1,794 results match your criteria: "Eisenmenger Syndrome"

Turner syndrome is a rare chromosomal abnormality in women that is caused by a partial or complete loss of one X chromosome and is often associated with a spectrum of congenital cardiac abnormalities, including cardiac shunts.  A 27-year-old woman with Turner syndrome was also found to have right ventricular dilation, partial anomalous pulmonary venous return, and possible atrial septal defect. She was scheduled for elective surgical repair.

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Background: Pulmonary arterial hypertension (PAH) is a severe complication among adult patients with congenital heart disease (ACHD). This study presents real-world data on risk stratification, pharmacotherapy and survival rates in PAH-ACHD.

Methods: Data from PAH-ACHD patients were analyzed using The Hellenic Pulmonary Hypertension Registry (HOPE), spanning eight specialized centers between 2015 and 2023.

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Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired large systemic-to-pulmonary shunts. The long-term data of adult patients who have PAH-CHD with elevated pulmonary vascular resistance (PVR) are limited. We aimed to investigate the survival of adults who had PAH-CHD with predominantly left-to-right (L-R) shunts with (1) borderline-to-high PVR and (2) treat-and-repair compared with those with Eisenmenger syndrome (ES).

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  • The OPUS/OrPHeUS studies focused on patients with pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD), collecting data on their real-world clinical practices and treatment outcomes while starting on macitentan.
  • A total of 272 patients (6.1% of the population) were identified with CHD-PAH, where most were in moderate functional health categories, and many began macitentan as part of combination therapy; a high percentage experienced adverse events, but survival rates were relatively positive over one and two years.
  • The findings indicate that macitentan is commonly used in CHD-PAH patients, with its safety profile matching existing knowledge,
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  • * The revised 2022 guidelines from the European Society of Cardiology/European Respiratory Society address PH but only briefly consider the needs of adults with congenital heart defects.
  • * The article aims to enhance understanding of ACHD management by exploring various aspects such as diagnostics, specialized therapies, and unique circumstances, thereby addressing gaps in current guidelines.
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  • * The 2022 guidelines by the European Society of Cardiology and the European Respiratory Society address PH management but only briefly cover the specific needs of adults with congenital heart defects.
  • * This article reviews various aspects of ACHDs and PH, including their epidemiology, risk factors, and management challenges, aiming to enhance awareness and care strategies for this patient population.
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Biventricular longitudinal strain analysis using cardiovascular magnetic resonance feature-tracking: Prognostic value in Eisenmenger syndrome.

J Cardiovasc Magn Reson

December 2024

Department of Cardiology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, PR China. Electronic address:

Article Synopsis
  • A study investigated the potential of biventricular longitudinal strain analysis as a prognostic tool for patients with Eisenmenger syndrome, hypothesizing that it could help in risk stratification.
  • They enrolled 57 patients who underwent cardiovascular imaging and catheterization, tracking their health outcomes over a median of 33 months, with 35.1% experiencing significant health events like mortality or hospital readmission.
  • Results showed that lower left and right ventricular strains were linked to worse outcomes, and the addition of these strain measurements improved prognostic predictions, indicating their potential clinical value.
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  • Pregnancy in women with Eisenmenger syndrome (ES) has high risks for both mothers and babies, prompting a study on pregnancy outcomes in this population.
  • The study reviewed eight cases of pregnant women with ES, revealing two maternal deaths related to severe complications, while four others diagnosed postpartum had no fatalities.
  • Although advanced care exists, the findings indicate persistently high maternal mortality rates without clear predictors for those at risk.
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  • * A case study of a 26-year-old female revealed the coexistence of CBA with a large patent ductus arteriosus (PDA) and a small atrial septal defect (ASD), resulting in Eisenmenger syndrome.
  • * Early diagnosis and intervention for CBA and associated congenital heart defects (CHD) are crucial to preventing serious complications like pulmonary arterial hypertension.
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  • Swyer-James-MacLeod syndrome makes one lung lobe look really empty on chest scans, and it is linked to other lung problems like air trapping and less blood flow.
  • If a child has a common heart issue called a ventricular septal defect and it isn't treated, it can lead to serious lung problems like Eisenmenger syndrome.
  • Identifying Swyer-James-MacLeod syndrome early is important for giving the right treatment to help patients with unusual lung conditions.
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Eisenmenger Syndrome Combined with Pulmonary Artery Aneurysm and Dissection.

Radiology

August 2024

From the Department of Radiology, Qilu Hospital of Shandong University Dezhou Hospital (Dezhou People's Hospital), Dezhou, Shandong, China (Zihao Li); and Department of Radiology, Liaocheng Hospital Affiliated to Shandong First Medical University (Liaocheng People's Hospital), No 67 Dongchang W Rd, Dongchangfu District, Liaocheng 252000, Shandong, China (Zhao Li).

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  • Erythrocytosis is a condition where there are too many red blood cells, and doctors frequently see it in their patients.
  • There are two types: primary erythrocytosis (like polycythemia vera) and secondary erythrocytosis, which can have different causes, including issues with the heart.
  • Eisenmenger syndrome is a serious condition related to untreated heart defects that can cause erythrocytosis, but doctors need to be careful with treatments like blood removal, only using them when necessary to avoid harm.
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Giant right pulmonary artery aneurysm in Eisenmenger syndrome.

Rev Esp Cardiol (Engl Ed)

August 2024

Key Laboratory of Cardiovascular Disease of Yunnan Province, Department of Cardiology, Yan'an Affiliated Hospital of Kunming Medical University, Kunming, China. Electronic address:

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  • Atrial septal defects (ASDs) are heart problems that are more common in girls and can be tricky to diagnose, especially a specific type called sinus venosus ASDs (SVASDs).
  • People under 40 usually have a good chance of recovery with surgery, but those over 40, especially women, can face serious health issues if their ASDs go undiagnosed.
  • A case study explores a 42-year-old woman who had trouble breathing, which was wrongly linked to another issue, and it took four years to find out she had an SVASD, leading to severe complications that prevented treatment.
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Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison.

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