The International Society for Heart and Lung Transplantation (ISHLT) Guidelines for the Care of Heart Transplant Recipients

The International Society for Heart and Lung Transplantion (ISHLT) Guidelines for the Care of Heart Transplant Recipients were originally published in 2010.635Costanzo MR Dipchand A Starling R et al.The International Society of Heart and Lung Transplantation Guidelines for the care of heart transplant recipients.J Heart Lung Transplant. 2010; 29: 914-956Google Scholar These guidelines provided the first comprehensive guideline for the care of Heart Transplant patients. A great deal has changed in the years after this initial unprecedented document. The ISHLT has made the commitment to convene experts in all areas of heart transplantation to develop a focused update to the original practice guidelines. Writers and Chairs were charged with reviewing the existing guidelines and where signifigant new literature exists, updating those original recommendations. Additionally, they were charged to add specific new areas of focus that were undeveloped, undiscovered, or unsupported at the time of the original publication. After a vast effort involving 39 writers from 11 countries worldwide, the “ISHLT Guidelines for the Care of Heart Transplant Recipients” has now been completed and the Executive Summary of these guidelines is the subject of this article. The document results from the work of 4 Task Force groups each co-chaired by a pediatric heart transplant clincian who had the specific mandate to highlight issues unique to the pediatric heart transplant population and to ensure their adequate representation.•Task Force 1 addresses the perioperative care of heart transplant recipients, including:○Pre-Transplant Optimization○Surgical Issues Impacting Care in the Immediate Post-operative Period○Considerations in Patients Bridged with Mechanical Circulatory Support○Early Post-Operative Care of the Heart Transplant Recipient○Evaluation of Allosensitization, Approaches to Sensitized Heart Transplant Recipients, and Hyperacute and Delayed Antibody-Mediated Rejection○Management of ABO “Incompatible” Heart Transplant Recipients○Coagulopathies in Heart Transplant Surgery○Documentation and Communication with the Multidisciplinary Team○Use of Extracorporeal Membrane Oxygenation for the Management of Primary Graft•Task Force 2 discusses the Immunosuppression and Rejection including:○Rejection Surveillance○Monitoring of Immunosuppressive Drug Levels○Principles of Immunosuppression and Recommended Regimens○Treatment of Acute Cellular Rejection○Treatment of Hyperacute and Antibody-Mediated Rejection○Management of Late Acute Rejection•Task Force 3 addresses the Long-term Care of Heart Transplant Recipients; Management of Complications including:○Minimization of Immunosuppression○Management of Neurologic Complications After Heart Transplantation○Cardiac Allograft Vasculopathy○Malignancy After Heart Transplantation○Chronic Kidney Disease After Heart Transplantation○Management of Cardiovascular Risk After Heart Transplantation○Other Complications of Chronic Immunosuppression○Arrhythmias○Anticoagulation after Heart Transplant○Monitoring Recipients of Organs from Donors at Higher Risk of Infectious Diseases○Graft Failure & Considerations for Cardiac Retransplantation•Taskforce 4 covers the Long-term Care of Heart Transplant Recipients. Prevention and Prophylaxis including:○Frequency of Routine Tests and Clinic Visits in Heart Transplant Recipients○Prophylaxis for Corticosteroid-Induced Bone Disease○Exercise, Nutrition and Physical Rehabilitation After Heart Transplantation○Management of Intercurrent Surgery in Heart Transplant Recipients○Reproductive Health After Heart Transplantation○Psychosocial and Psychologic Issues Particularly Related to Adherence to Medical Therapy in Heart Transplant Recipients○Substance Use & Abuse○Endocarditis Prophylaxis After Heart Transplantation○Return to Work or School and Occupational Restrictions After Heart Transplantation○Return to Operating a Vehicle After Heart Transplantation○Family Screening○Management of the Transition from Pediatric to Adult Care After Heart Transplantation○Principles of Shared Care