Bilateral Harmony™ valve placement in branch pulmonary arteries.

While newer self-expanding pulmonic valves were primarily designed for larger right ventricular outflow tracks, there are instances where even larger anatomies cannot accommodate these devices. In this report, we describe the successful implantation of two Harmony™ valves in bilateral branch pulmonary arteries after exhausting other options.

Participation in Competitive Sports by Patients With Congenital Heart Disease: AHA/ACC and EAPC/ESC/AEPC Guidelines Comparison.

Sports participation in patients with congenital heart disease is an evolving subject. The American Heart Association/American College of Cardiology released a set of guidelines that advise the type and level of sports participation based primarily on anatomical defects with secondary consideration given to hemodynamic effects. Recently, the European Association of Preventive Cardiology/European Society of Cardiology/Association for European Paediatric and Congenital Cardiology offered a contrasting approach to sports participation that is based on hemodynamic and electrophysiological profiles of each patient, regardless of anatomical consideration.

Five decades of Fontan palliation: What have we learned? What should we expect?

The Fontan procedure is the final palliative surgery in a series of staged surgeries to reroute the systemic venous blood flow directly to the lungs, with the ventricle(s) pumping oxygenated blood to the body. Advances in medical and surgical techniques have improved patients' overall survival after the Fontan procedure. However, Fontan-associated chronic comorbidities are common.

Congestive Heart Failure in an Adolescent With a Ruptured Sinus of Valsalva Aneurysm.

Sinus of Valsalva aneurysm (SVA) is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction and is rare in the pediatric population. This case report describes a unique case of a 16-year-old adolescent patient admitted with progressive heart failure symptoms and diagnosed with a ruptured noncoronary SVA. He underwent surgical repair of the SVA with autologous pericardial patches and had an uncomplicated postoperative course.

Intraoperative Sapien S3 Valve Placement in 2 Patients With Multi-Valvar Disease Operation: A Unique Hybrid Procedure.

There has been a paradigm shift in the management of patients with congenital heart disease with a move away from conventional surgical treatment in favor of a percutaneous catheter-based approach across the spectrum of valvular heart diseases. The Sapien S3 valve implantation in the pulmonary position has been previously reported using a conventional transcatheter approach in patients with pulmonary insufficiency due to an enlarged right ventricular outflow tract. In this report, we present 2 unique cases of intraoperative hybrid implantation of Sapien S3 valves in patients with complex pulmonic and tricuspid valvular disease.

COVID-19 in Adults With Congenital Heart Disease.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications.

Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes.

Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide.

Echocardiographic Findings in Children with Native Mitral Valve Masses Complicated by Systemic Embolization.

• Case of mitral valve IMT with embolization. • Case of mitral valve endocarditis with embolization. • Use of 2D and 3D echocardiography for management of mitral valve masses.

The Safety and Efficacy of Transcatheter Pulmonary Valve Replacement Combined with Electrophysiology Procedures.

The objective of this study was to evaluate the safety and efficacy of combining transcatheter pulmonary valve replacement (TPVR) and electrophysiology (EP) procedures. A retrospective review was undertaken to identify TPVR and EP procedures that were concomitantly performed in the cardiac catheterization laboratory at University of Iowa Stead Family Children's Hospital from January 2011 to October 2019. Procedural and follow-up data were compared between patients who underwent TPVR and EP procedures in the same setting to those who received TPVR or EP procedure separately and that were similar in age and cardiac anatomy.

Magnetic Resonance Imaging Assessment of Blood Flow Distribution in Fenestrated and Completed Fontan Circulation with Special Emphasis on Abdominal Blood Flow.

Objective: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI).

Materials And Methods: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.

Associations Between Age at Arterial Switch Operation, Brain Growth, and Development in Infants With Transposition of the Great Arteries.

Background: Brain injury, impaired brain growth, and long-term neurodevelopmental problems are common in children with transposition of the great arteries. We sought to identify clinical risk factors for brain injury and poor brain growth in infants with transposition of the great arteries undergoing the arterial switch operation, and to examine their relationship with neurodevelopmental outcome.

Methods: The brains of 45 infants with transposition of the great arteries undergoing surgical repair were imaged pre- and postoperatively using magnetic resonance imaging.

Interplay of brain structure and function in neonatal congenital heart disease.

Objective: To evaluate whether structural and microstructural brain abnormalities in neonates with congenital heart disease (CHD) correlate with neuronal network dysfunction measured by analysis of EEG connectivity.

