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.

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.

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.

Socio-economic factors determine maternal and neonatal outcomes in women with peripartum cardiomyopathy: A study of the ESC EORP PPCM registry.

BACKGROUND: Peripartum cardiomyopathy (PPCM) is a global disease with substantial morbidity and mortality. The aim of this study was to analyze to what extent socioeconomic factors were associated with maternal and neonatal outcomes. METHODS: In 2011, >100 national and affiliated member cardiac societies of the European Society of Cardiology (ESC) were contacted to contribute to a global PPCM registry, under the auspices of the ESC EORP Programme. We investigated the characteristics and outcomes of women with PPCM and their babies according to individual and country-level sociodemographic factors (Gini index coefficient [GINI index], health expenditure [HE] and human developmental index [HDI]). RESULTS: 739 women from 49 countries (Europe [33%], Africa [29%], Asia-Pacific [15%], Middle East [22%]) were enrolled. Low HDI was associated with greater left ventricular (LV) dilatation at time of diagnosis. However, baseline LV ejection fraction did not differ according to sociodemographic factors. Countries with low HE prescribed guideline-directed heart failure therapy less frequently. Six-month mortality was higher in countries with low HE; and LV non-recovery in those with low HDI, low HE and lower levels of education. Maternal outcome (death, re-hospitalization, or persistent LV dysfunction) was independently associated with income. Neonatal death was significantly more common in countries with low HE and low HDI, but was not influenced by maternal income or education attainment. CONCLUSIONS: Maternal and neonatal outcomes depend on country-specific socioeconomic characteristics. Attempts should therefore be made to allocate adequate resources to health and education, to improve maternal and fetal outcomes in PPCM.

Characterization of cardiac arrhythmias and maternal-fetal outcomes in pregnant women: A prospective cohort study.

INTRODUCTION AND OBJECTIVES: Cardiovascular disease is a common cause of morbidity and mortality in pregnant women. Arrhythmias are common complications during pregnancy; however, the data are limited. Our goal was to characterize the epidemiology, clinical presentation, and impact of cardiac arrhythmias on maternal-fetal outcomes. METHODS: A prospective cohort study from the Colombian Registry of Pregnancy and Cardiovascular Disease was carried out from 2016 to 2019. All patients with tachyarrhythmia or bradyarrhythmia and a minimum follow-up of six months after delivery were included. The primary outcome was a composite of cardiac events defined as pulmonary edema, symptomatic sustained arrhythmia requiring specific therapy, stroke, cardiac arrest, or maternal death. Secondary outcomes were other cardiac, neonatal, and obstetric events. RESULTS: Arrhythmias were the most common cause of referral to our dedicated cardio-obstetric clinic. A total of 92 patients were included, mean age 27±6 years; 8.7% had previous structural heart disease, and cardiology consultation was delayed in 79.4%. The most common arrhythmias were premature ventricular contractions (33%) and paroxysmal reentrant supraventricular tachycardias (15%); 11 patients (12%) had cardiac implantable electronic devices. Cardiac events occurred in 18.4% of patients, obstetric events occurred in 6.5%, and one caesarean was indicated in the context of symptomatic severe mitral stenosis. Adverse neonatal outcomes were observed in 24.3% of newborns. CONCLUSIONS: Arrhythmias were the most common cause of referral to a dedicated cardio-obstetric clinic; most had a benign course. Adverse maternal cardiovascular outcomes were significant and there was a high rate of obstetric and neonatal adverse events, underlining the importance of multidisciplinary care.

Serum Proteomic Analysis of Peripartum Cardiomyopathy Reveals Distinctive Dysregulation of Inflammatory and Cholesterol Metabolism Pathways.

