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BACKGROUND: Although pulmonary artery banding (PAB) has been generally acknowledged as an initial palliative treatment for patients having single ventricle (SV) physiology and unrestrictive pulmonary blood flow (UPBF), it may result in unfavorable outcomes. Performing bidirectional Glenn (BDG) surgery without initial PAB in some selected cases may avoid the complications associated with PAB and reduce the number of operative procedures for these patients. This research aimed to assess the outcome of BDG surgery performed directly without doing initial PAB in patients with SV-UPBF. METHODS: This Multicenter retrospective cohort includes all patients with SV-UPBF who had BDG surgery. Patients were separated into two groups. Patients in Group 1 included patients who survived till they received BDG (20 Patients) after initial PAB (28 patients), whereas patients in Group 2 got direct BDG surgery without first performing PAB (16 patients). Cardiac catheterization was done for all patients before BDG surgery. Patients with indexed pulmonary vascular resistance (PVRi) ≥ 5 WU.m2 at baseline or > 3 WU.m2 after vasoreactivity testing were excluded. RESULTS: Compared with patients who had direct BDG surgery, PAB patients had a higher cumulative mortality rate (32% vs. 0%, P = 0.016), with eight deaths after PAB and one mortality after BDG. There were no statistically significant differences between the patient groups who underwent BDG surgery regarding pulmonary vascular resistance, pulmonary artery pressure, postoperative usage of sildenafil or nitric oxide, intensive care unit stay, or hospital stay after BDG surgery. However, the cumulative durations in the intensive care unit (ICU) and hospital were more prolonged in patients with BDG after PAB (P = 0.003, P = 0.001respectively). CONCLUSION: Direct BDG surgery without the first PAB is related to improved survival and shorter hospital stays in some selected SV-UPBF patients.
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Procedimiento de Fontan , Cardiopatías Congénitas , Corazón Univentricular , Humanos , Lactante , Arteria Pulmonar/cirugía , Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Cuidados Paliativos/métodos , Ventrículos Cardíacos/cirugíaRESUMEN
Background: Percutaneous closure of residual ventricular septal defects (VSDs) after congenital heart surgery may provide a safer and more efficient alternative to redo surgery. This study aimed to evaluate the outcome of transcatheter closure of residual postoperative VSD. Methods: This multicenter retrospective cohort study was conducted at the tertiary care institutions of King Faisal Specialist Hospital and King Abdulaziz University Hospital, Saudi Arabia, from March 2012 to March 2022. All patients who underwent transcatheter closure of postoperative residual VSD were included. As catheter closure of VSD related to surgical patches is challenging, patients were divided into two groups. Group 1 comprised patients with VSD related to the surgical patches, while Group 2 included residual muscular VSD. Various occluders and approaches were utilized based on the patient's weight and the VSD type, size, and proximity to the cardiac valves. Demographic, echocardiographic, catheterization, and outcome data were collected and analyzed using descriptive and comparative statistics. Results: Thirty-three patients underwent 37 VSD catheter closure procedures. Twenty-two procedures were done to close residual VSD related to the surgical patch, while fifteen were done for additional muscular VSD. The median age of the patients was 3.3 years, and the interquartile range (IQR) ranged between 9 months and 7 years. The median weight was 13.1 kilograms, with an IQR of 5.1 to 16.8 kilograms. The median pulmonary to systemic flow ratio (QP/QS) was 1.6 with an IQR of 1.5 to 2.44; the median systolic pulmonary pressure was 46 mmHg with an IQR of 32 to 54 mmHg. The median procedure duration was 120 minutes, with an IQR of 90 to 160 minutes. Patients in Group 1 were older and had a lower mean pulmonary pressure than Group 2 (P=0.02, P=0.007, respectively). Of the 37 procedures, 35 (94.6%) were done successfully, while two patients had redo surgery due to failed procedures (one had device embolization). Ten successful catheterizations were performed for infants weighing ≤5 kilograms. The functional heart failure class improved significantly after the closure of the residual VSD. There were three documented mortalities, none related to the procedure. No significant difference between patient groups regarding hospital stay or survival (P=0.660, P=0.791, respectively). Conclusions: After congenital heart surgery, transcatheter closure of residual VSD may be a safe and effective alternative to surgical closure. It can be applied to various residual VSD using a variety of occluders with satisfactory results. Moreover, using specific approaches can close residual VSD, even in small infants.
