... School of RibeirEo Preto, University of SZo Paula, Ribeinio Preto, SEo Pa&o. ... A good surgical result was achieved by means of resection of the internal shelf ...
Iniernational Journal of Cardiology, 39 ( 1993) 109- 111 0 1993 Elsevier Scientific Publishers Ireland Ltd. All rights
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Congenital coarctation of the lower thoracic aorta. A rare surgically correctable cause of hypertension in the young - case report Fernando T.V. Amaral, Paulo J.F. Ribeiro and HClio C. Salgado Division of Paediatric Cardiology and Cardiac Surgery, Hospital do Coraqiio - Fundaqiio Waldemar B. Pessoa and Department of Physiology, Medical School of RibeirEo Preto, University of SZo Paula, Ribeinio Preto, SEo Pa&o. Bra:il (Received
2 November
1992; revision
accepted
7 January
1993)
We report a case of congenital coarctation of the lower thoracic aorta. The presenting with the signs of classical coarctation, had the diagnosis confirmed surgical result was achieved by means of resection of the internal shelf and pericardium patch. One year after the operation the patient has normal blood pulses. Key words: Coarctation;
Hypertension
Introduction Coarctation of the aorta located at sites other than the upper thoracic aorta is rare. This unusual lesion may be found at the abdominal segment of the aorta or more rarely, at the lower thoracic aorta [l]. The lesion may present as a long hypoplastic segment [2], nd feasibly corrected by surgery, or have a localized stricture, thus being amenable to surgical correction. Acquired inflammatory lesions have also been described as a cause of the coarctation [3,4]. This report describes a case of a young man succesfully operated on for a severe coarctation of the lower thoracic aorta. Case Report A 15year-old
patient, a 15year-old man, by an aortography. A good aortoplasty using a bovine pressure with good femoral
boy presented at the outpatient
Correspondence to: Fernando T.V. Amaral M.D., Avenue Independkncia 1379, 14025-390 Ribeirao Preto, Sao Paulo, Brazil.
clinic with hypertension, intermitent headaches and effort dyspnea over the last 2 years. Blood pressure in the right arm was 190/120 mmHg, the brachial pulses were prominent while femoral pulses were absent. A soft systolic murmur with no click was heard at the aortic area. Severe left ventricular hypertrophy was detected on the electrocardiogram and the chest X-ray was normal. A clinical diagnosis of severe coarctation of the aorta was made. Conventional cross-sectional and Doppler echocardiogram detected left ventricular hypertrophy but failed to show a coarctation in the usual site. Aortography revealed a stenotic lesion situated at the lower thoracic aorta extending to the diaphragmatic region (Fig. la). At operation through a left thoracotomy, a severe coarctation was found just above the diaphragm (Fig. 2). There was some degree of hypoplasia proximal and distal to the coarctation. Periaortic adhesions, usually found in aortitis, were not present at inspection. Resection of the internal shelf was done and the aorta repaired with a bovine pericardium
a
b
Fig. I. (a) Aortography: the tip of the catheter is in the lower thoracic aorta. Coarctation is visualized after contrast injections. (b) Postoperative aortography: the tip of the catheter is in the lower thoracic aorta. Mild deformity is visible at the coarctation site.
a
b Fig. 2. (a) Surgical
aspect
before operation.
(b) Surgical
aspect after operation.
III
patch (Fig. 2b). The postoperative course was complicated by paradoxical hypertension and convulsions despite preoperative beta blockade. The blood pressure was controlled with nitroprusside and propranolol. The patient was discharged home 15 days after operation, with a blood pressure of 140190 mmHg on propranolol 80 mgiday. At 1 year follow-up the patient is asymptomatic, with good femoral pulses and normal blood pressure on propranolol 40 mg/day. Repeat cardiac catheterization and aortography showed a very good repair with no pressure gradient (Fig. lb).
Discussion Congenital coarctation of the lower (descending) thoracic aorta is a rare disease. Since its first description in 1835 [1], apparently only 12 cases have been described [5-71. The lesion is a localized stricture similar to classical coarctation of the upper thoracic aorta with varying degrees of hypoplasia proximal and distal to the lesion. We believe one should be aware of the existence of this disease, despite its rarity. Except for local murmurs, there is no reported clinical feature which may lead us to think the coarctation is situated in the lower thoracic aorta. The clinical picture is similar to cases of classical coarctation and perhaps the first important clue for diagnosis would be the absence of coarctation at the usual site during cross-sectional echocardiography. In order to make a definitive anatomic diagnosis, angiography is, at the moment, mandatory. Contrast injection should be made in the lower thoracic aorta which could also image an abdominal coarctation. Long, hypoplastic descending aortic segment is a known cause of hypertension but unfortunately not amenable to surgery [2]. Acquired lesions such as difuse aortitis [3,4], may also mas-
querade a classical coarctation. This was ruled out in our patient by the absence of inflammatory signs in the peri-aortic region on gross examination at surgery. Localized lesions, such as the one we are reporting, should be clearly identified and treated by surgery if complications of long standing hypertension or endarteritis are to be avoided. Since the first catastrophic surgical attempt to correct the lesion [5], techniques have improved and a recent report [7] described a good result with dacron graft interposition. In our case, a bovine pericardium patch was used for reconstruction of the aorta, hoping to avoid late aneurysm formation. Prognosis seems to be good, however, due to the rarity of the disease, information about late follow-up is unavailable at the moment.
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