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A 21-year-old patient with complex cyanotic congenital heart disease and highly symptomatic severe common atrioventricular valve regurgitation was deemed too high risk for surgical intervention or transplantation. She successfully underwent transcatheter edge-to-edge repair with resulting considerable improvement in her symptoms, renal function, and quality of life.

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BACKGROUND: In light of prolonged hypoxia, children with cyanotic heart disase (CHD) are at a high risk of developing iron deficiency iron deficiency (ID) and iron deficiency anemia (IDA). Reticulocyte hemoglobin equivalent (Ret-He) is a novel and dependable indicator for assessing iron status. However, there has been no previous study regarding cut-off value in pediatric CHD group. The purpose of this study is to assess the role of Ret-He and to establish cut-off points in the diagnosis of iron deficiency and IDA in pediatric cyanotic heart disease. METHOD: This study was conducted in two tertiary hospitals in Jakarta, Indonesia. 59 children with CHD, aged 3 months to 18 years, were enrolled consecutively. To determine iron status, hematological parameters (hemoglobin, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin) and biochemical parameters for iron status (serum ferritin, transferrin saturation) were analysed and compared to Ret-He levels. The receiver operating characteristic (ROC) analysis was performed for the Ret-He cut-off points for ID and IDA. Sensitivity, specificity, positive and negative predictive value were calculated for each cut-off point. RESULT: Normal iron status was identified in 27 (45.8%) subjects, ID in 8 (13.5%) subjects, and IDA 24 (40.7%) subjects. The ID cut-off value for Ret-He is 28.8 pg (sensitivity 75%, specificity 85.2%, PPV 60%, NPV 92%, and AUC 0.828) and the Ret-He cut-off point for IDA is 28.15 pg (sensitivity 75%, specificity 88.9%, PPV 85.7%, NPV 80%, and AUC 0.824). Hemoglobin should be used in conjunction with Ret-He. ID might be detected in this cohort with Ret-He 28.8 pg and hemoglobin > 16,5 g/dL. While Ret-He 28.15 pg or Ret-He 28.15-28.8 pg with hemoglobin 16.5 g/dL could be used to diagnose IDA. CONCLUSION: The reticulocyte hemolgobin equivalent could be utilised as an iron status parameter in pediatric CHD, with a cut-off value of 28.8 pg for ID and 28.15 pg for IDA.

Asunto(s)

Anemia Ferropénica , Cardiopatías Congénitas , Hemoglobinas , Deficiencias de Hierro , Reticulocitos , Humanos , Anemia Ferropénica/diagnóstico , Anemia Ferropénica/sangre , Anemia Ferropénica/etiología , Preescolar , Masculino , Indonesia , Femenino , Lactante , Niño , Hemoglobinas/análisis , Reticulocitos/metabolismo , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/diagnóstico , Adolescente , Cianosis/sangre , Cianosis/etiología , Cianosis/diagnóstico , Curva ROC , Sensibilidad y Especificidad , Biomarcadores/sangre , Hierro/sangre , Ferritinas/sangre

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We present a case of a full-term newborn with complex congenital heart defects, including single-ventricle physiology and discontinuous pulmonary arteries. Prompt surgical intervention was performed, which involved pulmonary neoconfluence with autologous pericardium graft and systemic-to-pulmonary shunt placement. However, postoperative complications required stenting to address pulmonary artery stenosis.

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Background: Documented symptomatic progression of a paraganglioma (PGL) over many years is unusual. Our objective is to report a young man with such an occurrence. Case Report: A 27-year-old male presented with headache, sweating, and palpitation. He had a history of cyanotic congenital heart disease. Five years before presentation, he had 24-hour urine metanephrines 43 mcg/d (25-222), vanillylmandelic acid 3 mg/d (<6), and homovanillic acid 2.4 mg/d (1.6-7.5) levels and a 3.13 cm mass in the upper aortocaval space. Subsequent imaging showed slow growth of the mass. On admission, his blood pressure was 197/134 mm Hg, heart rate was 163 beats per minute, respiratory rate was 25 per minute, and oxygen saturation was 76% on room air. His 24-hour urine normetanephrine level was 2644 mcg/d (81-667) while metanephrine was 405 mcg/d (55-320). Plasma free metanephrine level was 0.92 nmol/L (0-0.49) and normetanephrine was 11.85 nmol/L (0-0.89). DOTATATE positron emission tomography-computed tomography revealed a 4.3 × 3.1 × 4.9 cm mass with activity in the right upper aortocaval space. He was treated with Prazosin. Two months later, he underwent resection of the mass. Pathology diagnosed a 4.9 cm PGL. He had improvement in metanephrine levels. Discussion: PGL is diagnosed by documenting excess catecholamines and identifying a lesion on imaging. False negative laboratory testing is rare but can occur. Patients with cyanotic congenital heart disease have a greater risk of developing PGL. Conclusion: It is crucial to evaluate a patient for PGL if clinical conditions suggest catecholamine excess, especially if a retroperitoneal tumor has grown or the patient has risk factors.

