Coarctation of aorta with right aortic arch. Report of surgical correction in 2 cases: one with associated anomalous origin of left circumflex coronary artery from the right pulmonary artery.

Two patients with right aortic arch, anomalous left subclavian artery, and coarctation of theaorta, have been operations for the relief of coarctation, successful in one but only partially successful in the other: in one case, a 'dacron' patch was inserted into the narrowed segment, but a mild recurrence of coarctation was found a year later; in the other, the proximal segment of the anomalous left subclavian artery was used for the repair, with complete relief of the coarctation, both immediately and at late follow-up. The first case had multiple associated congenital abnormalities, including anomalous origin of the left circumflex coronary artery from the right pulmonary artery, which was also corrected surgically: this anomaly has only been recorded once hitherto. The second had facial haemangiomata and a small ventricular septal defect. Four previously reported cases of coarctation and right arch are reviewed. These 6 cases are considered in the light of current views on the development and classification of aortic arch anomalies.     

Interrupted aortic arch. A series of 15 patients

Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.     

Anomalous origin of the left pulmonary artery from the ascending aorta in two children with pulmonary atresia, sub-aortic ventricular septal defect and right-sided major aorto-pulmonary collateral arteries

We report two rare cases of an anomalous origin of the left pulmonary artery (AOLPA) from the ascending aorta, associated with pulmonary atresia, a ventricular septal defect and a left aortic arch. The cases are unusual because AOLPA is more commonly associated with a right aortic arch and it is more usual for the right pulmonary artery to originate anomalously from the ascending aorta. The pulmonary blood supply to the right lung in both patients was absent and provided instead by major aorto-pulmonary collateral arteries which were stenosed at multiple levels. The AOLPA in both patients originated from the postero-lateral aspect of the ascending aorta just distal to the sino-tubular junction. Only one patient showed the more common association of an unusual aortic arch branching pattern in the form of an anomalous right subclavian artery. Neither patient was in heart failure and the chest X-ray in both revealed differential pulmonary perfusion with prominent vascularity of the left lung. Cardiac catheterisation showed systemic pressures within the anomalous left pulmonary artery. Karyotyping revealed normal chromosomes, and fluorescent in-situ hybridisation done in one patient was negative for chromosome 22q11.2 microdeletion. Both patients have been managed conservatively.     

Left common carotid artery arising from the pulmonary artery in a patient with DiGeorge syndrome.

A female infant, born at 33 weeks' gestation with tetralogy of Fallot, died of severe perinatal asphyxia 6 hours after birth. Necropsy disclosed two associated vascular anomalies: a right aortic arch with a left common carotid artery arising from the pulmonary artery (isolated left common carotid artery) and an aberrant left subclavian artery arising from the descending aorta. Agenesis of the thymus and parathyroid gland was also found, suggesting that the child also had DiGeorge syndrome. Origin of the left common carotid artery from the pulmonary artery is exceedingly rare. When planning surgical treatment it is important to be aware of the possibility of this anomaly occurring in association with congenital heart disease, particularly in the presence of tetralogy of Fallot, right sided aortic arch, or DiGeorge syndrome.     

Scanning suprasternal echocardiography.

Scanning suprasternal echocardiography was performed in 280 patients with a variety of cardiac anomalies. By using the special suprasternal transducer on the suprasternal notch, the aortic arch, right pulmonary artery, and left atrium were recorded superoinferiorly. From this reference position various scanning techniques were made to record the main pulmonary artery, pulmonary valve, ascending aorta, aortic valve, and distal aortic arch, wherever possible. These scans made it possible to assess (a) the integrity and size of right pulmonary artery and main pulmonary artery in pulmonary atresia, stenosis of origin of right pulmonary artery, pulmonary artery banding, kinked Waterston anastomosis, and aneurysm of pulmonary artery; (b) relative positions of aortic valve and pulmonary valve in malposition complexes; (c) the position and size of the aortic arch in tetralogy of Fallot and aortic coarctation. Measurements of the left atrium made by suprasternal echocardiography were consistently larger than those made by praecordial echocardiography. Changes in relative sizes of aortic arch, right pulmonary artery, main pulmonary artery, and left atrium were also documented in the various cardiac anomalies. The atrial baffle after Mustard repair for d-transposition of the great arteries and the atrial membrane in cor triatriatum were also demonstrated. The introduction of scanning techniques has thus widened the scope of suprasternal echocardiography.     

