Abstract
Vestibulopathy is a condition characterized by the onset of vertigo, nausea and vomiting, in the absence of hearing loss or tinnitus. The differential diagnosis includes peripheral otologic disorders and central disorders such as vascular insufficiency. Modified Blalock–Taussig (B–T) shunt, a polytetrafluoroethylene graft from the subclavian artery to the pulmonary artery, has become the palliative procedure of choice in cyanotic congenital heart disease patients. This palliative surgery creates a similar vascular haemodynamic to that seen in the subclavian steal syndrome and when associated with aplasia, hypoplasia or stenosis of the contralateral carotid, vertebral or posterior communicating arteries it may predispose to vascular hypoperfusion and peripheral hypofunctioning of the vestibular system. We report the case of a cyanotic congenital heart disease patient with vertigo and dizziness who underwent a modified B–T fistula on childhood and developed, many years later, a vertiginous syndrome probably of vascular origin.
1
Introduction
The subclavian steal syndrome is a condition that results from the stenosis or occlusion of the subclavian artery proximal to the origin of the vertebral artery causing subsequent retrograde blood flow in the homolateral vertebral artery to give blood supply to the arm. Most stenoses are due to atherosclerosis. Dissection, Takayasu arteritis, congenital atresia, trauma, surgical obstruction and external compression on the subclavian artery are rare causes. The term subclavian steal syndrome is applied when reversed vertebral artery flow causes symptoms from the compromised vertebrobasilar and subclavian arteries which include paroxysmal vertigo, drop attacks and/or arm claudication. However, many patients with subclavian artery stenosis or occlusion and reversed vertebral flow are asymptomatic while others present a clinical spectrum so variable that it is difficult to determine which symptoms, if any, are characteristic of the pure syndrome.
Reversal of vertebral artery flow from homolateral subclavian occlusion was first reported by Contorni in 1960 . Afterwards, many arteriographies illustrated this phenomenon and although once thought to be rare, the emergence of new imaging techniques has drastically improved its diagnosis and prevalence .
2
Case report
A 26-year-old male patient with a Levo-transposition of great arteries, also termed congenitally corrected transposition of the great arteries because conjugates atrioventricular and ventriculoarterial discordance, associated with a severe pulmonary stenosis and a large ventricular septal defect was operated on in childhood with a right and a left modified Blalock–Taussig (B–T) fistulas due to his cyanotic congenital heart disease.
The patient was categorized as New York Heart Association functional class II/IV and related, in the last months, vertigo and dizziness mostly with postural changes, often with nausea and vomiting and occasionally with spontaneous nystagmus. The patient denied hearing loss or tinnitus. Physical examination showed a high continuous machine-like systolic-diastolic murmur at the right infraclavicular level, a low continuous machine-like murmur at the left infraclavicular area and a systolic ejection murmur at the left sternal border. A 12-lead electrocardiogram exhibited right ventricular hypertrophy with systolic overload and the echocardiogram showed atrioventricular and ventriculoarterial discordance with levo-transposition of the great arteries, severe subpulmonary stenosis, a large ventricular septal defect, permeability of the right B–T fistula with the inability to visualize the left subclavian artery–pulmonary shunt. Blood analysis showed a slightly elevated hematocrit level of 47.3% and an oxygen pressure of 64%, while the rest of the parameters were within normal limits. Similarly, petrous and ears computed tomography (CT) and CT and magnetic resonance imaging of the brain and neck were within normality. The otolaryngology evaluation showed no spontaneous or position nystagmus and the patient had a normal Romberg, Babinski–Weil, Unterberger–Fukuda and Barany tests. Audiometry was normal and the neurological examination ruled out central pathology.
Due to his symptoms and the existence of a low intensity continuous machine-like murmur at the left infraclavicular area, raising suspicion of stenosis or occlusion of the left B–T fistula, a high-resolution computed tomography (CT) was done to rule it out. The CT showed a significant stenosis (60% of the area) of the left subclavian artery, between the origin of the left vertebral artery and the left B–T fistula. Also the CT exhibited an occluded right vertebral artery, in the context of a previous surgical clip, and a filiform vertebral vessel distal to the occlusion which filled by the cervical collateral circulation and gave rise to the ipsilateral posterior inferior cerebellar artery and the basilar artery ( Fig. 1 ). Because of this, an angiography was requested to assess the left B–T fistula. The angiography showed a significant stenosis of the left subclavian artery ( Fig. 2 A ), a reverse flow from the left vertebral artery, through the thyrocervical trunk, to the subclavian artery ( Fig. 2 B) and a non significant stenosis at distal end of the left B–T fistula. Due to the significant left subclavian artery stenosis direct stenting with a Genesis™ stent (Cordis Endovascular, Johnson & Johnson, Warren, New Jersey) of 7 × 24 mm was implanted. Some months later the patient persisted with a similar symptomatology, although less intense, needing symptomatic treatment and vestibular rehabilitation therapy to help in his dizziness and balance disorder. Actually, and three years after the stent implantation, the patient has only occasional vertigo and dizziness episodes remaining asymptomatic for long periods of time.
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