BACKGROUND: The pathophysiology of peripartum cardiomyopathy (PPCM) and its distinctive biological features remain incompletely understood. High-throughput serum proteomic profiling, a powerful tool to gain insights into the pathophysiology of diseases at a systems biology level, has never been used to investigate PPCM relative to nonischemic cardiomyopathy. OBJECTIVES: The aim of this study was to characterize the pathophysiology of PPCM through serum proteomic analysis. METHODS: Aptamer-based proteomic analysis (SomaScan 7K) was performed on serum samples from women with PPCM (n = 67), women with nonischemic nonperipartum cardiomyopathy (NPCM) (n = 31), and age-matched healthy peripartum and nonperipartum women (n = 10 each). Serum samples were obtained from the IPAC (Investigation of Pregnancy-Associated Cardiomyopathy) and IMAC2 (Intervention in Myocarditis and Acute Cardiomyopathy) studies. RESULTS: Principal component analysis revealed unique clustering of each patient group (P for difference <0.001). Biological pathway analyses of differentially measured proteins in PPCM relative to NPCM, before and after normalization to pertinent healthy controls, highlighted specific dysregulation of inflammatory pathways in PPCM, including the upregulation of the cholesterol metabolism-related anti-inflammatory pathway liver-X receptor/retinoid-X receptor (LXR/RXR) (P < 0.01, Z-score 1.9-2.1). Cardiac recovery by 12 months in PPCM was associated with the downregulation of pro-inflammatory pathways and the upregulation of LXR/RXR, and an additional RXR-dependent pathway involved in the regulation of inflammation and metabolism, peroxisome proliferator-activated receptor α/RXRα signaling. CONCLUSIONS: Serum proteomic profiling of PPCM relative to NPCM and healthy controls indicated that PPCM is a distinct disease entity characterized by the unique dysregulation of inflammation-related pathways and cholesterol metabolism-related anti-inflammatory pathways. These findings provide insight into the pathophysiology of PPCM and point to novel potential therapeutic targets.

Cardio-Obstetrics: A Focused Review.

PURPOSE OF REVIEW: Cardiovascular disease is the leading cause of maternal mortality in the USA. All cardiovascular care providers should have a foundational knowledge on the management of pregnant individuals with heart disease. This focused review touches on several key cardio-obstetric themes. RECENT FINDINGS: Many individuals with cardiovascular disease can safely undergo pregnancy, but should have counseling preconception to optimize cardiac status. There are several cardiovascular conditions that are high risk for maternal mortality and morbidity. These individuals should be adequately counseled preconception and offered reliable birth control. The approach to a high-risk pregnant patient with cardiac disease is best managed by a multidisciplinary team to address potential maternal and fetal complications. Identification of at risk individuals can be estimated preconception with several risk scores. The development of risk scores to stratify and identify those at elevated risk during pregnancy is an area of continued research and development.

Maternal and neonatal outcomes in pregnant women with heart disease with single evaluation vs. semi-structured evaluation by a cardio-obstetric team.

INTRODUCTION: Cardiovascular diseases in pregnant women are challenging, with high maternal and perinatal morbidity and mortality, so a cardio-obstetric team is recommended for their care. Even so, little data evaluates the impact of these teams. Therefore, the present study aims to compare the obstetric, maternal, and neonatal outcomes of semi-structured follow-up (SSF) in a Cardio-obstetric clinic concerning regular or unstructured follow-up (USF) in pregnant women with heart disease. METHODS: A prospective registry of pregnant women with heart disease was carried out. Patients with SSF by a cardio-obstetric team were compared with those with single evaluation or USF. The risk of events was calculated according to the modified World Health Organization (mWHO) classification and the CARPREG-II scale, and cardiac, obstetric, and neonatal outcomes were evaluated. RESULTS: One hundred sixty-eight patients were evaluated, 37 with SSF and 131 with single evaluation (USF). The primary diagnoses were congenital heart disease, arrhythmias, and valve disease. The average CARPREG-II in USF patients was 2.48 (SD 2.3); in SSF patients, it was 3.37 (SD 2.45; p = 0.041). The average of the mWHO in patients with USF was 2.1 (SD 1.6), and with SSF, it was 2.65 (SD 0.95; p = 0.0052). There were no significant differences in primary cardiac outcomes (13.8% in USF vs. 5.4% in SSF; p = 0.134), secondary cardiac (5.3% in USF vs. 2.7% in SSF; p = 0.410), obstetric (10% in USF vs. 16.2% in SSF; p = 0.253) and neonatal (35.9% in USF and 40.5% in SSF; p = 0.486) even though patients with SSF had a higher risk than patients with USF according to the mWHO and CARPREG-II scales. CONCLUSIONS: In pregnant women with heart disease, an SSF compared with a USF by a cardio-obstetric team did not show statistically significant differences in cardiovascular, obstetric, and neonatal outcomes. However, patients with SSF had a significantly higher risk of adverse outcomes due to the mWHO and CARPREG-II scales. This result suggests that the SSF achieves at least equal outcomes despite the higher risk of adverse events that patients in this group had.