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Background: Coronary artery stent implantation (CSI) in the pediatric population is rare. Only a few reports were published on managing postoperative coronary artery obstruction using coronary stents following surgical repair of congenital heart diseases (CHD). This study aimed to analyze the feasibility, indications, procedural technique, risk factors, and short-term outcomes of CSI after pediatric cardiac surgery. Methods: In this retrospective cohort study, we reviewed all pediatric patients who underwent surgical repair of CHD requiring postoperative CSI in two cardiac centers (King Abdulaziz University Hospital and King Faisal Specialist Hospital and Research Center) between 2012 and 2022. Survival to hospital discharge was the study's primary outcome. The secondary outcomes included procedural success, duration of mechanical ventilation, intensive care unit (ICU) stay, hospital stay, need for coronary reintervention, and late mortality. A descriptive analysis was performed for the collected data from the patients' medical records. Results: Eleven patients who underwent postoperative CSI were identified. The most common anatomic diagnosis was congenital aortic valve stenosis. All patients underwent cardiac catheterization on extracorporeal membrane oxygenation support except one patient, who presented with chest pain after cardiac surgery. Procedural success was achieved in all patients with excellent revascularization documented by post-procedural angiograms. Both patients who had late coronary events after cardiac surgery survived hospital discharge. There was no in-hospital mortality among the two patients who required stenting of only the right coronary artery. The four patients who required more than 120 minutes to complete the procedure had early mortality. After CSI, the median duration of mechanical ventilation and ICU stay was 12 and 17 days, respectively. Six patients (54.5%) survived hospital discharge post-CSI; they did not require re-intervention during the follow-up period (38-1,695 days). Conclusions: CSI in pediatric patients can be performed with excellent procedural success for treating coronary artery stenosis after cardiac surgery. It could be considered a potential treatment strategy for this population.
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Transcatheter closure of septal defects using specialized devices is a common procedure that has a high success rate. Embolization of Atrial septal defect devices is a known complication seen at a rate of 0.5%. We report a case of late ASD device occluder embolization immediately after brain MRI with clinical consequences and intraoperative evidence of right atrial wall thermal injury. To our knowledge Atrial septal occluder (ASO) device embolization post magnetic resonance imaging (MRI) was not reported before, although theoretically possible and that's why six to eight weeks post device implantation MRI is prohibited even with current MRI compatible devices.
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We present a case of percutaneous closure of main pulmonary artery pseudoaneurysm using an atrial septal occluder device in a seven-month-old infant. The infant had tetralogy of Fallot repair followed by transcatheter left pulmonary artery stenting. The occlusion of the wide neck of the pseudoaneurysm was performed successfully using the atrial septal occluder with no detected complications in his six-month follow-up post-catheterisation.
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Aneurisma Falso , Fibrilación Atrial , Defectos del Tabique Interatrial , Dispositivo Oclusor Septal , Humanos , Lactante , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Cateterismo Cardíaco , Aneurisma Falso/cirugía , Resultado del Tratamiento , Defectos del Tabique Interatrial/cirugíaRESUMEN
BACKGROUND: Cardiac catheterization is usually done routinely in patients with univentricular hearts before palliative Bidirectional Glenn (BDG) surgery. The objective of this study was to compare the outcomes of patients with physiological univentricular hearts and restrictive pulmonary flow that did not undergo routine cardiac catheterization before BDG with the patients that did have cardiac catheterization done. We retrospectively reviewed the data of all patients with single ventricle physiology and restrictive pulmonary blood flow who underwent BDG surgery from January 2016 till December 2020. Patients were divided into two groups: the catheterization and the non-catheterization groups. RESULTS: Out of 93 patients, 25 (27%) underwent BDG surgery without prior cardiac catheterization. The median age of patients was ten months, interquartile range (IQR) was 5-18 months. Tricuspid atresia represented 36% of the non-catheterization group, while unbalanced atrioventricular septal defect and hypoplastic left heart syndrome represented 19% and 17.6% of the catheterization group. No patients in the catheterization group were excluded from further BDG surgery based on the catheterization data. Moreover, no significant differences were found between the patients' groups regarding the length of hospital stay, length of intensive care unit stay, postoperative oxygen saturation, or survival (P = 0.266, P = 0.763, P = 0.543, P = 0456). CONCLUSIONS: Although pre-BDG cardiac catheterization is the routine and standard practice, in certain situations, some patients with single ventricle physiology and restrictive pulmonary blood flow may go directly to BDG without cardiac catheterization if noninvasive imaging is satisfactory on a case-by-case basis and according to center experience. Pre-BDG catheterization could be reserved for patients with limited echocardiographic studies, high-risk patients, or those indicated for catheter intervention before BDG surgery.
