Clinical Profile and Long-Term Follow-Up of Children with Brugada Syndrome.

Brugada syndrome (BrS) is a rare channelopathy associated with sudden cardiac death (SCD). Although outcome data of adult cohorts are well known, information on children are lacking. The aim of the present study was to analyze the clinical profile, treatment approach and long-term outcome of children affected with BrS. After a systematic review of the literature compiled from a thorough database search (PubMed, Web of Science, Cochrane Libary, Cinahl), data from a total of 4 studies which included 262 BrS patients were identified. The mean age of patients was 12.1 ± 5.5, 53.8% males and 19.8% spontaneous BrS type I. 80.2% of patients presented BrS ECG I after receiving sodium channel blockers. 76% of these patients were asymptomatic while only 17.9% suffered from recurrent syncope. Around 1.5% of the patients were admitted due to aborted SCD, and 3% suffered from atrial arrhythmias. Electrophysiological work-up was performed in 132 patients. Induction of ventricular tachycardia/ventricular fibrillation using programmed ventricular stimulation was inducible in 16 patients. 56 children received an ICD. 11 patients received quinidine. An electrical storm was documented in 1 patient. Appropriate shocks occured in 16% of the patients over a median follow-up period of 62.2 (54-64). ICD-related complications were observed in 11 patients (19.6%) with a predominance of inappropriate shocks and lead failure and/or fracture. Although BrS in the childhood is rare, diagnosis and management continues to be challenging. ICD therapy is an effective therapy in high-risk children with BrS, however, with relevant ICD-related complications.

Survival and Clinical Outcomes after Unconstrained Total Knee Arthroplasty for Tibial Plateau Fractures-A Retrospective Study with Minimum 4-Year Follow-Up.

This study investigated survival, complications, revisions, and patient-reported outcomes (PROs) for unconstrained total knee arthroplasty (TKA) in posttraumatic osteoarthritis (PTO) caused by intraarticular tibial plateau fractures with minimum four years follow-up. Forty-nine patients (71.4% male; 58.7 years) were included. Kaplan-Meier analysis was performed with failure defined as TKA removal. Patients without failure underwent pre- and postoperative evaluation (range of motion (ROM), Oxford Knee Score (OKS), Knee Society Score (KSS), anatomical femorotibial angle (aFTA), proximal tibial slope (PTS)) and Short Form-12 (SF-12) Physical (PCS) and Mental Component Summary (MCS) assessment at final follow-up. Fifteen (30.6%) patients had a complication, and eight (16.3%) patients underwent prosthesis removal at median 2.5 years. Cumulative survival rate of TKA was 79.6% at 20 years. A total of 32 patients with a mean follow-up of 11.8 years underwent further analyses. ROM (p = 0.028), aFTA (p = 0.044), pPS (p = 0.009), OKS (p < 0.001) and KSS (p < 0.001) improved significantly. SF-12 PCS was 42.3 and MCS was 54.4 at final follow-up. In general, one third of patients suffer a complication, and one in six patients has their prosthesis removed after TKA for PTO due to tibial plateau fractures. In patients who do not fail, TKA significantly improves clinical and radiographic outcomes at long-term follow-up.

Differences in Short QT Syndrome Subtypes: A Systematic Literature Review and Pooled Analysis.

BACKGROUND: Short QT syndrome (SQTS) is a rare syndrome and affects different types of genes. However, data on differences of clinical profile and outcome of different SQTS types are sparse. METHODS: We conducted a pooled analysis of 110 SQTS patients. Patients have been diagnosed between 2000 and 2017 at our institution (n = 12) and revealed using a literature review (n = 98). 29 studies were identified by analysing systematic data bases (PubMed, Web of Science, Cochrane Libary, Cinahl). RESULTS: 67 patients with genotype positive SQTS origin and 43 patients with genotype negative origin were found. A significant difference is documented between the sex with a higher predominance of male in genotype negative SQTS patients and predominance of females in genotype positive SQTS patients (male 52% versus 84%, female 45% versus 14%; p = 0.0016). No relevant difference of their median age (genotype positive 27 ± 19 versus genotype negative 29 ± 15; p = 0.48) was found. Asymptomatic patients and patients reporting symptoms such as syncope, sudden cardiac death, atrial flutter and ventricular fibrillation documented in both groups were similar except atrial fibrillation (genotype positive 19% versus genotype negative 0%; p = 0.0055). The QTc interval was not significantly different in both groups (genotype positive 315 ± 32 versus genotype negative 320 ± 19; p = 0.30). The treatments (medical treatment and ICD implantation) in both groups were comparable. Electrophysiology studies were not significantly higher documented in patients with genotype positive and negative origin (24% versus 9%; p = 0.075). Events at follow up such as VT, VF, and SCD were not higher presented in patients with genotype positive (13% versus 9%) (p = 0.25). 54% of genotype positive SQTS patients showed SQTS 1 followed by STQS 2 (21%) and SQTS 3 (10%). CONCLUSIONS: The long-term risk of a malignant arrhythmic event is not higher in patients with genotype positive. However, patients with genotype positive present themselves more often with AF with a female predominance. Also, other events at follow up such as syncope, atrial flutter and palpitation were not significantly higher (9% versus 0%; p = 0.079).