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Pulmonary atresia, a rare and complex congenital heart disease, is characterized by the absence of the central pulmonary artery and by the presence of a ventricular septal defect and aortopulmonary collaterals. Pregnancy reports concerning maternal and offspring outcome after palliative operation or repaired pulmonary atresia are sparse. We report here on the outcome of pregnancy in a woman, aged 36, with complex pulmonary atresia in whom palliative operation had been performed at the age of 23. We review the medical literature on pregnancy course as well as maternal and foetal outcome in cases involving this maternal congenital heart disease.