Ichinose E, Hirata K, Mikashima T, Sakamoto H, Toyoda O, Yoshioka F, Yokochi K, Kato H, Okada R
Department of Pediatrics and Child Health, Kurume University.
J Cardiogr. 1986 Sep;16(3):755-63.
We experienced three cases of unusual cardiac hypertrophy of the right ventricle or interventricular septum with severe congestive heart failure in the neonatal period. One patient had congenital heart disease consisting of membranous tricuspid atresia, absent pulmonary valve, patent ductus arteriosus, left single coronary artery and a hypoplastic pouch-like right ventricle. Very marked cardiac hypertrophy was observed in the right ventricle and interventricular septum. Histologically, there was no appreciable disorganization of the cardiac muscle. The etiology of the unusual hypertrophy of cardiac muscle in this patient is uncertain. The other two patients had asymmetrical septal hypertrophy of the left ventricle evidenced by two-dimensional echocardiography. Cardiac catheterization was performed for these two patients. There was no evidence of congenital heart disease; however, one patient had a significant pressure gradient in the outflow tracts of the left and right ventricles. These two patients' faces appeared unusual and they had minor anomalies of their fingers and ears. This unusual cardiac hypertrophy associated with cardiac anomalies and minor anomalies of the face and extremities comprise a specific type of cardiomyopathy in neonates. This should be distinguished from hypertropic cardiomyopathy of older children and adults.
我们遇到了三例新生儿期右心室或室间隔异常心肌肥厚并伴有严重充血性心力衰竭的病例。一名患者患有先天性心脏病，包括膜性三尖瓣闭锁、肺动脉瓣缺如、动脉导管未闭、左单冠状动脉以及发育不全的袋状右心室。在右心室和室间隔观察到非常明显的心肌肥厚。组织学上，心肌没有明显的结构紊乱。该患者心肌异常肥厚的病因尚不确定。另外两名患者经二维超声心动图证实有左心室不对称性室间隔肥厚。对这两名患者进行了心导管检查。没有先天性心脏病的证据；然而，一名患者在左、右心室流出道存在明显的压力梯度。这两名患者的面部外观异常，手指和耳朵有轻微畸形。这种与心脏异常以及面部和四肢轻微畸形相关的异常心肌肥厚构成了新生儿一种特殊类型的心肌病。这应与大龄儿童和成人的肥厚型心肌病相区分。
