Although the incidence of sudden cardiac death (SCD) is greater in men than in women, it represents an important mode of death also in the female gender. Sex-related differences have been identified not only in the prevalence of the phenomenon, but also in risk factors and etiology of SCD. The peripartum period represents a peculiar trigger of SCD in women with underlying cardiovascular substrates. Several lesions, both congenital and acquired, may be identified at postmortem in SCD victims, including coronary artery, myocardial, valve, and aortic diseases. While the incidence of SCD increases progressively with age in adult elderly women to reach a 1:1 male:female ratio after the age of 80 years, mostly due to the increasing incidence of atherosclerotic disease in the postmenopausal period, SCD in young women is a rare event and usually associated with non-atherosclerotic disease, such as mitral valve prolapse, spontaneous coronary dissection, myocarditis, inherited cardiomyopathies and congenital heart diseases. The heart can be found structurally normal and inherited ion channel diseases are often implicated. Gender differences in the risk of SCD deserve further attention, since they affect the evaluation of interventions designed to reduce the rate of female SCD.

The role of a gender effect (that means differences in clinical manifestations, access to therapies and response to treatments according to gender) in cardiomyopathies remains a matter of debate. Although recent studies have evaluated the differences in the clinical features and prognosis between the two sexes, many issues remain to be elucidated. At present, the only sex-specific condition that affects females is peripartum cardiomyopathy. Recent evidence suggests a pathogenetic role of a prolactin derivative, and ongoing clinical trials are investigating the possibility of targeted therapies using prolactin secretion inhibitors, such as bromocriptine and carbegoline. Although women were considered so far only carriers of X-linked diseases (Anderson-Fabry disease, Danon disease, Hunter syndrome and dystrophinopathies), clinical experience showed a wide spectrum of clinical manifestations in females due to random X chromosome inactivation. Conversely, in mitochondrial diseases (with matrilineal inheritance), cardiomyopathies may occur in the context of clinical multisystemic involvement without significant gender-related differences. Autosomal inherited cardiomyopathies also show different phenotypes and prognostic impact according to gender. The hypothesis of a premenopausal protective role of female hormones towards myocardial involvement has been raised by recent data on transtiretin-related amyloidosis and hypertrophic cardiomyopathy. Preexisting cardiomyopathies may affect pregnancy, labor and delivery in women, since all these conditions are associated with important hemodynamic changes. Women with low-risk hypertrophic cardiomyopathy (asymptomatic and without left ventricular outflow tract gradient) usually can tolerate pregnancy. Conversely, women who are symptomatic before pregnancy or have severe hypertrophy with important outflow tract gradient are at higher risk and should be referred to a tertiary center to be evaluated on a case by case basis. Pregnancy in women with dilated cardiomyopathy and significant left ventricular systolic dysfunction represents a high-risk condition. In addition, information on the clinical course and potential complications in pregnant women with arrhythmogenic right ventricular cardiomyopathy or restrictive cardiomyopathy is limited to individual reports.

Peripartum cardiomyopathy (PPCM) is an often fatal disease that affects pregnant women who are near delivery, and it occurs more frequently in women with pre-eclampsia and/or multiple gestation. The aetiology of PPCM, and why it is associated with pre-eclampsia, remain unknown. Here we show that PPCM is associated with a systemic angiogenic imbalance, accentuated by pre-eclampsia. Mice that lack cardiac PGC-1α, a powerful regulator of angiogenesis, develop profound PPCM. Importantly, the PPCM is entirely rescued by pro-angiogenic therapies. In humans, the placenta in late gestation secretes VEGF inhibitors like soluble FLT1 (sFLT1), and this is accentuated by multiple gestation and pre-eclampsia. This anti-angiogenic environment is accompanied by subclinical cardiac dysfunction, the extent of which correlates with circulating levels of sFLT1. Exogenous sFLT1 alone caused diastolic dysfunction in wild-type mice, and profound systolic dysfunction in mice lacking cardiac PGC-1α. Finally, plasma samples from women with PPCM contained abnormally high levels of sFLT1. These data indicate that PPCM is mainly a vascular disease, caused by excess anti-angiogenic signalling in the peripartum period. The data also explain how late pregnancy poses a threat to cardiac homeostasis, and why pre-eclampsia and multiple gestation are important risk factors for the development of PPCM.

Peripartum cardiomyopathy (PPCM) and acute fatty liver of pregnancy (AFLP) are rare complications of pregnancy affecting approximately 1/10,000 pregnancies each. We describe a patient who had biopsy-proven AFLP complicated by PPCM.Chart review and literature search.The patient is a 22-year-old G5P1213 obese African-American female who presented at 30 weeks gestation with abdominal pain. She had normal blood pressures and mildly elevated liver enzymes. After completion of a 24 hour urine protein collection that was consistent with pre-eclampsia, an induction of labor with uncomplicated vaginal delivery was accomplished. Following delivery, a computed tomography scan of the abdomen revealed significant cardiomegaly. An echocardiogram revealed global dysfunction with an ejection fraction of 10%. Liver biopsy showed AFLP. Attempts to establish a unifying etiology were unrevealing. The PPCM was treated with diuretics and intravenous immunoglobulin. The patient's clinical status deteriorated, eventually requiring continuous dialysis, intubation, pharmacologic and mechanical inotropic support, and a feeding tube. The patient was discharged to a long-term care facility where she subsequently passed away from multiorgan failure.AFLP and PPCM are rare complications of pregnancy. We present a patient who had both. Both diseases carry a high mortality rate, and together, are likely fatal.

Persistence of left ventricular (LV) systolic dysfunction after 6 months of diagnosis is believed to be a marker of an irreversible cardiomyopathy in peripartum cardiomyopathy (PPCM). We sought to determine the length of time required for recovery of LV systolic function (LVSF) in patients with PPCM.Forty-two consecutive women with PPCM were enrolled in this prospective study. The minimum required time of follow-up for inclusion was 30 months. Each patient underwent transthoracic echocardiography, and plasma brain natriuretic peptide (BNP) and C-reactive protein measurement at admission, and every 3 months. Early recovery was defined as normalization of LVSF at 6 months post-diagnosis. Delayed recovery was defined if the length of time required for recovery of LVSF was longer than 6 months. Persistent left ventricular dysfunction (PLVD) was defined as an ejection fraction of <50% at the end of follow-up. Twenty patients (47.6%) recovered completely, 10 died (23.8%), and 12 (28.6%) had PLVD. Average time to complete recovery was 19.3 months after initial diagnosis (3-42 months). Early recovery was observed only in six patients (30%), whereas delayed recovery was observed in 14 out of 20 patients (70%). Patients with complete recovery were more likely to have a higher LV ejection fraction and smaller LV end-systolic dimensions at baseline.Full recovery of LVSF in PPCM patients often requires longer than 6 months.

Peripartum cardiomyopathy (PPCMP) is a dilated and hypokinetic cardiomyopathy occurring during pregnancy or after delivery, with an estimated incidence between 1/1000 and 1/4000 births. It has been defined as a new onset of heart failure in the month preceding or following delivery, without demonstrated aetiology nor previously known heart disease, and with echocardiographic evidences of left ventricular (LV) dysfunction (LV ejection fraction<0.45). It's a multifactorial disease, immunologic, hormonal, and possibly viral mechanisms playing a determinant pathophysiological role. The classical clinical presentation is a rapid and unexpected onset of heart failure in a previously healthy woman, echocardiography being the key examination for positive and differential diagnosis, prognostication, therapeutic decision-making, and follow-up. The potential severity of PPCMP, and its unpredictable evolution in the first days following diagnosis, require that patients be referred to a tertiary care centre with a high skill in intensive cardiology care. Therapeutic management of PPCMP does not offer any specificity when compared to other causes of acute or chronic heart failure (from diuretics to extracorporeal life support), except for ACE-inhibitors, that are contraindicated before delivery. The high incidence of thrombo-embolic complications observed in the disease requires however rapid and curative anticoagulation, and immuno-suppressive treatment has been proposed in fulminant and highly inflammatory presentation, but its efficacy remains controversial. Very recently, promising results have been reported with bromocriptin-a prolactin secretion inhibitor-for reducing 6-month morbidity and mortality, but these findings have to be confirmed in larger scale randomised trials. As for the long-term evolution, approximately half of the patients will heal, while half of the women will keep some degree of LV dysfunction, 25% of them developing moderate to severe chronic heart failure.

