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Anomalous Left Coronary Artery

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  • • 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

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Pulmonary Arteriovenous Fistula (PAF)

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  • • 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

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Persistent Left Superior Vena Cava (LSVC)

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

    • Relatively common anomaly

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Endocardial Fibroelastosis

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  • • 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

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Cardiac Tumors

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  • • 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

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Epidemiology

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PAF

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

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Cardiac Tumors

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

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Symptoms and Signs

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LCA

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  • • Pallor, sweating

    • Tachycardia, episodic chest pain suggesting angina pectoris

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PAF

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

    • Cyanosis, clubbing, polycythemia in late childhood

    • Sometimes soft systolic/continuous murmur

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LSVC

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  • • Usually asymptomatic

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Endocardial Fibroelastosis

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  • • Symptoms and signs of left heart failure

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Cardiac Tumors

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

    • Include failure, outflow tract obstruction, arrhythmias

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Imaging Findings

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PAF

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

    Angiography/chest CT: Confirm diagnosis

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LCA

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  • • Echocardiography and catheterization with angiography make diagnosis

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Cardiac Tumors

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  • • Echocardiography, catheterization, MRI all useful

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  • • Echocardiography

    • Angiography if diagnosis unclear

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LCA

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