A 3-year-old boy underwent evaluation for dextrocardia. Echocardiograms showed features of corrected transposition physiology, a perimembranous ventricular septal defect (VSD) (Fig. 1), and aneurysmal tissue beneath the pulmonary valve that caused severe subpulmonary stenosis (Figs. 2–4). Eighteen months after VSD closure and resection of the aneurysmal tissue, the patient was asymptomatic with only mild residual pulmonary outflow tract obstruction.
Fig. 1.
Two-dimensional echocardiograms A) in apical 4-chamber view from the right chest and B) in color-flow Doppler mode show a moderate-to-large ventricular septal defect (VSD).
MLV = morphologic left ventricle; MRV = morphologic right ventricle
Supplemental motion image (3.2MB, mp4) is available for Figure 1B.
Fig. 2.
Echocardiograms (subcostal 4-chamber views). A) In the morphologic left ventricle (MLV), aneurysmal tissue (arrow) protrudes into the pulmonary outflow tract. B) Color-flow Doppler mode reveals turbulent flow (arrow) in the pulmonary outflow tract.
Supplemental motion image (2.9MB, mp4) is available for Figure 2A.
Fig. 3.
A) Continuous-wave Doppler echocardiographic recording across the pulmonary outflow tract shows a peak velocity >5 m/s, suggesting severe obstruction. B) Angiogram (60° left anterior oblique view) from the morphologic left ventricle (MLV) shows pulmonary outflow tract obstruction from aneurysmal tissue (arrows), the ventricular septal defect (arrowhead), and poststenotic dilation of the main pulmonary artery (MPA). A pigtail catheter (PC) is in the descending aorta.
Fig. 4.
Cineangiographic image from the A) left lateral view reveals the morphologic left ventricle (MLV) and the pulmonary outflow tract obstruction from aneurysmal tissue (arrows). B) The 60° left anterior oblique view reveals a severely narrowed right ventricular outflow tract (arrows). Both frames show poststenotic dilation of the main pulmonary artery (MPA) and a pigtail catheter (PC) in the descending aorta.
Supplemental motion image (3.3MB, mp4) is available for Figure 4B.
Comment
Congenitally corrected transposition of the great arteries (TGA) is usually associated with multiple cardiac defects. The hallmark finding is atrioventricular and ventriculoarterial discordance. Because of this double discordance, the circulatory physiology is normal: systemic venous return goes to the lungs, and pulmonary venous return goes to the body.1,2 The usual anatomic arrangement is levocardia, visceroatrial situs solitus, L-loop ventricular inversion, and an anterior aorta on the left of the pulmonary artery {S,L,L}. Our patient's anatomy was rarer: dextrocardia with situs inversus, D-loop of the ventricles, and a rightward anterior aorta {I,D,D}.
Prolapsing aneurysms of a membranous ventricular septum rarely cause left ventricular (LV) outflow tract obstruction (pulmonary obstruction) in patients who have normally related great vessels.2–5 However, in patients with TGA who have higher right ventricular pressure, such an aneurysm can protrude into the LV outflow tract and cause pulmonary outflow tract obstruction.6 Similarly, in patients with corrected TGA (who lack a conal septum and crista supraventricularis in the morphologic LV), even the proximity of a small aneurysm to the pulmonary valve can cause pulmonary outflow tract obstruction.
Doppler echocardiography and cardiac catheterization with selective cineangiography help to define the lesions and are the diagnostic tests of choice. Surgical aneurysm resection and VSD patch closure—with care to avoid injuring the vulnerable conduction system—is recommended.2–5
Supplementary Material
Acknowledgments
We thank Dr. William I. Douglas for his contribution to the clinical care of this patient.
Footnotes
Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute, 6624 Fannin St., Suite 2480, Houston, TX 77030
From: Department of Pediatrics, Division of Pediatric Cardiology, University of Texas at Houston Medical School and Children's Memorial Hermann Hospital, Houston Texas 77030
Dr. Yarrabolu is now at the Department of Pediatrics, Division of Pediatric Cardiology, Texas Tech University Health Science Center, Amarillo, Texas.
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