Contrast-enhanced magnetic resonance angiography of persistent fifth aortic arch in children

Background Cine angiography and echocardiography have been utilized to diagnose congenital aortic arch anomalies. However, the visualization of great vessels by echocardiography is limited, while cine angiography requires cardiac catheterization with ionizing radiation. Contrast-enhanced magnetic resonance angiography (MRA) is a noninvasive modality suitable for visualization of congenital aortic arch anomalies. Objective To evaluate the utility of contrast-enhanced MRA in the diagnosis of persistent fifth aortic arch, a rare congenital aortic arch anomaly, and to compare the diagnostic accuracy of MRA with that of echocardiography and cine angiography. Materials and methods In four pediatric patients, contrast-enhanced MRA studies were performed for diagnosing persistent fifth aortic arch. The findings of MRA were compared with echocardiographic findings and confirmed by cine angiography and operation. Results Transthoracic surface echocardiography noted an aberrant vessel arising from the ascending aorta in two of four patients; the etiology of this vessel was uncertain. In the other two patients a diagnosis of coarctation was made. Of the four patients, only one was diagnosed with interruption of the aortic arch. Contrast-enhanced MRA clarified uncertain echocardiographic findings, enabling the correct diagnosis of persistent fifth aortic arch with fourth aortic arch interruption in all four patients. Conclusion Contrast-enhanced MRA is a safe, accurate, and fast imaging technique for the evaluation of persistent fifth aortic arch and may obviate the need for conventional cine angiography. Cardiac catheterization may be reserved for some types of complicated congenital heart disease and for obtaining hemodynamic information.     

Rare types of aortic arch anomalies

Summary Rare types of aortic arch malformations include isolation of the right or left subclavian arteries in conjunction with a left or right aortic arch respectively. Isolation of the innominate or carotid arteries and an aberrant left innominate artery may exist in association with a right aortic arch. Other anomalies are persistent fifth aortic arch, subclavian artery as the first branch of the aortic arch, pulmonary artery, and ductus arteriosus sling. The angiocardiographic features and embryology of each of these malformations are discussed.     

Interruption of aortic arch and hypoplastic left heart syndrome

Interruption of the aortic arch and hypoplastic left heart syndrome in the same patient is exceptional. In the combined collections of the Registry of Cardiovascular Disease (St. Paul, Minnesota, U.S.A.) and the Registry of Congenital Heart Defects of the Rijksuniversiteit Gent (Gent, Belgium) three specimens were found with this unusual combination. These cases are herein described and compared with four similar case previously reported in the literature.     

Aortic atresia with aortopulmonary window and interruption of the aortic arch

Summary The cross-sectional echocardiographic and postmortem appearances of the heat from a patient with the rare association of aortic valve atresia, aortopulmonary window, and interrupted aortic arch are described. Differentiation of this anomaly from truncus arteriosus with interrupted aortic arch is important.     

A rare case of double aortic arch: Right thoracotomy and residual retroesophageal aortic arch

Summary The case of a 2-year-old boy with tetralogy of Fallot and a complete double aortic arch (both arches patent) is reported. The left dominant aortic arch ran retroesophageally to the right and joined with the right smaller arch to form the descending thoracic aorta on the right side. We employed a right thoracotomy and performed a division of the right nondominant arch at the connection with the descending aorta. The surgical implications of an unusual type of double aortic arch are discussed.     

Persistence of hypoplastic and recoarcted fifth aortic arch associated with type A aortic arch interruption: Surgical and balloon angioplasty results in an infant

Summary A rare aortic malformation, the persistence of the fifth aortic arch (PFAA) associated with coarctation of the aorta, is reported in a 3-month-old male infant who underwent an emergency surgical intervention at 10 days of life, to relieve a severe aortic coarctation by pericardial patch technique.A successful balloon angioplasty was performed 2 months later eliminating a recoarctation. The gradient fell from 77 mmHg to 0, but a definitive surgical intervention for recoarctation was necessary 14 months after angioplasty.     

