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Cardiovascular Diagnosis and Therapy Dec 2021Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited,...
BACKGROUND
Pulmonary arterial hypertension (PAH) is common in congenital heart disease (CHD). Because clinical-trial data on PAH associated with CHD (PAH-CHD) remain limited, registry data on the long-term course are essential. This analysis aimed to update information from the COMPERA-CHD registry on management strategies based on real-world data.
METHODS
The prospective international pulmonary hypertension registry COMPERA has since 2007 enrolled more than 10,000 patients. COMPERA-CHD is a sub-registry for patients with PAH-CHD.
RESULTS
A total of 769 patients with PAH-CHD from 62 specialized centers in 12 countries were included into COMPERA-CHD from January 2007 through September 2020. At the last follow-up in 09/2020, patients [mean age 45.3±16.8 years; 512 (66%) female] had either post-tricuspid shunts (n=359; 46.7%), pre-tricuspid shunts (n=249; 32.4%), complex CHD (n=132; 17.2%), congenital left heart or aortic valve or aortic disease (n=9; 1.3%), or miscellaneous CHD (n=20; 2.6%). The mean 6-minute walking distance was 369±121 m, and 28.2%, 56.0%, and 3.8% were in WHO functional class I/II, III or IV, respectively (12.0% unknown). Compared with the previously published COMPERA-CHD data, after 21 months of follow-up, the number of included PAH-CHD patients increased by 91 (13.4%). Within this group the number of Eisenmenger patients rose by 39 (16.3%), the number of "Non-Eisenmenger PAH" patients by 45 (26.9%). Currently, among the 674 patients from the PAH-CHD group with at least one follow-up, 450 (66.8%) received endothelin receptor antagonists (ERA), 416 (61.7%) PDE-5 inhibitors, 85 (12.6%) prostacyclin analogues, and 36 (5.3%) the sGC stimulator riociguat. While at first inclusion in the COMPERA-CHD registry, treatment was predominantly monotherapy (69.3%), this has shifted to favoring combination therapy in the current group (53%). For the first time, the nature, frequency, and treatment of significant comorbidities requiring supportive care and medication are described.
CONCLUSIONS
Analyzing "real life data" from the international COMPERA-CHD registry, we present a comprehensive overview about current management modalities and treatment concepts in PAH-CHD. There was an trend towards more aggressive treatment strategies and combination therapies. In the future, particular attention must be directed to the "Non-Eisenmenger PAH" group and to patients with complex CHD, including Fontan patients.
TRIAL REGISTRATION
www.clinicaltrials.gov, study identifier: Clinicaltrials.gov NCT01347216.
PubMed: 35070795
DOI: 10.21037/cdt-21-351 -
BMJ Case Reports Feb 2022A 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...
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.
Topics: Ciliary Arteries; Eisenmenger Complex; Fluorescein Angiography; Humans; Male; Ophthalmoscopy; Retinal Artery Occlusion
PubMed: 35228223
DOI: 10.1136/bcr-2021-246293 -
Archives of Cardiovascular Diseases May 2017The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial. (Observational Study)
Observational Study
BACKGROUND
The relationship between pulmonary arterial hypertension-specific drug therapy (PAH-SDT) and mortality in Eisenmenger syndrome (ES) is controversial.
AIMS
To investigate outcomes in patients with ES, and their relationship with PAH-SDT.
METHODS
Retrospective, observational, nationwide, multicentre cohort study.
RESULTS
We included 340 patients with ES: genetic syndrome (n=119; 35.3%); pretricuspid defect (n=75; 22.1%). Overall, 276 (81.2%) patients received PAH-SDT: monotherapy (endothelin receptor antagonist [ERA] or phosphodiesterase 5 inhibitor [PDE5I]) 46.7%; dual therapy (ERA+PDE5I) 40.9%; triple therapy (ERA+PDE5I+prostanoid) 9.1%. Median PAH-SDT duration was 5.5 years [3.0-9.1 years]. Events (death, lung or heart-lung transplantation) occurred in 95 (27.9%) patients at a median age of 40.5 years [29.4-47.6]. The cumulative occurrence of events was 16.7% [95% confidence interval 12.8-21.6%] and 46.4% [95% confidence interval 38.2-55.4%] at age 40 and 60 years, respectively. With age at evaluation or time since PAH diagnosis as time scales, cumulative occurrence of events was lower in patients taking one or two PAH-SDTs (P=0.0001 and P=0.004, respectively), with the largest differences in the post-tricuspid defect subgroup (P<0.001 and P<0.02, respectively) versus patients without PAH-SDT. By multivariable Cox analysis, with time since PAH diagnosis as time scale, New York Heart Association/World Health Organization functional class III/IV, lower peripheral arterial oxygen saturation and pretricuspid defect were associated with a higher risk of events (P=0.002, P=0.01 and P=0.04, respectively), and one or two PAH-SDTs with a lower risk of events (P=0.009).