The number of pregnant women with cardiac disease is increasing with improvements in technology. In addition, more people are part of the national health insurance plan. However, there are few reports concerning the best method for anesthesia and mode of delivery in these high-risk patients. We report a 29-year-old woman scheduled for a planned caesarean section, who had a history of severe peripartum cardiomyopathy requiring extracorporeal membrane oxygenation in a previous pregnancy. The patient had regular prenatal care in our obstetric clinic. At 29 weeks' gestation, she developed severe dyspnea. A chest radiograph revealed bilateral pulmonary edema and 2-dimensional echocardiography showed a global hypokinesis and severe valve regurgitation with left ventricular ejection fraction of 41.2%. She had an emergency caesarean section and a cardiovascular surgeon was consulted to stand-by. Anesthesia was induced by ketamine 25 mg, midazolam 2.5 mg and rocuronium 50 mg for rapid intubation. The patient tolerated the procedure well and was extubated on postoperative day 1. She was discharged one week after surgery. Postoperatively, the patient was followed in the obstetric and cardiovascular surgery outpatient departments and at 5 months after surgery she was in good condition without any complaints.

Maternal mortality in South Africa is rising, and heart conditions currently account for 41 per cent of indirect causes of deaths. Little is known about the burden of heart disease in pregnant South Africans.We systematically reviewed the contemporary epidemiology and peripartum outcomes of heart disease in South African women attending antenatal care. Searches were performed in PubMed, ISI Web of Science, the EBSCO Africa-Wide database, the South African Union Catalogue, and the Current and Completed Research database (South Africa). References of included articles were also hand-searched. Studies reporting epidemiologic data on antenatal heart disease in South Africa were included. Data on morbidity and mortality were also collected.Seven studies were included in the systematic review. The prevalence of heart disease ranged from 123 to 943 per 100,000 deliveries, with a median prevalence of 616 per 100,000. Rheumatic valvular lesions were the commonest abnormalities, although cardiomyopathies were disproportionately high in comparison with other developing countries. Peripartum case-fatality rates were as high as 9.5 per cent in areas with limited access to care. The most frequent complications were pulmonary oedema, thromboembolism, and major bleeding with warfarin use. Perinatal mortality ranged from 8.9 to 23.8 per cent, whilst mitral lesions were associated with low birth weight. Meta-analysis could not be performed due to clinical and statistical heterogeneity of the included studies.Approximately 0.6 per cent of pregnant South Africans have pre-existing cardiac abnormalities, with rheumatic lesions being the commonest. Maternal and perinatal morbidity and mortality continue to be very high. We conclude this review by summarising limitations of the current literature and recommending standard reporting criteria for future reports.

Limited research exists exploring contraceptive and pregnancy experiences of women with cardiovascular diseases.We conducted semistructured interviews with reproductive-age women with chronic hypertension or peripartum cardiomyopathy exploring thoughts and behaviors regarding future fertility. Transcribed interviews were coded and analyzed identifying salient themes.We interviewed 20 women with chronic hypertension and 10 women with peripartum cardiomyopathy. Women described a spectrum of perspectives regarding the relationship between disease and fertility: from complete disconnect to full integration of diagnosis and future fertility plans. Integration of reproductive and cardiovascular health was influenced by and reflected in circumstances of diagnosis, pregnancy-related experiences, contraception-related experiences and conceptualization of disease risk related to reproductive health.Providers must better understand how women perceive and consider their reproductive and cardiovascular health in order to optimize contraceptive care of women with cardiovascular disease and help them make safe, informed decisions about future fertility.