Current outcomes of one-stage surgical correction for Berry syndrome

Objectives

Berry syndrome is a combination of distal aortopulmonary window (APW), aortic origin of the right pulmonary artery (RPA), intact ventricular septum, and interrupted aortic arch. We present here our current experience of primary repair of this syndrome with the goal of optimizing treatment for this rare condition.

Mirror image Berry syndrome: a case report of complex aortopulmonary malformation in viscero-atrial situs inversus

We report a very rare case of an infant with viscero-atrial situs inversus presenting mirror image Berry syndrome (aortopulmonary window, interrupted aortic arch, anomalous origin of the left pulmonary artery, and intact ventricular septum). Successful surgical treatment was accomplished 56 days following the initial rescue bilateral pulmonary artery banding.     

Occlusion of left coronary artery ostium by an aortic valve cusp

Congenital anomalies of the aortic valve can be associated with other cardiac anomalies. In this report, we present a patient with an aortic valve anomaly associated with occlusion of left coronary ostia. In addition, we reviewed the literature and found 10 similar cases. Although compatible with life, this anomaly can lead to significant symptoms. Preoperative diagnosis as well as proper therapeutic planning should be tailored to correct valvular competence and restore coronary blood flow.     

Left inferior vena cava and aneurysm of the abdominal aorta

A case of isolated left-sided inferior vena cava, without situs inversus, in a patient with abdominal aortic aneurysm is reported. On the basis of a review of literature, the authors recall the incidence and the embryology of this anomaly, and analyze its surgical implications, with particular reference to abdominal aortic surgery.     

Unicuspid aortic valve in a middle-aged man: a rare case report

Unicuspid aortic valve is a rare congenital anomaly. Two forms of unicuspid aortic valve exist: acommisural and unicommisural forms, both with a predisposition to aortic dilatation. Most patients with this congenital abnormality will eventually require therapeutic intervention for resulting severe aortic stenosis during infancy or childhood. In adults, the most common treatment applied is aortic valvular replacement associated with replacement of the aortic root and ascending aorta. We describe a 43-year-old man admitted to our hospital complaining of chest pain with the diagnosis of acute coronary syndrome. Transthoracic and transesophageal echocardiography showed a unicommisural unicuspid aortic valve associated with an aneurysmally dilated ascending aorta. The patient underwent a Bentall operation and was discharged uneventfully.     

Congenital kinking of the aortic arch associated with aortic arch aneurysm: a case report

A surgical case of congenital kinking of the aortic arch associated with aortic arch aneurysm, which had been observed as the mediastinal tumor for 15 years, was reported. Twenty-two surgical cases of the aortic lesion, which were diagnosed pseudocoarctation or congenital kinking, have been reported in the literature. This paper focused on the pathogenesis, surgical treatment and terminology of this anomaly.     

Ebstein's anomaly of tricuspid valve with aortic stenosis and coarctation of aorta: Successful single-stage repair of a rare adult congenital heart disease

Ebstein's anomaly of the tricuspid valve is infrequently associated with left heart anomalies. The association of aortic stenosis in Ebstein's anomaly has been reported to be extremely rare and the association of coarctation of aorta is even rarer especially in adults. The combination of all three of these lesions is virtually unknown without any references in literature. We report here an unusual case of Ebstein's anomaly of the tricuspid valve and severe aortic stenosis with coarctation of aorta in an adult who presented to us with exertional dyspnoea in the third decade and underwent a successful single-stage intracardiac repair.     

Anomaly of the aortic arch: A case report

A case of the anomaly of the aortic arch was presented. The case showed subclavian steal syndrome due to the hypoplastic left subclavian artery associated with the double aortic arch with the obliterated left aortic arch. The hypoplastic left subclavian artery was replaced with the Dacron tube graft and the obliterated left arch was divided. Subclavian steal disappeared and the patient is in good condition at the present time.     

Dissecting thoracoabdominal aortic aneurysm associated with an isolated right-sided aortic arch.

