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Center for Congenital Heart Disease/Pediatric Cardiology Heart- and Diabetescenter NRW, University Clinic of Ruhr-University Bochum. Bad Oeynhausen, Germany
Department of Pharmacy, NewYork-Presbyterian Hospital Columbia University Irving Medical Center, Department of Pharmacy Practice, Arnold & Marie Schwartz College of Pharmacy, Long Island University. New York, NY
Table 1Hemodynamic profiles of pulmonary hypertension
Classification
Mean pulmonary artery pressure
Pulmonary capillary wedge pressure
Pulmonary vascular resistance
Isolated pre-capillary PH
>20 mm Hg
<15 mm Hg
>3 WU
Combined pre- and post-capillary PH
>15 mm Hg
>3 WU
Isolated post-capillary PH
>15 mm Hg
<3 WU
The 6th World Symposium on Pulmonary Hypertension defined three hemodynamic profiles of pulmonary hypertension (PH): isolated pre-capillary PH, combined. The pre- and post-capillary PH, and isolated post-capillary PH. WU, Wood units).
Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.
The Journal of Heart and Lung Transplantation.2014; 33: 289-297
*Aim for higher end of range when using mTOR to intensify immune suppression for CAV prevention in high-risk patients; aim for lower end of range when targeting reduced intensity immune suppression for PTLD, frequent infections, or renal failure.
EVL-I = more pericardial effusion = more AEs = more discontinuations due to AEs & serious AEs EVL-I = 48% non-significant increase in the relative risk of incidence of the primary endpoint (postoperative wound healing delays, pericardial effusion, pleural effusion needing drainage and acute renal insufficiency events)
Arora (2015) Andreassen (2016)
SCHEDULE: redCYA/EVL & CNI withdrawal at 7‐11 weeks vs CYA+ MMF
115
No significant differences between groups
-
No significant differences between groups for surgical events or wound complications
Eisen (2013)
CRAD 2310: redCyA/EVL 1.5mg vs redCyA/EVL 3mg (dc) vs CYA/MMF
721
EVL/redCYA = more anemia
-
EVL 3mg/redCYA arm = enrolment dc due to higher early mortality EVL/redCYA = more pericardial effusion
dc = discontinued; NS = not stated; SS = statistically significant; AE = adverse event
ISHLT CAV0 (Not significant): No detectable angiographic lesion
ISHLT CAV1 (Mild): Angiographic left main (LM) <50%, or primary vessel with maximum lesion of <70%, or any branch stenosis <70% (including diffuse narrowing) without allograft dysfunction
ISHLT CAV2 (Moderate): Angiographic LM <50%; a single primary vessel ≥70%, or isolated branch stenosis ≥70% in branches of 2 systems, without allograft dysfunction
ISHLT CAV3 (Severe): Angiographic LM ≥50%, or two or more primary vessels ≥70% stenosis, or isolated branch stenosis ≥70% in all 3 systems; or ISHLT CAV1 or CAV2 with allograft dysfunction (defined as LVEF ≤45% usually in the presence of regional wall motion abnormalities) or evidence of significant restrictive physiology (which is common but not specific; see text for definitions)
Definitions
a). A “Primary Vessel” denotes the proximal and Middle 33% of the left anterior descending artery, the left circumflex, the ramus and the dominant or co-dominant right coronary artery with the posterior descending and posterolateral branches.
b). A “Secondary Branch Vessel” includes the distal 33% of the primary vessels or any segment within a large septal perforator, diagonals and obtuse marginal branches or any portion of a non-dominant right coronary artery.
c). Restrictive cardiac allograft physiology is defined as symptomatic heart failure with echocardiographic E to A velocity ratio >2 (>1.5 in children), shortened isovolumetric relaxation time (<60 msec), shortened deceleration time (<150 msec), or restrictive hemodynamic values (Right Atrial Pressure >12mmHg, Pulmonary Capillary Wedge Pressure (PCWP) >25 mmHg (>15mmHg in children
Improved Detection of Cardiac Allograft Vasculopathy: A Multi-Institutional Analysis of Functional Parameters in Pediatric Heart Transplant Recipients.
The frequency of follow-up visits for HT recipients will depend on the time from HT and the post-operative clinical course. The frequency of follow-up should be increased if complications occur, particularly in patients with challenging medical or psychosocial conditions. In addition, in view of different local availabilities of newer noninvasive modalities (e.g., Gene Expression Profiling) and the lack of evidence about the optimal timing of echocardiographic studies in HT patients, it should be noted that the frequency of follow-up visits and schedule presented in the table serve merely as an example and should be tailored to each center. Furthermore, as noninvasive modalities improve, it is likely that the need for biopsies and serial conventional angiography will be reduced accordingly.
