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Objective: Pregnancy in women with Eisenmenger syndrome (ES) presents very high morbidity and mortality rates for both the mother and fetus. In this study, we aimed to evaluate the outcomes of pregnancies affected by ES., Methods: This retrospective cohort study reviewed pregnancies in women with ES at two university hospitals over the past 10 years. The primary outcomes examined were maternal, perinatal, and pregnancy-related outcomes in women with ES., Results: The study enrolled eight pregnant women diagnosed with ES. The average maternal age was 26.7 years (range 21-36 years). The causes of ES included ventricular septal defect (five patients), patent ductus arteriosus (two patients), and atrial septal defect (one patient). Four patients had been diagnosed with ES before pregnancy. Two patients died in this group; one developed right heart failure in the third trimester and unfortunately died immediately after delivery due to cardiogenic shock, and another died on the 10th postpartum day from sudden cardiac arrest despite having had a successful delivery. Four patients were diagnosed with ES during the postpartum period; none in this group died, and there were no cases of fetal mortality. We could not identify any clinical or echocardiographic predictors for postpartum mortality. The two deceased patients showed tendencies of severe right heart failure and lower oxygen saturation during the third trimester and before delivery., Conclusion: Despite advanced obstetric care and targeted treatments for pulmonary arterial hypertension, maternal mortality is still unacceptably high in patients with ES. No significant predictors of maternal mortality were identified.

Background: Congenital bronchial atresia (CBA) is a relatively benign and uncommon pulmonary anomaly that is usually an incidental radiological finding. Bronchial atresia may also be acquired. Its association with pulmonary vascular anomalies and congenital heart disease (CHD) has been described in literature as a handful of case reports only., Case Description: We report a case of a 26-year-old female having a rare coexistence of bronchial atresia with a large patent ductus arteriosus (PDA) and a small atrial septal defect (ASD), and had Eisenmenger syndrome (ES) at the time of presentation. PDA itself causes pulmonary arterial hypertension (PAH) if not corrected early within a few months of infancy. On the contrary, CBA can also lead to PAH and along with PDA may have contributed to the development of ES in our case., Conclusion: Timely diagnosis and treatment of CBA and its associated CHD may prevent or delay the complications occurring later on., (© Journal of the Association of Physicians of India 2024.)

Swyer-James-MacLeod syndrome is characterized radiologically by hyperlucency in a single lung lobe, accompanied by reduced vascularity, alveolar hyperdistention, and air trapping, without bronchial airway obstruction. The most common congenital heart defect in childhood, ventricular septal defect, leads to irreversible pulmonary hypertension and Eisenmenger syndrome if not treated promptly. This case report presents a 25-year-old patient with Swyer-James-MacLeod syndrome and Eisenmenger syndrome. It is crucial to include Swyer-James-MacLeod syndrome in the differential diagnosis of patients with atypically distributed pulmonary emphysema and unilateral hyperlucency for early diagnosis and timely intervention.

Abstract: Pregnancy in a patient with pulmonary hypertension carries a high risk of mortality. It poses multiple problems in the management of pregnancy, labor, and postpartum, thereby emphasizing the need for a multidisciplinary team for a successful outcome. We describe the successful management of a case of Eisenmenger syndrome who developed pre-eclampsia during her 28 weeks of pregnancy. As far as our knowledge, this is the first case report that describes the use of milrinone in a parturient for a successful outcome., (Copyright © 2024 Copyright: © 2024 Annals of Cardiac Anaesthesia.)

