The Pediatric-to-Adult Transition in the ICU: Caring for the Young Adult

A Comprehensive Review for Critical Care Practitioners

Dr Neeraj Manikath , claude.ai

Abstract

The transition of young adults with childhood-onset chronic diseases from pediatric to adult intensive care represents a complex challenge requiring specialized knowledge and multidisciplinary coordination. Advances in pediatric medicine have resulted in over 90% of children with chronic conditions now surviving into adulthood, creating a growing population of young adults with diseases traditionally managed in pediatric settings. This review examines the critical care management of transitioning patients, with particular emphasis on congenital heart disease and cystic fibrosis, while addressing the unique communication and psychosocial needs of this vulnerable population.

Introduction

The adult intensivist increasingly encounters young adults with diseases once considered exclusively pediatric. This demographic shift, termed the "transition population," presents unique clinical, psychosocial, and ethical challenges. Unlike typical adult ICU patients, these individuals bring complex congenital anatomies, multiorgan involvement, and psychological frameworks shaped by lifelong medical engagement. Understanding their specific needs is paramount for optimal outcomes and represents an essential competency for modern critical care practice.

Managing Congenital Heart Disease Patients Presenting to Adult ICUs

Epidemiology and Scope

Approximately 1.4 million adults in the United States live with congenital heart disease (CHD), with nearly half classified as having moderate or complex lesions. The adult CHD population now exceeds the pediatric CHD population, yet fewer than 50% receive specialized care in adult congenital heart disease (ACHD) centers. Consequently, general adult ICUs must be prepared to manage these patients during acute decompensation, perioperative care, or pregnancy-related complications.

Understanding Altered Anatomy and Physiology

Pearl #1: Never assume normal cardiac anatomy. Always obtain prior operative reports, echocardiograms, and catheterization data before implementing management strategies.

The fundamental challenge in managing CHD patients lies in appreciating their unique cardiovascular physiology. Common scenarios include:

Single Ventricle Physiology (Fontan Circulation): These patients have undergone staged palliation resulting in passive pulmonary blood flow without a subpulmonary ventricle. The Fontan circulation operates with elevated central venous pressures (12-18 mmHg) and depends critically on low pulmonary vascular resistance. Standard ICU interventions can be catastrophic:

Hack: Avoid positive pressure ventilation when possible; even modest PEEP (>5 cmH₂O) can dramatically reduce cardiac output by impeding venous return. If intubation is necessary, use low tidal volumes (4-6 mL/kg), minimal PEEP, and consider early extubation strategies.
Maintain preload meticulously; these patients cannot increase cardiac output through heart rate or contractility alone.
Treat arrhythmias aggressively, as they tolerate tachyarrhythmias and bradyarrhythmias poorly.

Transposition of the Great Arteries (TGA) with Atrial Switch (Mustard/Senning): The morphologic right ventricle supports the systemic circulation, with the tricuspid valve serving as the systemic AV valve. These patients develop right ventricular dysfunction, tricuspid regurgitation, and atrial arrhythmias.

Oyster: The "normal" ejection fraction on echo may be misleading; these right ventricles function at lower ejection fractions than morphologic left ventricles. An EF of 40% may represent severe dysfunction.
Avoid negative inotropes; use afterload reduction cautiously as systemic hypotension is poorly tolerated.

Eisenmenger Syndrome: Uncorrected left-to-right shunts eventually lead to pulmonary arterial hypertension with shunt reversal. These patients present with cyanosis and erythrocytosis.

Pearl #2: Never perform phlebotomy for "hyperviscosity" unless symptomatic and hematocrit exceeds 65%. The elevated hemoglobin is compensatory; unnecessary phlebotomy causes iron deficiency and worsens hyperviscosity.
Pulmonary vasodilators (sildenafil, bosentan, epoprostenol) are cornerstone therapies but must be initiated by specialists.
Pregnancy is absolutely contraindicated (maternal mortality 30-50%).