CONCLUSIONS
Outcomes are poor in ES, but seem better with PAH-SDT. ES with pretricuspid defects has worse outcomes despite the delayed disease onset.
Topics: Adolescent; Adult; Age Factors; Antihypertensive Agents; Arterial Pressure; Cause of Death; Chi-Square Distribution; Child; Disease Progression; Disease-Free Survival; Eisenmenger Complex; Female; France; Humans; Hypertension, Pulmonary; Kaplan-Meier Estimate; Longitudinal Studies; Male; Middle Aged; Multivariate Analysis; Proportional Hazards Models; Pulmonary Artery; Retrospective Studies; Risk Factors; Time Factors; Treatment Outcome; Young Adult
PubMed: 28286190
DOI: 10.1016/j.acvd.2017.01.006 -
The Journal of Veterinary Medical... Jun 2020A seven-month-old cat was referred for evaluation of exercise intolerance and open-mouth breathing. Based on ultrasonographic examination, caudal vena cava (CVC)...
A seven-month-old cat was referred for evaluation of exercise intolerance and open-mouth breathing. Based on ultrasonographic examination, caudal vena cava (CVC) aneurysm associated with right congestive heart failure resulting from congenital heart disease was diagnosed. Conservative treatment for alleviating pulmonary hypertension mildly improved the clinical signs and decreased the heart size and CVC aneurysm diameter. However, the improvements were transient and four months after initiating therapy, the cat developed dyspnea and uncontrollable seizures and was euthanized.
Topics: Aneurysm; Animals; Cat Diseases; Cats; Echocardiography; Eisenmenger Complex; Heart Defects, Congenital; Heart Failure; Hypertension, Pulmonary; Male; Ultrasonography; Vena Cava, Inferior
PubMed: 32418943
DOI: 10.1292/jvms.19-0518 -
BMJ Case Reports May 2022Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension and is associated with congenital heart disease. ES itself is a challenging...
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.
Topics: Adult; Critical Illness; Eisenmenger Complex; Familial Primary Pulmonary Hypertension; Heart Defects, Congenital; Humans; Pulmonary Arterial Hypertension
PubMed: 35580953
DOI: 10.1136/bcr-2021-245549 -
Medicine Sep 2019A single atrium is a rare congenital heart disease (CHD) involving zero atrial septal traces and preserved intact ventricular septum and atrioventricular valves,...
RATIONALE
A single atrium is a rare congenital heart disease (CHD) involving zero atrial septal traces and preserved intact ventricular septum and atrioventricular valves, requiring careful surgical intervention. However, developing to Eisenmenger syndrome (ES) makes the surgery complicated. Based on bidirectional cardiac shunting, vegetation easily develops in case of bacterial infection.
PATIENT CONCERN AND DIAGNOSES
We reported a 35-year-old woman with a single atrium, patent ductus arteriosus, pulmonary hypertension, and ES who developed infective endocarditis on her left ventricular outflow tract and complicated cerebral abscess and who underwent challenged medical treatment.
INTERVENTION
Infection was successfully controlled after 4-time change in antibiotics over 4 months. However, surgery is complicated for her.
OUTCOMES
The patient presented a relatively good outcome during follow-up for >6 months.
LESSONS
This case report suggests that patients with complex CHD should accept surgery therapy earlier before developing ES. It is imperative to avoid invasive interventions to prevent infectious endocarditis.
Topics: Adult; Anti-Bacterial Agents; Brain Abscess; Ductus Arteriosus, Patent; Eisenmenger Complex; Endocarditis; Female; Heart Atria; Humans
PubMed: 31490396
DOI: 10.1097/MD.0000000000017044 -
Journal of the American Heart... May 2024Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However,...
