High-Risk Congenital Heart Disease in Pregnancy.

High-risk congenital heart disease (CHD) in pregnancy presents a complex clinical challenge. With improved medical care and increased survival rates, a growing population of adults with complex CHD are surviving to adulthood, including women of reproductive age. This chapter focuses on risk stratification and management of pregnant women with high-risk CHD, emphasizing the importance of considering both anatomical and physiological complexity. Maternal physiological changes, such as blood volume increase, cardiac output changes, and alterations in vascular resistance, can significantly impact high-risk CHD patients. Management of high-risk CHD in pregnancy necessitates a multidisciplinary approach and individualized care.

Valvular Heart Disease in Pregnancy.

Valvular heart disease is a common cause of peripartum cardiovascular morbidity and mortality. The hemodynamic changes of pregnancy and their impact on preexisting valvular lesions are described in this paper. Tools for calculation of maternal and fetal risk during pregnancy are also discussed. The pathophysiology and management of valvular lesions, both obstructive and regurgitant, are then described, followed by discussion of mechanical and bioprosthetic valve complications during pregnancy.

Peripartum cardiomyopathy: A case report of decompensated heart failure in a hypertensive patient.

RATIONALE: Peripartum cardiomyopathy (PPCM) occurring in the context of hypertension presents a unique clinical challenge. This case contributes to the medical literature by highlighting the complexities of managing heart failure in postpartum women with pre-existing hypertensive disorders, particularly when complicated by a history of preeclampsia. PATIENT CONCERNS: Mrs. O.O., a 34-year-old hypertensive woman, presented with progressive dyspnea, bilateral leg swelling, and orthopnea. Notably, she had a history of previous preeclampsia and exhibited worsening symptoms over several months. DIAGNOSES: The patient was diagnosed with decompensated heart failure secondary to PPCM, exacerbated by hypertension and anemia. INTERVENTIONS: Therapeutic interventions included diuretics, angiotensin receptor-neprilysin inhibitors, digoxin, and anticoagulation. Additionally, lifestyle modifications and dietary restrictions were implemented. OUTCOMES: Following treatment adjustments, the patient demonstrated significant improvement in symptoms, exercise tolerance, and cardiac function. The transition from NYHA class III to class II heart failure indicated successful management. LESSONS: This case underscores the importance of a comprehensive approach to managing PPCM in hypertensive patients, with attention to cardiovascular and obstetric factors. It highlights the effectiveness of multidisciplinary care in achieving positive outcomes and emphasizes the need for heightened vigilance in postpartum women with cardiovascular risk factors.

Breastfeeding Practices in Patients with Heart Disease Stratified by Area Deprivation Index.

Objective: We sought to evaluate breastfeeding (BF) practices in patients with maternal cardiac disease (MCD) stratified by area deprivation index (ADI) to identity communities at risk. Study Design: Retrospective cohort of patients managed by the University of Alabama at Birmingham (UAB) Cardio-Obstetrics Program. Patients were included if they had ≥1 prenatal visit with the Cardio-Obstetrics team, delivered at UAB, and had a street address on file. The primary outcome was BF rate at hospital discharge. Secondary outcomes included BF intent on admission and BF at the postpartum (PP) visit. ADI reports socioeconomic disadvantage at the census tract level; 1 = least deprived and 100 = most deprived. Baseline characteristics and BF rates were compared by ADI categories: Low (ADI 1-33), medium (ADI 34-66), and high (ADI 67-100). Results: One hundred and forty-eight patients were included: 14 (10%) low, 42 (28%) medium, and 92 (62%) high ADI. Patients in the high ADI category were younger relative to those in the medium or low ADI (26 versus 28 versus 32 years; p < 0.01) and less likely to be married or living with a partner (30.4% versus 58.5% versus 71.4%; p < 0.01), There was no difference in BF intent between the lowest, medium, and highest ADI categories (85.7% versus 85.4% versus 81.6%; p = 0.38) or BF rates at hospital discharge (100% versus 92.7% versus 85.6%, p = 0.23). However, there was a significant difference in BF rates at the PP visit (90% versus 63.0% versus 38.6%; p < 0.01) even after controlling for differences in baseline characteristics (odds ratio = 0.11 (95% confidence interval [0.01-0.93]), p = 0.043). Conclusions: There was an association between living in a resource-poor community and early cessation of BF in our population of patients with MCD. Community-based interventions targeting mothers with heart disease living in high ADI communities may help these individuals achieve higher BF rates.

Peripartum cardiomyopathy unveiled: Etiology, diagnosis, and therapeutic insights.

