CONGENITAL HEART DISEASE

Incidence of CHD in Liveborn Infants

Overall incidence:   8–9÷1000 livebirths

◊  Most common CHD: 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%

Pulmonic stenosis 7.4%

Ostium 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 syndrome 1.3%

TAPVR 1.1%

Truncus arteriosus 1.0%

Single ventricle 0.3%

Double outlet right ventricle 0.2%

High-risk pregnancy:

(1)  Previous sibling with CHD:   2–5%

(2)  Previous 2 siblings with CHD:   10–15%

(3)  One parent with CHD:   2–10%

Congenital Abnormality of Aortic Valve

1.   Bicuspid aortic valve

2.  Unicuspid / unicomissural aortic valve

=  single commissure in (commonly) L posterior position

Incidence:   0.02%

Cx:   early aortic stenosis, aneurysmal dilatation of ascending aorta

3.   Quadricuspid aortic valve

Incidence:   200 cases reported

Cx:   regurgitation

4.   Aneurysmal dilatation of sinus of Valsalva

Congenital Abnormality of Mitral Valve

1.   Endocardial cushion defect

2.   Parachute mitral valve (Shone syndrome)

CHD Presenting in 1^st Year of Life

1.   VSD

2.   d-Transposition of great vessels

3.   Tetralogy of Fallot

4.   Isolated coarctation

5.   Patent ductus arteriosus

6.   Hypoplastic left heart syndrome

Most common causes for CHF + PVH in neonate:

1.   Left ventricular failure ← outflow obstruction

2.   Obstruction of pulmonary venous return

CHD Compatible with Relatively Long Life

1.   Mild tetralogy: mild pulmonic stenosis + small VSD

2.   Valvular pulmonic stenosis: with relatively normal pulmonary circulation

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

4.   Truncus arteriosus: delicate balance between systemic + pulmonary circulation

5.   Truncus arteriosus type IV: large systemic collaterals

6.   Tricuspid atresia + transposition + pulmonic stenosis

7.   Eisenmenger complex

8.   Ebstein anomaly

9.   Corrected transposition without intracardiac shunt

Classic Signs of Congenital Cardiovascular Anomalies

1.   “Boot-shaped” heart

2.   “Box-shaped” cardiomegaly

3.   “Egg-on-a-string” sign

4.   “Figure-of-3” and reverse “figure-of-3” sign

5.   “Gooseneck” sign

6.   “Scimitar” sign

7.   “Snowman” sign

Juxtaposition of Atrial Appendages

1.   Tricuspid atresia with transposition

2.   Complete transposition

3.   Corrected transposition of great arteries

4.   DORV

Continuous Heart Murmur

1.   PDA

2.   AP window

3.   Ruptured sinus of Valsalva aneurysm

4.   Hemitruncus

5.   Coronary arteriovenous fistula

Syndromes with CHD

5 p – (Cri-du-chat) Syndrome

Incidence of CHD:   20%

DiGeorge Syndrome

=  congenital absence of thymus + parathyroid glands

1.   Conotruncal malformation

2.   Interrupted aortic arch

Down Syndrome

=  MONGOLISM = TRISOMY 21

1.   Endocardial cushion defect (25%)

2.   Membranous VSD

3.   Ostium primum ASD

4.   AV communis

5.   Cleft mitral valve

6.   PDA

7.   11 rib pairs (25%)

8.   Hypersegmented manubrium (90%)

Ellis-van Creveld Syndrome

Incidence of CHD:   50%

•  polydactyly

√  single atrium

Holt-Oram Syndrome

=  UPPER LIMB-CARDIAC SYNDROME

Incidence of CHD:   50%

1.   ASD

2.   VSD

3.   Valvular pulmonary stenosis

4.   Radial dysplasia

Hurler Syndrome

Cardiomyopathy

Ivemark Syndrome

Incidence of CHD:   100%

•  asplenia

√  complex cardiac anomalies

Klippel-Feil Syndrome

Incidence of CHD:   5%

1.   Atrial septal defect

2.   Coarctation

Marfan Syndrome

=  ARACHNODACTYLY

1.   Annuloaortic ectasia

2.   Aortic aneurysm

3.   Aortic regurgitation

4.   Pulmonary aneurysm

Noonan Syndrome

1.   Pulmonary stenosis

2.   ASD

3.   Hypertrophic cardiomyopathy

Osteogenesis Imperfecta

1.   Aortic valve insufficiency

2.   Mitral valve insufficiency

3.   Pulmonic valve insufficiency

Postrubella Syndrome

•  low birth weight, deafness, 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