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Cardiac catheterization can affect clinical outcomes in patients on extracorporeal membrane oxygenation (ECMO) after congenital heart surgery; however, its effect in this group of patients remains unclear. This study aimed to evaluate the safety and outcome of cardiac catheterization in patients undergoing ECMO after congenital cardiac surgery and determine predictors that influence successful weaning. This retrospective cohort study included pediatric patients who underwent cardiac catheterization while on ECMO after congenital heart surgery in two cardiac centers between November 2012 and February 2020. Predictors of successful weaning from ECMO were studied using univariate and multivariate logistic regression analyses. Of 123 patients on ECMO support after congenital cardiac surgery, 60 patients underwent 60 cardiac catheterizations (31 diagnostic and 29 interventional). Thirty-four (56.7%) and 22 patients (36.7%) underwent successful decannulation from ECMO support and survived after hospital discharge, respectively. Patients who underwent earlier catheterization (within 24 h of ECMO initiation) had more successful weaning from ECMO and survival compared to others. Patients who underwent an interventional procedure (interventional catheterization or redo cardiac surgery after cardiac catheterization) had better survival than those who underwent only diagnostic catheterization (P = 0.038). Shorter durations of ECMO was the most important predictor of successful weaning from ECMO. Early cardiac catheterization greatly impacts successful weaning from ECMO and survival. Patients with correctable lesions amenable either by catheterization or redo surgery are more likely to survive. Shorter durations of ECMO could have a significant influence on successful weaning from ECMO and survival.
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Procedimientos Quirúrgicos Cardíacos , Oxigenación por Membrana Extracorpórea , Cardiopatías Congénitas , Cateterismo Cardíaco/efectos adversos , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Oxigenación por Membrana Extracorpórea/efectos adversos , Cardiopatías Congénitas/cirugía , Humanos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Ductal stenting is the preferred method of securing adequate pulmonary blood flow in patients with duct-dependent pulmonary circulation. The main limitation in most centers is the difficult vertical tubular or convoluted ducts that represent real challenges to interventional pediatric cardiologists. We present our experience in patent ductus arteriosus (PDA) stenting with some technical tips to overcome difficulties, especially in stenting tortuous or long tubular ducts. This study was conducted on all patients with cyanotic congenital heart disease who underwent PDA stenting between January 2011 and December 2018. RESULTS: We attempted to stent the PDA in 43 patients, with a success rate of 93% (40 patients) and only one procedural mortality. There was also one stent migration that needed to be treated with urgent surgery. Three-fourths of the patients had difficult ductal morphology and origin. One stent was used to cover the PDA in 27 patients (62.8%), two stents were used in 13 (30.2%), and three stents were used in 2 patients (4.6%). In-stent stenosis rate was 12.5% (5 patients) and the development of progressive left pulmonary artery stenosis was seen in two patients (5%). Pulmonary artery growth was adequate in all patients. CONCLUSIONS: PDA stenting is an effective method of palliation for patients with duct-dependent pulmonary circulation. It has low morbidity and mortality rates. Stenting difficult ducts have become more feasible with evolving materials and techniques.
