Endovascular Treatment of Bilateral Pulmonary Artery Stenoses and Superior Vena Cava Syndrome in a Patient with Advanced Mediastinal Fibrosis

Abstract

1 In severe cases, it can progress to obstruction of the superior vena cava (SVC), pulmonary vessels, esophagus, or airways. Whereas SVC obstruction is relatively common, pulmonary artery (PA) involvement is less frequently encountered.2 Very few reports of long-term outcomes exist in the medical literature of either SVC or PA stents in this patient population. We discuss a case in which MF resulted in PA and SVC stenoses that were treated endovascularly, with early symptom improvement and 5-year survival with primary patency. Case ReportA 54-year-old man with histoplasmosis presented at our institution with worsening right-sided chest tightness and difficulty sleeping on his right side because of shortness of breath. Three years before this episode, he had developed SVC syndrome from progressive MF that we had successfully treated endovascularly with an SVC stent. Therefore, differential diagnosis for the current episode of dyspnea included restenosis of the SVC stent, pulmonary embolism, pneumonia, and pulmonary hypertension. A computed tomographic angiogram (CTA) revealed high-grade right-PA stenosis caused by continued progression of MF. In addition, imaging revealed near-complete occlusion of the truncus anterior to the right PA. Results of CTA also revealed that the SVC stent remained widely patent.Subsequently, we performed pulmonary arteriography to evaluate right-PA stenosis and PA hypertension, and to judge the possibility of intervention. Using a femoral approach, we advanced a Mott pulmonary catheter over the wire and into the right ventricle. The catheter was next advanced into the main PA, where the pressure was 35/12 mmHg, and then into the left PA-where digital subtraction arteriography revealed no abnormalities. The left main PA pressure was 36/10 mmHg. The catheter was then modified to make it more amenable to entering the stenosed right main PA. We used a 260-cm, modified stiff Glidewire ® (Terumo Medical Corporation; Somerset, NJ), advancing the wire and catheter into the right main PA. Digital subtraction arteriography revealed approximately 75% stenosis at the origin of the right main PA, extending distally 3.6 cm (Fig. 1). A pressure recording obtained distal to the obstruction was 11/5 mmHg (pressure gradient, 22 mmHg). Given the patient's symptoms, imaging findings, and pressure gradient, intervention was judged necessary.

Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis

“…Catheter angiography was once the modality of choice for evaluation of vascular stenosis and/or compromise due to FM but now has been largely replaced by CT angiography and MR angiography. The current main role of conventional angiography is assessment of the extent of disease before placement of vascular stents and evaluation of stent and/ or vascular patency if CT angiographic and MR angiographic findings are indeterminate (36,37).…”

“…Nonsurgical and surgical procedures are reserved for patients with clinical symptoms directly related to compression and/or obstruction of mediastinal structures. Nonsurgical procedures such as percutaneous transluminal angioplasty with or without stent placement may be used in patients with symptomatic or bilateral vascular obstruction (Figs 19, 20) (36,37,41,43,(69)(70)(71)(72). Studies have shown these procedures to be generally safe and effective when performed by experienced providers and in the appropriate patient population.…”

Pulmonary Arterial Bypass Surgery for Fibrosing Mediastinitis Causing Severe Pulmonary Hypertension

“…Brown and colleagues [4] published a report about operative strategies in MF with PA occlusion, in which 5 patients with PA occlusion were described who The limitations for surgical intervention were listed as extensive fibrosis, calcification, and collateral vessels [4]. Stenting may be another option to relieve symptoms of pulmonary hypertension with an extraluminal component only [5]. Although successful PA stenting has been reported in case series, the long-term outcomes are mostly unknown.…”