title: Lost unilateral capillary perfusion during nonpulsatile pulmonary circulation: successful recovery by oral sildenafil.
abstract: Good status of pulmonary perfusion is essential for a successful outcome after the Fontan procedure. Increased pulmonary pressure and vascular resistance, small size of the pulmonary arteries, and significant branch stenoses reflect some of the main problems causing failing Fontan circulation. Here we report a child who underwent a staged Fontan procedure with subsequent subtotal loss of the left-sided pulmonary perfusion, although branch stenosis was successfully treated by stent implantation. Oral sildenafil caused restoration of the capillary vascular bed, improved left-sided lung perfusion, and resulted in significant clinical benefit.
authors: Department for Congenital Heart Disease/Pediatric Cardiology, Klinikum Links der Weser, Bremen, Germany. levent.celik@klinikum-bremen-ldw.de
authors: Levent
authors: L
authors: Celik
authors: Konstantin
authors: K
authors: Papakostas
authors: Markus
authors: M
authors: Lentschig
authors: Victor
authors: V
authors: Hraska
authors: Jan-Hendrik
authors: JH
authors: Nürnberg
mesh_terms: D002196:Capillaries / Q000187:drug effects; D002675:Child, Preschool; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D008168:Lung / Q000098:blood supply* / Q000187:drug effects; D008297:Male; D008833:Microcirculation / Q000187:drug effects; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use*; D011652:Pulmonary Circulation / Q000187:drug effects*; D011673:Pulsatile Flow; D011687:Purines / Q000494:pharmacology / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000494:pharmacology / Q000627:therapeutic use*; D014652:Vascular Diseases / Q000188:drug therapy* / Q000209:etiology; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate