Presentation

We present the multi-imaging approach and treatment of a 27-year-old female referred to our hospital with a 2-year history of dyspnea, fatigue, unintentional weight loss, and atypical chest pain. The patient referred in the last month worsening dyspnea (NYHA II), headache, upper back pain, and arthralgia in the left wrist and right elbow. Physical examination was remarkable for carotidynia, a systolic murmur on the pulmonic area, fixed splitting of S2, decreased right brachial and radial pulse, and unequal blood pressures (left arm 105/55 mm Hg, right arm 116/72 mm Hg, left leg 130/74 mm Hg, right leg 128/60 mm Hg). Transthoracic echocardiography (TTE) revealed an ostium secundum atrial septal defect (ASD) with a left-to-right shunt (Qp:Qs of 1.66:1), right ventricular dilation, pulmonary artery systolic pressure (PASP) of 61 mmHg, pulmonary vascular resistance (PVR) of 3.7 Wood units, and a large pericardial effusion (Fig. 1a) suggestive of pulmonary hypertension (PH). Computed tomography angiography (CTA) showed concentric thickening of the ascending aorta, aortic arch, pulmonary artery (PA), and right PA (RPA), confirming the diagnosis of Takayasu arteritis (TA) Numano IIa P + . Magnetic resonance angiography (MRA) showed stenotic areas in the superior lobar arteries of the RPA (Fig. 1b-c). Pericardial effusion was severe, with a maximum separation of 36 mm by TTE. A pericardial window and biopsy demonstrated leukocytoclastic vasculitis; pericardial fluid was positive for antinuclear antibodies. Medical treatment consisted of methylprednisolone and cyclophosphamide.

Fig. 1
figure 1

a. Transthoracic echocardiography (TTE) showing right ventricular dilation, pericardial effusion (white arrows), and increased tricuspid flow respiratory variation. b. Magnetic resonance angiography (MRA): Stenosis of the ascending branches (white arrows) of the RPA. Important pericardial effusion of 36 mm (red arrow). c. MRA: Significant reduction of the pulmonary flow in the right superior lobe (white arrow). d. Right pulmonary artery (RPA) fluoroscopy: Diffuse stenosis of the superior trunk of the RPA (white arrows). e. RPA fluoroscopy. Post-balloon dilation shows improved pulmonary blood flow after the percutaneous transluminal pulmonary angioplasty. f. Follow-up 2D Doppler TTE. Ostium secundum atrial septal defect with a left to right shunt

Full size image

During follow-up, cardiovascular and rheumatology teams decided on an interventional approach for treating the PH. Catheterization confirmed diagnosis of PH, with PASP of 50 mmHg, mean pulmonary artery pressure (MPAP) of 25 mmHg, transpulmonary gradient of 23 mmHg, PVR of 5.2 Wood units, and Qp:Qs of 2.5:1. Percutaneous transluminal pulmonary angioplasty (PTPA) was performed, requiring three dilations in the segmental lesions of the RPA superior lobar arteries, achieving a post-dilation PASP of 45 mmHg and an MPAP of 23 mmHg (Fig. 1d-e). Follow-up TTE 1 month after the PTPA showed a PASP of 40 mmHg (Fig. 1f).

Discussion

The prevalence of PA involvement (PAI) patients with TA has been reported in various studies as between 15.9% and 57%, depending on the patient population, imaging modality, and diagnostic criteria used [1, 2]. TA patients with PAI have a higher risk of developing PH than patients with only TA, and PH increases morbidity and mortality in these patients [3]. In a study by Sari et al., pericardial effusion was more frequent in TA patients with PH, but it did not reach statistical significance [4]. PH in patients with TA can often be secondary to causes other than PAI, such as left-heart disease or congenital heart defects, highlighting the importance of using multiple imaging modalities in cases of TA where PH is suspected. In the patient presented, both the ASD and TA contributed to the PH of the patient, as she presented PAI and a significant left-to-right shunt. The treatment of PH in TA is challenging and depends on the severity of PH, the underlying aetiology, and the patient's comorbidities. Although there is a lack of therapeutic experience with endovascular treatment in patients with TA, PTPA has become a promising approach for PH in these patients [5].