Key result
Reverse Potts shunt in a child with severe pulmonary arterial hypertension was complicated by significant hemothorax due to acquired von Willebrand disease.
Why the study?
Acquired von Willebrand disease is reported in children with severe PAH but had not previously been associated with bleeding following reverse Potts shunt.
Case Report (n=1)
Preoperative testing for acquired von Willebrand disease may be warranted in children with severe PAH undergoing reverse Potts shunt to prevent severe postoperative bleeding.
Acquired vWD may underlie bleeding after reverse Potts shunt in pediatric PAH; hypothesis-generating and requires prospective validation before screening changes practice.
The reverse Potts shunt is increasingly used as a palliative measure for end-stage pulmonary arterial hypertension (PAH) as a means to offload the right ventricle and improve functional status. This case report describes a child who developed significant hemothorax after reverse Potts shunt that required surgical exploration, blood product administration, and prolonged intensive care hospitalization. Despite lack of preoperative bleeding symptoms, testing revealed acquired von Willebrand disease (aVWD), with subsequent resolution of bleeding. Alterations in von Willebrand factor, including aVWD, have been reported in children with severe PAH but have not previously been associated with bleeding after reverse Potts shunt procedure. As bleeding is a recognized postoperative morbidity in PAH patients undergoing reverse Potts shunt, we highlight a potential role for preoperative testing for aVWD as perioperative factor replacement therapy may improve postoperative outcomes.
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Sullivan et al. (2022) conducted a case report in severe pediatric pulmonary arterial hypertension (n=1). Reverse Potts shunt was evaluated on Significant hemothorax and diagnosis of acquired von Willebrand disease. Reverse Potts shunt in a child with severe pulmonary arterial hypertension was complicated by significant hemothorax due to acquired von Willebrand disease.
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