Radiology Review Manual (Dahnert, Radiology Review Manual)

Authors: Dahnert, Wolfgang

Title: Radiology Review Manual, 6th Edition

Copyright 2007 Lippincott Williams & Wilkins

> Table of Contents > Heart and Great Vessels

Heart and Great Vessels

Differential Diagnosis of Cardiovascular Disorders

Congenital Heart Disease

Incidence of CHD in Liveborn Infants

• Most common CHD: mitral valve prolapse (5 20%), bicuspid aortic valve (2%) [usually not recognized before late infancy/childhood]

• ASD + VSD + PDA account for 45% of all CHD

• 12 lesions account for 89% of all CHD:

  Ventricular septal defect	30.3%
  Patent ductus arteriosus	8.6%
  Pulmonary stenosis	7.4%
  Septum secundum defect	6.7%
  Coarctation of aorta	5.7%
  Aortic stenosis	5.2%
  Tetralogy of Fallot	5.1%
  Transposition	4.7%
  Endocardial cushion defect	3.2%
  Hypoplastic right ventricle	2.2%
  Hypoplastic left heart	1.3%
  TAPVR	1.1%
  Truncus arteriosus	1.0%
  Single ventricle	0.3%
  Double outlet right ventricle	0.2%

P.580

Most common causes for CHF + PVH in neonate:

• Left ventricular failure due to outflow obstruction

• Obstruction of pulmonary venous return

CHD Presenting in 1st Year of Life

• VSD

• d-transposition of great vessels

• Tetralogy of Fallot

• Isolated coarctation

• Patent ductus arteriosus

• Hypoplastic left heart syndrome

CHD Compatible with Relatively Long Life

• Mild tetralogy: mild pulmonic stenosis + small VSD

• Valvular pulmonic stenosis: with relatively normal pulmonary circulation

• Transposition of great vessels: some degree of pulmonic stenosis + large VSD

• Truncus arteriosus: delicate balance between systemic + pulmonary circulation

• Truncus arteriosus type IV: large systemic collaterals

• Tricuspid atresia + transposition + pulmonic stenosis

• Eisenmenger complex

• Ebstein anomaly

• Corrected transposition without intracardiac shunt

Juxtaposition of Atrial Appendages

• Tricuspid atresia with transposition

• Complete transposition

• Corrected transposition of great arteries

• DORV

Continuous Heart Murmur

• PDA

• AP window

• Ruptured sinus of Valsalva aneurysm

• Hemitruncus

• Coronary arteriovenous fistula

Syndromes with CHD

5 p (Cri-du-chat) Syndrome

DiGeorge Syndrome

• = congenital absence of thymus + parathyroid glands

• 1. Conotruncal malformation

• 2. Interrupted aortic arch

Down Syndrome = MONGOLISM = TRISOMY 21

• Endocardial cushion defect (25%)

• Membranous VSD

• Ostium primum ASD

• AV communis

• Cleft mitral valve

• PDA

• 11 rib pairs (25%)

• Hypersegmented manubrium (90%)

Ellis-van Creveld Syndrome

• polydactyly

• single atrium

Holt-Oram Syndrome

• = UPPER LIMB-CARDIAC SYNDROME

• ASD

• VSD

• Valvular pulmonary stenosis

• Radial dysplasia

Hurler Syndrome

• Cardiomyopathy

Ivemark Syndrome

• asplenia

• complex cardiac anomalies

Klippel-Feil Syndrome

• Atrial septal defect

• Coarctation

Marfan Syndrome = ARACHNODACTYLY

• Aortic sinus dilatation

• Aortic aneurysm

• Aortic insufficiency

• Pulmonary aneurysm

Noonan Syndrome

• Pulmonary stenosis

• ASD

• Hypertrophic cardiomyopathy

Osteogenesis Imperfecta

• Aortic valve insufficiency

• Mitral valve insufficiency

• Pulmonic valve insufficiency

Postrubella Syndrome

• low birth weight

• deafness

P.581

• cataracts

• mental retardation

• 1. Peripheral pulmonic stenosis

• 2. Valvular pulmonic stenosis

• 3. Supravalvular aortic stenosis

• 4. PDA

Trisomy 13 15

• VSD, tetralogy of Fallot, DORV

Trisomy 16 18

• VSD, PDA, DORV

Turner Syndrome (XO) = OVARIAN DYSGENESIS

• Coarctation of the aorta (in 15%)

• Bicuspid aortic valve

• Dissecting aneurysm of aorta

Williams Syndrome = IDIOPATHIC HYPERCALCEMIA

• peculiar elfinlike facies

• mental + physical retardation

• hypercalcemia (not in all patients)

• 1. Supravalvular aortic stenosis (33%)

• 2. ASD, VSD

• 3. Valvular + peripheral pulmonary artery stenosis

• 4. Aortic hypoplasia, stenoses of more peripheral arteries

Shunt Evaluation

Evaluation of L-to-R Shunts

• AGE

  • Infants:

    • Isolated VSD

    • VSD with CoA/PDA/AV canal

    • PDA

    • Ostium primum

  • Children/adults:

    • ASD

    • Partial AV canal with competent mitral valve

    • VSD/PDA with high pulmonary resistance

    • PDA without murmur

• SEX

  • 99% chance for ASD/PDA in female patient

• CHEST WALL ANALYSIS

  • 11 pair of ribs + hypersegmented manubrium:

  • Down syndrome

  • pectus excavatum + straight back syndrome + funnel chest:

  • prolapsing mitral valve

  • rib notching

• CARDIAC SILHOUETTE

  • absent pulmonary trunk:

    • corrected transposition with VSD; pink tetralogy

  • left-sided ascending aorta:

    • corrected transposition with VSD

  • tortuous descending aorta:

    • aortic valve incompetence + ASD

  • huge heart:

    • persistent complete AV canal (PCAVC); VSD + PDA; VSD + mitral valve incompetence

  • enlarged left atrium:

    • intact atrial septum; mitral regurgitation (endocardial cushion defect, prolapsing mitral valve + ASD)

Shunt with Normal Left Atrium

• Precardiac shunt

  • Anomalous pulmonary venous connection

• Intracardiac shunt

  • ASD (8%)

  • VSD (25%)

• Postcardiac shunt

  • PDA (12%)

Aortic Size in Shunts

• Extracardiac shunts

  • aorta enlarged + hyperpulsatile

  • 1. PDA

• Pre- and intracardiac shunts

  • aorta small but not hypoplastic

  • 1. Anomalous pulmonary venous return

  • 2. ASD

  • 3. VSD

  • 4. Common AV canal

Cyanotic heart disease

• Decrease in hemoglobin delays detectability!

• Most common cause of cyanosis

Increased Pulmonary Blood Flow with Cyanosis

• = ADMIXTURE LESIONS

• = bidirectional shunt with 2 components:

  • mixing of saturated blood (L-R shunt) and unsaturated blood (R-L shunt)

  • NO obstruction to pulmonary blood flow

Evaluation process:

• cardiomegaly

• increased pulmonary blood flow

• concave main pulmonary artery:

  • PA segment absent = transposition

  • PA segment present:

    • L atrium normal (= extracardiac shunt) = TAPVR

    • L atrium enlarged (= intracardiac shunt) = truncus arteriosus

Admixture Lesions = T-lesions

• Transposition of great vessels = complete TGV VSD

  • Most common cause for cyanosis in neonate

P.582

• Tricuspid atresia with or without transposition + VSD

  • 2nd most common cause for cyanosis in neonate

• Truncus arteriosus

• Total anomalous pulmonary venous return (TAPVR) above diaphragm:

  • supracardiac

  • cardiac (coronary sinus/right atrium)

• Tingle = single ventricle

• Common atrium

• Aortic atresia

• Double-outlet right ventricle (DORV type I)/Taussig-Bing anomaly (DORV type II)

Clues:

• skeletal anomalies: Ellis-van Creveld syndrome (truncus/common atrium)

• polysplenia: common atrium

• R aortic arch: persistent truncus arteriosus

• ductus infundibulum: aortic atresia

• pulmonary trunk seen: supracardiac TAPVR; DORV; tricuspid atresia; common atrium

• ascending aorta with leftward convexity: single ventricle

• dilated azygos vein: common atrium + polysplenia + interrupted IVC; TAPVR to azygos vein

• left-sided SVC: vertical vein of TAPVR

• waterfall right hilum: single ventricle + transposition

• large left atrium (rules out TAPVR)

• prominent L heart border: single ventricle with inverted rudimentary R ventricle; levoposition of right atrial appendage (tricuspid atresia + transposition)

• age of onset 2 days: aortic atresia

Decreased Pulmonary Blood Flow with Cyanosis

• = two components of

  • impedance of blood flow through right heart due to obstruction/atresia at pulmonary valve/infundibulum

  • R-to-L shunt

• pulmonary circulation maintained through systemic arteries/PDA

• normal/decreased pulmonary blood flow

• concave main pulmonary artery

• cardiomegaly

• restrictive intracardiac R-to-L shunt

• Pulmonic stenosis with ASD

• Pulmonic atresia

• Tetralogy of Fallot

• Ebstein anomaly

• Tricuspid atresia with pulmonic stenosis

• Transposition of great vessels with pulmonic stenosis

• SHUNT AT VENTRICULAR LEVEL

  • 1. Tetralogy of Fallot

  • 2. Tetralogy physiology (associated with pulmonary obstruction):

    • complete/corrected transposition

    • single ventricle

    • DORV

    • tricuspid atresia (PS in 75%)

    • asplenia syndrome

  • prominent aorta with L/R aortic arch; inapparent pulmonary trunk

  • NORMAL R atrium (without tricuspid regurgitation)

  • NORMAL-sized heart (secondary to escape mechanism into aorta)

  Clues:

  • Skeletal anomaly (eg, scoliosis): tetralogy (90%)

  • Hepatic symmetry: asplenia

  • Right aortic arch: tetralogy, complete transposition, tricuspid atresia

  • Aberrant right subclavian artery: tetralogy

  • Leftward convexity of ascending aorta: single ventricle with inverted right rudimentary ventricle, corrected transposition, asplenia, JAA (tricuspid valve atresia)

• SHUNT AT ATRIAL LEVEL

  mnemonic:

  PET

  • Pulmonary stenosis/atresia with intact ventricular septum

  • Ebstein malformation + Uhl anomaly

  • Tricuspid atresia (ASD in 100%)

  • moderate to severe cardiomegaly

  • R atrial dilatation

  • R ventricular enlargement (secondary to massive tricuspid incompetence)

  • inapparent aorta

  • left aortic arch

Pulmonary Venous Hypertension with Cyanosis

• during 1st week of life

  • 1. Hypoplastic left heart syndrome

    • marked cardiomegaly

  • 2. TAPVR below diaphragm

    • normal cardiac size

• during 2nd week of life

  • 3. Aortic coarctation

  • 4. Aortic atresia

• during 4 6th week of life

  • 5. Critical aortic stenosis

  • 6. Endocardial fibroelastosis

  • 7. Anomalous origin of LCA

  • 8. Atresia of common pulmonary vein

Acyanotic heart disease

Increased Pulmonary Blood Flow without Cyanosis

• = indicates L-R shunt with increased pulmonary blood flow (shunt volume >40%)

• WITH LEFT ATRIAL ENLARGEMENT

  • indicates shunt distal to mitral valve = increased volume without escape defect

  • 1. VSD (25%): small aorta in intracardiac shunt

  • 2. PDA (12%): aorta + pulmonary artery of equal size in extracardiac shunt

  • 3. Ruptured sinus of Valsalva aneurysm (rare)

  • 4. Coronary arteriovenous fistula (very rare)

  • 5. Aortopulmonary window (extremely rare)

• WITH NORMAL LEFT ATRIUM

  • indicates shunt proximal to mitral valve = volume increased with escape mechanism through defect

  P.583

  • 1. ASD (8%)

  • 2. Partial anomalous pulmonary venous return (PAPVR) + sinus venosus ASD

  • 3. Endocardial cushion defect (ECD) (4%)

