Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman

Abstract

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and adult sudden death. As it predominantly presents in the first year of life, diagnosis in living adults is extremely rare. Current management is based on limited case series or extrapolated from pediatric cases. Modern advances in noninvasive cardiac imaging have substantially increased the number of diagnoses, uncovering a large adult population that has not been reviewed.

Hypothesis: The availability of newer diagnostic modalities correlates with an increasing incidence in an older cohort, and true association between sudden death and ALCAPA may be lower, especially among older patients.

Methods: A comprehensive literature search was performed for all case reports of ALCAPA on MEDLINE and PubMed using the keywords ALCAPA, Bland-White-Garland, and coronary anomaly; and augmented by references from published case reports from 1908 to 2008. All adult cases, defined by age 18 years and older, were reviewed for this article.

Results: One hundred fifty-one adult cases of ALCAPA are described, in addition to the case of an asymptomatic 53-year-old woman. The average reported age was 41 years old with the oldest being 83. Sixty-six percent of the patients presented with symptoms of angina, dyspnea, palpitations, or fatigue; 17% presented with ventricular arrhythmia, syncope, or sudden death; and 14% were asymptomatic. Twelve percent were diagnosed at autopsy. The majority had some form of surgical correction during their clinical course.

Conclusions: ALCAPA is a rare and life-threatening condition in adults. The availability of newer, less invasive diagnostic modalities has resulted in more frequent identification of this condition in an older cohort.

Figure 1

Coronary angiography at 30 degrees left anterior oblique and 20 degrees right anterior oblique. (A) Poor opacification of a dilated and tortuous right coronary artery (RCA) shows a patent vessel with extensive posterior and apical collateralization. (B) Retrograde filling of the left anterior descending artery and main pulmonary artery are suggested on delayed imaging after contrast injection of the RCA.

Figure 2

Multislice computed tomographic coronary angiography (Philips Medical Systems, Cleveland, OH). (A) Anterior volumetric rendering demonstrates a dilated right coronary artery (RCA; arrow) originating from the right coronary cusp of the aorta and passing under the right atrial appendage (^*). Extensive collateral vessels are present across the free wall of the right ventricle. The left anterior descending artery (LAD) (arrowhead) is also dilated, although not quite as large as the massively dilated RCA. (B) Volumetric rendering of the apex of the heart from the right side demonstrates a tortuous and dilated RCA (white arrow) that supplies a dilated right posterior descending artery (RPDA) (black arrow) with extensive apical collateralization (black arrowhead) to the wrap‐around LAD artery (white arrowhead). (C) Volumetric rendering in a left anterior oblique (LAO) projection demonstrates the dilated LAD artery (arrowhead) coursing around the left side of the pulmonary artery (PA) toward its origination from the posterior aspect of the main PA. (D) Sagittal maximum intensity projection view demonstrates the origin of the dilated left coronary artery (arrowhead) from the posterior aspect of the main PA. (E) Left circumflex artery branches (black arrowheads) are seen crossing over the inferolateral wall of the left ventricle to anastomose with branches of the RPDA (black^*) and posterior left ventricular branches from the distal RCA (white^*).

Figure 3

Time periods and modalities of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) diagnoses.