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.

Newborn Pulse Oximetry Screening for Detecting Congenital Heart Disease: Experience at a Tertiary Care Center.

Background: Congenital heart disease (CHD) remains the number one birth defect worldwide. Pulse oximetry screening (POS) is a widely used CHD screening modality effective in detecting critical lesions. This study is aimed at assessing the accuracy and cost-effectiveness of POS in a cohort of term well-babies admitted to a regular nursery in a tertiary care center. Methods: We reviewed the charts of term babies admitted to our regular nursery over a period of one year. The results of POS and the findings of echocardiography were collected. Similarly, we explored the records of our fetal echocardiography program to identify the fetuses screened for CHD during the same period. Results: 900 term babies were born and admitted to newborn nursery at our center, and 69 fetuses were evaluated by our fetal cardiology team during the study period. None of our term babies had a positive POS at birth or 24 hours of age. However, 56 babies had a cardiac echo before hospital discharge due to suspicious findings on physical examination or a family history of CHD. A simple noncritical CHD was noted in 10 of them. Additionally, 53 babies underwent echocardiography within the first five years of life; a simple CHD was noted in 6 of them. In parallel, 21 of our fetuses were found to have CHD: 16 simple CHD and 5 critical CHD (CCHD). Conclusion: Despite its cost-effectiveness and efficacy in screening for CCHD, POS is suboptimal for detecting simple CHD. In the absence of a proper prenatal screening and fetal echocardiography program, POS remains a cost-effective modality for detecting CCHD.

Three-dimensional printing of mitral valve models using echocardiographic data improves the knowledge of cardiology fellow physicians in training.

Background: High fidelity three-dimensional Mitral valve models (3D MVM) printed from echocardiography are currently being used in preparation for surgical repair. Aim: We hypothesize that printed 3DMVM could have relevance to cardiologists in training by improving their understanding of normal anatomy and pathology. Methods: Sixteen fellow physicians in pediatric and adult cardiology training were recruited. 3D echocardiography (3DE) video clips of six mitral valves (one normal and five pathological) were displayed and the fellows were asked to name the prolapsing segments in each. Following that, three still images of 3D MVMs in different projections: enface, profile and tilted corresponding to the same MVs seen in the clip were presented on a screen. Participating physicians were presented with a comprehensive questionnaire aimed at assessing whether the 3D MVM has improved their understanding of valvular anatomy. Finally, a printed 3D MVM of each of the valves was handed out, and the same questionnaire was re-administered to identify any further improvement in the participants' perception of the anatomy. Results: The correct diagnosis using the echocardiography video clip of the Mitral valve was attained by 45% of the study participants. Both pediatric and adult trainees, regardless of the year of training demonstrated improved understanding of the anatomy of MV after observing the corresponding model image. Significant improvement in their understanding was noted after participants had seen and physically examined the printed model. Conclusion: Printed 3D MVM has a beneficial impact on the cardiology trainees' understanding of MV anatomy and pathology compared to 3DE images.

Congenital heart disease research landscape in the Arab world: a 25-year bibliometric review.

Background: While research on congenital heart disease has been extensively conducted worldwide, comprehensive studies from developing countries and the Arab world remain scarce. Aim: This study aims to perform a bibliometric review of research on congenital heart disease in the Arab world from 1997 to 2022. Methods: We analyzed data from the Web of Science, encompassing various aspects such as topics, countries, research output, citations, authors, collaborations, and affiliations. This comprehensive science mapping analysis was done using the R statistical software's Bibliometrix Package. Results: The research output from Arab countries over the 25 years showed an average annual growth rate of 11.5%. However, Arab countries exhibited lower research productivity than the United States and Europe, with a 24-fold difference. There was substantial variation in research output among 22 Arab countries, with five countries contributing to 78% of the total publications. Most of the published research was clinical, with limited innovative contributions and a preference for regional journals. High-income Arab countries displayed higher research productivity and citation rates than their low-income developing counterparts. Despite being categorized as upper-middle-income, post-conflict countries exhibited low research productivity. About one-quarter of the published articles (26%) resulted from collaborative efforts among multiple countries, with the United States being the most frequent collaborator. Enhanced research productivity and impact output were strongly associated with increased international cooperation. Conclusion: Research productivity in the Arab region closely correlates with a country's GDP. Success hinges on governmental support, funding, international collaboration, and a clear research vision. These findings offer valuable insights for policymakers, educational institutions, and governments to strengthen research programs and nurture a research culture.