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BACKGROUND: Studies about the incidence and severity of coronavirus disease 2019 (COVID-19) in children are still significantly lower than those in adults. Moreover, data on the effect of COVID-19 in children with congenital heart disease (CHD) are limited. To the best of our knowledge, this study first reported mortality in a child with CHD who acquired COVID-19. CASE SUMMARY: A 16-month-old boy presented to the emergency department due to shortness of breath, fever, cough, and poor oral intake. He tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). He required mechanical ventilation for rapidly progressing respiratory failure. The patient had a large mid-muscular ventricular septal defect (VSD) that was closed percutaneously at the age of 13 months. Moreover, we followed his hospital sequelae from admission to death. DISCUSSION: This child had multiple risk factors, including malnutrition and persistent pulmonary hypertension (PH) after late closure of the VSD. The pre-existing PH could have been aggravated by the lung condition associated with COVID-19 and the respiratory failure triggered by SARS-CoV-2 infection. The patient presented with ventricular systolic dysfunction, elevated troponin serum levels and newly developed trifascicular block, which were indicative of myocardial injury. The elevated inflammatory markers and multi-organ dysfunction seem to corroborate multisystem inflammatory syndrome in children, which was described recently among paediatric patients with COVID-19.
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Neonates with congenital heart disease are at a high risk of vascular thrombosis. Thrombosis may occur due to vascular injury, increased blood viscosity secondary to polycythemia associated with congenital cyanotic heart diseases, or stasis of blood flow associated with low cardiac output (Schmidt B & Andrew M., Pediatrics 1995; 96: 939-943. Veldman A et al.,Vasc Health Risk Manag 2008; 4: 1337-1348).
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Cardiopatías Congénitas , Trombosis , Arterias , Niño , Cianosis , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Extremidad SuperiorRESUMEN
BACKGROUND: Cardiac catheterization after congenital heart surgery may play an important role in the diagnosis and management of patients with a complicated or unusual post-operative course. The main objective of this study was to evaluate the safety, efficacy, and outcome of cardiac catheterization performed in the early post-operative period following congenital heart surgery. All patients who underwent cardiac catheterization after congenital heart surgery during the same admission of cardiac surgery from November 2015 to May 2018 were included in the study. RESULTS: Thirty procedures were performed for 27 patients (20 interventional and 10 diagnostic). The median age of the patients was 15 months (15 days to 20 years), median weight was 8.2 kg (3.4 to 53 kg), and median time from surgery was 3 days (0-32 days). Eleven procedures were performed for 11 patients on extracorporeal membrane oxygenation (ECMO) support. The main indications for catheterization included the inability to wean from ECMO (10 procedures) and cyanosis (10 procedures). Interventional procedures included angioplasty using stents (10 procedures, success rate of 90%), angioplasty using only balloons (2 procedures, success rate of 50%), and occlusion for residual shunts (8 procedures, success rate of 100%). No mortality was recorded during any procedure. Vasoactive-inotropic score had significantly decreased 48 h after catheterization when compared to pre-catheterization scores (p = 0.0001). Moreover, 72% of patients connected to ECMO support were successfully weaned from ECMO after catheterization. Procedural complications were recorded in 3 interventional procedures. Survival to hospital discharge was 55.5% and overall survival was 52%. Patients on ECMO support had a higher mortality than other patients. CONCLUSION: Cardiac catheterization can be performed safely in the early post-operative period, and it could improve the outcome of the patient (depending on the complexity of the cardiac lesions involved).
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BACKGROUND: We reported our experience in managing patients with single ventricle (SV) physiology and increased pulmonary blood flow (PBF), aiming to assess if it is feasible to proceed with primary Bidirectional Glenn (BDG) without a prior operation to limit PBF. MATERIALS AND METHODS: This is a retrospective study with 51 consecutive patients who underwent BDG operation as a primary operation or a second stage prior to the definitive Fontan operation at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia between 2010 and 2018. Patients were categorized into two groups based on their PBF prior to the operation: Patients who had SV physiology and increased PBF (seven patients) vs. patients with SV physiology and restricted PBF (44 patients). RESULTS: The median age for the increased PBF group was 9.9 months [interquartile range (IQR): 2-16.9 months], and the median age for the restricted PBF group was 15.3 months (IQR: 6.7-42.6 months). Although the length of hospital stay was longer in patients with increased PBF (P = 0.039), we couldn't find a statistically significant difference in early mortality, duration of mechanical ventilation, length of pleural drainage, and length of intensive care unit (ICU) stay between the groups. CONCLUSION: In our experience, we found that primary BDG could be done safely for patients having SV physiology and increased PBF with acceptable short-term outcomes. It might further reduce the morbidity and mortality for those patients by avoiding the risk of initial pulmonary artery banding or aortopulmonary shunts.