After Heart Transplantation○Travelling After Heart Transplant○Emerging Pathogens, Epidemics and Pandemic Considerations for Heart Transplant Recipients International Society for Heart and Lung Transplantation Standards and Guidelines Committee Grading CriteriaTabled 1Class IEvidence and/or general agreement that a given treatment or procedure is beneficial, useful, and effectiveClass IIConflicting evidence and/or divergence of opinion about the usefulness/efficacy of the treatment or procedureClass IIaWeight of evidence/opinion is in favor of usefulness/efficacyClass IIbUsefulness/efficacy is less well established by evidence/opinionClass IIIEvidence or general agreement that the treatment or procedure is not useful or effective and in some cases may be harmfulLevel of evidence AData derived from multiple randomized clinical trials or meta-analysesLevel of evidence BData derived from a single randomized clinical trial or large non-randomized studiesLevel of evidence CConsensus of opinion of the experts and/or small studies, retrospective studies, registries Open table in a new tab Chair: Kumud Dhital Co-Chair: Estela Azeka Contributing Writers: Monica Colvin, Eugene DePasquale, Marta Farrero, Luis García-Guereta, Gina Jamero, Kiran Khush, Jacob Lavee, CJ Michaud, Jignesh Patel, Stephanie Pouch There is an important interplay between frailty and heart failure (HF). Frailty is an independent predictor for the development of HF.1Khan H Kalogeropoulos AP Georgiopoulou VV et al.Frailty and risk for heart failure in older adults: the health, aging, and body composition study.Am Heart J. 2013; 166: 887-894Google Scholar However, frailty is also associated with increased mortality and morbidity in the elderly and general HF population. The prevalence of frailty is high in advanced HF patients, accounting for over 30% amongst those referred for advanced HF therapies, including heart transplantation (HT). It is an independent prognostic factor for morbidity and mortality, especially in patients with lower peak oxygen consumption (VO2).2Moayedi Y Duero Posada JG Foroutan F et al.The prognostic significance of frailty compared to peak oxygen consumption and B-type natriuretic peptide in patients with advanced heart failure.Clin Transplant. 2018; 32: e13158Google ScholarA variety of methods have been utilized to assess frailty in HF with increasing support for its value in assessing HT patients. Currently, the modified Fried frailty criteria with five physical domains (fatigue, hand grip strength, gait speed, unintended weight loss and physical activity) and additional cognitive assessment (Montreal Cognitive Assessment [MoCA] tool) appears to be a reasonable resource for HT candidates.3Jha SR Hannu MK Chang S et al.The prevalence and prognostic significance of frailty in patients with advanced heart failure referred for heart transplantation.Transplantation. 2016; 100: 429-436Google Scholar While frailty is associated with increased morbidity and mortality in patients undergoing ventricular assist device (VAD) implantation and HT, it is also largely reversible following these procedures.3Jha SR Hannu MK Chang S et al.The prevalence and prognostic significance of frailty in patients with advanced heart failure referred for heart transplantation.Transplantation. 2016; 100: 429-436Google Scholar, 4Chung CJ Wu C Jones M et al.Reduced handgrip strength as a marker of frailty predicts clinical outcomes in patients with heart failure undergoing ventricular assist device placement.J Card Fail. 2014; 20: 310-315Google Scholar, 5Macdonald PS Gorrie N Brennan X et al.The impact of frailty on mortality after heart transplantation.J Heart Lung Transplant. 2021; 40: 87-94Google Scholar Prevalence of malnutrition in the heart failure population is high and represents an independent predictor of poor outcome and mortality.6Aggarwal A Kumar A Gregory MP et al.Nutrition assessment in advanced heart failure patients evaluated for ventricular assist devices or cardiac transplantation.Nutr Clin Pract. 