B iopsy ² (Other non-invasive methods as appropriate)
×
×
×
×
×
×
×
Right heart study
S tarting in the fifth year – to be done every other year alternating with coronary angiography
Dobutamine echo/SPECT/CTA
x
x
×
x
x
×
x
x
×
x
x
×
x
×
×
C MV DNA ³
×4
×
Coronary angiography
×
×
×
Urine 24h protein
×
Malignancy screening 4
×
×
×
Chest X-ray
×
×
×
PSA
x
x
×
×
x
×
PRA (DSA)5
×
×
×
Bone density
x
x
x
x
CPET
x
×
×
×
×
Skin-cancer screening clinic
x
x
x
x
Endocrinology clinic
x
x
x
x
Dental exam
The frequency of follow-up visits for HT recipients will depend on the time from HT and the post-operative clinical course. The frequency of follow-up should be increased if complications occur, particularly in patients with challenging medical or psychosocial conditions. In addition, in view of different local availabilities of newer noninvasive modalities (e.g., Gene Expression Profiling) and the lack of evidence about the optimal timing of echocardiographic studies in HT patients, it should be noted that the frequency of follow-up visits and schedule presented in the table serve merely as an example and should be tailored to each center. Furthermore, as noninvasive modalities improve, it is likely that the need for biopsies and serial conventional angiography will be reduced accordingly.
Single dose of etomidate, used during induction, has been shown to decrease serum concentration of cortisol for at least 24 hours. However, this has not been shown to be clinically relevant.
Cyclosporine has been described as prolonging muscle relaxants; this effect has not been shown in patients on mycophenolate mofetil and tacrolimus.
Although the apparent higher potential for infectious complications of spinal or epidural anesthesia, limited data have not demonstrated this occurrence for regional or neuraxial procedures
Given the complete cardiac denervation, drugs that work on the autonomic nervous system have minimal effects on the transplanted heart. Indirect-acting sympathomimetics such as ephedrine are therefore not very effective for treating hypotension and maintaining cardiac output; and ketamine may not display hemodynamic stability in heart transplant patients in extremis. Direct-acting sympathetic agents, like norepinephrine, epinephrine, isoproterenol, and dopamine, are effective, although the beta-adrenergic inotropic effects are attenuated early after HT. Phosphodiesterases have been shown to increase inotropy in the transplanted heart. The alpha-adrenergic response of phenylephrine is effective, but the reflex bradycardia is absent.
The indirect acting anticholinergics (atropine, glycopyrrolate) and anticholinesterases (neostigmine, edrophonium) have no effect on the heart rate of the cardiac allograft, and the safety of neuromuscular reversal has been demonstrated in a large-scale study with no instances of severe bradycardia or cardiac arrest. The direct neuromuscular blockade Sugammadex, which directly inhibits neuromuscular blocking agents, is devoid of any direct cholinergic effects, and is a reasonable alternative in HT recipients.
Appropriated photographic documentation of the intact and the sectioned hearts should be performed Preferably before fixation in 10% formalin, sampling of fresh myocardium from the four cavities taking multiple small fragments to be frozen for genetic and molecular analysis, and to be fixed in Karnovsky/glutaraldehyde for electron microscopy for diagnostic and for research purposes. Gross examination before sectioning according to standard protocols, which take into consideration the different types of pathologies, which have led to transplant. Sectioning according to the different types of pathologies: - for cardiomyopathies, ischemic heart disease, and valve diseases transverse cut from apex to the base of the heart. - for congenital heart disease the transverse cut is not recommended but use the sequential segmental approach 5) Histological sampling of the entire circumferential midventricular transverse cut and of the coronary arteries for multiple appropriate staining including immunohistochemistry 6) In case of mechanical assistance device implantation prior to transplant it would be important to evaluate grossly the device before removing it. In case of interventional procedures, both percutaneous and surgical, on the coronary arteries and on the valves particular care should be adopted for stents, valve, and vascular prosthesis with specific technique.
The International Society for Heart and Lung Transplantion (ISHLT) Guidelines for the Care of Heart Transplant Recipients were originally published in 2010. 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 peri-operative 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 Criteria
Tabled
1
Class I
Evidence and/or general agreement that a given treatment or procedure is beneficial, useful, and effective
Class II
Conflicting evidence and/or divergence of opinion about the usefulness/efficacy of the treatment or procedure
Class IIa
Weight of evidence/opinion is in favor of usefulness/efficacy
Class IIb
Usefulness/efficacy is less well established by evidence/opinion
Class III
Evidence or general agreement that the treatment or procedure is not useful or effective and in some cases may be harmful
Level of evidence A
Data derived from multiple randomized clinical trials or meta-analyses
Level of evidence B
Data derived from a single randomized clinical trial or large non-randomized studies
Level of evidence C
Consensus of opinion of the experts and/or small studies, retrospective studies, registries
] 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).[
]A 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.[
] 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.[
Reduced Handgrip Strength as a Marker of Frailty Predicts Clinical Outcomes in Patients With Heart Failure Undergoing Ventricular Assist Device Placement.
