Multi-modal treatment in a pregnant woman with untreated cardiac sarcoidosis complicated by cardiac dysfunction and ventricular arrhythmias: a case report and literature review.

Background: The treatment of cardiac sarcoidosis during pregnancy is inherently challenging owing to its impact on the foetus. Case summary: We report a case of a 30-year-old pregnant woman with untreated cardiac sarcoidosis. One year prior to admission, she underwent permanent pacemaker implantation for complete atrioventricular block. Left ventricular ejection fraction (EF) showed a declining trend, and ventricular tachycardia (VT) was documented. Following an extensive evaluation, the patient was diagnosed with active cardiac sarcoidosis, and the pregnancy was detected at the same time. Considering the high risk of mortality and cardiovascular complications in pregnant patients with decreased EF and VT, we meticulously discussed the optimal timing of multi-modal treatment, including bisoprolol, eplerenone, sotalol, and prednisolone and cardiac resynchronization therapy with a defibrillator, and its effect on the foetus. These interventions improved the EF to 49%, and the baby was successfully delivered without adverse events or neonatal complications developing. At 8 months' post-partum, the mother and the baby were doing well, and the EF was 45%. Discussion: Cardiac sarcoidosis can lead to adverse outcomes for both the mother and the foetus. However, with multi-modal treatment individually optimized and implemented by a multi-disciplinary team of specialists in each field, even pregnant women with untreated cardiac sarcoidosis who present with reduced EF and VT can achieve safe childbirth.

Diagnosis and Management of Vasospastic Angina in a Young Woman Wishing to Become Pregnant.

Vasospastic angina can sometimes induce acute myocardial infarction in pregnant women, potentially endangering the lives of mother and child. We describe a case of a young woman with suspected vasospastic angina who wished to become pregnant. Vasospasm provocation testing revealed severe vasospasm, and subsequent appropriate management resulted in successful delivery.

National Survey Identifying the Factors Affecting the Career Development of Cardiologists in Japan.

BACKGROUND: Equality in training opportunities, studying abroad, and satisfaction with work are not well investigated among Japanese cardiologists.Methods and Results: We studied cardiologists' career development using a questionnaire that was emailed to 14,798 cardiologists belonging to the Japanese Circulation Society (JCS) in September 2022. Feelings regarding equality in training opportunities, preferences for studying abroad, and satisfaction with work were evaluated with regard to cardiologists' age, sex, and other confounding factors. Survey responses were obtained from 2,566 cardiologists (17.3%). The mean (±SD) age of female (n=624) and male (n=1,942) cardiologists who responded to the survey was 45.6±9.5 and 50.0±10.6 years, respectively. Inequality in training opportunities was felt more by female than male cardiologists (44.1% vs. 33.9%) and by younger (<45 years old) than older (≥45 years old) (42.0% vs. 32.8%). Female cardiologists were less likely to prefer studying abroad (53.7% vs. 59.9%) and less satisfied with their work (71.3% vs. 80.8%) than male cardiologists. Increased feelings of inequality and lower work satisfaction were investigated among cardiologists who were young, had family care duties, and had no mentors. In the subanalysis, significant regional differences were found in cardiologists' career development in Japan. CONCLUSIONS: Female and younger cardiologists felt greater inequality in career development than male and older cardiologists. A diverse workplace may prompt equality in training opportunities and work satisfaction for both female and male cardiologists.

Awareness and Feasibility of Women Chairing Cardiology Sessions in Scientific Meetings: A Nationwide Survey by the Japanese Circulation Society.

Background: Diversity and inclusion remain a concern in the field of cardiology. Female cardiologists have less opportunity to chair sessions in scientific meetings than men. However, cardiologists' awareness and perspectives on feasibility of chairing sessions is poorly understood. Methods and Results: A web-based survey on awareness regarding the commitment of chairing sessions was sent to 14,798 certificated cardiologists registered with the Japanese Circulation Society (JCS). A total of 3,412 valid responses were obtained, such as 523 women and 2,889 men. Female cardiologists exhibited less interest in serving as chairpersons in Japanese and English sessions (71% women vs. 82% men, p < 0.001, 30% women vs. 40% men, p < 0.001). Influencing factors of chair acceptance in Japanese sessions for female cardiologists were being a cardiologist for over 10 years [odds ratio (OR) 1.84, 95% confidence interval (CI) 1.02-3.33], experience studying abroad (OR 3.35, 95% CI 1.93-5.81) and chairing sessions (OR 8.39, 95% CI 5.48-12.9), having a Doctor of Philosophy (OR 2.82, 95% CI 1.09-7.31), presence of 4 or more female cardiovascular specialists in the hospital (OR 1.70, 95% CI 1.10-2.61) and of role models (OR 2.86, 95% CI 1.93-4.24), and awareness of the JCS chairperson's manual (OR 10.7, 95% CI 6.67-17.1). The receiver operating characteristic (ROC) curve revealed that the number of female cardiovascular specialists in a hospital was a more sensitive predictor of chair acceptance among male than female cardiologists. Conclusions: Female cardiologists were less likely to accept chairing sessions compared with male cardiologists and the presence of female cardiovascular specialists positively influenced chair acceptance.

