Metabolic Exercise Testing in Hypertrophic Cardiomyopathy: Relation of Submaximal Parameters to Clinical Features

1998 ◽  
Vol 31 (2) ◽  
pp. 484A
Author(s):  
S Sharma
2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Kubo ◽  
H Takano ◽  
M Takayama ◽  
Y.L Doi ◽  
Y Minami ◽  
...  

Abstract Background Hypertrophic cardiomyopathy (HCM) is a most prevalent primary myocardial disorder with heterogeneous clinical features. However, there have been few studies on clinical features of HCM as a prospective cohort. In 2015, we established a large-scale registration survey of patients with HCM throughout Japan, named J-HCM registry study. Purpose The aim of this study was to clarify the clinical features of Japanese patients with HCM. Methods J-HCM registry study is a prospective, multicenter investigation, consisting of 24 hospitals. This time, we present the baseline clinical characteristics in this survey. Results Total 1484 patients were registered. The ages at registration and at diagnosis were 65±15 and 56±17 years, respectively, and 806 patients (54%) were men. Majority of the patients (95%) was NYHA class I or II. With regard to subtypes of HCM, there were 526 patients (36%) in the HCM with left ventricular (LV) outflow tract obstruction, 126 patients (8%) in the mid-ventricular obstruction, 57 patients (4%) in the end-stage phase characterized by LV ejection fraction <50%, and 197 patients (14%) in apical HCM. At registration, 80 patients (6%) had prior successful recovery from sustained ventricular tachycardia or ventricular fibrillation, 162 patients (11%) suffered from heart failure hospitalization, and 64 patients (4%) had history of embolic event. Regarding invasive treatment, 160 patients (10%) had prior septal reduction therapy and 162 patients (11%) had ICD implantation. According to the 2014 European Society of Cardiology Guidelines on sudden cardiac death (SCD) prevention, the study patients were divided into 3 categories by the HCM Risk-SCD calculator: patients distribution, 4% in the high risk group (≥6% calculated HCM Risk-SCD at 5 years), 7% in the intermediate risk group (4% to <6%), 69% in the low risk group (<4%), and 16% in the patients with extreme characteristics (Figure 1). Conclusions In this multicenter registration survey of patients with HCM, the baseline clinical characteristics were almost similar to several retrospective large-scale cohorts in Western countries except older age and less symptomatic state. This study will provide important knowledge regarding management of HCM. Figure 1 Funding Acknowledgement Type of funding source: None


Cardiology ◽  
2006 ◽  
Vol 106 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Cheng-Han Lee ◽  
Ping-Yen Liu ◽  
Li-Jen Lin ◽  
Jyh-Hong Chen ◽  
Liang-Miin Tsai

ESC CardioMed ◽  
2018 ◽  
pp. 1450-1455
Author(s):  
Albert Alain Hagège

The diagnosis of hypertrophic cardiomyopathy (HCM) in adults is based on the presence of left ventricular wall thickness greater than 15 mm using any imaging modality on at least one myocardial segment and not explained solely by abnormal cardiac loading conditions; a 13 mm threshold should be applied for familial screening in first-degree relatives. Diagnosis in children, in the elderly, in hypertensive individuals, and in elite athletes may be challenging. Initial evaluation should include a family pedigree, evaluation of signs and symptoms, electrocardiogram, and 48 h Holter electrocardiogram monitoring, exercise testing, imaging, and biochemistry. The presence of an intraventricular left ventricular obstruction, present in two-thirds of the patients at rest, during Valsalva or exercise, should be systematically evaluated using echocardiography. Cardiovascular magnetic resonance imaging with late gadolinium enhancement should always be considered, particularly to assess apical hypertrophy, left ventricular aneurysms, and fibrosis. This systematic approach is recommended to assist in the detection of HCM not caused by mutations in cardiac sarcomere protein genes (up to 10% of patients). Genetic tests should be performed and interpreted after a careful and complete clinical evaluation and genetic counselling. HCM is characterized by symptoms of dyspnoea, fatigue, chest pain, palpitations, and syncope, which are highly variable. Functional limitation may be difficult to evaluate and often necessitates cardiopulmonary exercise testing.


ESC CardioMed ◽  
2018 ◽  
pp. 1450-1455
Author(s):  
Albert Alain Hagège

The diagnosis of hypertrophic cardiomyopathy (HCM) in adults is based on the presence of left ventricular wall thickness greater than 15 mm using any imaging modality on at least one myocardial segment and not explained solely by abnormal cardiac loading conditions; a 13 mm threshold should be applied for familial screening in first-degree relatives. Diagnosis in children, in the elderly, in hypertensive individuals, and in elite athletes may be challenging. Initial evaluation should include a family pedigree, evaluation of signs and symptoms, electrocardiogram, and 48 h Holter electrocardiogram monitoring, exercise testing, imaging, and biochemistry. The presence of an intraventricular left ventricular obstruction, present in two-thirds of the patients at rest, during Valsalva or exercise, should be systematically evaluated using echocardiography. Cardiovascular magnetic resonance imaging with late gadolinium enhancement should always be considered, particularly to assess apical hypertrophy, left ventricular aneurysms, and fibrosis. This systematic approach is recommended to assist in the detection of HCM not caused by mutations in cardiac sarcomere protein genes (up to 10% of patients). Genetic tests should be performed and interpreted after a careful and complete clinical evaluation and genetic counselling. HCM is characterized by symptoms of dyspnoea, fatigue, chest pain, palpitations, and syncope, which are highly variable. Functional limitation may be difficult to evaluate and often necessitates cardiopulmonary exercise testing.


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