Pediatric Heart Defects

Overview of Pediatric Cardiovascular Dysfunction

Instructor and Institution

B. Kent Wilson, MSN RN CNE
BridgeValley Community and Technical College
Nursing 234 - Spring 2026

Objectives

  1. Design a plan for assisting children during cardiac diagnostic procedures.

  2. Demonstrate an understanding of hemodynamics and distinctive manifestations.

  3. Outline a care plan for an infant or child in heart failure.

  4. Describe the care for a child who has hypoxia.

  5. Describe the care for an infant or child with a congenital heart defect and its surgical repair.

  6. Discuss the nurse’s role in helping the child and family cope with congenital heart disease.

  7. Differentiate between rheumatic fever and rheumatic heart disease.

  8. List the criteria for selected cholesterol screening for children.

  9. Discuss the assessment and management of hypertension in children and adolescents.

  10. Outline a care plan for a child with Kawasaki disease.

  11. Describe the emergency treatment for shock, including anaphylaxis.

Key Concepts

  • Perfusion

  • Oxygenation

  • Inflammation

QSEN Competencies

  • Patient-Centered Care

  • Teamwork and Collaboration

  • Evidence-Based Practice

  • Quality Improvement

Classification of Cardiovascular Dysfunction

Cardiovascular dysfunction in pediatric patients can be categorized into two major groups:

  1. Congenital Heart Disease (CHD)

    • Present at birth; characterized by abnormal cardiac function due to structural anomalies

  2. Acquired Cardiac Disorders

    • Develop after birth due to various factors including infections, autoimmune responses, environmental factors, and familial tendencies

Congenital Heart Disease (CHD)

CHD can be divided into two subcategories:

  • Congestive Heart Failure (CHF)

  • Hypoxia

Causes of Congenital Heart Disease

  • Chromosomal/Genetic: 10-12%

  • Maternal Environment: 1-2%

    • Drug or alcohol use

    • Fetal alcohol syndrome: 80% have CHD

  • Maternal Illness:

    • Rubella within first 7 weeks of pregnancy: 50% risk of defects (e.g., PDA, pulmonary branch stenosis)

    • Other viral illnesses (CMV, Toxoplasmosis)

    • Diabetes mellitus, Lupus: IDM has a 10% risk of CHD (VSD, cardiomyopathy, TGA)

  • Family History:

    • Previous child with congenital heart disease increases risk

    • History of genetic cardiac disease should prompt referral

Notable Genetic Syndromes Associated with CHD:

  • Down syndrome (Trisomy 21)

  • Turner syndrome (45X0)

  • Noonan syndrome (Chr 12)

  • Williams syndrome (Chr 7; involves elastin gene mutation)

Incidence

  • The incidence of CHD is approximately 5-8 per 1000 live births.

  • About 2 to 3 of these cases are symptomatic within the first year of life.

  • CHD is considered a major cause of death in the first year of life (following prematurity).

  • The most common anomaly is a ventricular septal defect (VSD), which occurs in 28% of children with CHD; many of these children also have recognized anomalies such as trisomy 21, 13, and 18.

Classification of Congenital Heart Disease

Old Classification Versus More Useful Classification

  • Acyanotic: Can present as cyanotic

  • Cyanotic: Can appear pink; may develop CHF

Hemodynamic Characteristics Classification

The classification system based on hemodynamic characteristics and blood flow patterns includes:

  1. Increased pulmonary blood flow

  2. Decreased pulmonary blood flow

  3. Obstruction of blood flow out of the heart

  4. Mixed blood flow

Increased Pulmonary Blood Flow Defects

Definition:

  • Abnormal connections exist between the two sides of the heart, leading to increased blood volume on the right side of the heart, resulting in increased pulmonary blood flow and decreased systemic blood flow.

Types of Increased Pulmonary Blood Flow Defects

  1. Atrial Septal Defect (ASD)

    • An abnormal opening between atria (left atrium to right atrium).

    • Types: Ostium primum (lower septum, may have mitral valve issues); Ostium secundum (mid-septum); Sinus venosus defect (near junction of SVC and right atrium).

Pathophysiology:

  • The blood shifts from the higher pressure left atrium to the lower pressure right atrium, leading to an increase in right ventricular volume.

Signs & Symptoms (S&S):

  • May be asymptomatic; possible development of heart failure and atrial arrhythmias.

Treatment:

  • Surgical Dacron patch closure or non-surgical repair through catheterization.

  1. Ventricular Septal Defect (VSD)

    • An abnormal opening between the ventricles.

    • It is noted that about 20% of VSDs close spontaneously during the first year of life.

Pathophysiology:

  • As blood flows from the left ventricle (higher pressure) to the right ventricle (lower pressure), this causes a left-to-right shunt, increasing pressure in the right ventricle and possibly leading to hypertrophy and failure.

S&S:

  • Very common to see congestive heart failure.

Treatment:

  • Complete surgical repair or implantation of a closure device during cardiac catheterization.

  1. Patent Ductus Arteriosus (PDA)

    • Failure of the fetal ductus arteriosus to close within a few weeks after birth.

Pathophysiology:

  • Blood from the aorta flows back into the pulmonary artery, causing increased pulmonary blood flow, which results in added workload on the left side of the heart and pulmonary vascular congestion.

Signs & Symptoms (S&S):

  • May present with a machinery-like murmur, bounding pulses, risk for bacterial endocarditis, pulmonary vascular obstructive disease.

Treatment:

  • Possible litigation or administration of indomethacin.

  1. Atrioventricular Canal (Endocardial Cushion Defect)

    • Overview including clinical manifestations, diagnostic procedures, clinical therapy, prognosis to be detailed.

Deficits That Obstruct Systemic Blood Flow

  • Coarctation of the Aorta

    • Localized narrowing near the ductus arteriosus leading to increased pressure proximal to the defect, causing hypertension.

    • Symptoms may include bounding pulses in arms, weak femoral pulses, and cool lower extremities.

    • Treatment: balloon angioplasty.

  • Aortic Stenosis

    • Narrowing of the aortic valve leading to LV hypertrophy; signs may include decreased cardiac output and exercise intolerance.

    • Treatment includes management of symptoms and may lead to significant complications.

  • Pulmonic Stenosis

    • Narrowed pulmonary valve leading to decreased pulmonary blood flow and potential right ventricular hypertrophy.

    • Seen as a loud systolic ejection murmur, requires balloon angioplasty.

Nursing Management Considerations

For children with cardiac defects, nursing considerations will include anxiety management, potential for impaired growth and development, risk of infections, and ensuring adequate nutrition and oxygenation.

Evaluation and Teaching Plans

Prioritize teaching for parents, assess their anxiety, provide necessary literature, and ensure that teaching suits their child's developmental level, particularly before procedures such as cardiac catheterization.