[Adults with congenital heart disease presenting to the emergency department : Potential pitfalls]. / Erwachsene mit angeborenen Herzfehlern im Notaufnahmedienst : Was ist zu beachten?

The number of adults with congenital heart disease (ACHD) already exceeds the number of children with congenital heart disease in the industrialized world. ACHD patients often show complex pathophysiology and anatomy even after reparative cardiac surgery. In case of complications patients may rapidly deteriorate and become unstable, even when they were asymptomatic or had only mild symptoms before the onset of the complication. Compared to all patients seen by emergency physicians, emergencies in ACHD patients are still rare. This review is aimed to guide management in ACHD emergency situations. Approximately two-thirds of all emergency admissions are caused by arrhythmias or acute heart failure. Sustained arrhythmias may rapidly lead to acute cardiac decompensation in ACHD patients. If medical treatment fails or patients present in hemodynamically unstable conditions, prompt electrical cardioversion is mandatory. Symptomatic bradycardia may require urgent pacemaker implantation. Depending on the underlying heart defect, placement of temporary transvenous pacemaker leads may be impossible. Acute heart failure in ACHD patients is often caused by acute right heart failure. Other more frequent emergencies are infections, syncope, thromboembolic events, and aortic dissection. It is highly recommended to contact the tertiary care center that follows the patient regularly early in case of patient presentation to the emergency room.

Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study.

BACKGROUND: Current guidelines consider vitamin K antagonists (VKA) the oral anticoagulant agents of choice in adults with atrial arrhythmias (AA) and moderate or complex forms of congenital heart disease, significant valvular lesions, or bioprosthetic valves, pending safety data on non-VKA oral anticoagulants (NOACs). Therefore, the international NOTE registry was initiated to assess safety, change in adherence and quality of life (QoL) associated with NOACs in adults with congenital heart disease (ACHD). METHODS: An international multicenter prospective study of NOACs in ACHD was established. Follow-up occurred at 6 months and yearly thereafter. Primary endpoints were thromboembolism and major bleeding. Secondary endpoints included minor bleeding, change in therapy adherence (≥80% medication refill rate, ≥6 out of 8 on Morisky-8 questionnaire) and QoL (SF-36 questionnaire). RESULTS: In total, 530 ACHD patients (mean age 47 SD 15 years; 55% male) with predominantly moderate or complex defects (85%), significant valvular lesions (46%) and/or bioprosthetic valves (11%) using NOACs (rivaroxaban 43%; apixaban 39%; dabigatran 12%; edoxaban 7%) were enrolled. The most common indication was AA (91%). Over a median follow-up of 1.0 [IQR 0.0-2.0] year, thromboembolic event rate was 1.0% [95%CI 0.4-2.0] (n = 6) per year, with 1.1% [95%CI 0.5-2.2] (n = 7) annualized rate of major bleeding and 6.3% [95%CI 4.5-8.5] (n = 37) annualized rate of minor bleeding. Adherence was sufficient during 2 years follow-up in 80-93% of patients. At 1-year follow-up, among the subset of previous VKA-users who completed the survey (n = 33), QoL improved in 6 out of 8 domains (p ≪ 0.05). CONCLUSIONS: Initial results from our worldwide prospective study suggest that NOACs are safe and may be effective for thromboembolic prevention in adults with heterogeneous forms of congenital heart disease.

[Total thyroidectomy in patients with amiodarone-induced hyperthyroidism: when does the risk of conservative treatment exceed the risk of surgery?]. / Totale Thyreoidektomie bei amiodaroninduzierter Hyperthyreose: Wann übersteigt das Risiko der konservativen Therapie das Risiko der chirurgischen Behandlung?

Amiodarone plays a pivotal role in the treatment of ventricular and supraventricular arrhythmias. However, amiodarone-induced hyperthyroidism (AIH) is one of the most feared complications, which necessitates interdisciplinary treatment and careful balancing of the risks of conservative treatment against those of total thyroidectomy. In this article we discuss the pharmacological aspects of amiodarone and its diverse effects on the thyroid. Furthermore, we present diagnostic and therapeutic strategies and report our positive experiences with total thyroidectomy in patients with AIH. Particularly in patients for whom continuation of amiodarone treatment is compulsory, a well-timed total thyroidectomy is a reliable therapeutic option, with minimal complication rates and immediate amelioration of symptoms.

Heart transplantation in adolescent and adult patients with congenital heart disease: a case-control study.

BACKGROUND: The number of adolescent and adult patients with congenital heart disease undergoing heart transplantation is increasing. We aimed to better define the characteristics of these patients and their survival after transplantation. METHODS: We describe a group of patients with end-stage heart failure owing to congenital heart disease undergoing heart transplantation at a single tertiary center and compare their short- and long-term survival with a group of matched controls with dilated cardiomyopathy and the entire cohort of transplanted patients at our center. RESULTS: Between 1985 and 2006, a total of 322 orthotopic heart transplantations were performed at our center. Thirteen patients (mean age, 27.5 years) had a diagnosis of congenital heart disease with a wide spectrum of lesions. The survival of these 13 patients was 85% at 30 days, 1, 5, and 10 years and 77% at 20 years, which did not differ significantly to the short- and long-term survival of the entire cohort of patients with heart transplantation and to the survival of age-matched controls with dilated cardiomyopathy. CONCLUSION: In our single-center experience, short- and long-term survival after heart transplantation in a selected, small group of patients with end-stage heart failure owing to congenital heart disease did not differ significantly compared with a group of age-matched controls and the entire cohort.

[Recurrent pneumonia--case report and review of the literature]. / Rezidivierende Pneumonien.

We report the case of a 70-year old man with recurrent pneumonia due to aspiration from an otherwise asymptomatic small oesophageal diverticula in the mid-oesophageal region. The diagnosis was finally established by videofluoroscopy of the oesophagus after repeated bronchoscopies and CT scans in the proceeding months. After thoracoscopic removal of the diverticula the patient remained free of disease. Oesophageal diverticula as a rare cause of repeated pneumonias should be kept in mind, even though there are no symptoms of gastro-oesophageal disease.