Conditions resulting from abnormalities in the arteries branching from the ASCENDING AORTA, the curved portion of the aorta. These syndromes are results of occlusion or abnormal blood flow to the head-neck or arm region leading to neurological defects and weakness in an arm. These syndromes are associated with vascular malformations; ATHEROSCLEROSIS; TRAUMA; and blood clots.
An unnaturally deep or rough quality of voice.
Congenital or acquired paralysis of one or both VOCAL CORDS. This condition is caused by defects in the CENTRAL NERVOUS SYSTEM, the VAGUS NERVE and branches of LARYNGEAL NERVES. Common symptoms are VOICE DISORDERS including HOARSENESS or APHONIA.
Branches of the vagus (tenth cranial) nerve. The recurrent laryngeal nerves originate more caudally than the superior laryngeal nerves and follow different paths on the right and left sides. They carry efferents to all muscles of the larynx except the cricothyroid and carry sensory and autonomic fibers to the laryngeal, pharyngeal, tracheal, and cardiac regions.
Artery arising from the brachiocephalic trunk on the right side and from the arch of the aorta on the left side. It distributes to the neck, thoracic wall, spinal cord, brain, meninges, and upper limb.
Traumatic injuries to the RECURRENT LARYNGEAL NERVE that may result in vocal cord dysfunction.
Hereditary nonpolyposis colorectal neoplasms associated with other malignancies, more commonly of ovarian or uterine origin. When also associated with SEBACEOUS GLAND NEOPLASMS, it is called MUIR-TORRE SYNDROME.
Branches of the VAGUS NERVE. The superior laryngeal nerves originate near the nodose ganglion and separate into external branches, which supply motor fibers to the cricothyroid muscles, and internal branches, which carry sensory fibers. The RECURRENT LARYNGEAL NERVE originates more caudally and carries efferents to all muscles of the larynx except the cricothyroid. The laryngeal nerves and their various branches also carry sensory and autonomic fibers to the laryngeal, pharyngeal, tracheal, and cardiac regions.

Surgical treatment of nonaneurysmal aortic arch lesions in patients with systemic embolization. (1/58)

PURPOSE: Atherosclerotic lesions of the aortic arch are potential sources of arterial embolism. Here we investigate whether surgery, with the necessary circulatory supports, can be proposed as a good option for treatment of this problem. Study of these lesions on a national scale in France has made possible the assessment for future indications of techniques and results of the surgical management of aortic arch lesions, which retrospectively proved to be embolic. METHODS: Thirty-eight patients, (19 men and 19 women) underwent surgery between 1976 and 1996 in 17 French cardiovascular surgical centers. The average age at the time of surgery was 49 +/- 12 years (range, 31 to 82 years). Atherosclerotic lesions were detected with transesophagial echocardiography (n = 19), angiography of the aortic arch (n = 16), computed tomography (n = 9), and magnetic resonance imaging (n = 10). Surgery consisted of thrombectomy and endarterectomy (n = 22), aortic resection and graft replacement (n = 10), and patch aortoplasty (n = 5; one thrombus disappeared spontaneously before surgery was performed). RESULTS: The average postoperative period was 30 months (range, 3 to 82 months). Contact was lost with four patients after a follow-up period of 12 months. On pathologic specimens obtained at surgery, an atherosclerotic plaque was found in 73% of the cases (n = 28). In 15% of the cases, the aorta appeared normal (n = 6) and four other types of lesion were identified: angiosarcoma (n = 1), ectasia at the insertion of the remains of the ductus arteriosus (n = 1), rupture of tunica intima (n = 1), and a fibroblastic plaque (n = 1). A thrombus was identified in 26 cases, attached to the arterial wall in 18 cases. When transesophagial echocardiographic results showed mobile lesions (n = 22), histopathologic examination of specimens allowed the detection of a thrombus in 18 cases and an atherosclerotic plaque with a mobile projection in four cases. The postoperative mortality rate was 2.6%. The morbidity rate (28.9%; n = 11) was related to neurologic complications (n = 6), vascular complications (n = 4), and infection (n = 1). Four cases (12%) were reoperated. CONCLUSION: Nonaneurysmal aortic arch lesions are a frequent and still underestimated source of stroke and peripheral embolization. Surgery with circulatory support can be recommended in good operative candidates with recurrent critical events despite medical management and with high embolic potential (young patients with no calcified plaques).  (+info)

PARADOXES OF TAKAYASU'S DISEASE. (2/58)

Takayasu's disease (or arteritis) has been defined as an "idiopathic aortitis usually affecting young women." It can come to light from very spectacular and often quite puzzling clinical manifestations. Six cases of Takayasu's disease were investigated at the UCLA Hospital in the years 1961-1962, and signs and symptoms of central nervous system involvement were found in five of the patients. This relatively high incidence of neurological deficit prompted a review of case reports in the literature and this in turn led to a series of "unexpected" findings in the historical evolution of the illness as well as in its anatomopathological aspects. The study indicated that Takayasu's disease is frequently associated with neurological manifestations, at times very severe. In addition, the disease appears to be far more extensive than its classical description suggests. New criteria for the diagnosis of Takayasu's disease must include, among other things, special emphasis on the disseminated nature of the disease.  (+info)

Common variable immunodeficiency syndrome with right aortic arch: a case report. (3/58)

BACKGROUND: Common variable immunodeficiency syndrome predominantly affects adults. It is characterized by low production of all the major classes of immunoglobulins. We report a case of common variable immunodeficiency syndrome with right aortic arch. An association of right-sided arch and common variable immunodeficiency syndrome has not been previously reported. CASE PRESENTATION: A 41-year-old female patient presented with a history of recurrent pneumonia, sinusitis, otitis media, diarrhoea, cystitis since childhood. Biochemical and immunocytochemical analysis revealed common variable immunodeficiency syndrome and radiological evaluation confirmed right aortic arch and aberrant left subclavian artery. CONCLUSION: Common variable immunodeficiency syndrome syndrome is a clinical entity that should be kept in mind in patients with recurrent infections of different sites.  (+info)

Subclavian steal in Takayasu's arteritis. A hemodynamic study by means of ultrasonic Doppler flowmetry. (4/58)

Blood flow in the vertebral artery and the upper extremity was studied in five cases of Takayasu's arteritis with subclavian steal by use of ultrasonic Doppler flowmetry and finger plethysmography. The diagnosis of subclavian steal was made by observation of flow reversal in the vertebral artery on the subclavian steal side during grip exercise and, in addition, the vertebral flow change with brachial artery occlusion. The blood flow increase of both internal cartotid and non-affected (non-subclavian steal side) vertebral arteries during a common carotid compression was almost normal in patients with Takayasu's arteritis in this study. During carotid compression on the side of the subclavian steal, ipsilateral vertebral blood flow greatly decreased, and the amplitude the ipsilateral finger plethysmogram decreased slightly or moderately. It is suggested that there are significantly important factors in suppressing sumptoms of vertebrobasilar ischemia in these patients with Takayasu's arteritis with subclavian steal. These factors are believed to be (1) good function of the circle of Willis, (2) good blood supply to the brain stem, and (3) collateral circulation to the distal subclavian artery not via the vertebral artery.  (+info)

Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. (5/58)

BACKGROUND: Turner syndrome (TS) is associated with aortic coarctation and dissection; hence, echocardiographic evaluation of all patients is currently recommended. X-ray angiography in clinically symptomatic patients has suggested a range of other vascular anomalies, but the true prevalence of such lesions in TS is unknown. To better understand the prevalence and pathogenesis of cardiovascular defects in TS, we prospectively evaluated a group of asymptomatic adult volunteers with TS using magnetic resonance (MR) angiography. METHODS AND RESULTS: A total of 85 adults with TS and 27 normal female adult volunteers underwent gadolinium-enhanced 3D MR angiography. A high prevalence of aortic anomalies was seen in women with TS, including elongation of the transverse arch (49%), aortic coarctation (12%), and aberrant right subclavian artery (8%). Venous anomalies were also prominent, including persistent left superior vena cava (13%) and partial anomalous pulmonary venous return (13%). None of these anomalies were found in healthy female controls. The constellation of elongation of the transverse arch, aortic coarctation, and persistent left superior vena cava was significantly associated with women with TS. Neck webbing and increased thoracic anterior-to-posterior dimension diameters were strong predictors for arterial and venous anomalies. CONCLUSIONS: Thoracic vascular anomalies are common in TS, occurring in approximately 50% of a group not preselected for cardiovascular disease. The highly significant association between neck webbing, increased chest diameter, and these vascular anomalies suggests that in utero, centrally localized lymphatic obstruction may contribute to these cardiovascular deformities in TS. Improved recognition of these often-undetected vascular lesions may be important for identification of patients in need of closer cardiovascular monitoring.  (+info)