Critical Care Management Strategies

Hemodynamic Monitoring: Standard Swan-Ganz catheters may be impossible to place in complex anatomy. Consider:

Arterial catheters for beat-to-beat blood pressure monitoring
Central venous access for CVP trending (though interpretation varies by lesion)
Non-invasive cardiac output monitoring (pulse contour analysis, bioreactance)
Frequent echocardiography by experienced sonographers

Anticoagulation Considerations: Many CHD patients require chronic anticoagulation for mechanical valves, Fontan circulation, or atrial arrhythmias. Collaborate early with hematology and cardiology regarding bridging strategies.

Arrhythmia Management:

Hack: Obtain old ECGs for comparison; their "normal" may show dramatic axis deviation, conduction blocks, or paced rhythms.
Atrial tachyarrhythmias are common and poorly tolerated; aggressive rate or rhythm control is essential.
Maintain electrolyte homeostasis rigorously (K⁺ >4.0, Mg²⁺ >2.0).

Endocarditis Prophylaxis: These patients remain at lifelong high risk. Use appropriate antibiotic prophylaxis for procedures and maintain high suspicion for endocarditis with any fever or bacteremia.

Respiratory Management:

Avoid hypoxemia and hypercarbia, which increase pulmonary vascular resistance
Use non-invasive ventilation preferentially
If intubated, target normocapnia and optimize FiO₂ delivery while minimizing mean airway pressures

Pearl #3: Early consultation with ACHD specialists is not optional—it's essential. These subspecialists possess nuanced understanding of individual patient anatomy and can prevent catastrophic management errors.

Pregnancy-Related Complications

Pregnancy in CHD patients represents a high-risk scenario requiring multidisciplinary management. Women with Eisenmenger syndrome, severe left ventricular outflow obstruction, or systemic ventricular dysfunction face the highest mortality risk. Hemodynamic changes of pregnancy (increased blood volume, decreased systemic vascular resistance, hypercoagulability) can precipitate acute decompensation during the peripartum period. Consider early arterial access, invasive monitoring, and delivery in centers with ACHD and maternal-fetal medicine expertise.

Cystic Fibrosis and Other Childhood Diseases in the Adult Critical Care Setting

Cystic Fibrosis: From Pediatric Disease to Adult Chronic Illness

Cystic fibrosis (CF), once fatal in childhood, now sees median survival exceeding 50 years thanks to CFTR modulator therapies. However, adult CF patients experience progressive multiorgan complications requiring critical care expertise beyond traditional pulmonology.

Acute Respiratory Failure in CF

Clinical Presentation: CF patients develop chronic airway infection (typically Pseudomonas aeruginosa, Burkholderia cepacia, Staphylococcus aureus, nontuberculous mycobacteria), bronchiectasis, and progressive obstructive lung disease. Acute exacerbations manifest with increased cough, sputum production, dyspnea, and declining lung function.

Microbiological Considerations:

Pearl #4: Always reference prior sputum cultures; CF patients harbor multidrug-resistant organisms requiring tailored antibiotic regimens.
Burkholderia cepacia complex predicts poor outcomes and may preclude lung transplantation at some centers
Combination intravenous antibiotics are standard (usually β-lactam plus aminoglycoside or fluoroquinolone)
Extended dosing intervals for aminoglycosides (7-10 mg/kg once daily) due to altered pharmacokinetics

Respiratory Management:

Hack: Maximize airway clearance with vest therapy, directed coughing, and mucolytics (dornase alfa, hypertonic saline) even during mechanical ventilation
Non-invasive positive pressure ventilation (NIPPV) is first-line for respiratory failure; avoid intubation when possible as extubation may be extremely difficult
If intubated, perform frequent suctioning; consider bronchoscopy for mucus plugging
Target oxygen saturations of 88-92% for most patients (many have chronic hypoxemia)

Oyster: Hemoptysis is common in CF but rarely life-threatening. Massive hemoptysis (>240 mL/24h) requires bronchial artery embolization. Never anticoagulate these patients without compelling indication.

