Denfield S W, Rosenthal G, Gajarski R J, Bricker J T, Schowengerdt K O, Price J K, Towbin J A
Division of Pediatric Cardiology, Texas Children's Hospital, Houston, USA.
Tex Heart Inst J. 1997;24(1):38-44.
Restrictive cardiomyopathy is rare in childhood and little is known about the causes and outcome. This lack of information results in extrapolation of adult data to the care and management of children, who might require different treatment from that of adults. This study was undertaken retrospectively to evaluate the causes and natural history of restrictive cardiomyopathy in childhood. Twelve cases of restrictive cardiomyopathy were identified by database review of patient records from 1967 to 1994. The cases were selected on the basis of echocardiographic and cardiac catheterization criteria. Charts were reviewed for the following variables: age, sex, cause, right-and left-sided hemodynamics, pulmonary vascular resistance index, shortening fraction, therapy, and outcome. There were 6 males and 6 females with a mean age of 4.6 years at presentation (median, 3.4 yr; range, 0.9 to 12.3 yr). Etiologies included hypertrophic cardiomyopathy in 3 patients, cardiac hypertrophy with restrictive physiology in 3, idiopathic in 2, familial in 2 (twins), "chronic eosinophilia" in 1, and "post inflammatory" with no definitive causes in 1. At presentation the mean shortening fraction was 33% +/- 2% (mean +/- SEM), average right ventricular pressures were 44/13 +/- 3/1, average left ventricular pressures were 88/25 +/- 4/3, and the mean pulmonary vascular resistance index was 3.4 +/- 1.3 U.m2 (n = 9), but increased to 9.9 +/- 3.1 U.m2 (n = 5, p = 0.04) by 1 to 4 years after diagnosis. Four of the 12 patients had embolic events (1, recurrent pulmonary emboli; 1, saddle femoral embolus; 2, cerebrovascular accidents) and 9 of 12 died within 6.3 years despite medical therapies, which included diuretics, verapamil, propranolol, digoxin, and captopril. In conclusion, restrictive cardiomyopathy in childhood is commonly idiopathic or associated with cardiac hypertrophy, and the prognosis is poor. Embolic events occurred in 33% of our patients, and 9 of 12 patients died within 6.3 years. Within 1 to 4 years of diagnosis, patients may develop a markedly elevated pulmonary vascular resistance index; therefore, transplantation should be considered early.
限制型心肌病在儿童时期较为罕见，其病因和预后鲜为人知。由于缺乏相关信息，在对儿童的护理和管理中只能套用成人的数据，而儿童可能需要与成人不同的治疗方法。本研究通过回顾性研究来评估儿童限制型心肌病的病因和自然病史。通过对1967年至1994年患者记录的数据库回顾，确定了12例限制型心肌病病例。这些病例是根据超声心动图和心导管检查标准选取的。对病历进行了以下变量的审查：年龄、性别、病因、左右心血流动力学、肺血管阻力指数、缩短分数、治疗方法和预后。共有6名男性和6名女性，就诊时平均年龄为4.6岁（中位数为3.4岁；范围为0.9至12.3岁）。病因包括肥厚型心肌病3例、心脏肥大伴限制型生理学改变3例、特发性2例、家族性2例（双胞胎）、“慢性嗜酸性粒细胞增多症”1例、“炎症后”但病因不明1例。就诊时平均缩短分数为33%±2%（平均值±标准误），平均右心室压力为44/13±3/1，平均左心室压力为88/25±4/3，平均肺血管阻力指数为3.4±1.3U.m2（n = 9），但在诊断后1至4年增加到9.9±3.1U.m2（n = 5，p = 0.04）。12例患者中有4例发生栓塞事件（1例为复发性肺栓塞；1例为鞍状股动脉栓塞；2例为脑血管意外），12例中有9例在6.3年内死亡，尽管接受了包括利尿剂、维拉帕米、普萘洛尔、地高辛和卡托普利在内的药物治疗。总之，儿童限制型心肌病通常为特发性或与心脏肥大有关，预后较差。我们的患者中有33%发生栓塞事件，12例中有9例在6.3年内死亡。在诊断后1至4年内，患者的肺血管阻力指数可能会显著升高；因此，应尽早考虑进行移植。