INTRODUCCIÓN: Las enfermedades cardiovasculares en las gestantes son desafiantes, con alta morbimortalidad materna y perinatal, por lo que se recomienda un equipo cardio-obstétrico para su atención. Aun así, pocos datos evalúan el impacto de estos equipos. Por lo tanto, el presente estudio tiene como objetivo comparar los resultados obstétricos, maternos y neonatales del seguimiento semiestructurado (SSE) en una clínica cardio-obstétrica con respecto a un seguimiento usual o seguimiento no estructurado (SNE) en gestantes con enfermedad cardiaca. MÉTODOS: Se realizó un registro prospectivo de gestantes con cardiopatías. Se compararon las pacientes con SSE por un equipo cardio-obstétrico, contra aquellas con evaluación única o SNE. Se calculó el riesgo de eventos según la clasificación de la Organización Mundial de la Salud modificado (OMSm) y la escala del Cardiac Disease in Pregnancy Study II (CARPREG-II) y se evaluaron los desenlaces cardiacos, obstétricos y neonatales. RESULTADOS: Se evaluaron 168 pacientes, 37 con SSE y 131 con evaluación única (SNE). Los principales diagnósticos fueron cardiopatía congénita, arritmias y valvulopatías. La media del CARPREG-II en pacientes de SNE fue 2.48 (DE: 2.3) y en pacientes de SSE fue 3.37 (DE: 2.45; p = 0.041). La media de la OMSm en pacientes de SNE fue 2.1 (DE: 1.6) y con SSE fue 2.65 (DE: 0.95; p = 0.0052). No hubo diferencias significativas en los desenlaces cardiacos primarios (13.8% en SNE vs. 5.4% en SSE; p = 0.134), cardiacos secundarios (5.3 en SNE vs. 2.7 en SSE; p = 0.410), obstétricos (10% en SNE vs. 16.2% en SSE; p = 0.253) y neonatales (35.9% en SNE y 40.5% en SSE; p = 0.486) a pesar de que las pacientes con SSE tenían un riesgo mayor que las pacientes con SNE según las escalas de la OMSm y el CARPREG-II. CONCLUSIONES: En gestantes con cardiopatía, un SSE comparado con un SNE por un equipo cardio-obstétrico no mostró diferencias estadísticamente significativas en los desenlaces cardiovasculares, obstétricos y neonatales, a pesar de que las pacientes con SSE tenían un riesgo significativamente más alto de desenlaces adversos por las escalas de la OMSm y el CARPREG-II. Esto sugiere que el SSE logra al menos equiparar los desenlaces a pesar del mayor riesgo de eventos adversos que tenían las pacientes de este grupo.

Successful Management and Birth After Perimortem Cesarean Delivery and Stillbirth Due to Anaphylaxis.

