Maria Beatrice Musumeci1, Vittoria Mastromarino2, Matteo Casenghi2, Giacomo Tini2, Pietro Francia2, Antonello Maruotti3, Antonella Romaniello2, Damiano Magrì2, Rosa Lillo2, Carmen Adduci2, Massimo Volpe4, Camillo Autore2. 1. Cardiology Division, Clinical and Molecular Medicine Department, Sapienza University of Rome, Rome, Italy. Electronic address: beatrice.musumeci@uniroma1.it. 2. Cardiology Division, Clinical and Molecular Medicine Department, Sapienza University of Rome, Rome, Italy. 3. Dipartimento di Scienze Economiche, Politiche e delle Lingue Moderne, Libera Università Maria Ss. Assunta, Italy; Centre for Innovation and Leadership in Health Sciences, University of Southampton, Southampton, UK. 4. Cardiology Division, Clinical and Molecular Medicine Department, Sapienza University of Rome, Rome, Italy; IRCCS, Neuromed, Pozzilli, IS, Italy.
Abstract
BACKGROUND: Pulmonary hypertension (PH) in patients with hypertrophic cardiomyopathy (HCM) has been investigated in a small number of studies. Purpose of this study was to assess the prevalence and its association with outcome in a population of consecutive HCM outpatients. METHODS: We retrospectively analyzed data of 361 consecutive HCM outpatients in whom echocardiographic measurements of pulmonary artery systolic pressure (PASP) were available at initial and most recent evaluation. Four different clinical groups were specifically investigated: patients without left ventricular outflow tract obstruction (group A, 165), with obstruction (group B, 126), patients diagnosed at the age≥65 (group C, 50) and patients with end stage (ES) HCM (group D, 20). RESULTS: PH was identified in 41 (11.4%) of the 361 patients at initial evaluation while it has been recognized in 25 (7,8% [1.1%/year]) during a median follow-up of 3.4years. Analysis of subgroups showed that prevalence of PH increased from patient group A to D (8%, group A, 19%, group B, 28% group C, 70%, group D, respectively, p<0,01). During follow-up, patients with PH showed a significant higher HCM-related mortality (p=0.01) and morbidity (p<0.001) as compared with those without PH, but in multivariable analysis, PH resulted an independent risk factor only for HCM-related morbidity (HR=2.50, 95% CI 1.08-5.79, p=0.03). CONCLUSION: PH affects a significant proportion of patients with HCM. Its prevalence varies according to different clinical profiles. It is associated with an unfavorable clinical outcome and is an independent predictor of morbidity.
BACKGROUND:Pulmonary hypertension (PH) in patients with hypertrophic cardiomyopathy (HCM) has been investigated in a small number of studies. Purpose of this study was to assess the prevalence and its association with outcome in a population of consecutive HCM outpatients. METHODS: We retrospectively analyzed data of 361 consecutive HCM outpatients in whom echocardiographic measurements of pulmonary artery systolic pressure (PASP) were available at initial and most recent evaluation. Four different clinical groups were specifically investigated: patients without left ventricular outflow tract obstruction (group A, 165), with obstruction (group B, 126), patients diagnosed at the age≥65 (group C, 50) and patients with end stage (ES) HCM (group D, 20). RESULTS: PH was identified in 41 (11.4%) of the 361 patients at initial evaluation while it has been recognized in 25 (7,8% [1.1%/year]) during a median follow-up of 3.4years. Analysis of subgroups showed that prevalence of PH increased from patient group A to D (8%, group A, 19%, group B, 28% group C, 70%, group D, respectively, p<0,01). During follow-up, patients with PH showed a significant higher HCM-related mortality (p=0.01) and morbidity (p<0.001) as compared with those without PH, but in multivariable analysis, PH resulted an independent risk factor only for HCM-related morbidity (HR=2.50, 95% CI 1.08-5.79, p=0.03). CONCLUSION: PH affects a significant proportion of patients with HCM. Its prevalence varies according to different clinical profiles. It is associated with an unfavorable clinical outcome and is an independent predictor of morbidity.
Authors: Min Zhao; Min Liu; Jeffrey P Leal; Benjamin M W Tsui; Dean F Wong; Martin G Pomper; Yun Zhou Journal: PLoS One Date: 2019-03-20 Impact factor: 3.240