[Effective long-term noninvasive ventilation of a woman with kyphoscoliosis and sinus venosus atrial septal defect].

Atrial septal defects comprise one of the most common congenital cardiac malformations in adults and adolescents. However, such septal defects are rarely associated with chest wall diseases. When they are, respiratory failure soon develops and is severe. Cases in which the long-term course of disease is favorable with noninvasive ventilation have not been reported in the literature. We present the case of a 71-year-old woman with kyphoscoliosis, sinus venosus atrial septal defect, severe pulmonary hypertension, respiratory failure, and heart failure. We describe her response to treatment with noninvasive mechanical ventilation.