Early and mid-term outcomes of double-chambered right ventricle repair: An 8-year experience.

BACKGROUND/AIM: Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle. METHODS: Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively. RESULTS: An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths. CONCLUSIONS: The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.

Early and Mid-Term Outcomes of Primary Repair After Atrioventricular Canal Defect: A Single-Center Eight-Year Experience.

BACKGROUND/AIM: Surgical repair techniques and management of patients with atrioventricular septal defect (AVSD) have progressed over the last few decades. Early and definitive interventions have become the choice of treatment for these patients. Based on this background, we aimed to review the early and mid-term outcomes of primary AVSD repair. METHODS: A total of 53 patients with a mean age of 3.45 ± 5.67 years underwent definitive repair for AVSD between January 2014 and June 2021. The clinical data including age, type of defect, associated co-anomalies, symptoms, pulmonary hypertension, etc. were collected and assessed retrospectively. Mitral regurgitation (MR) as a clinical outcome was assessed at 0, 1, 2, and 5 years. RESULTS: Among the recruited patients, 35 (66.1%) were male and 18 (33.9%) were female. Of 53 patients, repair for the complete defect was done in 38 (71.69%) patients, repair for intermediate/partial defect was done in 15 (23.1%) patients, and one patient underwent repair for incomplete type. Other associated co-anomalies were anterior mitral leaflet (12 (22.6%)), atrial and ventricular septal defect (VSD) (30 (56.6%)), and patent ductus arteriosus (PDA) (11 (20.8%)). Different procedures for surgical repair included patch closure, cleft repair, and polytetrafluoroethylene (PTFE) VSD closure. After repair, the mean follow-up period was 46.73 ± 27.37 months. Overall mortality was 3.78% (2/53), and two patients underwent reintervention due to symptomatic severe MR. CONCLUSIONS: A definitive and timely correction of AVSD shows satisfactory early and mid-term results.

Effectiveness and Safety of the Ozaki Procedure for Aortic Valve Disease in Pediatric Patients: A Systematic Review and Meta-Analysis.

The surgical treatment options for pediatric aortic valve disease are limited and have debatable long-term durability. In the current situation, the Ross procedure is considered in children for aortic valve disease(s). It is a complex surgical procedure with the risk of neo-aortic dilatation, converting a single valve disease into double valve disease, and associated with future re-interventions. Conversely, the Ozaki procedure has shown promising results in adults. Thus, the present study aimed to provide comparative evidence on the effectiveness and safety of the Ozaki versus Ross procedure for pediatric patients by performing a meta-analytic comparison of reporting outcomes. A total of 15 relevant articles were downloaded and among them, seven articles (one prospective study, five retrospective studies, and one case series) were used in the analysis. Primary outcomes such as physiological laminar flow pattern and hemodynamic parameters, and secondary outcomes such as hospital stays, adverse effects, mortality, and numbers of re-intervention(s) were measured in the meta-analysis. There were no significant differences in the age of patients between children who underwent the Ozaki procedure and those who underwent the Ross procedure at the time of surgeries. The Ozaki procedure is a good solution to an aortic problem(s) similar to the Ross procedure. Unlike the Ross procedure, the Ozaki procedure has restored a physiological laminar flow pattern in the short-term follow-up without the bi-valvular disease. Mean hospital stays (p = 0.048), mean follow-up (p = 0.02), adverse effects (p = 0.02), death, and numbers of re-intervention(s) of children who underwent the Ozaki procedure were fewer than those who underwent the Ross procedure. The time required for re-intervention(s) is higher for children who underwent the Ozaki procedure than those who underwent the Ross procedure. None of the procedures, including the Ozaki procedure for aortic valve disease(s), has significant effects on hemodynamic parameters and the frequent death rate of children after surgeries. Based on our analysis, we may suggest the Ozaki procedure for aortic valve disease surgery in children.

Surgical Outcomes of Post-myocardial Infarction Ventricular Septal Defect/Rupture: A Systematic Review and Meta-Analysis.

Ventricular septal defect (VSD) is a catastrophic acute myocardial infarction (MI) complication. Despite a significant reduction in the prevalence of post-MI VSD with the advancement of surgical techniques, it is still considered fatal with a high mortality rate. The trends in the clinical outcomes of patients with post-MI VSD show discretion due to the complexity of the disease. Therefore, the present analysis aimed to evaluate the surgical outcomes and associated risks in the patients of post-MI VSD. A thorough literature survey resulted in 40 studies of our interest. The pooled proportion of differential variables, including the incidence of cardiogenic shock, 30-day survival, and overall mortality, were estimated using Bayesian hierarchical models. The risk difference was estimated for the location of MI and VSD and mortality in patients with coronary artery bypass graft (CABG). In addition, the heterogeneity tests for inconsistency and publication biases using Egger's and Begg's tests were also estimated. The analysis revealed a significant risk difference of 0.23 and 0.27 for the anterior vs. posterior location of MI and VSD, respectively. Further, the pooled proportion of 30-day survival and mortality was found to be 54.43% (95% credible interval (CI): 52.88-55.98%) and 48.22% (95% CI: 4-12.3%), respectively. Moreover, the heterogeneity test revealed significant inconsistencies in all the datasets with an I2 index of >90% (p<0.0001). Lastly, the publication bias results suggested no evidence of asymmetry and small-study effects. Conclusively, the surgical management of post-MI VSD patients is considered beneficial; however, the outcomes signify its fatal behavior.

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

Ultrasonic prediction of weaning failure in children undergoing cardiac surgery: A prospective observational study.

Background and Aim: To assess the utility of ultrasonic markers like B-line score (LUS), diaphragm thickness (DT), thickening fraction (DTF), and excursion (DE) as predictors of weaning outcomes in children on mechanical ventilation (MV) after cardiac surgery. Methods: This was a prospective observational study done in postcardiac surgical intensive care unit (ICU) of a tertiary care hospital. Children aged 1 month to 18 years, on MV after cardiac surgery from January to November 2017, were included. They were extubated after satisfying institutional weaning criteria. Ultrasound for LUS, DT, DTF, and DE was performed preoperatively, during pressure support ventilation (PSV) before extubation and 4 h after extubation. Results: Patients were divided into weaning failure and success groups based on reintubation within 48 h of extubation. Of the 50 evaluated patients, 43 (86%) were weaned successfully and 7 (14%) had weaning failure. The left DTF during PSV was lower in patients weaning failure (0.00%, interquartile range (IQR) 0.00-14.28 vs 16.67%, IQR 8.33-22.20, P = 0. 012). The left DTF≤ 14.64% during PSV (area under receiver's operating curve 0.795, P = 0.014), 85% sensitivity, and 57% specificity (positive likelihood ratio 1.97, negative likelihood ratio 0.25) could predict weaning failure. Conclusion: The left DTF during PSV is a good predictor of weaning failure in children on MV in postoperative ICU after congenital cardiac surgery. Take home message: In children on mechanical ventilation after cardiac surgery, left DTF during pressure support ventilation is a good predictor of weaning failure.

Utility of portal vein pulsatility fraction in patients undergoing corrective surgery for tetralogy of Fallot.

BACKGROUND: Right ventricle dysfunction is common after corrective surgery for tetralogy of Fallot and is associated with significant morbidity and mortality. We aimed to determine whether an increased portal vein pulsatility fraction (PVPF) was associated with worse clinical outcomes. METHODS: In a prospective, observational, single-centre study, PVPF and other commonly used parameters of right ventricle function were assessed in patients of all ages undergoing corrective surgery for tetralogy of Fallot intraoperatively, with transesophageal echocardiography, before and after bypass, and post-operatively, with transthoracic echocardiography, at days 1, 2, at extubation, and at ICU discharge. The correlation was tested between PVPF and mechanical ventilation duration, prolonged ICU stay, mortality, and right ventricle function. RESULTS: The study included 52 patients, and mortality was in 3 patients. PVPF measurement was feasible in 96% of the examinations. PVPF in the immediate post-operative period had sensitivity of 73.3% and a specificity of 74.3% in predicting the occurrence of the composite outcome of prolonged mechanical ventilation, ICU stay, or mortality. There was a moderate negative correlation of PVPF with right ventricle fractional area change and right ventricle global longitudinal strain (r = -0.577, p < 0.001 and r = 0.465, p < 0.001, respectively) and a strong positive correlation with abnormal hepatic vein waveform (rho = 0.749, p < 0.001). CONCLUSION: PVPF is an easily obtainable bedside parameter to assess right ventricular dysfunction and predict prolonged mechanical ventilation, prolonged ICU stay, and mortality.

"Y" incision and rectangular patch enlargement of aortic annulus and valve reconstruction with autologous pericardium in congenital aortic stenosis.

Surgical aortic valve replacement in children who have undergone prior balloon or surgical valvuloplasty or both is a formidable challenge. The aortic annulus is small, there is no ideal prosthesis and lifelong anticoagulation is highly undesirable. A "Y" incision and rectangular patch enlargement of the aortic annulus introduced by Dr. Bo Yang in 2020 combined with aortic valve reconstruction introduced by Dr. Shigeyuki Ozaki in 2011, is feasible, as described in this case.

Effect of perioperative use of oral triidothyronine for infants undergoing complex congenital cardiac surgeries under cardiopulmonary bypass: A double-blinded randomised controlled study.

Background: Thyroid hormone metabolism disrupts after cardiopulmonary bypass both in adults and pediatric patients. This is known as Euthyroid sick syndrome, and it is more evident in pediatric patients who were undergoing complex cardiac surgeries compared to adults. This decrease in serum T3 levels increases the incidence of low cardiac output, requirement of inotropes, prolonged mechanical ventilation, and prolonged intensive care unit (ICU) stay. Aims and Objectives: The primary objective was to compare the mean Vasoactive-inotropic score (VIS) at 72 hours postoperatively between T3 and Placebo groups. Materials and Methods: One hundred patients were screened, and 88 patients were included in the study. Triidothyronine 1 mic/kg 10 doses 8th hourly was given orally postoperatively to cases and sugar sachets to controls. The blood samples for analysis of FT3, FT4, and TSH were taken every 24 hours postoperatively, and baseline values were taken after induction. Mean VIS scores, ejection Fraction (EF), Left ventricular outflow tract velocity time integral (LVOT VTi), hemodynamics and partial pressure of oxygen/ fraction of inspired oxygen(PaO2/FiO2) were recorded daily. Results: The Mean VIS scores at 72 Hours postoperatively were significantly less in the T3 group (5.49 ± 6.2) compared to the Placebo group (13.6 ± 11.7). The PaO2/FiO2 ratios were comparatively more in the T3 group than the Placebo group. The serum levels of FT3 FT4 were significantly higher in the T3-supplemented group than the Placebo group. The VIS scores were significantly lower from 48 hours postoperatively in children < 6 months of age. Conclusion: In this study, we observed that supplementing T3 postoperatively decreases the ionotropic requirement from 72 hours postoperatively. This is more useful in children <6 months of age undergoing complex cardiac surgeries.

Utility of Transfontanelle Ultrasound in Predicting the Fluid Responsiveness in Children After Repair of Congenital Heart Diseases: A Prospective Observational Study.

OBJECTIVES: Postoperative fluid management in children undergoing cardiac surgery requires a balanced optimization. The blood flow velocity variation in the internal carotid artery (ΔICA) measured through transfontanelle ultrasound has been shown to predict fluid responsiveness during cardiac surgery. It may provide an excellent window to study fluid responsiveness in infants during the postoperative period when the intensivist is faced with the challenges of poor echocardiographic window. The authors aimed to observe the correlation between ΔICA measured on transfontanelle ultrasound with the respiratory variability of peak aortic blood flow velocity (ΔVpeak) on transthoracic echocardiography as a marker of fluid responsiveness in infants on mechanical ventilation during the postoperative period after cardiac surgery. DESIGN: A prospective observational study. SETTING: The postcardiac surgery intensive care unit (ICU) of a tertiary care hospital. PARTICIPANTS: Thirty infants undergoing congenital cardiac surgery. INTERVENTIONS: A fluid bolus of 10 mL/kg administered over 10 minutes at 1 and 6 hours after ICU admission. Patients were categorized into fluid responders and nonresponders based on >15% increase in the indexed stroke volume. MEASUREMENTS AND MAIN RESULTS: A total of 50 fluid boluses were administered in 30 patients. Among these, 22 (73.33%) were responders and 8 (26.67%) were nonresponders. There was moderate correlation between ΔICA and peak aortic blood flow velocity variation (ΔVpeak) (r = 0.59, p ≤ 0.001). The ΔVpeak >14.74% had 68% sensitivity and 75% specificity to predict fluid responsiveness (area under the receiver operating characteristic [AUROC], 0.749; p = 0.001; positive likelihood ratio, 2.71; negative likelihood ratio, 0.43). The ΔICA >9.85% could predict fluid responsiveness in infants (AUROC, 0.728; p = 0.003; 75% sensitivity; 60% specificity; positive likelihood ratio, 1.85; negative likelihood ratio, 0.42). Among children younger than 6 months, ΔICA >9.85% was a better predictor of fluid responsiveness (AUROC, 0.889; p = 0.009) than ΔVpeak >15% (AUROC, 0.778; p = 0.061). CONCLUSION: The ICA variability >9.85% measured via transfontanelle ultrasound is a good predictor of fluid responsiveness in infants, especially those younger than 6 months on mechanical ventilation after cardiac surgery.

A six-chambered heart: cor triatriatum sinister with double-chambered right ventricle in association with ventricular septal defect.

BACKGROUND: Cor triatriatum has been described as a heart with three atria in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two compartments by a fold of tissue, a membrane, or a fibromuscular band. Double-chambered right ventricle, on the other hand, is identified by the presence of an anomalous muscle bundle dividing the right ventricle into two chambers. CASE PRESENTATION: Here, we describe the case of a child who had a combination of both of these rare entities, effectively creating a heart with six chambers. The child underwent a successful intracardiac repair. CONCLUSIONS: The association of CTS with DCRV forming a "6-chambered heart" is extremely rare. Awareness of its existence and accurate preoperative diagnosis has important implications in its surgical repair with all the components of this disease spectrum, further increasing the complexity of a successful surgical repair.

Large collateral from the right coronary artery in a case of coarctation of the aorta with bilateral subclavian artery aneurysms in an adult.

Here, we present a case of a 26-year-old gentleman who presented with a rare combination of bilateral huge subclavian artery aneurysms, coarctation of the aorta with a large coronary artery collateral and a bovine aortic arch. Various mediastinal collaterals have been described in previous cases of coarctation of the aorta, but the association of coronary collaterals is a rare occurrence. To the best of our knowledge, this case has not been reported in literature yet.

Transposition of Great Arteries With Intact Ventricular Septum in an Adult: A Successful Outcome.

The management of dextrotransposition of the great arteries with left ventricular outflow tract obstruction continues to evolve. Even when large intracardiac shunts are present, it remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. We present our experience with a 25-year-old woman who presented with transposition of great arteries with severe left ventricular outflow tract obstruction and underwent a successful single-stage arterial switch operation with preservation of aortic and pulmonary valves.

Assessment of factors affecting short-term pulmonary functions following cardiac surgery: A prospective observational study.

BACKGROUND: Pulmonary complications after cardiac surgery are very common and lead to an increased incidence of post-operative morbidity and mortality. Several factors, either modifiable or non-modifiable, may contribute to the associated unfavorable consequences related to pulmonary function. This study was aimed to investigate the degree of alteration and factors influencing pulmonary function (forced expiratory volume in one second (FEV1) and forced vital capacity), on third, fifth, and seventh post-operative days following cardiac surgery. METHODS: This study was executed in 71 patients who underwent on-pump cardiac surgery. Pulmonary function was assessed before surgery and on the third, fifth, and seventh post-operative days. Data including surgical details, information about risk factors, and assessment of pulmonary function were obtained. RESULTS: The FEV1 and forced vital capacity were significantly impaired on post-operative days 3, 5, and 7 compared to pre-operative values. The reduction in FEV1 was 41%, 29%, and 16% and in forced vital capacity was 42%, 29%, and 19% consecutively on post-operative days 3, 5, and 7. Multivariate analysis was done to detect the factors influencing post-operative FEV1 and forced vital capacity. DISCUSSION: This study observed a significant impairment in FEV1 and forced vital capacity, which did not completely recover by the seventh post-operative day. Different factors affecting post-operative FEV1 and forced vital capacity were pre-operative FEV1, age ≥60, less body surface area, lower pre-operative chest expansion at the axillary level, and having more duration of cardiopulmonary bypass during surgery. Presence of these factors enhances the chance of developing post-operative pulmonary complications.

Utility of Lung Ultrasound in the Estimation of Extravascular Lung Water in a Pediatric Population-A Prospective Observational Study.

OBJECTIVE: Lung ultrasound (LUS) is a promising bedside modality for the estimation of extravascular lung water index (EVLWI), but has not been validated against objective measures in children. This study aimed to investigate the correlation of LUS B-line scoring with EVLWI, thresholds indicating elevated EVLWI, and its outcome following pediatric cardiac surgery. DESIGN: Prospective observational study. SETTING: Cardiothoracic surgical intensive care unit in a tertiary care teaching hospital. PARTICIPANTS: Children younger than 12 years undergoing elective complete surgical correction of cyanotic or acyanotic congenital heart disease (Aristotle score ≤9), excluding neonates, those weighing <3.5 kg, and those with thoracic deformities, pulmonary pathology, and hemodynamic instability. INTERVENTIONS: Extravascular lung water index measurement by transpulmonary thermodilution, along with concurrent LUS B-line and Chest-X ray (CXR) scoring. MEASUREMENTS AND MAIN RESULTS: LUS B-line score had a moderate correlation with EVLWI (Pearson's correlation coefficient 0.57; 95% CI 0.44-0.69). LUS B-line scores showed acceptable discrimination only for higher thresholds of EVLWI (sensitivity 82% and 79%, respectively, for EVLWI >20 mL/kg v sensitivity and specificity 57% and 80% for EVLWI >10 mL/kg). Age, body surface area, vasoactive-inotropic score (VIS), chest X-ray score, and EVLWI but not LUS B-line score were significant predictors for duration of mechanical ventilation in this cohort. CONCLUSIONS: LUS B-line scoring has limited utility in semiquantitative estimation of EVLWI at lower thresholds of EVLWI in pediatric cardiac surgical patients. It may have better discrimination and acceptable sensitivity and specificity at higher thresholds of EVLWI. Contrasting with multiple reports of clinical utility, these results call for wider evaluation of LUS and its clinical modifiers like age, pathology, and pretest probability in estimation of EVLWI.

Fetal cardiac intervention and fetal cardiac surgery: where are we in 21st century?

Fetal cardiac intervention is an in-utero cardiac procedure done in fetuses with heart diseases like severe aortic stenosis with evolving hypoplastic left heart syndrome, hypoplastic left heart syndrome with an intact or restricted atrial septum, pulmonary atresia with an intact ventricular septum, fetal heart block obstructed total anomalous pulmonary venous return, pericardial collection. The successful biventricular repair can be done in postnatal life after aortic or pulmonary valvuloplasty. Fetal bypass is very challenging because of different physiology. Low prime volume with the high flow can be used to prevent an inflammatory response.

Implications of left atrial enlargement and patent foramen ovale creation on early surgical outcomes in patients with total anomalous pulmonary venous connection.

INTRODUCTION: In patients with total anomalous pulmonary venous connection (TAPVC), left atrium (LA) is small and suprasystemic pulmonary artery (PA) pressures may be present in some patients. In our study, we studied the relationship between surgical LA enlargement and patent foramen ovale (PFO) creation separately on the outcomes of patients with TAPVC. MATERIALS AND METHODS: Out of the 130 patients operated in our institute between January 2014 and December 2020, LA was enlarged in 60 patients. LA enlargement was done using a larger patch for atrial septal defect (ASD) closure. Thus, the LA volume was increased by shifting the patch towards the right atrium (RA). Suprasystemic or high PA pressures were present in 60 patients. In 33 patients, PFO was created. Early surgical outcomes were determined on the basis of vasoactive inotropic score (VIS), hours of ventilation, hours of inotropic support, intensive care unit (ICU) stay, and hospital stay. RESULT: Between the LA enlarged and nonenlarged group there was statistically significant less VIS score (18 [13-27.5] vs. 24 [18-30], p value .019), hours of ventilation (23 [16-46.5] vs. 26 [18-60], p value .039), hours of inotropic support (45.5 [30-72] vs. 55 [38-84], p value .038), and ICU stay (7 [5-9] vs. 8 [7-10] p value .0352) and statistically nonsignificant less hospital stay (11.5 [9-13] vs. 12 [9-14], p value .424). In patients with preoperative suprasystemic or high PA pressures, there was a statistically significant less VIS score (16 [11-23.5] vs. 18 [13-25], p value .044), hours of ventilation (20 [14-37] vs. 22 [18-39], p value .038), hours of inotropic support (34 [29.5-71] vs. 38 [30-78], p value .042), and hospital stay (9 [5-12] vs. 11 [9-14], p value .038) and statistically nonsignificant less ICU stay (7 [5.5-9] vs. 7 [6-9], p value .886) in the group with a PFO with respect to the other group in which no PFO was created. CONCLUSION: In patients with TAPVC, LA can be enlarged by using a large ASD patch and thus shifting the septum towards RA. Early surgical outcomes were improved with LA enlargement. In patients with suprasystemic or high PA pressures, leaving a PFO improved the postoperative outcomes.

Iatrogenic aortic regurgitation following percutaneous coronary intervention: Role of transesophageal echocardiography in the detection and management.

The incidence aortic valve injury during percutaneous coronary intervention is scarce, mostly resulting in acute aortic regurgitation. However, rarely patients may remain asymptomatic in the immediate post-procedure period and present latter with chronic aortic regurgitation. Determining etiology of such an aortic regurgitation may be challenging. We present a case of a 51-year-old man with history of percutaneous coronary intervention for coronary artery disease and moderate aortic regurgitation scheduled for coronary artery bypass grafting and aortic valve replacement. Intra-operative transesophageal echocardiography was instrumental in deciding etiology of aortic regurgitation that change surgical management of the patient.

A single coronary artery with left circumflex artery crossing right ventricular outflow tract in tetralogy of Fallot with absent left pulmonary artery.

Tetralogy of Fallot (TOF) with unilateral absence of pulmonary artery and the anomalous coronary artery is a rare combination. Detailed preoperative evaluation of coronary artery anatomy is must to prevent injury to the major vessels crossing right ventricular outflow tract. We report a rare association of single coronary artery with left circumflex artery crossing right ventricular outflow tract close to the pulmonary annulus in tetralogy of Fallot with absent left pulmonary artery in 11-year-old girl. Though there is a great diversity of coronary anomalies in tetralogy of Fallot, the prepulmonic course of left circumflex artery crossing the right ventricular outflow tract (RVOT) close to the pulmonary annulus has rarely been described in the literature. The patient underwent successful primary single lung intracardiac repair. Right ventricular outflow tract obstruction was treated by handmade valved pericardial autologous conduit and release of the tethering of hypoplastic native unicuspid pulmonary valve leaflet maintaining its integrity.