Tsai C E, Chou Y H, Tsou K I, Huang S F, Lee C Y
Department of Pediatrics, College of Medicine, National Taiwan University, Taipei, Taiwan R.O.C.
J Formos Med Assoc. 1993 Sep;92(9):842-4.
A 3700-g male infant born at 37 weeks' gestation presented with cyanosis at birth. He was diagnosed as having persistent pulmonary hypertension of the newborn (PPHN) on the basis of persistent hypoxemia, despite continuous mechanical ventilatory support with 100% O2, and right-to-left shunting through the foramen ovale shown by Doppler echocardiography. Treatment with hyperventilation, and administration of tolazoline, prostaglandin E1 and MgSO4 failed to reverse his hypoxemia. High ventilator settings were required, and pneumothoraces ensued. Airway resistance increased gradually with development of hypercapnia and deterioration of hypoxemia. Bradycardia unresponsive to resuscitation occurred, and he died at eight days of age. Postmortem examination of the lungs revealed increased peripheral connective tissue and diffuse extension of medial smooth muscle to the precapillary pulmonary arteries. Excessive antenatal muscularization of the peripheral pulmonary arteries and resultant increased vasoconstriction capacity may have played an important role in the pathogenesis of PPHN in this case.
一名孕37周出生、体重3700克的男婴出生时即出现发绀。尽管给予100%氧气持续机械通气支持，但他仍持续低氧血症，并通过多普勒超声心动图显示卵圆孔存在右向左分流，据此被诊断为新生儿持续肺动脉高压（PPHN）。采用过度通气治疗，以及给予妥拉唑啉、前列腺素E1和硫酸镁治疗均未能纠正其低氧血症。需要设置高通气参数，随后出现气胸。随着高碳酸血症的发展和低氧血症的恶化，气道阻力逐渐增加。出现对复苏无反应的心动过缓，患儿于8日龄时死亡。肺部尸检显示外周结缔组织增多，且中膜平滑肌弥漫性延伸至肺小动脉前毛细血管。产前外周肺动脉过度肌化以及由此导致的血管收缩能力增加可能在该病例PPHN的发病机制中起了重要作用。
