Surgical experience in the rehabilitation and reimplantation of disconnected pulmonary arteries and its effectiveness in restoring pulmonary haemodynamics and function.

Objectives: We established a policy to restore disconnected pulmonary arteries (PAs), regardless of aetiology or cardiac pathology, using a protocol-based strategy. There are conflicting reports of the need to restore disconnected PA especially if severely hypoplastic with some groups advocating surgical repair of cardiac defects to single lung with moderate to high risk.

Methods: All patients referred to our PA reconstruction programme (2007-14) with complete separation of one branch PA were included. Our surgical strategy was based on the size of the PA. In patients with mild or no PA hypoplasia, we performed single-stage implantation of the disconnected PA and repair of intracardiac defects. Two-stage approach was used in patients with severe PA hypoplasia where the disconnected PA was retrieved inside the lung then connected to the aorta with a shunt to promote growth. After 6-12 months, the PA was implanted with simultaneous repair of intracardiac defects.

Results: Twenty patients were included (age range = 1 week to 8 years). Aetiologies were ductal origin of distal PA in 13 patients and iatrogenic in 7 patients. Group 1 (isolated lesion) consisted of 8 patients who presented with severe pulmonary hypertension. Group 2 consisted of 12 patients with associated intracardiac defects and was subdivided into Group 2a (biventricular hearts) 8 patients and Group 2b (univentricular hearts) 4 patients. Single-stage repair was achieved in 10 patients. Direct tissue-to-tissue connection was achieved in 18 (90%) patients. The follow-up was 100% complete (median = 53.8 months). All patients are alive and asymptomatic. Implanted PAs are patent with good growth demonstrated on serial echocardiography. Only two PAs required balloon dilatation for moderate restenosis at the anastomosis. No surgical reintervention was needed. All Group 1 patients had complete resolution of the pulmonary hypertension. Lung perfusion scans showed increased uptake (24-53% of the total uptake).

Conclusions: Our study demonstrated that, in paediatric age group, disconnected PAs can be restored with low risk and excellent outcomes even in patients with severely hypoplastic PA. Strategies to repair intracardiac defects to a single lung might not be the optimal approach.