A rare combination of post-ductal coarctation of the aorta and adjunct tortuous aneurysm: a neonate with Posterior fossa anomalies, Haemangiomas of the head and neck, Arterial, Cardiovascular, and Eye anomalies and ventral developmental defect syndrome

2022 ◽  
pp. 1-3
Author(s):  
Otohime Mori ◽  
Keiichi Fujiwara ◽  
Hisanori Sakazaki

Abstract A 4-day-old girl with Posterior fossa anomalies, Haemangiomas of the head and neck, Arterial, Cardiovascular, and Eye anomalies and ventral developmental defect syndrome comprising a facial haemangioma, aortic coarctation at the aortic arch, torturous aortic aneurysm distal to coarctation, and ductus arteriosus originating proximal to the coarctation is presented. The aortic arch was successfully reconstructed without cardiopulmonary bypass, and she is currently doing well after 4 years and 8 months.

PEDIATRICS ◽  
1956 ◽  
Vol 18 (4) ◽  
pp. 561-564
Author(s):  
Donald L. Alcott ◽  
Philip Sanfilippo ◽  
Jesse E. Edwards

A case of the Taussig-Bing complex with necropsy findings is reported. Of additional interest was the unusual occurrence of associated malformations of the aortic-arch system, including coarctation of the aorta and anomalous origin of the right subclavian artery proximal to the coarctation. A narrow patent ductus arteriosus was present and probably represented a temporary phase in the process of normal anatomic closure. A single coronary artery that divided into the two main coronary arteries shortly after its origin also was present. Origin of the anomalous right subclavian artery proximal to a zone of aortic coarctation is an extremely rare arrangement among malformations of the aortic-arch system.


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.


2013 ◽  
Vol 24 (1) ◽  
pp. 113-119 ◽  
Author(s):  
Stany Sandrio ◽  
Matthias Karck ◽  
Matthias Gorenflo ◽  
Tsvetomir Loukanov

AbstractBackgroundThe aim of this study was to evaluate the surgical treatment of complex aortic coarctation using partial cardiopulmonary bypass to increase the spinal cord protection.MethodsA total of 15 patients (age range from 7 to 48 years) underwent coarctation repair through a left posterolateral thoracotomy with cardiopulmonary bypass. Cannulation was performed via the descending aorta and the main pulmonary artery. In all, six surgeries were performed under hypothermic circulatory arrest and nine repairs were performed under mild hypothermia. The clinical outcome regarding the development of restenosis, as well as major neurologic complication, was studied.ResultsThere was no mortality. None of the patients developed paraplegia. Of the 15 patients, two developed a recurrent stenosis at the proximal anastomosis between the aortic arch and the aortic prothesis at a mean follow-up of 5.5 years. In the remaining 13 patients, echocardiography and magnetic resonance imaging showed no evidence of a significant gradient.ConclusionComplex aortic coarctation without hypoplasia of the proximal aortic arch and intra-cardiac anomalies can be repaired with low mortality and neurologic morbidity via a left thoracotomy using cardiopulmonary bypass. The use of cardiopulmonary bypass goes along with a low risk of spinal cord and lower body ischaemia and provides a sufficient amount of time for the anastomoses.


PEDIATRICS ◽  
1969 ◽  
Vol 44 (6) ◽  
pp. 1024-1028
Author(s):  
P. B. Deverall ◽  
J. F. N. Taylor ◽  
G. S. Sturrock ◽  
Eoin Aberdeen

Hemodynamic signs of coarctation of the aorta were present in a neonate dying in cardiac failure. A cerebral arteriovenous fistula was found at autopsy. No obstructive lesion of the aortic arch was present. Development of the aortic isthmus may be impaired if diminished flow through this segment is present. Reduced flow may be present if most of the systemic output is diverted to a fistula proximal to the isthmus, distal systemic flow being maintained by flow from right-to-left through the ductus arteriosus. Spontaneous duct closure after birth may then be followed by a reduction in distal systemic flow, resulting in signs suggestive of coarctation.


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


Author(s):  
Takuma Mikami ◽  
Takeshi Kamada ◽  
Hiroki Uchiyama ◽  
Yosuke Kuroda ◽  
Ryo Harada ◽  
...  

