Background
Antiphospholipid syndrome (APS) may cause chronic thromboembolic pulmonary hypertension
(CTEPH). Current knowledge regarding prevalence and risk factors for CTEPH among APS
patients is limited. We sought to determine clinical features and biomarkers that
could identify APS subjects suffering from CTEPH, and describe the prevalence, course
and treatment outcomes of patients with APS-CTEPH.
Methods
504 APS patients were treated in our center during 2008 to 2019. We studied clinical
and laboratory features of 69 APS patients, comparing 19 patients diagnosed with CTEPH
(APS-CTEPH) and treated accordingly, with 50 consecutive age and gender matched patients
with no evidence of pulmonary hypertension (APS-No-CTEPH).
Results
CTEPH prevalence was 3.8% in our APS cohort and was linked with the following parameters:
primary APS (p < 0.05); prior pulmonary embolism (p < 0.001); recurrent venous thromboembolism (VTE) (p < 0.001); lower platelet counts (p < 0.001); triple anti-phospholipid antibodies positivity (p < 0.001), higher titers of anti-cardiolipin IgG (p < 0.001), anti-B2GPI IgG (p < 0.001), and high Russell viper venom time ratio (RVVT-ratio) (p < 0.05). Additionally, history of catastrophic APS was more prevalent in APS-CTEPH
vs APS-No-CTEPH (p < 0.05). Of APS-CTEPH patients, 15/19 underwent pulmonary endarterectomy (PEA): In
12/15 the procedure was elective and resulted in good perioperative and long-term
outcomes, while only 1 of 3 patients that underwent urgent PEA survived.
Conclusions
CTEPH is relatively common in APS. Primary APS, prior PE, recurrent VTE, thrombocytopenia
and specific anti-phospholipid antibodies predict CTEPH in APS. Active assessment
for CTEPH in APS patients should be considered, as PEA was found to be effective and
relatively safe, especially if electively performed.
KEYWORDS
Abbreviations:
APS (antiphospholipid syndrome), aPL (antiphospholipid antibodies), ARDS (acute respiratory distress syndrome), CAPS (catastrophic antiphospholipid syndrome), CTEPH (chronic thromboembolic pulmonary hypertension), DVT (deep vein thrombosis), HIT (heparin induced thrombocytopenia), INR (international normalized ratio), mPAP (mean pulmonary arterial pressure), PE (pulmonary embolism), PEA (pulmonary endarterectomy), PH (pulmonary hypertension), PTT (partial thromboplastin time), RVVT (Russell viper venom time), SPAP (systolic pulmonary artery pressure), VTE (venous thromboembolism)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: October 27, 2021
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