Surgically Constructed Double-Outlet Right Ventricle
A 32-year-old woman with a history of fibrosing mediastinitis attributable to histoplasmosis presented to Mayo Clinic with occlusion of her right pulmonary artery. She underwent bypass of the occluded pulmonary artery with a 26-mm CryoLife homograft conduit from the right ventricle to the right pulmonary artery. Her postoperative course was uncomplicated, and postoperative cardiac MRI showed a patent right ventricle (RV) to right pulmonary artery homograft conduit, normal perfusion of the lobar branches of the right pulmonary artery, and normal pulmonary venous return (Figure 1). The patient did well after surgery and had an uncomplicated pregnancy 2 years later.
Fifteen years postoperatively, she presented with exertional dyspnea and signs of right heart failure. Chest computed tomography angiogram showed calcification and severe stenosis of the RV–right pulmonary artery conduit (Figure 2). Attempted dilatation of the conduit was unsuccessful. The patient’s clinical condition deteriorated with the development of oliguric acute kidney injury and progressive right heart failure refractory to inotropic support and diuresis. She was transferred to the Mayo Clinic for repeat surgical intervention.
At operation, the RV–right pulmonary artery conduit was replaced with a composite graft constructed with an 18-mm distal graft and a 24-mm proximal graft containing a 21-mm porcine bioprosthesis; also, a DeVega tricuspid annuloplasty was performed. A postoperative chest computed tomography angiogram performed showed a patent RV–right pulmonary artery conduit and an incidental pulmonary embolus located in the left pulmonary artery branches to the lingula (Figures 3 and 4, Movie I in the online-only Data Supplement). The patient was started on systemic anticoagulation and was dismissed home 9 days after surgery.
To our knowledge, this is the first case of surgically constructed double-outflow reconstruction of the RV with each of the outlets supplying a separate branch of the pulmonary artery. The only similar report in the literature is a patient with tetralogy of Fallot with severe RV outflow tract obstruction requiring placement of RV–main pulmonary artery conduit to augment blood flow around the stenotic native pulmonary valve.1
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.115.019312/-/DC1.
- © 2016 American Heart Association, Inc.