Objective: The association of hypoplastic left heart syndrome (HLHS) and restrictive atrial septum (RAS) carries a bad prognosis, related to profound cyanosis after birth. Relief of pulmonary hypertension in the fetal life may attenuate secondary pulmonary abnormalities. We report the outcome of a fetus with HLHS and highly RAS, submitted to intrauterine atrioseptostomy. Pulmonary structural changes were evaluated 6 months after birth.
Methods: Fetal atrioseptostomy was carried out at 33 weeks’ gestation. Atrial septum was dilated with a balloon catheter, relieving left atrial hypertension. The fetus was delivered at term, with stable hemodynamics and respiratory function. Hybrid procedure was carried out on the 5th day of life, with PDA stent and adjustable pulmonary artery bands. Patient developed a restrictive atrial communication at 3 weeks of life, requiring radio frequency septal access to implant a stent across the atrial septum. At 6 months, a comprehensive stage 2 procedure was performed.
Results: Patient did not survive stage 2 procedure due to hemorrhagic complications. Lung biopsy revealed signs of persistent edema of the interlobular septa, besides focal thickenning of the alveolar walls. There was thickening of the vein wall, with duplication of the elastic lamina, and lymphatic dilation as well. The arterial bed showed only mild hypertrophy of the medial layer.
Conclusions: Fetal atrioseptostomy allowed a relief of pulmonary venous hypertension, and probably alter secondary pulmonary vascular changes, since good respiratory function and stable hemodynamics were evident postnatally. The ideal timing of the procedure to completely reverse associated pulmonary abnormalities remains unclear.