Normal Pulmonary Blood Flow without Cyanosis

• OBSTRUCTIVE LESION

  • Right ventricular outflow obstruction

    • at level of pulmonary valve: subvalvular/valvular/supravalvular pulmonic stenosis

    • at level of peripheral pulmonary arteries:

      • peripheral pulmonary stenosis

  • Left ventricular inflow obstruction

    • at level of peripheral pulmonary veins:

      • pulmonary vein stenosis/atresia

    • at level of left atrium: cor triatriatum

    • at level of mitral valve:

      • supravalvular mitral stenosis, congenital mitral stenosis/atresia, parachute mitral valve

  • Left ventricular outflow obstruction

    • at level of aortic valve:

      • anatomic subaortic stenosis, functional subaortic stenosis (IHSS), valvular aortic stenosis, hypoplastic left heart, supravalvular aortic stenosis

    • at level of aorta:

      • interruption of aortic arch, coarctation of aorta

• CARDIOMYOPATHY

  • Endocardial fibroelastosis

  • Hypertrophic cardiomyopathy

  • Glycogen storage disease

• hyperdynamic STATE

  • Noncardiac AVM (cerebral AVM, vein of Galen aneurysm, large pulmonary AVM, hemangioendothelioma of liver)

  • Thyrotoxicosis

  • Anemia

  • Pregnancy

• MYOCARDIAL ISCHEMIA

  • Anomalous left coronary artery

  • Coronary artery disease (CAD)

Pulmonary vascularity

Normal Pulmonary Vasculature

• Vascular Distribution

  • pulmonary vessels within upper perihilum approximate 1/3 of total vascularity

  • pulmonary vessels within lower perihilum approximate 2/3 of total vascularity

• Vascular Tapering

  • pulmonary vessels taper near transition of middle 1/3 to outer 1/3 of lung

• Vascular Caliber

  • straight/slightly concave main pulmonary artery contour (mild convexity is normal in young females)

  • CT:

    • upper limits of normal for main pulmonary artery = 3 cm

    • pulmonary trunk measures <4.5 cm (leftward distance from vertical line at carina to most lateral aspect of main pulmonary artery contour)

    • right interlobar/intermediate pulmonary artery measures 10 15 mm in males and 9 14 mm in females on PA radiographs

    • pulmonary vessel size <1 2 mm in extreme lung periphery

    • arteries within 1st anterior intercostal space measure 3 mm

Normal Pulmonary Vascularity & Normal-sized Heart

• Myocardial ischemia

• Afterload (= pressure overload problems)

• Normal

Increased Pulmonary Vasculature

• OVERCIRCULATION

  • = shunt vascularity = arterial + venous overcirculation

  • (a) congenital heart disease (most common)

    • L-R shunts

    • Admixture cyanotic lesions

  • (b) high-flow syndromes

    • Thyrotoxicosis

    • Anemia

    • Pregnancy

    • Peripheral arteriovenous fistula

  • diameter of right descending pulmonary artery larger than trachea just above aortic knob

  • increased size of veins + arteries with size larger than accompanying bronchus (= kissing cousin sign), best seen just above hila on AP view

  • enlarged hilar vessels (lateral view)

  • visualization of vessels below 10th posterior rib

• PULMONARY VENOUS HYPERTENSION

  • redistribution of flow (not seen in younger children)

  • indistinctness of vessels with Kerley lines (= interstitial edema)

  • fine reticulated pattern

  • alveolar edema

• PRECAPILLARY HYPERTENSION

  • enlarged main + right and left pulmonary arteries

  • abrupt tapering of pulmonary arteries

• PROMINENT SYSTEMIC/AORTOPULMONARY COLLATERALS

  • Tetralogy of Fallot with pulmonary atresia (= pseudotruncus)

  • VSD + pulmonary atresia (single ventricle, complete transposition, corrected transposition)

  • Pulmonary-systemic collaterals

  • coarse vascular pattern with irregular branching arteries (from aorta/subclavian arteries)

  • small central vessels despite apparent increase in vascularity

Decreased Pulmonary Vascularity

• = obstruction to pulmonary flow

• vessels reduced in size and number

• hyperlucent lungs

• small pulmonary artery segment + hilar vessels

P.584

Pulmonary artery

Invisible Main Pulmonary Artery

• Underdeveloped = RVOT obstruction

  • Tetralogy of Fallot

  • Hypoplastic right heart syndrome (tricuspid/pulmonary atresia)

• Misplaced pulmonary artery

  • Complete transposition of great vessels

  • Persistent truncus arteriosus

Decreased Diameter of Pulmonary Artery

• Lung cancer

  • Tumor extending >180 of arterial circumference of main pulmonary artery indicates unresectability!

• Mediastinal fibrosis

• Takayasu arteritis

• Chronic thromboembolic disease

Unequal Pulmonary Blood Flow

• Tetralogy of Fallot

  • diminished flow on left side (hypoplastic/stenotic pulmonary artery in 40%)

• Persistent truncus arteriosus (esp. type IV)

  • diminished/increased blood flow to either lung

• Pulmonary valvular stenosis

  • increased flow to left lung secondary to jet phenomenon

Dilatation of Pulmonary Artery

• Idiopathic dilatation of pulmonary trunk

• Pulmonic valve stenosis

  • poststenotic dilatation of trunk + left pulmonary a.

• Pulmonary regurgitation

  • severe pulmonic valve insufficiency

  • absence of pulmonic valve (may be associated with tetralogy)

• Congenital L-to-R shunts

• Pulmonary arterial hypertension

• Aneurysm: mycotic/traumatic

• Intravascular pulmonary metastases

Filling Defect in Pulmonary Artery

• Thromboembolism (99%)

• Nonthrombotic embolus:

  • fat droplets, bubbles of air/nitrogen, tumor, foreign bodies (talc, polymethylmethacrylate particles)

  • Particles mostly too small to be visualized

• Primary sarcoma

Pulmaonary Hypertension

• = sustained pulmonary arterial pressure in systole >25 mm Hg at rest/ >30 mm Hg during exercise (normal levels, 10 and 15 mm Hg) determined by right heart catheterization secondary to reduction in cross-sectional area of the pulmonary vascular bed with concomitant increase in pulmonary vascular resistance

• often not clinically recognized until advanced stage

CT:

• vascular signs:

  • main pulmonary artery diameter >29 mm (87% sensitive, 89% specific) measured in the plane of PA bifurcation just lateral to ascending aorta

  • segmental artery-to-bronchus ratio >1 in three lobes

  • diameter ratio of main pulmonary artery to aorta >1 (strong correlation in patients <50 years)

  • diameter of left + right pulmonary artery >16 mm (poor indicator of pulmonary arterial hypertension)

  • pruning of peripheral pulmonary arteries

    • = disproportionate increase in caliber of central fibrous arteries (from sustained increase in flow by a factor of >2) + decrease in caliber of smaller muscular arteries (from vasoconstriction)

  • pulmonary veins

    • small: secondary to precapillary pulmonary HTN

    • enlarged: secondary to left-sided heart disease

  • enlargement of bronchial systemic arteries to >1.5 mm (more common in chronic thromboembolic pulmonary HTN than primary pulmonary HTN)

  • vascular complications:

    • subpleural pulmonary infarcts (with elevated pulmonary venous pressure/underlying malignancy)

    • calcified plaques of central pulmonary arteries (PATHOGNOMONIC)

    • dissection/massive thrombosis of central pulmonary arteries

• lung parenchymal signs:

  • mosaic perfusion without dilatation of bronchi (= increase in vessel diameter in areas of hyperattenuation + tapering of peripheral vessels in areas of hypoattenuation); most common in chronic pulmonary thromboembolism

• mediastinal & cardiac signs:

  • right heart enlargement + hypertrophy:

    • right heart dilatation = ratio of RV:LV > 1:1

    P.585

    • bowing of interventricular septum toward LV

    • RV myocardial thickness >4 mm

  • dilatation of IVC + coronary sinus

  • reflux of contrast medium into IVC + hepatic veins (usefulness diminishes with injection rate >3 mL/sec)

  • mild pericardial thickening/small pleural effusion

  • adenopathy + septal lines + ground-glass opacities suggestive of pulmonary veno-occlusive disease

  • NO increase of pulsations in middle third of lung

MR:

• RV hypertrophy (in almost 100% if RV systolic pressure exceeds 70 mm Hg):

  • mean thickness of RV wall = 6 mm

• RV dilatation:

  • mean RV end-diastolic volume = 80 mL

  • tricuspid regurgitation (from dilatation of tricuspid valve annulus)

• enlargement of RA + IVC + SVC

• septum straight/bowed toward LV

• diminished RV ejection fraction (mean of 45%)

• reduced distensibility of main pulmonary artery (mean of 8%)

• systolic intraluminal signals in pulmonary arteries (due to slow flow) correlating with severity of PAH

Pulmonary Arterial Hypertension (PAH)

Classification by anatomic level:

• Precapillary cause

  • = changes limited to pulmonary arterial circulation @ level of muscular arteries

  • 1. Chronic thromboembolic disease

  • 2. In-situ pulmonary arterial thrombotic disease (polycythemia, sickle-cell disease)

  • 3. Widespread pulmonary embolism from intra-vascular malignant cells/parasites/foreign material

  • 4. Pulmonary vasculitis (eg, collagen vascular disease, especially scleroderma, CREST, HIV)

  • 5. Long-standing L-to-R shunt = Eisenmenger syndrome with reversal of L-to-R shunt

  • 6. Alveolar hypoventilation syndromes

  • 7. Primary pulmonary hypertension (idiopathic)

• Pulmonary parenchymal disease

  • = mechanical interference with small pulmonary arteries (classified under precapillary cause)

  • Hypoxic lung disease:

    • Chronic obstructive pulmonary disease

    • Interstitial pulmonary fibrosis

    • Ventilatory failure from primary chest wall dysfunction

  • Connective tissue disorder:

    • Scleroderma

    • CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, telangiectasia)

  • Infection:

    • HIV (6 12 compared to general population)

    • Sarcoidosis

    • Tuberculosis

• Postcapillary cause

  • = Pulmonary venous hypertension

  • = findings located in pulmonary venous circulation + between capillary bed and left atrium

  • Left atrial obstruction (usually MV disease, left heart failure, left atrial tumor)

  • Mediastinal fibrosis (narrowing of pulmonary veins), may also affect precapillary vessels

  • Pulmonary veno-occlusive disease (idiopathic)

Pathogenesis:

• hyperkinetic CAUSES

  • L-to-R shunt

  • High cardiac output states: thyrotoxicosis, chronic anemia

• OBLITERATIVE CAUSES

  • vascular = precapillary pulmonary hypertension:

    • Primary plexogenic pulmonary arteriopathy = Primary pulmonary hypertension (PPH)

    • Arteritis (eg, Takayasu)

    • Embolization

      • chronic thromboembolic disease

      • tumor

        • lymphangitic carcinomatosis

      • parasites, eg, schistosomiasis

        • hepatosplenomegaly

      • talc crystals

        • micronodular opacities

        • perihilar fibrotic masses

    • Persistent fetal circulation

    • Pulmonary capillary hemangiomatosis

  • pleuropulmonic disease

    • Chronic interstitial lung disease

      • = cor pulmonale:

      • COPD, emphysema, chronic bronchitis, asthma, bronchiectasis, malignant infiltrate, granulomatous disease, cystic fibrosis, end-stage fibrotic lung, S/P lung resection, idiopathic hemosiderosis, alveolar proteinosis, alveolar microlithiasis

    • Pleural disease + chest deformity: fibrothorax, thoracoplasty, kyphoscoliosis

  • vasoconstrictive

    • Chronic alveolar hypoxia

      • = hypoxic pulmonary arterial hyperperfusion:

      • chronic high altitude, sleep apnea, chronic hypercapnea due to hypoventilation from neuromuscular disease/obesity

    • Portopulmonary hypertension (rare)

