Abstract
Aortic coarctation presenting with neurological complications as compressive myelopathy is rare. We report a case of a 43-year-old, hypertensive, female who presented with gradually progressive paraparesis over 4 years. She was diagnosed to be having coarctation of the aorta with intra-spinal collaterals causing compressive myelopathy. She underwent successful percutaneous endovascular implantation of a balloon-expandable aortic stent to relieve her aortic coarctation. This led to regression of her intra-spinal collaterals relieving her cord compression. This nonsurgical modality treatment proved to be safe and effective in relieving her hypertension and neurological complication of paraparesis.
Learning objectives
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To recognize that paraparesis can be a rare manifestation of coarctation of the aorta.
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To highlight the importance of treating the primary pathology of coarctation of the aorta in such critically ill therapeutically challenging patients.
Keywords: Coarctation of aorta, Paraparesis, Aortoplasty
Introduction
Coarctation of the aorta has been reported in 5–8% of all congenital heart defects [1]. A substantial number of subjects may present in adult life with upper extremity hypertension, heart failure, lower extremity claudication, or may be asymptomatic. Some may present with neurological complications which include subarachnoid hemorrhage, intracerebral hemorrhage due to rupture of berry aneurysm [1].
We report a rare case of severe congenital aortic coarctation who presented in the fifth decade with paraparesis due to spinal cord compression by intraspinal collaterals. There is scant literature pertaining to compressive myelopathy as a presenting feature of coarctation of aorta [2,3]. Further, presentation at such a late age is very rare. Also, a report on successful transcatheter endovascular stent implantation in aortic coarctation to successfully relieve spinal cord compression causing paraparesis is rare [4].
Case report
A 43-year-old female presented to the neurology department with a history of progressive worsening weakness in lower limbs for the past 4 years. She also had numbness in the lower limb and inability to micturate. Earlier she had progressive weakness in upper limbs also over 6 months which spontaneously improved.
On examination, her upper limb pulses were normal while lower limb pulses were not palpable. Blood pressure of the upper limb was 180/100 mmHg, while it was not recordable in the lower limbs. Cardiac examination showed loud A2. There was no audible murmur. Collateral bruit was heard on back. On examination of her nervous system, she had a mild decrease in tone in the upper limbs (distal) however power and reflexes were normal. In lower limbs, there was severe spasticity in the left lower limb. Power was 4+/5 in the right lower limb and 0–2/5 in the left lower limb. There was a decrease in touch, pain, temperature sensation more in the left leg than right. Dorsal tendon reflexes were brisk, left more than right. Plantar reflexes were up-going. There was no spinal tenderness or deformity. She was clinically diagnosed to be having coarctation of the aorta with spastic paraparesis.
Differential diagnosis
The differential diagnosis included spinal tuberculosis, spinal epidural hematoma, spinal tumors, spinal stenosis, herniation of intervertebral disc, and rarely spinal cord ischemia.
Investigations
A chest X-ray showed a notching of inferior margins of ribs with a normal cardiac shadow and lung parenchyma. Electrocardiography showed sinus rhythm with left ventricular hypertrophy. Her echocardiography showed concentric hypertrophy of the left ventricle with normal ejection fraction and valvular function. Magnetic resonance imaging (MRI) of the cervical spine showed multiple dilated tortuous T2 void signal intensity in extramedullary intradural space at C4-C7 level causing compression over the ventral and dorsal aspect of the spinal cord. The maximum dilated signal void was seen at C6 level causing near effacement of central spinal canal lumen causing significant compression and distortion of the spinal cord associated with mild short tau inversion recovery hyperintense edema. Computed tomography (CT) angiography showed post ductal coarctation of aorta with multiple arterial collaterals in the chest wall and spinal canal, bicuspid aortic valve, and left ventricular hypertrophy.
Management
After written and informed consent, she was planned for angiography, balloon angioplasty, and stenting of coarctation of the aorta to relieve her severe coarctation. The diameter of the aorta proximal and distal to coarctation was measured on a CT angiogram to determine the size of the aortic stent and balloon required.
Under local anesthesia, 6 French introducer sheath was placed percutaneously in the left brachial artery and 12 French in the left common femoral artery using the Seldinger technique. She underwent digital subtraction angiography by injecting contrast through the 6 French pigtail catheter passed through the left brachial artery to localize the site and severity of the coarctation segment. There was very tight coarctation of the aorta just distal to the left subclavian artery. (Fig. 1, Fig. 2A and Video 1) Through the retrograde femoral artery approach, the coarctation segment was initially crossed by Glidewire which is a hydrophilic coated 0.035″ diameter, 260 cm long, exchange length guidewire (Terumo Interventional Systems, Tokyo, Japan) using 6 F angled catheter. This Glidewire was exchanged with 0.035″ extra-stiff exchange length guidewire using the same angled catheter. Initially, the coarctation segment was pre-dilated using a 10 mm diameter, 4 cm long balloon. As the diameter of the aorta at the origin of the left subclavian artery was 19 mm and the diameter just distal to coarctation was 23 mm, we decided to use an aortic stent which can be expanded up to 20 mm. Cobalt chromium XXL bare Stent (Andramed Gmbh, Reutligen, Germany) was hand crimped on an 18 mm diameter, 6 cm long non-compliant Andra balloon (Andramed Gmbh). The stent mounted on the balloon was positioned across coarct segment over 0.035″ extra-stiff guidewire. The exact position was confirmed by an aortogram through a brachial pigtail catheter. The balloon was then inflated using diluted contrast and the stent was deployed under fluoroscopic guidance. The proximal and distal end of the stent was further flared up using a 20 × 40 mm balloon. The severe coarctation segment was well dilated leading to reduction of flow through collaterals (Fig. 2B and Video 2) and the peak systolic gradient across the coarctation improved from 80 mmHg to 10 mmHg. The femoral puncture site was closed using two Perclose Proglide suture-mediated vascular closure systems (Abbott Inc., Abbott Park, IL, USA).
