Repair of coarctation of the aorta in children with Turner syndrome

Summary Increased risk of perioperative hemorrhage has been described in patients with Turner syndrome and coarctation of the aorta. We have operated on 11 patients with Turner syndrome and coarctation of the aorta, two of whom developed bleeding. One patient died. End-to-end anastomosis was performed in all patients. Histologic studies of resected specimens did not reveal any abnormalities. The risk of bleeding was not related to age at the time of repair or postoperative hypertension. Although no specific abnormality has been identified in these patients, the risk of serious hemorrhage appears increased.     

Acquired coarctation of the aorta

Summary Coarctation of the aorta is usually caused by a congenital narrowing of the aorta. This report describes two children who developed hypertension secondary to an acquired coarctation of the aorta. In one patient the coarctation was temporally related to umbilical artery catheterization and was associated with thrombosis and aneurysmal dilatation of the aorta. In the second patient, the coarctation occurred after surgical aortotomy during the removal of an intrathoracic neuroblastoma. Patients who have interventional damage to the aorta should be periodically examined for the appearance of a coarctation. Although an acquired coarctation of the aorta is an infrequent complication of invasive or surgical procedures, it should be identified since it represents a remediable cause of hypertension in children.     

Balloon Dilatation of Native Aortic Coarctation in Two Patients with Turner Syndrome

We report successful balloon dilatation of native coarctation of the aorta in two patients with Turner syndrome, with favorable endovascular remodeling demonstrated by intravascular ultrasound imaging and angiography 1 year after the procedure.     

Anomalies of the abdominal aorta in Williams-Beuren syndrome – another cause of arterial hypertension

Vascular disease in Williams-Beuren syndrome is based on an elastin arteriopathy which may cause stenoses in small and great vessels. This study presents the pattern of stenotic lesions of the abdominal aorta and the incidence of arterial hypertension. From 112 patients with Williams-Beuren syndrome followed since 1975, 25 patients were studied by aortography. The diameter of the thoracic aorta and the change in diameter to the iliac bifurcation were compared with normal data. Renal artery stenosis was suspected when the proximal vessel diameter was less than 50% of the distal diameter. Of the 25 patients, 20 had vascular stenosis of whom 19 patients were affected by segmental narrowing either of the thoracic aorta (n=9) or the abdominal aorta (n = 7) or both (n = 3). Hypoplasia of the abdominal aorta was characterised by the smallest diameters at the renal artery level and an increased diameter of the infrarenal abdominal aorta. A total of 11 patients had renal arterial stenosis, associated with narrowing of other aortic segments in 10 cases. Only one patient had a solitary stenosis of the renal artery. Arterial hypertension was diagnosed in 17 patients, 2 of them had no vascular lesions; in the remaining 15 patients stenosis was present in more than one segment (aorta 6, renal artery stenosis 1, both 8). CONCLUSION: Narrowing of the abdominal aorta in patients with Williams-Beuren syndrome is a frequent morphological manifestation of the arteriopathy. Isolated renal arterial stenosis was rare, since it was more frequently combined with a narrowed aorta. Hypertension is a common symptom in the affected group and must be regarded as a manifestation of generalised arteriopathy rather than renal hypoperfusion.     

Turner's syndrome occurring with Horner's syndrome. Seen with coarctation of the aorta and aortic aneurysm.

A patient with 45, XO Turner's syndrome, subclinical coarctation of the aorta, and aortic aneurysm is described. She had left Horner's syndrome. The literature is reviewed for cases with the combination of aortic aneurysm and coarctation.     

Aortic anomalies in an adolescent with the Williams' elfin facies syndrome

An adolescent with classical Williams' syndrome who presented with hypertensive encephalopathy is described. He had the unusual combination of supravalvular aortic stenosis, long segment coarctation of the aorta, aortic hypoplasia and a high bifurcation of the abdominal aorta. Surgical resection of the coarctation was required; however, the patient has remained moderately hypertensive. The aortic anomalies in this syndrome are reviewed and their frequency and importance examined.     

