Unifocalization for Isolated Pulmonary Artery of Ductal Origin in a Two-Year-Old Child

Background 

In isolated pulmonary artery of ductal origin (IPADO), one or both pulmonary arteries arise from a ductus arteriosus, and it is observed in approximately 1.5 percent of patients with discontinuous pulmonary arteries (1,2). Embryologically, the condition is thought to result from involution of the proximal sixth aortic arch, resulting in the absence of the proximal pulmonary artery and the persistence of the connection between the hilar pulmonary artery and the distal sixth aortic arch, which later forms the ductus arteriosus (3, 4). If left untreated, this condition may lead to hypoplasia of the affected pulmonary artery, compensatory formation of aortopulmonary collateral vessels, and increased susceptibility to pulmonary infections and hemorrhage (5, 6). Given the potential for long-term pulmonary sequelae, early recognition and timely intervention are critical. 

The following case video illustrates the diagnostic pathway, anatomic findings, and management strategy in a two-year-old child with this rare congenital anomaly. In addition, this case represents a rare variant of the anomalous origin of the right pulmonary artery, featuring the presence of both ducts and an anomalous origin of the right pulmonary artery from the right-sided ductus arteriosus (7). 

Case Video Summary 

A two-year-old girl was referred for surgical treatment. At presentation, her oxygen saturation was 85 percent on furosemide, acetylsalicylic acid, and clopidogrel. Clopidogrel was prescribed as part of dual antiplatelet therapy to prevent stent thrombosis prior to definitive surgical repair. The preoperative angiography revealed a discontinuity between the left and right pulmonary arteries, and surgical reconstruction of the right pulmonary artery was deemed appropriate. Preoperative echocardiography showed dilated right heart chambers with left ventricular ejection fraction of 85 percent and mild-to-moderate pulmonary regurgitation. During the surgery, following the ligation of the patent ductus arteriosus, the right pulmonary artery was divided from its ductal connection. An 8 mm ringed graft was then anastomosed between the right pulmonary artery hilum and the main pulmonary artery using 6-0 Prolene sutures. The anterior wall of the main pulmonary artery was opened and extended toward the left side to minimize graft length. A modified LeCompte maneuver was performed to bring the pulmonary bifurcation anterior to the aorta, and the anterior pulmonary confluence was reconstructed with a bovine pericardial patch. Postoperatively, oxygen saturation improved to 96 percent. The postoperative echocardiography revealed a left ventricular ejection fraction of 62 percent with equal flow distribution into both pulmonary arteries. The patient’s oxygen saturation increased to 96 percent, and her postoperative course was uneventful. She was discharged on postoperative day six, receiving acetylsalicylic acid, furosemide, and clopidogrel. At two months, control angiography suggested mild narrowing of the right pulmonary artery without a significant gradient, and balloon angioplasty was performed. The patient has since remained in good health. 

Isolated pulmonary artery of ductal origin (IPADO) is a rare congenital anomaly that presents surgical challenges and significant consequences. Treatment strategies include ductal shunting prior to surgical correction to foster pulmonary arterial growth or performing early unifocalization. In this case, timely unifocalization following ductal stenting successfully restored continuity between the both pulmonary arteries, resulting in balanced pulmonary circulation and satisfactory early outcomes. 

 Limitations and Technical Challenges 

This case included several technical challenges and limitations during surgical reconstruction. Considerations related to somatic growth favor native-to-native tissue anastomosis or autologous tissue transfer whenever feasible. This principle guided the initial strategy of reversing the pulmonary artery flap in this patient. However, the stent within the right-sided patent ductus arteriosus extended distally to the level of the right pulmonary hilum. Although the ductal origin was distal to the head of the left pulmonary artery, the reversed flap was insufficient in length to reach a stent-free segment of the right pulmonary artery posterior to the aorta without excessive tension. 

The extension of the reconstruction using autologous tissue from the ascending aorta was considered; however, this would have required aortic cross-clamping, increased myocardial ischemic time, prolonged operative duration, and the creation of an additional aortic patch anastomosis with potential long-term consequences. 

Given these limitations, the modified Lecompte maneuver was adopted as a backup strategy rather than the primary plan. Once a growth-preserving native tissue reconstruction could not be safely achieved, anterior routing of the pulmonary artery reconstruction was selected to:  

1. Allow the use of a shorter graft  
2. Facilitate a wider and more controlled reconstruction of the pulmonary artery  
3. Preserve straightforward surgical access to the interposed graft if further reintervention becomes necessary.  

An 8 mm graft was chosen as the largest conduit compatible with the right pulmonary artery hilar anatomy; a larger diameter would have been preferable if a more extensive anastomotic surface been available. 

References

1. Mery CM, Molina KM, Krishnamurthy R, Fraser CD Jr, Justino H. Pulmonary artery resuscitation for isolated ductal origin of a pulmonary artery. J Thorac Cardiovasc Surg. 2014 Nov;148(5):2235-2244.e1.
2. Currarino G, Williams B. Causes of congenital unilateral pulmonary hypoplasia: a study of 33 cases. Pediatr Radiol. 1985;15(1):15-24.
3. Welch K, Hanley F, Johnston T, Cailes C, Shah MJ. Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up. Ann Thorac Surg. 2005 Apr;79(4):1399-402.
4. Apostolopoulou SC, Kelekis NL, Brountzos EN, Rammos S, Kelekis DA. “Absent” pulmonary artery in one adult and five pediatric patients: imaging, embryology, and therapeutic implications. AJR Am J Roentgenol. 2002 Nov;179(5):1253-60.
5. Pool PE, Vogel JH, Blount SG Jr. Congenital unilateral absence of a pulmonary artery. Am J Cardiol 1962;10:706–32.
6. Farghly E, Bousamra M II. Hemoptysis resulting from unilateral pulmonary artery agenesis. Ann Thorac Surg. 2002;74:255-7.

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