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Velocidad del Flujo Sanguíneo/fisiología , Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Circulación Pulmonar/fisiología , Preescolar , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/fisiopatología , Ventrículos Cardíacos/cirugía , Humanos , Lactante , Masculino , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
BACKGROUND: Currently, non-valved conduits are preferred for extracardiac total cavo-pulmonary connection (TCPC). However, previous work has failed to provide objective data comparing the postoperative outcome between non-valved TCPCs and bovine jugular vein valved xenograft (BJV) TCPCs. Hence, the objective of this study is to compare the postoperative outcomes in extracardiac TCPC patients who received BJV vs synthetic non-valved conduits and evaluate the effect of BJV on liver fibrosis. METHODS: Of 206 patients who had extracardiac TCPC from 2002 to 2017 were divided into three groups. Group A (n = 66) received BJV, group B (n = 37) received PET conduits and group C (n = 103) received polytetrafluoroethylene (PTFE) tube. Study endpoints were hospital outcomes, conduits thrombosis, reinterventions, and survival. Liver stiffness and fibrosis were assessed in eight patients with BJV. RESULTS: Preoperative parameters were comparable among groups. Thrombosis was significantly lower in group C (P < .0003) but no difference between groups A and B (P = .951). Reinterventions did not differ significantly among groups (Log-rank P = .598). Hospital deaths occurred in seven patients (3.4%). There was no difference in survival between groups (Log-rank P = .221). The median liver stiffness score was 18.65 kPa and the eight patients had advanced liver fibrosis (grade F3-4) in group A. CONCLUSION: PTFE is the recommended conduit for TCPC with a lower risk of thrombosis compared to BJV and PET. BJV conduits in TCPC circuits may not protect against liver fibrosis. BJV should not be considered as an option for TCPC.
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Bioprótesis , Procedimiento de Fontan/métodos , Cardiopatías Congénitas/cirugía , Venas Yugulares/trasplante , Cirrosis Hepática/prevención & control , Complicaciones Posoperatorias/prevención & control , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Trombosis/prevención & control , Trasplante Heterólogo/efectos adversos , Vena Cava Inferior/anomalías , Vena Cava Inferior/cirugía , Animales , Bioprótesis/efectos adversos , Bovinos , Niño , Preescolar , Femenino , Humanos , Cirrosis Hepática/etiología , Masculino , Politetrafluoroetileno , Complicaciones Posoperatorias/etiología , Trombosis/etiología , Resultado del TratamientoRESUMEN
Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated coronavirus disease 2019 (COVID-19) most commonly causes a mild respiratory illness; however, there are wide ranges of presenting symptoms and disease severity. It has a mortality rate around 7%. Case presentation: We present a case of a 9-year-old female patient with hypoplastic left heart syndrome status post heart transplantation at age of 7 days. She presented to our emergency room complaining of intermittent fever, chills, fatigue, poor appetite, and diarrhea.A throat swab nucleic acid test was positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Intravenous fluids therapy was used for correction of hydration status. To the best of our knowledge this is the first reported case of non-pulmonary presentation of coronavirus disease-2019 (COVID-19) in a pediatric heart transplant recipient, which was successfully managed conservatively. Conclusions: Gastrointestinal manifestations can be the only presenting symptom in pediatric heart transplant recipients with COVID-19. Conservative treatment could be used successfully. Immunomodulatory medications that are used in heart transplant recipients may have protective value in SARS-CoV-2 infection.
