Cardiothoracic Surgery: Fellow Case Report Poster - Cardiac and Transplant Surgery |

A Double Whammy: Platypnea-Orthodexia Syndrome Due to Both Trans-Pulmonary and Trans-Atrial Shunting FREE TO VIEW

Raju Reddy, MD; Ray Matthews, MD
Author and Funding Information

University of Southern California, Los Angeles, CA

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):40A. doi:10.1016/j.chest.2016.08.047
Text Size: A A A
Published online

SESSION TITLE: Fellow Case Report Poster - Cardiac and Transplant Surgery

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Platypnea-orthodeoxia syndrome (POS) is characterized by platypnea-orthodeoxia, right to left intra-cardiac shunting and normal right atrial (RA) pressures. We report a case of POS in a 53 year-old man with a history of cryptogenic liver cirrhosis, type 2 diabetes mellitus, hepatopulmonary syndrome (HPS) and a patent foramen ovale (PFO), who underwent a liver transplant followed by worsening hypoxemia unexpectedly.

CASE PRESENTATION: Pre-transplant, he required oxygen (O2) at 2 liters per minute (lpm) to maintain saturations (SaO2) ≥ 88%. However, post-transplantation, his oxygen requirements increased to 10 lpm at rest and up to 25 lpm during upright activity to maintain SaO2 ≥ 88%. Computed tomography angiogram of the chest was negative for pulmonary embolism, arteriovenous malformations or a parenchymal process. Contrast enhanced echocardiography showed both right to left intra-cardiac and trans-pulmonary shunting. However, right heart catheterization noted normal pressures in the RA, right ventricle and pulmonary artery. At 1 month post-transplant, he remained severely hypoxemic. The patient was treated with intra-venous methylene blue with transient improvement in O2 requirements. At 4 months post-transplant, reassessment of the patients’ shunt fraction showed significant progression from 11% pre-operatively to 28%. Trans-esophageal echocardiography again showed the presence of right to left intra-cardiac shunting. The PFO was closed percutaneously using an Amplatzer closure device. The patient’s oxygenation showed immediate improvement, reducing resting O2 from 10 to 4 lpm and from 25 to 10 lpm during upright activity.

DISCUSSION: The mainstay of therapy for HPS is supplemental O2 and liver transplantation both of which improve mortality. However, in about 6% - 21% of cases, hypoxemia worsens post-transplantation due to the presence of pulmonary microvascular dilations. In our case, hypoxemia was multifactorial - both pulmonary vascular dilations and the PFO. POS and intra-cardiac shunting can occur in the presence of normal right atrial pressures. This is a result of distortion of cardiac anatomy that favors the passage of blood that enters the right atrium into the inter-atrial communication especially with activity or valsalva maneuvers.

CONCLUSIONS: This case highlights the effectiveness of closure of intra-cardiac shunts in POS.

Reference #1: Mojadidi MK et al. The effect of patent foramen ovale closure in patients with platypnea-orthodeoxia syndrome. Catheter Cardiovasc Interv. 2015;86(4):701-7.

DISCLOSURE: The following authors have nothing to disclose: Raju Reddy, Ray Matthews

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543