Unusual Cause of Persistent Impairment of Ventricular Function after Repair of Coarctation of the Aorta

In neonates with coarctation of the aorta, left ventricular function may be impaired due to an increased afterload and an associated endocardial fibroelastosis. Repair of the coarctation usually results in improvement in left ventricular function over the postoperative weeks. We report a patient in whom, despite successful repair of coarctation, left ventricular performance continued to deteriorate. Investigation revealed the unusual coincidental finding of anomalous left coronary artery from the right pulmonary artery.     

Aortic dilation, dissection, and rupture in patients with Turner syndrome

We report two patients with Turner syndrome who had aortic dissection and rupture, one with prior repair of coarctation. We also note the high incidence (8.8%) of unrecognized aortic root dilation in a group of 57 patients with Turner syndrome whom we prospectively evaluated by echocardiography. Our analysis and review of previously reported cases suggests that multiple risk factors may exist for aortic dissection, including coarctation, bicuspid aortic valve, and systemic hypertension, but that these need not be present. Aortic root dilation may be an additional finding that suggests the patient with Turner syndrome is also at risk. When it is present, magnetic resonance imaging visualizes the entire aorta and allows quantification of the site and degree of dilation. In patients with dissection, the aorta often exhibits pathologic evidence of cystic medial necrosis similar to the finding in patients with Marfan syndrome. Therapeutic methods to decrease risk, such as those directed toward prevention of bacterial endocarditis, blood pressure control, and perhaps prophylactic beta blockade or surgical reconstruction, may need to be considered. Patients with Turner syndrome, their families, and the physicians who care for them should be aware of the significance of unexplained chest pain, dyspnea, or hypotension as potential manifestations of aortic dissection or rupture.     

A rare cause of arterial hypertension in children: Takayasu arteritis

Takayasu arteritis, a nonspecific inflammatory arteritis, is particularly rare in children. We report the case of a 6-year-old girl presenting with severe arterial hypertension in the upper segment associated with an inflammatory syndrome. Investigations showed coarctation of the abdominal aorta at different levels, due to Takayasu arteritis. The patient was treated with percutaneous dilatation and stent implantation as well as prolonged anti-inflammatory therapy. Arterial hypertension in children needs to be investigated until its cause, which may be rare, such as Takaysu arteritis, is determined.     

Coarctation of the Aorta in the Right Aortic Arch with Left Aberrant Innominate Artery

Right aortic arch with an aberrant left innominate artery is a rare combination. We report a unique case of coarctation of the aorta in a patient with right aortic arch and aberrant left innominate artery. The diverticulum of Kommerell gave rise to the left innominate artery and a left ligamentum arteriosum, completing a vascular ring. This unusual case raises interesting points regarding the development of coarctation in the context of a right-sided arch and the approach to management of these patients.     

Treatment of Coexistent Coarctation and Aneurysm of the Aorta with Covered Stent in a Pediatric Patient

Intravascular stents have emerged as an effective alternative to surgery and balloon angioplasty in the treatment of native and recoarctation of aorta. Balloon angioplasty, although effective in the treatment of native coarctation of the aorta, is associated with several complications such as recoarctation, aneurysm formation, aortic dissection, and vascular injury. In this report, we present the use of a covered stent to treat a patient who developed recoarctation of the aorta with an associated aneurysm following balloon angioplasty of her native coarctation.     

Neurological complications of balloon angioplasty in children

Summary We report on the occurrence of cerebrovascular accidents as a possible complication of balloon angioplasty in children. The first patient underwent balloon angioplasty for aortic stenosis and subsequently developed a right temporoparietal infarct in the vascular territory of the right middle cerebral artery. The second patient developed a right temporoparietal infarct also in the vascular territory of the right middle cerebral artery following balloon angioplasty of native coarctation of the aorta. This experience suggests that, in addition to the previously reported complications of balloon angioplasty, the occurrence of a potential cerebrovascular accident must be considered in the risk-benefit analysis of this procedure.     

