The follow-up surgical results of coarctation of the aorta procedures in a cohort of Chinese children from a single institution.

OBJECTIVES: The aim of this study was to evaluate the results following surgeries for the treatment of coarctation of the aorta in Chinese paediatric patients and to compare the surgery outcomes between simple and complex coarctation procedures. METHODS: Between January 2006 and December 2011, 107 consecutive paediatric patients with coarctation of the aorta underwent surgery. Forty-four patients (41.12%) were classified as having simple coarctations (group A), and 54 patients (50.47%) were classified as having complex coarctations (group B). Echocardiography and the resting systolic blood pressure were evaluated prior to the operation, at one month following the operation, and then once annually. RESULTS: Follow-up was 93.5% complete (100 patients), without significant differences between the two groups. Arch hypoplasias and bicuspid aortic valves were initially present in 10 (9.35%) and 11 (10.28%) of 107 patients, respectively. There were no deaths among the group A patients and three (5.56%) early deaths among the group B patients. There was a significant difference in the restenosis incidence rate between the two groups during the most recent follow-up consultations (p<0.05). Additionally, only 10 of 43 group A and 10 of 51 group B patients had persistently abnormal blood pressures during the annual follow-up consultations. CONCLUSIONS: The postoperative restenosis ratio was increased in the complex coarctation group compared with the simple coarctation group. Additionally, the complex coarctation patients who did not have restenosis at follow-up had a lower proportion of hypertension.

Interrupted aortic arch or extreme coarctation? A case report and review of the literature.

The hallmark feature of aortic interruption that is useful in differentiating it from aortic coarctation is the "complete absence" of continuity between both parts of the interrupted segment. In this study, we reviewed the 28 patients diagnosed with isolated interrupted aortic arch (IAA) who reached adult age (> 20 years), aimed to review the validity of the Celoria-Patton classification in the literature, and reported the first microscopic pathology of the IAA in an adult.

[Diagnostic value of 64 multislice CT in typing of congenital aortic anomaly in neonates and infants].

OBJECTIVE: To study diagnostic value of 64 multislice CT in typing of congenital aortic anomaly in neonates and infants. METHODS: 120 pediatric patients (under one year of age) with congenital heart disease (CHD) underwent 64 contrast-enhanced MSCT before a corrective operations. The diagnostic sensitivity, specificity and accuracy of 64 MSCT were evaluated and also compared with those of echocardiography with Doppler. The patients were randomly assigned to two groups (72 and 48 persons) respectively according to tube tension of 80 and 100 kV. The differences of the image qualities were compared between them. RESULTS: 36 congenital aortic anomalies were found (36/120, 30%) by 64 MSCT, which were furtherly distinguished into 2 cases in double aortic arch, 2 cases in left-sided aortic arch with aberrant right subclavian artery, 22 cases in right aortic arch (6 cases in right-sided aortic arch with aberrant left subclavian artery, 12 cases in right aortic arch with mirror image branching), 10 cases in coarctation of aorta. Diagnostic sensitivity, specificity and accuracy of 64 MSCT were all 100%. By contrast, those of echocardiography with Doppler were 27.8%, 97.6%, 76.7%, respectively. The quality scores were 4.69 ± 0.52, 4.58 ± 0.58 at 80 kV and 100 kV, respectively. No significant statistical difference was found between them (t = 1.08, P = 0.28). CONCLUSION: MSCT allows a detailed assessment of the anatomy of congenital aortic anomaly, which can be used as an important supplementary method in diagnosing CHD and offer important information for operation. 80 kV should be selected in CHD patients less than one year old for CT examination to reduce radiation exposure.

Hypertension in aortic coarctation.

Patients with aortic coarctation are prone to develop arterial hypertension at various stages throughout life. There are at least three different pathophysiologic pathways: re-stenosis at the aortic isthmus, paradoxical hypertension, and late hypertension at long-term follow-up. As the most common causes of death reported for coarctation patients are linked to hypertension, it is important to differentiate these pathways of hypertension carefully to provide optimal treatment for hypertensive coarctation patients. This review summarizes the actual data about those different pathologic pathways, about how to differentiate them from each other, and how to treat them adequately.

MR imaging of aortic coarctation.

Aortic coarctation accounts for 5%-10% of all congenital heart diseases and represents 7% of critically ill infants with heart disease. Magnetic resonance (MR) imaging allows the study of this disease with several advantages in comparison with conventional angiography, transesophageal echocardiography, and computed tomography. The MR protocol applied at our institution for both diagnosis and follow-up after surgical or endovascular treatment consists of four steps: morphologic study, cine MR study, flow analysis, and MR angiography (MRA). The first three sequences are acquired during breath-hold and with electrocardiographic gating. Anatomy is well depicted with dark-blood half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences. Cine true-fast imaging with steady-state precession (true-FISP) sequences show not only morphologic features but also blood-flow changes inside the aorta. Gradient-echo sequences for phase-velocity mapping allow flow analysis. Application of Bernoulli's equation--here briefly presented and discussed--allows for calculation of the pressure gradient caused by the coarctation. MRA, acquired with a breath-hold three-dimensional T1-weighted gradient-echo sequence and intravenous administration of paramagnetic contrast material, allows for optimal depiction of the aortic lumen, with a panoramic view of the whole aorta, its main branches and possible collateral circulation.

Quantification of morphologic and hemodynamic severity of coarctation of the aorta by magnetic resonance imaging.

OBJECTIVE: As the morpholgic severity of coarctation of the aorta is difficult to assess, especially after previous repair, the value of the technique of multiplanar reconstruction of magnetic resonance imaging data to achieve a 3-dimensional reconstruction of the aortic arch was evaluated and compared to hemodynamic measurements. METHODS AND RESULTS: We performed 30 examinations in 27 patients aged from 6 to 54 years, with a mean of 21 years, by magnetic resonance imaging using a 1.5Tesla scanner with a standard body coil. Measurements of flow across the coarctation were performed using phase shift velocity mapping, and peak velocity was calculated at the site of stenosis. Aortic cross-sectional area before, at, and beyond the stenosis was reconstructed 3-dimensionally to calculate a percentage degree of stenosis. Morphologic severity of stenosis was correlated to invasively assessed hemodynamic gradients and morphologic data from biplane angiography in 23 patients. Among the 30 examinations, 24 patients had been previously treated by either surgery, in 17 patients, or balloon dilation, while 6 had native coarctation. 3-dimensional reconstruction was possible in all and better delineated the anatomy concerning the hemodynamic relevance of stenoses even as compared with biplane angiography. The correlation between severity of narrowing assessed by diameter measurements in the biplane angiography and 2-dimensional magnetic resonance imaging was r = 0.94, and multiplanar reformation with 2-dimensional magnetic resonance imaging was r = 0.87 with a tendency of higher grading with the 3-dimensional technique (p = 0.0001). The correlation of 2-dimensional magnetic resonance imaging with invasively measured hemodynamic gradients was r = 0.67 versus r = 0.74 for the areas assessed by multiplanar reformation, indicating that the hemodynamic relevance of a morphological approach to evaluate the degree of a stenosis should better be assessed 3-dimensionally. CONCLUSIONS: The 3-dimensional reconstruction of the morphologic severity of coarctation offers additional information over conventional imaging especially in patients with kinking, complex geometry, or collaterals, in whom hemodynamic measurements can become unreliable.

Intravascular ultrasound imaging shortly after balloon angioplasty of native aortic coarctation in two neonates.

Two patients who had a balloon angioplasty for native coarctation of the aorta as neonates underwent redilatation for restenosis 1 or 2 months after the initial procedure. We recorded intravascular ultrasound (IVUS) images at redilatation. Before redilatation, there was an abnormal, localized area of ultrasound density equal to the inner layer of the aorta. The enlargement of this tissue was in proportion to the severity of stenosis and diminished after dilatations. No intimal flaps or tears were observed. From the IVUS images it appears that the mechanism to relieve the coarctation of our patients may be compression of this abnormal tissue.

Síndrome de shone asociado a hidramnios: a propósito de un caso y revisión de la literatura / Shone's syndrome associate a hydramnios: a purpose of a case and literature review

Presentamos un disco de diagnóstico prenatal de Síndrome de Shone, el cual fue llevado a término y extraído por cesárea. El diagnóstico prenatal se acompaño de hidramnios que pensamos se debio a compresión externa del esófago por la aurícula izquierda muy dilatada. Se establecio insuficiencia cardíaca intratable y dada la severidad de las lesiones se consideró inoperable, muriendo a las 48 horas de nacido. Los ecosonogramas rutinarios y de Doppler prenatales fueron registrados en cinta VHS. No hubo estudio anatomo-patológico

Surgical treatment of Takayasu arteritis.

