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Published on: August 8, 2022
Hypertrophic Cardiomyopathy in a Latin American Center: A Single Center Observational Study
Juan David López-Ponce de Leon1,2, Mayra Estacio3, Natalia Giraldo1,2
1Departamento de Cardiología, Fundación Valle del Lili, Cali 760032, Colombia.
Insights
Hypertrophic cardiomyopathy (HCM) is a complex heart condition. This Colombian study of 82 patients highlights its varied presentation, management, and significant morbidity and mortality, offering insights into rare disease care.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a rare, complex cardiac disorder with diverse phenotypes and challenges in diagnosis and treatment.
- Limited data exists on the prevalence and natural history of cardiomyopathies in South America.
- This study addresses the need for comprehensive data on HCM in a developing country context.
Purpose of the Study:
- To summarize the clinical features, management strategies, and outcomes of patients with hypertrophic cardiomyopathy (HCM) at a university hospital in Colombia.
- To provide insights into the characteristics and progression of HCM in a South American population.
- To contribute data on the management of rare cardiovascular diseases in resource-limited settings.
Main Methods:
- An observational retrospective cohort study was conducted.
- Data from 82 patients diagnosed with HCM between January 2010 and December 2021 were analyzed.
- Information was extracted from an institutional cardiomyopathy registry, including demographic, paraclinical, and outcome data.
Main Results:
- The cohort comprised 67.1% males, with a mean age at diagnosis of 49 years.
- Most patients (83%) were in NYHA functional class I or II, with dyspnea being the most common symptom (38%).
- 89% had preserved left ventricular ejection fraction (LVEF), and 65% showed an asymmetric septal pattern. Interventions included alcohol septal ablation (6%) and septal myectomy (5%). Overall mortality was 7.3%, with sudden cardiovascular death in 2.6%.
Conclusions:
- Hypertrophic cardiomyopathy (HCM) is a heterogeneous condition associated with considerable morbidity and mortality.
- This registry offers valuable, comprehensive data on the course and management of HCM in a developing nation.
- The findings underscore the complexities of managing rare cardiovascular diseases and the need for continued research and improved access to care.
Background:
Hypertrophic cardiomyopathy (HCM) is a complex disorder that includes various phenotypes, leading to different manifestations. It also shares different disadvantages typical of rare diseases, including limited recognition, lack of prospective studies assessing treatment, and little or delayed access to advanced treatment options. Reliable data about the prevalence and natural history of cardiomyopathies in South America are lacking. This study summarizes the features and management of patients with HCM in a university hospital in Colombia.
Methods:
This was an observational retrospective cohort study of patients with HCM between January 2010 and December 2021. Patient data were analyzed from an institutional cardiomyopathy registry. Demographic, paraclinical, and outcome data were collected.
Results:
A total of 82 patients during the study period were enrolled. Of these, 67.1% were male, and the mean age at diagnosis was 49 years. Approximately 83% were in NYHA functional class I and II, and the most reported symptoms were dyspnea (38%), angina (20%), syncope (15%), and palpitations (11%). In addition, 89% had preserved left ventricular ejection fraction (LVEF) with an asymmetric septal pattern in 65%. Five patients (6%) had alcohol septal ablation and four (5%) had septal myectomy. One patient required heart transplantation during follow-up. Sudden cardiovascular death was observed in 2.6%. The overall mortality during follow-up was 7.3%.
Conclusions:
HCM is a complex and heterogeneous disorder that presents with significant morbidity and mortality. Our registry provides comprehensive data on disease courses and management in a developing country.
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