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OBJECTIVE: This study aimed to compare the effectiveness of resection and extended end-to-end anastomosis between neonate and infant patients with coarctation. METHODS: This study was designed retrospectively and included 41 neonate (<30 days) and infant (30 days to 1 year) patients who were operated on using the resection and extended end-to-end anastomosis technique for aortic coarctation. Preoperative aortic annulus diameters and Z scores, all aortic arch diameters and Z scores, the presence of hypoplastic aortic segment, and the presence of prematurity were reviewed in both groups. Subsequently, we investigated whether these parameters were statistically related to the residual gradient in the operation area, whether there was a need for early re-intervention, and what was the incidence of mortality in the early postoperative period. In addition, the aortic arch Z scores of the patients at 6 months postoperatively were examined. RESULTS: While the mean age (p<0.001), body weight (p<0.001), and proximal arch Z score (p=0.029) were found to be significantly lower in the neonate group than in the infant group, the total length of the intensive care unit stay (p=0.013) and the total length of hospital stay (p=0.017) were found to be significantly higher. In addition, significant enlargement was detected in the proximal arch, distal arch, and isthmus segments in both patient groups. CONCLUSION: The resection and extended end-to-end anastomosis is an equally effective technique that can provide a marked decrease in gradient in the coarctation area and a significant enlargement of the aortic arch segments in the early period after coarctation repair in both neonate and infant patients.
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Coartação Aórtica , Tempo de Internação , Humanos , Coartação Aórtica/cirurgia , Recém-Nascido , Lactente , Resultado do Tratamento , Estudos Retrospectivos , Feminino , Masculino , Anastomose Cirúrgica/métodos , Aorta Torácica/cirurgia , Fatores EtáriosRESUMO
INTRODUCTION: Cyanotic congenital heart diseases constitute 40-45% of all congenital heart diseases. In patients who are not suitable for primary repair, modified BT (MBT) shunt and central shunt (CS) procedures are still frequently used. METHODS: This study included 62 pediatric patients who underwent MBT shunt or CS via median sternotomy. Patients' demographic, echocardiographic, operative, and postoperative data were collected retrospectively. The patients were classified as single ventricle and bi-ventricle according to their cardiac anatomy, and the presence of prematurity and heterotaxy was noted. Procedure details of the patients who underwent endovascular intervention prior to the surgery were investigated, and operation data were accessed from the surgery notes. Data regarding postoperative follow-ups were obtained and comparatively analyzed. RESULTS: Of the total 62 patients, 32 (51.6%) were newborns and 16 (25.8%) had a body weight < 3 kg. MBT shunt was applied to 48 patients (77.4%), while CS was applied to 14 patients (22.6%). There was no significant difference between the two surgical procedures in terms of requirement for urgent shunt or cardiopulmonary bypass, additional simultaneous surgical intervention, need for high postoperative inotropes, and in-hospital mortality (P>0.05). The rate of congestive heart failure in patients with in-hospital mortality was determined as 66.7% and it was significantly higher than in patients without heart failure (P<0.001). CONCLUSION: MBT shunt and CS are still frequently used in cyanotic patients. The use of small-diameter shunts, particularly when centrally located, can prevent the onset of congestive heart failure and lower mortality.
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Cardiopatias Congênitas , Humanos , Cardiopatias Congênitas/cirurgia , Estudos Retrospectivos , Masculino , Feminino , Lactente , Recém-Nascido , Pré-Escolar , Resultado do Tratamento , Criança , Mortalidade Hospitalar , Cianose/etiologia , Cianose/cirurgia , EcocardiografiaRESUMO
SUMMARY OBJECTIVE: This study aimed to compare the effectiveness of resection and extended end-to-end anastomosis between neonate and infant patients with coarctation. METHODS: This study was designed retrospectively and included 41 neonate (<30 days) and infant (30 days to 1 year) patients who were operated on using the resection and extended end-to-end anastomosis technique for aortic coarctation. Preoperative aortic annulus diameters and Z scores, all aortic arch diameters and Z scores, the presence of hypoplastic aortic segment, and the presence of prematurity were reviewed in both groups. Subsequently, we investigated whether these parameters were statistically related to the residual gradient in the operation area, whether there was a need for early re-intervention, and what was the incidence of mortality in the early postoperative period. In addition, the aortic arch Z scores of the patients at 6 months postoperatively were examined. RESULTS: While the mean age (p<0.001), body weight (p<0.001), and proximal arch Z score (p=0.029) were found to be significantly lower in the neonate group than in the infant group, the total length of the intensive care unit stay (p=0.013) and the total length of hospital stay (p=0.017) were found to be significantly higher. In addition, significant enlargement was detected in the proximal arch, distal arch, and isthmus segments in both patient groups. CONCLUSION: The resection and extended end-to-end anastomosis is an equally effective technique that can provide a marked decrease in gradient in the coarctation area and a significant enlargement of the aortic arch segments in the early period after coarctation repair in both neonate and infant patients.
