Intracardiac echocardiography in paediatric and congenital cardiac ablation shortens procedure duration and improves success without complications.

AIMS: Common to adult electrophysiology studies (EPSs), intracardiac echocardiography (ICE) use in paediatric and congenital heart disease (CHD) EPS is limited. The purpose of this study was to assess the efficacy of ICE use and incidence of associated complications in paediatric and CHD EPS. METHODS AND RESULTS: This single-centre retrospective matched cohort study reviewed EPS between 2013 and 2022. Demographics, CHD type, and EPS data were collected. Intracardiac echocardiography cases were matched 1:1 to no ICE controls to assess differences in complications, ablation success, fluoroscopy exposure, procedure duration, and arrhythmia recurrence. Cases and controls with preceding EPS within 5 years were excluded. Intracardiac echocardiography cases without an appropriate match were excluded from comparative analyses but included in the descriptive cohort. We performed univariable and multivariable logistic regression to assess associations between variables and outcomes. A total of 335 EPS were reviewed, with ICE used in 196. The median age of ICE cases was 15 [interquartile range (IQR) 12-17; range 3-47] years, and median weight 57 [IQR 45-71; range 15-134] kg. There were no ICE-related acute or post-procedural complications. There were 139 ICE cases matched to no ICE controls. Baseline demographics and anthropometrics were similar between cases and controls. Fluoroscopy exposure (P = 0.02), procedure duration (P = 0.01), and arrhythmia recurrence (P = 0.01) were significantly lower in ICE cases. CONCLUSION: Intracardiac echocardiography in paediatric and CHD ablations is safe and reduces procedure duration, fluoroscopy exposure, and arrhythmia recurrence. However, not every arrhythmia substrate requires ICE use. Thoughtful selection will ensure the judicious and strategic application of ICE to enhance outcomes.

An augmented hybrid multibaseline and referenceless MR thermometry motion compensation algorithm for MRgHIFU hyperthermia.

PURPOSE: A hybrid principal component analysis and projection onto dipole fields (PCA-PDF) MR thermometry motion compensation algorithm was optimized with atlas image augmentation and validated. METHODS: Experiments were conducted on a 3T Philips MRI and Profound V1 Sonalleve high intensity focused ultrasound (high intensity focused ultrasound system. An MR-compatible robot was configured to induce motion on custom gelatin phantoms. Trials with periodic and sporadic motion were introduced on phantoms while hyperthermia was administered. The PCA-PDF algorithm was augmented with a predictive atlas to better compensate for larger sporadic motion. RESULTS: During periodic motion, the temperature SD in the thermometry was improved from 1 . 1 ± 0 . 1 $$ 1.1\pm 0.1 $$ to 0 . 5 ± 0 . 1 ∘ $$ 0.5\pm 0.{1}^{\circ } $$ C with both the original and augmented PCA-PDF application. For large sporadic motion, the augmented atlas improved the motion compensation from the original PCA-PDF correction from 8 . 8 ± 0 . 5 $$ 8.8\pm 0.5 $$ to 0 . 7 ± 0 . 1 ∘ $$ 0.7\pm 0.{1}^{\circ } $$ C. CONCLUSION: The PCA-PDF algorithm improved temperature accuracy to <1°C during periodic motion, but was not able to adequately address sporadic motion. By augmenting the PCA-PDF algorithm, temperature SD during large sporadic motion was also reduced to <1°C, greatly improving the original PCA-PDF algorithm.

In-hospital Morbidity and Mortality After Modified Blalock-Taussig-Thomas Shunts.