Methods: We studied a prospective cohort of 20 neonates with CHD who underwent continuous EEG monitoring before surgery to assess functional brain maturation and network connectivity, structural magnetic resonance imaging (MRI) to determine the presence of brain injury and structural brain development, and diffusion tensor MRI to assess brain microstructural development.

Results: Neonates with MRI brain injury and delayed structural and microstructural brain development demonstrated significantly stronger high-frequency (beta and gamma frequency band) connectivity.

MRI reveals hemodynamic changes with acute maternal hyperoxygenation in human fetuses with and without congenital heart disease.

Objective: We investigated the physiologic impact of acute maternal hyperoxygenation (MH) in human fetuses with and without congenital heart disease (CHD) using fetal cardiac magnetic resonance (CMR) in order to explore the potential therapeutic benefits of chronic MH.

Methods: We examined 17 normal and 20 late gestation human fetuses with CHD on a 1.5 T CMR system.

Reduced fetal cerebral oxygen consumption is associated with smaller brain size in fetuses with congenital heart disease.

Background: Fetal hypoxia has been implicated in the abnormal brain development seen in newborns with congenital heart disease (CHD). New magnetic resonance imaging technology now offers the potential to investigate the relationship between fetal hemodynamics and brain dysmaturation.

Methods And Results: We measured fetal brain size, oxygen saturation, and blood flow in the major vessels of the fetal circulation in 30 late-gestation fetuses with CHD and 30 normal controls using phase-contrast magnetic resonance imaging and T2 mapping.

Postmortem imaging in congenital heart disease: preliminary experience.

Background: Despite the importance of the autopsy for quality improvement, autopsy rates have declined dramatically in recent decades due to poor acceptance by families and physicians and high costs to institutions.

Purpose: To compare postmortem imaging (PMI) with autopsy in patients with congenital heart defects to see if PMI could substitute in some or all cases and to compare costs of the two methods.

Material And Methods: Ten patients with congenital heart disease dying in hospital during the study period in whom an autopsy was planned underwent PMI using postmortem magnetic resonance imaging (PMMRI) (6 patients) and postmortem computed tomographic angiography (PMCTA) (10 patients) with permission of the family.

MRI shows limited mixing between systemic and pulmonary circulations in foetal transposition of the great arteries: a potential cause of in utero pulmonary vascular disease.

Objectives: To investigate the relationship between foetal haemodynamics and postnatal clinical presentation in patients with transposition of the great arteries using phase-contrast cardiovascular magnetic resonance.

Background: A severe and irreversible form of persistent pulmonary hypertension of the newborn occurs in up to 5% of patients with transposition and remains an important cause of morbidity and mortality in these infants. Restriction at the foramen ovale and ductus arteriosus has been identified as a risk factor for the development of pulmonary hypertension, and this can now be studied with magnetic resonance imaging using a new technique called metric optimised gating.

Novel mutations in geleophysic dysplasia type 1.

Geleophysic dysplasia (GD) is a rare genetic disorder characterized by acromelic dysplasia. Geleophysic dysplasia type 1 (MIM 231050) is autosomal recessive and is caused by homozygous or compound heterozygous mutation in the ADAMTSL2 (a disintegrin and metalloproteinase with thrombosponding repeats-like 2) gene. Geleophysic dysplasia type 2 (MIM 614185) is autosomal dominant and is caused by heterozygous mutation in the fibrillin 1 (FBN1) gene.

Diffusion MRI tractography of the developing human fetal heart.

Objective: Human myocardium has a complex and anisotropic 3D fiber pattern. It remains unknown, however, when in fetal life this anisotropic pattern develops and whether the human heart is structurally fully mature at birth. We aimed here to use diffusion tensor MRI (DTI) tractography to characterize the evolution of fiber architecture in the developing human fetal heart.

Postmortem imaging of antemortem myocardial ischaemia.

Objectives: To determine the minimum survival time for detection of antemortem myocardial ischaemia with postmortem imaging (PMI) techniques.

Methods: Nine pigs underwent ligation of the left anterior descending (LAD) (8) and/or right coronary artery (RCA) branch (4), and were killed 30 min-6 h after ligation. PMI (MRI and CT angiography) was performed 2-55 h after euthanasia.

Midterm survival of infants requiring postoperative extracorporeal membrane oxygenation after Norwood palliation.

This study reports the mid-term survival for neonates undergoing extracorporeal membrane oxygenation (ECMO) after Norwood palliation at a single center. Limited data exist on the mid-term survival of patients undergoing ECMO after Norwood palliation. We reviewed our ECMO experience from July 1994 to October 2008 and compared two groups: patients who required ECMO after Norwood palliation and patients who underwent Norwood palliation without ECMO.