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ABSTRACT Introduction: Cyanotic congenital heart diseases constitute 40-45% of all congenital heart diseases. In patients who are not suitable for primary repair, modified BT (MBT) shunt and central shunt (CS) procedures are still frequently used. Methods: This study included 62 pediatric patients who underwent MBT shunt or CS via median sternotomy. Patients' demographic, echocardiographic, operative, and postoperative data were collected retrospectively. The patients were classified as single ventricle and bi-ventricle according to their cardiac anatomy, and the presence of prematurity and heterotaxy was noted. Procedure details of the patients who underwent endovascular intervention prior to the surgery were investigated, and operation data were accessed from the surgery notes. Data regarding postoperative follow-ups were obtained and comparatively analyzed. Results: Of the total 62 patients, 32 (51.6%) were newborns and 16 (25.8%) had a body weight < 3 kg. MBT shunt was applied to 48 patients (77.4%), while CS was applied to 14 patients (22.6%). There was no significant difference between the two surgical procedures in terms of requirement for urgent shunt or cardiopulmonary bypass, additional simultaneous surgical intervention, need for high postoperative inotropes, and in-hospital mortality (P>0.05). The rate of congestive heart failure in patients with in-hospital mortality was determined as 66.7% and it was significantly higher than in patients without heart failure (P<0.001). Conclusion: MBT shunt and CS are still frequently used in cyanotic patients. The use of small-diameter shunts, particularly when centrally located, can prevent the onset of congestive heart failure and lower mortality.
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ABSTRACT The presence of persistent left superior vena cava to the left atrium connection without an innominate vein may give rise to technical challenges during intracardiac repair. In this report, the end-to-side anastomosis technique of the persistent left superior vena cava to the right superior vena cava is discussed in a patient with tetralogy of Fallot associated with persistent left superior vena cava draining directly into the left atrium. A successful end-to-side anastomosis between the persistent left superior vena cava and the right superior vena cava was performed and short-term anastomosis patency was documented via angiography.
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Native valve aortic endocarditis is rarely seen in the paediatric population. Although, the first-line of treatment is medical, surgical intervention may be indicated in patients with unrepairable valvular and subvalvular disease. Recently, the aortic valve neocuspidization (AVNeo) procedure has gained popularity both in adult and children in whom other repair techniques are not feasible. In this case report, we present an urgent aortic valve replacement using the AVNeo technique in a critically ill infant with a small annulus, severe left ventricular outflow tract stenosis, and severe aortic regurgitation.
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Insuficiência da Valva Aórtica , Estenose da Valva Aórtica , Endocardite , Próteses Valvulares Cardíacas , Adulto , Criança , Humanos , Lactente , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/diagnóstico , Insuficiência da Valva Aórtica/cirurgia , Estenose da Valva Aórtica/cirurgia , Endocardite/diagnóstico , Endocardite/cirurgiaRESUMO
The presence of persistent left superior vena cava to the left atrium connection without an innominate vein may give rise to technical challenges during intracardiac repair. In this report, the end-to-side anastomosis technique of the persistent left superior vena cava to the right superior vena cava is discussed in a patient with tetralogy of Fallot associated with persistent left superior vena cava draining directly into the left atrium. A successful end-to-side anastomosis between the persistent left superior vena cava and the right superior vena cava was performed and short-term anastomosis patency was documented via angiography.
