Medullary extradural hematoma revealing a coarctation of the aorta]

The authors report a spontaneous, unusual complication of coarctation of the aorta. An 11 year old child was admitted for investigation of chest pain. Cardiovascular examination revealed typical clinical signs of coarctation of the aorta. Neurological examination found neck stiffness without headache or deficit. The presumptive diagnosis of dissection of the aorta was infirmed by echocardiography and MRI. The latter investigation, with views of the spinal cord, revealed a compressive medullary extradural haematoma. Antihypertensive therapy and corticosteroids with strict bed rest resulted in complete regression of the haematoma and the coarctation was operated 6 months later. Medullary complications of coarctation of the aorta are usually postoperative. Spontaneous complications are exceedingly rare but very serious: medullary compression by the dilated anterior spinal artery or rupture of an aneurysmal collateral vessel. In this case, magnetic resonance imaging led to diagnosis and effective early treatment of this complication before the patient developed a neurological deficit and the coarctation was treated surgically thereafter.     

Origin of left coronary artery from right pulmonary artery co-existing with coarctation of the aorta

We report 2 infants with the unusual combination of origin of the left coronary artery from the right pulmonary artery as well as coarctation of the aorta. In the first case congestive cardiac failure persisted despite satisfactory repair of the coarctation. Further investigation revealed the anomalous origin of the left coronary artery. Corrective surgery, 24 days later was unsuccessful. When an identical diagnosis was made in the second child, it was decided, at a single operation, to combine correction of the coarctation together with re-implantation of the anomalous left coronary artery into ascending aorta. This patient has survived, and to our knowledge is the first such case with this outcome. The haemodynamic reasons for this approach are discussed.     

Clinical and hemodynamic follow-up of a patient after operation for dissection of an ascending aortic aneurysm secondary to coarctation of the aorta

We present clinical follow-up of a 20-year-old male with an aortic aneurysm secondary to aortic coarctation. The diagnosis of aortic aneurysm secondary to aortic coarctation was made in 1997. The patient did not agree to undergo any invasive or therapeutic procedures at that time. He presented to an emergency unit with severe chest pain after chest trauma obtained during judo exercises in 1998. Two-dimensional echocardiography showed bicuspid aortic valves, an ascending aortic aneurysm 6 cm in diameter with an intimal flap and false lumen, aortic coarctation distal to the left subclavian artery, and aortic insufficiency secondary to annular dilatation. Type II aortic dissection was confirmed by transesophageal echocardiography, which showed the dissection was confined to the ascending aorta. The dissection extended to the beginning of the arcus aorta. Following stabilization of the patient's clinical condition, balloon coarctation angioplasty was performed to reduce afterload and hypertension and to facilitate femoral artery cannulation for cardiopulmonary bypass. Surgical procedures included resection of the aortic valve and prosthetic valve implantation, resection of the ascending aorta, and interposition of a 22 mm Hamashied tubular vascular graft. At a follow-up visit 6 years later, the patient reported being easily fatigued and having palpitations. He had been suffering from hemolytic anemia and mild renal function impairment. Cardiac catheterisation and angiography showed a 40 mmHg gradient due to kinking of the aortic graft and no gradient at the coarctation site. We postulated the kinking of the aortic vascular graft may be related to an inappropriate vascular graft length. We also thought that the severe hemolysis was attributable to the disturbance of blood flow by a jet of blood at the site of the kinking aortic vascular graft. A second operation was performed because the renal function of the patient had decreased progressively and hemolysis symptoms increased. After the second operation, hemolysis on peripheral blood smears had disappeared and renal function had shown progressive improvements.     

Acute dissection of the ascending aorta complicating aortic coarctation successfully treated with surgery

Vascular complications and arterial hypertension are major risk factors in the evolution of disease connected with coarctation of the aorta. Supra- and sub-stenotic aortic aneurysms are classical findings, whereas a dissecting aneurysm is a rare occurrence. In this study, we report on a case of acute dissection of the ascending aorta complicating a poorly followed-up coarctation of the aorta in a ten-year-old girl. The diagnosis, determined by X-ray of the thorax, was confirmed by echocardiography and magnetic resonance imaging. The first operative stage consisted of treating the aneurysm. After resection of the latter, an impregnated dacron prosthesis was inserted in termino-terminal. The immediate result is satisfactory, and a second operation is planned for the aortic coarctation.     

