Renal artery angioplasty in Takayasu disease: Difficult anatomy addressed with a novel technique

Abstract

Takayasu arteritis is a well-established medical entity involving inflammatory changes in large arteries. We describe a characteristic case of Takayasu arteritis, in a young woman with significant renal artery stenosis, presenting with hypertensive urgency. Unsuccessful guide wire passage due to fibrotic septae in lumen of the left renal artery necessitated an innovative interventional approach, using an IMA-guide catheter and a UB3 coronary guidewire to cross the lesions, followed by placement of a coronary drug eluting stent, with an aim to avoid restenosis.

Introduction

Takayasu arteritis (TA) is a rare chronic inflammatory and granulomatous vascular disorder affecting large arteries of the body causing stenosis, occlusion, aneurysm(s), and other systemic manifestations.¹ The etiopathogenesis of TA remains an enigma, with implication of various infectious, genetic, and immune-mediated etiologies.² First-line management of TA includes drug therapy with the aim of immunosuppression and inflammation reduction if there is disease activity. However, in cases with severe vascular complications, endovascular interventions and revascularization procedures are the mainstay.³ Here, we present a young female who was found to have severe left renal artery (LRA) stenosis due to TA, causing multiple fibrotic septae within the renal artery, resulting in failure of guide wire crossing. This problem was eventually treated with a unique approach with modifications in both technique and equipment, while also successfully placing a coronary drug eluting stent (DES), which is an extremely rare scenario in such cases. This was done owing to extensive fibrosis, and with the aim to avoid restenosis, a well-known complication in TA patients.⁴

Case report

Patient presentation

A 27-year-old female patient, an international athlete, presented to the emergency ward of a tertiary care hospital with complaints of severe throbbing headache and tingling sensation in both hands. There was no history of chest pain/breathlessness/palpitations/syncope/trauma. She was a diagnosed case of hypertension on three drugs including a diuretic, and no other known comorbidities. The vitals were heart rate = 64 bpm, feeble pulse in left radial artery with right to left radioradial delay, blood pressure: RUL 166/116 mmHg, LUL 80/60 mmHg, RLL 146/110 mmHg, LLL 148/112 mmHg, respiratory rate = 20/min, O₂ saturation = 98% RUL, afebrile. All four limbs showed normal reflexes with normal motor power (5/5). Physical examination: body weight = 47 kg, height = 160 cm, and BMI = 18.35 kg/m². Precordium was normal, and no bruit was heard over any location. Other systems were clinically normal. A provisional diagnosis of hypertensive urgency with clinically diagnosed left subclavian artery stenosis and LRA stenosis was made. She was continued on oral Hydrochlorothiazide, Metoprolol, Amlodipine, and Telmisartan was added. Patient consent was obtained for use of images in the study.

Investigations

ECG was normal. Haematological and biochemical investigations reported all values within normal limits (ESR: 11 mm/h, C reactive protein: 1.5 mg/dL, serum creatinine: 0.74 mg/dL, serum sodium: 139 mEq/L, serum potassium: 4 mEq/L, eGFR: 113.7 mL/min/1.73 m²).

Arterial Doppler showed 90% stenosis of right subclavian artery (RSA) with a long segment occlusion of the left subclavian artery (LSA). Venous Doppler showed normal colour flow and no evidence of any superficial or deep vein thrombosis.

Renal sonography and Doppler revealed small size of left kidney (left kidney: 7.7 × 3.1 cm, right kidney: 10.8 × 3.9 cm) with LRA stenosis showing ‘parvus tardus effect’ distally in the segmental and interlobar arteries. Acceleration time was prolonged, resistive (RI) and pulsatile indices (PI) were normal (RI/PI values: at hilum: 0.53/0.76, upper polar artery: 0.73/1.14, interpolar artery: 0.63/0.99, lower polar artery: 0.56/0.81). Right renal Doppler findings were normal.

Abdominal sonography and Doppler revealed normal caliber of the abdominal aorta, with intimal thickening and interrupted focal and linear echogenic foci along the wall, suggestive of aorto-arteritis. Arch aortogram showed normal ascending aorta, arch of aorta and origin of the great vessels, except LSA, which showed ostial stenosis. CT angiography showed significant stenosis in LRA (80–90%) within its ostium, small caliber and multiple focal points of irregular narrowing giving it a “beaded appearance.” Overall, the multifocal narrowing within various visualized branches of the aorta were likely to be due to “arteritis.”

Coronary angiography revealed a normal, dominant right coronary artery. Left main coronary artery and circumflex artery were normal, left anterior descending artery (LAD) was a type-III vessel with a mid-segment myocardial bridge and 50% stenosis, indicative of nonobstructive LAD disease. Fractional flow reserve measurement across the LAD midsegment showed a Pd/Pa ratio: resting 0.9, maximal hyperemia 0.87, indicating no abnormality. Thus, medical line of management was advised for coronary artery disease. Renal angiography showed a normal patent right renal artery with a mild proximal plaque. LRA was seen as a small, shrunken vessel, with a reference diameter of 4 mm in the distal segment. Fig. 1(A) shows multiple septae in proximal/mid segment of LRA with 90% ostioproximal disease.

Fig. 1.

– Renal Artery Angiography: (A) Showing stenosis and multiple fibrotic septae in Left Renal Artery with a beaded appearance. (B) and (C) Showing Runthrough guidewire buckling at fibrotic septae on initial attempt.

