Sickle Cell Disease and Complex Congenital Cardiac Surgery: A Case Report and Review of the Pathophysiology and Perioperative Management

Sickle cell anemia and thalassemia are hemoglobinopathies rarely
encountered in the United States. Compounded with congenital heart
disease, patients with sickle cell disease (SCD) requiring
cardiopulmonary bypass and open-heart surgery represent the proverbial
“needle in the haystack”. As such, there is some trepidation on the part
of clinicians when these patients present for complex cardiac surgery.
SCD is an autosomal, recessive condition that results from a single
nucleotide polymorphism in the β-globin gene. Hemoglobin SS molecules
(HgbSS) with this point mutation can polymerize under the right
conditions, stiffening the erythrocyte membrane and distorting the
cellular structure to the characteristic sickle shape. This shape change
alters cellular transit through the microvasculature. As a result,
circumstances such as hypoxia, hypothermia, acidosis or diminished blood
flow can lead to aggregation, vascular occlusion and thrombosis.
Chronically, SCD can give rise to multiorgan damage secondary to
hemolysis and vascular obstruction. This review and case study details
an 11-year-old African-American male with known SCD who presented to the
cardiothoracic surgical service with congenital heart disease
consisting of an anomalous, intramural right coronary artery arising
from the left coronary sinus for surgical consultation and subsequent
surgical correction. This case report will include a review of the
pathophysiology and current literature regarding preoperative,
intraoperative and postoperative management of SCD patients.