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Updated: Oct 22, 2025
07:11
Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis
Published on: May 23, 2025
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A 57-year-old man with rapidly progressive pulmonary hypertension
Federico Raimondi1, Caterina Conti2, Luca Novelli3
1Pulmonary Medicine Unit, ASST Papa Giovanni XXIII Hospital, Bergamo; University of Milan. fraimondi@asst-pg23.it.
Summary
Pulmonary Tumor Thrombotic Microangiopathy (PTTM) is a rare, fatal condition causing pulmonary hypertension, often linked to cancer. Early consideration of PTTM in unexplained pulmonary hypertension is crucial for potential diagnosis.
Area of Science:
- Cardiovascular Medicine
- Oncology
- Pathology
Background:
- Pulmonary Tumor Thrombotic Microangiopathy (PTTM) is a rare condition.
- It is associated with neoplastic disorders, primarily gastric cancer.
- PTTM leads to pre-capillary Pulmonary Hypertension (PH).
Observation:
- The pathologic mechanism involves fibrocellular intimal proliferation in small pulmonary vessels.
- Carcinomatous cells lodged in pulmonary vasculature sustain this proliferation.
- Clinical presentation is nonspecific, including progressive dyspnea and dry cough.
Findings:
- Diagnosis of PTTM is challenging ante-mortem with a poor prognosis.
- A case of a middle-aged man with rapidly progressive dyspnea and PH, diagnosed post-mortem, is described.
- The patient had no known prior cancer history, highlighting diagnostic difficulty.
Implications:
- PTTM should be considered in patients with unexplained, rapidly progressive PH.
- Consider PTTM in cases poorly responsive to standard treatments or with suspected malignancy.
- Prompt diagnosis of PTTM can improve understanding of this under-recognized condition.
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