2013; 28: 112-119Google Scholar Pre transplant body mass index (BMI) is a factor that has been shown to correlate with survival post heart transplant. A United Network for Organ Sharing (UNOS) registry study showed the relationship between BMI and post-transplant survival to be U-shaped, with transplant candidates who were underweight (BMI <18.5 kg/m2) and candidates who were obese (BMI > 35 kg/m2) having significantly decreased survival from year 1 to 5.7Russo MJ Hong KN Davies RR et al.The effect of body mass index on survival following heart transplantation: do outcomes support consensus guidelines?.Ann Surg. 2010; 251: 144-152Google Scholar It is important to note, however, that in regards to nutritional screening and assessment of patients with heart failure, the accuracy of any single nutritional indicator may be compromised by many confounding factors, especially be edema. Edema is caused by fluid retention in addition to inflammatory responses, induced by cytoprotective responses to cellular damage caused by under perfusion of peripheral tissues. Both fluid retention and the inflammatory response affect anthropometric measures such as BMI, triceps skinfold measurement and mid-arm circumference, as well as serum markers, such as albumin and prealbumin. Given secondary confounding factors, multidimensional tools should be used to assess nutrition status.6Aggarwal A Kumar A Gregory MP et al.Nutrition assessment in advanced heart failure patients evaluated for ventricular assist devices or cardiac transplantation.Nutr Clin Pract. 2013; 28: 112-119Google Scholar,8Barge-Caballero E García-López F Marzoa-Rivas R et al.Prognostic value of the nutritional risk index in heart transplant recipients.Revis Española Cardio (English Edition). 2017; 70: 639-645Google Scholar,9Lin H Zhang H Lin Z Li X Kong X Sun G. Review of nutritional screening and assessment tools and clinical outcomes in heart failure.Heart Fail Rev. 2016; 21: 549-565Google Scholar Based on a systematic review of literature, the most commonly used tools that provide scores that were independent prognostic factors for mortality risk in heart failure patients, were the Mini Nutrinritional Assessment, MNA-short form, Nutritional Risk Index, and Geriatric Nutritional Risk Index.9Lin H Zhang H Lin Z Li X Kong X Sun G. Review of nutritional screening and assessment tools and clinical outcomes in heart failure.Heart Fail Rev. 2016; 21: 549-565Google Scholar Preliminary studies regarding prehabilitation, exercise, and nutrition interventions before surgery have shown promising results with improved outcomes postsurgery.10West MA Wischmeyer PE Grocott MPW. Prehabilitation and nutritional support to improve perioperative outcomes.Curr Anesthesiol Rep. 2017; 7: 340-349Google Scholar Interventions may include strategies to (1) improve appetite, such as appetite stimulating agents, including megestrol acetate and anabolic steroids; (2) augment caloric intake, including oral food supplements, or with enteral feedings via nasogastric feeding tube, or percutaneous endoscopic gastrostomy; and (3) directly provide micronutrients, carbohydrates and proteins, such as total parental nutrition.7Russo MJ Hong KN Davies RR et al.The effect of body mass index on survival following heart transplantation: do outcomes support consensus guidelines?.Ann Surg. 2010; 251: 144-152Google Scholar Lastly, post-transplant patients are at high risk for osteopenia and osteoporosis, largely due to use of glucocorticoids and calcineurin inhibitors. Transplant candidates should therefore be evaluated for bone disease by bone marrow density (BMD) and parameters of bone and mineral metabolism, so that appropriate therapies, such as vitamin D supplementation and bisphosphonates, can be initiated to minimize patient's risk for osteopenia following transplant.11Kulak CA Borba VZ Kulak Jr., J Custodio MR Osteoporosis after transplantation.Curr Osteoporos Rep. 2012; 10: 48-55Google Scholar,12Rahman A Jafry S Jeejeebhoy K Nagpal AD Pisani B Agarwala R. Malnutrition and cachexia in heart failure.J Parenter Enteral Nutr. 2016; 40: 475-486Google Scholar Cardiac rehabilitation has been shown to improve functional capacity and decrease hospital readmissions in HF patients, and is currently recommended by guidelines.13Piepoli MF Conraads V Corra U et al.Exercise training in heart failure: from theory