• Chronic pulmonary venous hypertension

Cor Pulmonale

• Thoracic deformity

• Idiopathic: primary pulmonary hypertension (1%)

• Chronic pulmonary embolism

• COPD

P.586

• Shunt (ASD, VSD, etc)

• Bronchiectasis

• Emphysema

• Vasculitis

Pulmonary Venous Hypertension (PVH)

• = INCREASED VENOUS PULMONARY PRESSURE = VENOUS CONGESTION

• = postcapillary pulmonary hypertension = primary findings located within pulmonary venous circulation between capillary bed + left atrium

Cause:

• LEFT VENTRICULAR INFLOW TRACT OBSTRUCTION

  • normal-sized heart with right ventricular hypertrophy

  • prominent pulmonary trunk

  • @ proximal to mitral valve:

    • normal-sized left atrium

    • pulmonary veins

      • TAPVR below the diaphragm

      • Primary pulmonary venoocclusive disease (PVOD)

      • Stenosis of individual pulmonary veins

      • Atresia of common pulmonary vein

    • mediastinum

      • Fibrosing mediastinitis (may also affect precapillary vessels)

      • Constrictive pericarditis

    • left atrium

      • Cor triatriatum

      • Left atrial mass: tumor, clot

      • Supravalvular ring of left atrium

  • @ at mitral valve level = mitral valve stenosis

    • enlarged left atrium

    • 1. Rheumatic mitral valve stenosis regurgitation (99%)

    • enlarged left atrial appendage

    • 2. Congenital mitral valve stenosis

    • 3. Parachute mitral valve (= single bulky papillary muscle)

• LEFT VENTRICULAR FAILURE

  • ABNORMAL PRELOAD with secondary mitral valve incompetence (= volume overload)

    • Aortic valve regurgitation

    • Eisenmenger syndrome (= R-to-L shunt in VSD)

    • High-output failure:

      • noncardiac AVM (cerebral AVM, vein of Galen aneurysm, large pulmonary AVM, hemangioendothelioma of liver, iatrogenic), thyrotoxicosis, anemia, pregnancy

  • ABNORMAL afterload

    • (= pressure overload)

    • = LV outflow tract obstruction

    • Hypoplastic left heart syndrome

    • Aortic stenosis (supravalvular, valvular, anatomic subaortic)

    • Interrupted aortic arch

    • Coarctation of the aorta

  • DISORDERS OF CONTRACTION AND RELAXATION

    • Endocardial fibroelastosis

    • Glycogen storage disease (Pompe disease)

    • Cardiac aneurysm

    • Cardiomyopathy

      • congestive (alcohol)

      • hypertrophic obstructive cardiomyopathy (HOCM), particularly in IDM

        • asymmetric septal hypertrophy (ASH)

        • idiopathic hypertrophic subaortic stenosis (IHSS)

  • MYOCARDIAL ISCHEMIA

    • Anomalous left coronary artery

    • Coronary artery disease (CAD)

Histo:

• primary changes: venous medial hypertrophy + intimal proliferation, marked thickening of venous internal elastic lamina

• secondary changes: capillary bed congestion with adjacent vascular proliferation, interlobular septal and pleural edema + fibrosis, lymphatic dilatation, alveolar hemosiderosis, paraseptal venous infarcts adjacent to complete venous occlusion

• equalization of pulmonary vascularity (PCWP 13 15 mm Hg)

• cephalization of pulmonary vascularity (PCWP 16 18 mm Hg)

• interstitial pulmonary edema (PCWP 19 24 mm Hg)

  • = fluid within peribronchovascular connective tissue:

  • peribronchial thickening/cuffing

  • indistinct vessel margins

  • Kerley B lines = short horizontal reticulations within lateral subpleural lung bases

  • Kerley A lines = 3 4-cm-long lines of interlobular septal thickening radiating from hila to mid and upper lung zones

  • perihilar haze = hilar interstitial edema

  • thickened pleural fissures/pseudoeffusion = fluid within subpleural connective tissue

• alveolar pulmonary edema (PCWP 25 mm Hg) = bilateral perihilar and basilar airspace opacification

• small pleural effusions

Cardiomegaly

• CHF

• Multivalvular disease

• Pericardial effusion

  • absence of pulmonary venous hypertension + hydrostatic edema

Cardiothoracic Ratio

• = widest transverse cardiac diameter widest inside thoracic diameter

• = primarily a measure of LV dilatation

P.587

Vascular Pedicle Width

• = distance on a horizontal line between (1) point where right mainstem bronchus + SVC cross and (2) point where left subclavian artery crosses horizontal line

Cardiomegaly in Newborn

• NONCARDIOGENIC

  • metabolic:

    • Ion imbalance in serum levels of sodium, potassium, and calcium

    • Hypoglycemia

  • decreased ventilation

    • Asphyxia

    • Transient tachypnea

    • Perinatal brain damage

  • erythrocyte function

    • Anemia

    • Erythrocythemia

  • endocrine

    • Glycogen storage disease

    • Thyroid disease: hypo-/hyperthyroidism

  • infant of diabetic mother

  • arteriovenous fistula

    • Vein of Galen aneurysm

    • Hepatic angioma

    • Chorioangioma

• CARDIOGENIC

  • Arrhythmia

  • Myo-/pericarditis

  • Cardiac tumor

  • Myocardial infarction

  • Congenital heart disease

Abnormal Heart Chamber Dimensions

• LEFT VENTRICULAR VOLUME OVERLOAD

  • VSD

  • PDA

  • Mitral incompetence

  • Aortic incompetence

• LEFT VENTRICULAR HYPERTROPHY

  • Coarctation

  • Aortic stenosis

• RIGHT VENTRICULAR VOLUME OVERLOAD

  • ASD

  • Partial APVR/total APVR

  • Tricuspid insufficiency

  • Pulmonary insufficiency

  • Congenital/acquired absence of pericardium

  • [Ebstein anomaly] not truly RV

• RIGHT VENTRICULAR HYPERTROPHY

  • Pulmonary valve stenosis

  • Pulmonary hypertension

  • Tetralogy of Fallot

  • VSD

• Fixed subvalvular aortic stenosis

• Hypoplastic left/right ventricle, common ventricle

• Congestive cardiomyopathy

Right Atrial Enlargement

• RA enlarges in rightward + posterior direction

PA CXR:

• prominent round superior border at junction with SVC

• >5.5 cm from midline to most lateral RA margin

• >2.5 cm from right vertebral margin

• >50% vertical height of RA compared with cardiovascular mediastinal height (from top of aortic arch to base of heart)

LAT CXR:

• sharp horizontal interface with lung above RV

• displacement of heart posterior to IVC mimicking LV enlargement

Right Ventricular Enlargement

• RV enlarges in anterior, superior + leftward direction causing levorotation of heart

PA CXR:

• Only extreme dilatation causes recognizable signs on frontal view!

• straightening/convexity of left upper cardiac contour

• upturned cardiac apex

• left upper cardiac margin parallels left mainstembronchus as a long convex curvature

• increased distance between left upper cardiac margin + left mainstem bronchus

• small appearance of rotated aortic arch + SVC

• large appearance of main pulmonary artery

LAT CXR:

• prominent convexity of anterior heart border >1/3 distance from anterior cardiophrenic sulcus to sternal angle

Left Atrial Enlargement

Cause:

• acquired: mitral stenosis/regurgitation, LV failure, LA myxoma

• congenital: VSD, PDA, hypoplastic left heart

• LA enlarges in multiple directions

PA CXR:

• right retrocardiac double density with inferomedial curvature (earliest sign)

• >7.0 (female)/7.5 (male) cm distance between midpoint of undersurface of left mainstem bronchus + right lateral LA shadow

• left retrocardiac double density

• >75 splaying of carina with horizontal orientation of distal left mainstem bronchus

P.588

• enlarged left-convex left atrial appendage calcifications (in 90% due to rheumatic heart disease)

LAT CXR:

• increased convexity of posterosuperior cardiac margin

• posterosuperior atrial convexity crosses vertical plane formed by tracheal midline + upper lobe bronchus

• posterior displacement of barium-filled esophagus

• posterior displacement of LUL bronchus

Left Ventricular Enlargement

Cause:

• pressure overload: hypertension, aortic stenosis

• volume overload: aortic or mitral regurgitation, VSD

• wall abnormalities: LV aneurysm, hypertrophic cardiomyopathy

• LV enlarges in posterior, inferior + leftward direction

PA CXR:

• leftward displacement of downturned cardiac apex = left ventricular configuration

• depression of left hemidiaphragm + gastric bubble (with diaphragmatic inversion)

LAT CXR:

• increased convexity of posteroinferior cardiac margin

• posterior cardiac margin projects >1.8 cm posterior to IVC measured at a point 2 cm above intersection of IVC with right hemidiaphragm (Hofman-Rigler rule)

Neonatal cardiac failure

• LEFT-SIDED OBSTRUCTIVE LESIONS

  • Segmental hypoplasia of aorta

  • Critical coarctation of the aorta

  • Aortic valve stenosis

  • Asymmetrical septal hypertrophy/hypertrophic obstructive cardiomyopathy

  • Mitral valve stenosis

  • Cor triatriatum

• VOLUME OVERLOAD

  • Congenital mitral valve incompetence

  • Corrected transposition with left (= tricuspid) AV valve incompetence

  • Congenital tricuspid insufficiency

  • Ostium primum ASD

• MYOCARDIAL DYSFUNCTION/ISCHEMIA

  • Nonobstructive cardiomyopathy

  • Anomalous origin of LCA from pulmonary trunk

  • Primary endocardial fibroelastosis

  • Glycogen storage disease (Pompe disease)

  • Myocarditis

• NONCARDIAC LESIONS

  • AV fistulas: hemangioendothelioma of liver, AV fistula of brain, vein of Galen aneurysm, large pulmonary AV fistula

  • Transient tachypnea of the newborn

  • Intraventricular/subarachnoid hemorrhage

  • Neonatal hypoglycemia (low birth weight, infants of diabetic mothers)

  • Thyrotoxicosis (transplacental passage of LATS hormone)

Congestive Heart Failure & Cardiomegaly

• Myocardial infarction

• anemia

• Malformation

• cardiomyopathy

• Coronary artery disease

• aortic insufficiency

• effusion

• Coarctation

Congenital Cardiomyopathy

• Cystic medial necrosis of coronary arteries

• Aberrant left coronary artery/Absent coronary a.