Interventions

Clinical diagnosis of “Takayasu Arteritis” was made based on the above investigations with peculiar findings, which revealed bilateral subclavian artery stenosis and significant LRA stenosis, consistent with the criteria laid down by the American College of Rheumatology for TA in 1990.⁵ Percutaneous balloon angioplasty was done for RSA. Repeated attempts to cross the occluded LSA were unsuccessful. 6F JR 3.5 and 7F RDC guide catheter was used to cannulate the LRA, with 7F sheath and right femoral artery access, without satisfactory lie. Hi-Torque Balance Middleweight Wire™ (BMW), ASAHI Fielder FC™, and Terumo Runthrough™ NS coronary wires could not cross the lesions in the LRA due to coiling of the wires in the septate compartments, as depicted in Fig. 1(B) and (C). LRA was then addressed with change in hardware and technique to perform a percutaneous transluminal renal angioplasty (PTRA) of the LRA. A 6F-IMA guide catheter was used to cannulate the LRA giving better coaxiality, and BMW wire was placed in the proximal segment as far as it would go. Over this, a Progreat micro-catheter was maneuvered as far ahead as possible, and the BMW was changed to an ASAHI ULTIMATEbros 3™ (UB3) 0.014″ × 180 cm coronary guide wire, which was used to puncture through the fibrotic septae. The microcatheter was advanced distally over this and wire was exchanged to a BMW. Following this, the vessel was predilated with 2 × 12mm and 3 × 15 mm semi-compliant coronary balloons. In view of significant recoil, and the fact that the vessel was only about 4 mm in size, lesion was eventually stented with a 4 × 22mm Medtronic Resolute Onyx™ Zotarolimus coronary DES at 14 Atm, ostium flared to 20 Atm (Fig. 2). Heparin 3000-IU was given IV. Postoperatively, within 24 h, the patient became normotensive without anti-hypertensives. She is currently on dual antiplatelet regime with Tab Aspirin 75 mg per day and Tab Ticagrelor 90 mg twice daily.

Fig. 2.

– Unique Approach with Modifications in Technique and Hardware used for Percutaneous Transluminal Renal Angioplasty and Stent Implantation in Left Renal Artery (LRA): (A) Curling of BMW wire in the proximal segment of LRA, inability to cross fibrous septae. (B) Microcatheter tip placed against a fibrous septum, having been passed over a BMW wire. (C) UB3 wire pushed through fibrous septae using microcatheter. (D) Microcatheter advanced over the UB3 wire. (E) Microcatheter in situ in distal LRA. (F) BMW wire passed distally through microcatheter which has been removed. (G) Balloon dilatation of LRA. (H) Implantation of 4×22 Medtronic Resolute Onyx Drug Eluting Stent (DES). (I) Ostial flaring. (J) Final result.

Discussion

Percutaneous transluminal renal angioplasty (PTRA) is a time-tested, safe, and promising procedure with successful results in treating TA-induced renal artery stenosis (TARAS) causing renovascular hypertension in patients.⁶ PTRA shows promising results in patients with TARAS in view of long term outcomes, success rates, and also significant reduction in anti-hypertensive therapy contributed by improvement in blood pressure. However, restenosis continues to be the main challenge in TARAS patients till date.⁷ In our patient, detailed investigations were carried out to rule out disease activity and to establish the viability of an already small kidney. PTRA was planned with a view to control hypertension and to attempt to save the kidney from certain irreversible ischemic damage.

DES are used widely in coronary artery disease, and there are studies which have also elaborated on the role played by DES in renal artery stenosis (RAS), showing good results and possible long-term benefit.^6,8,9 Evidence drawn from a study evaluating the comparison between bare metallic stent (BMS) and DES in atherosclerotic RAS cases from 2000 to 2014, clearly suggests that DES are superior to BMS, wherein the rate of in-stent restenosis (ISR) >50% was 18.6% in the BMS group and 7.2% in the DES group (p = 0.031) after a period of 12 months, and that, renal function was better achieved with DES than BMS.⁹ In our case, a DES was placed instead of the conventional BMS in the LRA to overcome the following: firstly, a small renal artery of only 4 mm diameter, renal stents being larger, and secondly, to decrease the chances of restenosis, as is seen commonly in cases of TA amounting to almost 14–16%.^4,7 Recently in 2022, Jariwala et al described a case of DES stent thrombosis after PTRA in a single functioning kidney in a patient with TA having bilateral RAS, possibly an inflammatory complication of TA resulting in acute kidney injury despite different drug regimens.¹⁰ This implies the undiscovered risk of TARAS complications progressing to subacute renal artery and stent thrombosis, which though an uncommon occurrence, needs to be recognized, investigated, and addressed immediately. That said, there is no long term documentation till date pertaining to DES use in a case of TA, especially in patients with severe RAS and such anatomical difficulties, as is seen in our case.

With respect to current available medical literature, our case report appears to be one of few documented cases of a DES being placed in a TARAS in a young female patient with such technical difficulties. Additionally, the different hardware used in this case, have not been used earlier in performing PTRA in TARAS patients with such a distinct presentation. This is probably a unique use of a guidewire designed for coronary chronic total occlusions, the UB3, which was used to cross fibrous septae in the renal artery. Coaxiality was achieved with the use of an IMA-guide catheter, the smaller curve of which was more suited to the relatively small aorta of our patient. An increased awareness and importance of DES in TARAS, with fueled research could certainly prove to highlight the possible long-term benefit of using DES as a mainstay in such cases.

Conclusion

Techniques and equipment used during revascularization interventions may be modified depending on the lesion complexity. DES may also be taken into consideration with the hope of a “restenosis-free” future. The long-term vascular and systemic effects of DES in TARAS patients is yet unknown. Advancements in endovascular and minimally invasive interventional technologies, both radiological and surgical, will certainly prove to be successful in the forthcoming years, in managing TA.

Patients/ Guardians/ Participants consent

Patients informed consent was obtained.

Ethical clearance

Not Applicable.

Source of support

Nil.

Disclosure of competing interest

The authors have none to declare.