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Veia Cava Superior Esquerda Persistente , Tetralogia de Fallot , Malformações Vasculares , Criança , Humanos , Veia Cava Superior/cirurgia , Tetralogia de Fallot/diagnóstico por imagem , Tetralogia de Fallot/cirurgia , Átrios do Coração/cirurgia , Anastomose CirúrgicaRESUMO
INTRODUCTION: Our aim was to present the effects of submammarian thoracotomy by our new approach to breast development in young female patients between 8 and 14 years old. METHODS: Atrial septal defect closure was performed in 55 female patients between 8 and 14 years old at the time of operation between January 2010 and January 2018 by median sternotomy or right anterolateral thoracotomy. These patients were called for a follow-up visits, breast examination were performed, and a questionnaire of 10 questions was applied. RESULTS: According to the answer given by the patients to the question of "how would you describe your scar" showed that the surgical method satisfies all patients cosmetically in study group. The overall satisfaction rate for the cosmetic results of operation was 100% versus 41.1%, respectively. The percentage of patients said no to the question "Do you experience lack of self-confidence related your scar" was significantly higher than the control group (p < 0.001). The percentage of those who said yes to the question "Would you like to be operated with the same approach if you will have surgery again?" in the study group was significantly higher than the control group (p = 0.002). CONCLUSIONS: We consider that our procedure is safe and effective with improved cosmetic results compared to median sternotomy which also confers many advantages such as less trauma, less pain, faster recovery, and shorter hospital stay. This study presents one of the largest series of paediatric patients operated by submammarian thoracotomy.
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Procedimentos Cirúrgicos Cardíacos , Comunicação Interatrial , Humanos , Criança , Feminino , Adolescente , Toracotomia/métodos , Cicatriz/cirurgia , Mama/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interatrial/cirurgia , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Anomalies in systemic venous return most commonly involve a persistent left supe-rior vena cava draining into the left atrium. Anomalous drainage of the inferior vena cava (IVC) into the left atrium is a rare congenital vascular disorder. The diagnosis was confirmed as anoma-lous drainage of the right superior pulmonary vein and large atrial septal defect following echo-cardiography. Anomalous drainage of the inferior vena cave was confirmed with computed tomog-raphy (CT). We report a rare combination of drainage of the inferior vena cava associated with atrial septal defect (ASD) and partial anomalous pulmonary venous return. CASE: A 14-year-old girl was referred to our hospital for the evaluation of palpitations, hypoxia, exertional dyspnea, and cyanosis. Transthoracic echocardiography (TTE) revealed a large sinus venosus ASD and anomalous right superior pulmonary venous return. A cardiac CT demonstrated IVC drainage to the left atrium and an anomalous right superior pulmonary vein draining into the right atrium. CONCLUSIONS: In older patients with cyanosis, further imaging methods together with TTE will be useful in detecting additional cardiac anomalies. Patients with inferior vena cava opening to the left atrium are different from caval type ASD`s and should be surgically repaired using a patch. Corrective surgery involves repositioning of the interatrial septum via a patch.
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Comunicação Interatrial , Veias Pulmonares , Veia Cava Inferior , Adolescente , Feminino , Humanos , Cianose/complicações , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/anormalidades , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/cirurgia , Comunicação Interatrial/complicações , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Veias Pulmonares/anormalidades , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Veia Cava Inferior/anormalidades , Veia Cava Superior/anormalidades , Veia Cava Superior/cirurgiaRESUMO
BACKGROUND: In this retrospective study, we compared the results from 2 pulmonary valve augmentation techniques in patients undergoing surgical repair of tetralogy of Fallot. METHODS: Between 2015 and 2018, 18 patients had anterior pulmonary valve repair at our institution, and 26 patients had both anterior and posterior pulmonary valve repair. RESULTS: Patients ranged from 6 months to 30 years of age. The median follow-up period was 8 months in the anterior augmentation group and 5 months in the anterior and posterior augmentation group. Postoperative echocardiograms indicated that only 2 patients (11%) in the anterior augmentation group had moderate or severe pulmonary insufficiency, compared with no patients in the anterior and posterior augmentation group. At follow-up, pulmonary insufficiency was seen in 3 patients (17%) in the anterior augmentation group and no patients in the anterior and posterior augmentation group. CONCLUSION: Reconstruction of the native pulmonary valve accompanied by pulmonary cusp augmentation can decrease or even circumvent postoperative pulmonary insufficiency. Both anterior augmentation and anterior and posterior augmentation techniques are easily applied; however, we believe that the anterior and posterior augmentation technique is superior in terms of early postoperative and follow-up pulmonary insufficiency outcomes.
