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Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
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Pulmonary tumour thrombotic microangiopathy
Laura C Price1, Athol U Wells, Stephen J Wort
1aNational Pulmonary Hypertension Service bInterstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
Current Opinion in Pulmonary Medicine
|July 9, 2016
Summary
Pulmonary tumour thrombotic microangiopathy (PTTM) is a rare cause of severe pulmonary hypertension linked to cancer. Early diagnosis and targeted therapies, including PDGF inhibition, are improving survival for this underdiagnosed condition.
Area of Science:
- Cardiovascular Medicine
- Oncology
- Pulmonary Medicine
Background:
- Pulmonary tumour thrombotic microangiopathy (PTTM) involves tumor cell microemboli causing fibrointimal remodeling in pulmonary vessels.
- This condition leads to severe and rapid onset pulmonary hypertension, often associated with carcinomas like gastric cancer.
- Vascular endothelial growth factor and platelet-derived growth factor (PDGF) signaling are implicated in the intimal remodeling process.
Purpose of the Study:
- To review the current understanding of Pulmonary Tumour Thrombotic Microangiopathy (PTTM).
- To highlight PTTM as an underdiagnosed cause of pulmonary hypertension.
- To discuss recent advances in diagnosis and treatment strategies for PTTM.
Main Methods:
- Review of existing literature on PTTM.
- Analysis of diagnostic modalities including right heart catheterization, HRCT, CT-PET, and histology.
- Evaluation of treatment outcomes with targeted therapies.
Main Results:
- PTTM is associated with poor prognosis, historically with survival of weeks to months.
- Diagnostic tools include aspiration of tumor cells, imaging (HRCT, CT-PET), and definitive histological analysis.
- Combination therapies involving vasodilators, anticoagulation, chemotherapy, and PDGF inhibitors show potential for prolonging survival.
Conclusions:
- PTTM is an increasingly recognized cause of pulmonary hypertension, often presenting with undiagnosed malignancy.
- Early recognition and diagnosis are crucial for improving patient outcomes.
- Targeted combination therapy offers improved survival prospects for patients with PTTM.
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