Combined tricuspid atresia- AV septal defect-a rare congenital cardiac abnormality.

INTRODUCTION: Congenital heart diseases (CHDs) are one of the most common birth defects worldwide with a prevalence of 1%. CHDs can be classified into cyanotic and acyanotic diseases based on the presence or absence of the characteristic bluish discoloration of skin and mucus membranes. A subset of cyanotic diseases is single ventricle malformations. This group of disorders comprises 1% of all CHDs. A remarkable yet rare and underreported entity of single ventricle malformations is combined tricuspid atresia (TA) and atrioventricular (AV) septal defect which is characterized by the anatomical features of both entities. Combined TA-AV septal malformation was first anatomically described in 1953 and further explored through echocardiography and cardiac catheterization in 1987 and then 1991. Since then, no studies have been documented in the literature prompting us to share our findings. METHODS: Herein we are describing a rare and underreported cardiac lesion based of a retrospective revision of medical charts at the American University of Beirut Medical Center (AUBMC) Children's Heart Center, a tertiary medical center in the Middle East RESULTS: Out of 200 cases with confirmed single ventricle physiology, we identified a few patients with characteristics of combined TA-AV septal defect. Our patients exhibited characteristic echocardiographic findings of primum ASD, VSD, atretic RAVV, and clefted LAVV. CONCLUSION: In short, TA-AV septal defect is a rare, underreported congenital malformation. Tracking our patients' clinical profiles will help improve our understanding of the prognosis of this entity. Our findings may also improve treatment modalities since replacing the left-sided valve is often overlooked if the defect is inaccurately diagnosed. In addition, such findings can help shed light on the embryological development of the rarely encountered variation of AV septal malformation.

Pre-operative assessment of pediatric congenital heart disease patients in the COVID-19 era: lessons learned.

BACKGROUND: Equal to COVID-19 patients, non-COVID-19 patients are affected by the medical and social drawbacks of the COVID-19 pandemic. A significant reduction in elective life-changing surgeries has been witnessed in almost all affected countries. This study discusses an applicable and effective pre-operative assessment protocol that can be applied during the COVID-19 era. METHODS: Our study is a descriptive retrospective observational study that involves children with CHD requiring open-heart surgeries at our tertiary care centre between March and November, 2020. We reviewed the charts of eligible patients aged 18 years and below. We identified the total numbers of scheduled, performed, and postponed surgeries, respectively. A thorough description of the clinical and physical presentation of the postponed cases, who tested positive for SARS-CoV-2, is provided. RESULTS: Sixty-eight open-heart surgeries were scheduled at our centre between March and November, 2020. Three surgeries (4%) were postponed due to COVID-19. The three patients were asymptomatic COVID-19 cases detected on routine SARS-CoV-2 polymerase chain reaction testing. No symptoms of cough, chest pain, dyspnea, rhinorrhea, diarrhea, abdominal pain, anosmia, and ageusia were reported by our patients. All patients were afebrile and hemodynamically stable. Owing to the pre-operative assessment protocol that was implemented after the first case was detected, only three healthcare workers were at risk of COVID-19 transmission and were imposed to infectious evaluation and home quarantine. CONCLUSIONS: Adopting our discussed preoperative COVID-19 assessment protocol for CHD patients is an effective method to detect COVID-19 infections, optimise patient care, and ensure healthcare workers' safety.

The use of steroids in treating chylothorax following cardiac surgery in children: a unique perspective.

BACKGROUND: Chylothorax is the accumulation of chyle fluid in the pleural space. The incidence of chylothorax is quite common post-cardiac surgeries in pediatrics especially in Fontan procedures. Although several treatment lines are known for the management of chylothorax, steroids were scarcely reported as a treatment option. We present a unique case of a 4-year-old child who underwent Fontan procedure and suffered a long-term consequence of chylothorax. The chylothorax only fully resolved after introducing corticosteroids as part of her management. METHODS: A literature review about management of chylothorax post-cardiac surgery in children using Ovid Medline (19462021), PubMed, and google scholar was performed. CONCLUSION: Conservative management without additional surgical intervention is adequate in most patients. Additionally, somatostatin can be used with variable success rate. However, a few cases mentioned using steroids in such cases. More research and reporting on the use of steroids in the treatment of chylothorax post-cardiac surgeries in children is needed to prove its effectivity. In this article, we describe a case of chylothorax post-Fontan procedure that supports the use of steroids.

