Conflict of Interest Declaration
The ILCOR Continuous Evidence Evaluation process is guided by a rigorous ILCOR Conflict of Interest policy. The following Task Force members and other authors were recused from the discussion as they declared a conflict of interest: none applicable.
The following Task Force members and other authors declared an intellectual conflict of interest, and this was acknowledged and managed by the Task Force Chairs and Conflict of Interest committees: none applicable.
CoSTR Citation
Raymond TT, Guerguerian AM, Acworth J, Scholefield B, Atkins, DL - on behalf of the International Liaison Committee on Resuscitation Pediatric Life Support Task Force. IHCA due to Suspected Cardiac Shunt/Stent Obstruction Consensus on Science with Treatment Recommendations [Internet] Brussels, Belgium: International Liaison Committee on Resuscitation (ILCOR) Pediatric Life Support Task Force, 2025 January XX. Available from: http://ilcor.org
Methodological Preamble and Link to Published Systematic Review
The continuous evidence evaluation process for the production of Consensus on Science with Treatment Recommendations (CoSTR) started with a systematic review (Raymond, 2024, PROSPERO CRD42024545261) conducted by the members of the PLS TF with involvement of clinical content experts. Evidence for pediatric literature was updated and considered by the Pediatric Life Support Task Force with assistance from Mary-Doug Wright (MDW), Information Specialist, ILCOR. Evidence was sought and considered by members of the Pediatric Life Support Task Force group when formulating the Treatment Recommendations.
This is a new PICOST (4030.25). Aortopulmonary shunts and/or patent ductus arteriosus stents are important tools for the palliation of patients with congenital heart disease. They are mainly used to augment or maintain a stable source of pulmonary blood flow as part of the palliation strategy in patients with single-ventricle physiology or to postpone a definitive biventricular repair in patients with comorbidities or those in which further somatic growth would allow for a better repair. PDA stents are selectively used as part of a hybrid palliation in patients with hypoplastic left heart syndrome. Systemic-to-pulmonary artery shunt or stent occlusion is one of the most important complications requiring urgent therapy in children with cardiac disease. It can develop gradually, or suddenly, thus resulting in a gradual or dramatic restriction of pulmonary artery flow, severe hypoxia,
cyanosis, and acidosis, leading to cardiac arrest. Current therapies of acute shunt obstruction can include the following: (1) administration of increased inspired oxygen to maximize alveolar oxygenation; (2) administration of vasoactive agents to maximize shunt perfusion pressure (e.g., phenylephrine, norepinephrine, epinephrine); (3) anticoagulation with heparin (50–100 U/kg bolus) to prevent clot propagation; (4) shunt intervention by catheterization or surgery; (5) stabilization with ECPR/ECMO, and/or (6) sternal opening in the post operative period that may relieve shunt compression. If the shunt obstruction produces persistent and profound arterial hypoxemia, myocardial performance will deteriorate rapidly.2,3,5-8
The only current ILCOR recommendations11 regarding cardiac arrest in the pediatric cardiac population involve the use of ECPR:1,9,10 “We suggest that ECPR may be considered as an intervention for selected infants and children (e.g., pediatric cardiac populations) with IHCA refractory to conventional CPR, in settings where resuscitation systems allow ECPR to be well performed and implemented (weak recommendation, very low-quality evidence). During cardiopulmonary arrest, it is reasonable to consider ECPR for patients with Fontan physiology. There is insufficient evidence to support or refute the use of ECPR in patients with hemi-Fontan or BDG physiology.”
This systematic review will further define what specific interventions other than standard cardiopulmonary resuscitation may improve clinical outcomes in pediatric in-hospital cardiac arrest due to suspected aortopulmonary shunt/stent obstruction in infants and children with cardiac disease as there are no specific recommendations regarding cardiac arrest in this population.
Systematic Review
No systematic review planned given the lack of data obtained from the search of the literature.
