[Two-staged correction for the coarctation of aorta complex (coarctation complex) and interrupted aortic arch (IAA)].
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Coarctation of the aorta is an important and treatable cause of secondary hypertension. The prevalence of aortic coarctation varies from 5% to 8% of all congenital heart defects. Neonates and infants, especially when they have other associated cardiac defects, may present with signs and symptoms of heart failure. Children beyond infancy are usually asymptomatic and are most often diagnosed because of a murmur or hypertension on a routine examination. Palpation of the brachial and femoral pulses simultaneously will show decreased and delayed or absent femoral pulses. On measurement of blood pressure from arms and legs, a pressure difference of more than 20 mm Hg in favor of the arms may be considered as evidence for coarctation of the aorta. The coarctation can be demonstrated on suprasternal notch two-dimensional echocardiographic views along with increased Doppler flow velocity across the coarctation site. Cardiac catheterization shows significant peak-to-peak systolic pressure gradient across the coarcted segment, and aortography demonstrates the degree and nature of the aortic narrowing. Aortic coarctation may be relieved by surgery or by balloon angioplasty; in asymptomatic patients, therapy during the ages of 2 and 5 years is suggested. Surgical relief of coarctation may be achieved by resection and end-to-end anastomosis or by subclavian flap or prosthetic path angioplasty. Although results of surgery are generally good, there are some problems with the procedure, namely, mortality, morbidity and recoarctation, particularly in neonates and young infants and development of aneurysm, paraplegia, and paradoxical hypertension. Balloon angioplasty has been used by some cardiologists with resultant relief of obstruction, but concern for development of aneurysms and arterial complications remain. Although the immediate results for surgical or balloon therapy for isolated coarctation are good, long-term prognosis is largely undetermined. Limited long-term follow-up studies suggest significantly lower survival rates compared with normal population; age at intervention and the degree and duration of hypertension before intervention may affect long-term survival.
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.
Coarctation of aorta is a rare cause of hypertension in pregnancy and the case material reported in literature is limited without a univocal line of clinical conduct. A case association between isthmic coarctation of the aorta and pregnancy in a 17 year-old primigravida woman is described.
Coarctation of the aorta is a common cardiovascular disorder with an unknown etiology. In the preductal type, blood flows from a patent ductus into the distal aorta. When the coarctation is juxtaductal or postductal, blood flows to the lower extremities by way of the subclavian arteries and collaterals. Plain films may show the reverse sign in postductal coarctation. Arteriography is the gold standard for making the diagnosis. However, magnetic resonance imaging will probably become an increasingly important diagnostic tool. The treatment of choice is surgery, with complete resection of the stenosed segment.
Coarctation of the aorta is one of the congenital heart diseases diagnosed primarily in childhood and early adolescence. A 67-year-old female was admitted to the hospital with chest pain. Angiography revealed a coarctation of the aorta, coronary ectasia and dilatation of the ascending aorta. This case attracted our attention because the first diagnosis was made at such an advanced age and coarctation was accompanied by coronary ectasia and dilatation of the ascending aorta. Therefore, we decided to report the case.
Coarctation of the aorta is a serious heart defect that can be successfully treated if identified early. Actual narrowing of the aortic isthmus is very difficult if not impossible to demonstrate on prenatal sonography; however, the indirect sign of discrepant ventricular sizes (right ventricle [RV] greater than left ventricle [LV]) is potentially useful to identify fetuses at risk for having coarctation of the aorta. We report on nine fetuses, 18 to 38 weeks gestation, in which the left ventricle was smaller than the right ventricle. After birth, four of the nine had coarctation of the aorta. One infant with Down's syndrome had a patent ductus arteriosus as well as foramen ovale. Another infant had a small left ventricle and parachute mitral valve but no coarctation. The last three infants had a normal cardiac workup at birth. We conclude that a small left ventricle compared to the right ventricle on prenatal sonography can be a sign of congenital heart disease and that one of the defects that can give this appearance is aortic coarctation. Careful neonatal follow-up is warranted.
Coarctation of the aorta is a relatively common congenital condition. Most infantile presentations of aortic coarctation are related to the associated congenital cardiac abnormalities leading to congestive heart failure or systemic hypoperfusion. We describe a 4-month-old infant who presented with stridor as the sole manifestation of coarctation. Radiologic studies revealed enlarged innominate artery due to the aortic coarctation that resulted in tracheal compression. After surgical correction, respiratory signs and symptoms completely resolved. This case report describes a unique cause of stridor in newborn infants and discusses the potential for vascular anomalies to result in tracheal narrowing.
BACKGROUND: Atheromatosis of the thoracic aorta and aortic arch is a well established source of systemic embolism. Acquired atheromatous coarctation of the aortic arch is a rare finding and not well documentated so far. CASE REPORT AND FINDINGS: Two patients presenting with intermittent claudication of the lower extremities were identified as having thromboatheromatous coarctation of the aortic arch as visualized by magnetic resonance tomography, fast CT scan, transesophageal echocardiography, cardiac catheterization and aortography. All findings including invasive hemodynamics resembled congenital coarctation of the aorta. One patient was treated surgically, while the other refused surgery and received long-term anticoagulation. CONCLUSION: Atheromatosis of the thoracic aorta and aortic arch not only cause systemic embolism, but may lead to the clinical and hemodynamic picture of coarctation of the aortic arch.
