Heart Failure in Congenital Heart Disease¶

Learning Objectives¶

By the end of this rotation, the resident should be able to:

Guideline anchors used in this topic

AHA-CHDHF-2024 = AHA Scientific Statement on chronic HF in CHD (Circulation 2024;150:e33–e50)

ISHLT-PedsHF-2025 = ISHLT Pediatric Heart Failure Guidelines (Update from 2014)

ISHLT-HTx-2024 = ISHLT Cardiac Transplant Candidate Guidelines

ISHLT-MCS-2023 = ISHLT Mechanical Circulatory Support Guidelines (10-year update)

ISHLT-PedsVAD-2021 = ISHLT Pediatric/CHD VAD Consensus Statement (AHA-endorsed)

Recognize & Diagnose Heart Failure in CHD¶

Recognize age-dependent HF presentations in CHD (infants: feeding intolerance, diaphoresis, tachypnea; older children: exercise intolerance, abdominal pain, fatigue) and distinguish HF from common mimics (bronchiolitis/asthma, sepsis, anemia)
Perform a focused "HF + CHD" bedside assessment (work of breathing, perfusion, pulses/BP gradient, hepatomegaly, edema, gallop, murmur changes, saturations/prehospital baseline)
Identify when "HF symptoms" are more likely a mechanical/lesion problem (e.g., shunt obstruction, coarctation, conduit stenosis, severe AV valve regurgitation) requiring urgent cardiology/surgical evaluation rather than medication escalation alone (AHA-CHDHF-2024)
Order and interpret initial tests in suspected CHD-HF: ECG, CXR, BNP/NT-proBNP, CMP (renal/hepatic), CBC/iron studies when indicated, lactate/blood gas when decompensated; emphasize trend interpretation rather than single values (AHA-CHDHF-2024; ISHLT-PedsHF-2025)
Recognize HF with preserved ejection fraction (HFpEF) can be common in CHD, so a "normal EF" does not exclude clinically meaningful HF; integrate diastolic/filling pressure thinking with cardiology (AHA-CHDHF-2024)

Hemodynamic Reasoning in CHD-HF¶

Classify the dominant mechanism of HF in CHD and connect it to lesions you'll actually see:
- Volume overload (left-to-right shunts, regurgitant valves)
- Pressure overload (outflow obstruction)
- Myocardial dysfunction (post-op injury, ischemia/anomalous coronaries, chronic pacing, myocarditis)
- Single-ventricle/Fontan physiology
- Systemic RV failure
- Pulmonary hypertension / RV failure
Use "congestion vs low output vs both" as a working bedside model and identify which findings point to each (tachypnea/hepatomegaly vs cool extremities/oliguria/rising lactate)
Apply a standardized staging language (Stage A–D framework) to communicate risk and severity in CHD-HF and to structure escalation/consult urgency (AHA-CHDHF-2024)
Identify decompensation triggers that residents can and should treat early (arrhythmia, infection/fever, dehydration/over-diuresis, anemia, medication nonadherence, thyroid dysfunction, pulmonary hypertensive crisis) (ISHLT-PedsHF-2025)

Initiate Management Safely¶

Start initial CHD-HF management appropriate to setting (clinic/ED/floor):
- Oxygen/ventilatory support when needed (and understand when oxygen is not the "fix" in certain mixing lesions)
- Diuresis and careful I/O/weights
- Early cardiology notification for known moderate/complex CHD
Select a diuretic strategy and monitoring plan (electrolytes, renal function, weight trajectory, urine output) and recognize signs of over-diuresis/renal injury
Explain what "guideline-directed medical therapy" (GDMT) can and cannot do in CHD-HF, acknowledging evidence gaps and lesion-specific cautions; use these agents in partnership with cardiology rather than reflexively applying adult HF playbooks (AHA-CHDHF-2024; ISHLT-PedsHF-2025)
Integrate nutrition as a primary therapy, not an afterthought: assess growth velocity, caloric needs, feeding fatigue, and when to push for early nutrition consultation and NG/GT support (AHA-CHDHF-2024)
Recognize end-organ dysfunction is common in CHD-HF (renal/hepatic/pulmonary) and incorporate that into medication choice, dosing vigilance, and escalation decisions (AHA-CHDHF-2024)

