VACTERL Association in a Fetus With a Normal Genetic Profile.

VACTERL association is a statistical association of vertebral(V), anal(A), cardiac(C), tracheoesophageal(TE), renal(R), and limb(L) abnormalities (VACTERL). Diagnosis of VACTERL can be made if anomalies in three or more organ systems indicated by the acronym are present and no other recognizable pattern of human malformations exists. We hereby present a case of a primigravida in her 20s, whose prenatal scan at 13 weeks of gestation revealed a fetus having a short spine, single outflow tract, and increased nuchal translucency. Chromosome microarray on amniotic fluid showed no quantitative genomic imbalance. A repeat scan at 21 weeks disclosed lumbar scoliosis with hemivertebrae, tetralogy of Fallot, and a single umbilical artery. After undergoing counselling, in light of the adverse outcomes and poor quality of life of the offspring, the parents decided to terminate the pregnancy. The abortus was subsequently sent to the histopathology lab for autopsy. The gross examination of the fetus unveiled an umbilical cord with only two vessels, right congenital talipes equinovarus (club foot), left congenital talipes equinovalgus, and right foot polydactyly. Internal examination of the cardiovascular system verified the presence of tetralogy of Fallot. The kidogram of the fetus was consistent with a butterfly vertebral body of D11 and D12 with right-side hemivertebrae. This case illustrates the importance of foetal autopsy for confirming the prenatal diagnosis and identifying abnormalities in syndromes or associations. The classification especially plays a crucial role in the case of recurrent abortions to point out the underlying aetiology and counsel the parents regarding the recurrence risk.

Rupture of a calcified right ventricle to pulmonary artery homograft by balloon dilation- emergency rescue by venus P-Valve.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent. CASE DETAILS: A 13-year-old boy had two previous operations of tetralogy of Fallot, one a total repair and the other a homograft valved conduit for pulmonary regurgitation. He presented with dyspnea and severe right ventricular outflow tract obstruction (RVOTO) and had a calcified outflow tract and main pulmonary artery. In the catheter laboratory, a non-compliant balloon dilation resulted in a contained rupture of the conduit. The patient remained hemodynamically stable, and the rupture was treated with a self-expandable Venus P-Valve without the need for a covered stent combined with a balloon-expandable valve or a further surgical procedure. DISCUSSION: Preprocedural evaluation with an inflating balloon is necessary to examine tissue compliance and determine suitability for PPVI. However, this condition is accompanied by a risk of conduit rupture. Risk factors of this complication are calcification and homograft use. These ruptures are mostly controlled with a prophylactic or therapeutic covered stent, with a low rate of requiring surgery. However, there are severe ruptures which lead to hemothorax and death. In the available literature, there was no similar reported case of conduit rupture, which a self-expandable Pulmonary valve stent has managed. It seems that fibrosis and collagen tissue around the heart, formed after open surgeries, can contribute to the control of bleeding in these cases. CONCLUSION (CLINICAL LEARNING POINT): The suitability of patients for the PPVI procedure should be examined more carefully, specifically patients with homograft and calcification in their conduit. Furthermore, conduit rupture might be manageable with self-expandable artificial pulmonary valves, specifically in previously operated patients, and the applicability of this hypothesis is worth examining in future research.

Long-term outcomes of ventricular tachycardia ablation in repaired tetralogy of Fallot: Systematic review and meta-analysis.

