Recovery from severe heart failure following peripartum cardiomyopathy

Objectives

Traditional concepts surrounding peripartum cardiomyopathy (PPCM) hold that if recovery does not occur within 6 months of diagnosis, it is unlikely to happen. The purpose of the study was to determine the length of time required for recovery of left ventricular systolic function.

Methods

Patients were identified from the Hôpital Albert Schweitzer PPCM Registry over the period 2000-2008. Echocardiography was carried out at diagnosis and every 6 months thereafter. Recovery of systolic heart function was defined as left ventricular ejection fraction greater than 0.50.

Results

Thirty-two out of 116 (27.6%) PPCM patients reached recovery levels, with 75% of patients taking over 12 months to recover. Shortest time to recovery was 3 months and longest time to recovery was 48 months.

Conclusion

Recovery of left ventricular systolic function in PPCM patients often requires longer than 6-12 months following diagnosis. It is important to continue effective treatment and follow-up for a sufficient period to assure maximum benefit.     

Cardiomyopathy in pregnancy

Cardiomyopathy during pregnancy is uncommon but potentially catastrophic to maternal health, accounting for up to 11% of maternal deaths. Peripartum cardiomyopathy is diagnosed in women without a history of heart disease 1 month before delivery or within 5 months postpartum. About half of all women will have full myocardial recovery within 6 months of diagnosis, but complications such as severe heart failure or death are not rare. African-American women have higher rates of diagnosis and adverse events. Women with preexisting cardiomyopathy, such as dilated or hypertrophic cardiomyopathy, followed closely during pregnancy often tolerate pregnancy and delivery. Risk factors for adverse outcomes include functional status at baseline, severity of systolic dysfunction or outflow tract gradient, or history of prior cardiac event, such as arrhythmia or stroke. The level of brain natriuretic peptide (BNP) can be used to risk stratify women for adverse events. Pregnant women with cardiomyopathy should be followed closely by a multidisciplinary team comprised of nurses, obstetricians, neonatologists, cardiologists, anesthesiologists, and cardiac surgeons.     

Risk of heart failure relapse in subsequent pregnancy among peripartum cardiomyopathy mothers

Objective

To quantify the level of risk for heart failure relapse in a subsequent pregnancy in women who have had peripartum cardiomyopathy (PPCM), and to test the hypothesis that meeting additional criteria may help lower the risk.

Methods

Prospectively-identified PPCM patients volunteering between 2003 and 2009 were identified from the PPCM Registry of Hôpital Albert Schweitzer, Deschapelles, Haiti, and an internet support group. Data were assessed for full adherence to monitoring and diagnostic criteria, clinical data, statistical analysis, and reporting.

Results

Of 61 post-PPCM pregnancies identified, there were 18 relapses (29.5%) of heart failure. Of 26 pregnancies with a left ventricular ejection fraction (LVEF) of less than 0.55 prior to the pregnancy, relapse occurred in 12 (46.2%) pregnancies. Of 35 pregnancies with an LVEF of 0.55 or greater prior to the pregnancy, relapse occurred in 6 (17.1%) (P < 0.01). No relapses occurred in 9 women who also demonstrated adequate contractile reserve.

Conclusion

The most important criterion associated with reduced risk for heart failure relapse in a post-PPCM pregnancy is recovery defined by an LVEF 0.55 or greater before the subsequent pregnancy. Exercise stress echocardiography showing adequate contractile reserve may help to identify women at an even lower risk of relapse.     

Peripartum cardiomyopathy

Objective

To determine the clinical and echocardiographic profiles of women with peripartum cardiomyopathy and ascertain the natural course of the disease.

Methods

Fifty-six women with peripartum cardiomyopathy were followed up for a mean period of 6.1 years and their clinical and echocardiographic profiles were studied as well as their outcomes.

