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Isolation and Functional Characterization of Human Ventricular Cardiomyocytes from Fresh Surgical Samples
Published on: April 21, 2014
Clinicopathologic features of hypertrophic cardiomyopathy managed by cardiac transplantation
J Shirani1, B J Maron, R O Cannon
1Pathology Branche, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland.
Insights
Cardiac transplantation offers a survival benefit for patients with end-stage hypertrophic cardiomyopathy. This study shows improved functional outcomes in survivors post-transplant.
Area of Science:
- Cardiology
- Cardiovascular Surgery
- Transplantation Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) can lead to severe congestive heart failure.
- Cardiac transplantation is a potential treatment for end-stage HCM.
- Understanding pre-transplant characteristics and post-transplant outcomes is crucial.
Purpose of the Study:
- To review clinical data of patients undergoing cardiac transplantation for hypertrophic cardiomyopathy.
- To evaluate the indications, pre-transplant physiological changes, and post-transplant outcomes.
Main Methods:
- Review of clinical records, electrocardiographic, echocardiographic, hemodynamic, and radionuclide data.
- Analysis of explanted hearts from 10 cardiac transplant recipients with hypertrophic cardiomyopathy.
- Longitudinal assessment of hemodynamic and functional parameters pre- and post-transplantation.
Main Results:
- Nine patients received transplants for severe congestive heart failure; one for syncope.
- Pre-transplant, these patients showed increased pulmonary artery wedge pressure, decreased ejection fraction, and ventricular dilation.
- Post-transplant, 6 of 10 patients survived long-term (mean 39 months) in functional class I or II.
Conclusions:
- Cardiac transplantation can be a viable option for carefully selected patients with end-stage hypertrophic cardiomyopathy.
- Long-term survival and improved functional capacity are achievable after transplantation.
- Explanted hearts often showed ventricular dilation and scarring, suggesting advanced disease progression.
Abstract:
Clinical records, electrocardiographic, echocardiographic, hemodynamic and radionuclide data, and the operatively excised hearts were reviewed in 10 patients (aged 19 to 46 years, mean 35) who had cardiac transplantation for hypertrophic cardiomyopathy. Severe congestive heart failure unassociated with outflow obstruction was the indication for transplantation in 9 patients. During a pretransplantation period ranging from 45 to 312 months (mean 137) in these 9 patients, pulmonary artery wedge pressure increased from 19 +/- 9 to 27 +/- 7 mm Hg, left ventricular ejection fraction decreased from 51 +/- 11% to 41 +/- 1%, left ventricular end-diastolic dimension increased from 42 +/- 6 to 48 +/- 4 mm, and total 12-lead QRS voltage decreased from 209 +/- 50 to 156 +/- 41 mm. In these 9 patients, the explanted hearts had dilated left ventricular cavities, and 8 had left ventricular scars without significant narrowing of the epicardial coronary arteries. The tenth patient had a nondilated left ventricular cavity and had transplantation because of recurrent, refractory syncope. Of the 10 patients, 3 died within the first month, and another died 8 months after transplantation. The remaining 6 patients have survived 20 to 54 months (mean 39) after transplantation and are in functional class I or II.
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Cardiomyopathy I: Introduction and Classification
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy V: Interprofessional Care
Cardiomyopathy VII: Pre and Post Operative Nursing Management

