Documenting adverse remodeling in absence of left ventricular outflow tract (LVOT) obstruction and mitral regurgitation in a classical hypertrophic cardiomyopathy (HCMP) phenotype is difficult. Changes in the left atrium (LA) are a consequence of progressive left ventricular (LV) fibrosis and have been shown to progress in a linear fashion. Therefore, studying LA changes for identifying adverse remodeling in HCMP patients is important. Materials and Methods:This was a prospective study which included HCMP patients and age-and gender-matched controls. Various echocardiographic parameters of adverse cardiac remodeling were investigated.Results: A total of 160 patients with HCMP and 75 age-and sex-matched controls were analyzed over a 5-year period. HCMP patients had an enlarged LA, greater segmental thickness, and mildly increased LV filling pressure. Patients with maximum LA volume >40 mL and global LA strain <21.5% showed greater maximum segmental thickness and increased ratio of pulse wave Doppler derived mitral E wave and tissue Doppler derived annular e wave with reduced LA strain and LV strain, LA emptying fraction, and strain-derived LV ejection fraction. In both groups, significant difference was not observed in age, sex, HCMP phenotype, presence or absence of LVOT obstruction, LV volume, and mitral Doppler ratio of pulse wave Doppler derived mitral E and A waves. Conclusion:Monitoring HCMP in asymptomatic patients is challenging. Assessment of adverse cardiac remodeling in classical HCMP phenotype is feasible using global LA strain and maximum LA volume. Global LA strain identifies early changes and maximum LA volume late changes of cardiac remodeling and therefore provide an early indication of disease progression in asymptomatic HCMP patients.
Background: Hypertrophic cardiomyopathy (HCM) is a genetic disorder with a heterogeneous phenotype. Historically, only obstructive and nonobstructive forms have been described. Improvements in imaging techniques have led to elaborative description of various new phenotypes, although there are limited epidemiological data regarding these phenotypes from within our population. Aims and Objectives: •To identify HCM patients from a cohort of cardiomyopathy patients. •To describe their demographic profile and mode of presentation based on the presenting symptoms. •To subclassify the selected HCM population into various phenotypes based on echocardiography. Results: There were 233 patients of HCM in this study. The mean age of the patients was 53 ± 14.5 years with male preponderance (70%). Thirty-six percent patients were asymptomatic, 37% mildly symptomatic (in class I-II), and 27% severely symptomatic (in class III-IV). Patients were classified into six phenotypes consisting of five classically described phenotypes and one atypical phenotype. Reverse curvature phenotype was most prevalent (49%) and symptomatic phenotype with 19.3% having significant resting left ventricular outflow tract (LVOT) obstruction. Other phenotypes were 19% apical mid, 11% neutral, 6% sigmoid, and 4% atypical. Male: female ratio for reverse curvature phenotype was 3:1, apical (mid) phenotype 2.4:1, and both apical and neutral phenotypes 2:1. Sigmoid phenotype had female-to-male ratio of 2.5:1. Conclusion: HCM patients require phenotype specific approach for complete assessment. Transthoracic echocardiography with myocardial contrast plays a pivotal role in identifying morphological phenotypes, effects on cardiac remodeling and hemodynamics which consequently influence mode of presentation. Reverse curvature, apical mid, and sigmoid phenotypes are comparatively more symptomatic, have larger left atrial size and segment thickness compared to less symptomatic apical and neutral phenotypes. Symptom status of the patient seems to be dependent on age, septal contour, severity of LVOT obstruction, left atrial volume, and maximal segment thickness.
Primary cardiac lymphoma is an extranodal non-Hodgkin's lymphoma exclusively located in the heart and/or pericardium, rare in immunocompetent patients, and more frequent in immunocompromised patients. We present 3 cases of primary cardiac lymphoma in immunocompetent patients and review other cases reported in the literature. Two patients were adults and third patient a child. Two patients presented with pericardial tamponade and third case with CHB. Initial diagnosis of tumor
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