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Chronic thromboembolic pulmonary hypertension and vascular transformation of the lymph node sinuses
M Meysman1, M Diltoer, H D Raeve
1Dept of Internal Medicine, Academic Hospital, University of Brussels (AZ-VUB), Belgium.
Insights
Chronic thromboembolic pulmonary hypertension (CTEPH) is often diagnosed late. This case highlights CTEPH mimicking interstitial lung disease, with unusual lymph node changes, emphasizing diagnostic challenges.
Area of Science:
- Cardiology
- Pulmonology
- Pathology
Background:
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive condition often diagnosed late.
- Current medical management does not reverse disease effects or prevent progression.
- Pulmonary thromboendarterectomy is the primary treatment for CTEPH.
Observation:
- A patient presented with symptoms initially mistaken for interstitial lung disease.
- Mediastinal lymph node enlargement was noted, initially attributed to the suspected lung disease.
- The lymph node enlargement was pathologically identified as vascular transformation of the lymph node sinuses (VTS).
Findings:
- The patient's condition was ultimately diagnosed as CTEPH.
- Vascular transformation of the lymph node sinuses (VTS) was an unusual finding associated with CTEPH in this case.
- This presentation underscores the potential for misdiagnosis in advanced CTEPH.
Implications:
- Recognizing unusual presentations of CTEPH is crucial for timely diagnosis and treatment.
- VTS should be considered in the differential diagnosis of mediastinal lymphadenopathy in patients with suspected pulmonary hypertension.
- Improved diagnostic strategies are needed to identify CTEPH earlier, especially in atypical cases.
Abstract:
Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease often not recognized until it is far advanced. Medical management does not reverse the effects of the disease, nor does it prevent its progression. Pulmonary thromboendarterectomy is the preferred treatment. We present the case of a patient in whom CTEPH was mistaken for an interstitial lung disease with mediastinal lymph node enlargement. The mediastinal lymph node enlargement was due to vascular transformation of the lymph node sinuses (VTS). This is an unusual case of chronic thromboembolic pulmonary hypertension with vascular transformation of the lymph node sinuses.