Chapter 26: The Child with a Cardiovascular Disorder

The Cardiovascular System in Children

The cardiovascular system is essential for transporting blood, oxygen, and nutrients throughout the body while also facilitating the removal of waste
products. Here’s an overview of the components and changes that occur in the cardiovascular system, especially at birth, and how it differs from that of
adults.

Components of the Cardiovascular System

1. Heart:
○ The heart is a muscular organ that pumps blood throughout the body.
○ It consists of four chambers: the right atrium, right ventricle, left atrium, and left ventricle.
2. Blood:
○ Blood is the fluid that carries oxygen, nutrients, hormones, and waste products.
○ It comprises red blood cells, white blood cells, platelets, and plasma.
3. Blood Vessels:
○ Blood vessels include arteries, veins, and capillaries, which are responsible for carrying blood to and from the heart and
throughout the body.

Changes at Birth

● At birth, significant physiological changes occur as the newborn transitions from fetal circulation, which relies on the placenta for oxygen
and nutrient exchange, to independent pulmonary circulation.
● Key changes include:
○ Closure of the ductus arteriosus: This structure connects the pulmonary artery to the aorta and allows blood to bypass the lungs
in utero. After birth, it closes and becomes the ligamentum arteriosum.
○ Closure of the foramen ovale: This opening between the right and left atria allows blood to bypass the lungs. After birth, it
usually closes due to increased pressure in the left atrium compared to the right.
○ Initiation of breathing leads to increased blood flow to the lungs, allowing for oxygenation.

Differences Between Pediatric and Adult Cardiovascular Systems

1. Heart Size and Rate:

○ A child's heart is smaller in size compared to an adult's heart, resulting in a higher heart rate.
○ The resting heart rate for infants can range from 100 to 160 beats per minute, gradually decreasing as they grow.
2. Blood Volume:
○ Total blood volume in children is less than in adults, averaging about 70-80 mL/kg in infants, compared to approximately 70
mL/kg in adults.
3. Cardiac Output:
○ Cardiac output (the amount of blood pumped by the heart per minute) is more reliant on heart rate in infants and young children
than in adults, who have a more stable stroke volume.
4. Vascular Resistance:
○ Systemic vascular resistance is lower in infants and children compared to adults, allowing for easier blood flow.
5. Developmental Considerations:
○ Children’s cardiovascular systems continue to develop throughout early life, with structural and functional changes occurring as
they grow. This includes growth in heart size, changes in heart rhythm, and adaptations in response to physical activity.
6. Physiological Immaturity:
○ Infants and young children exhibit greater variability in blood pressure and heart rate in response to stressors and
environmental changes compared to adults.
Signs Related to Suspected Cardiac Pathology in Infants

When assessing infants for potential congenital heart defects, nurses should be vigilant for specific signs and symptoms that may indicate underlying
cardiac issues. While the manifestations can vary based on the specific condition, the following observations are commonly associated with many
congenital cardiac problems:

1. Failure to Thrive or Poor Weight Gain:

○ Infants may exhibit inadequate growth and weight gain due to increased metabolic demands and decreased caloric intake, often
resulting from fatigue during feeding.
2. Cyanosis and Pallor:
○ Cyanosis refers to a bluish discoloration of the skin, particularly around the lips and extremities, indicating inadequate
oxygenation.
○ Pallor may also be observed, suggesting poor perfusion or anemia.
3. Visually Observed Pulsations in the Neck Veins:
○ Pulsations in the neck veins may be indicative of increased central venous pressure and are often seen in cases of right heart
failure or fluid overload.
4. Tachypnea and Dyspnea:
○ Tachypnea (rapid breathing) is common due to increased work of breathing and may be accompanied by dyspnea (shortness
of breath), indicating respiratory distress.
5. Irregular Pulse Rate:
○ An irregular pulse may suggest arrhythmias or hemodynamic instability, requiring further evaluation.
6. Clubbing of Fingers:
○ Finger clubbing is characterized by an abnormal enlargement of the fingertips and may indicate chronic hypoxia or underlying
cardiac shunts.
7. Fatigue During Feeding or Activity:
○ Infants may tire easily during feeding, leading to inadequate intake and further contributing to failure to thrive. Parents may
report that the infant is lethargic or less active than expected.
8. Excessive Perspiration, Especially Over the Forehead:
○ Increased sweating, particularly on the forehead, can be a sign of cardiac distress, indicating sympathetic nervous system
activation in response to poor oxygenation or heart failure.

Nursing Considerations

● Assessment: Regular and thorough assessments should be performed to monitor for these signs, especially in infants known to have risk
factors for congenital heart disease.
 ● Documentation: Accurate documentation of observations and any changes in the infant's condition is critical for ongoing evaluation and
intervention.
● Reporting: Promptly report any concerning findings to the healthcare provider for further investigation and potential intervention.
● Family Education: Educating parents about signs to watch for can help in early identification and management of cardiac issues.

Nursing Tip: Bradycardia may be a sign that cardiovascular arrest is imminent in children with hypoxia.

Congenital Heart Defects

Congenital heart defects (CHDs) are structural abnormalities of the heart that are present at birth. They can arise from genetic factors, maternal health
issues (such as drug use or infections like rubella), or environmental influences. Early detection and management of these conditions are crucial for the
health of affected infants.