Objective: This study assessed the long-term effects of triple therapy with prostanoids on patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), as there is limited information on the safety and efficacy of this treatment approach., Methods: A retrospective cohort study was conducted on patients with PAH-CHD who were actively followed up at our centre. All patients were already receiving dual combination therapy at maximum doses. Clinical characteristics, including functional class (FC), 6-minute walking test distance (6MWTD) and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, were documented before initiating triple therapy and annually for a 2-year follow-up period., Results: A total of 60 patients were included in the study, with a median age of 41 years and 68% being women. Of these, 32 had Eisenmenger syndrome, 9 had coincidental shunts, 18 had postoperative PAH and 1 had a significant left-to-right shunt. After 1 year of triple combination initiation, a significant improvement in 6MWTD was observed (406 vs 450; p=0.0027), which was maintained at the 2-year follow-up. FC improved in 79% of patients at 1 year and remained stable in 76% at 2 years. NT-proBNP levels decreased significantly by 2 years, with an average reduction of 199 ng/L. Side effects were experienced by 33.3% of patients but were mostly mild and manageable. Subgroup analysis showed greater benefits in patients without Eisenmenger syndrome and those with pre-tricuspid defects., Conclusions: Triple therapy with prostanoids is safe and effective for patients with PAH-CHD, improving FC, 6MWTD and NT-proBNP levels over 2 years. The treatment is particularly beneficial for patients with pre-tricuspid defects and non-Eisenmenger PAH-CHD., Competing Interests: Competing interests: RL-L, TSdlC, IM, WH, ACU, MTV, AM and PE-S have received study grants from Janssen and MSD., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES., Case Presentation: A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength., Conclusion: Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Hyperviscosity syndrome can present with haematological, neurological or cardiovascular manifestations. The common differential diagnoses for severe headache and altered sensorium in a patient with Eisenmenger syndrome include brain abscess, meningitis, cortical venous thrombosis and subarachnoid haemorrhage (SAH). We report a patient with Eisenmenger syndrome with hyperviscosity, presenting as pseudo-SAH, which was successfully treated with phlebotomy.

A case is presented where a patient with Eisenmenger's syndrome is successfully treated for infection of chronic pulmonary arterial thrombosis with good effect. The evidence for antibiotic and anticoagulant therapy in this patient group is discussed., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Despite advances in medical care, we still come across pregnancy in Eisenmenger syndrome. Eisenmenger syndrome represents the severe end of the spectrum for disease in pulmonary artery hypertension associated with CHD. Due to very high maternal and perinatal morbidity and mortality, pregnancy is contraindicated among these women. Current guidelines also recommend that the women who become pregnant should opt for early termination of pregnancy. Here, we present a case series of 11 women of Eisenmenger syndrome and their pregnancy outcome., Methods: It was a retrospective analysis of 12 pregnancies among 11 women with Eisenmenger syndrome who were managed in a tertiary care referral centre of Northern India., Results: The mean age of these women was 28 ± 4 years (range 22 to 36 years). Almost 80% of them (9/11) were diagnosed with Eisenmenger syndrome during pregnancy. The commonest cardiac lesion was Ventricular Septal defect (54.5%) followed by Atrial Septal defect (27.3%) and Patent Ductus arteriosus (9.1%). Only three women opted for medical termination of pregnancy, rest eight continued the pregnancy or presented late. Pregnancy complications found include pre-eclampsia (50%), abruption (22%), and fetal growth retardation (62.5%). There were three maternal deaths (mortality rate 27%) in postpartum period., Conclusion: This case series highlights the delay in diagnosis and treatment of CHD despite improvement in medical care. Women with Eisenmenger syndrome require effective contraception, preconceptional counselling, early termination of pregnancy, and multidisciplinary care.

Background: Eisenmenger syndrome (ES) is a rare condition seen in children with congenital heart disease (CHD). It is characterized by raised pulmonary vascular resistance (PVR) arising from a shunt reversal with the presence of desaturated blood in the systemic circulation. Proper timing and early intervention in children with congenital heart disease have made the syndrome a rare occurrence. However, this cannot be said in developing countries where facilities for the diagnosis and management of children with congenital heart disease are not optimal., Objectives: The aim of this narrative review is to highlight the importance of early diagnosis and to review the new techniques in the evaluation of children with ES. It also highlights in a snapshot the state of management of ES in a developing country., Methods: A search for published data on ES was done through several search engines such as Pubmed, google scholar citation, systematic reviews, and meta-analysis. This involves research done over the past 30 years. Keywords such as Eisenmenger'syndrome, 'congenital heart defect', 'Pulmonary hypertension', 'catherterization', 'echocardiography', and children' were used., Results: This review shows the new technique in the diagnosis, aetio-pathogenesis, management and treatment of children with ES in-depth descriptive analysis and new advances in the management of children with ES., Conclusion: Eisenmenger syndrome is a preventable disease that can be curbed by early diagnosis and treatment of children with congenital heart disease, especially in the developing world., Competing Interests: Copyright © 2023 by West African Journal of Medicine., (The Authors declare that no competing interest exists.)

Objectives: Maternal-fetal morbidity and mortality among pregnant women with pulmonary artery hypertension (PAH) and Eisenmenger syndrome are unacceptable, and management decision-making in these clinical scenarios remains debatable. This study aimed to compare and analyse clinical characteristics, management and pregnancy outcomes in PAH and Eisenmenger syndrome., Design: Prospective observational cohort study., Settings: A large tertiary care university hospital., Patients: Thirty patients with pulmonary artery hypertension and 20 patients with Eisenmenger syndrome., Methods: Data pertaining to clinical characteristics, anaesthetic, medical and obstetric management, and outcomes in pregnancy complicated by PAH and Eisenmenger syndrome were collected between July 2020 and June 2022. Each treating unit followed its management protocol in consultation with the multidisciplinary team., Main Outcome Measures: Maternal mortality and morbidity., Results: Maternal mortality was lower in the PAH group (6.6% versus 15%; p = 0.33). All mortalities were in the postpartum period. The incidence of new-onset or exacerbation of heart failure (23.3% versus 60%; p = 0.009) and hypoxaemia (13.3% versus 50%; p = 0.005) were significantly lower in the PAH group. In the Eisenmenger syndrome group, a significantly higher number of women received pulmonary hypertension and heart failure medications. Prematurity and neonatal intensive care unit admission were frequently noticed in Eisenmenger syndrome, whereas perinatal mortality, birthweight and APGAR score were comparable., Conclusions: Fetomaternal outcomes are inferior in Eisenmenger syndrome compared with PAH and are either lower or comparable to those reported from contemporary cohorts of developed nations., (© 2023 John Wiley & Sons Ltd.)

Background: The purpose of this registry was to provide insights into the characteristics, treatments and survival of patients with PAH-CHD in China., Methods: Patients diagnosed with PAH-CHD were enrolled in this national multicenter prospective registry. Baseline and follow-up data on clinical characteristics, PAH-targeted treatments and survival were collected., Results: A total of 1060 PAH-CHD patients (mean age 31 years; 67.9% females) were included, with Eisenmenger syndrome (51.5%) being the most common form and atrial septal defects (37.3%) comprising the most frequent underlying defect. Approximately 33.0% of the patients were in World Health Organization functional class III to IV. The overall mean pulmonary arterial pressure and pulmonary vascular resistance were 67.1 (20.1) mm Hg and 1112.4 (705.9) dyn/s/cm 5 , respectively. PAH-targeted therapy was utilized in 826 patients (77.9%), and 203 patients (19.1%) received combination therapy. The estimated 1-, 3-, 5-, and 10-year survival rates of the overall cohort were 96.9%, 92.9%, 87.6% and 73.0%, respectively. Patients received combination therapy had significantly better survival than those with monotherapy (p = 0.016). NT-proBNP >1400 pg/ml, SvO 2 ≤ 65% and Borg dyspnea index ≥ 3 and PAH-targeted therapy were independent predictors of mortality. Hemoglobin > 160g/L was a unique predictor for mortality in Eisenmenger syndrome., Conclusions: Chinese PAH-CHD patients predominantly exhibit Eisenmenger syndrome and have significantly impaired exercise tolerance and right ventricular function at diagnosis, which are closely associated with long-term survival. PAH-targeted therapy including combination therapy showed a favorable effect on survival in PAH-CHD. The long-term survival of Chinese CHD-PAH patients remains to be improved., Competing Interests: Disclosure statement None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose., (Copyright © 2023 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Introduction: Congenital heart disease (CHD) is the most common cardiac disorder in pregnancy in the western world (around 80%). Due to improvements in surgical interventions more women with CHD are surviving to adulthood and choosing to become pregnant., Areas Covered: Preconception counseling, antenatal management of CHDs and strategies to prevent maternal and fetal complications.Preconception counseling should start early, before the transition to adult care and be offered to both men and women. It should include the choice of contraception, lifestyle modifications, pre-pregnancy optimization of cardiac state, the chance of the child inheriting a similar cardiac lesion, the risks to the mother, and long-term prognosis. Pregnancy induces marked physiological changes in the cardiovascular system that may precipitate cardiac complications. Risk stratification is based on the underlying cardiac disease and data from studies including CARPREG, ZAHARA, and ROPAC., Expert Opinion: Women with left to right shunts, regurgitant lesions, and most corrected CHDs are at lower risk and can be managed in secondary care. Complex CHD, including systemic right ventricle need expert counseling in a tertiary center. Those with severe stenotic lesions, pulmonary artery hypertension, and Eisenmenger's syndrome should avoid pregnancy, be given effective contraception and managed in a tertiary center if pregnancy does happen.