Coarctation of the aorta is a cause of hypertension that can present dramatically in the severely ill infant or appear incidentally in the asymptomatic young adult. Coarctation is easily detected and there is good evidence that the extremely poor natural history can be altered favorably by surgical correction. The elevated blood pressure can be lowered in the majority of patients, and there is every expectation that the incidence of known complications of coarctation can be decreased. However, in about three fourths of the patients, significant residual cardiovascular defects persist, with their potential for future problems as yet incompletely defined. For this reason, patients who have undergone surgery for coarctation will need to be followed carefully for an indefinite period.
Coarctation of the aorta is frequently associated with left ventricular inflow tract abnormalities that may be difficult to detect even at cardiac catheterization. This study involved patients with coarctation who underwent comprehensive two-dimensional echocardiographic investigations emphasizing visualization of the mitral valve and its apparatus. Of the 56 patients studied, 23 had completely normal study results, while in 33 (59%) results showed abnormalities of the mitral complex. The latter were divided into two groups: those having major mitral abnormalities (n = 12) and those with minor anomalies of the valve and apparatus (n = 21). The first group included patients with a supravalve stenosing ring, congenital mitral stenosis, mitral valve prolapse, and parachute mitral valve. Minor anomalies were classified as abnormalities of the papillary muscles, chordae tendineae, or combinations of both. No mitral malformations were found in a control group of patients. We conclude that two-dimensional echocardiography is a sensitive and accurate noninvasive method for assessing either significant or subtle forms of left ventricular inflow disease in patients with aortic coarctation. Its use is recommended for the acute medical and surgical management of these patients as well as for their long-term follow-up evaluation.
Coarctation of the aorta is an uncommon cause of cerebral hemorrhage in the full- or near-term infant. The clinical, radiologic, and neurologic findings of four infants with aortic coarctation and cerebral hemorrhage are presented. In all four infants, cerebral hemorrhage was associated with only moderate elevation of systolic blood pressure (90-110 mmHg).
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 may seem to be a relatively simple lesion, but controversy persists about the optimal management strategy for this condition. A plethora of options are available, and strong evidence to guide clinical decision making is lacking. The age of the patient at presentation and anatomic variations have a significant effect on the type of therapy chosen and the outcome of intervention, which can include death, recurrence of obstruction, and persistent or late hypertension. The current trend in practice is to favor surgical repair in neonates and infants and transcatheter balloon dilation in older children and adults.
Coarctation of the aorta represents 5-7% of congenital heart defects. Symptoms and prognosis depend on the degree of stenosis, age at surgery, surgical method and the presence of other heart defects. Postoperative complications are hypertension, restenosis and an abnormal blood pressure response during exercise. This study includes 41 patients, 15-40 years old, operated in the period 1975-1996. All were exercised on a treadmill until maximal oxygen consumption was achieved. Blood pressure was measured in the right arm and leg before and immediately after exercise, and in the right arm during exercise. Oxygen consumption was monitored and we defined an aerobic phase, an isocapnic buffering phase and a hypocapnic hyperventilation phase. The resting systolic blood pressure correlates with the resting systolic blood pressure difference between right arm and leg. A resting systolic blood pressure difference between the right arm and leg of 0.13 kPa (1 mmHg) to 2.67 kPa (20 mmHg) corresponds with a slight increase in resting systolic blood pressure. This rise in blood pressure increases the aerobic phase of the exercise test, helping the patients to achieve higher maximal oxygen consumption. A resting systolic blood pressure difference of more than 2.67 kPa (20 mmHg) corresponds with severe hypertension and causes reduction in the aerobic phase and maximal oxygen consumption. Resting systolic blood pressure and resting systolic blood pressure difference between the right arm and leg are not indicators for blood pressure response during exercise. Exercise testing is important to reveal exercise-induced hypertension and to monitor changes in transition from aerobic to anaerobic exercise and limitation to exercise capacity.
Aorta coarctation results in hypertension (HTN) in the arterial tree proximal to stenosis and, as such, provides an ideal model to discern the effects of different levels of blood pressure on the vascular tissue in the same animal. Compelling evidence has emerged supporting the role of oxidative stress as a cause of HTN. However, whether or not HTN (independent of the circulating humoral factors) can cause oxidative stress is less certain. NAD(P)H oxidase isoforms are the main source of reactive oxygen species (ROS) in the vascular tissues. We therefore compared the expressions of NOX-I, gp91phox and the regulatory subunits of the enzyme in the aorta segments residing above and below coarctation in rats with abdominal aorta banding. Rats were studied 4 weeks after aorta banding above the renal arteries or sham operation. Subunits of NAD(P)H oxidase and its NOX-I isoform as well as endothelial NO synthase (eNOS) and nitrotyrosine (footprint of NO oxidation by superoxide) were measured in the aorta segments above and below coarctation. The gp91phox, p47phox, and p67phox subunits of NAD(P)H oxidase, NOX-I isoform, eNOS and nitrotyrosine were markedly increased in the aorta segment above coarctation (hypertensive zone), but were virtually unchanged in the segment below coarctation. Since, excepting blood pressure, all other conditions were constant, the upregulation of NAD(P)H oxidase isoforms and the increased NO oxidation in the aorta segment above, but not below, coarctation prove that HTN, per se, independent of circulating mediators can cause oxidative/nitrosative stress in the arterial wall. These observations suggest that HTN control may represent a specific form of antioxidant therapy for hypertensive disorders.
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