What are the American guidelines for congenital perimembranous subaortic Ventricular Septal Defect (VSD) with aortic prolapse and trivial aortic regurgure?

From the Guidelines

For a congenital perimembranous subaortic VSD with aortic prolapse and trivial aortic regurgitation, the American guidelines recommend close monitoring with regular cardiology follow-up every 6-12 months, including echocardiography to assess defect size, shunt severity, and progression of aortic valve involvement, as indicated by the 2018 AHA/ACC guideline for the management of adults with congenital heart disease 1. The management approach is based on the principle of balancing the risks of surgical intervention against the potential benefits of preventing progressive aortic regurgitation and its complications. Key considerations in the management of these patients include:

• The risk of progressive aortic regurgitation, which may be influenced by the size and location of the VSD, as well as the presence of aortic valve prolapse 1.
• The potential for spontaneous closure of small VSDs, which may occur in some cases, but is less likely in the presence of aortic valve prolapse 1.
• The importance of regular monitoring to assess for signs of progressive aortic regurgitation, such as increasing left ventricular volume overload or symptoms of heart failure 1. Surgical closure is indicated if there is progressive aortic regurgitation, significant left ventricular volume overload (Qp:Qs ratio >1.5), or development of symptoms such as exercise intolerance or heart failure, as these factors increase the risk of adverse outcomes and may necessitate earlier intervention 1. Medical management, including the use of diuretics and ACE inhibitors, may be considered in patients with heart failure symptoms, but is not a substitute for surgical intervention in cases where it is indicated 1. Overall, the goal of management is to balance the risks and benefits of intervention and to prevent long-term complications, such as irreversible aortic valve damage and heart failure, while also minimizing the risks associated with surgical intervention 1.

From the Research

American Guidelines for Congenital Perimembranous Subaortic VSD with Aortic Prolapse and Trivial Aortic Regurgitation

• The American guidelines for congenital perimembranous subaortic VSD with aortic prolapse and trivial aortic regurgitation are based on several studies, including 2, 3, 4, 5, and 6.
• According to these studies, the management of VSD with aortic valve prolapse and regurgitation depends on the severity of the regurgitation and the presence of other cardiac anomalies.

Indications for Surgery

• Surgery is indicated for patients with perimembranous VSD and aortic valve prolapse with more than trivial aortic regurgitation 2, 5.
• Patients with small perimembranous VSDs and cusp prolapse should undergo surgery only if there is clinical evidence of aortic regurgitation and progressive left ventricular enlargement 2.
• Surgery is also recommended for patients with subarterial VSD and aortic valve prolapse to prevent the development of aortic regurgitation 5.

Timing of Surgery

• The timing of surgery depends on the severity of the aortic regurgitation and the presence of other cardiac anomalies 3, 6.
• Patients with mild aortic regurgitation can be followed with serial echocardiography, while those with moderate or severe regurgitation require prompt surgical intervention 4, 6.

Surgical Outcomes

• The surgical outcomes for repair of aortic valve prolapse and regurgitation associated with VSD are generally good, with low rates of postoperative aortic regurgitation 6.
• However, patients with severe aortic regurgitation may require aortic valve replacement, and those with moderate regurgitation may require close follow-up to monitor for progression of disease 6.