Results

The mean ± SD age at presentation was 31 ± 5 years and mean parity was 2.6 ± 1. Of the 56 patients, 18 (32.1%) had NYHA Class II, 24 (42.9%) had NYHA Class III, and 14 (25%) had NYHA Class IV symptoms, and 21 (37.5%) and 35 (62.5%), respectively, presented with features of heart failure during pregnancy and the postpartum. During follow-up, the left ventricular ejection fraction improved from 31% ± 7.2% to 43% ± 8% (P ≤ 0.05). Nine patients (16.1%) became pregnant, with a mortality of 55.5% during pregnancy and 23.2% during follow-up.

Conclusion

Women with peripartum cardiomyopathy present with severe left ventricular dysfunction late in the pregnancy or early in the puerperium. A considerable number of women still die from this condition despite the use of angiotensin-converting enzyme inhibitors and β-blockers. A subsequent pregnancy carries a very high risk of mortality.     

围产期心肌病不同诊断标准的比较与分析

目的 探讨目前国内围产期心肌病诊断标准中产科标准及内科标准与国外Hibbard标准的符合情况及差异点.方法 1995年3月至2009年9月北京大学人民医院及中国医学科学院阜外心血管病医院住院的围产期心肌病患者49例.其中由北京大学人民医院产科按产科标准诊断的8例,内科按内科标准诊断的22例和由中国医学科学院阜外心血管病医院内科按内科标准诊断19例,按内科标准共诊断41例.(1)产科诊断标准:既往无心血管系统疾病史,于妊娠28周后至产后6个月内发生的扩张型心肌病即为围产期心肌病.(2)内科诊断标准:围产期心肌病是指发生于妊娠最后1个月或产后5个月内的不明原冈的心脏扩大和心功能衰竭,超声心动图诊断标准为:左心室舒张末期内径(LVEDd)>5.0 cm;左心室射血分数(LVEF)<45%和(或)左心室缩短分数(LVFS)<30%;或LVEDd>2.7 cnL/体表面积(m2);或LVEDd>年龄和体表面积预测值的117%.(3)Hibbard诊断标准:妊娠最后1个月至产后5个月内发生的心功能衰竭;既往无心脏病病史;无其他导致心功能衰竭的原凶;超声心动图标准为:LVEF<45%和(或)LVFS<30%;LVEDd>2.7 cm/m2;此4项标准必须全部符合才能诊断围产期心肌病.分析产科标准和内科标准诊断围产期心肌病与Hibbard标准的符合情况及不符合的原因.结果 (1)产科标准诊断围产期心肌病与Hibbard标准的符合情况:产科标准诊断的8例围产期心肌病患者中,不符合Hibbard标准有6例(6/8).其中,发病时间不符合2例,分别合并子痫前期和急性肾盂肾炎重度感染,可以成为导致心功能衰竭的原因;LVEF.均不符合超声心动图标准.另4例患者中,2例同时合并重度子痫前期和重度贫血,1例单纯合并子痫前期,1例单纯合并中度贫血,可以成为导致心功能衰竭的原因;同时该4例中LVEDd和LVEF均不符合标准者2例,LVEDd或LVEF不符合标准者各1例.(2)内科标准诊断围产期心肌病与Hibbard标准的符合情况:内科标准诊断的41例围产期心肌病患者中,不符合Hibbard标准者有7例(17%,7/41),与产科标准诊断的不符合率比较,差异有统计学意义(P<0.01).其中,发病时间不符合3例,均合并子痫前期,成为导致心功能衰竭的原因.另4例中LVEDd和INEF均不符合标准者1例,LVEF不符合标准者3例.(3)国内标准诊断围产期心肌病与Hibbard标准不符合的原因:国内标准诊断的49例围产期心肌病患者中共有13例不符合Hibbard标准,其中,发病时间不符合5例(10%,5/49),均为孕期发病患者;存在其他导致心功能衰竭的原因9例(18%,9/49),包括子痫前期5例、子痫前期合并贫血2例、贫血 1例、急性肾盂肾炎重度感染1例,均为孕期发病患者,合并子痫前期共7例(7/9);超声心动图检杏结果 不符合10例(20%,10/49).结论 与Hibbard标准相比,国内围产期心肌病诊断标准缺乏严格统一性;比较而言,内科标准有较好的符合性;而产科标准则有较大差异,成为导致产科诊断嗣产期心肌病符合率不高的主要原因;国内诊断围产期心肌病与Hibbard标准不符合的主要原因在于超声心动图检查结果 不达标.