Balloon Valvuloplasty for Congenital Aortic Stenosis: Experience at a Tertiary Center in a Developing Country.

BACKGROUND: Aortic valve stenosis accounts for 3-6% of congenital heart disease. Balloon aortic valvuloplasty (BAV) is the preferred therapeutic intervention in many centers. However, most of the reported data are from developed countries. MATERIALS AND METHODS: We performed a retrospective single-center study involving consecutive eligible neonates and infants with congenital aortic stenosis admitted for percutaneous BAV between January 2005 and January 2016 to our tertiary center. We evaluated the short- and mid-term outcomes associated with the use of BAV as a treatment for congenital aortic stenosis (CAS) at a tertiary center in a developing country. Similarly, we compared these outcomes to those reported in developed countries. RESULTS: During the study period, a total of thirty patients, newborns (n = 15) and infants/children (n = 15), underwent BAV. Left ventricular systolic dysfunction was present in 56% of the patients. Isolated AS was present in 19 patients (63%). Associated anomalies were present in 11 patients (37%): seven (21%) had coarctation of the aorta, two (6%) had restrictive ventricular septal defects, one had mild Ebstein anomaly, one had Shone's syndrome, and one had cleft mitral valve. BAV was not associated with perioperative or immediate postoperative mortality. Immediately following the valvuloplasty, a more than mild aortic regurgitation was noted only in two patients (7%). A none-to-mild aortic regurgitation was noted in the remaining 93%. One patient died three months after the procedure. At a mean follow-up of 7 years, twenty patients (69%) had more than mild aortic regurgitation, and four patients (13%) required surgical intervention. Kaplan-Meier freedom from aortic valve reintervention was 97% at 1 year and 87% at 10 years of follow-up. CONCLUSION: Based on outcomes encountered at a tertiary center in a developing country, BAV is an effective and safe modality associated with low complication rates comparable to those reported in developed countries.

Congenital Heart Disease in Syrian Refugee Children: The Experience at a Tertiary Care Center in a Developing Country.

The influx of Syrian refugees to Lebanon that began in 2012 created new health-care and financial stressors on the country with an increase in communicable and non-communicable diseases. This study aims to describe the presentations, diagnoses, management, financial burden, and outcomes among Syrian refugees with congenital heart disease (CHD) in Lebanon. This is a retrospective study that was conducted through reviewing the charts of all Syrian pediatric patients referred to the Children's Heart Center at the American University of Beirut Medical Center for evaluation between the years 2012 and 2017. We reviewed the charts of 439 patients. The mean age at presentation was 3.97 years, and 205 patients (46.7%) were females. 99 Patients (22.6%) were found to have no heart disease, 69 (15.7%) had simple, 146 (33.3%) had moderate, and 125 (28.5%) had complex heart diseases. 176 (40.1%) Patients underwent interventional procedures, with a surgical mortality rate of 10.1%, compared to a rate of 2.9% among non-Syrian children. The average cost per surgical procedure was $15,160. CHD poses a significant health and financial burden on the Syrian refugee population in Lebanon, a small country with very limited resources. The Syrian cohort had a higher frequency of complex cardiac lesions, presented late with additional comorbidities, and had a strikingly elevated surgical mortality rate. Securing appropriate funds can improve the lives of this population, ease the financial burden on the hosting country, provide adequate health-care services, and improve morbidity and mortality.

Large congenital left atrial wall aneurysm: An updated and comprehensive review of the literature.

BACKGROUND: Congenital left atrial wall aneurysms are rare abnormalities that arise from a developmental weakness in the muscular wall. It may be misdiagnosed or go undetected and the delay in diagnosis can lead to catastrophic consequences. CASE PRESENTATION: An updated and comprehensive review of the literature was performed for all patients with this abnormality under the age of 18. A total of 15 cases including ours are presented in this article. We present a 10-month-old boy who was referred to our center for cardiomegaly. Workup revealed a large atrial wall aneurysm that was successfully corrected with surgery. CONCLUSION: Historically, left atrial aneurysms were uncommon in the absence of valvular heart disease or other cardiac conditions. Congenital aneurysms are rare phenomenon because they arise without an acquired cardiac disease and surgical correction is crucial for survival.

Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.

An aortopulmonary window (APW) is a rare congenital heart defect involving an abnormal communication between the ascending aorta and the pulmonary trunk with separate aortic and pulmonary valves. This defect accounts for 0.2% of all congenital cardiac anomalies and if left untreated can lead to Eisenmenger syndrome, severe pulmonary hypertension, heart failure, and poor survival. The authors herein present a case of APW type III with Eisenmenger syndrome in an adult patient whose initial complaint was cyanosis, and provide a thorough review of the literature of cases of APW with Eisenmenger syndrome that have survived into adulthood.

Neurally adjusted ventilatory assist for children on veno-venous ECMO.

NAVA may improve veno-venous ECMO weaning in children. This is a retrospective small series, describing for the first time proof-of-principle for the use of NAVA in children on VV ECMO. Six patients (age 1-48 months) needed veno-venous ECMO. Controlled conventional ventilation was replaced with assisted ventilation as soon as lung compliance improved, and could trigger initiation and termination of ventilation. NAVA was then initiated when diaphragmatic electrical activity (EAdi) allowed for triggering. NAVA was possible in all patients. Proportionate to EAdi (1.8-26 µV), initial peak inspiratory pressures ranged from 21 to 34 cm H2O, and the tidal volume (Vt) from 3 to 7 ml/kg. During weaning, peak pressures increased proportionally to EAdi increase (5.2-41 µV), with tidal volumes ranging from 6.6 to 8.6 ml/kg. ECMO was weaned after a median time of 1.75 days on NAVA. Following ECMO weaning, the median duration of mechanical ventilation, and intensive care unit stay were 4.5 days, and 13.5 days, respectively. Survival to hospital discharge was 100%. In conclusion, combining NAVA to ECMO in paediatric respiratory failure is safe and feasible, and may help in a smoother ECMO weaning, since NAVA allows the patient to drive the ventilator and regulate Vt according to needs.

A Novel Somatic Variant in HEY2 Unveils an Alternative Splicing Isoform Linked to Ventricular Septal Defect.

Congenital heart defects (CHDs) are the leading cause of death in infants under 1 year of age. Aberrations in the expression and function of cardiac transcription factors (TFs) are a major contributor to CHDs. Despite the numerous studies undertaken to functionally characterize these TFs, their exact role in different stages of cardiogenesis is still not fully elucidated. Here we focused on HEY2, a basic helix loop helix transcriptional repressor, and its potential role in human ventricular septal defects. Genetic analysis was performed based on sequencing of DNA and cDNA obtained from post-operational cardiac tissues and blood of 17 Lebanese patients with various CHDs. The screen covered the entire coding regions of the GATA4, NKX2.5, TBX5, TBX20 and HEY2 genes. Our results revealed two novel somatic mutations, namely p.Ala229Thr and p.161_190 del, affecting HEY2 in the diseased cardiac tissues of two patients with VSD. These results suggest a potential role of HEY2 in regulating ventricular septation in humans.

Correction to: A Novel Somatic Variant in HEY2 Unveils an Alternative Splicing Isoform Linked to Ventricular Septal Defect.

The original version of this article unfortunately contained a mistake in the author name. The first author name should be Manal Fardoun instead of Manal Fardon. The original article has been corrected.

Transcatheter Closure of Atrial Septal Defects: Comparable Experience and Outcomes Between Developing and Developed Countries.

Atrial septal defect (ASD) is one of the most common congenital heart defects. Transcatheter device closure of ASDs is safe and effective with most of the reported data being described from developed countries. To evaluate the short and mid-term results and experience of device closure of ASDs at a tertiary center in a developing country and compare it to that from developed countries. Retrospective study based on data collection from all patients who have undergone transcatheter percutaneous device closure for ASD from January 2005 until December 2017 at the Children's Heart Center at the American University of Beirut, Medical Center, Lebanon. During the study period, a total of 254 cardiac catheterizations were performed for device closure of ASDs. The mean age of the patients was 18 ± 17.9 years with 37% being less than 6 years of age. Females were 54%. Defect size ranged from 7 to 37 mm and device size ranged from 8 to 40 mm. The procedure was executed with a success rate of 96%. Five patients had device embolization (2%); in one patient the device was snared and for the remainder the devices were removed surgically. None of the study patients had thrombus formation, neurological complications, bacterial endocarditis, or cardiac erosions. There was no mortality. Device closure of ASDs at our tertiary center in a developing country has an effective and safe profile with excellent results and low complications rates, which compare favorably to those reported from centers in developed countries.

