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Atrial Septal Defect Device Closure in Patients With Metabolic or Genetic Diseases Publisher



Zanjani KS1 ; Heidari M2 ; Nazari N3 ; Alimadadi H4 ; Mohsenipour R5
Authors
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Authors Affiliations
  1. 1. Cardiology Division, Pediatric Department, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Science, Tehran, Iran
  2. 2. Myelin Disorders Clinic, Department of Pediatric Neurology, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Science, Tehran, Iran
  3. 3. Anesthesiology Division, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Science, Tehran, Iran
  4. 4. Pediatric Gastroenterology and Hepatology Research Center, Tehran University of Medical Science, Tehran, Iran
  5. 5. Growth and Development Research Center, Children’s Medical Center, Pediatrics Center of Excellence, Tehran University of Medical Science, Tehran, Iran

Source: Iranian Journal of Pediatrics Published:2023


Abstract

Background: The coexistence of a metabolic or genetic disease can complicate the course of an atrial septal defect device closure. Methods: The database of our hospital was searched for patients who had undergone atrial septal defect (ASD) device closure and had concurrent metabolic and genetic diseases. Out of 188 such patients, 11 were identified. Results: This cohort study included 11 patients with type 1 diabetes mellitus, insulin resistance, mitochondrial diseases, rickets, Seckel syndrome, Alagille syndrome, cystic fibrosis, Down syndrome, and Crigler-Najjar syndrome type II. The patients were followed for a median of 4 years. Two patients experienced thromboembolic events. One procedure failed as the device was embolized. Large devices with a waist circumference greater than 1.5 times the body weight were used in 3 patients. One patient died 19 days after the procedure due to multi-organ failure, which was not related to device closure. Conclusions: In patients with metabolic or genetic diseases, this procedure may be complicated by factors such as small patient size, hypercoagulation, organ failure (cardiac, renal, or hepatic), vascular abnormalities, and issues with anesthesia or transesophageal echocardiography. It is recommended that careful attention be given to the specific challenges associated with each disease. The utilization of large devices can be considered safe, particularly in patients beyond 4 years of age. © 2023, Sayadpour Zanjani et al.