title: Hybrid Melody pulmonary valve replacement in an adult with severe pulmonary hypertension and pulmonary artery aneurysm.
abstract: A 48-year-old female with D-TGA, ventricular septal defect (VSD), pulmonary stenosis, pulmonary hypertension (PAH), and total anomalous pulmonary venous connection underwent hybrid intervention for a pulmonary artery (PA) aneurysm and replacement of a dysfunctional pulmonary valve (PV). She underwent a hemi-Mustard procedure at 9 years of age but remained cyanotic. She developed atrial fibrillation, heart failure, and functional decline at 43 years of age. A chest CT demonstrated a 6 cm PA aneurysm that upon re-imaging at 48 years had increased to 11 cm. A catheterization procedure revealed severe PS, PR, residual VSD, severe PAH with a pulmonary vascular resistance of 30 Wood units. She was evaluated and turned down for heart-lung transplantation at another institution. She was subsequently referred to our institution for heart-lung transplantation but was felt to be at unacceptably high risk given the complexity of her anatomy, imaging suggesting liver cirrhosis and liver biopsy with extensive fibrosis. After extensive discussion of risk and benefits, the patient agreed to proceed with a hybrid intervention, consisting of surgical aneurysm resection/PA repair, tricuspid valve repair; PV replacement with a Melody valve, and VSD closure. There were no complications and she was discharged home within 2 weeks. Six months post procedure, she is not on oxygen, her resting room air saturation is 94%, and echocardiography shows stable Melody valve function. This case highlights the utility of a hybrid approach in the treatment of an adult with complex congenital heart disease, heart failure and severe PAH, considered at the highest risk for adverse surgical outcomes. The short-term efficacy of the Melody valve in severe PAH is reassuring.
authors: B.S. Physiological Science UCLA, Research Associate Ahmanson/UCLA Adult Congenital Heart Disease Center, Department of Medicine, Division of Cardiology, 650 Charles E. Young Drive South, Los Angeles, California.
authors: Gwendolyn
authors: G
authors: Derk
authors: Hillel
authors: H
authors: Laks
authors: Jamil
authors: J
authors: Aboulhosn
mesh_terms: D000783:Aneurysm / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D006328:Cardiac Catheterization / Q000295:instrumentation*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000295:instrumentation* / Q000379:methods; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D011474:Prosthesis Design; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: congenital; hybrid; melody; pulmonary hypertension; transposition; ventricular septal defect