Clinicopathologic Study of Placentas From Women With a Fontan Circulation.

BACKGROUND: Pregnant women with a Fontan circulation have a high risk of obstetric complications, such as preterm delivery and small for gestational age (SGA), which may be affected by low blood flow to the placenta and hypoxia. This study investigated placental pathology in a Fontan circulation.Methods and Results:Eighteen pregnancies in 11 women with a Fontan circulation were reviewed. Pregnancy outcomes showed 9 miscarriages and 9 live births, with 4 preterm deliveries. Five neonates were SGA (<5th percentile). Eight placentas from live births in 7 women were available for the study. Five placentas had low weight placenta for gestational age, and 7 grossly showed a chronic subchorionic hematoma. Histological examination revealed all placentas had some form of histological hypoxic lesions: maternal vascular malperfusion in 7, fetal vascular malperfusion in 1, and other hypoxia-related lesions in 8. Quantitative analyses, including immunohistochemistry (CD31, CD68, and hypoxia inducible factor-1α antibodies) and Masson's trichrome staining, were also performed and compared with 5 control placentas. Capillary density and the area of fibrosis were significantly greater in placentas from women with a Fontan circulation than in control placentas. CONCLUSIONS: Placentas in a Fontan circulation were characterized by a high frequency of low placental weight, chronic subchorionic hematoma, and constant histological hypoxic changes, which could reflect altered maternal cardiac conditions and lead to poor pregnancy outcomes.

Two cases of pregnancy after achieving complete remission of chronic active Epstein-Barr virus infection.

Chronic active Epstein-Barr virus (CAEBV) infection characterized by persistent infectious mononucleosis-like symptoms can lead to cardiovascular diseases. We encountered two pregnant women with CAEBV histories complicated with cardiovascular diseases. A 36-year-old woman with a history of myocardial infarction due to CAEBV and coronary artery bypass grafting became pregnant. Her left ventricular ejection fraction (LVEF) decreased, and cesarean section was performed at 36 weeks of gestation. Her LVEF recovered after delivery. A 32-year-old woman with a history of CAEBV and chronic hypertension was diagnosed with mild pulmonary arterial hypertension (PAH) after conception. She strongly desired to continue the pregnancy. She became complicated with severe superimposed preeclampsia at 31 weeks of gestation, and cesarean section was performed. Her PAH did not deteriorate during pregnancy or the postpartum period. Women treated for CAEBV, even with complete remission, require a preconception evaluation focused on the cardiovascular system and careful management of their pregnancy.

Cardiovascular disease among pregnant women after anticancer therapy.

Recent advances in cancer therapies have enabled many women diagnosed with malignancies during childhood and adolescence to survive longer and therefore to reach an age where they wish to conceive. When providing cancer treatment such as anticancer drugs and radiotherapy, attention is often paid to preserving future fertility, but little is known about maternal pregnancy risks, and in particular cardiovascular complications. Recent studies have shown that cardiovascular diseases such as cardiomyopathy, heart failure, and arrhythmias often occur during and soon after anticancer therapy. This has led to the emergence of the specialized field of "onco-cardiology" or "cardio-oncology," in which oncologists and cardiologists collaborate, as well as the publication of multiple clinical practice guidelines. The interdisciplinary onco-cardiology team plays an important role in further improving the prognosis of cancer survivors. The current recommendation for women after anticancer therapy who wish to conceive is to undergo cardiovascular screening, regardless of whether there is a history of cardiovascular complications or not, in order to provide preconception counseling. Pregnancies in cancer-survivors, who experience cardiovascular complications, should be managed perinatally by a multidisciplinary team including obstetricians and cardiologists. Absence of cardiovascular disease on screening does not preclude the possibility that new cardiac dysfunction may occur during the perinatal period, especially in women with a history of high-dose anthracycline drug administration and/or radiation therapy. In such high-risk cases, follow-up of cardiac function throughout the perinatal period is required.

