Presentation This is a 33-year-old African American lady who presented with exertional dyspnea. She presented at 3 months of age with wheezing and cough and was noted to have cardiomegaly on chest x-ray. She was treated with diuretics and digoxin. Cardiac catheterization, done at age 2 years, reportedly showed both coronary arteries. At the age of 16 years, when we first saw her, she was asymptomatic and was able to participate in high school activities and athletics without problems.
Diagnostic Tests EKG at 24 years age showed a Q wave of 7 mm and QR pattern in lead 1. Echo at 32 years showed normal left ventricular (LV) function, unusual thickening of the mitral valve (MV) leaflets, and LV endocardium. MV prolapse and mild mitral regurgitation (MR) were noted. She also had tricuspid regurgitation, pulmonary hypertension, and pulmonary regurgitation on echocardiogram. Cardiac catheterization showed reflux (arrowhead in Figure) of contrast from right coronary artery (RCA) via collaterals into left coronary artery (LCA) and into pulmonary artery (PA). LCA could not be cannulated from the aorta. Shunt series revealed a mild step up in oxygen saturation (PaO2) between the right ventricle (RV) and the main PA (PaO2 61% in RV and 65% in PA).
Discussion The patient has a congenital anomalous origin of LCA from PA (ALCAPA). After transposition of LCA from PA to aorta along with MV repair, her symptoms improved remarkably. Q wave and QR pattern on EKG, lead 1 also disappeared. Echo several months post surgery showed hypokinesis in left anterior descending artery distribution and persistent thickening of LV endocardium.
Conclusion Presence of symptoms or signs of cardiac ischemia, heart failure, MR, and endocardial fibroelastosis need to point us toward ALCAPA as a possibility, especially in pediatric, adolescent, or sometimes adult patients. Q wave depth of more than 3 mm, Q wave width greater than 30 ms, and a QR pattern in at least one of leads I, aVL, V5-V7, is present in 100% of EKGs of patients with ALCAPA. Further evaluation with coronary angiography, SpO2 assessments in cardiac chambers and prompt treatment with transposition of anomalous coronary artery to aorta is indicated.
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