Anomalous Left Coronary Artery From the Pulmonary Artery Differential Diagnoses

Updated: Aug 10, 2020
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Syamasundar Rao Patnana, MD  more...
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Diagnostic Considerations

Important considerations

The most serious, yet avoidable, complication is the mistaking of symptoms in the patient older than several months who presents with anomalous left coronary artery from the pulmonary artery (ALCAPA) as idiopathic dilated cardiomyopathy [9, 10] or end stage myocarditis. [9] The initial presentation may be entirely similar but the treatments are dramatically different. The clinical and initial laboratory findings may be remarkably similar. If there is significant pulmonary artery hypertension, the diagnostic electrocardiographic (ECG) findings may not be apparent. Careful definition of the origin of the coronary arteries by echo-Doppler ultrasonographic studies in every patient presenting with the above clinical scenario should be undertaken. Selective cine-aortography to evaluate the origin of coronary arteries is no longer routinely necessary because of the usefulness of echocardiographic studies.

A high index of suspicion is necessary to make the diagnosis of ALCAPA, as surgical treatment is extremely successful and results in an excellent long-term prognosis.

Special concerns

With successful surgical revascularization and resolution of myocardial dysfunction in female patients, pregnancy and normal vaginal delivery are not contraindicated.

In patients with persistent myocardial dysfunction, mitral valve insufficiency, or coronary ischemia, the ability to carry a pregnancy to term varies. These patients should be considered high risk and require close observation throughout the pregnancy by both a cardiologist and a perinatologist because the increase in blood volume may be deleterious to the patient's hemodynamic status. A normal vaginal delivery may also be contraindicated in this situation.

In a relatively recent study, the pregnancy and delivery outcomes of six patients who had coronary artery reimplantation for ALCAPA were examined. [11] Six women delivered 10 babies, of which 8 babies were delivered vaginally and 2 were by cesarean section because of maternal congestive heart failure (CHF). All did well with the exception of one cesarean section patient, with both maternal and infant complications. The authors concluded that pregnancy is safe in asymptomatic repaired ALCAPA. Pregnant patients with symptomatic CHF secondary to myocardial damage should be carefully monitored and managed. [11]

Differential Diagnoses