Steroids as a possible effective therapy in the management of large isolated chylopericardium following open heart surgery.

BACKGROUND: Chylopericardium is the collection of lymph fluid inside the pericardial cavity. The incidence of chylopericardium is very low, as this diagnosis is rarely reported following cardiac procedures in children. While some reports were published worldwide on isolated chylopericardium after cardiac surgeries for diverse reasons, it has never been reported after repair for partial anomalous pulmonary venous return. In addition, management of this diagnosis ends up being surgical with minimal concentration on medical treatment which proved unsuccessful. We present a medical approach with corticosteroids as an effective method to treat isolated chylopericardium. CASE PRESENTATION: In this manuscript, we present an approach to treat isolated post-operative chylopericardium in a child following repair of partial anomalous pulmonary venous return. Chylous drainage responded to corticosteroids and completely ceased. There was no need for surgical intervention. CONCLUSION: Until now, isolated chylopericardium has never been reported to occur with partial anomalous pulmonary venous return repair. A review of the literature showed that most patients follow a conservative approach consisting of diuretics and non-steroidal anti-inflammatory agents with some of them undergoing surgical re-intervention. With future research on the topic still needed, we hope that this will encourage physicians worldwide to consider administering a trial of corticosteroids as an option to treat chylopericardium.

Percutaneous closure of patent ductus arteriosus in children using amplatzer duct occluder II: relationship between PDA type and risk of device protrusion into the descending aorta.

OBJECTIVES: To compare the efficacy and safety of Amplatzer Duct Occluder II (ADOII) among the various patent ductus arteriosus (PDA) types, and to assess the association between development of aortic obstruction and the PDA type in terms of measurable parameters as the device angulation and distance of upper end protrusion into the aortic lumen. METHODS: Retrospective cohort study involving 50 consecutive subjects who underwent ADO II device closure of PDA. RESULTS: The median age and weight at intervention were 13 months (5.5 months to 18 years) and 11 (6-67) kg respectively. The median smallest ductal diameter by angiography was 3.2 (1.9-5.4) mm. Thirty two patients had type A PDA, 5 had type C, 5 had type D, and 8 had type E. Residual shunt was seen in only 1 patient who had a tubular PDA and resolved within 2 months of the procedure. No device embolization or pulmonary side protrusion were noted. There was a 16% aortic protrusion rate. The median distance of protrusion of the upper end of the device into the aortic lumen was 3.1 (0-9) mm and the median angle formed between the aortic end of the device and the PDA take-off was 10.4 (0-80.6) degrees. These latter parameters of aortic obstruction were significantly higher in the non-conical PDA group as compared to the conical PDA. Nevertheless, there was no significant coarctation due to aortic retention disc protrusion. CONCLUSION: Device closure of PDA using the ADO II is a safe procedure for chosen types of PDA. We demonstrated a novel technique for objective assessment of device protrusion into the descending aorta based on measurable parameters. ADOII device closure of non-conical PDAs warrants closer follow ups.

[Supravalvular aortic stenosis with and without coronary lesions in pediatrics. A Lebanese study]. / STÉNOSE SUPRAVALVULAIRE AORTIQUE AVEC OU SANS LÉSIONS CORONAIRES EN PÉDIATRIE--ÉTUDE LIBANAISE.