Escalate, Stabilize, and Know "When This Is ICU"¶

Identify red flags requiring ICU-level escalation (shock physiology, rising lactate, worsening mental status, escalating respiratory support, refractory arrhythmia, progressive end-organ dysfunction)
Differentiate cardiogenic shock from septic shock in a child with CHD and initiate immediate stabilizing actions while mobilizing cardiology/ICU support
Determine when escalation from oral to IV therapy is warranted (e.g., IV diuresis, IV inotropes under specialist guidance) and articulate the goal (improve perfusion, relieve congestion, bridge to intervention)
Recognize when to activate an "advanced HF" pathway (frequent HF admissions, persistent growth failure, inability to wean IV support, unacceptable quality of life, progressive end-organ injury) (AHA-CHDHF-2024; ISHLT-PedsHF-2025)

Advanced Therapies: VAD/ECMO/Transplant¶

Explain the basic roles and timelines of ECMO (short-term rescue) vs durable VAD (bridge to transplant/recovery) and what residents must do before/after initiation (infection screening, anticoagulation vigilance, neuro checks, end-organ monitoring) (ISHLT-MCS-2023; ISHLT-PedsVAD-2021)
Identify indications for transplant evaluation referral and the practical "resident actions" that improve candidacy (optimize nutrition/conditioning, monitor renal/hepatic function, ensure psychosocial support, medication adherence) (ISHLT-HTx-2024; AHA-CHDHF-2024)
Recognize CHD-specific transplant/VAD modifiers residents should flag early (pulmonary vascular disease, Fontan-associated liver disease, complex anatomy, prior surgeries, sensitization risk) and communicate these in handoffs (ISHLT-HTx-2024)

Communication, Goals of Care, and Systems¶

Deliver a clear family-facing explanation of CHD-HF: what HF means in their child's anatomy, what the meds are trying to accomplish, and what "worsening HF" looks like at home
Teach "return precautions" that actually prevent harm (increasing work of breathing, feeding intolerance, decreased urine output, syncope, chest pain, new cyanosis, severe lethargy)
Initiate or support goals-of-care discussions for Stage D/advanced HF and involve palliative care early for symptom support and decision-making—without framing it as "giving up" (AHA-CHDHF-2024; ISHLT-PedsHF-2025)
Coordinate multidisciplinary HF care (nutrition, pharmacy, social work, PT/OT, palliative care) and produce a safe discharge plan (med reconciliation, weight plan, follow-up timing, caregiver teach-back)
Plan transition elements for adolescents with HF/CHD: medication ownership, reproductive counseling basics, and linkage to adult congenital + advanced HF programs (AHA-CHDHF-2024)

Key Guidelines¶

AHA Scientific Statement (CHD-HF): Evaluation and Management of Chronic Heart Failure in Children and Adolescents With Congenital Heart Disease Circulation. 2024;150(2):e33–e50. Epub 2024-05-29 PMID: 38808502 | DOI: 10.1161/CIR.0000000000001245

ISHLT Pediatric HF Guideline Update (covers CHD + cardiomyopathy + acquired disease) J Heart Lung Transplant. 2025;44(10):e21–e71. Epub 2025-08-21 PMID: 40838915 | DOI: 10.1016/j.healun.2025.06.003

ISHLT Guideline: Evaluation and Care of Cardiac Transplant Candidates (includes pediatric considerations) J Heart Lung Transplant. 2024;43(10):1529–1628.e54. Epub 2024-08-08 PMID: 39115488 | DOI: 10.1016/j.healun.2024.05.010

ISHLT Mechanical Circulatory Support Guidelines (10-year update) J Heart Lung Transplant. 2023;42(7):e1–e222. Epub 2023-05-25 PMID: 37245143 | DOI: 10.1016/j.healun.2022.12.004

ISHLT Consensus Statement: Pediatric/CHD Patients on VADs (AHA-endorsed) J Heart Lung Transplant. 2021;40(8):709–732. Epub 2021-05-20 PMID: 34193359 | DOI: 10.1016/j.healun.2021.04.015

