An unexpected cause of pulmonary hypertension in a young woman: a case report

Abstract

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 dyspnoea and syncope. The transthoracic echocardiographic findings were consistent with a cor pulmonale. Right heart catheterization (RHC) 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 tumour thrombotic microangiopathy the most probable cause of PH. Follow-up after successful oncologic treatment and cumulative 10 months of specific PH medication showed an excellent clinical response with complete remission of PH confirmed by RHC at rest.

Discussion: Tumour-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 tumoural emboli in pulmonary microcirculation in ∼26% of patients with a solid tumour, markedly associated with adenocarcinoma. Prompt initiation of cancer treatment on tumoural PH is essential. To our knowledge, this report documents the first full recovery of tumoural 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.

Keywords: Pulmonary hypertension WHO group 5; case report, tumoral pulmonary hypertension; pulmonary tumor thrombotic microangiopathy.

Figure 1

Initial electrocardiogram (A) and follow-up at week 14 (B).

Figure 2

Computed tomography of the lung. (A) Week 1: normal lung parenchyma. (B) Week 4: bipulmonary subpleural nodular ground glass opacities with ‘feeding vessel sign’.

Figure 3

Ventilation–perfusion scintigraphy with follow-up. Week 1: (A) homogenous ventilation and (B) small peripheral perfusion defects. Week 47: (C) homogenous ventilation and (D) homogenous perfusion.