Dykes E H, Raine P A, Arthur D S, Drainer I K, Young D G
J Pediatr Surg. 1985 Feb;20(1):49-52. doi: 10.1016/s0022-3468(85)80391-2.
Five infants with Pierre Robin syndrome developed evidence of carbon dioxide retention and congestive cardiac failure despite measures to alleviate upper airway obstruction. Investigations included chest radiography, electrocardiography, echocardiography, and cardiac catheterization; pulmonary hypertension was diagnosed. In two cases raised main pulmonary artery pressures of 40 mm Hg and 120 mm Hg were recorded. Relief of upper airway obstruction was achieved by tracheostomy in three cases and nasopharyngeal intubation in two cases, with reversal of signs of cor pulmonale in each. Four patients progressed well with no recurrence of cardiac problems but one died suddenly one month after apparently successful management by tracheostomy.
五名患有皮埃尔·罗宾综合征的婴儿尽管采取了缓解上呼吸道梗阻的措施，但仍出现了二氧化碳潴留和充血性心力衰竭的迹象。检查包括胸部X线摄影、心电图、超声心动图和心导管检查；诊断为肺动脉高压。在两例病例中，记录到主肺动脉压力分别升高至40毫米汞柱和120毫米汞柱。三例行气管切开术、两例行鼻咽插管术缓解了上呼吸道梗阻，每例患者的肺心病体征均得到逆转。四名患者病情进展良好，未再出现心脏问题，但有一名患者在气管切开术治疗看似成功一个月后突然死亡。