Genetic and Phenotypic Landscape of Peripartum Cardiomyopathy.

BACKGROUND: Peripartum cardiomyopathy (PPCM) occurs in ≈1:2000 deliveries in the United States and worldwide. The genetic underpinnings of PPCM remain poorly defined. Approximately 10% of women with PPCM harbor truncating variants in TTN (TTNtvs). Whether mutations in other genes can predispose to PPCM is not known. It is also not known if the presence of TTNtvs predicts clinical presentation or outcomes. Nor is it known if the prevalence of TTNtvs differs in women with PPCM and preeclampsia, the strongest risk factor for PPCM. METHODS: Women with PPCM were retrospectively identified from several US and international academic centers, and clinical information and DNA samples were acquired. Next-generation sequencing was performed on 67 genes, including TTN, and evaluated for burden of truncating and missense variants. The impact of TTNtvs on the severity of clinical presentation, and on clinical outcomes, was evaluated. RESULTS: Four hundred sixty-nine women met inclusion criteria. Of the women with PPCM, 10.4% bore TTNtvs (odds ratio=9.4 compared with 1.2% in the reference population; Bonferroni-corrected P [P*]=1.2×10-46). We additionally identified overrepresentation of truncating variants in FLNC (odds ratio=24.8, P*=7.0×10-8), DSP (odds ratio=14.9, P*=1.0×10-8), and BAG3 (odds ratio=53.1, P*=0.02), genes not previously associated with PPCM. This profile is highly similar to that found in nonischemic dilated cardiomyopathy. Women with TTNtvs had lower left ventricular ejection fraction on presentation than did women without TTNtvs (23.5% versus 29%, P=2.5×10-4), but did not differ significantly in timing of presentation after delivery, in prevalence of preeclampsia, or in rates of clinical recovery. CONCLUSIONS: This study provides the first extensive genetic and phenotypic landscape of PPCM and demonstrates that predisposition to heart failure is an important risk factor for PPCM. The work reveals a degree of genetic similarity between PPCM and dilated cardiomyopathy, suggesting that gene-specific therapeutic approaches being developed for dilated cardiomyopathy may also apply to PPCM, and that approaches to genetic testing in PPCM should mirror those taken in dilated cardiomyopathy. Last, the clarification of genotype/phenotype associations has important implications for genetic counseling.

Aortic dissection during pregnancy and puerperium: A Japanese nationwide survey.

AIM: This study aimed to characterize the incidence and clinical significance of pregnancy-related aortic dissection using a large-scale survey in Japan. METHODS: A questionnaire requesting the detailed information included in the clinical charts of pregnancy-related aortic dissection cases (without any personally identifying information) was designed between 2013 and 2017 and administered to 407 perinatal centers in Japan. The response rate was 70.5%. Seventeen cases of pregnancy-related aortic dissection were identified. RESULTS: Maternal death due to aortic dissection was observed in nine patients (56.2%) while seven survived (43.8%). Dissection occurred during the postpartum period in 10 cases (62.5%), the third trimester in 4 (25.0%), labor in 1 (6.2%), and the second trimester in 1 (6.2%). The most common underlying diseases were: Marfan syndrome (25.0%), Loeys-Dietz syndrome (6.2%), hypertension (6.2%), and Takayasu aortitis (6.2%). Stanford type A aortic dissection was associated with maternal death during both pregnancy and the postpartum period. However, deceased patients showed lower rates of pre-diagnosed connective tissue disease than did survivors. CONCLUSIONS: The mortality rate of aortic dissection that occurred during pregnancy or postnatal periods was more than 50%. Aortic dissection occurred more frequently in the postnatal period than during pregnancy, and less frequently in women previously diagnosed with connective tissue disease than in women without any medical history of aortic disorders. If symptoms suggestive of aortic dissection, such as severe back pain, are observed, even after the end of pregnancy, exhaustive diagnostic examinations should be carried out.

Clinical management of coagulation status and placenta previa in a pregnant woman with Marfan's syndrome after mitral and aortic mechanical heart valve replacement.

Pregnancy with a mechanical heart valve (MHV) is high risk for valve thrombosis because pregnancy is a hyper-coagulation state. In addition, warfarin use during pregnancy is restricted due to its fetotoxicity, and postpartum bleeding may be increased with anticoagulation. In particular, placenta previa under anticoagulation may cause massive postpartum bleeding. The optimal anticoagulation for a pregnant woman with mitral and aortic double MHVs is not known. In addition, suitable techniques for control of bleeding in a case of placenta previa under anticoagulation are not known. Thus, a case of a pregnant woman with mitral and aortic double MHVs and placenta previa is presented. The case was managed without valve thrombosis through precise unfractionated heparin dose adjustment and frequent activated partial thromboplastin time monitoring, along with maintenance of antithrombin levels. Compression sutures were found to be effective for controlling bleeding from the attachment site of placenta previa even under anticoagulation.