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Bombay blood group is one of the rarest blood types with a prevalence of 1 per 10,000 population in India. Children and adults of this blood group can receive autologous blood or blood from an individual with a Bombay phenotype only. Children with grown-up uncorrected cyanotic heart disease are associated with a high risk of perioperative hemorrhagic diathesis and may require multiple blood transfusions. Arrangement of adequate Bombay blood group units for pediatric cardiac surgery is a unique challenge. The COVID-19 pandemic brought about additional difficulties in the procurement of blood components due to donor hostility. Despite the associated risks, pre-operative multi-session autologous blood harvest under monitored anesthesia care was planned in a 16-year-old child and four units of autologous blood were harvested and preserved for performing total correction surgery.

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Antígenos de Grupos Sanguíneos , Procedimientos Quirúrgicos Cardíacos , Adulto , Humanos , Niño , Adolescente , Pandemias , Transfusión Sanguínea , Corazón

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Background Intraoperative hypofibrinogenemia is a major factor associated with increased postoperative blood loss in adult cardiac surgery. However, previous pediatric studies on this topic did not sufficiently adjust for potential confounders and variations in surgeons' techniques. Therefore, evidence for the association between hypofibrinogenemia and postoperative blood loss after cardiac surgery in children remains insufficient. In this study, we aimed to evaluate the association between postoperative blood loss and hypofibrinogenemia by adjusting for potential confounders and the effects of differences in surgeons' techniques. Methodology This single-center, retrospective, cohort study included children who underwent cardiac surgery with cardiopulmonary bypass from April 2019 to March 2022. Multilevel logistic regression models with mixed effects were used to evaluate the association of major blood loss in the first six hours postoperatively with fibrinogen concentration at the end of cardiopulmonary bypass. The difference in the surgeon's techniques was adjusted as a random effect for the model. The model included potential confounders identified as risk factors in previous studies. Results A total of 401 patients were included. A fibrinogen concentration ≤150 mg/dL (adjusted odds ratio (aOR) = 2.08; 95% confidence interval (CI) = 1.18-3.67; p = 0.011) and the presence of cyanotic disease (aOR = 2.34; 95% CI = 1.10-4.97; p = 0.027) were associated with major blood loss in the first six postoperative hours. Conclusions A fibrinogen concentration ≤150 mg/dL and the presence of cyanotic disease were associated with postoperative blood loss in pediatric cardiac surgery. Maintaining a fibrinogen concentration >150 mg/dL is recommended, especially for patients with cyanotic diseases.

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Tetralogy of Fallot (TOF) presenting with superior vena cava (SVC) syndrome is an exceedingly rare scenario. The diagnosis of this condition is still a difficult task due to its unspecific clinical features and unclear recommendations for the best diagnostic tools. We report an interesting case of a 23-year-old male who was found to have TOF with the presentation of SVC syndrome after a sequential diagnosis. The timely diagnosis helped in the successful management of the patient. Through this case, we want to make clinicians aware of such a rare association and its diagnosis.

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Most adult patients who experience recurrent hemoptysis have respiratory or coagulopathy-related causes and cardiac aetiology in very few cases. In this rare case of 56 years aged male patient who presented to us with chronic recurrent hemoptysis, Tetralogy of Fallot (TOF) was the culprit aetiology, and he was successfully managed by minimal intervention.

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BACKGROUND: Proboscis lateralis (PL) is an uncommon congenital facial deformity marked by the protrusion of a primitive tubular structure made up of skin and soft tissue that generally emerges from the eye's medial canthus and is associated with some craniofacial deformities. We report the first case of PL with multiple craniofacial, neurological, cardiac, and spinal anomalies. CASE PRESENTATION: A full-term female baby delivered by cesarean section cried immediately at birth. The mother reported having a normal pregnancy but has a history of x-ray during her first trimester. The baby was born with a rare presentation of proboscis lateralis which was accompanied by multiple anomalies, including but not limited to bilateral colpocephaly, corpus callosum agenesis, complex cyanotic congenital heart disease, and hemivertebra of the T10 body. CONCLUSION: PL is an uncommon congenital condition that causes a variety of craniofacial abnormalities. Multiple additional defects affecting various organ systems should also be evaluated in a person diagnosed with PL.

Asunto(s)

Anomalías Múltiples , Anomalías Craneofaciales , Embarazo , Lactante , Recién Nacido , Humanos , Femenino , Nariz/anomalías , Cesárea , Cara , Anomalías Craneofaciales/diagnóstico , Anomalías Craneofaciales/diagnóstico por imagen

RESUMEN

Our patient was a 50-year-old woman with tricuspid atresia who had undergone palliation with a Potts shunt to the left pulmonary artery as an infant and a classic Glenn shunt to the right pulmonary artery as a young child. Under general anesthesia, she underwent transcatheter edge-to-edge repair of the mitral valve for severe symptomatic mitral regurgitation. (Level of Difficulty: Advanced.).

