DOCSLIB.ORG

Coarctation of the Thoracic Aorta Associated with Cerebral Arterial Occlusive Disease

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Coarctation of the Thoracic Aorta Associated with Cerebral Arterial Occlusive Disease 577 Coarctation of the Thoracic Aorta Associated with Cerebral Arterial Occlusive Disease 3 Harold A. Baltaxe," 2 Solomon Bloch,' and Paul K. Mooring Since coarctation of the aorta was first described by extremely poor and no bl ood pressure could be obtained in them. Johann Friederich Meckel in 1760 [1], a vast literature has Blood pressure in his arm s was 122 / 58 mm Hg. appeared on its associated conditions and treatment. This Laboratory findings were noncontributory. Chest rad iography anomaly of the descending thoracic aorta is usually located revealed left ventricular prominence and a notch-shaped left con­ distal to the left subclavian artery and is commonly found in tour of the descending aorta suggesti ve of coarctation. El ectro­ cardiography revealed left ventricular and left atrial hypertrophy. coexistence with intra- and extracardiac pathology. Ventric­ Heart catheterization and angiocard iography revealed a 90% coarc­ ular and/ or atrial septal defects [2], as well as discrete tation of the aorta distal to the left subclavi an artery with poststen­ subvalvular stenosis, represent the accompanying intracar­ otic dilatation of th e descending th oracic aorta. The vessels to the diac conditions [3, 4]. The presence of a bicuspid aortic head appeared anatomically norm al in their takeoff from the arch. valve was first noted by Edwards [5] and is found in 85% of Selective cerebral arteriography was perform ed. Injecti on of the patients afflicted by coarctation of the aorta [6]. Supraval­ right common carotid artery demonstrated a norm al extracranial vular stenosis is only rarely an associated anomaly [7], course; intracranially, multiple discrete stenoses were noted mainly whereas the presence of patent ductus arteriosus is rather in the horizontal part of the ri ght middle cerebral artery and its common [8]. Dilatation of the ascending aorta and aneu­ angular branch (figs. 1 A- 1 C). The appearance of these stenoses rysms of the sinus of Valsalva combined with coarctation was consistent with th at of fibromuscular dysplasia or some other form of vasculopath y. A left carotid arteriogram with cross compres­ have also been reported [9]. Whenever the patient does not sion of the right common caroti d artery revealed a norm al ri ght suffer from Turner syndrome [10], anomalies involving or­ anteri or cerebral artery and norm al left carotid branches. gans other than the heart and the thoracic aorta are rarely Computed tomography (CT) of th e head demonstrated an area of seen in coarctation. However, aneurysms of the circle of increased uptake of contrast materi al in the ri ght hemisphere that Willis were noted by Hodes et al. [11] and are the cause of was consistent with an ischemi c infarct. A brain scan supported this cerebral hemorrhage, which is a major complication in the diagnosis. natural history of coarctation. Coarctation of the aorta in A mu scle bi opsy of the gastrocnemius muscle revealed no ab­ association with cerebral arterial occlusive disease, to our norm alities. The coarctation of the aorta was repaired leaving a 25 knowledge, has not been reported previously. We report mm Hg residual gradient. Hi stologic examination of aortic tissue was not made. two patients with such a combination. The question to be The patient underwent reassessment of his cerebral status 5 considered is whether this represents a fortuitous associa­ years later. Cerebral angiography revealed an occlusion of the ri ght tion or whether both entities have a common etiology. middle cerebral artery at its ori gin . Its branches filled distall y in a retrograde fashion mainly from the ri ght posteri or cerebral artery (figs. 1 E and 1 F). CT showed a well defined area of encephaloma­ Case Reports lacia on the right side in the middle cerebral artery distribution consistent with an old infarct (fig. 10). Case 1 A 4-year-old boy was admitted 2 weeks after ac ute onset of a left Case 2 hemiparesis. His father found him lying on the bathroom floor with what seemed to be choreoathetotic movements on the right, while A 2-year-old boy underwent cardiac catheteri zati on and angiog­ the left side of his body was limp. He was alert and cooperative with raphy, revealing coarctation of the aorta distal to th e left subclavian left hemiparesis and left facial paralysis. A grade III / VI harsh systolic artery. At age 5 years, he sustain ed a cerebrovascul ar accident murmur heard at the upper left sternal border was transmitted to resulting in left hemiparesis. He underwent surgical correction of the back and to both cflrotid arteri es. The pulses in the legs were his coarctation 1 month later. The procedure had to be repeated at This article appears in the September/ October 1982 issue of AJNR and the December 1982 issue of AJR. I Department of Radiology, University of Nebraska Medical Center, Omaha, NB 68 105. 2Present address: Department of Radiology, University of California, Davi s, UCD Professional Bldg., 4301 X St. , Sacramento, CA 95817 . Address reprint requests to H. A. Baltaxe. 3Department of Pediatric Cardiology, University of Nebraska Medical Center, Omaha, NB 68105. AJNR 3:577-580, September/ October 1982 0 195- 6 108 / 82/ 0305-0577 $00.00 © American Roentg en Ray Society 578 BALTA XE ET AL. AJNR:3, September/ October 1982 B c D E F Fig. 1.-Case 1. A, Right lateral common carotid arteriogram. Multiple angiogram. Almost complete obstruction at origin of the middle cerebral discrete linea r filling defects with mild poststenotic dilatations in angular artery (straight arrow). Distally there is earl y retrograde filling of distal middle branch of middle cerebral art ery. Anterior cerebral artery not visible. B, Local cerebral artery branch via anterior and posterior cerebral arteries (curved mag nified view. Angular branch with multiple defects (arrows). C, AP right arrows ). F, AP right carotid angiogram . Almost complete block at right middle carotid angiogram. Multiple discrete linear fi ll in g defects in middle cerebral cerebral artery origin with opacificati on onl y of ascending frontoparietal artery (arrows ). D, 5 years later. CT scan with intravenous contrast enhance­ branch. ment demonstrates old right frontal in farct. E, Right lateral common carotid Fig. 2.-Case 2. A, Left lateral com­ mon carotid angiogram. Almost com­ plete occlusion at supraclinoid part of internal carotid artery (arrowhead). Me­ dial posterior choroidal artery (straight arrow) and perforating arteries of pos­ terior cerebral artery show marked dila­ tation (curved arrow). B, Right vertebral angiogram. Prominent perforating ves­ sels within basal ganglia consistent with Moya Moya syndrome. A B AJNR:3, September I Octaber 1982 COARCTATION OF THE AORTA 579 age 9 years due to restenosis. Aortic tissue was not examined hypertension have been reported in neurofibromatosis histologically. In the interim he apparently sustained one or two [15]. The renal artery stenosis in neurofibromatosis is more cerebrovascular accidents. caused by the mesenchymal abnormali ty. Cerebrovascular At age 9 years the residual gradient across the site of his occlusive disease has also been described in neurofibro­ previously repaired coarctation was 30 mm Hg. Blood pressure in matosis [16]. Levisohn et al. [17] reported two such cases both arms was normal. A left common carotid angiogram revealed with brain infarction diagnosed by CT, caused by occlusive an almost complete occlusion at the level of the supraclinoid part of the internal carotid artery with dilatation of the perforating arterial intracranial disease with resulting extensive coll aterali za­ supply to the basal ganglia (fig. 2A). A right vertebral angiogram tion. Angiographically, the vessels presented with segmental revealed marked dilatation of the thalamoperforating arteries to the constrictions and areas of ectasia, similar to the cerebral basal ganglia producing an appearance consistent