Related Experiment Video

Updated: Sep 16, 2025

Implantation of Total Artificial Heart in Congenital Heart Disease
07:27

Implantation of Total Artificial Heart in Congenital Heart Disease

Published on: July 18, 2014

24.8K

Management strategy for congenital hyperinsulinism with atrial septal defect and diazoxide-induced pulmonary

Sayuri Koizumi1, Ikuma Musha1,2, Koichi Toda3

  • 1Department of Pediatrics, Saitama Medical University Hospital, Saitama, Japan.

Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology
|July 10, 2025
PubMed

Insights

Congenital hyperinsulinism (CHI) treatment with diazoxide can cause pulmonary hypertension (PH). This case study shows surgically closing an atrial septal defect enabled safe diazoxide reintroduction, achieving good glycemic control and managing PH.

Area of Science:

  • Pediatric Endocrinology
  • Cardiology
  • Medical Case Reports

Background:

  • Congenital hyperinsulinism (CHI) is a condition of excessive insulin secretion causing hypoglycemia.
  • Diazoxide is the primary medical treatment for CHI but can induce pulmonary hypertension (PH).

Observation:

  • A 2-month-old infant with CHI and an atrial septal defect (ASD) developed dose-dependent PH while on diazoxide.
  • Alternative medical management proved insufficient for stable glycemic control.
  • Surgical closure of the ASD was performed to facilitate safer diazoxide use.

Findings:

  • The combined approach of surgical ASD closure, diazoxide therapy, and anti-PH medication successfully managed PH.
  • This strategy also achieved stable blood glucose levels in the patient.

Implications:

  • This case demonstrates a novel strategy for managing CHI patients with co-existing cardiac conditions.
  • Surgical intervention for cardiac defects may allow for the safe reintroduction of essential medications like diazoxide.
  • This approach offers a potential alternative to more invasive treatments like pancreatic resection.

Keywords:
atrial septal defectcongenital hyperinsulinismdiazoxidepulmonary hypertension

More Related Videos

Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China08:42

Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China

Published on: February 11, 2022

3.6K
Induction of Right Ventricular Failure by Pulmonary Artery Constriction and Evaluation of Right Ventricular Function in Mice09:40

Induction of Right Ventricular Failure by Pulmonary Artery Constriction and Evaluation of Right Ventricular Function in Mice

Published on: May 13, 2019

10.7K

Related Concept Videos

Mitral Stenosis III: Medical Management01:26

Mitral Stenosis III: Medical Management

Mitral stenosis, a condition marked by the narrowing of the mitral valve, necessitates an integrated approach for effective management. This approach includes preventative measures, medical therapy, and surgical interventions to reduce symptoms and prevent complications.PreventionPrevention of mitral stenosis primarily focuses on reducing the incidence of bacterial infections, particularly streptococcal infections, which can lead to rheumatic fever and subsequent valvular damage. Timely...
28
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
38
Heart Failure V: Medical Management01:30

Heart Failure V: Medical Management

Medical Management of Acute Decompensated Heart Failure (ADHF)The primary goals of therapy for patients hospitalized with acute decompensated heart failure (ADHF) include:Relieving symptomsOptimizing volume statusSupporting oxygenation and ventilationMaintaining cardiac output (CO) and end-organ perfusionIdentifying and addressing the cause of ADHFPreventing complicationsProviding patient education on factors precipitating HF exacerbationPlanning for dischargeOngoing monitoring and assessment...
29
Mitral Stenosis IV: Nursing Management01:27

Mitral Stenosis IV: Nursing Management

A comprehensive nursing assessment is essential for patients with valvular heart disease, which involves any dysfunction of the heart valves that could impact blood flow and overall heart function.Subjective Data Collection:Chief Complaint and Present Illness: Start with the patient's primary concerns, focusing on the onset, duration, and progression of cardiac symptoms such as dyspnea, fatigue, chest pain, and palpitations.Past Medical History: Collect detailed information on any previous...
49
Cardiomyopathy VII: Pre and Post Operative Nursing Management01:28

Cardiomyopathy VII: Pre and Post Operative Nursing Management

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular outflow tract (LVOT) obstruction who remain symptomatic despite optimal medical therapy may undergo a septal myectomy (Morrow procedure). This procedure involves excising a portion of the hypertrophied septum below the aortic valve using a heart-lung machine to improve blood flow through the LVOT. Effective preoperative and postoperative nursing management ensures successful patient outcomes, minimizes complications, and...
29
Heart Failure VI: Adjunct Therapies01:22

Heart Failure VI: Adjunct Therapies

Additional therapies for treating patients with heart failure (HF) may include procedural interventions, supplemental oxygen, the management of sleep disorders, and nutritional therapy.Procedural InterventionsImplantable Cardioverter-Defibrillator: For patients at risk of life-threatening arrhythmias due to severe left ventricular dysfunction, an Implantable Cardioverter-Defibrillator (ICD) can detect and terminate these arrhythmias, preventing sudden cardiac death and improving survival rates.
36