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.

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.

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.

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.

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.

COVID-19: potential therapeutics for pediatric patients.

The global spread of COVID-19 has imparted significant economic, medical, and social burdens. Like adults, children are affected by this pandemic. However, milder clinical symptoms are often experienced by them. Only a minimal proportion of the affected patients may develop severe and complicated COVID-19. Supportive treatment is recommended in all patients. Antiviral and immunomodulatory medications are spared for hospitalized children with respiratory distress or severe to critical disease. Up till now, remdesivir is the only USFDA-approved anti-COVID-19 medication indicated in the majority of symptomatic patients with moderate to severe disease. Dexamethasone is solely recommended in patients with respiratory distress maintained on oxygen or ventilatory support. The use of these medications in pediatric patients is founded on evidence deriving from adult studies. No randomized controlled trials (RCTs) involving pediatric COVID-19 patients have assessed these medications' efficacy and safety, among others. Similarly, three novel monoclonal anti-SARS-CoV-2 spike protein antibodies, bamlanivimab, casirivimab and imdevimab, have been recently authorized by the USFDA. Nonetheless, their efficacy has not been demonstrated by multiple RCTs. In this review, we aim to dissect the various potential therapeutics used in children with COVID-19. We aspire to provide a comprehensive review of the available evidence and display the mechanisms of action and the pharmacokinetic properties of the studied therapeutics. Our review offers an efficient and practical guide for treating children with COVID-19.

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.

Placement of Labcor Pulmonary Conduit Results in a High Incidence of Postoperative Fever.

BACKGROUND: Fever in the postoperative period in cardiac patients is common. The purpose of this study is to recognize the risk factors for prolonged postoperative fever in cardiac patients with pulmonary conduit insertion. METHODS: Patients were identified retrospectively by looking at the International Classification of Diseases, Ninth Revision, Clinical Modification procedure code for pulmonary conduit insertion between June 2009 and December 2015 at the American University of Beirut Medical Center. Data about preoperative, perioperative, and postoperative variables were collected. Data entry and analysis were performed using SPSS version 22. RESULTS: The study identified 59 patients. The most common type of pulmonary conduit used was the Contegra type (57.6%) (n = 34), followed by the Labcor type (20.3%; n = 12). Postoperative fever occurred in 61% of patients (n = 36). Fourteen patients (38.8%) had a prolonged fever that lasted for more than seven days. Prolonged postoperative fever was significantly associated with the Labcor pulmonary conduit (P value < .001) and a longer duration of pacing wires (P value: .039). Significantly prolonged fever that lasted for more than 21 days occurred in five patients who all had inserted the Labcor pulmonary conduit. CONCLUSIONS: The Labcor pulmonary conduit type is a risk factor for prolonged postoperative fever. The protracted use of pacing wires could be a consequence of the prolonged fever rather than a cause. In the absence of a demonstrable infectious etiology for prolonged postoperative fever in cardiac patients with pulmonary conduit insertion, the Labcor pulmonary conduit could be the underlying cause. Alternative management of such cases may lead to decreased antibiotic use and morbidity.

Founder Mutation in N Terminus of Cardiac Troponin I Causes Malignant Hypertrophic Cardiomyopathy.

BACKGROUND: Cardiac troponin I (TNNI3) gene mutations account for 3% of hypertrophic cardiomyopathy and carriers have a heterogeneous phenotype, with increased risk of sudden cardiac death (SCD). Only one mutation (p.Arg21Cys) has been reported in the N terminus of the protein. In model organisms, it impairs PKA (protein kinase A) phosphorylation, increases calcium sensitivity, and causes diastolic dysfunction. The phenotype of this unique mutation in patients with hypertrophic cardiomyopathy remains unknown. METHODS: We sequenced 29 families with hypertrophic cardiomyopathy enriched for pediatric-onset disease and identified 5 families with the TNNI3 p.Arg21Cys mutation. Using cascade screening, we studied the clinical phenotype of 57 individuals from the 5 families with TNNI3 p.Arg21Cys-related cardiomyopathy. We performed survival analysis investigating the age at first SCD in carriers of the mutation. RESULTS: All 5 families with TNNI3 p.Arg21Cys were from South Lebanon. TNNI3 p.Arg21Cys-related cardiomyopathy manifested a malignant phenotype-SCD occurred in 30 (53%) of 57 affected individuals at a median age of 22.5 years. In select carriers without left ventricular hypertrophy on echocardiogram, SCD occurred, myocyte disarray was found on autopsy heart, and tissue Doppler and cardiac magnetic resonance imaging identified subclinical disease features such as diastolic dysfunction and late gadolinium enhancement. CONCLUSIONS: The TNNI3 p.Arg21Cys mutation has a founder effect in South Lebanon and causes malignant hypertrophic cardiomyopathy with early SCD even in the absence of hypertrophy. Genetic diagnosis with this mutation may be sufficient for risk stratification for SCD.