Aortic root replacement with a freestyle stentless valve for aortitis syndrome with ascending aortic aneurysm and aortic regurgitation. (6/58)

A 47-year-old woman who had been diagnosed as having aortitis syndrome underwent aortic root replacement for an ascending aortic aneurysm and aortic regurgitation. Because the patient has been treated with steroids for more than 20 years, a Freestyle stentless valve was used to avoid the risk of valve detachment. There were no complications observed during the postoperative course. Although long-term follow-up will be necessary to observe the valve durability, the Freestyle stentless valve seems to be useful for aortic root replacement in patients at high risk of valve detachment due to aortitis syndrome.  (+info)

Protruding aortic arch thrombus: treatment with minimally invasive surgical approach. (7/58)

BACKGROUND: Protruding aortic arch thrombus is associated clinically with life-threatening emboli. Definitive treatment for aortic arch thrombus removal has demanded complicated vascular surgical procedures, with high morbidity and mortality. METHODS AND RESULTS: Transesophageal echocardiography (TEE) enabled diagnosis of a protruding thrombus at the aortic arch in 5 patients, and a simultaneous lesion in the descending aorta in 1 patient. Four patients had visceral emboli, coinciding with peripheral emboli in 2 patients, and the fifth patient had peripheral and cerebral emboli. One patient had had ischemic stroke and femoral emboli a few months previously. Mean patient age was 51 years. None had clinical evidence of coronary or peripheral atherosclerotic occlusive disease. Risk factors included hypertension (n = 2), smoking (n = 4), and preexisting thrombophilia (n = 4). Five patients underwent TEE-guided aortic balloon thrombectomy from the arch with a 34-mm occluding balloon catheter. One patient also underwent balloon thrombectomy from the descending aorta with a 14F Foley catheter. Access into the aorta was obtained through the iliac artery (n = 4) during laparotomy because of visceral ischemia or through the transfemoral approach (n = 2). Previous procedures included superior mesenteric embolectomy (n = 3), segmental bowel resection (n = 1), splenectomy (n = 1), and peripheral arterial embolectomy n = 3). Real-time intraoperative TEE enabled visualization of the protruding thrombus and assisted with maneuvering of the balloon catheter. At completion peripheral thrombectomy thrombus material was retrieved in 4 patients. Postoperatively there were no clinically proved new procedure-related visceral emboli, and all patients received anticoagulant therapy thereafter. Follow-up TEE within 2 weeks and up to 7 years revealed no recurrent aortic arch thrombus. CONCLUSIONS: TEE-guided aortic balloon thrombectomy used in 6 procedures was effectively completed without visceral or peripheral ischemic complications. It enabled removal of the life-threatening source of emboli from the proximal aorta, thereby averting the need of major aortic surgery.  (+info)

Unusual vascular ring anomaly in a foal. (8/58)

A 2.5-month-old filly was presented with signs of esophageal obstruction. The filly was euthanized and postmortem examination revealed a vascular ring anomaly. The vascular ring anomaly was not caused by a persistent right aortic arch, which is the only vascular ring anomaly reported to occur in horses.  (+info)

Aortic arch syndromes are a group of conditions that affect the aortic arch, which is the curved portion of the aorta that arises from the left ventricle of the heart and gives rise to the major branches of the arterial system. These syndromes are typically caused by congenital abnormalities or degenerative changes in the aorta and can result in various complications, such as obstruction of blood flow, aneurysm formation, and dissection.

There are several types of aortic arch syndromes, including:

1. Coarctation of the Aorta: This is a narrowing of the aorta at the point where it leaves the heart, just distal to the origin of the left subclavian artery. It can cause hypertension in the upper extremities and reduced blood flow to the lower extremities.
2. Aortic Arch Aneurysm: This is a localized dilation or bulging of the aorta in the region of the aortic arch. It can lead to dissection, rupture, or embolism.
3. Aortic Arch Dissection: This is a separation of the layers of the aortic wall, which can result from hypertension, trauma, or genetic disorders such as Marfan syndrome. It can cause severe chest pain, shortness of breath, and shock.
4. Kommerell's Diverticulum: This is an outpouching or bulge in the aorta at the origin of the ligamentum arteriosum, which is a remnant of the ductus arteriosus. It can cause compression of the airways or esophagus and increase the risk of dissection or rupture.
5. Abernethy Malformation: This is a rare congenital anomaly in which there is an abnormal connection between the portal vein and systemic venous circulation, leading to the bypass of the liver. It can cause various complications such as hepatic encephalopathy, pulmonary hypertension, and liver tumors.

The diagnosis and management of aortic arch syndromes require a multidisciplinary approach involving cardiologists, radiologists, surgeons, and other specialists. Treatment options may include medications, endovascular procedures, or surgical interventions depending on the severity and location of the lesion.

Hoarseness is a condition characterized by an abnormal change in the quality of voice, making it sound rough, breathy, strained, or weak. Medically, it's described as a disorder of phonation, which is the process of producing sound by vibrating the vocal cords in the larynx (voice box). Hoarseness can be caused by various factors, such as inflammation, irritation, or injury to the vocal cords, and may result in symptoms like altered voice pitch, volume, and clarity. It's essential to consult a healthcare professional if hoarseness persists for more than two weeks, especially if it's accompanied by other concerning symptoms like difficulty swallowing or breathing.

Vocal cord paralysis is a medical condition characterized by the inability of one or both vocal cords to move or function properly due to nerve damage or disruption. The vocal cords are two bands of muscle located in the larynx (voice box) that vibrate to produce sound during speech, singing, and breathing. When the nerves that control the vocal cord movements are damaged or not functioning correctly, the vocal cords may become paralyzed or weakened, leading to voice changes, breathing difficulties, and other symptoms.

The causes of vocal cord paralysis can vary, including neurological disorders, trauma, tumors, surgery, or infections. The diagnosis typically involves a physical examination, including a laryngoscopy, to assess the movement and function of the vocal cords. Treatment options may include voice therapy, surgical procedures, or other interventions to improve voice quality and breathing functions.

The Recurrent Laryngeal Nerve (RLN) is a branch of the vagus nerve (cranial nerve X), which is a mixed sensory, motor, and autonomic nerve. The RLN has important functions in providing motor innervation to the intrinsic muscles of the larynx, except for the cricothyroid muscle, which is supplied by the external branch of the superior laryngeal nerve.

The recurrent laryngeal nerve supplies all the muscles that are responsible for adduction (bringing together) of the vocal cords, including the vocalis muscle, lateral cricoarytenoid, thyroarytenoid, and interarytenoid muscles. These muscles play a crucial role in voice production, coughing, and swallowing.

The right recurrent laryngeal nerve has a longer course than the left one. It loops around the subclavian artery in the chest before ascending to the larynx, while the left RLN hooks around the arch of the aorta. This anatomical course makes them vulnerable to injury during various surgical procedures, such as thyroidectomy and neck dissection, leading to potential voice impairment or vocal cord paralysis.

The subclavian artery is a major blood vessel that supplies the upper limb and important structures in the neck and head. It arises from the brachiocephalic trunk (in the case of the right subclavian artery) or directly from the aortic arch (in the case of the left subclavian artery).

The subclavian artery has several branches, including:

1. The vertebral artery, which supplies blood to the brainstem and cerebellum.
2. The internal thoracic artery (also known as the mammary artery), which supplies blood to the chest wall, breast, and anterior mediastinum.
3. The thyrocervical trunk, which gives rise to several branches that supply the neck, including the inferior thyroid artery, the suprascapular artery, and the transverse cervical artery.
4. The costocervical trunk, which supplies blood to the neck and upper back, including the posterior chest wall and the lower neck muscles.

The subclavian artery is a critical vessel in maintaining adequate blood flow to the upper limb, and any blockage or damage to this vessel can lead to significant morbidity, including arm pain, numbness, weakness, or even loss of function.

Recurrent laryngeal nerve injuries refer to damages or trauma inflicted on the recurrent laryngeal nerve, which is a branch of the vagus nerve that supplies motor function to the intrinsic muscles of the larynx, except for the cricothyroid muscle. This nerve plays a crucial role in controlling vocal fold movement and swallowing.

Injuries to this nerve can result in voice changes, hoarseness, or even complete loss of voice, depending on the severity and location of the injury. Additionally, it may also lead to breathing difficulties, coughing, and choking while swallowing due to impaired laryngeal function.

Recurrent laryngeal nerve injuries can occur due to various reasons, such as surgical complications (particularly during thyroid or neck surgeries), tumors, infections, inflammation, or direct trauma to the neck region. In some cases, these injuries may be temporary and resolve on their own or through appropriate treatment; however, severe or prolonged injuries might require medical intervention, including possible surgical repair.

Lynch Syndrome II is a genetic disorder also known as Hereditary Non-Polyposis Colorectal Cancer (HNPCC) type II. It is characterized by an increased risk of developing certain types of cancer, including colorectal, endometrial, stomach, small intestine, pancreas, kidney, urinary tract, brain, and skin cancers.

