引用本文：Wang JA, Lin XP, Jiang JB, Liu XB, Jiang J, Pu ZX, Wang LH, Li HJ, Lv F. Alcohol septal ablation for the treatment of hypertrophic obstructive cardiomyopathy in a patient with prior transcatheter aortic valve replacement. Cardiol Plus 2020;5:97-100.
Abstract
A 68-year-old man presented with chest distress recurring for the past 10 years. An echocardiogram demonstrated bicuspid aortic valve malformation with severe aortic stenosis and ventricular septal thickness of 22 mm. The patient underwent successful transcatheter aortic valve replacement (TAVR). Six months later, he complained of worsening dyspnea and chest distress (New York Heart Association Class III) on exertion. Besides a functional normal AV prosthesis, the echocardiography indicated the left ventricular outflow tract obstruction peak gradient of 122 mmHg at rest. Alcohol septal ablation was performed as the patient was unable to tolerate morrow procedure. His symptoms were relieved immediately after ablation, and no major cardiovascular events were observed during the 20 month follow up. In conclusion, among patients with concomitant hypertrophic obstructive cardiomyopathy and severe aortic valvular stenosis, consideration for TAVR and alcohol septal ablation should only be made for patients who are at high surgical risk or cannot tolerate thoracotomy.
摘要
一名68歲的男子在過去10年中反覆出現胸部不適，超聲心動圖顯示二尖瓣主動脈瓣畸形並伴有嚴重的主動脈瓣狹窄，室間隔厚度為22 mm。在該患者成功接受了經導管主動脈瓣置換術（TAVR）六個月後，發現在勞累後產生了呼吸困難和胸痛症狀（NYHA心功能分級III級）。超聲心動圖顯示AV假體功能正常，此外，靜止時左心室流出道梗阻的峰值梯度為122 mmHg。由於患者無法耐受後續手術，因此進行了酒精室間隔消融術。術後他的症狀立即緩解，並且在20個月的隨訪期間未觀察到重大心血管事件。
Keywords
Ablation techniques; alcohols; ventricular outflow obstruction; heart valve prosthesis Implantation; transcatheter aortic valve replacement
關鍵詞
消融術；酒精；心室流出道梗阻；心臟瓣膜假體植入；經導管主動脈瓣置換
Introduction
Hypertrophic obstructive cardiomyopathy is defined as increased thickness of the left ventricular (LV) wall that is not solely explained by abnormal loading conditions. LV outflow tract obstruction (LVOTO) is defined as an instantaneous peak LVOT pressure gradient of ≥30 mmHg as seen on Doppler study; this may occur at rest or during physiological provocations such as the Valsalva maneuver, standing, and exercise. Most people with LVOTO present symptoms such as exertional shortness of breath, chest pain, and/or syncope. The most commonly performed invasive procedures to treat LVOTO are ventricular septal myectomy (Morrow procedure), septal alcohol ablation, and the Liwen procedure (percutaneous intramyocardial septal radiofrequency ablation). The simultaneous occurrence of both aortic valve disease and hypertrophic obstructive cardiomyopathy is extremely rare. Herein, we present the successful treatment of a patient diagnosed with ypertrophic obstructive cardiomyopathy after undergoing transcatheter aortic valve replacement (TAVR) for concomitant aortic stenosis.
介紹
肥厚性梗阻性心肌病的定義為不僅僅由異常負荷情況引起的左心室（LV）壁厚度增加。左室流出道梗阻(LVOTO)的定義為在都卜勒超聲檢測中瞬時LVOT壓力梯度峰值≥30 mmHg，這種情況可能發生在休息或受到生理刺激時，大多數LVOTO患者會表現出勞累性氣短，胸痛和暈厥等症狀。治療LVOTO的最常用的侵入性治療方法是室間隔肌切除術（Morrow手術），室間隔酒精消融術和「Liwen術式」（中國劉麗文主任團隊開發的經皮心肌內室間隔射頻消融術）。患者同時發生主動脈瓣疾病和肥厚性梗阻性心肌病的情況極為罕見。本文中，我們報導了一例因主動脈瓣狹窄進行經導管主動脈瓣置換術（TAVR）治療，後被診斷為肥厚性梗阻性心肌病患者的治療成功案例。
Discussion
Managing concurrent aortic valve stenosis and subaortic LVOTO is a significant challenge. Echocardiogram via the modified Bernoulli equation may not enable clinicians to accurately determine the relative hemodynamic contribution of the different levels of obstruction; therefore, subaortic LVOTO in patients with severe aortic stenosis can be challenging as it may be masked by the high fixed afterload.