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Insuficiência da Valva Pulmonar , Valva Pulmonar , Tetralogia de Fallot , Humanos , Lactente , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/etiologia , Estudos Retrospectivos , Tetralogia de Fallot/diagnóstico , Tetralogia de Fallot/cirurgia , Resultado do TratamentoRESUMO
AIM: Minimally invasive techniques in the surgical treatment of congenital heart diseases have gained popularity recently. Right infra-axillary vertical thoracotomy (RIAVT) is one of these techniques. The aim of this study is to investigate the postoperative morbidity and cosmetic results of the right infraaxillary thoracotomy technique and compare them with the results in the literature. METHOD: All patients who had undergone cardiac surgery using RIAVT at the Ankara City Hospital Pediatric Cardiovascular Surgery Clinic between 2019 and 2021 were enrolled in the study. Preoperative echocardiographic diagnoses, intraoperative surgical data and postoperative morbidity data of the patients was obtained. In addition, surgical incision length was measured and recorded for the evaluation of cosmetic outcome. This data was then compared with data from similar studies in the literature. RESULT: Between January 2019 and December 2021, 59 patients were operated on using RIAVT. Of the RIAVT patients, 45 (76%) were female and 14 (24%) were male. The mean weight of the patients was 22.38±12.48 kg. Although the youngest patient was a 5-month-old infant weighing 4 kg, the oldest one was 15-years-old weighing 57 kg. Isolated secundum atrial septal defect (ASD) repair (primary or patch) was performed in 47 patients. One (1) patient with Primum ASD and mitral cleft, 1 patient with secundum ASD and perimembranous ventricular septal defect (VSD) repair, nine patients with high venosum ASD and PAPVC, and one patient with a discrete subaortic membrane were operated on. The surgical incision length was between 4 to 5 cm. CONCLUSION: RIAVT is an important surgical alternative to median sternotomy in selected cases with certain pathologies. It can be performed safely in various congenital heart anomalies and provides excellent functional and cosmetic results in the long-term when compared to other surgical methods.
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Cardiopatias Congênitas , Comunicação Interatrial , Ferida Cirúrgica , Adolescente , Criança , Feminino , Cardiopatias Congênitas/cirurgia , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , Humanos , Lactente , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Estudos Retrospectivos , Ferida Cirúrgica/cirurgia , Toracotomia/métodos , Resultado do TratamentoRESUMO
OBJECTIVE: To determine the safety of on-site bedside patent ductus arteriosus (PDA) ligation, performed by a regional roving surgical team at different neonatal intensive care units (NICUs) in preterm infants with hemodynamically significant PDA (hsPDA). STUDY DESIGN: A descriptive study. PLACE AND DURATION OF STUDY: Department of Paediatric Cardiovascular Surgery, Ankara City Hospital, Ankara, Turkey, between January 2018 and December 2020. METHODOLOGY: Medical data of 48 premature infants with hsPDA, who underwent bedside PDA ligation by the same roving surgical team at five different NICU centres in Ankara province, were evaluated. Demographic and clinical data of the patients were extracted from the institutional databases and medical records. Postoperative complications were recorded. RESULTS: Mean body-weight of the infants was 1113.1 ± 392.8 grams. Forty patients were under respiratory support before surgery. Majority of the patients (n=45, 93.7%) no longer needed respiratory support after the third and seventh days of surgery. No complications related to surgery or anaestesia were observed. Non of the patients developed post-PDA ligation cardiac syndrome. No surgical wound infection or sepsis was observed in any patient. The overall mortality rate was 4.2%. CONCLUSION: Bedside PDA ligation at different NICUs by the roving surgical team is safe and effective. Owing to an extremely low risk, this procedure can be performed successfully in the hands of professional and experienced surgeons. It seems to be a feasible option for premature infants with hsPDA, who do not respond to medical treatment. Key Words: Infant, Patent ductus arteriosus, Ligation, Intensive care unit, Newborn, Surgery.
