Successful Cardiac Surgical Management in a Trisomy 21 Child After Long-Term Hospitalization Associated With Bronchopneumonia and Hepatitis C Virus Seropositivity: A Case Report.

A 31-month-old girl with trisomy 21 (Down syndrome) was seen in the emergency department of pediatrics because of oxygen desaturation associated with features of lower respiratory tract infections. She was born at full term and diagnosed with congenital heart disease (CHD) having ventricular septal defect (VSD), and patent ductus arteriosus (PDA); consequently, she underwent corrective surgery after adequate optimization of treatment. Incidentally, she was detected to have the presence of anti-hepatitis C virus (HCV) antibodies. In this case report, we mainly focus on the multi-modal approach to medical and surgical management.

Conservative Surgical Management of a Pulmonary Hydatid Cyst in an Adolescent Having Extra-pulmonary Lesions by a Multi-disciplinary Approach.

Echinococcus granulosus causes hydatid cysts, a significant zoonotic and pulmonary parasitic disease that can mimic various pathologies and is often harder to manage than the disease itself. A hydatid cyst is considered a significant health problem in India, Iran, China, and Mediterranean countries, which lack satisfactory environmental health, preventive medicine, and veterinarian services. Echinococcosis continues to be a major community health burden in several countries, and in some terrains, it constitutes an emerging and re-emerging disease. Cystic echinococcosis is the most common human disease of this genus, and it accounts for a significant number of cases worldwide. Herein, a case involving an 11-year-old presenting with fever, dry cough, and right hypochondrial pain is presented, where imaging revealed a hydatid cyst in the lung. Surgical removal of the cyst was achieved through right posterolateral thoracotomy under one-lung ventilation and anesthesia using intubation with a double-lumen endotracheal tube (DLET or DLT), highlighting surgery as the primary treatment despite the lack of consensus on surgical methods. This case underscores the effectiveness of individualized, parenchyma-preserving surgery for even large, uncomplicated cysts, indicating a positive prognosis.

Surgical Management of Congenital Pulmonary Airway Malformations (CPAM) in an Infant and a Toddler: Case Report Depicting Two Distinct Surgical Techniques With Successful Outcomes.

Congenital pulmonary airway malformations (CPAM) compose the major part of congenital lung malformations (CLM) and have traditionally been treated by pulmonary lobectomy. In terms of surgical strategy, lobectomy has conventionally been the preferred treatment for CPAM localized to a single lobe. More recently, alternative approaches including lung-sparing resections (LSR), such as wedge or non-anatomic resections and segmentectomy, have been suggested. In asymptomatic CPAM early surgical resection is often shown to reduce infection and malignancy development. We describe two patients who were diagnosed with CPAM when being evaluated for respiratory tract infection. Patient 1 (P1) was a two-month-old infant weighing 4 kg with glucose-6-phosphate dehydrogenase (G6PD) deficiency and Patient 2 (P2) was a toddler aged one year, nine months weighing 9 kg. P1 underwent LSR for the CPAM diagnosed in the left upper lobe of the lung with conventional mechanical ventilation whilst right upper lobectomy was performed in P2 using one/single lung ventilation. In both cases, LSR and right upper lobectomy led to an uneventful postoperative recovery with no complications reported.

Identifying left ventricular dysfunction using prospective electrocardiogram-triggered coronary computed tomography angiography.