OBJECTIVE: This study aims to analyze several parameters concerning the supravalvular aortic stenosis (SVAS) in children such as age of diagnosis, place of residence, the existence of a metabolic disorder or dysmorphic syndrome and possible damage to the coronary ostia, the means of diagnosis and outcome of these patients. MATERIALS AND METHODS: A large group of patients (2868) with congenital heart disease enrolled between 1 May 1999 and 30 April 2010 at the National Register of Pediatric and Congenital Heart Disease, Lebanese Society of Cardiology. RESULTS: SVAS were found in 14 patients (0.5%) aged 8.5 months to 15 years. The Williams Beuren syndrome was the most common etiology (6 cases) without ostial stenosis, asymptomatic and not treated, followed by forms without dysmorphic syndrome (5 cases) and without ostial stenosis, one patient was operated because of severe SVAS; finally, 3 cases of homozygous familial hypercholesterolemia treated differently: a patient had a successful liver transplantation at age of 4.5 years but has developed, despite the normalization of cholesterol level, a SVAS associated with severe ostial lesions 10 years after transplantation, another treated by coronary artery bypass graft surgery and the latter treated medically. CONCLUSION: SVAS is a very rare disease, but its discovery must lead to search for coronary lesions especially in presence of homozygous familial hypercholesterolemia. The ultrasound monitoring is mandatory and is designed to detect this anomaly and early coronary lesions. The slightest suspicion should carry out more extensive explorations to detect ostial stenosis.

[Linear insertion of atrioventricular valves in children with and without Down's syndrome]. / Insertion linéaire des valves auriculo-ventriculaires chez les enfants avec et sans syndrome de Down.

OBJECTIVE: Study various parameters encountered in the linear insertion of the atrioventricular valves (LIAVV) in patients with and without Down's syndrome (DS). MATERIAL: A group of patients (n = 6240) aged from 0 to 16 years with and without congenital heart disease (CHD) registered over 6 years (1.07.2005-30.06.2011) in the National Registry of the Pediatric and Congenital Heart Disease of the Society of Cardiology in Lebanon. METHOD: Children were divided in two groups; Group I: children with DS and group II: children without DS. In the two groups, the frequency of LIAVV whether isolated or associated with other CHD (excluding the atrioventricular canal), the distribution boy-girl and the various associated cardiac anomalies were studied. We used nonparametric tests for comparing the two groups. The p value < 0.05 was considered significant. RESULTS: Group I consists of 155 patients including 31 (20%) with LIAVV, isolated in 22 cases (14.2%) and associated with CHD in 9 cases (5.8%). Group II was composed of 5995 patients; the incidence of LIAVV was 0.2% (14 patients) with apvalue < 0.00001. Regarding sex, there was a marked male predominance: respectively 78% and 56% in groups I and II. In patients with isolated LIAVV, 86% of patients were males. Family history of DS is found in 3/7 children without T21 with isolated LIAVV. A patient, in whom an atrial septal defect ostium primum type with LIAVV was diagnosed during the first month of life, has seen his septal defect closed spontaneously five months later. CONCLUSION: Encountered predominantly among a male population, the isolated LIAVV is less frequent in children without DS but often with family history of Down's syndrom.

Hybrid stage 1 palliation for HLHS: the experience of a tertiary center in a developing country.

Background: Hypoplastic left heart syndrome (HLHS) accounts for 2.6% of congenital heart disease and is an invariably fatal cardiac anomaly if left untreated. Approximately 33,750 babies are born annually with HLHS in developing countries. Unfortunately, the majority will not survive due to the scarcity of resources and the limited availability of surgical management. Aim: To describe and analyze our experience with the hybrid approach in the management of HLHS in a developing country. Methods: We performed a retrospective single-center study involving all neonates born with HLHS over five years at the Children's Heart Center at the American University of Beirut. The medical records of patients who underwent the hybrid stage 1 palliation were reviewed, and data related to baseline characteristics, procedure details and outcomes were collected to describe the experience at a tertiary care center in a developing country. Results: A total of 18 patients were diagnosed with HLHS over a five-year period at our institution, with male to female ratio of 1:1. Of those, eight patients underwent the hybrid stage I procedure. The mean weight at the time of the procedure was 3.3 ± 0.3 kg with an average age of 6.4 ± 4 days. The mean hospital length of stay was 27.25 days, with an interquartile range of 33 days. The cohort's follow-up duration averaged 5.9 ± 3.5 years. The surgical mortality was zero. Only one mortality was recorded during the interstage period between stage I and II and was attributed to sepsis. Notably, all surviving patients maintained preserved and satisfactory cardiac function with good clinical status. Conclusion: Our limited experience underscores the potential of developing countries with proper foundations to adopt the hybrid procedure for HLHS, yielding outcomes on par with those observed in developed countries. This demonstrates the viability of establishing a more balanced global landscape for children with congenital heart disease.

Congenital Blood Cyst of a Child: A case report and review of literature.

