Anomalous Left Coronary Artery From the Pulmonary Artery Clinical Presentation

Updated: Aug 10, 2020
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) usually do well for a short period then gradually become fussy and irritable. Typically, they may display pallor, irritability, and diaphoresis after feeding, which are often attributed to colic.

Signs and symptoms of congestive heart failure, including tachypnea, tachycardia, diaphoresis, and poor feeding, eventually ensue, leading to poor weight gain. Usually no obvious evidence of a systemic illness is noted.

In rare instances, children outgrow these symptoms and gradually become asymptomatic, although periodic dyspnea, angina pectoris, syncope, or sudden death may still occur in adulthood.


Physical Examination

If congestive heart failure (CHF) is present, the infant appears distressed and exhibits tachypnea, tachycardia, diaphoresis, and irritability.

Auscultation may demonstrate a systolic murmur of mitral valve regurgitation and, possibly, a diastolic rumble of relative mitral stenosis best located at the apical left precordial region. A moderate third heart sound may be heard.

Rarely, a soft continuous murmur may be detected at the upper left sternal border that is reminiscent of a coronary artery fistula or a small patent ductus arteriosus.

The left ventricular precordial impulse may appear prominent and displaced both inferiorly and laterally.

The second heart sound may seem narrowly split with increased intensity of the pulmonic component, if left ventricular failure causes pulmonary artery hypertension secondary to elevated left atrial pressure.

In cases of severe CHF, hepatic enlargement may be observed, and the peripheral pulses may be diminished in intensity secondary to low cardiac output.