Incidence of CHD:   35%

1.   Coarctation of the aorta (in 15%)

2.   Bicuspid aortic valve

3.   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

A.  AGE

›  Infants:

(1)  Isolated VSD

(2)  VSD with CoA / PDA / AV canal

(3)  PDA

(4)  Ostium primum

›  Children / adults:

(1)  ASD

(2)  Partial AV canal with competent mitral valve

(3)  VSD / PDA with high pulmonary resistance

(4)  PDA without murmur

B.  SEX

99% chance for ASD / PDA in female patient

C.  CHEST WALL ANALYSIS

√  11 pair of ribs + hypersegmented manubrium → Down syndrome

√  pectus excavatum + straight back syndrome + funnel chest → prolapsing mitral valve

√  rib notching

D.  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

A.  Precardiac shunt

1.   Anomalous pulmonary venous connection

B.  Intracardiac shunt

1.   ASD (8%)

2.   VSD (25%)

C.  Postcardiac shunt

1.   PDA (12%)

Aortic Size in Shunts

A.  Extracardiac shunts

√  aorta enlarged + hyperpulsatile

1.   PDA

B.  Pre- and intracardiac shunts

√  aorta small but not hypoplastic

1.   Anomalous pulmonary venous return

2.   ASD

3.   VSD

4.   Common AV canal

Left-to-Right Shunts Missed on Echocardiography

1.   Sinus venosus defect

2.   Patent ductus arteriosus

3.   Anomalous pulmonary venous return

SITUS

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

Segmental Analysis of CHD

=  3-part Van Praagh notation of congenital heart disease corresponding to atria & ventricles & great vessels

◊  Only one type of situs anomaly is possible in any patient!

Step 1.   Visceroatrial situs {X,_,_}:

solitus ={S}, inversus = {I}, ambiguus = {A}

(a)  establish atrial situs by morphology of atrial appendages

√  broad blunt (triangular) appendage = RA

√  narrow pointed tubular (fingerlike) appendage = LA

◊  Morphologic RA is on patient’s right side

(b)  establish atrial situs by lung morphology:

›  morphologic RT lung

√  main RT lung bronchus is eparterial

›  morphologic LT lung

√  main LT bronchus is hyparterial

(c)  establish visceral situs:

√  largest lobe of liver on RT; stomach + spleen on LT

(b)  establish thoracoabdominal situs:

√  supradiaphragmatic IVC connects to anatomic RA

√  RT lung + largest lobe of liver on right side

◊  Atrial + thoracoabdominal situs are usually concordant!

Step 2.   Ventricular loop orientation {_,X,_}:

Embryology:

components of craniocaudal tube are truncus arteriosus (future great vessels), bulbus cordis (future right ventricle), embryonic LV, primitive atria, sinus venosus; tube curves toward right (D-loop) placing RV to right of LV

(a)  identify morphologic RV and LV

(b)  determine location of RV relative to LV

Hand rule:   approaching heart from anteriorly place right / left hand in RV with palm against IVS. If right thumb is in inflow tract + right fingers in outflow tract = D-loop

Step 3.   Position of great vessels {_,_,X}:

(a)  identify morphologic aorta (Ao supplies at least 1 coronary artery + most systemic arteries) and main pulmonary artery (MPA supplies right + left pulmonary artery)

(a)  designate position of Ao relative to MPA at level of aortic + pulmonic valves:

›  normal or inverse position: solitus ={S}, inversus = {I}

›  transposition of great arteries: dextrotransposition = {D-TGV}, levotransposition = {L-TGV}

