Ana Teixeira

Percutaneous closure of atrial right-to-left shunt in patients with Ebstein's anomaly of the tricuspid valve

AIMS Patients with Ebsteins anomaly of the tricuspid valve may have right-to-left shunt at atrial level resulting in hypoxaemia, high haematocrit and hyperviscosity syndrome. The purpose of this study was to assess the results of percutaneous closure of atrial right-to-left shunt in patients with Ebsteins anomaly. METHODS AND RESULTS Records of patients treated between January 2002 and June 2010 were reviewed. Their condition before and after shunt closure (clinical data, oxygen saturation and haematocrit) were studied. During this period nine selected patients with Ebsteins anomaly and right-to-left shunt at atrial level were treated. Ages ranged from six to 67 years; seven were male. Mean pulmonary artery pressures were under 25 mmHg in all. Three patients had previous episodes of stroke and three had very high haematocrit, two of whom required therapeutic phlebotomies. Test occlusion of the shunt was performed in all patients with a balloon catheter, revealing an increase in systemic oxygen saturation, with right atrial pressures remaining <18 mmHg in all. Percutaneous closure of atrial shunt was achieved in all. There were no major complications. Arterial oxygen saturations increased in all patients from 85.0 ± 4.5% to 96.7 ± 1.5% (mean ± standard deviation). Medium follow-up was five years. The three patients with very high haematocrit levels had a decrease in its values from 62.9 ± 2.8% to 45.5 ± 3.9% after device occlusion. Both therapeutic phlebotomy programs were discontinued. All patients reported a marked improvement in effort tolerance. CONCLUSIONS Percutaneous closure of atrial right-to-left shunt in selected patients with Ebsteins anomaly offers significant improvement, abolishing hypoxaemia and hyperviscosity and preventing paradoxical embolisation.

Tetralogy of Fallot with absent pulmonary valve

of life due to hypoxemia and grade IV/VI systolic murmur. She was diagnosed remotely with tetralogy of Fallot with absent pulmonary valve on the first day of life and was transferred to Hospital Santa Cruz with body weight of 3.695 kg (<5th percentile) for surgical correction of cyanotic heart disease. She was slightly pale and markedly polypneic, with systolic and diastolic murmurs on the left sternal border, but no other relevant alterations on physical

Tetralogia de Fallot com agenesia da válvula pulmonar

of life due to hypoxemia and grade IV/VI systolic murmur. She was diagnosed remotely with tetralogy of Fallot with absent pulmonary valve on the first day of life and was transferred to Hospital Santa Cruz with body weight of 3.695 kg (<5th percentile) for surgical correction of cyanotic heart disease. She was slightly pale and markedly polypneic, with systolic and diastolic murmurs on the left sternal border, but no other relevant alterations on physical

Ressincronização cardíaca em doente com transposição congenitamente corrigida das grandes artérias

Congenitally corrected transposition of the great arteries is a rare heart defect that can be associated with systemic ventricular dysfunction and conduction disturbances. The use of cardiac resynchronization therapy in patients with congenital heart disease is not fully established. The authors report a case of cardiac resynchronization therapy in a 31-year-old woman with a history of congenitally corrected transposition of the great arteries and heart failure, who had undergone two heart surgeries and had a DDDR pacemaker implanted. To our knowledge this is the first case reported in Portugal of cardiac resynchronization therapy by transvenous access in a patient with congenitally corrected transposition of the great arteries.

Embolização percutânea de fístulas coronárias – Experiência de um centro

INTRODUCTION A coronary fistula is a connection between one of the coronary arteries and a cardiac chamber or great artery. It is a rare defect and usually occurs in isolation. Two-dimensional echocardiography has an important role in diagnosis but coronary or CT angiography is essential to delineate the anatomy. Surgery is the traditional therapeutic approach but percutaneous closure is now the recommended method, with excellent results and few complications in experienced centers. METHODS We describe our experience with percutaneous treatment of 15 coronary fistulas in 12 patients between 1996 and 2011. Eight (67%) were male and median age was 25 years. The most frequent symptoms were murmur and/or fatigue. All fistulas were congenital. Five patients (42%) had concomitant cardiac disease: pulmonary atresia with intact ventricular septum (1), patent ductus arteriosus (1), ostium secundum atrial septal defect (1), stenotic bicuspid aortic valve (1), and critical pulmonary stenosis operated in the neonatal period (1). Three patients had two fistulas, while the others had a single lesion. All fistulas were hemodynamically significant. They originated in the territory of the right coronary (10), left coronary (3) and circumflex (2), draining into the right ventricle (5), pulmonary artery (6), right atrium (2) coronary sinus (1) and left ventricle (1). Embolization materials included standard coils, controlled-release coils, microcoils (standard, GDC or IDC) and an Amplatzer(®) duct occluder. RESULTS Embolization was achieved in all patients. There was no mortality. One patient with a large fistula and a very small right coronary artery distally to the origin of the fistula had a right ventricular infarction. In three patients there were minor complications: inadvertent coil embolization, recovered in the same procedure (1), transient arrhythmia (1) and femoral pseudo-aneurysm (1). In a mean follow-up of 4.9 years (one month to 14 years), there were no procedure-related complications. Echocardiographic and/or angiographic control showed complete and permanent occlusion in ten patients and minimal residual flow in two patients through small collaterals with no hemodynamic significance. CONCLUSION Percutaneous embolization represents an effective form of treatment for selected coronary fistulas. A wide range of embolization devices must be available to ensure the best therapeutic approach.