Pulmonary Vascular Disease: Student/Resident Case Report Poster - Pulmonary Vascular Disease I |

Anti-Phospholipid Syndrome in a Patient With Class IV PAH FREE TO VIEW

Sarah Robison, DM; Jeffrey Garland, DM
Author and Funding Information

Mayo Clinic Florida, Jacksonville Beach, FL

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

Chest. 2016;150(4_S):1204A. doi:10.1016/j.chest.2016.08.1313
Text Size: A A A
Published online

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease I

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: Coagulation disorders contribute to a minority of patients with pulmonary arterial hypertension secondary to chronic thromboembolic disease. However, a high index of suspicion should be taken when the patient has recurrent pulmonary emboli.

CASE PRESENTATION: A 53yo gentleman had multiple venous thromboembolic (VTE) events between 2012 and 2015 despite anticoagulation with warfarin and rivaroxaban. A work-up for coagulopathy was negative. He was diagnosed with CTEPH by right heart catheterization and was treated with riociguat, furosemide and warfarin. He established care at our institution for a second opinion. A CT angiogram of the chest showed new acute thrombi. He was started on a heparin infusion. Prior to initiation of the heparin, he had an elevated partial thromboplastin time (aPTT), 76 sec. Within 12 hours after starting the heparin, his aPTT was 138 sec. The heparin was discontinued and 48 hours later, the aPTT was still elevated. Given the prolonged aPTT, the possiblity of a coagulopathy was investigated. Lab studies for vasculitis and autoimmune disorders, including antiphospholipid antibodies and beta-2 glycoprotein1 antibodies, were negative. In a mixing study, the PT corrected completely and the aPTT partially corrected. Factors V, VII, and X were decreased. The dilute Russell viper venom time and the StaClot LA were positive, consistent with lupus anticoagulant. The patient was diagnosed with anti-phospholipid syndrome based on these studies. Factor Xa levels were used to monitor anticoagulation with heparin (as opposed to aPTT levels) during his hospitalization. At the time of discharge, he was transitioned to low molecular weight heparin, and recommended to continue this therapy indefinitely.

DISCUSSION: The majority of patients with chronic thromboembolic pulmonary hypertension do not have an identifiable coagulopathy. Initially, it seemed this gentleman was such a patient. However, a single abnormal lab test (an elevated partial thromboplastin time) prompted a repeat hematologic evaluation and lupus anticoagulant was identified. This is important because it had a significant impact on his hospital and outpatient management. Moreover, the diagnosis of antiphospholipid syndrome had an influence on future hospitalizations. When he was admitted to the ICU 3 months later for acute kidney injury, new thromboses and thrombocytopenia catastrophic antiphospholipid syndrome was considered in the differential.

CONCLUSIONS: Pro-thrombotic conditions change the management of patients with chronic thromboembolic pulmonary hypertension; accurate diagnosis is essential.

Reference #1: Piazza G, et al. Chronic Thromboembolic Pulmonary Hypertension. N Engl J Med (2011); 364:351-60.

Reference #2: Wolf M, et al. Thrombotic risk factors in pulmonary hypertension. Eur Respir J (2000); 15:395-9.

Reference #3: Sciascia S, et al. Catastrophic antiphospholipid syndrome (CAPS). Best Practice & Research Clinical Rheumatology (2012); 26:535-41.

DISCLOSURE: The following authors have nothing to disclose: Sarah Robison, Jeffrey Garland

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