Iatrogenic Anomalous Origin of the Right Pulmonary Artery From the Aorta: A Rare Complication of Ligation of Aortopulmonary Window.

An aortopulmonary window (APW) is a communication between the main pulmonary artery (MPA) and the ascending aorta in the presence of two separate semilunar valves. Surgical strategies described for the management of APW include ligation, division and suturing, patch closure of APW (transaortic or transpulmonary or transwindow), and device closure. Ligation of a type-2 APW can result in stenosis or complete closure of the right pulmonary artery (RPA) off the MPA, while leaving the aorta still opening into the RPA. We describe a delayed presentation of iatrogenic anomalous origin of the RPA from the aorta, post APW ligation, and its management.

A Valved Expanded Polytetrafluoroethylene Conduit can Acquire Endothelium After Implantation.

We demonstrate histopathology, neointimal proliferation, and neo-endothelialization in an explanted valved expanded polytetrafluoroethylene (ePTFE) conduit 40 months postimplantation that was void of calcification and inflammation, confirmed by CD-31 positivity on immunohistochemistry. Grossly, there was no distortion with preserved leaflets and lack of calcification. Good biocompatibility, nonreactivity, and low antigenicity, combined with neointimal and endothelial layer generation within the conduit might explain the low infection rates and minimal thrombogenicity. These findings support the use of handmade, valved ePTFE conduits as an economically viable option as a right ventricle to pulmonary artery conduit.

The Role of Shunt Occlusion During Extracorporeal Life Support.

INTRODUCTION: The current recommendation for systemic to pulmonary artery shunt (SPS) patients requiring extracorporeal life support (ECLS) is to keep the shunt open, maintaining a higher pump flow. The practice in our center is to totally occlude the shunt while on ECLS, and we are presenting the outcome of this strategy. METHODS: This is a retrospective analysis of patients who underwent SPS for cyanotic congenital heart disease with decreased pulmonary blood flow and required postoperative ECLS between January 2016 and December 2020. ECLS indication was excessive pulmonary blood flow, leading to either refractory low cardiac output syndrome (LCOS) or cardiac arrest. All patients had their shunts totally occluded soon after ECLS establishment. RESULTS: Of the 27 SPS patients who needed postoperative ECLS (13 refractory LCOS, 14 extracorporeal cardiopulmonary resuscitation), wherein the strategy of occluding the shunt on ECLS initiation was followed, 16 (59.3 %) survived ECLS weaning and eight (29.6%) survived to discharge. CONCLUSION: Increased flow to maintain systemic circulation for a SPS patient while on ECLS is an accepted strategy, but it should not be applied universally. A large subset of SPS patients, who require ECLS either due to cardiac arrest or refractory LCOS due to excessive pulmonary flow, might benefit from complete occlusion of the shunt soon after commencement of ECLS, especially in cases with frank pulmonary edema or haemorrhage in the pre-ECLS period. A prospective randomized trial could be ethically justified for the subset of patients receiving ECLS for the indication of excessive pulmonary blood flow.

The Role of Shunt Occlusion During Extracorporeal Life Support

ABSTRACT Introduction: The current recommendation for systemic to pulmonary artery shunt (SPS) patients requiring extracorporeal life support (ECLS) is to keep the shunt open, maintaining a higher pump flow. The practice in our center is to totally occlude the shunt while on ECLS, and we are presenting the outcome of this strategy. Methods: This is a retrospective analysis of patients who underwent SPS for cyanotic congenital heart disease with decreased pulmonary blood flow and required postoperative ECLS between January 2016 and December 2020. ECLS indication was excessive pulmonary blood flow, leading to either refractory low cardiac output syndrome (LCOS) or cardiac arrest. All patients had their shunts totally occluded soon after ECLS establishment. Results: Of the 27 SPS patients who needed postoperative ECLS (13 refractory LCOS, 14 extracorporeal cardiopulmonary resuscitation), wherein the strategy of occluding the shunt on ECLS initiation was followed, 16 (59.3 %) survived ECLS weaning and eight (29.6%) survived to discharge. Conclusion: Increased flow to maintain systemic circulation for a SPS patient while on ECLS is an accepted strategy, but it should not be applied universally. A large subset of SPS patients, who require ECLS either due to cardiac arrest or refractory LCOS due to excessive pulmonary flow, might benefit from complete occlusion of the shunt soon after commencement of ECLS, especially in cases with frank pulmonary edema or haemorrhage in the pre-ECLS period. A prospective randomized trial could be ethically justified for the subset of patients receiving ECLS for the indication of excessive pulmonary blood flow.

