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目的:分析新生儿三尖瓣腱索断裂引起的三尖瓣脱垂合并重度肺动脉高压的临床特点及治疗方法。方法:对河北省儿童医院新生儿科2018年11月收治的1例三尖瓣腱索断裂、三尖瓣脱垂合并重度肺动脉高压新生儿的临床资料进行回顾性分析。中文以“新生儿、三尖瓣脱垂、乳头肌或腱索断裂、肺动脉高压”为检索词在中国知网、万方数据库，英文以“（neonate OR newborn） AND （tricuspid valve prolapsed） AND （rupture OR necrosis） AND （papillary muscle OR chordae tendineae） AND （pulmonary hypertension）”为检索式在PubMed数据库中检索自建库至2020年9月报道的临床资料完整的文献，总结该病的临床特点及治疗方法。结果:患儿 男，9小时龄，因“生后皮肤青紫9 h”入院。患儿系足月剖宫产出生，生后10 min出现全身皮肤发绀、呼吸发憋，出生医院正压通气不能缓解，予气管插管、呼吸机辅助通气，生后9 h病情无好转转至河北省儿童医院。入院查血气分析提示动脉氧分压22.5 mmHg（1 mmHg=0.133 kPa），胸部正位X线片提示双肺透光度正常，超声心动图表现为三尖瓣前叶脱垂、三尖瓣大量反流、重度肺动脉高压、卵圆孔未闭，卵圆孔水平右向左分流。予继续呼吸机辅助呼吸，吸入一氧化氮、静脉用曲前列尼尔联合降肺动脉压治疗，患儿低氧血症逐渐缓解，好转出院。患儿在11月龄时行三尖瓣瓣叶成形术+三尖瓣瓣环成形术+卵圆孔未闭缝合术，术中证实为三尖瓣腱索断裂导致三尖瓣前叶脱垂，随访至22月龄发育状况良好。文献检索未发现中文文献，检索到英文文献12篇，加上本例共分析21例患儿资料，其中仅1例出生胎龄33周早产儿，其余20例均为足月新生儿；21例患儿中男12例。21例患儿均在生后早期出现发绀，接受气管插管机械通气，其他治疗包括吸入一氧化氮、静脉用米力农、血管活性药、利尿剂、前列环素类药物等，6例患儿接受了体外膜氧合治疗。早期报道的2例生后因心肺功能衰竭死亡，其余病例均在接受外科手术后存活，术中或尸检均发现三尖瓣脱垂、三尖瓣乳头肌或腱索断裂。结论:乳头肌或腱索断裂导致三尖瓣脱垂在新生儿期罕见，患儿可发生严重低氧血症，早期识别、充分心肺支持稳定血流动力学，适时外科手术可显著降低病死率。“,”Objective:To analyze the clinical characteristics and treatment of tricuspid valve prolapse caused by chordal rupture complicated with persistent pulmonary hypertension in neonates.Methods:The clinical data of a male neonate with tricuspid valve prolapse complicated with persistent pulmonary hypertension admitted to the Neonatal Intensive Care Unit of Children′s Hospital of Hebei Province in November 2018 was analyzed retrospectively. Related literature up to September 2020 was searched with the strategy of “(neonate OR newborn) AND (tricuspid valve prolapse) AND (rupture OR necrosis) AND (papillary muscle OR chordae tendineae) AND (pulmonary hypertension)” in Wanfang, CNKI and PubMed database in Chinese and English. The characteristics of the disease were summarized.Results:A male full-term neonate was admitted due to presenting severe cyanosis for 9 hours. He was born by caesarean section and presented severe cyanosis and dyspnea at 10 min of ages, unresponsive to the positive airway pressure resuscitation. After 9 hours of mechanical ventilation, there was no improvement. Thus he was transferred to Children′s Hospital of Hebei Province. On admission, the initial blood gas analysis showed an arterial partial pressure of oxygen of 22.5 mmHg (1 mmHg=0.133 kPa). The echocardiography revealed prolapsed anterior leaflet of tricuspid valve, severe tricuspid regurgitation (TR) and pulmonary artery hypertension, and right to left shunt via a patent foramen ovale. The arterial duct was closed. The chest X-ray was normal. The boy was treated with nitric oxide, milrinone, and continued mechanical ventilation initially. Addition of prostacyclin analog (treprostinil) on day 3 led to significant improvement of pulmonary blood flow, oxygenation, and stabilization, so that the extracorporeal membrane oxygenation therapy was avoided. At 11 months after birth, the boy underwent cardiac surgery. At surgery, the rupture of chordal tendineae in anterior leaflet of tricuspid valve was found. Tricuspid annuloplasty, valvuloplasty and repair of patent foramen ovale were successfully performed. The follow-up echocardiogram at postoperative 3 months showed only mild tricuspid insufficiency. The boy was well at last follow-up at 22 months of age with normal cognitive skill development. According to literature, 20 cases of papillary muscle or chordae tendineae rupture in neonates had been reported in 12 English papers. Among the total 21 neonates, there were 12 male infants and only one premature infant with gestational age of 33 weeks. They presented with profound cyanosis soon after birth. All of them received endotracheal intubation and mechanical ventilation. Other treatments included inhalation of nitric oxide, intravenous milrinone, vasoactive drugs, diuretics and prostacyclin, etc. Extracorporeal membrane oxygenation (ECMO) was used in 6 infants as a bridge to surgical treatment. Two cases reported earlier death of cardiopulmonary failure without operation and the rest 19 survived after surgery. The followed surgery or autopsy revealed that all of them had tricuspid valve prolapse, rupture of papillary muscle or chordae tendineae.Conclusions:The severe TR resulting from rupture of papillary muscle or chordate tendineae in neonates is rare and could cause severe hypoxemia. Early recognition, adequate cardiopulmonary support to stabilize the hemodynamic status and timely surgery can significantly reduce the mortality.