Surgical management of aortopulmonary window: 24 years of experience and lessons learned

Aortopulmonary window represents 0.2-0.3% of all congenital heart lesions. Progressive pulmonary arterial hypertension and its consequences are more common with this anomaly. The purpose of this study was to share 24 years of surgical experience in managing a spectrum of 55 cases of aortopulmonary window, followed up to 17 years in a single institution.

This retrospective study was done from November 1991 to November 2015 of 55 patients with aortopulmonary window who underwent successful surgical repair. Age ranged from 5 months to 31 years with 45 children (12 years and younger) and 10 adults (older than 12 years). The male:female ratio was 2.2:1. The mean weight at operation was 14.63 kg (range 3.5-50 kg). An initial diagnosis was obtained from 2D echocardiography, which showed echo dropout in the parasternal short-axis view. Cardiac catheterization and angiography were performed in 54 out of 55 patients. Cardiac catheterization was not done in 1 patient who was 4 months of age. The mean right ventricular systolic pressure (RVSP) was 94 ± 2 mmHg, and the pulmonary artery mean pressure was 68 ± 2 mmHg. The average left to right shunt was 5.2:1, and the pulmonary vascular resistance index in room air was 7.97 ± 0.5 Wood units, whereas after oxygen administration, it declined to 2.0 ± 0.5 Wood units. Four surgical techniques were used based on the size of the communication and the anatomical conditions.

There were no early or late deaths. There were no pulmonary hypertensive crises. All patients underwent echocardiography before discharge; none showed a residual shunt. Mild left ventricular dysfunction was seen in 2 patients. This dysfunction regressed with afterload reduction and diuretics on follow-up. All patients were followed up at intervals of 3 months, 1, 5 and 10 years, with the longest follow-up being 17 years. The mean follow-up period was 7 years. At follow-up, all patients were New York Heart Association class I. The mean RVSP on echocardiography was 32 mmHg at 3 months and 30 mmHg at 7 years with no change on further follow-up. Residual pulmonary hypertension was seen in 3 patients: 2 had mild pulmonary hypertension at 8-years follow-up and 1 had moderate hypertension at 3-months follow-up who required sildenafil postoperatively.

Aortopulmonary window is a rare but well identified and surgically correctable anomaly. Operative repair should be offered as soon as the diagnosis is established, regardless of the patient's age. Irreversible pulmonary hypertension with a right to left shunt despite oxygen administration is the only contraindication for surgery. Various surgical techniques can be applied depending on the size of the communication. Associated arch anomalies may require technically challenging approaches and surgical strategies. Early and long-term outcomes after surgical correction are excellent regardless of age or pulmonary vascular resistance.