to practice. A consensus document of the Heart Failure Association and the European Association for Cardiovascular Prevention and Rehabilitation.Eur J Heart Fail. 2011; 13: 347-357Google Scholar,14Ponikowski P Voors AA Anker SD et al.2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: the task force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC)Developed with the special contribution of the Heart Failure Association (HFA) of the ESC.Eur Heart J. 2016; 37: 2129-2200Google Scholar Prehabilitation has been shown to decrease post-operative complication after cardiovascular or abdominal surgery.15Barberan-Garcia A Ubre M Roca J et al.Personalised prehabilitation in high-risk patients undergoing elective major abdominal surgery: a randomized blinded controlled trial.Ann Surg. 2018; 267: 50-56Google Scholar,16Drudi LM Tat J Ades M et al.Preoperative exercise rehabilitation in cardiac and vascular interventions.J Surg Res. 2019; 237: 3-11Google Scholar Physical activity was related to increased event-free survival on the HT waiting list17Spaderna H Vogele C Barten MJ Smits JMA Bunyamin V Weidner G. Physical activity and depression predict event-free survival in heart transplant candidates.Health Psychol. 2014; 33: 1328-1336Google Scholar and better functional capacity and health-related quality of life in heart failure, heart transplant, or left ventricular assist device (LVAD) patients.18Karapolat H Engin C Eroglu M et al.Efficacy of the cardiac rehabilitation program in patients with end-stage heart failure, heart transplant patients, and left ventricular assist device recipients.Transplant Proc. 2013; 45: 3381-3385Google Scholar Pre-transplant psychosocial factors, including patients’ history of medical adherence, mental health, substance use, and social support, can predict outcomes following heart transplantation. Certain factors, such as noncompliance to medical regimen, smoking and alcohol abuse, psychiatric conditions such as depression, and minimal or no social support, have been shown to lead to behaviors of continued or relapse of nonadherence to medical regimen, relapse of substance use, poor self-care, and poor coping. These behaviors lead to poor health-related quality of life with increased morbidity and mortality post-transplant. To maximize outcomes, efforts should be made, before transplant, to optimize factors that are modifiable, based on pretransplant psychosocial evaluation. Interventions may include support groups for substance use, ongoing counseling or therapy, optimization of medical therapy for psychiatric illnesses, and utilization of community resources.19Dew MA DiMartini AF Dobbels F et al.The 2018 ISHLT/APM/AST/ICCAC/STSW recommendations for the psychosocial evaluation of adult cardiothoracic transplant candidates and candidates for long-term mechanical circulatory support.J Heart Lung Transplant. 2018; 37: 803-823Google Scholar The presence of pretransplant pulmonary hypertension (PH) in heart organ recipients increases the risk of post-transplant PH and deterioration in right ventricular function in the donor heart. Large registry studies show pretransplant PH is associated with significantly worse short-term survival post HT compared to patients without pretransplant PH.20Crawford TC Leary PJ Fraser CD et al.Impact of the new pulmonary hypertension definition on heart transplant outcomes.Chest. 2020; 157: 151-161Google Scholar,21Yost G Gregory M Bhat G Short-form nutrition assessment in patients with advanced heart failure evaluated for ventricular assist device placement or cardiac transplantation.Nutr Clin Pract. 2014; 29: 686-691Google Scholar However, assessment of isolated pulmonary hypertension, related to left ventricular failure and reversibility following transplant, remains challenging. In 2018, the 6th World Health Symposium on Pulmonary Hypertension developed two main changes in the definition and classification of PH.22Tedford RJ Beaty CA Mathai SC et al.Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.J Heart Lung Transplant. 