A 27-year-old male truck driver presented with recurrent rapid palpitations associated with near-syncope for 6 months. He was noted to have had “isolated” dextrocardia soon after birth. There was no family history of congenital heart disease. Before a herniorrhaphy at age 4 years, a chest radiograph demonstrated anomalous venous drainage of the right lung. He was otherwise asymptomatic, with a normal exercise tolerance.
On examination, he was acyanotic, in sinus rhythm, with a blood pressure of 120/80 mm Hg. His apical impulse was not palpable, and heart sounds were best heard to the right of the sternum. The first heart sound was normal, and the second sound was widely split, with a holosystolic murmur of tricuspid regurgitation.
A chest radiograph showed dextrocardia and a small right lung with a prominent anomalous right pulmonary vein. Two-dimensional echocardiography demonstrated an enlarged right heart but an intact atrial septum by contrast injection. Cardiac-gated MRI showed dextrocardia, a dilated and hypertrophied right ventricle, and a large pulmonary vein that joined the inferior vena cava (IVC) below the level of the diaphragm (Fig 1⇓). Three-dimensional (3D) MR angiography with gadolinium contrast enhancement unequivocally demonstrated that the venous drainage of the whole of the diminutive right lung was by an anomalous vein whose entire course to the suprahepatic IVC was visualized. The arterial supply of the right lung was seen to originate from a normal right pulmonary artery (Figs 2⇓ and 3⇓). A 24-hour ECG monitor revealed recurrent long R-P narrow complex tachycardia, with rates of up to 230 bpm. Cardiac catheterization showed a pulmonary artery systolic pressure of 38 mm Hg and a left-to-right shunt ratio of 1.8:1.0.
The patient underwent electrophysiological testing and atrioventricular nodal modification to control his supraventricular tachycardia. He is off all medications and feeling well, without recurrence of arrhythmia, 8 months after the procedure and is being conservatively managed with respect to his anomalous venous drainage.
The authors acknowledge the contributions of Jeffrey Goldman, MD, and Janice Dawson, RN, and the expert technical assistance of Norman Butler and Tanya Kurtz.
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.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1–267, Houston, TX 77030.
- Copyright © 1998 by American Heart Association