Abstract：

Objective To compare the differences and similarities between the diagnostic criteria of obstetrics and internal medicine in China with that of Hibbard for peripartum cardiomyopathy (PPCM).Methods From March 1995 to September 2009, a total of 49 patients were diagnosed as PPCM at the Peking University People's Hospital and the Fu Wai Hospital in Beijing, China. Obstetric diagnostic criteria was:PPCM was one of dilated cardiomyopathy,occurred during the third trimester of pregnancy through the 6th month postpartum,and without cardiovascular diseases before. Internal medicine diagnostic criteria was:PPCM was unexplained cardiomegaly and heart failure, occurred during the last month of pregnancy through the 5th month postpartum, and meet the echocardiographic criteria of dialated cardiomyopathy as follows:left ventricular end-diastolic dimension (LVEDd) greater than 5.0 cm; left ventricular ejection fraction (LVEF)less than 45% , and(or) left ventricular fractional shortening (LVFS) less than 30% ; or LVEDd greater than 2. 7 cm/body surface area (m2) ; or LVEDd > 117% of age and body surface area predictive value. Hibbard diagnostic criteria was: All four of the following: (1) heart failure within last month of pregnancy and 5 months postpartum; (2) absence of prior heart disease; (3) no determinable cause; (4) strict echocardiographic indication of left ventricular dysfunction; LVEF less than 45% , and/or LVFS less than 30% , and LVEDd greater than 2. 7 cm/m2. The compliance between obstetric and internal criteria with Hibbard criteria, and the reasons of incompliance between Chinese and international criteria were analyzed. Results Eight patients were diagnosed of PPCM by obstetricians according to Chinese obstetric criteria. Among them, 6 patients (6/8) did not meet Hibbard criteria. 2 of the six did not reach the time regulated in the criteria. All of the six had other determinable causes for heart failure, and their echocardiographic results did not meet the diagnostic standard either. The other 41 patients were diagnosed of PPCM by physicians according to Chinese internal medicine criteria. Among them, 7 patients (17%) did not meet Hibbard criteria, 3 of the seven did not reach the time regulated in the criteria, and had other determinable causes for heart failure either. 4 of the seven did not meet the echocardiographic standard part in the criteria. The Chinese internal medicine diagnostic criteria has a significant higher coincidence rate with Hibbard criteria, compared to Chinese obstetric criteria (83% vs. 25% ; P <0. 01). Among all 13 patients whose PPCM diagnosis did not meet Hibbard criteria, 5 cases did not reach the time regulated in the criteria, 9 cases had other determinable causes for heart failure, and 10 cases did not meet the echocardiographic standard part in the criteria. Preeclampsia was the most common determinable causes for heart failure, accounted for 7 cases. Conclusion There is obvious difference between Chinese and Hibbard diagnostic criteria for PPCM, especially Chinese obstetric criteria.     

Symptomatic recovery and pharmacological management in a clinical cohort with peripartum cardiomyopathy

Aim: We aimed to characterize the clinical course with focus on pharmacological management of peripartum cardiomyopathy (PPCM) in Sweden.

Methods: Twenty-four consecutive patients were retrospectively identified among women presenting with PPCM in Western Sweden. Of these, 14 had concomitant preeclampsia. There was only one fatality. The mean (standard deviation) left ventricular ejection fraction (LVEF) at diagnosis was 35.0?±?9.9%. Ten women, 47.6%, required intensive care unit (ICU) admission. All patients received β-blockers (BB) and angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers (ACE-I/ARB), which were tapered off over a mean/median period of 3.3/2.5?years with only one case of worsening heart failure. The mean follow-up for medication was 7.9?±?2.6?years. Early and late/non-recovery was defined as New York Heart Association (NYHA) functional class I and NYHA II–IV at one?year, respectively. Late recovery was associated with larger LVEDD at diagnosis (56.8 versus 62.4?mm) was associated with late recovery, p?=?.02.