Transcatheter occlusion of a hepatic vein-to-left atrium fistula: Should we close venovenous collateral vessels following Fontan operation?

Right-to-left shunt due to abnormal systemic venous drainage to the left heart is among the causes of hypoxemia following Fontan operation. There are conflicting data regarding the closure of the venovenous collaterals (VVCs) post-Fontan, showing decreased survival in older patients. In a child with visceral heterotaxy, we describe a rare fistula draining a right-sided hepatic vein into hepatic venous plexus and a right-sided pulmonary venous atrium. The patient presented with severe hypoxemia post-Fontan and underwent fistula occlusion with AMPLATZER™ Vascular Plug II, successfully improving hemodynamic status with resolution of the hypoxemia. Younger patients with cyanosis due to VVCs may benefit from percutaneous occlusion post-Fontan.

Cardiac manifestations and short-term outcomes of multisystem inflammatory syndrome in Middle Eastern children during the COVID-19 pandemic: a case series.

We herein report on a series of four patients presented to our tertiary care centre with features of multisystem inflammatory syndrome in children and cardiac involvement. Two of our patients had recent exposure to a COVID-19-positive patient, one had recent documented infection, and another had no known positive contact. All the patients were tested positive for severe acute respiratory syndrome coronavirus 2 immunoglobulin G antibody at the time of presentation. All of them fulfilled the diagnostic criteria according to the World Health Organization Centers for Disease Control or the British guidelines for MIS-C (fever for ≥3 days, multisystem involvement (at least two), elevated markers of inflammation, and no other alternative diagnosis).Cardiac involvement was variable ranging from isolated ectasia of the coronary arteries to full-blown pancarditis such as severe biventricular dysfunction, multi-valvar involvement, and pericardial effusion.All our patients received intravenous immunoglobulin (2 g/kg), methylprednisolone, and aspirin and some required inotropic support and ICU admission.Remarkably, all our patients showed significant improvement in their cardiac disease within a few days as evident on serial echocardiographic evaluation. However, we stress the need for long-term follow-up as one of our patients demonstrated mild LV myocardial scarring as evident by gadolinium late enhancement on a cardiac MRI.

Right Ventricular Volumes, Ejection Fraction, and Systolic Function Indices in Normal Neonates by Three-Dimensional Speckle-Tracking Echocardiography.

The aim of this study was to test the feasibility of measuring right ventricular (RV) volumes, ejection fraction (EF), and systolic function indices in neonates using three-dimensional speckle-tracking echocardiography (3D-STE). Thirty-eight neonates underwent complete echocardiographic evaluation, including the acquisition of three full-volume 3D datasets or more from each of the apical, parasternal, and subcostal windows while naturally sleeping. Datasets were analyzed using a commercially available software (Tomtec). Global RV 3D volumes and EF were measured. In addition, 2D free wall longitudinal strain (LS), tricuspid valve annulus (TVA), tricuspid annular plane systolic excursion (TAPSE) and its index to RV length (TAPSEi), and fractional area change (FAC) were obtained from a non-shortened apical 4-chamber view of the RV, derived from the 3D dataset. Three or more datasets obtained from the apical window were available for analysis for each subject. At least one dataset was adequate for analysis in all subjects. Mean indexed 3D diastolic, systolic, stroke volumes, and EF were measured at 28.5 ± 3.4 ml/m2, 13 ± 2.0 ml/m2, 15.6 ± 1.9 ml/m2, and 54.6 ± 3.2%, respectively. Free wall 2D LS was calculated at (- 27.9 ± 2.5%). In addition, mean TVA measured 11.1 ± 0.8 mm, TAPSE measured 6.8 ± 0.9 mm, and TAPSEi and FAC were calculated at 24.2 ± 2.1 and 46 ± 3.4%, respectively. 3D-STE is feasible in normal neonates without the need for sedation. Reference values of RV 3D volumes and 2D indices of systolic function were obtained. These data could be helpful in patients where the size or systolic function of the RV is in question. Larger studies are required to establish nomograms for the above indices in this age group.

Endovascular Stent Repair of Aortic Coarctation in a Developing Country: A Single-Center Experience.