Pathophysiology

● Fetal Circulation: During fetal development, the fetus relies on the mother's circulation for oxygen and nutrients, which is facilitated by
specialized structures like the placenta. Consequently, congenital heart defects may not significantly affect the fetus.
● Transition at Birth: At birth, the infant's circulatory system must assume responsibility for oxygenating the body. Any anatomical heart
defect or open fetal pathways (such as patent ductus arteriosus) can lead to inadequate oxygenation and result in clinical symptoms.
● Prevalence: CHDs occur in approximately 2 to 3 per 1,000 live births, and about 50% of these infants exhibit signs and symptoms before
reaching one year of age. Some defects, like mitral valve prolapse, may manifest later in life .
● Mortality: Heart defects are the leading cause of death in the first year of life among congenital anomalies. Therefore, proactive prenatal
care and regular well-baby checkups are essential to detect potential issues early.

Diagnosis

Diagnosis of congenital heart defects typically involves:

● Clinical Assessment: Observations of symptoms, including poor weight gain, cyanosis, and abnormal heart sounds.
● Diagnostic Tests: Echocardiography, chest X-rays, electrocardiograms (ECGs), and cardiac catheterization can provide further insight into
the structural and functional aspects of the heart.

Common Symptoms of Congenital Heart Defects

● Cyanosis
● Tachypnea
● Fatigue during feeding
● Heart murmurs
● Failure to thrive

Treatment

The management of congenital heart defects often requires surgical intervention:

● Surgical Approaches:
○ Thoracotomy: A surgical incision in the chest to access the heart.
○ Cardiopulmonary Bypass: During surgery, this machine takes over the heart's pumping function, allowing surgeons to operate
on a still heart while oxygenating the blood.
○ Hypothermia: The body’s temperature may be lowered to decrease metabolic demands and oxygen needs during surgery.
● Heart Transplant: In severe cases (e.g., hypoplastic left heart syndrome or a three-chambered heart), a heart transplant may be
necessary.
● Interventional Cardiac Catheterization: Some defects, like atrial septal defects (ASDs) or ventricular septal defects (VSDs), can be
corrected using less invasive techniques, such as cardiac catheterization.

Classification of Congenital Heart Defects

Congenital heart defects (CHDs) can be classified based on their impact on blood circulation, specifically how they affect the flow of oxygenated and
deoxygenated blood. The two main categories are cyanotic and acyanotic defects. However, a more precise classification considers the
hemodynamic effects, which is essential for understanding the physiological implications of each defect.

1. Hemodynamic Classification
 ● Cyanotic Defects: These defects result in a significant amount of deoxygenated blood entering systemic circulation, leading to cyanosis (a
bluish discoloration of the skin). Examples include:
○ Tetralogy of Fallot
○ Transposition of the great arteries
○ Tricuspid atresia
● Acyanotic Defects: These defects generally do not cause cyanosis because they do not significantly mix deoxygenated blood with
oxygenated blood in the systemic circulation. Examples include:
○ Atrial septal defect (ASD)
○ Ventricular septal defect (VSD)
○ Patent ductus arteriosus (PDA)

Physiological Effects on Blood Flow

Congenital heart defects can be organized based on their effect on blood flow as follows:

● Lesions that Increase Pulmonary Blood Flow:

○ These defects cause blood to flow from the left side of the heart to the right side, resulting in excess blood circulating through
the lungs.
○ Pathophysiology: Blood flows from areas of high pressure to low pressure, and in these cases, oxygenated blood from the left
atrium moves back to the right atrium through a defect, such as ASD or VSD. This leads to increased pulmonary blood flow,
which can eventually cause pulmonary congestion.
○ Examples:
■ Atrial Septal Defect (ASD): Blood flows from the left atrium to the right atrium, leading to increased right atrial and
right ventricular volumes.
■ Ventricular Septal Defect (VSD): Blood flows from the left ventricle to the right ventricle, causing increased
pressure and volume in the right ventricle and lungs.
■ Patent Ductus Arteriosus (PDA): The ductus arteriosus fails to close after birth, allowing blood to flow from the
aorta to the pulmonary artery.
● Lesions that Restrict Blood Flow:
○ These defects impede the normal flow of blood through the heart or great vessels, leading to reduced cardiac output.
○ Examples:
■ Coarctation of the Aorta: A narrowing of the aorta that can lead to higher blood pressure in the upper body and
lower blood pressure in the lower body.
■ Aortic Stenosis: A narrowing of the aortic valve that restricts blood flow from the left ventricle to the aorta.
● Lesions that Decrease Pulmonary Blood Flow:
○ These defects restrict the amount of blood that reaches the lungs for oxygenation, resulting in decreased pulmonary blood flow
and often leading to cyanosis.
○ Examples:
■ Tetralogy of Fallot: This condition includes four defects, one of which is a right ventricular outflow tract obstruction
that decreases blood flow to the lungs.
■ Tricuspid Atresia: The tricuspid valve fails to develop, leading to no direct connection between the right atrium and
ventricle, severely limiting blood flow to the lungs.
● Mixed Blood Flow Lesions:
○ These defects lead to a mixture of oxygenated and deoxygenated blood due to abnormal connections between the chambers of
the heart or vessels.
○ Examples:
■ Transposition of the Great Arteries: The aorta and pulmonary artery are switched, leading to two separate
circulations that do not adequately mix.