• Viral myocarditis

P.589

• Endocardial fibroelastosis

• Glycogen storage disease (Pompe)

• Infant of diabetic mother/Ischemia

Acquired heart disease

• LA enlargement = MV disease

• dilated ascending aorta = aortic valve disease

• RA enlargement = tricuspid valve disease

Pressure Overload

• Systemic hypertension

• Aortic stenosis

• Mitral stenosis

Decreased Compliance

• Myocardial infarction

• Hypertrophic cardiomyopathy

• Restrictive cardiomyopathy

Volume Overload

• Aortic insufficiency

• Mitral insufficiency

• Tricuspid insufficiency

Aorta

Enlarged Aorta

• PA CXR:

  • aortic knob >4.0 cm measured from indented trachea to most lateral margin of aorta

  • right convex contour above RA margin + lateral displacement of SVC (= dilatation of ascending aorta)

• INCREASED VOLUME LOAD

  • Aortic insufficiency

  • PDA

• POSTSTENOTIC DILATATION

  • Valvular aortic stenosis

• INCREASED INTRALUMINAL PRESSURE

  • Coarctation

  • Systemic hypertension

• MURAL WEAKNESS/INFECTION

  • Cystic media necrosis: Marfan/Ehlers-Danlos syndrome

  • Congenital aneurysm

  • Syphilitic aortitis

  • Mycotic aneurysm

  • Atherosclerotic aneurysm (compromised vasa vasorum)

• LACERATION OF AORTIC WALL

  • Traumatic aneurysm

  • Dissecting hematoma

Aortic Wall Thickening

• Intramural hematoma

  • = aortic dissection without intimal tear

• Aortitis

  • segments of aortic arch + branch vessels

• Atherosclerotic plaque

  • irregular narrowing of aortic lumen

• Adherent thrombus

Aortic Calcifications

Intimal Calcification

• discrete punctate lesion of radiograph

Medial Calcification

• linear deposit along elastic lamellae resembling railroad tracks (when severe)

Double Aortic Arch

• Most common + serious type of a complete vascular ring; usually isolated condition

P.590

• usually asymptomatic

• stridor, dyspnea, recurrent pneumonia

• dysphagia (less common than respiratory symptoms, more common after starting baby on solids)

• two separate arches arise from single ascending aorta

• each arch joins to form a common descending aorta

• trachea in midline:

  • impressions may be present on both sides of trachea: usually R > L (in older children)

  • trachea narrowed and displaced posteriorly with small anterior impression

• Esophagogram:

  • broad horizontal posterior indentation at the level of 3rd/4th thoracic vertebra (by right arch crossing obliquely to join left arch)

  • bilateral esophageal indentations with a reversed S-shaped configuration (= right indentation higher than left)

• CT:

  • four-artery sign = each arch gives rise to 2 dorsal subclavian + 2 ventral carotid arteries evenly spaced around trachea on section cephalad to aortic arch

  DDx:	right arch with aberrant left subclavian artery (indistinguishable by esophagogram when dominant arch on right side)

Right Aortic Arch

Right Aortic Arch

P.591

Incidence of right aortic arch in CHD:

Rare anomalies:

• Corrected transposition

• Pseudotruncus

• Asplenia

• Pink tetralogy

Right Aortic Arch with Aberrant Left Subclavian Artery

• = RAA with ALSA

• = interruption of embryonic left arch between left CCA and left subclavian artery

• Most common type of right aortic arch anomaly

• 2nd most common cause of vascular ring after double aortic arch

• usually asymptomatic (loose ring around trachea + esophagus)

• may be symptomatic in infancy/early childhood provoked by bronchitis + tracheal edema

• may be symptomatic in adulthood provoked by torsion of aorta

• left common carotid artery is first branch of ascending aorta

• left subclavian artery arises from descending aorta via the remnant of the left dorsal aortic root

• bulbous configuration of origin of LSA (= remnant of embryonic left arch) = retroesophageal aortic diverticulum = diverticulum of Kommerell (N.B.: originally described as diverticular outpouching at origin of right subclavian artery with left aortic arch):

  • small rounded density left lateral to trachea

  • impression on left side of esophagus simulating a double aortic arch (by aortic diverticulum or ductus/ligamentum arteriosum)

• vascular ring (= left ductus extends from aortic diverticulum to left pulmonary artery):

  • impression on tracheal air shadow (by right aortic arch)

  • right esophageal indentation (by right aortic arch)

  • masslike density silhouetting top of aortic arch just posterior to trachea on LAT CXR (by aberrant left subclavian artery)

  • broad posterior impression on esophagus (left subclavian artery/aortic diverticulum)

  • small anterior impression on trachea (by left common carotid artery)

  • descending aorta on right side

Right Aortic Arch with Mirror-image Branching

• 2nd most common aortic arch anomaly: 24 60%

• = interruption of embryonic left arch between left subclavian artery and descending aorta; dorsal to left ductus arteriosus

Interruption of Left Aortic Arch Distal to Ductus Arteriosus = Type 1 (common)

• 25% of patients with tetralogy have right aortic arch!

• 37% of patients with truncus arteriosus have right aortic arch!

• NO vascular ring, NO retroesophageal component

• NO structure posterior to trachea

• R arch impression on tracheal air shadow

• NORMAL barium swallow

Interruption of Left Aortic Arch Proximal to Ductus Arteriosus = Type 2 (rare)

• = true vascular ring (if duct persists); rarely associated with CHD

Right Aortic Arch with Isolated Left Subclavian Artery

• 3rd most common right aortic arch anomaly: 2%

• = interruption of embryonic left arch between

  • left CCA and left subclavian artery and

  • left ductus and descending aorta

  • resulting in a connection of left subclavian artery with left pulmonary artery

• left common carotid artery arises as the first branch

• left subclavian artery attaches to left pulmonary artery through PDA

• NO vascular ring, NO retroesophageal component

• congenital subclavian steal syndrome

Right Aortic Arch with Aberrant Left Brachiocephalic Artery

• Similar in appearance to R aortic arch + aberrant L subclavian artery

Left Aortic Arch

Left Aortic Arch with Aberrant Right Subclavian Artery

• = right subclavian artery arises as 4th branch from proximal descending aorta

P.592

• asymptomatic/dysphagia lusoria (rare)

• soft-tissue opacity crossing the esophagus obliquely upward toward the right shoulder (PATHOGNOMONIC)

• masslike opacity in right paratracheal region

• rounded opacity arising from superior aortic margin posterior to trachea + esophagus on LAT CXR

• dilated origin of aberrant right subclavian artery (in up to 60%) = diverticulum of Kommerell = remnant of embryonic right arch

• unilateral L-sided rib notching (if aberrant right subclavian artery arises distal to coarctation)

Anomalous Innominate Artery Compression Syndrome

• = origin of R innominate artery to the left of trachea coursing to the right

• anterior tracheal compression

• ablation of right radial pulse by rigid endoscopic pressure

• posterior tracheal displacement

• focal collapse of trachea at fluoroscopy

• pulsatile indentation of anterior tracheal wall by innominate artery on MRI

Vascular rings

• = anomaly characterized by encirclement of trachea + esophagus by aortic arch + branches

• Usually symptomatic lesions

  • chronic stridor, wheezing, recurrent pneumonia

  • dysphagia, failure to thrive

  • Double aortic arch with R descending aorta + L ductus arteriosus

  • R aortic arch with R descending aorta + aberrant L subclavian artery + persistent L ductus/ligamentum arteriosum

    N.B.:

    left obliterated ductus arteriosus (= ligament of Botallo) passes from L pulmonary a. to descending aorta/L subclavian a.

    • symptoms + radiographic findings identical to double aortic arch

    P.593

    • indentation on right lateral esophageal wall (by aortic arch)

    • impression on the anterolateral esophageal wall (by ligament of Botallo)

    • origin of L subclavian artery frequently dilated

  • L arch with L descending aorta + R ductus/ligamentum

  • Aberrant L pulmonary artery = pulmonary sling

  • Frequency of CXR findings:

    • frontal CXR:

      • right aortic arch (85%)

      • focal indentation of distal trachea (73%)

    • lateral CXR:

      • anterior tracheal bowing (92%)

      • increased retrotracheal opacity (79%)

      • focal tracheal narrowing (77%)

• OCCASIONALLY SYMPTOMATIC LESIONS

  • Anomalous R innominate artery

  • Anomalous L common carotid artery/common trunk

  • R aortic arch with L descending aorta + L ductus/ligamentum

• USUALLY ASYMPTOMATIC LESIONS

  • L aortic arch + aberrant R subclavian artery

  • L aortic arch with R descending aorta

  • R aortic arch with R descending aorta + mirror-image branching

  • R aortic arch with R descending aorta + aberrant L subclavian artery

  • R aortic arch with R descending aorta + isolation of L subclavian artery

  • R aortic arch with L descending aorta + L ductus/ligamentum

Aortic stenosis

• ACQUIRED

  • Takayasu aortitis

  • Radiation aortitis

  • Aortic dissection

  • Infected aortic aneurysm with abscess

  • Pseudoaneurysm from laceration

  • Atherosclerosis (rare)

  • Syphilitic aortitis (rare)

• CONGENITAL

  • Williams syndrome

  • Neurofibromatosis

  • Rubella

  • Mucopolysaccharidosis

  • Hypoplastic left heart syndrome

Abnormal Left Ventricular Outflow Tract

• LVOT = area between IVS + aML from aortic valve cusps to mitral valve leaflets

• Membranous subaortic stenosis

  • = crescent-shaped fibrous membrane extending across LVOT + inserting at aML

  • diffuse narrowing of LVOT

  • abnormal linear echoes in LVOT space (occasionally)

• Prolapsing aortic valve vegetation

• Narrowed LVOT (<20 mm)

  • Long-segment subaortic stenosis

    • aortic valve closure in early systole with coarse fluttering

    • high-frequency flutter of mitral valve in diastole (aortic regurgitation)

    • symmetric LV hypertrophy

  • ASH/IHSS

    • asymmetrically thickened septum bulging into LV + LVOT

    • systolic anterior motion of aML (SAM)

  • Mitral stenosis

  • Endocardial cushion defect

Situs

• = position/site/location referring to the position of the atria and viscera relative to the midline

  • systemic/right atrium

    • has a broad-based appendage

    • receives blood from IVC

    • has terminal crest + coarse pectinate muscles

  • pulmonary/left atrium

    • has a small narrow appendage

    • receives blood from pulmonary veins

• SITUS SOLITUS = normal/usual situs

  on right side:	systemic atrium trilobed lung liver gallbladder IVC
  on left side:	pulmonary atrium bilobed lung stomach single spleen aorta

  Associated with:

  (a) levocardia	: 0.6 0.8% chance for CHD
  (b) dextrocardia	: 95% chance for CHD

• SITUS INVERSUS

  • = mirror-image arrangement of situs solitus

  Frequency:	0.01%
  on left side:	systemic atrium trilobed lung liver gallbladder IVC
  on right side:	pulmonary atrium bilobed lung stomach single spleen aorta

  Associated with:

  • dextrocardia = situs inversus totalis (usual variant): 3 5% chance for CHD, eg, Kartagener syndrome (in 20%)

  • levocardia (extremely rare): 95% chance for CHD

• SITUS AMBIGUUS = HETEROTAXIA

  • = visceral malposition + dysmorphism associated with indeterminate atrial arrangement

  • Subclassification:

    • Asplenia syndrome = double right-sidedness = right isomerism = Ivemark syndrome

      P.594

      • both lungs have 3 lobes

      • eparterial bronchi = main bronchus passes superior to ipsilateral main pulmonary artery

    • Polysplenia syndrome = double left-sidedness = left isomerism

      • both lungs have 2 lobes

      • hyparterial bronchi = main bronchus passes inferior to ipsilateral main pulmonary artery

Cardiac Malposition

• location of heart other than within left hemothorax in situs solitus

• location of heart within left hemithorax when other organs are ambiguous

• Determined by base-apex axis; no assumption is made regarding cardiac chamber/vessel arrangement

• POSITION OF CARDIAC APEX

  • Levocardia = apex directed leftward

  • Dextrocardia = apex directed rightward

  • Mesocardia = vertical/midline heart (usually with situs solitus)

  • atrial septum characteristically bowed into left atrium in cardiac situs solitus with dextrocardia + cardiac situs inversus with levocardia (DDx: juxtapositioned atrial appendages)

• CARDIAC DISPLACEMENT

  • by extracardiac factors (eg, lung hypoplasia, pulmonary mass)

  • Dextroposition

    • suggests hypoplasia of ipsilateral pulmonary artery (PAPVR implies scimitar syndrome)

  • Levoposition

  • Mesoposition

• CARDIAC INVERSION

  • alteration of normal relationship of chambers

  • D-bulboventricular loop

  • L-bulboventricular loop

• TRANSPOSITION

  • = alteration of anterior-posterior relationship of great vessels

Cardiac tumor

• Rare often asymptomatic tumors until very large!

• symptoms of cardiopulmonary diseases:

  • congestive heart failure: dyspnea, orthopnea, peripheral edema, paroxysmal nocturnal dyspnea

  • palpitations, heart murmur

  • cough, chest pain

• symptoms caused by peripheral emboli to cerebral/systemic/coronary circulation:

  • syncope

• weight loss, fever, malaise

CXR:

• cardiomegaly, pericardial effusion

• signs of CHF

• abnormal cardiac contour

• pleural effusion

Benign Heart Tumor in Adults

• More common than malignant neoplasms

• Myxoma (most common primary cardiac tumor; 50% of all primary cardiac tumors)

• Papillary fibroelastoma (10% of all primary cardiac tumors; most common valvular tumor)

• Lipoma

• Hydatid cyst (uncommon):

  • localized bulge of left cardiac contour

  • curvilinear/spotty calcifications (resembling myocardial aneurysm)

Malignant Heart Tumors

• Sarcoma:

  • Majority of primary malignant cardiac neoplasms!