CF-Related Diabetes (CFRD)

Nearly 50% of adults with CF develop diabetes due to pancreatic insufficiency and destruction of islet cells. CFRD differs from types 1 and 2 diabetes:

Maintain blood glucose 90-180 mg/dL; avoid hypoglycemia
Insulin is the only recommended therapy; metformin and SGLT2 inhibitors are contraindicated
During acute illness, nutritional support is essential (CF patients require 120-150% of predicted caloric needs)

Liver Disease and Portal Hypertension

CF-related liver disease affects 30-40% of patients, manifesting as cirrhosis, portal hypertension, and variceal bleeding. Management parallels other cirrhotic populations but consider:

Maintain nutrition aggressively (malnutrition accelerates hepatic decompensation)
Fat-soluble vitamin supplementation (A, D, E, K)
Standard variceal bleeding protocols apply

Pneumothorax Management

Pneumothorax complicates CF in 3-4% annually, typically occurring with advanced disease. Standard chest tube management applies, but:

Hack: Avoid pleurodesis in potential transplant candidates as it complicates future surgery; consult transplant teams early
Recurrent pneumothorax may necessitate VATS or transplant evaluation

Transplant Considerations

Pearl #5: Initiate transplant discussions early in critical illness. The window for successful transplantation narrows rapidly with acute decompensation.

Lung transplantation criteria for CF include:

FEV₁ <30% predicted
Rapid lung function decline
Pulmonary hypertension
Increasing antibiotic requirements
Life-threatening hemoptysis
Respiratory failure requiring mechanical ventilation

Bridge to transplant options include invasive and non-invasive ventilation, though outcomes decline precipitously with prolonged mechanical ventilation. ECMO bridging is controversial but increasingly utilized at high-volume centers.

Other Childhood Diseases in Adult ICU Settings

Sickle Cell Disease (SCD): Acute chest syndrome represents a leading cause of ICU admission and mortality. Distinguish from pneumonia (though both may coexist). Management includes:

Oxygen supplementation targeting SpO₂ >95%
Incentive spirometry and analgesia to prevent splinting
Broad-spectrum antibiotics (cover atypicals)
Transfusion strategy: Simple transfusion if Hgb <9 g/dL; exchange transfusion for severe disease or rapid progression (target HbS <30%)
Early hematology consultation
Hack: Avoid over-hydration; SCD patients are prone to pulmonary edema

Muscular Dystrophies: Duchenne and Becker muscular dystrophy patients survive into adulthood with cardiomyopathy and restrictive lung disease. Key management points:

Many use chronic non-invasive ventilation; continue during hospitalization
Avoid succinylcholine (risk of rhabdomyolysis and hyperkalemia)
Cardiomyopathy requires standard heart failure management
Cough-assist devices and mechanical insufflation-exsufflation aid secretion clearance

Spina Bifida: Adults with spina bifida face neurogenic bladder, latex allergy, and shunt-dependent hydrocephalus.

Pearl #6: Always use latex-free equipment
High index of suspicion for shunt malfunction with altered mental status
Urological complications common; early urology consultation for any genitourinary concerns

Communication and Psychosocial Support for Young Adults and Their Families

The Developmental Context

Young adults (ages 18-30) navigate a critical developmental period characterized by identity formation, autonomy development, and establishment of adult relationships. For those with chronic childhood illness, this transition is further complicated by medical complexity, psychological burden, and disrupted normal developmental trajectories.

Challenges in the Transition Population

Medical Complexity: These patients possess sophisticated knowledge of their disease developed over years yet may lack understanding of adult healthcare systems, insurance navigation, or self-advocacy skills traditionally developed during healthy adolescence.

Psychological Burden: Chronic illness during childhood and adolescence increases risks of depression, anxiety, post-traumatic stress, and adjustment disorders. ICU admission may represent loss of hard-won stability and trigger existential distress.

Social Isolation: Medical demands often limit educational opportunities, employment, and peer relationships, resulting in smaller support networks compared to healthy young adults.

Family Dynamics: Parents of children with chronic illness develop expertise as medical advocates and decision-makers. The transition to adult care requires renegotiating roles, which may be incomplete when critical illness strikes.

Communication Strategies

Pearl #7: Address the patient directly, even when parents are present. Use language that respects their adult status while acknowledging their expertise in their disease.

