Implications of structural right ventricular involvement in patients with hypertrophic cardiomyopathy
Yu Zhang1, Yuming Zhu1, Mo Zhang2
1State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China.
Insights
Right ventricular involvement in hypertrophic cardiomyopathy (HCM) predicts worse outcomes, including cardiovascular death. Genetic factors are also linked to RV involvement in HCM patients.
Area of Science:
- Cardiology
- Genetics
- Clinical Medicine
Background:
- Right ventricular (RV) involvement in hypertrophic cardiomyopathy (HCM) is understudied.
- Understanding RV manifestations is crucial for risk stratification in HCM.
Purpose of the Study:
- To evaluate the risk prediction value of RV involvement in HCM.
- To investigate the genetic characteristics associated with RV involvement in HCM patients.
Main Methods:
- 893 HCM patients were assessed for RV hypertrophy, obstruction, or late gadolinium enhancement.
- Survival analyses evaluated cardiovascular death, all-cause death, and heart failure (HF)-related death.
- Genetic testing was performed on 669 patients to identify genetic variants.
Main Results:
- RV involvement was identified in 12.8% of patients and was an independent risk factor for adverse cardiovascular outcomes.
- Patients with RV involvement showed a higher frequency of genotype-positive results and MYBPC3 variants.
- Logistic analyses confirmed an independent correlation between RV involvement and genetic factors.
Conclusions:
- RV involvement is a significant independent risk factor for mortality in HCM patients.
- Genetic factors likely play a role in the development of RV involvement in HCM.
Aims:
In the clinical practice, the right ventricular (RV) manifestations have received less attention in hypertrophic cardiomyopathy (HCM). This paper aimed to evaluate the risk prediction value and genetic characteristics of RV involvement in HCM patients.
Methods And Results:
A total of 893 patients with HCM were recruited. RV hypertrophy, RV obstruction, and RV late gadolinium enhancement were evaluated by echocardiography and/or cardiac magnetic resonance. Patients with any of the above structural abnormalities were identified as having RV involvement. All patients were followed with a median follow-up time of 3.0 years. The primary endpoint was cardiovascular death; the secondary endpoints were all-cause death and heart failure (HF)-related death. Survival analyses were conducted to evaluate the associations between RV involvement and the endpoints. Genetic testing was performed on 669 patients. RV involvement was recognized in 114 of 893 patients (12.8%). Survival analyses demonstrated that RV involvement was an independent risk factor for cardiovascular death (P = 0.002), all-cause death (P = 0.011), and HF-related death (P = 0.004). These outcome results were then confirmed by a sensitivity analysis. Genetic testing revealed a higher frequency of genotype-positive in patients with RV involvement (57.0% vs. 31.0%, P < 0.001), and the P/LP variants of MYBPC3 were more frequently identified in patients with RV involvement (30.4% vs. 12.0%, P < 0.001). Logistic analyses indicated the independent correlation between RV involvement and these genetic factors.
Conclusion:
RV involvement was an independent risk factor for cardiovascular death, all-cause death and HF-related death in HCM patients. Genetic factors might contribute to RV involvement in HCM.
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