Swiss Adult Congenital HEart disease Registry (SACHER) - rationale, design and first results.

Background: In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in Switzerland, providing detailed data on disease characteristics and outcomes: Swiss Adult Congenital HEart disease Registry (SACHER). Its aim is to improve the knowledge base of outcomes in adults with CHD. The registry design and baseline patient characteristics are reported.

Impact of growing cohorts of adults with con-genital heart disease on clinical workload: a 20-year experience at a tertiary care centre.

Background: Population based studies show a steady increase in adult patients with congenital heart defects. The aim of this study was to assess the evolution of such a patient cohort and its burden on clinical care at a dedicated tertiary care centre.

Methods: All patients with congenital heart disease followed up by a dedicated multidisciplinary team at our institution were identified (n = 1725).

Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

Background: Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes.

Methods: Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed.

High prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries.

Aims: To determine the prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries, as these may predispose to paradoxical embolic events, particularly in patients with transvenous pacemaker or defibrillator leads.

Methods And Results: We routinely perform contrast echocardiography with agitated saline in all patients after atrial switch operations. For this study, we analysed patients who had saline contrast echocardiography between 2010 and 2012.

Plasma levels of B-type natriuretic peptide in patients with tetralogy of Fallot after surgical repair.

Objective: To evaluate the utility of plasma BNP measurement in the long term follow-up of patients with surgically repaired tetralogy of Fallot (TOF).

Methods: From 2002 to 2008, plasma BNP concentration was measured in 130 patients with TOF at the age of mean 16.1+/-7.

A heart within the heart: double-chambered left ventricle.

We describe a rare congenital anomaly in a 49-year-old woman who presented with palpitations and slightly reduced exercise capacity. A double-chambered left ventricle was suspected on echocardiography and confirmed by cardiac computed tomography scanning, cardiac magnet resonance imaging, and invasive angiography.

Pulsating thoracic tumor caused by extragenital endometriosis in a patient with Noonan syndrome.

We present the case of a 36-year-old woman with Noonan syndrome and congenital heart disease in whom extragenital endometriosis caused an extraordinarily large jelly-like paracardiac and abdominal mass. Protrusion through an intercostal space caused a pulsating subcutaneous tumor on the left lateral thoracic wall. The patient never complained of menstruation-related pain.