  • 2nd most common primary cardiac neoplasm

• Rhabdomyosarcoma

  • Most common primary in children

P.595

• Metastatic disease

  • 20 1000 times more frequent than primary tumor!

    • to peri-/epicardium lung > breast > lymphoma > leukemia (most common)

    • myocardium: malignant melanoma; secondary extension to myocardium from epicardium

    • endocardial/intracavitary (in only 5%)

• Lymphoma (rare)

• Malignant teratoma

• Multiple cardiac myxomas

Congenital Cardiac Tumor

• Rhabdomyoma (58%): usually multiple masses

• Teratoma (20%): intrapericardiac, extracardiac

  • multicystic mass

• Fibroma (12%): intramural

  May be associated with:	Gorlin syndrome
  Location:	free LV wall/interventricular septum

  • may be pedunculated

  • calcification and cystic degeneration centrally

  • tendency for slow growth

  Cx:	fetal hydrops secondary to obstruction, pericardial effusion, fetal arrhythmia, fetal death

• Hemangioma (arise from R atrium, pericardial effusion, skin hemangiomas), lymphangioma, neurofibroma, myxoma, mesothelioma:

  • mass-occupying lesion impinging upon cardiac cavities

Cardiac Tumor by Location

• ENDOCARDIAL/INTRACAVITARY

  • Myxoma

  • Thrombus

  • Myofibroblastic sarcomas (MFH, leiomyosarcoma, fibrosarcoma, myxosarcoma)

• VALVULAR

  • Papillary fibroelastoma

  • Vegetations

  • Thrombus

  • Myxoma

• MYOCARDIAL/INTRAMURAL

  • Rhabdomyoma: multifocal, high signal on T2WI

  • Fibroma: always solitary, calcifications, cystic degeneration, low signal on T2WI

• ENDO-/MYO-/EPICARDIAL

  • Lipoma

  • Sarcoma

  • Primary cardiac lymphoma: right heart, multifocal, extension into pericardium

Cardiac calcifications

Detected by:

• fluoroscopy (at low-beam energies 75 kVp is 57% sensitive)

• < digital subtraction fluoroscopy

• < conventional CT < ultrafast CT (96% sensitive)

• @ Coronary arteries (see below)

• @ Cardiac valves

  • Valvar calcification means stenosis its amount is proportionate to degree and duration of stenosis!

• Aortic valve

  • Valve calcium best seen on lateral view!

  • good correlation between amount of calcium and degree of stenosis:

    • heavy calcification = significant stenosis

    • no calcification = aortic stenosis unlikely

  Cause:	congenital bicuspid valve (70 85%) > atherosclerotic degeneration > rheumatic aortic stenosis (rare), syphilis, ankylosing spondylitis
  Location:	above + anterior to a line connecting carina + anterior costophrenic angle (lateral view)

  • Congenital bicuspid aortic valvular stenosis

    • calcium first detected at an average age of 28 years

    • In patients <30 years aortic valve calcifications are mostly due to a bicuspid aortic valve!

    • usually extensive cluster of heavy dense calcific deposits:

      • nearly circular calcification with interior linear bar (DIAGNOSTIC)

    • poststenotic dilatation of ascending aorta

  • Isolated rheumatic aortic stenosis

    • calcium first detected at an average age of 47 years

    • In patients 30 60 years of age aortic valve calcification suggests rheumatic valve disease!

    • cluster of heavy dense calcific deposits without bicuspid contour

  • Degenerative aortic stenosis

    • calcium first detected at an average age of 54 years

    • In patients >65 years aortic valve calcification in 90% due to atherosclerosis!

    • curvilinear shape of calcium outlining tricuspid leaflets

    • diffuse dilatation + tortuosity of aorta (NO poststenotic dilatation)

• Mitral valve (MV)

  Cause:	rheumatic heart disease (virtually always), infected endocarditis, tumor attached to mitral valve, mitral valve prolapse
  Location:	inferior to a line connecting carina + anterior costophrenic angle (on lateral view)

  • calcium first detected in early thirties when patients become overtly symptomatic

  • a severely calcified MV is usually stenotic, but MV stenosis frequently exists without calcium

  • delicate calcification similar to coronary arteries (DDx: calcium in RCA/LCX)

  • superior-to-inferior motion

• Pulmonic valve

  Cause:

  tetralogy of Fallot, pulmonary stenosis, atrial septal defect

  • calcific pattern similar to calcified mitral valve

• Tricuspid valve (extremely rare)

  Cause:

  rheumatic heart disease, septal defect, tricuspid valve defect, infective endocarditis

• @ Annulus

  • = valve rings serve as fibrous skeleton of the heart for attachment of myocardial fibers + cardiac valves

  • Mitral annulus

    Cause:	degenerative (physiologic in elderly)
    Age:	>65 years; M:F = 1:4
    May be associated with:	mitral valve prolapse

    P.596

    • Commonly associated with:

      • aortic valve calcium (= aortic stenosis), hypertension, hypertrophic cardiomyopathy

    • dense bandlike calcification starting at posterior aspect + progressing laterally frequently forming a reversed C / O / U / J

    Cx:	mitral insufficiency (due to impaired anterior mitral leaflet), atrial fibrillation, heart block (due to infiltration into posterior wall conduction pathway)
    Prognosis:	doubles risk of stroke

  • Aortic annulus

    • usually in combination with degenerative aortic valve calcification

  • Tricuspid annulus

    Associated with:	long-standing RV hypertension
    Location:	right AV groove

    • bandlike C-shaped configuration

• @ Pericardium

  Cause:	idiopathic pericarditis, rheumatic fever (5%), tuberculosis, viruses, uremia, trauma, radiotherapy to mediastinum
  Location:	calcification over less pulsatile right-sided chambers along diaphragmatic surface, atrioventricular grooves, pulmonary trunk

  • 50% of patients with constrictive pericarditis show pericardial calcifications!

  • clumpy amorphous calcium deposits, frequently in atrioventricular groove

  • diffuse eggshell calcification sparing LA (not covered by pericardium)

  Cx:	constrictive pericarditis

• @ Myocardium

  Cause:	infarction, aneurysm, rheumatic fever, myocarditis
  Frequency:	in 8% post myocardial infarction; M > F
  Location:	apex/anterolateral wall of LV (coincides with LAD vascular distribution + typical location of LV aneurysms)

  • thin curvilinear contour outlining the aneurysm

  • shaggy laminated calcification suggests calcification of associated mural thrombus

  • coarse amorphous calcifications caused by trauma, cardioversion, infection, endocardial fibrosis

• @ Interventricular septum

  Location:

  triangular fibrous area between mitral + tricuspid annuli (= trigona fibrosa) representing the basal segment of interventricular septum, closely related to bundle of His

  Always associated with:

  • heavy calcification of mitral annulus/aortic valve

  Cx:	heart block

• @ Left atrial wall

  Cause:

  rheumatic endocarditis

  • diffuse sheetlike form

    • patient usually in CHF + atrial fibrillation

    • curvilinear calcification sparing interatrial septum + posterolateral wall on right side

    • shaggy nodular deposits in atrial appendage

    Cx:	mural thrombus formation + emboli

    • LA wall calcification indicates atrial fibrillation!

  • localized form

    • nodular calcific scar in posterior wall (= McCallum patch) due to injury from a forceful jet in mitral valve insufficiency

• @ Cardiac tumor

  • atrial myxoma (in 5 10% calcified), rhabdomyoma, fibroma, angioma, osteosarcoma, osteoclastoma

• @ Endocardium

  Cause:

  cardiac aneurysm, thrombus, endocardial fibroelastosis

• @ Pulmonary artery

  Cause:

  severe precapillary pulmonary arterial hypertension, syphilis

• @ Ductus arteriosus

  (a) in adults:	indicates patency of ductus with associated long-standing precapillary pulmonary hypertension
  (b) in children:	ductus likely closed

  • calcium deposition in ligament of Botallo

Coronary Artery Calcification

• The amount of coronary calcification correlates with the extent of atherosclerosis!

• The absence of calcification implies the absence of angiographically significant coronary vessel stenosis!

Pathophysiology:

• injury to endothelium allows circulating histiocytes to lodge in vessel wall where they are transformed into macrophages; these accumulate lipids ( fatty streaks beneath surface endothelium); lipids calcify; the thin fibrous cap overlying lipid deposits may rupture allowing circulating blood to mount a thrombogenic reaction resulting in narrowing of lumen

• Calcium is deposited as calcium hydroxyapatite in hemorrhagic areas within atheromatous plaques!

CXR (detection rate up to 42%):

• Indicating more severe coronary artery disease

• parallel calcified lines (lateral view)

Fluoroscopy: (promoted as inexpensive screening test)

• asymptomatic population

  • calcifications in 34% in asymptomatic male individuals

  • in 35% of patients with calcifications exercise test will be positive (without calcifications only in 4% positive)

  P.597

  • calcifications indicate >50% stenosis with 72-76% sensitivity, 78% specificity); frequency of coronary artery calcifications with normal angiogram increases with age; predictive values in population <50 years as good as exercise stress test

• symptomatic population

  • in 54% of symptomatic patients with ischemic heart disease

  • In symptomatic patients 94% specificity for obstructive disease (>75% stenosis) of at least one of the three major vessels!

CT:

Clinical outcome:

• for coronary calcifications detected at fluoroscopy: 5.4% event risk at 1 year (vs. 2.1% without calcification)

• for electron beam CT a calcification score of 100 is highly predictive to identify patients with events

Pericardium

Pericardial Effusion

• = pericardial fluid >50 mL

• dyspnea, fatigue

• symptoms of cardiac tamponade (50%)

Etiology:

• SEROUS FLUID = transudate congestive heart failure, hypoalbuminemia, irradiation, Dressler syndrome, postpericardiotomy syndrome, myxedema

• BLOOD = hemopericardium

  • iatrogenic: cardiac surgery/catheterization, anticoagulants, chemotherapy, radiation

  • trauma: penetrating/nonpenetrating

  • acute myocardial infarction/rupture

  • rupture of ascending aorta/pulmonary trunk

  • coagulopathy

  • neoplasm: mesothelioma, sarcoma, teratoma, fibroma, angioma, metastasis (lung, breast, lymphoma, leukemia, melanoma)

• LYMPH

  • neoplasm, congenital, cardiothoracic surgery, obstruction of hilum/SVC

• FIBRIN = exudate

  • infection: viral, pyogenic, tuberculous

  • uremia: 18% in acute uremia; 51% in chronic uremia; dialysis patient

  • collagen vascular disease: SLE, rheumatoid arthritis, acute rheumatic fever

  • hypersensitivity

• Collagen vascular disease

• Uremia

• Metastasis

• Trauma

• Acute myocardial infarction

• Purulent infection

• Post MI syndrome

• Idiopathic

• Tuberculosis

• Rheumatoid arthritis

• Virus

• CXR:

  • normal with fluid <250 mL/in acute pericarditis

  • water bottle configuration = symmetrically enlarged cardiac silhouette

  • loss of retrosternal clear space

  • fat-pad sign = separation of retrosternal from epicardial fat line >2 mm (15%) by water density

  • rapidly appearing cardiomegaly + normal pulmonary vascularity

  • differential density sign = increase in lucency at heart margin secondary to slight difference in contrast between pericardial fluid + heart muscle

  • diminished cardiac pulsations

• CT:

  • fluid of water/higher (blood, infection) density

• MR:

  • transudative fluid is hypointense on T1WI + hyperintense on T2WI

  • proteinaceous/hemorrhagic fluid is hyperintense compared with transudate on T1WI

  • inflammatory pericarditis of uremia/TB may demonstrate pericardial thickening

• ECHO:

  • separation of epi- and pericardial echoes extending into diastole (rarely behind LA)

  • volume estimates by M-mode:

    • separation only posteriorly = <300 mL

    • separation throughout cardiac cycle = 300 500 mL

    • plus anterior separation = >1000 mL

Pneumopericardium

• thick shaggy soft-tissue density of fibrous pericardium separated by air from cardiac density

• air limited to distribution of pericardial reflection

Pericardial Tumor

• Pericardial teratoma: benign tumor of infants + children

• Pericardial mesothelioma: malignant tumor of adulthood

Vena Cava

Vena cava anomalies

Circumaortic Left Renal Vein

• venous collar encircling aorta

• superior left renal vein crosses aorta anteriorly

P.598

• inferior left renal vein receives left gonadal vein + crosses aorta posteriorly 1 2 cm below the superior left renal vein

Duplicated IVC

• = DOUBLE IVC

DOUBLE IVC WITH RETROAORTIC RIGHT RENAL VEIN AND AZYGOS CONTINUATION OF IVC

DOUBLE IVC WITH RETROAORTIC RIGHT RENAL VEIN AND HEMIAZYGOS CONTINUATION OF IVC

• right IVC and right renal vein join the left IVC and continue cephalad as the hemiazygos vein

• hemiazygos vein follows alternative pathways:

  • crosses posterior to aorta at T8-9 and joins the rudimentary azygos vein

  • continues cephalad + joins coronary vein via persistent left SVC

  • accessory hemiazygos continuation to left brachiocephalic vein

• hepatic segment of IVC drains into right atrium

Interrupted IVC with Azygos/Hemiazygos Continuation

Left IVC

• = TRANSPOSITION OF IVC = SOLITARY LEFT IVC

• left IVC usually joins left renal vein

• crossover as left renal vein usually anterior/rarely posterior to aorta

Persistent Left SVC

• = BILATERAL SVCs

• left SVC drains into enlarged coronary sinus (common)

• left SVC drains into LA (rare) creating a R-to-L shunt (increased prevalence of CHD)

• hemiazygos arch formed by left superior intercostal vein + persistent left SVC (20%)

• absent/small left brachiocephalic vein (65%)

• absence of right SVC (10 18%)

• anastomosis between right + left anterior cardinal veins (in 35%)

Retroaortic Left Renal Vein

• crossover usually below/occasionally at level of right renal vein

IVC Obstruction

• INTRINSIC OBSTRUCTION

  • neoplastic (most frequent)

    • Renal cell carcinoma (in 10%), Wilms tumor

    • Adrenal carcinoma, pheochromocytoma

    • Pancreatic carcinoma, hepatic adenocarcinoma

    • Metastatic disease to retroperitoneal lymph nodes (carcinoma of ovary, cervix, prostate)

  • nonneoplastic

    • Idiopathic

    • Proximally extending thrombus from femoroiliac veins

    • Systemic disorders: coagulopathy, Budd-Chiari syndrome, dehydration, infection (pelvic inflammatory disease), sepsis, CHF

    • Postoperative/traumatic phlebitis, ligation, plication, clip, cava filter, severe exertion

• INTRINSIC CAVAL DISEASE

  • neoplastic

    • Leiomyoma, leiomyosarcoma, endothelioma

  • nonneoplastic

    • Congenital membrane

• EXTRINSIC COMPRESSION

  • neoplastic

    • Retroperitoneal lymphadenopathy (adults) due to metastatic disease, lymphoma, granulomatous disease (TB)

    • Renal + adrenal tumors (children)

    • Hepatic masses

    • Pancreatic tumor

    • Tumor-induced desmoplastic reaction (eg, metastatic carcinoid)

  • nonneoplastic

    • Hepatomegaly

    • Tortuous aorta/aortic aneurysm

    • Retroperitoneal hematoma

    • Massive ascites

    • Retroperitoneal fibrosis

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• FUNCTIONAL OBSTRUCTION

  • Pregnant uterus

  • Valsalva maneuver

  • Straining/crying (in children)

  • Supine position with large abdominal mass

• COLLATERAL PATHWAYS

  • Deep pathway: ascending lumbar veins to azygos vein (right) + hemiazygos vein (left) + intravertebral, paraspinal, extravertebral plexus (Batson plexus)

  • Intermediate pathway: via periureteric plexus + left gonadal vein to renal vein

  • Superficial pathway: external iliac vein to inferior epigastric vein + superior epigastric vein + internal mammary vein into subclavian vein

  • Portal pathway: retrograde flow through internal iliac vein + hemorrhoidal plexus into inferior mesenteric vein + splenic vein into portal vein

Vasculitis

= inflammation and necrosis of vessel wall

• LARGE-VESSEL VASCULITIS

  • Giant cell (temporal) arteritis

  • Takayasu disease

• MEDIUM-SIZED VESSEL VASCULITIS

  • Polyarteritis nodosa

  • Kawasaki disease

  • Drug-induced vasculitis:

    • methamphetamine

    • cocaine: neurovascular, cardiovascular complications, aortic dissection, venous thrombosis, mesenteric artery thrombosis, renal infarction

• SMALL-VESSEL VASCULITIS

  • ANCA-associated small-vessel vasculitis

    • (= antineutrophil cytoplasmic autoantibodies)

    • Wegener granulomatosis

    • Churg-Strauss syndrome

    • Microscopic polyangitis

  • immune-complex small-vessel vasculitis

    • Henoch-Sch nlein purpura

    • Essential cryoglobulinemic vasculitis

    • Cutaneous leukocytoclastic angitis

    others:

    lupus, rheumatoid, Sj gren, Beh et, Goodpasture, serum sickness, drug-induced, hypocomplementemic urticaria

  • inflammatory bowel disease vasculitis

Multiple Aneurysms

• Polyarteritis nodosa

• Rheumatoid vasculitis

• Systemic lupus erythematosus

• Churg-Strauss syndrome

Cardiac surgery

Surgical Procedures

• AORTICOPULMONARY WINDOW SHUNT

  • = side-to-side anastomosis between ascending aorta and left pulmonary artery (reversible procedure)

  • Tetralogy of Fallot

• BLALOCK-HANLON PROCEDURE

  • = surgical creation of ASD

  • Complete transposition

• BLALOCK-TAUSSIG SHUNT

  • = end-to-side anastomosis of subclavian artery to pulmonary artery, performed ipsilateral to innominate artery/opposite to aortic arch

  • Modified Blalock-Taussig shunt uses synthetic graft material such as polytetrafluoroethylene (Gore-Tex ) in an end-to-side anastomosis between subclavian artery + ipsilateral branch of pulmonary artery

  • Tetralogy of Fallot, tricuspid atresia with pulmonic stenosis

• FONTAN PROCEDURE

  • external conduit from right atrium to pulmonary trunk (= venous return enters pulmonary artery directly)

  • closure of ASD: floor constructed from flap of atrial wall and roof from piece of prosthetic material

  • Tricuspid atresia

• GLENN SHUNT

  • = end-to-side shunt between distal end of right pulmonary artery and SVC; reserved for patients with cardiac defects in which total correction is not anticipated

  • Tricuspid atresia

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• NORWOOD PROCEDURE

  • construction of neoaorta from aortic arch + descending aorta + main pulmonary artery supplying coronary and systemic circulation

  • communication between RV as systemic ventricle and systemic circulation

  • shunt between innominate artery + main pulmonary artery to control pulmonary arterial blood flow

  • excision of distal ductus arteriosus + atrial septum to prevent pulmonary venous hypertension

  • Hypoplastic left heart syndrome

• POTT SHUNT

  • = side-to-side anastomosis between descending aorta + left pulmonary artery

  • Tetralogy of Fallot

• MUSTARD PROCEDURE

  • removal of atrial septum

  • pericardial baffle placed into common atrium such that systemic venous blood is rerouted into left ventricle and pulmonary venous return into right ventricle and aorta

  • Complete transposition

• RASHKIND PROCEDURE

  • = balloon atrial septostomy

  • Complete transposition

• RASTELLI PROCEDURE

  • external conduit (Dacro) with porcine valve connecting RV to pulmonary trunk

  • Transposition

• WATERSTON-COOLEY SHUNT

  • = side-to-side anastomosis between ascending aorta and right pulmonary artery;

    • extrapericardial (WATERSTON)

    • intrapericardial (COOLEY)

  • Tetralogy of Fallot

Postoperative Thoracic Deformity

• ON RIGHT SIDE

  • Systemic-PA shunt: Blalock-Taussig shunt, Waterston-Cooley shunt, Glenn shunt, central conduit shunt

  • Atrial septectomy: Blalock-Hanlon procedure

  • VSD repair: through RA

  • Mitral valve commissurotomy

• ON LEFT SIDE

  • PDA

  • Coarctation

  • PA banding

  • Mitral valve commissurotomy

  • Systemic-PA shunt: Blalock-Taussig shunt, Pott shunt

Heart Valve Prosthesis

• Starr-Edwards

  • caged ball

  • Predictable performance from large long-term experience

• Bj rk-Shiley/Lillehei-Kaster/St. Jude

  • tilting disk

  • Excellent hemodynamics, very low profile, durable

• Hancock/Carpentier-Edwards (= porcine xenograft) Ionescu-Shiley (= bovine xenograft)

  • Low incidence of thromboembolism, no hemolysis, central flow, inaudible

Pulsus Alternans

= alternating arterial pulse height with regular cardiac rhythm

• Intrinsic myocardial abnormality

  • severe left ventricular dysfunction (CHF, aortic valvular disease, hypothermia, hypocalcemia, hyperbaric stress, ischemia)

• Alternating end-diastolic volumes abnormalities in venous filling + return (obstructed venous return, IVC balloon

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Cardiovascular Anatomy

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Heart size

Cardiothoracic Ratio

• = widest transverse cardiac diameter widest inside thoracic diameter

Dependent on:

• lung volume: CT ratio enlarges in expiration

• patient position: CT ratio increases on supine film

Pitfalls:

• no change unless LV volume increases by >2/3

• no change in moderate enlargement of LA / RV

Heart valve positions

PA CXR:

• reference line = oblique line drawn from distal left mainstem bronchus to right cardiophrenic angle

• aortic valve resides in profile superior to this line overlying the thoracic spine

• pulmonic valve just inferior to left mainstem bronchus

• mitral valve resides inferior to this line centrally located within cardiac silhouette

• tricuspid valve inferior to this line more basilar and midline

LAT CXR:

• reference line = oblique line drawn from carina / right pulmonary artery shadow to anterior cardiophrenic sulcus

• aortic valve resides superior to this line

• pulmonic valve anterior + superior to aortic valve

• mitral valve resides inferoposteriorly to this line

• tricuspid valve inferior to this line anteriorly

Atria

Right Atrium

• Eustachian valve = valve of IVC

  • located at junction of IVC + RA

  • directs blood from IVC to foramen ovale in fetus

  • thin linear structure, not routinely imaged

• Crista terminalis

  • line of fusion between anterior trabeculated portion + smooth-walled posterior portion of RA

  • vertically oriented smooth muscular ridge

Interatrial Septum

• thin septum, difficult to image

• may contain small amount of fat sparing fossa ovalis

Left Atrium

• ridge of smooth muscle ( bulbous tip) at junction of left atrial appendage and entrance of left superior pulmonary vein

Aortic arch branching patterns

• Standard branching pattern (65 75%)

  • brachiocephalic trunk, left CCA, left subclavian artery

• Common origin of brachiocephalic trunk + left CCA (13%)

• Bovine aortic arch (9%)

  • = origin of left CCA from brachiocephalic trunk

• Vertebral artery (usually left) arising from aortic arch (3%)

• Left and right brachiocephalic trunks (1%)

• Aberrant right subclavian artery as the last branch of the aortic arch (<1%)

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Cervical Aortic Arch

• pulsatile neck mass

• upper airway obstruction

• dysphagia

• mediastinal widening

• absence of normal aortic knob

• aortic arch near lung apex

• tracheal displacement to opposite side + anteriorly

• apparent cutoff of tracheal air column (secondary to crossing of descending aorta to side opposite of arch)

