We report on the first prospective trial of chemotherapy for infants and children targeting the presence of myofibroblastic cells within the lesions of PVS based on myofibroblastic proliferation associated with desmoid tumors of infancy. The toxicity profile resulted in numerous treatment delays and interruptions that, combined with limited information on the natural history of PVS in this patient population, hampered our ability to determine the true efficacy of this approach. These results will be important as a baseline for clinical trials in this patient population.
Bilateral disease and earlier age at diagnosis are independent predictors of poor survival in patients with CHD and PVS, while patients with unilateral disease presenting at an older age have a better prognosis. These findings are helpful in risk stratification of patients with CHD and multivessel PVS.
Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor. Recurrence of atresia can take place as early as a few months in the presence of underlying lung disease, prematurity, or history of recurrent pulmonary vein atresia. Further research is needed to identify mechanisms for attaining and sustaining pulmonary vein patency after surgery.
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