PREVENTION-ACHD: PRospEctiVE study on implaNTable cardioverter-defibrillator therapy and suddeN cardiac death in Adults with Congenital Heart Disease; Rationale and Design.

BACKGROUND: Many adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) may prevent SCD, but the evidence for primary prevention indications is still unsatisfactory. STUDY DESIGN: PREVENTION-ACHD is a prospective study with which we aim to prospectively validate a new risk score model for primary prevention of SCD in ACHD patients, as well as the currently existing guideline recommendations. Patients are screened using a novel risk score to predict SCD as well as current ICD indications according to an international Consensus Statement. Patients are followed up for two years. The primary endpoint is the occurrence of SCD and sustained ventricular arrhythmias. The Study was registered at ClinicalTrials.gov (NCT03957824). CONCLUSION: PREVENTION-ACHD is the first prospective study on SCD in ACHD patients. In the light of a growing and aging population of patients with more severe congenital heart defects, more robust clinical evidence on primary prevention of SCD is urgently needed.

Outcomes in patients with pulmonary hypertension undergoing percutaneous atrial septal defect closure.

OBJECTIVES: To examine the outcomes in patients with moderate or severe pulmonary arterial hypertension (PAH) undergoing percutaneous atrial septal defect (ASD) closure. DESIGN: Retrospective study. SETTING: Teaching hospital-based study. PATIENTS: Fifty-four patients with moderate (n = 34) or severe PAH (n = 20) who underwent successful device implantation between 1999 and 2004 were included in the study. Clinical and transthoracic echocardiographic data were reviewed. Pulmonary hypertension was classified as moderate (50-59 mm Hg) or severe (>or=60 mm Hg) according with the right ventricular systolic pressure (RVSP) calculated by echocardiography. RESULTS: At the early follow-up (mean (SD) 2.3 (1.2) months) all patients were alive and the baseline RVSP decreased from 57 (11) mm Hg to 51 (17) mm Hg (p = 0.003). At the late follow-up (n = 39, mean (SD) duration 31 (15) months) two patients had died and the baseline RVSP decreased from 58 (10) mm Hg to 44 (16) mm Hg (p = 0.004). Although the overall mean RVSP decreased at late follow-up, only 43.6% (17/39) of patients had normalisation (<40 mm Hg) of the RVSP and 15.4% (6/39) had persistent severe PAH. CONCLUSION: Transcatheter closure in patients with secundum ASD and PAH can be successfully performed in selected subjects and is associated with good outcomes. Early improvements in RVSP are seen in patients with moderate or severe PAH undergoing transcatheter ASD closure. Continued improvement in RVSP occurs in late follow-up. Despite decreases in the mean RVSP in late follow-up, many patients do not have complete normalisation of pressures.

Symptomatic atrial arrhythmias and transcatheter closure of atrial septal defects in adult patients.

OBJECTIVE: To determine whether transcatheter device closure of a secundum atrial septal defect (ASD) will reduce the risk of developing subsequent atrial arrhythmias. DESIGN: The incidence and predictors of symptomatic atrial tachyarrhythmias (AT) were examined in adults undergoing transcatheter closure of ASDs. SETTING: Toronto Congenital Cardiac Centre for Adults. PATIENTS: 132 consecutive patients, mean (SD) age 44 (16) years; 74% female. MAIN OUTCOME MEASURE: Sustained or symptomatic atrial arrhythmias at early follow up (six weeks; n = 115) and intermediate follow up (last clinic visit 17 (11) months post surgery; n = 121). RESULTS: 15% of the patients (20 of 132) had AT before the procedure (14 paroxysmal, six persistent). Patients without a history of arrhythmia had a low incidence of AT during early follow up (6%) and intermediate follow up (1%/year), while all patients with persistent AT before closure remained in atrial fibrillation or flutter. Of patients in sinus rhythm but with a previous history of AT, two thirds remained arrhythmia-free at follow up, with overall incidences of paroxysmal and persistent AT of 17%/year and 11%/year. A history of AT before closure (risk ratio (RR) 35.0, 95% confidence interval (CI) 7.2 to 169.0) and age > or = 55 years at the time of device insertion (RR 5.6, 95% CI 1.2 to 25.0) predicted AT after closure. CONCLUSIONS: Device closure of an ASD before the onset of atrial arrhythmias may protect against the subsequent development of arrhythmia, in particular in patients less than 55 years of age.