You are following up a 28-year-old male in clinic who was referred by his GP for increasing breathlessness and intermittent palpitations. He has come back for the result of his TOE which has shown evidence of a superior sinus venosus ASD with normal pulmonary venous drainage and moderate right heart dilatation.
He asks you about the likely treatment.
A. Transcatheter ASD device closureThis patient has a sinus venosus ASD with symptoms and evidence of haemodynamic anatomical change. The defect is in the superior atrium at the entrance of the SVC into the RA (almost as if the SVC overrides the atrial septum). Currently, transcatheter solutions are not available and surgery is the treatment of choice. Sinus venosus ASDs are often associated with anomalous pulmonary venous drainage which requires surgical correction. If the shunt is not corrected, this may result in irreversible right heart dysfunction, pulmonary hypertension (late), or the development of atrial arrhythmias such as AF which complicate the situation. ACE inhibitors reduce LA pressure, and therefore in theory can reduce the shunt, but this is not a suitable treatment.