Safety Alert!: In congenital heart disease, cyanosis is not always a clinical sign.

Overview of Common Congenital Heart Defects

1. Atrial Septal Defect (ASD)

ASD involves an abnormal opening between the right and left atria, allowing oxygenated blood from the left atrium to flow back into the right atrium.
Though many patients are asymptomatic, a heart murmur may be detected during routine examinations.

● Diagnosis: Cardiac catheterization, electrocardiogram (ECG), and echocardiography.

● Treatment:
○ Spontaneous Closure: Some ASDs close on their own.
○ Surgical Repair: Dacron patch or repair using a portion of the pericardium. Robotic surgery or open cardiac surgery may be
required.
○ Nonsurgical Repair: Can sometimes be achieved via cardiac catheterization.
○ Post-Surgical Care: Low-dose aspirin is typically prescribed for 6 months following surgery.
● Complications: Risk of stroke if untreated.
 ● Prognosis: Excellent after treatment.

2. Ventricular Septal Defect (VSD)

VSD is the most common congenital heart anomaly. It features an opening between the right and left ventricles, causing oxygenated blood from the left
ventricle to flow into the right ventricle (left-to-right shunt).

● Symptoms: A loud, harsh murmur, often accompanied by a systolic thrill. Severity varies.
● Diagnosis: A murmur is typically detected during a physical examination, confirmed by imaging.
● Treatment:
○ Spontaneous Closure: Often occurs in small defects during the first year of life.
○ Surgical Repair: The defect is closed with sutures or a synthetic patch, often performed under hypothermia with a heart-lung
bypass machine.
● Prognosis: Excellent, with normal growth and development typically achieved within 1-2 years after surgery.

3. Patent Ductus Arteriosus (PDA)

PDA occurs when the ductus arteriosus, a fetal vessel that connects the pulmonary artery to the aorta, fails to close after birth, leading to oxygenated
blood flowing back into the lungs from the aorta.

● Symptoms: Dyspnea, full and bounding pulse, wide pulse pressure, and a characteristic machinery murmur.
● Diagnosis: Two-dimensional echocardiography visualizes the PDA and determines blood flow.
● Treatment:
○ Medication: Premature infants with hypoxia may respond to intravenous indomethacin or ibuprofen to close the PDA.
○ Surgical Closure: Performed via visual-assisted thoracoscopic surgery (VATS), where the PDA is ligated.
○ Nonsurgical Options: Insertion of coils via catheterization to occlude the PDA.
○ Medication to Maintain Patency: Prostaglandin E1 can be used to keep the ductus open if needed before surgery for
conditions like hypoplastic heart.
● Prognosis: Excellent.

4. Coarctation of the Aorta

Coarctation involves the narrowing of the aorta, leading to increased blood pressure before the constriction and decreased pressure afterward.

● Symptoms: Significant difference in blood pressure and pulse strength between the upper and lower extremities. In severe cases,
symptoms may not appear until later in childhood.
● Diagnosis: X-rays may show cardiac enlargement and "notching" of the ribs, and echocardiography can confirm the diagnosis.
● Treatment:
○ Medical Management: Infants with congestive heart failure (CHF) are stabilized with medications before surgery.
○ Surgical Repair: Narrowed portions of the aorta are resected and joined through an anastomosis.
○ Balloon Angioplasty: Preferred for older children, with stent insertion if necessary.
○ Postsurgical Care: Monitoring for complications such as hypertension, gastrointestinal symptoms, and restenosis. Balloon
angioplasty can relieve restenosis if it occurs.
○ Complications: Hypertension, CHF, and infective endocarditis if untreated.
● Prognosis: Good if there are no other defects and surgery is performed at an optimal time.

Nursing Tip: The systolic blood pressure is normally 10 to 15 mm Hg higher in the legs than the arms. Systolic blood pressure that is lower in the legs
than in the arms should be reported, as this could be a sign of coarctation of the aorta.
Safety Alert!: A significant difference in the blood pressure between the upper extremities and the lower extremities is a characteristic sign of
coarctation of the aorta.

Defects that Decrease Pulmonary Blood Flow

Defects that decrease pulmonary blood flow allow unoxygenated blood to enter the aorta and general circulation, leading to cyanosis due to the
presence of deoxygenated blood. One of the most significant congenital heart defects in this category is Tetralogy of Fallot.

Tetralogy of Fallot (ToF)

Tetralogy of Fallot consists of four specific anatomical defects that contribute to reduced pulmonary blood flow:
 1. Pulmonary Stenosis: Narrowing of the pulmonary artery, which limits blood flow to the lungs.
2. Right Ventricular Hypertrophy: Thickening of the right ventricle due to increased workload from pumping blood through the narrowed
artery.
3. Dextroposition of the Aorta: The aorta is positioned over the ventricular septal defect (VSD) and receives blood from both ventricles,
including unoxygenated blood.
4. Ventricular Septal Defect (VSD): An opening between the right and left ventricles that allows the mixing of oxygenated and unoxygenated
blood.

Clinical Manifestations

● Cyanosis: Cyanotic episodes increase with age as unoxygenated blood enters the systemic circulation.
● Clubbing of Fingers and Toes: Caused by chronic hypoxia.
● Squatting Position: Children may squat to improve blood flow and ease breathing; this position alters systemic venous return and helps
alleviate symptoms.
● Failure to Thrive: Due to feeding difficulties, increased energy expenditure, and growth retardation.
● Frequent Respiratory Infections: Increased vulnerability due to compromised circulation.
● Tet Spells: Sudden episodes of profound cyanosis, weakness, respiratory distress, and syncope. These episodes can last from a few
minutes to several hours, after which the child may feel lethargic. It’s essential for caregivers to position the child in a knee-chest position
during these episodes to improve venous return and reduce symptoms.

Diagnosis

● Chest X-ray: Reveals a characteristic "boot-shaped" heart due to right ventricular hypertrophy.
● Electrocardiogram (ECG): Provides information on heart rhythm and morphology.
● Echocardiography: Three-dimensional echocardiography helps visualize the heart’s anatomy and blood flow.
● Cardiac Catheterization: Assists in confirming the diagnosis and evaluating the severity of the defects.

Complications

● Cerebral Thrombosis: Thickened blood due to polycythemia (an increase in red blood cells) can lead to blood clots, especially if
dehydration occurs.
● Iron Deficiency Anemia: Can develop from decreased appetite and increased energy demands during feeding.
● Bacterial Endocarditis: Higher risk due to abnormal blood flow; prophylactic antibiotic therapy is necessary before certain procedures or
surgeries.

Treatment

● Immediate Management: Treatment aims to increase pulmonary blood flow and alleviate hypoxia.
○ Blalock-Taussig Shunt: A temporary surgical shunt can be performed on newborns or premature infants to increase blood flow
to the lungs.
● Surgical Correction: Open-heart surgery is typically performed between 4 months and 2 years of age to correct all defects, with excellent
outcomes expected.
● Postoperative Care: Monitoring for signs of congestive heart failure, irregular heartbeat, and other complications is critical.

et position. Infants and children with

tetralogy of Fallot can have paroxysmal hypercyanotic or “tet” spells. Placing the child in the tet position (a knee-chest position) relieves these
symptoms. Older children will spontaneously squat when a tet spell occurs.

Nursing Tip: There are four defects in tetralogy of Fallot:

1. Pulmonary artery stenosis
2. Hypertrophy of the right ventricle
3. Dextroposition of aorta
4. VSD

Defects that Cause Mixed Pathology

Hypoplastic Left Heart Syndrome (HLHS) is a critical congenital heart defect characterized by the underdevelopment of the left side of the heart.
This condition is particularly complex and requires immediate attention and management to ensure the survival of affected infants.

Overview of Hypoplastic Left Heart Syndrome

● Definition: HLHS involves a poorly developed left ventricle and hypoplasia of the ascending aorta. The heart’s left side is unable to
effectively pump oxygenated blood to the body.
● Diagnosis: HLHS can often be diagnosed prenatally through ultrasound. Early diagnosis allows for placement on a transplant list,
preparing for necessary surgical interventions immediately after birth.

Physiological Mechanisms

● Dependence on Patency: For infants with HLHS, survival initially relies on the presence of a patent foramen ovale (a natural opening
between the atria) and a patent ductus arteriosus (a vessel connecting the pulmonary artery and aorta). These structures allow oxygenated
blood to flow to the body.
● Prostaglandin E1 Administration: Prostaglandin E1 is given to maintain the patency of the ductus arteriosus, ensuring adequate blood
flow until surgical intervention.

Clinical Manifestations

● Cyanosis: Infants often present with a grayish-blue coloration of the skin and mucous membranes, indicating inadequate oxygenation.
● Congestive Heart Failure (CHF) Symptoms: Signs may include:
○ Dyspnea: Difficulty breathing due to inadequate blood circulation.
○ Weak Pulses: Reduced perfusion can lead to weak pulses.
○ Cardiac Murmur: Abnormal heart sounds may be noted during auscultation.
● Other Anomalies: Infants with HLHS may have other congenital heart defects or anomalies that need assessment and management.

Management and Surgical Intervention

● Initial Support: Immediate medical management focuses on maintaining circulation and oxygenation. Without surgical intervention,
survival beyond the first few months is rare.
● Surgical Procedure: A three-stage surgical approach is typically required:
1. Norwood Procedure: Performed in the first week of life, it reconstructs the heart to improve blood flow.
2. Glenn Procedure: Performed between 4-6 months, it reduces the workload on the heart by connecting the superior vena cava
to the pulmonary artery.
3. Fontan Procedure: Usually performed around age 2-4, it directs systemic venous return to the pulmonary arteries.
● Heart Transplant: In cases where surgical repair isn’t viable or fails, heart transplantation becomes a critical option. Early referral to a
transplant center is essential.

Postoperative Care and Nursing Management

1. Monitoring: Post-surgery, infants are monitored closely in an intensive care unit (ICU) for complications such as bleeding, infection, and
organ rejection.
2. Supportive Care: Nurses provide routine care, monitor vital signs, and ensure the child is comfortable and stable.
3. Immunosuppressive Therapy: After transplantation, infants require medications to prevent organ rejection.
4. Family Education: Parents need guidance on recognizing signs of complications, administering medications, and ensuring follow-up
appointments.
5. Nutritional Support: Adequate nutrition is crucial for growth and development, with attention to preventing anemia and dehydration.

