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| http://dx.doi.org/10.14797/mdcj-16-2-168 | DOI Listing |
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Intraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome.
Case Rep Cardiol
December 2024
Department of Anesthesiology and Pain Management, Cleveland Clinic, Cleveland, Ohio, USA.
Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome.
View Article and Find Full Text PDFPulmonary arterial hypertension with left to right shunts: When to treat and/or close?
Int J Cardiol Congenit Heart Dis
September 2024
Royal Brompton Hospital, Part of Guys St Thomas NHS Trust, and National Heart and Lung Institute, Imperial College London, London, United Kingdom.
Pulmonary arterial hypertension (PAH) is defined as increase in mean pulmonary arterial pressure and pulmonary vascular resistance (PVR). It can be associated with congenital heart disease (CHD) with the following subtypes: 1) uncorrected left-to-right (L-R) intracardiac shunt leading to overload of the pulmonary circulation and a progressive increase of PVR; 2) Eisenmenger syndrome, appearing when a large post-tricuspid shunt is left uncorrected and pulmonary vascular disease (PVD) is severe, so the shunt becomes bidirectional or right-to-left, causing cyanosis; 3) PAH after shunt closure, when PVR arises after a defect correction; and 4) PAH associated with small or coincidental defects. While the treatment of patients with Eisenmenger syndrome is well established, the treatment of patients with PAH in whom there is a L-R shunt (with no cyanosis) remains unclear and requires expertise.
View Article and Find Full Text PDFUpdate on Eisenmenger syndrome - Review of pathophysiology and recent progress in risk assessment and management.
Int J Cardiol Congenit Heart Dis
September 2024
The Cincinnati Adult Congenital Heart Disease Program, Heart Institute, Cincinnati Children's Hospital, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Longstanding left-to-right shunting associated with congenital heart disease (CHD) can ultimately lead to pulmonary vascular remodeling, pulmonary arterial hypertension, and shunt reversal, the hallmark feature of Eisenmenger Syndrome (ES). ES is a multisystem disease, with hematologic, cardiovascular, renal, neurologic, immune, and other manifestations, each of which inform its management. Many of the most distinct and clinically important consequences relate to chronic hypoxemia.
View Article and Find Full Text PDFTurner 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.
View Article and Find Full Text PDFSurvival prospects of pulmonary arterial hypertension associated with congenital heart disease: Insights from the HOPE registry.
Int J Cardiol
December 2024
AHEPA University General Hospital, Pulmonary Hypertension and Congenital Heart Disease Unit, Thessaloniki, Greece. Electronic address:
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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