Restrictive cardiomyopathy is the least common type of cardiomyopathy, being defined by diastolic dysfunction and often unimpaired systolic function. Restrictive cardiomyopathies can be classified as familial or non-familial. Patients with familial restrictive cardiomyopathy can develop signs and symptoms of this condition anytime from childhood to adulthood. The evolution of the disease is towards signs and symptoms of pulmonary and systemic congestion and, without treatment, there is a fiveyear mortality rate of approximately 30% in these patients. We discuss the case of a 43-year-old patient diagnosed with familial restrictive cardiomyopathy with positive genetic tests for mutations of MYH7 gene and ABCC9 gene, who was first hospitalized in 2011 for palpitations. The echocardiography performed in evolution showed a continuous alteration of right ventricle function, without important differences of left ventricular function. She developed heart failure symptoms six years after diagnosis and she had seven hospitalizations in the past two years, currently with an increasing need of diuretics and persistent hepatic dysfunction. Cardiac transplantation or left ventricular assist device therapy should be considered in patients with severe heart failure symptoms and no longer effective treatment. However, elevated pulmonary vascular resistance excludes the patient from cardiac transplantation.
BACKGROUND: Testosterone is an important factor that influences the quality of life in men. The purpose of this study is to evaluate how testosterone level impacts the quality of life in patients with dilated cardiomyopathy. METHODS: This cross-sectional single-center included 97 male patients with dilated cardiomyopathy, in whom serum testosterone was measured. Health-related quality of life was measured using the translated validated version of the Kansas City Cardiomyopathy Questionnaire (KCCQ-12). We used correlation and multivariable regression to assess the association between KCCQ12 score, serum testosterone level, and clinical and paraclinical variables. RESULTS: The mean age of study participants was 58 (range 29–88). The mean LVEF was 25 ±8.61%. The average total serum testosterone level was 3.13 ±2.72 (range 0.19–13.5 ng/ml). The median global KCCQ-12 score was 44.8 (6.2–90.6) representing a poor to fair impairment in quality of life. There was an inverse correlation between the KCCQ-12 score and NYHA class (Pearson coefficient r = 0.847 p<0.001) and a direct correlation with LVEF (r=0.445, p<0.001). Also, the KCCQ-12 score correlated with hemoglobin level (r=0.214, p=0.037) and plasmatic creatinine level (r=-0.296 p= 0.004). In multivariable regression, the independent predictors of health-related quality of life were testosterone, LVEF, and NYHA class. CONCLUSIONS: The results of this study showed for the first time a significant direct relationship between serum testosterone levels and quality of life in patients with dilated cardiomyopathy.
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