Abstract
BackgroundFetuses with hypoplastic left heart syndrome (HLHS) and intact interatral septum (IS) have a high perinatal mortality due to the impossibility to guarantee oxygenation at birth. The most effective way to manage this condition seems to create an interatrial communication in utero. We describe a case of IS stenting, performed through an alternative technical approach.Case presentationA 32 weeks gestation baby presented with HLHS with intact IS. A left side approach was electively planned, targeting a grossly dilated left pulmonary vein. This maneuver, avoiding the direct puncture of a cardiac chamber, allowed to more safely use a larger cannula and to deploy a larger stent. The procedure was uneventful and granted an adequate flow until the delivery.ConclusionsElective interatrial septoplasty through a left side approach seems to be promising in terms of safety and efficacy.
Highlights
Fetuses with hypoplastic left heart syndrome (HLHS) and intact interatral septum (IS) have a high perinatal mortality due to the impossibility to guarantee oxygenation at birth
Elective interatrial septoplasty through a left side approach seems to be promising in terms of safety and efficacy
The procedure is accomplished through the right atrium using a small (18 gauge) cannula
Summary
HLHS (Hypoplastic left heart syndrome) fetuses with intact interatrial sepum (IS) represents a clinical subset with a high mortality, primarily due to an almost unmanageable hemodynamic condition at birth. This is the first report of a 32 weeks fetus with HLHS syndrome and an intact IS who underwent IS stenting, contemplating a new alternative approach targeting the left pulmonary vein (LPV). Case presentation HLHS with intact IS was diagnosed at 32 weeks of gestation, as documented by absence of flow across the septum, and dilated pulmonary veins with a biphasic flow (Fig. 1a). The IS appeared thickened and protruding toward a large right atrium. Owing to this particular anatomy, we planned a procedure of septal stenting from the LPV. The stent was properly positioned and completely patent ensuring an unrestricted left to right shunt (Fig. 1d). Upon Norwood stage one procedure, the stent appeared firmly placed across the septum and was removed. Thereafter, the child underwent a second stage palliation and, total cavo-pulmonary connection uneventfully
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