Intestinal obstruction caused by malrotation of the gut in atrial isomerism

Area of Science:

• Pediatric Cardiology
• Pediatric Surgery
• Medical Genetics

Background:

• Atrial isomerism encompasses a spectrum of congenital conditions characterized by abnormalities in the development of the heart and other organs.
• Intestinal malrotation is a known complication associated with certain congenital syndromes, including those involving atrial isomerism.
• Historically, extracardiac anomalies in these syndromes, apart from asplenia, have been underestimated in their impact on surgical outcomes.

Purpose of the Study:

• To highlight the significant risk of intestinal obstruction in children with atrial isomerism due to gut malrotation.
• To emphasize the importance of early gastrointestinal symptom evaluation in patients with atrial isomerism.
• To underscore the need for prompt surgical intervention for intestinal complications in this population.

Main Methods:

• Case series review of pediatric patients diagnosed with atrial isomerism.
• Analysis of clinical presentation, diagnostic findings, and surgical outcomes for intestinal obstruction.
• Correlation of cardiac status and extracardiac anomalies with gastrointestinal complications.

Main Results:

• Five pediatric patients with atrial isomerism presented with intestinal obstruction secondary to malrotation.
• The surgical outcomes for intestinal obstruction in this cohort were noted to be poor.
• Asplenia was the most frequently recognized extracardiac anomaly, while others were often overlooked.

Conclusions:

• Intestinal obstruction due to malrotation is a critical concern in children with atrial isomerism.
• Improved cardiac management necessitates increased focus on early detection and intervention for gastrointestinal symptoms.
• Proactive gastrointestinal assessment and timely surgical management are vital for improving patient outcomes in atrial isomerism syndromes.