PICOST
PICOST |
Description (with recommended text) |
Population |
Among infants and children in cardiac arrest in the in-hospital setting who have suspected aortopulmonary shunt/stent obstruction |
Intervention |
does any intervention [administration of oxygen, vasoactive agents to increase shunt/stent perfusion pressure, extracorporeal cardiopulmonary resuscitation (ECPR), heparin, sternal opening, catheter-based intervention, surgical intervention] or a combination of these interventions |
Comparison |
compared to standard resuscitation |
Outcomes |
Any clinical outcomes, including (not exclusive) - ROSC - ROC with extracorporeal membrane oxygenation (ECMO) - survival to hospital discharge - survival with favorable neurological outcome as per Pediatric Core Outcome Set for Cardiac Arrest1 |
Study Design |
Randomized controlled trials (RCTs) and non-randomized studies (non-randomized controlled trials, interrupted time series, controlled before-and-after studies, cohort studies) that directly concern the population and intervention described above are eligible for inclusion. If it is anticipated that there will be insufficient studies from which to draw a conclusion, case series may be included in the initial search. The minimum number of cases for a case series to be included was set by the taskforce at 5. Unpublished studies (e.g., conference abstracts, trial protocols) are excluded. All relevant publications in any language are included as long as there is an English abstract. |
Timeframe |
All years. |
PROSPERO Registration CRD42024545261
Consensus on Science
No studies compared any intervention (administration of oxygen, vasoactive agents to increase shunt/stent perfusion pressure, ECPR, heparin, sternal opening, catheter-based intervention, surgical intervention] or a combination of these interventions to standard resuscitation for infants and children with IHCA due to suspected shunt/stent obstruction.
The search strategy was developed by a professional medical librarian (MDW) trained in knowledge synthesis provided by ILCOR. Databases searched were Embase 1974 to 2024 June 05; MEDLINE(R) ALL 1946 to 1974 to June 05, 2024 (multi-database search via Ovid); and Cochrane Central Register of Controlled Trials (Cochrane Library via Wiley Online). Final database searches were conducted June 6, 2024. Screening resulted in 721 articles, title and abstract screening resulted in 15 articles being screened by full text with zero final articles obtained.
Treatment Recommendations
There is insufficient evidence to make a specific treatment recommendation for infants and children in cardiac arrest in the in-hospital setting who have suspected aortopulmonary shunt/stent obstruction other than standard resuscitation.
Justification and Evidence to Decision Framework Highlights
A systematic review revealed no studies directly comparing interventions such as oxygen, ECPR, administration of vasoactive agents, heparin, or catheter-based interventions with standard resuscitation in this patient population with suspected shunt/stent obstruction in cardiac arrest. As such, the PLS task force can make no treatment recommendations other than following standard resuscitation recommendations.
Link to ETD summary table: None
Knowledge Gaps
- Limited Direct Evidence: There is an absence of randomized controlled trials (RCTs) or comparative studies focused on interventions for IHCA due to aortopulmonary shunt or stent obstruction, making it difficult to establish evidence-based treatment protocols and the ability to recommend one intervention over another with confidence.
- Outcome Data for Specific Interventions: More data are needed regarding the effectiveness of individual interventions (e.g., vasoactive agents, heparin) or their combinations in improving outcomes like return of spontaneous circulation (ROSC), survival to hospital discharge, and neurological outcomes.
- ECPR Effectiveness in Specific Physiology: The evidence for using ECPR in patients with specific cardiac anatomies, like those with single ventricle physiology status post shunt or stent, is currently insufficient. Further research is required to determine its effectiveness and potential risks in these subgroups.
- Outcome Data for Neurological Recovery: Data on survival rates and neurological outcomes following cardiac arrest due to shunt obstruction in pediatric patients is scarce. Understanding the long-term impacts of different interventions on neurological health is crucial for making evidence-based decisions.
- Optimal Management Strategies: The ideal timing and combination of therapies (e.g., vasoactive agents, anticoagulation, surgical intervention) remain undefined, with no clear consensus on a standardized approach. Research to identify optimal management protocols could significantly improve outcomes for this vulnerable patient group.