with a Moya arteries of our patients. Neurofibromatosis, however, is a Moya syndrome (fig. 2B). heredofamilial disorder with maldevelopment of the neural At age 14 years, he still had a left hemiparesis and appeared ectodermal tissue. Nothing suggested that our patients were somewhat mentally diminished. There appeared to be no progres­ afflicted by such a condition. However, it is possible that the sion of the neurologic deficit. abnormality that affects th e fibroelastic tissue in coarctati on may sometimes also involve th e intracerebral vessels. Coarctation of the aorta has been reported in association Discussion with aneurysms of the cerebral vessels [11]. The mechani sm Whenever several pathologic conditions coexist, it is im­ for the formation of these aneu rysms has never been eluci­ portant to determine whether their association is fortuitous dated. Long-standing hypertension restri cted to the upper or whether they have a common etiology. Because coarc­ body as seen in coarctation may produce changes in the tation of the aorta is a congenital disease, an initial assump­ cerebral vessels that eventually may result in the formation tion would be that the intracranial lesions in our patients are of aneurysms. Could hypertension have produced occlusive due to an inherent rather than an acquired defect. Never­ vascular disease in our patients? Even if long-standing theless, the possibility that the cerebral arterial occlusions hypertension would have been present, the cerebrovascular were secondary to the hypertension caused by coarctation changes noted in our patients could probably not have taken must be considered also. place without an underl ying disposition. The exact etiology of coarctation of the aorta is unknown. These two cases cannot be dismissed without suggesting In 1841, Craigie (cited in [2]) first proposed the " skodiac the possibility of a vasculitis. Ferri s and Levine [18] classi­ theory, " which stipulates that the aortic narrowing is sec­ fied cerebral arteritides according to their etiology. Bacte­ ondary to the closure of the ductus. Coarctation of the aorta, rial, viral, and collagen diseases must be considered when however, is often associated with a patent ductus, and , faced with intracranial vascular changes in children.
Load more

Recommended publications
  • Coarctation of the Aorta Interrupted Aortic Arch
    T HE P EDIATRIC C ARDIAC S URGERY I NQUEST R EPORT Coarctation of the aorta In the normal heart, blood flows to the body through the aorta, which connects to the left ven- tricle and arches over the top of the heart.In coarc- tation of the aorta,the aorta is pinched (in medical terms, coarcted) at a point somewhere along its length. This pinching restricts blood flow from the heart to the rest of the body. Often, the baby also has other heart defects, such as a narrowing of the aortic arch (in medical terms,transverse aortic arch hypoplasia). Usually no symptoms are apparent at birth, but can develop within a week of birth with the closure of the ductus arteriosus. The possible conse- Diagram 2.9 Coarctation of the aorta quences of this condition are poor feeding, an 1 – pinched or coarcted aorta enlarged heart, an enlarged liver and congestive Flow patterns are normal but are reduced below the coarctation. Blood pressure is increased in ves- heart failure. Blood pressure also increases above sels leaving the aorta above the coarctation. The the constriction. broken white arrow indicates diminished blood flow through the aorta. Timing of surgery depends on the degree of coarctation. Surgery may be delayed with mild coarctation (which sometimes is not detected until adoles- cence). However, if a baby develops congestive heart failure or high blood pressure, early surgery is usually required. A baby with severe coarctation should have early surgery to prevent long-term high blood pres- sure. The coarctation can be repaired in a number of ways without opening the heart.The surgeon can remove the narrowed part of the aorta and sew the ends together, thereby creating an anastomosis.
