Abstract：
BACKGROUND: Peripartum cardiomyopathy (PPCM) is a rare, life-threatening form of heart disease, frequently associated with gene alterations and, in some cases, presenting with advanced heart failure. Little is known about ventricular assist device (VAD) implantation in severe PPCM cases. We describe long-term follow-up of PPCM patients who were resistant to medical therapy and received mechanical circulatory support or heart transplant.
RESULTS: A total of 13 patients were included with mean follow-up of eight years. Mean age of PPCM onset was 33.7 ± 7.7 years. All patients were initially treated with angiotensin-converting enzyme inhibitors and beta-blockers, and four received bromocriptine. Overall, five patients received VADs (three biventricular, two isolated left ventricular) at median 27 days (range: 3 to 150) following childbirth. Two patients developed drive line infection. Due to the short support time, none of those patients had a stroke or VAD thrombosis. In total, five patients underwent heart transplantation, of which four previously had implanted VADs. Median time to transplantation from PPCM onset was 140 days (range: 43 to 776), and time to transplantation from VAD implantation were 7, 40, 132, and 735 days, respectively. All patients survived until most recent follow up, with the exception of one patient who died following unrelated abdominal surgery two years after PPCM recovery.
CONCLUSIONS: In patients with severe, life-threatening PPCM refractory to medical management, mechanical circulatory support with or without heart transplantation is a safe therapeutic option.
RESULTS: A total of 13 patients were included with mean follow-up of eight years. Mean age of PPCM onset was 33.7 ± 7.7 years. All patients were initially treated with angiotensin-converting enzyme inhibitors and beta-blockers, and four received bromocriptine. Overall, five patients received VADs (three biventricular, two isolated left ventricular) at median 27 days (range: 3 to 150) following childbirth. Two patients developed drive line infection. Due to the short support time, none of those patients had a stroke or VAD thrombosis. In total, five patients underwent heart transplantation, of which four previously had implanted VADs. Median time to transplantation from PPCM onset was 140 days (range: 43 to 776), and time to transplantation from VAD implantation were 7, 40, 132, and 735 days, respectively. All patients survived until most recent follow up, with the exception of one patient who died following unrelated abdominal surgery two years after PPCM recovery.
CONCLUSIONS: In patients with severe, life-threatening PPCM refractory to medical management, mechanical circulatory support with or without heart transplantation is a safe therapeutic option.
摘要：
背景：围产期心肌病（PPCM）是一种罕见的，危及生命的心脏病,经常与基因改变相关，在某些情况下,表现为晚期心力衰竭。对于严重PPCM病例中的心室辅助装置（VAD）植入知之甚少。我们描述了对药物治疗有抵抗力并接受机械循环支持或心脏移植的PPCM患者的长期随访。
结果：共纳入13例患者，平均随访8年。PPCM发病的平均年龄为33.7±7.7岁。所有患者最初接受血管紧张素转换酶抑制剂和β受体阻滞剂治疗，四个人接受了溴隐亭。总的来说,五名患者接受了VAD(三个双心室，两个孤立的左心室）在分娩后的中位数27天（范围：3至150）。两名患者发生驱动线感染。由于支持时间短,这些患者均无卒中或VAD血栓形成.总的来说,五名患者接受了心脏移植，其中四个以前植入了VAD。从PPCM开始的中位移植时间为140天（范围：43至776），从VAD植入到移植的时间分别为7、40、132和735天，分别。所有患者都存活到最近的随访，除了一名患者在PPCM恢复后两年因无关的腹部手术死亡。
结论：在重症患者中，危及生命的PPCM难以接受医疗管理，有或没有心脏移植的机械循环支持是一种安全的治疗选择。
结果：共纳入13例患者，平均随访8年。PPCM发病的平均年龄为33.7±7.7岁。所有患者最初接受血管紧张素转换酶抑制剂和β受体阻滞剂治疗，四个人接受了溴隐亭。总的来说,五名患者接受了VAD(三个双心室，两个孤立的左心室）在分娩后的中位数27天（范围：3至150）。两名患者发生驱动线感染。由于支持时间短,这些患者均无卒中或VAD血栓形成.总的来说,五名患者接受了心脏移植，其中四个以前植入了VAD。从PPCM开始的中位移植时间为140天（范围：43至776），从VAD植入到移植的时间分别为7、40、132和735天，分别。所有患者都存活到最近的随访，除了一名患者在PPCM恢复后两年因无关的腹部手术死亡。
结论：在重症患者中，危及生命的PPCM难以接受医疗管理，有或没有心脏移植的机械循环支持是一种安全的治疗选择。