BACKGROUND: The modified Blalock-Taussig-Thomas shunt is a critically important palliation for patients with insufficient pulmonary blood flow associated with congenital heart disease. After creating a modified Blalock-Taussig-Thomas shunt patients experience high rates of early postoperative morbidity and mortality. METHODS: This is a single-institution retrospective cohort study. A query of The Society of Thoracic Surgeons database identified relevant patients whose health records were manually queried for echocardiography and operative reports. Patients with ductal-dependent systemic circulation were excluded. Primary outcomes were early serious adverse events and in-hospital mortality. Secondary outcomes were time to primary outcomes and postoperative lengths of stay. We investigated the correlation of demographics, presence of competitive pulmonary blood flow, and surgical and anatomic factors on outcomes. RESULTS: After exclusions our cohort comprised 155 patients. Thirty-three patients (21.3%) experienced an early serious adverse event, 10 (6.5%) early shunt malfunction, and 11 (7.1%) in-hospital mortality. Smaller shunt size, smaller shunted pulmonary artery size, surgical approach, and site of proximal shunt anastomosis were independently associated with morbidity and mortality. CONCLUSIONS: Anatomic elements imparting increased resistance along the modified Blalock-Taussig-Thomas shunt predispose to increased morbidity and mortality, particularly in the early postoperative period. Despite the significant heterogeneity of patients receiving such shunts, similar risk profiles are observed regardless of lesion or presence of competitive flow. A surgical approach using thoracotomy with shunt anastomosis to the subclavian artery, where feasible, results in the subclavian artery as the point of natural resistance, allowing for placement of larger shunts and yielding lower morbidity and mortality.

Childhood malnutrition within the indigenous Wayuú children of northern Colombia.

Malnutrition contributes to nearly half of all preventable deaths in children under the age of five. While the burden of disease is heaviest in Sub-Saharan Africa, South, and Southeast Asia, malnutrition in Latin America remains high, especially within indigenous communities. This study evaluates the prevalence of malnutrition and its relationship with access to healthcare resources within 172 indigenous Wayuú communities in La Guajira, Colombia. Healthcare workers administered a health questionnaire and collected anthropometric measurements on all children 6 months to 5 years of age within the Wayuú households. These data were utilised to calculate the prevalence of acute malnutrition, stunting, and underweight. Of all surveyed Wayuú children, 22.9% and 18.3% met criteria for moderate and severe malnutrition, 33.4% and 28.1% met criteria for moderate and severe stunting, and 28.1% and 16.6% were moderately and severely underweight. Across all categories, malnourished children were older, less likely to have had a medical professional present at birth, less likely to have received medical care after birth, and more likely to have been born in a non-medical, community setting. The prevalence of malnutrition is much higher than national levels in Colombia. This population requires urgent assistance to address their disproportionately high rates of malnutrition.

Incidentally identified genetic variants in arrhythmogenic right ventricular cardiomyopathy-associated genes among children undergoing exome sequencing reflect healthy population variation.

BACKGROUND: With expanding use of clinical whole exome sequencing (WES), genetic variants of uncertain significance are increasingly identified. As pathologic mutations in genes associated with arrhythmogenic right ventricular cardiomyopathy (ARVC) carry a risk of sudden death, determining the diagnostic relevance of incidentally identified variants associated with these genes is critical. METHODS: WES variants from a large, predominantly pediatric cohort (N = 7,066 probands) were obtained for nine ARVC-associated genes (Baylor Miraca). For comparison, a control cohort was derived from the gnomAD database and an ARVC case cohort (N = 1,379 probands) was established from ARVC cases in the literature. Topologic mapping was performed and signal-to-noise analysis was conducted normalizing WES, or case variants, against control variant frequencies. Retrospective chart review was performed of WES cases evaluated clinically (Texas Children's Hospital). RESULTS: Incidentally identified variants occurred in 14% of WES referrals and localized to genes which were rare among ARVC cases yet similar to controls. Amino acid-level signal-to-noise analysis of cases demonstrated "pathologic hotspots" localizing to critical domains of PKP2 and DSG2 while WES variants did not. PKP2 ARM7 and ARM8 domains and DSG2 N-terminal cadherin-repeat domains demonstrated high pathogenicity while normalized WES variant frequency was low. Review of clinical data available on WES referrals demonstrated none with evidence of ARVC among variant-positive individuals. CONCLUSIONS: Incidentally identified variants are common among pediatric WES testing with gene frequencies similar to "background" variants. Incidentally identified variants are unlikely to be pathologic.

Fulminant Haemophilus Influenzae Type a Infection in a 4-year-old with Previously Undiagnosed Asplenic Heterotaxy.

The post Hib-vaccine era has resulted in increased cases of invasive, non-Hib, Haemophilus influenzae infections. One of the encapsulated species, type a, displays the most similarity in virulence to type b. Our patient, a fully vaccinated 4-year-old female, with newly diagnosed asplenia in the setting of heterotaxy, had a prolonged hospital course with disseminated Haemophilus influenzae type a infection.