The incidence of heart disease in pregnancy ranges from 0.5% to 3.0% and is regarded as one of the top three causes of maternal death. The mortality rate of patients with pulmonary hypertension and Eisenmenger syndrome is as high as 16.7%-50%. Changes in haemodynamics during pregnancy and childbirth increase the burden on the heart, and induced pulmonary hypertension crisis is one of the main causes of maternal death. Extracorporeal Membrane Oxygenation (ECMO) is the last-resort treatment strategy to treat patients with pulmonary hypertension crisis. We report a ventricular septal defect in a pregnant woman with pulmonary hypertension and Eisenmenger's syndrome, which is a postpartum pulmonary hypertension crisis that leads to respiratory and circulatory disorders. The patient was successfully treated with venous-venous extracorporeal membrane oxygenation.

Eisenmenger syndrome is a life-threatening complication of congenital heart defects (CHD). Since Eisenmenger syndrome among children of repaired CHD is rare, very few studies have had the necessary data to investigate its distribution in children. The current study used data collected in rural China to investigate the prevalence of Eisenmenger syndrome in children with unrepaired CHD. Data were from the 2006 to 2016 patient medical records of China California Heart Watch, which is a traveling cardiology clinic in Yunnan Province, China. Patients were included if they (1) aged 18 or below, (2) had CHD(s), and (3) the defect was not repaired by the time of the clinic visit. The prevalence of Eisenmenger syndrome was calculated in each age and defect group. Using logistic regression models, we tested whether oxygen saturation, Down syndrome, sex, and age were significantly associated with Eisenmenger syndrome. Of the 1301 study participants, ventricular septum defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA) were the most common CHD. About one-sixth of the patients had pulmonary hypertension and 1.5% had Eisenmenger syndrome. The percentages of Eisenmenger syndrome were 1.8% in VSD patients, 0 in ASD patients, and 0.9% in PDA patients. Patients in the age group between 15 and 18 years had the highest percentages of Eisenmenger syndrome (11.5%). Age and presence of Down syndrome were significantly associated with the presence of Eisenmenger syndrome. Our finding highlights the importance of early detection and correction of CHD., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Myocardial fibrosis is a common pathophysiological process involved in many cardiovascular diseases. However, limited prior studies suggested no association between focal myocardial fibrosis detected by cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) and disease severity in Eisenmenger syndrome (ES). This study aimed to explore potential associations between myocardial fibrosis evaluated by the CMR LGE and T1 mapping and risk stratification profiles including exercise tolerance, serum biomarkers, hemodynamics, and right ventricular (RV) function in these patients., Methods: Forty-five adults with ES and 30 healthy subjects were included. All subjects underwent a contrast-enhanced 3T CMR. Focal replacement fibrosis was visualized on LGE images. The locations of LGE were recorded. After excluding LGE in ventricular insertion point (VIP), ES patients were divided into myocardial LGE-positive (LGE + ) and LGE-negative (LGE - ) subgroups. Regions of interest in the septal myocardium were manually contoured in the T1 mapping images to determine the diffuse myocardial fibrosis. The relationships between myocardial fibrosis and 6-min walk test (6MWT), N-terminal pro-brain natriuretic peptide (NT-pro BNP), hematocrit, mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance index (PVRI), RV/left ventricular end-systolic volume (RV/LV ESV), RV ejection fraction (RVEF), and risk stratification were analyzed., Results: Myocardial LGE (excluding VIP) was common in ES (16/45, 35.6%), and often located in the septum (12/45, 26.7%). The clinical characteristics, hemodynamics, CMR morphology and function, and extracellular volume fraction (ECV) were similar in the LGE + and LGE - groups (all P > 0.05). ECV was significantly higher in ES patients (28.6 ± 5.9% vs. 25.6 ± 2.2%, P < 0.05) and those with LGE - ES (28.3 ± 5.9% vs. 25.6 ± 2.2%, P < 0.05) than healthy controls. We found significant correlations between ECV and log NT-pro BNP, hematocrit, mPAP, PVRI, RV/LV ESV, and RVEF (all P < 0.05), and correlations trends between ECV and 6MWT (P = 0.06) in ES patients. An ECV threshold of 29.0% performed well in differentiating patients with high-risk ES from those with intermediate or low risk (area under curve 0.857, P < 0.001)., Conclusions: Myocardial fibrosis is a common feature of ES. ECV may serve as an important imaging marker for ES disease severity., (© 2022. The Author(s).)