    [Show full text]
  • Thoracic Aorta
    GUIDELINES AND STANDARDS Multimodality Imaging of Diseases of the Thoracic Aorta in Adults: From the American Society of Echocardiography and the European Association of Cardiovascular Imaging Endorsed by the Society of Cardiovascular Computed Tomography and Society for Cardiovascular Magnetic Resonance Steven A. Goldstein, MD, Co-Chair, Arturo Evangelista, MD, FESC, Co-Chair, Suhny Abbara, MD, Andrew Arai, MD, Federico M. Asch, MD, FASE, Luigi P. Badano, MD, PhD, FESC, Michael A. Bolen, MD, Heidi M. Connolly, MD, Hug Cuellar-Calabria, MD, Martin Czerny, MD, Richard B. Devereux, MD, Raimund A. Erbel, MD, FASE, FESC, Rossella Fattori, MD, Eric M. Isselbacher, MD, Joseph M. Lindsay, MD, Marti McCulloch, MBA, RDCS, FASE, Hector I. Michelena, MD, FASE, Christoph A. Nienaber, MD, FESC, Jae K. Oh, MD, FASE, Mauro Pepi, MD, FESC, Allen J. Taylor, MD, Jonathan W. Weinsaft, MD, Jose Luis Zamorano, MD, FESC, FASE, Contributing Editors: Harry Dietz, MD, Kim Eagle, MD, John Elefteriades, MD, Guillaume Jondeau, MD, PhD, FESC, Herve Rousseau, MD, PhD, and Marc Schepens, MD, Washington, District of Columbia; Barcelona and Madrid, Spain; Dallas and Houston, Texas; Bethesda and Baltimore, Maryland; Padua, Pesaro, and Milan, Italy; Cleveland, Ohio; Rochester, Minnesota; Zurich, Switzerland; New York, New York; Essen and Rostock, Germany; Boston, Massachusetts; Ann Arbor, Michigan; New Haven, Connecticut; Paris and Toulouse, France; and Brugge, Belgium (J Am Soc Echocardiogr 2015;28:119-82.) TABLE OF CONTENTS Preamble 121 B. How to Measure the Aorta 124 I. Anatomy and Physiology of the Aorta 121 1. Interface, Definitions, and Timing of Aortic Measure- A. The Normal Aorta and Reference Values 121 ments 124 1.
    [Show full text]
  • Atypical Coarctation of Aorta
    Atypical coarctation of aorta Authors: Professor Harald Kaemmerer1 and Doctor Alfred Hager Creation Date: January 2003 Scientific Editor: Professor Marie-Christine Seghaye 1Department of pediatric cardiology, Deutsches Herzzentrum München, Lazarettstr. 36, 80636 München, Germany. [email protected] Abstract Keywords Disease name and synonyms Definition Frequency Clinical presentation Etiology Diagnostic methods Treatment References Abstract Atypical coarctation of aorta (CoA) is characterized by localized or extended narrowing of the ascending aorta, of the descending thoracic aorta at the level of the diaphragm, or of the abdominal aorta. Symptoms are attributed to upper body arterial hypertension and may include headache, abdominal angina, leg fatigue at exercise, cold feet and intermittent claudication. A pressure difference between arms and legs may exist as it is the case for the isthmic CoA. A systolic vascular murmur can be heard over the region of stenosis. This disease is often caused by an arteritis (Takayasu arteritis or aortitis) or a fibromuscular dysplasia. Some forms may be congenital. It can be associated with neurofibromatosis such as von Recklinghausen disease or Williams syndrome. This rare condition affects 0.5% to 2% of individuals with CoA. The symptomatic treatment consists of antihypertensive medication. The causal treatment is either percutaneous transluminal angioplasty with or without endovascular stent placement or surgery. Keywords Coarctation, aortic isthmus, atypical, ectopic, Takayasu arteritis, neurofibromatosis (Recklinghausen disease), Williams syndrome, rubella syndrome, tuberous sclerosis, Alagille syndrome. Disease name and synonyms Atypical CoA is characterized by a localized or • Atypical coarctation of the aorta extended narrowing of the ascending aorta, of • Coarctation of the abdominal aorta the descending thoracic aorta at the level of the • Middle aortic syndrome diaphragm or of the abdominal aorta.
