Recoarctation after fracture of stent implanted for native aortic coarctation: case report.

We report the case of a 20-year-old patient with severe arterial hypertension in whom coarctation of the aorta was diagnosed. She underwent cardiac catheterization with balloon dilation and stent implantation, with a good initial result. After this procedure she improved and her blood pressure was under control for six months. Her condition then worsened and she developed signs of recoarctation, with an increased Doppler pressure gradient in the descending aorta. On recatheterization a stent fracture was detected and treated with percutaneous implantation of a covered stent inside the previous one, with sustained improvement. Stent fracture is a rare complication of stent implantation in the aorta. Several etiological hypotheses and treatment options are reviewed.

Adolescent with neurological manifestations of cardiac cause treated surgically.

Cardiac myxomas are a rare but important cause of embolic events and cerebrovascular disease in children and adolescents. The authors report the case of a 12-year-old girl with sudden dizziness and right-sided paresthesia of the lips and fingers of the right hand. She was observed by a pediatric neurologist and brain magnetic resonance imaging suggested demyelinating lesions or vasculitis. Echocardiography showed a left atrial mass compatible with an atrial myxoma. The myxoma was surgically removed without complications. This case highlights the need for cardiac evaluation to rule out a cardiac tumor with embolic potential in the presence of any child with neurological manifestations of cerebrovascular disease with inconclusive brain imaging, even with no previous evidence of cardiac disease. Prompt clinical recognition and surgical removal are essential to prevent serious complications.

Primary cardiac tumors in children: a 16-year experience.

INTRODUCTION: Primary cardiac tumors are rare in children. The majority are benign and noninvasive, although they may have significant hemodynamic consequences depending on their location. The most frequent type in children is rhabdomyoma, usually multiple and intraventricular. OBJECTIVES: To assess the presentation and outcome of patients with cardiac tumors followed by the Department of Pediatric Cardiology of Hospital de Santa Cruz. METHODS: A retrospective analysis was performed of the clinical records of patients diagnosed with primary cardiac tumors between January 1992 and March 2008. RESULTS: Eleven children with a diagnosis of primary cardiac tumor were identified, three of them detected antenatally. In the remaining cases, median age at diagnosis was 27.5 months, ranging from two days to 13 years. Seven (64%) were male. In three cases diagnosis was made during fetal echocardiography, and in the others by transthoracic echocardiogram. In six patients magnetic resonance imaging was used to confirm the diagnosis. All were benign (eight rhabdomyomas, one papillary fibroelastoma, one myxoma and one pericardial tumor with histological features of Castleman disease). Six patients were asymptomatic at the time of diagnosis; two cases presented with arrhythmias; one with heart failure; and two were diagnosed following stroke, one with right hemiparesis, and the other with paresthesias of the lip and fingers of the right hand. In four cases there was total or partial tumor regression. Surgery was performed in four patients, due to tumor location and/ or significant left ventricular outflow tract obstruction. One of these patients died following surgery due to multiorgan system failure. FINAL REMARKS: Primary cardiac tumors are uncommon in children. Their treatment depends mainly on location and histological type. The most serious complications are ventricular outflow tract obstruction and thromboembolic events, which are associated with a poor prognosis even when surgery is attempted.

[DiGeorge syndrome and vascular ring. An unusual association with multidisciplinary approach]. / Síndrome de DiGeorge e anal vascular. Associação invulgar e abordagem multidisciplinar.

Velo-cardio-facial syndrome/DiGeorge/CATCH 22 is a spectrum of association, characterized by unusual face, cleft or incompetent palate, congenital heart disease with defects of the outflow tracts, absence of the thymus and parathyroid glands, often associated with developmental and behavioral disorders. This association is caused by a microdeletion in chromosome band 22q11.2. In a 4-month-old infant, with obstructive lower respiratory distress and poor weight gain since 2 months of age, truncus arteriosus was diagnosed and surgically corrected. On the postoperative period maintained dependency on mechanical ventilation, with persistent hypoventilation of the left lung. Fiberoptic bronchoscopy revealed complete obstruction of the left main bronchus by an extrinsic compression due to a vascular ring diagnosed by cardiac catheterization that showed a common anomalous origin of both right and left subclavian arteries and the ligamentum arteriosum. A second surgery by left lateral thoracotomy corrected the vascular ring. The maintenance of the collapse of the left main bronchus led to selective endobronchial stenting. The migration of the stent to the trachea, with acute respiratory distress, required emergent endoscopic removal of the stent. Thereafter, the evolution was uneventful. The association of DiGeorge syndrome with vascular ring is unusual. Unexpected evolution in these patients require a multidisciplinary technical approach for diagnosis and eventual emergent intervention.

