BACKGROUND: Children with pulmonary hypertension secondary to large left-to-right, post-tricuspid valve shunts can eventually have severe and/or irreversible pulmonary vascular disease, yielding them inoperable for conventional surgery. It has been shown, however, that unloading of the pulmonary hypertension can result in remodeling of the pulmonary vasculature and, thus, improvement of the pulmonary hypertension. METHODS: This study explored whether such patients might experience a significant reduction in pulmonary vascular resistance (PVR) after pulmonary artery band (PAB) placement. Pulmonary hypertension hemodynamics were evaluated by cardiac catheterization in 4 patients with pulmonary hypertension secondary to nonrestrictive left-to-right, post-tricuspid valve shunts before and after PAB placement. Two patients with severe pulmonary hypertension who were considered high risk for conventional surgery benefited from PAB placement with a significant reduction in their PVR, permitting subsequent complete intracardiac repair. RESULTS: The medium-term follow-up for these 2 patients demonstrated good outcomes. The PVR failed to improve after PAB placement in the remaining 2 patients, leading to medical therapy for pulmonary hypertension. There was 1 late death, presumably related to pulmonary hypertension. Current practice provides 3 relatively unattractive options for patients with severe pulmonary hypertension secondary to nonrestrictive left-to-right, post-tricuspid valve shunts: transplantation, high-risk intracardiac repair, or palliative medical therapy. CONCLUSION: Our study suggests that a staged approach with initial PAB placement can be considered for select patients with large left-to-right, post-tricuspid valve shunts and high PVR prior to committing them to other high-risk therapeutic options.
BACKGROUND:Children with pulmonary hypertension secondary to large left-to-right, post-tricuspid valve shunts can eventually have severe and/or irreversible pulmonary vascular disease, yielding them inoperable for conventional surgery. It has been shown, however, that unloading of the pulmonary hypertension can result in remodeling of the pulmonary vasculature and, thus, improvement of the pulmonary hypertension. METHODS: This study explored whether such patients might experience a significant reduction in pulmonary vascular resistance (PVR) after pulmonary artery band (PAB) placement. Pulmonary hypertension hemodynamics were evaluated by cardiac catheterization in 4 patients with pulmonary hypertension secondary to nonrestrictive left-to-right, post-tricuspid valve shunts before and after PAB placement. Two patients with severe pulmonary hypertension who were considered high risk for conventional surgery benefited from PAB placement with a significant reduction in their PVR, permitting subsequent complete intracardiac repair. RESULTS: The medium-term follow-up for these 2 patients demonstrated good outcomes. The PVR failed to improve after PAB placement in the remaining 2 patients, leading to medical therapy for pulmonary hypertension. There was 1 late death, presumably related to pulmonary hypertension. Current practice provides 3 relatively unattractive options for patients with severe pulmonary hypertension secondary to nonrestrictive left-to-right, post-tricuspid valve shunts: transplantation, high-risk intracardiac repair, or palliative medical therapy. CONCLUSION: Our study suggests that a staged approach with initial PAB placement can be considered for select patients with large left-to-right, post-tricuspid valve shunts and high PVR prior to committing them to other high-risk therapeutic options.