Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome.

We performed a secondary analysis of the Pediatric Heart Network (PHN) Marfan Trial public-use database to evaluate associations between extracardiac features and cardiac and aortic phenotypes in study participants. Aortic aneurysm phenotype was defined as aortic root Z-score ≥4.5, aortic root growth rate ≥75th percentile, aortic dissection, and aortic surgery. Severe cardiac phenotype was defined as aortic dissection, aortic Z-score ≥4.5, aortic valve surgery, at least moderate mitral regurgitation, mitral valve surgery, left ventricular dysfunction, or death. Extracardiac manifestations were characterized by specific organ system involvement and by a novel aggregate extracardiac score (AES) that was created for this study based on the original Ghent nosology. Mixed effects logistic regression analysis compared AES and systems involvement to outcomes. Of 608 participants (60% male), the median age at enrollment was 10.8 years (interquartile range: 6, 15.4). Aortic aneurysm phenotype was observed in 71% of participants and 64% had severe cardiac phenotype. On univariable analysis, skeletal (OR: 1.95, 95% CI: 1.01, 3.72; p = 0.05), skin manifestation (OR: 1.62, 95% CI: 1.13, 2.34; p = 0.01) and AES (OR: 1.17, 95% CI: 1.02, 1.34; p = 0.02) were associated with aortic aneurysm phenotype but were not significant in multivariable analysis. There was no association between extracardiac manifestations and severe cardiac phenotype. Thus, the severity of cardiac manifestations in Marfan syndrome (MFS) was independent of extracardiac phenotype and AES. Severity of extracardiac involvement did not appear to be a useful clinical marker for cardiovascular risk-stratification in this cohort of children and young adults with MFS.

Extracardiac Manifestations Fail to Predict the Severity of Cardiac Phenotype in Children and Young Adults with Marfan Syndrome.

We performed a secondary analysis of the Pediatric Heart Network Marfan Trial public-use database to evaluate associations between extracardiac features and cardiac and aortic phenotypes in study participants. Aortic aneurysm phenotype was defined as aortic root Z-score ≥ 4.5, aortic root growth rate ≥ 75th percentile, aortic dissection, and aortic surgery. Severe cardiac phenotype was defined as aortic dissection, aortic Z-score ≥4.5, aortic valve surgery, at least moderate mitral regurgitation, mitral valve surgery, left ventricular dysfunction, or death. Extracardiac manifestations were characterized by specific organ system involvement and by a novel aggregate extracardiac score that was created for this study based on the original Ghent nosology. Logistic regression analysis compared aggregate extracardiac score and systems involvement to outcomes. Of 608 participants (60% male), the median age at enrollment was 10.8 years (interquartile range: 6, 15.4). Aortic aneurysm phenotype was observed in 71% of participants and 64% had severe cardiac phenotype. On univariate analysis, skeletal (OR: 1.95, 95% CI: 1.01, 3.72; p = 0.05), skin manifestation (OR: 1.62, 95% CI: 1.13, 2.34; p = 0.01) and aggregate extracardiac score (OR: 1.17, 95% CI: 1.02, 1.34; p = 0.02) were associated with aortic aneurysm phenotype but were not significant in multivariate analysis. There was no association between extracardiac manifestations and severe cardiac phenotype. Thus, the severity of cardiac manifestations in Marfan syndrome was independent of extracardiac phenotype and aggregate extracardiac score. Severity of extracardiac involvement did not appear to be a useful clinical marker for cardiovascular risk-stratification in this cohort of children and young adults with Marfan syndrome.

COVID-19 vaccine triggered autoimmune hepatitis: case report.

