What are the guideline sources, guidelines and references for Hypoplastic left heart syndrome ?
Guideline sources The following summarized guidelines for the evaluation and management of hypoplastic left heart syndrome (HLHS) are prepared by our editorial team based on guidelines from the Association for European Paediatric and Congenital Cardiology (AEPC/EACTS 2020) and the American Heart Association (AHA 2018). 1 2 Calculator NYHA functional classification f Guidelines 1. Screening and diagnosis https://web.pathway.md/diseases/rec9a3ruUUM6kGOlZ 1/5 6/24/23, 12:47 PM Hypoplastic left heart syndrome Pathway Prenatal diagnosis: obtain an obstetrical fetal anomaly scan at 18-22 weeks of gestation. Include at minimum a four-chamber view, outflow tract views, and three-vessel tracheal view in the cardiac imaging during obstetrical fetal anomaly scan. B Show 4 more Postnatal diagnosis: Obtain TTE to rule out congenital heart disease, including HLHS, in undiagnosed neonates with persistent tachypnea or desaturation. B Assess global cardiac function, structures by four- chamber view, outflow tract views, aortic arch, coeliac trunk, and cerebral blood flow. B Confirm the diagnosis of HLHS by a pediatric cardiologist and provide counseling to the parents in conjunction with a pediatric cardiac surgeon. B 2. Diagnostic investigations Cardiac imaging: obtain echocardiographic evaluation of HLHS to define the HLHS type, including the situs and cardiac variants, and assess the left ventricle and its function, ventricular septal defect, atrial septum, pulmonary veins (anatomy and Doppler flow patterns), aortic arch including flow patterns (antegrade, retrograde), RV function, and tricuspid valve. Obtain cardiac MRI as the standard for measurement of right ventricle volume and function. B Electrocardiogram: obtain an ECG to exclude cardiac arrhythmias. B Chest X-ray: obtain CXR by clinical indication only. B Genetic testing: obtain testing for chromosomal abnormalities in patients with extracardiac abnormalities to provide additional information for postnatal counseling and prognosis. B 3. Medical management Setting of delivery: allow delivery of a fetus with HLHS, in the absence of risk factors, to occur spontaneously up to 40 weeks. Offer elective delivery planning (induction of labor or Cesarean delivery) no earlier than 39 weeks as long as there are no obstetrical risk factors. B Show 2 more Initial resuscitation: Perform resuscitation, including intubation and ventilation and placement of central and invasive monitoring lines, in unstable neonates. B Administer oxygen and perform intubation and ventilation only by strict indications. B Ensure minimal handling in a tranquil environment with HR, respiratory rate, SaO , and BP monitoring in stable neonates. B Prostaglandin E1: administer continuous, low-dose prostaglandin E1 infusion to preserve patency of the ductus arteriosus before stage I procedure. B Hemodynamic support: administer inodilators (milrinone) to support RV function before stage I procedure, if adequate coronary perfusion pressure is maintained. B https://web.pathway.md/diseases/rec9a3ruUUM6kGOlZ 2/5 6/24/23, 12:47 PM Hypoplastic left heart syndrome Pathway Show 3 more 4. Perioperative care Preoperative imaging: obtain TTE for planning initial surgery in patients with classical HLHS. Consider obtaining 3D echocardiography to assist in imaging the tricuspid valve to assess morphology and regurgitation. B Show 3 more Intraoperative imaging: obtain intraoperative transesophageal or epicardial echocardiography in conjunction with hemodynamic assessment during the Norwood procedure. Consider obtaining exit angiography or CT for clinical concerns, particularly for anatomy less accessible to echocardiography. B Postoperative imaging: obtain TTE as the initial imaging modality for postoperative assessment including all operated regions (atrial communication, neoaortic valve, aortic-pulmonary artery connection, coronary flow, arch reconstruction, source of pulmonary blood flow, and branch pulmonary arteries after after the Norwood procedure, and atrial communication, ductus arteriosus stent, aortic arch flow, bilateral pulmonary artery banding after the hybrid procedure). Consider obtaining angiography or CT for clinical concerns unanswered by echocardiography. B 5. Surgical interventions Prenatal interventions: Consider performing fetal intervention to augment the size of the atrial septal communication to improve perinatal and longer-term outcomes in the fetus with a restrictive foramen ovale. C Consider performing fetal intervention to improve antegrade flow across the aortic valve in an effort to achieve a biventricular outcome in fetuses with critical aortic stenosis and features of emerging HLHS. C Immediate postnatal interventions: consider performing immediate transcatheter or surgical intervention in neonates with HLHS and restrictive ductus arteriosus, restrictive foramen ovale, totally anomalous pulmonary venous drainage, pulmonary valve stenosis, or non-restrictive foramen ovale with high pulmonary blood flow. C Pre-stage 1 interventions: Perform balloon atrial septostomy to treat pulmonary congestion and to increase pulmonary blood flow in cases of insufficient inter-circulatory mixing. B Perform bilateral pulmonary banding for low cardiac output based on a high pulmonary blood flow and widely patent ductus arteriosus. B Norwood stage 1 palliation: Use the following techniques for the management of pulmonary arteries: patch reconstruction of the central pulmonary arteries B direct closure of the central pulmonary arteries B https://web.pathway.md/diseases/rec9a3ruUUM6kGOlZ 3/5 6/24/23, 12:47 PM Hypoplastic left heart syndrome Pathway division of the branch pulmonary arteries B ligature of the proximal ductus arteriosus, no patch. B Show 5 more Hybrid stage 1 palliation: perform hybrid stage 1 palliation electively in the first postnatal week. B Show 2 more Considerations for anesthesia: reduce systemic vascular resistance and prevent surges in afterload to improve systemic perfusion and maintain a balanced pulmonary blood flow/systemic blood flow. B Show 3 more 6. Specific circumstances Patients with tricuspid regurgitation: recognize that tricuspid regurgitation moderately increases mortality and morbidity rates and prevents completion of the single ventricle pathway. B Show 5 more Patients with restrictive atrial septal defect: recognize that intact atrial septum or restrictive foramen ovale are predictors of poor outcome in HLHS. B Show 4 more Patients with pulmonary vein anomalies: perform surgery as early as possible in patients with anomalies of pulmonary venous connection with obstruction. Repair the anomalous connection by anastomosis between the common pulmonary vein and the most convenient part of the common atrium. B Patients with coronary anomalies: Recognize that coronary anomalies can negatively impact survival after stage 1 palliation. Obtain preoperative echocardiographic evaluation if a coronary anomaly is suspected. B Use a particular strategy for stage 1 palliation in patients with aortic atresia/MS associated with a ventriculocoronary connection as it is associated with a higher mortality rate. B 7. Follow-up and surveillance Early postoperative care: ensure compliance with an operating room checklist and a rigorous handover to the ICU team based on an algorithm. A Show 8 more Interstage 1 monitoring: As per EACTS 2020 guidelines, include the following components in the interstage 1 monitoring programs: dedicated interstage 1 care team B https://web.pathway.md/diseases/rec9a3ruUUM6kGOlZ 4/5 6/24/23, 12:47 PM Hypoplastic left heart syndrome Pathway interstage 1 clinic B provision of red flag action plan B standardized discharge process including family education and rooming in B written communication with primary pediatrician and cardiologist B involvement of a registered dietitian B feeding/nutrition protocol including clear daily weight change goals B use of telehealth visits B use of tablet or mobile apps. B As per AHA 2018 guidelines, consider providing a home monitoring program including the following components for the interstage period to reduce interstage morbidity and mortality: daily oxygen saturations daily weights diet record close communication with discharging facility. C 8. Quality improvement Analysis of outcomes: Use the International Paediatric and Congenital Cardiac Code in all registries, databases, and research studies in pediatric and congenital cardiac care, including cases of HLHS. B Ensure that all pediatric and congenital cardiac teams routinely assess their data against national and international benchmarks using multi-institutional databases and registries. B References 1. Nelson Alphonso, Annalisa Angelini, David J Barron et al. Guidelines for the management of neonates and infants with hypoplastic left heart syndrome: The European Association for Cardio-Thoracic Surgery EACTS and the Association for European Paediatric and Congenital Cardiology AEPC Hypoplastic Left Heart Syndrome Guidelines Task Force. Eur J Cardiothorac Surg. 2020 Sep 1;58 3 416 499. Open 2. Bradley S Marino, Sarah Tabbutt, Graeme MacLaren et al. Cardiopulmonary Resuscitation in Infants and Children With Cardiac Disease: A Scientific Statement From the American Heart Association. Circulation. 2018 May 29;137 22):e691-e782. Open https://web.pathway.md/diseases/rec9a3ruUUM6kGOlZ 5/5