Diffuse myocardial fibrosis is uncommon in people with perinatally acquired human immunodeficiency virus infection.

BACKGROUND: Cardiovascular disease (CVD) remains a leading cause of death in people living with HIV. Myocardial fibrosis is well-described in HIV infection acquired in adulthood. We evaluate the burden of fibrosis by cardiac magnetic resonance in people with perinatal HIV infection. METHODS: Individuals with perinatally acquired HIV (pnHIV) diagnosed before 10 years-old and on antiretroviral treatment for ≥ 6 months were matched with uninfected controls. Patients with significant cardiometabolic co-morbidities and pregnancy were excluded. Diffuse fibrosis was assessed by cardiac magnetic resonance (CMR) with native T1 mapping for calculation of extracellular volume fraction (ECV). Viability was assessed with late gadolinium enhancement. The normality of fibrosis was assessed using the Komogrov-Smirnov test. Fibrosis between the groups was analyzed using a Mann-Whitney U test, as the data was not normally distributed. Statistical significance was defined as a p-valve < 0.05. RESULTS: Fourteen adults with pnHIV group and 26 controls (71% female and 86% Black race) were assessed. The average (± standard deviation) age in the study group was 29 (± 4.3) years-old. All pnHIV had been on ART for decades. Demographic data, CMR functional/volumetric data, and pre-contrast T1 mapping values were similar between groups. Diastolic function was normal in 50% of pnHIV patients and indeterminate in most of the remainder (42%). There was no statistically significant difference in ECV between groups; p = 0.24. CONCLUSION: Perinatally-acquired HIV was not associated with diffuse myocardial fibrosis. Larger prospective studies with serial examinations are needed to determine whether pnHIV patients develop abnormal structure or function more often than unaffected controls.

Impact of pulmonary stenosis on right ventricular global longitudinal strain in repaired tetralogy of Fallot patients post transcatheter pulmonary valve replacement.

BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Society for Cardiovascular Magnetic Resonance 2020 Case of the Week series.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). Case of the week is a case series hosted on the SCMR website ( https://www.scmr.org ) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. Each case consists of the clinical presentation and a discussion of the condition and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2020 Case of the Week series of 11 cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Coronary artery ectasia in post-pericardiotomy syndrome.

Post-pericardiotomy syndrome (PPS) is a common inflammatory process following cardiac surgery, in which the pericardial space was opened. Pericardial effusion (PE) is a common manifestation in PPS; however, coronary artery dilation is not associated with PPS. Inflammatory vasculitis in children are known to cause coronary dilation, in conditions such as in Kawasaki Disease (KD). We report a patient with PPS and concomitant coronary dilation by transthoracic echocardiography (TTE) following repair of her ventricular septal defect (VSD).

Causes of Death in Infants and Children with Congenital Heart Disease.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code. Patients who could be linked to a NC death certificate between 2008 and 2016 were included. Patients were divided by CHD subtypes (severe, shunt, valve, other). COD was compared between groups. Records of 35,542 patients < 20 years old were evaluated. There were 15,277 infants with an annual mortality rate of 3.5 deaths per 100 live births. The most frequent COD in infants (age < 1 year) were CHD (31.7%), lung disease (16.1%), and infection (11.4%). In 20,265 children (age 1 to < 20 years), there was annual mortality rate of 9.7 deaths per 1000 at risk. The most frequent COD in children were CHD (34.2%), neurologic disease (10.2%), and infection (9.5%). In the severe subtype, CHD was the most common COD. In infants with shunt-type CHD disease, lung disease (19.5%) was the most common COD. The mortality rate in infants was three times higher when compared to children. CHD is the most common underlying COD, but in those with shunt-type lesions, extra-cardiac COD is more common. A multidisciplinary approach in CHD patients, where development of best practice models regarding comorbid conditions such as lung disease and neurologic disease could improve outcomes in this patient population.

Left-sided congenitally unguarded tricuspid valve with congenitally corrected transposition of the great arteries: A rare diagnosis confirmed by three-dimensional echocardiography.

Left-sided unguarded tricuspid valve disease with congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation, only reported a few times in the literature. Two-dimensional echocardiography (2DE) uses standard views to diagnose tricuspid valve disease. Advanced imaging techniques, such as three-dimensional echocardiography, allow for simultaneous visualization of the tricuspid valve annulus and all leaflets. Three-dimensional echocardiography (3DE) may be useful in distinguishing unguarded tricuspid valve orifice from other forms of tricuspid valve disease.

Sedated Echocardiograms Better Characterize Branch Pulmonary Arteries Following Bidirectional Glenn Palliation with Minimal Risk of Adverse Events.