Fig. 1.
Pre-stenting multidetector computed tomography angiography images. (A, B) 3D volume-rendered angiographic image (A) with surface shaded colored projection (B) showing tight post-ductal coarctation of the aorta (arrowhead) with post stenotic dilation and multiple prominent tortuous anterior abdominal wall (solid arrow), posterior intraspinal (hollow arrow) collaterals. (C, D) Axial multiplanar reformation images in soft tissue window at lower cervical (C) and upper dorsal (D) levels showing prominent para-vertebral (white arrows) and intraspinal (black arrows) collaterals.
Fig. 2.
Pre (A) and post (B) stenting digital subtraction angiography (DSA) image. (A) Pre-stenting DSA showing tight post-ductal coarctation of the aorta with large collaterals. (B) Post-stenting DSA showing a reduction in collaterals and well-expanded coarctation segment after aortic stent implantation.
The patient was observed in the post-catheterization intensive care unit. The improvement in power started within a week. On follow up the patient regained power in her lower limbs, paraparesis gradually improved, and after one month the patient could walk around herself. Repeat CT angiography showed regression of collaterals in the spinal canal which were compressing the spinal cord (Fig. 3).
Fig. 3.
Pre (A, B) and post (C, D) stenting multidetector computed tomography angiography images. (A) 3D volume-rendered colored projection showing tight post-ductal coarctation of the aorta with post stenotic dilation, prominent tortuous anterior abdominal wall, posterior intraspinal collaterals. (B) Coronal maximum intensity projection (MIP) images (thickness 5 mm) showing shelf-like tight coarctation of the aorta with prominent paravertebral collaterals (white arrow). Post stenting 3D volume-rendered colored projection (C) and coronal MIP images (thickness 10 mm) (D) showing metallic stent across the stenotic segment with normalization of post stenotic aortic caliber and complete resolution of anterior abdominal wall, paravertebral collaterals.
Discussion
Endovascular stent implantation for coarctation of the aorta is safe and efficacious in adults with native and recurrent coarctation of the aorta [5,6]. It is now considered the preferred option in the treatment of aortic coarctation in most adults and adolescents. Aortic stent implantation improves luminal diameter, results in minimal residual peak pressure gradient, and sustained hemodynamic benefit. By providing a rigid endovascular prosthesis it prevents vascular recoil resulting in re-coarctation and can tack intimal flaps to the aortic wall allowing the wall to heal, reducing the risks of dissection and aneurysm. Use of bare-metal stents is recommended for the majority of patients with coarctation of the aorta, with covered stents reserved for older patients, patients with Turner syndrome, and those with near atresia of the aorta, or with pre-existing aneurysm formation [7]. Also, with bare stents compared to covered stents, there is a lesser risk of partial obstruction of the left subclavian artery in case of juxta-ductal coarctations. However, we should keep covered stents on our shelf as a back-up option for aortic rupture if any. Besides, these patients should be carefully followed up for the development of any pseudoaneurysm.
In aortic coarctation, collaterals develop to decompress the proximal high-pressure segment into the aorta distal to coarctation stenosis which has lower pressure. The collateral flow usually is through the subclavian arteries through thoracoacromial to intercostal, internal mammary, and scapular arteries [4]. Rarely, collaterals may develop inside the spinal canal [5]. These vessels can cause myelopathy by spinal cord compression in the closed space. Increased intra-spinal pressure can also lead to lymph venous congestion which can lead to the development of chronic myelopathy [5]. It may also cause hemorrhage due to the rupture of these collateral vessels. In the present case, apart from a hypertrophied anterior spinal artery, the peri spinal collaterals caused spinal cord compression leading to paraparesis. Also, compression due to vascular channels can have a dynamic course because of associated spinal cord edema, as the edema subsides there may be an improvement in the neurological deficit [8].
In the present case, endovascular bare-metal stent implantation relieved her coarctation, improved her hypertension, and reduced intra-spinal collaterals compressing the spinal cord, thus relieving paraparesis.
Follow up
The patient's resolution of paraparesis has persisted for 2 years. Her hypertension is well controlled on medications and she is living an active life.
Conclusion
Paraparesis as a presenting manifestation of aortic coarctation is rare. Endovascular stent implantation in coarctation of aorta to relieve compressive myelopathy is a safe and effective non-surgical option in such critically ill patients.
The following are the supplementary data related to this article.
DSA showing the tight coarctation with abundant peri-spinal collaterals.
DSA showing well expanded and apposed aortic stent with a reduction in collaterals.
Funding
None.
Declaration of competing interest
The authors declare that there is nothing to disclose.
Acknowledgment
None.
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Associated Data
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Supplementary Materials
DSA showing the tight coarctation with abundant peri-spinal collaterals.
DSA showing well expanded and apposed aortic stent with a reduction in collaterals.