Cardiologia Pediátrica
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Browsing Cardiologia Pediátrica by Subject "Adult"
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- Endovascular Treatment of Pediatric Ischemic Stroke: a Single Center Experience and Review of the LiteraturePublication . Fragata, I; Morais, T; Silva, R; Paiva Nunes, A; Loureiro, P; Martins, JD; Pamplona, J; Carvalho, R; Baptista, M; Reis, JIntroduction: Mechanical thrombectomy is standard treatment for large vessel occlusion (LVO) in adults. There are no randomized controlled trials for the pediatric population. We report our single-center experience with thrombectomy of LVO in a series of pediatric patients, and perform a review of the literature. Methods: Retrospective review of consecutive pediatric thrombectomy cases between 2011 and 2018. Demographic variables, imaging data, technical aspects and clinical outcome were recorded. Results: In a period of 7 years, 7 children were treated for LVO at our center. Median age was 13 (2-17), and median Ped-NIHSS was 15 (3-24), and the median ASPECTS was 8 (2-10). Five patients had cardiac disease, and 2 of them were under external cardiac assistance. Median time from onset of symptoms to beginning of treatment was 7h06m (2h58m-21h38m). Five patients had middle cerebral artery occlusions. Thrombectomy was performed using a stentriever in 3 patients, aspiration in 3 patients, and combined technique in 1 patient. Six patients had good recanalization (TICI 2 b/3). There were no immediate periprocedural complications. At 3 months, 4 patients (57%) were independent (mRS score <3). Two patients died, one after haemorrhagic transformation of an extensive MCA infarct, and one due to extensive brainstem ischemia in the setting of varicella vasculitis. Discussion: Selected pediatric patients with LVO may be treated with mechanical thrombectomy safely. In patients under external cardiac assistance and under anticoagulation, thrombectomy is the only alternative for treatment of LVO. A multidisciplinary approach in specialized pediatric stroke centers with trained neurointerventionalists are essential for good results.
- Long-Term Evaluation of Endothelial Function in Kawasaki Disease PatientsPublication . Pinto, MF; Laranjo, S; Paramés, F; Freitas, I; Mota-Carmo, MBACKGROUND: Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce. Aim To evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT. METHODS: We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for >5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index. RESULTS: A total of 35 individuals (21 males, age 21 ± 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 ± 0.49 versus 2.31 ± 0.53; p = 0.001). Augmentation index was similar in both groups (-10 ± 7 versus -11 ± 5; p > 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls. CONCLUSIONS: Endo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.
- Percutaneous Occlusion of Vascular Malformations in Pediatric and Adult Patients: 20-Year Experience of a Single CenterPublication . Pereira-da-Silva, T; Martins, JD; Sousa, L; Fiarresga, A; Trigo Pereira, C; Cruz Ferreira, R; Pinto, MFOBJECTIVE: A case series on different vascular malformations (VM) treated with percutaneous occlusion in children and adults is presented. BACKGROUND: Percutaneous occlusion is usually the preferred treatment method for VM. Previous series have mostly focused on single types of devices and/or VM. METHODS: Retrospective analysis of all patients who underwent percutaneous occlusion of VM in a single center, from 1995 to 2014, excluding patent ductus arteriosus. Clinical and angiographic data, procedural details, implanted devices, and complications were assessed. Procedural success was defined as effective device deployment with none or minimal residual flow. Predictors of procedural failure and complications were determined by multivariate analysis. RESULTS: A total of 123 VM were intervened in 47 patients with median age of 12 years (25 days-76 years). The VM included 55 pulmonary arteriovenous fistulae, 39 aortopulmonary collaterals, 10 systemic venovenous collaterals, 8 peripheral arteriovenous fistulae, 5 Blalock-Taussig shunts, 4 coronary fistulae, and 2 Fontan fenestrations. The 143 devices used included 80 vascular plugs, 38 coils, 22 duct occluders, and 3 foramen ovale or atrial septal defect occluders. Median vessel size was 4.5 (2.0-16.0) mm and device/vessel size ratio was 1.4 (1.1-2.0). Successful occlusion was achieved in 118 (95.9%) VM, including three reinterventions. Four (3.3%) clinically relevant complications occurred, without permanent sequelae. Lower body weight was independently associated with procedural failure and complications. CONCLUSION: To our knowledge, this is the largest series on different VM occluded percutaneously in children and adults, excluding patent ductus arteriosus. Percutaneous occlusion was effective and safe, using different devices.
- Quality of Life Among Adults with Repaired Tetralogy of Fallot: a Literature ReviewPublication . Rodrigues, C; Silva, M; Cerejo, R; Rodrigues, R; Sousa, L; Trigo, C; Branco, LM; Agapito, A; Fragata, JTetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. Survival into adulthood is currently expected following surgical repair, leading to a growing population of adults with repaired TOF. In this literature review, we aim to summarize the current state of knowledge on the quality of life of adults with repaired TOF. A search was conducted on PubMed and results were reviewed for articles published between January of 2010 and June of 2020. Search terms included "Tetralogy of Fallot", "repaired", "adults" and "quality of life". For the subjective health status evaluation, most published studies used Short-Form-36. Most studies agree that physical complex status is poorer for adult patients with repaired TOF than for controls. Mental complex status was also lower. Patients reported similar satisfaction with their lives and levels of social participation. Most patients had a college or university degree. Higher education, male gender and having a partner were positively associated with being employed. Studies found no difference in the proportion of patients that are married or living with a partner, compared to control groups. Patients operated for TOF have a lower reproduction rate compared with the background population. A consistent finding of these studies is abnormal physical parameters compared to psychosocial issues. The diverse needs of adults with repaired TOF require a multidisciplinary care, that takes into consideration all aspects that affect their quality of life. Despite abnormal physical functional status, it is reassuring that most adult patients with TOF lead independent and productive lives.
- RoPE Score As a Predictor of Recurrent Ischemic Events After Percutaneous Patent Foramen Ovale ClosurePublication . Morais, LA; Sousa, L; Fiarresga, A; Martins, JD; Timóteo, AT; Viveiros Monteiro, A; Soares, C; Agapito, A; Pinto, MF; Cruz Ferreira, RThe 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.
- Uhl's Disease: an Uncommon Presentation of a Rare DiseasePublication . Aguiar Rosa, S; Agapito, A; António, M; de Sousa, L; Oliveira, JA; Laranjo, S; Martins, S; Jalles Tavares, N; Pinto, MF; Cruz Ferreira, RUhl's disease, also known as Uhl anomaly, is a rare disease secondary to selective but uncontrolled apoptosis of right ventricular myocytes during the perinatal period, after complete cardiac development, leading to the absence of right ventricular myocardium and the direct apposition of endocardium to epicardium without a myocardial layer in between, resulting in right ventricular failure. The present paper describes a case of Uhl's disease with an uncommon presentation. A 28-year-old man was admitted with dyspnea and cyanosis. Transthoracic echocardiography showed severe dilation of the right chambers, impaired right ventricular systolic function and a large ostium secundum atrial septal defect (ASD). Cardiac catheterization revealed pulmonary hypertension, with increased pulmonary capillary wedge pressure (mean 19mmHg) and Qp:QS 0.88:1. At this point, the authors considered that a main diagnosis of ASD could not explain the clinical features and hemodynamic data. A primary disease of the right ventricle was the most likely hypothesis and cardiac magnetic resonance imaging was performed, which demonstrated an extremely thin-walled right ventricle, with almost complete absence of right ventricular free wall myocardium, compatible with Uhl's disease.