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Management of tetralogy of Fallot with absent pulmonary valve: early and mid-term results of a uniform approach.

The operative management of absent pulmonary valve syndrome remains controversial regarding palliative or one-stage correction, the need for pulmonary valve implantation and pulmonary arterioplasty. This retrospective report summarises the experience of a single centre with a view to provide some answers to this controversy. Forty-six consecutive patients including five infants, aged 2 months to 43 years, underwent primary surgical correction during the last 8.5 years. All the patients underwent two-dimensional echocardiography and cardiac catheterisation. Nine patients had mild and 10 moderate pulmonary artery hypertension. Repair consisted of patch closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract. A valve was incorporated in the pulmonary position in 19 patients. Pulmonary arterioplasty was performed only in infants. Overall hospital mortality was 4 out of 46 patients (8.6%). Two out of five infants died accounting for 40 percent mortality. Forty-two survivors were followed up from 4 to 101 months; 40 patients are in functional class I and two in class II. Actuarial survival at 8.5 years was 91 percent. It is concluded that reconstruction of the right ventricular outflow tract with a transannular patch is sufficient in majority of patients. A selective approach to pulmonary valve insertion is recommended in patients with pulmonary hypertension or other anomalies. Pulmonary arterioplasty should be performed as the primary treatment in infants.