General Treatment Considerations for Congenital Heart Defects

● Advances in Care: Technological improvements have made less invasive procedures, such as catheterizations for valvuloplasty and
angioplasty, viable alternatives to traditional open-heart surgeries.
● Family Support and Education: It’s vital to provide emotional support to families and educate them about managing their child’s condition,
including:
 ○ Health Maintenance: Emphasizing hygiene, nutrition, and regular check-ups.
○ Activity Restrictions: Avoiding competitive sports and overexertion to prevent stress on the heart.
○ Preventive Measures: Ensuring appropriate vaccinations and dental care with antibiotic prophylaxis to prevent infections.

Medication Safety Alert!: Complementary and alternative medicine (CAM) therapy with ginkgo, ginseng, and St. John’s wort may interact with drugs
used for congenital heart disease and should not be used

Acquired Heart Disease

● Definition: A cardiac issue that develops after birth.

● Causes: May result from:
○ Complications of congenital heart disease
○ Respiratory infection
○ Sepsis
○ Hypertension
○ Severe anemia
● Heart Failure: Occurs when cardiac output is insufficient to meet the body’s metabolic needs.

Congestive Heart Failure (CHF):

● Right-sided CHF:
○ Function: Moves unoxygenated blood to the pulmonary circulation.
○ Failure: Causes backup of blood in the systemic venous system.
● Left-sided CHF:
○ Function: Moves oxygenated blood from the lungs to the systemic circulation.
○ Failure: Results in blood backing up into the lungs.
● Compensation:
○ Peripheral vasoconstriction: Leads to cold or blue hands and feet, tachycardia, and tachypnea.
○ Progression: Failure usually begins on one side but eventually affects both sides.

Manifestations in Infants:

● Cyanosis or pallor
● Rapid respiration (tachypnea)
● Rapid pulse (tachycardia)
● Feeding difficulties
● Fatigue and weak cry
● Excessive sweating, especially on the forehead
● Failure to gain weight
● Edema
● Frequent respiratory infections

Safety Alert!: The following early signs of CHF in infants should be reported:
• Tachycardia at rest
• Fatigue during feeding
• Sweating around scalp and forehead
• Dyspnea
• Sudden weight gain

Cyanosis:

● Nurse observes if cyanosis is general or localized (e.g., hands, feet, lips, or around the mouth).
● Depth and duration: Record if cyanosis is deep, light, constant, or transient.
● Color changes during crying are significant.
● African American infants: Check palms and soles for cyanosis.
● Clubbing of fingers/toes: May result from chronic hypoxia.
● Other signs: Pale or mottled skin, sweating (especially on the head).

Rapid Respiration (Tachypnea):

● Defined as more than 60 breaths per minute in a newborn at rest, indicating distress.
● Accompanied by:
○ Dyspnea (shortness of breath)
 ○ Flaring nostrils
○ Mouth breathing, grunting
○ Sternal retractions
○ Difficulty breathing when lying flat
● Air hunger: Evident by irritability and restlessness.
● Weak, hoarse cry.

Rapid Pulse (Tachycardia):

● One of the first signs of CHF: Heart beats faster to increase oxygen supply to tissues.
● Infant’s compensation: Increase in heart rate (due to limited ability to increase stroke volume).

Feeding Difficulties:

● Infants tire easily during feeding and may stop sucking after a few ounces.
● Choking, gagging, and difficulty breathing while feeding.

Poor Weight Gain:

● Failure to gain weight.

● Sudden weight increase may indicate edema and the start of heart failure.

Edema:

● Decreased kidney function leads to fluid and sodium retention.

● Signs: Puffiness around the eyes, legs, feet, and abdomen.
● Decreased urine output.

Frequent Respiratory Tract Infections:

● Infants are at higher risk due to compromised heart and lung function.
● Prevention: Immunizations are reviewed and updated, and exposure to sick children is minimized.

Treatment and Nursing Care:

● Goals:
○ Reduce heart workload.
○ Improve respiration.
○ Maintain nutrition.
○ Prevent infection.
○ Reduce anxiety.
○ Support and instruct parents.
● Care Organization: Minimize disturbances, e.g., delay baths/linen changes if not necessary.
● Feeding: Small, frequent feedings; soft nipple with large holes; high-calorie formulas (24 cal/oz).
● Oxygen administration: Relieves dyspnea and helps the infant relax.
● Comforting care: Soft voice, gentle care, holding the infant during feeding.

Medication Management:

● Digitalis (e.g., Lanoxin):

○ Nursing considerations: Count pulse for 1 minute before administering; withhold if pulse < 100 bpm (infants) or < 70 bpm
(older children).
○ Toxicity signs: Nausea, vomiting, irregular pulse, anorexia.
○ Medication safety: Two nurses verify dose; reconfirm dose over 0.05 mg.
● ACE Inhibitors (Captopril, Enalapril, Lisinopril):
○ Watch for hypotension, cough, renal dysfunction, hyperkalemia.
● Diuretics (e.g., Furosemide, Spironolactone):
○ Monitor electrolytes, particularly potassium levels.
○ Weigh diapers for urine output, and monitor daily weight.
● Other medications: β-Adrenergic agonists, resynchronization pacing for left ventricular failure in ICU settings.

Monitoring:
 ● Input/output records are essential.
● Signs of dehydration: Thirst, dry skin, poor skin turgor, decreased urination.
● Pneumonia prevention: Regular position changes to avoid hypostatic pneumonia.

Family Support:

● Support parents and provide reassurance without minimizing the condition.