Perimortem cesarean delivery is an effective procedure for cardiopulmonary resuscitation during pregnancy. However, there are no reports documenting long-term outcomes in perimortem cesarean delivery survivors. This may be the first report of a successful live birth, occurring two years after perimortem cesarean delivery. A 29-year-old primipara was transferred to the emergency center on account of cardiopulmonary arrest, at 33 weeks of gestation. She was resuscitated 47 min after cardiopulmonary arrest by perimortem cesarean delivery amongst other treatment modalities, although the fetus died. Two months later, she was discharged with a preserved uterus, and no neurological damage. The couple suffered from posttraumatic stress disorder, which they overcame with the support of the multidisciplinary team, then gave birth to a healthy baby 2 years later. To overcome cardiopulmonary arrest during pregnancy, a seamless approach by a multidisciplinary team is essential for a good patient outcome.

Trends in Cardiovascular Health Counseling Among Postpartum Individuals.

Importance: Poor prepregnancy cardiovascular health (CVH) and adverse pregnancy outcomes (APOs) are key risk factors for subsequent cardiovascular disease (CVD) in birthing adults. The postpartum visit offers an opportunity to promote CVH among at-risk individuals. Objective: To determine prevalence, predictors, and trends in self-reported CVH counseling during the postpartum visit. Design, Setting, and Participants: Serial, cross-sectional analysis of data from 2016-2020 from the Pregnancy Risk Assessment Monitoring System (PRAMS), a nationally representative, population-based survey. The primary analysis included individuals who attended a postpartum visit 4 to 6 weeks after delivery with available data on receipt of CVH counseling, self-reported prepregnancy CVD risk factors (obesity, diabetes, and hypertension), and APOs (gestational diabetes, hypertensive disorders of pregnancy, and preterm birth) (N = 167 705 [weighted N = 8 714 459]). Exposures: Total number of CVD risk factors (0, 1, or ≥2 prepregnancy risk factors or APOs). Main Outcomes and Measures: Annual, age-adjusted prevalence of self-reported postpartum CVH counseling per 100 individuals, defined as receipt of counseling for healthy eating, exercise, and losing weight gained during pregnancy, was calculated overall and by number of CVD risk factors. Average annual percent change (APC) assessed trends in CVH counseling from 2016 through 2020. Data were pooled to calculate rate ratios (RRs) for counseling that compared individuals with and without CVD risk factors after adjustment for age, education, postpartum insurance, and delivery year. Results: From 2016 through 2020, prevalence of self-reported postpartum CVH counseling declined from 56.2 to 52.8 per 100 individuals among those with no CVD risk factors (APC, -1.4% [95% CI, -1.8% to -1.0%/y]), from 58.5 to 57.3 per 100 individuals among those with 1 risk factor (APC, -0.7% [95% CI, -1.3% to -0.1%/y]), and from 61.9 to 59.8 per 100 individuals among those with 2 or more risk factors (APC, -0.8% [95% CI, -1.3% to -0.3%/y]). Reporting receipt of counseling was modestly higher among individuals with 1 risk factor (RR, 1.05 [95% CI, 1.04 to 1.07]) and with 2 or more risk factors (RR, 1.11 [95% CI, 1.09 to 1.13]) compared with those who had no risk factors. Conclusions and Relevance: Approximately 60% of individuals with CVD risk factors or APOs reported receiving CVH counseling at their postpartum visit. Prevalence of reporting CVH counseling decreased modestly over 5 years.

Peripartum cardiomyopathy: A review.

