Hypoplastic Right Heart Syndrome (HRHS)

Hypoplastic right heart syndrome refers to a congenital heart defect characterized by underdevelopment of the right-sided structures of the heart. These defects cause inadequate blood flow to the lungs and thus, a blue or cyanotic infant. This defect involves the pulmonary valve atresia which has not formed, a very small right ventricle, a small tricuspid valve and a small hypoplastic pulmonary artery.  As the ventricle has failed to grow and develop the ventricles muscle structure is poor, so additional problems are encountered as the heart attempts to pump blood to the pulmonary valve for transfer to the lungs. HRHS requires immediate emergency treatment, as the heart begins to fail almost immediately after birth.

Depakote and Hypoplastic Right Heart Syndrome (HRHS)

Recent studies suggest a potential link between infants born with HRHS and mothers who were taking the prescription drug Depakote during pregnancy. Besides Depakote and Spina Bifida, recent medical data also suggests that other heart related deficiencies may arise as a result of Depakote use during pregnancy.

Due to the severe birth defects associated with Depakote the FDA has now cautioned women about taking this drug during pregnancy.(See Depakote FDA Warning)  Many experts now agree that even these recent warnings are inadequate to convey the true risk of birth defects such as heart defects.  Despite the congenital birth defects, such as heart defects, which can result from a pregnant woman taking Depakote has still failed to require pregnancy tests and mandatory birth control for women of child bearing age who are taking the drug.