Pregnancy outcomes in women with dilated cardiomyopathy: Peripartum cardiovascular events predict post delivery prognosis.

BACKGROUND: The number of pregnant women with dilated cardiomyopathy (DCM) is relatively small, and therefore their prognosis after pregnancy is unknown. This study aims to elucidate pregnancy outcomes among women with DCM, as well as the long-term prognosis after pregnancy. METHODS: Thirty-five pregnancies and deliveries in 30 women, diagnosed with DCM before pregnancy, were retrospectively analyzed. RESULTS: All women had a left ventricular ejection fraction (LVEF) over 30% and belonged to the New York Heart Association (NYHA) class I or II before pregnancy. The mean gestational age at delivery was 36 weeks with 15 (43%) preterm deliveries. Eight pregnancies (23%) were complicated by peripartum cardiac events including 1 ventricular arrhythmia, 6 heart failures, and 1 significant deterioration in LVEF requiring termination of pregnancy. NYHA class II, pre-pregnancy use of angiotensin-converting enzyme inhibitor/angiotensin II receptor blocker/diuretics, elevated brain natriuretic peptide (BNP), and advanced diastolic dysfunction assessed by Doppler echocardiography were defined as risk factors for cardiac events. Although the more severe cases took beta-blockers during pregnancy, the rates of cardiac events and decreasing LVEF did not differ significantly between those taking beta-blockers and those who were not. Values of LVEF decreased by almost 10% after the average 4-year post-delivery follow-up period. The long-term event-free survival was considerably worse among women with peripartum cardiac events than in those without (p<0.0001). CONCLUSIONS: DCM women with pre-pregnancy LVEF over 30% tolerated pregnancy, but the rate of preterm delivery was high. Peripartum cardiovascular events occurred more often in women with NYHA class II, as well as those who received medications before and during pregnancy and showed more elevated BNP and advanced diastolic dysfunction before pregnancy. Beta-blockers likely allowed similar outcomes for DCM patients with lower initial LVEFs. Close monitoring later in life is required, particularly among the women with peripartum cardiac events.

Anesthesia for Cesarean Section and Postpartum Cardiovascular Events in Congenital Heart Disease: A Retrospective Cohort Study.

OBJECTIVE: To clarify the association between anesthetic technique and maternal and neonatal outcomes in parturients with congenital heart disease (CHD). DESIGN: Retrospective, observational cohort study. SETTING: An academic hospital. PARTICIPANTS: A total of 263 consecutive parturients with CHD who underwent cesarean section from 1994 to 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The authors compared postpartum cardiovascular events (composite of heart failure, pulmonary hypertension, arrhythmia, and thromboembolic complications) and neonatal outcomes (intubation and Apgar score <7 at one or five minutes) by anesthetic technique. Among 263 cesarean sections, general anesthesia was performed in 47 (17.9%) parturients and neuraxial anesthesia in 214 (81.3%) parturients. Cardiovascular events were more common in the general anesthesia group (n = 7; 14.9%) than in the neuraxial anesthesia group (n = 17; 7.9%). Generalized linear mixed models assuming a binomial distribution (ie, mixed-effects logistic regression), with a random intercept for each modified World Health Organization classification for maternal cardiovascular risk, revealed that general anesthesia was not significantly associated with cardiovascular events (odds ratio [OR], 1.00; 95% confidence interval [CI], 0.30-3.29). In addition, general anesthesia was associated with composite neonatal outcomes (Apgar score <7 at one or five minutes or need for neonatal intubation; OR, 13.3; 95% CI, 5.52-32.0). CONCLUSION: Anesthetic technique is not significantly associated with postpartum composite cardiovascular events. General anesthesia is significantly associated with increased need for neonatal intubation and lower Apgar scores.

Elaborate evaluation of serial changes in electrocardiograms of atrial septal defects after transcatheter closure for a better understanding of the recovery process.