BACKGROUND/PURPOSE: CoA remains one of the most common congenital heart diseases and is associated with significant morbidity and mortality and if untreated. We aim to evaluate the safety, feasibility, and outcomes of endovascular stenting of Coarctation of the aorta (CoA) in a developing country with limited resources and compare it to available benchmarks. MATERIALS/METHODS: A retrospectively review of all patients who underwent endovascular stent repair of aortic coarctation at our tertiary center since 2009 was done. RESULTS: 18 patients were identified, sixteen had native CoA, while two had recurrent CoA. mean age at the time of procedure was 21.2 ± 9.8 years (range 10-45 years), and 12(66%) patients were males. The mean follow-up duration was 4 ± 2.8 years. Post stenting, the average ascending-to-descending aorta systolic gradient decreased by 42.9 ± 20.4 mmHg (p < 0.001). After the intervention, 13(72.2%) patients achieved normal BP while 5(27.8%) had residual hypertension. Fourteen patients received bare-metal stents, and four had covered stents. Attempted stent implantation was successful in all patients. Our procedural success rate was 94%. On follow-up, no dissections or aneurysmal changes were detected, four patients underwent re-expansion of the stent, one patient with suboptimal stenting result required surgery 6 months after stenting, and two patients had minor post-operative complications. CONCLUSIONS: Endovascular stenting for de-novo or recurrent CoA in children and adults at a tertiary center in a developing country is feasible and safe with outcomes comparable to developed countries. A multidisciplinary team approach is paramount in achieving good results and low complication rates in limited-resource settings.

Acute Kidney Injury Post-cardiac Surgery in Infants and Children: A Single-Center Experience in a Developing Country.

Introduction: The incidence of acute kidney injury (AKI) in pediatric patients following cardiac surgery varies between 15 and 64%, with a mortality rate of 10-89% among those requiring dialysis. This variation in the incidence and mortality of AKI across studies is probably due to the inconsistent definitions used for AKI. The purpose of this study is to present our experience with AKI post-cardiac surgery with emphasis on predisposing or aggravating factors. Patients and Methods: We evaluated the incidence of AKI using the KDIGO criteria in 150 infants and children undergoing cardiac surgeries between 2015 and 2017. Post-operatively, all patients were admitted to the pediatric intensive care unit (PICU) at a tertiary care center in a developing country. This is a retrospective chart review in which data collected included age, gender, type of heart disease, prior cardiac surgeries, RACHS-1 category, and pre- and post-operative creatinine levels. Neonates were not included in this study. Results: Six percent of the studied patients were below 1 year of age, 84% 1-10 years, and 10% 10-18 years. Fourteen patients (9.3%) developed AKI. Patients with cyanotic heart disease were more prone to develop AKI (78%) compared to those with non-cyanotic heart disease (44%). Children with AKI had a higher length of stay in PICU, 2.56 ± 1.44 vs. 4 ± 2.66 (p- 0.02). Serum lactic acid was higher in patients who developed AKI with a mean value of 6.8 ± 6.9 vs. 2.85 ± 1.55 mmol/l in the non-AKI group (p- 0.03). Lower hemoglobin levels and hyperlactic acidemia were significantly more prevalent in the AKI group. There were five deaths in this series (3.3%), and four of those (80%) were in the AKI group. Conclusion: Using the KDIGO criteria, the incidence of AKI in infants and children following cardiac surgery was 9.3%. This is slightly lower than in previously published studies where the range was between 15 and 64%. Children with cyanotic cardiac disease, hyperlactic acidemia, and anemia were more prone to developing AKI. Identifying patients at risk might help decrease the risk of post-operative AKI.

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.

Measurement of Left Ventricular Dimensions and Ejection Fraction in Neonates by Three-Dimensional Echocardiography: A Comparative Study Between Philips QLAB and TOMTEC Software-Are the Values Interchangeable?

