Longest Survivor of Pulmonary Atresia With Ventricular Septal Defect
Well-Developed Major Aortopulmonary Collateral Arteries Demonstrated by Multidetector Computed Tomography
A 59-year-old woman was admitted because of cyanosis and dyspnea on exertion and at rest. In her childhood, she was suspected of having ventricular septal defect (VSD), but she refused to undergo cardiac catheterization and operation. Dyspnea on exertion gradually developed after adolescence.
On admission, chest roentgenography demonstrated enlarged cardiac silhouette with elevated cardiac apex, a right aortic arch, and enlargement of the main pulmonary arteries and their major branches with increased pulmonary arterial vascularity (Figure 1). Echocardiography revealed a large VSD which lay beneath the dilated aorta that overrides the interventricular septum, hypertrophied right ventricle, and the blind outflow tract of the right ventricle (Figure 2 and online-only Data Supplement Movies I and II). These findings suggested pulmonary atresia (PA) with VSD (PA-VSD).
The volume-rendering 64-multidetecter computed tomography (MDCT) images visualized multiple well-developed arterial collaterals, namely major aortopulmonary collateral arteries (MAPCAs) (Figure 3A). The mediastinum was occupied by the multiple MAPCAs, mimicking the cavernous tissue (Figure 3B). Several MAPCAs originated from the descending aorta (Figure 3C).
Pulmonary atresia with VSD is the ultimate form of tetralogy of Fallot and is estimated to represent 5% to 10% of tetralogy of Fallot patients.1 The survival rate without surgical repair is as low as 50% at 1 year of age and 8% at 10 years.2 Adult survivors of PA-VSD are quite rare: Marelli et al reported that the mean life expectancy without operation did not exceed 3 decades,3 and the oldest reported survivors were 54 years old.3,4 To the best of our knowledge, this patient is the oldest reported survivor of PA-VSD.
Survival of PA-VSD patients is dependent on the adequacy of pulmonary blood flow derived from direct or indirect aortopulmonary collateral vessels. The well-developed MAPCAs might have enabled this patient to survive to nearly 60 years of age. Multidetecter computed tomography is a valuable noninvasive imaging modality to evaluate the development and sources of MAPCAs in PA-VSD.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.111.035469/-/DC1
- © 2011 American Heart Association, Inc.
- Perloff J
- Perloff J
- Marelli AJ,
- Perloff JK,
- Child JS,
- Laks H