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Altered expression of dystrophin within the thoracic aorta in coarctation.

Although persisting endothelial dysfunction has been established in the vasculature of patients following surgical repair of coarctation, it is unknown whether there are alterations in the cytoskeleton of the aorta in such patients. We compared staining of N-terminus dystrophin in the smooth muscle of the aortic wall of a patient with coarctation to that in a patient without coarctation, the latter undergoing surgical treatment of a double aortic arch. There was a marked difference in the pattern of expression of dystrophin between the two, with the coarcted specimen demonstrating marked fragmentation but normal intensity of staining. As far as we are aware, ours is the first report to demonstrate the presence of dystrophin in the smooth muscle of the aorta. Alterations in the cytoskeletal structure may account for underlying aberrations in endothelial function in such patients, and is a topic that warrants further investigation.

Aorta, Thoracic↗

Coarctation of aorta with right aortic arch. Report of surgical correction in 2 cases: one with associated anomalous origin of left circumflex coronary artery from the right pulmonary artery.

Two patients with right aortic arch, anomalous left subclavian artery, and coarctation of theaorta, have been operations for the relief of coarctation, successful in one but only partially successful in the other: in one case, a 'dacron' patch was inserted into the narrowed segment, but a mild recurrence of coarctation was found a year later; in the other, the proximal segment of the anomalous left subclavian artery was used for the repair, with complete relief of the coarctation, both immediately and at late follow-up. The first case had multiple associated congenital abnormalities, including anomalous origin of the left circumflex coronary artery from the right pulmonary artery, which was also corrected surgically: this anomaly has only been recorded once hitherto. The second had facial haemangiomata and a small ventricular septal defect. Four previously reported cases of coarctation and right arch are reviewed. These 6 cases are considered in the light of current views on the development and classification of aortic arch anomalies.

Adolescent↗

Balloon angioplasty for recurrent coarctation of aorta. Immediate and long-term results.

BACKGROUND: As angioplasty techniques have been refined and larger low-profile balloons developed, a nonsurgical approach to recoarctation has become available. Several reports have documented both the efficacy and safety of this procedure. However, there are little data available on the long-term follow-up of these patients. This report details the initial results and long-term evaluation of both the relief of obstruction and the presence of hypertension after balloon angioplasty for recurrent coarctation. METHODS AND RESULTS: Balloon angioplasty for recurrent coarctation of the aorta was performed 29 times in 26 patients at a median age of 4 years and 9 months (range, 4 months to 29 years), with eight patients less than 1 year old. Initial surgical techniques were end-to-end anastomosis in 11 patients, subclavian flap aortoplasty in 11 patients, and patch aortoplasty in four patients. Angioplasty was performed at a median interval of 2 years and 7 months (range, 4 months to 23 years) after surgery. Mean peak systolic pressure difference across the coarctation decreased from 40.0 +/- 16.8 to 10.3 +/- 9.5 mm Hg (p less than 0.05) after the initial angioplasty, and mean diameter of the aortic lumen at the coarctation site increased from 5.8 +/- 3.5 to 9.0 +/- 4.3 mm (p less than 0.05). There was no mortality, and only one patient developed an aneurysm (4%). Three patients underwent repeat angioplasty for a pressure difference of more than 20 mm Hg. Long-term follow-up is available on 24 of 26 patients with a mean follow-up of 42 +/- 24 months (range, 12-88 months). Mean peak systolic pressure difference across the area of coarctation decreased from 40.3 +/- 17.4 before angioplasty to 8.5 +/- 8.3 mm Hg after final angioplasty (p less than 0.05) and 7.5 +/- 7.5 mm Hg at follow-up. Mean peak systolic blood pressure in the upper extremities decreased from 133.1 +/- 14.9 before angioplasty to 111.1 +/- 14.1 mm Hg at long-term follow-up (p less than 0.05). CONCLUSIONS: Balloon angioplasty should be considered the treatment of choice for relief of recurrent aortic coarctation.

Angioplasty, Balloon↗

Covered stents deployed for coarctation of aorta with aneurysm.

Three patients presented to us with upper extremity hypertension and aortic coarctation. Aortic angiograms and spiral computerized tomography delineated the anatomy at the site of coarctation and the associated small aneurysmal dilation. They were taken up for percutaneous stenting of the coarctation segment with cheathum-platinum covered stents. Post-deployment, there was a significant fall in pullback gradients and exclusion of the aneurysms.

Adolescent↗

[Non-invasive continuous hemodynamic measurement during operation for a child with coarctation of aorta].