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BACKGROUND: Transcatheter closure of secundum atrial septal defect is routinely performed under general anesthesia and transesophageal echocardiography guidance. If patients have good echo windows, the procedure could be performed under transthoracic echo guidance. AIM OF STUDY: To evaluate safety and efficacy of the intervention using fluoroscopy and echo guidance. METHODS: In a case control study design, 180 patients underwent atrial septal defect closure between January 2010 and December 2016. In 32 patients, the intervention was performed under fluoroscopy and transthoracic echo guidance. Our study group consisted of 22 out of 32 patients (<13â¯years old). For the other 10 patients, we could not find a matching pair. The data of the study group were compared with an age, weight, and height matched group (controls), who underwent the procedure under transesophageal echocardiography guidance. RESULTS: The diameter of the atrial septal defect, septal length, and most of the rims were comparable. The superior rim and inferior rims were longer in the study group. The devices chosen for the cases were larger than the control group. Procedure time and fluoroscopy times were shorter in the study group. Success rate was comparable. On follow-up, both groups had almost no or minimal incidence of residual shunt. CONCLUSION: We conclude that transcatheter closure of atrial septal defect under fluoroscopy and transthoracic echo guidance is safe and successful in selected patients who have single central atrial septal defect with adequate septal lengths and adequate septal rims, with high incidence of complete occlusion rate.
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BACKGROUND: Isolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described. CASE PRESENTATION: We describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully. CONCLUSIONS: The superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.
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Embolización Terapéutica , Atrios Cardíacos/anomalías , Cardiopatías Congénitas/cirugía , Malformaciones de la Vena de Galeno/terapia , Vena Cava Superior/anomalías , Anomalías Múltiples/terapia , Atrios Cardíacos/diagnóstico por imagen , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Lactante , Masculino , Malformaciones de la Vena de Galeno/complicaciones , Vena Cava Superior/diagnóstico por imagenRESUMEN
OBJECTIVE: To assess the implementation and efficiency of medical education among the caregivers of children with congenital heart disease (CHD) and to investigate its success factors. MATERIAL AND METHOD: A cross-sectional study was conducted from February 2014 to March 2014 in a tertiary healthcare center. Study included 120 caregivers of children with CHD visiting the Paediatric Cardiology Clinic that included 93% mothers, aged 31.72 ± 6.67 years, 38.3% Saudi citizens and 45.0% had a high educational level. Caregivers were interviewed through a semi-structured questionnaire that included socio-demographic data of participants and demographic and clinical data of patients; awareness about the diagnosis and self-assessed understanding of thedisease; disease dimensions explained, educational material used andefficacy of whole medical education received in alleviating caregivers' anxiety. RESULTS: Of children who had CHD, around 42.5% were males aged 5.65 ± 3.99 years and 37.3% had ventral septal defect, 14.4% had atrial septal defectand 19.5% had a complex CHD. Study results showed that 83.3% of caregivers were aware of the correct diagnosis and 64.2% had good understanding of the nature and effect of the disease. Frequently explained disease dimensions were impact on child's growth (65.8%), complications (57.5%), and outcomes (55.0%). Least frequently explained dimensions were those related to infective endocarditis (IEC) including risk factors (15.8%), prophylaxis (17.5%), complications (19.2%) and symptoms (22.5%). The whole medical education received was efficient to enable good understanding of the disease and alleviate anxiety in 70.8% and 62.5% of the cases, respectively. Time dedicated to educate, use of illustrating educational materials, explaining various disease dimensions (aetiology, complications, prognosis, long-term management, etc.) and the number of dimensions explained were all significant predictors for both good understanding of the child's disease and anxiety relief among caregivers.
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INTRODUCTION: The term cor triatriatum is used to describe a fibromuscular partition that divides an atrium into two compartments. It was first used by Borst in 1905, although the lesion had been described before by Church in 1868. Both described divided left atrial chamber (also referred to as cor triatriatum sinister). The incidence of the divided left atrial chamber among patients with congenital heart disease is reported at 0.1% and 0.4% in an autopsy study. Divided right atrial chamber - also referred to as cor triatriatum dexter - is even rarer to an extent that there is no reported incidence and only few hundred cases have been reported so far. A search through pubMed library revealed only 90 publications on humans, either under the term "dexter" or "dextrum". We would like to report a new association of bilateral divided atrial chambers and pulmonary vein stenosis, with a review of the literature.
