What is the medical management of Atrioventricular (AV) canal defect?

From the Guidelines

Medical management of atrioventricular (AV) canal defects should focus on controlling symptoms and preventing complications, with most patients requiring no regular medication in the absence of specific problems, as stated in the ACC/AHA 2008 guidelines 1.

Medical Management

The primary goal of medical management is to stabilize the patient's condition and optimize their status before surgical correction.

• Treatment may include diuretics like furosemide to reduce pulmonary congestion and fluid overload.
• Angiotensin-converting enzyme (ACE) inhibitors such as enalapril may be used to decrease afterload and improve cardiac function in patients with AV valve regurgitation and symptoms of chronic heart failure 1.
• Pulmonary vasodilation therapy may be indicated in patients with pulmonary arterial hypertension (PAH) and no significant left-to-right shunt who are deemed to be at high risk for surgical repair, but this should be approached with caution due to the potential for producing a significant right-to-left shunt 1.

Monitoring and Follow-up

Regular monitoring includes echocardiograms to assess defect progression and pulmonary pressures.

• The timing of surgery depends on symptom severity and the development of pulmonary hypertension, with most repairs performed within the first year of life.
• Nutritional support is crucial, often requiring high-calorie formulas or feeding tubes to ensure adequate growth.
• Respiratory infections must be treated promptly with appropriate antibiotics.
• Infective endocarditis prophylaxis may be recommended before certain procedures, as outlined in the ACC/AHA guidelines 1.

Current Guidelines

The most recent guidelines for the management of adults with congenital heart disease, including AV canal defects, are outlined in the 2018 AHA/ACC guideline 1.

• However, the 2008 ACC/AHA guidelines 1 provide specific recommendations for the medical management of AV canal defects, which should be followed in clinical practice.
• The European Society of Cardiology (ESC) guidelines for the management of grown-up congenital heart disease also provide valuable information on the diagnosis and treatment of AV canal defects 1.

From the Research

Medical Management of AV Canal Defect

• The medical management of AV canal defect is largely focused on surgical intervention, as long-term medical therapy is ineffective 2.
• Surgical options for repair of AV canal defects are dependent on the morphology of the valve and symptoms 3.
• The traditional single-patch technique for repair of complete AV canal requires surgical division of the superior and inferior common leaflets, but selective application of the modified single-patch technique (Australian technique) can minimize manipulation of the AV valve leaflet tissue 4.
• For patients with complete AV canal defect and aortic arch obstruction, a staged approach with arch obstruction repaired first, followed by repair of the complete AV canal defect, may be a reasonable treatment option 5.
• While there is no direct evidence on the medical management of AV canal defect using diuretics, a study on acute heart failure suggests that early administration of intravenous diuretics (e.g., furosemide) within the first hour of presentation can be associated with favorable outcomes 6.

Surgical Considerations

• The risk of corrective surgery for AV canal defect in infancy has decreased due to improvements in surgical techniques, anesthesia, and postoperative management 2.
• Selective application of the single-patch technique can allow for excellent results for surgical repair in the neonatal period or early infancy 4.
• The choice of surgical technique depends on the individual patient's anatomy and symptoms, and may involve a combination of traditional and modified techniques 3, 4.