Acquired Cardiovascular Diseases in Patients with Pulmonary Hypertension Due to Congenital Heart Disease: A Case Report.

Background: Advances in the diagnosis and treatment of congenital heart diseases (CHDs) have resulted in improved survival rates for CHD patients. Up to 90% of individuals with mild CHD and 40% with complex CHD now reach the age of 60. Previous studies have indicated an elevated risk of atherosclerotic cardiovascular disease (ASCVD) and associated risk factors, morbidity, and mortality in adults with congenital heart disease (ACHD). However, there were no comprehensive guidelines for the prevention and management of acquired cardiovascular diseases (CVDs) in ACHD populations until recently. Case presentation: A 55-year-old man with Eisenmenger syndrome and comorbidities (arterial hypertension, heart failure, dyslipidemia, hyperuricemia, and a history of pulmonary embolism (PE)) presented with progressive breathlessness. The electrocardiogram (ECG) revealed signs of right ventricle (RV) hypertrophy and overload, while echocardiography showed reduced RV function, RV overload, and severe pulmonary hypertension (PH) signs, and preserved left ventricle (LV) function. After ruling out a new PE episode, acute coronary syndrome (ACS) was diagnosed, and percutaneous intervention was performed within 24-48 h of admission. Conclusions: This case highlights the importance of increased awareness of acquired heart diseases in patients with pulmonary hypertension due to CHD.

Factores pronósticos en el trasplante cardiopulmonar: experiencia a 30 años en un centro de referencia / Prognosis factors in heart–lung transplantation: Thirty years experience in a reference center

Introducción: El trasplante cardiopulmonar (TCP) ha presentado una disminución progresiva en el número de procedimientos. En nuestro país existe poca información al respecto, siendo el objetivo de este estudio analizar la experiencia de un hospital de referencia. Métodos: Estudio observacional unicéntrico de una cohorte histórica en el periodo entre 1990 y 2021. Las asociaciones entre categorías se evaluaron mediante la prueba de X2 o la f de Fisher. La supervivencia se analizó a través del método de Kaplan-Meier. Las diferencias se evaluaron con el estudio de log-rank y el análisis multivariante con el método de Cox. Resultados: Se observó una reducción del número de procedimientos realizados en el último decenio (2000-2009: 19 [44,2%]; 2010-2021: 15 [34,8%]). La mortalidad posoperatoria precoz fue de 23,3%, reduciéndose a 13,3% a partir del 2010. La intrahospitalaria fue de 41%, disminuyendo a 33% en 2010. Los factores asociados a la mortalidad fueron cirugía torácica previa, corticoterapia, circulación extracorpórea (CEC) mayor a 200 min, tiempo de isquemia mayor a 300 min y dehiscencia traqueal (p < 0,005). La supervivencia global a uno, cinco y 10 años fue de 58, 44,7 y 36,1%, respectivamente. Los factores asociados a menores tasas de supervivencia fueron cirugía torácica previa, donante masculino, CEC mayor 200 min, tiempo de isquemia mayor a 300 min, dehiscencia traqueal y diferencia de pesos (p < 0,005). Conclusiones: Existe una disminución en el número de procedimientos, siendo más evidente en la última década, pero evidenciando una mejora tanto de la mortalidad posoperatoria y supervivencia.(AU)

Introduction: Heart–lung transplantation has shown a progressive decrease in the number of procedures. There is a lack of information about this field in Spain. The main goal of this study is to analyze the experience of a national reference hospital. Methods: We performed a retrospective study of a historical cohort of heart–lung transplanted patients in a single center, during a 30 years period (from 1990 to 2021). The associations between variables were evaluated using the χ2 test or Fisher's exact test. Survival was analyzed using the Kaplan–Meier method. Differences were evaluated using the log-rank test and multivariate analysis with the Cox method. Results: A decrease in the number of procedures performed in the last decade was observed [2000–2009: 19 procedures (44.2%); 2010–2021: 15 procedures (34.8%)]. Early postoperative mortality was 23.3%, falling to 13.3% from 2010. In-hospital mortality was 41%, falling to 33% from 2010. Main factors related to higher mortality: previous thoracic surgery, corticosteroid therapy, extracorporeal circulation (ECLS) greater than 200 min, ischemia time greater than 300 min, and tracheal dehiscence (p < 0.005). Overall survival at one, five, and ten years was 58%, 44.7%, and 36.1%, respectively. Factors associated with lower survival rates: previous thoracic surgery, male donor, extracorporeal circulation greater than 200 min, ischemia time greater than 300 min, tracheal dehiscence and weight difference (p < 0.005). Conclusions: There has been a progressive decrease in the number of heart–lung transplantations, being more evident in the last decade, but showing an improvement in both mortality and survival.(AU)

