Incidence and Risk Factors for the Development of Aortopulmonary Collaterals in Patient with Hypoplastic Left Heart Syndrome

Background: Development of aortopulmonary collateral arteries (APCs) is more common in patients with hypoplastic left heart syndrome (HLHS) compared with other types of functional single ventricle. This study was performed to clarify the incidence of APCs and to identify risk factors for development of APCs in patients with HLHS.

Method: A total of 141 patients with HLHS who completed three staged palliation by means of Norwood procedure, bidirectional cavopulmonary shunt (BCPS) and total cavo-pulmonary connection (TCPC) between 2003 and 2019 were included. Incidence and risk factors for the development of APCs were examined.

Results: APCs were observed pre-BCPS in 32 patients (23%), pre-TCPC in 65 (46%), and after TCPC in 41 (29%). Origins of APCs were from RIMA in 77 patients (55%), LIMA in 44 (31%), right SCA in 37 (26%), left SCA in 23 (16%), and descending aorta in 17 (12%). Aortic and mitral atresia (AA/MA) was associated with higher incidence of APCs at pre-PCPC (44 vs. 18%, p = 0.002). Sano shunt had higher incidence of APCs from descending aorta, compared with modified Blalock-Taussig shunt (20 vs. 2%, p = 0.002). Age at BCPS, hemoglobin level, mean pulmonary artery pressure, or arterial saturation (SO₂) did not influence the development of APCs at BCPS. Risk factors of APCs at TCPC were lower systemic ventricular pressure (88 vs. 81 mm Hg, p = 0.01) and mean aortic pressure (59 vs. 55 mm Hg, p = 0.04). Risk factor after TCPC was lower SO2 at pre-TCPC cardiac catheterization (83 vs. 81%, p = 0.02). Intervention for APCs were performed before BCPS in 10 (7%) patients, after BCPS in 7 (5%), pre-TCPC in 22 (16%), and after TCPC in 32 (23%). Lower hemoglobin level before BCPS (12.4 vs. 14.2 g/dL, p = 0.02) was associated with interventional closure of APCs before BCPS. Lower weight (4.7 vs. 5.2 kg, p = 0.02) and smaller Nakata Index at BCPS (114 vs. 139 mm²/m², p = 0.03) was associated with interventional closure of APCs before TCPC. Lower SO2 at TCPC (80 vs. 84%, p < 0.01) was associated with interventional closure of APCs after TCPC.

Conclusion: APCs can develop at each stage of palliation, most frequently before TCPC. AA/MA is a risk factor for development of APCs. Lower systemic blood pressure and cyanosis should be avoided to prevent the development of APCs at the time of TCPC.

Publication History

Article published online:
03 February 2022