The patient was a nine-month-old, 8.8 kg infant who was born with anomalous origin of the right pulmonary artery (RPA) from the ascending aorta. She underwent an initial median sternotomy with translocation of the RPA to the main pulmonary artery and anterior translocation of the RPA. The patient developed stenosis of the RPA and underwent pulmonary homograft patching of the RPA that did not last long. She had her third sternotomy where repeat patching of the RPA was performed using a bovine pericardial patch.
The follow-up showed right lung hypoperfusion, severe RPA stenosis, and persistent hypoplasia. The decision was made to proceed with repeat surgery. At this stage, the team did not feel that repatching of the RPA would be of any benefit, so it was decided to completely remove this segment and replace it with an oversized ringed Gore-Tex graft.
The procedure was performed through a fourth sternotomy and, after initial mediastinal dissection, the RPA was isolated and fully mobilized from its origin to the origin of the lobar branches. Heparin was administered (100 Units/kg) systemically and a test clamping of the RPA showed the patient tolerated the clamping, so the team proceeded with proximal and distal control of the RPA. The stenotic, hypoplastic segment was resected and a 12 mm Gore-Tex graft was anastomosed to the distal RPA using running 6-0 Prolene sutures. This was followed by completion of the proximal anastomosis to the proximal RPA using running 5-0 Prolene sutures. The graft position was satisfactory, and echocardiography confirmed good flow in the distal RPA.
The anterior pericardium was reconstructed with a 0.1 mm Gore-Tex membrane and the chest was closed in a routine fashion.
The patient was extubated in the operating room, received no transfusion, and the remaining postoperative course was uneventful. She was discharged five days later. She continued to do well during her follow up and a repeat CTA scan one year postoperatively showed satisfactory growth of the RPA, with a well-patent main pulmonary artery and branches. The position of the RPA graft also looked good.
This option represents a good alternative for severely hypoplastic and repeatedly obstructed branch pulmonary arteries when previous traditional surgical measures failed. The graft must be oversized to allow for growth.
References
- Yoshino M, Saitoh Y, Chiyo M, Hoshino H, Sekine Y, Wada H, Suzuki H, Yoshino I. Surgical outcome of pulmonary artery reconstruction using the expanded polytetrafluoroethylene patch in patients with lung cancer. Surg Today. 2019 Sep;49(9):778-784
- Sughimoto K, Konstantinov IE, Brizard CP, d’Udekem Y. Hilum-to-hilum Gore-Tex tube replacement of central pulmonary arteries. Ann Thorac Surg. 2015 Jan;99(1):340-2
- Miyazaki T, Yamagishi M, Maeda Y, Yamamoto Y, Taniguchi S, Sasaki Y, Yaku H. Expanded polytetrafluoroethylene conduits and patches with bulging sinuses and fan-shaped valves in right ventricular outflow tract reconstruction: multicenter study in Japan. J Thorac Cardiovasc Surg. 2011 Nov;142(5):1122-9
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2 Comments
Congratulations on successfully performing this very special surgery.
A very nice technical result and an effective palliation (ringed gortex). I have dealt with a couple cases of what invariably becomes a progressively difficult situation as these patients achieve adulthood b/c this is not dTGA s/p ASO LeCompte but anterior RPA with interposition graft that will invariable demonstrate relative foreshortening due to somatic growth with impact on the aorta, pseudo-kinking/pancaking at MPA takeoff and competition against the sternum. Because of these experiences, I strongly advocate for all anomalous or discontinuous RPA variants (duct or aortic origins) that the original palliation should always strive to achieve a retro-aortic position of the new RPA b/c, though technically more difficult, it can be achieved in a neonate/young infant – but at this stage and forevermore is/will be impossible. Excellent video.