Interrupted aortic arch iaa is a rare form of critical neonatal heart disease in which there is lack of continuity between the ascending aorta and the descending thoracic aorta. Interrupted aortic arch is usually diagnosed before birth with a fetal echocardiogram echo or immediately after birth. Interrupted aortic arch iaa is rare, accounting for only 1% of critically ill neonates with congenital heart disease. First, the aortic arch does not form a complete tube and is divided, or interrupted see upper arrow in diagram. This used to be done in a twostage operation but is now done most of the time in one stage. Iaa occurs when part of the aortic arch that carries blood to the head and neck is missing. We report a patient with cat eye syndrome and interrupted aortic arch type b, a typical finding in the 22q11. Part of the aorta is absent and this leads to severe obstruction to blood flow to the lower part of the body.
Incomplete double aortic arch radiology reference article. Aortic arch aneurysms are one of the most complicated conditions to treat. Interrupted aortic arch cs mott childrens hospital. Interrupted aortic arch iaa is an uncommon congenital cardiovascular anomaly where there is a separation between the ascending and descending aorta. Interrupted aortic arch in an asymptomatic adult chest. Floating thrombus in aortic arch pubmed central pmc. Floating thrombi in the aortic arch are very rare and an unusual source of systemic embolism.
Although most cases occur in normally connected great arteries, interrupted aortic arch. Aortic disease continued aortic arch aneurysm repairs in 2011, cleveland clinic surgeons performed 222 procedures to repair aortic arch aneurysms. It happens when a babys aorta doesnt develop correctly, leaving a gap between two parts of the aorta that are normally connected. This means that the heart cannot send blood through the aorta. In newborns with interrupted aortic arch, the only way for blood to bypass the blockage is via the patent ductus arteriosus 4. Interrupted aortic arch is a serious heart defect that requires surgery soon after birth. The aortic arch is the part of the aorta the major vessel that carries oxygenrich blood from the heart to the bodys tissues that curves directly above the heart and begins the descent to the lower body. Interrupted aortic arch iaa is the absence or discontinuation of a portion of the aortic arch. The aortic arches or pharyngeal arch arteries previously referred to as branchial arches in human embryos are a series of six paired embryological vascular structures which give rise to the great arteries of the neck and head. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. Coarctation of the aorta coa coa is defined as a constriction or narrowing of the aorta which is variable in severity. Interrupted aortic arch iaa refers to a rare but very serious congenital heart defect in which there is a gap between the ascending and descending segments of the aorta, namely, in the arch of.
Chapter 8 coarctation of the aorta and interrupted aortic arch. In the immediate newborn period blood flows through the ductus into the descending aorta and hence reaches the lower part of the circulation. Interrupted aortic arch is a very rare heart defect affecting 3 per million live births in which the aorta is not completely developed. Interrupted aortic arch was associated mainly with a ventricular septal defect 85 % and left ventricular outflow tract obstruction lvoto, %. The aortic arch, arch of the aorta, or transverse aortic arch english. We report two cases of interrupted right aortic arch associated with isolated left pulmonary artery and bilateral ductus arteriosus in the first case and with truncus arteriosus in the second case. In 1760, the prussian anatomist, johann friedreich meckel, characterized coarctation of the aorta as an extraordinary dilatation of the heart which came from the fact that the aortic conduit was too narrow. Even though the condition is very rare, interrupted aortic arch is a lifethreatening situation and needs to be managed immediately. The ascending aorta begins above the aortic root and extends towards the neck until it begins to turn and give rise to the aortic arch. Aortic arch replacement main line health philadelphia. Mar, 2020 in patients with acute aortic dissection, it is reasonable to repair the entire aortic arch when the arch is aneurysmal, or there is extensive aortic arch destruction and leakage. Association of chromosome 22q11 deletion with isolated. In a sense it is the complete form of a coarctation of the aorta.
Pdf interrupted aortic arch type b in a patient with cat. As the only source of blood for the lower body after birth in the case of interrupted aortic arch is through the patent ductus arteriosus, the medication prostaglandin e1 is administered soon after birth to keep that vessel open. Mar 04, 2015 the aortic arch is the portion of the main artery that bends between the ascending and descending aorta. May 10, 2019 calculate and compare zscores of the aortic arch, including the aortic isthmus.
Interrupted aortic arch type b was the major anomaly in 48 patients and persistent truncus arteriosus in 37. The ascending aorta supplies blood to the head and arms depending on the type of interrupted aortic arch, while the lower body receives blood that would otherwise pass through to the lungs. If iaa is untreated, the median age at death is 410 days, usually following physiological closure of the ductus arteriosus. The upmc heart and vascular institute s center for thoracic aortic disease. Approximately 30 percent to 40 percent of the infants with. It was a floating, highly mobile thrombus attached to the lesser curvature of the aortic arch. The goal of surgery is to create remove the blockage. The ascending aorta is more frequently affected by aneurysms and dissections and requires open heart surgery to be repaired. The interruption occurs just beyond the left subclavian artery. Chromosomal analysis and fluorescent in situ hybridization fish showed a supernumerary bisatellited isodicentric marker chromosome derived from chromosome 22. Mar 29, 2016 interrupted aortic arch iaa is a relatively rare genetic disorder that usually occurs in association with a nonrestrictive ventricular septal defect vsd and ductus arteriosus or, less commonly, with a large aortopulmonary window or truncus arteriosus.