Percutaneous transluminal angioplasty of congenital coarctation of the aorta

Two cases of successful dilatation of congenital coarctation of the aorta using the Grüntzig technique are reported. In a 3-week-old boy and an 11-month-old girl, systolic gradients across the narrowed areas were lowered from 50 to 8 mm Hg and from 23 to 8 mm Hg. Although the femoral pulses later disappeared in the younger patient, surgery was avoided. The second patient's gradient has remained minimal for 8 months and no surgery has been performed.     

Coarctation of the aorta

Coarctation of the aorta is an important, treatable cause of secondary hypertension. Its prevalence varies from 5% to 8% of all congenital heart defects. This condition is most often detected because of a murmur or hypertension found on routine examination. Delayed or absent femoral pulses and an arm/leg systolic blood pressure difference of 20 mm Hg or more in favor of the arms may be considered as evidence for aortic coarctation. The coarctation may be demonstrated on a suprasternal notch two-dimensional echocardiographic view along with increased Doppler flow velocities across the coarctation site. Cardiac catheterization reveals significant systolic pressure gradient (> 20 mm Hg) across the coarctation and angiography demonstrates the degree and type of aortic narrowing. Aortic obstruction may be relieved by surgery or by transcatheter techniques; the latter include balloon angioplasty and stent implantation. In the past, surgery has been used exclusively, but because of morbidity and complications associated with surgery, catheter techniques are increasingly used in the management of aortic coarctation. Balloon angioplasty in children and stents in adolescents and adults are becoming initial therapeutic options for management of coarctation. Studies evaluating long-term follow-up results of the interventional techniques are needed.     

Coarctation of the aorta presenting as a pulsatile occipital mass

A healthy 18 months old boy, is referred to our hospital for a thrilling pulsatile mass behind the left ear. Aortography in the ascending aorta shows a severe coarctation with almost interruption of the aorta between the left carotid and left subclavian artery. Both external carotid arteries provide an important collateral pathway through occipital arteries to two dilated vertebral arteries. Descending aorta is feeding by a reverse blood flow into vertebral and subclavian arteries. The child has been operated. This is an original presentation of severe coarctation of the aorta with development of an important and vital collateral pathway.     

Coarctation of the aorta and Fallot's tetralogy. Apropos of 2 cases

Two cases of the association of Fallot's tetralogy and coarctation of the aorta are reported. In both cases there was a right aortic arch. In addition, the second case had a cervical aorta and a left subclavian artery isolated from the aorta and vascularised by the left vertebral artery. The two children underwent complete repair: the first patient had a Waterston shunt followed by repair of the Fallot's tetralogy, followed by repair of the coarctation; the second patient first had a Gore-tex tube inserted from the ascending to the descending aorta and then underwent repair of the Fallot's tetralogy. The association of a coarctation with an obstructive lesion of the right heart is very rare; only two cases have previously been reported: one Fallot's tetralogy and one tricuspid atresia with pulmonary stenosis. Both cases also had a right aortic arch. This seems to go against the haemodynamic theory of coarctation: blood flow in the ascending aorta increases while flow in the pulmonary artery decreases. Coarctation could be caused by a partial involution of the terminal segment of the right primitive dorsal aorta as proposed in cases of coarctation with a right sided aortic arch. The second case also presented two uncommon features: a cervical aortic arch and a left subclavian artery isolated from the aorta.     

Temporal flow velocity profile in the descending aorta in coarctation

The temporal blood flow velocity profile in the descending aorta at the diaphragm was investigated using pulsed Doppler echocardiography in patients with documented coarctation of the aorta and in control patients. The ratio of peak systolic to peak diastolic frequency shift, an angle-independent index of pulsatility derived from the Doppler curve, proved to be an excellent indicator of aortic arch obstruction in the absence of a patent ductus arteriosus. Control groups included patients with a systemic right ventricle, systemic ventricular dysfunction and aortic stenosis, to determine if these conditions would confound the test. None of these factors were found to influence significantly the frequency shift ratio. However, the Doppler curves in six patients with interrupted aortic arch or severe coarctation, whose descending aorta was supplied by way of a large patent ductus arteriosus, were similar to those seen in control patients. Two indexes of wave transmission velocity, the rate of acceleration and the time to peak velocity, discriminated less effectively between patients with coarctation and the control patients, particularly those with aortic stenosis. After surgery for coarctation, the Doppler indexes and contour of the Doppler curve discriminated completely between patients with and without residual coarctation.     