Abstract Here we report a rare case of pseudoaneurysm at the site of aortic coarctation. Aortic coarctation and a saccular aortic aneurysm protruding from the site of this coarctation were detected in a 50-year-old woman. Owing to the shape of the aneurysm and high risk of rupture, an open surgical repair was performed. The pathological findings of the removed aneurysm revealed a pseudoaneurysm consisting of only a thin adventitial wall. Adult uncorrected aortic coarctation has a poor prognosis. One of its causes may be the formation of such a pseudoaneurysm.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
E A Khalifa ◽  
S Helmy ◽  
S F Mohamed ◽  
M Alkuwari

Abstract Introduction Aneurysms are found following all types of surgical repair of aortic coarctation, especially after Dacron patch aortoplasty. We describe the finding of an aortic aneurysm in an asymptomatic 52-year-old male, who was managed by Dacron patch aortoplasty for native coarctation of the aorta 34 years earlier. Case report A 52-year male, smoker, hypertensive on medication He had previous history of surgical repair of aortic coarctation at age of 18 years . Repair was by Dacron patch aortoplasty. Since then, his regular follow up was unremarkable. Recently, he was referred for cardiac evaluation as a part of pre-employment general check-up. He was asymptomatic with no history of shortness of breath or chest pain. Physical examination revealed that the pulse in the left arm was reduced in volume in comparison to the right one. The heart sounds were essentially normal but a pericardial murmur was audible, perhaps reflecting residual collateral flow. Blood pressure was 156/83 mmHg in right arm and 142/81 in the left arm. Transthoracic echocardiography revealed mild left ventricular hypertrophy with normal global and regional contractility and an ejection fraction of 58%. Supra sternal window images showed dilatation of the three aortic arch branches. The distal portion of aortic arch just distal to origin of left subclavian artery was narrowed with a peak systolic gradient across of 34 mmHg. A cystic structure (1.7 cm x 1.9 cm) was visualized attached to the narrowed segment of the aorta, suggestive of a saccular aneurysm, (figures A&B&C). Computed tomography aortogram showed a narrow-necked aneurysm arising from the posterolateral aspect of the distal aortic arch (anticipated site of the coarctation repair graft anastomosis). A small laminated thrombus was also noted within. Aneurysm measured approximately 2.2 x 3.3 cm in its craniocaudal and anteroposterior dimensions respectively, with no evidence of aortic luminal compromise. (figures D&E&F). Management Aneurysmectomy was performed subsequently. Interposition polyester grafts were used to reconstruct the aortic arch and proximal descending aorta and to connect this aortic segment to the subclavian artery via a lateral thoracotomy. The postoperative course thereafter was uneventful. Conclusion: This is a rare insidious complication of Dacron patch aortoplasty that occurred after more than 3 decades, which highlights the importance of diagnostic imaging in the follow up of these patients Abstract P1494 Figure.


2012 ◽  
Vol 60 (9) ◽  
pp. 575-577
Author(s):  
Masatoshi Shimada ◽  
Takaya Hoashi ◽  
Koji Kagisaki ◽  
Tatsuya Oda ◽  
Isao Shiraishi ◽  
...  

2012 ◽  
Vol 22 (5) ◽  
pp. 610-614 ◽  
Author(s):  
James R. Bentham ◽  
Nilesh Oswal ◽  
Robert Yates

AbstractObjectiveTo describe endovascular stent placement using partially covered stents to preserve flow in head and neck vessels.BackgroundEndovascular stent placement has become established as a first-line therapy for native coarctation of the aorta or re-coarctation in older children and adults. Increasingly covered stents are becoming the preferred option over bare-metal stents because of the perceived lower risk of aneurysm formation. Open-cell bare-metal stents are chosen when there is a high likelihood of jailing a head and neck vessel. Here we describe partial uncovering of a covered stent before implantation to allow flow through the uncovered portion of the stent to the branch vessel but preserve the covering over the majority of the remaining stent.MethodsWe describe two cases with aortic arch hypoplasia and re-coarctation, both of which required two partially uncovered stents for a satisfactory result.ConclusionsEndovascular stent placement is becoming the preferred option in the management of coarctation of the aorta in older children and adults. Strategies to deal with transverse arch hypoplasia and multiple levels of aortic arch obstruction frequently involving branch vessels or aneurysms need to be considered before these procedures are embarked upon. Partially uncovering stents may afford more protection than using bare-metal stents in the transverse and distal arch while preserving flow in head and neck branches, and is a technically straightforward procedure.


Sign in / Sign up

Export Citation Format

Share Document