Variability in the Echocardiographic Evaluation of the Systemic Right Ventricle.

Background: Echocardiographic evaluation of the systemic right ventricle (sRV) remains challenging in patients with transposition of the great arteries (TGA) corrected by an atrial switch (AS) and with congenitally corrected TGA (ccTGA). The aim of this study was to determine the interobserver and intraobserver variability of echocardiographic parameters for sRV size and systolic function.

Methods: Six independent observers retrospectively interpreted 44 previously acquired echocardiograms (25 patients with TGA/AS and 19 patients with ccTGA).

Late Onset Postcapillary Pulmonary Hypertension in Patients With Transposition of the Great Arteries and Mustard or Senning Baffles.

Background: There is a paucity of data regarding late-onset pulmonary hypertension (PH) in patients with transposition of the great arteries and atrial switch surgery.

Methods And Results: A retrospective cohort study was conducted on 140 adults with transposition of the great arteries and atrial switch surgery, age 37.3±7.

Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood.

Background: Shone complex consists of a constellation of left-sided, usually obstructive, cardiac lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation. Incomplete Shone complex consists of a mitral valve anomaly associated with lesions involving the subaortic region, aortic valve, or thoracic aorta. There is a paucity of data regarding long-term outcomes in adults with Shone complex.

The impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot.

Background: Sudden death of presumed arrhythmic etiology is the leading cause of mortality in adults with tetralogy of Fallot.

Objectives: To assess the impact of exercise on ventricular arrhythmias in adults with tetralogy of Fallot.

Methods: Adults with repaired tetralogy of Fallot were randomized in a 2:1 ratio to an open-label trial of standard care versus 12weeks of supervised combined aerobic/resistance training with continuous Holter monitoring.

Risks and Benefits of Exercise Training in Adults With Congenital Heart Disease.

Exercise capacity in adults with various forms of congenital heart disease is substantially lower than that of the general population. Although the underlying congenital heart defect, and its sequelae, certainly contribute to observed exercise limitations, there is evidence suggesting that deconditioning and a sedentary lifestyle are important implicated factors. The prevalence of acquired cardiovascular comorbidities is on the increase in the aging population with congenital heart disease, such that obesity and a sedentary lifestyle confer increased risk.

Adult congenital heart disease: a growing epidemic.

Medical and surgical breakthroughs in the care of children born with heart defects have generated a growing population of adult survivors and spawned a new subspecialty of cardiology: adult congenital heart disease. The prevalence of adult congenital heart disease is escalating at a rampant rate, outpacing the relatively static prevalence of pediatric congenital heart disease, because adults now surpass children in numbers by a ratio of 2:1. As such, congenital heart disease can no longer be considered primarily a pediatric specialty.

Cardiac, obstetric, and fetal outcomes during pregnancy after biological or mechanical aortic valve replacement.

Background: The aim of this study was to assess pregnancy-related cardiac, maternal, and fetal outcomes in women who underwent aortic valve replacement (AVR).

Methods: From 1978-2011, 67 women < 40 years of age underwent 74 isolated AVRs (52 mechanical prostheses and 22 bioprostheses). All patients were prospectively followed at our dedicated valve clinic.

Aortic coarctation and the retinal microvasculature.

Background: Aortic coarctation has been associated with generalized vascular disease, yet little is known about retinal vascular patterns and their changes over time.

Objectives: The aim of this study is to characterize the nature and extent of retinal vascular disease in adults with aortic coarctation, and explore age-related effects and associations with cardiovascular outcomes.

Methods: A prospective cross-sectional seroepidemiological study was conducted on 60 consecutive adults with repaired aortic coarctation, age 42.

Initial clinical experience with the GORE septal occluder for the treatment of atrial septal defects and patent foramen ovale.

Aims: To describe the initial experience with the GORE® septal occluder (GSO), a new septal occluder for the treatment of atrial septal defects (ASD) and patent foramen ovale (PFO).

Methods And Results: This was a prospective single-centre review of patients undergoing percutaneous closure for a PFO or ASD with the GSO. A clinical evaluation and follow-up echocardiography were performed at three months with transoesophageal echocardiography (TEE) in case of PFO and transthoracic echocardiography (TTE) in ASDs.

Fontan circulation: success or failure?

Fontan surgery represents a milestone in the evolution of congenital heart disease management. It achieved the seemingly improbable (ie, restoration of a noncyanotic state by entirely bypassing the subpulmonary ventricle). In so doing, it has allowed a generation of children who may have otherwise succumbed to their severe congenital heart defect to survive to adulthood.

Tetralogy of Fallot and aortic root disease.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease and accounts for 10% of all congenital heart defects. Corrective surgery has dramatically improved long-term prognosis, with nearly 90% of patients now surviving well into adulthood. Aortic root dilation is a well-recognized feature of unrepaired tetralogy of Fallot, and is increasingly reported in patients, years after corrective surgery.

Impaired arm development after Blalock-Taussig shunts in adults with repaired tetralogy of Fallot.

Background: Many adults with repaired tetralogy of Fallot have had prior Blalock-Taussig shunts. These shunts may theoretically hinder growth and development of the ipsilateral arm.

