Cardiac MRI predictors of adverse outcomes in adults with a systemic right ventricle.

Aims: Predicting risk in individuals with a systemic right ventricle (SRV) remains difficult. We assessed the value of cardiac MRI (CMR) for predicting death, heart transplantation (HT), or need for a ventricular assist device (VAD) in adults with D-transposition of the great arteries (DTGA) post Mustard/Senning and in adults with congenitally corrected transposition of the great arteries (ccTGA) at two large academic centres.

Methods And Results: Between December 1999 and November 2020, 158 adult patients with an SRV underwent CMR.

Importance of surgical expertise in septal myectomy for obstructive hypertrophic cardiomyopathy.

Objective: In 2011, a multidisciplinary hypertrophic cardiomyopathy (HCM) program with a dedicated myectomy surgeon was implemented at our institution. We hypothesized that a dedicated approach allows better identification and management of mitral regurgitation (MR) during septal myectomy (SM) for obstructive HCM with significant mitral regurgitation.

Methods: Between 2006 and 2018, 181 patients had SM at our institution.

Heart failure in adult congenital heart disease: tetralogy of Fallot.

The number of rTOF patients who survive into adulthood is steadily rising, with currently more than 90% reaching the third decade of life. However, rTOF patients are not cured, but rather have a lifelong increased risk for cardiac and non-cardiac complications. Heart failure is recognized as a significant complication.

Predictors of Late Mortality in D-Transposition of the Great Arteries After Atrial Switch Repair: Systematic Review and Meta-Analysis.

Background Existing data on predictors of late mortality and prevention of sudden cardiac death after atrial switch repair surgery for D-transposition of the great arteries (D-TGA) are heterogeneous and limited by statistical power. Methods and Results We conducted a systematic review and meta-analysis of 29 observational studies, comprising 5035 patients, that reported mortality after atrial switch repair with a minimum follow-up of 10 years. We also examined 4 additional studies comprising 105 patients who reported rates of implantable cardioverter-defibrillator therapy in this population.

Myocardial Contraction Fraction Predicts Cardiovascular Events in Patients With Hypertrophic Cardiomyopathy and Normal Ejection Fraction.

Background: Myocardial contraction fraction (MCF), the ratio of left ventricular stroke volume to myocardial volume, is a novel parameter that can distinguish between pathologic and physiologic hypertrophy. However, its prognostic value in hypertrophic cardiomyopathy (HCM) has never been examined. The objective was to determine if MCF is associated with functional capacity and predicts adverse cardiovascular outcomes in patients with HCM and normal left ventricular ejection fraction (LVEF).

Virtual septal myectomy for preoperative planning in hypertrophic cardiomyopathy.

Objective: Although septal myectomy (SM) is the preferred treatment for medication-refractory obstructive hypertrophic cardiomyopathy, the procedure remains subjective. We have developed a virtual myectomy (VM) technique using 3-dimensional reconstruction of gated cardiac computed tomography (CT) to assist intraoperative objective assessment of the adequacy of the resection.

Methods: We retrospectively reviewed patients 15 patients who underwent a SM guided by preoperative VM at our program between March 2016 and July 2017.

Multi-Modality Imaging in the Evaluation and Treatment of Tricuspid Regurgitation.

Purpose Of Review: The goal of this review is to cover the epidemiology of tricuspid regurgitation (TR), anatomy of the tricuspid valve (TV), and the mechanisms and modern treatment of TR. The focus will be on the role of echocardiography, cardiac CT, and MRI to determine the mechanism, severity, and management strategies of TR.

Recent Findings: The evaluation and management of TR is a rapidly growing field with significant advances in both imaging and interventions.

Long-term Management of the Arterial Switch Patient.

Purpose Of Review: This review paper describes the management of patients with dextro-transposition of the great arteries (D-TGA) with a focus on the complications seen and the appropriate care required to identify and prevent adverse events.

Recent Findings: D-TGA is a form of cyanotic congenital heart disease (CHD) representing ~ 3% of all CHD and almost 20% of all cyanotic CHD. Since the late 1980s, standard of care is to repair these patients with an arterial switch operation (ASO) as opposed to a Mustard/Senning operation.

Role of computed tomography angiography for HeartMate II left ventricular assist device thrombosis.

Introduction: Device thrombosis is one of the most devastating complications of HeartMate II left ventricular assist devices. The purpose of this study is to assess the anatomical properties that the inflow and outflow computed tomography angiography provides in assessing for left ventricular assist device thrombosis, as well as their impact on clinical management and postoperative outcomes.

Methods: Between April 2010 and December 2016, 22 patients who received a HeartMate II left ventricular assist device implantation were readmitted for suspected device thrombosis and underwent a computed tomography angiography for workup.

Scimitar Syndrome in Children and Adults: Natural History, Outcomes, and Risk Analysis.

Background: Scimitar syndrome involves congenital anomalous pulmonary venous return to the inferior vena cava. Optimal management remains controversial. We describe the natural history of disease, nonsurgical and surgical outcomes, and risk factors for poor outcomes at our institution.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association.

Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD.

Outcomes and Prognostic Factors for Adult Patients With Congenital Heart Disease Undergoing Primary or Reoperative Systemic Atrioventricular Valve Surgery.

Background: Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery.

Methods: We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation.

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.

Background: The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified.

Methods And Results: A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed.

Postoperative tricuspid regurgitation after adult congenital heart surgery is associated with adverse clinical outcomes.

Objective: Many patients with adult congenital heart disease will require cardiac surgery during their lifetime, and some will have concomitant tricuspid regurgitation. However, the optimal management of significant tricuspid regurgitation at the time of cardiac surgery remains unclear. We assessed the determinants of adverse outcomes in patients with adult congenital heart disease and moderate or greater tricuspid regurgitation undergoing cardiac surgery for non-tricuspid regurgitation-related indications.

Pheochromocytoma and paraganglioma in cyanotic congenital heart disease.

Context: Aberrant cellular oxygen sensing is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL).

Objective: The objective of the study was to test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD) increases the risk for PHEO-PGL.

Design/setting/participants: We investigated the association between CCHD and PHEO-PGL with two complementary studies: study 1) an international consortium was established to identify congenital heart disease (CHD) patients with a PHEO-PGL diagnosis confirmed by pathology or biochemistry and imaging; study 2) the 2000-2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with noncyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes.

Adult congenital heart disease and pregnancy.

Adults with congenital heart disease now form the largest group of women with cardiac disease becoming pregnant in the developed world. This is both a mark of impressive steps forward in the management of congenital heart disease and also a challenge to the medical community to develop systems of care that will best serve these women and their babies. Each woman with congenital heart disease presents a unique pattern of challenges for the cardiologist, obstetrician, and anesthesiologist, and their care should be tailored to deal with their individual circumstances.