Background: Ventricular tachycardia (VT) remains a risk in repaired Tetralogy of Fallot (rTOF); however, long-term benefits of VT ablation have not been established. This study compares the outcomes of rTOF patients with and without VT ablation. Methods: We searched multiple databases examining the outcomes of rTOF patients who had undergone VT ablation compared to those without ablation. Primary outcomes were VT recurrence, sudden cardiac death (SCD), and all-cause mortality. Subgroup analysis was conducted based on the type of ablation (catheter and surgical). Slow-conducting anatomical isthmus (SCAI)-based catheter ablation (CA) was also analyzed separately. The secondary outcome was the risk factors for the pre-ablation history of VT. Results: Fifteen cohort studies with 1459 patients were included, 21.4% exhibited VTs. SCAI was found in 30.4% of the population, with 3.7% of non-inducible VT. Factors significantly associated with VT before ablation included a history of ventriculostomy, QRS duration ≥180 ms, fragmented QRS, moderate to severe pulmonary regurgitation, high premature ventricular contractions burden, late gadolinium enhancement, and SCAI. Ablation was only beneficial in reducing VTs recurrence in SCAI-based CA (risk ratio (RR) 0.11; 95% CI 0.03 to 0.33. p < 0.001; I2 = 0%) with no recurrence in patients with preventive ablation (mean follow-up time 91.14 ± 77.81 months). The outcomes of VT ablation indicated a favorable trend concerning SCD and all-cause mortality (RR 0.49 and 0.44, respectively); however, they were statistically insignificant. Conclusions: SCAI-based CA has significant advantages in reducing VT recurrence in rTOF patients. Risk stratification plays a key role in determining the decision to perform ablation.

Identification and analysis of inflammation-related biomarkers in tetralogy of Fallot.

Background: Studies have revealed that inflammatory response is relevant to the tetralogy of Fallot (TOF). However, there are no studies to systematically explore the role of the inflammation-related genes (IRGs) in TOF. Therefore, based on bioinformatics, we explored the biomarkers related to inflammation in TOF, laying a theoretical foundation for its in-depth study. Methods: TOF-related datasets (GSE36761 and GSE35776) were downloaded from the Gene Expression Omnibus (GEO) database. The differentially expressed genes (DEGs) between TOF and control groups were identified in GSE36761. And DEGs between TOF and control groups were intersected with IRGs to obtain differentially expressed IRGs (DE-IRGs). Afterwards, the least absolute shrinkage and selection operator (LASSO) and random forest (RF) were utilized to identify the biomarkers. Next, immune analysis was carried out. The transcription factor (TF)-mRNA, lncRNA-miRNA-mRNA, and miRNA-single nucleotide polymorphism (SNP)-mRNA networks were created. Finally, the potential drugs targeting the biomarkers were predicted. Results: There were 971 DEGs between TOF and control groups, and 29 DE-IRGs were gained through the intersection between DEGs and IRGs. Next, a total of five biomarkers (MARCO, CXCL6, F3, SLC7A2, and SLC7A1) were acquired via two machine learning algorithms. Infiltrating abundance of 18 immune cells was significantly different between TOF and control groups, such as activated B cells, neutrophil, CD56dim natural killer cells, etc. The TF-mRNA network contained 4 mRNAs, 31 TFs, and 33 edges, for instance, ELF1-CXCL6, CBX8-SLC7A2, ZNF423-SLC7A1, ZNF71-F3. The lncRNA-miRNA-mRNA network was created, containing 4 mRNAs, 4 miRNAs, and 228 lncRNAs. Afterwards, nine SNPs locations were identified in the miRNA-SNP-mRNA network. A total of 21 drugs were predicted, such as ornithine, lysine, arginine, etc. Conclusions: Our findings detected five inflammation-related biomarkers (MARCO, CXCL6, F3, SLC7A2, and SLC7A1) for TOF, providing a scientific reference for further studies of TOF.

Effects of intranasal dexmedetomidine versus intranasal midazolam as premedication in children with tetralogy of Fallot undergoing corrective cardiac surgery: A randomized trial.

Background: This prospective, randomized, double-blind trial was done to compare intranasal dexmedetomidine and intranasal midazolam as premedication for sedation and ease of child-parent separation in pediatric patients of tetralogy of Fallot (TOF) undergoing corrective cardiac surgery. Materials and Methods: Forty children with TOF, between 1 and 10 years, undergoing corrective cardiac surgery were included in the study and, after randomization, were given intranasal midazolam (0.2 mg/kg) or intranasal dexmedetomidine (1 µg/kg), 30 min before shifting to the operation room (OR). Patients were assessed for sedation and child-parent separation, along with hemodynamic parameters, respiratory rate, and oxygen saturation (SpO2) 30 min after drug administration, at the time of shifting inside the OR, and at the time of induction of anesthesia. Results: Both groups had comparable child-parent scores, hemodynamic parameters, SpO2, and respiratory rate. However, the dexmedetomidine group had significantly better sedation levels than the midazolam group patients at the time of shifting inside the OR (dexmedetomidine group: 3.55 ± 0.82 vs. midazolam group: 2.80 ± 0.83; P = 0.007) and at the time of induction of anesthesia (dexmedetomidine group: 3.40 ± 0.75 vs. midazolam group: 2.70 ± 0.86; P = 0.009). Conclusion: Intranasal dexmedetomidine provides better sedation than midazolam, with similar child-parent separation scores and hemodynamic parameters, respiratory rate, and SpO2. No adverse events were observed in both groups. A study on a larger population will help in further establishing the safety and superiority of dexmedetomidine and will further its regular use as an intranasal premedication.