Peripartum cardiomyopathy (PPCM) remains a significant challenge in maternal health, marked by its unpredictable onset and varied clinical outcomes. With rising incidence rates globally, understanding PPCM is vital for improving maternal care and prognosis. This review aims to consolidate current knowledge on PPCM, highlighting recent advancements in its diagnosis, management, and therapeutic approaches. This comprehensive review delves into the epidemiology of PPCM, underscoring its global impact and demographic variations. We explore the complex etiology of the condition, examining known risk factors and discussing the potential pathophysiological mechanisms, including oxidative stress and hormonal influences. The clinical presentation of PPCM, often similar yet distinct from other forms of cardiomyopathy, is analyzed to aid in differential diagnosis. Diagnostic challenges are addressed, emphasizing the role of advanced imaging and biomarkers. Current management strategies are reviewed, focusing on the absence of disease-specific treatments and the application of general heart failure protocols. The review also discusses the prognosis of PPCM, factors influencing recovery, and the implications for future pregnancies. Finally, we highlight emerging research directions and the urgent need for disease-specific therapies, aiming to provide a roadmap for future studies and improved patient care. This review serves as a crucial resource for clinicians and researchers, contributing to a deeper understanding and better management of PPCM.

Pregnancy outcomes in Chinese women with mechanical heart valves receiving warfarin treatment throughout pregnancy: 14-year experience.

OBJECTIVE: The purpose was to evaluate pregnancy outcomes and risk factors associated with fetal complications in Chinese pregnant women with mechanical heart valves (MHVs) taking low-dose warfarin, aiming to fill in the research gap of this area. METHODS: Between June 2010 and Aug 2023, 122 patients with MHVs who had 151 pregnancies and received warfarin throughout pregnancy were included. We compared them with 302 paired pregnancies without warfarin treatment. Binary logistic regression analyses were performed to explore risk predictors of fetal complications. RESULTS: Pregnancy loss rate was 37.1 % in women taking warfarin, compared to only 4.6 % for those without warfarin exposure in pregnancy (RR = 8.00, 95 % CI: 4.61-13.90). In pregnant women with MHVs, there were 34 spontaneous abortions, 22 stillbirths and 1 neonatal malformation. In the first, second and third pregnant trimesters of women with MHVs, fetal complication incidences were 19.2 %, 9.9 % and 8.0 %, respectively. 86.0 % of fetal complications occurred in women taking a warfarin dose ≤5 mg/d, accounting for 94.0 % of the total population. The newborns' birth weight, gestational age and 1-minute Apgar score were significantly lower in pregnancies treated with warfarin compared to those without warfarin exposure. Only 2.0 % of postpartum hemorrhage and no thrombosis or maternal mortality data were collected in pregnant women on warfarin in this study. CONCLUSION: Most Chinese pregnant women take a warfarin daily dose ≤5 mg and they might have only around 60 % chance of giving birth to a live baby without maternal complications.

Guyton perspective in managing peripartum cardiomyopathy patient with pulmonary edema: a case report.

BACKGROUND: Peripartum cardiomyopathy (PPCM) is a potentially life-threatening pregnancy-related condition characterized by left ventricular dysfunction and heart failure, typically occurring in the peripartum period. Individuals with a history of preeclampsia and hypertension are particularly prone to developing PPCM. Recent research suggests that the condition may be triggered by vascular dysfunction influenced by maternal hormones in the late stages of gestation. The onset of left heart failure results in decreased cardiac output, leading to insufficient perfusion, which in turn, contributes to pulmonary edema and exacerbates tissue hypoxia. This cardiovascular response activates the neurohumoral system, causing peripheral vasoconstriction and elevating both mean capillary filling pressure (MCFP) and central venous pressure (CVP). Early administration of furosemide reduces volume overload due to negative cumulative fluid balance gaining and vasodilation, which increases the velocity of intravascular refilling and causes interstitial edema to resolve. This will decrease interstitial fluid pressure, resulting in decreased mechanical compression to systemic capillary and systemic vein pressure, thus decreasing MCFP and CVP subsequently. Reduced CVP also contributes to increased venous return by decreasing the gradient pressure between MCFP and CVP, resulting in increased cardiac output (CO) and improved tissue oxygenation. CASE: A 33-year-old Asian woman, para 3 at full term pregnancy, admitted to the intensive care unit (ICU) after c-section and tubectomy due to shortness of breath and palpitation. Based on history taking, physical examination and echocardiography the patient fulfilled the criteria of PPCM which was also complicated by pulmonary edema. Despite impending respiratory failure, the patient rejected intubation and continuous positive airway pressure (CPAP), and was given oxygen supplementation through nasal cannula. Furosemide was given rapidly continued by maintenance dose and CVP was monitored. Antihypertensive drug, anticoagulants, and bromocriptine were also administered. After achieving negative cumulative fluid balance the patient's symptoms resolved and was discharged one week later. CONCLUSION: There is a correlation between negative cumulative fluid balance and reduced central venous pressure after early furosemide therapy. Suspicion for PPCM should not be lowered in the presence of preeclampsia, it could delay appropriate treatment and increase the mortality.