● Integrating children with heart conditions into family life helps build a healthy personality.
● Rest: Routine naps and early bedtimes are encouraged.
● Activity limitation: Children typically self-regulate activity levels as they grow.

Medication Safety Alert!: Two nurses should check dosage of drugs such as digoxin. A single dose larger than 0.05 mg, or 50 mcg, should be
reconfirmed with the health care provider.
Medication Safety Alert!: Before administering a digoxin medication, the resting apical pulse should be counted for 1 full minute.

Rheumatic Fever (RF)

Rheumatic Fever (RF) is a systemic inflammatory disease that affects various tissues, including the joints, heart, central nervous system (CNS), skin,
and subcutaneous tissues. It primarily affects children between 5 and 15 years of age and is a serious complication of untreated group A
beta-hemolytic streptococcus (GAS) throat infections. Early detection and treatment of strep throat infections are critical in preventing the
development of RF.

Pathophysiology:

RF damages connective tissues, with a particular effect on the heart, often causing scarring and potential malfunction of the mitral valve. This disease
is associated with immune-mediated inflammation and primarily occurs in low-income or overcrowded areas, especially during winter and spring
seasons.

Manifestations:

1. Migratory Polyarthritis:
○ Involves larger joints such as the knees, elbows, and ankles.
○ Joints become painful, tender, and swollen but resolve without permanent damage.
2. Skin Eruptions (Erythema Marginatum):
○ Rash with red margins and pale centers appearing mainly on the trunk.
3. Chorea (Sydenham’s Chorea):
○ A nervous disorder with involuntary muscle movements, seen more in girls before puberty.
○ Children may have behavioral changes and clumsiness.
4. Rheumatic Carditis:
○ Involves the heart and can lead to inflammation of the myocardium, pericardium, or endocardium.
○ Mitral stenosis and Aschoff’s bodies (myocardial lesions) can develop.
5. Fever and Fatigue:
○ Pallor, anorexia, and possible nosebleeds.
○ Symptoms range from mild to severe and may develop 1-6 weeks after a streptococcal infection.

Diagnosis:

The diagnosis of RF is guided by Jones criteria, which require the presence of two major criteria (e.g., carditis, polyarthritis, chorea) or one major and
two minor criteria (e.g., fever, elevated ESR), along with evidence of a recent streptococcal infection. Laboratory tests such as antistreptolysin O
(ASO) titers, ESR, and C-reactive protein levels help confirm the diagnosis.

Treatment and Nursing Care:

The treatment focuses on:

● Elimination of Streptococcal Infection:

○ Antibiotics like penicillin or erythromycin.
● Anti-inflammatory Therapy:
○ Aspirin for joint pain and steroids for severe carditis.
● Bed Rest:
○ During the acute phase to reduce the workload on the heart.
● Management of Chorea:
 ○ Phenobarbital to control muscle spasms and emotional support for behavioral changes.
● Cardiac Failure Management:
○ If heart failure occurs, supportive measures including diuretics, and in severe cases, advanced treatments like digoxin may be
needed.

Nursing care includes:

● Encouraging rest to reduce heart strain.

● Providing emotional support to the child and family, as RF can cause long-term anxiety.
● Educating the family about the importance of completing antibiotics and adhering to follow-up care.
● Preventing infections, especially subacute bacterial endocarditis, by ensuring proper oral hygiene and administering prophylactic
antibiotics before dental procedures.

Prevention:

Preventing RF primarily involves treating streptococcal throat infections promptly with antibiotics and educating families on the importance of
completing the prescribed treatment even if symptoms improve.

Manifestations of rheumatic fever.

Nursing Tip: The nurse should teach parents about the need for prophylactic antibiotic therapy before any dental procedure.

Systemic Hypertension

Systemic Hypertension in children and adolescents is becoming more prevalent and is a critical condition requiring early detection and management to
prevent complications in adulthood, such as renal disease, stroke, or left ventricular dysfunction.

Pathophysiology:

Hypertension (high blood pressure) results from an increase in cardiac output or peripheral vascular resistance. Blood pressure naturally rises with age
and varies according to age, sex, and height. It is categorized as:

● Normal blood pressure: Below the 90th percentile for age, height, and sex (or <120/80 mm Hg in adolescents).
● Elevated blood pressure: Between the 90th and 95th percentile + 11 mm Hg (or 120/80–129/80 mm Hg in adolescents).
● Stage 1 hypertension: Above the 95th percentile to 95th percentile + 12 mm Hg (or 130–139/80–89 mm Hg in adolescents).
● Stage 2 hypertension: Above the 95th percentile + 12 mm Hg, or ≥140/90 in adolescents.
Hypertension can be primary (essential, with no underlying cause) or secondary (caused by other conditions such as renal, congenital, vascular, or
endocrine disorders). Risk factors include heredity, obesity, stress, poor diet, and lack of exercise. Emerging evidence suggests that essential
hypertension may begin in childhood, possibly even during fetal development.

Manifestations:

High blood pressure in children is often asymptomatic and discovered during routine exams. The American Academy of Pediatrics recommends
confirming abnormal readings with auscultation on three different occasions before diagnosing hypertension.