    [Show full text]
  • Coarctation of the Aorta: from Fetal Life to Adulthood
    Cardiology Journal 2011, Vol. 18, No. 5, pp. 487–495 10.5603/CJ.2011.0003 Copyright © 2011 Via Medica REVIEW ARTICLE ISSN 1897–5593 Coarctation of the aorta: From fetal life to adulthood Damien Kenny, Ziyad M. Hijazi Rush Center for Congenital and Structural Heart Disease, Rush University Medical Center, Chicago, IL, USA Abstract Coarctation of the aorta was once viewed as a simple discrete narrowing of the aortic isthmus that could be ‘cured’ by surgical intervention. It is now clear that this condition may: (1) affect the aortic arch in a highly variable manner; (2) be associated with a host of other left sided heart lesions; (3) represent a wider vasculopathy within the pre-coarctation arterial tree, leading to significant prevalence of hypertension by adolescence, and subsequent risk of early morbidity and death. This review outlines the evaluation and treatment of this disease from pre-natal to adult life. (Cardiol J 2011; 18, 5: 487–495) Key words: surgery, echocardiography, stenting, hypertension, vasculopathy Introduction Coarctation of the aorta (CoA) is the fifth most common congenital heart defect, accounting for 6–8% of live births with congenital heart disease, with an estimated incidence of 1 in 2,500 births [1–3]. It is likely that the incidence is higher in stillborn babies [4]. It affects more male babies than female, with a reported ratio in males of between 1.27:1 and 1.74:1 [5, 6]. It usually manifests as a discrete con- striction of the aortic isthmus (Fig. 1). However, it is more likely to represent a spectrum of aortic nar- rowing from this discrete entity to tubular hypopla- sia, with many variations seen in between these two extremes.
    [Show full text]
  • The Aorta, and Pulmonary Blood Flow
    Br Heart J: first published as 10.1136/hrt.36.5.492 on 1 May 1974. Downloaded from British HeartJournal, I974, 36, 492-498. Relation between fetal flow patterns, coarctation of the aorta, and pulmonary blood flow Elliot A. Shinebourne and A. M. Elseed From the Department of Paediatrics, Brompton Hospital, National Heart and Chest Hospitals, London Intracardiac anomalies cause disturbances in fetal flow patterns which in turn influence dimensions of the great vessels. At birth the aortic isthmus, which receives 25 per cent of the combinedfetal ventricular output, is normally 25 to 30 per cent narrower than the descending aorta. A shelf-like indentation of the posterior aortic wall opposite the ductus characterizes thejunction of the isthmus with descending aorta. In tetralogy ofFallot, pulmonary atresia, and tricuspid atresia, when pulmonary blood flow is reduced from birth, the main pul- monary artery is decreased and ascending aorta increased in size. Conversely in intracardiac anomalies where blood is diverted away from the aorta to the pulmonary artery, isthmal narrowing or the posterior indentation may be exaggerated. Analysis of I62 patients with coarctation of the aorta showed 83 with an intracardiac anomaly resulting in increased pulmonary bloodflow and 21 with left-sided lesions present from birth. In contrast no patients with coarctation were found with diminished pulmonary flow or right-sided obstructive lesions. From this evidence the hypothesis is developed that coarctation is prevented whenflow in the main pulmonary artery is reduced in thefetus. http://heart.bmj.com/ The association of coarctation with left-sided establishing the complete intracardiac diagnosis in I62 obstructive lesions such as mitral and aortic stenosis patients with coarctation.
    [Show full text]
  • Aortic Coarctation and Interrupeted
    Aortic Coarctation and Interrupted Aortic Arch 25 Keila N. Lopez, Sebastian C. Tume, Stuart R. Hall, Aimee Liou, S. Kristen Sexson Tejtel, Carlos M. Mery Coarctation of the aorta (CoA), aortic arch hypoplasia, and interrupted aortic arch (IAA) are congenital conditions where a portion of the aorta (not including the aortic valve) measure small for BSA or a segment of the arch is atretic or missing. ese lesions can present in the newborn period, childhood, and more rarely, in the adult period. Timing of presentation is dependent on the severity of the lesion (degree and extent of arch narrowing) as well as the presence of a PDA and aortopulmonary collaterals. Arch lesions may also be accompanied by other left-sided obstructive lesions including an abnormal aortic valve, mitral valve, or LV. For multilevel left-heart hypoplasia, see Chapter . e aortic arch is usually divided into dierent segments (Figure -): • Proximal arch: between the innominate artery and left carotid artery • Distal arch: between the left carotid artery and the left subclavian artery • Isthmus: between the left subclavian artery and the ductus arteriosus or ligamen- tum arteriosum e dimensions of each of the aortic arch segments are important to dene the clin- ical presentation and management of patients with aortic arch hypoplasia. Focal CoA classically presents with signicant narrowing at the level of the isthmus. IAA is classied into types depending on the aortic arch segment involved with the interruption: • Type A: the interruption is distal to the left subclavian artery • Type B: the interruption is between the left common carotid artery and the left subclavian artery • Type C: the interruption is between the innominate artery and the left common carotid artery Pathophysiology and Clinical Presentation Fetal Mild aortic arch lesions are often dicult to detect in utero, and may only be detected postnatally, after the PDA closes.