Blood-filled cysts of the heart valves are frequently reported at postpartum autopsies of infants. They are seen as round nodules mostly in the paediatric age group in infants less than 2 months of age and disappear spontaneously within 6 months of life. We report a unique case of an 11-month-old girl who presented at a tertiary healthcare hospital in 2022 with a blood-filled cyst on the posterior leaflet of the pulmonary valve that was successfully treated. This case report highlights the characteristics and course of a paediatric patient with blood-filled cysts. Further studies are yet needed to better understand the diagnostic approaches to blood-filled cysts as well as treatment modalities to fill the gap in clinical settings.

Pulmonary artery banding: a 20-year experience at a tertiary care center in a developing country.

Aim: Pulmonary artery banding serves as an important palliative procedure used for the management of several congenital heart lesions. This study aims to describe a 20-year experience of pulmonary artery banding at a tertiary care center in a developing country. Methods: This is a retrospective chart review of patients who underwent pulmonary artery banding over a 20-year period between January 2000 and July 2020 in a tertiary care center in a developing country. Data regarding demographics, indications, diagnosis, echocardiographic findings, postoperative complications, hospital stay, and outcomes were recorded. Results: A total of 143 patients underwent pulmonary artery banding between 2000 and 2020, with a decrease from approximately 15 surgeries per year in 2012 to 1-2 surgeries a year in 2020. At the time of banding, the median age of patients was approximately 90 days [interquartile range, IQR, 30-150 days]. Four patients (2.8%) died during the band placement. No significant association was observed between baseline characteristics or type of heart defect at presentation and postoperative morbidity and mortality. Conclusion: Pulmonary artery banding remains useful in a subset of congenital heart lesions and as a surgical palliation, with relatively low mortality, allowing postponement of total correction to a higher weight. This technique continues to be valuable in developing countries or for heart surgical programs with limited resources.

Pulmonary migration of a fragment of plastic coating sheared from a stylet.

BACKGROUND: In trauma patients, particularly with head immobilization, tracheal intubation without the use of a stylet may be impossible. OBJECTIVES: To report a very rare but potentially fatal complication that may happen in any Emergency Department: fracture of the plastic sheath of an intubation stylet, reported only twice before in the literature. CASE REPORT: Two large plastic fragments detached from a stylet while intubating a trauma patient. One piece was removed from the endotracheal tube a few hours later in the operating room. The second fragment migrated asymptomatically into the pulmonary airway. It was successfully retrieved from the right bronchus 24 h later. CONCLUSION: This potentially life-threatening event may go unnoticed after intubation if the endotracheal tube is not obstructed by the fragment. Gentle withdrawal of the stylet from the tube is essential to avoid stylet fracture. Careful examination of the stylet after intubation may suggest a stylet fracture.

Pathogenesis and distribution of infective endocarditis in the pediatric population: a 20-year experience in a tertiary care center in a developing country.

Introduction: Infective endocarditis is an infection of the endothelial surfaces of the heart. It is more prevalent in adults but its incidence in the pediatric population has been on the rise. The most important factor remains congenital heart disease and the most isolated pathogen is viridans group streptococcus. Methods: In this manuscript, we present a 20-year experience of a major referral tertiary care center in diagnosing and treating pediatric patients with endocarditis. A retrospective analysis of records of patients who were diagnosed with infective endocarditis under the age of 18 years is presented in this study. Variables relating to the demographic, imaging, microbiologic and pathologic data are described. Outcomes relating to complications and need for surgical repair are also portrayed. Results: A total of 70 pediatric patients were diagnosed with endocarditis in this time interval. The medical records of 65 patients were comprehensively reviewed, however the remaining 5 patients had severely missing data. Of the 65 patients, 55.4% were males, and the mean age at diagnosis was 7.12 years. More than half of the population (58.5%) had vegetation evident on echocardiography. The pulmonary valve was the most commonly affected (50%), followed by the mitral valve and tricuspid valves (15.6%). Most patients received empiric treatment with vancomycin and gentamicin. Viridans group streptococcus was the most frequently isolated organism (23.4%). Conclusion: Among pediatric patients diagnosed with endocarditis in this study, data pertaining to valve involvement and microbiologic information was consistent with the published literature. The incidence of complications and the need for surgical repair are not significantly correlated with demographic and clinical variables.