Download S2006_74.DOC ENDOCARDIAL FIBROELASTOSIS

S2006_74.DOC
ENDOCARDIAL FIBROELASTOSIS AND MITRAL REGURGITATION IN A YOUNG WOMAN
H. Bogabathina, P. Manchikalapati, T.J. Wool, J.H. Halanych, K.J Wool. UAB Montgomery Internal
Medicine Residency Program; Montgomery AL.
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 7mm 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: 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 towards ALCAPA as a possibility, especially in
pediatric, adolescent or sometimes adult patients. Q wave depth of more than
3mm, 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, Sp02 assessments in cardiac
chambers and prompt treatment with transposition of anomalous coronary
artery to aorta is indicated.