Results and conclusions: PPCM had an overall good prognosis in this cohort. Left ventricular dilation at presentation was a predictor of worse prognosis. Concurrent preeclampsia was common, but was associated with better prognosis. Medication was safely discontinued in 75% of patients.     

Pregnancy in women with arrhythmogenic right ventricular cardiomyopathy/dysplasia

OBJECTIVES: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a cardiac disease characterised by myocardial necrosis followed by fibro-fatty substitution leading to the onset of ventricular arrhythmias. The aim of the present study was to analyse pregnancy in women affected by this condition. STUDY DESIGN: Six women affected by ARVC/D who underwent a pregnancy were studied with a follow-up programme, consisting of 12-lead ECG, signal-averaged ECG, 24-h ECG and two-dimensional and Doppler echocardiogram performed before the beginning of the pregnancy, at 3rd and 7th month of gestation and after the delivery. RESULTS: All women were on antiarrhythmic therapy during pregnancy; two complained of palpitations in the last 3 months. Delivery was performed at full terms in all, with caesarean section and epidural anaesthesia in four. Mean weight at birth was 3490g. No adverse reactions on the newborns were detected. All patients were advised against breast-feeding. No significant morphological changes were detected. During the period following the delivery (1-6years, mean 2,6years) one subject experienced a sustained ventricular tachycardia. CONCLUSIONS: Pregnancy seems to be well tolerated in patients affected by ARVC/D, but a programmed clinical protocol is mandatory particularly in the last trimester and puerperium, due to increased risk of ventricular arrhythmias.     

High prevalence of viral genomes and inflammation in peripartum cardiomyopathy

Molecular pathologic investigation of endomyocardial biopsy specimens from 26 patients with peripartum cardiomyopathy revealed viral genomes (parvovirus B19, human herpes virus 6, Epstein-Barr virus, and human cytomegalovirus) in 8 patients (30.7%) that were associated immunohistologically with interstitial inflammation. These findings indicate a high prevalence of virus-associated inflammatory changes in peripartum cardiomyopathy.     

我国围产期心肌病流行病学特点及转归荟萃分析

目的了解我国围产期心肌病（peripartum candiomyophthy,PPCM）流行病学及临床发病的特点和转归。方法 检索1986年4月至2010年4月在中国期刊全文数据库（CNKI）和万方数据库中围产期心肌病的研究资料,总结分析我国PPCM的流行趋势、发病特点和临床转归。结果①共检索到文献170篇,根据文献资料入选及排除标准,经筛选并纳入本次荟萃的文献共有68篇,累计PPCM患者1 097例;②资料显示我国PPCM初产妇病例数多于经产妇（53.3%vs 46.7%）;农村患者占81.5%;③产后3个月内发生PPCM者比例最高（67.3%）,其次是妊娠最后1个月内（20.4%）;④PPCM患者合并贫血比例较高（51.4%）,其次是高血压（48.5%）;⑤31.2%患者治愈,59.7%患者好转,未愈/死亡患者占9.1%。结论深入了解我国围产期心肌病的流行病学及发病特点和转归,有利于我们对本病的防治。     