Aortic isthmus variants

Aortic Isthmus

• = narrowing of the aorta in newborn between left subclavian artery and ductus arteriosus

Aortic Spindle (16%)

• = congenital narrowing of the aorta at the ligamentum arteriosum with distal fusiform dilatation

Ductus Diverticulum

• = localized bulge along anteromedial aspect of aortic isthmus

• focal bulge with smooth uninterrupted margins:

  • gently sloping symmetric shoulders (classic ductus diverticulum)

  • shorter steeper slope superiorly + more gentle slope inferiorly (atypical ductus diverticulum)

Prominent Bronchial-intercostal Trunk

Coronary arteries

Anatomy of Right Coronary Artery (RCA)

• arises from anterior right coronary sinus;

• travels within right atrioventricular sulcus;

• rounds the acute margin of the heart

• Conus artery (CB)

  • = 1^st branch from RCA (in 50% directly from aorta) to supply RVOT

• Sinoatrial node artery (SANA)

  • = 2^nd branch from RCA (in >50%)

• Acute / RV marginal branches (M1, M2, etc)

  • have an anterior course

• Posterior descending artery (PDA)

  • originates from RCA near crux or from a distal acute marginal branch;

  • supplies posterior third of ventricular septum + diaphragmatic segment of LV;

  • supplies blood to posteromedial papillary muscle

• Atrioventricular node artery (AVNA)

  • = small branch to AV node

• Posterolateral segment arteries (PLSA)

  • supplies posterolateral wall of LV

Anatomy of Left Coronary Artery (LCA)

• arises from left posterior coronary sinus

• Left main coronary artery (LM)

  • 0.5 2.0 cm short stem before bi- / trifurcation

• Left anterior descending (LAD)

  • travels within anterior interventricular groove, gives blood supply to anterolateral papillary muscle

  • (a) Diagonal branches (D1, D2, etc)

    • arise from LAD and course over anterolateral wall of LV

    mnemonic:

    Diagonals from LAD occasionally trifurcation into LAD + LCx + ramus intermedius (in a course similar to D1)

  • (b) Septal branches (S)

    • for anterior interventricular septum

• Left circumflex artery (LCx)

  • travels within left atrioventricular sulcus;

  • terminates at obtuse margin of heart

  • (a) Obtuse marginal branches (OM1, OM2, etc)

    • for lateral wall of LV

  • (b) Left atrial circumflex artery (LACX)

    • for atrium

Coronary Artery Territory

Coronary Artery Dominance

• determined by the artery that crosses the crux and serves as origin of the posterior descending artery (PD), which supplies the inferior portion of LV:

• from RCA in 85% (= right dominance)

• from LCx in 8% (= left dominance)

• RCA + LCA = codominance / balanced supply (7%)

Coronary Arteriography

Contrast agents:

• Monomeric ionic contrast material:

  P.605

  • negative inotropic = depression of myocardial contractility due to hyperosmolality of sodium + decrease in total calcium

  • peripheral vasodilatation

• Meglumine diatrizoate (contains small quantities of sodium citrate + EDTA)

• Nonionic contrast material = slight increase in LV contractility

Risk factors associated with death:

• Multiple ventricular premature contractions

• Congestive heart failure

• Systemic hypertension

• Severe triple-vessel coronary artery disease (highest risk)

• LV ejection fraction <30%

• Left main coronary artery stenosis

Clues for projection:

45 70 LAO:

• ribs slanting to left side of image

• catheter in descending aorta on right side of image

15 30 RAO:

• ribs slanting to right side of image

• catheter in descending aorta on left side of image

Technique:

• 20 30 of cranial / caudal angulation variably used

• Catheter in left coronary orifice:

  • (a) LAO + caudocranial angulation:

    • proximal 1/3 of LAD + origin of first diagonal branch

  • (b) LAO + craniocaudal angulation = spider view :

    • LCA, proximal LCx, first marginal / diagonal branches

  • (c) RAO + craniocaudal angulation:

    • proximal third of LCx + origin of its branches

  • (d) RAO + caudocranial angulation:

    • separation of LAD from diagonal branches

• Catheter in right coronary artery orifice: LAO plusmn; RAO

False-negative interpretation:

• eccentric lesion in 75%

• foreshortening of vessel

• overlap of other vessels remedied by angulated projections: improved diagnosis (50%), upgrade to more significant stenosis (30%), lesion unmasked (20%)

Coronary Artery Collaterals

• intracoronary COLLATERALS

  • = filling of a distal portion of an occluded vessel from the proximal portion

  • tortuous course outside the normal path

• intercoronary COLLATERALS

  • = between different coronary arteries / between branches of the same artery

  P.606

  Right and Left Coronary Artery Angiograms

  P.607

  Coronary Artery Angiograms of Varying Dominance

  Location:

  on epicardial surface, in atrial / ventricular septum, in myocardium

  • Proximal RCA to distal RCA

    • by way of acute marginal branches

    • from sinoatrial node artery (SANA) to atrioventricular node artery (AVNA) = Kugel collateral

  • RCA to LAD

    • between PDA and LAD through ventricular septum / around apex

    • conus artery (1st branch of RCA) to proximal part of LAD

    • acute marginals of RCA to right ventricular branches of LAD

  • Distal RCA to distal LCx

    • posterolateral segment artery of RCA to distal LCx (in AV groove)

    • AVNA of RCA to LCx (through atrial wall)

    • posterolateral branch of RCA to obtuse marginal branches of LCx (over left posterolateral ventricular wall)

  • Proximal LAD to distal LAD

    • proximal diagonal to distal diagonal artery of LAD

    • proximal diagonal to LAD directly

  • LAD to obtuse marginal of LCx

Coronary Artery Anomalies

Anomaly of Origin

• High takeoff (6%)

  • = origin of RCA / LCA above the junctional zone between sinus + tubular part

• Multiple ostia

  • RCA + conus branch arise separately

  • LAD + LCx arise separately without LCA (0.41%)

• Single coronary artery (0.0024 0.044%)

• Anomalous origin from the pulmonary artery

  • Bland-White-Garland syndrome

    • = LCA arises from PA + RCA arises from aorta

    Prevalence:

    1:300,000 live births

    • collateral circulation between RCA + LCA

    • coronary steal into PA

    Prognosis:

    death in 1st year of life in 90%

• Origin of coronary artery from opposite sinus / noncoronary sinus

  • RCA arising from left coronary sinus (0.03 0.17%)

  • LCA arising from right coronary sinus (0.09 0.11%)

  • LCx / LAD arising from right coroanry sinus (0.32 0.67%)

  • LCA / RCA arising from noncoronary sinus

  may take the following course:

  • interarterial (between aorta + pulmonary trunk) with a high risk of sudden cardiac death

  • retroaortic

  • prepulmonic

  • septal (subpulmonic / beneath RVOT

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Anomaly of Coronary Artery Course

• Myocardial bridging

  • = band of myocardial muscle overlying a segment of a coronary artery

• Duplication of arteries, eg., LAD

Anomaly of Termination

• Coronary artery fistula (0.1 0.2%)

  • = communication between coronary artery (RCA in 60%, LCA in 40%, both in <5%) and cardiac chamber (RV in 45%, RA in 25%) / pulmonary artery (in 15%) / coronary sinus / SVC

  • dilated tortuous coronary artery

  • single / multiple communications / network of fine vessels

  Cx:	myocardial ischemia

• Coronary arcade

  • = angiographically demonstrable communication between RCA and LCA in the absence of a coronary artery stenosis

  • prominent straight connection near crux

  DDx:	tortuous collateral vessel

• Extracardiac termination

  Cause:	atherosclerotic CAD
  Receiver:	bronchial, internal mammary, pericardial, anterior mediastinal, superior / inferior phrenic, intercostal arteries

Pericardium

• FIBROUS PERICARDIUM

  • = outer fibrous layer

• SEROUS PERICARDIUM

  • = inner serous sac forming the pericardial cavity

  • contains 20 50 mL of serous fluid

  • (a) inner visceral layer = epicardium

    • intimately connected to heart + epicardial fat

  • (b) outer parietal layer

    • lines fibrous pericardium

Pericardial sinuses and Recesses

• = extensions of pericardial cavity

• Recesses of pericardial cavity proper

  • Postcaval recess (23%*)

    • behind and right lateral to SVC

  • Right pulmonic vein recess (29%*)

    • behind and right lateral to SVC

  • Left pulmonic vein recess (60%*)

    • behind and right lateral to SVC

• Transverse sinus

  • posterior to ascending aorta and pulmonary trunk + above left atrium (95%*)

  • 1. Superior aortic recess

    • along ascending aorta; may be divided into anterior, posterior, right lateral portion

    DDx:	aortic dissection on NECT

  • 2. Left pulmonic recess

    • below left pulmonary artery + posterolateral to proximal right pulmonary artery

  • 3. Right pulmonic recess

    • below right pulmonary artery + above left atrium

  • 4. Inferior aortic recess

    • between ascending aorta + inferior SVC / right atrium

    • extending down to level of aortic valve

• Oblique sinus (89%*)

  • behind left atrium + anterior to esophagus

  • separated from transverse sinus by double reflection of pericardium (and fat) between right + left superior pulmonic veins

  • 1. Posterior pericardial recess (67%*)

    • behind distal right pulmonary artery + medial to bronchus intermedius

      P.609

      DDx:	lymph nodes, esophageal / thymic process, vascular abnormality, pericardial cyst / tumor

    • * = percentages give depiction on HRCT

Embryogenesis Of Vena Cava

Origin:

• Vitelline (omphalomesenteric) venous system:

  • blood from yolk sac to sinus venosus

• Umbilical venous system

  • blood from chorionic villi to sinus venosus via ductus venosus

• Intraembryonic cardinal venous system

  • continuous appearance + regression of 3 paired embryonic veins

  • (1) Cardinal veins

    • join to form common cardinal vein, which enters left + right sinus horns

    • (a) anterior cardinal veins

      • drain the cranial region

    • (b) posterior cardinal veins

      • drain body of embryo + mesonephros + anterior extremities

    Location:

    dorsolateral part of urogenital fold

  • (2) Subcardinal veins

    • drain urogenital system of metanephros + suprarenal glands

      Location:

      ventromedial to posterior cardinal veins + ventrolateral to aorta

    • Intersubcardinal anastomoses form anterior to aorta below superior mesenteric artery and connect left + right subcardinal veins

  • (3) Supracardinal veins

    • drain body wall via intercostal veins

    Location:

    dorsomedial to posterior cardinal vein + dorsolateral to aorta

    • CRANIAL

      • azygos vein on the right

        • drains 4 11 right intercostal veins

      • portion of superior intercostal vein

        • drains 2 3 left intercostal veins

      • accessory hemiazygos

        • drains 4 7 left intercostal veins

      • hemiazygos vein

        • drains left 8 11 intercostal veins

    • CAUDAL: lumbar veins

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Inferior Vena Cava

• Hepatic = posthepatic segment

  Origin:

  terminal part of right vitelline vein

• Suprarenal segment

  Origin:

  subcardinal-hepatic anastomosis

• Renal segment

  Origin:

  part of right subcardinal vein + supracardinal-subcardinal anastomoses

• Infrarenal segment

  Origin:

  right supracardinal / sacrocardinal vein

Venous system of lower extremity

Deep Veins of Lower Extremity

• 3 paired stem veins of the calf accompany the arteries as venae commitantes + anastomose freely with each other:

• 1. Anterior tibial veins

  • draining blood from dorsum of foot, running within extensor compartment of lower leg close to interosseous membrane

• 2. Posterior tibial veins

  • formed by confluence of superficial + deep plantar veins behind ankle joint

• 3. Peroneal veins

  • directly behind + medial to fibula

• 4. Calf veins

  • Soleal muscle veins

    • baggy valveless veins in soleus muscle (= sinusoidal veins); draining into posterior tibial + peroneal veins or lower part of popliteal vein