PURPOSE: Coronary computed tomography angiography (CCTA) is an important non-invasive tool for the assessment of coronary artery disease and the delivery of information incremental to coronary anatomy. CCTA measured left ventricular (LV) mid-diastolic volume (LVMDV) and LV mass (LVMass) have important prognostic information but the utility of prospectively ECG-triggered CCTA to predict reduced left ventricular ejection fraction (LVEF) is unknown. The objective of this study was to determine if indexed LVMDV (LVMDVi) and the LVMDV:LVMass ratio on CCTA can identify patients with reduced LVEF. MATERIALS/METHODS: 8179 patients with prospectively ECG-triggered CCTA between November 2014 and December 2019 were reviewed. A subset derivation cohort of 4352 healthy patients was used to define normal LVMDVi and LVMDV:LVMass. Sex-specific thresholds were tested in a validation cohort of 1783 patients, excluded from the derivation cohort, with cardiac disease and known LVEF. The operating characteristics for 1 SD above the mean were tested for the identification of abnormal LVEF, LVEF≤35 â€‹% and ≤30 â€‹%. RESULTS: The derivation cohort had a mean LVMDVi of 61.0 â€‹± â€‹13.7 â€‹mL/m2 and LVMDV:LVMass of 1.11 â€‹± â€‹0.24 â€‹mL/g. LVMDVi and LVMDV:LVMass were both higher in patients with reduced LVEF than those with normal LVEF (98.8 â€‹± â€‹40.8 â€‹mL/m2 vs. 63.3 â€‹± â€‹19.7 â€‹mL/m2, p â€‹< â€‹0.001, and 1.32 â€‹± â€‹0.44 â€‹mL/g vs. 1.05 â€‹± â€‹0.28 â€‹mL/g, p â€‹< â€‹0.001). Both mean LVMDVi and LVMDV:LVMass increased with the severity of LVEF reduction. Sex-specific LVMDVi thresholds were 79 â€‹% and 80 â€‹% specific for identifying abnormal LVEF in females (LVMDVi â€‹≥ â€‹69.9 â€‹mL/m2) and males (LVMDVi â€‹≥ â€‹78.8 â€‹mL/m2), respectively. LVMDV:LVMass thresholds had high specificity (87 â€‹%) in both females (LVMDVi:LVMass â€‹≥ â€‹1.39 â€‹mL/g) and males (LVMDVi:LVMass â€‹≥ â€‹1.30 â€‹mL/g). CONCLUSION: Our study provides reference thresholds for LVMDVi and LVMDV:LVMass on prospectively ECG-triggered CCTA, which may identify patients who require further LV function assessment.

Hemi-Diaphragm Plication and/or Tracheostomy Are Valuable Adjunctive Procedures After Repair of Congenital Heart Defects in Children: A Systematic Review.

Diaphragmatic paralysis (DP), whether unilateral or bilateral, often leads to extended recovery and more severe complications, particularly in neonates and infants undergoing congenital heart surgery. This condition's impact is most pronounced after single-ventricle palliative procedures. Tracheostomy prevalence is rising in pediatric patients with congenital heart disease (CHD) despite its association with high resource utilization and in-hospital mortality. This study examines the reported incidence of diaphragmatic paralysis and timing of tracheostomy in pediatric patients undergoing surgery for congenital heart disease in the literature and a retrospective analysis of cases in our institution between 2018 and 2023, offering insights for prospective management. An electronic search of PubMed databases retrieved 10 studies on pediatric tracheostomy and 11 studies on DP. Our retrospective analysis included 15 patients, of whom 10 underwent tracheostomy, four underwent diaphragmatic plication, and one underwent both. Postoperative tracheostomy had an 11.8% mortality rate in our systematic review, rising to 40% in our observational study. Diaphragm repair and early diagnosis can reduce morbidity, prevent complications, and improve patients' quality of life.

Validation of a Clinical Prediction Rule for Distinguishing Bacterial and Aseptic Meningitis in Pediatric Patients.

Introduction The treatments and prognosis of bacterial meningitis differ greatly from those of aseptic meningitis, making early identification and differentiation essential. Several different clinical prediction rules have been developed to distinguish bacterial meningitis from aseptic meningitis. We sought to validate one clinical prediction rule for pediatric patients utilizing a centralized data warehouse that collects daily data from 184 hospitals across the United States. Methods We retrospectively collected data on all patients aged 29 days to 14 years who presented to Hospital Corporation of America (HCA) Healthcare hospitals from January 1, 2016, to May 31, 2021, with a diagnosis of meningitis. Our study replicated the original study of the meningitis score for emergencies (MSE) for the pediatric clinical prediction rule and assigned 3 points for procalcitonin (PCT) >1.2 ng/dL, 2 points for CSF protein >80 mg/dL, and 1 point for each of the other variables of C-reactive protein (CRP) >40 mg/L and CSF absolute neutrophil count >1000 cells per mm3. Patients were categorized either as having bacterial or aseptic meningitis. Using the clinical prediction rule, a calculation of the sensitivity, specificity, positive predictive value, negative predictive value, and receiver operating characteristic (ROC) curve was performed. Results The optimum test characteristic was found to have a score of ≥ 3, showing a sensitivity of 92.86% (95% CI, 83.3-100), a specificity of 65.22% (95% CI, 51.5-79), a positive predictive value of 61.90% (95% CI, 47.2-76.6), and a negative predictive value of 93.75% (95% CI, 85.4-100). The ROC curve from this study showed an area under the curve (AUC) of 0.7892 (95% CI, 0.681-0.897). Conclusion Our study validated a high sensitivity for distinguishing bacterial meningitis from aseptic meningitis, suggesting the clinical prediction rule has clinical utility as a predictive screening tool. Although the original MSE advised a cutoff score of ≥1, our study suggests that a score ≥3 would give the best test performance.

Revival of Brock's Operation for Intermediary Palliation of Fallot's Tetralogy in Children Anatomically Unsuitable for One-Stage Total Correction of the Anomaly: Interim Results of Two Cases.

One-stage total correction is known to be anatomically unsuitable for correcting tetralogy of fallot (TOF) in a certain proportion of children. Surgeons are thus faced with dilemmas regarding which preliminary operation for the anomaly to do first. Brock's primary postulation suggests that pulmonary trunk and annulus enlargement leading to the correction of the outflow obstruction will favor the subsequent total correction. In line with this, the current article presents two patients who were 6 months and 5 years old. The first patient underwent primary Brock's operation while the second patient had a blocked modified Blalock-Taussig's shunt (MBTS) done off-pump. Following the discontinuation of anti-platelet medications, the MBTS blocked and the patient was subsequently considered for secondary Brock's operation. The outcome of both procedures involved the patients' discharge with uneventful hospital stays and regular follow-ups at specified intervals. Thus, Brock's operation is an excellent preliminary palliative procedure for one-stage total correction of TOF. There is a need to revive 'Brock's procedure' for patients with TOF and poor pulmonary artery anatomy as the procedure of choice. The first direct intra-cardiac operation aimed at directly addressing the pathological anatomy on its Diamond Jubilee Year.

Mid-Term Outlook Following Modified Senning's Operation for the Correction of Transposition of the Great Arteries: A Case Series and Review of Literature.

At the time of writing, two patients who underwent modified Senning's operation (MSO) for the treatment of transposition of great arteries (TGAs) were followed up. At the time of surgery, the patients were three months and 15 years old, respectively. The duration of the follow-up was three years, during which there was a good prognosis, and hence no further invasive treatments were required. There was normal functioning of the right ventricle (RV) in both patients, with the exception of a minor baffle leak in the three-month-old patient. At the annual three-year follow-up, the tricuspid regurgitation (systemic atrioventricular valve) status was moderate in the three-year-old child and mild in the 18-year-old girl. Both patients maintained sinus rhythm and are assigned classification as New York Heart Association (NYHA) Classes I and II. This study aims to assess the midterm outlook after MSO in order to identify and manage future long-term complications. Our report shows a positive outcome in terms of survival and functional activities among children with d-TGA; however, there is a strong need for future research to evaluate the prognosis in the long term (LT) and to assess the functioning of RV.

Utility of Conventional but Late Pulmonary Artery Banding in Complex Cyanotic Congenital Heart Disease in a Toddler - A Single Case Scenario.

