Hsieh W S, Yang P H, Fu R H
Department of Pediatrics, Chang Gung Children's Hospital, Chang Gung University, 199, Tung Hwa North Road, Taipei, 105 Taiwan.
Acta Paediatr Taiwan. 2001 Mar-Apr;42(2):94-100.
Persistent pulmonary hypertension of the newborn (PPHN) remains one of the most challenging situations in the neonatal intensive care unit, and it is associated with high mortality and morbidity. The optimal treatment for PPHN is controversial. We report our 9-year experience in the management of PPHN through a retrospective review of 29 neonates with persistent pulmonary hypertension. The diagnosis of PPHN is made by echocardiography and/or preductal and postductal oxygen tension difference. The treatment modalities include supportive medical care, vasodilator therapy, mechanical ventilation and correction of underlying conditions. The wide diversity of etiologies of PPHN, the complications of vasodilator therapy, the management of assisted ventilation, the mortality and the morbidity are evaluated. There are 29 patients enrolled in this study, including 18 male and 11 female babies. Twenty-two patients (72%) are referred from other hospitals. The mean birth body weight is 2707 +/- 693 grams (range: 1450-4100 grams) and the mean gestational age is 37.1 +/- 3.1 weeks (range: 31-41 weeks). The underlying clinical conditions include meconium aspiration syndrome (n = 8), perinatal asphyxia (n = 7), respiratory distress syndrome (n = 5), sepsis and/or pneumonia (n = 4), congenital diaphragmatic hernia (n = 3) and idiopathic persistent fetal circulation (n = 2). In addition to supportive medical care and correction of underlying clinical conditions, most of the patients receive vasodilator therapy (Tolazoline) and nonhyperventilation respirator management. The overall mortality rate is 27.6% (8/29). The duration on ventilator therapy in the survival group (9.3 +/- 8.6 days) is not significantly different from in the mortality group (6.0 +/- 7.1 days) (p = 0.13). There is also no statistically significant difference between these two groups both in the maximal alveolar-arterial oxygen tension difference (594 +/- 53 mmHg and 613 +/- 37 mmHg, p = 0.145) and in the maximal oxygenation index (49.7 +/- 29.6 and 61.1 +/- 36.9, p = 0.172) before vasodilator therapy. However, twenty-four hours after treatment, these two parameters change significantly with the former changes to 426 +/- 198 mmHg and 643 +/- 7 mmHg, respectively (p < 0.001), and the latter changes to 21.6 +/- 15.8 and 82.3 +/- 54.8, respectively (p < 0.001). Skin rash, gastrointestinal hemorrhage, hypotension and hyponatremia are the most common complications of Tolazoline therapy. Eight patients have pulmonary complications including pneumothorax (n = 5) and pulmonary interstitial emphysema (n = 3). Two patients develop chronic lung disease. Three patients have neurodevelopmental handicap. In conclusion, we achieve a survival rate of nearly 75% in PPHN mainly with the administration of Tolazoline therapy and the nonhyperventilation respirator approach. Further well-controlled and multicenter studies with newer treatment modalities are crucial for the improvement of survival of PPHN in Taiwan.
新生儿持续性肺动脉高压(PPHN)仍然是新生儿重症监护病房中最具挑战性的情况之一,并且与高死亡率和高发病率相关。PPHN的最佳治疗方法存在争议。我们通过对29例持续性肺动脉高压新生儿的回顾性研究报告了我们9年的PPHN管理经验。PPHN的诊断通过超声心动图和/或导管前和导管后氧分压差值来确定。治疗方式包括支持性医疗护理、血管扩张剂治疗、机械通气以及纠正潜在疾病。对PPHN病因的广泛多样性、血管扩张剂治疗的并发症、辅助通气的管理、死亡率和发病率进行了评估。本研究共纳入29例患者,其中男婴18例,女婴11例。22例患者(72%)从其他医院转诊而来。平均出生体重为2707±693克(范围:1450 - 4100克),平均胎龄为37.1±3.1周(范围:31 - 41周)。潜在的临床情况包括胎粪吸入综合征(n = 8)、围产期窒息(n = 7)、呼吸窘迫综合征(n = 5)、败血症和/或肺炎(n = 4)、先天性膈疝(n = 3)以及特发性持续性胎儿循环(n = 2)。除了支持性医疗护理和纠正潜在临床情况外,大多数患者接受血管扩张剂治疗(妥拉唑啉)和非过度通气呼吸管理。总体死亡率为27.6%(8/29)。存活组的机械通气治疗时间(9.3±8.6天)与死亡组(6.0±7.1天)无显著差异(p = 0.13)。在血管扩张剂治疗前,两组在最大肺泡 - 动脉氧分压差(594±53 mmHg和613±37 mmHg,p = 0.145)和最大氧合指数(49.7±29.6和61.1±36.9,p = 0.172)方面也无统计学显著差异。然而,治疗24小时后,这两个参数发生显著变化,前者分别变为426±198 mmHg和643±7 mmHg(p < 0.001),后者分别变为21.6±15.8和82.3±54.8(p < 0.001)。皮疹、胃肠道出血、低血压和低钠血症是妥拉唑啉治疗最常见的并发症。8例患者出现肺部并发症,包括气胸(n = 5)和肺间质气肿(n = 3)。2例患者发展为慢性肺病。3例患者有神经发育障碍。总之,我们主要通过妥拉唑啉治疗和非过度通气呼吸方法使PPHN的存活率达到近75%。采用更新的治疗方式进行进一步严格控制的多中心研究对于提高台湾PPHN的存活率至关重要。