Aortic valve replacement in an adult 20 years after a single-stage extra-anatomic repair of an interrupted aortic arch

Interrupted aortic arch is a rare congenital malformation, which is defined as a loss of luminal continuity between the ascending and descending aorta. Usually, there is a considerable distance between the ascending and descending parts of the aorta. According to the classification system of Celoria and Patton, three subtypes have to be differentiated. We describe a single-stage, extra-anatomic repair in an adolescent patient with a rare type C (the interruption is proximal to the left common carotid artery) interrupted aortic arch and his subsequent aortic valve replacement 19 years later.     

Interrupted aortic arch in an asymptomatic adult

Isolated interrupted aortic arch is a rare congenital cardiac disorder believed at one time to be incompatible with life once the ductus arteriosus closed. To our knowledge, only 12 cases have been reported in the literature and mostly in children. The diagnosis was made in all of them by catheterization. We report the first adult patient with an asymptomatic interrupted aortic arch diagnosed by computed tomography.     

Interrupted aortic arch in an adult female

Interrupted aortic arch is a rare and usually lethal malformation, representing approximately 1% of congenital heart disease. This presents as a missing segment of the aortic arch and is divided into three types: A-called extreme form of coarctation, and is characterized by disruption of aorta's continuity distal to the left subclavian artery (30-40%), B-disruption between the left subclavian and the left carotid arteries (55-60%), and C-the most uncommon type, interruption proximal to the left common carotid artery. The suspicion of coarctation of the aorta can be made from a combination of physical findings including systolic ejection murmur, the murmurs of collateral blood vessels, diminished or absent femoral pulse, and difference in blood pressure between arms and legs. Interrupted aortic arch is an extremely rare anomaly in adult patients. To our knowledge, the world medical literature contains only about 13 reports of interrupted aortic arch diagnosed in adults.     

Single-stage aortic valve-sparing root replacement and extra-anatomic bypass for aortic arch interruption in an adult

We describe the treatment of an extremely rare case of interrupted aortic arch, annuloaortic ectasia, and aortic regurgitation in a 34-year-old man who presented with dyspnea and palpitation. We performed a single-stage operation involving aortic root reconstruction and valve repair with concomitant extra-anatomic bypass from the ascending to the descending aorta with a Dacron tube graft. The patient made a full recovery. To the best of our knowledge, this is the first such report in the English medical literature.     

Interrupted aortic arch in an adolescent male

Interrupted aortic arch (IAA) is an uncommon and usually lethal congenital malformation. The present report describes an unusual case of IAA, an aneurysmal ascending aorta and a bicuspid aorta in a 15-year-old boy. He presented with general malaise, weakness of his legs, headache and hypertension that began six months earlier. He had suffered from effort intolerance since childhood. A three-dimensional gadolinium contrast-enhanced magnetic resonance angiogram demonstrated IAA and a markedly developed collateral circulation. IAA is an uncommon disease that is rarely encountered in an adolescent patient with nonspecific symptoms or hypertension. The present case also shows the clinical value of three-dimensional gadolinium contrast-enhanced magnetic resonance angiography for noninvasive assessment of the aortic arch, and magnetic resonance angiography can replace angiography in the assessment of aortic arch anomalies and visualization of well-developed collaterals.     

Interrupted aortic arch: an eight-year experience

INTRODUCTION: Interrupted aortic arch (IAA) is a rare congenital heart defect with a high mortality rate in the neonatal period. Surgical correction of associated intracardiac anomalies can be performed in a one-stage (primary) or two-stage approach. OBJECTIVES: Case review of children with IAA operated in our center and to evaluate the surgical outcomes and the occurrence of complications. METHODS: A retrospective chart review of children operated from June 1998 to October 2006. RESULTS: Twelve children (nine girls and three boys) were operated. Nine patients had ventricular septal defect with septal malalignment, two had univentricular hearts and two had transposition of the great arteries. Primary correction was performed in eleven patients (aged between two and 38 days), including two Norwood procedures. There was no early mortality. The follow-up time ranged from 30 days to six years (median 2.6 years), with late mortality of 33%. Two children required reintervention for aortic arch restenosis, which was successfully treated by catheterization. DISCUSSION: Our experience is that early primary repair of IAA has low early and late mortality and is the method of choice most situations. Late mortality depends the severity of associated anomalies.     

Interrupted aortic arch with cardiac heart failure in young adult

The interrupted aortic arch (IAA) is a rare cardiopathy, with high morbimortality when treatment is delayed. This study is a case of IAA with atypical clinical behavior in a 19-year-old patient. The history and the clinical assessment were compatible with recent heart failure, associated to syncope episodes and reduction of pulses in the lower limbs. The electrocardiogram showed biventricular and biatrial overload. The echocardiogram showed severe ventricular dysfunction and altered blood flow in the descending aorta. The angiotomography disclosed occlusion of the thoracic aorta after the left subclavian artery. The attained diagnosis was interrupted aortic arch and surgical treatment was indicated.     

