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Prognosis in hypertrophic cardiomyopathy--echocardiographic follow-up and histopathological study
R Emoto1, Y Yokota, H Fukuzaki
11st Department of Internal Medicine, Kobe University School of Medicine, Japan.
Insights
Echocardiography can track heart changes in hypertrophic cardiomyopathy (HCM) patients. This study shows echocardiographic follow-up helps predict disease progression and patient outcomes, especially in heart failure cases.
Area of Science:
- Cardiology
- Medical Imaging
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex cardiac condition requiring effective monitoring.
- Understanding the progression of myocardial lesions and patient prognosis is crucial for managing HCM.
- Echocardiography offers a non-invasive method for assessing cardiac structure and function.
Purpose of the Study:
- To evaluate morphologic and functional cardiac changes in hypertrophic cardiomyopathy (HCM) patients using echocardiography.
- To compare echocardiographic indices among different patient groups, including those who survived and those who died from various causes.
- To determine the utility of echocardiographic follow-up in predicting HCM progression and prognosis.
Main Methods:
- Echocardiographic evaluation of 30 deceased and 50 surviving HCM patients over an average of 3.9 years.
- Comparison of echocardiographic indices (e.g., left ventricular end-diastolic dimension, fractional shortening, normalized rapid filling rate) across five patient groups.
- Correlation of echocardiographic findings with histopathological data from autopsied patients.
Main Results:
- Patients who died of heart failure (HF) or experienced sudden death (SD) showed increased left ventricular end-diastolic dimension and decreased fractional shortening and normalized rapid filling rate during follow-up.
- Massive fibrosis in the left ventricle was significantly larger in deceased groups, particularly HF, compared to non-cardiac death (NC) groups.
- Extensive myocardial fibrosis correlated strongly with adverse echocardiographic parameters, including larger left ventricular dimensions and reduced cardiac function.
Conclusions:
- Echocardiographic follow-up is a valuable tool for monitoring the progression of myocardial lesions in HCM.
- Changes in echocardiographic indices over time can predict the prognosis of hypertrophic cardiomyopathy patients.
- The extent of myocardial fibrosis, assessed indirectly through echocardiography, is a key determinant of HCM outcomes.
Abstract:
In 30 consecutive hypertrophic cardiomyopathy (HCM) patients who eventually died and 50 who survived, morphologic and functional changes in the heart during a follow-up period of an average of 3.9 years were echocardiographically evaluated. Echocardiographic indices were compared among 5 groups of patients with HCM, consisting of 4 groups of patients who eventually died (SD: 17 patients who suffered sudden death, ED: 4 who died of embolism, HF: 4 who died of congestive heart failure, NC: 5 who died of a noncardiac event) and 1 group of patients who survived with nondilated left ventricle (S: 50 patients). These indices at the last evaluation before death were compared with histopathological findings of left ventricles in 12 autopsied patients. At the initial evaluation ED and HF patients had a larger left ventricular end-diastolic dimension (LVDd), and HF patients had a smaller percent fractional shortening (%FS) and a slower normalized rapid filling rate (nRFR: mean rapid filling rate/LVEDV) than S patients, but these indices showed no differences between SD and S patients. During follow-up, no echocardiographic indices changed in S patients, but LVDd was increased and %FS and nRFR were decreased in SD and HF patients. Mean myocyte diameter and % area of disarray showed no differences among the 4 death groups. However, compared with NC, the other 3 groups, especially HF, had larger % area of massive fibrosis. The % area of massive fibrosis was correlated with LVDd (r = 0.80, p less than 0.005), %FS (r = -0.64, p less than 0.05), and nRFR (r = -0.95, p less than 0.001) at the last evaluation. These results suggested that echocardiographic follow-up was useful in predicting the progression of the myocardial lesion and the prognosis of HCM.