What is anomalous left coronary artery from the pulmonary artery (ALCAPA)?

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

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but serious congenital cardiac anomaly.

ALCAPA was first described in 1866. The first clinical description in conjunction with autopsy findings was described by Bland and colleagues in 1933, so the anomaly is also called Bland-White-Garland syndrome. [1] In 1962, Fontana and Edwards reported a series of 58 postmortem specimens that demonstrated that most patients had died at a young age. [2]

The prognosis for patients with ALCAPA has dramatically improved as a result of both early diagnosis using echocardiography with color flow mapping and improvements in surgical techniques, including myocardial preservation.

The ALCAPA anomaly may result from (1) abnormal septation of the conotruncus into the aorta and pulmonary artery, or from (2) persistence of the pulmonary buds together with involution of the aortic buds that eventually form the coronary arteries.


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