前列腺素E_1治疗围产期心肌病与扩张性心肌病疗效的超声评价

目的 :用超声心动图评价前列腺素E1治疗围产期心肌病的疗效。方法 :选择围产期心肌病 (PCM) 2 1例、原发性扩张型心肌病 (DCM) 33例和正常对照 35例 ,分别在常规治疗基础上给予前列腺素E1治疗 (10mg/日 ,静脉滴注 ,疗程为 2 0天 )。于治疗前及治疗 10天、2 0天时行心脏超声检查 ,测量左心室 (LVD)、左心房 (LA)、右心室 (RV)、右心房 (RA)内径 ,室间隔和左室后壁厚度、肺动脉收缩压及左室射血分数 (EF) ,并加以比较。结果 :(1)治疗 10天及 2 0天时 ,PCM组LVD均较治疗前显著缩小 ,肺动脉压显著降低 ,EF值显著升高 (P <0 .0 5 ,P <0 .0 1) ,且治疗 2 0天时肺动脉压较治疗 10天时显著降低。DCM组LVD亦均较治疗前显著缩小 ,肺动脉压显著降低 ,EF值显著升高 (P <0 .0 5 ) ,但治疗 2 0天时 ,上述各指标较治疗 10天时无显著改善。 (2 )治疗 2 0天时 ,PCM组在LVD、肺动脉平均压及EF值方面的改善与DCM组比较均有显著性差异。结论 :前列腺素E1治疗PCM疗效优于DCM。     

Clinical outcomes and maternal associated conditions between antepartum and postpartum-onset of peripartum cardiomyopathy

ObjectivePeripartum cardiomyopathy (PPCM) developed from late pregnancy to five months after delivery. Women with PPCM have the risk of mortality or non-recovered cardiac function. We aimed to investigate women with PPCM in Taiwan.Materials and methodsThe retrospective study recruited patients with PPCM from January 2002 to October 2018 in a tertiary center. We evaluated the presentations, onset, associated conditions, maternal and fetal outcomes, follow-up cardiac function, and subsequent pregnancies. The clinical data were compared between antepartum and postpartum-onset of PPCM.ResultsThirty women were identified and seventeen (56.6%) patients were antepartum-onset. The delivery time, ranged from 26 to 40 weeks, was mostly at 35 weeks. Twenty-one patients had cardiac function follow-up and seven (33.3%) were non-recovered in six months. The associated conditions of PPCM included age >30, primiparity, preeclampsia or hypertension, obesity, twin pregnancy, and tocolysis. The maternal characteristics and associated conditions were not significant different, but early preterm (32.8 ± 3.6 vs. 35.5 ± 2.4 weeks, p = 0.042) and lower Apgar scores in one (7 vs. 9, p = 0.002) and 5 min (9 vs. 10, p = 0.005) were observed in the antepartum-onset group.ConclusionIn conclusion, PPCM commonly occurred around 35 weeks of gestation, ranged from 26 to 40 weeks. Additionally, there were risks of early preterm and low Apgar scores in women with antepartum-onset of PPCM.     

围产期心肌病诊治

围产期心肌病（PPCM）典型症状以心力衰竭、呼吸困难为主要表现,超声心动图是首选检查。溴隐亭及口服抗心力衰竭药物是治疗主要手段,对于血流动力学不稳定的心力衰竭孕妇,无论孕周大小均应终止妊娠。孕产妇病死率的预测指标为纽约心脏病协会（NYHA）Ⅲ/Ⅳ分级和左心室射血分数（LVEF）＜40%。     

Emergency peripartum hysterectomy in the nulliparous patient.

OBJECTIVE: To find out the incidence, indications and outcomes of emergency peripartum hysterectomy in the nulliparous woman. METHOD: Retrospective study at the Korle Bu Teaching Hospital in Accra, Ghana, between January 1995 and December 2003. RESULTS: During the eight-year study period there were 92,966 deliveries out of which 36,550 (39.5%) were for the nulliparous. Peripartum hysterectomy was performed for 39 nulliparous women, thus giving an incidence of 1 per 1000 nulliparous deliveries. The indications for the hysterectomy were: atonic uterus 28 (71.8%), ruptured uterus 8 (20.5%) and placenta praevia/accreta 3 (7.7%). The perinatal losses were 8 (20.5%) and the total blood loss ranged from 1 to 4.5 l. There were no maternal deaths but there were 7 near-missed fatalities. CONCLUSION: Though rare, peripartum hysterectomy in the nulliparous patients carries high maternal morbidity and perinatal mortality.     

