RESUMO
In this report, we describe the unusual but highly informative clinical course of a high-risk hypertrophic cardiomyopathy patient who has to date experienced 10 appropriate primary prevention implantable cardioverter-defibrillator therapies terminating ventricular fibrillation over a 19-year period. Most patients in the Tufts HCM cohort with primary prevention experienced either 1 such therapy (66%) or 1 to 3 interventions (91%) over time. These observations underscore the opportunity for sudden death prevention in hypertrophic cardiomyopathy attributable to efficacy of the implanted defibrillator and the reliability of the American Heart Association/American College of Cardiology individual risk marker strategy.
Assuntos
Cardiomiopatia Hipertrófica , Desfibriladores Implantáveis , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/terapia , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle , Humanos , Reprodutibilidade dos Testes , Medição de Risco , Fatores de RiscoRESUMO
For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in obstructive hypertrophic cardiomyopathy (HCM). However, given the relatively youthful adult ages at which HCM surgery is usually performed, it is informative to evaluate longer-term results of myectomy after ≥10 years. We identified 139 consecutive obstructive HCM patients (50 ± 15 years of age; 55% men) who underwent surgical myectomy, 2003 to 2010 at Tufts HCM Center and followed 11.3 ± 2.7 years (range to 17). Operative mortality was low (0.6%) and left ventricular (LV) outflow gradients at rest were reduced from 56 ± 40 mm Hg preoperatively to 1 ± 7 mm Hg postoperatively, durable over the study period, with no patient requiring reoperation for the residual gradient. Over follow-up, 129 of 139 patients (93%) were alive ≥10 years after myectomy, including 17 patients ≥15 years. Of 118 patients with complete long-term clinical follow-up data, 109 (92%) experienced clinical improvement to New York Heart Association classes I or II. In 9 patients (8%) refractory class III/IV symptoms reoccurred 6.6 ± 3.9 years postoperatively, including 4 who ultimately underwent a heart transplant. After myectomy, there were 2 late HCM-related deaths, but none suddenly; notably 6 patients (12%) with prophylactic implantable cardioverter-defibrillators experienced appropriate therapy terminating ventricular tachycardia/ventricular fibrillation after myectomy. Survival following myectomy was 91% at 10 years (95% confidence interval: 85, 96%) not different from the age- and gender-matched general United States population (log-rank pâ¯=â¯0.64). In conclusion, myectomy provides permanent abolition of outflow gradients with reversal of heart failure and highly favorable long-term survival, representing a low-risk:high-benefit option when performed in experienced HCM centers. Myectomy did not protect absolutely against arrhythmic sudden death events, underscoring the importance of risk stratification in operative patients.
Assuntos
Cardiomiopatia Hipertrófica/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Septo Interventricular/cirurgia , Adulto , Idoso , Cardiomiopatia Hipertrófica/complicações , Cardiomiopatia Hipertrófica/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Anuloplastia da Valva Mitral , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/cirurgia , Modelos de Riscos Proporcionais , Volume Sistólico , Resultado do Tratamento , Obstrução do Fluxo Ventricular Externo/complicações , Obstrução do Fluxo Ventricular Externo/fisiopatologiaRESUMO
BACKGROUND: For over 50 years, surgical septal myectomy has been the preferred treatment for drug-refractory heart failure symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM). Over this time in the United States, the majority of myectomy operations have been performed in a small number of select referral centers. METHODS: We have taken the opportunity to report results from the relatively new Tufts HCM Center and surgical program, incorporated 13 years ago, during which 507 myectomies (52±14 years of age; 56% male) were performed by one cardiothoracic surgeon, Dr. Hassan Rastegar. RESULTS: Resting left ventricular (LV) outflow gradients were reduced from 56±42 mmHg preoperatively to 1.2±6.8 mmHg on most recent echocardiogram 2.0±2.5 years after surgery, and 94% of patients showed clinical improvement to NYHA functional class I or II. The first 200 myectomies were performed without mortality or major complications. Among all patients, 30-day mortality rate was 0.8%. Over follow-up of 3.2±2.8 years, 11 patients died (four due to HCM causes) with long-term survival after myectomy of 94% at 5 years (95% CI: 89-96%) and 91% at 10 years (95% CI: 84-95%), which did not differ from the age- and gender-matched general U.S. population (log-rank P=0.9). CONCLUSIONS: This experience demonstrates that, with the appropriate support, new HCM surgical programs can provide patients successful relief of outflow obstruction, extended longevity and restored of quality of life.