探讨孕妇双胎妊娠合并肥厚型心肌病(HCM)的临床诊治及特点，并进行文献复习。

选取2017年10月12日，因孕龄为33⁺⁵孕周时皮肤瘙痒5 d，血液生化指标异常，于四川大学华西第二医院产科入院治疗的1例双胎妊娠合并HCM孕妇为研究对象。回顾性分析其临床资料、临床特点和诊疗过程，并进行相关文献复习。设定文献检索策略为，以"肥厚型心肌病""双胎""心肌病""twin pregnancy""hypertrophic cardiomyopathy""cardiomyopathy"为关键词，在中国知网、万方数据知识服务平台、维普中文期刊服务平台、PubMed及Web of Science(WOS)数据库中检索相关文献，检索时间设定为上述数据库建库至2020年1月1日。本研究符合2013年修订的《世界医学协会赫尔辛基宣言》要求。

①本例患者在本院诊治结果如下。病史采集：30岁，孕龄为33⁺⁵孕周，G₂P₀⁺¹，体外受精-胚胎移植技术(IVF-ET)术后(移植冻胚2枚)，HCM史4⁺年，反复告知孕妇夫妇妊娠风险，并由多学科团队(MDT)充分评估后，孕妇在产科及心内科共同监管下定期产前检查。因胎膜早破(PROM)后临产，于胎龄34⁺¹周在全身麻醉下急诊剖宫产术分娩，手术顺利。术后立即转入重症监护病房(ICU)。患者术后出现急性心力衰竭，经呼吸支持、镇静、抗感染、减轻容量负荷、改善心功能等治疗后病情平稳，经心血管科医师评估，给予口服美托洛尔控制心室率、降低心肌氧耗治疗，术后2 d病情好转。目前随访显示母婴情况均良好。②文献复习结果：根据本研究设定的文献检索策略，未检索到相关孕妇双胎妊娠合并HCM的相关报道，遂扩大检索范围，将检索词由"双胎"改为"妊娠"，共计检索到12篇国内外相关文献，涉及260例妊娠合并HCM患者，共计284次妊娠，所有患者均为单胎妊娠。这284次妊娠中，孕产妇发生心力衰竭为28次，2例死亡，其余患者均治疗有效。其中52次妊娠数据中新生儿未报道，余232次妊娠中，胎儿早产为46例，胎儿宫内死亡为5例。

多数无明显症状或轻症妊娠合并HCM孕妇可耐受妊娠及分娩，并且母儿结局多良好。但是，对于多胎妊娠合并HCM患者，即使孕妇孕期未发生相关不良心血管事件，分娩后由于血容量变化，对于本身罹患心肌病者是巨大挑战。

To investigate clinical characteristics of twin pregnancy combined with hypertrophic cardiomyopathy (HCM) and review related literature.

A pregnant woman with a gestational age of 33^{+ 5} gestational weeks who admitted to the Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University with twin pregnancy combined with HCM on October 12, 2017 was included into this study. The clinical data, characteristics and treatment process were retrospectively analyzed, and related literature were reviewed. In this study, the literature review of HCM in twin pregnancy was conducted with the following keywords " hypertrophic cardiomyopathy" " twin pregnancy" " cardiomyopathy" " twin pregnancy" " hypertrophic cardiomyopathy" and " cardiomyopathy" . The search period was set from database inception to January 1, 2020, and the clinical characteristics and treatment protocols of the disease were summarized. This study met the requirements of World Medical Association Declaration of Helsinki revised in 2013.

The clinical data and consultation process of this patient were collected as follows. ①This patient was 30 years old, post-in vitro fertilization-embryo transfer (IVF-ET) (two frozen embryos were transferred), with a history of HCM for 4⁺ years. The patient was repeatedly informed of the risks and after full assessment by the multiple disciplinary team (MDT), the patient underwent regular prenatal check up under the supervision of the Department of Obstetrics and Cardiology. Due to premature rupture of membranes (PROM), the patient underwent an emergency cesarean section at 34^{+ 1} weeks′ gestation under general anesthesia after assessment by the MDT, and the operation was successful. Immediately after surgery, she was transferred to the intensive care unit (ICU) for monitoring. Postoperatively, the patient developed acute heart failure, which stabilized after treatment with respiratory support, sedation, anti-infection, reduction of volume load and improvement of cardiac function, etc.. The patient was evaluated by a cardiovascular specialist and given beta-lactone to control the ventricular rate and reduce myocardial oxygen consumption. Two days after surgery, the condition was improved and this patient was transferred back to the obstetrics ward. She is still being followed up and both mother and baby are in good condition. ②Results of literature review were as follow. According to the literature search strategy of this study, no relevant reports of twin pregnancy combined with HCM were retrieved, so the search was expanded by changing keywords from " twin" to " pregnancy" , and a total of 12 articles were retrieved. Among these 12 articles, a total of 260 cases of pregnancy with HCM were retrieved, involving a total of 284 pregnancies, all of which were singleton pregnancy. Among these 284 pregnancies, 28 cases of heart failure occurred; 2 patients died, and the remaining patients with heart failure were treated effectively and their condition improved. Except for 52 pregnancies in which the neonatal outcome was unknown, there were 46 cases of preterm fetal delivery and 5 cases of intrauterine fetal death in the remaining 232 pregnancies.

Most patients with asymptomatic or mild HCM can tolerate pregnancy and have good maternal and infant outcomes. However, in patients with HCM in multiple pregnancies, even if no maternal adverse cardiovascular events occurred during pregnancy, the rapid change in blood volume after delivery will be a great challenge for patients with cardiomyopathy.