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The pulmonary artery-to-left atrium (LA) fistula is one of the rare and unique structural causes of silent cyanosis. This correctable abnormality can be identified by having a high index of clinical suspicion and appropriate investigations using echocardiography and cardiac computed tomography (CT). We report an eight-year-old child who had worsening exertional dyspnea, long-standing central cyanosis, and recurrent infections. A large-sized fistula connecting the right pulmonary artery (RPA) to the LA with all the right- and left-sided pulmonary veins showed normal drainage into the LA, suggesting a type I RPA-to-LA fistula, which was diagnosed on cardiac CT. Percutaneous closure using the occluder device is planned as further management for the patient.

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OBJECTIVE: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes. STUDY DESIGN: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the Australia and New Zealand Fontan Registry. RESULTS: Participants (mean age: 11.5 ± 2.6 years, 62% male) reported lower overall HRQOL (P < .001), and lower scores across all HRQOL domains (all P < .0001), compared with normative data. Median global QOL score was 7.0 (IQR 5.8-8.0), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of congenital heart disease explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes. CONCLUSIONS: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population.

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While significant progress has been made in reducing disparities within the US health care system, notable gaps remain. This article explores existing disparities within pediatric congenital heart disease care. Congenital heart disease, the most common birth defect and a leading cause of infant death, has garnered substantial attention, revealing certain disparities within the US health care system. Factors such as race, ethnicity, insurance coverage, socioeconomic status, and geographic location are all commonalities that significantly affect health disparities in pediatric congenital heart disease. This comprehensive review sheds light on disparities from diverse perspectives in pediatric care, demonstrates the inequities and inequalities leading to these disparities, presents effective solutions, and issues a call to action for providers, institutions, and the health care system. Recognizing and addressing these disparities is imperative for ensuring equitable care and enhancing the long-term well-being of children affected by congenital heart disease. Implementing robust, evidence-based frameworks that promote responsible and safe interventions is fundamental to enduring change.

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Pediatric arterial ischemic stroke is a rare but increasingly acknowledged disorder. Large vessel occlusions in this population have been treated off-label with endovascular thrombectomy. However, there is limited evidence to guide management. Small children, before the age of five when the cerebrovasculature reaches adult size, present additional challenges. We report the case of cardioembolic basilar occlusion in a two-year-old and the technical details of endovascular management, currently lacking in published literature. We employed a 5 French slender sheath, typically used for radial access, as a femoral short sheath. We accessed the dominant vertebral artery with a 5 French intermediate catheter, navigated with a typical 0.027-inch microcatheter and 0.014-inch microwire, and performed direct aspiration thrombectomy of the basilar clot. No closure device was employed. The patient had a near-complete and durable recovery. Small children present additional challenges for the endovascular management of stroke. Pre-procedural imaging can be used to design an aspiration-capable system appropriate for the child's size. Endovascular thrombectomy in children is feasible with some modifications to adult protocols.

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This retrospective study addresses the intersection of Down syndrome (DS) and congenital heart defects (CHD), including the prevalence and characteristics of CHD in trisomy 21 patients at a secondary hospital in the Eastern Province of Saudi Arabia. The study investigated the electronic medical records retrieved from the Qatif Central Hospital database, specifically targeting individuals diagnosed with DS (identified by the International Classification of Diseases, Tenth Revision (ICD-10) code Q90.9) between March 2012 and March 2022. The primary aim was to detect cardiac anomalies diagnosed via echocardiography performed at the hospital, along with subsequent follow-up assessments and documented patient outcomes. Among the 161 patients reviewed, the study revealed a significant prevalence of diagnosed heart defects through echocardiograms, constituting approximately 72.7%. Notably, patent ductus arteriosus was the most common condition, found in 29.81% of cases, followed by atrial septal defect (27.95%) and atrioventricular septal defect (17.39%). Among the study participants, 22.98% required surgical intervention. Unfortunately, mortality impacted 32.3% of individuals, while the majority (60.87%) remained alive. In addition, a small percentage (6.83%) discontinued follow-up within our center. This study contributes significant data on cardiac anomalies in DS patients in Saudi Arabia, highlighting a high prevalence of CHD with specific patterns of anomalies. The need for early diagnosis, timely surgical intervention, and ongoing management is evident. These findings provide a foundation for improving clinical practices and shaping public health policies tailored to the needs of this population in Saudi Arabia and similar regions.

