Lung function and gas exchange in Eisenmenger syndrome and their impact on exercise capacity and survival.

Background: Eisenmenger physiology may contribute to abnormal pulmonary mechanics and gas exchange and thus impaired functional capacity. We explored the relationship between lung function and gas exchange parameters with exercise capacity and survival.

Methods: Stable adult patients with Eisenmenger syndrome (N=32) were prospectively studied using spirometry, lung volumes, diffusion capacity, and blood gas analysis, as well as same day measurement of 6-minute walk distance and cardiopulmonary maximal treadmill exercise.

Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.

Objective: Patients with repaired tetralogy of Fallot (TOF) experience increased rates of mortality and morbidity in adulthood. This study was designed to identify risk factors for death and ventricular tachycardia (VT) in a large contemporary cohort of patients with repaired TOF.

Methods: Subjects with repaired TOF from four large congenital heart centres in the USA, Canada and Europe were enrolled.

Pulmonary hypertension related to congenital heart disease: a call for action.

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH). Despite this, little emphasis has been given to this group of patients until recently, when compared with idiopathic PAH. This is largely because of the complexity and the wide range of underlying cardiac anatomy and physiology, with a multitude of adaptive mechanisms not fully understood.

Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of Fallot and potential prognostic value of preoperative cardiopulmonary exercise testing.

Background: Indications for surgical pulmonary valve replacement (PVR) after repair of tetralogy of Fallot have recently been broadened to include asymptomatic patients.

Methods And Results: The outcomes of PVR in adults after repair of tetralogy of Fallot at a single tertiary center were retrospectively studied. Preoperative cardiopulmonary exercise testing was included.

Percutaneous patent foramen ovale occlusion: current evidence and evolving clinical practice.

Patent foramen ovale (PFO) has long been implicated with cryptogenic stroke, migraine and decompression illness. PFO is common and its implicated pathologies cause devastating neurological sequelae; and hence have drawn the attention of medical practitioners across disciplines. The pathogenesis is hypothesized to be caused by micro-emboli or neuro-hormones which would otherwise being filtered by the lungs, astraying into the systemic circulation via the atrial communication especially during Valsalva maneuver.

Meeting the challenge: the evolving global landscape of adult congenital heart disease.

Background: Only limited information is available on the worldwide distribution and volume of adult congenital heart disease (ACHD) centers. We aimed to assess the centers using a bibliometric approach.

Methods And Results: We identified publications presenting original research in the field of ACHD between 1995 and 2011.

Six-minute walk test distance and resting oxygen saturations but not functional class predict outcome in adult patients with Eisenmenger syndrome.

Background: Eisenmenger syndrome (ES) represents the extreme manifestation of pulmonary arterial hypertension in patients with congenital heart disease, associated with significant exercise intolerance and mortality. Even though of six-minute-walk-test (6MWT) is routinely used in these patients, little is known about its prognostic value in comparison to functional class.

Methods And Results: We included 210 adult patients with ES who underwent a total of 822 6MWTs.

Pregnancy and heart disease: time for a randomized controlled trial.

Cardiac disease is the most important cause of maternal mortality and despite considerable efforts by cardiologists and obstetricians alike, the numbers of deaths continue to rise. The literature on the subject is limited and almost entirely made up of case series. In this editorial we consider the need for randomized controlled trials in key areas, some will be harder than others to complete, but all are required to help reverse the growing numbers of women who die from heart disease during pregnancy.

Congenital heart disease beyond the age of 60: emergence of a new population with high resource utilization, high morbidity, and high mortality.

Aims: The population of adults with congenital heart disease (ACHD) is growing and ageing. Limited information about the diagnostic spectrum of this emerging population, its resource utilization at tertiary ACHD centres, and especially about prognostic parameters is available.

Methods And Results: Retrospective cohort study on all ACHD patients ≥60 years of age under active follow-up.

Great vessel and coronary artery anatomy in transposition and other coronary anomalies: a universal descriptive and alphanumerical sequential classification.

In patients with transposition of the great arteries, the identification of coronary anatomy is fundamental to optimal surgical outcome. A number of classifications describing the coronary vessels' origin and course in transposition of the great arteries have been published. However, all are limited to operative or pathological case series.

Right atrial area and right ventricular outflow tract akinetic length predict sustained tachyarrhythmia in repaired tetralogy of Fallot.

Aims: Repaired tetralogy of Fallot (rtoF) patients are at risk of atrial or ventricular tachyarrhythmia and sudden cardiac death. Risk stratification for arrhythmia remains difficult. We investigated whether cardiac anatomy and function predict arrhythmia.

Histopathology of the great vessels in patients with pulmonary arterial hypertension in association with congenital heart disease: large pulmonary arteries matter too.

