Repair of Congenital Cardiac Disease Requiring Bypass for a Pediatric Patient With Paroxysmal Nocturnal Hemoglobinuria

Background

Paroxysmal nocturnal hemoglobinuria (PNH) is a life-threatening disease characterized by cytopenias, hemolysis, and thrombosis. There are only a handful of documented cases of cardiac bypass surgery performed on patients with PNH, none in the pediatric population.

Case Summary

A 16-year-old male adolescent presented with abdominal pain and fatigue, with laboratory studies revealing severe anemia, thrombocytopenia, and leukopenia. Based on flow cytometry, he was diagnosed with PNH. During cardiac screening prior to central line placement, he was incidentally found to have superior sinus venosus atrial septal defect with partial anomalous pulmonary venous return. The patient required surgical repair of the cardiac defect prior to PNH treatment with stem cell transplant.

Discussion

Cardiac surgery in patients with PNH requires careful multidisciplinary planning, including cardiology, cardiothoracic surgery, hematology, and infectious disease.

Take-Home Message

The presence of PNH alongside a cardiac defect in a pediatric patient highlights the complexity and challenges of performing cardiac bypass in this population.