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Constricción Patológica/diagnóstico por imagen , Corazón Triatrial/diagnóstico por imagen , Venas Pulmonares/diagnóstico por imagen , Ascitis/etiología , Constricción Patológica/complicaciones , Corazón Triatrial/complicaciones , Descompresión Quirúrgica , Ecocardiografía Doppler en Color , Femenino , Humanos , Lactante , Derrame Pleural/etiología , Vena Cava Inferior/diagnóstico por imagenRESUMEN
BACKGROUND: Protrusion of the patent ductus arteriosus (PDA) stent can occur into the lumen of the main pulmonary artery (MPA) branch, the aorta, or both. This protrusion can vary from trivial to major, causing potential obstruction to the vessel lumen, which may cause flow obstruction or risk of thromboses. As far as we know, no one has followed these patients with protruding stents to see whether they do pose a risk of obstruction or thromboses. METHODS: A retrospective, descriptive, cross-sectional study reviewing charts of all included patients who received stents in the MPA branches with residual protrusion into the pulmonary artery branch lumen (total, 87 patients; 34 patients with protruding stents) was performed to determine whether this protrusion caused any undesired effects on flow or coagulation. The patients were divided into two groups: the protruding stents group (group 1); and the non-protruding stent group that served as a control group (group 2). Each group was then categorized into 3 sections according to the stent position, the PDA, the MPA branches, and the Blalock-Taussig shunt. RESULTS: The only risk factor that had statistical significance was the number of stents in the PDA site. CONCLUSION: Protruding stents do not cause an increased risk of thrombosis in patients on aspirin. Mild protrusion is more likely in PDA stents and severe protrusion is more likely in the MPA branch stents. Severe protrusion is more likely when more stents are used in the PDA location. There is no statistical evidence that protrusion can cause lung perfusion defects from the small numbers we have.
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Cateterismo Cardíaco/métodos , Conducto Arterioso Permeable/terapia , Cardiopatías Congénitas/terapia , Cuidados Paliativos/métodos , Stents/efectos adversos , Procedimiento de Blalock-Taussing , Preescolar , Estudios Transversales , Femenino , Humanos , Lactante , Masculino , Arteria Pulmonar/fisiología , Circulación Pulmonar/fisiología , Estudios Retrospectivos , Factores de Riesgo , Trombosis/epidemiología , Resultado del TratamientoRESUMEN
Moderate to severe pulmonary hypertension is considered to be an absolute contraindication to the performance of bidirectional Glenn (BDG) shunting. However, BDG shunting has been performed in young children with pulmonary hypertension associated with unrestricted pulmonary blood flow. In this study, the medical records of patients who underwent BDG starting from October 2000 to March 2004 were reviewed. Patients were divided into 2 groups on the basis of indexed pulmonary vascular resistance (PVRI) measured in room air: a high-risk group (n = 12) with PVRI > or = 3.5 Woods units (WU)/m(2) and a low-risk group (n = 28) with PVRI <3.5 WU/m(2) in room air. The 2 groups were comparable with respect to age, weight, ventricular morphology, pulmonary arterial anatomy, and atrioventricular valve function. Mean pulmonary arterial pressure and PVRI were significantly higher in the high-risk group compared with the low-risk group (39.2 +/- 20.7 vs 15.1 +/- 6.25 mm Hg, p <0.002, and 6.0 +/- 2.5 vs 1.6 +/- 0.82 WU/m(2), p <0.0005, respectively). The ratio of pulmonary flow to systemic flow was 1.45 +/- 0.76 in the high-risk group and 1.24 +/- 1.2 in the low-risk group. In the high-risk group, mean PVRI decreased to 2.0 +/- 1.0 WU/m(2) on 100% oxygen (p <0.0005). A contraindication to Glenn shunting was PVRI >3.5 WU/m(2) on 100% oxygen. Hospital mortality was 17% (2 of 12) in the high-risk group and 4% (1 of 28) in the low-risk group. Of 10 survivors in the high-risk group, 1 had undergone a Kawashima procedure, 7 had undergone Fontan procedures (with 1 death), and 2 were awaiting the completion of Fontan procedures as of this writing. In conclusion, these preliminary data suggest that in young children with increased pulmonary flow, BDG shunting can be safely performed, despite the apparent elevation of pulmonary arterial pressure to inoperable levels, provided PVRI decreases to < or = 3.5 WU/m(2) on 100% oxygen.