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Platypnea–orthodeoxia syndrome

Orthostatic dyspnea and possible pathophysiological substrates

Platypnoe-Orthodeoxie-Syndrom

Orthostatische Dyspnoe und mögliche pathophysiologische Substrate

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Abstract

Platypnea–orthodeoxia syndrome (POS) is a rare disorder characterized by the emergence of a right-to-left shunt at the intracardiac or intrapulmonary level. The clinical picture is distinguished by shortness of breath that worsens on standing due to an accentuation of oxygen desaturation, and instead improves, at least partly, in the recumbent position. In this article we present a brief review of the pathophysiology of POS, as well as its clinical picture, diagnostic assessment, and preferential therapeutic options. Pathophysiological issues that are still not completely understood or much debated are outlined. The currently accepted pathophysiological concepts are presented and a summary of the main diagnostic and therapeutic tools is provided.

Zusammenfassung

Das Platypnoe-Orthodeoxie-Syndrom (POS) ist eine seltene Erkrankung, die durch die Entstehung eines intrakardialen oder intrapulmonalen Rechts-links-Shunts charakterisiert ist. Das klinische Bild ist von Kurzatmigkeit geprägt, die sich im Stehen aufgrund einer Akzentuation der Sauerstoffentsättigung verschlechtert und im Liegen, zumindest teilweise, verbessert. In diesem Artikel präsentieren die Autoren einen kurzen Review über die Pathophysiologie des POS sowie dessen klinisches Bild, diagnostische Untersuchungen und bevorzugte therapeutische Möglichkeiten. Pathophysiologische Probleme, die noch nicht vollständig verstanden sind oder über die viel diskutiert wird, werden angesprochen. Die derzeit akzeptierten pathophysiologischen Konzepte werden vorgestellt, und es wird eine Zusammenfassung der wichtigsten diagnostischen und therapeutischen Maßnahmen gegeben.

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Authors and Affiliations

  1. Cardiology Unit, Presidio Sanitario Intermedio “Elena d’Aosta”, via Cagnazzi 29, ASL Napoli 1 Centro, 80137, Naples, Italy

    R. De Vecchis, C. Ariano, A. Giasi & C. Cioppa

  2. Heart Department, Interventional Cardiology, A.O.U. “San Giovanni di Dio e Ruggi d’Aragona”, Salerno, Italy

    C. Baldi

  3. via P. Gaurico 21, 80125, Naples, Italy

    R. De Vecchis

Authors
  1. R. De Vecchis
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  2. C. Baldi
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  3. C. Ariano
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  4. A. Giasi
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  5. C. Cioppa
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Correspondence to R. De Vecchis.

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R. De Vecchis, C. Baldi, C. Ariano, A. Giasi, and C. Cioppa state that they have no competing interest.

This article does not contain any studies with human participants or animals performed by any of the authors.

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De Vecchis, R., Baldi, C., Ariano, C. et al. Platypnea–orthodeoxia syndrome. Herz 42, 384–389 (2017). https://doi.org/10.1007/s00059-016-4479-4

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