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Heart (British Cardiac Society) Feb 2024This study assessed the long-term effects of triple therapy with prostanoids on patients with 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.
Topics: Humans; Female; Adult; Male; Pulmonary Arterial Hypertension; Eisenmenger Complex; Hypertension, Pulmonary; Vasodilator Agents; Retrospective Studies; Heart Defects, Congenital; Familial Primary Pulmonary Hypertension; Prostaglandins
PubMed: 37903556
DOI: 10.1136/heartjnl-2023-323015 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Oct 2023Pulmonary arterial hypertension (PAH) is a profoundly destructive condition marked by the gradual narrowing and restructuring of small pulmonary arteries, leading to a...
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.
Topics: Humans; Eisenmenger Complex; Pulmonary Artery; Coronary Vessels; Coronary Stenosis; Tomography, X-Ray Computed; Coronary Angiography; Angioplasty, Balloon, Coronary; Aneurysm; Stents; Ultrasonography, Interventional
PubMed: 37861262
DOI: 10.5543/tkda.2023.56585 -
West African Journal of Medicine Sep 2023Eisenmenger syndrome (ES) is a rare condition seen in children with congenital heart disease (CHD). It is characterized by raised pulmonary vascular resistance (PVR)... (Review)
Review Meta-Analysis
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.
Topics: Child; Humans; Eisenmenger Complex; Hypertension, Pulmonary; Syndrome; Echocardiography
PubMed: 37768185
DOI: No ID Found -
Expert Review of Cardiovascular Therapy 2023Congenital heart disease (CHD) is the most common cardiac disorder in pregnancy in the western world (around 80%). Due to improvements in surgical interventions more... (Review)
Review
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.
Topics: Adult; Child; Pregnancy; Female; Humans; Risk Factors; Pregnancy Complications, Cardiovascular; Heart Defects, Congenital; Eisenmenger Complex; Prognosis
PubMed: 37470417
DOI: 10.1080/14779072.2023.2237886 -
Journal of Cardiothoracic Surgery Jul 2023Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between the main pulmonary artery and ascending aorta. There are various...
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.
Topics: Female; Humans; Adult; Eisenmenger Complex; Aneurysm, False; Thoracic Surgical Procedures; Lung Transplantation; Plastic Surgery Procedures
PubMed: 37393254
DOI: 10.1186/s13019-023-02305-2 -
European Heart Journal Jun 2023
Topics: Humans; Eisenmenger Complex; Pulmonary Artery; Lung; Pulmonary Circulation; Aneurysm; Aortic Dissection
PubMed: 37140033
DOI: 10.1093/eurheartj/ehad258 -
BJOG : An International Journal of... Sep 2023Maternal-fetal morbidity and mortality among pregnant women with pulmonary artery hypertension (PAH) and Eisenmenger syndrome are unacceptable, and management... (Observational Study)
Observational 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.
Topics: Infant, Newborn; Female; Pregnancy; Humans; Eisenmenger Complex; Pulmonary Artery; Prospective Studies; Cesarean Section; Hypertension, Pulmonary; Pregnancy Outcome; Heart Failure
PubMed: 37039249
DOI: 10.1111/1471-0528.17474 -
The Journal of Heart and Lung... Jul 2023The purpose of this registry was to provide insights into the characteristics, treatments and survival of patients with PAH-CHD in China.
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/cm, 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 ≤ 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.
Topics: Female; Humans; Adult; Male; Pulmonary Arterial Hypertension; Eisenmenger Complex; Hypertension, Pulmonary; Heart Defects, Congenital; Familial Primary Pulmonary Hypertension; Registries
PubMed: 37002152
DOI: 10.1016/j.healun.2023.02.1494 -
European Journal of Preventive... Sep 2023Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal...
AIMS
Previous studies in adult congenital heart disease (CHD) have demonstrated a link between renal dysfunction and mortality. However, the prognostic significance of renal dysfunction in CHD and decompensated heart failure (HF) remains unclear. We sought to assess the association between renal dysfunction and outcomes in adults with CHD presenting to our centre with acute HF between 2010 and 2021.
METHODS AND RESULTS
This retrospective analysis focused on the association between renal dysfunction, pre-existing and on admission, and outcomes during and after the index hospitalization. Chronic kidney disease (CKD) was defined as an estimated glomerular filtration rate <60 mL/min/1.73 m2. Cox regression analysis was used to identify the predictors of death post-discharge. In total, 176 HF admissions were included (mean age 47.7 ± 14.5 years, 43.2% females). One-half of patients had a CHD of great complexity, 22.2% had a systemic right ventricle, and 18.8% had Eisenmenger syndrome. Chronic kidney disease was present in one-quarter of patients. The median length of intravenous diuretic therapy was 7 (4-12) days, with a maximum dose of 120 (80-160) mg furosemide equivalents/day, and 15.3% required inotropic support. The in-hospital mortality rate was 4.5%. The 1- and 5-year survival rates free of transplant or ventricular assist device (VAD) post-discharge were 75.4% [95% confidence interval (CI): 69.2-82.3%] and 43.3% (95% CI: 36-52%), respectively. On multivariable Cox analysis, CKD was the strongest predictor of mortality or transplantation/VAD. Highly complex CHD and inpatient requirement of inotropes also remained predictive of an adverse outcome.
CONCLUSION
Adult patients with CHD admitted with acute HF are a high-risk cohort. CKD is common and triples the risk of death/transplantation/VAD. An expert multidisciplinary approach is essential for optimizing outcomes.
Topics: Female; Humans; Adult; Middle Aged; Male; Heart Defects, Congenital; Retrospective Studies; Aftercare; Patient Discharge; Heart Failure; Renal Insufficiency, Chronic
PubMed: 36974357
DOI: 10.1093/eurjpc/zwad094 -
Hellenic Journal of Cardiology : HJC =... 2023Data 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...
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.
Topics: Humans; Eisenmenger Complex; Retrospective Studies; Prognosis; Heart Septal Defects, Ventricular; Heart Septal Defects, Atrial
PubMed: 36924996
DOI: 10.1016/j.hjc.2023.03.004