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酒精室间隔消融术在药物难治性肥厚梗阻性心肌病的年轻患者中有作用吗?

Is There a Role for Alcohol Septal Ablation in Young Patients with Medically Refractory Hypertrophic Obstructive Cardiomyopathy?

机构信息

Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, 4A, Dr J J Nagar, Mogappair, Chennai, 600089, India.

出版信息

Pediatr Cardiol. 2024 Mar;45(3):648-659. doi: 10.1007/s00246-023-03145-6. Epub 2023 Mar 30.

Abstract

Surgical myectomy is recommended for symptomatic hypertrophic obstructive cardiomyopathy (HOCM) after optimal pharmacological therapy. Percutaneous transluminal septal myocardial ablation (PTSMA) is reserved for high-risk adults. Symptomatic patients below 25 years underwent either surgery or PTSMA after heart-team discussion and informed consent. Echocardiography assessed gradients in surgical group. PTSMA group underwent invasive transseptal hemodynamic assessment, selective coronary angiography and super-selective cannulation of septal perforators using microcatheters. Contrast echocardiography through the microcatheter identified the myocardial target for PTSMA. Hemodynamic and electrocardiographic monitoring guided alcohol injection. Both groups were continued on beta-blockers. Symptoms, echocardiographic gradients and Brain natriuretic peptide (NTproBNP) measurements were assessed on follow-up. Twelve patients aged 5-23 years (11-98 kg) formed the study group. Indications for PTSMA in 8 patients included abnormal mitral valve anatomy warranting replacement (n = 3), Jehovah's witness (n = 2), severe neurodevelopmental and growth retardation (n = 1) and refusal of surgery (n = 2). PTSMA targeted first perforator (n = 5), second perforator (n = 2) and anomalous septal artery from left main trunk (n = 1). Outflow gradient reduced from 92.5 ± 19.7 to 33.1 ± 13.5 mmHg. At a median follow-up of 38 months (range 3-120 weeks), the peak instantaneous echocardiographic gradient was 32 ± 16.5 mmHg. Gradient reduced in four surgical patients from 86.5 ± 16.3 mmHg to 42 ± 14.7 mm Hg. All patients were in NYHA class I/II on follow-up. The mean NTproBNP in PTSMA group reduced from 6084 ± 3628 pg/ml to 3081 ± 2019 pg/ml; it was 1396 and 1795 pg/ml in surgery. PTSMA may be considered in medically refractory high-risk young patients. It relieves symptoms and reduces gradient. Though surgery is preferred in young patients, PTSMA may have a role in selected patients.

摘要

对于经过最佳药物治疗后仍有症状的肥厚型梗阻性心肌病(HOCM),建议进行外科心肌切除术。经皮腔内间隔心肌消融术(PTSMA)保留给高危成年人。经心脏团队讨论并征得知情同意后,25 岁以下有症状的患者接受手术或 PTSMA 治疗。超声心动图评估手术组的梯度。PTSMA 组进行了有创经间隔血流动力学评估、选择性冠状动脉造影和使用微导管对间隔穿通支进行超选择性插管。通过微导管进行对比超声心动图确定 PTSMA 的心肌靶标。血流动力学和心电图监测指导酒精注射。两组均继续使用β受体阻滞剂。在随访时评估症状、超声心动图梯度和脑利钠肽(NTproBNP)测量值。12 名年龄 5-23 岁(11-98kg)的患者组成了研究组。8 例患者行 PTSMA 的适应证包括需要更换的异常二尖瓣解剖结构(n=3)、耶和华见证人(n=2)、严重神经发育和生长迟缓(n=1)和拒绝手术(n=2)。PTSMA 的目标为第一间隔穿通支(n=5)、第二间隔穿通支(n=2)和左主干异常间隔动脉(n=1)。流出道梯度从 92.5±19.7mmHg 降至 33.1±13.5mmHg。在中位数为 38 个月(范围 3-120 周)的随访中,峰值瞬时超声心动图梯度为 32±16.5mmHg。4 例手术患者的梯度从 86.5±16.3mmHg 降至 42±14.7mmHg。所有患者在随访时均处于 NYHA Ⅰ/Ⅱ级。PTSMA 组的平均 NTproBNP 从 6084±3628pg/ml 降至 3081±2019pg/ml;手术组为 1396 和 1795pg/ml。对于药物难治性高危年轻患者,可考虑 PTSMA。它可缓解症状并降低梯度。尽管手术是年轻患者的首选,但 PTSMA 在某些患者中可能具有一定作用。

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