A uniform strategy of primary repair of tetralogy of Fallot: Transventricular approach results in low reoperation rate in the first decade.

OBJECTIVES: To review outcomes after a uniform strategy of transventricular repair of tetralogy of Fallot. METHODS: A total of 244 consecutive patients underwent transventricular primary repair of tetralogy of Fallot from 2004 to 2019. Median age at operation was 71 days; 57 (23%) patients were premature; 57 (23%) patients had low birth weight (<2.5 kg), and 40 (16%) had genetic syndromes. The diameter of pulmonary valve annulus, right pulmonary artery (PA), and left PA were 6.0 ± 1.8 mm (z score, -1.7 ± 1.3), 4.3 ± 1.4 mm (z score, -0.9 ± 1.2) and 4.1 ± 1.5 mm (z score, -0.5 ± 1.3). RESULTS: Three (1.2%) operative deaths were recorded. Ninety patients (37%) underwent transannular patching. Postoperative echocardiographic peak right ventricular outflow tract gradient decreased from 72 ± 27 mm Hg to 21 ± 16 mm Hg. Median intensive care unit and hospital stay were 3 and 7 days. The survival rate at 10 years was 94.6% ± 1.8%. Reintervention was required 86 times (55 catheter interventions) in 56 patients following tetralogy of Fallot repair. The freedom from all-cause reintervention rate at 10 years was 70.5% ± 3.6%. Cyanotic spells (hazard ratio, 2.14; 95% CI, 1.22-3.90; P < .01) and smaller pulmonary valve annulus z score (hazard ratio, 1.26; 95% CI, 1.01-1.59; P = .04) were associated with increasing risk of all reinterventions. Freedom from redo surgery for right ventricular outflow tract obstruction and right ventricular dilatation at 10 years were, respectively, 85.0% ± 3.1% and 98.7% ± 0.9%. Freedom from valve implantation was 96.7% ± 1.5% at 10 years. CONCLUSIONS: A uniform strategy of primary repair of tetralogy of Fallot through a transventricular approach resulted in low reoperation rate in the first decade. The need of pulmonary valve implantation was limited to <4% at 10 years.

Novel handheld ultrasound technology to enhance non-expert screening for rheumatic heart disease in the Republic of Palau: A descriptive study.

AIM: Non-expert training in rheumatic heart disease (RHD) detection is a valuable strategy in resource-limited settings. Here we present an innovative handheld ultrasound application featuring views of correct probe position, imaging protocol and echocardiographic loops of RHD pathology versus normal, accessible during real-time scanning. METHODS: This prospective study was implemented into a pre-existing school health screening programme at an elementary school in Koror, Palau. Six learners with no prior ultrasound experience were taught a simplified screening protocol in which a mitral regurgitation jet ≥1.5 cm and/or presence of aortic insufficiency were considered a positive screen. All consented children underwent echocardiographic screening by experts with standard portable echocardiography. All positive cases and 25% of negative cases were referred for handheld scans by learners. RESULTS: A total of 26 (4.1%) children were diagnosed with borderline or definite RHD. Mean sensitivity and specificity compared to expert RHD diagnosis over all learners was 71% (standard deviation (SD) 11.3) and 92% (SD 4.9), respectively. For the three highest scanning learners, mean sensitivity was 88% (SD 4.9) and mean specificity was 90% (SD 5.7). For all definite RHD cases, sensitivity was 100% for all but one learner, who achieved sensitivity of 60%. The novel application was used in 229 of 624 (36%) of all handheld scans and 50 of 112 (45%) of expert-diagnosed positive scans, with protocol and reference features most frequently used. Utilisation of the novel application overall decreased per day of scanning per learner. CONCLUSION: Adjunctive handheld ultrasound technology may help ease non-experts into RHD screening.

Prevalence of group A ß-hemolytic streptococcal throat carriage and prospective pilot surveillance of streptococcal sore throat in Ugandan school children.

OBJECTIVES: Group A ß-hemolytic Streptococcus (GAS), also known as Streptococcus pyogenes, is responsible for an annual 600 million cases of acute pharyngitis globally, with 92% of those infections occurring in low-resource settings. Further knowledge of the acute streptococcal pharyngitis burden in low-resource settings is essential if serious post-streptococcal complications - rheumatic fever (RF) and its long-term sequel rheumatic heart disease (RHD) - are to be prevented. METHODS: Two studies were conducted in school-aged children (5-16 years): a cross-sectional study of streptococcal pharyngeal carriage followed by a prospective cohort study of streptococcal sore throat over 4 weeks from March to April 2017. RESULTS: The cross-sectional study revealed an overall prevalence of GAS carriage of 15.9% (79/496, 95% confidence interval 12.8-19.5%). Among 532 children enrolled in the prospective cohort study, 358 (67%) reported 528 sore throats, with 221 (41.1%) experiencing at least one GAS-positive sore throat. The overall GAS-positive rate for sore throat was 41.8% (221/528). CONCLUSIONS: The GAS pharyngeal carriage rates seen in Uganda (15.9%, 95% confidence interval 12.8-19.5%) are higher than the most recent pooled results globally, at 12% (range 6-28%). Additionally, pilot data suggest a substantially higher percentage of sore throat that was GAS-positive (41.8%) compared to pooled global rates when active recruitment is employed.

Prevalence, Clinical Features and Antibiotic Susceptibility of Group A Streptococcal Skin Infections in School Children in Urban Western and Northern Uganda.