BACKGROUND
Pretricuspid shunts have been associated with poorer survival rates in patients with Eisenmenger syndrome compared with postricuspid shunts and complex lesions. However, the risk stratification for persistent pulmonary hypertension (PH) in this population remains uncertain.
METHODS AND RESULTS
We retrospectively enrolled 103 patients with pretricuspid shunts with high total pulmonary resistance >4.5 Wood units (estimated pulmonary vascular resistance ≥3 Wood units). During a mean±SD follow-up of 20.95±24.84 months, 32 patients developed postoperative persistent PH after shunt correction. We identified 3 significant predictors of postoperative persistent PH, including mean pulmonary artery pressure after inhaled oxygen ≥40.5 mm Hg (odds ratio [OR], 7.78 [95% CI, 2.02-30.03]; <0.01), total pulmonary resistance after inhaled oxygen ≥6.5 Wood units (estimated pulmonary vascular resistance ≥5 Wood units; OR, 12.23 [95% CI, 2.12-70.46]; <0.01), and artery oxygen saturation at rest <95% (OR, 3.34 [95% CI, 1.07-10.44]; =0.04). We established the prediction model with the C-statistics of 0.85 (95% CI, 0.77-0.93; <0.01), and the C-statistic was 0.83 (95% CI, 0.80-0.86) after bootstrapping 10 000 times with a good performance of the nomogram calibration curve for predicting persistent PH.
CONCLUSIONS
Our study presents a multivariable risk stratification model for persistent PH after shunt correction in adults with pretricuspid shunts. This model, based on 3 hemodynamic predictors after inhaled oxygen, may assist in identifying individuals at higher risk of persistent PH after shunt correction.
Topics: Humans; Female; Male; Hypertension, Pulmonary; Retrospective Studies; Nomograms; Adult; Vascular Resistance; Risk Assessment; Pulmonary Artery; Middle Aged; Risk Factors; Predictive Value of Tests; Treatment Outcome; Cardiac Surgical Procedures; Heart Defects, Congenital; Arterial Pressure
PubMed: 38639332
DOI: 10.1161/JAHA.123.032412 -
Multidisciplinary approach for the management of term pregnancy complicated by Eisenmenger syndrome.Journal of Zhejiang University.... Jan 2023Pregnancy 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...
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.
Topics: Female; Humans; Pregnancy; Cesarean Section; Eisenmenger Complex; Hypertension, Pulmonary; Maternal Mortality; Pregnancy Complications, Cardiovascular; Pregnancy Outcome
PubMed: 36632753
DOI: 10.1631/jzus.B2200368 -
Open Heart Apr 2021Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on...
OBJECTIVE
Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies.
METHODS
This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios.
RESULTS
82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios.
CONCLUSIONS
Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.
Topics: COVID-19; Eisenmenger Complex; Europe; Fontan Procedure; Global Burden of Disease; Heart Defects, Congenital; Humans; Outcome Assessment, Health Care; Prognosis; Pulmonary Arterial Hypertension; Risk Assessment; Risk Factors; SARS-CoV-2; Societies, Medical; Surveys and Questionnaires
PubMed: 33883228
DOI: 10.1136/openhrt-2020-001455 -
Journal of Cardiac Surgery Oct 2019Dextrocardia is a rare congenital condition which presents important challenges for surgical management. We discuss a patient with dextrocardia, atrial septal defect,...
Management of a young patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome with venous-venous extracorporeal membrane oxygenation and heart-lung transplantation.
Dextrocardia is a rare congenital condition which presents important challenges for surgical management. We discuss a patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome, which ultimately led to decompensated end-stage lung disease and heart-lung transplant. Venous-venous extracorporeal membrane oxygenation was an important strategy to bridge the patient until donor organs became available. Transplantation of a heart-lung block allowed for the treatment of the patient's underlying congenital heart defect, anatomic reversal of dextrocardia with appropriate venous and arterial connections, and management of pulmonary damage from pulmonary hypertension.
Topics: Abnormalities, Multiple; Adult; Dextrocardia; Eisenmenger Complex; Extracorporeal Membrane Oxygenation; Female; Heart Septal Defects, Atrial; Heart-Lung Transplantation; Humans; Radiography, Thoracic; Tomography, X-Ray Computed
PubMed: 31332841
DOI: 10.1111/jocs.14168