Unlike Lynch Syndrome I (HNPCC type I), which primarily involves mutations in the MLH1 or PMS2 genes, Lynch Syndrome II is caused by mutations in the MSH2 or MSH6 genes. These genes are responsible for DNA mismatch repair, and their malfunction leads to an accumulation of errors during DNA replication, increasing the risk of cancer development.

Individuals with Lynch Syndrome II have a higher lifetime risk of colorectal cancer (up to 80%) and endometrial cancer (up to 60%). The onset of these cancers tends to occur at an earlier age compared to sporadic cases. It is essential for individuals with Lynch Syndrome II to undergo regular cancer screening, including colonoscopies and gynecological examinations, to facilitate early detection and treatment of potential malignancies.

In addition to increased cancer risks, individuals with Lynch Syndrome II may also experience other clinical features such as café-au-lait spots or sebaceous gland tumors. Genetic counseling and testing are recommended for individuals with a family history suggestive of Lynch Syndrome II to assess their risk and develop appropriate surveillance strategies.

The laryngeal nerves are a pair of nerves that originate from the vagus nerve (cranial nerve X) and provide motor and sensory innervation to the larynx. There are two branches of the laryngeal nerves: the superior laryngeal nerve and the recurrent laryngeal nerve.

The superior laryngeal nerve has two branches: the external branch, which provides motor innervation to the cricothyroid muscle and sensation to the mucous membrane of the laryngeal vestibule; and the internal branch, which provides sensory innervation to the mucous membrane of the laryngeal vestibule.

The recurrent laryngeal nerve provides motor innervation to all the intrinsic muscles of the larynx, except for the cricothyroid muscle, and sensation to the mucous membrane below the vocal folds. The right recurrent laryngeal nerve has a longer course than the left one, as it hooks around the subclavian artery before ascending to the larynx.

Damage to the laryngeal nerves can result in voice changes, difficulty swallowing, and respiratory distress.