Treatment and Nursing Care:

1. Lifestyle Management:
○ Nutritional Counseling: Aimed at reducing weight, sodium intake, and improving diet.
○ Exercise: A structured aerobic exercise program tailored to the child’s age is recommended.
○ Dietary Approaches to Stop Hypertension (DASH): A low-sodium, plant-based diet that emphasizes high-potassium, calcium,
and magnesium foods (e.g., fruits, vegetables, low-fat dairy, fish, poultry).
○ Limit sedentary activities (e.g., screen time) to 2 hours per day and encourage 30-60 minutes of physical activity daily.
2. Counseling on Drug, Alcohol, and Tobacco Use: Adolescents should be educated about the negative effects of these substances
on blood pressure.
3. Pharmacological Management:
○ In stage 2 hypertension, medications may be required, such as:
■ ACE inhibitors (e.g., captopril)
■ Calcium channel blockers (e.g., felodipine)
■ Angiotensin receptor blockers (ARBs) (e.g., candesartan)
■ Thiazide diuretics
○ These drugs are added if lifestyle changes alone are insufficient in reducing blood pressure.
4. Non-compliance Support: Adolescents often struggle with long-term adherence to diet, medication, and exercise plans. Nurses should
provide positive support to encourage compliance and successful outcomes.

Prevention:

The focus of prevention is on education. Nurses can:

● Promote awareness through school programs, parent-teacher meetings, and community health fairs.
● Encourage regular blood pressure screenings during annual physical exams.
● Address risk factors, such as obesity, high cholesterol, inactivity, and use of drugs, alcohol, or tobacco.

Nursing Tip: The assessment of blood pressure levels should be part of every physical examination during childhood for children over 3 years of age.
Children under 3 years of age with comorbidities, such as prematurity or genetic anomalies, should have blood pressure assessed at every clinic visit.
Nursing Tip: Using the proper size blood pressure cuff is essential to obtaining an accurate blood pressure in children. The bladder length of the cuff
should be 80% to 100% of the circumference of the arm, and the width should be at least 40%

Hyperlipidemia

Hyperlipidemia refers to an excessive level of lipids (fats) in the blood, which can increase the risk of cardiovascular disease later in life. It is
particularly important to monitor and manage lipid levels in children, especially those with a family history of high cholesterol or early cardiac death.

Lipoproteins:

Lipoproteins are made up of lipids and proteins and are responsible for transporting fats in the bloodstream. The two main types are:

● Low-Density Lipoproteins (LDLs): These contain lower amounts of triglycerides but higher levels of cholesterol and some protein. LDLs
transport cholesterol to the cells for use in metabolism and steroid production but can lead to plaque buildup in arteries if levels are too
high, increasing the risk of heart disease.
● High-Density Lipoproteins (HDLs): These contain higher levels of protein and lower amounts of triglycerides and cholesterol. HDLs carry
cholesterol from the bloodstream back to the liver for excretion, reducing the risk of plaque formation.

Risk Factors and Screening:

Children with a parental history of high cholesterol (levels exceeding 240 mg/dL) or a family history of early cardiac death (before age 55) should
be screened for high cholesterol at an early age. Hyperlipidemia is influenced by genetics, but lifestyle factors such as diet and physical activity also
play a crucial role.
Screening for hyperlipidemia is important because adult cardiovascular disease risk factors, like high cholesterol, can begin in fetal life—especially if
the mother is diabetic or obese. Additionally, high-fat diets, obesity, and a sedentary lifestyle during childhood contribute to an increased risk of
cardiovascular disease later in life.

The National Lipid Association (NLA) and the American Academy of Pediatrics (AAP) recommend:

● Universal cholesterol screening between the ages of 9 and 11 and again at ages 17 and 21.
● Earlier screening (as early as age 2) for children with a family history of high cholesterol or cardiovascular disease.

In children with risk factors such as diabetes, hypertension, tobacco use, obesity, or a family history of heart disease, nonfasting lipid screening
may be conducted at younger ages.

Management and Prevention:

● Lifestyle Changes: The initial approach to managing borderline or high lipid levels in children is lifestyle modification rather than immediate
treatment. This includes:
○ Dietary adjustments: Emphasizing a low-fat, high-fiber diet.
○ Physical activity: Encouraging regular exercise.
○ Calorie reduction: For obese children with high triglycerides, reducing calorie intake is essential.
● These changes can help control lipid levels and promote cardiovascular health. Children younger than 2 years of age should not follow a
fat-restricted diet, as fats and calories are necessary for central nervous system (CNS) development.
● Family Education: Education and involvement of the entire family in adopting healthy eating habits and physical activity is critical. A
family-based approach to lifestyle changes improves adherence and long-term health outcomes for the child.

Prevention:

● Healthy Eating: Encouraging lifelong heart-healthy eating habits for the entire family.
● Physical Activity: Promoting active lifestyles and reducing sedentary behaviors, such as excessive screen time.
● Annual Screenings: Regular health checks to monitor blood lipid levels and assess risk factors.