Emergency peripartum hysterectomy in a tertiary Istanbul hospital

OBJECTIVE: To evaluate the incidence, risk factors, indications, outcomes and complications of emergency peripartum hysterectomy performed after cesarean and vaginal deliveries. METHOD(S): We analyzed retrospectively 28 cases of emergency peripartum hysterectomy operations performed between February 2001 and February 2007 at the Istanbul Goztepe Training and Research Hospital, which is a teaching hospital operating under the Turkish Ministry of Health. The indications, risk factors and the associated complications were compared with control groups. Statistical analysis was performed using the STATA version 7.0 statistical package (Stata Corporation, College Station, TX, USA). RESULT(S): The overall incidence of emergency peripartum hysterectomy at our hospital is 0,37 in 1,000 deliveries. Abnormal placental adherence and uterine atony comprised 85% of the indications for peripartum hysterectomy. Postoperative maternal morbidity occurred in 15 cases (54%). Most had a febrile morbidity and depression. Seven patients underwent postpartum histerectomy due to consumptive coagulopathy. There was one maternal mortality (4%) and five perinatal mortalities (18%). The maternal death was due to consumptive coagulopathy after placental abruption. All patients had to receive blood transfusions. The median number of postoperative hospitalization days was 7. CONCLUSION(S): Peripartum hysterectomy is still a dramatic life-saving operation with high risks. The most common reason for abnormal placental adherence is previous uterine procedures.     

Intimate partner and nonpartner violence against pregnant women in rural Haiti

OBJECTIVE: To examine the association between violence experienced by pregnant Haitian women in the previous 6 months and pregnancy-related symptom distress. METHODS: A total of 200 women seeking prenatal care at community health dispensaries in the Artibonite Valley were interviewed. RESULTS: Over 4 in 10 women (44.0%) reported that they had experienced violence in the 6 months prior to interview; 77.8% of these women reported that the violence was perpetrated by an intimate partner. Those who experienced intimate partner violence reported significantly greater pregnancy-related symptom distress (beta=0.23, P=0.001). No significant differences between violence perpetrated by family members or others and reporting of symptoms were observed (beta=0.06, P=0.38). CONCLUSION: The findings indicate the need to integrate violence screening, resources, and primary prevention into prenatal care in rural Haiti.     

Emergency peripartum hysterectomy in a tertiary London hospital

Objective The objective was to review all emergency peripartum hysterectomies performed at a tertiary hospital in London, UK, and to identify the risk factors for emergency peripartum hysterectomy.Method A retrospective case control study. The cases consisted of all women who had emergency peripartum hysterectomy between 1 January 1993 and 31 December 2003. Controls were women who delivered immediately before and after the indexed case. Demographic data, medical and surgical histories, pregnancy, intrapartum and postpartum data were collected. Differences between cases and controls were compared with ², Fisher exact and Student t tests. Multiple logistic regression analysis was performed to identify independent risk factors for emergency peripartum hysterectomy.Results There were 15 cases of emergency peripartum hysterectomy in 31,079 deliveries, giving a rate of 0.48 per 1,000. Women who had emergency peripartum hysterectomy were significantly older (mean age 37 years vs. 29 years, P<0.001) and multiparous (P=0.02). More of the cases had a history of uterine surgery (67 vs. 30%, P=0.01), placenta praevia (60 vs. 3%, P<0.0001) and were delivered by caesarean section (86.7 vs. 30%, P=0.003). Eighty percent of the hysterectomies were performed in the daytime and all were done by consultants. Haemorrhage due to placenta praevia was the main indication for emergency peripartum hysterectomy (47%). Independent risk factors for emergency peripartum hysterectomy were older age (odds ratios [OR] 1.2, 95% confidence interval [95% CI] 1.2–1.6), multiparity (OR 2.6, 95% CI 1.3–10.2), history of previous caesarean section (OR 13.5, 95% CI 2.7–65.4), caesarean delivery in index pregnancy (OR 11.6, 95% CI 2.1–68.6) and caesarean delivery in index pregnancy for placenta praevia (OR 18, 95% CI 3.6–69).Conclusion Caesarean deliveries, especially repeat caesareans in women with placenta praevia, significantly increase the risk of emergency peripartum hysterectomy.