Hypertrophic Cardiomyopathy Center, Heart and Vascular Institute and Children's Hospital, Cleveland Clinic, Cleveland, Ohio.
Hypertrophic Cardiomyopathy Center, Heart and Vascular Institute and Children's Hospital, Cleveland Clinic, Cleveland, Ohio.
Am J Cardiol. 2021 Feb 1;140:110-117. doi: 10.1016/j.amjcard.2020.10.060. Epub 2020 Nov 4.
We describe characteristics and outcomes of contemporary pediatric hypertrophic cardiomyopathy (PHC) patients. We studied 398 consecutive pediatric HC patients (<18 years, median 14 years, 65% boys) seen at our center between 2002 and 2018. Baseline clinical and pediatric echocardiographic data was collected. Left ventricular outflow tract gradient (LVOTG), LV fractional shortening and Z-score for left ventricular (LV) wall thickness were calculated. Sudden cardiac death (SCD), appropriate internal defibrillator discharge (ICD), myectomy, and orthotopic heart transplant (OHT) were composite primary endpoint. A total of 133 (33%) had symptoms (71 [18%] dyspnea, 77 [19%] angina, and 19 [5%] syncope), 109 (27%) were on beta-blockers; 179 (45%) had family history of HC. A total of 146 (37%) underwent genetic testing (of which 91 (62%) were HC-gene positive). Basal septal LV thickness, septal LV z-score and fractional shortening were 1.2 ± 0.6 cm, 4.8 ± 5.6, and 42% ± 8, whereas 23% had extreme LV hypertrophy (z-score > 6) and 8% had LVOTG >30 mm Hg (range 0 to 139 mm Hg). At a median of 5.9 years (interquartile range 2.4, 9), there were 23 (6%) ICD's placed, and 47 (12%) primary composite events (9 [2%] deaths, 3 [1%] appropriate ICD discharge, 29 [7%] myectomy, and 8 [2%] OHT). There were no in hospital deaths following myectomy/OHT. Presence of symptoms (Hazard ratio or HR 2.45), ventricular tachycardia (HR 1.52), and higher basal septal LV z-score (HR 1.10) were independently associated with primary composite outcomes. LV septal z-score >4 was independently associated with events on spline analysis. Rate of SCD/appropriate ICD discharge was 0.5%/year. In conclusion, contemporary PHC patients seen at an experienced center have excellent outcomes with presence of symptoms and higher LV septal thickness associated with primary composite events.
我们描述了当代儿科肥厚型心肌病（PHC）患者的特征和结局。我们研究了 2002 年至 2018 年间在我们中心就诊的 398 例连续儿科 HC 患者（<18 岁，中位年龄 14 岁，65%为男性）。收集了基线临床和儿科超声心动图数据。计算了左心室流出道梯度（LVOTG）、左心室（LV）壁厚度的 LV 短轴缩短率和 Z 分数。心脏性猝死（SCD）、适当的体内除颤放电（ICD）、心肌切除术和原位心脏移植（OHT）是复合主要终点。共有 133 例（33%）有症状（71 例[18%]呼吸困难，77 例[19%]心绞痛，19 例[5%]晕厥），109 例（27%）服用β受体阻滞剂；179 例（45%）有肥厚型心肌病家族史。共有 146 例（37%）接受了基因检测（其中 91 例[62%]为 HC 基因阳性）。基础室间隔 LV 厚度、室间隔 LVZ 分数和短轴缩短率分别为 1.2 ± 0.6cm、4.8 ± 5.6 和 42% ± 8%，而 23%有严重 LV 肥厚（Z 分数>6），8%有 LVOTG>30mmHg（范围 0 至 139mmHg）。中位随访 5.9 年（四分位间距 2.4，9），有 23 例（6%）放置 ICD，47 例（12%）发生原发性复合事件（9 例[2%]死亡，3 例[1%]适当 ICD 放电，29 例[7%]心肌切除术，8 例[2%] OHT）。心肌切除术/OHT 后无院内死亡。有症状（危险比或 HR 2.45）、室性心动过速（HR 1.52）和更高的基础室间隔 LVZ 分数（HR 1.10）与原发性复合结局独立相关。LV 室间隔 Z 分数>4 与样条分析中的事件独立相关。SCD/适当 ICD 放电的发生率为 0.5%/年。总之，在经验丰富的中心就诊的当代儿科 PHC 患者具有良好的结局，有症状和更高的 LV 室间隔厚度与原发性复合事件相关。
