When Repair is not Feasible: Prosthesis Selection in Children and Adults with Congenital Heart Disease

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Abstract

Congenital heart surgeons face many challenges when dealing with valvular pathology in the pediatric population. Because of the concerns related to growth, repair should be the main goal. However, this is not always feasible and valve replacement becomes the only other alternative. Valve replacement also represents one of the most common procedures performed for adults with congenital heart disease, with several valve options existing including homografts, xenografts, autografts, and other artificial prostheses. The choice sometimes may be difficult because there are advantages and disadvantages to each valve substitute. In this article, we will address the different options of valve replacement in children and adults with congenital heart disease, and review the current literature that supports current practice.

Introduction

Valvular pathology and the need for reinterventions represent an ongoing challenge to congenital heart surgeons. In infants and children, valve repair should be the top priority because of ongoing growth issues. However, this is not always possible and then replacement will be needed despite the expected need for reoperation because of somatic outgrowth. Adults with congenital heart disease (CHD) are a fast-growing population because of improved survival and surgical outcomes of those who have undergone surgery for CHD during infancy and childhood. Therefore, there is a growing need for reoperation; many patients require numerous surgical procedures or interventions over a lifetime.

It is estimated that there is more than a million adults with CHD in the United States alone.1 This is the result of the significant improvement in surgical techniques and perioperative care, with at least 95% of newborns with CHD surviving to adulthood.2 However, despite this improvement, residual or recurrent lesions may progress over years and decades, resulting in an increase in the need for reoperation. It is expected that the number of adults with CHD will continue to increase as the population increases.

We recently reviewed our experience with reoperation in adults with CHD, which included 984 adults with a mean age of 36.4 years; the majority of these reoperations (85%) were related to valve dysfunction,3 including 25% with multivalve pathology. The most common valve requiring replacement was the pulmonary valve, followed by the aortic valve. However, among these repairs, the most common was the tricuspid valve followed by the mitral valve (MV).

In this article we will review the current literature related to valve replacement in children and adults with CHD and discuss the different valvular substitutes that are available.

Section snippets

The Ideal Prosthetic Valve

The ideal prosthesis chosen by the surgeon to replace the native valve should possess the following favorable characteristics4:

  • •

    available in different sizes

  • •

    has excellent handing features

  • •

    growth potential, especially in the younger population

  • •

    low infectious potentials

  • •

    low risk of thrombosis and dysfunction

  • •

    known long-term function, and

  • •

    low cost.

Unfortunately, this so-called ‘ideal prosthesis’ does not exist. However, the above-listed criteria may not all be required or needed to be applied to every

Aortic Valve Replacement

Aortic valve (AV) replacement is one of the most common procedures performed for adults with CHD, and AV pathology remains one of the most common congenital cardiac defects. Congenital aortic stenosis caused by bicuspid AV continues to be a common indication for valve replacement in a high percentage of patients over their lifetimes.5 Other congenital diagnoses that may require AV replacement include tetralogy of Fallot, truncus arteriosus, transposition of the great arteries, and aortic

Mitral Valve Replacement

Mitral valve (MV) pathology can be seen in a number of CHDs, including congenital MV anomalies, Shone complex, atrioventricular septal defect, cleft MV, corrected transposition of the great arteries (MV position), and acquired lesions such as rheumatic heart disease and endocarditis. Because of the success with MV repair and the documented long-term benefit, every effort should be made to repair the valve. Unfortunately, despite these efforts, occasionally the need for valve replacement exists.

Pulmonary Valve Replacement

Pulmonary valve replacement (PVR) with or without RVOT reconstruction is the most common valve requiring replacement in the congenital population. The most common associated diagnoses are congenital pulmonary valve pathology (stenosis and or regurgitation), tetralogy of Fallot, and other conotruncal abnormalities (truncus arteriosus and transposition of great arteries). Different prostheses are available as native PV substitutes, including pulmonary or aortic homografts, xenografts, bovine

Tricuspid Valve Replacement

The most common congenital diagnoses that require tricuspid valve (TV) interventions are congenital TV dysplasia, Ebstein malformation, atrioventricular septal defects, and conotruncal anomalies. Our experience with Ebstein repair spans over decades and we adopted the cone repair strategy recently with excellent early and midterm results.46 We also recently published our experience with re-repair of Ebstein malformation using the cone technique and demonstrated its feasibility after previous TV

Conclusion

In summary, the majority of reoperations in adults with CHD are secondary to valvular pathology, and valve replacement in children and young adults is a complex task because of ongoing growth and patient size. Several prosthetic options are available and the surgeon should assess every patient individually before deciding about the prosthesis. Important considerations should be given to patient anatomy, lifestyle, anticipated durability, and future reoperations.

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