Risk factors for early pulmonary valve replacement after valve disruption in congenital pulmonary stenosis and tetralogy of Fallot.

OBJECTIVE: Congenital heart defects with a component of pulmonary stenosis are often palliated in childhood by disrupting the pulmonary valve, either by means of dilation or excision. It is unclear what factors affect a patient's ability to tolerate long-term pulmonary insufficiency before requiring pulmonary valve replacement. We analyze potential factors that are related to the interval between pulmonary valve disruption and pulmonary valve replacement.
METHODS: One hundred seven patients were analyzed. They had a congenital diagnosis of pulmonary stenosis or tetralogy of Fallot, had their first pulmonary valve replacement between 2002 and 2008, and had a known interval between pulmonary valve disruption and pulmonary valve replacement. The median age at the time of surgical intervention was 2 years for pulmonary valve disruption (range, 0-56 years) and 26 years for pulmonary valve replacement (range, 1-72 years). The median interval was 23 years (range, 0-51 years). Potential related factors were sex, race, initial diagnosis and procedure, age at pulmonary valve disruption, prior shunt operation, presence of branch pulmonary artery stenosis, and degree of pulmonary regurgitation.
RESULTS: As determined by using univariate analysis, male patients had a shorter interval than female patients (median, 16 vs 26 years; P = .01), and African American patients had a shorter interval than white patients (median, 16 vs 25 years; P = .049). A significant correlation was also identified between age at the time of pulmonary valve disruption and the subsequent interval to pulmonary valve replacement. Overall, the interval tended to increase as age at disruption increased (P < .0001). Although the presence of branch pulmonary artery stenosis determined by the need for concomitant pulmonary arterioplasty was associated with a significantly shorter interval to pulmonary valve replacement (21 vs 24 years, P = .02), stenosis determined based on small branch pulmonary artery diameter was correlated to a prolonged interval to pulmonary valve replacement (P = .009). Initial diagnosis, prior palliative shunt operation, and degree of pulmonary regurgitation had no effect on the interval between pulmonary valve disruption and subsequent pulmonary valve replacement. As determined by using multivariate analysis, only male sex and small pulmonary artery diameter remained significant factors.
CONCLUSIONS: Male sex appears to shorten the interval between pulmonary valve disruption and pulmonary valve replacement, whereas small branch pulmonary artery diameter appears to lengthen the interval. Knowing which factors are detrimental and which are protective might help identify patients who are prone to a more rapid progression of right heart failure from free pulmonary insufficiency, possibly steering them toward more frequent follow-up or more aggressive heart failure medical regimens.