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.

Port-a-Cath fracture and migration in paediatric cancer patients: incidence and management at a tertiary care centre - a 15-year experience.

INTRODUCTION: Port-a-Cath or chemoport provides prolonged central venous access for cancer patients requiring prolonged chemotherapy. Prolonged use of chemoport is associated with many complications. Dislodgement and migration of chemoport catheter is a rare and reportable complication with potentially serious consequences. METHODS: The medical charts of 1222 paediatric cancer patients admitted to the Children's Cancer Center in Lebanon who had chemoports inserted for long-term chemotherapy were retrospectively reviewed. Descriptive analysis of data was conducted. RESULTS: Chemoport fracture and migration were found in seven cases with an incidence of 0.57%. The duration of chemoport use before the event of dislodgement varied from 2 months to 102 months. Non-functioning chemoport was the most common presentation. Totally, six cases were managed successfully by loop snaring, three cases by paediatric cardiology team, and three cases by interventional radiology team. One case was managed surgically during chemoport removal. CONCLUSION: Fracture and migration of chemoport catheter is a rare complication of uncertain aetiology and with potentially serious consequences. Percutaneous retrieval, done by experienced cardiologist or interventional radiologist, is the first choice for management of this complication as it is considered as a safe and effective approach.

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.

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.

Novel EIF2AK4 mutations in histologically proven pulmonary capillary hemangiomatosis and hereditary pulmonary arterial hypertension.

BACKGROUND: Pulmonary hypertension (PH) remains one of the rarest and deadliest diseases. Pulmonary Capillary Hemangiomatosis (PCH) is one of the sub-classes of PH. It was identified using histological and molecular tools and is characterized by the proliferation of capillaries into the alveolar septae. Mutations in the gene encoding the eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) have recently been linked to this particular subgroup of PH. METHODS: In our effort to unveil the genetic basis of idiopathic and familial cases of PH in Lebanon, we have used whole exome sequencing to document known and/or novel mutations in genes that could explain the underlying phenotype. RESULTS: We showed bi-allelic mutations in EIF2AK4 in two non-consanguineous families: a novel non-sense mutation c.1672C > T (p.Q558*) and a previously documented deletion c.560_564drlAAGAA (p.K187Rfs9*). Our histological analysis coupled with the CT-scan results showed that the two patients with the p.Q558* mutation have PH. In contrast, only one of the individuals harboring the p.K187Rfs9* variant has a documented PCH while his older brother remains asymtomatic. Differential analysis of the variants in the genes of the neighboring network of EIF2AK4 between the two siblings identified a couple of interesting missense mutations that could account for this discrepancy. CONCLUSION: These findings represent a novel documentation of the involvement of EIF2AK4 in the different aspects of pulmonary hypertension. The absence of a molecular mechanism that relates the abrogated function of the protein to the phenotype is still a major hurdle in our understanding of the disease.

Atrial septal defect closure complicated by anomalous inferior vena cava return to the left atrium: a case report of a 5-year-old child.

BACKGROUND: The anatomy of the sinus venosus atrial septal defect (ASD) of the inferior vena caval type is complex. Limited rate of complications during its closure has been described. One of the unusual complications, with few case reports, is the iatrogenic diversion of the inferior vena cava (IVC) to the left atrium (LA). CASE SUMMARY: We report the case of a 5-year-old boy who underwent previous surgical closure of sinus venosus ASD of the inferior vena caval type aged 2 years. Three years after surgery, he was diagnosed as having iatrogenic diversion of the IVC to the LA. DISCUSSION: Cardiologists and cardiac surgeons should be aware of this rare but significant complication following routine ASD surgical repair and vigilant follow-up should be performed routinely.

Non-familial cardiomyopathies in Lebanon: exome sequencing results for five idiopathic cases.

BACKGROUND: Cardiomyopathies affect more than 0.5% of the general population. They are associated with high risk of sudden cardiac death, which can result from either heart failure or electrical abnormalities. Although different mechanisms underlie the various types of cardiomyopathies, a principal pathology is common to all and is usually at the level of the cardiac muscle. With a relatively high incidence rate in most countries, and a subsequent major health burden on both the families and governments, cardiomyopathies are gaining more attention by researchers and pharmaceutical companies as well as health government bodies. In Lebanon, there is no official data about the spectrum of the diseases in terms of their respective prevalence, clinical, or genetic profiles. METHODS: We used exome sequencing to unravel the genetic basis of idiopathic cases of cardiomyopathies in Lebanon, a relatively small country with high rates of consanguineous marriages. RESULTS: Five cases were diagnosed with different forms of cardiomyopathies, and exome sequencing revealed the presence of already documented or novel mutations in known genes in three cases: LMNA for an Emery Dreifuss Muscular Dystrophy case, PKP2 for an arrhythmogenic right ventricle dysplasia case, and MYPN for a dilated cardiomyopathy case. Interestingly two brothers with hypertrophic cardiomyopathy have a novel missense variation in NPR1, the gene encoding the natriuretic peptides receptor type I, not reported previously to be causing cardiomyopathies. CONCLUSION: Our results unravel novel mutations in known genes implicated in cardiomyopathies in Lebanon. Changes in clinical management however, require genetic profiling of a larger cohort of patients.