References
1. Alsoufi B, Awan A, Manlhiot C, Guechef A, Al-Halees Z, Al-Ahmadi M, McCrindle BW, Kalloghlian A. Results of rapid-response extracorporeal cardiopulmonary resuscitation in children with refractory cardiac arrest following cardiac surgery. Eur J Cardiothorac Surg. 2014;45(2):268-75.
2. Bonnet M, Petit J, Lambert V, Brenot P, Riou JY, Angel CY, Belli E, Baruteau AE. Catheter-based interventions for modified Blalock-Taussig shunt obstruction: a 20-year experience. Pediatr Cardiol. 2015;36(4):835-41. doi: 10.1007/s00246-014-1086-0.
3. Guzzetta NA, Foster GS, Mruthinti N, Kilgore PD, Miller BE, Kanter KR. In-hospital shunt occlusion in infants undergoing a modified blalock-taussig shunt. Ann Thorac Surg. 2013;96(1):176-82. doi: 10.1016/j.athoracsur.2013.03.026.
4. Jolley M, Thiagarajan RR, Barrett CS, Salvin JW, Cooper DS, Rycus PT, Teele SA. Extracorporeal membrane oxygenation in patients undergoing superior cavopulmonary anastomosis. J Thorac Cardiovasc Surg. 2014; 148:1512–1518.
5. Krasemann T, Tzifa A, Rosenthal E, Qureshi SA. Stenting of modified and classical Blalock–Taussig shunts – lessons learned from seven consecutive cases. Cardiology in the Young. 2011;21(4):430-435.
6. MacMillan M, Jones TK, Lupinetti FM, et al. Balloon angioplasty for Blalock–Taussig shunt failure in early postoperative period. Catheter CardiovascInterv.2005;66:585–589.
7. Marino BS, Tabbutt S, MacLaren G, Hazinski MF, Adatia I, Atkins DL, Checchia PA, DeCaen A, Fink EL, Hoffman GM, Jefferies JL, Kleinman M, Krawczeski CD, Licht DJ, Macrae D, Ravishankar C, Samson RA, Thiagarajan RR, Toms R, Tweddell J, Laussen PC; American Heart Association Congenital Cardiac Defects Committee of the Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Emergency Cardiovascular Care Committee. Cardiopulmonary resuscitation in infants and children with cardiac disease: a scientific statement from the American Heart Association. Circulation. 2018;137:e691–e782.
8. Moszura T, Ostrowska K, Dryzek P, et al. Thrombolysis and stent implantation in a child with an acute occlusion of the modified Blalock–Taussig shunt—a case report. Kardiol Pol. 2004;60:354–356.
9. Polimenakos AC, Wojtyla P, Smith PJ, Rizzo V, Nater M, El Zein CF, Ilbawi MN. Post-cardiotomy extracorporeal cardiopulmonary resuscitation in neonates with complex single ventricle: analysis of outcomes. Eur J Cardiothorac Surg. 2011;40(6):1396-405; discussion 1405.
10. Polimenakos AC, Rizzo V, El-Zein CF, Ilbawi MN. Post-cardiotomy Rescue Extracorporeal Cardiopulmonary Resuscitation in Neonates with Single Ventricle After Intractable Cardiac Arrest: Attrition After Hospital Discharge and Predictors of Outcome. Pediatr Cardiol. 2017;38(2):314-323.
11. Topjian AA, Scholefield BR, Pinto NP, Fink EL, Buysse CMP, Haywood K, Maconochie I, Nadkarni VM, de Caen A, Escalante-Kanashiro R, Ng KC, Nuthall G, Reis AG, Van de Voorde P, Suskauer SJ, Schexnayder SM, Hazinski MF, Slomine BS. P-COSCA (Pediatric Core Outcome Set for Cardiac Arrest) in Children: An Advisory Statement From the International Liaison Committee on Resuscitation. Circulation. 2020 Oct 20;142(16):e246-e261.