Disease features and management of cardiomyopathies in women.

Over the last years, there has been a growing interest in the clinical manifestations and outcomes of cardiomyopathies in women. Peripartum cardiomyopathy is the only women-specific cardiomyopathy. In cardiomyopathies with X-linked transmission, women are not simply healthy carriers of the disorder, but can show a wide spectrum of clinical manifestations ranging from mild to severe manifestations because of heterogeneous patterns of X-chromosome inactivation. In mitochondrial disorders with a matrilinear transmission, cardiomyopathy is part of a systemic disorder affecting both men and women. Even some inherited cardiomyopathies with autosomal transmission display phenotypic and prognostic differences between men and women. Notably, female hormones seem to exert a protective role in hypertrophic cardiomyopathy (HCM) and variant transthyretin amyloidosis until the menopausal period. Women with cardiomyopathies holding high-risk features should be referred to a third-level center and evaluated on an individual basis. Cardiomyopathies can have a detrimental impact on pregnancy and childbirth because of the associated hemodynamic derangements. Genetic counselling and a tailored cardiological evaluation are essential to evaluate the likelihood of transmitting the disease to the children and the possibility of a prenatal or early post-natal diagnosis, as well as to estimate the risk associated with pregnancy and delivery, and the optimal management strategies.

Stretch-induced compliance mechanism in pregnancy-induced cardiac hypertrophy and the impact of cardiovascular risk factors.

Pressure overload-induced hypertrophy compromises cardiac stretch-induced compliance (SIC) after acute volume overload (AVO). We hypothesized that SIC could be enhanced by physiological hypertrophy induced by pregnancy's chronic volume overload. This study evaluated SIC-cardiac adaptation in pregnant women with or without cardiovascular risk (CVR) factors. Thirty-seven women (1st trimester, 1stT) and a separate group of 31 (3rd trimester, 3rdT) women [healthy or with CVR factors (obesity and/or hypertension and/or with gestational diabetes)] underwent echocardiography determination of left ventricular end-diastolic volume (LVEDV) and E/e' before (T0), immediately after (T1), and 15 min after (T2; SIC) AVO induced by passive leg elevation. Blood samples for NT-proBNP quantification were collected before and after the AVO. Acute leg elevation significantly increased inferior vena cava diameter and stroke volume from T0 to T1 in both 1stT and 3rdT, confirming AVO. LVEDV and E/e' also increased immediately after AVO (T1) in both 1stT and 3rdT. SIC adaptation (T2, 15 min after AVO) significantly decreased E/e' in both trimesters, with additional expansion of LVEDV only in the 1stT. NT-pro-BNP increased slightly after AVO but only in the 1stT. CVR factors, but not parity or age, significantly impacted SIC cardiac adaptation. A distinct functional response to SIC was observed between 1stT and 3rdT, which was influenced by CVR factors. The LV of 3rdT pregnant women was hypertrophied, showing a structural limitation to dilate with AVO, whereas the lower LV filling pressure values suggest increased diastolic compliance.NEW & NOTEWORTHY The sudden increase of volume overload triggers an acute myocardial stretch characterized by an immediate rise in contractility by the Frank-Starling mechanism, followed by a progressive increase known as the slow force response. The present study is the first to characterize echocardiographically the stretch-induced compliance (SIC) mechanism in the context of physiological hypertrophy induced by pregnancy. A distinct functional adaptation to SIC was observed between first and third trimesters, which was influenced by cardiovascular risk factors.

Peripartum cardiomyopathy in low- and middle-income countries.

Peripartum cardiomyopathy (PPCM) causes pregnancy-associated heart failure, typically during the last month of pregnancy, and up to 6 months post-partum, in women without known cardiovascular disease. PPCM is a global disease, but with a significant geographical variability within and between countries. Its true incidence in Africa is still unknown because of the lack of a PPCM population-based study. The variability in the epidemiology of PPCM between and within countries could be due to differences in the prevalence of both genetic and non-genetic risk factors. Several risk factors have been implicated in the aetiopathogenesis of PPCM over the years. Majority of patients with PPCM present with symptoms and signs of congestive cardiac failure. Diagnostic work up in PPCM is prompted by strong clinical suspicion, but Echocardiography is the main imaging technique for diagnosis. The management of PPCM involves multiple disciplines - cardiologists, anaesthetists, intensivists, obstetricians, neonatologists, and the prognosis varies widely.