Corazón univentricular sin cirugía de Fontan: el tipo de paliación importa / Univentricular hearts not undergoing Fontan: the type of palliation matters

Introducción y objetivos: La información sobre pacientes con fisiología univentricular (FU) y flujo pulmonar restrictivo no sometidos a cirugía de Fontan es escasa. El objetivo de este estudio es comparar la supervivencia y los eventos cardiovasculares en estos pacientes según el tipo de paliación.MétodosLos datos de pacientes con FU se obtuvieron de las bases de datos de 7 centros con unidades de cardiopatías congénitas del adulto. Se excluyó a los pacientes que completaron la circulación de Fontan o desarrollaron un síndrome de Eisenmenger. Se crearon 3 grupos según la fuente de flujo pulmonar: G1, flujo anterógrado pulmonar restrictivo; G2, shunt cavopulmonar, y G3, shunt aortopulmonar±shunt cavopulmonar. El objetivo principal fue la muerte.ResultadosSe identificó a 120 pacientes. La media de edad en la primera visita fue 32,2 años. El seguimiento medio fue de 7,1 años. Se asignó a 55 pacientes (45,8%) al G1, 30 (25%) al G2 y 35 (29,2%) al G3. Los pacientes del G3 tenían peores función renal, clase funcional y fracción de eyección en la primera visita y mostraron una disminución más marcada de la fracción de eyección durante el seguimiento, especialmente en comparación con el G1. Veinticuatro pacientes (20%) fallecieron, 38 (31,7%) ingresaron por insuficiencia cardiaca y 21 (17,5%) presentaron aleteo/fibrilación auricular durante el seguimiento. Estos eventos fueron más frecuentes en el G3 y al compararlos con los del G1 se encontraron diferencias significativas en muerte (HR=2,9; IC95%, 1,14-7,37; p=0,026) y aleteo/fibrilación auricular (HR=2,9; IC95%, 1,11-7,68; p=0,037).ConclusionesEl tipo de paliación de los pacientes con FU y flujo pulmonar restrictivo no sometidos a cirugía de Fontan identifica distintos perfiles. Los pacientes paliados con derivaciones aortopulmonares presentan un peor pronóstico, con más morbilidad y mortalidad. (AU)

Introduction and objectives: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation.MethodsSVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death.ResultsWe identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037).ConclusionsThe type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality. (AU)

Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease.

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.

Lung infarction secondary to infected pulmonary arterial thrombosis in a patient with Eisenmenger's syndrome.

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.

Intravascular Ultrasound-Guided Stent Implantation in the Left Main Coronary Artery Extrinsic Compression by Pulmonary Artery Aneurysm Due to Eisenmenger Syndrome.

Pulmonary arterial hypertension (PAH) is a profoundly destructive condition marked by the gradual narrowing and restructuring of small pulmonary arteries, leading to a rise in pulmonary vascular resistance (PVR), causing right-sided heart failure and, ultimately, mortality. During more advanced stages of this disease, patients may present with rare manifestations of pulmonary artery aneurysm (PAA) which are exertional chest pain, and hoarseness. The left main coronary artery compression (LMCA-Co) has been an increasingly recognized and possibly life-threatening entity in patients with severe PAH. The lack of well-established decision-making strategies for the management of both PAA and LMCA-Co has been remained as an unsolved issue in this setting. In this report, we present a case of percutaneous intervention of LMCA-Co with the guidance of intracoronary imaging in a patient with patent ductus arteriosus-Eisenmenger syndrome. Percutaneous intervention with intravascular guidance appears to be a safe and effective option for relieving symptoms and achieving positive clinical outcomes in patients with LMCA-Co.

Eisenmenger Syndrome: A Revisit of a Hidden but Catastrophic Disease.

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.

