SHORT REPORT Fabienne Marchau Æ Derize Boshoff Æ Marc Gewillig Luc Mertens Excluded right pulmonary artery in a neonate Received: 1 September 2003 / Revised: 8 December 2003 / Accepted: 9 December 2003 / Published online: 4 February 2004 Ó Springer-Verlag 2004 A case of ‘‘absent’’ right pulmonary artery in a 3-day- old newborn is presented. Initial treatment consisted of stenting the arterial duct with subsequent surgical correction. Congenital unilateral ‘‘absence’’ of a pulmonary artery is a rare malformation. ‘‘Absent’’ right pulmonary artery is mostly found as an isolated lesion whereas an ‘‘absent’’ left pulmonary artery is commonly associated with tetralogy of Fallot, in particular with pulmonary atresia and duct-dependent pulmonary circulation. Patients with isolated unilateral ‘‘absence’’ of a pulmo- nary artery are mostly asymptomatic, this being an incidental finding, e.g. on a chest X-ray film [1, 6, 7, 8,9]. Some patients develop recurrent respiratory infections, dyspnoea, exercise intolerance, haemoptysis and pul- monary hypertension in the contra lateral pulmonary artery. The affected pulmonary artery is not actually absent and the distal portion and the intrapulmonary vessels are always present but may be occluded. Perfu- sion of these distal vessels was occasionally described by an arterial duct originating from the aorta or from the ipsilateral brachiocephalic artery [1, 5,7]. Thus, only the central portion of the affected pulmonary artery is absent causing discontinuity between the intrapulmonary artery and the pulmonary trunk. The search for the patency of this occult intrapulmonary artery is of great importance in order to try to restore blood flow to the affected side. This stimulates further normal vessel and lung growth thus preventing pulmonary hypertension in the contralateral lung. We report a case in which an excluded right pulmonary artery was detected in the neonatal period. A 2-day-old 2.6 kg term male newborn presented with grunting, mild tachypnoea and wheezing. A chest X-ray film (Fig. 1) showed bilateral hyperinflation and discrete diminished pulmonary vascular markings in the right lung fields. There was a small pleural effusion. Echocardiography showed a normal cardiac anatomy with a left aortic arch. The left pulmonary artery origi- nated from the pulmonary trunk but the right pulmo- nary artery could not be identified, neither at the inner curve of the ascending aorta nor at the right lung hilus. There was a small right pleural effusion. No patent left or right arterial duct could be identified echocardio- graphically. Intravenous prostaglandins were started with the diagnosis of excluded right pulmonary artery from a closed right arterial duct. Cardiac catheterisation was performed which confirmed the diagnosis of a central pulmonary artery only connected with the left pulmonary artery (Fig. 1). The left duct had reopened on prostaglandin therapy. A dimple from the closed right duct was noted on the brachiocephalic artery (Fig. 2). Retrograde right pulmonary venous wedge injection showed a nicely developed right pulmonary artery without thrombus. There was minimal washout from collateral arteries. The closed right duct could easily be crossed with a guide wire. Angiography demonstrated the right duct between the brachiocephalic artery and the right pulmonary artery (Fig. 3). A 3 mm coronary stent was implanted in the right duct resulting in good perfusion from the aorta to the right lung (Fig. 4). Immediately after the proce- dure, reperfusion pulmonary oedema was observed. This resolved within hours and the child could easily be weaned from the ventilator. Clinically there was abun- dant flow to the right lung, with mild failure. One month after the procedure, a severe abdominal problem was noted caused by necrotising enterocolitis. This was associated with an adenoviral infection and required a temporary ileostomy. Progressive respiratory distress was also observed. Repeat cardiac catheterisation was performed 2 months after stent implantation. This showed a patent stented duct with prominent perfusion Sponsored in part by the Belgian Foundation for Research in Paediatric Cardiology. L.M. is clinical researcher for the Foun- dation for Scientific Research (FWO). F. Marchau (&) Æ D. Boshoff Æ M. Gewillig Æ L. Mertens Department of Paediatric Cardiology, University Hospital Leuven, Herestraat 49, 3000 Leuven, Belgium Eur J Pediatr (2004) 163: 274–276 DOI 10.1007/s00431-003-1399-0