Atypical Coarctation of the Aorta with Bilateral Vertebral-Subclavian Pathway: A Report of the First Known Case of Preligamental Aortic Arch Interruption and its Surgical Correction

Author(s):  
Per Zetterqvist
2020 ◽  
Vol 26 (4) ◽  
pp. 4-12
Author(s):  
A.А. Malska ◽  
◽  
O.B. Kuryliak ◽  

Aim. To determine the rate of aortic coarctation, the correlation of its anatomical forms - critical and not critical, and the frequency of combination with the associated pathology; to define the features of the clinical course of its different anatomical forms; and to analyze the remote results of the surgical correction of this defect. Material and Methods. The article represents the statistical analysis of outpatient medical records and case histories of 86 children with aortic coarctation in Lviv region. In the course of the research, retrospective and epidemiological studies were carried out; clinical (data acquisition of medical history, physical examination), instrumental (Doppler echocardiography, ECG, X-ray imaging of organs of the thoracic cavity), and statistical methods were used. Results and Discussion. It was determined that over the period of 2008-2020 years, out of 74 neonates with CoA registered at Lviv Regional Children's Hospital (Health Care of Mother and Child) 40,54% had the critical CoA form, while 59 (46%) - uncritical CoA form. In children with the critical CoA form, the most frequent findings were hypoplasia of the aortic arch (56,67%), open aortic duct (53,33%), and open oval window (53,33%); in 36,6% cases CoA was combined with the bicuspid aortic valve, interatrial septal defect, and transposition of great vessels. However, the uncritical CoA form was more frequently combined with the bicuspid aortic valve (52,27%), and hypoplastic aortic arch (31,82%); aortic stenosis was revealed in 20,45% of children. After the surgical correction in 43,59% of the operated patients with uncritical CoA, excessive arterial hypertension was observed, while in critical form, the frequency of excessive arterial hypertension among the operated patients amounted to 10%. According to our research, after the plasty of the critical CoA, aortic recoarctation was observed in 3.33% of the operated patients, whereas, in case of the uncritical CoA form, it occurred in 30,77% of the operated ones. Conclusions. Coarctation of the aorta is a congenital heart disease with relatively high incidence, amounting to 5-8% out of all congenital defects of the heart. In newborns, it is manifested by acute cardiac failure, while in elder children it is presented with arterial hypertension. Echocardiographic examination after Doppler analysis is the basic procedure of diagnostics and allows for precise determination of the CoA anatomy. The majority of cardiologists recommend prompt surgical intervention after the diagnosis has been made, and, particularly, in patients with hypertension. At present, the available surgical methods include surgical excision of the aortic obstruction, and catheter intervention (the balloon angioplasty and stent implantation) . After the surgical correction has been performed, arterial hypertension persists. The duration of hypertension after the coarctation correction depends on its duration before the diagnosis is made and the timing of surgical correction of the defect. With the child's growth , recoarctation may occur. In such patients, normal arterial pressure can be determined in the state of rest, but it may increase in the upper extremities during physical exertion. Key words: aortic coarctation, associated pathology, excessive arterial hypertension, recoarctation


2016 ◽  
Vol 20 (2) ◽  
pp. 66 ◽  
Author(s):  
I. A. Soynov ◽  
Yu. S. Sinelnikov ◽  
I. A. Kornilov ◽  
N. R. Nichay ◽  
E. N. Gasanov ◽  
...  

<p><strong>Aim:</strong> The paper focuses on assessment of the late results and causes of complications when repairing coarctation with distal aortic arch hypoplasia.<br /><strong>Methods:</strong> This prospective randomized study included 54 patients who underwent repair of coarctation of the aorta. All patients were divided in 2 groups depending on the correction type: reconstruction using the modified reverse left subclavian artery flap plasty (Group I, 27 pts) or extended end-to-end anastomosis (Group II, 27 pts).<br /><strong>Results:</strong> A postoperative follow-up period was 26 (21;31) months. Recoarctation of the aorta during long-term follow-up was found in 1 patient (3.84%) in Group I and in 2 patients (7.7%) in Group II (p=0.5). Two patients in the Group I (7.7%) and 8 patients in the Group II (30.8%) had arterial hypertension (p=0.03).<br /><strong>Conclusion:</strong> With modern types of surgical correction used, the anastomotic area tended to proportionally grow in different segments of the aortic arch and did not influence the postoperative complications rate. Endocardial fibroelastosis and ascending aortic wall rigidity could be considered as predictors of arterial hypertension in the late period after coarctation repair in infants. Low body weight before surgery is a factor of aortic recoarctation development in the late postoperative period. Arterial hypertension persistence depends on the correction type. One-stage reconstruction of the distal aortic arch allows for reducing the rate of arterial hypertension from 30.8% to 7.7%. Despite a range of complications in the late postoperative period, the infants’ quality of life was not affected and was high in both groups.</p>