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Congenital heart disease (CHD) are genetically complex and comprise a wide range of structural defects that often predispose to - early heart failure, a common cause of neonatal morbidity and mortality. Transcriptome studies of CHD in human pediatric patients indicated a broad spectrum of diverse molecular signatures across various types of CHD. In order to advance research on congenital heart diseases (CHDs), we conducted a detailed review of transcriptome studies on this topic. Our analysis identified gaps in the literature, with a particular focus on the cardiac transcriptome signatures found in various biological specimens across different types of CHDs. In addition to translational studies involving human subjects, we also examined transcriptomic analyses of CHDs in a range of model systems, including iPSCs and animal models. We concluded that RNA-seq technology has revolutionized medical research and many of the discoveries from CHD transcriptome studies draw attention to biological pathways that concurrently open the door to a better understanding of cardiac development and related therapeutic avenue. While some crucial impediments to perfectly studying CHDs in this context remain obtaining pediatric cardiac tissue samples, phenotypic variation, and the lack of anatomical/spatial context with model systems. Combining model systems, RNA-seq technology, and integrating algorithms for analyzing transcriptomic data at both single-cell and high throughput spatial resolution is expected to continue uncovering unique biological pathways that are perturbed in CHDs, thus facilitating the development of novel therapy for congenital heart disease.

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BACKGROUND: Congenital heart disease (CHD) is a common occurrence in live births, with some exhibiting critical congenital heart disease; therefore, cardiology services should be available around the clock to ensure timely diagnosis and management. This study aims to describe the workload and the need for pediatric cardiac services in a maternity hospital for newborn referrals. Moreover, the study describes the indications for neonatal cardiology consultations. METHODS: The prospective cohort study was conducted over four months, from January to April 2022, in the Prince Sultan Cardiac Center Al Qassim region of Saudi Arabia. Prince Sultan Cardiac Center's pediatric cardiology department provides cardiac services to the Maternity and Children Hospital Buraidah Al Qassim. Out of the total 2,606 live births during the study period, the cardiology team evaluated 352 neonates. Neonates less than 30 days of age who were born in the maternity hospital were enrolled in the study. The outborn babies referred from other centers as suspected congenital heart disease for whom a cardiac evaluation was done were excluded. In addition, babies assessed in the emergency room and born elsewhere were excluded. Only new consultations have been considered, excluding follow-up consultations. STATISTICAL ANALYSIS: Data about patients' demographic, clinical and echocardiographic findings were recorded on Google Forms and converted to a Google spreadsheet. The Google spreadsheet's inbuilt statistical software was used for analysis. Categorical data were presented as percentages, and numerical data as median and range. RESULTS: The cardiology team evaluated 352 neonates from 2,606 live births over four months, accounting for 13.5 per 100 live births. The median weight was 2.8 kilograms, with a 0.5-4.3 kilogram range. Males comprised 187 (53%), and females comprised 165 (47%). Moreover, full-term, preterm, and post-term accounted for 236 (67%), 113 (32%), and 3 (0.8%) of patients, respectively. The common indications for neonatal cardiac referral were respiratory distress 60 (17%), infants born to diabetic mothers 50 (14%), abnormal fetal echocardiogram 49 (13.9%), family history of abortion or neonatal death 31 (8.8%), and congenital anomalies 30 (8.5%). Systolic murmur was the commonest clinical finding that prompted cardiology referrals 82 (23.2%), followed by desaturation 38 (10.7%) and dysmorphic features 31 (8.8%). Among the congenital cardiac defects, an isolated atrial septal defect (ASD) was seen in 66 (18.5%), isolated patent ductus arteriosus in 50 (14.2%), and ventricular septal defect in 21 (5.9%). Moreover, 13 (4.4%) lesions were critical CHDs. Finally, 27 (7.6%) had a diagnosis of pulmonary hypertension. CONCLUSION: Knowing the burden of neonatal cardiac assessment on pediatric cardiology services in any maternity center may help the healthcare authorities to allocate resources and optimize the delivery of cardiac services among the neonatal population. Properly allocating pediatric cardiologists to the needed centers may optimize neonatal cardiac services. Moreover, it may decide on the number of pediatric cardiologists that need to be trained each year to meet the requirements of neonatal cardiac services.

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While congenital heart disease is not uncommon, cyanotic congenital heart disease (CCHD) accounts for a minor fraction of them. However, when cyanosis is present, it usually indicates a severe or critical illness. Tetralogy of Fallot (TOF) is one of the common CCHDs, representing 7-10% of all congenital cardiac malformations. Double-outlet right ventricle (DORV) is another CCHD similar to the TOF and associated with decreased pulmonary flow, ventricular septal defect (VSD), and aorta receiving blood from both ventricles. Reduced oxygen arterial saturation and increased viscosity by polycythemia induce focal cerebral ischemia, often in the area supplied by the middle cerebral artery leading to brain abscess. Brain abscesses require craniotomy, which is a major surgery. These patients also often show features of sepsis and increased intracranial pressure. The presence of CCHD further complicates the situation, making perioperative management even more challenging. There are studies in the literature on the management of similar cases, and they report successful management in most of them. However, not all such cases need intensive postoperative management. We present four pediatric cases who had either TOF or DORV and had to undergo craniotomy for brain abscess or ventriculoperitoneal shunt placement. We describe case management and highlight the critical features and cases that require prolonged postoperative critical care management.

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The literature on tetralogy of Fallot (TOF) is abundant. It is a fairly common cyanotic congenital heart disease. It results from the anterior malalignment of the conal septum resulting in the aberrant formation of the ventricular septum leading to a defect. This presents a very important clinical significance because the prognosis usually depends on the clinical evaluation and initiation of timely therapy. We present a two-week-old baby with normal birth history and an uncomplicated newborn nursery course. He also passed the Critical Congenital Heart Disease screen at 24 hours of life. The routine examination by the pediatrician led to further investigations and treatments, highlighting the importance of good history-taking and clinical examination skills.

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BACKGROUND: To evaluate the great vessels in young children with complex congenital heart disease (CHD) using non-contrast cardiovascular magnetic resonance angiography (CMRA) based on three-dimensional relaxation-enhanced angiography without contrast (REACT) in comparison to contrast-enhanced steady-state CMRA. METHODS: In this retrospective study from April to July 2021, respiratory- and electrocardiogram-gated native REACT CMRA was compared to contrast-enhanced single-phase steady-state CMRA in children with CHD who underwent CMRA at 3T under deep sedation. Vascular assessment included image quality (1 = non-diagnostic, 5 = excellent), vessel diameter, and diagnostic findings. For statistical analysis, paired t-test, Pearson correlation, Bland-Altman analysis, Wilcoxon test, and intraclass correlation coefficients (ICC) were applied. RESULTS: Thirty-six young children with complex CHD (median 4 years, interquartile range, 2-5; 20 males) were included. Native REACT CMRA was obtained successfully in all patients (mean scan time: 4:22 ± 1:44 min). For all vessels assessed, diameters correlated strongly between both methods (Pearson r = 0.99; bias = 0.04 ± 0.61 mm) with high interobserver reproducibility (ICC: 0.99 for both CMRAs). Native REACT CMRA demonstrated comparable overall image quality to contrast-enhanced CMRA (3.9 ± 1.0 vs. 3.8 ± 0.9, P = 0.018). With REACT CMRA, better image quality was obtained at the ascending aorta (4.8 ± 0.5 vs. 4.3 ± 0.8, P < 0.001), coronary roots (e.g., left: 4.1 ± 1.0 vs. 3.3 ± 1.1, P = 0.001), and inferior vena cava (4.6 ± 0.5 vs. 3.2 ± 0.8, P < 0.001). In all patients, additional vascular findings were assessed equally with native REACT CMRA and the contrast-enhanced reference standard (n = 6). CONCLUSION: In young children with complex CHD, REACT CMRA can provide gadolinium-free high image quality, accurate vascular measurements, and equivalent diagnostic quality compared to standard contrast-enhanced CMRA.

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Chylothorax is a critical complication after surgery for congenital heart disease, which markedly compromises the postoperative course with increased mortality. As the cardiovascular load additively causes stagnation of the thoracic duct, chylothorax after palliative cardiac surgery can be highly refractory to the therapies. Here we report a case of two patients with refractory chylothorax attributed to hemodynamic load which was successfully treated with minocycline pleurodesis. In combination with congenital heart disease, extremely low birth weight coupled with prematurity in case 1 and venous obstruction with excessive volume load due to additional aortopulmonary shunt in case 2 additively increased resistance to the therapies, including fasting with total parenteral nutrition (TPN), XIII factor supplementation, octreotide infusion, as well as the use of steroids. As pleural effusion was sustained at more than 50 ml/kg/day, the condition of both patients deteriorated severely; pleurodesis using minocycline was urgently introduced. Pleural effusion declined at every session and both cases were in remission in a few sessions without unfavorable acute reaction. No symptoms suspecting chronic adverse effects were observed during follow-up, including respiratory dysfunction, pulmonary hypertension, tooth staining, or abnormal bone mineralization. Although the application of minocycline for children should be minimized, minocycline pleurodesis can be an option for patients with refractory and life-threatening chylothorax.

