Association And Prognosis Analysis Of Hypertrophic Cardiomyopathy And Pulmonary Arterial Hypertension

Objective:To study the independent risk factors of pulmonary hypertension in patients with hypertrophic cardiomyopathy and establish a PH prediction model.To explore the risk factors of poor prognosis in patients with HCM with PH.Methods:A total of 154 patients were diagnosed with hypertrophic cardiomyopathy from January 2016 to January 2021 were retrospectively analyzed,which were divided into control group(119 cases)and case group(35 cases)according to whether or not PH was combined.The patients in the case group were divided into PH 1 group(25 cases)and PH 2 group(10 cases)according to the level of pulmonary artery systolic pressure.Record and analyze the echocardiographic and clini cal data were recorded and analyzed,and follow-up results of patients.Multivariate logistic regression was used to analyze the independent risk factors of PH in HCM patients.The PH prediction model was constructed and the ROC curves were drawn to verify its prediction efficacy.COX regression analysis was used to analyze the risk factors of poor prognosis in patients with HCM with PH KM survival curves were plotted for the survival risk analysis of patients with HCM complicated with PH.Results:The prevalence of PH in this study was 22.73%.Compared with patients in the control group,patients in the case group had larger atrial diameter,were more prone to combine moderate to severe mitral and tricuspid regurgitation,wider pulmonary artery diameter,lower ejection fraction and higher E/e’ values.There were also statistically significant differen ces between the two groups in the comparison of atrial diameter ratio:atrioventricular coupling index and left ventricular mass fraction(P<0.05).Logistic regression analysis showed that atrial diameter ratio(OR 0.5,95%CI 0.000～0.003)and pulmonary axtery diameter(OR 1.891:95%CI 1.482～2.412)were independently related to the occurrence of PH in HCM patients(P<0.01).According to the results,the PH prediction model was constructed.The sensitivity of the model was 77.4%:the specificity was 75.8%,and the area under the curve was 0.826(P<0.001):indicating that the model had high prediction efficiency.The incidence of adverse events in HCM patients in this study was 25.97%.The overall survival rates of patients in the control group and the case group at 12 and 24 months were 100%,94.1%,and 31.4%,5.7%,respectively,with statistically significant differences between the groups(P<0.05).COX regression analysis showed that PASP(HR 1.123,95%CI 1.033～1.221)and E/e’(HR 1.131,95%CI 1.054～1.213)were independent risk factors for adverse events in HCM patients with PH within 2 years(P<0.05).There was significant difference between PH 1 group and PH 2 group in cardiac function deterioration and new heart failure symptoms.The cumulative survival rates of patients in the two groups at 12 and 24 months were 40%,4%and 10%,10%,respectively,with statistically significant differences between the two groups(P<0.05).According to the post-stratification analysis of E/e’ level,the occurrence of adverse events in patients of HCM with PH was significantly correlated with left ventricular diastolic dysfunction(P<0.001).Conclusion:HCM with PH was associated with sex,atrial diameter ratio,atrioventricular coupling index,left ventricular mass fraction,pulmonary artery diameter,moderate and severe mitral and tricuspid regurgitation,LVEF and E/e’ values.Atrial diameter ratio and pulmonary artery diameter are independently related to the occurrence of PH in HCM patients.PH and PASP levels were associated with poor prognosis in HCM patients.Both PASP and E/e’ values were independent risk factors for developing poor prognosis in patients of HCM with PH,and impairment of left ventricular diastolic function was also significantly related with its poor prognosis.The results of the study are helpful for the clinical evaluation of the risk and poor prognosis of PH in HCM patients,and have certain clinical application value.