Cardiovocal syndrome caused by aortic arch pseudoaneurysm". Journal of the Saudi Heart Association. 28 (4): 266-269. doi: ... Escribano JF, Carnès J, Crespo MA, Antûn RF (January 2006). "Ortner's Syndrome and Endoluminal Treatment of a Thoracic Aortic ... A second Ortner's syndrome, Ortner's syndrome II, refers to abdominal angina. Due to its low frequency of occurrence, more ... Aortic dissection More commonly affects the right recurrent laryngeal nerve as the most common type of aortic dissection is ...
... (especially Type B) is often associated with DiGeorge syndrome. Patients with an interrupted aortic ... They are: Type A: The aortic arch is interrupted after the left subclavian artery. Type B: The aortic arch is interrupted ... Chin, Alvin J (Oct 2, 2007). "Interrupted Aortic Arch". eMedicine. Retrieved May 27, 2009. "Interrupted Aortic Arch , Types, ... an interrupted aortic arch can be associated with an intracranial aneurysm. Signs of ischemia due to interrupted aortic arch ...
Aortic arch anomaly peculiar facies intellectual disability syndrome". www.orpha.net. Retrieved 2022-07-16. "Aortic arch ... Strong, W. B. (December 1968). "Familial syndrome of right-sided aortic arch, mental deficiency, and facial dysmorphism". The ... Rare genetic syndromes, Syndromes affecting the aorta, Syndromes with intellectual disability, Genetic anomalies). ... "OMIM Entry - 107500 - AORTIC ARCH ANOMALY WITH PECULIAR FACIES AND MENTAL RETARDATION". www.omim.org. Retrieved 2022-05-21. ( ...
... (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of ... Milan B, Josip K (November 1967). "Ocular manifestations of the aortic arch syndrome (pulseless disease; Takayasu's disease) ( ... In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than Takayasu's ... Fainting may result from subclavian steal syndrome or carotid sinus hypersensitivity. There is also often anemia and marked ...
"Tbx1 haploinsufficieny in the DiGeorge syndrome region causes aortic arch defects in mice". Nature. 410 (6824): 97-101. doi: ... Shprintzen syndrome, DiGeorge sequence/syndrome, Sedlackova syndrome, and conotruncal anomaly face syndrome. All are now ... DiGeorge syndrome, also known as 22q11.2 deletion syndrome, is a syndrome caused by a microdeletion on the long arm of ... "DiGeorge syndrome (22q11.2 deletion syndrome)". Mayo Clinic. Retrieved 22 May 2020. "DiGeorge (22q11.2 deletion) syndrome: ...
March 2001). "Tbx1 haploinsufficieny in the DiGeorge syndrome region causes aortic arch defects in mice". Nature. 410 (6824): ... TBX1 is thought to operate on the same developmental pathway as CHD7 which can be mutated in CHARGE syndrome. Most cases of ... Jerome LA, Papaioannou VE (March 2001). "DiGeorge syndrome phenotype in mice mutant for the T-box gene, Tbx1". Nature Genetics ... Baldini A (October 2003). "DiGeorge's syndrome: a gene at last". Lancet. 362 (9393): 1342-1343. doi:10.1016/S0140-6736(03)14671 ...
Aortic arch anomalies and the pathogenesis of the DiGeorge syndrome". The American Journal of Pathology. 118 (2): 179-193. PMC ...
Patients with PHACE syndrome may also experience symptoms such as: Anomalies of the aortic arch. Narrowing, abnormal growth, or ... Cardiac defects, aortic coarctation and other aortic abnormalities. Eye anomalies. Sometimes an "S" is added to PHACE making ... "PHACE Syndrome Community". Drolet, Beth. "PHACE Syndrome handbook". Children's Hospital of Wisconsin. Archived from the ... "PHACE syndrome". Children's Hospital of Wisconsin. Retrieved 2018-10-25. Lanes, Marsha. "PHACE Syndrome". NORD (National ...
Variable defects in blood vessels have been described, including hypoplastic aortic arch, middle cerebral artery, pulmonary ... Rare genetic syndromes, Syndromes affecting the nervous system, Genetic disorders with OMIM but no gene, Syndromes affecting ... Adams-Oliver syndrome (AOS) is a rare congenital disorder characterized by defects of the scalp and cranium (cutis aplasia ... "Orphanet: Adams Oliver syndrome". www.orpha.net. Retrieved 16 May 2019. Mašek, Jan; Andersson, Emma R. (2017-05-15). "The ...
It has also been found in association with other genetic syndromes such as Trisomy 21 (Down syndrome).[citation needed] During ... the aortic arch is formed by the left fourth aortic arch and the left dorsal aorta. In people with a right-sided aortic arch, ... a right-sided aortic arch is visualized on chest radiography, by the aortic knob (the prominent shadow of the aortic arch) that ... Right-sided aortic arch is a rare anatomical variant in which the aortic arch is on the right side rather than on the left. ...
These defects are common in DiGeorge syndrome and include interrupted aortic arch and LVOT obstruction (IAA/LVOTO); aortic ... and aortic atresia with interrupted aortic arch and aortopulmonary window. This procedure allows the surgeon to keep the left ... "Primary repair of interrupted aortic arch and severe aortic stenosis in neonates". The Journal of Thoracic and Cardiovascular ... Tchervenkov, Christo I.; Jacobs, Jeffrey P.; Sharma, Kapil; Ungerleider, Ross M. (2005). "Interrupted aortic arch: surgical ...
However, the association with chromosome 22q11 deletion (DiGeorge Syndrome) implies that a genetic component is likely in ... Double aortic arch with right dominant arch: Normally there is only one (left) aortic arch. In patients with double aortic arch ... Double aortic arch is a subtype of complete vascular ring. There are three types of double aortic arch: Right dominant arch ( ... Double aortic arch with left dominant arch: In this less common condition, as the name indicates, the left arch is the larger ...
The proximal pulmonary artery is connected to the aortic arch, while the narrowed segment of the pulmonary trunk is repaired. ... Sharma J, Friedman D, Schiller M, Flynn P, Alonso ML (December 1997). "Aortic stenosis in hypoplastic right heart syndrome, ... Hypoplastic right heart syndrome is both less common and less severe than hypoplastic left heart syndrome. Within the United ... It can be associated with aortic stenosis. "Hypoplastic Right Heart Syndrome (HRHS): Diagnosis & Treatment , SSM Health". ...
... arch a series of bony "loops" present in fish, which support the gills. Branchial artery, also known as aortic arches ... First and second branchial arch syndrome, also known as hemifacial microsomia. Ultimo-branchial bodies, also known as ...
... one of arteries originating off of the aortic arch. Subclavian steal syndrome has potential to affect flow in the circle of ... In subclavian steal syndrome, blood is "stolen" from the vertebral artery on the affected side to preserve blood flow to the ... Subclavian steal syndrome results from a proximal stenosis (narrowing) of the subclavian artery, ... "Contribution of the Circle of Willis to the Subclavian Steal Syndrome". Circulation. 40 (6): 871-878. doi:10.1161/01.cir.40.6. ...
Placing the balloon too distal from the aortic arch may induce occlusion of the renal artery and subsequent kidney failure. ... Since the device is placed in the femoral artery and aorta it could provoke ischemia, and compartment syndrome. The leg is at ... The following conditions will always exclude patients for treatment: Severe aortic valve insufficiency Aortic dissection Severe ... "Intra-Aortic Balloon Pump Overview". 5 January 2019. Usage of Intra-Aortic Balloon Pump in High Risk Coronary Artery Bypass ...
... and measure inflammatory aortic arch syndrome. The results from the study were that MRI and FDG-PET were unreliable techniques ... Inflammatory aortic aneurysm (IAA), also known as Inflammatory abdominal aortic aneurysm (IAAA), is a type of abdominal aortic ... "MRI and FDG-PET in the assessment of inflammatory aortic arch syndrome in complicated courses of giant cell arteritis". Ann. ... The rupture of an aortic aneurysm is a catastrophic, life-threatening event. Aortic aneurysms are often encountered during an X ...
This fibrous arch forms the anterior aspect of the aortic hiatus, through which the aorta, thoracic duct, and azygos vein pass ... It has also been called Harjola-Marable syndrome and Marable syndrome. Nutcracker syndrome Superior mesenteric artery syndrome ... also known as celiac artery compression syndrome, celiac axis syndrome, celiac trunk compression syndrome or Dunbar syndrome) ... Median arcuate ligament syndrome (celiac artery compression syndrome)". Radiographics. 13 (6): 1400-2. doi:10.1148/ ...
In the normal embryological development of the aortic arches, the right dorsal aorta regresses caudal to the origin of the 7th ... Aberrant subclavian artery, or aberrant subclavian artery syndrome, is a rare anatomical variant of the origin of the right or ... This abnormality is the most common congenital vascular anomaly of the aortic arch, occurring in approximately 1% of ... Chaoui, R; Rake, A; Heling, KS (2008). "Aortic arch with four vessels: aberrant right subclavian artery". Ultrasound in ...
Other outcomes of aortic arch artery anomalies includes a double aortic arch, variable absence of the carotid arteries and left ... Frank D. U. et al "FGF8 mouse mutant phenocopies human 22q11 deletion syndrome." Development October 2002, 129(19) p4591 - 4603 ... The group of cells found in the third aortic arch gives rise to common carotid arteries. Cells found in the fourth aortic arch ... There is a reduction in the quantity of endothelial tubes of ectomesenchyme in pharyngeal arches that surround the aortic arch ...
... syndrome Antley-Bixler syndrome Anton-Babinski syndrome Aortic arch syndrome Aortocaval compression syndrome Apert syndrome ... syndrome Wende-Bauckus syndrome Werner syndrome Wernicke-Korsakoff syndrome West syndrome Westerhof syndrome Wet lung syndrome ... syndrome Activation syndrome Acute aortic syndrome Acute brain syndrome Acute chest syndrome Acute coronary syndrome Acute HME ... syndrome Fetal warfarin syndrome FG syndrome Fibrinolysis syndrome Fibromyalgia syndrome Fibromyalgia First arch syndrome Fish ...
... including septal defects and aortic arch anomalies, renal cysts, urinary tract malformations, and distal limb hypoplasia. Most ... Syndromes affecting the nervous system, Syndromes affecting the respiratory system, Rare genetic syndromes). ... noted the phenotypic overlap between Fryns syndrome and the Pallister-Killian syndrome (601803), which is a dysmorphic syndrome ... a new lethal syndrome". J Genet Hum. 28 (1): 57-60. PMID 7400786. Fitch N (February 1988). "Fryns syndrome". J. Med. Genet. 25 ...