Comparison Between Histidine-Tryptophan-Ketoglutarate Cardioplegia and Cold Blood Cardioplegia for Myocardial Protection in Tetralogy of Fallot Patients Undergoing Surgical Repair: A Randomized Clinical Trial.

OBJECTIVES: This study was conducted to compare the efficacy of histidine-tryptophan-ketoglutarate (HTK) cardioplegia and cold blood cardioplegia (CBC), especially for postoperative right ventricular (RV) function after tetralogy of Fallot repair. DESIGN: Randomized, double-blinded, parallel-group, controlled clinical trial. SETTING: Ain Shams University Hospitals. PARTICIPANTS: A total of 100 children (1 to 5 years old) scheduled for tetralogy of Fallot repair. INTERVENTIONS: Patients were allocated (double-blinded 1:1 allocation ratio) to either the HTK group that received HTK cardioplegia (30 mL/kg via antegrade route) or the CBC group that received cold blood cardioplegia with blood-to-Ringer solution (4:1) in a volume of 20 mL/kg. MEASUREMENTS AND MAIN RESULTS: The HTK group showed a statistically significant reduction of the vasoactive inotropic score on admission to the pediatric intensive care unit (13.0 ± 4.1) in comparison to the CBC group (15.5 ± 5.4), with a p value of 0.011. However, after 24 hours, the vasoactive-inotropic score was comparable. Lactate level during the first 24 hours was 6.2 ± 0.7 mmol/L in the HTK group and 6.9 ± 0.4 mmol/L in the CBC group (p < 0.0001). Serial troponin measurements were lower in the HTK group (1.49 ± 0.45) compared to the CBC group (1.69 ± 0.18) at the first 72 hours postoperatively (p = 0.005). Postoperative echocardiographic assessment of RV function by means of tricuspid annular plane systolic excursion and myocardial performance index were better in the HTK group than in the CBC grpup (p < 0.05). CONCLUSIONS: HTK cardioplegia may offer better cardiac protection to pediatric patients undergoing tetralogy of Fallot repair than our institutional standard CBC with better recovery for the hypertrophic RV.

Early outcomes of experience warm surgery in children undergoing complete repair of tetralogy of Fallot in developing countries.

OBJECTIVES: While significant evidence supports the benefits of normothermic cardiopulmonary bypass (NCPB) over hypothermic techniques, many institutions in developing countries, including ours, continue to employ hypothermic methods. This study aimed to assess the early postoperative outcomes of normothermic cardiopulmonary bypass (NCPB) for complete surgical repair via the Tetralogy of Fallot (TOF) within our national context. METHODS: We conducted this study in the Pediatric Cardiac Intensive Care Unit (PCICU) at the University Children's Hospital. One hundred patients who underwent complete TOF repair were enrolled and categorized into two groups: the normothermic group (n = 50, temperature 35-37 °C) and the moderate hypothermic group (n = 50, temperature 28-32 °C). We evaluated mortality, morbidity, and postoperative complications in the PCICU as outcome measures. RESULTS: The demographic characteristics were similar between the two groups. However, the cardiopulmonary bypass (CPB) time and aortic cross-clamp (ACC) time were notably longer in the hypothermic group. The study recorded seven deaths, yielding an overall mortality rate of 7%. No significant differences were observed between the two groups concerning mortality, morbidity, or postoperative complications in the PCICU. CONCLUSIONS: Our findings suggest that normothermic procedures, while not demonstrably effective, are safe for pediatric cardiac surgery. Further research is warranted to substantiate and endorse the adoption of this technique.

