Dramatic Left Atrial and Ventricular Hypertrophy in an Adult With Cyanotic Congenital Heart Disease
A 29-year-old man with history of atrial tachyarrhythmias and a remote Potts anastomosis for treatment of tricuspid atresia presented with palpitations and chest pain. His medications included digoxin, furosemide, prednisone, methimazole, and amiodarone. He was afebrile. His pulse was 176 beats per minute with a blood pressure of 160/80 mm Hg. He was cyanotic with clubbed fingers and toes. The apical impulse was displaced to the 6th intercostal space in the mid-axillary line. Auscultation revealed normal S1, single S2, and a short systolic murmur arising from the Potts shunt that was heard over the left precordium and back. No diastolic component was appreciated.
Chest radiography revealed cardiomegaly with prominent pulmonary vasculature and enlarged pulmonary arteries. The admission ECG showed supraventricular tachycardia (SVT) at 176 beats per minute (Figure 1A) that terminated with intravenous adenosine injection. The subsequent ECG in sinus rhythm (Figure 1B) demonstrated marked atrial and left ventricular hypertrophy. The P waves in V1 during tachycardia (Figure 1A) were so large that they were initially misconstrued as being T waves. The QRS voltage was the largest recorded in our laboratory (106 mV in V6). Echocardiography demonstrated findings consistent with tricuspid atresia and a Potts (direct descending aorta to left pulmonary artery) shunt. The left ventricle was markedly dilated and hypertrophied, with an end-diastolic dimension of 60 mm and wall thickness of 18 mm; there was marked bi-atrial enlargement, and right-to-left shunting across the atrial septal defect (Figure 2). Doppler examination of the Potts shunt showed a low velocity systolic flow, which suggested severe pulmonary hypertension. The striking abnormalities in the ECG and the echocardiogram reflect the hemodynamic impact of long-standing aortopulmonary shunting with severe left ventricular volume overload leading to both marked left ventricular hypertrophy and pulmonary plethora that resulted in profound pulmonary hypertension and eventual closure of the shunt.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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