Yonsei Med J.  2016 May;57(3):799-802. 10.3349/ymj.2016.57.3.799.

Platypnea-Orthodeoxia Syndrome Two Decades after Definitive Surgical Repair of Pulmonary Atresia with Intact Ventricular Septum

• Lee ML
• Chiu IS

• KMID: 2374105
• DOI: http://doi.org/10.3349/ymj.2016.57.3.799

Abstract

A 20-year-old female had undergone definitive surgical repair for pulmonary atresia with intact ventricular septum soon after birth. She was referred to our institution with the chief complaint of clubbing fingers. A thorough examination revealed platypnea-orthodeoxia syndrome due to an interatrial right-to-left shunt through a secundum atrial septal defect. Percutaneous closure with an Amplatzer Septal Occluder resulted in resolution of the syndrome.

Keyword

Clubbing fingers; platypnea-orthodeoxia syndrome; secundum atrial septal defect; Amplatzer Septal Occluder

MeSH Terms

Dyspnea/*diagnosis/*etiology/surgery
Female
Heart Defects, Congenital/complications/*surgery
Heart Septal Defects, Atrial/*complications/*diagnosis/surgery
Humans
Hypoxia
Pulmonary Atresia/complications/*surgery
*Septal Occluder Device
Syndrome
Treatment Outcome

Figure

• 

  Fig. 1 Platypnea-orthodeoxia syndrome treated by implantation of Amplatzer Septal Occluder (ASO). (A) Clubbing fingers were remarkable. (B) Contrast trans-thoracic echocardiography showed multiple bubbles in the right atrium and the right ventricle (open arrows), indicating the presence of interatrial right-to-left shunt. (C) Balloon sizing showed an indentation with a diameter of 21.84 mm. Intracardiac echocardiography showed (D) opening of left-side disc of ASO in the left atrium, (E) opening of right-side disc of ASO in the right atrium, and (F) complete occlusion of the secundum atrial septal defect after deployment of ASO. It is worthy of note that the ASO lie in (G) the present patient was somewhat more oblique than (H) the usual patients with a secundum atrial septal defect under fluoroscope (lateral 70 degrees; cranial 20 degrees).

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