Left ventricular structural integrity on tetralogy of Fallot patients: approach using longitudinal relaxation time mapping.

Purpose: Tetralogy of Fallot (TOF) is a congenital heart disease, and patients undergo surgical repair early in their lives. The evaluation of TOF patients is continuous through their adulthood. The use of cardiac magnetic resonance imaging (CMR) is vital for the evaluation of TOF patients. We aim to correlate advanced MRI sequences [parametric longitudinal relaxation time (T1), extracellular volume (ECV) mapping] with cardiac functionality to provide biomarkers for the evaluation of these patients. Methods: A complete CMR examination with the same imaging protocol was conducted in a total of 11 TOF patients and a control group of 25 healthy individuals. A Modified Look-Locker Inversion recovery (MOLLI) sequence was included to acquire the global T1 myocardial relaxation times of the left ventricular (LV) pre and post-contrast administration. Appropriate software (Circle cmr42) was used for the CMR analysis and the calculation of native, post-contrast T1, and ECV maps. A regression analysis was conducted for the correlation between global LV T1 values and right ventricular (RV) functional indices. Results: Statistically significant results were obtained for RV cardiac index [RV_CI= -32.765 + 0.029 × T1 native; p = 0.003 ], RV end diastolic volume [RV_EDV/BSA = -1023.872 + 0.902 × T1 native; p = 0.001 ], and RV end systolic volume [RV_ESV/BSA = -536.704 + 0.472 × T1 native; p = 0.011 ]. Conclusions: We further support the diagnostic importance of T1 mapping as a structural imaging tool in CMR. In addition to the well-known affected RV function in TOF patients, the LV structure is also impaired as there is a strong correlation between LV T1 mapping and RV function, evoking that the heart operates as an entity.

A Rare Confluence: Brain Abscess in an Adult With Tetralogy of Fallot.

Tetralogy of Fallot (TOF) is a common congenital heart disease (CHD) characterized by four distinct cardiac abnormalities. Brain abscess, though rare, is a life-threatening complication in patients with cyanotic congenital heart disease (CCHD), including TOF. This case report describes a 24-year-old female with unrepaired TOF who presented with symptoms of a brain abscess, including altered sensorium, fever, projectile vomiting, and headache. Diagnostic imaging with non-contrast-enhanced computed tomography (NCCT) revealed a well-defined hypodense lesion with a midline shift, prompting urgent drainage of the abscess. Subsequent cultures of the pus material identified Streptococcus intermedius as the causative agent, and the patient was maintained on antibiotics. This case highlights the importance of early diagnosis and surgical repair of TOF to prevent severe complications such as brain abscess, thereby reducing morbidity and mortality.

Identification of hub lncRNAs correlated with tetralogy of fallot based on weighted gene co-expression network analysis.

The most prevalent cyanotic congenital heart disease, Tetralogy of Fallot (TOF), has an unknown etiology. Long-stranded non-coding RNAs (lncRNAs) have been linked to cardiac development and congenital heart disease, as evidenced by an increasing number of studies; nevertheless, additional research is necessary to fully understand the function that TOF-related lncRNAs play in the condition. This study constructed lncRNA-mRNA co-expression networks, performed functional enrichment analysis, and screened hub lncRNAs using Weighted Gene Co-expression Network Analysis (WGCNA) using the Gene Expression Omnibus dataset GSE36761. Ten hub lncRNAs, including IRF1-AS1, AC012360.6, HLA-F-AS1, RP1-253P7.4, NPTN-IT1, RP11-166P13.4, RP5-1183I21.2, SNHG14, CH17-98J9.1, and RP11-894P9.1, were identified by WGCNA analysis as potentially significant contributors to the development of TOF. Based on functional enrichment analysis, lncRNA mainly contributes to TOF by altering gene splicing patterns. New insights on the mechanism underlying TOF occurrence are provided by identifying hub lncRNAs associated with the disease and analyzing their regulatory networks.

Deep Learning-Based Electrocardiogram Analysis Predicts Biventricular Dysfunction and Dilation in Congenital Heart Disease.

