Hypertrophic Cardiomyopathy Institute, Divisions of Cardiology and Cardiac Surgery, Tufts Medical Center, Boston, Mass.
Hypertrophic Cardiomyopathy Institute, Divisions of Cardiology and Cardiac Surgery, Tufts Medical Center, Boston, Mass.
Am J Med. 2018 Jun;131(6):e235-e239. doi: 10.1016/j.amjmed.2017.12.031. Epub 2018 Jan 31.
Surgical myectomy reverses heart failure symptoms in the vast majority of obstructive hypertrophic cardiomyopathy patients. However, a small subgroup fails to experience sustained postoperative improvement despite relief of obstruction. Clinical profile of such patients has not been well defined.
Consecutive obstructive hypertrophic cardiomyopathy patients undergoing myectomy at Tufts Medical Center for drug-refractory New York Heart Association III/IV heart failure symptoms, 2004 to 2017, were followed postoperatively for 2.5 ± 2.8 years and assessed for outcome.
Of the 503 patients, there were 4 postoperative deaths (0.8%); 480 patients (96%) had sustained improvement to New York Heart Association classes I or II (responders), but 19 (3.8%) developed advanced symptoms (classes III or IV) in the absence of obstruction (nonresponders). Compared with responders, nonresponders were younger (40 ± 13 vs 53 ± 14 years; P < .001) and had greater septal thickness (25 ± 9 vs 20 ± 4 mm; P < .001). Massive hypertrophy (≥30 mm) was 5-fold more common in nonresponders (P < .01). Seven nonresponders developed systolic dysfunction (ejection fraction 20%-47%), 2 days to 6.1 years postoperatively. Four nonresponders underwent heart transplant 3.4 to 9.2 years after myectomy, and 2 others have been listed.
Surgical myectomy is highly effective at reversing heart failure symptoms in the vast majority of patients with obstructive hypertrophic cardiomyopathy. However, a small minority experience persistent functional limitation despite surgical relief of outflow obstruction. Predictors of adverse postoperative course were substantial/massive septal thickness and youthful age. Patients who failed to respond symptomatically to myectomy were considered for advanced heart failure treatment, including heart transplantation.
外科心肌切除术在绝大多数梗阻性肥厚型心肌病患者中逆转心力衰竭症状。然而，一小部分患者尽管梗阻得到缓解，但仍未能持续改善。此类患者的临床特征尚未明确。
连续接受心肌切除术治疗药物难治性纽约心脏协会 III/IV 级心力衰竭症状的梗阻性肥厚型心肌病患者，于 2004 年至 2017 年在塔夫茨医疗中心接受治疗，术后随访 2.5±2.8 年，评估其结局。
503 例患者中，术后死亡 4 例（0.8%）；480 例（96%）患者纽约心脏协会心功能分级持续改善至 I 级或 II 级（有反应者），但 19 例（3.8%）在无梗阻的情况下出现晚期症状（III 级或 IV 级）（无反应者）。与有反应者相比，无反应者更年轻（40±13 岁 vs 53±14 岁；P<0.001）且室间隔厚度更大（25±9 毫米 vs 20±4 毫米；P<0.001）。无反应者中，巨大肥厚（≥30 毫米）更常见（5 倍；P<0.01）。7 例无反应者术后 2 天至 6.1 年出现收缩功能障碍（射血分数 20%-47%）。4 例无反应者在心肌切除术后 3.4 至 9.2 年接受心脏移植，另有 2 例患者被列入名单。
外科心肌切除术在绝大多数梗阻性肥厚型心肌病患者中高度有效，可逆转心力衰竭症状。然而，少数患者尽管流出道梗阻得到手术缓解，但仍持续存在功能受限。术后不良病程的预测因素是室间隔厚度大/巨大和年轻。对于心肌切除术症状无反应的患者，考虑采用先进的心力衰竭治疗方法，包括心脏移植。
