Cardiopulmonary Responses and Prognosis in Hypertrophic Cardiomyopathy. A Potential Role for Comprehensive Noninvasive Hemodynamic Assessment

OBJECTIVES: The study sought to discover the key determinants of exercise capacity, maximal oxygen consumption (oxygen uptake [Vo2]), and ventilatory efficiency (ventilation/carbon dioxide output [VE/Vco2] slope) and assess the prognostic potential of metabolic exercise testing in hypertrophic cardiomyopathy (HCM). BACKGROUND: The intrinsic mechanisms leading to reduced functional tolerance in HCM are unclear. METHODS: The study sample included 156 HCM patients consecutively enrolled from 2007 to 2012 with a complete clinical assessment, including rest and stress echocardiography and cardiopulmonary exercise test (CPET) with impedance cardiography. Patients were also followed for the composite outcome of cardiac-related death, heart transplant, and functional deterioration leading to septal reduction therapy (myectomy or septal alcohol ablation). RESULTS: Abnormalities in CPET responses were frequent, with 39% (n = 61) of the sample showing a reduced exercise tolerance (Vo2 max 34). The variables most strongly associated with exercise capacity (expressed in metabolic equivalents), were peak cardiac index (r = 0.51, p 34 (hazard ratio: 3.14; 95% CI: 1.26 to 7.87; p = 0.014), and LAVI >40 ml/m2 (hazard ratio: 3.32; 95% CI: 1.08 to 10.16; p = 0.036). CONCLUSIONS: In HCM, peak cardiac index is the main determinant of exercise capacity, but it is not significantly related to ventilatory efficiency. Peak Vo2, ventilatory inefficiency, and LAVI are associated with an increased risk of major events in the short-term follow-up