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Background: The aim of the study was to compare the predictive value of Sonoclot analysis and thromboelastography (TEG) for postoperative bleeding in children younger than 12 years coming for cardiac surgery for congenital cyanotic heart disease. Methods: This is a prospective, observational study carried out in a single tertiary care military hospital. Ninety patients of the paediatric age group undergoing bypass cardiac surgery for correction of congenital cyanotic heart defect were included in the study. Laboratory-derived values to assess coagulation status (prothrombin time, international normalisation ratio, activated partial thromboplastin time) and point-of-care Sonoclot- and TEG-derived parameters were noted at the start of surgery and postoperatively in all patients. Bleeders were predefined on the basis of chest tube drainage. Results: The incidence of bleeders was 42.2% (38/90 patients), whereas 57.8% (52/90 patients) were non-bleeders. The postoperative R value and preoperative gbPF test were predictive for postoperative bleeders on multivariate analysis. Postoperative gbPF had the highest area under the curve (0.72), with a cut-off value of 1.75, and gbPF had 82% sensitivity and 71% specificity in predicting significant postoperative bleeding in paediatric cyanotic congenital heart surgeries. Transfusion requirements and mechanical ventilation duration were higher in bleeders; however; length of intensive care unit stay, incidence of sepsis and mortality were similar in both the groups. Conclusion: Bleeding in patients undergoing corrective surgery for cyanotic congenital heart disease could be predicted by the preoperative gbPF and postoperative R value. Among these, preoperative gbPF has the maximum predictive value.

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Relentless human curiosity to understand the basis of every aspect of medical science has led humanity to unlock the deepest secrets about the physiology of human existence and, in the process, has reached milestones that a century ago could only be imagined. Recent ground-breaking breakthroughs have helped scientists and physicians all over the world to update the scientific basis of diseases and hence further improve treatment outcomes. According to recent studies, scientists have found a link between intestinal flora and the pathogenesis of diseases, including cardiovascular diseases. Any change in the typical habitat of gut microbiota has been shown to result in the culmination of various metabolic and cardiac diseases. Therefore, gut microbiota can be credited for influencing the course of the development of a disease. Any change in the composition and function of bacterial species living in the gut can result in both beneficial and harmful effects on the body. Gut microbiota achieves this role by numerous mechanisms. Generations of various metabolites like TMAO (trimethylamine N-oxide), increased receptibility of various bacterial antigens, and disruption of the enzyme action in various metabolic pathways like the bile acids pathway may result in the development of metabolic as well as cardiovascular diseases. Even if they may not be the only etiological factor in the pathogenesis of a disease, they may very well serve as a contributing factor in worsening the outcome of the condition. Studies have shown that they actively play a role in the progression of cardiovascular diseases like atherosclerotic plaque formation and rising blood pressure. The focus of this review article is to establish a relation between various cardiovascular diseases and gut microbiota. This could prove beneficial for clinicians, health care providers, and scientists to develop novel therapeutic algorithms while treating cardiac patients.

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PURPOSE: Representative neonatal surgical diseases are often complicated by congenital heart disease (CHD). We reviewed our decade of experience from the perspective of the prognosis and report on the management of infants with CHD. METHODS: Cases with and without CHD between 2011 and 2020 were retrospectively compared. Qualitative data were analyzed using a chi-square test with Yates' correction, and quantitative data were compared using Student's t-test. RESULTS: Of the 275 neonatal surgical cases, 36 had CHD (13.1%). Ventricular septal defect was the most common cardiac anomaly, followed by atrial septal defect. Esophageal atresia showed the highest complication rate of CHD (43.8%, 14/32) followed by duodenal atresia (38.5%, 10/26). The mortality rates of patients with and without CHD (22.2% [8/36] vs. 1.3% [3/239]) were significantly different (χ2 = 30.6, p < 0.0001). Of the eight deaths with CHD, six patients had cyanotic complex CHD. Notably, four of these patients died from progression of inappropriate hemodynamics in the remote period after definitive non-cardiac surgery. CONCLUSION: Considering its high-mortality, the presence of CHD, especially cyanotic heart disease, is an important issue to consider in the treatment of neonatal surgical diseases. Pediatric surgeons should be alert for changes in hemodynamics after surgery, as these may affect mortality.

