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.

Anesthetic management of a parturient with Eisenmenger’s syndrome: A case report

Eisenmenger’s syndrome results from certain uncorrected congenital abnormalities of the heart facilitating left to right shunt and chronic volume overload of the pulmonary vasculature, leading to irreversible changes in the pulmonary vasculature. Pregnancy in the patients of this syndrome is associated with a high risk of cardiovascular decompensation, thromboembolic complications, and sudden cardiac death. We present the case of a 25-year-old pregnant woman with Eisenmenger’s syndrome who delivered through the vaginal route at 37 weeks of gestation, under labor epidural anesthesia. A healthy male baby was delivered within 40 min of drug deposition in the epidural space. The postpartum period was essentially uneventful with successful maternal and neonatal outcomes.

EISENMENGER'S SYNDROME AND PREGNANCY: ASPECTS OF ETIOPATHOGENESIS AND THERAPEUTIC TACTICS

In the modern world, the problem of maintaining a woman's reproductive potential and preserving the resulting pregnancy comes to the fore. An important aspect remains the increasing number of concomitant extragenital diseases. The most common pathology of the cardiovascular and respiratory systems. A rare and often fatal complication is Eisenmenger's syndrome. Eisenmenger's syndrome is a pathological process that occurs due to bidirectional blood discharge or right-left blood discharge due to the presence of a message between the right and left parts of the heart, clinically manifested by severe pulmonary hypertension with diffuse cyanosis. It occurs against the background of an existing decompensated heart defect - a defect of the atrial septum, interventricular septum, open arterial duct, anomalies of the pulmonary veins. Previously, it was believe that the threat of developing Eisenmenger syndrome is an absolute indication for termination of pregnancy, and this pathology is not compatible with gestation. Currently, the effect of pregnancy on the cardiovascular system has been study and recommendations for the management of such patients have been develop. It found that due to an increase in the volume of circulating blood, cardiac output, a decrease in total peripheral resistance and blood pressure, there is an increase in the load on the cardiovascular system and a change in the pharmacokinetics of drugs, which leads to a revision of the treatment tactics used before pregnancy. The situation is aggravated by an increase in blood clotting factors, fibrinogen, platelet aggregation and a decrease in fibrinolysis factors, which, together with the restriction of taking low-molecular-weight heparins due to the tendency of patients with Eisenmenger syndrome to bleeding and thrombosis, causes difficulties in determining the tactics of patient management, emphasizing the importance and relevance of the problem.

A CASE SERIES OF PREGNANCY IN EISENMENGER'S SYNDROME- A MANAGEMENT CONUNDRUM

Eisenmenger's syndrome(ES) refers to the development of pulmonary hypertension and reversal of shunt in patients with long standing congenital heart defects with left-to-right shunt. The cardiovascular changes in pregnancy, labour and puerperium add to the morbidity and mortality associated with this high-risk cardiac condition. Fetal outcome is also poor with increased incidence of stillbirth and prematurity. We report 3 such patients of ES with severe pulmonary hypertension. The rst patient presented in advanced pregnancy with unbooked status. She had a spontaneous preterm precipitate labour before cardiac optimization and delivered vaginally with successful outcome. The second patient presented to us in early third trimester with right heart failure, which was stabilized after caesarean section. The third patient also had a good outcome, despite additional complications of pulmonary edema. Neonatal outcome in our case series was marred by complications like fetal growth restriction, prematurity, perinatal asphyxia and one neonatal death. A multidisciplinary team approach involving obstetric medicine specialists, cardiologists, anaesthesiologists and neonatologists, is required for peripartum care of these patients to improve the maternal and perinatal outcomes.

A RARE CASE REPORT ON EISENMENGER’S SYNDROME WITH PREGNANCY : A GLIMPSE OF LIFE IN OCEAN OF DEATH

Pregnancy complicated with Eisenmenger syndrome is associated with high risk to the fetus as well as the mother. There is approximately 50% risk of sudden maternal death, frequently occuring a few days postpartum and the overall fetal wastage is reported to be up to 75%. Patients with Eisenmenger syndrome are advised to refrain from pregnancy or to terminate pregnancy by the end of rst trimester itself. Management of these patients requires a co- ordinated multi-specialist care when such pregnancies reach a stage where safe termination is not advisable. However, in spite of all the risks, a few patients deliver successfully with a good maternal and neonatal outcome. We present 2 cases reported till third trimester and delivered a healthy baby and were subsequently discharged on the 10th postpartum day without any serious complications.

EXPRESS: Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Lessons Learnt from the Large Turkish Nationwide Registry (THALES)

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1,034 patients aged 3 months–79 years (median 11.2 [Q1–Q3: 2.2–24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I–II at the time of diagnosis (46.6%). Mean 6-minute walk distance (6MWD) was 369±120 m. Mean pulmonary arterial pressure was 54.7±22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data was available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p=0.009), brain natriuretic peptide level <300 ng/L (p <0.001). Follow-up 6MWD >165 m (p <0.0001), brain natriuretic peptide level <300 ng/L (p=0.031), and targeted therapies (p=0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

Chronic rheumatic heart disease and congenital heart disease complicating pregnancy: a study of the cardiac events, the maternal and perinatal outcome during 2011-2013 at tertiary care centre

Background: Objective of this study was to assess the prevalence of chronic rheumatic heart disease and congenital heart disease complicating pregnancy, study the maternal and perinatal outcome, and indications for termination of pregnancy.Methods: Preconception counseling, antenatal care by pregnancy heart team as per protocol. One 2nd gravida (G2A1) with 26 weeks gestation, underwent mitral valve replacement during 26th week gestation i/v/o infective endocarditis associated with severe mitral regurgitation.Results: Authors had CRHD: CHD = 29:21, out of 50 cases, the ratio was 1.3:1 in this study. Atrial septal defect (ASD) was the predominant lesion in this study -29% ASD alone and 9% associated with pulmonary artery hypertension. Eisenmenger's syndrome, was associated with ASD in three and VSD in two. Corrected lesions were 24%. In the rheumatic heart disease, mitral stenosis was the predominant lesion and PBMV was done in four (13.7%) cases. In CRHD cases, surgically corrected by prosthetic heart valve were -11 (37.9%). In CRHD total corrected cases, by prosthetic heart valve and percutaneous balloon mitral valvotomy (PBMV) account for 51.7%. One patient had PBMV procedure during 5th month of present pregnancy i/v/o severe mitral stenosis with mitral valve area -0.8 cm2 and another patient had PBMV during her first pregnancy. In this study 42% were in NYHA class I. 14% were in NYHA class IV. CHF was seen in 10%. Termination of pregnancy was necessary in 6 with CHD and 5 with CRHD. There were 39 deliveries with one set of twins. All the babies were alive. Maternal mortality was confirmed in one case with Eisenmenger’s syndrome + HELLP syndrome. Live birth rate was higher in cases with NYHA class I/II than in those with NYHA class III/IV (82.8% versus 66.60%).Conclusions: Management by a pregnancy heart team as per guidelines would reduce mortality.