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[Advanced sequelae of apical hypertrophic cardiomyopathy: report of two cases with wall motion abnormalities]
J Kusukawa1, M Suwa, Y Nakayama
1Third Division of Internal Medicine, Osaka Medical College, Takatsuki.
Insights
This study reports two cases of apical hypertrophic cardiomyopathy (apical HCM) that progressed to heart failure, showing thinning of the left ventricle apex. These findings highlight the potential for deterioration in apical HCM patients.
Area of Science:
- Cardiology
- Cardiovascular Diseases
- Internal Medicine
Background:
- Apical hypertrophic cardiomyopathy (apical HCM) is a distinct form of HCM.
- Limited reports exist on the long-term progression and systolic function deterioration in apical HCM.
Observation:
- Two patients with apical HCM developed congestive heart failure and apical thinning over 8-10 years.
- Case 1 showed left ventricular hypertrophy progressing to apical aneurysm and embolism.
- Case 2 exhibited apical hypertrophy, fibrosis, and eventual severe cardiac failure with atrial fibrillation.
Findings:
- Progressive thinning and abnormal motion of the left ventricular apex were observed in both cases.
- Reduced thallium-201 uptake indicated myocardial damage in the apical region.
- Histological analysis revealed interstitial fibrosis without myocyte hypertrophy.
Implications:
- Apical HCM can lead to significant left ventricular systolic dysfunction and heart failure.
- Long-term monitoring is crucial for identifying progression and managing complications.
- Further research is needed to understand the pathophysiology and long-term outcomes of apical HCM.
Abstract:
In view of the paucity of reports describing symptoms of increased degree, and deterioration of left ventricular systolic function in patients with apical hypertrophic cardiomyopathy (apical HCM), two cases with congestive heart failure and progressive thinning of previously hypertrophied apical portions of the left ventricle are reported. These were among 13 patients observed from eight to 10 years. Case 1: A 56-year-old man was diagnosed as having apical HCM at the age of 49 years. Severe left ventricular hypertrophy and prominent ST-T changes were observed on ECG during his first admission. His left ventricular end-diastolic pressure (LVEDP) was 24 mmHg and a left ventriculo-gram revealed a decrease in the left ventricular cavity in the apex and marked hypertrophy of the apical wall. Moderate interstitial fibrosis without hypertrophy or disarray of myocytes was observed in a left ventricular endomyocardial biopsy specimen. In two episodes of cardiac arrest he was successfully resuscitated at the age of 50 years. At the age of 55 years, two-dimensional echocardiography revealed thinning and abnormal motion in the apical wall, and a defect in 201T1 accumulation was observed in the same region by perfusion scintigraphy. This patient was readmitted with a diagnosis of cerebral embolism at the age of 56 years. Cardiac catheterization revealed normal LVEDP (8 mmHg), and a left ventriculogram revealed an aneurysm in the left ventricular apex with normal major epicardial coronary arteries. He has been under treatment with antiarrhythmic medications, calcium antagonists and anticoagulants, and has become relatively asymptomatic. Case 2: A 69-year-old-man was diagnosed as having apical HCM after a complete evaluation, including cardiac catheterization, at the age of 59 years. His LVEDP was elevated (17 mmHg), and a left ventricular angiogram revealed marked hypertrophy localized to the apex. Ejection fraction was 64%. A left ventricular endomyocardial biopsy revealed interstitial fibrosis without hypertrophy of myocytes. Thereafter, he has been followed as a New York Heart Association functional class III to IV with occasional elevation of cardiac enzymes but without chest pain or acute changes in his ECGs. However, atrial fibrillation with complete right bundle branch block developed at the age of 60 years. Apical wall thinning and dyskinesis were diagnosed by 2D echocardiography and a defect in the 201T1 accumulation was observed at about 65 years of age. He was readmitted in severe cardiac failure at the age of 69 years, and he was diagnosed as having cardiac asthma with pulmonary capillary wedge pressure of 35 mmHg.(ABSTRACT TRUNCATED AT 400 WORDS)