Perianeurysmal retroperitoneal fibrosis: An unusual cause of renal failure

Retroperitoneal fibrosis causing ureteral obstruction in association with an abdominal aortic aneurysm has been reported infrequently. However, the clinical presentation of patients with this entity and the histopathologic findings at surgery are similar to those in patients with idiopathic retroperitoneal fibrosis. We describe a patient with perianeurysmal fibrosis and bilateral ureteral obstruction who presented with severe renal failure. The diagnosis of an abdominal aortic aneurysm with perianeurysmal fibrosis was made only at the time of surgery to repair bilateral ureteral obstruction. Previous case reports of perianeurysmal fibrosis are reviewed, and possible pathogenetic mechanisms are discussed. It is important to consider the presence of an occult abdominal aortic aneurysm in patients suspected of having retroperitoneal fibrosis because of the serious prognostic and therapeutic implications.     

Congenital tricuspid incompetence simulating pulmonary atresia with intact ventricular septum: a report of two cases.

Two infants with isolated congenital tricuspid incompetence appeared to have associated right ventricular outflow tract obstruction at diagnostic evaluation, including catheterization and cineangiography. One infant died during surgery; the other improved rapidly and survived with medical therapy. We discuss the problem of establishing the presence of patency of the right ventricular outflow tract in infants with massive tricuspid incompetence and outline an approach to the management of infants whose diagnosis remains unclear even after careful evaluation.     

Idiopathic pulmonary trunk aneurysm causing airway obstruction in an infant

We report a case of idiopathic pulmonary trunk aneurysm in an infant, in which the main clinical picture was airway obstruction. Aneurysmorraphy was the chosen surgical approach and showed to be successful on 13 months follow-up.     

Left ventricular aneurysm without coronary arterial obstruction or occlusion

The most common cause of left ventricular aneurysm is severe obstruction or occlusion of a major coronary artery. Among the last 2,500 patients studied in our laboratory with selective coronary cinearteriography and left ventriculography, we found 9 with unquestionable evidence of left ventricular aneurysm and no or minimal coronary arterial obstruction. Eight of the nine patients had a history of previous myocardial infarction and classic electrocardiographic changes. We believe that in these patients coronary arterial obstruction had led to the development of left ventricular aneurysm. Later, complete or partial regression of the coronary obstruction ensued, leaving a normal or a minimally compromised vascular lumen.     

Polyarteritis nodosa of the coronary arteries: Report of a case in an infant with rupture of an aneurysm and intrapericardial hemorrhage

A case of polyarteritis nodosa in a white female infant 9 months old is reported. The process was confined to the coronary arteries and was complicated by rupture of an aneurysm with subsequent intrapericardial hemorrhage.     

A mediastinal tumor simulated by a sacculated aneurysm of the azygos vein

A rare case of a true idiopathic aneurysm of the azygos vein is reported. A 54 year old female with signs of obstruction of the superior caval vein was submitted to a chest X-ray-examination. It showed a giant mass in the right chest with a diameter of 12 cm. Mediastinal phlebography, CT and NMR-scans of the thorax yielded an aneurysmatic tumor, which seemed to originate from the superior caval vein near to the right atrium. With the clinical diagnosis of an aneurysm of the superior caval vein a right thoracotomy was carried out. Intraoperatively we found a sacculated aneurysm of the azygos vein near to the superior caval vein. Despite of extensive, clinical and radiographic investigations no cause for the aneurysm could be detected. This is one of the very rare cases of congenital aneurysm of the azygos vein.     

Echocardiographic evidence of outflow tract obstruction in Pompe''s disease (glycogen storage disease of the heart)

A 7 month old black female infant with the clinical findings of Pompe's disease is presented. The diagnosis of an infiltrative myocardiopathy with left ventricular outflow tract obstruction presenting with a pronounced systolic anterior motion of the anterior mitral valve leaflet was made by echocardiography. This diagnosis was confirmed by cardiac catheterization and angiocardiography. Pathologic findings were consistent with Pompe's disease (type II glycogen storage disease). The presence of systolic anterior motion of the mitral valve in this patient suggests that this finding is not pathognomonic of idiopathic hypertrophic subaortic stenosis.     

Aneurysm of the nonpatent ductus arteriosus

Clinical and necropsy findings are described in a 6 week old infant with aneurysm of the ductus arteriosus. It was obliterated at the pulmonary arterial end and patent at the aortic end. Observations in this case and in 60 previously described cases of ductal aneurysm disclosed that 46 of the aneurysms occurred in infants less than 2 months old, 4 in children, and 11 in adults. The aortic end of the ductal aneurysm is always patent. The pulmonary arterial end may or may not be patent. Since complications (rupture, embolism or infection) tend to develop in nearly half of the ductal aneurysms, operative resection appears indicated.     

Two-dimensional echocardiographic recognition and repair of subvalvular mitral aneurysm of the left ventricle in an infant

The clinical features, diagnostic studies and surgical treatment of a subvalvular mitral aneurysm of the left ventricle are described. The infant presented at 9 weeks of age with large apical ventricular septal defects and pulmonary hypertension. The subvalvular aneurysm was an incidental finding. Both lesions were treated surgically.     

Respiratory insufficiency caused by an aneurysm with multiple vascular lesions

A 71-year-old woman, who presented tracheobronchial obstruction caused by a thoracic aortic aneurysm, was admitted to our institution. Although she had multiple cerebral infarctions, old myocardial infarction, bilateral iliofemoral atherosclerotic lesions with abdominal aortic aneurysm, and superior vena cava syndrome, aneurysmectomy was undertaken in order to rescue her from respiratory insufficiency. The operation successfully relieved her of exertional dyspnea and dysphagia.     