Type b occurs when the left fourth aortic arch regresses be fore normal ascension of the left subclavian artery to its expected position. Pdf interrupted aortic arch a rare abnormality, is the discontinuity of the ascending and descending aorta. Isolated interrupted of aortic arch diagnosed using ct angio. Aortic arch disease can result from blood pressure changes, clots, trauma, a congenital disorder one that is present from birth, or takayasus arteritis, an autoimmune disorder that inflames the aorta and the pulmonary artery the main artery of the lung. Strong associations of arch anomalies with chromosomal and. The aorta is the hearts main pipeline, carrying oxygenrich blood to the body. Pdf interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births. Daa is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea andor esophagus. An interrupted aortic arch iaa is a rare heart condition that happens when the aorta doesnt form completely. Coarctation of the aorta and interrupted aortic arch. The diagnosis was made in all of them by catheterization. Presented at the 73rd scientific sessions of the american heart association, new orleans, louisana, november 1115, 2000. Account for 15% to 20% of all congenital cardiovascular diseases. An ascending aortic aneurysm is a bulge in the portion of the aorta closest to the heart.
Farber professor of surgery and radiology director, unc aortic network university of north carolina. Interrupted aortic arch is a rare condition in which the aortathe large blood vessel arising from the heart that carries blood to the rest of the bodyis incomplete, or interrupted. Interrupted aortic arch is managed with surgery to repair the aorta and reestablish the correct arch. References included conform to ase guideline methodology. It can either be complete or connected by a remnant fibrous band. Determinants of type ii and type iii aortic arch in humans. Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. Aai should be distinguished anatomically from atresia of the aortic arch where. The clinical presentation is similar to that of coa, so is discussed here.
Herein, a case of a 3cm thrombus in the aortic arch is reported. Iaa is a defect of the aorta, which is the main blood vessel that carries oxygenrich blood from the heart to the rest of the body. In a child with interruption of the aortic arch, there is a disconnection a break between the top part of the aortic arch and the lower, descending aorta. Aortic arch anomalies are a type of congenital heart condition, which means it is a disease or abnormality that is present from birth. More than 97% of the cases also have associated cardiac anomalies complicating their treatment. Interrupted aortic arch iaa is defined as a complete luminal and anatomical interrupt. Interrupted aortic arch congenital heart disease cove. Interrupted aortic arch iaa is a rare congenital, meaning present at birth, heart defect. An accompanying large ventricular septal defect vsd andor patent ductus arteriosus pda is frequently present. Interrupted right aortic arch is a rare congenital cardiovascular anomaly typically associated with other forms of congenital heart disease. Aai should be distinguished anatomically from atresia of. It is important to diagnose this defect as soon after birth as possible. Interrupted aortic arch occurs in association with a wide variety of intracardiac malformations. Repair of interrupted aortic arch the annals of thoracic surgery.
In patients with acute aortic dissection, it is reasonable to repair the entire aortic arch when the arch is aneurysmal, or there is extensive aortic arch destruction and leakage. Iaa type b is specifically associated with a posterior malalignment ventricular septal defect vsd with resultant. Interrupted aortic arch is characterized by interruption of the aortic arch distal to the left subclavian artery type a, proximal to the left subclavian artery type b, or between the innominate artery and the left common carotid artery type c. Interrupted aortic arch, type b alberto sosa olavarria, md. Incomplete double aortic arch is a rare vascular ring anomaly wherein a segment of the minor aortic arch, usually the left, is atretic. The calcium content of the aortic arch, measured from the aortic root to the descending thoracic aorta at the level of the carina, was determined by using a coronary calcium score grade. Three arteries, feeding the head, neck and arms, branch off directly from the aortic arch, in this order. Interrupted aortic arch iaa is a congenital anomaly where there is interruption or discontinuation of a segment of the aortic arch. Other reports have also suggested that a direct anastomotic repair without a patch for iaa leads to. New branched and fenestrated devices for the aortic arch mark a. They are ventral to the dorsal aorta and arise from the aortic sac. The arch of the aorta gives off branches to the head and arms. Interrupted aortic arch iaa is an uncommon congenital anomaly representing approximately 1% of congenital heart disease.
Interrupted aortic arch iaa is a ductus dependent lesion. The first vessel is a left innominate artery, the second and third are the right common carotid and right subclavian arteries fig. Isolated interrupted aortic arch is a rare congenital cardiac disorder believed at one time to be incompatible with life once the ductus arteriosus closed. Type b occurs when the left fourth aortic arch regresses before normal ascension of the left subclavian artery to its expected position. Result from errors in the embryologic development of the branchial arches, including errors of involution or migration, or abnormal persistence of vascular structures. When two ventricles are present, there is almost always an interventricular communication, 1 and frequently there is some degree of obstruction or hypoplasia of the outflow tract of the subaortic ventricle.
