Acute superficial and deep necrosis of lower limb following femoral arterial cannulation in a neonate undergoing arterial switch operation for transposition of great arteries.

Arterial lines are routinely used for hemodynamic monitoring and blood sampling in the operating room and in cardiac surgery intensive care unit. The complications related to arterial line insertion are very low; the knowledge of the relevant artery anatomy, skills and the experience of the operator and selection of a right size cannula plays a vital role in reducing morbidity related to arterial line insertion. We describe extensive superficial and deep necrosis of lower limb following arterial cannula insertion in a preterm neonate undergoing arterial switch procedure and discuss measures to prevent such a complication.

Impact of Type of Enteral Feeds on Early Postoperative Outcomes After Congenital Heart Surgery in Neonates and Young Infants: A Single Center Experience in a Limited Resource Environment.

Background: Breast milk is known to prevent infections and is recommended for enteral feeding of infants after congenital heart surgery (CHS). During the Covid-19 pandemic, expressed breast milk (EBM) was not always available; hence, feeding after CHS was maintained with EBM or infant formula (IF) or both; we evaluated the impact of enteral feed type on early postoperative outcomes after CHS. Methods: In a prospective observational study, consecutive neonates and infants <4 months undergoing CHS were divided into EBM, IF, or EBM+IF groups; incidences of postoperative infections, ventilation duration, intensive care unit (ICU) stay, and mortality were studied. Results: Among 270 patients; 90 (33.3%) received EBM, 89 (32.9%) received IF, and 91 (33.7%) received EBM+IF. IF group had more neonates (78.7%[IF] vs 42.2%[EBM] and 52.7%[EBM+IF], P < 0.001) and greater surgical complexity. Postoperative infections were 9 (10.0%) in EBM; 23 (25.8%) in IF; and 14 (15.4%) in EBM+IF (P = .016). IF group (OR 2.58 [1.05-6.38], P = .040), absence of preoperative feeding (OR 6.97 [1.06-45.97], P = .040), and increase in cardiopulmonary bypass time (OR 1.005 [1.001-1.010], P = .027) were associated with postoperative infection. Ventilation duration in hours was 26 (18-47.5) in EBM; 47 (28-54.5) in IF; and 40 (17.5-67) in EBM+IF (P = .004). ICU stay in days was 4 (3-7) in EBM; 6 (5-9) in IF; and 5 (3-9) in EBM+IF (P = .001). Mortality did not differ (P = .556). Conclusion: IF group had a greater proportion of neonates with higher surgical complexity. Patients who received EBM after CHS had fewer postoperative infections and better postoperative outcomes compared to those receiving IF or EBM+IF.

Comparison of efficacy of two different doses of tranexamic acid in prevention of post operative blood loss in patients with congenital cyanotic heart disease undergoing cardiac surgery.

Background: The optimal dose of tranexamic acid in minimizing perioperative bleeding is uncertain. We compared efficacy of two different doses of tranexamic acid in reducing post-operative blood loss and its side effects in patients with congenital cyanotic heart disease undergoing cardiac surgery. Settings and Design: Prospective observational study at a pediatric cardiac center in South India. Methods: Consecutive cyanotic patients undergoing cardiac surgery were divided into groups I and II to receive either 10 mg/kg or 25 mg/kg of tranexamic acid administered as triple dose regime after induction, during cardiopulmonary bypass, and after protamine. Post-operative blood loss at 24 hours, blood component utilization, incidence of renal dysfunction and seizures were compared. Results: Totally, 124 patients were recruited, 62 in each group. The pre-operative variables and cardiopulmonary bypass time were comparable. Patients receiving 25 mg/kg had lower post-operative blood loss compared to patients in lower dose group (8.04 ± 8.89 vs 12.41 ± 19.23 ml/kg/24 hours, P = 0.03). There was no difference in the transfused volume of packed red cells (9.21 ± 7.13 ml/kg vs 12.41 ± 9.23 ml/kg, P = 0.712), fresh frozen plasma (13.91 ± 13.38 ml/kg vs 11.02 ± 8.04 ml/kg, P = 0.19), platelets (9.03 ± 6.76 ml/kg vs 10.90 ± 6.9 ml/kg, P = 0.14) or cryoprecipitate (0.66 ± 0.59 ml/kg vs 0.53 ± 0.54 ml/kg, P = 0.5) in group II and I, respectively. Two patients developed renal dysfunction secondary to low cardiac output in lower dose group. There were no seizures. Conclusions: Tranexamic acid administered at a dose of 25 mg/kg as triple dose regime is associated with lower post-operative blood loss compared to a lower dose of 10 mg/kg in cyanotic patients undergoing cardiac surgery without causing major adverse effects.

Conversion of prior univentricular repairs to septated circulation: Case selection, challenges, and outcomes.

OBJECTIVES: Complex congenital heart defects that present earlier in life are sometimes channelled in the single ventricle pathway, because of anatomical or logistic challenges involved in biventricular correction. Given the long-term functional and survival advantage, and with the surgeons' improved understanding of the cardiac anatomy, we have consciously explored the feasibility of a biventricular repair in these patients when they presented later for Fontan completion. We present a single institution's 10-year experience in achieving biventricular septation of prior univentricular repairs, the technical and physiological challenges and the surgical outcomes. METHODS: Between June 2010 and December 2019, 246 patients were channelized in the single ventricle pathway, of which 32 patients were identified as potential biventricular candidates at the time of evaluation for Fontan palliation, considering their anatomic feasibility. The surgical technique involves routing of the left ventricle to the aorta across the ventricular septal defect, ensuring an adequate sized right ventricular cavity, establishing right ventricle-pulmonary artery continuity and taking down the Glenn shunt with rerouting of the superior vena cava to the right atrium. This is a retrospective study where we reviewed the unique physiological and surgical characteristics of this subset of patients and analysed their surgical outcomes and complications. RESULTS: Biventricular conversion was achieved in all cases except in 3 patients, who had the Glenn shunt retained leading to a one and a half ventricle repair. The average age of the patients was 4.9 years of whom 18 were male. The average cardiopulmonary bypass time was 371 min with an average cross clamp time of 162 min. There was one mortality in a patient with corrected transposition of great arteries (c-TGA) with extensive arterio-venous malformations (AVMs). At a median follow-up of 60 months, all patients remained symptom free except two with NYHA II symptoms, one being treated for branch pulmonary artery stenosis with balloon dilatation and the other with multiple AVMs who needed coil closure. One patient with branch pulmonary artery (PA) stenosis required balloon dilatation and stent placement. CONCLUSION: The possibility of achieving the surgical goal in this unique subset of patients evolves with the progressive experience of the congenital heart surgeon. Case selection is a crucial aspect in achieving the desired outcome, and this 'borderline' substrate is often recognized at the time of evaluation for the Fontan completion. A comprehensive preoperative imaging and planning helps in achieving the surgical septation and reconnection to achieve the desired physiological circulation. Though technically challenging, the surgery has excellent short- and mid-term outcomes as evidenced by our 10-year experience.

Catheter-based palliation for infants with tetralogy of Fallot.

BACKGROUND: The optimal management of symptomatic tetralogy of Fallot in neonates and younger infants with unfavourable anatomy is unclear and is further constrained by resource limitations in low and middle income countries. METHODS: Retrospective medical record review of infants with tetralogy of Fallot undergoing corrective or palliative procedures between January 2016 and June 2019. RESULTS: The study included 120 infants; of whom 83 underwent primary complete repair, four underwent surgical palliation, and 33 underwent catheter-based palliation, including balloon pulmonary valvuloplasty (n = 18), right ventricular outflow tract stenting (n = 14), and stenting of the patent arterial duct (n = 1). Infants undergoing catheter-based procedures were younger in age (median 32 days; inter-quartile range (IQR) 7-144 versus 210 days; IQR 158-250), with lower baseline saturation (65 ± 12% versus 87 ± 7%) and had smaller pulmonary artery z-scores compared to the complete repair cohort. Follow-up was available for 31/33 (94%) infants (median 7 months [IQR 4-11]) who underwent trans-catheter palliation; 12 underwent complete repair, 10 are well, awaiting repair, eight required further palliation (catheter: 6; surgical: 2), and one died post-discharge from non-cardiac causes. CONCLUSION: Catheter-based palliation is a safe and effective alternative in infants with tetralogy of Fallot who are at high risk for primary surgical repair.

Comparison of Fontan Surgical Options for Patients with Apicocaval Juxtaposition.

Apicocaval juxtaposition (ACJ) is a rare form of viscerocardiac malpositions in association with single-ventricle congenital heart defects. The Fontan surgery is the common palliation, and possible surgical options include ipsilateral, contralateral, and intra-atrial conduits. Concerns include lower hemodynamic performances or risks of conduit compression by the cardiac mass. This study investigates the hemodynamics and clinical outcomes of ACJ patients and potential surgical improvements. Ten consecutive ACJ patients were included, along with a reference cohort of ten non-ACJ patients. Magnetic resonance images were acquired at 6 ± 0.6 year follow-up for anatomical analysis and hemodynamic assessments using computational fluid dynamics. Metrics of interest are deformation index (DI), indexed power loss (iPL), and hepatic flow distribution (HFDoff). A "virtual" surgery was performed to explore potential hemodynamic improvements using a straightened conduit. DI for ACJ patients fell within the DI range of non-ACJ patients. Contralateral conduits had insignificantly higher iPL (0.070 [0.032,0.137]) than ipsilateral conduits (0.041 [0.013,0.095]) and non-ACJ conduits (0.034 [0.011,0.061]). HFDoff was similar for the ipsilateral (21 [12,35]), contralateral (26 [7,41]), and non-ACJ Fontan conduits (17 [0,48]). Virtual surgery demonstrated that a straightened conduit reduced HFDoff and iPL for the contralateral and ipsilateral conduits, potentially leading to improved clinical outcomes. In this limited sample, the hemodynamic performance of ACJ patients was not significantly different from their non-ACJ counterparts. The use of a straightened conduit option could potentially improve patient outcomes. Additionally, the fear of significant compression of conduits for ACJ patients was unsupported.

Accuracy of a New Echocardiographic Index to Predict Need for Trans-annular Patch in Tetralogy of Fallot.

The long-term outcome after repair of tetralogy of Fallot (TOF) is critically dependent pulmonary valve competence that is compromised by trans-annular patch (TAP). We compared a new echocardiographic index [pulmonary annulus index (PAI)] to conventional methods of predicting need for TAP in infants undergoing TOF repair. Consecutive infants undergoing TOF repair were prospectively studied. Pre-operative aortic and pulmonary annuli and main pulmonary artery (MPA) diameters were measured and z scores determined. PAI was a ratio of observed to expected pulmonary annulus (PA) diameter. TAP was based on intra-operative sizing by surgeons blinded to PAI values. Receiver operator curves (ROC) were generated for all PAI, MPA z scores and pulmonary annulus z scores. Of 84 infants (8.6 ± 2.6 months; 7.5 ± 1.3 kg), 36 needed TAP (43%). All the three indices viz. PAI, Pulmonary annulus and MPA z scores performed similarly in predicting need for TAP (ROC curves ~ 80%). Combining cut-offs of MPA z scores (> - 3.83) with either PAI (> 0.73) or PA z score (> - 1.83) predicted avoidance of TAP with ~ 90% accuracy. When both PAI and MPA z scores were below the cut-offs there was an 80% likelihood of TAP. Failure to predict TAP was associated with unicommisural pulmonary valves. PAI was equal to PA z scores in predicting need for TAP during repair of TOF. Combining either with MPA z scores was the most accurate method of prediction. Failure of prediction was mainly due to presence of a unicommissural pulmonary valve.

Pulmonary Hemorrhage Due to Unrecognized Bronchial Collateral After Arterial Switch Operation.

We report a neonate with transposition of great arteries and intact ventricular septum who had a massive pulmonary hemorrhage soon after an arterial switch operation. An emergency cardiac catheterization revealed a large bronchial collateral artery from the descending aorta feeding the right lung. The hemorrhage was controlled by coil embolization of the collateral, and the patient recovered after prolonged intensive care.

Three-dimensional-printed cardiac prototypes aid surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases: Early experience and proof of concept in a resource-limited environment.

INTRODUCTION: Three-dimensional. (3D) printing is an innovative manufacturing process that allows computer-assisted conversion of 3D imaging data into physical "printouts" Healthcare applications are currently in evolution. OBJECTIVE: The objective of this study was to explore the feasibility and impact of using patient-specific 3D-printed cardiac prototypes derived from high-resolution medical imaging data (cardiac magnetic resonance imaging/computed tomography [MRI/CT]) on surgical decision-making and preoperative planning in selected cases of complex congenital heart diseases (CHDs). MATERIALS AND METHODS: Five patients with complex CHD with previously unresolved management decisions were chosen. These included two patients with complex double-outlet right ventricle, two patients with criss-cross atrioventricular connections, and one patient with congenitally corrected transposition of great arteries with pulmonary atresia. Cardiac MRI was done for all patients, cardiac CT for one; specific surgical challenges were identified. Volumetric data were used to generate patient-specific 3D models. All cases were reviewed along with their 3D models, and the impact on surgical decision-making and preoperative planning was assessed. RESULTS: Accurate life-sized 3D cardiac prototypes were successfully created for all patients. The models enabled radically improved 3D understanding of anatomy, identification of specific technical challenges, and precise surgical planning. Augmentation of existing clinical and imaging data by 3D prototypes allowed successful execution of complex surgeries for all five patients, in accordance with the preoperative planning. CONCLUSIONS: 3D-printed cardiac prototypes can radically assist decision-making, planning, and safe execution of complex congenital heart surgery by improving understanding of 3D anatomy and allowing anticipation of technical challenges.

A life-threatening infective pseudoaneurysm of the left ventricle in a toddler.

Pseudoaneurysms of the ventricle are an uncommon occurrence in children. They may be secondary to previous cardiac surgery or infection of the pericardial space. Infective pseudoaneurysms require urgent surgery because rupture of the pseudoaneurysm may have catastrophic consequences. The outcome with surgery is excellent. We report a toddler with ruptured pseudoaneurysm secondary to purulent pericarditis who recovered with an emergency surgical closure of the opening of the pseudo-aneurysm.

Anomalous left coronary artery from nonfacing pulmonary sinus: direct aortic reimplantation.

We report a new technique of establishing a direct coronary transfer for anomalous left coronary artery arising from the nonfacing sinus of the pulmonary artery. This easily reproducible technique was successfully used in 2 patients. It achieves a dual coronary repair without the use of complex aortic or pulmonary arterial flaps and without causing any distortion to the great vessels.

Two-ventricle repair for complex congenital heart defects palliated towards single-ventricle repair.

OBJECTIVES: Complex congenital heart defects that present earlier in life are sometimes channelled towards single-ventricle repair, because of anatomical or logistic challenges involved in two-ventricle correction. Given the long-term functional and survival advantage, we have been consciously exploring the feasibility of a biventricular repair in these patients when they present later for Fontan completion. METHODS: Since June 2009, 71 patients were referred for staged completion of the Fontan procedure. Following detailed evaluation that included three-dimensional echocardiography and magnetic resonance imaging, 10 patients (Group 1-median age 6 years) were identified and later underwent complex biventricular repair with takedown of Glenn shunt, while completion of extracardiac Fontan repair was done in 61 patients (Group 2-median age 7 years). RESULTS: Two-ventricle repair was accomplished in all the 10 Group 1 patients. One patient developed complete heart block requiring permanent pacemaker insertion. Late patch dehiscence occurred in another (awaiting repair). At a median follow-up of 15 months, there was no mortality among the Group 1 patients and all except for 1 patient were symptom free. There were 2 early deaths (3.3%) in the Group 2 patients. CONCLUSIONS: Two-ventricular repair, although surgically challenging, should be considered in all patients with two functional ventricles who come for Fontan completion. Comprehensive preoperative imaging and meticulous planning helps in identifying suitable candidates.

Stage one Norwood procedure in an emerging economy:Initial experience in a single center.

OBJECTIVE: The evolution of surgical skills and advances in pediatric cardiac intensive care has resulted in Norwood procedure being increasingly performed in emerging economies. We reviewed the feasibility and logistics of performing stage one Norwood operation in a limited-resource environment based on a retrospective analysis of patients who underwent this procedure in our institution. METHODS: Retrospective review of medical records of seven neonates who underwent Norwood procedure at our institute from October 2010 to August 2012. RESULTS: The median age at surgery was 9 days (range 5-16 days). All cases were done under deep hypothermic cardiopulmonary bypass and selective antegrade cerebral perfusion. The median cardiopulmonary bypass (CPB) time was 240 min (range 193-439 min) and aortic cross-clamp time was 130 min (range 99-159 min). A modified Blalock-Taussig (BT) shunt was used to provide pulmonary blood flow in all cases. There were two deaths, one in the early postoperative period. The median duration of mechanical ventilation was 117 h (range 71-243 h) and the median intensive care unit (ICU) stay was 12 days (range 5-16 days). Median hospital stay was 30.5 days (range 10-36 days). Blood stream sepsis was reported in four patients. Two patients had preoperative sepsis. One patient required laparotomy for intestinal obstruction. CONCLUSIONS: Stage one Norwood is feasible in a limited-resource environment if supported by a dedicated postoperative intensive care and protocolized nursing management. Preoperative optimization and prevention of infections are major challenges in addition to preventing early circulatory collapse.

An unusual example of isolated double-orifice tricuspid valve.

Double-orifice tricuspid valve (DOTV) is a very rare congenital cardiac anomaly. We report an unusual example of DOTV where one orifice was committed to the left ventricle. This was identified preoperatively and managed surgically.

Surgical repair of double-chambered right ventricle in adulthood.

The aim of this study was to investigate a rare subset of adult patients with double-chambered right ventricle, pertaining to clinical presentation, rhythm disturbances, surgical approach, and results of surgical repair. A retrospective review of our records revealed that 14 adults had undergone surgical repair of double-chambered right ventricle from 1990 to 2010. Their median age was 25.5 years, and their median weight was 50 kg. Patients presented with various symptoms, the most common being dyspnea on exertion. The mean systolic pressure gradient across the obstruction was 69.8 ± 23.1mm Hg. The most common associated anomaly was ventricular septal defect. Significant rhythm disturbances were present in 4 patients. Transatrial repair was undertaken in 5 patients, a combined transatrial and transpulmonary approach was used in 4, and a right ventriculotomy in 5. There was no mortality at a median follow-up of 5.1 years. A mild right ventricular outflow tract gradient was noted in 2 patients. Medical management was required for rhythm disturbances in 2 patients. Double-chambered right ventricle can present in adulthood, often with vague symptoms. The midterm results of surgical correction are satisfactory. Long-term follow-up is necessary to detect late arrhythmias.

Anomalous origin of left coronary artery from pulmonary artery in older children and adults: direct aortic implantation.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presents rarely in adulthood and is treated by different surgical techniques with varying results. This study was undertaken to evaluate the feasibility of reestablishment of a dual coronary system in older children and adults with emphasis on direct aortic implantation. METHODS: From 2002 to 2010, 10 patients aged 10 to 51 years (median age, 24 years) underwent surgical correction of ALCAPA. Median weight was 44.5 kg. All the patients except 2 were symptomatic. Two patients were in atrial fibrillation. Eight patients had varying degrees of mitral regurgitation, which was severe in 2. In all patients, the left coronary artery was arising from the facing sinus of the pulmonary artery. Direct left main coronary implantation into the aorta was feasible in all patients. Mitral valve replacement was done in 4 patients. RESULTS: At a median follow-up of 5.5 years there was no new angina or infarction. One patient died 3 months postsurgery due to intracerebral bleed. Echocardiography demonstrated normal antegrade flow in the transferred left coronary artery, with 2 patients having persistent moderate mitral regurgitation. Coronary angiography done on follow-up in one patient revealed a patent transferred left coronary artery with distal development of new atherosclerotic lesion. CONCLUSIONS: Direct reimplantation of the ALCAPA is considered technically more difficult and hazardous in adults. With increased experience with coronary transfer technique, direct aortic implantation is feasible in adult cases. This provides a more physiologic correction and reestablishment of a dual coronary system with a better outcome.

Anomalous pulmonary venous connection to superior vena cava: Warden technique.

OBJECTIVE: Repair of anomalous pulmonary venous connection (APVC) to superior vena cava (SVC) with internal pericardial patch may be complicated by the obstruction of the SVC or pulmonary veins and sinus node dysfunction. Moreover, the shrinkage of the pericardium may lead to late obstruction. Various techniques are practiced, which have reduced the incidence of obstruction to the venous drainage, but sinus node dysfunction continues to be an issue. We reviewed our experience with the Warden technique, predominantly in children, for various types of APVC to the SVC. METHODS: Between 2006 and 2010, 32 patients with anomalous drainage of the pulmonary veins to SVC underwent repair by the Warden technique in our institute. The median age at operation was 4 years (range 3 months-34 years). Partial anomalous pulmonary venous connection (PAPVC) was present in 28 patients, while four patients had total anomalous pulmonary venous connection (TAPVC) to the SVC, where one or more pulmonary veins joined the SVC separately from the entry of the common chamber. Clinical data, echocardiography and operative details were collected from our database, retrospectively. RESULTS: At a median follow-up of 24 months, there was no mortality. One patient had transient rhythm disturbance. On follow-up, it was revealed that all patients are in sinus rhythm with no evidence of systemic or pulmonary venous obstruction. CONCLUSION: The Warden technique is a simple and effective surgical option, which should be preferred for patients with anomalous drainage of the pulmonary veins to SVC. Though arrhythmias are rare in the early follow-up, longer follow-up is required to rule out their late development.