Total Aortic Arch and Neoaortic Root Replacement Following Stage III Palliation for Hypoplastic Left Heart Syndrome.

Progressive dilatation of the neoaortic root and reconstructed aortic arch is a serious complication after the Norwood procedure. There are no clear guidelines on the management of this complex anatomy in the setting of single ventricle physiology, and the surgical treatment of such an entity remains anecdotal. We describe a successful surgical repair in a 15-year-old girl presenting with a severely dilated neoaortic root and aortic arch causing compression and narrowing of the left pulmonary artery after successful three-stage palliation for hypoplastic left heart syndrome.

Neonatal aortic dilatation secondary to vitamin A deficiency.

We report on a term neonate with unexplained respiratory distress, dilatation of the descending aorta, and low serum retinol concentration. The respiratory distress did not respond to conventional medical management and persisted for 22 days requiring an inspired oxygen fraction of 0.4 to 0.5 to maintain adequate arterial oxygen saturation. One week after intramuscular vitamin A therapy, the respiratory distress and requirement for supplementary oxygen resolved. Dilatation of the distal aorta resolved at 7 weeks of age. An association between vitamin A deficiency and aortic dilatation has previously been described in rats, but the association in humans has rarely been reported. We suggest that unexplained neonatal respiratory distress and a dilated aorta should prompt suspicion of vitamin A deficiency. An underlying infective or inflammatory process may give rise to a falsely low serum retinol concentration. Serum retinol and retinol binding protein concentrations in both the mother and infant should be used to guide vitamin A status, treatment, and subsequent response.

Absent pericardium: at risk for endocarditis?

A 16-year-old girl with no previous cardiac problems presented to the casualty with septic shock. Investigations revealed endocarditis involving the mitral valve. At the time of surgery, she was noted to have a large left atrial appendage herniating into the left pleural cavity due to partial absence of the pericardium. Complete or partial absence of the pericardium is an uncommon congenital anomaly. Though various complications and presentations are reported, it has not been identified as a risk factor for endocarditis. We discuss the possible mechanism, and need for prophylaxis against endocarditis, in patients with absence of the pericardium.