2014; 33: 289-297Google Scholar First, PH is defined by a mean PAP (mPAP) greater than 20 mm Hg (previously greater than 25 mm Hg). The lower parameter reflects recent studies suggesting that individuals with mPAP 21 to 24 mm Hg are at increased risk of poor outcomes and tend to progress to “overt PH” (mPAP 25 or greater) more often than patients with lower mPAP(20 mm Hg or less).23Condon DF Nickel NP Anderson R Mirza S de Jesus Perez VA The 6th world symposium on pulmonary hypertension: what's old is new.F1000Research. 2019; 8: 888Google Scholar,24Tsukashita M Takayama H Takeda K et al.Effect of pulmonary vascular resistance before left ventricular assist device implantation on short- and long-term post-transplant survival.J Thorac Cardiovasc Surg. 2015; 150 (e2): 1352-1361Google Scholar In addition, PH was further subclassified by pulmonary vascular resistance (PVR) to help stratify pre-capillary PH (as seen in PAH), and isolated post-capillary PH (IpcPH, related to left ventricle (LV) dysfunction, as well as combined pre- and post-capillary PH (CpcPH) (Table 1). While subcategorization and method of detecting CpcPH remains controversial, current evidence suggests that CpcPH is a distinct entity from PAH or IpcPH and carries a different prognosis both before and after HT.22Tedford RJ Beaty CA Mathai SC et al.Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.J Heart Lung Transplant. 2014; 33: 289-297Google Scholar,25Vakil K Duval S Sharma A et al.Impact of pre-transplant pulmonary hypertension on survival after heart transplantation: a UNOS registry analysis.Int J Cardiol. 2014; 176: 595-599Google ScholarTable 1Hemodynamic Profiles of Pulmonary HypertensionaThe 6th World Symposium on Pulmonary Hypertension defined three hemodynamic profiles of pulmonary hypertension (PH): isolated precapillary PH, combined. The pre- and postcapillary PH, and isolated postcapillary PH).22ClassificationMean pulmonary artery pressurePulmonary capillary wedge pressurePulmonary vascular resistanceIsolated pre-capillary PH>20 mm Hg<15 mm Hg>3 WUCombined pre- and post-capillary PH>15 mm Hg>3 WUIsolated post-capillary PH>15 mm Hg<3 WUWU, wood units.a The 6th World Symposium on Pulmonary Hypertension defined three hemodynamic profiles of pulmonary hypertension (PH): isolated precapillary PH, combined. The pre- and postcapillary PH, and isolated postcapillary PH).22Tedford RJ Beaty CA Mathai SC et al.Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.J Heart Lung Transplant. 2014; 33: 289-297Google Scholar Open table in a new tab WU, wood units. Right heart catheterization should be performed on all adult candidates in preparation for listing, and periodically when patients are listed.26Mehra MR Canter CE Hannan MM et al.The 2016 International Society for Heart Lung Transplantation listing criteria for heart transplantation: a 10-year update.J Heart Lung Transplant. 2016; 35: 1-23Google Scholar Strategies to assess and optimize elevated pulmonary artery (PA) pressures should be utilized to determine reversibility in order to prevent right ventricular failure post-transplant. Medical therapies include diuretics, inotropes, and vasoactive agents, both inhaled (i.e., nitric oxide and prostacyclins), and intravenous (i.e., nitroglycerin and nitroprusside). Phosphodiesterase-3 (PDE-3) inhibitors (i.e., milrinone) have shown immediate hemodynamic effects, however, with no long-term effects on clinical outcomes in PH due to LV failure. Other therapies typically used for WHO Group 1 PH (pulmonary arterial hypertension) have been utilized for WHO group 2 PH (due to LV failure) with varying results. PDE-5 inhibitors (i.e., sildenafil) has demonstrated some beneficial effects. Additionally, endothelin receptor antagonists (ERAs) such as bosentan and tezosentan have shown some improvement in hemodynamics in preclinical and small studies albeit with adverse effects, including hepatic dysfunction. Newer ERAs, such as macetentan, without adverse effects on hepatic function are currently being studied. Finally, PH refractory to medical therapy has been effectively treated with mechanical circulatory support, such as LVADs, with improvement in PH and successful bridging to transplant.27Koulova A Gass AL Patibandla S Gupta CA Aronow WS Lanier GM Management of pulmonary hypertension from left heart disease in candidates for