Zhang Yu, Liu Ming-Hao, Zhang Mo, Wu Gui-Xin, Liu Jie, Wang Ji-Zheng, Sun Xiao-Lu, Jiang Wen, Wang Dong, Kang Lian-Ming, Wu Xue-Yi, Zou Yu-Bao, Song Lei
State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Cardiomyopathy Ward, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
J Geriatr Cardiol. 2023 Feb 28;20(2):109-120. doi: 10.26599/1671-5411.2023.02.007.
To determine the different clinical characteristics and outcomes of hypertrophic cardiomyopathy (HCM) patients with and without hypertension (HT).
A total of 696 HCM patients were included in this study and all HCM diagnoses were confirmed by the genetic test. Patients were analyzed separately in the septal reduction therapy (SRT) cohort and the non-SRT cohort. The primary endpoint was cardiovascular death and the secondary endpoint was all-cause death. Outcome analyses were conducted to evaluate the associations between HT and outcomes in HCM. Medications before enrollment and at discharge were collected in the analyses.
HCM patients without HT were younger, had a lower body mass index, were more likely to have a family history of HCM, and had a smaller left ventricular (LV) end-diastolic diameter than those with HT in both cohorts. A thicker LV wall, a higher level of N-terminal pro-B-type natriuretic peptide, and a higher extent of LV late gadolinium enhancement were additionally observed in patients without HT in the non-SRT cohort. The presence of HT did not alter the distribution pattern of late gadolinium enhancement, as well as the constituent ratio of eight disease-causing sarcomeric gene variants in both cohorts. Outcome analyses showed that in the non-SRT cohort, patients without HT had higher risks of cardiovascular death (HR = 2.537, = 0.032) and all-cause death (HR = 3.309, = 0.032). While such prognostic divergence was not observed in the SRT cohort. Further analyses in the non-SRT cohort found that patients without HT received fewer non-dihydropyridine calcium channel blockers and angiotensin-converting enzyme inhibitors/angiotensin receptor blockers before enrollment and at discharge.
HCM patients without HT had worse clinical conditions and higher mortality than patients with HT overall, which may result from active medical therapy in HT patients. Active SRT may have a substantial de-risking effect on patients meeting the indications.
确定伴有和不伴有高血压（HT）的肥厚肥厚性肥厚型心肌病（HCM）患者的不同临床特征及预后。
本研究共纳入696例HCM患者，所有HCM诊断均经基因检测确诊。患者在室间隔减容治疗（SRT）队列和非SRT队列中分别进行分析。主要终点为心血管死亡，次要终点为全因死亡。进行预后分析以评估HT与HCM患者预后之间的关联。分析中收集了入组前和出院时的用药情况。
在两个队列中，不伴有HT的HCM患者比伴有HT的患者更年轻，体重指数更低，更可能有HCM家族史，左心室（LV）舒张末期直径更小。在非SRT队列中，不伴有HT的患者还观察到左心室壁更厚、N末端B型利钠肽前体水平更高以及左心室晚期钆增强程度更高。HT的存在并未改变晚期钆增强的分布模式，以及两个队列中八种致病肌节基因突变体的构成比。预后分析表明，在非SRT队列中，不伴有HT的患者心血管死亡风险（HR = 2.537， = 0.032）和全因死亡风险（HR = 3.309， = 0.032）更高。而在SRT队列中未观察到这种预后差异。在非SRT队列中的进一步分析发现，不伴有HT的患者在入组前和出院时接受非二氢吡啶类钙通道阻滞剂和血管紧张素转换酶抑制剂/血管紧张素受体阻滞剂的次数更少。
总体而言，不伴有HT的HCM患者临床状况比伴有HT的患者更差，死亡率更高，这可能是由于HT患者接受了积极的药物治疗。积极的SRT可能对符合适应症的患者有显著的降低风险作用。