Transcatheter treatment of pulmonary stenosis and coarctation of the aorta: experience with percutaneous balloon dilatation.

Twenty two children (age range 4 months-20 years) with pulmonary valve stenosis and ten children (age range 1 month-11 years) with coarctation of the aorta underwent balloon dilatation in the 29 month period between October 1983 and February 1986. Number 5-9 French catheters with 5-20 mm balloons were used according to the size of the angiographically measured pulmonary valve annulus or coarcted segment and the aorta proximal to coarctation. The peak inflation pressure used in the balloons varied from 2-5 atm (202-505 kN/m2) for pulmonary valve stenosis and 4-8 atm (404-808 kN/m2) for coarctation, and inflation lasted 8-15 s. At least four balloon dilatations were performed in every case. After balloon dilatation the mean (SD) peak systolic pressure gradient across the pulmonary valve fell from 98 (39) to 33 (13) mm Hg. The cardiac index did not change. After balloon dilatation of aortic coarctation the systolic pressure in the descending aorta rose from 86.4 (17.7) to 107.8 (20) mm Hg and the peak systolic pressure difference across the coarctation fell from 44.1 (19.1) to 8.5 (8.5) mm Hg. The diameter of the angiographically measured coarcted segment increased, the Doppler estimate of the pressure difference across the coarctation decreased, and the femoral pulses improved. There were no important complications. Long term follow up results for balloon dilatation of pulmonary valve stenosis were excellent. Similar follow up was not available for patients after dilatation of aortic coarctation. Percutaneous balloon dilatation for pulmonary stenosis and coarctation of the aorta is a safe and effective alternative to operation. It is the treatment of choice for pulmonary valve stenosis and it may become so for coarctation of the aorta in young children if long term results are favourable.     

Coarctation of the abdominal aorta. Treatment by stent placement]

We report a nine-year-old boy referred for evaluation of suspected coarctation of the aorta. He presented with hypertension and absent femoral pulses. A 50 mmHg gradient was detected in the abdominal aorta by color flow doppler echocardiography but the level of coarctation was impossible to locate. The diagnosis was made by magnetic resonance imaging and aortography, showing segmentary hypoplasia in the upper abdominal segment of the descending aorta, immediately below the celiac axis and above the renal arteries.Treatment consisted in placement of a stent Palmaz, which was effective in significantly decreasing the gradient. Some clinical aspects and different therapeutic strategies for this unusual case are discussed.     

Percutaneous angioplasty of native coarctation of the aorta. Results in children and young adults

The authors report the experience of one centre in the treatment of native coarctation of the aorta by percutaneous angioplasty. Between 1999 and August 2005, 22 patients (7 girls, 15 boys) underwent dilatation of their coarctation by a balloon catheter at an average age of 11.8 +/- 5 years and body weight of 44 +/- 21 kg. In 20 patients, balloon angioplasty alone was performed and, in the other two, the angioplasty was associated with the insertion of a covered CP stent. The dilatation was performed with a balloon/coarctation ratio of 2.18 +/- 0.6 (1.7 to 3.5) and a balloon/isthmus ratio of 1.0 +/- 0.23 (0.7 to 1.87). After the procedure, ascending aortic pressure decreased from 134.7 +/- 23.4 mmHg to 125 +/- 22.7 mmHg (p= 0.0003); descending aortic pressure increased from 93.4 +/- 14.9 mmHg to 104.8 +/- 21.7 mmHg (p= 0.003); transisthmic pressure gradient decreased from 41.7 +/- 14.1 mmHg to 19.8 +/- 9.5 mmHg (p< 0.0001) and the diameter of the coarctation increased from 5.9 +/- 2.6 mm to 9.3 +/- 2.6mm (p= 0.0015). The two patients treated by covered CP stents had excellent immediate results without significant residual gradients. After dilatation (sometimes repeated, N= 13), a gradient > 20 mmHg persisted in ten patients (36% of cases) but no predictive factor was found on statistical analysis. There were no cases of acute aneurysm. In one patient, the femoral pulse decreased but returned to normal with heparin therapy. No patient required emergency surgical treatment after the angioplasty. During the clinical follow-up, echocardiography and MRI showed no signs of aneurysm and one patient had mild irregularity of the isthmic region. Three patients underwent surgery by resection suture for persistent coarctation (one combined with a Ross procedure); another patient was treated by a CP stent. In conclusion, percutaneous angioplasty of native coarctation of the aorta gives satisfactory results with few complications in bigger children and young adults. The results can be improved by using a slightly higher balloon/coarctation ratio.     