Accidental detection of a giant right atrial aneurysm in an asymptomatic infant

We present a 4-month old infant boy accidentally detected to have cardiomegaly on a chest radiograph, and on further investigation discovered to have a giant right atrial aneurysm and a secundum atrial septal defect. Congenital right atrial aneurysm or diverticulum is a rare anomaly, usually presenting with atrial or supraventricular tachycardia. Previously reported patients did not have any associated congenital heart defect. Although asymptomatic our patient needs close follow-up, and surgical resettion of the aneurysm and closure of the atrial septal defect in view of potential complications.     

Left ventricular outflow tract obstruction due to anomalous mitral valve: successful mitral valve replacement in a four month old infant.

A four month old infant was investigated for heart failure was found to have mitral incompetence and severe subvalvar aortic stenosis. The left ventricular outflow tract obstruction was found to be due to an anatomically anomalous mitral valve. The obstruction could only be relieved by removal of the mitral valve and its replacement with a St Jude's prosthesis. Two years after operation the child is fit and active. There have been no difficulties with anticoagulant treatment.     

Left ventricular outflow tract obstruction due to anomalous mitral valve: successful mitral valve replacement in a four month old infant

A four month old infant was investigated for heart failure was found to have mitral incompetence and severe subvalvar aortic stenosis. The left ventricular outflow tract obstruction was found to be due to an anatomically anomalous mitral valve. The obstruction could only be relieved by removal of the mitral valve and its replacement with a St Jude's prosthesis. Two years after operation the child is fit and active. There have been no difficulties with anticoagulant treatment.     

Prolapsed left atrium behind the left ventricular posterior wall: Two dimensional echocardiographic and angiographlc features

Left atrial enlargement can usually be detected accurately using M mode echocardiography. However, in the presence of heart disease, asymmetric enlargement may lead to inaccurate assessment of left atrial size and shape. Pericardial effusion can usually be diagnosed on the basis of characteristic M mode echocardiographic findings. However, false positive patterns sometimes occur with the use of this single dimensional technique. Three patients with a greatly enlarged left atrium are described whose M mode echocardiogram suggested significant posterior pericardial fluid accumulation. In each patient, two dimensional echocardiography detected portions of a huge left atrium that prolapsed behind the left ventricular posterior wall and mimicked an isolated posterior pericardial effusion. In one case a right anterior oblique left ventricular cineangiogram suggested the presence of a ventricular septal defect or a false aneurysm of the left ventricle due to the prolapsed left atrium. Because two dimensional echocardiography can provide accurate spatial orientation with visualization of intracardiac structures in relation to one another in real time, it can identify the presence of left atrial prolapse and play an important role in the differential diagnosis of isolated echo-free spaces behind the left ventricle detected with M mode echocardiography.     

Duodenal Obstruction by Abdominal Aortic Aneurysms

We report a case of duodenal obstruction caused by an abdominal aortic aneurysm. The duodenum was firmly attached to the lateral wall of the aneurysm by a tough, fibrous band. Duodenal obstruction is a rare presenting complaint in a patient with an abdominal aortic aneurysm. Anatomic features have not been routinely described in previous patients. We propose a mechanism for the cause of this entity and a review of the literature. The treatment of choice is resection of the aneurysm with graft replacement of the aorta.     

Interventional cardiac catheterization therapy for combined coarctation of the aorta and patent ductus arteriosus: Successful outcome in two infants

We report two infants each with coarctation of the aorta and small patent ductus arteriosus who had balloon angioplasty therapy for aortic obstruction at 3–6 months of age followed by anterograde placement of a single Gianturco coil to close the ductus at a separate catheterization. One infant developed recoarctation that resolved with repeat angioplasty prior to closure of the ductus. Follow-up evaluations have demonstrated a trivial aortic gradient, no aortic aneurysm, and no residual shunt. © 1996 Wiley-Liss, Inc.     

ECTOPIC PANCREAS AND CYSTIC FIBROSIS

The case is reported of an infant with cystic fibrosis and duodenal obstruction relieved by surgery and caused by an affected submucosal ectopic pancreas in the third part of the duodenum.     

Childhood traumatic infarction causing left ventricular aneurysm: diagnosis by two-dimensional echocardiography

After being struck by an automobile, a 9 year old boy developed transient right bundle branch block followed by electrocardiographic changes of inferior wall myocardial infarction and an increase of serum cardiac enzymes. Two-dimensional echocardiography demonstrated a prominent septal aneurysm. Subsequent cardiac catheterization confirmed septal aneurysm and demonstrated an additional inferior aneurysm. Six weeks after the accident, resection of the inferior aneurysm was performed. At 1 year follow-up study, residual septal aneurysm and cardiomegaly are present, but the boy is asymptomatic.     

Endocarditis with aneurysm involving an aortic homograft used to correct a truncus arteriosus: medical-surgical salvage.

An aneurysm of an aortic homograft conduit, used to correct a type I truncus arteriosus anomaly in a four month old infant, developed when the patient was 15. Blood cultures grew Staphylococcus aureus. The aneurysm was detected by magnetic resonance imaging and digital subtraction angiocardiography. An emergency open heart operation, guided by these investigations, was performed to remove the original homograft and replace it with another valved aortic homograft. Postoperative antibiotic treatment had to be stopped when profound neutropenia developed. This responded to treatment with recombinant human granulocyte colony stimulating factor. Three years later she was symptom free and did not require medication. Chest x rays and echocardiograms showed a normally functioning heart and conduit valve.