Connection of discontinuous pulmonary arteries in patients with a superior or total cavopulmonary circulation

Abstract

Background: Discontinuous pulmonary arteries (PAs) may develop in patients with single-ventricle heart disease from a variety of causes. We investigated factors associated with successful connection of nonconfluent PAs in patients with a cavopulmonary circulation.

Methods: We reviewed 49 patients who underwent connection of discontinuous PAs with or after a bidirectional Glenn (n = 29) or Fontan (n = 20) procedure at a median age of 7.9 years. PA continuity was established by direct anastomosis in 27, interposition graft in 19, and transcatheter recanalization in 3. Survival was 92% +/- 4% at 1 year and 89% +/- 5% at 5 years.

Results: Recurrent PA occlusion was documented in 7 patients, 5 within 10 days of PA connection. The only factor associated with shorter freedom from PA occlusion was sole supply of blood flow to 1 lung by systemic-to-PA collaterals before connection (66% +/- 14% vs 95% +/- 4% freedom from occlusion at 6 months, p = 0.03). Among the 45 early survivors, freedom from PA reintervention or occlusion was 83 +/- 6% at 1 year and 55 +/- 9% at 3 years.

Conclusions: Discontinuous PAs can be successfully connected in most patients with a cavopulmonary circulation, although nonconfluent PAs appear to increase the risk of poor outcome after Fontan. Recurrent PA occlusion was usually diagnosed in the early postoperative period. In patients with sole supply to 1 lung through collaterals, shunt placement before PA connection may optimize outcome. A low threshold for investigation of the reconnected PA is warranted.

Figure 1

(A) Discontinuous pulmonary arteries (PAs) after a classic Glenn procedure, with a (B) systemic-to-PA shunt providing flow to the left PA. The umbrella occlusion device at the junction of the superior vena cava and right PA in Panel A was placed to close a residual superior vena cava–right atrial communication. (C) A tube graft was used successfully to reconstruct the central PAs at the time of Fontan completion.

Figure 2

Discontinuous pulmonary arteries (PAs) due to ductal closure, with consequent left PA atresia. (A) The initial palliation in this patient with a right-sided systemic-to-PA shunt, which is seen supplying the right PA and filling the central PA and main PA remnant. (B) After diagnosis of discontinuous PAs, an additional systemic-to-PA shunt was placed to supply with left lung, (C) before the reconnection of the left and right PAs at the time of bidirectional superior cavopulmonary anastomosis.

Figure 3

Flow diagram demonstrates outcome of pulmonary artery (PA) reconstruction among patients with sole supply to 1 lung by systemic-to-PA collaterals at the time of diagnosis of PA discontinuity.

Figure 4

Kaplan-Meier curves depict freedom from PA occlusion (top, solid line) and freedom from either PA occlusion or reintervention (bottom, solid line). The dotted lines show the 95% confidence intervals.