Patients post-bidirectional Glenn (BDG) operation are at risk of left and right pulmonary artery (LPA and RPA) hypoplasia. Transthoracic echocardiograms (TTE) in active children can miss essential elements of anatomy. Procedural sedation improves image quality but increases risk of adverse events. We hypothesized that echocardiograms performed with sedation in patients post-BDG would improve visualization of branch pulmonary arteries with minimal adverse events. Patients post-BDG between 2007-2016 were identified. Exclusion criteria were > 12 months of age, absence of complete TTE before discharge, death before discharge, conversion to shunt physiology, and prolonged post-operative course > 7 weeks. Of 254 post-BDG patients, 153 met inclusion/exclusion criteria. TTE reports were reviewed for visualization of LPA/RPA and hypoplasia of LPA/RPA. Blinded assessment of image quality was performed (scale of 1[poor] to 5[excellent]). Pertinent clinical data were recorded. Pearson's chi-squared and Wilcoxon Rank Sum tests used for statistical analysis. The median age at surgery and hospital stay were 4.8 months and 10 days. Twenty-three patients underwent sedated TTE (15%). Sedated TTE significantly improved visualization of the RPA (100% vs 82%, p = 0.029) and LPA, though this did not reach statistical significance (100% vs 91%, p = 0.129). Sedated TTEs has significantly better image quality (median of 4 vs 3, p < 0.001). There were no serious adverse events due to sedation. Sedated TTE early post-BDG is safe, improves visualization of the RPA and LPA, and improves overall image quality. Routine sedated TTE in these patients should be considered. Implications for long-term outcome need to be further analyzed.

Pediatric Myocarditis.

Myocarditis is a condition caused by acute or chronic inflammation of the cardiac myocytes, resulting in associated myocardial edema and myocardial injury or necrosis. The exact incidence is unknown, but is likely underestimated, with more mild cases going unreported. Diagnosis and appropriate management are paramount in pediatric myocarditis, as it remains a recognized cause of sudden cardiac death in children and athletes. Myocarditis in children is most often caused by a viral or infectious etiology. In addition, there are now two highly recognized etiologies related to Coronavirus disease of 2019 (COVID-19) infection and the COVID-19 mRNA vaccine. The clinic presentation of children with myocarditis can range from asymptomatic to critically ill. Related to severe acute respiratory syndrome-Coronavirus 2 (SARs-CoV-2), children are at greater risk of developing myocarditis secondary to COVID-19 compared to the mRNA COVID-19 vaccine. Diagnosis of myocarditis typically includes laboratory testing, electrocardiography (ECG), chest X-ray, and additional non-invasive imaging studies with echocardiogram typically being the first-line imaging modality. While the reference standard for diagnosing myocarditis was previously endomyocardial biopsy, with the new revised Lake Louise Criteria, cardiac magnetic resonance (CMR) has emerged as an integral non-invasive imaging tool to assist in the diagnosis. CMR remains critical, as it allows for assessment of ventricular function and tissue characterization, with newer techniques, such as myocardial strain, to help guide management both acutely and long term.

Diffuse Myocardial Fibrosis is Uncommon in People with Perinatally Acquired Human Immunodeficiency Virus Infection.

Background: Cardiovascular disease (CVD) remains a leading cause of death in people living with HIV. Myocardial fibrosis is well-described in HIV infection acquired in adulthood. We evaluate the burden of fibrosis by cardiac magnetic resonance in people with perinatal HIV infection. Methods: Individuals with perinatally acquired HIV (pnHIV) diagnosed before 10 years-old and on antiretroviral treatment for ≥ 6 months were matched with uninfected controls. Patients with significant cardiometabolic co-morbidities and pregnancy were excluded. Diffuse fibrosis was assessed by cardiac magnetic resonance (CMR). with native T1 mapping for calculation of extracellular volume fraction (ECV). Viability was assessed with late gadolinium enhancement. The normality of fibrosis was assessed using the Komogrov-Smirnov test. Fibrosis between the groups was analyzed using a Mann-Whitney U test, as the data was not normally distributed. Statistical significance was defined as a p-valve < 0.05. Results: Fourteen adults with pnHIV group and 26 controls (71% female and 86% Black race) were assessed. The average (± standard deviation) age in the study group was 29 (± 4.3) years-old. All pnHIV had been on ART for decades. Demographic data, CMR functional/volumetric data, and pre-contrast T1 mapping values were similar between groups. Diastolic function was normal in 50% of pnHIV patients and indeterminate in most of the remainder (42%). There was no statistically significant difference in ECV between groups; p = 0.24. Conclusion: Perinatally-acquired HIV was not associated with diffuse myocardial fibrosis. Early exposure to ART may be cardioprotective against development of myocardial fibrosis in patients with perinatal HIV.

The Association of Race and Ethnicity with Mortality in Pediatric Patients with Congenital Heart Disease: a Systematic Review.