Newborns with untreated single ventricles develop pulmonary vascular diseases early in their lives. At that age, during the first eight weeks after birth, clinicians perform pulmonary artery (PA) banding to reduce the blood flow to the lung, decreasing the likelihood of future high vascular resistance or pressure. PA banding is also considered an initial stage in the process of single ventricle palliation procedures. We report a case of a 16-month-old toddler (7 kg) with room air saturation of 82%, diagnosed with tricuspid valve atresia, large atrial and ventricular septal defect, and hypoplastic right ventricle with severe pulmonary arterial hypertension. The baby underwent a successful surgical procedure of PA banding and was discharged after 13 days of hospital stay with a room air saturation of 89%. This case highlighted the benefit of PA banding beyond the stipulated period.

Detecting and Quantifying Residual Intracardiac Shunts Using Oximetric Step-Up Methods: A Prospective Observational Study.

Background & aims  Intracardiac shunts are abnormal channels of blood circulation within the heart that develop either as an additional blood flow pathway or as a replacement for the normal channels of blood circulation. They are the commonest types of congenital heart defects. Various methods are available in the present times to identify, localize or quantify left-to-right intracardiac shunts. Methods may vary in sensitivity, indicators, or types of equipment available. One such method used in almost all cardiac centers for a long time has been oximetry run to detect step-up differences in oxygen saturation values. In the oximetry run the main approach to detect and estimate the left-to-right (L-->R) shunts requires the oxygen concentration expressed as a proportion of saturation to be evaluated in blood samples which are obtained from the right atrium (RA) and pulmonary artery (PA), respectively. A left-to-right shunt can be considered if there is a significant increase (step-up) in blood saturation. A significant step-up is defined as a substantial rise in blood oxygen content or saturation that is higher than normal values. Methods  Using a prospective observational design, this article investigates the application of the step-up method in detecting intracardiac shunts. The study was conducted between 2021 and 2022 on 35 pediatric cardiac patients (males/females, 24/11) diagnosed with post-tricuspid shunts. The pulmonary artery and right atrium were sampled before and after cardiopulmonary bypass surgery and analyzed using a blood gas test. As a result, nearly 91% of the patients had a saturation below 8%. However, the difference between PA oxygen saturation (SO2) & RASO2 before and after surgery was significant. As a result, the difference in O2 saturation helped detect the residual ventricular septal defect (VSD) after the surgery. Results  There were no deaths or complications in this study. There were no re-interventions for post-tricuspid shunt surgery, though one patient had a step-up of >15% and residual VSD status was moderate to large on two-dimensional (2D) echocardiography. Conclusion A combination of physical findings, chest radiography, electrocardiogram (ECG), and echocardiography is routinely done for all these patients undergoing pediatric cardiac surgery. Echocardiography can detect the occurrence of shunt but does not calculate the shunt ratio. Transesophageal or epicardial echocardiography is the standard of care but has its limitations like perception difference between the operating surgeon and the person performing echocardiography. In this study, we have added an oximetry analysis of blood-gas samples before and after surgery and compared it to 2D echocardiography to test the validation of oximetry in isolation and comparison to 2D echocardiography.

On-Pump Bidirectional Glenn Shunt Compared to Off-Pump Shunt Is a Better Influencing Determinant of Perioperative Morbidity During Palliation of Univentricular Heart: Compilation of Two Cases.

The bidirectional Glenn (BDG) or hemi-Fontan technique, performed under cardiopulmonary bypass (CPB) and often utilized as first-stage palliation for various cyanotic congenital heart diseases, as a part of the single-ventricle repair is associated with adverse side effects and high expenditures. Previous studies have shown that BDG is safe even without CPB, which thus necessitates further investigation. This manuscript presents the case of two patients with complex cyanotic congenital heart diseases. The first case was an 11-month-old baby who presented with fever, cough, and cold, while the second case was a two-year-old baby who was underweight due to poor feeding status. Both patients underwent a BDG and main pulmonary artery partial ligation following the requisite preoperative medical evaluations on CPB. Moreover, case 1 had atrial septectomy, while case 2 had significant aortopulmonary collateral arteries ligation. Both patients were discharged following an uneventful postoperative outcome. We found that an adequate selection of patients for the BDG procedure, with or without CPB, could lead to identical postoperative outcomes with regard to mortality, morbidity, and supply usage.

