P1338 Anomalous left coronary artery from pulmonary artery syndrome diagnosed in adulthood: case of a mother of five
Authors: Szabo L , Borszeki K , Rabai E , Dohy Z , Czimbalmos C , Suhai F I , Toth A , Hartyanszky I , Merkely B , Vago H 
Published: January 2020

Funding Acknowledgements

Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary

Introduction

Bland-White-Garland syndrome or ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital disorder with a prevalence of 1: 300,000. This condition is one the most common causes of infant myocardial infarction. It can ultimately lead to heart failure before the age of one, however when pronounced left and right coronary artery collateralization is present, patients may remain asymptomatic until older age. Due to the development and availability of non-invasive imaging techniques, these patients diagnosed more frequently in recent years.

Case description: We present a 53-year-old patient with treated hypertension and five uncomplicated pregnancies. At the age of 40, the patient was referred to coronary angiography due to her dyspnea on exertion. The examination showed that the left main coronary artery (LMCA) originates from the pulmonary artery 7 mm above the pulmonary valve and the right coronary artery is significantly dilated. Based on the mild complaints and good left ventricle systolic function, conservative therapy was recommended by the cardiac surgery team. Since 2018 the patient has had gradually worsening complaints despite of the therapy adjusted for chronic heart failure. Q-waves were depicted on the 12-lead ECG in aVL and V1-V6 leads. Echocardiography showed dilated left ventricle (LV), LV hypertrophy, diffuse hypokinesia, decreased LV systolic function and diastolic dysfunction. At our clinic, we performed cardiac magnetic resonance imaging (CMR) which showed moderately reduced LV ejection fraction, diffuse LV hypokinesia, increased LV volume, septal LV hypertrophy. We visualized the retrograde flow on the LMCA, the shunt volume at the anomalous artery was 1,27 L/min. The CMR also showed irreversible damage of the subendocardial region of the myocardium via late gadolinium enhancement in the area supplied by the LMCA. Since we planned to reevaluate the surgical possibilities, the collateral network with tortuous and dilated left and right coronary arteries were visualized by coronary CT angiography. The surgical team at our institute decided to perform myocardial revascularization and restoration of dual coronary artery supply.

Discussion

ALCAPA is usually diagnosed in infancy however, in about 10-15% of cases the disease is only recognized in adulthood. The long-term morphological and functional characteristics of the syndrome may include reversible/irreversible ischemic damage, systolic and diastolic dysfunction, mitral regurgitation, significant collateral circulation, left-to-right shunt, dilated coronary arteries. Surgical myocardial revascularization and restoration of dual coronary artery supply is recommended even in adult patients.

Abstract P1338 Figure. CTA reconstruction and LGE CMR