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Cyanosis in atrial septal defect without pulmonary hypertension: a case of Platypnea-orthodeoxya syndrome

Department of Cardiac Surgery, Silvestrini Hospital, Perugia, Italy

*Corresponding author: Divisione di Cardiochirurgia, Ospedale Silvestrini, S. Andrea delle fratte, Perugia, Italy. Tel.: + 39-75-5782213; fax: + 39-75-5782214. E-mail address: isidorodibella{at}yahoo.it (l. Di Bella).

Cyanosis in atrial septal defect typically occurs when pulmonary hypertension develops. Platypnea-orthodeoxya is an uncommon syndrome, still under debate, characterized by breathlesness and arterial oxygen desaturation exacerbated in the upright position. An interatrial communication is a common finding in this syndrome, but the absence of a right to left pressure gradient complicates the physiopathological picture. To explain the right to left shunt, it is generally advocated a concomitant condition that alterates the sterical relationship between inferior vena cava orifice and the atrial septal defect. A case of a 58-year-old male with platypnea-orthodeoxya syndrome related to a fenestrated redundant interatrial septum without any additional pathologic condition is reported. Possibly, this isolated anatomical abnormality could lead to a right to left shunt in the absence of other coexisting predisposing factors. It is reasonable to hypothesize the septum secundum bulging like a ‘spinnaker’ into the right atrium, so that it deviates the inferior vena cava venous blood towards the left atrium. Echocardiographic evaluation is mandatory to achieve a correct diagnosis and to decide the therapeutic strategy.

Key Words: Atrial septal defect; Intracardiac shunt; Cyanosis