BACKGROUND: Artificial intelligence-enhanced electrocardiogram (AI-ECG) analysis shows promise to detect biventricular pathophysiology. However, AI-ECG analysis remains underexplored in congenital heart disease (CHD). OBJECTIVES: The purpose of this study was to develop and externally validate an AI-ECG model to predict cardiovascular magnetic resonance (CMR)-defined biventricular dysfunction/dilation in patients with CHD. METHODS: We trained (80%) and tested (20%) a convolutional neural network on paired ECG-CMRs (≤30 days apart) from patients with and without CHD to detect left ventricular (LV) dysfunction (ejection fraction ≤40%), RV dysfunction (ejection fraction ≤35%), and LV and RV dilation (end-diastolic volume z-score ≥4). Performance was assessed during internal testing and external validation on an outside health care system using area under receiver-operating curve (AUROC) and area under precision recall curve. RESULTS: The internal and external cohorts comprised 8,584 ECG-CMR pairs (n = 4,941; median CMR age 20.7 years) and 909 ECG-CMR pairs (n = 746; median CMR age 25.4 years), respectively. Model performance was similar for internal testing (AUROC: LV dysfunction 0.87; LV dilation 0.86; RV dysfunction 0.88; RV dilation 0.81) and external validation (AUROC: LV dysfunction 0.89; LV dilation 0.83; RV dysfunction 0.82; RV dilation 0.80). Model performance was lowest in functionally single ventricle patients. Tetralogy of Fallot patients predicted to be at high risk of ventricular dysfunction had lower survival (P < 0.001). Model explainability via saliency mapping revealed that lateral precordial leads influence all outcome predictions, with high-risk features including QRS widening and T-wave inversions for RV dysfunction/dilation. CONCLUSIONS: AI-ECG shows promise to predict biventricular dysfunction/dilation, which may help inform CMR timing in CHD.

Right Ventricular Strain With 4-Dimensional Computed Tomography Identifies Pulmonary Hypertension in Adults With Repaired Tetralogy of Fallot.

BACKGROUND: This study evaluated right ventricular (RV) volume, strain, and morphology using cardiac 4-dimensional computed tomography (4D-CT) to detect pulmonary hypertension (PH) in adults with repaired tetralogy of Fallot (TOF) scheduled for transcatheter pulmonary valve implantation (TPVI). METHODS AND RESULTS: Using cardiac 4D-CT data, we calculated RV strain in 3 different geometries and RV outflow tract (RVOT) mass in 42 patients with repaired TOF. We compared RV strain and RVOT mass between patients with and without PH. Receiver operating characteristic (ROC) analysis was conducted to evaluate the diagnostic performance of these measurements for identifying PH. Four-chamber (4ch) strain was significantly smaller for patients with (n=10) than without (n=32) PH (8.8±1.7% vs. 11.1±2.4%, respectively; P<0.01), whereas RVOT mass was significantly larger in the PH group (12.5±3.5 vs. 9.2±3.2 cm2; P<0.01). ROC analysis of the diagnostic performance revealed that the respective sensitivity and specificity was 70% and 84% (area under the curve [AUC]=0.784) for 4ch strain of 8.8%; 80% and 69% (AUC=0.766) for RVOT mass of 10.7 cm2; and 80% and 81% (AUC=0.844) for a 4ch strain/RVOT mass ratio of 0.97. CONCLUSIONS: RVOT mass and 4ch strain obtained from cardiac 4D-CT may be helpful for identifying PH in patients with repaired TOF.

Outcomes following multivalve reoperation in adults with congenital heart disease: A 30-year, single-center study.