›  malposition if both arteries override / originate from same ventricle: Ao rightward from MPA = {D-MGV}, Ao leftward from MPA = {L-MGV}

Situs Solitus = Normal / Usual Situs

Associated with:

SITUS ANOMALY

Associated with:   CHD, primary ciliary dyskinesia, abdominal anomalies (eg, intestinal malrotation)

Situs Inversus

=  mirror-image arrangement of situs solitus

Frequency:    0.01%Associated with:

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

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

Situs Ambiguus = Heterotaxy

[heteros, Greek = other / different; taxis, Greek = arrangement]

=  visceral malposition + dysmorphism associated with indeterminate atrial arrangement

Associated with:   CHD in 50–100%

Subclassification:

1.   Asplenia syndrome = double / bilateral right-sidedness

2.   Polysplenia syndrome = double / bilateral left-sidedness

Bronchial Situs

Classification:

1.   Right bronchial isomerism

√  bilateral eparterial upper lobe bronchi = morphologically right lung

2.   Left bronchial isomerism

√  bilateral hyparterial upper lobe bronchi = morphologically left lung

Less reliable:   number of lobes, ratio of left to right main bronchial lengths, bronchial branching pattern

Cardiac Malposition

=  location of heart other than within left hemothorax in situs solitus or within left hemithorax when other organs are ambiguous

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

A.  POSITION OF CARDIAC APEX

1.   Levocardia = apex directed leftward

2.   Dextrocardia = apex directed rightward

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

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

◊  Does not imply a particular internal structure / specific location of other thoracoabdominal organs!

B.  CARDIAC DISPLACEMENT

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

1.   Dextroposition

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

2.   Levoposition

3.   Mesoposition

C.  CARDIAC INVERSION

= alteration of normal relationship of chambers

1.   D-bulboventricular loop

2.   L-bulboventricular loop

D.  TRANSPOSITION

=  alteration of anterior-posterior relationship of great vessels

CYANOTIC HEART DISEASE

◊  Decrease in hemoglobin delays detectability!

Most common cause of cyanosis:

N.B.:     tricuspid atresia = the great mimicker

Increased Pulmonary Blood Flow with Cyanosis

=  ADMIXTURE LESIONS

=  bidirectional shunt with 2 components:

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

(b)  NO obstruction to pulmonary blood flow

Evaluation process:

√  cardiomegaly

√  increased pulmonary blood flow

√  concave main pulmonary artery:

√  PA segment absent = transposition

√  PA segment present:

(a)  L atrium normal (= extracardiac shunt) = TAPVR

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

N.B.:   Overcirculation + cyanosis = complete transposition until proven otherwise!

Admixture Lesions = T-lesions

mnemonic:   5 T’s + CAD

Transposition of great vessels = complete TGV ± VSD

◊  Most common cause for cyanosis in neonate

Tricuspid atresia with or without transposition + VSD

◊  2^nd most common cause for cyanosis in neonate

Truncus arteriosus

Total anomalous pulmonary venous return (TAPVR) above diaphragm:

(a)  supracardiac

(b)  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 RV; levoposition of appendage of RA (tricuspid atresia + transposition)

√  age of onset ≤ 2 days: aortic atresia

Decreased Pulmonary Blood Flow with Cyanosis

=  two components of

(a) impedance of blood flow through right heart ← obstruction / atresia at pulmonary valve / infundibulum

(b) 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

mnemonic:   P2 TETT

Pulmonic stenosis with ASD

Pulmonic atresia

Tetralogy of Fallot

Ebstein anomaly

Tricuspid atresia with pulmonic stenosis

Transposition of great vessels with pulmonic stenosis

A.  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 RA (without tricuspid regurgitation)

√  normal-sized heart ← escape mechanism into aorta

Clues:

1.   Skeletal anomaly (eg, scoliosis): tetralogy (90%)

2.   Hepatic symmetry: asplenia

3.   Right aortic arch: tetralogy, complete transposition, tricuspid atresia

4.   Aberrant right subclavian artery: tetralogy

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

B.  SHUNT AT ATRIAL LEVEL

mnemonic:   PET

1.   Pulmonary stenosis / atresia with intact ventricular septum

2.   Ebstein malformation + Uhl anomaly

3.   Tricuspid atresia (ASD in 100%)

√  moderate to severe cardiomegaly

√  RA dilatation

√  RV enlargement ← massive tricuspid incompetence

√  inapparent aorta

√  left aortic arch

Pulmonary Venous Hypertension with Cyanosis

(a)  during 1^st week of life

1.   Hypoplastic left heart syndrome

√  marked cardiomegaly

2.   TAPVR below diaphragm

√  normal cardiac size

(b)  during 2^nd week of life

3.   Aortic coarctation

4.   Aortic atresia

(c)  during 4^th–6^th 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%)

A.  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)

B.  WITH NORMAL LEFT ATRIUM indicates shunt proximal to mitral valve = volume increased with escape mechanism through defect

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

A.  OBSTRUCTIVE LESION

1.   Right ventricular outflow obstruction

(a)  at level of pulmonary valve: subvalvular / valvular / supravalvular pulmonic stenosis

(b)  at level of peripheral pulmonary arteries:

peripheral pulmonary stenosis

2.   Left ventricular inflow obstruction

(a)  at level of peripheral pulmonary veins:

pulmonary vein stenosis / atresia

(b)  at level of left atrium: cor triatriatum

(c)  at level of mitral valve:

supravalvular mitral stenosis, congenital mitral stenosis / atresia, “parachute” mitral valve

3.   Left ventricular outflow obstruction

(a)  at level of aortic valve:

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

(b)  at level of aorta:

interruption of aortic arch, coarctation of aorta

B.  CARDIOMYOPATHY

1.   Endocardial fibroelastosis

2.   Hypertrophic cardiomyopathy

3.   Glycogen storage disease

C.  HYPERDYNAMIC STATE

1.   Noncardiac AVM: cerebral AVM, vein of Galen aneurysm, large pulmonary AVM, hemangioendothelioma of liver

2.   Thyrotoxicosis

3.   Anemia

4.   Pregnancy

D.  MYOCARDIAL ISCHEMIA

1.   Anomalous left coronary artery

2.   Coronary artery disease (CAD)

ACQUIRED HEART DISEASE

√  LA enlargement = MV disease

√  dilated ascending aorta = aortic valve disease

√  RA enlargement = tricuspid valve disease

Pressure Overload

1.   Systemic hypertension

2.   Aortic stenosis

3.   Mitral stenosis

Decreased Compliance

1.   Myocardial infarction

2.   Hypertrophic cardiomyopathy

3.   Restrictive cardiomyopathy

Volume Overload

1.   Aortic insufficiency

2.   Mitral insufficiency

3.   Tricuspid insufficiency

Systolic Anterior Motion of Mitral Valve

1.   Hypertrophic Cardiomyopathy

2.   Hypertensive heart

3.   Diabetes mellitus

4.   Acute myocardial infarction

5.   Mitral valve dysfunction / repair

Concentric Left Ventricular Hypertrophy

1.   Symmetric hypertrophic cardiomyopathy

2.   Amyloidosis

3.   Cardiac sarcoidosis

4.   Athlete’s heart

√  ↑ LV mass, ↑ LV diastolic cavity dimension, ↑ LV wall thickness

√  lack of areas of delayed myocardial hyperenhancement

Dx (80% sensitive, 99% specific):

LV Thicknessdiastole ÷ Volumeend-diastole (corrected to body surface area) < 0.15 mm/m2/mL

5.   Fabry disease

=  rare X-linked autosomal recessive metabolic storage disorder caused by lysosomal a-galactosidase

√  concentric thickening of LV wall

√  delayed hyperenhancement of LV midwall on CEMR

6.   Adaptive LV hypertrophy: hypertension, aortic stenosis

CARDIOMEGALY

1.   CHF

2.   Multivalvular disease

3.   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

Falsely normal:   with LV enlargement in up to 66%, moderate enlargement of LA + RV

Falsely elevated:   in expiration, in recumbent position

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

A.  NONCARDIOGENIC

(a)  metabolic:

1.   Ion imbalance in serum levels of sodium, potassium, and calcium

2.   Hypoglycemia

(b)  decreased ventilation

1.   Asphyxia

2.   Transient tachypnea

3.   Perinatal brain damage

(c)  erythrocyte function

1.   Anemia

2.   Erythrocythemia

(d)  endocrine

1.   Glycogen storage disease

2.   Thyroid disease: hypo- / hyperthyroidism

(e)  infant of diabetic mother

(f)  arteriovenous fistula

1.   Vein of Galen aneurysm

2.   Hepatic angioma

3.   Chorioangioma

B.  CARDIOGENIC

1.   Arrhythmia

2.   Myo- / pericarditis

3.   Cardiac tumor

4.   Myocardial infarction

5.   Congenital heart disease

Abnormal Heart Chamber Dimensions

A.  LEFT VENTRICULAR VOLUME OVERLOAD

1.   VSD

2.   PDA

3.   Mitral incompetence

4.   Aortic incompetence

B.  LEFT VENTRICULAR HYPERTROPHY

1.   Coarctation

2.   Aortic stenosis

C.  RIGHT VENTRICULAR VOLUME OVERLOAD

1.   ASD

2.   Partial APVR / total APVR

3.   Tricuspid insufficiency

4.   Pulmonary insufficiency

5.   Congenital / acquired absence of pericardium

[6.  Ebstein anomaly] – not truly RV

D.  RIGHT VENTRICULAR HYPERTROPHY

1.   Pulmonary valve stenosis

2.   Pulmonary hypertension

3.   Tetralogy of Fallot

4.   VSD

E.  Fixed subvalvular aortic stenosis

F.  Hypoplastic left / right ventricle, common ventricle

G.  Congestive cardiomyopathy

Right Atrial Enlargement

Cause:    tricuspid stenosis / regurgitation, ASD, atrial fibrillation, dilated cardiomyopathy, Ebstein anomaly, pulmonary atresia

◊  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

Cause:    pulmonary valve stenosis, cor pulmonale, ASD, tricuspid regurgitation, dilated cardiomyopathy, secondary to LV failure

◊  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 mainstem bronchus 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 (most sensitive view):

√  filling of retrosternal air space by prominent convexity of anterior heart border > ⅓ of distance from sternodiaphragmatic angle to the point where trachea meets sternum

Left Atrial Enlargement

Cause:

(a)  acquired: mitral stenosis / regurgitation, LV failure, LA myxoma

(b)  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

√  enlarged left-convex left atrial appendage ± calcifications (← rheumatic heart disease in 90%)

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:

(a)  pressure overload: hypertension, aortic stenosis

(b)  volume overload: aortic or mitral regurgitation, VSD

(c)  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)

Flattening / Inversion of Interventricular Septum

=  position determined by pressure difference between RV + LV

√  normally convex shape with slight right-sided bulge during ventricular filling (LV diastolic pressure > RV pressure)

(a)  RV volume / pressure overload:

1.   ASD

2.   Acute / chronic cor pulmonale

(b)  pericardial abnormality:

1.   Cardiac tamponade (↑ pericardial pressure)

2.   Constrictive pericarditis (↓ pericardial compliance)

Enlargement of Coronary Sinus

Conditions Causing RA Dilatation

1.   Tricuspid stenosis / regurgitation

2.   Right ventricular dysfunction (failure): CHF, cardiomyopathy, atrial fibrillation, atrioventricular node reentrant tachycardia

3.   Severe pulmonary hypertension

4.   Right atrial hypertension

5.   Cardiac tamponade

Congenital Structural Anomaly

(a)  increased volume of systemic venous blood

(1)  persistent left SVC draining into coronary sinus

(2)  interrupted IVC with hemiazygos continuation to a left SVC / hepatic veins connecting directly into coronary sinus

(b)  L-to-R shunt: oxygenated blood

›  low-pressure shunt:

1.  Interatrial shunt (= coronary sinus-ASD) LA → unroofed coronary sinus

2.  TAPVR

›  high-pressure shunt:

3.  Coronary arteriovenous fistula

Small Coronary Sinus

A.  ACQUIRED

1.   Atrial systole

2.   Lipomatosis

3.   Small heart

4.   Stenosis

B.  CONGENITAL

1.   Atresia

2.   Hypoplasia: corrected TGA

3.   Unroofed coronary sinus

4.   CHD: corrected TGA, tetralogy of Fallot, Ebstein

Neonatal Cardiac Failure

A.  LEFT-SIDED OBSTRUCTIVE LESIONS

1.   Segmental hypoplasia of aorta

2.   Critical coarctation of the aorta

3.   Aortic valve stenosis

4.   Asymmetrical septal hypertrophy / hypertrophic obstructive cardiomyopathy

5.   Mitral valve stenosis

6.   Cor triatriatum

B.  VOLUME OVERLOAD

1.   Congenital mitral valve incompetence

2.   Corrected transposition with left (= tricuspid) AV valve incompetence

3.   Congenital tricuspid insufficiency

4.   Ostium primum ASD

C.  MYOCARDIAL DYSFUNCTION / ISCHEMIA

1.   Nonobstructive cardiomyopathy

2.   Anomalous origin of LCA from pulmonary trunk

3.   Primary endocardial fibroelastosis

4.   Glycogen storage disease (Pompe disease)

5.   Myocarditis

D.  NONCARDIAC LESIONS

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

2.   Transient tachypnea of the newborn

3.   Intraventricular / subarachnoid hemorrhage

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

5.   Thyrotoxicosis (transplacental passage of LATS hormone)

Congestive Heart Failure & Cardiomegaly

mnemonic:   Ma McCae & Co.

Myocardial infarction

anemia

Malformation

cardiomyopathy

Coronary artery disease

aortic insufficiency

effusion

Coarctation

Congenital Cardiomyopathy

mnemonic:   CAVE GI

Cystic medial necrosis of coronary arteries

Aberrant left coronary artery / Absent coronary artery

Viral myocarditis

Endocardial fibroelastosis

Glycogen storage disease (Pompe)

Infant of diabetic mother / Ischemia

Delayed Myocardial Hyperenhancement on MRI

A.  Ischemic heart disease (MI)

B.  Nonischemic

1.   Dilated / hypertrophic cardiomyopathy

2.   Myocarditis

3.   Sarcoidosis

4.   Systemic sclerosis

5.   Endocardial fibroelastosis

6.   Fabry disease

7.   Chagas disease

8.   RV pressure overload

9.   Amyloidosis

10.   Cardiac transplantation

11.   Uremia

Subendocardial Enhancement

1.   Ischemic heart disease

2.   Amyloidosis

3.   Löffler endocarditis (hypereosinophilic syndrome)

Transmural Enhancement

1.   Ischemic heart disease

2.   Myocarditis

Subepicardial Enhancement

1.   Myocarditis

Mesocardial Enhancement

=  delayed enhancement in midinterventricular septum

1.   Hypertrophic cardiomyopathy

2.   Dilated cardiomyopathy

Nodular Patchy Enhancement

1.   Amyloidosis

2.   Myocarditis

3.   Cardiac sarcoidosis

Cardiac Accumulation of FDG

Myocardial Uptake

1.   Physiologic FDG uptake ← depending on levels of blood glucose and free fatty acids

√  none / diffuse / focal uptake in walls of ventricles

Remedy:   uptake suppressed by low-carbohydrate dinner followed by 12-hour fast

2.   Drug interaction

(a)  lowering uptake:

›  Bezafibrate for treating hyperlipidemia

›  Levothyroxine (thyroid hormone)

(b)  increasing uptake:

›  benzodiazepines: Diazepam

3.   Sarcoidosis

√  hypermetabolic myocardial foci in nonperivascular distribution

4.   Biventricular chemotherapy-induced cardiomyopathy

√  increased metabolic activity in ventricular myocardium

5.   Ventricular enlargement associated with systemic + pulmonary hypertension and valvular heart disease

√  diffuse uniform increase in myocardial activity

6.   Inflammatory myocarditis

7.   Endocarditis ← infected valve

8.   Neoplasia: sarcoma, metastasis (breast, lung, melanoma, lymphoma), myxoma

9.   Coronary artery disease ← shift from fatty acid metabolism to glucose utilization ← ischemia

ATRIAL UPTAKE

1.   Atrial fibrillation

2.   Radiofrequency ablation for atrial fibrillation

3.   Crista terminalis: ? physiologic

Pericardial Uptake

1.   Post–radiation pericarditis

2.   Malignant pericardial effusion

3.   Epipericarditis

4.   Lipomatous hypertrophy interatrial septum

CARDIAC TUMOR

◊  Extremely rare; often asymptomatic 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

Location:   intracavitary (obstruction, emboli), intramural (arrhythmia), pericardial (tamponade)

CXR:

√  cardiomegaly, pericardial effusion

√  signs of CHF

√  abnormal cardiac contour

√  pleural effusion

DDx:   thrombus, pericardial and bronchogenic cyst, intrathoracic neoplasm, gastrointestinal hernia

Primary Heart Tumor

Prevalence:   0.002–0.250%; 0.001–0.030% (autopsy series)

Benign Heart Tumor in Adults (75%)

◊  More common than malignant neoplasms

1.   Myxoma (with almost 50% the most common primary cardiac tumor; 25% of all cardiac tumors)

2.   Papillary fibroelastoma (10% of all primary cardiac tumors; with 75% most common valvular tumor)

3.   Lipoma (10% of all cardiac tumors)

4.   Hydatid cyst (uncommon):

√  localized bulge of left cardiac contour

√  curvilinear / spotty calcifications (resembling myocardial aneurysm)

Cx:  may rupture into cardiac chamber / pericardium

Malignant Heart Tumors (25%)

Prevalence:   25% of all cardiac tumors in adults;
10% of all cardiac tumors in children

1.   Sarcomas

2.   Rhabdomyosarcoma

3.   Lymphoma (rare)

4.   Pericardial mesothelioma

5.   Malignant teratoma

6.   Multiple cardiac myxomas

Secondary (Metastatic) Heart Tumor

◊  Most common cause of a neoplastic cardiac mass!

Age:     typically > 60 years with disseminated tumor

Prevalence:   metastatic÷primary disease = 30÷1; 10–12% of cancer patients develop metastases to the heart (autopsy)

Origin:   lung, breast, esophagus, melanoma, lymphoma, leukemia

◊  Melanoma has the highest prevalence of cardiac seeding of any neoplasm!

•  asymptomatic (frequently)

•  arrhythmia (most common clinical sign)

•  chest wall pain ← transient ischemic attack

•  CHF (shortness of breath, fatigue, peripheral edema)

Site:

(a)  peri- / epicardium (most common): lung > breast > lymphoma > leukemia > breast > esophagus

(b)  myocardium: malignant melanoma; secondary extension to myocardium from epicardium

◊  Most common sites for myocardial lesions are LV free wall + interventricular septum (= greatest myocardial mass)

(c)  endocardial / intracavitary (in only 5%)

(d)  transvenous extension: RCC, hepatoma, adrenal adenocarcinoma

√  nonspecific imaging features

ECHO:   iso- / hyperechoic

US/CT/MR:   enhancement

MR:   T1-hypointense + T2-hyperintense

Exception:   T1-hyperintensity of melanoma, hemorrhagic lesion

Prognosis:  death (⅓) ← cardiac tamponade / CHF / coronary artery invasion, sinoatrial node invasion

Cavoatrial Tumor Extension

=  neoplasm arising in infradiaphragmatic sites

1.   Hepatocellular carcinoma
extension into hepatic veins / IVC (7.5%), into RA (4%)

2.   RCC
invasion of renal vein / IVC (5–15%), into RA (1%)

4.   Wilms tumor
into renal veins / IVC (4–10%), into RA (1%)

5.   Adrenocortical carcinoma

6.   Uterine leiomyoma

7.   Leiomyosarcoma of IVC

8.   Osteogenic sarcoma of pelvis

N.B.:   sudden death may occur when tumor in RA intermittently obstructs tricuspid valve leading to low cardiac output

Congenital Cardiac Tumor

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