Aortic origin of the left pulmonary artery in an infant with Fallot's tetralogy

We report the case of a male neonate who had a prenatal diagnosis of Fallot's tetralogy. He presented with respiratory distress during the second week of life. Chest x-ray showed an enlarged right ventricle and pulmonary edema. Echocardiography demonstrated characteristic features of Fallot's tetralogy. However, cardiac catheterization disclosed that, in this case of Fallot's tetralogy, the left pulmonary artery had an anomalous origin in the ascending aorta. At 23 days of age, the patient underwent total surgical correction of the defects, during which a direct anastomosis was employed without conduit placement. 10 days later, he was discharged. We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition.     

Importance of Absent Ductus Arteriosus in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Tetralogy of Fallot without pulmonary valve syndrome is almost always associated with an absent ductus arteriosus. Patients with right aortic arch and retroesophageal left subclavian artery have a vascular ring if the left ductus arteriosus or its remnant and the Kommerell diverticulum are present. We report the cases of 2 infants in whom the role of an absent ductus arteriosus or its remnant is noteworthy. Both patients had a combination of tetralogy of Fallot with absent pulmonary valve syndrome and right aortic arch with retroesophageal left subclavian artery without a vascular ring. The absence of the ductus arteriosus has a role in the pathogenesis of tetralogy of Fallot with absent pulmonary valve syndrome. The absence of a ductus arteriosus in the right aortic arch with retroesophageal left subclavian artery precludes a vascular ring.     

Ectopic aortic origin of the right pulmonary artery in tetralogy of Fallot

A case is described wherein Fallot's tetralogy was associated with aortic origin of the right pulmonary artery. The left pulmonary artery was the continuation of the pulmonary trunk. Although a number of examples of Fallot's tetralogy associated with aortic origin of the left pulmonary artery are known, combination of Fallot's tetralogy and aortic origin of the right pulmonary artery is rare and has been described in only two cases previously.     

Obstructed coarctation in a right aortic arch in an adult female

Coarctation of a right aortic arch is rare congenital anomaly. We report a rare case of a 24-year-old female with coarctation of the right aortic arch with aberrant left subclavian artery between the right common carotid and right subclavian arteries. The coarctation progressed into complete obstruction as the interruption of the aorta in adulthood. To prevent cerebral complications and progression to heart failure, surgical procedure was selected. Extraanatomical bypass grafting between the ascending and descending aorta was successfully performed using cardiopulmonary bypass. Some patients diagnosed with interruption of the aortic arch in adulthood might be displaying progression of undiagnosed coarctation, as our in case. Three-dimensional computed tomography was useful to detect the obstructive lesion and to determine the surgical approach and methods.     

Surgical Treatment of Vascular Ring Including Right Cervical Aortic Arch

Abstract Cervical aortic arch is a developmental entity consisting of persistence of the right or left third branchial arch and regression of the fourth branchial arches. In most cases, the aorta is redundant and crosses behind the esophagus to the opposite side. In the presence of an aberrant subclavian artery contralateral to the side of the aortic arch and a ligamentum arteriosum, a vascular ring is formed around the trachea and esophagus. Two young patients with right-sided cervical aortic arch, aberrant left subclavian artery, and ligamentum arteriosum presented with dys-phagia and exertional dyspnea. In one patient, through a left thoracotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were dissected thoroughly above and below the level of the ring. In addition, the aberrant left subclavian artery was divided at its origin from a large diverticulum and implanted into the left common carotid artery; the aortic di-verticulum was resected. In the other patient, who had associated 22q11 chromosomal deletion, in addition to left-sided compression of the trachea and esophagus, there was additional marked compression of the right anterolateral trachea by the redundant ascending aorta. Through a median sternotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were widely mobilized; an additional aortopexy of the ascending aorta to the right of the sternum resulted in the absence of tracheal compression. The cases of the two reported patients illustrate the clinical variability of vascular ring, including a right cervical aortic arch and the consequently versatile surgical approach that is needed to successfully address this combination of vascular anomalies.     