OBJECTIVE: As patients with congenital heart disease increasingly live into adulthood, reoperative surgery is frequently required. Although half of these are valve-related procedures, little is known regarding early and late outcomes, and factors associated with adverse outcomes. METHODS: From 1993 to 2022, a total of 1960 adult patients with congenital heart disease underwent repeat median sternotomy at our institution. Of these, 502 patients (26%) underwent intervention on 2 or more valves and constituted the study cohort. RESULTS: The median age was 39 (27-51) years, and 275 patients (55%) were female. A second sternotomy was performed in 265 patients (53%), a third sternotomy was performed in 135 patients (27%), a fourth sternotomy was performed in 75 patients (15%), and a fifth or more sternotomy was performed in 27 patients (5%). Interventions were performed on 2 valves in 436 patients (87%), 3 valves in 62 patients (12%), and 4 valves in 4 patients (1%). The most common combinations were pulmonary and tricuspid in 241 patients (48%), followed by mitral and tricuspid in 85 patients (17%), aortic and pulmonary in 42 patients (8%), and aortic and mitral in 41 patients (8%). Early mortality was 4.2% overall and 2.7% for elective operations. Nonelective operations and congenital heart disease of major complexity were independently associated with early mortality. Median follow-up was 14 years. One, 5-, and 10-year survivals were 93.6%, 89.3%, and 79.5%, respectively. Factors independently associated with overall mortality were age, ventricular dysfunction, coronary artery disease, renal failure, double valve replacement, nonelective operations, and bypass time. CONCLUSIONS: Multiple valve interventions are common and confer low early mortality in the elective setting. Referral before ventricular dysfunction and in an elective setting optimizes outcomes.

Severe pulmonary insufficiency caused by Fallot-type absent pulmonary valve syndrome: A rare reason for neonatal central cyanosis.

INTRODUCTION: Absent Pulmonary Valve Syndrome (APVS) is a rare birth defect where the pulmonary valve is missing or underdeveloped. APVS often occurs alongside Tetralogy of Fallot, (TOF) another heart defect. PRESENTATION OF CASE: A 33-year-old woman gave birth to a male infant with severe pulmonary stenosis (PS) and a large ventricular septal defect (VSD). The infant underwent surgery to close the VSD and resect the stenotic ring. Two years later, he remained asymptomatic with a closed VSD and no pulmonary valve gradient. DISCUSSION: Despite high mortality rates, long-term survival has improved with advancements in surgical repair. This case underscores the significance of early detection and personalized surgical strategies for complex congenital heart defects. CONCLUSION: Early identification of subtle symptoms is crucial for timely intervention, while individualized surgical strategies optimize outcomes. Further research is needed to understand the complex interplay of cardiac anomalies in APVS, particularly the absence of a patent ductus arteriosus in this case.

Prognostic value of myocardial deformation parameters for outcome prediction in tetralogy of Fallot.

BACKGROUND: The prognostic value of myocardial deformation parameters in adults with repaired tetralogy of Fallot (rTOF) has not been well-elucidated. We therefore aimed to explore myocardial deformation parameters for outcome prediction in adults with rTOF using cardiovascular magnetic resonance imaging (CMR). METHODS: Adults with rTOF and at least moderate pulmonary regurgitation were identified from an institutional prospective CMR registry. Left ventricular (LV) and right ventricular (RV) global strains were recorded in longitudinal (GLS), circumferential (GCS), and radial (GRS) directions. Major adverse cardiovascular events (MACE) were defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia (>30 seconds), or heart failure (hospital admission >24 hours). In patients with pulmonary valve replacement (PVR), pre- and post-PVR CMR studies were analyzed to assess for predictors of complete RV reverse remodeling, defined as indexed RV end-diastolic volume (RVEDVi) <110 mL/m2. Logistic regression models were used to estimate the odds ratio (OR) per unit change in absolute strain value associated with clinical outcomes and receiver operator characteristic curves were constructed with area under the curve (AUC) for select CMR variables. RESULTS: We included 307 patients (age 35 ± 13 years, 59% (180/307) male). During 6.1 years (3.3-8.8) of follow-up, PVR was performed in 142 (46%) and MACE occurred in 31 (10%). On univariate analysis, baseline biventricular ejection fraction (EF), mass, and all strain parameters were associated with MACE. After adjustment for LVEF, only LV-GLS remained independently predictive of MACE (OR 0.822 [0.693-0.976] p = 0.025). Receiver operator curves identified an absolute LV-GLS value less than 15 and LVEF less than 51% as thresholds for MACE prediction (AUC 0.759 [0.655-0.840] and 0.720 [0.608-0.810]). After adjusting for baseline RVEDVi, RV-GCS (OR 1.323 [1.094-1.600] p = 0.004), LV-GCS (OR 1.276 [1.029-1.582] p = 0.027) and LV-GRS (OR 1.101 [1.0210-1.200], p = 0.028) were independent predictors of complete remodeling post-PVR remodeling. CONCLUSION: Biventricular strain parameters predict clinical outcomes and post-PVR remodeling in rTOF. Further study will be necessary to establish the role of myocardial deformation parameters in clinical practice.

