M S Lee1, R Zimmer2, J Kobashigawa2. 1. Division of Cardiology, UCLA Medical Center, Los Angeles, California. Electronic address: michaelsblee@gmail.com. 2. Division of Cardiology, Cedars-Sinai Medical Center, Los Angeles, California.
Abstract
BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease characterized by asymmetric or symmetric ventricular hypertrophy in the absence of an obvious clinical cause. Orthotopic heart transplantation (OHT) has been performed in patients who have refractory symptoms despite medical therapy and surgical septal myectomy. However, there is a paucity of data on outcomes of HCM patients who undergo OHT. METHODS: Data on 462 consecutive patients who underwent OHT at UCLA Medical Center from 1996 to 2004 were retrospectively collected. The clinical data on the 11 patients with HCM were identified. RESULTS: The majority of the HCM patients were male (64%). The mean age of the patient was 45 ± 8 years, and the mean donor age was 35 ± 18 years. The mean ischemia time was 226 ± 60 minutes. There was 1 in-hospital death secondary to septic shock. At a median duration of follow-up of 4.5 years (mean, 4.4 ± 3.2 years), there were 3 additional deaths. Compared with the 451 OHT patients who did not have HCM, there was no difference in survival (P = .13), development of cardiac allograft vasculopathy (P = .46), or rejection (P = .71). There was no evidence of HCM recurrence in biopsies from the donor heart. CONCLUSIONS: OHT is a viable treatment option for patients with end-stage HCM refractory to standard therapies.
BACKGROUND:Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease characterized by asymmetric or symmetric ventricular hypertrophy in the absence of an obvious clinical cause. Orthotopic heart transplantation (OHT) has been performed in patients who have refractory symptoms despite medical therapy and surgical septal myectomy. However, there is a paucity of data on outcomes of HCM patients who undergo OHT. METHODS: Data on 462 consecutive patients who underwent OHT at UCLA Medical Center from 1996 to 2004 were retrospectively collected. The clinical data on the 11 patients with HCM were identified. RESULTS: The majority of the HCM patients were male (64%). The mean age of the patient was 45 ± 8 years, and the mean donor age was 35 ± 18 years. The mean ischemia time was 226 ± 60 minutes. There was 1 in-hospital death secondary to septic shock. At a median duration of follow-up of 4.5 years (mean, 4.4 ± 3.2 years), there were 3 additional deaths. Compared with the 451 OHT patients who did not have HCM, there was no difference in survival (P = .13), development of cardiac allograft vasculopathy (P = .46), or rejection (P = .71). There was no evidence of HCM recurrence in biopsies from the donor heart. CONCLUSIONS: OHT is a viable treatment option for patients with end-stage HCM refractory to standard therapies.