HJAR Jan/Feb 2022

36 JAN / FEB 2022 I  HEALTHCARE JOURNAL OF ARKANSAS CHILDREN’S HEALTH COLUMN CHILDREN’S HEALTH CONGENITALHEARTDISEASE is the most common birth defect in the United States, affecting 8 out of every 1,000 infants. Many forms of congenital heart disease involve stenosis or narrowing of the right ven- tricular outflow tract, pulmonary valve or main pulmonary artery. Congenital anom- alies such as tetralogy of Fallot can limit blood flow into the lungs, causing cyanot- ic heart disease or “blue baby syndrome,” which needs complex surgical intervention during infancy. This open-heart procedure requires both surgical expertise as well as the proper support for the rest of the body while the heart is being worked on (cardio- pulmonary bypass). While successful in relieving the stenosis, this surgery often leaves the patient with significant pulmonary valve regurgitation. This valvular insufficiency is well-toler- ated by most infants and children, but the progressive dilation of the right ventricle can have consequences later in life. As the chamber size grows to adapt to the volume overload caused by the leakage of the pul- monary valve, it can reach a point where the contractility of the heart is unable to keep up with the increased workload. Children who are thus affected gradually progress from having no symptoms to de- veloping fatigue and exercise intolerance as symptoms of right heart failure. Pediatric and adult congenital cardiologists moni- tor these patients with surveillance echo- cardiography and advanced imaging with cardiac magnetic resonance imaging (MRI) to determine when re-intervention will be indicated. Decades ago, the only option for these patients was to undergo another open- heart surgery to replace the dysfunctional pulmonary valve with a homograft conduit or a bioprosthetic valve created from bo- vine or porcine tissue. This re-intervention carried an increased risk of complications and procedural mortality as compared to the initial surgery. In addition, the prosthet- ic valve itself is an imperfect solution, with the new pulmonary valve failing in 10 to 15 years and needing surgical replacement. Each subsequent surgical intervention car- ries a progressively higher risk of compli- cations, and a patient can expect to need several valve replacements in their lifetime. Music for a Broken Heart: Nonsurgical Pulmonary Valve Replacement for Congenital Heart Disease