Real-world experiences and treatment patterns among congenital heart disease patients with associated pulmonary vascular disease: Results from a real-world survey in the United States.

Background: Limited data exist on the use of drugs in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). Therefore, we evaluated their real-world patient journey, including symptomatology, diagnostic pathway, treatment patterns, and guideline adherence.

Methods: Data were drawn from the Adelphi Real World pulmonary hypertension congenital heart disease Disease Specific Programme (DSP), a cross-sectional survey of clinicians and patients with PAH-CHD or Fontan circulation associated with elevated pulmonary vascular resistance (PVR).

CMR Findings in the Long-Term Outcomes After Multisystem Inflammatory Syndrome in Children (MUSIC) Study.

Background: Multisystem Inflammatory Syndrome in Children is characterized by high rates of acute cardiovascular involvement with rapid recovery of organ dysfunction. However, information regarding long-term sequelae is lacking. We sought to characterize the systolic function and myocardial tissue properties using cardiac magnetic resonance (CMR) imaging in a multicenter observational cohort of Multisystem Inflammatory Syndrome in Children patients.

Transcatheter thrombectomy of acute pulmonary embolism in an adult Fontan patient: a case report.

Background: The Fontan operation is a well-described palliative procedure for functional single-ventricle patients. This population has an increased risk of thrombo-embolic events. Adequate imaging, and therefore diagnosis, requires an understanding of the unique anatomy and physiology of a Fontan.

Utility of Cardiac Magnetic Resonance Imaging in Predicting Atrial Arrhythmias in Repaired Tetralogy of Fallot.

Arrhythmias are the leading cause of morbidity and mortality in repaired tetralogy of Fallot (TOF), and over 20% of these patients will develop a sustained atrial arrhythmia during their lifetimes. Cardiac magnetic resonance imaging (cMRI) is frequently performed in TOF, although its ability to identify patients at risk of atrial arrhythmias is uncertain. Adult TOF patients (n = 175) with no history of atrial arrhythmia who underwent cMRI between 2003 and 2020 at a single tertiary care center were identified.

Emergency department evaluation of chest pain among adult congenital heart disease patients.

Background: Data regarding emergency department (ED) assessment of acute chest pain (CP) and incidence of myocardial infarction (MI) among adult congenital heart disease (ACHD) patients, relative to the non-congenital population, is lacking.

Objectives: To describe MI risk in ACHD patients presenting to the ED with chest pain and to compare clinical characteristics, diagnostic testing patterns, and outcomes to controls.

Methods: We retrospectively identified a cohort of ACHD patients presenting with acute CP and matched them with non-ACHD controls at a large tertiary-level ED during the period 1998-2018.

Impact of Pulmonary Hypertension on Survival Following Device Closure of Atrial Septal Defects.

Pulmonary hypertension (PH), defined as mean pulmonary arterial pressure ≥25 mm Hg, may be a complication of a secundum atrial septal defect (ASD). This study sought to evaluate the impact of PH at time of ASD device closure on patient survival. A prospectively collected database of ASD closures was utilized.

Outcomes of lung disease-associated pulmonary hypertension and impact of elevated pulmonary vascular resistance.

Background: The clinical characteristics, hemodynamic changes and outcomes of lung disease-associated pulmonary hypertension (LD-PH) are poorly defined.

Methods: A prospective cohort of PH patients undergoing initial hemodynamic assessment was collected, from which 51 patients with LD-PH were identified. Baseline characteristics and long-term survival were compared with 83 patients with idiopathic pulmonary arterial hypertension (iPAH).

Effect of Patent Foramen Ovale in Patients With Pulmonary Hypertension.

Septostomy reduces right ventricular (RV) workload at the expense of hypoxemia in patients with advanced pulmonary hypertension (PH). A patent foramen ovale (PFO) may serve as a "natural" septostomy, but the incidence and impact of a PFO in PH remains uncertain. We prospectively examined echocardiograms in 404 PH patients referred for initial hemodynamic assessment.

Coronary Disease and Modifying Cardiovascular Risk in Adult Congenital Heart Disease Patients: Should General Guidelines Apply?

There are >1.4 million adult congenital heart disease (CHD; ACHD) patients living in the United States. Coronary artery disease (CAD) is at least as prevalent in ACHD patients as in the general population and has become a leading cause of their mortality.

Hospital Readmissions in Children with Pulmonary Hypertension: A Multi-Institutional Analysis.

Objective: To assess the rate of and risk factors for 30-day hospital readmission in children with pulmonary hypertension.

Study Design: The Pediatric Health Information System database was analyzed for patients ≤18 years old with pulmonary hypertension (International Classification of Diseases, Ninth Revision, diagnosis codes of 416.0, 416.

Characteristics of pediatric pulmonary hypertension trials registered on ClinicalTrials.gov.

The investigation of pediatric pulmonary hypertension (PH) drugs has been identified as a high priority by the United States National Institutes of Health (NIH). Studying pediatric PH is challenging due to the rare and heterogeneous nature of the disease. We sought to define the pediatric PH clinical trials landscape, to evaluate areas of trial success or failure, and to identify potential obstacles to the study of pediatric PH drugs.

Atrial ectopic tachycardia in a patient with marfan syndrome.

The fibrillin defect central to Marfan syndrome is believed to affect myocardial conduction and predispose affected patients to various arrhythmias, including ventricular tachycardia, atrial fibrillation, and atrioventricular nodal reentry tachycardia. Here we describe an adult Marfan patient with atrial ectopic tachycardia. To our knowledge, this is the first reported case of atrial ectopic tachycardia in the setting of Marfan syndrome.