Quick Reference: Heart Failure Management Algorithm

Epidemiology¶

HF incidence rising among children/adolescents with CHD
CHD is leading cause for pediatric HF-related ED visits and hospitalizations
Improved surgical survival → more patients living with chronic HF
Distinct from cardiomyopathy-related HF

Pathophysiology¶

Chronic pressure overload (AS, PS, CoA)
Chronic volume overload (VSD, PDA, valvular regurgitation)
Ischemic damage (anomalous coronaries, surgical complications)
Post-operative complications (RV dysfunction, conduit failure)

Single Ventricle Physiology¶

Mitochondrial dysfunction
Oxidative stress
Chronic volume loading
All Fontan patients are functionally in HF

Comorbidities Worsening Outcomes¶

Arrhythmias
Chronic cyanosis
Pulmonary hypertension
Protein-losing enteropathy

HF Staging Framework¶

Stage	Description	Examples
A	At risk for HF	Unrepaired CHD, prior chemotherapy
B	Structural/functional abnormality without symptoms	Asymptomatic LV dysfunction
C	Symptomatic HF	Current or prior symptoms
D	Advanced/refractory HF	Requiring specialized interventions

Diagnostic Evaluation¶

Clinical Assessment¶

Growth parameters and nutritional status
Exercise tolerance (formal testing if able)
Feeding difficulties in infants
Respiratory symptoms

Laboratory¶

BNP/NT-proBNP (serial trending most useful)
CBC, CMP, liver function
Iron studies (high prevalence of deficiency)
Thyroid function

Imaging¶

Echocardiography (serial assessment)
Cardiac MRI (volumetric assessment, scar/fibrosis)
Cardiac catheterization (hemodynamics, filling pressures)

Medical Management¶

Pharmacotherapy¶

Drug Class	Agents	Considerations
ACE inhibitors	Captopril, enalapril, lisinopril	First-line; caution with renal dysfunction
ARBs	Losartan, valsartan	ACEi alternative; avoid combining
ARNI	Sacubitril/valsartan	Emerging pediatric data
Beta-blockers	Carvedilol, metoprolol	Limited pediatric CHD data
Diuretics	Furosemide, spironolactone	Symptom management
Digoxin	-	Limited role; narrow therapeutic window

Key Considerations¶

Evidence largely extrapolated from adult trials
Hepatic/renal dysfunction common in CHD (dose adjust)
Monitor electrolytes closely with diuretics
Be cautious with beta-blockers in single ventricle

Nutritional Support¶

Critical component of CHD-HF management: - High metabolic demands + poor oral intake = failure to thrive - NG/GT feeding often required - Caloric fortification of feeds - Monitoring growth velocity - Early nutrition consultation

Multidisciplinary Care¶

Essential team members: - Pediatric cardiologist - Heart failure specialist - Cardiac surgeon - Nutrition/dietitian - Social work - Palliative care (advanced stages) - Transplant team (when appropriate)

Advanced Therapies¶

Mechanical Circulatory Support¶

VAD (for bridge to transplant/recovery)
ECMO (short-term support)
See Topic 28: Heart Transplantation

Transplant Evaluation¶

Indications for referral: - Stage D heart failure - Refractory to medical therapy - Repeated hospitalizations - Unacceptable quality of life

Board Pearls¶

Pearl: CHD is the leading cause of pediatric HF hospitalizations

More common than cardiomyopathy in causing HF-related ED visits

Pearl: All Fontan patients are functionally in chronic HF

No subpulmonary ventricle = chronically elevated CVP + reduced CO

Pearl: Nutritional support is CRITICAL in pediatric HF

High metabolic demands with poor intake; early GT/NG feeding often needed

Self-Assessment¶

Q1: A 2-year-old with unrepaired large VSD presents with tachypnea, hepatomegaly, and poor weight gain. What is the most likely HF stage?

Answer

**Answer**: Stage C - Symptomatic heart failure **Rationale**: The child has structural heart disease (VSD with volume overload) AND current symptoms (tachypnea, hepatomegaly, failure to thrive). This meets criteria for Stage C HF.

Q2: Which medication class has the strongest evidence for reducing mortality in pediatric CHD-related HF?