orthotopic heart transplantation.J Thorac Dis. 2017; 9: 2640-2649Google Scholar Patients with HF refractory to optimal medical therapy, with hemodynamic instability and/or progressive end organ dysfunction, should be considered for short-term and/or long-term mechanical circulatory support (MCS). MCS therapy should be directed by the trajectory of HF progression and clinical status.28den Uil CA Akin S Jewbali LS et al.Short-term mechanical circulatory support as a bridge to durable left ventricular assist device implantation in refractory cardiogenic shock: a systematic review and meta-analysis.Eur J Cardiothorac Surg. 2017; 52: 14-25Google Scholar, 29Feldman D Pamboukian SV Teuteberg JJ et al.The 2013 International Society for Heart and Lung Transplantation Guidelines for mechanical circulatory support: executive summary.J Heart Lung Transplant. 2013; 32: 157-187Google Scholar, 30Nagpal AD Singal RK Arora RC Lamarche Y. Temporary mechanical circulatory support in cardiac critical care: a state of the art review and algorithm for device selection.Can J Cardiol. 2017; 33: 110-118Google Scholar, 31Peura JL Colvin-Adams M Francis GS et al.Recommendations for the use of mechanical circulatory support: device strategies and patient selection: a scientific statement from the American Heart Association.Circulation. 2012; 126: 2648-2667Google Scholar, 32Potapov EV Antonides C Crespo-Leiro MG et al.2019 EACTS expert consensus on long-term mechanical circulatory support.Eur J Cardiothorac Surg. 2019; 56: 230-270Google Scholar, 33Rihal CS Naidu SS Givertz MM et al.2015 SCAI/ACC/HFSA/STS clinical expert consensus statement on the use of percutaneous mechanical circulatory support devices in cardiovascular care: endorsed by the American Heart Assocation, the Cardiological Society of India, and Sociedad Latino Americana de Cardiologia Intervencion; Affirmation of Value by the Canadian Association of Interventional Cardiology-Association Canadienne de Cardiologie d'intervention.J Am Coll Cardiol. 2015; 65: e7-e26Google Scholar, 34Sánchez-Enrique C Jorde UP González-Costello J Heart transplant and mechanical circulatory support in patients with advanced heart failure.Rev Española Cardiol (English Edition). 2017; 70: 371-381Google Scholar Selection of pediatric recipients is a multifactorial process including specific considerations of factors that will directly impact posttransplant outcome. Furthermore, the spectrum of advanced therapies as well as donor polices, public initiatives and published studies have significantly changed approaches in the management and care of this special population. Candidate selection and waitlist removal are a multidisciplinary process that balances the risks and benefits for the transplant procedure.35Chen CK Manlhiot C Mital S et al.Prelisting predictions of early postoperative survival in infant heart transplantation using classification and regression tree analysis.Pediatr Transplant. 2018; 22: e13105Google Scholar,36Peng D Schumacher K. Risk factors for early and late mortality in pediatric heart transplantation.Pediatr Heart Transplant. 2019; : 224-248Google Scholar Pediatric risk factor models have been studied in early and late mortality.35Chen CK Manlhiot C Mital S et al.Prelisting predictions of early postoperative survival in infant heart transplantation using classification and regression tree analysis.Pediatr Transplant. 2018; 22: e13105Google Scholar Risk factors for early mortality include: recipient variables such as diagnosis, age, gender, sensitization, pulmonary vascular resistance, noncardiac end organ status, mechanical ventilation, extracorporeal membrane oxygenation, VADs; donor-related factors including ischemic time, donor graft function, cause of death. Small center volume has been described as a potential variable for increased post-transplant mortality. A model for in-hospital mortality after pediatric transplantation has been studied using variables available in Organ Procurement Transplantation Network (OPTN) which includes hemodynamic support; Extracorporeal Membrane Oxygenation (ECMO), VAD, ventilator and medical therapy, cardiac diagnosis, renal dysfunction, and serum total bilirubin. This model has C-statistics of 0.75 and 0.81.37Almond CS Gauvreau K Canter CE Rajagopal SK Piercey GE Singh TP. A risk-prediction model for in-hospital mortality after heart transplantation in US children: risk prediction in pediatric heart transplant.Am J Transplant. 