Normalized Subendocardial Myocardial Attenuation on Coronary Computed Tomography Angiography Predicts Postoperative Adverse Cardiovascular Events: Coronary CTA VISION Substudy.

BACKGROUND: Abnormalities in computed tomography myocardial perfusion has been associated with coronary artery disease and major adverse cardiovascular events (MACE). We sought to investigate if subendocardial attenuation using coronary computed tomography angiography predicts MACE 30 days postelective noncardiac surgery. METHODS: Using a 17-segment model, coronary computed tomography angiography images were analyzed for subendocardial and transmural attenuation and the corresponding blood pool. The segment with the lowest subendocardial attenuation and transmural attenuation were normalized to the segment with the highest subendocardial and transmural attenuation, respectively (SUBnormalized, and TRANSnormalized, respectively). We evaluated the independent and incremental value of myocardial attenuation to predict the composite of cardiovascular death or nonfatal myocardial infarction. RESULTS: Of a total of 995 coronary CTA VISION (Coronary Computed Tomographic Angiography and Vascular Events in Noncardiac Surgery Patients Cohort Evaluation Study) patients, 735 had available images and complete data for these analyses. Among these patients, 60 had MACE. Based on Revised Cardiovascular Risk Index, 257, 302, 138, and 38 patients had scores of 0, 1, 2, and ≥3, respectively. On coronary computed tomography angiography, 75 patients had normal coronary arteries, 297 patients had nonobstructive coronary artery disease, 264 patients had obstructive disease, and 99 patients had extensive obstructive coronary artery disease. SUBnormalized was an independent and incremental predictor of events in the model that included Revised Cardiovascular Risk Index and coronary artery disease severity. Compared with patients in the highest tertile of SUBnormalized, patients in the second and first tertiles had an increased hazards ratio for events (2.23 [95% CI, 1.091-4.551] and 2.36 [95% CI, 1.16-4.81], respectively). TRANSnormalized, as a continuous variable, was also found to be a predictor of MACE (P=0.027). CONCLUSIONS: Our study demonstrates that SUBnormalized and TRANSnormalized are independent and incremental predictors of MACE 30 days after elective noncardiac surgery. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT01635309.

Functional consequences of postnatal interventions in a mouse model of Fragile X syndrome.

BACKGROUND: Fragile X syndrome (FXS) is a leading genetic cause of autism and intellectual disability with cortical hyperexcitability and sensory hypersensitivity attributed to loss and hypofunction of inhibitory parvalbumin-expressing (PV) cells. Our studies provide novel insights into the role of excitatory neurons in abnormal development of PV cells during a postnatal period of inhibitory circuit refinement. METHODS: To achieve Fragile X mental retardation gene (Fmr1) deletion and re-expression in excitatory neurons during the postnatal day (P)14-P21 period, we generated CreCaMKIIa/Fmr1Flox/y (cOFF) and CreCaMKIIa/Fmr1FloxNeo/y (cON) mice, respectively. Cortical phenotypes were evaluated in adult mice using biochemical, cellular, clinically relevant electroencephalogram (EEG) and behavioral tests. RESULTS: We found that similar to global Fmr1 KO mice, the density of PV-expressing cells, their activation, and sound-evoked gamma synchronization were impaired in cOFF mice, but the phenotypes were improved in cON mice. cOFF mice also showed enhanced cortical gelatinase activity and baseline EEG gamma power, which were reduced in cON mice. In addition, TrkB phosphorylation and PV levels were lower in cOFF mice, which also showed increased locomotor activity and anxiety-like behaviors. Remarkably, when FMRP levels were restored in only excitatory neurons during the P14-P21 period, TrkB phosphorylation and mouse behaviors were also improved. CONCLUSIONS: These results indicate that postnatal deletion or re-expression of FMRP in excitatory neurons is sufficient to elicit or ameliorate structural and functional cortical deficits, and abnormal behaviors in mice, informing future studies about appropriate treatment windows and providing fundamental insights into the cellular mechanisms of cortical circuit dysfunction in FXS.