Serial changes of electrocardiograms (ECG) could be used to assess their clinical features in atrial septal defects (ASD) after transcatheter closure together with other clinical parameters. We retrospectively studied 100 ASD patients who underwent transcatheter closure. Complications of persistent atrial fibrillation occurred in five ASD patients, and they were excluded. We divided the other 95 patients according to PQ intervals before closure (normal: < 200 ms, n = 51; prolonged: ≥ 200 ms, n = 44) to evaluate their clinical characteristics and parameters such as echocardiography, chest X-rays, and brain natriuretic protein (BNP) levels. Individuals in the prolonged PQ group were significantly older, had higher incidences of paroxysmal atrial fibrillation (PAF) and heart failure (HF) treated with more ß-blockers and diuretics, and with a higher tendency of NYHA functional classification and BNP levels than the normal PQ group. The prolonged PQ group also had a significantly higher incidence of complete right bundle branch block, wider QRS intervals, and larger cardiothoracic ratios in chest X-rays accompanied by larger right atrial-areas and larger left atrial dimensions in echocardiograms. Furthermore, the prolonged PQ intervals with less PQ interval shortening after transcatheter closure revealed that the patients were the oldest at the time of closures and showed less structural normalization of the right heart and left atrium after ASD closure. PAF and HF also occurred more frequently in this subgroup. These results suggested that the ASD patients with prolonged PQ intervals with less PQ shortening were accompanied by more advanced clinical conditions. Together with other clinical parameters, detailed analyses of ECG and their changes after closure could elucidate the clinical characteristics and status of ASD patients with transcatheter closure and were useful for predicting structural normalization after transcatheter closure.

Risk Factors for Cardiovascular Events among Pregnant Women with Cardiovascular Disease.

Objective Cardiovascular disease increases the risk of maternal mortality. This study examined the risk factors for cardiovascular events in pregnant women with cardiovascular disease. Methods This was a case-control study conducted in 2 phases at Japanese maternal and fetal care centers. The primary survey, using an interviewer-administered questionnaire, investigated whether the institutions had managed pregnant women with cardiovascular disease from April 2014 to March 2016. From 424 individual facilities surveyed, 135 facilities were found to have experience in managing pregnant women. In the secondary survey, the 135 institutions were asked to complete a web-based questionnaire, which collected detailed clinical information about cases, including cardiovascular disease, cardiovascular events, maternal background, and the perinatal outcome. Results Information on 302 pregnant women with cardiovascular disease was collected. None of the 302 patients died. There were 25 women with cardiovascular events (cardiovascular event group) and 277 women without cardiovascular events (non-cardiovascular event group); the two groups were compared. No significant differences were found in the perinatal outcomes. Medication use before pregnancy was identified as a risk factor for cardiovascular events (adjusted odds ratio, 23.28; 95% confidence interval, 8.15-66.47; p<0.001). In pregnant women with cardiovascular disease, New York Heart Association (NYHA) functional class II or III before pregnancy was associated with a higher risk of cardiovascular events in comparison to NYHA functional class I (p<0.001 for both). Conclusion Medication use before pregnancy and NYHA functional class >I were risk factors for cardiovascular events in pregnant women with cardiovascular disease.

Appropriate delivery method for cardiac disease pregnancy based on noninvasive cardiac monitoring.

Background There are numerous significant physiological changes occurring in circulation during labor. To detect these rapid hemodynamic changes, invasive and intermittent measurement techniques are not reliable. To suggest a suitable delivery method for pregnancy with cardiac disease, this study analyzed how each delivery method influences cardiac function using a noninvasive and continuous measurement technique. Methods A prospective study was accomplished at the National Cerebral and Cardiovascular Center in Japan from October 1, 2014, to November 30, 2018. The classification of the healthy heart pregnant women was according to the delivery method: vaginal delivery (VD) without epidural anesthesia, VD with epidural anesthesia, and caesarean section (CS). The hemodynamic parameters cardiac index (CI), stroke volume index (SI), and heart rate (HR) were evaluated regularly throughout delivery by noninvasive electrical cardiometry monitor. Results Ten cases were examined for each group. CI and HR were significantly increased before VD, while the increase in CI and HR was mild in the epidural group in comparison to the nonepidural group. SI was increased toward the delivery in the epidural group, and it was constant in the nonepidural group. However, there was no alteration in the level of outcomes of the two groups. In CS, SI increased and HR decreased before delivery. After delivery, SI continued to increase, while HR did not change but CI increased. Conclusion In VD, the increase in venous circulation according to the autotransfusion is managed by increasing HR. By epidural anesthesia, the increase in HR was suppressed and SI was increased. However, as epidural anesthesia increases the vascular capacity, the level of SI outcome was comparable. In CS, the HR was decreased because of the spinal anesthesia and the SI was increased because of many factors like hydration. As there are many factors to control in CS, VD with epidural anesthesia will be the first preference for most cardiac patients.