Total anomalous origin of the coronary arteries from the pulmonary artery

Abstract

The clinicopathologic features of four patients with total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) are presented and compared with 21 previously reported cases. Patients with TCAPA usually present with left ventricular heart failure, angina, or associated cardiovascular anomalies. Of the 19 patients in whom a clinical history was available, 16 were symptomatic before three days of age. All patients died with 60% dying before two weeks of age. Longer survival was associated with additional cardiovascular anomalies that increased pulmonary arterial perfusion pressure, oxygen saturation, or both. Seventeen (68%) patients had additional cardiovascular anomalies, most commonly atrial (nine cases) or ventricular (eight cases) septal defects and tetralogy of Fallot or other variants of pulmonary atresia (four cases). Only five (22%) of 23 had noncardiovascular anomalies. The coronary arteries arose equally from either one ostium or from two, and the number of ostia was not related to either anomalous coronary artery distribution or to the presence of additional cardiovascular anomalies. Cardiomegaly was present in 56% of cases and the majority of patients had myocardial fibrosis or infarction. Embryology is reviewed and evidence is presented to support the theory of involution-persistence of coronary artery anlagen as the pathogenetic mechanism of TCAPA.

Anomalous left coronary artery from pulmonary artery (ALCAPA) in infants: a 5-year review in a defined birth cohort

“…If an infant presents in cardiac failure with no obvious structural cause, ALCAPA should be suspected and appropriate investigations undertaken. The examination of the coronary arteries by echocardiogram is technically difficult, but there are other findings that may indicate anomalous coronary artery [7]. It is always necessary to exclude ALCAPA in an infant presenting with congestive cardiac failure associated with echocardiogram findings of a dilated left ventricle, depressed left ventricular function and/or mitral regurgitation.…”

Echocardiographic diagnosis of coronary artery fistula in both dizygotic twin brothers

“…The embryological basis of coronary artery to pulmonary artery fistula can be founded on Hackensellner's hypothesis [3]. This theory proposes that there are six buttons in the primitive truncus; two persist in the aortic sinuses and give rise to the coronary arteries, whereas the others revert.…”

A large coronary arteriovenous fistula with MRI and angiographic images

“…CAVF are thought to arise due to the persistence of embryological sinusoidal connections that allow direct communication between the coronary artery and a cardiac chamber or great vessel 1. It is the most commonly encountered congenital coronary artery anomaly during cardiac catheterisation with a reported incidence of approximately 0.1–0.2% 2…”