Transcatheter closure of a fenestration in intracardiac Fontan circulation.

We present a case of tricuspid atresia and the treatment that was used in a child now aged 12. The malformation was corrected at the age of five through intracardiac Fontan circulation. Later, the child developed a right-to-left shunt due to dehiscence in the intra-atrial conduit, causing significant desaturation. This was treated by transcatheter closure with a 10 mm Amplatzer device at 8 years of age. Currently the child is asymptomatic with no residual shunts, and the position of the device is normal. The percutaneous closure of Fontan fenestrations using the Amplatzer device is a therapeutic approach that provides good results. It results in normal oxygen saturation and has low morbidity. The technique is simple, available to most cardiac catheterization laboratories, safe, and offers a reasonable cost-benefit ratio. In addition, the design of the device is suitable for this type of defect.

Aortopulmonary window--a review of nine cases.

INTRODUCTION: Aortopulmonary window (APW) is a rare anomaly, accounting for 0.1% of congenital heart defects. It consists of a communication between the ascending aorta and the pulmonary artery in the presence of normal separate aortic and pulmonary valves. Early treatment is usually required in order to prevent the development of irreversible pulmonary hypertension. OBJECTIVE: To assess the results of treatment in all patients diagnosed at our institution between January 1994 and November 2007, based on a retrospective longitudinal study. RESULTS: Nine patients treated for APW were identified. Their ages at diagnosis ranged from two days to 23 years; eight were infants aged 2 +/- 2.9 months. In this group clinical presentation was congestive heart failure in all cases. Five patients had associated lesions (interrupted aortic arch: 2; coarctation of the aorta: 2; VSD: 1; ASD: 3). In seven cases the diagnosis was made on the basis of echocardiography only. In the other two it was by cardiac catheterization, one patient with coarctation of the aorta and the other an adult patient with a smaller lesion who was initially misdiagnosed as having ductus arteriosus. All patients had corrective surgery, via a transaortic approach, with implantation of an autologous pericardial patch. All patients are alive and only one case has a small residual shunt at the correction site. CONCLUSION: A diagnosis of APW should always be kept in mind in the differential diagnosis of a child with congestive heart failure. Associated congenital heart anomalies should be excluded. Surgical repair appears to provide good short- and long-term results.

Treatment of aortic aneurysm with covered stent. Case report.

The authors present the case of a thirteen-year-old boy with aortic coarctation, which was surgically corrected at the age of 2.5 months. He subsequently developed recoarctation, which was treated with balloon dilatation. Recently, recoarctation and an aortic aneurysm were observed, which were corrected with covered stent implantation. We discuss various techniques for correction of aortic coarctation, as well as their advantages and disadvantages.

Intracranial aneurysms as the first sign of coarctation of the aorta.

The case of a twenty-seven-year old man with coarctation of the aorta who suffered rupture of cerebral aneurysms is presented. Treatment was successful for both lesions.

Systemic-to-pulmonary blood supply in Tetralogy of Fallot with pulmonary atresia.

Tetralogy of Fallot with pulmonary atresia is one of the most challenging congenital cardiac malformations, for the morphologist, cardiologist and surgeon alike. Much of the difficulty in this lesion concerns the nature and development of pulmonary arterial supply, and the manner in which complete segmental supply to the lungs can be successfully restored or maintained. In this review, we discuss the anatomy and nomenclature of the lesion, emphasising the variability that can occur in pulmonary arterial anatomy, particularly in the presence of systemic-to-pulmonary collateral arteries. We speculate on the likely embryologic origins of these connections. Then by means of anatomic-clinical correlations, we emphasise the diagnostic approach to delineating the origin and extent of the pulmonary vasculature.