Eisenmenger Syndrome Research Articles

Thrombosis of aneurysmal pulmonary arteries in patent ductus arteriosus with Eisenmenger syndrome.

ABSTRACTA 45-year-old lady with patent ductus arteriosus with Eisenmenger's syndrome had presented with hemoptysis. Computed tomography revealed aneurysmally dilated pulmonary arteries with a large calcified organized thrombus.

An Unusual Tricuspid Annular Mass in an Adult With Eisenmenger Syndrome Secondary to Uncorrected Aorto-Pulmonary Window

We report a case of a tricuspid annular mass in a 55-year-old man with Eisenmenger syndrome (ES) secondary to uncorrected aorto-pulmonary window (APW). The patient’s congenital heart disease was diagnosed in his late teens. He had stable NYHA II symptoms in subsequent decades. In 2017, aged 50 years, he presented for routine review. TTE demonstrated dilated RV with marked hypertrophy and mildly impaired contractility, elevated pulmonary pressures (RV-RA 81mmHg), and large APW (4.6cm antero-posterior).

Pregnancy with congenital heart disease

Pregnancy is complicated by maternal cardiovascular disease in 1%-4% of cases. With advances in management of congenital heart diseases (CHDs), the survival to adulthood and childbearing age is increasing all over the world. The physiological adaptation during pregnancy adds to the hemodynamic burden of CHD, and, hence, many women are diagnosed with CHD for the first time during pregnancy, more so in developing countries. The type of underlying CHD and pre-pregnancy hemodynamics determine the risk of developing complications during pregnancy. Hence, pre-pregnancy risk stratification and counseling are a crucial part of management plan. Some of the serious CHDs are best treated in the preconception stage. The maximum chance of developing complications is between 28 and 32 weeks of gestation, during labor, and up to two weeks after delivery. Common complications in women with CHD during pregnancy and labor include heart failure, arrhythmias, bleeding/thrombosis, infective endocarditis, and rarely maternal death. Fetal complications include abortion, stillbirth, prematurity, low birth weight, and CHD. Comprehensive knowledge of these complications and their management is very important as an experienced multidisciplinary team is critical for improving outcome of these patients. Special care is required for pregnant women who have pulmonary hypertension, due to either Eisenmenger syndrome or other causes, severe valve stenosis, aortopathy associated with bicuspid aortic valve/coarctation, or severe cyanotic CHDs. Most women with CHD are at low risk, and successful pregnancy is feasible in the majority with optimal management.

Cardiovascular Diseases Associated with Pregnancy: An Overview

Valvular heart disease is the most common cardiac problem complicating pregnancy, and pregnancy in most women with heart disease has a favourable maternal and fetal outcome. With the exception of patients with Eisenmenger syndrome, pulmonary vascular obstructive disease, and Marfan syndrome with aortopathy, maternal death during pregnancy in women with heart disease is rare. However, pregnant women with heart disease do remain at risk for other complications including heart failure, arrhythmia, and stroke. Women with congenital heart disease now comprise the majority of pregnant women with heart disease seen at referral centres. The next largest group includes women with rheumatic heart disease. Peripartum cardiomyopathy, though infrequent, will be discussed in view of its unique relation to pregnancy. Two groups of conditions not discussed further are coronary artery disease which is infrequently encountered, and isolated mitral valve prolapse, which generally has an excellent outcome. Hormonally mediated increases in blood volume, red cell mass, and heart rate result in a major increase in cardiac output during pregnancy; cardiac output peaks during the second trimester, and remains constant until term. Gestational hormones, circulating prostaglandins, and the low resistance vascular bed in the placenta result in concomitant decreases in peripheral vascular resistance and blood pressure. During labour and delivery, pain and uterine contractions result in additional increases in cardiac output and blood pressure. Immediately following delivery, relief of caval compression and autotransfusion from the emptied and contracted uterus produce a further increase in cardiac output. Most haemodynamic changes of pregnancy resolve by two weeks postpartum.

Anesthetic Management in Patient with Suspected Brain Abscess, Atrium Septal Defect, and Eisenmenger Syndrome

Introduction: Patients with congenital heart disease especially with systemic shunting between systemic and pulmonary circulation often develop pulmonary hypertension and left-to-right shunt (Eisenmenger syndrome) if left untreated. These patients are at risk of developing spontaneous brain abscess due to brain infarction caused by polycythemia, impaired immune function, and loss of lung phagocytosis. Such patients were often admitted to the emergency room with signs of increased intracranial pressure (ICP), and needed specific consideration during surgery. Case: a 31-year old female diagnosed with intracranial space occupying lesion (SOL) due to suspected brain abscess with concurrent heart defects (atrial septal defect / ASD and Eisenmenger syndrome) was consulted to the operating theatre for emergency burrhole aspiration. The surgery was performed for an hour and the postoperatively the patient was admitted to the intensive care unit (ICU). Conclusion: perioperative management of patients with brain abscess and concurrent ASD and Eisenmenger syndrome consists of preoperative management, methods of anesthesia, monitoring, and interventions to prevent the worsening of left-to-right shunt and increasing intracranial pressure. These managements consist of optimal pain management, perioperative oxygen therapy, and prevention of precipitating factor that increases left-to-right shunting.

A Review on Eisenmenger Syndrome: Pathophysiology, Investigations, and Treatment

A Review on Eisenmenger Syndrome: Pathophysiology, Investigations, and Treatment

Characteristics Of Pulmonary Arterial Hypertension in Children with Acyanotic Congenital Heart Disease

Background: Congenital heart disease is the leading cause of death in infants related to birth defects and can cause chronic defects (1). The most common complication is pulmonary hypertension (PH). The worst manifestation of PAH is Eisenmenger syndrome. If it has manifested into Eisenmenger syndrome, the defect in CHD cannot be corrected (2,3). Research on the characteristics of PAH in CHD needs to be known for better treatment. Methods: This is a descriptive study in pediatric patients with acyanotic congenital heart disease. In this research we used medical records. Data analysis was carried out descriptively. Results: The results of this study showed that the prevalence of PAH was 7.08%, and most of the patients were toddlers (33 patients or 57.9%) and female (38 patients of 66.7%). The most common diagnosis of CHD lesions was ASD, found in 19 patients (33.3%). Most patients came with severe PAH conditions. Pharmacological therapy in the form of sildenafil was mostly given. The most common symptoms were shortness of breath and murmurs on physical examination. There was no relationship between intervention variables, pharmacological therapy, and mortality and between diagnosis of CHD and severity of PAH. Conclusion: Although the prevalence of PAH was not high in this study, the mortality rate was quite high. It is necessary to educate the parents of patients with congenital heart disease so that further complications can be prevented early on. It is also necessary to make services better to improve the nutritional status of patients. Pharmacological therapy and intervention measures need to be reviewed and improved to reduce mortality in patients.

Case Report Onventricular Septal Defect (VSD) with Pulmonary Hypertention (Eisenmenger Syndrome) with LSCS

Background: A ventricular septal defect is a defect of the ventricular septal wall where the abnormal opening is present in between the two ventricles. Eisenmenger syndrome is a condition it may develop due to unrepaired heart defect and complication of unrepaired heart defect who is born congenitally. The congenital heart defects lead to Eisenmenger syndrome it developed due to abnormal blood circulate throughout the heart and lungs.
 Case Report: The 23-year-old female was admitted to AVBRH with the chief complaints of a history of amenorrhoea since 8 months, complaints of breathlessness during exertion since the 7th month of pregnancy and she was referred to AVBRH from Amravati on date 27/1/2021. At the age of 14 years, she had a history of cyanosis at the time of fever and had recurrent episodes of fever for 2-3 yrs, lasting for 2-3 weeks. She is primigravida and had undergone LSCS on date 3/2/2021. She delivered a male child on date 3/2/2021 of birth weight 1.9kg at 12:15 pm.
 Discussion: Patient with pulmonary hypertension and Eisenmenger syndrome where the surgery is contraindicated because in Eisenmenger syndrome surgery is life-threatening. Only medical management is available to treat Eisenmenger syndrome.
 Conclusion: Ventricular septal defect in adult females where the pregnancy is contraindicated. Epidural anesthesia should be preferred while the patient undergoes the lower segment cesarean section. This case report concludes that in this condition emergency LSCS is the first choice of treatment and oxygen therapy.

Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients

Having discussed current definitions and classification of pulmonary hypertension associated with congenital heart defects, the authors consider characteristic clinical symptoms and data of physical examination separately for every subgroup. An increase in pulmonary vascular resistance after radical correction and with small / concomitant defects leads to insufficient filling of the systemic ventricle and the progression of symptoms of low cardiac output , i.e. shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. At late stages, due to transient systemic hypotension the patients feel dizziness, lightheadedness and fainting, as well as signs of congestive right ventricular failure – peripheral edema, liver enlargement, ascites and swelling of the cervical veins. The patients with Eisenmenger syndrome develop cyanosis and signs of systemic complications – polycythemia, deformation of the distal phalanges of the fingers like «drumsticks» and «watch glasses», posture disturbance due to osteoarthropathy and scoliosis, pulmonary and paradoxical systemic thrombosis and embolism, bleeding, symptoms of gout and cholelithiasis, impaired renal function. Even mild pulmonary hypertension after Fontaine’s surgery causes venous hypertension with congestive heart failure, hydrothorax, protein deficiency enteropathy and plastic bronchitis, as well as insufficient blood flow to the systemic ventricle with low cardiac output, shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. All forms of pulmonary hypertension cause pain in the region of the heart, hemoptysis, pulmonary hemorrhage, arrhythmias, and developmental delay in children. The patient shall be examined «from head to toe.» The diagnostic value of auscultation depends on the complexity of the congenital malformation leading to pulmonary hypertension Pulse oximetry should be performed separately on the arms and legs at rest and exercise.

The use of pulmonary arterial hypertension therapies in Eisenmenger syndrome

ABSTRACT 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.

The Heroic Chamber – an Outlook on the Right Ventricle in Eisenmenger Syndrome

Abstract Despite being at the extreme spectrum of congenital heart disease-associated pulmonary hypertension, patients with Eisenmenger syndrome have better outcomes compared to other types of pulmonary arterial hypertension, especially in the case of post-tricuspid shunts. This survival advantage seems to be at least partly due to significant resilience of the right ventricle and a relative resistance to failure. This paper aims to review the concept of right ventricular adaptive remodeling in Eisenmenger syndrome, its impact on prognosis and the role of multimodality imaging in the right ventricle's assessment in this setting.

Effect of oxygen therapy on exercise performance in patients with cyanotic congenital heart disease: Randomized-controlled trial

BackgroundPatients with unrepaired cyanotic congenital heart disease (CHD) suffer from aggravated hypoxemia during exercise. We tested the hypothesis that supplemental oxygen improves exercise performance in these patients. MethodsIn this randomized, sham-controlled, single-blind, cross-over trial cyanotic CHD-patients underwent four cycle exercise tests to exhaustion, while breathing either oxygen-enriched (FiO2 0.50, oxygen) or ambient air (FiO2 0.21, air) using incremental (IET) or constant work-rate (CWRET) exercise test protocols (75% of maximal work rate achieved under FiO2 0.21). Pulmonary gas-exchange, electrocardiogram, arterial blood gases, oxygen saturation (SpO2), cerebral and quadriceps muscle tissue oxygenation (CTO and QMTO) by near-infrared spectroscopy were measured. ResultsWe included seven patients with cyanotic CHD (4 Eisenmenger syndrome, 3 unrepaired cyanotic defects, 4 women) median (quartiles) age 36 (32;50) years, BMI 23 (20;26) kg/m2 and SpO2 at rest 87 (83;89) %. When comparing supplemental oxygen with air during exercise, maximal work-rate in IET increased from 76 (58;114) Watts to 83 (67;136) Watts, median difference 9 (0;22) W (p = 0.046) and CWRET-time increased from 412 s (325;490) to 468 s (415;553), median increase 56 (39;126) s (p = 0.018). In both IET and CWRET SpO2 was significantly higher and ventilatory equivalent for carbon dioxide significantly lower at end-exercise with oxygen compared to air, whereas CTO and QMTO did not significantly differ. ConclusionsPatients with cyanotic CHD significantly improved their exercise performance, in terms of maximal work-rate and endurance time along with an improved arterial oxygenation and ventilatory efficiency with supplemental oxygen compared to air.

Myocardial fibrosis and its correlation with the predictors of morbidity and mortality in Eisenmenger syndrome

Myocardial fibrosis and its correlation with the predictors of morbidity and mortality in Eisenmenger syndrome

C43. Eisenmenger Syndrome The Challenge of Untreated Large Ventricular Septal Defect in Pregnant Women: A Case report

Abstract Background Eisenmenger syndrome is a constellation of symptoms that arise from an uncorrected large congenital heart disease that have a reversed shunt to right to left. Eisenmenger syndrome in pregnancy is rare because pregnancy in this patient is not recommendeed due to high mortality for mother and baby. The objective of this case report is to highlight the argument of Eisenmenger syndrome in pregnancy. Case Summary A 38-years pregnant woman presented to the emergency room with dyspnea since the third trimester. Reported history of the untreated ventricular septal defect. Central cyanosis, systolic murmur and lower extremity oedema were found. ECG showed a right axis deviation with right ventricular hypertrophy. Cardiomegaly was found from a chest x-ray. Left ventricular diastolic dysfunction, large ventricular septal defect with Right-Left shunt, and pulmonary hypertension were found from echocardiography. Based on characteristic appearance, a diagnosis of Eisenmenger syndrome was made. Caesarean delivery was performed and the baby delivered safely, but the mother passed away. Discussion Eisenmenger syndrome in pregnancy comes with high mortality to mother and baby. A ventricular septal defect is a common cause of Eisenmenger syndrome. The choice for medical treatment from Eisenmenger syndrome in pregnancy is still controversial. Both caesarean section and vaginal delivery could be harmful to the patient due to vulnerability to hemodynamic change.

C42. Eisenmenger Syndrome, Could Its Pathophysiological Changes Serve as Protective Factors Against Severe COVID-19 Infection?

BackgroundCongenital heart disease (CHD) and pulmonary artery hypertension (PAH) are comorbidities to severe COVID-19 infection. We reported a case of adult congenital heart disease (ACHD) with Eisenmenger Syndrome (ES) who survived COVID-19 without progression to severe infection.Case DescriptionA 39 years old male with history of uncorrected ACHD complained of low-grade fever and cough for 3 days. Initial oxygen saturation was 85%, no tachypnoea, no tachycardia, and blood pressure was normal. SARS-CoV2 RT-PCR results was positive. Thorax CT showed lung fibrosis, minimal GGO, and prominent pulmonary notch. Notable laboratory result was haemoglobin: 20.4g/dL. Echocardiography showed a large secundum ASD with bidirectional shunt, dilated RA and RV, normal ventricular systolic functions, mild TR with increased tricuspid gradient (TR V max 4.27m/s) which concluded the diagnosis of ES. The patient was treated according to moderate COVID-19 guidelines and ES management. His response to treatment was favourable and was discharged in 3 weeks uneventfully.DiscussionStudies have documented CHD and PAH as comorbid to severe COVID-19 infection, but our patient had moderate COVID-19 infection and recovered without progression to severe disease. We proposed several pathophysiological characteristics of ES which could serve as protective factors against severe COVID-19, which are: 1) Pre-existing PAH, where pulmonary vasculopathy and remodelling lessened endothelial response to inflammation, and reduced expression of ACE2 receptors, 2) Chronic hypoxia, which prepared cells and tissues against hypoxic environment, and 3) Polycythaemia, might be beneficial in haemoglobinopathy directly caused by SARS-CoV2.

C43. Eisenmenger Syndrome The Challenge of Untreated Large Ventricular Septal Defect in Pregnant Women: A Case report

Abstract Background Eisenmenger syndrome is a constellation of symptoms that arise from an uncorrected large congenital heart disease that have a reversed shunt to right to left. Eisenmenger syndrome in pregnancy is rare because pregnancy in this patient is not recommendeed due to high mortality for mother and baby. The objective of this case report is to highlight the argument of Eisenmenger syndrome in pregnancy. Case Summary A 38-years pregnant woman presented to the emergency room with dyspnea since the third trimester. Reported history of the untreated ventricular septal defect. Central cyanosis, systolic murmur and lower extremity oedema were found. ECG showed a right axis deviation with right ventricular hypertrophy. Cardiomegaly was found from a chest x-ray. Left ventricular diastolic dysfunction, large ventricular septal defect with Right-Left shunt, and pulmonary hypertension were found from echocardiography. Based on characteristic appearance, a diagnosis of Eisenmenger syndrome was made. Caesarean delivery was performed and the baby delivered safely, but the mother passed away. Discussion Eisenmenger syndrome in pregnancy comes with high mortality to mother and baby. A ventricular septal defect is a common cause of Eisenmenger syndrome. The choice for medical treatment from Eisenmenger syndrome in pregnancy is still controversial. Both caesarean section and vaginal delivery could be harmful to the patient due to vulnerability to hemodynamic change.

C42. Eisenmenger Syndrome, Could Its Pathophysiological Changes Serve as Protective Factors Against Severe COVID-19 Infection?

BackgroundCongenital heart disease (CHD) and pulmonary artery hypertension (PAH) are comorbidities to severe COVID-19 infection. We reported a case of adult congenital heart disease (ACHD) with Eisenmenger Syndrome (ES) who survived COVID-19 without progression to severe infection.Case DescriptionA 39 years old male with history of uncorrected ACHD complained of low-grade fever and cough for 3 days. Initial oxygen saturation was 85%, no tachypnoea, no tachycardia, and blood pressure was normal. SARS-CoV2 RT-PCR results was positive. Thorax CT showed lung fibrosis, minimal GGO, and prominent pulmonary notch. Notable laboratory result was haemoglobin: 20.4g/dL. Echocardiography showed a large secundum ASD with bidirectional shunt, dilated RA and RV, normal ventricular systolic functions, mild TR with increased tricuspid gradient (TR V max 4.27m/s) which concluded the diagnosis of ES. The patient was treated according to moderate COVID-19 guidelines and ES management. His response to treatment was favourable and was discharged in 3 weeks uneventfully.DiscussionStudies have documented CHD and PAH as comorbid to severe COVID-19 infection, but our patient had moderate COVID-19 infection and recovered without progression to severe disease. We proposed several pathophysiological characteristics of ES which could serve as protective factors against severe COVID-19, which are: 1) Pre-existing PAH, where pulmonary vasculopathy and remodelling lessened endothelial response to inflammation, and reduced expression of ACE2 receptors, 2) Chronic hypoxia, which prepared cells and tissues against hypoxic environment, and 3) Polycythaemia, might be beneficial in haemoglobinopathy directly caused by SARS-CoV2.

C38. Diagnosis and Management of Atrial Septal Defects (ASD) and Pulmonary Hypertension (PH) in Pregnancy

Abstract Background Atrial Septal Defect (ASD) is a congenital heart disease that is often found in adulthood, including women in pregnancy. Women with ASD are at greater risk for cardiovascular complications during pregnancy especially with Pulmonary Hypertension (PH), so comprehensive management is essential for this case. Case Summary A 31-year-old woman with gravida 32-33 weeks complaint of shortness of breath increasing since 3 days before entering the hospital with central cyanosis, clubbing finger, peripheral oxygen saturation 74%, from auscultation showed hardened P2 sound, indistinct wide fixed split, grade 3/6 systolic murmur. Echocardiography showed ASD with High Probability PH (Pulmonary Hypertension). The patient treated with furosemide 2x20 mg, sildenafil 3x12.5 mg, 3x20 mcg berraprost. The pregnancy was electively terminated with cesarean delivery and also got tubectomy. After 18 days of treatment in hospital, patient was discharged with stable and good condition. The patient was planned for right heart catheterization from outpatient clinic. Discussion Based on Guidline ESC on the management of Cardiovascular disease during Pregnancy in 2011, patients with ASD-PH are included in class IV WHO classification, where are contraindicated for pregnancy. If the pregnancy continues, recommended for patients to control regularly to the cardiologists and obstetricians during pregnancy, delivery and the puerperium. From literature, cesarean delivery is recommended for pregnant women with Pulmonary Hypertension (including Eisenmenger's Syndrome). Special attention and comprehensive management are required for pregnant patients with ASD-PH. The choice of intervention method and some medical therapy in this patient must also be considered.

C38. Diagnosis and Management of Atrial Septal Defects (ASD) and Pulmonary Hypertension (PH) in Pregnancy

Abstract Background Atrial Septal Defect (ASD) is a congenital heart disease that is often found in adulthood, including women in pregnancy. Women with ASD are at greater risk for cardiovascular complications during pregnancy especially with Pulmonary Hypertension (PH), so comprehensive management is essential for this case. Case Summary A 31-year-old woman with gravida 32-33 weeks complaint of shortness of breath increasing since 3 days before entering the hospital with central cyanosis, clubbing finger, peripheral oxygen saturation 74%, from auscultation showed hardened P2 sound, indistinct wide fixed split, grade 3/6 systolic murmur. Echocardiography showed ASD with High Probability PH (Pulmonary Hypertension). The patient treated with furosemide 2x20 mg, sildenafil 3x12.5 mg, 3x20 mcg berraprost. The pregnancy was electively terminated with cesarean delivery and also got tubectomy. After 18 days of treatment in hospital, patient was discharged with stable and good condition. The patient was planned for right heart catheterization from outpatient clinic. Discussion Based on Guidline ESC on the management of Cardiovascular disease during Pregnancy in 2011, patients with ASD-PH are included in class IV WHO classification, where are contraindicated for pregnancy. If the pregnancy continues, recommended for patients to control regularly to the cardiologists and obstetricians during pregnancy, delivery and the puerperium. From literature, cesarean delivery is recommended for pregnant women with Pulmonary Hypertension (including Eisenmenger's Syndrome). Special attention and comprehensive management are required for pregnant patients with ASD-PH. The choice of intervention method and some medical therapy in this patient must also be considered.

C80. Pulmonary Embolism in Congenital Heart Disease with Pulmonary Arterial Hypertension: a Case Series

Abstract Background Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease (CHD), notably in patients with left-to-right shunts. Patients with severe PAH due to CHD, Eisenmenger syndrome (ES), represent a high-risk group of pulmonary artery (PA) thrombosis patients. We present serial adult cases of CHD with PAH complicated by pulmonary embolism (PE). Case Description We found 3 cases of pulmonary embolism on adult congenital heart disease in our center. All of the patients were female, with an age range of 36 to 61 years old. The presenting symptoms were shortness of breath, WHO classification class II-III. All patients were hemodynamically stable. CHD and estimated RV systolic pressure were evaluated with echocardiography. CT-PA was performed in all patients, which findings were supporting PE. All patients were treated with anticoagulation. After three months, right heart catheterization was conducted in two patients, and one patient was refused. Discussion The prevalence of PE among adult patient with CHD and PAH range from 17 to 21%. PE was frequently found in older patients, women, and patients with lower oxygen saturation. To date, no uniformity regarding routine prophylaxis anticoagulation therapy in ES due to the high bleeding risk in this population despite being at risk of PA thrombosis. We conclude that risk stratification for PA thrombosis in adult CHD-PAH patients is essential, and further research is needed to prevent PE in this population.