Heart Lesions, Congenital

Anomalous Left Coronary Artery

• • Left coronary artery (LCA) arises from pulmonary artery (PA)

  • Causes myocardial ischemia, heart failure, myocardial infarction (MI), heart dilation, and fibrosis

  • R coronary normal, supplies entire myocardium, retrograde into L coronary causing large coronary steal

Pulmonary Arteriovenous Fistula (PAF)

• • Rare anomaly, associated with Rendu-Osler-Weber syndrome in 50%

  • Large AV fistulas not communicating with alveolar capillaries

  • Most common in lower lobes

  • Pulmonary blood shunts into veins causing cyanosis

Persistent Left Superior Vena Cava (LSVC)

• • LSVC connects left jugular and subclavian veins to coronary sinus

  • Relatively common anomaly

Endocardial Fibroelastosis

• • Nonoperable lesion

  • Associated w/ aortic coarctation, aortic stenosis, anomalous LCA, mitral valve disease

  • Hyperplasia of subendocardial elastic and collagenous tissue and proliferation of capillaries cause markedly thickened and smooth glistening lining of LV wall

  • Trabeculae obliterated, papillary muscles and chordae contracted

  • Affects principally LV and LA

  • May result from subendocardial ischemia in utero

  • Affects 1-2% of patients with congenital heart disease, often without other disease

Cardiac Tumors

• • Uncommon; primary tumors more common than metastatic lesions

  • > 90% are benign

  • Rhabdomyoma: Most common benign tumor

  • -Most arise from ventricular septum

    -Pale, gray nodules

    -Associated with tuberous sclerosis in 50%

  • Fibroma: Second most common benign tumor

  • -More common in ventricular septum

    -Solid white lesion with distinct borders

  • In infants, teratoma more common than fibroma; hamartoma also common

  • Myxoma rare in children

  • Malignant tumors rare; malignant teratoma and sarcoma most common

Epidemiology

PAF

• • 50% associated with multiple telangiectases (Rendu-Osler-Weber syndrome)

Cardiac Tumors

• • 0.002-0.008% of patients, uncommon in infancy and childhood

Symptoms and Signs

LCA

• • Pallor, sweating

  • Tachycardia, episodic chest pain suggesting angina pectoris

PAF

• • Dyspnea, cyanosis, right heart failure occasionally in infants

  • Cyanosis, clubbing, polycythemia in late childhood

  • Sometimes soft systolic/continuous murmur

LSVC

• • Usually asymptomatic

Endocardial Fibroelastosis

• • Symptoms and signs of left heart failure

Cardiac Tumors

• • Symptoms due to space occupying lesion or involvement of conduction system

  • Include failure, outflow tract obstruction, arrhythmias

Imaging Findings

PAF

• • Chest x-ray: Irregular opacified lesion in peripheral lung fields at site of fistula

  • Angiography/chest CT: Confirm diagnosis

LCA

• • Echocardiography and catheterization with angiography make diagnosis

Cardiac Tumors

• • Echocardiography, ...