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

Published online by Cambridge University Press:  22 January 2020

Amr A. Matoq
Affiliation:
Nemours Cardiac Center, Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USA
Takeshi Tsuda*
Affiliation:
Nemours Cardiac Center, Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USA Department of Pediatrics, Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, PA 19107, USA
*
Author for correspondence: T. Tsuda, MD, FAAP, FACC, Nemours Cardiac Center, Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Rd, Wilmington, DE 19803, USA. Tel: (302) 651-6677; Fax: (302) 651-6601; E-mail: [email protected]

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.

Type
Original Article
Copyright
© Cambridge University Press 2020

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