Asunto(s)

Cardiopatías Congénitas , Defectos del Tabique Interventricular , Niño , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/cirugía , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/cirugía , Humanos , Lactante , Recién Nacido , Pronóstico , Estudios Retrospectivos

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Paragangliomas (PGL) in paediatric patients are rarely diagnosed with neuroendocrine tumours. Anaesthetic management of paraganglioma is challenging due to exaggerated haemodynamic alterations. Further associated cardiovascular diseases like congenital cyanotic heart disease (CCHD) with paragangliomas make its management daring and if not properly managed can increase morbidity. We herein discuss the successful anaesthetic management of a 10-year-old child with paraganglioma and associated atrial septal defect (ASD) with pulmonary stenosis (PS) for adrenalectomy. Overlapping the clinical spectrum between CCHD and catecholamine-secreting tumour makes the case very challenging. Management of the patient with CCHD and PGL needs a multidisciplinary approach, and intensive vigilance and monitoring are needed for the successful management of such challenging cases.

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Procedimiento de Fontan , Cardiopatías Congénitas , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/cirugía , Humanos , Lactante , Arteria Pulmonar/cirugía , Estudios Retrospectivos , Resultado del Tratamiento

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BACKGROUND: Women with tetralogy of Fallot especially uncorrected are at increased risk of adverse maternal and perinatal outcomes. METHOD: We reviewed the maternal complications and pregnancy outcomes of women with tetralogy of Fallot (ToF), having corrected or uncorrected lesion during the period from 2011 to 2019 attending a south-Indian tertiary care center. Data regarding demographics, clinical course, and medications received and echocardiographic diagnosis regarding ToF, labor and delivery details, and postpartum follow-up was collected from the records. For each case, four women matched for age (±2 years), without a diagnosis of heart disease, were included as controls. We compared the outcomes in pregnant women with ToF and controls as well between those who have undergone repair with those without repair and also with the controls. RESULTS: There were 27 pregnancies in 19 patients with ToF, including eight pregnancies in seven women with uncorrected lesions. There was no maternal death or occurrence of infective endocarditis. Women with uncorrected lesion had significantly higher rates of fetal growth restriction (37.5% vs 6.7%), preterm birth (50% vs. 13.3%) resulting in lower birth weight (1907.5 g vs. 2607.3 g)) compared to those with a corrected lesion. Women with corrected lesion had similar outcomes to the controls (p > .5). CONCLUSION: Women with uncorrected ToF are at increased risk of perinatal complications compared to those with corrected lesion and require care under a multidisciplinary team, with an individualized plan for delivery to optimize the outcomes.

Asunto(s)

Complicaciones Cardiovasculares del Embarazo , Nacimiento Prematuro , Tetralogía de Fallot , Femenino , Humanos , India/epidemiología , Recién Nacido , Embarazo , Complicaciones Cardiovasculares del Embarazo/epidemiología , Resultado del Embarazo/epidemiología , Mujeres Embarazadas , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/cirugía

RESUMEN

Brain abscesses are rare but life-threatening conditions associated with morbidity and mortality. They are encountered in unoperated and partially treated cyanotic congenital heart diseases. Our patient is a diagnosed case of transposition of great arteries with a ventricular septal defect. She had recurrent abscesses for which a combination of antimicrobial therapy and surgical excision was performed. Surgical excision carries a great risk of rupture of abscess into the ventricular system and is associated with poorer outcomes. The outcome of brain abscess primarily depends on Glasgow Coma Score (GCS) at the time of admission and ventricular extension of the abscess. Fortunately, our patient showed good results without any apparent neurological sequelae. Early diagnosis of the brain abscess and timely administration of antibiotics help in a good outcome.

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BACKGROUND: We aimed to establish a clinical registry for patients with congenital heart disease who referred to multidetector computed tomography in our country, to describe the pattern and clinical profile of such patients and document the safety and efficacy of the procedure in our daily practice. RESULTS: A total 2310 studies were analyzed after excluding studies with missed, and lost data. Half of our study population-1215 patients-52.5% were males. The median age of the patients was 12 months (IQR 37 months), and the youngest patient was 3 days old. The eldest patient was 50 years old. 68.27% of the patients were less than 2 years old, and two-third of the whole studied population 66.7% had cyanotic heart disease. Minor local access complications, complications related to anesthetic drugs, and allergic reactions were the most commonly encountered complications, with only single mortality mainly due to multiple associated multisystem congenital malformation. CONCLUSIONS: Most of our patients with congenital heart disease referred for MDCT study were infants and young children. The majority of them had complex cyanotic heart disease. The study is safe, with excellent diagnostic yield and safe with very low incidence of complications.

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Complete heart block is a common complication for adults with congenital heart disease (CHD). Epicardial pacing is preferred in patients with septal shunting due to risk of thromboembolism. Anatomic changes in complex CHD may preclude surgical epicardial lead placement. Thromboembolism risk reduction in such patients requiring endocardial pacing remains questionable. (Level of Difficulty: Advanced.).