Necrotizing enterocolitis (NEC) is a relatively rare but devastating entity associated classically with the preterm cohort in the neonatal intensive care unit. Preterm and term babies with congenital heart disease are at risk of a number of comorbidities because of the hemodynamic derangements due to a structurally abnormal heart and the corrective procedures adopted. Necrotizing enterocolitis is one of the dreaded complications associated with this cohort and impacts the course of these babies in the hospital in a major way. A large majority of term babies with NEC are in the backdrop of a significant congenital cardiac lesion. This review article summarizes the literature and elaborates this entity including its specific features, risk factors associated with its causality, histopathology and related aspects of hemodynamics, and feeding in this vulnerable population. It also provides insight into modifiable risk factors and early markers of detection of gut necrosis to facilitate prevention and early detection. It highlights the subtle but definite difference in outcome variables to help physicians enable the parents of babies with heart disease to develop a better understanding of the entity and its expected course while counseling.
Arterial tortuosity syndrome (ATS) is a rare, autosomal recessive, connective tissue disorder. It predominantly involves the arterial tree with clinical features reflecting the systems involved. There have been few cases of ATS suspected during antenatal screening ultrasound in high-risk families, but none confirmed. We present the first case of ATS confirmed antenatally in the fetus with cascade testing, detecting the disease in the mother as well.
Accelerated idioventricular rhythm (AIVR) is an uncommon but benign rhythm, seen most commonly in children. It is associated with reperfusion injury after myocardial infarction in adults. In children, it is usually seen as an idiopathic finding in the absence of heart disease. We present a case of AIVR in an adolescent associated with acute presentation of inflammatory bowel disease. Prompt treatment of the systemic inflammation led to the remission of both inflammatory bowel disease and AIVR. This report emphasizes the diverse causes of AIVR in children and our limited understanding of its pathophysiology. Treatment of the underlying condition resolved the arrhythmia.
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