Echocardiographic ‘brainstorm’ to detect anomalous origin of the left coronary artery from the pulmonary artery

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), so-called Bland-WhiteGarland syndrome, is a rare congenital malformation with a clinical course depending on the extent of collateralization between right coronary artery (RCA) and left coronary artery (LCA). The mortality rate in untreated patients affected by the so-called 'infant type' of ALCAPA is over 90% in the first year of life. Only 10% of patients reach adulthood because of adequate coronary collateralization but are still at high risk of sudden death or congestive heart failure ('older type'). [1][2][3][4][5][6][7][8][9][10] The purpose of this case study is to highlight the suggestive clue elements to detect this rare malformation in adulthood during a routine standard transthoracic echocardiographic exam. Methods and resultsA 38-year-old Caucasian woman with a clinical history of cyanosis during infancy and childhood was referred to our echo laboratory for acute worsening of heart failure symptoms. The standard two-dimensional echocardiographic study alone was able to suggest the diagnosis of ALCAPA according to the following findings: dilated proximal RCA with concordant flow pattern; hypocontractile left ventricle with papillary muscle calcification and functional mitral regurgitation; multiple septal intramyocardial coronary flow signals visualized across the surface of the right ventricle; and an abnormal vessellike structure inserting into the pulmonary trunk with continuous shunt at colour Doppler mapping in the diastolic phase. A transoesophageal echocardiography examination (TEE) was also performed but it did not provide an additional diagnostic value, due to the Research letterFig. 1Parasternal and apical views. (a) Long-axis view of a dilated proximal right coronary artery arising from the aorta. (b) In short-axis view an atypical vessel-like structure (yellow arrows) simulates a dilated left coronary artery arising from the aorta; the thin linear echo-free space is related to the transverse sinus of the pericardium (white arrows). (c) Apical four-chamber view of the markedly calcified heads of antero-lateral papillary muscle (white arrows). Ao, aorta; LV, left ventricle; RCA, right coronary artery.

Case Report: Neonatal Complex Congenital Heart Disease With Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery: Analysis of Missed Diagnosis and Improvement Procedures

“…If combined with PDA or VSD, the pulmonary artery pressure and pulmonary artery oxygen partial pressure will not be significantly reduced, with no secondary changes of myocardial ischemia, and the collateral circulation of the coronary artery will not be formed (9). In this situation, echocardiography can only see the abnormal vascular opening of the pulmonary artery, while there is no opening of the left coronary artery of the aorta (10). The abnormal opening of the left coronary artery can be located at any part of the pulmonary artery.…”

Misdiagnosis of anomalous origin of the left coronary artery from the pulmonary artery by echocardiography: Single‐center experience from China

“…The diagnosis should be considered when the above signs appeared and a jet from an abnormal vessel was found shunting into the root of pulmonary artery. If the abnormal blood vessel was composed of several small branches, it is more suggestive of this disease …”

“…At this time, the presentation of myocardial ischemia had not yet occurred, and the collateral circulation was not formed. The only sign was abnormal vessel opening into the PA. Because of no shunt in the pulmonary artery, the operator could not be alerted, resulting in missed diagnosis . (b) In ALCAPA patients with complications of complete transposition of great arteries, the pressure of well‐developed pulmonary artery was high.…”