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  • Title: [Pulmonary atresia with ventricular septal defect: the significance of collateral lung perfusion for the prognosis of corrective operations].
    Author: Hofbeck M, Singer H, Schlaud B, Staender S, Rein JG, vd Emde J.
    Journal: Z Kardiol; 1987 Jun; 76(6):364-70. PubMed ID: 2442896.
    Abstract:
    From 1970 to 1986, 43 patients with pulmonary atresia and ventricular septal defect (PA-VSD) were diagnosed in our hospital. 19 children had a collateral lung perfusion via a ductus botalli (DB), three children had a DB and systemico-pulmonary collateral arteries (SPCA), and 21 children had only SPCA. Patients with a DB alone had significantly larger pulmonary arteries. Children with SPCA always had hypoplastic pulmonary arteries and intrapulmonary arborization anomalies and in a high percentage (19 patients) they showed intrapulmonary stenoses. 26 palliative operations were performed in 24 children. After palliative operations in 15 cases (patients with DB: RVOT-Patch: one patient, aortopulmonary shunt: three patients, Brock procedure: one patient, patients with SPCA: RVOT-Patch: five patients, aortopulmonary shunt: four patients, Brock procedure: one patient) we checked the possibility of corrective surgery with the equation of Alfieri. According to this equation, corrective surgery without excessive right ventricular pressure would have been possible in all post-operatively catheterized patients with DB after primary palliative operations but, due to the pulmonary artery anomalies, only in seven out of nine patients with SPCA (one patient of the latter group was operated twice, Brock procedure and RVOT-Patch). In four cases we could prove the value of the Alfieri equation after corrective operations. There was an excellent correlation between the predicted relation of PRV/PLV and the actually measured pressures. In our opinion, the Alfieri equation forms a valuable aid in the preoperative assessment of children with PA-VSD.
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