    [Show full text]
  • Repaired Coarctation of the Aorta, Persistent Arterial Hypertension and the Selfish Brain Jonathan C
    Rodrigues et al. Journal of Cardiovascular Magnetic Resonance (2019) 21:68 https://doi.org/10.1186/s12968-019-0578-8 RESEARCH Open Access Repaired coarctation of the aorta, persistent arterial hypertension and the selfish brain Jonathan C. L. Rodrigues1,2,3*, Matthew F. R. Jaring4, Melissa C. Werndle4, Konstantina Mitrousi2, Stephen M. Lyen4, Angus K. Nightingale5, Mark C. K. Hamilton4, Stephanie L. Curtis6, Nathan E. Manghat4, Julian F. R. Paton2,5,7† and Emma C. Hart2,5*† Abstract Background: It has been estimated that 20–30% of repaired aortic coarctation (CoA) patients develop hypertension, with significant cardiovascular morbidity and mortality. Vertebral artery hypoplasia (VAH) with an incomplete posterior circle of Willis (ipCoW; VAH + ipCoW) is associated with increased cerebrovascular resistance before the onset of increased sympathetic nerve activity in borderline hypertensive humans, suggesting brainstem hypoperfusion may evoke hypertension to maintain cerebral blood flow: the “selfish brain” hypothesis. We now assess the “selfish brain” in hypertension post-CoA repair. Methods: Time-of-flight cardiovascular magnetic resonance angiography from 127 repaired CoA patients (34 ± 14 years, 61% male, systolic blood pressure (SBP) 138 ± 19 mmHg, diastolic blood pressure (DBP) 76 ± 11 mmHg) was compared with 33 normotensive controls (42 ± 14 years, 48% male, SBP 124 ± 10 mmHg, DBP 76 ± 8 mmHg). VAH was defined as < 2 mm and ipCoW as hypoplasia of one or both posterior communicating arteries. Results: VAH + ipCoW was more prevalent in repaired CoA than controls (odds ratio: 5.8 [1.6–20.8], p = 0.007), after controlling for age, sex and body mass index (BMI). VAH + ipCoW was an independent predictor of hypertension (odds ratio: 2.5 [1.2–5.2], p = 0.017), after controlling for age, gender and BMI.
    [Show full text]
  • Bicuspid Aortic Valve and Aortic Coarctation in Congenital Heart Disease—Important Aspects for Treatment with Focus on Aortic Vasculopathy
    788 Mini-Review Bicuspid aortic valve and aortic coarctation in congenital heart disease—important aspects for treatment with focus on aortic vasculopathy Christoph Sinning1,2#, Elvin Zengin1#, Rainer Kozlik-Feldmann3, Stefan Blankenberg1,2, Carsten Rickers3, Yskert von Kodolitsch1, Evaldas Girdauskas4 1Department of General and Interventional Cardiology, University Heart Center Hamburg, Hamburg, Germany; 2German Center of Cardiovascular Research (DZHK), Partner Site Hamburg/Kiel/Lübeck, Germany; 3Department of Pediatric Cardiology, 4Department of Cardiac and Cardiovascular Surgery, University Heart Center Hamburg, Hamburg, Germany Contributions: (I) Conception and design: C Sinning, E Zengin, Y von Kodolitsch, E Girdauskas; (II) Administrative support: None; (III) Provision of study materials or patients: None; (IV) Collection and assembly of data: C Sinning, E Zengin; (V) Data analysis and interpretation: C Sinning, E Zengin, Y von Kodolitsch, E Girdauskas; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors. #These authors contributed equally to this work. Correspondence to: Christoph Sinning, MD. Department of General and Interventional Cardiology, Universtiy Heart Center Hamburg, Martinistr. 52, 20246 Hamburg, Germany. Email: [email protected]. Abstract: Prevalence of congenital heart disease (CHD) is constantly increasing during the last decades in line with the treatment options for patients ranging from the surgical as well to the interventional spectrum. This mini-review addresses two of the most common defects with bicuspid aortic valve (BAV) and coarctation of the aorta (CoA). Both diseases are connected to aortic vasculopathy which is one of the most common reasons for morbidity and mortality in young patients with CHD. The review will focus as well on other aspects like medication and treatment of pregnant patients with BAV and CoA.