Aortic anomaly with atypical coarctation A report of three cases presenting coarctation between the origin of the left carotid and the left subclavian artery

Three cases of faulty development of the aorta are presented. In Case 1 the aortic arch between the origin of the left common carotid and the left subclavian artery was tortuous, narrow and situated high in the thorax. Two aneurysms were located in this portion. The left vertebral and left internal mammary arteries were not demonstrated. The narrowed part of the aorta was resected under hypothermia with a good result.

Case 2 showed a pronounced coarctation between the left common carotid artery and the left subclavian artery. This latter vessel was filled almost completely via an ample collateral network. The left common carotid artery had an anomalous origin and the right subclavian artery showed a slight coarctation in the proximal part; a poststenotic dilatation was distal to this.

Case 3 showed an aortic arch situated high in the thorax. The bending of the aortic arch was very sharp, producing a functional coarctation with differences in the blood pressures in the right and left arm; there was no anatomic coarctation in the ordinary sense.

Two cases were corrected by surgery; the former by resection of the tortuous part and end to end anastomosis, the latter by incision and removal of the membrane.

The different theories concerning the development of coarctation of the aorta are discussed. It is suggested that the cases presented might best be explained by faulty development.     

Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching

OBJECTIVES

The purpose of this study was to determine the frequency of chromosome 22q11 deletions in patients with isolated anomalies of the aortic arch and its branches.

BACKGROUND

Chromosome 22q11 deletions are often present in patients with certain forms of congenital cardiovascular disease, including tetralogy of Fallot, truncus arteriosus and interruption of the aortic arch. Among patients with these anomalies, chromosome 22q11 deletion is more common in those with abnormal aortic arch laterality or branching.

METHODS

We studied 66 patients with isolated anomalies of the aortic arch and no associated intracardiac defects for deletions within chromosome 22q11, using fluorescence in situ hybridization with the cosmid probe N25 (D22S75). Arch anomalies included: double aortic arch (n = 22); right aortic arch with aberrant left subclavian artery (n = 28); right aortic arch with mirror-image branching and a vascular ring formed by a left-sided ductus from the descending aorta (n = 5); right aortic arch with mirror-image branching and no vascular ring (n = 4); and left aortic arch with aberrant right subclavian artery (n = 7). In addition, four patients had a cervical aortic arch, four had aortic coarctation and six had hypoplasia/atresia of the proximal pulmonary arteries.

RESULTS

Chromosome 22q11 deletions were found in 16 patients (24%) across the full spectrum of anomalies studied. Among the morphologic variables analyzed, only hypoplasia/atresia of the proximal pulmonary arteries correlated with the deletion (p = 0.03). Among patients with a double arch, the frequency of chromosome 22q11 deletion was higher in those with an atretic minor arch than it was in those with a patent minor arch (p = 0.02).

CONCLUSIONS

Chromosome 22q11 deletion is associated with isolated anomalies of laterality or branching of the aortic arch in 24% of cases in our series. These findings should alert the clinician to consider deletion screening in patients with isolated anomalies of the aortic arch.     