Background: Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary hypertension on the larger proximal pulmonary arteries. This study aims to investigate the structural changes in the great arteries of adults who developed PAH in association with congenital heart disease (CHD), with severe cases termed Eisenmenger syndrome.

Methods: We performed macroscopic and light microscopy analyses on the great arteries of 10 formalin-fixed human hearts from patients with PAH/CHD and compared them to age-matched healthy controls.

Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival.

Background: Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease.

Methods And Results: A total of 1188 patients with adult congenital heart disease (age, 33.

Magnetic navigation in adults with atrial isomerism (heterotaxy syndrome) and supraventricular arrhythmias.

Aims: We analysed the type and mechanism of supraventricular arrhythmias encountered in a series of symptomatic adults with atrial isomerism undergoing catheter ablation procedures.

Methods And Results: The study population included consecutive adults with atrial isomerism who had previously undergone surgical repair or palliation of the associated anomalies. Patients underwent electrophysiological study for symptomatic arrhythmia in our institution between 2010 and 2012 using magnetic navigation in conjunction with CARTO RMT and three-dimensional (3D) image integration.

The safety and efficacy of trans-baffle puncture to enable catheter ablation of atrial tachycardias following the Mustard procedure: a single centre experience and literature review.

Background: Targets for catheter ablation of atrial tachyarrhythmias (AT) in post-Mustard procedure patients are often located in the pulmonary venous atrium (PVA). Traditional access to this chamber is retrograde via the aorta. However trans-baffle puncture may be a key determinant of successful ablation in many cases.

Echocardiographic predictors of outcome in eisenmenger syndrome.

Background: Eisenmenger syndrome differs significantly from other types of pulmonary arterial hypertension in its physiology and prognosis. We sought to assess the relationship between the echocardiographic characteristics of patients with Eisenmenger syndrome and mortality.

Methods And Results: Clinical and echocardiographic variables were assessed in 181 consecutive patients with Eisenmenger syndrome, excluding those with complex congenital heart disease.

Outcome in adult patients after arterial switch operation for transposition of the great arteries.

Background: The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist.

Methods And Results: We studied 145 adult patients (age>16, median 25 years) with ASO followed at our institution.

Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland.

Rationale: Incident pulmonary arterial hypertension was underrepresented in most pulmonary hypertension registries and may have a different disease profile to prevalent disease.

Objectives: To determine the characteristics and outcome of a purely incident, treatment-naive cohort of idiopathic, heritable, and anorexigen-associated pulmonary arterial hypertension and to determine the changes in presentations and survival over the past decade in the United Kingdom and Ireland.

Methods: All consecutive newly diagnosed patients from 2001 to 2009 were identified prospectively.

Rationale and design of an International Multicenter Registry of patients with repaired tetralogy of Fallot to define risk factors for late adverse outcomes: the INDICATOR cohort.

Although early survival after tetralogy of Fallot (TOF) repair in the modern era is excellent, studies on late outcomes have shown increasing rates of mortality and morbidity. Despite multiple publications on factors associated with late complications, risk factors for major outcomes (death and sustained ventricular tachycardia [VT]) remain poorly defined. Consequently, the International Multicenter TOF Registry (INDICATOR) was established.

Tricuspid valvar regurgitation in congenital absence of the pericardium.

Congenital absence of the pericardium (CAP) is a rare disorder in which most patients are asymptomatic. The association between this condition and the development of significant tricuspid valvar regurgitation (TR) has been reported, but the causal relationship between them is not clear. Herein is provided a review of all reported cases of CAP associated with TR, and the potential pathogenetic mechanisms behind this association are discussed.

Systemic right ventricular longitudinal strain is reduced in adults with transposition of the great arteries, relates to subpulmonary ventricular function, and predicts adverse clinical outcome.

Background: Transposition of the great arteries (TGA) after atrial switch operation and congenitally corrected TGA (ccTGA) are commonly associated with impaired systemic right ventricular (RV) function and impaired prognosis. We aimed to investigate the value of indices of myocardial deformation on speckle-tracking echocardiography for quantifying ventricular function and their potential role in assessing ventricular-ventricular interaction and outcome in patients with a systemic RV.

Methods And Results: A total of 129 patients (87 with TGA and atrial switch and 42 with ccTGA, 71 men, age 35 ± 12 years) were investigated, and biventricular myocardial deformation was compared with findings in healthy subjects (n = 38, age 36 ± 10 years).

Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of fallot.

Background: Sudden cardiac death and life-threatening ventricular arrhythmia remain a concern in adult patients with repaired tetralogy of Fallot. Longitudinal left ventricular (LV) function is sensitive in detecting early myocardial damage and may have prognostic implications in this setting.

Methods And Results: We included 413 tetralogy of Fallot patients (age, 36 ± 13 years; QRS duration, 148 ± 27 milliseconds; LV ejection fraction, 55 ± 10%).