For non-invasive hemodynamic monitoring, DYNEMO 3000 (SOMETEC Inc) has a 10 mHz ultrasonic echo scan and a 5 mHz Doppler velocimeter connected to an echo-oesophageal probe allowing continuous measurement of the aortic diameter and of the blood flow velocity in the descending aorta. This device thus can only measure the aortic blood flow, but also calculate the stroke volume and the systemic vascular resistance. Moreover, the systolic time intervals and the length of the pre-ejection period can be measured with the oscillometric tensiometer and the electrocardiography. This paper describes a case in which this device was used for a child with coarctation of the aorta. The hemodynamic changes were measured continuously during coarctectomy. During the temporary bypass, the aortic blood flow and the stroke volume decreased, but on the other hand, the total vascular resistance increased at the same time. After the bypass, these parameters returned to the values before the bypass. We think that this device is useful as a non-invasive monitor to observe the hemodynamic changes continuously during operation for a child with congenital heart disease.

Adolescent↗

[The structural reorganization of the arteries of the brain and kidneys in experimental aortic coarctation].

Structural changes of cerebral and renal arteries were studied in 25 dogs with hemodynamic model of aorta coarctation and 10 control animals using histological, histochemical and morphometric methods. It was established in aorta coarctation due to growth and decrease of blood supply into cerebral and renal basins a series++ of compensatory adaptive, adaptive and pathological changes develop in vessels. The first are manifested in cerebral arteries through hypertrophic and hyperplastic changes of media smooth myocyte circular musculature, the second in renal arteries they are manifested through these musculature atrophy. The second--through the development of oblique-longitudinal musculature; the latter--through the reduction of these vessels lumen and the developments of angiosclerosis and angiohyalinosis.

Animals↗

[Elimination of stress-induced activation of DNA reparative synthesis in the myocardium by increasing the load on the heart].

The rate of DNA reparative synthesis was studied in the nucleus of myocardial cells in the heart compensatory hyperfunction (HCH) induced by the aorta coarctation and in animals exposed to surgical stress without the aorta coarctation. It was established that both surgery and emotional painful stress activated the DNA reparative synthesis in myocardial cells. For example, 12-24 hrs following the surgery the DNA reparation rate exceeded control values by 50-74%. HCH induced no changes in the DNA reparation rate in nuclei of myocardial cells. The mechanism of stress-induced DNA damage is discussed and a hypothesis is put forward on stabilizing the effect of hyperfunction on the DNA structure.

Animals↗

[Aneurysm of the ascending aorta in its coarctation].

In long-term period after resection of aorta coarctation, 9-14% patients developed aortic aneurysms, one third of them localized in the ascending aorta. From 146 patients operated on for aneurysm of the ascending aorta, 3 had aortic coarctation. In 2 patients aneurysms of the ascending aorta formed late after resection of the coarctation, the third patient was hospitalized with clinical picture of cardiac insufficiency at terminal stage due to coarctation of the aorta and a giant aneurysm of the ascending aorta with significant aortic insufficiency. The patients underwent successful surgeries: 1) ascending aorta grafting; 2) aortic valve and ascending aorta grafting by Bentallo de Bono method; 3) aortic valve and ascending aorta grafting by Bentallo de Bono method with bypass of descending aorta from the conduit. Cystic medianecrosis and two-volume aortic valve were revealed in all the patients. It is concluded that patients after surgery for coarctation of the aorta require long-term follow-up to defect cardiovascular complications early.

Adolescent↗

Histo-anatomical backgrounds of subclavian flap aortoplasty in coarctation of the aorta.

After repair of coarctation of the aorta using the technique of resection and end-to-end anastomosis, the internal diameters of the aortic isthmus and descending aorta often fail to increase. Better results seem possible with aortoplasty using the left subclavian flap technique. In order to clarify this matter, we investigated the structure of the left subclavian artery comparing it with that of the descending aorta and aortic isthmus: we studied the internal diameter, the thickness of the tunica media and the packing density of its elastic fibers in these vascular elements using a postmortem material of children with a coarctation of the aorta. The ages ranged from 4 days to 13 months with one child of 8 years. All 16 cases had one or more additional cardiac lesions. Operation had been performed in 3 children: 2 end-to-end anastomoses and one subclavian bypass of the aortic arch. Data were compared with observations on autopsy cases of children without cardiovascular abnormalities. The mean findings were that the calibers of the left subclavian artery and the descending aorta were within normal limits but that the caliber of the aortic isthmus was smaller than in normal children. The measurements on the tunica media showed that although, generally, the thickness of the media of the left subclavian artery was smaller than that of the aortic isthmus and descending aorta of the same individual, it contained relatively more elastic fibers than the matching vessels. This may indicate that the structure of the left subclavian artery is well suited to grow out as a part of the aortic arch.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