October 2016). "Ortner's syndrome: Cardiovocal syndrome caused by aortic arch pseudoaneurysm". Journal of the Saudi Heart ... January 2006). "Ortner's Syndrome and Endoluminal Treatment of a Thoracic Aortic Aneurysm: A Case Report". Vascular and ... M. Hirata et al, (2018) Cardiovocal syndrome (Ortner syndrome) associated with secondary pulmonary arterial hypertension in a ... the definition has expanded to include aneurysms of the aortic arch, pulmonary hypertension due to mixed connective tissue ...
... syndrome 747 Other congenital anomalies of circulatory system 747.1 Coarctation of aorta 747.11 Interruption of aortic arch ... Down syndrome 758.1 Patau's syndrome 758.2 Edward's syndrome 758.3 Autosomal deletion syndromes 758.31 Cri du chat syndrome ... syndrome XO syndrome 758.7 Klinefelter syndrome 758.8 Other conditions due to sex chromosome anomalies Snyder-Robinson syndrome ... 759.8 Other specified congenital anomalies 759.81 Prader-Willi syndrome 759.82 Marfan syndrome 759.83 Fragile X syndrome 759.89 ...
Tricuspid atresia Interrupted aortic arch Pulmonary atresia (PA) Pulmonary stenosis (critical) Eisenmenger syndrome (reversal ... Tetralogy of Fallot (ToF) Total anomalous pulmonary venous connection Hypoplastic left heart syndrome (HLHS) Transposition of ...
... such as aortic outflow tract obstruction, mitral stenosis, aortic arch syndrome) etc. A bounding pulse signifies high pulse ... It is an unusual physical finding typically seen in patients with aortic valve diseases if the aortic valve does not normally ... unless there is coexisting aortic regurgitation). The delay can also be observed in supravalvar aortic stenosis. Several pulse ... It is seen in aortic valve stenosis. Pulsus paradoxus: a condition in which some heartbeats cannot be detected at the radial ...
... right-sided aortic arch, in 25% coronary artery anomalies, in 10% a patent foramen ovale or atrial septal defect, in which case ... Genetically, it is most commonly associated with Down syndrome and DiGeorge syndrome. Down syndrome and other chromosomal ... Mutations of this gene have been shown to result in dysfunctional lymphatic syndrome and TOF. GATA4 aids in cardiac development ... "Tetralogy" denotes four parts, here implying the syndrome's four anatomic defects. This is not to be confused with the ...
... persistent truncus arteriosus with aortopulmonary window that affects the aortic arch, left-to-right shunts, anomalous right ... or in surgery to correct absent pulmonary valve syndrome. It is also used in corrective surgeries for Tetralogy of Fallot where ... "The Lecompte Maneuver for Relief of Airway Compression in Absent Pulmonary Valve Syndrome". The Annals of Thoracic Surgery. 81 ...
... critical aortic stenosis, and interrupted aortic arch). Alprostadil is sold in the United States as urethral suppositories and ... hypoplastic left heart syndrome, pulmonary atresia/stenosis, tricuspid atresia/stenosis, transposition of the great arteries) ...
If the fetal aortic stenosis is critical it may lead to hypoplastic left heart syndrome. Hypoplastic Left Heart Syndrome (HLHS ... The atrial septum is removed, the aortic arch is reconstructed to remove any hypoplasia, and then the main pulmonary artery is ... Fetal aortic stenosis is a disorder that occurs when the fetus' aortic valve does not fully open during development. The aortic ... Fetal aortic stenosis impairs left ventricular development, which can lead to hypoplastic left heart syndrome. If untreated, ...
Aortic arch syndrome refers to a group of signs and symptoms associated with structural problems in the arteries that ... Aortic arch syndrome refers to a group of signs and symptoms associated with structural problems in the arteries that ... The aortic arch is the top part of the main artery carrying blood away from the heart. ... The aortic arch is the top part of the main artery carrying blood away from the heart. ...
... encirclement of the trachea and esophagus by connected segments of the aortic arch and its branches. ... Several types of vascular rings have aortic arches that are right sided. Although the specific anatomic details of the various ... Associated syndromes and noncardiac conditions. Vascular ring with a right aortic arch is associated with a band 22q11 deletion ... Other forms of aortic arch anomaly occur in which a vascular ring is not present.) A right aortic arch is formed when the right ...
Pulseless Disease; Occlusive Thromboaortopathy; Aortic Arch Syndrome; Takayasus Arteritis). By Alexandra Villa-Forte , MD, MPH ... and heart attacks Acute Coronary Syndromes (Heart Attack; Myocardial Infarction; Unstable Angina) Acute coronary syndromes ... A person with angina usually has discomfort or pressure beneath the... read more or a heart attack Acute Coronary Syndromes ( ... Heart Attack; Myocardial Infarction; Unstable Angina) Acute coronary syndromes result from a sudden blockage in a coronary ...
Cardiovocal syndrome caused by aortic arch pseudoaneurysm". Journal of the Saudi Heart Association. 28 (4): 266-269. doi: ... Escribano JF, Carnès J, Crespo MA, Antûn RF (January 2006). "Ortners Syndrome and Endoluminal Treatment of a Thoracic Aortic ... A second Ortners syndrome, Ortners syndrome II, refers to abdominal angina. Due to its low frequency of occurrence, more ... Aortic dissection More commonly affects the right recurrent laryngeal nerve as the most common type of aortic dissection is ...
Examination of aorta showed a second/inner aortic arch just as a tube lying inside the aortic arch. The inner aortic arch was ... Keywords: Aortic rupture, Chronic aortic dissection, Marfan syndrome, Sudden death, Telescopic aortic arch ... Telescopic aortic arch is a consequence of aortic dissection in Marfans syndrome, which has not been reported previously. This ... Telescopic aortic arch: A new entity in marfan syndrome Teleskopik arkus aorta: Marfan sendromunda yeni bir antite ...
... a right aortic arch, and a left brachiocephalic artery arising from a left ductus arteriosus. ... reported the first case of right aortic arch and isolation of left innominate artery in 1966 [3]. A right aortic arch, defined ... I. Occurrence of right aortic arch in various types of congenital heart disease. II. Right aortic arch, right descending aorta ... The aortic arch was noted to be right sided with bilateral ductus arteriosus. The aortic valve was mildly hypoplastic and the ...
Ehlers-Danlos syndrome (EDS) comprises a group of inherited heterogeneous disorders that share a common decrease in the tensile ... including views of the aortic arch and aorta. Recent studies indicate a risk for thoracic aortic enlargement in patients with ... encoded search term (Genetics of Ehlers-Danlos Syndrome) and Genetics of Ehlers-Danlos Syndrome What to Read Next on Medscape ... Vascular Ehlers-Danlos syndrome: imaging findings. AJR Am J Roentgenol. 2007 Sep. 189(3):712-9. [QxMD MEDLINE Link]. ...
Ebstein anomaly, hypoplastic left heart syndrome. , interrupted aortic arch, pulmonary atresia with intact septum. , single ... Some of these genetic conditions, such as Down syndrome, Turner syndrome, and 22q11.2 deletion syndrome, result from changes in ... Other conditions, including Noonan syndrome and Alagille syndrome, result from mutations in single genes. ... the heart defects associated with CCHD can also occur as part of genetic syndromes that have additional features. ...
Hypoplastic Left Heart Syndrome. *Interrupted Aortic Arch. *Pulmonary Atresia. *Single Ventricle. *Tetralogy of Fallot ... What is cardiofaciocutaneous syndrome?. Cardiofaciocutaneous syndrome or CFC syndrome is a rare disease that affects about 1 in ... CFC syndrome generally affects an individuals heart (cardio), face (facio), and skin (cutaneous). CFC syndrome affects people ... When the genetic test results came back, Nolan was found to have Cardiofaciocutaneous Syndrome or CFC syndrome. "On one hand, ...
Hypoplastic Left Heart Syndrome. *Interrupted Aortic Arch. *Patent Ductus Arteriosis. *Pulmonary Artery Sling ...
Hypoplastic Left Heart Syndrome. *Interrupted Aortic Arch. *Patent Ductus Arteriosis. *Pulmonary Artery Sling ...
Aortic Arch Anomaly with Peculiar Facies and Mental Retardation Aortic Arch Syndromes + ... urofacial syndrome + A syndrome that is characterized by inverted facial expressions in association with a severe and early- ... Cerebellar Vermis Aplasia with Associated Features suggesting Smith-Lemli-Opitz Syndrome and Meckel Syndrome ... urofacial syndrome (DOID:0050816). Annotations: Rat: (2) Mouse: (2) Human: (2) Chinchilla: (2) Bonobo: (2) Dog: (2) Squirrel: ( ...
Ullrich-Turner syndrome was associated with severe narrowing of the whole aortic arch. In all four groups of chromosomally ... It is postulated that narrowing of the aortic isthmus may be the basis of increased nuchal translucency thickness in all four ... The group consisted of 60 fetuses with trisomy 21, 29 with trisomy 18, 17 with trisomy 13 and 6 with Ullrich-Turner syndrome. ... abnormal fetuses, the aortic isthmus was significantly narrower than in normal fetuses and the degree of narrowing was ...
Pneumococcal endocarditis, meningitis, and rupture of the aortic valve. AMA Arch Intern Med. 1957;99:539-44.PubMedGoogle ... Austrian syndrome in a child-aortic valve endocarditis following pneumococcal meningitis. Int J Cardiol. 2004;94:321-2. DOI ... One case of Austrian syndrome has been reported in the pediatric age group, in a 7-year-old girl in whom aortic valve ... an association now known as Austrian syndrome. This syndrome mainly occurs in middle-age men who have predisposing factors, ...
Aortic Aneurysm / etiology * Aortic Arch Syndromes / epidemiology* * Aortic Valve Insufficiency / etiology * Female ... aortic regurgitation and aortic or arterial aneurysm) attributed to Takayasus disease at the time when the diagnosis was ...
... , also referred to as pulseless disease and aortic arch syndrome, is a rare chronic inflammatory vasculitis ... aortic coarctation or middle aortic syndrome; unlikely to have constitutional symptoms. *Marfan syndrome - look for ... Ehlers-Danlos syndrome - look for fragile skin, easy bruising, joint hyperextensibility, frequent dislocations ... aortic regurgitation, hypertension, or absent pulses. Rarely, patients older than 40 years meet the criteria for Takayasu ...
