A female patient in her early 30s, with a medical history of pulmonary arterial hypertension associated with congenital heart disease, lost to follow-up, was referred to a pulmonary hypertension reference centre. The patient presented at a WHO functional class of II in a low-risk category. A transthoracic echocardiogram demonstrated severe pulmonary hypertension with right ventricular dysfunction. A cardiac MRI demonstrated a right aortic arch with anomalous origin of the left pulmonary artery from the ascending thoracic aorta and a patent ductus arteriosus. A right heart catheterisation confirmed the presence of severe pulmonary hypertension. The patient was started on combined vasodilator therapy with an improvement in symptoms and remaining in a low-risk category. This represents one of the oldest described cases of such an anomaly and the first description of response to vasodilator therapy, highlighting the importance of a structured approach in a reference centre for achieving optimal outcomes.
- Pulmonary hypertension
- Cardiovascular system
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Contributors JGS was responsible for the acquisition and interpretation of data, being the author who drafted the work. FF developed the conception and design of the work and together with SA were paramount in the analysis and interpretation of data. HP was responsible for overseeing the work, for the critical revision and the approval of the final version.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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