Pulmonary Hemorrhage During Cardiac Catheterization is Common in Children With Alagille Syndrome

Background:
Children with Alagille syndrome (AGS) are known to have a high incidence of bleeding complications during invasive procedures. It has been our impression that catheterization-associated pulmonary hemorrhage is more common in children with AGS, but there are no published data on this topic. We reviewed our experience to evaluate the incidence and severity of pulmonary hemorrhage in children with AGS, along with factors associated with pulmonary hemorrhage.

Methods:
This is a retrospective single institution study of children with AGS undergoing catheterization. Pulmonary hemorrhage was defined as extravasated blood in the lung parenchyma by fluoroscopy or frank blood suctioned from the endotracheal tube, and severity was defined by duration of mechanical ventilation. Univariate comparisons were made between catheterizations that did and did not have hemorrhages.

Results:
Thirty children with AGS underwent 87 catheterizations, 14 (44%) with interventions on the branch pulmonary arteries (PA). There were 26 (30%) procedures with hemorrhage, the majority (65%) of which were self-limited or required less than 24 hours of mechanical ventilation. Moderate and severe hemorrhages occurred only in children with variants of Tetralogy of Fallot (ToF). Higher right ventricular (RV) to aorta (Ao) pressure ratio (0.99 ± 0.28 vs. 0.83 ± 0.32, p = 0.039) and interventions on the branch PAs (14/26 (53.8%) vs. 18/61 (29.5%), p = 0.032) were associated with hemorrhage.

Conclusions:
Pulmonary hemorrhage is common in children with AGS undergoing cardiac catheterization, and is associated with ToF, high RV:Ao pressure, and interventions on the branch PAs.