UNASSIGNED: Long QT syndrome (LQTS) is an autosomal dominant inherited cardiac condition characterized by a QT interval prolongation and risk of sudden death. There are 17 subtypes of this syndrome associated with genetic variants in 11 genes. The second most common is type 2, caused by a mutation in the KCNH2 gene, which is part of the potassium channel and influences the final repolarization of the ventricular action potential. This case report presents an Ecuadorian teen with congenital Long QT Syndrome type 2 (OMIM ID: 613688), from a family without cardiac diseases or sudden cardiac death backgrounds.
UNASSIGNED: A 14-year-old girl with syncope, normal echocardiogram, and an irregular electrocardiogram was diagnosed with LQTS. Moreover, by performing Next-Generation Sequencing, a pathogenic variant in the KCNH2 gene p.(Ala614Val) (ClinVar ID: VCV000029777.14) associated with LQTS type 2, and two variants of uncertain significance in the AKAP9 p.(Arg1654GlyfsTer23) (rs779447911), and TTN p. (Arg34653Cys) (ClinVar ID: VCV001475968.4) genes were identified. Furthermore, ancestry analysis showed a mainly Native American proportion.
UNASSIGNED: Based on the genomic results, the patient was identified to have a high-risk profile, and an implantable cardioverter defibrillator was selected as the best treatment option, highlighting the importance of including both the clinical and genomics aspects for an integral diagnosis.

BACKGROUND: The subcutaneous implantable cardioverter defibrillator (S-ICD) and leadless pacemaker (LP) are alternative options for patients at high risk of infection requiring ICD and pacing therapy. In this analysis, we described the simultaneous implantation of S-ICD and LP in patients with high infectious risk.
METHODS: The study cohort comprised patients referred to our institution for ICD implantation due to high-risk factors of infection.
RESULTS: Between 2018 and 2022, 13 patients were referred, including 11 with infected ICD and 2 for first ICD implantation in the presence of high-risk factors. In cases of infected ICD, successful extraction was performed using a mechanical dilatation technique. Reimplantation was delayed until resolution of infection with antibiotic therapy. The devices were implanted during a single procedure, with S-ICD implantation following LP placement for verification of sensing adequacy through surface ECG screening. Suitable vectors for sensing during inhibited and ventricular pacing were identified in all patients. Defibrillation testing was effective, and no issues with double counting or undersensing were observed. The postoperative period was uneventful, and during a median follow-up of 35 months, no complications or infections were reported. The median ventricular pacing percentage was 5%, and a single inappropriate shock episode due to myopotential interference was reported and resolved by reprogramming the sensing vector.
CONCLUSIONS: Simultaneous implantation of S-ICD and LP is feasible and safe in patients at high risk of infection requiring both ICD and pacing therapy. This combined approach provides an effective solution for these patients.

A previous randomized study demonstrated that the subcutaneous implantable cardioverter defibrillator (S-ICD) was noninferior to transvenous ICD with respect to device-related complications and inappropriate shocks. However, that was performed prior to the widespread adoption of pulse generator implantation in the intermuscular (IM) space instead of the traditional subcutaneous (SC) pocket. The aim of this analysis was to compare survival from device-related complications and inappropriate shocks between patients who underwent S-ICD implantation with the generator positioned in an IM position in comparison with an SC pocket.
We analysed 1577 consecutive patients who had undergone S-ICD implantation from 2013 to 2021 and were followed up until December 2021. Subcutaneous patients (n = 290) were propensity matched with patients of the IM group (n = 290), and their outcomes were compared. : During a median follow-up of 28 months, device-related complications were reported in 28 (4.8%) patients and inappropriate shocks were reported in 37 (6.4%) patients. The risk of complication was lower in the matched IM group than in the SC group [hazard ratio 0.41, 95% confidence interval (CI) 0.17-0.99, P = 0.041], as well as the composite of complications and inappropriate shocks (hazard ratio 0.50, 95% CI 0.30-0.86, P = 0.013). The risk of appropriate shocks was similar between groups (hazard ratio 0.90, 95% CI 0.50-1.61, P = 0.721). There was no significant interaction between generator positioning and variables such as gender, age, body mass index, and ejection fraction.
Our data showed the superiority of the IM S-ICD generator positioning in reducing device-related complications and inappropriate shocks.
Clinical Trial Registration: ClinicalTrials.gov; NCT02275637.

A two-incision technique, in association with inter-muscular positioning of the subcutaneous defibrillator (S-ICD), is now the most frequently adopted implantation approach in Europe. Ultrasound-guided serratus anterior plane block (SAPB) has been proposed to provide anesthesia/analgesia during S-ICD implantation.
We performed a case-control analysis in which a standardized SAPB approach was compared with the typical local anesthesia and sedation approach.
Ninety-one consecutive patients underwent implantation of an S-ICD with the SAPB approach for anesthesia/analgesia at 10 centers. The control group consisted of 55 consecutive patients who underwent S-ICD implantation with a standard local approach.
The mean procedure duration was 59 ± 15 minutes in the SAPB group and 76 ± 23 minutes in the control group (P < .001). No operative complications were reported in either group. During the procedure, 79 (87%) patients in the SAPB group and 25 (46%) patients in the control group (P < .001) remained awake. Lower values of pain intensity at the device pocket (P = .005) and the lateral tunneling site (P = .046) were reported in the SAPB group. The difference in static (P = .002) and dynamic (P = .007) pain intensity between the groups persisted at 1 hour, while no differences were observed 6 hours after the end of the procedure.
SAPB is feasible and effective in providing anesthesia/analgesia during S-ICD implantation. The procedures were successfully accomplished and no complications occurred in either group. However, SAPB was associated with lower pain levels, enabling the need for sedation to be reduced and more patients to remain awake. Moreover, it resulted in shorter procedure durations.

Kearns-Sayre syndrome (KSS), a rare form of mitochondrial myopathy, is a triad of chronic progressive external ophthalmoplegia, bilateral pigmentary retinopathy, and cardiac conduction abnormalities. In this report, we show how a combined spinal epidural anesthesia can be useful for cesarean delivery, as we illustrate in a dual-chamber and VVI implantable defibrillator pacemaker/defibrillator parturient with a KSS and preeclampsia.