Methods: We prospectively enrolled consecutive patients with tetralogy of Fallot in a cross-sectional study to measure arm length and assess handgrip strength.

Multiplanar transesophageal echocardiography for the evaluation and percutaneous management of ostium secundum atrial septal defects in the adult.

The purpose of this paper is to review the usefulness of multiplanar transesophageal echocardiography before, during and after percutaneous transcatheter closure of secundum atrial septal defects. Transesophageal echocardiography imaging techniques,including their role in patient selection, procedural guidance and immediate assessment of technical success and complications are described and discussed in this review. Percutaneous transcatheter closure is indicated for ostium secundum atrial septal defects of less than 40 mm in maximal diameter.

Pregnancy in women with Fontan physiology.

Advances in the care of patients with a univentricular heart have enabled the majority to survive well into adulthood. Consequently, an increasing number of women with Fontan palliation are contemplating pregnancy. This review summarizes physiologic modifications related to pregnancy in the setting of single-ventricle physiology, discusses potential maternal cardiovascular concerns and describes commonly encountered obstetrical and neonatal complications.

Recent advances in adult congenital heart disease.

As a result of major achievements in pediatric cardiac care, a growing number of patients with congenital heart disease (CHD) are flourishing well into adulthood. This heterogeneous and aging population of patients, many of whom represent the first generation of middle-age survivors, faces unique issues and challenges. As a field, adult CHD has evolved markedly during the past decade on several fronts, including imaging, arrhythmia management, percutaneous interventions, surgical techniques, research, and multidisciplinary care that extends beyond the cardiac realm.

Structure and process measures of quality of care in adult congenital heart disease patients: a pan-Canadian study.

Background: There are more adults than children with congenital heart disease. Of over 96,000 ACHD patients in Canada, approximately 50% require ongoing expert care. In spite of published recommendations, data on the quality of care for ACHD patients are lacking.

Prediction of complications in pregnant women with cardiac diseases referred to a tertiary center.

Background: Prediction of adverse maternal and neonatal events in women with heart disease is not well established. We aimed to assess cardiac, obstetrical and neonatal complications in pregnant women with heart disease referred to our tertiary care center and validate a previously proposed risk index.

Methods: We included 227 women with cardiac disease followed for 312 pregnancies at our tertiary center from 1992 to 2007.

Clinical assessment of arrhythmias in tetralogy of Fallot.

As a major success story of an early surgical era, corrective surgery transformed the prognosis of patients born with tetralogy of Fallot. With lifelong care, healthy survival well into adulthood is now the norm, albeit somewhat blemished by long-term sequelae. Arrhythmias are an important source of morbidity and a leading cause of mortality.

Pulmonary arterial hypertension in patients with transcatheter closure of secundum atrial septal defects: a longitudinal study.

Background: Pulmonary arterial hypertension (PAH) may develop in patients with atrial septal defects (ASD); however, little is known about associated risk factors and its evolution after transcatheter ASD closure.

Methods And Results: We conducted a cohort study on 215 adults with attempted transcatheter ASD closure from 1999 to 2006. Patients were classified according to baseline systolic pulmonary artery pressures as having no (I, <40 mm Hg), mild (II, 40 to 49 mm Hg), moderate (III, 50 to 59 mm Hg), or severe (IV, >or=60 mm Hg) PAH.

Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.

Background: Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators.

Methods And Results: We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications.

Evaluation of adult congenital heart disease by cardiac magnetic resonance imaging.

Cardiovascular magnetic resonance (CMR) imaging plays an essential role in the evaluation and follow-up of adult congenital heart disease (ACHD), providing safe, high-resolution imaging of some of the most complex anatomies encountered. Unlimited by acoustic windows and capable of tissue characterization, CMR is devoid of ionizing radiation and provides superior three-dimensional spatial resolution to transthoracic echocardiography and superior temporal resolution to computed tomography, making it the gold standard for various cardiac and great vessel imaging indications in ACHD. In this state-of-the art review, we provide an overview of CMR examination methods and detail the various approaches and classical findings in the more common forms of ACHD.

Risk stratification in surgically repaired tetralogy of Fallot.

Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients. Indeed, sudden death of presumed arrhythmic etiology is the most frequent mode of demise in patients with surgically repaired tetralogy of Fallot, often in early-to-mid adulthood. Nevertheless, the overall annual incidence of sudden death is actually low.

Nocturnal breathing in cyanotic congenital heart disease.

Background: Sleep disordered breathing is frequently observed in patients with cardiovascular disease. Even in the absence of heart disease, acute and chronic hypoxia have been shown to promote sleep-related periodic breathing with central apnea characterized by a repetitive reduction or lack of respiratory activity. Cyanotic congenital heart disease (CCHD) is associated with chronic hypoxia, regardless of whether an increase in pulmonary artery pressures coexists.

Laser lead extraction in adult congenital heart disease.

Background: In adults with congenital heart disease (ACHD), lead extraction procedures are expected to parallel increasing transvenous pacemaker and defibrillator implantations. We sought to assess the safety and feasibility of laser lead extraction in ACHD.

Methods And Results: All laser lead extractions (Spectranectics, Colorado Springs, CO, USA) performed at the Montreal Heart Institute between September 2000 and August 2005 were prospectively registered.