CONTEXTE: Le syndrome d'Eisenmenger (SE) est une affection rare observée chez les enfants atteints de cardiopathie congénitale. Il se caractérise par une augmentation de la résistance vasculaire pulmonaire (RVP) due à l'inversion d'un shunt et à la présence de sang désaturé dans la circulation systémique. Le syndrome est devenu rare chez les enfants atteints de cardiopathie congénitale grâce à un choix judicieux du moment et à une intervention précoce. Toutefois, il n'en va pas de même dans les pays en développement où les moyens de diagnostic et de prise en charge des enfants atteints de cardiopathies congénitales ne sont pas optimaux. OBJECTIFS: L'objectif de cette revue narrative est de souligner l'importance d'un diagnostic précoce et de passer en revue les nouvelles techniques d'évaluation des enfants atteints de SE. Elle met également en lumière, sous forme d'un instantané, l'état de la prise en charge de l'ES dans un pays en développement. MÉTHODES: Une recherche de données publiées sur l'ES a été effectuée à l'aide de plusieurs moteurs de recherche tels que Pubmed, google scholar citation, revues systématiques et méta-analyses. Il s'agit de recherches effectuées au cours des 30 dernières années. Des mots clés tels que "syndrome d'Eisenmenger", "malformation cardiaque congénitale", "hypertension pulmonaire", "cathétérisme", "échocardiographie" et "enfants" ont été utilisés. RÉSULTATS: Cette revue présente les nouvelles techniques de diagnostic, d'étio-pathogénie, de prise en charge et de traitement des enfants atteints de SE, ainsi qu'une analyse descriptive approfondie et les nouvelles avancées dans la prise en charge des enfants atteints de SE. CONCLUSION: Le syndrome d'Eisenmenger est une maladie évitable qui peut être enrayée par un diagnostic et un traitement précoces des enfants atteints de cardiopathies congénitales, en particulier dans les pays en développement. Mots-clés: Syndrome d'Eisenmenger; Enfants; Cardiopathie congénitale; Hhypertension pulmonaire; Prise en charge.

Pseudoaneurysm after aortopulmonary window repair and bilateral lung transplantation for eisenmenger syndrome: a case report.

BACKGROUND: Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various surgical techniques, and the short- and long-term results are excellent if the surgical repair is performed early in life. To our knowledge, there have been no reports of pseudoaneurysm after APW repair. Herein, we present a case of a 30-year-old woman with an ascending aortic pseudoaneurysm found at the site of APW repair nine months after the APW repair and bilateral lung transplantation. CASE PRESENTATIONS: A 30-year-old woman presented with APW and Eisenmenger syndrome. The patient underwent APW repair and bilateral lung transplantation. We transected the communication between the aorta and pulmonary artery and closed the aortic side directly with strips of felts. Nine months after the surgery, the patient complained of chest pain. Cardiac computed tomography revealed an ascending aortic pseudoaneurysm at the anastomotic site. Emergent graft replacement of the ascending aorta was performed and the postoperative course was uneventful. CONCLUSIONS: We have presented a case of a pseudoaneurysm at the anastomotic site after APW repair and bilateral lung transplantation. The choice of surgical technique should be based on the patient's background requiring lung transplantation, and in these cases close postoperative follow-up is required.

Pregnancy in women with congenital heart disease: a focus on management and preventing the risk of complications.

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.

Management and outcomes of pulmonary artery hypertension and Eisenmenger syndrome during pregnancy: a prospective observational cohort study.

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.

Characteristics, treatments and survival of pulmonary arterial hypertension associated with congenital heart disease in China: Insights from a national multicenter prospective registry.

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/cm5, 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, SvO2 ≤ 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.

Impact of current targeted drug therapy on the prognosis of Eisenmenger syndrome: A large-scale retrospective analysis.

BACKGROUND: Data regarding the prognosis of Eisenmenger syndrome (ES) and effect of targeted drugs are limited. This study aimed to analyze the prognosis and impact of targeted drug therapy on the survival rate of patients with ES in the Chinese population. METHODS: The data of patients with ES referred to our hospital between January 2010 and December 2020 were retrospectively analyzed. Data included baseline demographics, echocardiographic parameters, and clinical diagnoses. All patients were followed up via telephone interviews in February 2022. The primary endpoint was mortality. RESULTS: Overall, 1,021 patients with ES were included. The 1-, 3-, 5-, 7-, 10-, and 12-year survival rates were 91.6%, 84.2%, 80.7%, 73.8%, 71.4%, and 69.9%, respectively. Patients with atrial septal defects had the best prognosis than those with ventricular septal defects, patent ductus arteriosus, and complex congenital heart disease (CHD) (P < 0.0001). Patients who visited between 2016 and 2020 received increased targeted drug therapy and had a better prognosis than those who visited between 2010 and 2015 (all P < 0.05). Cox regression analysis revealed age, pulmonary arterial systolic pressure, post-tricuspid shunt CHD, targeted drugs, and year of the first hospital visit to be predictors of death (P < 0.05). CONCLUSIONS: Survival rates associated with an increased use of combined targeted drugs significantly improved in patients with ES. However, numerous factors that predict increased mortality remain to be elucidated.

Successful Eisenmenger syndrome-targeted drug therapy in pregnant women: a case series and literature review.

OBJECTIVE: To clarify the real-world outcomes in pregnant women with Eisenmenger syndrome (ES) in the new therapeutic era and provide a literature review. DESIGN: Retrospective case and literature review. SETTING: Tertiary referral hospital (The Second Xiangya Hospital of Central South University). SAMPLE: Thirteen women with ES delivered between 2011 and 2021. METHODS: Respective study and literature reviews. MAIN OUTCOMES MEASURES: Maternal and neonatal mortality and morbidity. RESULTS: 12/13 (92%) pregnant women were treated with targeted drugs. 9/13 (69%) of patients had heart failure, but no maternal deaths occurred. 12/13 (92%) of women chose caesarean delivery. One pregnant woman gave birth at 37+1  weeks, and the remaining 12 (92%) patients had preterm birth. 10/13 (77%) women gave birth to live infants, of which 9/10 (90%) were low birthweight infants with a mean birthweight of 1575 g. The infant mortality rate was 1/10 (10%). Cardiac functional class improved during pregnancy, probably due to therapy; 11/13 (85%) of the pregnant women were in cardiac functional level III/IV at admission and 12 (92%) were in cardiac functional class II/III at discharge. Our literature review identified 72 cases of pregnancy with ES from 11 studies, which were characterised by a low rate of targeted drug use (28%) and a high maternal mortality rate of 24% in the perinatal period. CONCLUSION: Our case series and literature review suggest that targeted drugs may be key to improving maternal mortality in ES.

Outcomes of heart-lung transplantation in Eisenmenger syndrome compared to primary pulmonary hypertension.

OBJECTIVE: Heart-Lung Transplantation (HLTX) is required both in primary pulmonary hypertension (PPH) and Eisenmenger syndrome (ES) when there is associated end-stage heart disease. Although PPH is associated with an otherwise structurally normal heart, ES is associated with congenital heart defects, which may increase the complexity of the operation. This study analyzes if the diagnosis (PPH vs. ES) is related to short-term outcomes after HLTX. METHODS: Patients ≥18 years of age with PPH and ES who underwent HLTX were identified in the United Network for Organ Sharing database from 2005 to 2021. Patients were propensity score matched on heart and lung listing status at the time of transplant. Univariable, multivariable, and Kaplan-Meir survival analyses were performed. RESULTS: The unmatched cohort had 128 PPH and 44 ES patients, and the matched cohort had 44 patients in each group. PPH patients had lower waitlist times and PA pressures but higher FEV1, heart, and lung listing status and ECMO bridge. There were no differences in immediate postoperative outcomes such as dialysis, stroke, and airway dehiscence. PPH patients had a higher treatment rejection in the first year. The 30-day, 1-year, and 3-year survival were better in the PPH group. However, a landmark analysis excluding deaths within 30 days eliminated differences in survival between the groups. Post-transplant dialysis and postoperative mechanical ventilation >5 days were risk factors for 1-year mortality in ES. CONCLUSION: The short-term outcomes of HLTX are inferior in ES compared to PPH and much of the attrition in ES occurs in the immediate postoperative period.

Multidisciplinary approach for the management of term pregnancy complicated by Eisenmenger syndrome.

Pregnancy in patients with Eisenmenger syndrome (ES) is associated with high maternal mortality rates of 30%‒50%, or even up to 65% in the case of a cesarean section (Yuan, 2016). Here, we report a case of term pregnancy complicated with ES and severe pulmonary artery hypertension (PAH), which was managed by a multidisciplinary team (MDT) and resulted in an uncomplicated delivery via elective cesarean section. The goal of this study is to emphasize the importance of multidisciplinary approach in the management of pregnancy with ES, which can profoundly improve maternal and infant outcomes.

Pregnancy in Eisenmenger syndrome: a case series from a tertiary care hospital of Northern India.

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.

Use of venous-venous extracorporeal membrane oxygenation in the treatment of postpartum pulmonary hypertension crisis.

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.