Survival prospects of pulmonary arterial hypertension associated with congenital heart disease: Insights from the HOPE registry.

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.

Desaturation after Fontan procedure due to systemic venous collateral and improvement after closure with embolization.

Key Clinical Message: Systemic to pulmonary venous collaterals (SPVCs) are common findings in patients with Fontan and may be responsible for desaturation in this group of patients. In selected cases, percutaneous closure of SPVCs with coils or vascular plugs could be offered as a safe and effective treatment modality for these patients.

Abstract: A 27-year-old female patient who had operated primarily with Glenn operation and she underwent later a lateral tunnel Fontan operation.

Left ventricular structural integrity on tetralogy of Fallot patients: approach using longitudinal relaxation time mapping.

Purpose: Tetralogy of Fallot (TOF) is a congenital heart disease, and patients undergo surgical repair early in their lives. The evaluation of TOF patients is continuous through their adulthood. The use of cardiac magnetic resonance imaging (CMR) is vital for the evaluation of TOF patients.

Haemolytic Anaemia-Related Pulmonary Hypertension.

Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH, and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassaemia and 14.

Use of apixaban in adults with congenital heart disease and atrial arrhythmias: The PROTECT-AR study.

Background: Adults with congenital heart disease (ACHD) and atrial arrhythmias (AA) face an increased risk of thromboembolic events. Limited data exist on the use of non-vitamin K oral anticoagulants for thromboprophylaxis in ACHD. We aimed to assess the effectiveness and safety of apixaban in ACHD patients with AA.