Left ventricular recovery in an African cohort of patients with peripartum cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is a rare and potentially life-threatening disease associated with pregnancy. There are limited data regarding the outcome of PPCM and its predictive factors in sub-Saharan African patients. We prospectively conducted a double-center (cardiology unit of the department of medicine, Regional Hospital Center of Tenkodogo, Burkina Faso and the department of cardiology of the National Referral Teaching Hospital of N´Djamena, Chad) cohort study in patients with PPCM. Patients were consecutively enrolled from January 2015 to December 2017. Outcomes of interest were left ventricular recovery and poor outcome at one year. Ninety-four patients enrolled with a median age of 28 years. At one-year follow-up, 40.5% of them recovered their left ventricular function. Cox multiple regression analysis revealed that higher left ventricle ejection fraction (LVEF), lower natremia and use of betablockers were baseline variables predicting this end-point. Of the entire study population, 26.60% exhibited the composite end-point of death (n=15) or remaining in New York Heart Association (NYHA) class III-IV or LVEF < 35%. Predictors of poor outcome were lower LVEF at baseline, hyponatremia and use of digoxin. The current cohort study demonstrated that PPCM in sub-Saharan Africa is associated with limited myocardial recovery and significant rate of poor outcome at one year. Therefore, additional studies are needed to better address the disease.

Placental Abruption and Cardiovascular Event Risk (PACER): Design, data linkage, and preliminary findings.

BACKGROUND: Obstetrical complications impact the health of mothers and offspring along the life course, resulting in an increased burden of chronic diseases. One specific complication is abruption, a life-threatening condition with consequences for cardiovascular health that remains poorly studied. OBJECTIVES: To describe the design and data linkage algorithms for the Placental Abruption and Cardiovascular Event Risk (PACER) cohort. POPULATION: All subjects who delivered in New Jersey, USA, between 1993 and 2020. DESIGN: Retrospective, population-based, birth cohort study. METHODS: We linked the vital records data of foetal deaths and live births to delivery and all subsequent hospitalisations along the life course for birthing persons and newborns. The linkage was based on a probabilistic record-matching algorithm. PRELIMINARY RESULTS: Over the 28 years of follow-up, we identified 1,877,824 birthing persons with 3,093,241 deliveries (1.1%, n = 33,058 abruption prevalence). The linkage rates for live births-hospitalisations and foetal deaths-hospitalisations were 92.4% (n = 2,842,012) and 70.7% (n = 13,796), respectively, for the maternal cohort. The corresponding linkage rate for the live births-hospitalisations for the offspring cohort was 70.3% (n = 2,160,736). The median (interquartile range) follow-up for the maternal and offspring cohorts was 15.4 (8.1, 22.4) and 14.4 (7.4, 21.0) years, respectively. We will undertake multiple imputations for missing data and develop inverse probability weights to account for selection bias owing to unlinked records. CONCLUSIONS: Pregnancy offers a unique window to study chronic diseases along the life course and efforts to identify the aetiology of abruption may provide important insights into the causes of future CVD. This project presents an unprecedented opportunity to understand how abruption may predispose women and their offspring to develop CVD complications and chronic conditions later in life.

A novel score to predict left ventricular recovery in peripartum cardiomyopathy derived from the ESC EORP Peripartum Cardiomyopathy Registry.

BACKGROUND AND AIMS: There are no established clinical tools to predict left ventricular (LV) recovery in women with peripartum cardiomyopathy (PPCM). Using data from women enrolled in the ESC EORP PPCM Registry, the aim was to derive a prognostic model to predict LV recovery at 6 months and develop the 'ESC EORP PPCM Recovery Score'-a tool for clinicians to estimate the probability of LV recovery. METHODS: From 2012 to 2018, 752 women from 51 countries were enrolled. Eligibility included (i) a peripartum state, (ii) signs or symptoms of heart failure, (iii) LV ejection fraction (LVEF) ≤ 45%, and (iv) exclusion of alternative causes of heart failure. The model was derived using data from participants in the Registry and internally validated using bootstrap methods. The outcome was LV recovery (LVEF ≥50%) at six months. An integer score was created. RESULTS: Overall, 465 women had a 6-month echocardiogram. LV recovery occurred in 216 (46.5%). The final model included baseline LVEF, baseline LV end diastolic diameter, human development index (a summary measure of a country's social and economic development), duration of symptoms, QRS duration and pre-eclampsia. The model was well-calibrated and had good discriminatory ability (C-statistic 0.79, 95% confidence interval [CI] 0.74-0.83). The model was internally validated (optimism-corrected C-statistic 0.78, 95% CI 0.73-0.82). CONCLUSIONS: A model which accurately predicts LV recovery at 6 months in women with PPCM was derived. The corresponding ESC EORP PPCM Recovery Score can be easily applied in clinical practice to predict the probability of LV recovery for an individual in order to guide tailored counselling and treatment.