Peripartum cardiomyopathy is a rare type of heart failure manifesting towards the end of pregnancy or in the months following delivery, in the absence of any other cause of heart failure. There is a wide range of incidence across countries reflecting different population demographics, uncertainty over definitions and under-reporting. Race, ethnicity, multiparity and advanced maternal age are considered important risk factors for the disease. Its etiopathogenesis is incompletely understood and is likely multifactorial, including hemodynamic stresses of pregnancy, vasculo-hormonal factors, inflammation, immunology and genetics. Affected women present with heart failure secondary to reduced left ventricular systolic function (LVEF <45%) and often with associated phenotypes such as LV dilatation, biatrial dilatation, reduced systolic function, impaired diastolic function, and increased pulmonary pressure. Electrocardiography, echocardiography, magnetic resonance imaging, endomyocardial biopsy, and certain blood biomarkers aid in diagnosis and management. Treatment for peripartum cardiomyopathy depends on the stage of pregnancy or postpartum, disease severity and whether the woman is breastfeeding. It includes standard pharmacological therapies for heart failure, within the safety restrictions for pregnancy and lactation. Targeted therapies such as bromocriptine have shown promise in early, small studies, with large definitive trials currently underway. Failure of medical interventions may require mechanical support and transplantation in severe cases. Peripartum cardiomyopathy carries a high mortality rate of up to 10% and a high risk of relapse in subsequent pregnancies, but over half of women present normalization of LV function within a year of diagnosis.

Cardiovascular Complications of Pregnancy.

The physiologic changes in pregnancy predispose the pregnant patient to a variety of potential cardiovascular complications. In this article, we discuss the major cardiovascular disorders of pregnancy and their management, highlight specific diagnostic challenges, and discuss new developments in the field. Topics covered in this article include venous thromboembolism, acute myocardial infarction, peripartum cardiomyopathy, and aortic dissection.

Considerations for specific cardiomyopathies during pregnancy.

PURPOSE OF REVIEW: Maternal mortality in the United States continues to increase. Cardiovascular disease, and in particular, cardiomyopathy and heart failure, serves as one of the principal causes of pregnancy-related mortality. The goal of this review is to summarize current knowledge on various cardiomyopathies in pregnancy, with an emphasis on cardiomyopathies beyond the most common in pregnancy, peripartum cardiomyopathy. RECENT FINDINGS: Although existing literature is somewhat limited, outcome studies of cardiomyopathy in pregnancy have demonstrated increased risk for adverse cardiovascular events and maternal morbidity and mortality. Herein, we review and synthesize recent studies focusing on preconception risk and counseling, pharmacologic management, and maternal outcomes in various cardiomyopathies during pregnancy including: dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, noncompaction cardiomyopathy, and chemotherapy-induced cardiomyopathy. SUMMARY: In an effort to optimize maternal outcomes, it is critical to recognize the risk associated with various cardiomyopathies in pregnancy and understand the tools for risk stratification and antepartum management. Further research on less common cardiomyopathies in pregnancy is warranted to provide more standardized recommendations.

Peripartum cardiomyopathy: risk factors and predictors of outcome.

PURPOSE OF REVIEW: Peripartum cardiomyopathy (PPCM) contributes significantly to maternal morbidity and mortality worldwide. In this review, we describe the present-day epidemiology and current understanding of the pathogenesis of PPCM. We provide an updated approach to diagnosis and management of PPCM, and discuss risk factors and predictors of outcome. RECENT FINDINGS: The highest incidences of PPCM have been reported in African, Asian, and Caribbean populations. Contemporary literature supports a 'two-hit' hypothesis, whereby the 'first hit' implies a predisposition, and the 'second hit' refers to an imbalanced peripartal hormonal milieu that results in cardiomyopathy. Whereas a half of patients will have left ventricular (LV) recovery, a tenth do not survive. Clinical findings and special investigations (ECG, echocardiography, cardiac MRI, biomarkers) can be used for risk stratification. Frequent prescription of guideline-directed medical therapy is associated with improved outcomes. SUMMARY: Despite advances in elucidating the pathogenesis of PPCM, it remains unclear why only certain women develop the disease. Moreover, even with better diagnostic work-up and management, it remains unknown why some patients with PPCM have persistent LV dysfunction or die. Future research should be aimed at better understanding of the mechanisms of disease and finding new therapies that could improve survival and LV recovery.