In our case, TAVR was performed first because echocardiography indicated that the aortic valve was bilobate with obvious calcification and dilatation of the ascending aorta. We found a significant increase in septal thickness within 4 months of the TAVR, indicating the role of afterload reduction influencing the geometry as well as the rapid growth of the septum. The patient reported no chest pain or syncope and was not determined to have any atrial fibrillation or bradycardia at 2 year follow up after the alcohol septal ablation. Surgical operation is the most suitable treatment for concurrent aortic valve stenosis and subaortic LVOTO. However, if the patient’s Society of Thoracic Surgeons score indicates a very high risk because of which the patient may not tolerate thoracotomy, we have to investigate other therapeutic options. This case implies that it may be feasible to perform TAVR and alcohol septal ablation in such cases. Liu et al. reported that echocardiography guided percutaneous intramyocardial septal radiofrequency ablation was effective for treating hypertrophic obstructive cardiomyopathy.
However, more observational data are needed to describe the hemodynamic and clinical outcomes of TAVR or septal ablation in patients with high surgical risk.
討論
同時處理主動脈瓣狹窄和主動脈瓣下左室流出道梗阻（LVOTO）是一個重大挑戰。僅通過超聲心動圖可能無法使臨床醫生準確確定不同程度的阻塞對血流動力學的影響。嚴重的主動脈瓣狹窄患者的主動脈下左室流出道梗阻（LVOTO）可能被掩蓋， 因此對醫生來說極具挑戰性。
我們首先進行TAVR，因為超聲心動圖顯示主動脈瓣為雙葉狀，且升主動脈明顯鈣化和擴張。我們發現TAVR術後4個月內患者室間隔厚度顯著增加，這表明後負荷減少對室間隔幾何形狀的影響以及室間隔快速生長的影響。在酒精室間隔消融術後的2年隨訪中，患者沒有報告胸痛或暈厥，也沒有發生任何房顫或心動過緩。對於並發主動脈瓣狹窄和主動脈瓣下左室流出道梗阻（LVOTO），手術是最合適的治療方法。然而，如果胸外科醫生評估患者有很高的手術風險，那麼可能無法進行開胸手術，必須制定其他治療方案。此病例提示在此類病例中實施TAVR和酒精隔消融是可行的。Liu等報導超聲心動圖引導下經皮心間隔內射頻消融術治療肥厚性梗阻性心肌病是有效的。然而，我們需要更多的觀察數據來描述高風險患者TAVR或室間隔消融的血流動力學和臨床結果。
Conclusion
Our case indicates that among patients with concomitant hypertrophic obstructive cardiomyopathy and severe aortic valvular stenosis, consideration for TAVR and alcohol septal ablation should only be made for patients who are at high surgical risk or cannot tolerate thoracotomy.
總結
我們的病例提示，在合併肥厚性阻塞性心肌病和嚴重主動脈瓣狹窄的患者中，只有手術風險高或不能耐受開胸手術的患者才應考慮TAVR和酒精間隔消融術。
關於本文的更多詳情，請閱覽Cardiology Plus 2020年第二期第91頁《Alcohol septal ablation for the treatment of hypertrophic obstructive cardiomyopathy in a patient with prior transcatheter aortic valve replacement》， 通訊作者為浙江大學醫學院附屬第二醫院心血管內科王建安教授和劉先寶教授。
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