title: Successful palliation of the "absent" pulmonary valve syndrome by banding of the pulmonary trunk.
abstract: The "absent" pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary trunk, stenosis of the ventriculo-arterial junction with or without malalignment of the outlet septum, and ventricular septal defect. When the outlet septum is malaligned, the morphology resembles that of tetralogy of Fallot. We report our experience with 4 infants with this syndrome. All were in severe respiratory distress and cardiac failure when first seen. Cardiac catheterization was performed at 0.5-4.5 months of age in 3 of them. In the other, the clinical and echocardiographic features were considered sufficient to establish the diagnosis. Banding of the pulmonary trunk was carried out at the age of 2.5-5 months. The distal pulmonary arterial pressure in 3 cases dropped to 12-19 mm Hg. These patients could be extubated within one week postoperatively. Their course 1-3 years later is excellent, with rare episodes of mild respiratory problems only and markedly diminished pulmonary insufficiency. One child, weighing 3250 g at surgery, whose pulmonary arterial pressure did not drop below 29 mm Hg, could not be weaned off the respirator. Corrective surgery was undertaken 17 days later, but the patient died of respiratory complications. Based on clinical and Doppler sonographic findings, on control catheterization data and on haemodynamic findings in 3 surviving infants and two further patients with an uneventful course who, as yet, have not undergone surgery, we conclude that the beneficial effect of banding is the combined result of reduced pulmonary arterial pressure and decreased pulmonic regurgitation.
authors: Universitäts-Kinderklinik, Abteilung Pädiatrische Kardiologie, Freiburg, F.R.G.
authors: R
authors: R
authors: Mocellin
authors: D
authors: D
authors: Henglein
authors: A
authors: A
authors: Beitzke
authors: A
authors: A
authors: Ahmadi
authors: C
authors: C
authors: Suppan
authors: B
authors: B
authors: Rigler
mesh_terms: D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010166:Palliative Care / Q000379:methods*; D011183:Postoperative Complications / Q000628:therapy; D011651:Pulmonary Artery / Q000601:surgery*; D011664:Pulmonary Valve / Q000002:abnormalities*; D011665:Pulmonary Valve Insufficiency / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000601:surgery; D011669:Pulmonary Wedge Pressure; D012127:Respiratory Distress Syndrome, Newborn / Q000628:therapy; D013577:Syndrome; D013771:Tetralogy of Fallot / Q000601:surgery