Establishing the Primary Relationship: Begin each interaction by clarifying who the patient wants involved in medical discussions. While respecting parental concern, center the patient as the primary decision-maker unless cognitive limitations exist. Sample opening: "I'd like to understand how you prefer to discuss your medical care. Do you want your parents involved in all discussions, or are there times you'd prefer to talk with me alone?"

Acknowledging Expertise: These patients are experts in their disease. Validating their knowledge builds therapeutic alliance: "You've lived with this your whole life—help me understand what's normal for you and what concerns you about what's happening now."

Avoiding Pediatric Language: Eliminate terms like "honey," "sweetie," or references to parents as "mom and dad" without patient's explicit preference. Speak as you would to any competent adult.

Managing Parental Anxiety: Parents face profound distress when their child enters adult critical care. Acknowledge their expertise while redirecting to the patient:

"I can see you've been instrumental in managing [patient name]'s care. As we move forward, I'll be primarily directing questions to [patient name], but I value your input when they want you involved."
Offer scheduled family meetings to address parental concerns without undermining patient autonomy

Hack: Schedule separate conversations with patient and family if conflict arises around decision-making or information sharing. Clarify the patient's wishes privately before engaging in family discussions.

Developmental-Stage-Appropriate Communication

Young adults vary in developmental maturity, medical literacy, and coping capacity. Tailor communication:

For Younger Patients (18-22): May benefit from:

More frequent check-ins
Explanation of ICU routines and expectations
Permission to ask questions without judgment
Acknowledgment that this experience differs from pediatric care

For Older Patients (23-30): Often prefer:

Direct, efficient communication
Greater autonomy in decision-making
Recognition of competing life responsibilities (work, relationships, finances)
Realistic prognosis discussions

Addressing Goals of Care

Pearl #8: Initiate goals-of-care conversations early, before crisis mandates urgent decisions. Frame discussions around quality of life, not just quantity.

Young adults with chronic illness have often contemplated mortality more than healthy peers but may not have articulated preferences. Useful prompts include:

"Help me understand what gives your life meaning and what you hope for in your future."
"Have you thought about what medical treatments you would or wouldn't want if your health deteriorated?"
"If your health doesn't improve, what would be most important to you?"

Navigating Family Disagreements: When patients and families disagree about treatment goals, clarify:

Patient's decision-making capacity (formal assessment if unclear)
Patient's explicitly stated preferences
Duty to patient supersedes parental preferences for competent adults

Involve palliative care and ethics consultation early when conflicts arise.

Psychosocial Support Interventions

Multidisciplinary Team Engagement:

Social work: Assess support systems, financial concerns, insurance coverage, and connect with community resources
Psychology/psychiatry: Screen for depression, anxiety, PTSD; provide coping strategies and consider pharmacologic intervention when indicated
Child life specialists: Some institutions extend child life services to young adults; these specialists understand chronic illness's developmental impact
Spiritual care: Explore existential and spiritual concerns regardless of religious affiliation

Peer Support: Connect patients with peer support groups (many disease-specific organizations offer young adult programs). Peer mentorship provides validation that medical teams cannot replicate.

Maintaining Normalcy:

Allow personal items, photographs, and music in the ICU
Facilitate video calls with friends when appropriate
Encourage families to maintain normal conversation topics, not only medical discussions
Hack: Coordinate care to allow consolidated sleep periods; chronic illness patients often experience poor sleep quality at baseline

Supporting Parents: Parents face role ambiguity, grief, and helplessness. Interventions include:

Acknowledging their lifelong caregiving role while supporting new dynamics
Providing specific tasks (coordinating visitors, liaising with extended family) to channel their need to help
Connecting with parent support organizations
Respite periods encouraged (parents may resist leaving)

Addressing End-of-Life Care

When transition to palliative care becomes appropriate, young adults and families face unique grief. Death in young adulthood represents "off-time" mortality, violating expected life trajectories. Compassionate end-of-life care includes:

Developmentally Appropriate Legacy Work:

Written letters or video messages to loved ones
Completion of "unfinished business" (relationship mending, expressing gratitude)
Life review emphasizing accomplishments despite illness burden
Memory-making with photography or handprint molds

Family-Centered Care:

Liberal visitation policies
Space for siblings (often forgotten grievers)
Bereavement support referrals
Follow-up after death (condolence cards, optional bereavement meetings)

Oyster: Withdrawal of life support in young adults generates profound moral distress among healthcare teams. Debrief with staff after difficult cases; normalize emotional responses and reinforce ethical appropriateness of honoring patient preferences.