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Perioperative autonomic nervous system (ANS) measurements are evolving toward increasing import and utility. We present a three-year-old male with Down Syndrome who underwent ambulatory autonomic monitoring during surgery followed by cardiac magnetic resonance (CMR) imaging. Autonomic data from both environments are compared to age-related norms. We are the first to describe a method for acquiring and trending autonomic data from clinically indicated CMR scans in order to monitor autonomic function. These data are proof of concept for the use of routinely collected CMR data as a surrogate for autonomic data in children, noting differences in the autonomic effects of anesthetic techniques.

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Coronary artery lesions represent rare conditions in pediatric congenital heart disease and mainly include coronary artery stenoses (CAS) or coronary artery fistulae (CAF). Due to the small vessel size, pediatric percutaneous coronary interventions (PCI) are demanding and studies concerning long-term results are missing. In this retrospective study, we analyzed indications, procedural details, and post-procedural outcomes in pediatric patients who underwent PCI in our institution. For CAS treatment, procedural success was defined as efficient coronary revascularization with a significant improvement of coronary perfusion. CAF treatment was considered successful, when no residual shunt was detectable. From 1995 to 2020, 32 pediatric patients aged ≤ 18 years received interventional treatment for CAS (n = 24/32) or CAF (n = 8/32). Reasons for CAS were post-surgical (n = 15/24) or post-transplant (n = 9/24). Interventional treatment strategies included coronary angioplasty (20/43), stent placement (10/43), and a combination of both (13/43). In-hospital mortality occurred in 6/24 patients and late mortality in 5/24 patients leading to an overall 5-year survival of 62.5%. Early mortality mainly occurred due to post-ischemic myocardial failure. CAF occlusion was performed using coil embolization (n = 3), placement of vascular plugs (n = 3), a combination of both (n = 1), or a combination of coil embolization and a covered stent (n = 1). Treatment of coronary fistulae was successful in all patients with excellent post-procedural results and no follow-up death. PCI in pediatric patients with congenital heart disease can be performed safely and effectively. However, the overall 5-year survival probability of patients with CAS is reduced due to severe ischemic myocardial damage.

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Conjoined twins represent a very rare congenital anomaly, and the dicephalic dibrachius dipus (DDD) type of conjoined twinning is so rare that the exact prevalence is unknown. Only a few published case studies have mentioned this anomaly. Not enough data are available where antenatal ultrasonography (USG) and MRI have been employed in the workup of such cases. This study describes the case of a 24-year-old woman who came to our department for an anomaly scan at 25 weeks of gestation and was diagnosed with a dicephalic type of conjoined twinning with multiple anomalies. However, USG could not differentiate between DDD twinning and craniopagus parasiticus; hence, the patient was referred for fetal MRI. On MRI, the diagnosis of DDD was confirmed. In craniopagus parasiticus twinning, the surgical removal of the parasitic head can allow an everyday life. However, DDD twinning with multiple anomalies is not compatible with life, and the mother was thoroughly explained the grave prognosis. In such doubtful cases, fetal MRI should always be employed to ascertain the diagnosis for proper management and counseling.

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OBJECTIVES: We report a multicenter experience with simultaneous right ventricular outflow tract (RVOT) stenting and transcatheter pulmonary valve implantation using the Melody valve (Medtronic). BACKGROUND: Prestenting the RVOT before Melody valve implantation is now the standard of care. Prestenting is usually performed as a separate step. The "one-step" technique for simultaneous landing zone stenting and Melody delivery was previously reported using only Max LD stents (Medtronic). We report a multicenter experience of simultaneous stenting and Melody implantation using multiple stent types in combination. METHODS: This retrospective cohort study includes 33 patients from 3 centers who underwent simultaneous stenting and Melody valve implantation between 2017 and 2020. Key variables were compared with 31 patients from the same centers who underwent standard (non-simultaneous) prestenting followed by Melody implantation during the same time frame. RESULTS: The 2 groups were similar in terms of age, weight, sex, and total procedure time. The 2 groups had similar clinical results and safety profiles, with no difference between the postimplantation right ventricle (RV) to pulmonary artery systolic pressure gradient, RV to aortic pressure ratio, and complication rate. The simultaneous group had lower radiation exposure as measured by dose area product. Up to 3 stents were safely placed simultaneously with a Melody valve. CONCLUSIONS: Simultaneous RVOT stenting and Melody valve implantation can safely be used to place up to 3 stents outside a Melody valve. This approach can simplify the catheterization procedure and potentially reduce radiation dose.