Narrowing of aortic arch, infants otherwise good health prompt physicians to move Baby JJs Glenn procedure up one month. ... Unborn child diagnosed with rare syndrome when mom was five months pregnant; parents now prepare for difficult road ahead. ... Baby JJ scheduled to have second hypoplastic left heart syndrome surgery Tuesday ... Trussville couple sharing baby, family journey with rare hypoplastic left heart syndrome ...
Narrowing of aortic arch, infants otherwise good health prompt physicians to move Baby JJs Glenn procedure up one month. ... Unborn child diagnosed with rare syndrome when mom was five months pregnant; parents now prepare for difficult road ahead. ... Baby JJ scheduled to have second hypoplastic left heart syndrome surgery Tuesday ... Trussville couple sharing baby, family journey with rare hypoplastic left heart syndrome ...
Takeyasus arteritis, also known as aortic arch syndrome or nonspecific aortoarteritis, predominately affects young to middle- ... Progressive weakness of your artery wall results in aortic aneurysms, essentially blisters of the walls of the blood vessels, ...
Holt and Oram first described this syndrome in 1960. ... Holt-Oram syndrome (HOS) (OMIM 142900) is a heart-upper limb ... for the coarctation of the aortic arch and patent ductus arteriosus in a 3-day-old neonate with the Holt-Oram syndrome. Int J ... SALL4 mutations in Okihiro syndrome (Duane-radial ray syndrome), acro-renal-ocular syndrome, and related disorders. Hum Mutat. ... Holt-Oram syndrome with intermediate atrioventricular canal defect, and aortic coarctation: functional characterization of a de ...
... hypoplastic left heart syndrome (Q23.4), coarctation of aorta (Q25.1), interrupted aortic arch (Q25.21), and total anomalous ... aortic arch. 115. 102,776. 320. 199,973. 95. 77,770. 60. 69,036. 25. 10,323. 615. 142,438. ... interrupted aortic arch ($199,973), and diaphragmatic hernia ($195,456) (Table 2). Although mean costs per nonbirth ... Other syndrome affecting. multiple systems. 1,725. 66,503. 3,555. 92,066. 4,560. 31,502. 5,065. 28,560. 14,145. 21,905. 29,050 ...
Double Choker: Double Aortic Arch with Bilateral Aortic Coarctation Associated with Heterotaxy-Asplenia Syndrome and Complex ... Transcatheter device closure of a congenital aortic-left atrial tunnel.. Sun HY, Buccola KJ, Punn R, Silverman NH, Peng LF, ... Fetal Diagnosis of Dextroposition, Left Pulmonary Artery Sling, Partial Anomalous Left Pulmonary Artery, and Aortic Coarctation ...
Mild degrees of aortic arch obstruction are common following Norwood palliation for hypoplastic heart syndrome. The generally ... Cheatham et al has demonstrated that even complex transverse aortic arch obstructions can be successfully approached with open ... Likewise pulmonary artery narrowing or distortion can be approached in the same manner as aortic arch obstructions equalizing ... Common examples include mild aortic arch obstruction, branch pulmonary stenosis, mild to moderate degrees of atrioventricular ...
The ascending aorta was affected in 270 of the patients (type A AAS), whereas only the descending aorta or aortic arch was ... Diagnosis, management and mortality in acute aortic syndrome: results of the Spanish Registry of Acute Aortic Syndrome (RESA-II ... AAS, acute aortic syndrome; CT, computed tomography; TEE, transesophageal echocardiography.. Values represent No. (%) or mean± ... AAS, acute aortic syndrome; CT, computed tomography; TEE, transesophageal echocardiography.. Values represent No. (%) or mean± ...
Hypoplastic left heart syndrome. Hypoplastic right heart. Interrupted aortic arch. Intrapericardial teratoma. ... Split hand split foot syndrome. Thanatophoric dysplasia. Thanatophoric dysplasia, Type I. Thanatophoric dysplasia, 2nd ...
ARCH) helps diagnose children with midaortic syndrome and related conditions, including renovascular hypertension. ... Middle aortic syndrome, also known as midaortic syndrome - narrowing of part of the aorta (the main artery of the heart) and ... ARCH helps to diagnose children with midaortic syndrome and related conditions, including renovascular hypertension (high blood ... Why choose the Aortic and Renovascular Center for Hypertension?. As the only center of its kind in the St. Louis area and one ...
Coarctation of the aorta, interrupted aortic arch, and hypoplastic left heart syndrome in three generations. By gerbonis 03 Mar ... Coarctation of the aorta, interrupted aortic arch, and hypoplastic left heart syndrome in three generations. S Gerboni, G ... Williams-Beuren Syndrome: Computed Tomography Imaging Review By gerbonis 24 Nov, 2014 English, neonatal cardiology, review ... Beuren, wbs syndrome, Williams Williams-Beuren Syndrome: Computed Tomography Imaging Review Karuna M. Das,Tarek S. Momenah,Sven ...
Coarctation of the aorta, interrupted aortic arch, and hypoplastic left heart syndrome in three generations. By gerbonis 03 Mar ... Coarctation of the aorta, interrupted aortic arch, and hypoplastic left heart syndrome in three generations. S Gerboni, G ... Williams-Beuren Syndrome: Computed Tomography Imaging Review By gerbonis 24 Nov, 2014 English, neonatal cardiology, review ... Beuren, wbs syndrome, Williams Williams-Beuren Syndrome: Computed Tomography Imaging Review Karuna M. Das,Tarek S. Momenah,Sven ...
... early to intermediate results of 120 patients with particular reference to aortic arch repair. J Thorac Cardiovasc Surg 117:920 ... 2001) Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis. Arch Dis ... 1998) Outcome after prenatal diagnosis of the hypoplastic left heart syndrome. Heart 79:371-373. ... 2000) Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution ...
  • The aortic valve was mildly hypoplastic and the main pulmonary artery moderately dilated along with suspicion for a single coronary artery. (heraldopenaccess.us)
  • The previously noted right aortic arch was mildly hypoplastic and gave rise to the right common carotid and right subclavian arteries. (heraldopenaccess.us)
  • Mild degrees of aortic arch obstruction are common following Norwood palliation for hypoplastic heart syndrome. (hgexperts.com)
  • Coarctation of the aorta, interrupted aortic arch, and hypoplastic left heart syndrome in three generations. (gerboni.net)
  • In this issue of Archives of Disease in Childhood , the Guy's group present their experience with staged reconstructive surgery for hypoplastic left heart syndrome (HLHS). (bmj.com)
  • Preoperative and postoperative Norwood stage I circulation in hypoplastic left heart syndrome: the balance between the systemic and pulmonary circulations is crucial. (bmj.com)
  • Our journey begins at the 20 week scan, when our Little Beatrice was diagnosed with Hypoplastic Left Heart Syndrome. (tinytickers.org)
  • At our next scan they told us her heart had in fact grown and was repairing itself .It was definitely not Hypoplastic Left Heart Syndrome, but she would need a Bi Ventricular repair as the left side of her heart was still slender. (tinytickers.org)
  • Photograph showing hypoplastic right thumb of the right hand of a 6-month-old infant with Holt-Oram syndrome. (medscape.com)
  • In a baby with a coarctation, the aortic arch also might be smaller than usual ( hypoplastic ). (kidshealth.org)
  • Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year experience. (chop.edu)
  • This right sided arch showed no evidence of coarctation. (heraldopenaccess.us)
  • Fetal Diagnosis of Dextroposition, Left Pulmonary Artery Sling, Partial Anomalous Left Pulmonary Artery, and Aortic Coarctation. (rchsd.org)
  • Double Choker: Double Aortic Arch with Bilateral Aortic Coarctation Associated with Heterotaxy-Asplenia Syndrome and Complex Atrioventricular Canal Defect. (rchsd.org)
  • A cervical arch on either side, variable laterality of the descending thoracic aorta, coarctation of the major arch, and/or discontinuity of the central pulmonary arteries may be present. (medscape.com)
  • These patients were classified according to the presence and severity of four major complications (Takayasu's retinopathy, secondary hypertension, aortic regurgitation and aortic or arterial aneurysm) attributed to Takayasu's disease at the time when the diagnosis was established: no complications (group I) or mild single complication (group IIa) and severe single complication (group IIb) or multiple complications (group III). (nih.gov)
  • Abdominal Aortic Aneurysm (AAA), a focal enlargement of the abdominal aorta is an ongoing process that can be affected by many parameters. (asme.org)
  • Aortic media necrosis and aortic dissection without aneurysm formation]. (bvsalud.org)
  • As normal cardiovascular morphogenesis proceeds, a patterned regression and persistence of the various arches and right-sided dorsal aorta occur, ultimately resulting in the mature configuration of the thoracic aorta and its branches. (medscape.com)
  • The third, fourth, and sixth arches, along with the seventh intersegmental arteries and the left dorsal aorta, are the primary contributors to the normal aortic arch and its major thoracic branches (see image below). (medscape.com)
  • The segments of the bilateral aortic arch system that normally regress include the distal portion of the sixth arch and the right-sided dorsal aorta. (medscape.com)
  • Normally, the left fourth arch becomes the aortic arch, the right fourth arch contributes to the innominate artery, the distal left sixth arch becomes the ductus arteriosus, the proximal sixth arches bilaterally contribute to the proximal branch pulmonary arteries, the left dorsal aorta becomes the descending thoracic aorta, and the dorsal intersegmental arteries bilaterally become the subclavian arteries. (medscape.com)
  • A right aortic arch is formed when the right dorsal aorta remains patent and either the left fourth arch or the left dorsal aorta regress abnormally (see image below). (medscape.com)
  • Examination of aorta showed a second/inner aortic arch just as a tube lying inside the aortic arch. (bezmialem.edu.tr)
  • The inner aortic arch was arrised 2.5 cm above aortic valves and lasted at the beginning of the descending aorta. (bezmialem.edu.tr)
  • The reported incidence is 1-1.5 in 10,000 live births.Right-sided aortic arch with isolation of the innominate artery is an extremely rare congenital anomaly, in which the innominate artery loses its connection with the ascending aorta and is supplied by either a patent ductus or mediastinal collaterals [1]. (heraldopenaccess.us)
