A Novel Technique to Repair Absence of Right Pulmonary Artery

Objectives: Unilateral isolated absence of a pulmonary artery (UAPA) is a rare congenital lesion, which can lead to pulmonary hypoplasia and pulmonary hypertension. To avoid these complications, early surgical repair of UAPA is recommended. Surgical approaches include creation of an aorto-pulmonary shunt, direct anastomosis between pulmonary artery and main pulmonary artery (MPA) or bridging the “gap” with prosthetic interposition grafts. We present a novel surgical technique using a total autologous interposition tube graft using tissue from the roof of the MPA.

Methods: An 8-days-old female (weight: 2.8 kg; size: 48 cm) with severe respiratory distress (SaO2 80%) was referred to our center for suspected pulmonary anomaly. Chest radiograph showed hyperlucent, symmetrical lungs and cardiomegaly. Echocardiography revealed an absent RPA without other cardiac malformations. Catheterization depicted a normal-sized left PA and a ductus diverticulum at the brachiocephalic artery indicating a closed right arterial duct originating from the hilar RPA.

In surgery, at first the right arterial duct was excised. After initiation of cardiopulmonary bypass, a rectangular graft (1.5 × 3 cm) was resected from the anterior wall of the main pulmonary artery . To create a “tube,” the graft was sutured around a 3mm Hegar pin with PDS 7x0. Next, the “tube” graft was anastomosed to the hilar RPA and to the MPA. The MPA roof was augmented with an autologous pericardial patch.

Results: Early follow up was complicated by an anastomotic stenosis at the level of the hilar RPA. Therefore balloon dilatation (Savvy 3 mm/20 cm; SeQuent Please 4.0 × 17 mm) was performed two month after initial surgery and the hilar RPA diameter was enlarged from 1 to 2 mm. After 7 month, the hilar anastomosis was still tight, hence stent implantation (Formula Stent 8 × 20 mm) was undertaken with a good angiographic result. Now, 16 months after the operation, echocardiography shows an acceptable diameter of RPA (4 mm). In addition, the patient is thriving very well with no signs of pulmonary maldevelopment.

Conclusion: We describe a novel pulmonary graft interposition technique for UAPA repair using complete autologous material. Hence, the chance of future growth of the new RPA is increased with our approach. However, graft stenosis is of concern, mandating close follow-up and tailored treatment.

No conflict of interest has been declared by the author(s).