Completion of an interventional extracardiac Fontan.

The extracardiac Fontan can be completed via transcatheter perforation of the pericardial membrane created during the preparatory stage, thus establishing continuity between the inferior vena cava and the pulmonary artery. This step is followed by deployment of a covered stent to isolate the systemic and pulmonary circuits. The procedure avoids the morbidity associated with cardiac reoperation and is a safe option for patients who present late with prohibitive pulmonary artery pressures in whom primary Fontan completion may not be feasible.

Preparatory stage of interventional extracardiac Fontan.

The extracardiac Fontan can be completed as an interventional procedure when the preparatory stage for Fontan completion is performed at the time of the bidirectional Glenn operation. In this video tutorial, we present the technique for the preparatory stage of an interventional extracardiac Fontan. The interventional aspect offers the advantage of avoiding a redo sternotomy, which involves the risk of cardiac injury, injury to the mediastinal collaterals due to the single-ventricle physiology, the acquired deranged coagulation that may incur morbidity associated with sternal re-entry, and the problems pertinent to cardiopulmonary bypass and/or cardioplegic arrest in this subset of patients.

Coronavirus disease 2019 convalescent children: outcomes after congenital heart surgery.

BACKGROUND: Children with exposure to coronavirus disease 2019 in recent times (asymptomatic or symptomatic infection) approaching congenital heart surgery programme are in increasing numbers. Understanding outcomes of such children will help risk-stratify and guide optimisation prior to congenital heart surgery. OBJECTIVE: The objective of the present study was to determine whether convalescent coronavirus disease 2019 children undergoing congenital heart surgery have any worse mortality or post-operative outcomes. DESIGN: Consecutive children undergoing congenital heart surgery from Oct 2020 to May 2021 were enrolled after testing for reverse transcription-polymerase chain reaction or rapid antigen test and immunoglobulin G antibody prior to surgery. Convalescent coronavirus disease 2019 was defined in any asymptomatic patient positive for immunoglobulin G antibodies and negative for reverse transcription-polymerase chain reaction or rapid antigen test anytime 6 weeks prior to surgery. Control patients were negative for any of the three tests. Mortality and post-operative outcomes were compared among the groups. RESULTS: One thousand one hundred and twenty-nine consecutive congenital heart surgeries were stratified as convalescence and control. Coronavirus disease 2019 Convalescent (n = 349) and coronavirus disease 2019 control (n = 780) groups were comparable for all demographic and clinical factors except younger and smaller kids in control. Convalescent children had no higher mortality, ventilation duration, ICU and hospital stay, no higher support with extracorporeal membrane oxygenation, high flow nasal cannula, no higher need for re-intubations, re-admissions, and no higher infections as central line-associated bloodstream infection, sternal site infection, and ventilator-associated pneumonia on comparison with coronavirus disease 2019 control children. CONCLUSIONS: Convalescent coronavirus disease 2019 does not have any unfavourable outcomes as compared to coronavirus disease 2019 control children. Positive immunoglobulin G antibody screening prior to surgery is suggestive of convalescence and supports comparable outcomes on par with control peers.

Branch Pulmonary Artery Jailing During Patent Ductus Arteriosus Stenting: Recruitment and Immediate Outcomes.

BACKGROUND: Branch pulmonary artery (PA) occlusion during patent ductus arteriosus (PDA) stenting procedure is the main reason why branch PA origin stenosis was considered as a contraindication for PDA stenting. This study was designed to assess the incidence of branch PA jailing during PDA stenting for cyanotics with duct-dependent pulmonary circulation and its immediate outcome. METHODS: All the completed PDA stenting patients in our hospital between April 2017 and June 2019 were retrospectively analyzed for branch PA jailing and its outcome. RESULTS: Of 63 completed PDA stenting, there was branch PA jailing in 13 (20.6%) patients, all successfully recruited either by strut dilatation or by surgery. The median duration of ventilation was 16 (interquartile range [IQR]: 8-22) hours for jailed patients and 17.5 (IQR: 5.25-34.25) hours for nonjailed patients (P = .978). Median intensive care unit [ICU] stay was 69 (IQR: 47.75-96) hours for jailed patients and 79.5 (IQR: 66.75-135.25) hours for nonjailed patients (P = .394). Procedural mortality was 1 (7.6%) for jailed patients and 3 (6%) for nonjailed patients. Since all the jailed pulmonary arteries were recruited, there was proportionate growth of branch PA till the most recent follow-up. CONCLUSION: Jailing of branch PA does not increase the ventilation duration, ICU stay, or mortality risk if recruited immediately. Proportionate growth of branch PAs can be achieved in spite of jailing, if addressed aggressively. Branch PA stenosis should not be considered as a contraindication for PDA stenting.

Staged Hybrid Approach for Ductal Origin of the Pulmonary Artery in a Low Birth Weight Neonate.

Early recruitment of a discontinuous branch pulmonary artery will give the best long-term outcome for congenitally disconnected pulmonary arteries. This may be achieved using a staged approach. Even in low birth weight neonates, a hybrid approach with the first stage consisting of transcervical (via the common carotid artery) patent ductus arteriosus stenting can be performed to recruit the pulmonary artery where conventional treatment strategies may not be feasible or may be associated with higher risk.

Hybrid Approach for Embolized Right Ventricular Outflow Tract Stent.

Displacement and embolization of the stent is one of the major complications of right ventricular outflow tract (RVOT) stenting. Since embolized stents cannot be retrieved percutaneously as they cannot be crimped into pre-stenting state (unlike duct occluders and septal devices, which can be pulled back into the sheath), surgery remains the gold standard treatment. We describe a hybrid approach for the retrieval of the embolized RVOT stent, which will decrease morbidity when compared to the standard surgical approach that is otherwise required.

Improved Technique for Interventional Extracardiac Fontan.

We have previously reported a simple technique for preparatory staging and subsequent interventional completion of an extracardiac Fontan procedure that anatomically and hemodynamically closely mimics a standard extracardiac Fontan. We describe herein modifications that simplify the original procedural sequence and that may allow wider application. Percutaneous completion can be achieved even without a radiofrequency probe, using electrocautery. Fenestration is also easy to perform if there is a clinical indication.

Late Complication of Amplatzer Duct Occluder: Erosion and Pseudoaneurysm.

We are presenting a delayed complication of an Amplatzer duct occluder (ADO) in which a patient presented with increasing hemoptysis owing to ADO eroding the vessel wall and forming a pseudoaneurysm that communicated with the left main bronchus. Although ADO is considered as the safest for catheter-based patent ductus arteriosus closures, isolated complication as reported can occur. Just like atrial septal defect device closures, which were considered safe initially and found to be having an erosion incidence of 0.1% to 0.3%, this case could represent an isolated event (the index case) or a long-term complication occurring at a very low incidence.

Handmade trileaflet valved ePTFE right ventricle to pulmonary artery conduit - How do we do it?

Right ventricle to pulmonary artery conduits such as homografts and pre-manufactured synthetic conduits are widely employed in the present era of complex congenital cardiac surgeries for disorders involving right ventricle - pulmonary artery discontinuity and major coronary artery crossing the right ventricular outflow tract. The key drawback of homograft conduits is the need for reoperation to replace them as a result of degeneration over time or in cases where a child has outgrown the conduit and cost is a major drawback to using commercially available conduits. The advantages of expanded polytetrafluoroethylene are its long-term durability in terms of conduit calcification/degeneration and valve stenosis/regurgitation and its cost-effectiveness.  In this video presentation, we demonstrate the preparation and intraoperative usage of a handmade, trileaflet, valved polytetrafluoroethylene conduit in a case of double outlet right ventricle with valvular and subvalvular pulmonary stenosis and the left anterior descending artery crossing the right ventricular outflow tract. Our experience with this handmade conduit is close to 120 cases and we have seen excellent postoperative recovery and results. Our medium-term follow-up echocardiographic evidence shows conduit patency and preserved valve function.

Durability of tissue-engineered bovine pericardium (CardioCel®) for a minimum of 24 months when used for the repair of congenital heart defects.

OBJECTIVES: This study aims to assess the performance of tissue-engineered bovine pericardium (CardioCel®) at 24 months and beyond when used for the repair of congenital heart defects. METHODS: Between October 2012 and November 2014, CardioCel was implanted in 135 patients (140 procedures and 195 implants). Applications included the closure of septal defects (98, 50.3%), repair of pulmonary arteries (63, 32.3%), intra-atrial/intraventricular baffles (10, 5.1%), repair of systemic arteries (15, 7.7%), valve repair (5, 2.6%), repair of systemic veins (3, 1.5%) and Fontan operation (1, 0.5%). There were 19 (13.6%) procedures performed in neonates, 77 (55%) in infants and 44 (31.4%) in children older than 365 days. RESULTS: Eight patients (n = 135, 5.9%) required reintervention in 12 instances (n = 195, 6.2%, 6 catheters and 6 surgical). There was no echocardiographic or radiological evidence of calcification in any patient. Ten of the reinterventions (83%) occurred within the first 12 months. All the reinterventions occurred within 36 months. Freedom from reintervention at both 12 and 24 months was 95% [confidence interval (CI) 91-97] and at 36 months was 94% (CI 89-97). There was no statistical difference in freedom from reintervention when stratified by age or patch position. CONCLUSIONS: At 24 months and beyond the follow-up, the performance of CardioCel remains acceptable with good haemodynamic performance. There was no echocardiographic or radiological evidence of calcification in any patient. CardioCel performs comparably in systemic and pulmonary circulations.

Long-term performance of homografts versus stented bioprosthetic valves in the pulmonary position in patients aged 10-20 years.

OBJECTIVES: We aimed to compare the long-term performance of pulmonary homografts and stented bioprosthetic valves in the pulmonary position in patients aged 10-20 years. METHODS: Between January 1995 and December 2015, 188 patients aged 10-20 years undergoing pulmonary valve replacement were identified retrospectively from hospital databases in both congenital cardiac centres in Brisbane. Valve performance was evaluated using previously described standard criteria. Propensity score matching was used to balance the 2 treatment groups. RESULTS: Freedom from structural valve degeneration in homografts (n = 131) was 97%, 92% and 85% at 3, 5 and 10 years, respectively, and 91% and 53% at 3 and 5 years, respectively, in the bioprosthesis group (n = 57). Freedom from reintervention in homografts was 96%, 93% and 88% at 3, 5 and 10 years, respectively, and 93% and 68% at 3 and 5 years, respectively, in the bioprosthesis group. The unadjusted Cox regression analysis demonstrated that a bioprosthesis was at 5.64 times the risk of structural valve degeneration and 3.89 times the risk of reintervention. The Cox regression analysis performed on the propensity matched sample (45 pairs of patients) revealed that a bioprosthesis was at almost 10 times the risk of experiencing structural valve degeneration [hazard ratio (HR) = 9.18] and at more than 8 times the risk of undergoing a reintervention (HR = 8.34). CONCLUSIONS: In our patient population, pulmonary homografts outperformed stented bioprosthetic valves within 5 years when implanted in the pulmonary position in patients aged 10-20 years. We recommend the use of a pulmonary homograft for pulmonary valve replacement in this age group in patients undergoing surgery for congenital heart disease.

Histologic Evaluation of Explanted Tissue-Engineered Bovine Pericardium (CardioCel).

CardioCel is a bovine pericardium that is subjected to a novel anticalcification tissue-engineering process. We present the histopathologic findings of human explants of CardioCel that were used in operations for congenital heart disease in children. Six explants were identified from 140 patients undergoing CardioCel implants from October 2012 to March 2015. CardioCel explants were evaluated histologically using hematoxylin and eosin, Masson trichrome, and immunohistochemical staining. A variable inflammatory response was seen in the surrounding native tissue, but not within the CardioCel graft in any of the explants. A neointimal layer of varying thickness developed on the visceral surface of 5 CardioCel explants with endothelialization of the longest duration explant. A granulation tissue layer developed on the parietal surface of the graft (consistently thicker than the neointima). Maintained collagen fiber architecture (laminated) and variable fibroblastic invasion (which increased with the age of the implant) were identified in all 6 cases. Scattered capillary vessels were noted in the majority of the explants with new collagen fibers in one, suggesting early remodeling. Calcium was seen in 1 explant at the interface of the graft and inflammatory response on its parietal surface. Evidence of graft remodeling was noted in the majority of the explants without inflammatory cells or calcification within the explanted graft material. A noticeable feature was the differential thickness of the host reaction to the parietal compared with the visceral surface of the graft. We will continue to evaluate CardioCel as a cardiovascular substitute for extracardiac and intracardiac reconstructions.

A systematic surgical approach to hepatoblastoma with intracardiac extension.

Hepatoblastoma is very uncommon in children, and intracardiac extension is rare. The SIOPEL-4 regime for metastatic hepatoblastoma has extended the surgical options with good results. We successfully treated a boy aged 2 years and 9 months with hepatoblastoma, using a multidisciplinary surgical strategy involving an extended left hepatectomy, left adrenalectomy, biopsy of the paraaortic and coeliac lymph nodes, and resection of the inferior vena caval-right atrial extension of the hepatoblastoma, under cardiopulmonary bypass and deep hypothermia.