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Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that may present isolated or may be associated with other cardiac malformations. Most of the patients develop symptoms during infancy but some may remain asymptomatic up to adulthood. Symptoms range from mild distress to severe irritability and feed intolerance. We report a case of a five-month-old male child who presented with congestive heart failure and was diagnosed as a case of ALCAPA with left atrial and left ventricular dilation based on two-dimensional echocardiography and computed tomography (CT) coronary angiogram. Left main coronary artery was shown to be arising from the posteroinferior wall of main pulmonary artery. Various surgical approaches have been suggested in the repair but the Takeuchi technique was preferred owing to its origin from the non-facing sinus of the pulmonary artery and co-existing dilatation of left atria and ventricle. The surgery was uneventful and there were no postoperative complications. A cardiac CT dynamic study was also done on the follow-up visit five months later and no signs of abnormality or complications were reported. Early intervention is necessary to prevent irreversible cardiac complications and early mortality.

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INTRODUCTION: Congenital heart diseases (CHD) are one of the most commonly occurring congenital anomalies. Echocardiography is usually the initial investigation for suspected CHD. However, it is operator-dependent and limited by available chest windows. Multidetector computed tomography (MDCT) scan provides superior temporal and spatial resolution producing excellent cross-sectional anatomical images. MDCT is specifically helpful for pulmonary artery anomalies if not clearly visible on an echocardiogram. OBJECTIVE AND METHODS: The study aims to compare measurements of branch pulmonary arteries, pulmonary valve, and main pulmonary artery obtained from trans-thoracic echocardiography measurements and MDCT. Forty-nine patients younger than 17 years of age underwent MDCT, and an echocardiogram was included in the study. The measurements of the pulmonary valve, main pulmonary artery, and branch pulmonary arteries were measured on MDCT and echocardiogram. RESULTS: Bland-Altman analysis revealed the mean difference (95% confidence limits) in measurements of diameter between echocardiogram and MDCT for the right pulmonary artery, left pulmonary artery, pulmonary valve, and main pulmonary artery, which were -0.5 (-3.1, 2.2) mm, -0.6 (-3.3, 2.1) mm, 0.7 (-2.5, 3.9) mm, and 1.2 (-6.9, 4.5) mm, respectively. CONCLUSION:  The analysis revealed acceptable agreement in measurements of the pulmonary valve, main pulmonary artery, and branch pulmonary arteries obtained from MDCT and echocardiogram. The difference was marginally more for the main pulmonary artery compared to the pulmonary valve and branch pulmonary arteries.

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OBJECTIVE: The objective was to assess the effectiveness and safety of peripheral regional anesthesia in congenital cardiac surgical patients undergoing thoracotomy for aortic coarctation. DESIGN: A retrospective chart review of pediatric patients (<18 years) who underwent surgical repair of congenital heart diseases via thoracotomy between September 2013 and July 2018 was done. Among patients who underwent coarctation repair, a propensity score was used to match patients who received a regional catheter (C) versus traditional medical treatment only (M). SETTING: A single center children's hospital. PARTICIPANTS: The median age was 172 days (IQR 64-1315) in group C and 176 days (IQR 71-1146) in group M (SMD = 0.07). The median weight was 6.8 kg (IQR 4.8-13.6) in group C and 7.7 kg (4.6-17.4) in group M (SMD = 0.003). MEASUREMENTS AND MAIN RESULT: Outcomes assessed were postoperative hospital length of stay, median pain scores in the first 24 and 48 hours, and total morphine equivalent use in the first 24 and 48 hours. Complications related to the catheters were reviewed. The median oral morphine equivalent dose administered in the first 24 hours was lower in group C than group M (0.8 mg/kg, IQR 0.5-1.1 vs. 1.4 mg/kg, IQR 0.9-1.7, p = 0.019). There were no major complications related to the catheters, including hematoma. CONCLUSIONS: Peripheral regional catheters may be used to reduce opioid requirements in patients after CoA repair. Due to the low risk of these catheters, they should be considered as part of a pain management strategy for pediatric patients undergoing thoracotomy and should be incorporated into strategies to improve outcomes.

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In pediatrics, reported coronavirus disease-19 (COVID-19) cases showed that this category of patients usually presented with no symptoms or mild to moderate COVID-19 disease. However, infants less than one-year-old had a more severe presentation or even critical condition and respiratory failure. Patients with chronic disease and congenital heart disease (CHD) may have serious effects on course of COVID-19 in neonate and early infancy. There is very limited data about confirmed COVID-19 cases with CHD in neonates and early infancy. We report our case with confirmed COVID-19 diagnosed in the neonatal period with multiple ventricular septal defects (VSDs) and patent ductus arteriosus (PDA) who presented with respiratory distress and respiratory acidosis.