José Diogo Ferreira Martins

Percutaneous Occlusion of Vascular Malformations in Pediatric and Adult Patients: 20-Year Experience of a Single Center

A case series on different vascular malformations (VM) treated with percutaneous occlusion in children and adults is presented.

The Role of Propranolol in the Treatment of Infantile Hemangioma

INTRODUCTION Infantile hemangioma (IH) is one of the most common childhood tumors. There are various medical or surgical therapeutic options, all with suboptimal results. Recently, the successful use of propranolol for involution of IH was described. We report the results of a single-center experience with this therapeutic option. OBJECTIVE To prospectively assess the efficacy and safety of propranolol in children with infantile hemangioma. METHODS We performed a prospective analysis of clinical data of all patients with IH referred to a pediatric cardiology center for baseline cardiovascular assessment prior to propranolol therapy. Propranolol was given at a starting dose of 1 mg/kg/day and titrated to a target dose of 2-3 mg/kg/day according to clinical response. Efficacy was assessed through a photograph-based severity scoring scale. Safety was assessed by collecting data regarding significant side effects. RESULTS Starting in 2010, 30 patients (15 female) were referred for propranolol treatment of IH, at a median age of six months (1-63 months). The mean target propranolol dose was 2.8 mg/kg/day, with a mean duration of therapy of 12 months. All patients experienced significant reduction of IH size and volume. There were no side effects. CONCLUSIONS In our experience propranolol appears to be a useful and safe treatment option for severe or complicated IH, achieving a rapid and significant reduction in their size. No adverse effects were observed, although until larger clinical trials are completed, potential adverse events should be borne in mind and consultation with local specialists is recommended prior to initiating treatment.

Initial use of endothelial progenitor cells capturing stents in paediatric congenital heart disease.

INTRODUCTION Stenosis, mediated by neointimal hyperplasia and thrombosis, is a major limiting factor in successful stent implantation. The introduction of a stent, coated in its endoluminal surface by antihuman CD34 antibodies with endothelial progenitor cell-capturing properties, opens the possibility of promoting a rapid and normal functioning coverage by endothelium and thus avoids both an excessive cell proliferation within stent and the need for long-term dual antiplatelet therapy. These stents, developed for adult coronary artery disease, have not yet been implanted in children or in those with congenital heart disease. OBJECTIVE AND METHODS In this paper, we describe the implantation of Genous® stents in three children with cyanotic congenital heart disease and obstructed systemic-to-pulmonary shunts. We describe the use of this stent and address its potential feasibility in paediatric congenital heart disease. RESULTS To maintain the patency of two modified Blalock-Taussig shunts and one ductus arteriosus, four Genous® stents were implanted in three infants with cyanotic heart disease. All procedures were immediately successful, with resolution of stenosis and improvement in transcutaneous oxygen saturation from 66% ± 3.6% to 92% ± 2.6%. In the follow-up, one stent had no occlusion; however, the remaining two had partial occlusion after 5 and 5.5 months, which were successfully managed with balloon dilatation preceding elective definitive surgical correction. CONCLUSION In our preliminary experience, we demonstrated that Genous® stent implantation was feasible in infants with complex congenital heart disease. Additional studies with larger samples and longer follow-up are required to confirm the potential benefits of this technology in this clinical setting.

Repair of near-atretic coarctation of the aorta in children with a new low-profile covered stent.

This article, which shows a technically very challenging angioplasty of a near-atretic coarctation of the aorta, underlines the importance of an anticipated procedure planning and of the right selection of available technology. Additionally, the performance and clinical setting of utilization of a new small premounted stent--the Advanta Atrium stent--is highlighted.

Válvula Aórtica Quadricúspide. Casuística de 10 Anos e Revisão da Literatura

INTRODUCTION Quadricuspid aortic valve is a rare malformation, with an estimated incidence of 0.003 to 0.043% of all congenital heart disease. It usually appears as an isolated congenital anomaly, but may also be associated with other malformations, the most common being coronary artery anomalies. Current technology enables noninvasive diagnosis in most cases. This entitys natural history is progression to valve regurgitation, which is rare before adulthood. OBJECTIVE Case review of quadricuspid aortic valve patients diagnosed in the last 10 years in a tertiary pediatric cardiology center. METHODS Retrospective chart review of patients diagnosed with quadricuspid aortic valve between January 2000 and December 2009. RESULTS Over the past 10 years, four cases of quadricuspid aortic valve were diagnosed in children aged between 6 months and 8 years, two male. In three cases, the four leaflets were of similar size, which is the most common finding. Two of the valves functioned normally and two had minimal regurgitation. All patients had associated cardiac malformations (one atrial and two ventricular septal defects, one supravalvular aortic stenosis and one quadricuspid pulmonary valve). One patient was also diagnosed with Williams syndrome. During a median follow-up of 2 years (0-9), all patients remained asymptomatic and none required medical or surgical treatment of the aortic valve. CONCLUSION Diagnosis of quadricuspid aortic valve is rare, especially in children, since most patients are asymptomatic and have normally functioning valves. In this study, half the patients had minimal aortic regurgitation. Contrary to what is described in the literature, all patients had concomitant cardiac malformations. We provide the first description of this entitys association with Williams syndrome. Clinical follow-up should be maintained in these patients in order to promptly detect the onset or worsening of functional alterations and to enable appropriate therapeutic intervention.

