Apico-aortic conduit for severe hypertrophic cardiomyopathy: sudden death despite conduit patency 31 years postoperatively.
Although small experiences have been described with the use of apico-aortic valved conduit in the treatment of hypertrophic cardiomyopathy (HCM), the long-term follow-up has never been previously reported. In a young female patient with symptomatic HCM and a prognostically unfavorable phenotype, apico-aortic conduit was chosen instead of conventional myectomy because severe ventricular hypertrophy involved the whole ventricle, making outflow tract cavity virtually absent in systole. Close clinical and imaging follow-up was postoperatively performed. The patient remained asymptomatic, without cardioactive drug therapy for 30 years, also experiencing 2 successful pregnancies. A striking finding was the perfect patency of the conduit at the last follow-up control (31 years), with computed tomography and echocardiography showing no calcification of the porcine Hancock bioprosthesis inside the graft. Nevertheless, the disease slowly evolved towards the dilative phase and the patient experienced sudden death while scheduled for implantation of defibrillator in waiting list for heart transplant.The present case could suggest that, in selected cases of HCM not treatable by myectomy, apico-aortic conduit may be an option. The relief of the obstruction can provide even long-term freedom from symptoms, however, late evolution to end-stage cannot be prevented.
Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, c/o V. Monaldi Hospital, via L Bianchi, Naples, Italy.
Gianantonio
G
Nappi
Giovanni
G
Dialetto
Marisa
M
De Feo
Raffaela
R
Provenzano
Maurizio
M
Cotrufo
Pasquale
P
Santè
Alessandro
A
Della Corte
D001011:Aorta / Q000503:physiopathology / Q000601:surgery*; D001021:Aortic Valve / Q000503:physiopathology / Q000601:surgery*; D001705:Bioprosthesis; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000295:instrumentation*; D024741:Cardiomyopathy, Hypertrophic, Familial / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D017147:Defibrillators, Implantable; D018450:Disease Progression; D004452:Echocardiography; D004554:Electric Countershock / Q000295:instrumentation; D017809:Fatal Outcome; D005260:Female; D006333:Heart Failure / Q000209:etiology / Q000628:therapy; D016027:Heart Transplantation; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000295:instrumentation*; D006801:Humans; D008875:Middle Aged; D011474:Prosthesis Design; D012720:Severity of Illness Index; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014654:Vascular Patency; D014850:Waiting Lists; D055815:Young Adult