Three-dimensional echocardiography is being used to evaluate left ventricular dimensions and ejection fraction in clinical practice. The validity and normal values have been established in a large group of normal adults, children and neonates. The aim of this study was to compare left ventricular dimensions and ejection fraction obtained from the same 3-dimensional echocardiography datasets using 2 commercially available applications: Philips QLAB and Tomtec 4D LEFT VENTRICLE-ANALYSIS. Fifty consecutive newborns or neonates coming to their first newborn visit within the first 3 weeks of live, were recruited. 38 babies underwent full Echocardiographic evaluation, including the acquisition of several 3-dimensional datasets, while naturally sleeping. Left ventricular dimensions, volumes and ejection fraction were measured using Philips QLAB version 9.0 and results were compared to results of the same datasets, analysed using TomTec 4D LV software. Mean left ventricular diastolic, and systolic volumes indexed to body surface area and ejection fraction were: 24.7 ± 3.6 ml/m2, 9.2 ± 1.3 ml/m2 and 62 ± 3.8% using TomTec and 26.6 ± 3.8 ml/m2, 10.4 ± 2 ml/m2 and 63 ± 3.1% using QLAB, respectively. Mean indexed left ventricular diastolic, and systolic volumes measured with QLAB were significantly higher as compared to TomTec with insignificant difference in the ejection fraction. Normal left ventricular indices obtained from 3-dimensional echocardiography datasets were established, using Philips QLAB and TomTec 4D LV ANALYSIS software. Measurements obtained were significantly different between those venders, and hence, may not be used interchangeably.

Arrhythmias in Neonates and Infants at a Tertiary Care Center.

Background Limited data about arrhythmias in neonates and infants are coming out from the Middle East. Objectives To evaluate different types of arrhythmias in neonates and infants at the American University of Beirut Medical Center (AUBMC), a tertiary care center in Lebanon, with the focus on the nature of arrhythmia, treatment modalities and relation to surgery. Methods Data were collected retrospectively from the hospital records. We included all neonates and infants presenting to AUBMC between 2013 and 2017. Collected data included: the type of arrhythmia, the treatment modality used and its success, the need for additional modes of treatment, the relationship to congenital heart diseases, and the cardiac surgeries performed. Results Of 16,346 subjects admitted to AUBMC between 2013 and 2017, 90 subjects developed arrhythmias that required medical intervention. The most frequent types of arrhythmias were supraventricular tachycardia (62.22%), junctional ectopic tachycardia (13.33%), complete heart block (7.78%), atrial flutter (5.56%), multifocal atrial tachycardia (3.33%), Wolf Parkinson White Syndrome (3.33%), non-sustained ventricular tachycardia (2.22%), sinus pause (1.11%), and premature ventricular contractions (1.11%). Bivariate analysis showed a significant difference between arrhythmias not related to cardiac surgery and arrhythmias related to cardiac surgery in terms of the type of arrhythmia developed, the presence of congenital heart defect, prematurity, and electrolyte disturbances (P-value <0.005). However, multivariate logistic regression showed no significant difference between the two groups after adjustment for the significant variables (P-value > 0.05). Conclusion There is a significant difference between arrhythmias not related to cardiac surgery and arrhythmias related to cardiac surgery in neonates and infants at AUBMC. However, the difference disappears after adjusting for different variables.

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.

Establishing a High-Quality Congenital Cardiac Surgery Program in a Developing Country: Lessons Learned.

Background: Developing countries are profoundly affected by the burden of congenital heart disease (CHD) because of limited resources, poverty, cost, and inefficient governance. The outcome of pediatric cardiac surgery in developing countries is suboptimal, and the availability of sustainable programs is minimal. Aim: This study describes the establishment of a high quality in-situ pediatric cardiac surgery program in Lebanon, a limited resource country. Methods: We enrolled all patients operated for CHD at the Children's Heart Center at the American University of Beirut between January 2014 and December 2018. Financial information was obtained. We established a partnership between the state, private University hospital, and philanthropic organizations to support the program. Results: In 5 years, 856 consecutive patients underwent 993 surgical procedures. Neonates and infants constituted 22.5 and 22.6% of our cohort, respectively. Most patients (82.6%) underwent one cardiac procedure. Our results were similar to those of the Society of Thoracic Surgeons (STS) harvest and to the expected mortalities in RACHS-1 scores with an overall mortality of 2.8%. The government (Public) covered 43% of the hospital bill, the Philanthropic organizations covered 30%, and the Private hospital provided a 25% discount. The parents' out-of-pocket contribution included another 2%. The average cost per patient, including neonates, was $19,800. Conclusion: High standard pediatric cardiac surgery programs can be achieved in limited-resource countries, with outcome measures comparable to developed countries. We established a viable financial model through a tripartite partnership between Public, Private, and Philanthropy (3P system) to provide high caliber care to children with CHD.

The first Fetal Echocardiography experience for Prenatal diagnosis of Congenital Heart Disease in Lebanon: Successes and challenges.