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Permeabilidade do Canal Arterial , Doenças do Prematuro , Criança , Permeabilidade do Canal Arterial/cirurgia , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Prematuro , Resultado do TratamentoRESUMO
Pseudoaneurysms of the ascending aorta are rarely seen in paediatric patients. They may occur following complex cardiovascular surgery and are associated with a high risk of mortality due to their nature of spontaneous rupture. In this case report, we discuss a 1-year-old paediatric patient with an ascending aortic pseudoaneurysm following Norwood Stage 1 surgery with Sano modification for hypoplastic left heart syndrome and the patient's successful recovery following aneurysmectomy with Norwood Stage 2 operation.
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Anomalous origin of coronary artery originating from the pulmonary artery in conjunction with the aorticopulmonary window (APW) is a rare but a significant anomaly in the era of congenital cardiac diseases. The occurrence of anomalous origin of the right coronary artery from the pulmonary artery among the associated anomalies is less than 5%. The severity of the clinical condition of these patients depends on the degree of left-right shunt and compromise of the pulmonary blood flow. We report surgical management of a case of a 45-day-old infant with APW, ventricular septal defect, and anomalous origin of coronary artery originated from the pulmonary artery.
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Defeito do Septo Aortopulmonar , Anomalias dos Vasos Coronários , Defeito do Septo Aortopulmonar/diagnóstico por imagem , Defeito do Septo Aortopulmonar/cirurgia , Anomalias dos Vasos Coronários/diagnóstico por imagem , Anomalias dos Vasos Coronários/cirurgia , Humanos , Lactente , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Circulação PulmonarRESUMO
Background/aim: Systemic to pulmonary shunts (SPS) have proven to be highly effective for the palliation of neonates with cyanotic congenital heart disease. Mortality after SPS surgery in neonates has multifactorial basis. We aimed to investigate the clinical results of the SPS in relation to the underlying cardiac disease and to identify the risk factors contributing to an adverse outcome.Material and method: All neonates who underwent first shunt insertion for cyanotic congenital heart disease during the study period from 1 January 2014 to 31 December 2017 were included. A retrospective review of patient records was done. Patients were grouped into two different categories: survived with or without any reintervention and death before or after any reintervention till discharge.Result: During the study period, 47 patients underwent SPS shunt placement. Patients who survived with or without any reintervention were in Group 1 and patients who died before or after any reintervention till discharge were in Group 2. Preoperative epinephrine requirement and mechanical ventilation and postoperative erythrocyte transfusion need were statistically significant.Conclusion: Although primary cardiac pathology is the most important prognostic factor, some other preoperative and postoperative factors like preoperative epinephrine requirement, and postoperative erythrocyte transfusion might also affect the prognosis. As there are very few centers in the region that specialize in pediatric cardiac surgery, a multicenter approach will be helpful in reaching reliable conclusions.
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Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Criança , Cardiopatias Congênitas/cirurgia , Humanos , Recém-Nascido , Prognóstico , Artéria Pulmonar , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Blunt abdominal aortic injuries are very rare in childhood. In these injuries, abdominal aorta and intra-abdominal organs can be damaged to various degrees. In this article, we report a unique pediatric case of isolated middle sacral artery injury following trauma.
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The new coronavirus infection, which was first seen in China in late December, 2019 and eventually became a worldwide pandemic, poses a serious threat to public health. After a high spike in the number of new COVID-19 infection cases following the increase in overall daily death toll in Turkey, Turkish Ministry of Health has taken immediate precautions to postpone elective surgeries in order to reduce the burden to the healthcare system which might be challenged. Whereas different areas of medicine were able to suspend their operative procedures during this period, this was not completely possible in paediatric cardiovascular surgery due to the severity and urgency of congenital heart disease patients requiring operation. Based on the guideline that was published by the Turkish Paediatric Cardiology and Cardiac Surgery Association, in which the patients requiring surgical intervention during the COVID-19 pandemic period are ranked according to the priority, directions were given regarding the operations that hereby, be delayed, we report our experience in 29 cases retrospectively, regarding the pre-operative evaluation of these patients, makings of an emergency operation decision, and strategies taken about intra-operative and post-operative management and arrangements during the pandemic period. In this article, we present crucial precautions that were applied in paediatric cardiovascular surgery and extensive list of cases in order to deliver highest level of the patient safety and protection for the surgical team.
