What are the American guidelines for a 5-year-old boy with congenital perimembranous subaortic Ventricular Septal Defect (VSD) and aortic prolapse with trivial aortic regurgity?

From the Guidelines

For a 5-year-old boy with congenital perimembranous subaortic ventricular septal defect (VSD) with aortic valve prolapse and trivial aortic regurgitation, surgical closure of the VSD is recommended. This approach is based on the most recent guidelines, including the 2018 AHA/ACC guideline for the management of adults with congenital heart disease 1, which emphasizes the importance of managing VSDs, especially those with complications like aortic valve prolapse. Although the guideline primarily focuses on adults, the principles of managing VSDs to prevent long-term complications such as progressive aortic regurgitation, left ventricular volume overload, and potential endocarditis risk are applicable to pediatric patients as well.

Key Considerations

• The presence of aortic valve prolapse with trivial aortic regurgitation in a child with a perimembranous subaortic VSD indicates a need for intervention to prevent progression of aortic valve disease, as noted in the guideline 1.
• Surgical closure of the VSD can help prevent long-term complications, including worsening aortic regurgitation, which may necessitate aortic valve replacement if left untreated.
• The guideline suggests that small restrictive defects may be watched conservatively, but the presence of aortic valve prolapse and the potential for progressive aortic regurgitation justify a more proactive approach 1.
• Regular follow-up with a cardiologist and periodic echocardiography are crucial for monitoring the child's condition post-surgery and addressing any potential issues early.

Management Approach

• Surgical closure of the VSD by a pediatric cardiac surgeon at a center experienced in congenital heart disease is the recommended course of action.
• Pre-surgical evaluation should include assessment of the VSD size, aortic valve function, and ventricular dimensions through echocardiography.
• Post-surgical care should involve regular cardiology follow-up every 6-12 months to monitor for any signs of complication or progression of disease.
• Antibiotic prophylaxis for dental procedures should be administered according to current guidelines to prevent endocarditis, typically amoxicillin 50 mg/kg orally one hour before the procedure (maximum 2g).

Quality of Life and Activity

• Physical activity need not be restricted if the child is asymptomatic with normal exercise tolerance and no significant hemodynamic abnormalities.
• The goal of surgical intervention is not only to prevent long-term cardiac complications but also to ensure the child can lead a normal life with minimal restrictions, thus improving quality of life.

From the Research

American Guidelines for Congenital Perimembranous Subaortic VSD with Aortic Prolapse and Trivial Aortic Regurgitation in a 5-Year-Old Boy

• The management of ventricular septal defect (VSD) with aortic valve prolapse and trivial aortic regurgitation is a complex issue, and the decision to operate depends on various factors, including the size of the VSD, the degree of aortic regurgitation, and the presence of other cardiac anomalies 2, 3.
• According to the study by 2, children with small perimembranous VSDs and cusp prolapse require surgery only if there is clinical evidence of aortic regurgitation and progressive left ventricular enlargement.
• The study by 3 suggests that the anatomical features of the septal defect may influence clinical management and outcome, and that aortic valvuloplasty is required more often in doubly committed VSDs and in the perimembranous type without associated anomalies.
• The pathophysiology of aortic valve prolapse and aortic insufficiency in patients with VSD is explained by the Venturi effect, which creates a low-pressure zone that impacts the adjacent aortic valve cusp, resulting in prolapse and subsequent insufficiency 4.
• The risk of development of aortic insufficiency increases during childhood, peaking at 5 to 10 years of age, and VSD closure eliminates the low-pressure zone that is the cause of ongoing aortic valve cusp deformity and, if performed early, prevents development of aortic insufficiency 4.
• The study by 5 suggests that all moderate and severe aortic regurgitation with small VSD in older patients with more than one cusp prolapse will need intervention to their aortic valve during the closure of VSD.

Indications for Surgery

• The indications for surgery in patients with VSD and aortic valve prolapse include:
  • Clinical evidence of aortic regurgitation and progressive left ventricular enlargement 2
  • Presence of moderate or severe aortic regurgitation 5
  • Small VSD in older patients with more than one cusp prolapse 5
  • Doubly committed VSDs or perimembranous VSDs without associated anomalies 3

Timing of Surgery

• The timing of surgery depends on various factors, including the size of the VSD, the degree of aortic regurgitation, and the presence of other cardiac anomalies.
• Early surgery is recommended to prevent the development of aortic insufficiency and to eliminate the low-pressure zone that is the cause of ongoing aortic valve cusp deformity 4.