ALCAPA in an Octogenarian Woman: An Enigma

Abstract

ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery) is an exceedingly rare disease but lethal with clinical expression from myocardial infarction, congestive heart failure to death during early infancy and rare survival to adulthood. A 75-year-old woman with ALCAPA syndrome presented with angina (Canadian Cardiovascular Society functional class II) over past 8 months. Physical examination was within normal limits except pan-systolic murmur at the apex. Electrocardiography displayed poor R progression in precordial leads and signs of left ventricular hypertrophy. Echocardiography established presence of continuous flow entering the pulmonary trunk and normal systolic function. Coronary angiogram showed absence of left coronary artery originating from aorta, dilated and tortuous right coronary artery (RCA) and abundant Rentrop grade 3 intercoronary collateral communicating with left coronary artery originating from pulmonary trunk which was also confirmed on CT angiogram thus establishing diagnosis of ALCAPA. Survival in eight decade with this anomaly is still an enigma.

Keywords: ALCAPA syndrome; Arrythmia; Collateral circulation; Ischemic cardiomyopathy.

Figure 1

Transthoracic echo-parasternal short-axis view showing presence of continuous flow entering the pulmonary trunk.

Figure 2

RAO (right anterior oblique) view of dilated and tortuous right coronary artery.

Figure 3

RAO (right anterior oblique) view of late phase showing abundant intercoronary anastomoses (Rentrop grade 3 intercoronary collateral) communicating with left coronary artery.

Figure 4

RAO (right anterior oblique) view of LAD and LCX arteries opacifying the LMCA and its origin from the main pulmonary artery.

Figure 5

Reconstructed 3D CT angiogram showing dilated and tortuous right coronary artery with abundant intercoronary anastomoses communicating with left coronary artery and absence of its origin from aorta.