Although a right-sided aortic arch is not a rare anomaly, an aortic dissection involving an isolated right-sided aortic arch is extremely rare and remains a complicated entity for surgical therapy because of its anatomical characteristics. Previous reports that we have identified in English literature include only six surgical cases of aortic dissection involving a right-sided aortic arch. We report on a 75-year-old female who had a chronic thoracoabdominal aortic aneurysm following type B aortic dissection in a right-sided aortic arch. Graft replacement including reconstruction of Adamkiewicz artery and a celiac trunk was performed. The postoperative course was uneventful except for a prolonged ventilatory support. This case seems to be the first successful case of extended graft replacement for this pathology.     

Endovascular repair of a right-sided descending thoracic aortic aneurysm with a right-sided aortic arch and aberrant left subclavian artery

Aneurysms involving a right-sided aortic arch and a right-sided descending thoracic aorta with an aberrant origin of the left subclavian artery are rare. We describe the successful surgical repair of this vascular anomaly by the combined use of a left carotid to subclavian artery bypass followed by endovascular stent-graft placement to exclude the aortic aneurysm. We also review the literature associated with this particular anatomic presentation.     

Shone-Anomalie

Shone's anomaly was first described in 1963 as a developmental complex of four potentially obstructive cardiac lesions including a supravalvular fibrous mitral ring, deformity of the mitral and/or subvalvular apparatus, subvalvular aortic stenosis and coarctation of the aorta. While paediatric patients with Shone's anomaly have been reported in the literature, only a few adult patients presenting with this anomaly have been described in the perioperative period. However, patients with an undiagnosed, incomplete form of Shone's anomaly might occasionally present for non-cardiac surgery as adults. In this case report we describe the anaesthetic management of an adult patient scheduled for a non-cardiac operation, who suffered from Shone's anomaly that was unrecognised prior to the operation.     

Ruptured thoracoabdominal aortic aneurysm in a renal transplant patient with Alport's syndrome

Alport's syndrome is a rare genetic disorder of type IV basement membrane collagen synthesis that typically presents with nephropathy, deafness, and ocular abnormalities. To the best of our knowledge, this is the first report in the world's literature of ruptured thoracoabdominal aortic aneurysm in a young patient with Alport's syndrome and a renal transplant. Hypotheses on an association between collagen disease in Alport's syndrome and aortic aneurysms are discussed.     

Ruptured aortic dissecting aneurysm in Turner's syndrome: a case report and review of literature.

Cardiovascular malformations are frequently observed in Turner's syndrome. Bicuspid aortic valve and coarctation of the aorta are commonly associated with Turner's syndrome whereas aortic dissection is rare but its rupture results in death. We experienced a case of ruptured dissecting aneurysm (Stanford type A) in a 30-year-old female with Turner's syndrome. Emergent total arch replacement was performed successfully. A literature review revealed 32 cases of aortic dissection in patients with Turner's syndrome, including 15 cases of rupture. However, survival after rupture was reported only two cases. To our knowledge, this report descries the third known case of successful surgical management of ruptured aortic dissection in Turner's syndrome.     

Thrombotic occlusion and late lysis in the arteria subclavia lusoria dextra]

Case history of a 45 years aged male patient with an aortic arch anomaly. Thrombotic occlusion of the A. lusoria dextra with symptoms of a subclavian-steal syndrome and anoxia in the right hand. Spontaneous lysis after 8 weeks is documentated by arteriography.     

