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Hypertrophic cardiomyopathy with extreme increase in left ventricular wall thickness: functional and morphologic
Insights
This study on hypertrophic cardiomyopathy found that severe left ventricular hypertrophy presents diverse clinical outcomes. Many patients remained stable or improved, though some experienced deterioration or arrhythmias.
Area of Science:
- Cardiology
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex cardiac condition.
- Severe left ventricular hypertrophy (LVH) is a key feature of HCM.
- Understanding the clinical spectrum of severe LVH in HCM is crucial.
Purpose of the Study:
- To analyze the clinical and morphologic features of patients with marked left ventricular hypertrophy in hypertrophic cardiomyopathy.
- To investigate the prevalence of subaortic obstruction and the natural history of these patients.
Main Methods:
- Retrospective analysis of 34 patients with hypertrophic cardiomyopathy and septal thickness ≥ 35 mm.
- Echocardiography and hemodynamic assessments were used to evaluate left ventricular morphology and subaortic obstruction.
- Clinical follow-up data were collected for at least 1 year.
Main Results:
- 90% of patients exhibited diffuse hypertrophy involving the left ventricular free wall.
- 29% had basal subaortic obstruction, while 71% did not have obstruction at rest.
- During a mean 6-year follow-up, 30% showed clinical deterioration (including heart block and ventricular fibrillation), but 70% remained stable or improved, with 67% asymptomatic or mildly symptomatic.
Conclusions:
- Patients with severe left ventricular hypertrophy in hypertrophic cardiomyopathy show a wide range of clinical presentations and natural histories.
- Subaortic obstruction is not universally present in this subgroup.
- A significant proportion of patients experience long-term stability or improvement despite marked hypertrophy.
Abstract:
Clinical and morphologic features of 34 patients with hypertrophic cardiomyopathy and particularly marked left ventricular hypertrophy were analyzed. Only patients with a ventricular septal thickness of at least 35 mm (range to 52 mm) were selected for the study; 31 (90%) had a diffuse pattern of hypertrophy also involving substantial portions of the left ventricular free wall. Despite similar left ventricular morphology, these patients exhibited a broad spectrum of clinical findings and natural history. Ten patients (29%) had hemodynamic or echocardiographic evidence of basal subaortic obstruction (average gradient, 63 mm Hg); however, the majority (24 [71%]) had no evidence of obstruction at rest, despite substantial hypertrophy of the basal anterior portions of septum and free wall. Although the electrocardiograms of most patients (76%) showed patterns of left ventricular hypertrophy, the magnitude of precordial QRS complexes was not markedly increased (S wave in lead V1 or V2, 27 +/- 15 mm; R wave in lead V5 or V6, 21 +/- 9 mm). The clinical course was variable in 30 patients who were followed up for at least 1 year (mean 6 years). Although no patient died, nine (30%) have exhibited clinical deterioration, including two who spontaneously developed complete heart block and one who collapsed with ventricular fibrillation but survived. However, the clinical condition of the majority of patients (21 [70%]) remained unchanged or improved. At the most recent evaluation, 20 (67%) of the 30 patients were asymptomatic or only mildly symptomatic, including 7 who remained without symptoms throughout the period of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)