Aortic atresia with interruption of the aortic arch and an aortopulmonary fistulous tract: Case report

We present a case of interruption of the aortic arch (IAA) in association with aortic atresia. A pulmonary artery to ascending aorta fistulous channel at the level of the sinuses of Valsalva of the pulmonary artery was present and supplied the ascending aorta and coronary arteries. The communication had a significant length, so embryologically it is not an aortopulmonary window. There have been only three reported cases in the literature of IAA with aortic atresia; in none was a pulmonary artery to ascending aorta fistulous tract present.     

Right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch

We present a rare case of right circumflex retro-oesophageal aortic arch with coarctation of a high-positioned right arch. A 7-month-old boy presented with a cardiac murmur. Cardiac situs was normal and there was no evidence of an intracardiac shunt or patent ductus arteriosus. MR aortography revealed a right aortic arch that was high-positioned, tortuous and narrowed. This right aortic arch crossed the midline behind the oesophagus and continued as a left-sided descending aorta. The left common carotid and subclavian arteries arose from a large branching vascular structure that derived from the top of the left-sided descending aorta. The right common carotid artery arose from the ascending aorta. The proximal portion of the right common carotid artery showed very severe stenosis and poststenotic dilatation. The right subclavian artery originated distal to the narrowed and tortuous segment of the aortic arch.     

The subclavian artery as the first branch of the aortic arch

Summary Four patients in whom the subclavian artery arose as the first branch of the ascending aortic arch are presented: two with four separate brachiocephalic arteries and two with a common trunk supporting both carotid arteries.     

主肺动脉窗合并右肺动脉起源于主动脉和主动脉弓离断患儿诊治分析

目的探讨主肺动脉窗(APW)合并右肺动脉起源于主动脉(AORPA)及主动脉弓离断(IAA)患儿的诊断与治疗。方法回顾性分析1例APW合并AORPA及IAA患儿的临床资料。结果患儿,女,4个月,外院诊断为室间隔缺损,保守治疗无效,无法撤离呼吸机,入院后超声心动图提示为APW、AORPA、动脉导管未闭、重度肺动脉高压;外科手术后患儿出现少尿,上、下肢动脉压差大,经CT检查提示合并IAA,再次外科手术矫治;术后患儿顺利恢复并出院,随访28个月,生长发育与同龄儿无明显差别。结论 APW合并AORPA及IAA患儿一经诊断需尽早手术治疗,一期外科纠治效果满意,术前应完善心血管造影等检查防止漏诊。     

Right aortic arch with isolation of the left subclavian artery

A 5-year-old boy with cyanotic heart disease and weak pulses in the left arm is described. Cardiac catheterization and cineangiography confirmed the diagnosis of tetralogy of Fallot and right aortic arch with isolation of the left subclavian artery. In addition to the aortogram and right ventricular cineangiography, pulmonary angiography was performed, demonstrating that blood did not reach the left subclavian artery through a left ductus arteriosus.     

Interruption of aortic arch type A in two siblings

The recurrence of interruption of aortic arch (IAA) in siblings is rare. According to previous reports concerning siblings with IAA, all cases were IAA type B according to the classification proposed by Celoria and Patton. In this report we present the first cases of brothers with IAA type A. The type of IAA was confirmed by autopsy findings in the elder brother and by operative findings in the younger brother. The etiology of IAA was unclear. Monogenic inheritance was thought unlikely because congenital heart disease was not recognized in other members of the family. It would seem prudent to separate recurrence risks for IAA type A from IAA type B when genetic counselling is provided, but it must be borne in mind that the recurrence of IAA type A can occur among siblings.     