  • Perform a baseline echocardiogram, including views of the aortic arch and aorta. (medscape.com)
  • and proximal dilatation of the aorta can cause aortic regurgitation , dilated cardiomyopathy , and congestive heart failure . (logicalimages.com)
  • When a child is diagnosed with midaortic syndrome - a rare condition where part of the aorta (the largest blood vessel coming from the heart) and its major branches narrow - this can lead to an impaired blood flow to vital organs. (stlouischildrens.org)
  • Middle aortic syndrome, also known as midaortic syndrome - narrowing of part of the aorta (the main artery of the heart) and its major branches, leading to impaired blood flow to vital organs in the chest, abdomen and the lower limbs. (stlouischildrens.org)
  • Right: Mature anatomy of a vascular ring formed by a right aortic arch with an aberrant left subclavian artery arising from a retroesophageal diverticulum with a left-sided ligamentum arteriosum to the left pulmonary artery. (medscape.com)
  • 4 7 Conotruncal heart defects most commonly found in DGS/VCFS patients with 22q11.2 deletions are interrupted aortic arch (IAA) type B, truncus arteriosus (TA), and tetralogy of Fallot (TOF). (bmj.com)
  • When considering cardiovocal syndrome, the most common historical cause is a dilated left atrium due to mitral stenosis, but other causes, including pulmonary hypertension, thoracic aortic aneurysms, an enlarged pulmonary artery and aberrant subclavian artery syndrome have been reported compressing the nerve. (wikipedia.org)
  • Progressive weakness of your artery wall results in aortic aneurysms, essentially blisters of the walls of the blood vessels, to form. (healthline.com)
  • Double aortic arch is one of the 2 most common forms of vascular ring, a class of congenital anomalies of the aortic arch system in which the trachea and esophagus are completely encircled by connected segments of the aortic arch and its branches. (medscape.com)
  • Although the double aortic arch has various forms, the common defining feature is that both the left and right aortic arches are present. (medscape.com)
  • The easiest way to understand the anatomy and development of double aortic arch and other forms of vascular ring is to begin by considering the bilateral system of pharyngeal arch vessels in the early embryo. (medscape.com)
  • A double aortic arch is formed when both fourth arches and both dorsal aortas remain present. (medscape.com)
  • Double aortic arch has various forms. (medscape.com)
  • In more than 75% of patients with double aortic arch, the right arch is dominant. (medscape.com)
  • Most people with double aortic arch will require surgery. (umms.org)
  • Babies who have breathing problems due to double aortic arch usually continue to have breathing problems for a few weeks to months after surgery because the cartilage in the trachea is still soft. (umms.org)
  • Fetal echocardiographic features associated with bicuspid aortic valve. (uchicago.edu)
  • Aortopathy Including Hereditary Disease (Marfan Syndrome, Bicuspid Aortic Valve, etc.) -- 14. (nshealth.ca)
  • An inflammatory disease called Takayasu syndrome may result in narrowing (stenosis) of the vessels of the aortic arch. (medlineplus.gov)
  • Common examples include mild aortic arch obstruction, branch pulmonary stenosis, mild to moderate degrees of atrioventricular and semilunar valve insufficiency, and aortopulmonary collateral flow. (hgexperts.com)
  • [11] Other upper airway abnormalities that can be seen in CHARGE syndrome include: laryngomalacia, tracheomalacia, tracheoesophageal fistula, and subglottic stenosis. (aao.org)
  • Transluminal dilatation of innominate stenosis in aortic arch syndrome]. (bvsalud.org)
  • [9] Cardiac defects can include Tetralogy of Fallot, aortic arch interruption, double outlet right ventricle with arch vessel abnormalities, and atrioventricular septal defects (AVSD). (aao.org)
  • However, the heart defects associated with CCHD can also occur as part of genetic syndromes that have additional features. (medlineplus.gov)
  • Case Report: Airway and Concurrent Hemodynamic Management in a Neonate with Oculo-Auriculo-Vertebral (Goldenhar) Syndrome, Severe Cervical Scoliosis, Interrupted Aortic Arch, Multiple Ventricular Septal Defects, and an Unstable Cervical Spine. (stanford.edu)
  • [ 3 ] In addition, mutations of the gene encoding chromodomain-helicase DNA-binding protein 7 ( CHD7 ) have been found in some patients with Kallmann syndrome or idiopathic hypogonadotropic hypogonadism, some of whom have features of the CHARGE syndrome (characterized by delayed growth and development, congenital cardiac defects, dysmorphic ears, hearing loss, coloboma of the eyes). (medscape.com)
  • OBJECTIVE: To evaluate the hypothesis that childhood survival for individuals with Down syndrome (DS) and congenital heart defects (CHDs) has improved in recent years, approaching survival of those with DS without CHDs. (cdc.gov)
  • Takayasu arteritis, also referred to as pulseless disease and aortic arch syndrome, is a rare chronic inflammatory vasculitis that primarily affects large- and medium-sized vessels. (logicalimages.com)
  • When the minor arch is atretic, the atretic segment almost always is distal to the left subclavian artery, although atresia may also occur between the left common carotid and subclavian arteries. (medscape.com)
  • Approximately 65% of patients with CHARGE syndrome may have obstructed breathing due to choanal atresia at birth. (aao.org)
  • Aortic dissection More commonly affects the right recurrent laryngeal nerve as the most common type of aortic dissection is type A (Figure 2). (wikipedia.org)
  • Telescopic aortic arch is a consequence of aortic dissection in Marfan's syndrome, which has not been reported previously. (bezmialem.edu.tr)
  • This paper presents the first case of telescopic aortic arch secondary to chronic aortic dissection, as an incidental finding of a forensic autopsy. (bezmialem.edu.tr)
  • Although the specific anatomic details of the various forms differ, they share the defining feature of all vascular rings, namely, encirclement of the trachea and esophagus by connected segments of the aortic arch and its branches. (medscape.com)
  • The two arches encircle the trachea and esophagus and often cause compression on these structures. (umms.org)
  • Schematic diagram (right) shows the segments of the pharyngeal arch system that regress (shown in black) in the normal formation of the thoracic great arteries. (medscape.com)
  • Unstable Angina) Acute coronary syndromes result from a sudden blockage in a coronary artery. (merckmanuals.com)
  • Mortality is high in acute aortic syndrome (AAS), which therefore requires early treatment. (revespcardiol.org)
  • Introduction: A cluster of pneumonia cases of unknown origin was first reported in Wuhan China then the causa- tive pathogen was identified and named severe acute respiratory syndrome coronavirus 2 (SARS-Cov2) and the associated disease was named coronavirus disease 2019 (COVID-19). (who.int)
  • The two beta coronaviruses, severe acute respiratory symptoms associated with COVID-19 infection are syndrome (SARS-CoV) and Middle East respiratory fever (accounting for 98% of the symptoms), myalgia syndrome coronavirus (MERS-CoV) have caused or fatigue, and shortness of breath. (who.int)
  • Ortner's syndrome is a rare cardiovocal syndrome and refers to recurrent laryngeal nerve palsy from cardiovascular disease. (wikipedia.org)
  • Cardiofaciocutaneous syndrome or CFC syndrome is a rare disease that affects about 1 in 800,000 people, possibly more. (maximhealthcare.com)
  • A region of homozygosity within 22q11.2 associated with congenital heart disease: recessive DiGeorge/velocardiofacial syndrome? (bmj.com)
  • [ 1 ] The presence or absence of a vascular ring in the setting of a right aortic arch depends on the branching of the brachiocephalic vessels and the location of the ductus arteriosus, as discussed below. (medscape.com)
  • Some examples of reported cardiovascular causes include: Congenital abnormalities: Atrial septal defect Aortopulmonary window Ebstein's Anomaly Patent Ductus Arteriosus (PDA) Surgical intervention: Transcatheter closure of a PDA Incidence: due to the close proximity of the LRLN to the aortic arch, transient paralysis can occur in 10% of cases while permanent effects can occur in 1% of cases. (wikipedia.org)
  • We present a case of left isomeric heterotaxy, a right aortic arch, and a left brachiocephalic artery arising from a left ductus arteriosus. (heraldopenaccess.us)
  • The aortic arch was noted to be right sided with bilateral ductus arteriosus. (heraldopenaccess.us)
  • The definition of Ortner's syndrome has since then expanded to encompass all possible causes of left recurrent laryngeal nerve palsy with cardiac etiologies. (wikipedia.org)
  • Patients with Holt-Oram syndrome may require dietary modification because of their specific cardiac abnormality. (medscape.com)
  • Malignant hyperthermia-like manifestations in a two-month-old child with Holt-Oram syndrome undergoing cardiac surgery. (medscape.com)
  • Cardiac malformations are found in 75-85% of patients with CHARGE syndrome. (aao.org)
  • The cardiac apex, aortic arch, and stomach are all normally on the left. (uab.edu)
  • In situs inversus totalis, the cardiac apex, stomach and aortic arch are all on the right. (uab.edu)
  • Complex cardiac anomalies are usually found in this syndrome. (uab.edu)
  • The cardiac anomalies associated with polysplenia syndrome are less complex giving this syndrome a better prognosis than asplenia syndrome. (uab.edu)
  • The majority of patients with DiGeorge syndrome are recognized to have immunodeficiency in the first few months of life when they are being evaluated for cardiac malformations that are highly associated DiGeorge syndrome and/or deletions of chromosome 22q11.2. (lu.se)
  • The proximal (p) sixth arches develop into the proximal pulmonary arteries, and the distal (d) sixth arches become the arterial ducts. (medscape.com)
  • The easiest way to understand the anatomy and development of vascular rings with a right aortic arch is to begin by considering the bilateral system of pharyngeal arch vessels in the early embryo. (medscape.com)
