Graça Nogueira

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.

Enhanced nonsynaptic epileptiform activity in the dentate gyrus after kainate-induced status epilepticus

Understanding the mechanisms that influence brain excitability and synchronization provides hope that epileptic seizures can be controlled. In this scenario, non-synaptic mechanisms have a critical role in seizure activity. The contribution of ion transporters to the regulation of seizure-like activity has not been extensively studied. Here, we examined how non-synaptic epileptiform activity (NEA) in the CA1 and dentate gyrus (DG) regions of the hippocampal formation were affected by kainic acid (KA) administration. NEA enhancement in the DG and suppression in area CA1 were associated with increased NKCC1 expression in neurons and severe neuronal loss accompanied by marked glial proliferation, respectively. Twenty-four hours after KA, the DG exhibited intense microglial activation that was associated with reduced cell density in the infra-pyramidal lamina; however, cellular density recovered 7 days after KA. Intense Ki67 immunoreactivity was observed in the subgranular proliferative zone of the DG, which indicates new neuron incorporation into the granule layer. In addition, bumetanide, a selective inhibitor of neuronal Cl(-) uptake mediated by NKCC1, was used to confirm that the NKCC1 increase effectively contributed to NEA changes in the DG. Furthermore, 7 days after KA, prominent NKCC1 staining was identified in the axon initial segments of granule cells, at the exact site where action potentials are preferentially initiated, which endowed these neurons with increased excitability. Taken together, our data suggest a key role of NKCC1 in NEA in the DG.

Congenital Heart Disease in Children with Down Syndrome: What Has Changed in the Last Three Decades?

INTRODUCTION The prevalence of Down syndrome has increased in the last 30 years; 55% of these children have congenital heart disease. MATERIAL AND METHODS A retrospective longitudinal cohort study; clinical data from 1982 to 2013 databases with the diagnosis of Down syndrome or trisomy 21 in a reference hospital in pediatric cardiology and cardiac surgery. OBJECTIVE to assess the progress in the last three decades of cardiological care given to children with Down syndrome and congenital heart disease. RESULTS We studied 102 patients with Down syndrome and congenital heart disease subjected to invasive therapy: corrective or palliative cardiac surgery and therapeutic catheterization. The referral age was progressively earlier in patients referred in the first year of life. The most frequent diagnosis was complete atrioventricular sptal defect (41%). There was a trend towards increasingly early corrective surgery in patients under 12 months (p < 0.001). Since 2000, the large majority of patients were operated before reaching six months of age. The main cardiac complications were rhythm dysfunction and low output. More frequent noncardiac complications were pulmonary and infectious. The 30-day mortality rate was 3/102 cases (2.9%). Of patients in follow-up, 89% are in NYHA class I. DISCUSSION AND CONCLUSION The early surgical correction seen over the past 15 years follows the approach suggested in the literature. The observed 30-day mortality rate is overlapping international results. Patients with Down syndrome subjected to corrective surgery of congenital heart disease have an excellent long-term functional capacity.

Assistência ventricular esquerda numa criança de 5 anos – ponte para recuperação num caso de miocardite viral

INTRODUCTION Viral myocarditis can lead to heart failure that is refractory to medication. In these cases, a ventricular assist device is a good therapeutic option that can be used as a bridge to transplantation or recovery. We describe the first case in Portugal of recovery with ventricular assistance after severe myocarditis. CASE REPORT A five-year-old boy with no previous cardiac disease presented with severe viral myocarditis, refractory to medical treatment, with positive serology for parvovirus B19 and Ebstein-Barr virus. A Berlin Heart Excor(®) was implanted 15 days after diagnosis. A biopsy at the time of implantation showed subendocardial fibrosis. After 40 days of assistance ventricular function recovered and the device was explanted. The patient was discharged from hospital 15 days later. DISCUSSION Survival of children with ventricular assistance has improved significantly because of earlier implantation and coagulation monitoring. The presence of subendocardial fibrosis in the context of myocarditis is not a mandatory indication for transplantation.

Terapêutica de ressincronização na miocardiopatia induzida pelo pacing ventricular direito em doente pediátrico

A 2-day-old male infant required a conventional VVI pacemaker for congenital atrioventricular block. Three years later, he developed progressive heart failure due to left ventricular (LV) dysfunction and mitral regurgitation despite optimized medical treatment, and a cardiac resynchronization therapy (CRT) device was implanted. This is the first Portuguese report of CRT in a pediatric patient. One-year echocardiographic follow-up showed that LV shortening fraction had improved and LV end-diastolic dimension and mitral regurgitation had decreased. New York Heart Association class had improved from III-IV to I at 1-year follow-up.

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.