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COVID-19/prevenção & controle , Cardiologia , Cardiopatias Congênitas/cirurgia , Cuidados Pré-Operatórios/métodos , Cirurgia Torácica , Adolescente , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Serviço Hospitalar de Emergência , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Lactente , Recém-Nascido , Controle de Infecções/métodos , Masculino , Equipamento de Proteção Individual , Guias de Prática Clínica como Assunto , Estudos Retrospectivos , TurquiaRESUMO
BACKGROUND: Optimal management strategy for native aortic coarctation in neonates and young infants is still a matter of debate. The surgical procedure, histopathologic research, and clinical outcome in 15 neonates who underwent surgery after successful balloon angioplasty is the basis of this study. METHOD: Between 01 October, 2014 and 01 August, 2017, we enrolled 15 patients with native aortic coarctation for this study. These patients had complications regarding recoarctation, following balloon angioplasty intervention at our institute and other centres. Surgically extracted parts were examined histopathologically and patient's data were collected retrospectively.ResultThe reasons for recurrence of recoarctation after balloon angioplasty are as follows: patients with higher preoperative echocardiographic gradients had recoarctation earlier, neointimal proliferation, aortic intimal fibrosis at the region of ductal insertion, and ductal residual tissue debris after balloon angioplasty. No repeat intervention was required in the 15 patients who underwent surgery followed by balloon angioplasty. Early mortality was seen in one patient after surgery. Postoperative complication in the surgical group occurred in the form of chylothorax in one patient. CONCLUSION: In centres in which the neonatal ICU is inexperienced, balloon angioplasty is particularly recommended. In developing neonatal clinics, balloon angioplasty, when performed on patients at their earliest possible age, delays actual corrective operation to a later date, which in turn provides less risky surgical outcomes in infants who are gaining weight, growing, and do not have any haemodynamic complaints.
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Angioplastia com Balão , Aorta Torácica/patologia , Coartação Aórtica/patologia , Neointima/patologia , Aorta Torácica/cirurgia , Coartação Aórtica/cirurgia , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Período Pós-Operatório , Estudos RetrospectivosRESUMO
Pulmonary valvular stenosis is a relatively common disorder, accounting for approximately 10% of all CHDs. Pulmonic valvular disease can get clinically detected at different ages of life. The more severe the obstruction, the earlier detected the valvular abnormality. Surgical pulmonary valvotomy has been available as a treatment since 1956. This article is about a case of pulmonary annular and valvular stenosis in a 1-year-old child, and it also explores surgical operation of this condition. Transannular patches are usually used within the 1st year of age in pulmonary annular and valvular stenosis. In recent years, anterior leaflet augmentation has been preferred for annulus enlargements. In our 1-year-old case, we expanded the annulus by the anterior leaflet expansion technique and we also augmented other leaflets by polytetrafluoroethylene patch.
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Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/cirurgia , Estenose da Valva Pulmonar/diagnóstico por imagem , Estenose da Valva Pulmonar/cirurgia , Ponte Cardiopulmonar/métodos , Ecocardiografia Doppler em Cores , Feminino , Humanos , Lactente , Valva Pulmonar/cirurgiaRESUMO
Ochronosis is an autosomal recessive metabolic disorder which may cause aortic valve stenosis. In this article, we report a 58-yearold male patient with alkaptonuria who presented to our institute with severe aortic valve stenosis. He underwent successful aortic valve replacement with a mechanical prosthesis. The deposition of foreign material in valvular tissue is a rare case scenario which may result in serious problems, the management of which may necessitate valve replacement with prosthetic heart valves.