Coarctation of aorta presenting as acute abdominal pain

Coarctation of aorta is the most common cardiovascular cause of secondary hypertension. The condition is correctable although early diagnosis is often not made. We report the case of a 9-year-old child who was admitted with severe intractable abdominal pain as the presenting symptom of post-subclavian coarctation of aorta with hypertension. His pain resolved after control of hypertension with parenteral antihypertensives and the narcotic analgesics. He subsequently underwent transcatheter balloon dilation of the coarctation of aorta and remains well with normal blood pressure on follow-up.     

Coarctation of the aorta associated with pulmonary valve stenosis

A 7-year-old patient had coarctation of the aorta associated with moderate and dysplastic pulmonary valve stenosis. The child underwent corrective surgery of the coarctation. This unusual association cannot be satisfactorily explained by the theory of fetal flow patterns.     

Evaluation of persistent coarctation of aorta after surgery with blood pressure measurement and exercise testing

In 16 patients with coarctation of the aorta blood pressure in the arms and legs was measured before and after exercise using a treadmill and the Bruce protocol to achieve a standardized level of exercise. Three patients had had no previous operation; 13 had had a previous surgical repair of the coarctation. Three patients were studied both before and after operation. Two of the 13 patients studied postoperatively were found to have significant residual coarctation on the basis of a postexercise arm to leg pressure gradient that had not been appreciated on routine postoperative examination, and one of these patients was found to have residual coarctation after his second operation. One patient not operated on was believed to have mild coarctation of no clinical significance. Eleven of the 13 patients previously operated on (85 percent) were found to have had a satisfactory repair. A postexercise arm to leg systolic blood pressure gradient of more than 35 mm Hg after repair of coarctation of the aorta is suggested as an indication for recatheterization.     

The effect of chronic constriction of the aorta on arterial blood pressure in dogs: An attempt to produce coarctation of the aorta

The aortae of dogs may be constricted or occluded at the arch without the development of arterial hypertension proximal to the occlusion. Hypotension of moderate to marked degree occurs distal to the occlusion, and is usually more marked a few hours after clamping than later. the return toward normal of the pressure distal to the clamp was attributed, among other causes, chiefly to the development of collateral circulation and acute and chronic thining of the vessel walls within the clamp. After several days the pressure in the femoral artery may fall progressively, without simultaneous reduction of carotid pressure. The oxygen content of the blood does not appear to be reduced significantly until a great reduction of femoral pressure has occurred.The failure to produce, in dogs, hypertension proximal to a clamp constricting the aorta at or near the site where aortic coarctation occurs in human beings is attributed to the ready development in dogs of an extensive collateral circulation.Hypertension of renal origin occurs when the aorta is closed a few centimeters below the origin of the renal vessels, and, in addition, constricted or closed in the thorax at a level corresponding to the dome of the diaphragm. Removal of the kidneys reduces or abolishes this hypertension. The hemodynamic changes are sufficiently similar to suggest the possibility that the hypertension of human coarctation is of renal origin, and that the manner in which collateral circulation develops differentiates the response to constriction of the aorta in dogs and men.     

Double isthmic coarctation of the aorta: "tandem" coarctation

A second case of double coarctation of the thoracic aorta is reported, the first having been observed at the Marie-Lannelongue Surgical Center in an older child. This case was a 3 months infant in which the missed pre- and postoperative diagnosis led to reoperation after control catheter and angiographic studies. These investigations were carried out one month after the first operation because of persistent severe cardiac failure. Surgical cure in two stages consisted in a Waldhausen plastic enlargement procedure and a Crafoord-type resection anastomosis, ensuring the best chances for a good result.     