1971 ◽  
Vol 11 (6) ◽  
pp. 593-597 ◽  
Author(s):  
D. Alton Murphy ◽  
George Collins ◽  
A.R.C. Dobell

Aorta ◽  
2019 ◽  
Vol 07 (03) ◽  
pp. 084-086
Author(s):  
Duccio Federici ◽  
Samuele Bichi ◽  
Gianfranco Montesi ◽  
David Matiashvili ◽  
Cristina Agostinis ◽  
...  

AbstractAortic coarctations in adults are mainly represented by recurrent critical narrowing at the site of previous surgical correction, or less frequently by native forms of complex obstructive malformations of the distal arch and isthmus. We present our experience with an unusual form of native adult aortic coarctation presenting as a complete interruption of the aortic arch.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Xinjian He ◽  
Jiaoyang Chen ◽  
Gaoyang Li

Abstract Background The purpose of this study was to explore echocardiographic views and methods of aortic arch anomalies in infants, so as to improve the screening sensitivity and diagnostic accuracy. Methods 140 children with abnormal aortic arch diagnosed by ultrasound in Children’s Hospital of Hebei Province from January 2014 to December 2019 were selected for retrospective analysis. All were confirmed by surgery or/and computerized tomography angiography. Series of views for aortic arch (the three-vessel and tracheal view, aortic arch short axis view, left aortic arch long axis view, aortic arch long axis continuous scan views) were performed in all cases on the basis of the routine views of echocardiography. The screening sensitivity and diagnostic coincidence rate of different echocardiographic views for aortic arch anomalies were analyzed. Results Among the 140 infants, right aortic arch were 21 cases (6/21 were accompanied by mirror branch and 15/21 were with aberrant left subclavian artery). Left aortic arch with aberrant right subclavian artery were 2 cases, and double aortic arch with both arches open were 20 cases. Double aortic arch with left arch atresia were 2 cases, and atresia of the proximal aorta with aortic arch dysplasia was 1 case. Coarctation of the aorta were 67 cases, and interruption of aortic arch were 27 cases. All the patients were correctly diagnosed except that 2 infants with interruption of aortic arch were incorrectly diagnosed as coarctation of the aorta, and 1 infant with coarctation of the aorta was misdiagnosed as interruption of aortic arch by echocardiography. The screening sensitivities of four views and four-view combination for abnormal aortic arch were 99.3, 73.6, 87.1, 99.3, and 100%; the diagnostic coincidence rates were 85.7, 27.1,66.4, 95.0%, and 97.9% respectively. On the basis of traditional left aortic long axis view, other three views had their own advantages. The screening sensitivity and diagnostic coincidence rate of four-view combination were significantly improved. Conclusions The three-vessel trachea view is simple and feasible, which is suitable for screening abnormal aortic arch. The combination of four views conduces to improving screening sensitivity and diagnostic accuracy of aortic arch abnormalities.


2004 ◽  
Vol 14 (2) ◽  
pp. 206-209 ◽  
Author(s):  
Gerald Wendelin ◽  
Erwin Kitzmüller ◽  
Ulrike Salzer-Muhar

The acronym PHACES summarizes the most important manifestations of a rare neurocutaneous syndrome. Specifically, “P” accounts for malformation of the brain in the region of the posterior fossa, “H” stands for haemangiomas, “A” is for arterial anomalies, and “C” is for coarctation of the aorta along with cardiac defects, “E” is for abnormalities of the eye, and “S” for clefting of the sternum, and/or a supraumbilical abdominal raphe. Our objective is to introduce the syndrome to paediatric cardiologists. Our patient has stenosis of the aortic arch, multiple malformations of the great vessels arising from the aortic arch, intracranial vascular abnormalities, a sternal malformation with a supraumbilical raphe, and facial haemangiomas. We stress that it is important always to consider the existence of this syndrome in all patients with facial haemangiomas.


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