Internal Cardioverter Defibrillator Indications and Therapies after Atrial Baffle Procedure for d-Transposition of the Great Arteries: A Multicenter Analysis.

BACKGROUND: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure. METHODS: Demographic and clinical data as well as data on device implantation, programming, ICD discharges, and complications after atrial baffle procedure for d-TGA from four German centers were analyzed retrospectively. RESULTS: ICD implantation was undertaken in 33 subjects. ICD implantation was undertaken as primary prevention in 29 (88%) and secondary prevention in four (12%) individuals. There were 21 (64%) subjects with atrial reentrant tachycardia (IART). During a median follow-up of 4.8 years, seven appropriate ICD therapies were delivered in three (10%) individuals with primary prevention indication. No appropriate shocks were documented in subjects with secondary prevention indication. A total of 12 inappropriate ICD discharges occurred in eight (24%) individuals due to IART (n = 6) or lead failure (n = 2). ICD-related complications were noted in seven individuals (21%): lead dislodgement/failure in five (15%) and ICD infection in two subjects (6%). CONCLUSIONS: The majority of individuals received an ICD for primary prevention of SCD, thus representing a liberal attitude of physicians for ICD implantation. During a median follow-up of 4.8 years, the rate of appropriate ICD therapies was low and clearly exceeded by inappropriate ICD discharges. Lead failure and IART were present in >20% of the individuals and were frequent reasons for inappropriate ICD discharges. Facing these results, rigorous treatment of IART and careful ICD programming seems mandatory.

[Cardiac arrhythmias in adults with congenital heart disease]. / Herzrhythmusstörungen bei Erwachsenen mit angeborenen Herzfehlern.

In patients with congenital heart disease, cardiac arrhythmias are complex and require a thorough understanding of the anatomy, past surgical and interventional procedures, and the specific electric processes. Supraventricular tachycardias commonly present as emergency situations and should be treated immediately, particularly when there is an underlying complex malformation. Establishing sinus rhythm is usually superior to pure frequency control for hemodynamic reasons. Catheter ablation should be preferred over medical treatment, even though several procedures are often necessary. In addition, bradycardia is seen more frequently in congenital heart defects; this could be aggravated by antiarrhythmic drugs. There are significant differences between the indications and techniques used for pacemaker implantation in patients with congenital heart defects and those without. Patients with complex congenital heart diseases have an increased risk of thromboembolism; therefore, an individual and early indication for low-threshold oral anticoagulation is necessary; direct oral anticoagulants can also be used for this purpose. In risk stratification for sudden cardiac death, the principles of general guidelines are often not applicable, and individualized decisions are required. Recently, a new general risk score for congenital heart disease has been developed. The treatment of cardiac arrhythmias in patients with congenital heart disease should always be performed in close cooperation with specialized centers.

Creation of a restrictive atrial communication in heart failure with preserved and mid-range ejection fraction: effective palliation of left atrial hypertension and pulmonary congestion.

BACKGROUND: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect. METHODS AND RESULTS: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %. Cardiomyopathy was classified as a restrictive (n = 4) or hypertrophic (n = 2). (75% related to congenital heart defects) Three patients had a systemic right ventricle; in the majority of patients, HFpEF was associated to complex congenital heart defects (n = 18). Mean pulmonary arterial pressures (PAP systolic/diastolic) were 56/28 (± 24/13), left atrial pressures (LAP, v-, a-wave, mean) 26/25/20 (± 7/10/6). Trans-septal puncture was used in 22 patients; foramen ovale dilatation in 2 patients. Median balloon size was 12 (range 6-18) mm; procedure time including diagnostic measures 125 (83-221) min. No procedural death or complications were observed. Mean LA-pressures decreased significantly to 19/19/15 ± 6/8/5 mmHg (p = 0.05); median brain natriuretic peptide (BNP) decreased from 392 (range 93-4401) pg/ml median BNP to 314 (range 61-1544) pg/ml (p = 0.05). Three patients died; one patient received orthotopic heart and one patient a heart-lung transplantation. No patient required so far an assist device. Clinical improvement occurred in all patients, in some after additional surgical or interventional approach. CONCLUSIONS: Transcatheter LA decompression is an age-independent, effective palliation treating patients with HFpEF.