Effective Cibenzoline Treatment in a Patient With Midventricular Obstruction After Transcatheter Aortic Valve Implantation.
Effective Cibenzoline Treatment in a Patient With Midventricular Obstruction After Transcatheter Aortic Valve Implantation.
复制标题
西苯唑啉对经导管主动脉瓣植入后心室中梗阻患者的有效治疗。
DOI：
10.1161/circheartfailure.115.002629
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发表时间：
2016
期刊：
影响因子：
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通讯作者：
Fukuda K.
中科院分区：
文献类型：
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作者：
Isobe S;Ieda M;Kohno T;Nishiyama T;Maekawa Y;Tsuruta H;Murata M;Yashima F;Yanagisawa R;Tanaka M;Inami S;Nasuno T;Haruyama A;Sano M;Hayashida K;Fukuda K.
一名80岁女性因严重主动脉瓣狭窄入院接受经导管主动脉瓣植入术（TAVI）。她患有纽约心脏协会功能性 III 级充血性心力衰竭。她的合并症包括多发性骨髓瘤、慢性类固醇治疗的特发性血小板减少性紫癜、高血压、糖尿病和持续性心房颤动。超声心动图显示三尖瓣主动脉瓣钙化，瓣叶受限，与严重主动脉瓣狭窄一致（图 1A；数据补充中的影片 I）。多普勒成像上主动脉瓣的瞬时峰值速度为 5.0 m/s，平均压力梯度为 53 mm Hg（图 1B）。利用连续性方程计算出主动脉瓣面积为0.49 cm2。在室间隔中观察到同心左心室 (LV) 肥大，测量结果为 13 毫米，在后壁中观察到肥大，测量结果为 14 毫米。左心室腔较小，舒张末期左心室内部尺寸为 39 mm。左心室射血分数为80%。静息时观察到 25 mm Hg 的中腔 LV 压力梯度后期达到峰值，没有 LV 流出道阻塞和二尖瓣小叶收缩期前移（图 1C）。冠状动脉造影未发现明显的冠状动脉狭窄。鉴于她有严重的主动脉瓣狭窄和心力衰竭症状，并伴有严重血小板减少症（血小板计数：15000/μL）的合并症，该患者通过经股动脉途径接受了 TAVI。成功植入 23 毫米 Sapien 瓣膜（Edwards Lifesciences，尔湾，加利福尼亚州）。手术过程中和手术后患者的血流动力学特征保持稳定。她的症状有所改善，出院后接受了 β 受体阻滞剂、血管扩张剂和利尿剂的门诊治疗。两个月后，她因反复劳力性呼吸困难入院。查体发现颈静脉怒张，双下肢浮肿，胸骨左下缘有收缩期喷射性杂音6中3中。心电图显示房颤伴心动过缓（心率30-50次/分钟）。鉴于她患有心力衰竭、容量超负荷和心动过缓，静脉注射呋塞米并减少β受体阻滞剂的剂量。然而，她的症状因低血压和肾功能障碍而恶化。超声心动图显示爱德华兹智人瓣膜假体功能正常，瓣周反流极少。左心室收缩功能高动力，收缩期左心室向心肥厚，观察到明显的心室中段梗阻，静息时峰值压力梯度为 69 mm Hg（图 2A 和 2B）。未观察到明显的左室流出道梗阻和二尖瓣收缩期前移。鉴于上述发现，患者的病情恶化归因于肥厚性梗阻性心肌病生理学，在消除左心室压力超负荷后出现心室中段梗阻。因此，暂停使用利尿剂和全身血管扩张剂。由于心动过缓，β受体阻滞剂剂量无法增加，患者因合并症拒绝手术心肌切除术和酒精间隔消融术。因此，我们决定开始使用西苯唑啉，一种 Ia 类抗心律失常药物，用作肥厚性梗阻性心肌病 (HOCM) 患者的二线治疗。开始西苯啉治疗后，她的血流动力学有所改善，超声心动图显示心室中层的峰值压力梯度降至 35 mm Hg（图 2C）。她的呼吸困难有所改善，而且她……
An 80-year-old woman with severe aortic stenosis was admitted to our hospital for transcatheter aortic valve implantation (TAVI). She had New York Heart Association functional class III congestive heart failure. Her comorbidities included multiple myeloma, idiopathic thrombocytopenic purpura treated with chronic steroids, hypertension, diabetes mellitus, and persistent atrial fibrillation. Echocardiography demonstrated a calcified tricuspid aortic valve with leaflet restriction consistent with severe aortic stenosis (Figure 1A; Movie I in the Data Supplement). The instantaneous peak velocity across the aortic valve on Doppler imaging was 5.0 m/s, and the mean pressure gradient was 53 mm Hg (Figure 1B). The aortic valve area calculated by using the continuity equation was 0.49 cm2. A concentric left ventricular (LV) hypertrophy was observed in the septum, measuring 13 mm, and in the posterior wall, measuring 14 mm. The LV cavity was small, with an LV internal dimension of 39 mm at end diastole. LV ejection fraction was 80%. Late peaking of the midcavitary LV pressure gradient of 25 mm Hg at rest was observed, without LV outflow tract obstruction and systolic anterior motion of the mitral valve leaflet (Figure 1C). Coronary angiography revealed no significant coronary artery stenosis. Given her severe aortic stenosis and heart failure symptoms with comorbidities of severe thrombocytopenia (platelet count: 15 000 per μL), the patient underwent TAVI via the transfemoral approach. A 23-mm Sapien valve (Edwards Lifesciences, Irvine, CA) was successfully implanted. The hemodynamic profile of the patient throughout and after the procedure was stable. Her symptoms improved, and she was discharged and prescribed with a β-blocker, vasodilators, and diuretics as outpatient treatments. Two months later, she was admitted to our hospital for recurrent exertional dyspnea. Her physical examination revealed jugular venous distension, bilateral legs edema, and a 3 of 6 systolic ejection murmur at the lower left sternal border.Electrocardiography showed atrial fibrillation with bradycardia (heart rate: 30–50 beats per minute). Given her conditions of heart failure with volume overload and bradycardia, intravenous furosemide was administered and the dose of the β-blocker was reduced. However, her symptoms worsened with hypotension and renal dysfunction. Echocardiography revealed that the Edwards Sapien valve prosthesis was functioning properly with minimal paravalvular regurgitation. LV systolic function was hyperdynamic, and significant midventricular obstruction was observed with concentric LV hypertrophy during systole, with a peak pressure gradient of 69 mm Hg at rest (Figure 2A and 2B). No significant LV outflow tract obstruction and systolic anterior motion of the mitral valve were observed. Given the above-mentioned findings, the patient’s deterioration was attributed to hypertrophic obstructive cardiomyopathic physiology with the development of midventricular obstruction after the removal of the LV pressure overload. Therefore, the diuretics and systemic vasodilators were suspended. The β-blocker dose could not be increased because of bradycardia, and the patient refused surgical myectomy and alcohol septal ablation because of her comorbidities. Thus, we decided to start cibenzoline, a class Ia antiarrhythmic drug, which is used as a second-line therapy for patients with hypertrophic obstructive cardiomyopathy (HOCM). After starting the cibenzoline therapy, her hemodynamics improved and the peak pressure gradient at the midventricular level decreased to 35 mm Hg on echocardiography (Figure 2C). Her dyspnea improved, and she was …