Background
Pulmonary hypertension (PH) is defined as a progressive disease that leads to right heart failure and death if untreated. This case report presents a young woman with reversible precapillary PH in the setting of a gastric cancer.
Case Summary
A 37-year-old woman presented with exertional dyspnea and syncope. The transthoracic echocardiographic findings were consistent with a cor pulmonale. Right heart catheterization proved a precapillary PH. Specific PH therapy with macitentan and tadalafil was initiated. Shortly thereafter, a gastric carcinoma was diagnosed and oncologic treatment with neoadjuvant chemotherapy and subsequent gastrectomy was promptly initiated. Retrospectively, we considered a pulmonary tumor thrombotic microangiopathy the most probable cause of PH. Follow-up after successful oncologic treatment and cumulative ten months of specific PH medication showed an excellent clinical response with complete remission of PH confirmed by RHC at rest.
Discussion
Tumor-related PH is very rare and might be largely underdiagnosed as the clinical course often results in a rapid deterioration and fatal outcome before diagnostics are completed. Post mortem studies have documented tumoral emboli in pulmonary microcirculation in up to 26% of patients with a solid tumor, markedly associated with adenocarcinoma. Pprompt initiation of cancer treatment on tumoral PH is essential. To our knowledge, this report documents the first full recovery of tumoral PH at rest after successful cancer treatment and temporary specific PH medication. We therefore conclude that a multidisciplinary approach with an initially combined oncologic and PH therapy may be most beneficial with the potential of complete remission of PH.