The hybrid approach for palliation of hypoplastic left heart syndrome: Intermediate results of a single-center experience

INTRODUCTION Hypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. METHODS We reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. RESULTS Nine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. CONCLUSIONS In our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.

RoPE Score as a Predictor of Recurrent Ischemic Events After Percutaneous Patent Foramen Ovale Closure

The benefits of patent foramen ovale (PFO) closure for cryptogenic stroke secondary prevention are still debated. The Risk of Paradoxical Embolism (RoPE) study developed a score to improve patient selection for this procedure. We proposed to assess the validity of this score to assess the prognostic impact of PFO closure.From 2000 to 2014, all consecutive patients submitted to PFO closure were included in a prospective registry in a university center. The primary endpoint was recurrent ischemic cerebrovascular events and the secondary endpoints were all-cause, neurological, and cardiac mortality rates and new-onset atrial fibrillation (NOAF) rates. In total, 403 patients were included in the study (women: 52.1%; mean age: 44.7 ± 10.9 years). The mean follow-up period was 6.4 ± 3.7 years. Immediate success was achieved in 97% patients. There were 23 (5.8%) ischemic cerebrovascular events, 8 (2.0%) deaths, and 17 (4.3%) NOAFs. The mean RoPE score was 6.10 ± 1.79. Smoker status, coronary artery disease, lower RoPE score, and higher left atrial dimensions were predictors of the primary endpoint. However, a lower RoPE score and coronary artery disease remained independent predictors in multivariate analysis.RoPE score was shown to be an independent predictor of recurrent ischemic cerebrovascular events, and a score of ≤ 6 was shown to identify patients with significantly higher risk of mortality and recurrent ischemic events.

Successful Percutaneous Closure of a Residual Atrial Septal Defect Due to Device Failure

A 39-year-old woman underwent uneventful percutaneous occlusion of an ostium secundum atrial septal defect (ASD) with a 22 mm Ultrasept ASD Occluder®. Transesophageal echocardiography (TEE) performed two years after implantation revealed a de novo residual left-to-right shunt through the correctly implanted device. Three-dimensional transesophageal echocardiography (3D TEE) further clarified this finding by showing a perforation of the device membrane coating. The patient underwent transcatheter closure of the residual shunt with a 20 mm Ultrasept PFO® device. The procedure was guided by fluoroscopy and real-time 3D TEE. At the end of the procedure 3D TEE documented correct device deployment with complete defect coverage and absence of residual shunt.

Image in CardiologyPulmonary arteriovenous malformation closure in a preterm newbornEncerramento de fístula arteriovenosa pulmonar em recém-nascido pré-termo

A 23-day-old preterm baby (1900 g) presenting with severe hypoxemia secondary to a large pulmonary arteriovenous malformation (PAVM), diagnosed by thoracic computed tomography (CT), was referred to our department. He was born at 32 weeks of gestation weighing 1945 g and, after birth, presented with severe hypoxemia (peripheral oxygen saturation [SpO2] 65%). The cardiac assessment was unremarkable. The initial diagnosis was congenital pneumonia with persistent pulmonary hypertension of the newborn and he was treated with antibiotics and ventilated with nitric oxide. In the absence of clinical improvement a thoracic CT scan was performed, which raised suspicion of a PAVM (Figure 1). Cardiac catheterization performed under general anesthesia demonstrated a large PAVM in the right lower lobe

Bridging thoracic endovascular aneurysm repair for a late rupture following aortic coarctation angioplasty.

UNLABELLED Introduction Thoracic endovascular aneurysm repair has been employed to treat late complications after aortic coarctation correction. However, its use in children has seldomly been reported. Case report We present the case of a 15-year-old child who presented with a ruptured aneurysm of the descending aorta complicated later by an aortic-oesophageal fistula following aortic coarctation stenting that was managed with multiple bridging endovascular interventions until a definitive repair was performed. CONCLUSION Thoracic endovascular aneurysm repair may be used successfully as a bridging intervention to a definitive repair in children with life-threatening aortic complications following aortic coarctation repair.