Conclusion

The pediatric-to-adult transition population represents a growing and complex cohort in adult critical care. Success requires more than disease-specific knowledge—it demands appreciation of altered anatomy and physiology, understanding of developmental psychology, and commitment to patient-centered communication. By integrating specialized medical expertise with developmentally appropriate psychosocial support, intensivists can optimize outcomes while honoring the unique experiences and perspectives of young adults navigating critical illness.

The adult ICU must evolve to embrace this population with curiosity rather than apprehension. Each encounter offers opportunity for learning and for providing compassionate, expert care to patients who have demonstrated remarkable resilience throughout their lives. As the transition population continues to expand, developing institutional protocols, fostering subspecialty collaborations, and pursuing ongoing education will ensure that young adults receive the sophisticated, holistic care they deserve.

Key Pearls Summary

Always obtain prior records before managing CHD patients
Never perform routine phlebotomy for erythrocytosis in Eisenmenger syndrome
Early ACHD consultation is mandatory, not optional
Reference prior cultures in CF patients for antibiotic selection
Initiate transplant discussions early in critical illness
Use latex-free equipment for spina bifida patients
Address young adult patients directly, respecting their autonomy
Begin goals-of-care conversations early, before crisis

References

Marelli AJ, Ionescu-Ittu R, Mackie AS, et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130(9):749-756.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease. Circulation. 2019;139(14):e698-e800.
Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102(14):1081-1086.
Cystic Fibrosis Foundation Patient Registry. 2022 Annual Data Report. Bethesda, MD; 2023.
Flume PA, Mogayzel PJ, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med. 2009;180(9):802-808.
Moran A, Pillay K, Becker DJ, et al. ISPAD Clinical Practice Consensus Guidelines 2018: Management of cystic fibrosis-related diabetes in children and adolescents. Pediatr Diabetes. 2018;19(Suppl 27):64-74.
Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342(25):1855-1865.
Gabriel P, McManus M, Rogers K, White P. Outcome evidence for structured pediatric to adult health care transition interventions: a systematic review. J Pediatr. 2017;188:263-269.
White PH, Cooley WC. Supporting the Health Care Transition From Adolescence to Adulthood in the Medical Home. Pediatrics. 2018;142(5):e20182587.
Sawicki GS, Lukens-Bull K, Yin X, et al. Measuring the transition readiness of youth with special healthcare needs: validation of the TRAQ—Transition Readiness Assessment Questionnaire. J Pediatr Psychol. 2011;36(2):160-171.
Broberg CS, Hinton RB, Rosenkranz E, et al. Pregnancy in Women With Adult Congenital Heart Disease. JACC Heart Fail. 2020;8(5):371-382.
Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med. 2019;381(19):1809-1819.
Hayes D Jr, Higgins RS, Kirkby S, et al. Impact of pulmonary hypertension on survival in patients with cystic fibrosis undergoing lung transplantation. Am J Respir Crit Care Med. 2014;190(12):1456-1464.
Jeppesen J, Green A, Steffensen BF, Rahbek J. The Duchenne muscular dystrophy population in Denmark, 1977-2001: prevalence, incidence and survival in relation to the introduction of ventilator use. Neuromuscul Disord. 2003;13(10):804-812.
Bomba F, Herrmann-Garitz C, Schmidt J, Schmidt S, Thyen U. An assessment of the experiences and needs of adolescents with chronic conditions in transitional care: a qualitative study to develop a patient education program. Health Soc Care Community. 2017;25(2):652-666.

Corresponding Author Declaration: This manuscript represents expert consensus and literature review intended for educational purposes in critical care medicine training programs.

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Thursday, November 6, 2025