Heart-Healthy Guidelines for Children

Infants
● Provide breastmilk or formula for 1 year.
● Provide rice or other single-grain cereal for 4 to 6 months.
● Provide a balanced mixture of cereal, vegetables, fruits, and meats for the second 6 months of life.
● Baby foods are labeled regarding calories and nutrient composition; avoid foods with added sugar or salt. Most baby foods, with the
exception of combined foods and desserts, do not have these additives.
● Infants do not need desserts to grow; fruits are more nutritious.
● Fats do not have to be restricted in healthy infants.
Toddlers and Preschoolers
● Avoid excessive fats, salt, and refined sugars.
● Avoid salty snacks and sweet desserts.
● Offer heart-healthy snacks of vegetables, fruits, and finger foods.
● Offer a variety of foods from the basic food groups.
● Discourage the consumption of large amounts of milk, which can lead to nutritional imbalances.
School-Age Children
● Provide heart-healthy school lunches. Limit juice intake.
● Role-model good daily exercise.
● Screen children with family history of congenital heart disease (cholesterol, triglycerides, blood pressure).
● Avoid obesity. Use recommended portion sizes.
● Discourage smoking.
● Limit noneducational screen time to 2 hours per day.
Adolescents
● Emphasize the importance of heart-healthy foods to improve endurance and good body image.
● Avoid a sedentary lifestyle. Limit sedentary activities to 2 hours per day with at least 60 minutes per day of active exercise.
● Discourage the excessive intake of dietary saturated fat, sodium, sugar, and excess calories.
● Be a nonsmoking parent as a model for the adolescent.
● Assess stress management capabilities; counsel accordingly.
● Screen periodically for serum cholesterol elevations and blood pressure measurements.
● Provide serial monitoring of adolescents deemed to be at high risk (sustained high blood pressure readings on at least three separate
occasions).
Nursing Tip: Contact in the clinic or during hospitalization provides an excellent opportunity for the nurse to review heart-healthy information. Reviews
of family history, lifestyle, and eating patterns are suitable interventions, even in the absence of high risks.

Kawasaki Disease (KD)

Kawasaki Disease (KD) is a serious inflammatory condition that affects blood vessels, primarily in children under 5 years of age. It is the leading
cause of acquired cardiovascular disease in children in the United States. The exact cause is unknown, though it may be triggered by a viral
infection in genetically predisposed children. Early diagnosis and treatment are critical to prevent serious complications, such as coronary aneurysms.

Pathophysiology

Kawasaki Disease causes inflammation of blood vessels (vasculitis), including the coronary arteries, which can weaken the vessel walls and lead to
the formation of aneurysms (dilated blood vessels). Without treatment, about 40% of children develop coronary artery aneurysms, potentially leading
to serious, life-threatening complications such as thrombus formation (blood clots).

Manifestations

KD has distinct clinical features, as there are no specific laboratory findings for diagnosis:

● Abrupt onset of fever: Often above 40°C (104°F), lasting for more than 5 days and unresponsive to antipyretics(fever reducers) or
antibiotics.
● Conjunctivitis: Red eyes without discharge.
● Oropharyngeal signs: Fissured lips, "strawberry tongue" (enlarged, red papillae), and inflamed mouth and pharyngeal membranes.
● Lymphadenopathy: Enlarged, nontender lymph nodes, particularly in the neck.
● Skin changes: A generalized erythematous rash (red rash), swollen hands, and peeling (desquamation) of the skin on the palms and
soles.
● Irritability: A hallmark of KD, children may become very irritable and agitated.
● Cardiac signs: Some children may develop cardiac complications during the course of the disease, with abnormalities detectable on an
echocardiogram by the 10th day.

Laboratory Findings

Though not diagnostic, lab results often show:

● Elevated C-reactive protein (CRP)

● Elevated erythrocyte sedimentation rate (ESR)
● Increased white blood cell (WBC) count

Treatment and Nursing Care for Kawasaki Disease focuses on reducing inflammation and preventing complications such as coronary artery damage.
Early intervention is essential to minimize the risk of life-threatening cardiovascular issues.

Treatment

1. Intravenous Immune Globulin (IVIG):

○ Early administration of IVIG within the first 10 days of illness significantly reduces the risk of coronary artery abnormalities.
○ IVIG is usually given in a single infusion, but a second dose may be prescribed if the child does not respond to the first.
2. Aspirin Therapy:
○ Salicylate (aspirin) is administered for its anti-inflammatory and antithrombotic properties.
○ Initially given in high doses to reduce fever and inflammation, then continued at low doses for its antithrombotic effect to prevent
blood clots, particularly if coronary abnormalities are present.
○ Long-term, low-dose aspirin therapy may be required to prevent clot formation, though compliance can be a challenge,
especially as the child begins to feel better.
3. Steroid Therapy:
○ Prednisolone may be used as an adjunct treatment if the child does not respond to IVIG, as it has been shown to improve
coronary outcomes.
Nursing Care

1. Symptomatic and Supportive Care:

○ Nurses focus on monitoring the child’s condition and managing symptoms such as fever, discomfort, and irritability.
○ Hydration, rest, and comfort measures are essential during the acute phase.
2. Parental Education:
○ Postpone live vaccinations for 11 months after IVIG administration, as IVIG suppresses the immune response.
○ Teach parents the signs and symptoms of potential cardiac complications, such as chest pain, shortness of breath, or fatigue,
and when to seek emergency care.
○ Encourage parents to keep their CPR skills up to date in case of cardiac emergencies.
3. Long-term Care:
○ Emphasize the importance of medication compliance, especially with long-term low-dose aspirin therapy, even when the child
feels well.
○ Regular follow-up with a cardiologist may be necessary to monitor for any long-term cardiovascular issues.

Peeling of the fingertips. The appearance of fingertip or toe tip

peeling is characteristic of the subacute phase of Kawasaki disease.