Refining current risk stratification guidelines for pregnant women with Fontan circulation: lessons from PROFAT registry.

Background: Pregnancy in women with a Fontan circulation carries increased risk. Given the relative evidence void, pregnancy counseling requires considerable nuance and experience.

Objectives: This study aimed to identify risk factors for maternal and fetal complications and to contrast risk estimates obtained from existing risk stratification tools, including the modified WHO, CARPREG II, and ZAHARA risk scores.

Addressing Complexities in Fontan Combined Heart-Liver Transplantation: A Collaborative Perspective.

In Fontan circulatory failure, heart or combined heart-liver transplantation (CHLT) is considered a final therapeutic option. Fontan patients present unique challenges for the cardiac and liver transplant teams. Improving outcomes of CHLT in Fontan patients requires a multidisciplinary team approach to patient selection, meticulous surgical planning, intraoperative and postoperative management, and continuous learning.

Hybrid Transcatheter Pulmonary Valve Replacement With SAPIEN S3 Following Right Ventricular Outflow Tract Reduction Using Tricuspid Annuloplasty Rings.

We describe a novel variation of hybrid TPVR using a tricuspid annuloplasty ring which can be considered for patients with very large RVOT, short pulmonary artery, and limited vascular access and to avoid the risk of cardiopulmonary bypass. The utilization of Carpentier-Edwards Physio Tricuspid annuloplasty ring can reduce the size of the RVOT and at the same time the ring served as an effective docking site for the implantation of the SAPIEN S3 (Edwards Lifesciences). All three reported cases showed satisfactory immediate hemodynamic and clinical outcomes as well as at mid-term follow up.

The Growing Role of Intracardiac Echo in Congenital Heart Disease Interventions.

Advancements in congenital heart disease (CHD) care have significantly improved survival, leading to a growing population of adults with congenital heart disease (ACHDs). Many of these patients require ongoing interventions for residual defects, conduit or valve dysfunction, and arrhythmia management, often performed using transcatheter techniques. Imaging plays a critical role in ensuring procedural success and safety.

Organization and structure of intensive care management of adult congenital heart disease in the United States.

Background: The growing number of adults with congenital heart disease has led to the formation of dedicated centers to provide specialized care. There is a paucity of published data on the organization, staffing, and resources available when adult congenital heart disease (ACHD) patients are hospitalized in intensive care settings. This study aims to describe the delivery of intensive care for ACHD patients in the United States and identify key differences between accredited centers or those pending accreditation and non-accredited Adult Congenital Heart Association (ACHA) centers.

Percutaneous Treatment of Proximal Migration of an Alterra Prestent.

Treatment of native right ventricular outflow tract (RVOT) dysfunction with predominant pulmonary regurgitation has become a catheter-based procedure since the introduction of the RVOT reducer devices such as the Alterra prestent (Edwards Lifesciences). These devices have potential complications. One such complication is the migration or embolization of the stent.

Interventions for adult congenital heart disease.

Advances in imaging diagnostics, surgical techniques and transcatheter interventions for paediatric patients with severe congenital heart disease (CHD) have substantially reduced mortality, thereby extending the lifespan of these individuals and increasing the number of adults with complex CHD. Transcatheter interventions have emerged as an alternative to traditional open-heart surgery to mitigate congenital defects. The evolution of techniques, the introduction of new devices and the growing experience of operators have enabled the treatment of patients with progressively more complex conditions.

Novel Use of a Catheter-Based Device Retrieval System for Percutaneous Treatment of Recurrent Atrial Myxoma.

Surgical resection is standard of care for the treatment of atrial myxoma. However, the optimal management strategy for recurrent cardiac tumors is less clear. Here we report the novel use of a catheter-based device retrieval system for the removal of a recurrent cardiac myxoma.

Age-Related Ventricular Tachycardia Substrate Characteristics for Repaired Tetralogy of Fallot Before Transcatheter Pulmonary Valve Placement.