COARCTATION OF THE AORTA WITH MULTIPLE ARTERY ANOMALIES IN IDIOPATHIC HYPERCALCEMIA OF INFANCY

A case of idiopathic hypercalcemia of infancy with multiple anomalies of the arteries is described. This patient demonstrated a combination of supravalvular pulmonary artery stenosis, supravalvular aortic stenosis, hypoplasia of the aortic arch, coarctation of the aorta and multiple stenoses of the branches of the abdominal aorta. The pathogenesis of arterial hypertension in this syndrome is discussed.     

Fraser-Cryptophthalmos syndrome with cardiovascular malformations: a rare association

Fraser-Cryptophthalmos syndrome is a multiple malformation disorder associated variably with cryptophthalmos (hidden eye), anomalies of the head, nose and ears; syndactyly, renal and genital malformations. In this report, we describe a case of Fraser syndrome with cardiovascular malformations: coarctation of aorta, an association not previously described.     

Pulmonary Atresia with Intact Ventricular Septum Coexisting with Coarctation of the Aorta

A newborn with pulmonary atresia, intact ventricular septum, and a bicommissural aortic valve underwent percutaneous pulmonary valvotomy at 1 day of age. Severe coarctation of the aorta became apparent only after spontaneous closure of the ductus arteriosus at 5 weeks of age, requiring repair by surgical end-to-end anastomosis. This is the first report of a child with pulmonary atresia with intact ventricular septum in association with coarctation of the aorta.     

A rare cause of neonatal ulcero-necrotizing enterocolitis: aortic coarctation syndrome

The authors report a case of necrotizing enterocolitis which appeared in the first hours of life of a full-term neonate without signs of sepsis. This neonate presented with a severe hypoplasia of the horizontal aorta and very tight coarctation responsible for hepatic, renal and mesenteric ischemia. Reports of enterocolitis as a complication of congenital heart disease are rare and related most often to hypoplastic left heart than to coarctation of the aorta.     

Growth hormone treatment in a child with Williams-Beuren syndrome: a case report

Growth retardation is a consistent finding in Williams-Beuren syndrome. The cause of short stature in this syndrome is unknown. Endocrine studies have failed to reveal abnormalities in the growth hormone – insulin-like growth factor I axis. We report a boy with confirmed Williams-Beuren syndrome, who was found to have classical growth hormone deficiency and responded well to growth hormone therapy. Conclusion Although growth hormone deficiency is not likely to be a common cause of short stature in Williams-Beuren syndrome, we nevertheless recommend evaluation of the growth hormone – insulin-like growth factor I axis in all cases. Received: 14 February 1998 / Accepted in revised form: 4 April 1998     

A Case of Coarctation in a Right Aortic Arch with an Aneurysmal Right Subclavian Artery and Aberrant Left Brachiocephalic Artery

This is the second reported case of coarctation in a right aortic arch with an aberrant left brachiocephalic artery. In this patient, the right subclavian artery is aneurysmal, making this case unique. There is a pressure gradient of 34 mm Hg between ascending and descending aorta. Both the conventional and computed tomography angiographic images are presented. This is the first reported case to include imaging of this anatomy prior to surgery. It is also the first report of primary surgical management following a correct initial diagnosis.     

Early revealing of Williams-Beuren syndrome by digestive disorders]

Williams-Beuren syndrome is a rare syndrome for which diagnosis is usually made during early childhood. It includes mental retardation, friendly outgoing personality, typical facies, supravalvular aortic stenosis and hypercalcemia. CASE REPORT: We report the case of a newborn whose gastroesophageal reflux led to the diagnosis of Williams-Beuren syndrome. Hypercalcemia is known to precipitate digestive symptoms but was not present in this case. CONCLUSION: Announcing such a diagnosis in the neonatal period is difficult and may destabilize the family, but at least allows early care of the cardiovascular pathologies that may lead to death.