2012; 12: 1240-1248Google Scholar The risk factor model using donor variables on 1-year or late mortality post-transplant has been studied using the OPTN registry38Zafar F Jaquiss RD Almond CS et al.Pediatric Heart Donor Assessment Tool (PH-DAT): a novel donor risk scoring system to predict 1-year mortality in pediatric heart transplantation.J Heart Lung Transplant. 2018; 37: 332-339Google Scholar including ischemic time, stroke as the cause of death, donor-to recipient height ratio, donor left ventricular ejection fraction, and donor glomerular filtration rate. This model can be useful when assessing acceptability of a prospective organ in a recipient. Therefore, risk factors models can provide an impact on wait list management after acknowledgement of unmeasured and confounding factors. Nutritional status in most pediatric chronic conditions is a major determinant of childhood well-being. Chronic HF in children is a major cause of malnutrition.39Godown J Friedland-Little JM Gajarski RJ et al.Abnormal nutrition affects waitlist mortality in infants awaiting heart transplant.J Heart Lung Transplant. 2014; 33: 235-240Google Scholar, 40Heuschkel RB Gottrand F Devarajan K et al.ESPGHAN position paper on management of percutaneous endoscopic gastrostomy in children and adolescents.J Pediatr Gastroenterol Nutr. 2015; 60: 131-141Google Scholar, 41Kirk R Dipchand AI Rosenthal DN et al.The International Society for Heart and Lung Transplantation Guidelines for the management of pediatric heart failure: executive summary.J Heart Lung Transplant. 2014; 33: 888-909Google Scholar, 42Schwarz SM Gewitz MH See CC et al.Enteral nutrition in infants with congenital heart disease and growth failure.Pediatrics. 1990; 86: 368-373Google Scholar, 43Spillane NT Kashyap S Bateman D Weindler M Krishnamurthy G. Comparison of feeding strategies for infants with hypoplastic left heart syndrome: a randomized controlled trial.World J Pediatr Congenital Heart Surg. 2016; 7: 446-453Google Scholar Malnutrition is an imbalance of nutrients between intake and nutritional requirements. The body is unable to meet metabolic demands in the setting of cardiac dysfunction. The pathophysiology of heart failure involves activation of compensatory pathways, proinflammatory cytokines, neurohormonal abnormalities, increased metabolic demands, reduced intake, and malabsorption.44Lewis KD Conway J Cunningham C Larsen BMK. Optimizing nutrition in pediatric heart failure: the crisis is over and now it's time to feed.Nutr Clin Pract. 2018; 33: 397-403Google Scholar These mechanisms lead to starvation, malabsorption nutritional loss, and hypermetabolism which result in malnutrition and suboptimal growth. Therefore, it is recommended that nutritional status should be addressed by history, and nutritional and physical assessment. The basic tools for initial evaluation include a history of energy, protein and fluid intake, weight, length, head circumference measurements on sex- and age-specific growth curves44Lewis KD Conway J Cunningham C Larsen BMK. Optimizing nutrition in pediatric heart failure: the crisis is over and now it's time to feed.Nutr Clin Pract. 2018; 33: 397-403Google Scholar,45WHO Child Growth Standards. Available at: https://wwwwhoint/childgrowth/standards/en/. 2021.Google Scholar (weight for age, length for age, body mass index) on which individual patient's values can be plotted and detection of growth velocity deviation. Nutritional support includes hypercaloric feeds, oral supplements, and enteral and parenteral nutrition. Enteral nutrition is required when oral intake is insufficient. Conditions such as severe cord dysfunction, dysphagia, or oral aversion can interfere with adequate oral intake. Nasojejunal tube feeds may be used when nasogastric tube feeds are not tolerated. Nutritional support via gastrostomy can be effective at reversing malnutrition, in maintaining nutritional status, and may be indicated in