Background: Ventricular tachycardia (VT) substrate characteristics before transcatheter pulmonary valve replacement (TPVR) in repaired tetralogy of Fallot (rTOF) are unknown.

Objectives: In this study, the authors sought to evaluate substrates for sustained monomorphic VT before TPVR in rTOF.

Methods: Retrospective (2017 to 2021) and prospective (commencing 2021) rTOF patients with native right ventricular outflow tract referred for electrophysiology study (EPS) before TPVR were included.

Propensity Score Analysis of Possible Medication Effects on Outcomes in Patients With Systemic Right Ventricles.

Background: Patients with systemic right ventricle (SRV), either d-transposition of the great arteries following an atrial switch procedure or congenitally corrected transposition of the great arteries, develop severe right ventricular dysfunction, prompting appropriate medical therapy. However, the efficacy of beta-blockers and angiotensin receptor blockers or angiotensin-converting enzyme inhibitors (ACEI) in SRV patients is unproven.

Objectives: The objective of this study was to determine the effects of ACEI/ARB and beta-blockers on outcomes in SRV patients after accounting for likely cofounders affecting their use.

Covered Stent Correction for Sinus Venosus Atrial Septal Defects, an Emerging Alternative to Surgical Repair: Results of an International Registry.

Background: Covered stent correction for a sinus venosus atrial septal defect (SVASD) was first performed in 2009. This innovative approach was initially viewed as experimental and was reserved for highly selected patients with unusual anatomic variants. In 2016, increasing numbers of procedures began to be performed, and in several centers, it is now offered as a standard of care option alongside surgical repair.

Multicenter Pivotal Study of the Alterra Adaptive Prestent for the Treatment of Pulmonary Regurgitation.

Background: A dilated native right ventricular outflow tract (RVOT) presents unique challenges for transcatheter management using balloon-expandable valves. The Alterra Adaptive Prestent was designed to expand transcatheter therapy to treat patients with dilated RVOTs.

Objectives: The aim of this study was to report 2-year outcomes of the main cohort of the ALTERRA (Multicenter Study of Congenital Pulmonic Valve Dysfunction Studying the SAPIEN 3 THV With the Alterra Adaptive Prestent) pivotal trial using the prestent with transcatheter pulmonary valve replacement.

Update on Transcatheter Interventions in Adults with Congenital Heart Disease.

The field of adult congenital interventions is unique in the world of cardiac catheterization, combining the structural concepts commonly employed in pediatric heart disease and applying them to adult patients, who are more amenable to device intervention as they no longer experience somatic growth. Rapid advances in the field have been made to match the growing population of adult patients with congenital heart disease, which currently surpasses the number of pediatric patients born with congenital heart disease. Many congenital defects, which once required surgical intervention or reoperation, can now be addressed via the transcatheter approach, minimizing the morbidity and mortality often encountered within adult congenital surgeries.

Extravascular protrusion of the Alterra adaptive prestent identified on surveillance computed tomography imaging.

Background: The Alterra adaptive prestent is a novel self-expanding device designed to provide a landing zone for the 29 mm SAPIEN 3 valve to treat pulmonary regurgitation in patients with a right ventricular outflow tract that is too large for a balloon expandable valve alone. The mechanism of fixation for the Alterra prestent is radial force from the self-expanding stent frame, combined with a unique set of flared "tines" that protrude from both ends of the stent.

Aims, Methods, And Results: In this report, we describe 6 patients who underwent uncomplicated transcatheter pulmonary valve replacement with an Alterra adaptive prestent and SAPIEN 3 valve and had surveillance chest computed tomography (CT) scans performed 1 day to 21 months after implant.

Cardiovascular Outcomes in Fontan Patients With Right vs Left Univentricular Morphology: A Multicenter Study.

Background: There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle.

Objectives: The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV.

Methods: The Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America.