CONTEXT: Congenital heart disease (CHD) is a common condition with high morbidity and mortality and is subject to racial and ethnic health disparities. OBJECTIVE: To conduct a systematic review of the literature to identify differences in mortality in pediatric patients with CHD based on race and ethnicity. DATA SOURCES: Legacy PubMed (MEDLINE), Embase (Elsevier), and Scopus (Elsevier) STUDY SELECTION: English language articles conducted in the USA focused on mortality based on race and ethnicity in pediatric patients with CHD. DATA EXTRACTION: Two independent reviewers assessed studies for inclusion and performed data extraction and quality assessment. Data extraction included mortality based on patient race and ethnicity. RESULTS: There were 5094 articles identified. After de-duplication, 2971 were screened for title and abstract content, and 45 were selected for full-text assessment. Thirty studies were included for data extraction. An additional 8 articles were identified on reference review and included in data extraction for a total of 38 included studies. Eighteen of 26 studies showed increased risk of mortality in non-Hispanic Black patients. Results were heterogenous in Hispanic patients with eleven studies of 24 showing an increased risk of mortality. Results for other races demonstrated mixed outcomes. LIMITATIONS: Study cohorts and definitions of race and ethnicity were heterogenous, and there was some overlap in national datasets used. CONCLUSION: Overall, racial and ethnic disparities existed in the mortality of pediatric patients with CHD across a variety of mortality types, CHD lesions, and pediatric age ranges. Children of races and ethnicities other than non-Hispanic White generally had increased risk of mortality, with non-Hispanic Black children most consistently having the highest risk of mortality. Further investigation is needed into the underlying mechanisms of these disparities so interventions to reduce inequities in CHD outcomes can be implemented.

An Unusual Association: Total Anomalous Pulmonary Venous Return and Aortic Arch Obstruction in Patients with Cat Eye Syndrome.

Cat eye syndrome (CES) is a rare genetic defect, characterized by iris colobomas, preauricular skin tags, and anal malformations. Affecting 1 in 150,000 people, this defect is caused by duplication or triplication of the proximal long (q) arm of chromosome 22. Congenital heart disease is associated with CES. One of the most common heart defects in patients with CES is total anomalous pulmonary venous return (TAPVR). In this article, we reported patients with a rare association of concomitant TAPVR and aortic arch obstruction: one with interrupted aortic arch and the other with coarctation of the aorta with an aberrant right subclavian artery.

Two new Prenolepis species (Hymenoptera: Formicidae) from Indomalaya and Australasia, with a redescription of P. dugasi from Vietnam.

Prenolepis is a lineage of formicine ants with its center of diversity in the Old World tropics. Three more Prenolepis species are added to the Indomalayan and Australasian fauna and another is synonymized, bringing the total number of Prenolepis species worldwide to 19. Two new species are described: P. nepalensis from Nepal and P. lakekamu from Papua New Guinea, the latter being the first in the genus east of Wallace's Line. Additionally, P. dugasi Forel (comb. rev.) from Vietnam is transferred from Nylanderia and redescribed. Based on morphology, each of the three species appears to be most closely-related to other species found predominantly in or nearest to their respective bioregions: P. nepalensis most resembles P. darlena, P. fisheri, and P. fustinoda; P. lakekamu bears strongest resemblance to P. jacobsoni, P. jerdoni, and P. subopaca; and P. dugasi most resembles P. melanogaster. Descriptions, illustrations and images are provided for all three species. One new synonymy is proposed: P. angulinoda Chen Zhou 2018 = P. fustinoda Williams LaPolla 2016. An updated key for workers of all extant Prenolepis species is also included.

Taxonomic revision and phylogeny of the ant genus Prenolepis (Hymenoptera: Formicidae).

The formicine ant genus Prenolepis is here revised for the first time. Thirteen extant species are recognized of which four are described as new. A key for the worker caste is provided, and the worker of each species is imaged, with males and queens imaged in species where they are known. Worker-based characters were used to construct a species-level phylogeny of Prenolepis. Both maximum parsimony and Bayesian inference methods were used for the phylogenetic analyses. A morphological diagnosis for the genus is provided, with a discussion of useful morphological characters for separating Prenolepis from other genera in the Prenolepis genus-group. Major taxonomic changes are proposed. The new species are: P. darlena, P. fustinoda, P. mediops, and P. shanialena. Prenolepis jerdoni subopaca is elevated to full species. Three species are excluded from Prenolepis and transferred to Nylanderia and Paratrechina as new combinations: N. emmae, N. flaviabdominis, and P. umbra. Two species are excluded from Paratrechina and transferred to Nylanderia and Paraparatrechina as new combinations: N. guanyin and P. kongming. One species, Z. darlingtoni, is excluded from Nylanderia and transferred to Zatania as a new combination. Several synonyms are proposed: Prenolepis sphingthoraxa = Nylanderia flaviabdominis; P. imparis arizonica, P. imparis colimana, P. imparis coloradensis, and P. imparis veracruzensis = P. imparis; P. melanogaster carinifrons and P. nigriflagella = P. melanogaster; P. longiventris and P. magnocula = P. naoroji; and P. septemdenta = Nylanderia opisopthalmia.