BACKGROUND: Group A Streptococcus (GAS) skin infections can lead to invasive sepsis, poststreptococcal glomerulonephritis, and potentially rheumatic heart disease (RHD). Within a study to identify predisposing factors of RHD in Ugandan schoolchildren, we determined the prevalence of skin infections and assessed the clinical features and antibiotic susceptibility of GAS skin infection. METHODS: Cross-sectional study conducted at 3 urban primary schools in Western and Northern Uganda in March 2017. A dermatologist rendered clinical diagnoses and obtained a skin swab specimen from lesions with signs of bacterial infection. Beta-hemolytic colonies underwent Lancefield grouping, species identification by polymerase chain reaction and antimicrobial susceptibility testing. RESULTS: From 3265 schoolchildren, we observed 32% with ≥1 fungal, 1.8% with ≥1 bacterial, 0.9% with ≥1 viral, and 0.2% with ≥1 ectoparasitic infection. Of 79, 25 (32%) specimens were GAS-positive, of which one-third demonstrated tetracycline resistance. Of 17 impetigo cases, 13 (76%) were located on the leg/foot and 3 (18%) on the head/neck. Prevalence of GAS skin infection was 0.8% (25 of 3265). In Northern Uganda, where subclinical definite RHD prevalence is 1.1%, GAS skin infection prevalence was 1.2% (4 of 343) and 0.9% (3 of 352). CONCLUSION: This study identifies tetracycline-resistant GAS in Ugandan communities, suggests modified skin examination of exposed anatomic locations may be appropriate for population-based GAS skin infection studies, and underscores need for clear case definitions of GAS skin infection. Future studies are needed to evaluate the role of GAS skin infection in development of RHD in Ugandan communities.

Decreased Prevalence of Rheumatic Heart Disease Confirmed Among HIV-positive Youth.

BACKGROUND: There is geographical overlap between areas endemic for rheumatic heart disease (RHD) and those endemic for HIV. A recent pilot study demonstrated that children living with HIV might be at less risk for RHD development; however, the sample size was too small to make definitive conclusions. Our objective was to determine the prevalence of RHD among HIV-positive children in Uganda. METHODS: We conducted a prospective, cross-sectional study of HIV-positive children (5-15 years of age) receiving care at the Baylor Uganda HIV Clinic, Kampala, Uganda. A focused echocardiogram and chart review was performed. A sample size of 988 children was needed to provide 80% power to detect a difference in population prevalence between HIV-positive children and the general population, 2.97% [95% confidence interval (CI): 2.70-3.24%], based on previous reports. RESULTS: Screening echocardiography of 993 HIV-positive children found 15 individuals (1.5%; 95% CI: 0.88%-2.54%) with RHD. Of these 15, 2 were classified as definite RHD and 13 as borderline RHD. The majority of children had isolated mitral valve disease (93%). Children found to have RHD were older than those without RHD, 12 versus 10 years of age (P = 0.004). When separated based on geographic location, the prevalence of RHD among HIV-positive children from Kampala was 1.28% (95% CI: 0.63%-2.51%) compared with 2.1% (95% CI: 0.89%-4.89%) in those from outside Kampala. CONCLUSIONS: Children living with HIV have a lower prevalence of RHD than the general pediatric population. Further studies are needed to explore this protective association.

Intervening for RPA stenosis following Waterston shunt: Importance of anatomical definition of the coronary arteries.

Right pulmonary artery (RPA) stenosis following Waterston shunt is common. We report a case of RPA stenosis many years following tetralogy of Fallot repair with take-down of a Waterston shunt and demonstrate an unusual and important anatomic variant of proximity of the left coronary artery to the mid-portion of the RPA.

Cold tolerance, cold-induced hyperphagia, and nonshivering thermogenesis are normal in α1-AMPK-/- mice.

Recent studies indicate that a substantial amount of metabolically active brown adipose tissue (BAT) exists in adult humans. Given the unique ability of BAT to convert calories to heat, there is intense interest in understanding the regulation of BAT metabolism in hopes that its manipulation might be an effective way of expending excess calories. Because of the established role of AMP-activated protein kinase (AMPK) as a "metabolic master switch" and its extremely high levels of activity in BAT, it was hypothesized that AMPK might play a central role in regulating BAT metabolism. To test this hypothesis, whole body α(1)-AMPK(-/-) (knockout) and wild-type mice were studied 1) under control (room temperature) conditions, 2) during chronic cold exposure (14 days at 4°C), and 3) during acute nonshivering thermogenesis (injection of a ß(3)-adrenergic agonist). Under control conditions, loss of α(1)-AMPK resulted in downregulation of two important prothermogenic genes in BAT, thyrotropin-releasing hormone (-9.2-fold) and ciliary neurotrophic factor (-8.7-fold). Additionally, it caused significant upregulation of α(2)-AMPK activity in BAT, white adipose tissue, and liver, but not cardiac or skeletal muscle. During acute nonshivering thermogenesis and chronic cold exposure, body temperature was indistinguishable in the α(1)-AMPK(-/-) and wild-type mice. Similarly, the degree of cold-induced hyperphagia was identical in the two groups. We conclude that α(1)-AMPK does not play an obligatory role in these processes and that adaptations to chronic loss of α(1)-AMPK are able to compensate for its loss via several mechanisms.