Degenerated hair follicle cells and partial loss of sebaceous and eccrine glands in a familial case of axenfeld-rieger syndrome: An emerging role for the FOXC1/NFATC1 genetic axis.

BACKGROUND: Cutaneous malformations are at times associated with some forms of congenital heart defects. Many a times subtle cutaneous phenotypes maybe overlooked as their significance on the lives of individuals is minimal. Lebanon represents an area of high consanguinity, where the rates can go beyond 70% in some districts. For the past 6 years, we have been studying several genodermatoses in Lebanon including those with cardiac malformations. OBJECTIVES: The main aim of this study is to document the genetic basis of a familial case of Axenfeld-Rieger Syndrome (ARS) with a mild cutaneous phenotype represented histologically with degeneration/ absence of hair follicles and incomplete formation of sebaceous and eccrine glands, in addition to the cardiac and ocular phenotypes. METHODS: Whole exome sequencing was performed on two identical-twins with ARS along with their affected father and non-affected mother. Sanger sequencing was used to confirm the mutation, and the effects of the mutations on protein function was assessed in vitro using transient transfections. RESULTS: A novel mutation inFOXC1 designated p.L240Rfs*75 was found in both twins and their father. The affected individuals share also a rare documented variant in NFATC1 designated p.V197 M. Both were absent from 200 Lebanese exomes. Our in vitro results suggested a gain of function activity of the FOXC1/NFATC1 complex, confirming its documented role in controlling murine hair follicle stem cells quiescence and regeneration. CONCLUSION: This is the first documented human case with a mutation inFOXC1 regulating multi-organ developmental pathways that reflect a conserved mechanism in cell differentiation and proliferation.

Surgical repair of partial atrioventricular defect.

Long-term survival rate of patients operated for partial atrioventricular (AV) canal is lower than that of the general population, and late complications are relatively significant: between 10 and 30% of operated patients present with left AV valve regurgitation, and up to 25% have to be reoperated for valve repair or replacement, left ventricular outflow tract obstruction or residual atrial septal defect. Because the left AV valve regurgitation is the most common complication following surgery, technical details in the surgical management of the mitral valve are the most important aspects of this procedure; for example, the decision to close the cleft and to perform an annuloplasty. The presence of mitral valve anomalies in 7-28% of the cases complicates further the surgical management of these valves. This article will describe in detail the operative technique of partial AV canal repair, and review the relevant literature.

Surgical repair of complete atrioventricular defect (Nunn technique).

Two procedures have been traditionally used for the surgical repair of complete atrioventricular canal. The single-patch technique includes the division of valve leaflets, and the use of one patch to close the ventricular and the atrial septal defects, whereas the double-patch technique uses two separate patches, without the division of the bridging leaflets. Between 1997 and 2007, another technique emerged, the modified single-patch technique, or the 'Australian' technique, whereby the ventricular septal defect (VSD) is closed by the direct apposition of the bridging leaflets against the crest of the defect. Because of the absence of the ventricular septal patch, concerns have been raised about the possible left ventricular outflow tract obstruction (LVOTO), or atrioventricular valve (AVV) distortion, especially in case of a deep VSD, or if the defect extends superiorly. The results of the modified single-patch technique in terms of mortality, immediate and long-term AVV function and LVOTO have been similar to the standard techniques in most reports. This article will describe in detail the operative technique and review the relevant literature.

Noninvasive Nitric Oxide Therapy in Right Ventricular Systolic Dysfunction Following Arterial Switch Procedure.

Right ventricular dysfunction is uncommon following surgical repair of dextrotransposition of great arteries (d-TGA) by the arterial switch procedure. Inhaled nitric oxide (iNO) is a specific pulmonary vasolidator that has proven successful in treating pulmonary hypertension when delivered via endotracheal tube. We present a five-month-old infant with d-TGA and ventricular septal defect (VSD) who developed pulmonary hypertensive crisis, causing severe right ventricular dysfunction with hemodynamic deterioration at day 6 postsurgical repair by VSD closure and arterial switch. He was successfully treated with iNO via nonrebreather face mask avoiding endotracheal reintubation. This modality should be considered in the management of such infants.