  • Gastrocnemius veins

    • thin straight veins with valves; draining into lower + upper parts of popliteal vein

• 5. Popliteal vein

  • formed by stem veins of lower leg

• 6. Femoral / superficial femoral vein

  • continuation of popliteal vein; receives deep femoral vein about 9 cm below inguinal ligament

• 7. Deep femoral vein

  • draining together with superficial femoral vein into common femoral vein; may connect to popliteal vein (38%)

• 8. Common femoral vein

  • formed by confluence of deep + superficial femoral vein; becomes external iliac vein as it passes beneath inguinal ligament

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Superficial Veins of Lower Extremity

• Greater saphenous vein

  • formed by union of veins from medial side of sole of foot with medial dorsal veins; ascends in front of medial malleolus; passes behind medial condyles of tibia + femur

  • (a) Posterior arch vein

    • connected to deep venous system by communicating veins

  • (b) Anterior superficial tibial vein

  • (c) Posteromedial superficial thigh vein

    • often connects with upper part of lesser saphenous vein

  • (d) Anterolateral superficial thigh vein

  • (e) Tributaries in fossa ovalis

    • superficial inferior epigastric vein

    • superficial external pudendal vein

    • superficial circumflex iliac vein

• Lesser saphenous vein

  • originates at outer border of foot behind lateral malleolus as continuation of dorsal venous arch; enters popliteal vein between heads of gastrocnemius in popliteal fossa within 8 cm of knee joint (60%) or joins with greater saphenous vein via posteromedial / anterolateral superficial thigh veins (20%)

Communicating = Perforating Veins

• >100 veins in each leg

• MEDIAL

  • Submalleolar communicating vein

  • Cockett group

    • group of 3 veins located 7, 12, 18 cm above the tip of medial malleolus connecting posterior arch vein with posterior tibial vein

  • Boyd vein

    • located 10 cm below knee joint connecting main trunk of greater saphenous vein to posterior tibial veins

  • Dodd group

    • group of 1 or 2 veins passing through Hunter canal (= subsartorial canal) to join greater saphenous vein with superficial femoral vein

• LATERAL

  • Lateral communicating vein

    • located from just above lateral malleolus to junction of lower-to-mid thirds of calf connecting lesser saphenous vein with peroneal veins

  • Posterior mid-calf communicating veins

    • located posteriorly 5 + 12 cm above os calcis joining lesser saphenous vein to peroneal veins

  • Soleal + gastrocnemius points

    • joining short saphenous vein to soleal / gastrocnemius veins

Contents of femoral triangle

• Nerve

• Artery

• Vein

• Empty space

• Lymphatics

Pulsatility

• = assessment of vascular resistance (increased resistance reduces diastolic flow)

• Can be assessed in vessels too small / tortuous to be imaged (Doppler angle unnecessary)!

• Index should be calculated for each of several cardiac cycles (5 heartbeats adequate) an average value taken

  • S = A = maximal systolic shift

  • D = B = end-diastolic frequency shift

• 1. Full pulsatility index of Gosling (PI_F) = 1/A₀² SA_i²

• 2. Simplified pulsatility index (PI) = (S - D)/mean

• 3. Resistance index (RI) = Pourcelot index = (S- D)/S or 1 - (D/S)

• 4. Stuart index = A/B ratio = S/D ratio

• 5. B/A ratio = B(100%)/A

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Cardiovascular Disorders

Aberrant Left Pulmonary Artery

• = LEFT PULMONARY ARTERY SLING

Classification:

• Normal bronchial pattern

• Malformation of bronchotracheal tree

  Associated with:

  • napkin-ring trachea = absent pars membranacea (50%)

  • Stenosis of long tracheal segment

  • PDA (most common), ASD, persistent left SVC

• stridor (most common), wheezing, apneic spells, cyanosis

• respiratory infection

• feeding problems

• deviation of trachea to left

• inverted-T appearance of mainstem bronchi = horizontal course secondary to lower origin of right mainstem bronchus

• anterior bowing of right mainstem bronchus

• carrot-shaped trachea = narrowing of tracheal diameter in caudad direction resulting in functional tracheal stenosis

• obstructive emphysema/atelectasis of RUL + LUL

• low left hilum

• separation of trachea + esophagus at hilum by soft-tissue mass

• anterior indentation on esophagogram

Amyloidosis

• = extracellular deposits of insoluble fibrillar protein

• asymptomatic/CHF (restrictive cardiomyopathy), arrhythmia

CXR:

• normal/generalized cardiomegaly

• pulmonary congestion

• pulmonary deposits of amyloid

NUC:

• striking uptake of Tc-99m pyrophosphate greater than bone (50 90%)

ECHO:

• granular sparkling appearance of myocardium

• LV wall thickening

• decreased LV systolic + diastolic function

Anomalous Left Coronary Artery

• = left coronary artery arises from pulmonary trunk (left sinus of Valsalva)

Hemodynamics:

• with postnatal fall in pulmonary arterial pressure perfusion of LCA drops (ischemic left coronary bed), collateral circulation from RCA with flow reversal in LCA

  • adequate collateral circulation = lifesaving

  • inadequate collateral circulation = myocardial infarction

  • large collateral circulation = L-to-R shunt with volume overload of heart

• episodes of sweating, ashen color (angina symptomatology)

• ECG: anterolateral infarction

• continuous murmur (if collaterals large)

• dilatation of LV

• enlargement of LA

• normal pulmonary vascularity/redistribution

Anomalous Pulmonary Venous Return

• = lack of an appropriate connection of the primitive pulmonary vein to the left atrium with persistence of fetal pulmonary + systemic connections

Total Anomalous Pulmonary Venous Return

• = entire pulmonary venous return directed to RA = TAPVR = admixture lesion because of the combination of cyanosis + increased pulmonary vascularity (L-to-R and R-to-L shunt)

• cyanosis

Hemodynamics:

• obstruction along the pulmonary venous pathway

Associated with:

• asplenia; ASD/patent foramen ovale (necessary for survival), bronchopulmonary sequestration, pulmonary arteriovenous malformation, cystic adenomatoid malformation

MR:

• retroatrial sign = presence of veins posterior to LA

• small LA without pulmonary venous connections

• patent foramen ovale/ASD

• anomalous vein with variable location

P.614

Supradiaphragmatic TAPVR (82%)

Hemodynamics:

• functional L-to-R shunt from pulmonary veins to RA

• increased pulmonary blood flow (= overcirculation)

• obligatory R-to-L shunt via usually patent foramen ovale/ASD restores oxygenated blood to left side

• normal systemic venous pressure with increased flow through widened SVC

• after birth CHF secondary to

  • mixture of systemic + pulmonary venous blood in RA

  • volume overload of RV

• cyanosis

• neck veins undistended (shunt level distally)

• R ventricular heave (= increased contact of enlarged RV with sternum)

• systolic ejection murmur (large shunt volume)

• overall heart size notably normal:

  • slightly enlarged RV (= volume overload with time)

  • normal/enlarged RA

  • normal LA (= ASD acts as escape valve)

• dilated SVC + left vertical vein:

  • figure of 8 / snowman configuration of cardiac silhouette (= dilated SVC + left vertical vein)

  • pretracheal density on lateral film (= left vertical vein)

• increased pulmonary blood flow (= overcirculation)

• absent connection of pulmonary veins to LA

Sub-/Infradiaphragmatic TAPVR (12%)

• = type III

• = drainage into portal vein/IVC/ductus venosus/left gastric vein with constriction of descending pulmonary vein by diaphragm en route through esophageal hiatus leading to pulmonary venous hypertension + RV pressure overload; >90% obstructed

• intense cyanosis + respiratory distress (R-to-L shunt through ASD)

• unique appearance of pulmonary edema + pulmonary venous congestion with normal-sized heart (DDx: hyaline membrane disease)

• low anterior indentation on barium-filled esophagus

Mixed Type of TAPVR (6%)

• = type IV

• = with various connections to R side of heart (6%)

Partial Anomalous Pulmonary Venous Return

• = PAPVR = only a portion of the pulmonary venous circulation connects to the LA

• May occur in isolation

May be associated with:

• Atrial septal defect (25%)

  • RUL pulmonary vein enters SVC/RA (66%)

    • 90% of patients with sinus venosus type ASD have PAPVR

    • 50% of patients with PAPVR have sinus venosus type ASD

    • RUL vein courses in a horizontal direction

  • LUL pulmonary vein enters brachiocephalic vein (33%)frequently associated with: ostium secundum type ASD (10 15%)

    • vertical mediastinal density lateral to aortic knob extending upward and medially with smooth curvilinear border (DDx: persistent left SVC)

• Hypogenetic lung as a component of congenital pulmonary venolobar syndrome

  • = Scimitar Syndrome

  • = lower part/all of the hypogenetic lung is drained by an anomalous vein

  • Anomalous vein drains into:

    • IVC below right hemidiaphragm (33%)

    • suprahepatic portion of IVC (22%)

    • hepatic veins

    • portal vein (11%)

    • azygos vein

    • coronary sinus

    • right atrium (22%)

    • left atrium = meandering pulmonary vein

      • Drainage into suprahepatic portion of IVC/right atrium may be a clue for interruption of intrahepatic portion of IVC!

  May be associated with:	systemic arterialization of the lung without sequestration
  Location:	almost exclusively on right side

  • tubular structure paralleling the right heart border in the configuration of a Turkish sword = scimitar (PA view)

  • shift of heart + mediastinum into right chest

• acyanotic

• ASD symptomatology

• radiographic findings similar to ASD

• anomalous course of draining vein

• enlargement of draining site: SVC, IVC, azygos vein

CECT:

• nodular/tubular opacity (= anomalous vein), which opacifies in phase with pulmonary vein

Aortic Aneurysm

Cause:

• Atherosclerosis (73 80 90%): descending aorta

• Traumatic (15 20%): following transection; descending aorta

• Congenital (2%): aortic sinus, post coarctation, ductus diverticulum

• Syphilis (19%): ascending aorta + arch

• Mycotic = bacterial dissection; anywhere

P.615

• Cystic media necrosis (Marfan/Ehlers-Danlos syndrome, annuloaortic ectasia): ascending aorta

• Inflammation of media + adventitia:

  • Takayasu arteritis, giant cell arteritis, relapsing polychondritis, rheumatic fever, rheumatoid arthritis, ankylosing spondylitis, Reiter syndrome, psoriasis, ulcerative colitis, systemic lupus erythematosus, scleroderma, Beh et disease, radiation

• Increased pressure:

  • systemic hypertension, aortic valve stenosis

• Abnormal volume load: severe aortic regurgitation

True Aneurysm

• = permanent dilatation of all layers of weakened but intact wall

False Aneurysm

• = focal perforation with all layers of wall disrupted; escaped blood contained by adventitia/perivascular connective tissue + organized blood

Fusiform Aneurysm (80%)

• = circumferential involvement

Saccular aneurysm

• = involvement of portion of wall

Abdominal Aortic Aneurysm (AAA)

• There is no consensus regarding the definition of an atherosclerotic AAA!

• = focal widening >3 cm (ultrasound literature); twice the size of normal aorta/>4 cm [Bergan, Ann Surg 1984]

• Normal size of abdominal aorta >50 years of age:

  • 12 19 mm in women; 14 21 mm in men

Associated with:

• visceral + renal artery aneurysm (2%)

• isolated iliac + femoral artery aneurysm (16%):

• common iliac (89%), internal iliac (10%), external iliac (1%)

• stenosis/occlusion of celiac trunk/SMA (22%)

• stenosis of renal artery (22 30%)

• occlusion of inferior mesenteric artery (80%)

• occlusion of lumbar arteries (78%)

Growth rate of aneurysm of 3 6 cm in diameter:

• 0.39 cm/year

• asymptomatic (30%)

• abdominal mass (26%)

• abdominal pain (37%)

• Imaging should provide information about:

  • the proximal extent of the aneurysm, which determines the site of clamping of the aorta (origin of renal arteries)

  • the course of the left renal vein (retroaortic?)!