Atrial septal defect closure as second-line therapy in refractory heart failure: a successful case report in a 6-year-old child with phosphoglucomutase 1 deficiency (PGM1-CDG)
These videos have been matched automatically. Contact us if they are not relevant.
These videos have been matched automatically. Contact us if they are not relevant.
View abstract on PubMed
Summary
This summary is machine-generated.Atrial septal defect (ASD) closure can be a life-saving option for patients with impaired left ventricular compliance. Percutaneous closure using a fenestrated device successfully treated a child with heart failure due to ASD and myocarditis.
Area Of Science
- Cardiology
- Pediatrics
- Congenital Heart Disease
Background
- Atrial septal defect (ASD) is a common congenital heart defect.
- ASD typically remains asymptomatic in childhood but can cause heart failure when left ventricular (LV) compliance is impaired.
Purpose Of The Study
- To report a case of successful percutaneous ASD closure in a child with CDG-PGM1 and heart failure.
- To highlight ASD closure as a therapeutic option in selected patients with impaired LV compliance.
Main Methods
- A 6-year-old boy with CDG-PGM1 and a large ASD presented with acute refractory heart failure.
- He underwent a balloon occlusion test followed by percutaneous closure of the ASD using a hand-made fenestrated device.
Main Results
- The percutaneous ASD closure was successful, leading to rapid weaning from inotropes and full clinical recovery.
- At 6-month follow-up, LV function normalized, and the patient remained asymptomatic.
Conclusions
- ASD closure can be a life-saving therapeutic option in selected patients with impaired LV compliance.
- Hemodynamic evaluation with balloon occlusion testing is crucial for assessing closure feasibility and device selection.
These videos have been matched automatically. Contact us if they are not relevant.
These videos have been matched automatically. Contact us if they are not relevant.
Related Concept Videos
Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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...
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...
Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Mitral regurgitation (MR) is characterized by retrograde blood circulation from the left ventricle into the left atrium due to inadequate mitral valve closure. The severity of the condition, symptoms, and underlying cause determine treatment strategies.Monitoring and Pharmacological TreatmentPatients with mild to moderate MR typically do not need immediate intervention but regular monitoring to assess progression and guide treatment. Patients with mild MR should have an echocardiogram every 3-5...