  • Situs ambiguous with bilateral 'right-sidedness' is known as Asplenia syndrome. (uab.edu)
  • Situs ambiguous with bilateral 'left-sidedness' is called Polysplenia syndrome. (uab.edu)
  • A 2-dimensional echocardiographic picture taken from subxiphoid window showing a large secundum atrial septal defect (arrow) in a 7-year-old boy with Holt-Oram syndrome. (medscape.com)
  • In all four groups of chromosomally abnormal fetuses, the aortic isthmus was significantly narrower than in normal fetuses and the degree of narrowing was significantly greater in fetuses with high nuchal translucency thickness. (nih.gov)
  • Deficient hypothalamic GnRH secretion underlies the markedly abnormal gonadotropin secretion patterns in most patients with Kallmann syndrome or idiopathic hypogonadotropic hypogonadism. (medscape.com)
  • Vena caval syndrome occurs when heartworms reside in the vena cava (the big vein returning blood from the liver and lower body to the heart). (irishdogs.ie)
  • The correct diagnosis for patients with Ehlers-Danlos syndrome (EDS) is critical and must be determined, if possible. (medscape.com)
  • Recent studies indicate a risk for thoracic aortic enlargement in patients with classical-type EDS (types I and II). (medscape.com)
  • Pregnancy represents a special issue in patients with certain types of Ehlers-Danlos syndrome (EDS). (medscape.com)
  • Among patients with a right-dominant double arch, those with a patent minor arch outnumber those with an atretic minor arch. (medscape.com)
  • In approximately 20% of patients, the left arch is dominant. (medscape.com)
  • In these patients, the minor right arch is typically patent. (medscape.com)
  • A report identified this syndrome in 4% of patients with radial longitudinal deficiency. (medscape.com)
  • [4] It was found that 90-95% of patients fulfilling the formal diagnostic criteria for CHARGE syndrome are heterozygous for a CDH7 mutation or deletion. (aao.org)
  • In addition to visual impairment, colobomas predispose CHARGE syndrome patients to retinal detachment . (aao.org)
  • [7] Other ophthalmic features that can occur in patients with CHARGE syndrome include microphthalmia, microcornea, cataracts, strabismus, cranial nerve VII palsy, and ptosis. (aao.org)
  • Genital hypoplasia is a common feature in patients with CHARGE Syndrome. (aao.org)
  • One study showed that 70% of CHARGE syndrome patients have an IQ less than 70. (aao.org)
  • [21] Patients with CHARGE syndrome are also at risk for hypothyroidism [22] and recurrent suppurative ear and chest infections. (aao.org)
  • By definition, either anosmia (lack of sense of smell) or severe hyposmia is present in patients with Kallmann syndrome, in contrast to patients with idiopathic hypogonadotropic hypogonadism, whose sense of smell is normal. (medscape.com)
  • MRI of the brain in patients with Kallmann syndrome (KS) and idiopathic hypogonadotropic hypogonadism (IHH). (medscape.com)
  • Mutations of the KAL1 gene, which encodes a putative neural cell adhesion molecule (anosmin), have been described in several patients with X-linked Kallmann syndrome. (medscape.com)
  • Loss-of-function mutations of the gene encoding fibroblast growth factor receptor 1 (FGFR1) have been described in patients with autosomal dominant Kallmann syndrome. (medscape.com)
  • Mutations of the gene encoding fibroblast growth factor 8 have been found in a small minority of patients with autosomal dominant Kallmann syndrome. (medscape.com)
  • E ditor -DiGeorge syndrome (DGS, MIM 18840) and velocardiofacial syndrome (VCFS, MIM 192430) are associated with interstitial deletions of chromosome 22q11.2 and are considered to be phenotypic variations of the same underlying genetic defect. (bmj.com)
  • There is no known cause of vascular rings, thought it can be associated with DiGeorge syndrome. (umms.org)
  • DiGeorge Syndrome by The Jeffrey Modell Foundation (JMF). (lu.se)
  • In the most frequent form of vascular ring with a right aortic arch, an aberrant origin of the left subclavian artery from a retroesophageal diverticulum (diverticulum of Kommerell) is present, which originates as the last branch of the aortic arch (distal to the right subclavian artery). (medscape.com)
  • The aortic arch is the top part of the main artery carrying blood away from the heart. (medlineplus.gov)
  • Left: Schematic diagram depicting the segments of the pharyngeal arch system that regress (shown in black) in order for the development of a right aortic arch with aberrant left subclavian artery. (medscape.com)
  • Likewise pulmonary artery narrowing or distortion can be approached in the same manner as aortic arch obstructions equalizing pulmonary blood flow and decreasing already elevated caval and lymphatic pressures in addition to reducing the total resistance the single ventricle faces. (hgexperts.com)
  • Holt-Oram syndrome associated with anomalies of the feet. (medscape.com)
  • In 1957, an American internist reported the preference of Streptococcus pneumoniae for the aortic valve and its frequent association with meningitis and pneumonia ( 1 ), an association now known as Austrian syndrome. (cdc.gov)
  • One case of Austrian syndrome has been reported in the pediatric age group, in a 7-year-old girl in whom aortic valve endocarditis developed after pneumococcal meningitis infection ( 2 ). (cdc.gov)
  • In pneumococcal endocarditis, the native aortic valve is the most frequent location of vegetation. (cdc.gov)
  • Surgery is often needed to treat the underlying cause of aortic arch syndrome. (medlineplus.gov)
  • ARCH offers world-class care from Washington University specialists in pediatric nephrology, pediatric interventional cardiology and vascular surgery, along with their fellow St. Louis Children's Hospital clinicians to provide unified care of the whole patient in a single center. (stlouischildrens.org)
  • She had surgery to repair a narrow aortic arch, close a VSD and shut her PDA duct. (tinytickers.org)
  • After numerous daily scans, they decided Beatrice needed open heart surgery to repair the narrow Aortic Arch, close the VSD and to shut the PDA duct. (tinytickers.org)
  • Only people with symptoms due to the vascular rings from a right-sided aortic arch will require surgery. (umms.org)
  • Most people with a right aortic arch never need surgery. (umms.org)
  • Several types of vascular rings have aortic arches that are right sided. (medscape.com)
  • Ullrich-Turner syndrome was associated with severe narrowing of the whole aortic arch. (nih.gov)
  • Many children with midaortic syndrome experience severe hypertension, which is addressed using medical, endovascular, and surgical approaches depending on each patient's need. (stlouischildrens.org)
  • Pseudoaneurysm Notable case: A male with long-standing uncontrolled hypertension and hoarseness of voice attributed to life-long smoking was found to have a pseudoaneurysm of the aortic arch which was compressing the LRLN. (wikipedia.org)
  • A right aortic arch may occur without forming a vascular ring. (medscape.com)
  • Other forms of aortic arch anomaly occur in which a vascular ring is not present. (medscape.com)
  • [8] High refractive errors and amblyopia also occur in CHARGE syndrome. (aao.org)
  • Left: Schematic diagram of the primitive pharyngeal arch system showing the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, the fourth (IV) and sixth (VI) pharyngeal arches, distal pulmonary arterial segments (PA), dorsal aortas (DA), and seventh intersegmental arteries (VII). (medscape.com)
  • Some of these genetic conditions, such as Down syndrome , Turner syndrome , and 22q11.2 deletion syndrome , result from changes in the number or structure of particular chromosomes. (medlineplus.gov)
  • Individuals with 22q11.2 deletion syndrome (22q11.2DS) can present with a wide range of features that are highly variable, even within families. (nih.gov)
  • Kartageners syndrome may also be seen in connection with situs inversus. (uab.edu)
  • Kartageners syndrome represents a triad of situs inversus (seen in 50% of cases), sinusitis, and bronchiectasis. (uab.edu)
  • When the genetic test results came back, Nolan was found to have Cardiofaciocutaneous Syndrome or CFC syndrome. (maximhealthcare.com)
  • KLF13 is a genetic modifier of the Holt-Oram syndrome gene TBX5. (medscape.com)
  • Li B, Chen S, Sun K, Xu R, Wu Y. Genetic analyses identified a SALL4 gene mutation associated with Holt-Oram syndrome. (medscape.com)
  • A cardiomelic developmental field has also been postulated to relate the genetic heterogeneity of HOS (and other similar syndromes) to a cascade of molecules, including the brachyury, sonic hedgehog, bone morphogenetic protein, retinoic acid receptor, and transforming growth factor beta families. (medscape.com)
  • CHARGE Syndrome is a rare genetic syndrome that produces a constellation of clinical features. (aao.org)
  • Classic Kallmann syndrome (KS) and idiopathic hypogonadotropic hypogonadism (IHH) are rare genetic conditions that encompass the spectrum of isolated hypogonadotropic hypogonadism. (medscape.com)
  • It's also common in girls born with Turner syndrome , a genetic disorder in which one of a girl's two X chromosomes is incomplete or missing. (kidshealth.org)
  • Aortic arch syndrome refers to a group of signs and symptoms associated with structural problems in the arteries that branch off the aortic arch. (medlineplus.gov)
  • Heterotaxy syndrome is a complex set of abnormalities related to arrangements of the internal thoracic-abdominal structures across the left-right axis. (heraldopenaccess.us)
  • It is postulated that narrowing of the aortic isthmus may be the basis of increased nuchal translucency thickness in all four chromosomal abnormalities. (nih.gov)
  • CFC syndrome generally affects an individual's heart (cardio), face (facio), and skin (cutaneous). (maximhealthcare.com)
  • Takeyasu's arteritis, also known as aortic arch syndrome or nonspecific aortoarteritis, predominately affects young to middle-aged females of Asian descent. (healthline.com)
  • Williams-Beuren Syndrome: Computed Tomography Imaging Review Karuna M. Das,Tarek S. Momenah,Sven G. Larsson,Shehla Jadoon, Abdullah S. Aldosary,Edward Y. Lee Abstract Williams-Beuren syndrome (WBS) affects young infants and children. (gerboni.net)