Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.
Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.
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DOI：
10.1161/circheartfailure.116.003689
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发表时间：
2017-04
期刊：
影响因子：
--
通讯作者：
Maron BA
中科院分区：
文献类型：
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作者：
Covella M;Rowin EJ;Hill NS;Preston IR;Milan A;Opotowsky AR;Maron BJ;Maron MS;Maron BA
关于阻塞性肥厚型心肌病（HCM）和晚期心力衰竭患者肺动脉高压的患病率、病理生理学和治疗意义的数据有限。为了评估HCM伴心力衰竭患者心肺血流动力学测量值的临床意义，我们回顾性评估了162例连续患者的右心导管插入术数据，这些患者的流出道压差（中位数[四分位距]）为90 [70-110] mmHg，年龄为59±11岁，49%为男性，主要为NYHA III/IV级状态。82例患者（51%）存在肺动脉高压（平均肺动脉压[mPAP] ≥25 mmHg），其中29例（18%）被视为中度至重度（mPAP ≥35 mmHg），28例（34%）肺血管阻力（PVR）增加>3.0 WU。54%的患者肺动脉楔压（PAWP）≤15 mmHg，表明大多数患者无左房高压。值得注意的是，9例患者（11%）符合毛细血管前肺动脉高压的血流动力学标准（mPAP ≥25 mmHg，PVR >3.0 WU，PAWP ≤15 mmHg）。手术肌切除术（或酒精室间隔消融术）后中位随访327 [90-743]天，分别有92%和95%的术前肺动脉高压患者无症状或轻度症状。一例59岁女性患者术后死亡，发生急性呼吸衰竭，mPAP=65 mmHg。阻塞性肥厚型心肌病合并晚期心力衰竭患者肺动脉高压常见。虽然肺动脉高压可能是导致术前心力衰竭的原因，但并没有显着影响临床和手术结果。值得注意的是，发现了一个新的患者亚组，其静息侵入性血流动力学与肺血管疾病一致。
There are limited data regarding the prevalence, pathophysiology and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy (HCM) and advanced heart failure. To assess the clinical significance of measured cardiopulmonary hemodynamics in HCM patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range]) (90 [70–110] mmHg), 59±11 years old, and 49% male, predominately NYHA Class III/IV status. Pulmonary hypertension (mean pulmonary artery pressure [mPAP] ≥25 mmHg) was present in 82 patients (51%), including 29 (18%) regarded as moderate-severe (mPAP ≥35 mmHg) and 28 (34%) that also had increased pulmonary vascular resistance (PVR) >3.0 WU. The pulmonary artery wedge pressure (PAWP) was ≤15 mmHg in 54%, indicating that left atrial hypertension was absent in a majority of patients. Notably, 9 patients (11%) met hemodynamic criteria for pre-capillary pulmonary hypertension (mPAP ≥25 mmHg, PVR >3.0 WU, PAWP ≤15 mmHg). Over a median follow-up of 327 [90–743] days after surgical myectomy (or alcohol septal ablation), 92% and 95% of patients with or without preoperative pulmonary hypertension, respectively, were asymptomatic or mildly symptomatic. One postoperative death occurred in a 59-year old woman with acute respiratory failure and mPAP=65 mmHg. Pulmonary hypertension was common in obstructive HCM patients with advanced heart failure. While possibly a contributor to pre-operative heart failure, pulmonary hypertension did not significantly influence clinical and surgical outcome. Notably, a novel patient subgroup was identified with resting invasive hemodynamics consistent with pulmonary vascular disease.