] 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.[
] 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.[
] 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.[
Preliminary studies regarding prehabilitation, exercise, and nutrition interventions prior to surgery have shown promising results with improved outcomes post-surgery.[
] 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.[
] 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.[
Cardiac rehabilitation has been shown to improve functional capacity and decrease hospital readmissions in HF patients, and is currently recommended by guidelines.[
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.
van der Meer P and Group ESCSD 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.
] and better functional capacity and health-related quality of life in heart failure, heart transplant or left ventricular assist device (LVAD) patients.[
Efficacy of the cardiac rehabilitation program in patients with end-stage heart failure, heart transplant patients, and left ventricular assist device recipients.
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, prior to transplant, to optimize factors that are modifiable, based on pre-transplant 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.[
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.
The Journal of Heart and Lung Transplantation.2018; 37: 803-823
The presence of pre-transplant 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 pre-transplant PH is associated with significantly worse short-term survival post HT compared to patients without pre-transplant PH.[
] 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.[
Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.
The Journal of Heart and Lung Transplantation.2014; 33: 289-297
] First, PH is defined by a mean PAP (mPAP) greater than 20mmHg (previously greater than 25mmHg). The lower parameter reflects recent studies suggesting that individuals with mPAP 21-24mmHg 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(20mmHg or less).[
] 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.[
Prognostic value of the pre-transplant diastolic pulmonary artery pressure–to–pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension.
The Journal of Heart and Lung Transplantation.2014; 33: 289-297
] 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 (Figure 1). 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 pre-clinical 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.[
Figure 1Established effects of some targeted interventions. Blue bars symbolize depleting or reducing effect. Anti-CD20 antibodies show strong effect on naïve, effector, and memory B cells but no effect on plasma cells, which are not expressing CD20. Proteasome inhibitors show strong effect on PC and moderate effect on memory B cells. Anti-CD19 cells target PC but are currently not available as an effective clinical therapeutic for transplant. Effect of all of these therapies on LLPC is unclear but appears to be limited. (IL: interleukin, LLPC: long lives plasma cell, PC: plasma cell)
Consideration of mechanical circulatory support (MCS) for Bridging to Transplant
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 MCS. MCS therapy should be directed by the trajectory of HF progression and clinical status.[
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.
European Journal of Cardio-Thoracic Surgery.2017; 52: 14-25
International Society for H and Lung T. The 2013 International Society for Heart and Lung Transplantation Guidelines for mechanical circulatory support: executive summary.
Recommendations for the Use of Mechanical Circulatory Support: Device Strategies and Patient Selection: A Scientific Statement From the American Heart Association.
Society for Cardiovascular A, Interventions, Heart Failure Society of A, Society of Thoracic S, American Heart A and American College of C. 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.
Impact of Pediatric Risk Models on Wait-list Management
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.[
] Risk factors for early mortality include: recipient variables such as diagnosis, age, gender, sensitization, pulmonary vascular resistance, non-cardiac 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.[
] 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 Assessment, Nutritional Rehabilitation and Nutritional Interventions in the Pediatric Population
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.[
] 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, pro-inflammatory cytokines, neurohormonal abnormalities, increased metabolic demands, reduced intake, and malabsorption.[
] 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 curves[
] (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 children requiring prolonged enteral tube feeding. Multidisciplinary discussions surrounding the risk of surgical intervention and anesthesia are required in these cases.
Consideration of Bridge to Transplant with MCS in Pediatric Recipients
The use of VADs in pediatric patients for the treatment of advanced HF has increased significantly in the past decade and has supplanted ECMO as the most common form of MCS as a bridge to HT. The percentage of children with MCS as a bridge to transplantation has increased from 25% in 2010 to 36% in 2019. The majority of MCS implants in the pediatric population are INTERMACS profiles 1 or 2 with significantly decreased waitlist mortality. However, the ISHLT registry data demonstrates no survival difference between children with or without VAD support, except for worse outcomes in those bridged with ECMO.[
The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-second pediatric heart transplantation report –2019; Focus theme: Donor and recipient size match.
The Journal of Heart and Lung Transplantation.2019; 38: 1028-1041
Modified PCWP threshold in children based on
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