    [Show full text]
  • Coarctation of the Aorta What Is Coarctation of the Aorta? Mal Mitral Valve, and Coarctation
    HEART MATT ERS A PUBLICATION OF THE ADULT CONGENITAL HEART ASSOCIATION ACHA Q and A: Coarctation of the Aorta What is coarctation of the aorta? mal mitral valve, and coarctation. Your family members may People with coarctation of the aorta are born with an aorta want to get tested for heart defects. This usually involves an that is too narrow . The aorta is the longest blood vessel in the echocardiogram, which is an ultrasound picture of the heart . body . Blood is pumped from the left ventricle into the aorta. What other defects are common in coarctation? It then branches out into the right arm, head , and left arm . In 50-85% of coarctation patients also have a bicuspid aortic coarctation the aorta can be narrow in different places and in valve. This means the aortic valve has two flaps instead of different ways. Some people have a short narrow section. three. Some people with COA are also born with a hole in the Others have long areas of their aorta that are too narrow. If lower wall of the heart (ventricular septal defect). A smaller the aorta is very narrow, blood will not be able to pass. This number have problems with other heart valves such as the kind of coarctation is usually found at birth. If the narrowing mitral valve. People with coarctation are also at risk from is less severe, it may not be detected until later in life . swelling in their blood vessels (aneurysms). The causes and How common is coarctation? risks of aneurysms are discussed below.
    [Show full text]
  • Pathology and Molecular Mechanisms of Coarctation of the Aorta and Its Association with the Ductus Arteriosus
    J Physiol Sci (2017) 67:259–270 DOI 10.1007/s12576-016-0512-x REVIEW Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus 1 1 2 Utako Yokoyama • Yasuhiro Ichikawa • Susumu Minamisawa • Yoshihiro Ishikawa1 Received: 27 June 2016 / Accepted: 6 December 2016 / Published online: 20 December 2016 Ó The Physiological Society of Japan and Springer Japan 2016 Abstract Coarctation of the aorta (CoA) is defined as a Introduction congenital stenosis of the thoracic aorta and is one of the most common congenital cardiovascular diseases. Despite Early diagnosis and appropriate management of coarctation successful surgical treatment for CoA, arterial abnormali- of the aorta (CoA) have resulted in a low mortality rate ties, including refractory hypertension, aortic aneurysm, associated with the repair of CoA. It has been suggested and proatherogenic phenotypic changes, frequently affect that patients with CoA are at increased risk of myocardial patients’ quality of life. Emerging evidence from mor- infarction because systemic hypertension and premature phological and molecular biological investigations suggest atherosclerosis tend to develop early in life even after that the area of CoA is characterized by phenotypic mod- successful surgical treatment [1, 2]. The increased cardio- ulation of smooth muscle cells, intimal thickening, and vascular risk in this patient group is the result of both impaired elastic fiber formation. These changes extend to frequent relapses of hypertension and proatherogenic the pre-and post-stenotic aorta and impair arterial elastic- abnormalities that are present even in the absence of ity. The aim of this review is to present current findings on arterial hypertension [3, 4].