Hemoptysis, accompanying various chronic lung diseases, some systemic diseases, infections, structural heart diseases, or syndromes is a clinical condition that is quite mortal when it is massive. Hemoptysis is a common complication of Eisenmenger syndrome. Its frequency increases with age. It is an important cause of mortality in patients with Eisenmenger syndrome. Embolization of systemic-pulmonary collateral arteries is an effective method in the treatment of hemoptysis in eligible patients with Eisenmenger syndrome. In this case report, a patient with Eisenmenger syndrome, developed due to large patent ductus arteriosus, received dual pulmonary arterial hypertension-specific treatment, after the development of hemoptysis, medical stabilization was provided with initial inhaled nitric oxide therapy and then treated with bronchial artery embolization without complications is presented.

Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension and is associated with congenital heart disease. ES itself is a challenging condition to manage, further compounded if the patient is critically ill and acutely decompensated. We share our experience of managing a critically ill adult patient with ES who presented with acute decompensation due to sepsis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Congenital shunt location is related to Eisenmenger syndrome (ES) survival. Moreover, right ventricular (RV) remodeling is associated with poor survival in pulmonary hypertension., Purpose: To investigate RV remodeling using comprehensive magnetic resonance imaging (MRI) techniques and identify its relationship with prognosis in ES subgroups classified by shunt location., Study Type: Prospective observational study., Population: Fifty-four adults with ES (16 with pre-tricuspid shunt and 38 with post-tricuspid shunt)., Field Strength/sequence: 3.0 T/cine MRI with balanced steady-state free precession sequence, late gadolinium enhancement with inversion recovery segmented gradient echo sequence and phase-sensitive reconstruction, and T1 mapping with modified Look-Locker inversion recovery sequence., Assessment: Demographics, clinical characteristics, hemodynamics, RV remodeling features (morphology, systolic function, RV-pulmonary artery (PA) coupling and myocardial fibrosis), and prognosis were compared between ES subgroups. The adverse endpoint was all-cause mortality or readmission for heart failure., Statistical Tests: The independent samples t-test, Fisher's exact test or Chi-squared test, and the Kaplan-Meier method were used. P < 0.05 was considered significant., Results: Compared to patients with post-tricuspid shunt, patients with pre-tricuspid shunt were significantly older and had higher N-terminal pro-B-type natriuretic peptide concentrations and poorer exercise tolerance. Pre-tricuspid shunt showed significantly larger RV dimensions (end-diastolic volume index: 185.81 ± 37.49 vs. 98.20 ± 36.26 mL/m 2 ), worse RV ejection fraction (23.54% ± 12.35% vs. 40.82% ± 10.77%), and RV-PA decoupling (0.35 ± 0.31 vs. 0.72 ± 0.29). Biventricular myocardial fibrosis was significantly more severe in pre-tricuspid shunt than post-tricuspid shunt (extracellular volume, left ventricle: 35.85% ± 2.58% vs. 29.10% ± 5.20%; RV free wall: 30.93% ± 5.65% vs. 26.75% ± 5.15%). In addition, pre-tricuspid shunt demonstrated a significantly increased risk of adverse endpoint (hazard ratio: 2.938, 95% confidence interval: 1.204-7.172)., Data Conclusion: ES with pre-tricuspid shunt might be a unique subtype with worse clinically decompensated RV remodeling and poor prognosis., Level of Evidence: 2 Technical Efficacy Stage: 5., (© 2021 International Society for Magnetic Resonance in Medicine.)

Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study. PH was determined by cardiac catheterization data or echocardiography reports. In accord with the World Symposia on Pulmonary Hypertension, CHD was further categorized as simple, severe, or complex CHD (including pulmonary atresia-ventricular septal defect and single-ventricle anomalies). There were 4301 patients (55.6% women), 15.7% with severe and 3.9% with complex CHD. The cumulative incidence of PH was 4.4% (95% CI, 3.8-5.0). Our multivariable regression model indicated 4.2-fold mortality increase (95% CI, 3.0-5.9) in the presence of PH, with age, female sex, and severe or complex CHD linked to higher incidence of PH. Only 49% of patients received PH-specific therapy. Five- and 10-year survival rates of patients with PH (n=190) were 72.3% (95% CI, 65.1%-78.4%) and 58.8% (95% CI, 50.1%-66.5%), respectively. Survival rates in those with Eisenmenger syndrome, PH after defect correction, and complex CHD were similar. Low oxygen saturation and high uric acid levels were associated with increased mortality. Conclusions In this sizable Asian adult CHD cohort, the cumulative incidence of PH was aligned with that of Western countries. Mortality proved higher in patients with PH versus without PH. Although complex CHD carried greater risk of PH compared with other adult CHD subsets, survival rate was similar.

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond., Competing Interests: Funding Support and Author Disclosures Dr Arvanitaki is the recipient of the International Training and Research Fellowship EMAH Stiftung Karla Voellm. Dr Gatzoulis has received unrestricted educational grants from Actelion, Pfizer, and GlaxoSmithKline. Dr Opotowsky has received funding from Actelion, Janssen, and Johnson and Johnson, not directly related to the current work; and has received funding by the Heart Institute Research Core (HIRC) supporting research in the Cincinnati Children’s Heart Institute. Dr Khairy is supported by the André Chagnon Research Chair in Electrophysiology and Congenital Heart Disease. Dr Diller has received funding from Actelion, Daiichi-Sankyo, and Bayer, unrelated to the current work; and has received funding by the Karla Völlm Foundation, supporting research in the Department. Dr Giannakoulas has receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, Galenica-Ferrer, GlaxoSmithKline, Pfizer, Lilly, Merck Sharp & Dohme, and United Therapeutics, not directly related to this work. Dr Grünig has received fees for lectures and/or consultations from Actelion, Bayer, GlaxoSmithKline, Janssen, Merck Sharp & Dohme, Pfizer, and United Therapeutics. Dr D’Alto has received funding from Actelion-Janssen, Merck Sharp & Dohme, GlaxoSmithKline, and Dompé, not directly related to the current work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background and Objectives : Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients' well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. Materials and Methods : The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. Results : Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. Conclusions : On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes.

BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal extramedullary and non-hepatosplenic extramedullary hematopoiesis a 43-year-old man with a history of congenital Eisenmenger syndrome. CASE REPORT We present the case of a 43-year-old patient with complex heart disease and full-blown Eisenmenger syndrome associated with ventricular septal defect, bicuspid right ventricle, and pulmonary hypertension. In July 2020, the patient began to report neurological symptoms in the form of lower-limb numbness and weakness of lower-limb strength, with additional increasing lower-limb edema. A CT scan of the abdomen and pelvis revealed a 63×102×103 mm soft-tissue mass in the pelvis, located behind the urinary bladder. Due to the suspicion of proliferative disease, mainly of the lymphatic system, a diagnostic trephine biopsy was performed first, but no tumor cell infiltration was found. Then, the patient was qualified for diagnostic surgery. During the operation, tumor sections were harvested. Histopathological examination of the tissue sections showed extramedullary hematopoiesis in the tumor lining. Hydroxycarbamide was used as first-line treatment. However, it was not effective in controlling clinical symptoms. Therefore, the patient was qualified for radiotherapy as a second-line palliative treatment. CONCLUSIONS This report presents a patient with cyanotic heart disease and extramedullary and non-hepatosplenic hematopoiesis presenting as masses that mimicked malignancy. In this case, palliative radiotherapy effectively reduced the symptoms due to the size of the mass lesion.