Abdominal aortic aneurysm associated with crossed renal ectopia without fusion: case report and literature review

Genitourinary anomalies can present a formidable challenge to the vascular surgeon at abdominal aortic reconstruction. We saw a case of crossed renal ectopia without fusion, a rare anomaly, associated with abdominal aortic aneurysm. Because of risk for injury to the kidney during surgery, preoperative evaluation of this anomaly must include computed tomography, angiography, and intravenous pyelography. Preoperative placement of a ureteral catheter may prevent injury to the anomalous ureter. Renal failure of the ectopic kidney during aortic reconstruction can be a serious problem. We used in situ hypothermic perfusion with cold (4 degrees C) Ringer solution for renal protection, and reimplanted the aberrant renal artery. The postoperative course was good, without major complications. The procedure for renal preservation must be selected on the basis of anatomic findings. We review the literature and present the first case of crossed renal ectopia.     

Diverticula in association with the proximal left subclavian artery: a cause of mediastinal mass lesions.

The case reports of 5 patients with an aortic arch diverticulum associated with the proximal left subclavian artery are presented. In 3 patients, the left subclavian artery arose from a diverticulum off a right aortic arch and traversed the retroesophageal area to the opposite arm. In 1 patient, the left subclavian artery arose from a diverticulum off a left aortic arch; this represents the second instance of this anomaly to be reported in the literature. In 1 patient, the left subclavian artery was not attached to the aorta, and a diverticulum was present in the expected area of attachment. Confusion of these anomalies with solid mediastinal mass lesions and with aortic aneurysms may occur. Treatment is predicted on symptomatology, and resection is usually not indicated. It is necessary to remain cognizant of aortic diverticula and to differentiate them from other mediastinal mass lesions.     

Aortic dissection of Kommerell's diverticulum in Marfan's syndrome.

We report a surgical case of chronic aortic dissection with an entrance tear on Kommerell's diverticulum, a rare embryologic anomaly, with an extension to the arteria lusoria, in a patient with Marfan's syndrome. The operation consisted of aortic root and arch replacement with reimplantation of the right subclavian artery in the right carotid artery under circulatory arrest and deep hypothermia.     

Fibromuscular hyperplasia in an aberrant subclavian artery and neurogenic thoracic outlet syndrome: An unusual combination

An aberrant right subclavian artery arising distal to the origin of the left subclavian artery is the most common anomaly of the aortic arch. Degenerative diseases of aberrant subclavian arteries including aneurysms and occlusive disease have been reported previously. We believe that this case is the first reported case of fibromuscular hyperplasia affecting an aberrant subclavian artery. A 25-year-old woman admitted with a history consistent with neurogenic thoracic outlet syndrome was found to have a reduced pulse and blood pressure on the ipsilateral side caused by fibromuscular hyperplasia of an aberrant subclavian artery. A carotid-subclavian bypass via a supraclavicular incision was performed at the same time as thoracic outlet decompression. Histologic examination confirmed the presence of fibromuscular hyperplasia in the aberrant subclavian artery. This case is discussed with reference to the available literature. (J VASC SURG 1994;20:834-8.)     

Persistent fifth aortic arch left subclavian aneurysm in an adult

Persistent fifth aortic arch is so rare an anomaly that only seven cases have been reported. We treated an adult with this anomaly in which there was a single lumen aortic arch, single arterial trunk and left subclavian aneurysm. The patient underwent corrective surgery. From an analysis of the eight cases, this anomaly can be divided into two groups, depending on the aortic arch. The first group of six had double lumen aortic arch. All in this group had intra and/or extra cardiac anomalies, and only two with only PDA underwent ligation. The second group of two had a single lumen aortic arch and corrective surgery was done. Thus, those patients with a persistent fifth aortic arch should be classed into the first or second group, according to the angiographical findings.     

Surgical Treatment of Cardiovascular Complications in Patients with Marfan Syndrome: a Report of two Cases and Literature Review

Cardiovascular disease is the main cause of morbidity and mortality in patients with Marfan syndrome. The most life threatening complication is aortic root aneurysms leading to aortic dissection or rupture. It can be prevented by regular aortic follow-up and prophylactic aortic surgery. Modern aortic surgery has led to a substantial increase in the life expectancy of these patients. We report two cases of Marfan syndrome with cardiovascular complications. Their management is discussed according to the most recent literature.