The rare association of truncus arteriosus with a cervical double aortic arch presenting with left main bronchial compression

Truncus arteriosus, a double aortic arch, and a cervical aortic arch are all rare cardiovascular anomalies. We experienced a unique female newborn with the rare combination of truncus arteriosus with a cervical double aortic arch, which probably resulted from abnormal persistence of the bilateral 2nd or 3rd rather than the 4th embryonic aortic arches and failure of regression of the right 8th somitic segment of the right dorsal aorta. She presented with respiratory distress soon after birth, which was initially attributed to the vascular ring and hypertensive pulmonary arteries. Our inability to relieve her respiratory compromise by surgical division of the vascular ring and main pulmonary artery banding prompted the diagnosis of left main bronchial compression caused by a posteriorly displaced dilated ascending aorta that compressed the right pulmonary artery and left main bronchus against the descending aorta. The patient then underwent successful left main bronchus stent implantation. We speculate the cervical double aortic arch is redundant in nature and is a loose ring that may not cause tracheal compression. Nevertheless, a posteriorly displaced dilated ascending aorta in patients with truncus arteriosus may compress the right pulmonary artery and the main bronchus on the side of the aortic arch against the descending aorta.     

Left cervical aortic arch associated with multiple vascular anomalies

Cervical aortic arch is a rare anomaly occasionally associated with other cardiovascular abnormalities. We present a case of tortuous left cervical aortic arch associated with hypoplastic transverse arch, coarctation of the aorta, and right brachiocephalic arteries arising below the coarctation and stenotic origin of the left subclavian artery. These multiple anatomic anomalies, which are associated in our case, have not been described in a single patient previously.     

Histo-anatomical backgrounds of subclavian flap aortoplasty in coarctation of the aorta

After repair of coarctation of the aorta using the technique of resection and end-to-end anastomosis, the internal diameters of the aortic isthmus and descending aorta often fail to increase. Better results seem possible with aortoplasty using the left subclavian flap technique. In order to clarify this matter, we investigated the structure of the left subclavian artery comparing it with that of the descending aorta and aortic isthmus: we studied the internal diameter, the thickness of the tunica media and the packing density of its elastic fibers in these vascular elements using a postmortem material of children with a coarctation of the aorta. The ages ranged from 4 days to 13 months with one child of 8 years. All 16 cases had one or more additional cardiac lesions. Operation had been performed in 3 children: 2 end-to-end anastomoses and one subclavian bypass of the aortic arch. Data were compared with observations on autopsy cases of children without cardiovascular abnormalities. The mean findings were that the calibers of the left subclavian artery and the descending aorta were within normal limits but that the caliber of the aortic isthmus was smaller than in normal children. The measurements on the tunica media showed that although, generally, the thickness of the media of the left subclavian artery was smaller than that of the aortic isthmus and descending aorta of the same individual, it contained relatively more elastic fibers than the matching vessels. This may indicate that the structure of the left subclavian artery is well suited to grow out as a part of the aortic arch.(ABSTRACT TRUNCATED AT 250 WORDS)     

Complete repair of the interrupted aortic arch by an anterior approach. Apropos of 9 cases

Interrupted aortic arch is a duct-dependent heart disease, and this is why the use of prostaglandins E, which keep the ductus arteriosus open to feed the descending aorta, has transformed its formerly sombre prognosis. The authors report the cases of 9 neonates and infants presenting with interrupted aortic arch associated with ventricular septal defect who underwent total repair of the malformations under extracorporeal circulation. Five children were less than 10 years' old. The aortic lesions were of type A in 2 cases (interruption distal to the left subclavian artery) and of type B in 7 cases (interruption between the left carotid and subclavian arteries). In the second group the lesions were of type B1 in 5 cases (without retro-oesophageal right subclavian artery) and of type B2 in 2 cases (right retro-oesophageal artery distal to the interruption). There was one death (11 p. 100). So far, no child has been reoperated upon for residual aortic obstruction or reopened ventricular septal defect. This technique of total repair in one stage, with closure of the ventricular septal defect, seems to be preferable to the two-stage technique in which stage one includes, in addition to aortic arch repair, cerclage of the pulmonary artery, a procedure with numerous disadvantages and a higher overall mortality rate.     