Balloon-expandable stent treatment of experimental coarctation of the aorta: early hemodynamic and pathological evaluation

The present study was intended to evaluate the acute and short-term hemodynamic, angiographic, and pathological response to balloon-expandable stent treatment of experimental coarctation of the aorta. A discrete thoracic coarctation was surgically created in six mongrel dogs (two adults, four puppies). Two months postoperatively all six dogs (8.9-30 kg) underwent left heart catheterization and coarctation stenting performed through a femoral artery cutdown. A Palmaz PS-30 stent was advanced to the coarctation through a 10 French sheath, and expanded with an angioplasty balloon chosen to equal the diameter of the proximal aorta (9-12 mm). Stent implantation was successful in each dog. The systolic pressure gradient decreased from 26.3 +/- 9.1 mmHg (mean +/- SE) to 0.5 +/- 0.5 mmHg (P = 0.04), and the coarctation diameter improved from 50 +/- 6% to 82 +/- 6% of the diameter of the proximal descending aorta (P less than 0.01). Follow-up catheterization 4-7 weeks after stenting documented no stent migration, early restenosis, thrombosis, obstruction of arterial side branches, or aneurysm formation. Pathological evaluation of the explanted segments of stented aorta documented that by 6-7 weeks the stents are covered by a neointima composed of intimal proliferation and fibrosis with an endothelial cell surface. These data suggest that balloon-expandable stainless steel stents provide excellent acute and short-term relief of coarctation in this experimental model. Larger and longer-term studies are needed to better assess the incidence of restenosis or aneurysm formation following stenting of coarctation of the aorta.     

Primary balloon dilatation of coarctation of the aorta in neonates

Primary balloon dilatation of coarctation of the aorta was attempted in 10 consecutive neonates (age range 2-23 days). The coarctation site was crossed and the balloon inflated in all but one patient. In two patients with associated severe isthmal hypoplasia there was no change in the gradient after dilatation. In the remainder, the residual gradients were trivial and angiography showed complete relief of coarctation. Severe recoarctation developed 5-12 weeks after dilatation in five patients, each considered to have had an excellent initial result. The coarctation was rapidly progressive in three patients in whom Doppler studies within two weeks of the development of recoarctation had shown no significant gradient. In the other two patients progressive restenosis was charted by Doppler examinations over the course of 6-8 weeks. Three patients had a second, initially successful, dilatation procedure. One patient remained well with no residual gradient 18 weeks later. Stenosis recurred within eight weeks in the other two, and both have undergone successful surgical repair. Balloon dilatation of a native coarctation of the aorta gave excellent immediate results in most neonates. Severe isthmal hypoplasia is, however, a contraindication to balloon dilatation and early restenosis is an important problem. These results do not support the continued use of primary balloon dilatation of coarctation of the aorta in neonates.     

Primary balloon dilatation of coarctation of the aorta in neonates.

Primary balloon dilatation of coarctation of the aorta was attempted in 10 consecutive neonates (age range 2-23 days). The coarctation site was crossed and the balloon inflated in all but one patient. In two patients with associated severe isthmal hypoplasia there was no change in the gradient after dilatation. In the remainder, the residual gradients were trivial and angiography showed complete relief of coarctation. Severe recoarctation developed 5-12 weeks after dilatation in five patients, each considered to have had an excellent initial result. The coarctation was rapidly progressive in three patients in whom Doppler studies within two weeks of the development of recoarctation had shown no significant gradient. In the other two patients progressive restenosis was charted by Doppler examinations over the course of 6-8 weeks. Three patients had a second, initially successful, dilatation procedure. One patient remained well with no residual gradient 18 weeks later. Stenosis recurred within eight weeks in the other two, and both have undergone successful surgical repair. Balloon dilatation of a native coarctation of the aorta gave excellent immediate results in most neonates. Severe isthmal hypoplasia is, however, a contraindication to balloon dilatation and early restenosis is an important problem. These results do not support the continued use of primary balloon dilatation of coarctation of the aorta in neonates.