Over the past few decades, we’ve made great strides in congenital heart surgery, which has increased survivorship among long-term sufferers of congenital heart disease. While many options for congenital heart disease and defect repairs are available, patients may require future additional surgeries.
Tetrology of Fallot is one of the most common types of congenital heart defects, and is a prime example of a condition that could require pulmonary valve replacement. Tetraology of Fallot (TOF) actually refers to four heart defects present from the time of birth: a ventricular septal defect (hole between the ventricular chambers which allows blue and red blood to mix), pulmonary stenosis (narrowing that makes it difficult for blue blood to reach the lungs), right ventricular hypertrophy (thickening of the heart muscle from pumping blood past the narrowing) and an overriding aorta. These defects often cause an infant to have cyanosis, or blue-tinged color of the skin, resulting from lack of oxygen-rich blood.
Fortunately, the prognosis for children with this condition has greatly improved over the last several decades (assuming proper diagnosis and treatment is administered). During surgical repair of TOF, the hole is closed with a patch, and the narrowing from the diseases pulmonary valve is removed, which can leave patients with a ‘leaky’ pulmonary valve. Although some patients may do well for a decade or more with the leaky valve, the pulmonary valve will eventually need to be replaced in most children born with TOF. Perhaps the most challenging aspect of treatment for patients suffering from this condition is exactly when to time treatment.
Other repairs may be associated with the need for pulmonary valve replacement in the future, including repair for pulmonary atresia with ventricular septal defect (VSD), truncus arteriosus, the Ross procedure for aortic valve disease, and double outlet right ventricle and d-transposition of the great arteries with VSD.
Surgical replacement of the pulmonary valve is the standard treatment for pulmonary valve disease. Valve replacement surgery involves the replacement of one (or more) of the valves of the heart, typically with an artificial heart valve or a bioprosthesis (a prosthesis consisting of an animal part or animal tissue).
Pulmonary valve replacement is the most common operation performed in the adult congenital heart disease population. This surgery can be performed with extremely low morbidity and mortality. Patients are typically out of the hospital within the first week, and able to perform their normal daily routine. They’re fully recovered within 4 weeks. A surgically placed pulmonary valve is expected to last 10-15 years, or longer.
For very select patients who have already had conduit replacement of the pulmonary valve, transcatheter pulmonary valve replacement may be an option. While this option has a shorter recovery time, the longevity of the valve remains unknown. Early findings show that transcatheter PVR could be used as an alternative to traditional surgical methods in appropriate patients. The percutaneous PVR approach involves the placement of a bovine valve inside a balloon stent.
As physicians, we’re encouraged by the great strides we’re making from both surgical and technological standpoints with pulmonary valve replacement and congenital heart disease in general.
Do you have questions regarding any of these medical advances? If so, please be sure to let me know in the comments section.
About Brian E. Kogon, MD:
Dr. Kogon is an assistant professor of surgery and director of the congenital cardiac surgery fellowship at Emory. Additionally, he’s the surgical director of adult congenital cardiac surgery at Emory University Hospital. His clinical interests include pediatric cardiac surgery, cardiac transplantation, and adult congenital heart surgery. Dr. Kogon has been with Emory as a faculty member since 2004.