Right-sided aorta with atypical coarctation involving only the left subclavian artery. Hypertension

A case has been described of right-sided aorta with coarctation of the left subclavian artery. The diagnosis was made clinically and by ordinary x-ray film and then confirmed by angiocardiographic films.A small or absent left radial pulse in the presence of a hypertension in the right arm and a normal expected blood pressure in the lower extremities should lead to the consideration of the diagnosis of a localized coarctation of the left subclavian artery. Such causes as cervical rib, anomalous course of the left radial artery, tumors, and aortic aneurysm must first be investigated.With a localized coarctation of the left subclavian artery, just as with the typical coarctation of the aorta, a hypertension is usually present. It is probably a reflex mechanism originating from the nerves in the aortic arch and producing an increased vascular tone in all the extremities.Localized coarctation of the left subclavian artery frequently is discovered by accident. The patient presents no anatomic or physiologic defect in the left upper extremity except the small or absent left radial pulse. Hard work is quite compatible with the lesion.     

Aortic root dilatation in tetralogy of Fallot long-term after repair--histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy

The ascending aorta or pulmonary trunk in congenital heart disease may dilate out of proportion to hemodynamic or morphogenetic expectations, may become aneurysmal, and may rupture. A bicuspid aortic valve and/or coarctation of the aorta are consistently associated with ascending aortic and para-coarctation medial abnormalities. Congenital heart diseases such as single ventricle, truncus arteriosus, transposition of the great arteries and tetralogy of Fallot are also associated with aortic medial abnormalities. Aortic regurgitation in unrepaired tetralogy of Fallot imposes volume overload on both ventricles. A significant subset of adults late after repair of tetralogy of Fallot exhibits progressive aortic root dilatation which may lead to regurgitation and predispose to dissection and rupture which can be fatal, and necessitating aortic valve and aortic root surgery. The aortic dilatation relates medial abnormalities coupled with previous long-standing volume overload of the ascending aorta. Risk factors for aortic dilatation and regurgitation in tetralogy of Fallot relate to specific hemodynamic abnormalities such as pulmonary atresia, right aortic arch and a history of an aorto-pulmonary shunt, and patient demographics such as male sex and the association of chromosome 22q11 deletion. There is no current consensus on beta-blocker administration for limiting progressive dilatation of the aortic root in patients with congenital heart disease and repaired tetralogy of Fallot. Aortic root surgery should be considered for these patients and address aortic regurgitation and or prevent the risk of aortic dissection. Meticulous follow-up of the aortic root after repair, tetralogy of Fallot are thus recommended.     

Quantitative morphology of the aortic arch in neonatal coarctation

It has been speculated that neonatal coarctation results from postnatal constriction of the aortic isthmus and ductus arteriosus. However, aortic arch hypoplasia is present in some neonates with coarctation and is presumed to be due to decreased aortic arch blood flow in utero. To measure the degree of aortic arch hypoplasia and to analyze the distribution of blood flow in neonatal coarctation, quantitative morphometric analysis of the great vessels from two-dimensional echocardiograms was performed in 14 neonates with isolated coarctation and 14 normal control neonates, all less than 1 month old. Measurements of the aortic valve, pulmonary valve, ascending aorta, transverse aortic arch between the carotid and subclavian arteries, aortic isthmus, descending aorta, main pulmonary artery and brachiocephalic vessels were obtained to the nearest 0.5 mm. In coarctation patients the transverse arch and isthmus were significantly smaller than in control subjects (p less than 0.001). In addition, pulmonary valve and main pulmonary artery diameters were significantly greater in neonates with coarctation than in normal neonates. Transverse arch hypoplasia and increased pulmonary valve and pulmonary artery diameters are present in neonates with coarctation. This suggests decreased aortic arch flow and increased pulmonary and ductus arteriosus flow in utero, which produce a characteristic echocardiographic appearance of transverse arch hypoplasia with a large main pulmonary artery.     

Anomalous origin of left pulmonary artery branch from the aorta with Fallot's tetralogy

We report the case of a ten-year-old boy who had often presented with respiratory distress since birth. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated TGE characteristic features of Fallot's tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin from the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged.We present the results of a literature review of the incidence,physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.