OBJECTIVES: To describe the first Lebanese fetal echocardiography experience for prenatal diagnosis of congenital heart diseases (CHD), showcase successes, and hurdles. METHODS: This was a retrospective study from January 2014 to December 2017. A total of 350 fetal echocardiograms for 299 fetuses were performed at the Children's Heart Center at the American University of Beirut, the only fetal center in Lebanon. Data were collected regarding diagnosis, reasons for referral, and timing of referral. RESULTS: The mean gestational age at presentation was 25.3 weeks (standard deviation 4.9 weeks). The primary reasons for referral were abnormal anomaly scan (81 27%), history of previous child with CHD (48 16%), and pre-existing maternal congenital heart disease (15 5%). A total of 144 fetal echocardiograms were normal and 155 patients were diagnosed prenatally with CHD giving a detection rate of 44%. The most identified cardiac lesions were ventricular septal defects (31, 20%), atrial septal defects (15, 9.7%). Significant CHD defined as major abnormalities which would impact pregnancy and future quality of life of the baby were identified in 78 fetuses, with a detection rate of 22%. CONCLUSION: High rates of detection are mainly due to low rates of referral when indicated and possibly parental anxiety regarding CHD diagnosis.

Establishing an ECMO program in a developing country: challenges and lessons learned.

AIM: The ECMO (extracorporeal membrane oxygenation) Program at the American University of Beirut Medical Center was established in November 2015 as the first program serving adult and pediatric population in a low-resource setting. The aim of the study is to describe the challenges faced during the establishment of the program and factors leading to its success. METHODS: The program establishment is described. The preparation phase, included the strategic, financial, and clinical planning by administration, nursing, and a multidisciplinary team of physicians. The training and education phase included all the involved nurses, perfusionists, and physicians. Concerns were heard from various stakeholders, and the challenges were analyzed and discussed. RESULTS: The preparation committee chose the adequate equipment, responded to the concerns, defined roles and responsibilities through credentialing and privileging, wrote policies and protocols, and established a strategy to decide for the ECMO indication. Selected team of nurses, physicians, and perfusionists are identified and trained locally, and abroad. A full-time ECMO physician was recruited to launch the program. Twelve patients (6 adults, 3 children, and 3 neonates) were supported by ECMO, for cardiac and respiratory indications. Eleven patients were supported by veno-arterial ECMO, and 1 patient (a neonate) with veno-venous ECMO. Overall, 75% survived to decannulation and 41% survived to discharge. CONCLUSION: With limited human and financial resources, new ECMO centers need to carefully establish selection criteria that may differ from those used in developed countries. Indications should be discussed on a case by case basis, taking into account clinical, social, and financial issues. This experience might help other institutions in developing countries to build their own program despite financial and human limitations.

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.

Successful use of calcium channel blocker for management of arterio-venous malformations following Fontan procedure.

In diffuse forms of arteriovenous malformation following Fontan procedure, "classical" medical therapy, inhaled nitric oxide and sildenafil, may play a role, until re-direction of hepatic flow to pulmonary circulation cures it. However, in refractory cases, as reported in our 2-year-old patient, unusual medications such as calcium channel blockers can be tried and continued if patients respond adequately.

Gothic aortic arch and cardiac mechanics in young patients after arterial switch operation for d-transposition of the great arteries.

BACKGROUND: In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. HYPOTHESIS: GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE). METHODS: We studied one hundred and five asymptomatic patients, who have undergone first stage ASO for d-TGA, with normal left ventricular ejection fraction (LVEF ≥53%). RESULTS: Forty-six (44%) patients showed a GA (mean age 11.5±7.2years, 26 males) while fifty-nine (56%) patients (mean age 9.6±6.7years, 37 males) did not present GA. The two groups were comparable for age, sex, BSA, and office blood pressure values. In group GA aortic root was significantly dilated (27.4±7.5mm vs. 21.2±6.9mm, p<0.0001), aortic stiffness index (Group GA=1.8±1.2 vs. 1.4±0.7, p=0.025) was significantly increased, left atrial volume was larger (p=0.0145), global longitudinal strain (Group GA=-18.4±2.5% vs. -20.1±3.3%, p=0.012) and basal LV longitudinal strains (Group GA=-16.9±4.8% vs. -20.4±7.0%, p=0.013) were significantly reduced. CONCLUSIONS: After ASO the presence of a GA is associated with a significantly dilated aortic root, stiffer aortic wall, larger left atrial volume, and worse LV longitudinal systolic deformations, well known predictors of cardiovascular morbidity and mortality.