An unusual case of pulmonary arterial hypertension
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A 51-year-old Hispanic female with no relevant medical history exhibited progressive dyspnoea on exertion without haemoptysis, fever, malaise, skin lesions, or arthritis. She denied having oral or genital aphthae, as well as ocular or neurological diseases. Physical examination showed jugular ingurgitation, a wide splitting of the second heart sound, with a loud pulmonary component. Lung auscultation was normal. She had no clubbing or peripheral oedema.

Transthoracic echocardiogram revealed severe pulmonary hypertension (PH), with an estimated systolic pulmonary artery pressure of 91?mmHg, and signs of chronic right ventricle pressure overload.

Computed tomography angiography revealed pulmonary arteries with severe tortuosity and multiple foci of dilatation and stenosis, showing a ‘string of beads’ appearance, without evidence of pulmonary embolism. There were no lesions either in the carotid or renal arteries, suggestive of fibromuscular dysplasia.

Complete blood count, C reactive protein, and sedimentation rate were normal. A serological evaluation for systemic vasculitis and connective tissue diseases was negative.

Right heart catheterization showed precapillary PH (mean pulmonary artery pressure = 49?mmHg; pulmonary capillary wedge pressure = 9?mmHg; pulmonary vascular resistance = 11 Wood units), without intracardiac shunts. Pulmonary angiogram revealed multiple, bilateral aneurysms, scattered areas of narrowing with few intraluminal filling defects, and severely compromised perfusion of both lungs.

Specific therapy for pulmonary arterial hypertension was started, including Sildenafil, Ambrisentan, and inhaled Treprostinil.

Bilateral pulmonary artery aneurysms are very unusual findings, sometimes associated with congenital heart diseases with left-to-right shunting and systemic vasculitis, particularly Behçet disease. In this case, a patient with idiopathic PH developed this severe form of vasculopathy. Although the mechanism remains largely unknown, there appears to be a link between the vasa vasorum in the adventitia and hyper-proliferative plexiform lesions seen in advanced idiopathic PH, which could lead to aneurysm formation.