Browsing by Author "Costa, G"
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- Perioperative Ultrasound-Guided Continuous Caudal Epidural Analgesia in Newborns: A Case Series in a Tertiary Medical CenterPublication . Portela, F; Costa, G; Cenicante, TBackground Caudal epidural anesthesia technique is a relevant method for postoperative analgesia in newborns, allowing for the reduction of drug-induced respiratory depression. The threading of a catheter is, however, uncommon in clinical practice. Our main purpose was to describe our experience regarding caudally inserted epidural catheters in neonates undergoing major abdominal surgery. Methods We included every full-term neonate undergoing surgery under combined caudal epidural-general anesthesia from 2017 to 2022 in our institution. After induction of general anesthesia, an ultrasound-guided caudal epidural injection was performed, and an epidural catheter was inserted for perioperative analgesia. An epidural bolus of ropivacaine was administered to every patient before the surgical incision, and an epidural infusion of ropivacaine 0.05% was administered for 24 hours. Results Retrospectively obtained data included six full-term neonates with American Society of Anesthesiologists (ASA) physical status II to IV. Intraoperatively, good analgesia was achieved without hemodynamic instability or need for additional systemic opioids after induction. At the end of surgery, five of the six neonates were extubated without adverse respiratory events. Postoperatively, effective analgesia was achieved in four cases with an epidural infusion of ropivacaine 0.05%, at a rate between 0.2 and 0.4 mg/kg/h, and intravenous paracetamol. Epidural pain control was not successful in one neonate, and thus an intravenous fentanyl infusion was added. The sixth neonate remained intubated for prolonged mechanical ventilation due to surgical complications, and thus an intravenous fentanyl infusion was introduced for sedation in the neonatal intensive care unit (NICU), not allowing to evaluate the effectiveness of the epidural infusion alone. No other complications related to the epidural catheters were reported. Conclusion Continuous caudal epidural analgesia may be a valuable technique with a low risk of complications, decreasing the incidence of respiratory adverse events in this patient population. Although more cases are needed for a stronger conclusion, it has become a useful analgesic strategy for major abdominal surgery in neonates in our institution.
- The Hybrid Approach for Palliation of Hypoplastic Left Heart Syndrome: Intermediate Results of a Single-Center ExperiencePublication . Laranjo, S; Costa, G; Freitas, I; Martins, JD; Bakero, L; Trigo, C; Fragata, I; Fragata, J; Pinto, MFINTRODUCTION: 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.
- The Role of Propranolol in the Treatment of Infantile HemangiomaPublication . Laranjo, S; Costa, G; Paramés, F; Freitas, I; Martins, JD; Trigo, C; Pinto, MFINTRODUCTION: 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.
- Transthyretin Amyloidosis: Chaperone Concentration Changes and Increased Proteolysis in the Pathway to DiseasePublication . Costa, G; Ribeiro-Silva, C; Ribeiro, R; Gilberto, S; Gomes, R; Ferreira, A; Mateus, E; Barroso, E; Coelho, A; Ponces Freire, A; Cordeiro, CTransthyretin amyloidosis is a conformational pathology characterized by the extracellular formation of amyloid deposits and the progressive impairment of the peripheral nervous system. Point mutations in this tetrameric plasma protein decrease its stability and are linked to disease onset and progression. Since non-mutated transthyretin also forms amyloid in systemic senile amyloidosis and some mutation bearers are asymptomatic throughout their lives, non-genetic factors must also be involved in transthyretin amyloidosis. We discovered, using a differential proteomics approach, that extracellular chaperones such as fibrinogen, clusterin, haptoglobin, alpha-1-anti-trypsin and 2-macroglobulin are overrepresented in transthyretin amyloidosis. Our data shows that a complex network of extracellular chaperones are over represented in human plasma and we speculate that they act synergistically to cope with amyloid prone proteins. Proteostasis may thus be as important as point mutations in transthyretin amyloidosis.
- Válvula Aórtica Quadricúspide. Casuística de 10 Anos e Revisão da LiteraturaPublication . Gouveia, S; Martins, JD; Costa, G; Paramés, F; Freitas, I; Rebelo, M; Trigo, C; Pinto, MFIntrodução: A válvula aórtica quadricúspide é uma malformação rara, com uma incidência estimada de 0,003 a 0,043% de todas as cardiopatias congénitas. Surge habitualmente como uma anomalia congénita isolada, podendo igualmente estar associada a outras malformações, sendo as mais frequentes as anomalias das artérias coronárias. A tecnologia actual permite o diagnóstico não invasivo na grande maioria das situações. A sua história natural é a evolução para a insuficiência, rara antes da idade adulta. Objectivos: Revisão dos casos de válvula aórtica quadricúspide diagnosticados nos últimos 10 anos num centro terciário de Cardiologia Pediátrica. Material e Métodos: Revisão retrospectiva do processo clínico dos doentes aos quais foi detectada uma válvula aórtica quadricúspide, entre Janeiro de 2000 e Dezembro de 2009. Resultados: Nos últimos 10 anos, foram diagnosticados quatro casos de válvula aórtica quadricúspide, em crianças com idades compreendidas entre os 6 meses e os 8 anos, duas do sexo masculino. Em três casos, os quatro folhetos eram de dimensões semelhantes, que é o achado mais frequente. Duas das válvulas eram normofuncionantes e duas apresentavam insuficiência mínima. Todos os doentes apresentavam outras malformações cardíacas associadas (uma comunicação interauricular, duas comunicações interventriculares, uma estenoseçupravalvular aórtica e uma válvula pulmonar quadricúspide). Um doente tinha também o diagnóstico de Síndrome de Williams. Com um tempo de seguimento mediano de 2 anos [0 --- 9], todos os doentes se mantiveram assintomáticos e não requereram tratamento médico ou cirúrgico para a válvula aórtica. Conclusão: O diagnóstico de válvula aórtica quadricúspide é raro, sobretudo em idade pediátrica, quando a maioria dos doentes são assintomáticos e apresentam válvulas normofuncionantes. Nesta casuística, metade apresentava insuficiência aórtica mínima. Ao contrário do que está descrito na literatura, todos os doentes apresentavam malformações cardíacas concomitantes. Descrevemos pela primeira vez a associação com a Síndrome de Williams. Estes doentes deverão manter seguimento em ambulatório, de forma a detectar atempadamente o aparecimento ou agravamento de alterações funcionais e permitir uma intervenção terapêutica oportuna.