Esophageal atresia and right aortic arch

A right aortic arch occurs in 5% of patients with esophageal atresia. Its presence has significant implications; there is a high association of other anomalies, especially cardiac types. Repair of the atresia has been regarded as difficult with the usual approach through the right chest. We report our experience with five cases of right aortic arch and esophageal atresia treated in the past 5 years. In four cases this association was not recognized preoperatively and the right aortic arch was not an obstacle in repairing the atresia through the standard right thoracotomy. In one case the right aortic arch was diagnosed before surgery; repair was done through the left chest. Offprint requests to: G. Stringel     

Multislice CT angiography of interrupted aortic arch

Interrupted aortic arch (IAA) is defined as complete luminal and anatomic discontinuity between the ascending and descending aorta. Because almost all patients with IAA become critically ill during the neonatal period, they should undergo urgent corrective surgery. This clinical urgency necessitates a fast and accurate noninvasive diagnostic method. Although echocardiography remains the primary imaging tool for this purpose, it is not always sufficient for planning surgical correction of IAA, principally due to a limited acoustic window and the inexperience of imagers. In this context, multislice CT angiography is regarded as an appropriate imaging technique complementary to echocardiography because it is fast, accurate, and objective for the diagnosis of IAA. In this article we describe what cardiac radiologists should know about IAA in their clinical practice, including clinicopathological features, CT features with contemporary surgical methods and postoperative complications, and differentiation from coarctation of the aorta and aortic arch atresia.     

Percutaneous balloon aortoplasty for restenosis after extended aortic arch anastomosis for type B interrupted aortic arch

A 4-month-old boy underwent a percutaneous balloon aortoplasty for restenosis after extended aortic arch anastomosis for type B interrupted aortic arch (IAA). Balloon aortoplasty resulted in a decrease in the peak systolic pressure gradient across the obstructive segment from 84 mmHg to 19 mmHg and in an increase in diameter from 2.5 mm to 4.3 mm. No complications related to the procedure were observed. We assume that balloon aortoplasty can be effective and safe for relieving postoperative aortic obstruction associated with IAA.     

Retroesophageal right aortic arch

Summary Right aortic arch with left descending aorta and a retroesophageal aortic segment is an uncommon congenital vascular malformation which may cause symptoms of compression from a vascular “ring.” The presence of this malformation may be suspected by the findings on barium swallow and the diagnosis confirmed by angiocardiography. Surgical intervention may be necessary in patients who have severe symptoms.     

Single arterial trunk arising from the aortic arch associated with coarctation of the aorta

A 6-day-old baby with a single arterial trunk arising from the aortic arch is reported. The baby had coarctation of aorta with patent ductus arteriosus and ostium primum atrial septal defect. A review of the literature in English reveals no other case reported of an infant with this association of anomlies.     

Markedly hypoplastic circumflex retroesophageal right aortic arch: MR imaging and surgical implications

Background Circumflex retroesophageal right aortic arch with a markedly hypoplastic retroesophageal segment is a rare anomaly of the aortic arch. Adequate surgical management relies on precise diagnosis, which might not be feasible with echocardiography. Objective To demonstrate the utility of MR imaging in establishing the diagnosis and to describe potential pitfalls in making the diagnosis. Materials and methods Three patients with a circumflex retroesophageal right aortic arch with a markedly hypoplastic retroesophageal segment were retrospectively evaluated. All patients underwent evaluation by echocardiography and MR imaging. The MR imaging consisted of 3-D MR angiography in two patients and fast gradient recalled echo with cardiac-triggered segmented acquisition in one patient. Surgical confirmation was obtained on all three patients. Results The arch anatomy was accurately depicted in all three patients by MR imaging and in none of the patients by echocardiography. Conclusion MR imaging is extremely useful in establishing the diagnosis of markedly hypoplastic retroesophageal circumflex right aortic arch and thus helps in surgical planning.     

Biphasic flow velocity pattern in the descending aorta in double aortic arch

We report a case of double aortic arch in which the biphasic flow velocity pattern in the descending aorta changed to a monophasic flow velocity pattern after corrective surgery. The hypoplasia of left aortic arch might prolong the acceleration time of the flow velocity in the left aortic arch.