Surgically Constructed Double-Outlet Right Ventricle.

Abstract

HomeCirculationVol. 133, No. 8Surgically Constructed Double-Outlet Right Ventricle Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBSurgically Constructed Double-Outlet Right Ventricle Alexander C. Egbe, MD, MPH, Heidi M. Connolly, MD, FACC, Muhammad Y. Qureshi, MBBS and Hartzell V. Schaff, MD, FACC Alexander C. EgbeAlexander C. Egbe From Division of Cardiovascular Diseases (A.C.E., H.M.C.), Division of Pediatric Cardiology (M.Y.Q.), and Division of Cardiovascular Surgery (H.V.S.), Mayo Clinic, Rochester, MN. Search for more papers by this author , Heidi M. ConnollyHeidi M. Connolly From Division of Cardiovascular Diseases (A.C.E., H.M.C.), Division of Pediatric Cardiology (M.Y.Q.), and Division of Cardiovascular Surgery (H.V.S.), Mayo Clinic, Rochester, MN. Search for more papers by this author , Muhammad Y. QureshiMuhammad Y. Qureshi From Division of Cardiovascular Diseases (A.C.E., H.M.C.), Division of Pediatric Cardiology (M.Y.Q.), and Division of Cardiovascular Surgery (H.V.S.), Mayo Clinic, Rochester, MN. Search for more papers by this author and Hartzell V. SchaffHartzell V. Schaff From Division of Cardiovascular Diseases (A.C.E., H.M.C.), Division of Pediatric Cardiology (M.Y.Q.), and Division of Cardiovascular Surgery (H.V.S.), Mayo Clinic, Rochester, MN. Search for more papers by this author Originally published23 Feb 2016https://doi.org/10.1161/CIRCULATIONAHA.115.019312Circulation. 2016;133:765–767A 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.Download figureDownload PowerPointFigure 1. A, Computed tomography angiogram performed before the first surgical intervention, showing severe calcification (arrow) in the area of the right pulmonary artery. B, Three-dimensional reconstructed image of MRI after initial surgery (insertion of conduit from right ventricle to right pulmonary artery). The artifact from the sternal wires precludes assessment of ventricular end of the conduit. A indicates anterior; LPA, left pulmonary artery; MPA, main pulmonary artery; P, posterior; and RPA, right pulmonary artery.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.Download figureDownload PowerPointFigure 2. Computed tomography angiogram after 15 years of the initial surgery showing calcification of the conduit with significant stenosis at the ventricular and the arterial ends, in maximum-intensity projection in near-axial plane (A) and 3-dimensional reconstructed image (B). A indicates anterior; F, feet; H, head; LPA, left pulmonary artery; MPA, main pulmonary artery; P, posterior; and RPA, right pulmonary arteryAt 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.Download figureDownload PowerPointFigure 3. Three-dimensional reconstruction of computed tomography angiogram after revision of the conduit from right ventricle to right pulmonary artery showing 2 outlets from the right ventricle: the native pulmonary valve and main pulmonary artery supplying blood to the left pulmonary artery and the valved conduit supplying blood to the right pulmonary artery. F indicates feet; H, head; L, left; LPA, left pulmonary artery; MPA, main pulmonary artery; R, right; and RV, right ventricle.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.1Download figureDownload PowerPointFigure 4. Computed tomography angiogram showing small pulmonary embolism (arrow) in the left lower and lingular branches of pulmonary artery. A indicates anterior; and P, posterior.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.115.019312/-/DC1.Correspondence to Hartzell V. Schaff, MD, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail [email protected]