From 1959 to 1991, 93 patients underwent vascular reconstruction for Takayasu arteritis at our institution. The details of the cases were as follows: 16 were of type I (brachiocephalic ischemia), 48 type II (hypertension), 13 type III (extensive lesions with cerebral ischemia and hypertension), and 16 type IV (aneurysms). Carotid reconstruction, repair of atypical aortic coarctation, renovascular reconstruction, and aneurysm repair were performed independently or in combination. Nine operative deaths occurred, 8 cases of which were operated before 1970. The most serious of the delayed complications was suture line aneurysm formation, which was encountered in ten cases. The aneurysms were often found long after the operation, some of them developing even after more than 20 years. Takayasu arteritis is characterized by extensive inflammation and destruction of the medial elastic fibers and long term postoperative observation is mandatory to improve the late survival rate.

Role of extended aortoplasty related to the definition of coarctation of the aorta.

One hundred thirty-nine patients underwent operation for coarctation of the aorta. Age ranged from 1 day to 21 years and weight, from 1.5 to 70.4 kg. Numerous methods of repair were used. The operative mortality was low (1.3%), and 17 patients (11.3%) died late. Recoarctation occurred in 13 patients (9.4%). We attempted to correlate mortality and recoarctation with the surgical procedure. A review of the literature revealed no classifications of coarctation that applied to the anatomical and pathological variations we found at the time of operation. Therefore, we devised a surgical classification to separate the various entities in the spectrum of coarctation: type I = primary coarctation; type II = coarctation with isthmus hypoplasia; and type III = coarctation with tubular hypoplasia involving the isthmus and segment between the left carotid and left subclavian arteries. Each of these types has subtypes: A = with ventricular septal defect and B = with other major cardiac defects. We believe that rather than labeling one procedure as "the procedure of choice," providing this classification will allow the surgeon to use a method of repair that is suited to the anatomical variation.

Coarctation of the aorta.

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.

Balloon angioplasty of aortic coarctation: a review.

Since the initial report of balloon coarctation angioplasty in 1982, several workers used this technique in native coarctation and postoperative recoarctation. Immediate and intermediate-term follow-up results are generally good with a small chance for recoarctation and aneurysmal formation at the site of coarctation. The causes of recoarctation were identified and include age less than 1 year, isthmus hypoplasia, and a small coarcted aortic segment. Despite good immediate and follow-up results, recommendations for use of balloon angioplasty as a treatment procedure of choice are clouded by the reports of development of aneurysms at the site of coarctation. We feel that balloon coarctation angioplasty is the treatment of choice in neonates and small infants, while general use of this technique in both native and postoperative coarctations in older children should await follow-up results in larger numbers of children at selected centers.

Coarctation and other obstructive aortic arch anomalies: their relationship to the ductus arteriosus.

Forty-three specimens with an obstructive aortic arch anomaly, obtained from infants that had died under the age of one year, were studied to establish the relationship between the arch anomalies and the ductus arteriosus. All specimens were studied morphologically. Twenty of the 43 were also studied histologically. The morphological part of the study included measurements of all the segments of the aortic arch and establishment of the localisation of any associated coarctation. The specimens were divided according to the appearance of the isthmus. The isthmus was not present in 4, was atretic in 2, hypoplastic in 19 and had a normal diameter in 18 cases. In all cases, except 5 (2 with atresia and 3 with hypoplasia of the isthmus) there was an associated coarctation. The majority of the coarctations (28) was located preductally. All such preductal coarctations had ductal tissue in the obstructing ridge. There were 5 paraductal coarctations. Of these, two also had ductal tissue in the ridge. The distribution of the ductal tissue among the two types of coarctation suggested a spectrum of anomalies rather than two completely different entities. The patterns of the aortic arches appeared predictive for the type of coarctation present. The arches that tapered normally towards the isthmus either presented with a preductal coarctation (20 cases) or without a coarctation (3 cases). All 4 specimens in which the isthmus was lacking also had a preductal coarctation. The 5 paraductal coarctations were found in arches that showed a "reverse" pattern, tapering towards the brachiocephalic arteries. All the preductal coarctations contained ductal tissue whereas only 2 of the 5 paraductal coarctations had ductal tissue in the obstructing ridge. It is concluded that careful evaluation of all the segments of the aortic arch may be helpful in the clinical evaluation of infants with an obstructive aortic arch anomaly.