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Effective myocardial perfusion and concomitant haemodynamic status determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.

Abstract：

BACKGROUND:Anomalous left coronary artery from the pulmonary artery is a rare congenital heart disease (CHD) with diverse clinical presentations despite the same anatomy. Factors determining this heterogeneous presentation are not well understood. METHOD AND RESULTS:We retrospectively investigated 14 patients (12 females) who underwent surgical repair of anomalous left coronary artery from the pulmonary artery. These patients were divided into three groups based upon the severity of initial presentation: (1) severe, life-threatening condition (n = 5), (2) mild-to-moderate distress (n = 6), and (3) asymptomatic (n = 3). All patients presented with left ventricular dilation and retrograde flow in left coronary artery by echocardiogram. Eight patients in (1) and (2) presented with severe left ventricular dysfunction. All but one showed abnormal ECG consistent with myocardial ischemia or infarction. Asymptomatic patients had preserved left ventricular systolic function despite ischemic findings on ECG. In 13 patients after surgical repair, all but one normalised left ventricular geometry and systolic function, suggesting nearly full myocardial recovery upon improvement of myocardial perfusion; 8 patients had residual echogenic papillary muscle with variable degree of mitral regurgitation. CONCLUSIONS:Evidence of myocardial ischemic injury was present in all patients with anomalous left coronary artery from the pulmonary artery regardless of their initial presentation. Retrograde flow in left coronary artery, implying collateral vessel development from right coronary artery to left coronary artery, was noted in all patients, yet only few patients had preserved systolic function at the time of diagnosis. The balance between effective myocardial perfusion and a deleterious fistulous flow provided by these collaterals and the simultaneous haemodynamic status are what determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.

journal_name

Cardiol Young

journal_title

Cardiology in the young

authors

Matoq AA,Tsuda T

doi

10.1017/S1047951119003299

subject

Has Abstract

pub_date

2020-03-01 00:00:00

pages

362-368

issue

eissn

1047-9511

issn

1467-1107

pii

S1047951119003299

journal_volume

pub_type

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Effective myocardial perfusion and concomitant haemodynamic status determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.