Background .– Partial atrioventricular septal defect (PAVSD) is usually operated in childhood with excellent long-term results. However, some patients may present during adulthood and their management as well as their outcome is still unclear. We sought to analyze the clinical characteristics at presentation and the outcome of patients with PAVSD, non-operated or operated during adulthood.

Methods .– Between January 2000 and March 2013, 31 adult patients with PAVSD presented and were followed in our care network. The mean age at study entry was 34 (±16.6) years.

Results .– Dyspnea was the most frequent symptom at presentation in 19 cases (61%). Left atrioventricular valve regurgitation (LAVVR) and right ventricular overload were higher in patients undergoing surgical repair ( P = 0.01). Twenty-two patients (71%) had their surgical repair at a mean age of 39.4 (±15.3) year-old with ostium primum closure and partial or complete suture of the left atrioventricular cleft without postoperative death or major complication. There was one late reoperation for a residual shunt. Nine patients (29%) were not operated. After a mean follow-up of 7.4 (±7.1) years, 26 patients (84%) are in NYHA class I or II whereas five (16%) are in NYHA class III or IV. Operated patients have a lower NYHA class ( P < 0.01), a lower grade of LAVVR ( P = 0.03) and a lower systolic pulmonary artery pressure ( P < 0.01) than unoperated patients at last follow-up. The onset or persistence of supraventricular arythmias (SVA) after surgery was associated with an older (> 40-year-old) age at repair ( P = 0.02), a higher systolic pulmonary artery pressure ( P = 0.04) and a higher grade of LAVVR ( P = 0.03).

Conclusion .– PAVSD repair is indicated in adult patients because it carries an excellent prognosis and operated patients have better long-term clinical and hemodynamic status than unoperated patients. Moreover, SVA incidence decreases when surgery is performed before 40 years of age.