Pulmonary arterial hypertension unveils itself: a cancer-like progression - a case report.

ABSTRACT:

Background

Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis, characterized by progressive remodelling of the small pulmonary arteries that precede the clinical and haemodynamic manifestations of the disease. Thus, a prompt diagnosis and early intervention are crucial.

Case summary

A 39-year-old pregnant women presented with persistent severe hypoxaemia after the diagnosis of influenza B and an elective caesarean delivery at 33 weeks. Ten months after, an extensive and inconclusive investigation that included a lung biopsy, despite of a spontaneous improvement in oxygen saturation, clinical deterioration led to further testing, namely genetic screening. It revealed a fast-progressing case of hereditary PAH caused by BMRP2 mutation.

Discussion<

SUBMITTER: Santos-Ferreira C 

PROVIDER: S-EPMC8183656 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature