title: Hypoplastic left heart syndrome with intact atrial septum--attempt of an interventional palliation by ductal and interatrial stent implantation.
abstract: In patients with hypoplastic left heart syndrome (HLHS) and intact atrial septum, the blood entering the left atrium cannot egress. Emergency treatment interventionally or surgically is mandatory immediately after birth. We describe a patient with HLHS and intact atrial septum who underwent successful transvenous atrial septostomy immediately after birth. When the interatrial communication became restrictive, stent implantation into the arterial duct and into the atrial septum was performed on the 7th day of life. Despite good hemodymanic response, the lung damage was severe and persistent, rendering staged surgical correction impossible. The child died on the 23rd day of life. Autopsy showed patent and correct placed stents in the duct and the atrial septum. There was severe dilatation of pulmonary lymphatic and venous vessels, suggestive of long-standing pulmonary venous hypertension. In conclusion, this form of HLHS has a poor prognosis despite early and aggressive interventional treatment.
authors.affiliation: Klinik für Kinderkardiologie, Herzzentrum Leipzig GmbH, Universität Leipzig, Strümpellstr. 39, 04289 Leipzig, Germany. michael.weidenbach@medizin.uni-leipzig.de
authors.forename: Michael
authors.initials: M
authors.lastname: Weidenbach
authors.forename: Paul
authors.initials: P
authors.lastname: Caffier
authors.forename: Taja
authors.initials: T
authors.lastname: Harnisch
authors.forename: Ingo
authors.initials: I
authors.lastname: Daehnert
mesh_terms: D002404:Catheterization; D015897:Comorbidity; D017809:Fatal Outcome; D006325:Heart Atria; D006346:Heart Septum; D006801:Humans; D006976:Hypertension, Pulmonary / Q000151:congenital / Q000453:epidemiology; D018636:Hypoplastic Left Heart Syndrome / Q000453:epidemiology / Q000628:therapy*; D007231:Infant, Newborn; D010166:Palliative Care; D010996:Pleural Effusion / Q000453:epidemiology; D011379:Prognosis; D015607:Stents