A young boy in his 20s presented with sudden diminution of vision in right eye 2 days ago. Best-corrected visual acuity was hand movement in right eye and 20/20 in left eye. Funduscopy demonstrated diffuse retinal opacification and swelling at the posterior pole with a cherry red spot at the macula. There was sparing of the superonasal macula with a patent cilioretinal artery. OCT showed diffuse inner retinal thickening of the temporal macula. He was diagnosed as central retinal artery occlusion with cilioretinal sparing in right eye. He was a known case of Eisenmenger syndrome secondary to a large congenital ventricular septal defect. Medical history was notable for prominent pulmonary hypertension and shortness of breath on exertion. Physical examination revealed a harsh systolic murmur. Extremities showed digital erythema and severe clubbing. Chest X-ray demonstrated cardiomegaly. Laboratory examination revealed haemoglobin-220 g/L, haematocrit-60%, serum iron-20 µg/dL., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis. The primary management strategy for most patients with PAH-CHD is medical therapy, although defect closure is considered in select cases., (Copyright © 2021 Elsevier Inc. All rights reserved.)

A 52-year-old man who was diagnosed with Eisenmenger syndrome due to a muscular-type ventricular septal defect 30 years previously, visited our emergency room after experiencing six hours of severe left flank pain and vomiting. On laboratory examination, azotemia and microscopic haematuria were identified. Contrast-enhanced computed tomography also revealed pulmonary embolism (PE) and bilateral acute renal infarction. The flank pain resolved after heparin was administered for anti-coagulation and aspiration thrombectomy was performed. The patient was discharged on warfarin as anticoagulant therapy. In this case, a paradoxical embolism was considered to have been the cause of PE and bilateral acute renal infarction in a patient with Eisenmenger syndrome.

Introduction: For many years, treatment options for patients with Eisenmenger physiology had been restricted to conservative measures to alleviate multi-system complications. The use of pulmonary arterial hypertension (PAH)-targeted therapies in patients with Eisenmenger syndrome (ES) changed the course of the disease, since they substantially improved clinical outcomes and increased survival., Areas Covered: In this review, we primarily focus on the use of PAH pharmacotherapies in ES. A literature search was carried out in PubMed, Scopus and Cochrane Database up to May 2021. We thoroughly discuss current evidence about mechanisms of action, safety, and efficacy of these agents and present challenges and gaps in literature regarding the recommended treatment approach., Expert Opinion: Unlike other forms of PAH, we usually treat patients with ES more conservatively as we lack evidence that aggressive management is safe and effective in this complex population. Several issues on the time of initiation of PAH-targeted therapies, choice between monotherapy vs. upfront combination therapy, and time of escalation still remain challenging and require further investigation. Therapeutic management should be guided by patients' individual evaluation based on available prognostic markers. More well-designed trials are warranted to assess the benefits of new PAH-targeted agents and combination therapies.

After reviewing the current definitions and classification of pulmonary hypertension (PH) associated with congenital heart disease (CHD), based on an analysis of 59 clinical trials (of which 14 are randomized controlled trials) drugs registered in the Russian Federation, the evidence base for PH therapy in adults with CHD is provided. The presence of a randomized controlled trial of bosentan BREATHE-5 and uncontrolled trials of other drugs became the basis for a higher class and level of evidence of bosentan (IB) compared to other drugs (IIaC) for Eisenmenger syndrome in the current European (ERS/ESC 2015) and updated Russian (2020) guidelines. According to the updated European (ESC 2020) guidelines for congenital heart disease in adults, in Eisenmenger patients with reduced exercise capacity (6MWT distance 450 m), a treatment strategy with initial endothelin receptor antagonist monotherapy should be considered followed by combination therapy if patients fail to improve (IIaB), in low- and intermediate-risk patients with repaired simple lesions and pre-capillary PH, initial oral combination therapy or sequential combination therapy is recommended and high-risk patients should be treated with initial combination therapy including parenteral prostanoids (IA) and endothelin receptor antagonists and phosphodiesterase 5 inhibitors may be considered in selected patients with elevated pulmonary pressure/resistance in the absence of elevated ventricular end diastolic pressure (IIbC). Only three (bosentan, macitentan and selexipag) out of seven specific pulmonary vasodilators registered in the Russian Federation have indications for pulmonary arterial hypertension associated with congenital heart disease and Eisenmenger syndrome or pulmonary arterial hypertension associated with corrected simple congenital heart disease in the instructions for use.

Pulmonary arterial hypertension (PAH) - a complex and progressive disease that carries significant morbidity and mortality despite optimal medical treatment. Combination therapy for PAH can be more effective than monotherapy. The present randomized trial compared the safety and efficacy of sildenafil ambrisentan combination therapy with sildenafil monotherapy. Twenty-two patients of Eisenmenger syndrome and five patients of idiopathic PAH were randomized to two arms. There was a significant improvement in NYHA functional class and mean pulmonary artery pressure, while an insignificant improving trend was observed for 6-min walk distance and oxygen saturation, following the 12 weeks of combination therapy. An upfront combination therapy was found to be safe and effective in the management of PAH patients., Competing Interests: Declaration of competing interest There is no conflict of interest on any of the authors about the present study., (Copyright © 2021 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.)

Eisenmenger syndrome is a multisystem disorder and the most severe form of pulmonary arterial hypertension in adult congenital heart disease. Pulmonary arterial hypertension represents a fatal disease, characterized by increased pulmonary vascular resistance, right heart failure, and death. Although therapeutic management has rapidly advanced in recent years, these patients were not included in randomized controlled trials for specific pulmonary arterial hypertension drugs, except for bosentan. However, in clinical practice we apply treatment strategies combining drugs targeting multiple pathobiological pathways. We present 3 patients with Eisenmenger syndrome and their improvement after starting treatment with selexipag, an oral selective IP prostacyclin receptor agonist., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era., (Copyright: © 2021 The Author(s).)

Introduction : Eisenmenger syndrome describes a condition in which a congenital heart defect has caused severe pulmonary vascular disease, resulting in reversed (right-left) or bidirectional shunting and chronic cyanosis. Areas covered : In this paper, the progression of congenital heart defects to Eisenmenger syndrome, including early screening, diagnosis and operability are covered. The mechanisms of disease progression in Eisenmenger syndrome and management strategies to combat this, including the role of pulmonary arterial hypertension therapies, are also discussed. Expert opinion/commentary : Patients with congenital heart disease (CHD) are at increased risk of developing pulmonary arterial hypertension with Eisenmenger syndrome being its extreme manifestation. All CHD patients should be regularly assessed for pulmonary hypertension. Once Eisenmenger syndrome develops, shunt closure should be avoided. The clinical manifestations of Eisenmenger syndrome are driven by the systemic effects of the pulmonary hypertension, congenital defect and long-standing cyanosis. Expert care is essential for avoiding pitfalls and preventing disease progression in this severe chronic condition, which is associated with significant morbidity and mortality. Pulmonary arterial hypertension therapies have been used alongside supportive care to improve the quality of life, exercise tolerance and the outcome of these patients, although the optimal timing for their introduction and escalation remains uncertain.

Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and respiratory tract infections. Most patients die during childhood. We report a 53-year-old male patient with a large aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta, with Eisenmenger syndrome and without surgery.

Purpose of Review: Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common association adversely affecting quality of life and survival in these patients. We provide herewith recent advances in the understanding and management of PAH-CHD., Recent Findings: Significant progress has been made in disease-targeting therapy with pulmonary vasodilators for the treatment of Eisenmenger syndrome, the most severe form of PAH-CHD. Important gaps, however, still exist in the assessment and management of patients with PAH-CHD with systemic to pulmonary shunts. The choice of therapy, either interventional, medical, or both is an on-going dilemma that requires more long-term data. PAH after defect closure represents the most concerning subgroup of patients with the worst prognosis, requiring close follow-up and proactive disease-targeting therapy treatment. Small defects are not considered responsible for patients who have severe PAH and therefore, present different subgroup of patients similar to idiopathic PAH., Summary: Even with advances in diagnosis and treatment PAH-CHD remains a challenging field requiring lifelong follow-up and meticulous treatment in centres specialized in both CHD and PAH.

Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults.