Harlequin effect and central veno-arterial extracorporeal life support.

"Harlequin effect" may be observed in the watershed region of a patient with pulmonary dysfunction, receiving peripheral veno-arterial extracorporeal membrane oxygenation via the femoral vessels. In such cases, retrograde oxygenated blood from the peripheral inflow cannula converges with the antegrade deoxygenated blood ejected from the left ventricle. This occurs when the left ventricle is ejecting significantly but the recovery of pulmonary function lags behind. Herein, we describe the occurrence of "Harlequin effect" in the setting of central veno-arterial extracorporeal membrane oxygenation that ensues due to the persistence of right ventricular dysfunction in the presence of an interatrial communication. This results in right to left shunting at the atrial level while weaning the patient from extracorporeal life support.

Tetralogy of Fallot with pulmonary atresia and aortopulmonary window may mimic common arterial trunk.

Tetralogy of Fallot with pulmonary atresia is a group of congenital cardiac malformations, which is defined by the absence of luminal continuity between both ventricles and the pulmonary artery, and an interventricular communication. Pulmonary arterial supply in patients with tetralogy of Fallot with pulmonary atresia can be via the arterial duct or from collateral arteries arising directly or indirectly from the aorta (systemic-to-pulmonary artery collaterals), or rarely both. The rarest sources of pulmonary blood flow are aortopulmonary window and fistulous communication with the coronary artery.Herein, we describe an outflow tract malformation, tetralogy of Fallot with pulmonary atresia and aortopulmonary window, which was misdiagnosed as common arterial trunk. We emphasise the morphological differences.

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.

The aortic arch in tetralogy of Fallot: types of branching and clinical implications.

INTRODUCTION: Tetralogy of Fallot is the most common form of cyanotic CHD, with an incidence of 421 cases per million live births, constituting around 10% of CHD. Variations in aortic arch anatomy associated with tetralogy of Fallot, other than the incidence of right aortic arch (13-34%), are not frequently reported. A comprehensive analysis of a large number of tetralogy of Fallot cases is required to arrive at a compendious description of aortic arch anatomy. MATERIALS AND METHODS: All patients with a diagnosis of tetralogy of Fallot who had CT or MRI either pre or post procedures between 1 January 2010 and 31 December 2019 at our hospital were included in the study. Using radiology consultants' reports and analysis of individual images, we identified the various morphological patterns of aortic arches prevalent in these patients. RESULT: Out of 2684 patients who met the inclusion criteria, 1983 patients had left aortic arch (73.9%), 688 patients had right aortic arch (25.7%), four patients had cervical aortic arch (0.15%), eight patients had double aortic arch (0.3%), one patient had an aorto-pulmonary window (0.04%), and none of the patients had interrupted aortic arch. Sub-classification and clinical implications of the arch variations are described. CONCLUSION: Up to 10% of tetralogy of Fallot patients may have significant anatomical variations of aortic arch that would necessitate changes or additional steps in their surgical or interventional procedures.

Anatomic classification of the right aortic arch.

The term right aortic arch is used for an aorta that arches over the right bronchus. Right aortic arch was classified into two types by Felson et al, based on branching patterns, with a proposed embryological explanation, and into three types by Shuford et al. Other anatomical variants of right aortic arch were described later, including isolated left brachiocephalic artery and aberrant left brachiocephalic artery. We have classified right aortic arch anatomy into 10 variants, supported by radiological evidence, and with reference to possible embryology. This classification will help in understanding the morphological basis for the formation of different types of right aortic arch and the course of the recurrent laryngeal nerve in such cases.

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.

Right ventricular outflow tract histology post-stenting and in-stent stenosis.

The right ventricular outflow tract (RVOT) stenting is used for the treatment of Fallot-type lesions when not amenable to complete correction or when surgical palliation carries a substantial risk. Despite the increasing clinical application, there is a lack of data that describe the RVOT morphology after stenting. This article elucidates post-RVOT stenting and in-stent stenosis, which is thought to be a zonal phenomenon, in this case, predominantly occurring proximally, in the portion of the stent apposing the RVOT infundibulum.

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.

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.

Abnormally proximal aortic origin of the brachiocephalic artery: Surgical implications.

Abnormal proximal aortic origin of the brachiocephalic artery is a very rare condition. It can occur in isolation or associated with complex congenital heart disease affecting the right ventricular outflow tract. Its recognition carries relevant surgical implications for the safe conduct of cardiopulmonary bypass and for any surgical procedures that directly involve the proximal ascending aorta and its branches.

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.

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.

Splenic mass and isolated gastric varices: a rare presentation of a neuroendocrine tumor of the pancreas.

CONTEXT: Splenic involvement in neuroendocrine pancreatic tumors is well known but rarely presents as a primary splenic mass. CASE REPORT: A rare case of a neuroendocrine tumor involving the tail of the pancreas, splenic hilum and splenic flexure of the colon, forming a conglomerate mass and presenting as isolated gastric varices is described. A 75-year-old male presented with hematemesis and melena. Esophagogastroduodenoscopy revealed isolated gastric varices. A CT scan revealed a mass predominantly involving the spleen and a small part of the pancreas. CONCLUSION: A splenic mass with isolated gastric varices should be kept in mind as one of the presentations of a pancreatic neuroendocrine tumor.

Choledocholithiasis: endotherapy versus surgery.

When multiple treatment options are available, debate invariably persists regarding the optimal option. Confusion and controversy must then be resolved based on scientific evidence, but one needs to be practical because options depend on the available expertise. We aimed to evaluate the efficacy of endotherapy vis-à-vis surgery in patients with choledocholithiasis. The records of 349 patients with stone disease from February 2005 to January 2010 were analyzed. A total of 349 patients were analyzed: 279 patients with gallstones alone, 56 with choledocholithiasis, 3 with stones with stricture, 5 with common bile duct (CBD) and pancreatic duct (PD) stones, and 6 with combined choledocholithiasis and hepatolithiasis. In the 56 patients with choledocholithiasis alone, preoperative endoscopic retrograde cholangio pancreatography (ERCP) and endotherapy were followed by cholecystectomy within 48 hours. Endotherapy was successful in 15 patients, whereas surgery was required in the remaining 41 patients. Surgery is an efficacious option and can be carried out safely with acceptable morbidity and no mortality, even in difficult situations.

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.

Hybrid approach for aortic embolization of Amplatzer duct occluder.

Embolization of the patent ductus arteriosus (PDA) device is a known adverse event of percutaneous PDA closure, which can lead to complications. Embolization can occur into the pulmonary artery or into the aorta. Device embolization can be moderate adverse event (when retrieved percutaneously) or major adverse event (when retrieved surgically). We are describing a hybrid approach for aortic embolization of PDA device when the percutaneous retrieval fails, where device retrieval and PDA ligation can be done through thoracotomy incision, thus decreasing the complications.

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.