Acute Cerebellitis and Obstructive Hydrocephalus: An Unseen Neurological Complication After Surgical Repair for Tetralogy of Fallot.

Acute cerebellitis with obstructive hydrocephalus post-Tetralogy of Fallot surgery is extremely rare but can present aggressively in pediatric cases. Early diagnosis is critical for prompt medical and surgical intervention. We report a fatal case in a 7-year-old boy post-surgery, where neurological symptoms rapidly progressed, leading to drowsiness and intermittent response to commands. Despite initial computed tomography scans showing no abnormality, subsequent scans revealed cerebellitis and hydrocephalus. Treatment with steroids, antibiotics, and cerebrospinal fluid drainage was unsuccessful, and the condition's etiology remained unclear despite negative serological tests and cultures. This highlights the challenge of diagnosing and treating acute cerebellitis, especially when no specific cause is found and when deterioration is swift. The role of opioids in pediatric patients and their potential association with neurosurgical complications is also discussed, prompting further inquiry into postoperative symptoms and opioid-related risks in susceptible individuals.

From Triumph to Trial: A Case Study of Non-Tuberculous Mycobacterium After Tetralogy of Fallot (TOF) Correction in an Immunocompetent Child.

Mycobacterium simiae, a slow-growing non-tuberculous mycobacterium (NTM), presents diagnostic challenges due to its resemblance to Mycobacterium tuberculosis and other NTMs. While NTM infections and tuberculosis share clinical and radiological features, their management strategies differ. Accurate differentiation is pivotal, as misdiagnosing NTM infections such as MDR-TB can lead to ineffective treatments. A case involving an 11-year-old female with tetralogy of Fallot (TOF) and a pulmonary M. simiae infection underscores the importance of precise diagnosis. Enhancing diagnostic methods is imperative to prevent mismanagement of NTM infections and ensure appropriate care.

Propranolol Therapy in Tetralogy of Fallot: Treating the Echocardiogram or Treating the Patient.

Hypercyanotic spells are one of the defining clinical features of Tetralogy of Fallot (TOF). Limited data exist on peak Doppler right ventricular outflow tract (RVOT) gradient as a risk factor for the development of hypercyanotic spells, frequency of prophylactic use of propranolol based on peak RVOT gradient, and its impact on preventing the occurrence of hypercyanotic spells. We aimed to quantify peak RVOT gradients as measured on transthoracic echocardiography in infants with unrepaired TOF and assess for correlation with clinical symptoms of hypercyanotic spells. We also assessed the frequency of pre-operative use of propranolol, indication for medication initiation, and occurrence of hypercyanotic spells with or without propranolol use. Retrospective analysis was performed on patients at our institution who were born between February 1, 2011 and May 31, 2023. Patients were excluded if they were maintained on prostaglandin infusion or underwent palliative shunt placement or balloon valvuloplasty prior to complete surgical repair. Demographics, occurrence of hypercyanotic spells, propranolol use, peripheral oxygen saturation, age at surgical repair, and peak RVOT gradient at the time of propranolol initiation were collected from the electronic medical record. If no propranolol use was recorded, the single highest maximum RVOT gradient prior to surgery was collected. 203 patients were identified, of which 92 patients were included in analysis. Thirty-six (39%) patients received propranolol and 19% of patients developed hypercyanotic spells prior to surgery. Patients with higher peak RVOT gradients were more likely to be started on propranolol even in the absence of overt symptoms, and they also demonstrated more systemic desaturation. Additionally, peak RVOT gradient was found to be a poor predictor for the development of hypercyanotic spells. Wide clinical variation exists in the prophylactic use of propranolol for prevention of hypercyanotic spells. Peak RVOT gradient is not a reliable tool for prophylactic propranolol initiation to prevent hypercyanotic spells.