Autoimmune hepatitis (AIH) is a non-contagious, chronic, inflammatory autoimmune disease in which one's own immune system attacks healthy, normal hepatic cells. The exact cause of AIH is unknown; however, the combination of genetic, environmental (eg, drugs and natural infection) and immunological factors may lead to AIH. AIH may also be potentiated with the use of vaccines: this case reports one such event following immunisation, along with 1 year of follow-up. A female patient in her late 20s presented to the hospital with yellowish discolouration of eyes, urine and stools. Her medical history revealed that she had been vaccinated with the first dose of a COVID-19 vaccine 10 days earlier. She had a history of asymptomatic COVID-19 infection 3 months ago and a history of chronic analgesic consumption for migraine. She was diagnosed as having AIH through extensive clinical and laboratory workup. This case may be an immediate enhancement of a hidden autoimmune disorder triggered by the vaccination. This adverse event following immunisation has an adequate temporal relationship with her COVID-19 vaccine. The causality can be categorised as 'indeterminate' and may be considered as a potential signal following COVID-19 vaccination.

Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning. AIM: To evaluate the role of cardiac computed tomography (CT) in this setting. METHODS: Retrospective review of 13 infants who underwent TAPVC repair associated with SV from May 2016 to October 2021. Anatomy, incidence, and mechanisms of pulmonary venous obstruction (PVO) were described. Cardiac CT diagnostic yield was compared to echocardiography (echo). RESULTS: Of 13 infants, median age and weight were 24 days (range 2-303 days) and 3.2 (range 2.6-9.1) kg, 8 (62%) were male, 4 (31%) premature, and 11 (85%) had heterotaxy syndrome. All infants had pre- and postoperative echo; 13 had preoperative and 8 (62%) had postoperative cardiac CT. Type of TAPVC: six (46%) supracardiac, two (15%) intracardiac, one (8%) infracardiac, and four (31%) mixed, with pulmonary veins draining in >1 confluence in nine (69%). PVO was present in 6/13 (46%) preoperatively and 5/13 (31%) postoperatively. Mechanisms of PVO: 9/11 (82%) stenosis, 1/9 (9%) membrane formation, and 1/9 (9%) external compression. The sensitivity to diagnose PVO was 45.5% for echo and 100% for cardiac CT, the specificity was 100% for both. No discrepancy was found between cardiac CT and intraoperative findings, but echo had a complete preoperative diagnosis in 1/13 (8%) (p < .00001, Fisher exact test). CONCLUSIONS: Cardiac CT is essential to evaluate pre- and postoperative TAPVC in SV for surgical decision making and long term follow up.

Pre- and post-operative cardiovascular CT in Stage I single ventricle palliation.

OBJECTIVES: The aim of this study is to describe clinical utility of low dose cardiac computed tomography (CT) in the evaluation of single ventricle physiology before and after Stage I palliation. BACKGROUND: Despite the increased utilization of CT imaging and advancement of CT technology, there are limited studies describing the routine clinical use of cardiac CT and radiation dose parameters in the single ventricle Stage I palliation. METHODS: This single center, retrospective study included 57 infants with single ventricle physiology who underwent cardiac CT scans between January 1, 2016 and November 30, 2020. Patients' demographic information, diagnosis, indication, total dose length product (DLP), computed tomographic dose index volume (CTDIvol), cardiac CT findings and intraoperative or intraprocedural findings were reviewed. Estimated effective radiation dose was calculated using a previously published conversion rate. RESULTS: The studies were performed using different generations of CT scanners over the 4 years period: Somatom AS 128, Somatom definition edge, Somatom Force (Siemens Medical Solutions). The studies performed with dual source scanner with prospective gated technique have lower radiation dose exposure with median effective radiation dose of 0.32 mSv. CONCLUSION: Pre- and post-operative cardiovascular CT in Stage I single ventricle palliation using newer generation scanners with prospective gated technique can be done with minimal radiation exposure and good image quality. Cardiac CT is a powerful imaging modality for better management planning in this group of patients.

Adverse events following COVID-19 vaccination: first 90 days of experience from a tertiary care teaching hospital in South India.

BACKGROUND: The COVID-19 vaccination program was introduced in India on 16 January 2021. The Government-issued fact sheet was the only source of information regarding Adverse Events Following Immunizations (AEFIs) for these vaccines. The objective of this study was to assess the AEFIs reported following COVID-19 vaccination in a tertiary care teaching hospital. MATERIALS AND METHODS: The spontaneous reporting method was used for data collection for a period of 3 months. A data collection form was designed to collect the data from the study population who reported adverse events. Collected data were analyzed and categorized by severity and seriousness. The causality assessment team performed causality assessment of the AEFIs using the World Health Organization's causality assessment algorithm. RESULTS: A total of 11,656 doses of COVID-19 vaccine were administered at the study site during the study period, of which 9292 doses were COVISHIELD™ and 2364 doses were COVAXIN™. In all, 445 AEFIs were reported from 269 subjects with an incidence rate of 3.48%. The majority of the subjects with AEFIs belonged to the age group of 18-45 years. Out of the total 445 AEFIs, 418 AEFIs were expected as per the fact sheets, 409 with COVISHIELD™ and 9 with COVAXIN™. Most of the AEFIs [62.02% (n = 276)] were observed at the system organ class of 'General disorders and administration site conditions'. After the causality assessment, out of 433 AEFIs to COVISHIELDTM vaccine, 94.22% (n = 408) of events were categorized to have 'consistent causal association with immunization'. Out of 12 adverse events following COVAXIN™, 8 (66.66%) events were categorized as 'consistent causal association with immunization'. All of them recovered from their adverse events without any sequelae. CONCLUSION: Spontaneous reporting is one of the cheapest methods that can be used for the reporting of AEFI. This method helps health care professionals to identify rare events and potential signals.

Operative treatment for tricuspid valve endocarditis in a premature neonate.

Infective endocarditis is a significant cause of morbidity and mortality in hospitalized patients, especially with the increasing use of indwelling catheters in critically ill children. Surgical excision is sometimes essential to relieve valvar dysfunction and reduce burden of infection. Here we present a preterm infant who developed refractory infective endocarditis and right heart failure with tricuspid valve vegetation likely related to an indwelling umbilical venous catheter. Infective endocarditis resolved after resection of the vegetation and tricuspid valve repair, followed by a 6-week course of antibiotics.

Management of the Adult with Arterial Switch.

Dextro-transposition of the great arteries (d-TGA) is a lethal congenital heart defect in which the great arteries-the pulmonary artery and aorta-are transposed to create ventriculoarterial discordance. Corrective surgical interventions have resulted in significant improvements in morbidity and mortality for this once-fatal congenital heart defect. The initial palliative surgery for d-TGA was the atrial switch operation, which provided physiological correction. The Mustard and Senning "atrial switch" procedures, in which an atrial baffle is created to produce a discordant atrioventricular connection on the existing discordant ventriculoarterial connection, showed preliminary success for the correction of d-TGA. However, follow-up evaluations demonstrated increasing complications from the right ventricle utilized as a systemic ventricle, resulting in progressive right ventricular dysfunction. Thus, the search continued for an anatomical correction of d-TGA to return the great arteries to their normal ventricular connections. The arterial switch operation (ASO), though attempted and theorized by many, was first successfully performed by Dr. Jatene and colleagues in 1975. For ASO, the distal main pulmonary artery and the distal ascending aorta are transected and then anastomosed to their respective ventricles with relocation of the coronary arteries to the neoaorta. The ASO has replaced the atrial switch operation since the 1980s and is now the standard surgical correction for d-TGA. As more patients who have undergone ASO are living into adulthood, late complications of this procedure have become more evident. The most common late postoperative complications include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency, and neopulmonic stenosis. Adults who have undergone ASO in childhood will need follow-up with surveillance imaging and evaluation of new symptoms or declining function to prevent and manage late postoperative complications. This review describes the management strategies for common late complications in patients who have undergone ASO.