Insight Into the Multifactorial Nature of Platypnea-Orthodeoxia Syndrome: Not Just a Solo

Gabriele De Palma; Rosaria Barracano; Nunzia Borrelli; Mario Giordano; Giuseppe Paone; Federica Lo Presti; Rosa Cauteruccio; Giancarlo Scognamiglio; Alessandro Della Corte; Berardo Sarubbi

doi:10.1016/j.jaccas.2026.107353

Insight Into the Multifactorial Nature of Platypnea-Orthodeoxia Syndrome: Not Just a Solo

JACC Case Rep. 2026 Apr 15;31(15):107353. doi: 10.1016/j.jaccas.2026.107353. Epub 2026 Mar 14.

Affiliations

¹ University of Campania Luigi Vanvitelli, Naples, Italy; Adult Congenital Heart Disease and Congenital and Familial Arrhythmias Unit, Monaldi Hospital, Naples, Italy.
² Adult Congenital Heart Disease and Congenital and Familial Arrhythmias Unit, Monaldi Hospital, Naples, Italy.
³ Pediatric Cardiology Unit, Monaldi Hospital, Naples, Italy.
⁴ Department of Translational Medical Sciences, Cardiac Surgery Unit, University of Campania Luigi Vanvitelli, Naples, Italy.
⁵ Department of Respiratory Pathophysiology and Rehabilitation, Monaldi Hospital, Naples, Italy.
⁶ Adult Congenital Heart Disease and Congenital and Familial Arrhythmias Unit, Monaldi Hospital, Naples, Italy; University of Naples Parthenope, Naples, Italy. Electronic address: berardo.sarubbi@ospedalideicolli.it.

PMID: 41823912
DOI: 10.1016/j.jaccas.2026.107353

Abstract

Background: Platypnea-orthodeoxia syndrome (POS) is a rare condition with complex pathophysiology resulting from an anatomical site of shunting, mostly a patent foramen ovale (PFO), and factors promoting a right-to-left (R-L) shunt.

Case summary: A 54-year-old man was referred to our center for unexplained respiratory failure, exacerbated by sitting. A suspicion of POS was raised, and transesophageal echocardiography demonstrated a wide stretched PFO with R-L shunting, related to the presence of an aortic root aneurysm and right hemidiaphragm elevation, both compressing the right atrium, as well as a prominent eustachian valve. The patient underwent valve-sparing aortic root replacement and concomitant PFO closure, with resolution of symptoms.

Take-home messages: Multiple factors promoting R-L shunt have been associated with POS. The treatment of choice is represented by the correction of the anatomical shunt. Multidisciplinary team discussion is paramount for the diagnosis and management of this complex condition.

Keywords: ascending aorta aneurysm; hemidiaphragm elevation; patent foramen ovale; platypnea-orthodeoxia syndrome.

Publication types

Case Reports