Interrupted aortic arch in adulthood

Interrupted aortic arch is one of the rarest anomalies among the congenital cardiac defects. Patients must be treated early in life, otherwise death is inevitable. Reports on adult patients with aortic interruption are very rare in the literature. In this report, we present a 21-year-old patient with type A interrupted aortic arch. The patient underwent successful anatomical reconstruction of the descending aorta.     

Right-sided cervical aortic arch with stenosis--treatment with an extra-anatomic bypass graft

Right-sided cervical aortic arch is a very rare vascular anomaly that may lead to stenosis development. Anatomic repair may be impeded by its high course or by abnormal branching of the supraaortic vessels, or both. This report will describe the treatment of a stenotic right-sided cervical aortic arch using an extra-anatomic bypass graft without extracorporeal support in an 11-year-old girl.     

Interrupted right aortic arch in DiGeorge syndrome

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 parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.     

Interrupted right aortic arch in DiGeorge syndrome.

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 parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.     

Interrupted aortic arch associated with aortopulmonary window in a 20 year-old young adult

Interrupted aortic arch (IAA) and aortopulmonary window (APW) are rare but well-described congenital malformations. However, coexistence of these anomalies in young adults is an unusual event. Patients rarely reach adulthood without surgical correction in the neonatal period. The authors describe a 20-year-old patient, who presented with exertional dyspnea and was diagnosed as having IAA and concomitant APW. IAA just distal to the left subclavian artery and a large APW were demonstrated by chest multi-slice computed tomography angiography and cardiac catheterization.     

Interrupted aortic arch: brief review and summary of an eighteen-year experience

Interrupted aortic arch (IAA) is an uncommon congenital anomaly representing approximately 1% of congenital heart disease. More than 97% of the cases also have associated cardiac anomalies complicating their treatment. Because the median age at death in untreated cases is 10 days, this condition usually occurs as a complicated neonatal surgical emergency. There are three types of IAA: Type A is interrupted distal to the left subclavian artery. Type B, which is the most common form, is interrupted between the left common carotid and the left subclavian arteries. Type C, which is the most uncommon type, is interrupted between the innominate and left common carotid arteries. Since the first patient with this congenital anomaly was treated at our institution in 1965, 39 patients have undergone surgical treatment. Two of these (4.6%) had no associated intracardiac lesions, and both survived bypass grafting without complications. There were 12 Type A (30%), 26 Type B (67%) and one Type C (3%). A variety of individualized techniques were used to repair the defects. The three basic techniques were (1) direct aortic to aortic anastomosis bridging the gap, (2) "turndown" or "turnup" of one of the arch vessels to the aorta across the gap, or (3) bypass of the interruption with graft material. Because aortic arch and associated cardiac anomalies represent a neonatal surgical emergency, our therapeutic plan consists of treatment for biventricular failure and PGE(1) infusion to maintain ductal patency, and surgical correction as soon as feasible.     

Interruption of aortic arch in adults: surgical experience with extra-anatomic bypass

We reviewed our 3-year experience in treating interruption of the aorta in adult patients. Clinical profiles, surgical management, and results of early and mid-term follow-up are presented. From August 2001 through June 2003, 7 adult patients underwent an extra-anatomic bypass procedure to repair interruption of the aortic arch. Five patients underwent ventral aortic repair through a mid-sternotomy and an upper midline laparotomy, and 2 patients underwent repair through a left posterolateral thoracotomy. A bovine collagen-impregnated polyester fiber graft was used in 6 patients, and a Gore-Tex graft was interposed in 1 patient. All repairs were performed without cardiopulmonary bypass. Follow-up was complete in all patients. The mean follow-up was 1728 +/- 1 months (range, 9-31 months). No neurologic, renal, or gastrointestinal complications were noted in any patient. There was no in-hospital or late mortality or need for re-intervention. All patients were asymptomatic; however, 5 patients had mild residual hypertension. Graft patency in all the patients was confirmed by computed tomographic angiography. Interruption of the aorta is rare in adults. Ventral aortic repair through a midline approach is our preferred technique for surgical repair of this entity, because it avoids the extensive network of collateral vessels on the chest wall, enables simultaneous treatment of associated lesions, and in all likelihood reduces morbidity and mortality.     

Interrupted aortic arch: A misdiagnosed cause of hypertension

We present the case of a 47-year-old man with hypertension for over 20 years, referred to our hospital due to mild aortic dilatation detected on a transthoracic echocardiogram. On physical examination weak lower limb pulses and a blood pressure differential of >50 mmHg between arms and legs were detected. Complete interruption of the aortic arch below the left subclavian artery was diagnosed by computed tomography angiography. With this case we aim to draw attention to aortic coarctation and interrupted aortic arch as potential causes of hypertension and to highlight the importance of the physical examination in the diagnosis of secondary causes of hypertension.     

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.