    [Show full text]
  • Moyamoya Disease and Aortic Coarctation in a Patient With
    Case Report Olgu Sunumu 85 Moyamoya disease and aortic coarctation in a patient with common brachiocephalic trunk Moyamoya hastal›¤› ve aort koarktasyonunun efllik etti¤i bir brakiyosefalik kütük olgusu Kadir Babao¤lu, Tevfik Demir*, Levent Salt›k*, fieyhan Kutlu¤**, Civan Ifllak*** Department of Pediatric Cardiology, Kocaeli University, Faculty of Medicine, Kocaeli, Turkey Departments of *Pediatric Cardiology, **Pediatrics and ***Radiology, Cerrahpafla Faculty of Medicine, Istanbul University, Istanbul, Turkey Introduction yond which severe stenosis of the both carotid arteries were seen. The network of collateral vessels was demonstrated at the dien- Moyamoya disease is characterized by a slowly progressive cephalic region (Fig. 2). A child had been treated with anticonvul- stenosis and obliteration of the large vessels at the base of the sant and subsequently had been advised to have physiotherapy. At brain, affecting mainly the supraclinoid segment of the internal ca- the last control, upper limb hypertension was established. The rotid artery and the initial portion of the anterior or middle cereb- systolic pressure gradient between the right upper and lower limbs ral arteries and the posterior cerebral arteries (1). Due to the slow was 30 mmHg. Echocardiography revealed a high systolic gradient progression of the disease and in response to progressive cereb- (45 mmHg) at the aortic isthmus. Cardiac catheterization and angi- ral ischemia, a large network of collateral vessels is formed from ography revealed CBT, coarctation of the aorta and stenosis at the the external carotid arteries, the vertebra-basilar system and ot- origin of left subclavian artery (Fig. 3). A cerebral angiography was her vessels (2). done at the same time and confirmed the diagnosis of Moyamoya The idiopathic or primary form of Moyamoya disease, which is disease.
    [Show full text]
  • Hypoplastic Aortic Arch, Coarctation of the Aorta and Poststenotic Aneurysm
    272 Türk Kardiyol Dern Arş - Arch Turk Soc Cardiol 2015;43(3):272-274 doi: 10.5543/tkda.2015.89084 A rare combination of vascular anomalies: Hypoplastic aortic arch, coarctation of the aorta and poststenotic aneurysm Nadir bir damar anomalisi kombinasyonu: Hipoplastik aort yayı, aort koarktasyonu ve poststenotik anevrizma Nermin Bayar, M.D., Şakir Arslan, M.D., Çağın Mustafa Üreyen, M.D., Selçuk Küçükseymen, M.D., Bekir Erol, M.D.# Department of Cardiology, Antalya Training and Research Hospital, Antalya #Department of Radiology, Antalya Training and Research Hospital, Antalya Summary– Coarctation of the aorta is the fifth most com- Özet– Aort koarktasyonu erişkinlerde beşinci sıklıkta görü- mon congenital cardiac anomaly encountered in adults. It len doğumsal kalp anomalisidir. Prognoz için erken tanı ve is important for prognosis to diagnose and treat this anom- tedavi önemlidir. Aort koarktasyonu bulunan olgularda tu- aly early. An aneurysm might develop due to tunica media nika medya anormallikleri anevrizma gelişimine neden ola- abnormalities in patients with coarctation of the aorta. We bilmektedir. Bu yazıda, çıkan aort anevrizması, hipoplastik hereby present an adult case with a very rare combination aort yayı, aort koarktasyonu ve poststenotik anevrizmayı of vascular anomalies including ascending aorta aneurysm, içeren çok nadir damar anomalisi birlikteliği olan erişkin bir hypoplastic aortic arch, coarctation of the aorta and postste- hasta sunuldu. notic aneurysm. oarctation of the aorta constitutes 5-8% of all pressure was regulated. Abbreviations: congenital cardiac anomalies.[1] Moreover, bi- Physical examination re- C 3D Three-dimensional cuspid aortic valve and ventricular septal defect may vealed a II/IV diastolic CT Computed tomography accompany coarctation of the aorta.[2] An aortic aneu- murmur in the meso- TTE Transthoracic echocardiograhy rysm may also develop owing to medial abnormalities cardiac area.
    [Show full text]