Martin S Maron1, Ethan J Rowin2, Iacopo Olivotto3, Susan A Casey4, Anna Arretini3, Benedetta Tomberli3, Ross F Garberich4, Mark S Link2, Raymond H M Chan5, John R Lesser4, Barry J Maron4. 1. Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts. Electronic address: mmaron@tuftsmedicalcenter.org. 2. Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts. 3. Regional Referral Center for Myocardial Disease, Azienda Ospedaliera Careggi, Florence, Italy. 4. The Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota. 5. Department of Medicine (Cardiovascular Division) and Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
Abstract
BACKGROUND: Left ventricular outflow tract gradients are absent in an important proportion of patients with hypertrophic cardiomyopathy (HCM). However, the natural course of this important patient subgroup remains largely unresolved. OBJECTIVES: The authors systematically employed exercise (stress) echocardiography to define those patients without obstruction to left ventricular outflow at rest and/or under physiological exercise and to examine their natural history and clinical course to create a more robust understanding of this complex disease. METHODS: We prospectively studied 573 consecutive HCM patients in 3 centers (44 ± 17 years; 66% male) with New York Heart Association functional class I/II symptoms at study entry, including 249 in whom left ventricular outflow tract obstruction was absent both at rest and following physiological exercise (<30 mm Hg; nonobstructive HCM) and retrospectively assembled clinical follow-up data. RESULTS: Over a median follow-up of 6.5 years, 225 of 249 nonobstructive patients (90%) remained in classes I/II, whereas 24 (10%) developed progressive heart failure to New York Heart Association functional classes III/IV. Nonobstructive HCM patients were less likely to experience advanced limiting class III/IV symptoms than the 324 patients with outflow obstruction (1.6%/year vs. 7.4%/year rest obstruction vs. 3.2%/year provocable obstruction; p < 0.001). However, 7 nonobstructive patients (2.8%) did require heart transplantation for progression to end stage versus none of the obstructive patients. HCM-related mortality among nonobstructive patients was low (n = 8; 0.5%/year), with 5- and 10-year survival rates of 99% and 97%, respectively, which is not different from expected all-cause mortality in an age- and sex-matched U.S. population (p = 0.15). CONCLUSIONS: HCM patients with nonobstructive disease appear to experience a relatively benign clinical course, associated with a low risk for advanced heart failure symptoms, other disease complications, and HCM-related mortality, and largely without the requirement for major treatment interventions. A small minority of nonobstructive HCM patients progress to heart transplant.
BACKGROUND: Left ventricular outflow tract gradients are absent in an important proportion of patients with hypertrophic cardiomyopathy (HCM). However, the natural course of this important patient subgroup remains largely unresolved. OBJECTIVES: The authors systematically employed exercise (stress) echocardiography to define those patients without obstruction to left ventricular outflow at rest and/or under physiological exercise and to examine their natural history and clinical course to create a more robust understanding of this complex disease. METHODS: We prospectively studied 573 consecutive HCM patients in 3 centers (44 ± 17 years; 66% male) with New York Heart Association functional class I/II symptoms at study entry, including 249 in whom left ventricular outflow tract obstruction was absent both at rest and following physiological exercise (<30 mm Hg; nonobstructive HCM) and retrospectively assembled clinical follow-up data. RESULTS: Over a median follow-up of 6.5 years, 225 of 249 nonobstructive patients (90%) remained in classes I/II, whereas 24 (10%) developed progressive heart failure to New York Heart Association functional classes III/IV. Nonobstructive HCM patients were less likely to experience advanced limiting class III/IV symptoms than the 324 patients with outflow obstruction (1.6%/year vs. 7.4%/year rest obstruction vs. 3.2%/year provocable obstruction; p < 0.001). However, 7 nonobstructive patients (2.8%) did require heart transplantation for progression to end stage versus none of the obstructive patients. HCM-related mortality among nonobstructive patients was low (n = 8; 0.5%/year), with 5- and 10-year survival rates of 99% and 97%, respectively, which is not different from expected all-cause mortality in an age- and sex-matched U.S. population (p = 0.15). CONCLUSIONS: HCM patients with nonobstructive disease appear to experience a relatively benign clinical course, associated with a low risk for advanced heart failure symptoms, other disease complications, and HCM-related mortality, and largely without the requirement for major treatment interventions. A small minority of nonobstructive HCM patients progress to heart transplant.
Authors: Anna Axelsson Raja; Hoshang Farhad; Anne Marie Valente; John-Paul Couce; John Lynn Jefferies; Henning Bundgaard; Kenneth Zahka; Harry Lever; Anne M Murphy; Euan Ashley; Sharlene M Day; Mark V Sherrid; Ling Shi; David A Bluemke; Charles E Canter; Steven D Colan; Carolyn Y Ho Journal: Circulation Date: 2018-08-21 Impact factor: 29.690
Authors: Nabil Ghorayeb; Ricardo Stein; Daniel Jogaib Daher; Anderson Donelli da Silveira; Luiz Eduardo Fonteles Ritt; Daniel Fernando Pellegrino Dos Santos; Ana Paula Rennó Sierra; Artur Haddad Herdy; Claúdio Gil Soares de Araújo; Cléa Simone Sabino de Souza Colombo; Daniel Arkader Kopiler; Filipe Ferrari Ribeiro de Lacerda; José Kawazoe Lazzoli; Luciana Diniz Nagem Janot de Matos; Marcelo Bichels Leitão; Ricardo Contesini Francisco; Rodrigo Otávio Bougleux Alô; Sérgio Timerman; Tales de Carvalho; Thiago Ghorayeb Garcia Journal: Arq Bras Cardiol Date: 2019-03 Impact factor: 2.000
Authors: Michele Covella; Ethan J Rowin; Nicholas S Hill; Ioana R Preston; Alberto Milan; Alexander R Opotowsky; Barry J Maron; Martin S Maron; Bradley A Maron Journal: Circ Heart Fail Date: 2017-04 Impact factor: 8.790