Delsing Bas, Lopriore Enrico, Blom Nico, Te Pas Arjan B, Vandenbussche Frank P, Walther Frans J
Division of Neonatology, Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands.
Neonatology. 2007;92(2):134-8. doi: 10.1159/000101433. Epub 2007 Mar 29.
Chronic twin-to-twin transfusion syndrome (TTTS) is a complication of monochorionic twin gestations and is associated with high perinatal mortality and increased neurological, cardiovascular and renal morbidity.
To report the risk of severe persistent pulmonary hypertension of the newborn (PPHN) in TTTS and discuss the possible association between severe PPHN and TTTS.
All cases of monochorionic twins with severe PPHN at birth admitted to our nursery between June 2002 and July 2006 were reviewed retrospectively. We compared the incidence of severe PPHN in monochorionic twins with and without TTTS. Severe PPHN was diagnosed according to clinical and ultrasound criteria when an infant with a structurally normal heart had (1) severe hypoxemia and (2) evidence of a right-to-left shunt on persistent ductus arteriosus or foramen ovale, requiring treatment with inhaled nitric oxide (iNO).
In a consecutive series of 73 twin pregnancies with TTTS, 4 of the 135 live-born twins (3%) were affected by severe PPHN. All reacted promptly to treatment with iNO. The incidence of severe PPHN in monochorionic twins without TTTS was 0% (0/161).
In view of the severe clinical course in PPHN and need for adequate and prompt treatment with iNO, perinatologists should be aware of the increased risk of severe PPHN in TTTS.
慢性双胎输血综合征（TTTS）是单绒毛膜双胎妊娠的一种并发症，与围产期高死亡率以及神经、心血管和肾脏发病率增加相关。
报告TTTS患儿发生新生儿重度持续性肺动脉高压（PPHN）的风险，并探讨重度PPHN与TTTS之间可能存在的关联。
回顾性分析2002年6月至2006年7月间入住我院新生儿重症监护室的所有出生时患有重度PPHN的单绒毛膜双胎病例。比较了合并和未合并TTTS的单绒毛膜双胎中重度PPHN的发生率。当心脏结构正常的婴儿出现（1）严重低氧血症和（2）动脉导管未闭或卵圆孔水平存在右向左分流的证据，且需要吸入一氧化氮（iNO）治疗时，根据临床和超声标准诊断为重度PPHN。
在连续73例TTTS双胎妊娠中，135例存活双胎中有4例（3%）患重度PPHN。所有患儿对iNO治疗反应迅速。未合并TTTS的单绒毛膜双胎中重度PPHN的发生率为0%（0/161）。
鉴于PPHN严重的临床病程以及需要及时进行充分的iNO治疗，围产医学专家应意识到TTTS患儿发生重度PPHN的风险增加。