Interruption of the aortic arch childrens hospital of. Double aortic arch is a relatively rare congenital cardiovascular malformation. Interrupted aortic arch iaa is a relatively rare genetic disorder that usually occurs in association with a nonrestrictive ventricular septal defect vsd and ductus arteriosus or, less commonly, with a large aortopulmonary window or truncus arteriosus. Interrupted aortic arch flash animation, less than 70kb how is it treated. To our knowledge, only 12 cases have been reported in the literature and mostly in children. Martin, md interrupted aortic arch, two cases elena andreeva, md. Interrupted aortic arch is a rare congenital malformation of the aortic arch that occurs in 3 per million live births.
The ascending aorta begins at the hearts left ventricle and extends to the aortic arch, or the bend in the aorta. In an interrupted aortic arch, part of the aorta is missing, leaving a gap. The aortic arch aa is classified as type i, ii and iii based on the vertical distance between the origin of brachiocephalic artery and the top of the arch 1. Interrupted aortic arch iaa for parents nemours kidshealth. Interrupted aortic arch pediatric echocardiography. In particular, the chss reported that any technique other than direct anastomosis with patch augmentation is a risk factor for arch reintervention. Interrupted aortic arch in an adult pubmed central pmc. Therefore, in about half of the patients with digeorge syndrome the major anomaly was. Interrupted aortic ayortik arch is a rare congenital present at birth problem. The aorta is the main artery that carries blood with oxygen out of the heart to the body. An open patent ductus arteriosus is necessary to allow blood flow to the lower part of the body. In the usual right aortic arch, there is mirrorimage branching of the arch vessels compared to the normal left arch. Handout on aortic arch interruption part of the aorta is absent and this leads to severe obstruction to blood flow to the lower part of the body.
The site of the defect varies 2 and may or may not involve the takeoff of the head and neck vessels. The majority of patients have a left ductus arteriosus arising from the left pulmonary artery and inserting into the left subclavian artery. Interrupted aortic arch radiology reference article. As the aorta carries blood from the heart to the rest of the body, it arches upward before bending around and down. Diagnosis and treatment of interrupted aortic arch. Interrupted aortic arch iaa is the absence or discontinuation of a portion of the aortic arch, the section of the aorta that turns downward toward the lower half of the body. Type c is seen when the ventral portion of the left third aortic arch and left fourth aortic arch involute, and there is a persistent ductus caroticus, a structure that normally regresses. We report the first adult patient with an asymptomatic interrupted aortic arch diagnosed.
Data from multiple references including recent data particularly suited for infants with arch hypoplasia or coarctation. The arch travels backward, so that it ultimately runs to the left of the trachea. The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with digeorge syndrome congenital absence or hypoplasia of the thymus and. Almost all patients also have other cardiac anomalies, including a. Increased aortic arch calcification in patients older than 75.
There are three types of interruption of the aortic arch, based on the location of the break in the aorta. The aortic arch is the portion of the main artery that bends between the ascending and descending aorta. In most babies with interrupted aortic arch 7090% there is also a large ventricular septal defect 3. Ascending and aortic arch aneurysm symptoms and repair. An interrupted aortic arch is often accompanied by a ventricular septal defect vsd. Interrupted aortic arch iaa is an uncommon congenital cardiovascular malformation characterized by the lack of continuity between the ascending and descending thoracic aorta as described by steidele in 1778. Please use one of the following formats to cite this article in your essay, paper or report. Interrupted aortic arch an overview sciencedirect topics. Oxygenated blood flows across the vsd to the right ventricle, where it mixes with deoxygenated and is pumped through the pulmonary artery to the lungs. Aortic arch interruption aai is a rare disease with estimated incidence of 0. Interrupted aortic arch iaa is an extremely rare chd defined as the loss of luminal continuity between the ascending and descending aorta. Interrupted aortic arch iaa is a rare genetic disorder of the cardiovascular system, present in approximately two cases per 100,000 live births and comprising 1.
Interrupted aortic arch, pediatric cardiac center delaware. Interruption of the aortic arch iaa nicklaus children. Surgical repair of interrupted aortic arch iaa remains challenging and is associated with significant mortality and incidence of late arch obstruction, as recently reported by the congenital heart surgeons society chss. Repair is also indicated when there is an infected mycotic aneurysm or when there is significant atherosclerosis plaque in the arch that has resulted in strokes. New branched and fenestrated devices for the aortic arch.
Takayasus mostly affects asian females between the ages of 10 and 30. Of these, 152 were elective and 70 were emergency surgeries. The two ends of the aorta are sewn together 1 and the patent ductus arterious 2 is tied off. Outcomes in patients with interrupted aortic arch and.
Defined as a loss of luminal continuity between the ascending and descending portions of the aorta, this anomaly entails a very poor. Caregivers may also hear a murmur due to blood flowing across the ventricular septal defect or the patent ductus arteriosus. This prevents oxygenated blood from reaching the rest of the body. Clinical presentation as in the case of other vascular rings, this anomaly can cause 1. Aortic arch calcium content and arch classification were examined in consecutive patients undergoing thoracic computed tomography scans. There are three types of interrupted aortic arch, and they are classified according to the site of the interruption.