Chronic Illness: Week 4 - Congenital Heart Defects

Heart Failure: Pediatrics (cont.)

For school-aged child and children in day-care, teach parents to make sure the school has an AED with child paddles. The chance of sudden death are very little as most do well and live a healthy adult life, but parents worry about this. Parents need to see genetic counselor before having another baby as the chance of having another baby with a heart defect is higher.

Tetralogy of Fallot

Four heart defects are present at birth: Ventricular septal defect (VSD) Overriding aorta straddles the VSD Pulmonary valve stenosis Right ventricle hypertrophy Most cases corrected surgically in early infancy. Many have residual lesions that may complicate pregnancy. Prenatal evaluation to assess RV and LV function, pulmonary insufficiency and stenosis is vital for healthy outcomes.

Family Education: Tetralogy of Fallot, cont.

A child with Tetralogy of Fallot is at higher risk of infections. Remind about benefits of all recommended immunizations. Limit visitors. Call pediatrician immediately if fever, vomiting, poor feeding, diarrhea. Daily Weights. Keep record of intake and output amounts. Teach about medications, especially Digoxin (Lanoxin) and Side Effects. Teach to hold the child often. Teach to respond to crying right away - THERE ARE NO "BAD" BABIES!

A 9-year-old girl reports that she cannot keep up during play and that her feet are "always freezing" compared with her hands. On exam, the physician notes a short stature, wide shoulders, and webbing of the neck. Her cardiac exam is without murmur, but the blood pressure in her right arm is 146/79 mm Hg and in the right thigh is 91/45 mm Hg. By palpation, her femoral pulse is delayed compared with her radial artery pulse. The physician orders numerous diagnostic tests including an aortic angiogram (shown). What is the most likely diagnosis causing the patient's symptoms? A. Coarctation of the aorta B. Hypoplastic left heart syndrome C. Aortic stenosis D. Patent ductus arteriosus (PDA)

A. Coarctation of the aorta

What Could Go Wrong?

Atrial Septal Defect: Abnormal communication between the atria allows blood to shunt from left to right, includes right atrial enlargement. Often asymptomatic, diagnosed by a murmur. Ventricular Septal Defect: Abnormal communication between the ventricles, accounts for 25-33% of congenital heart lesions, also can be asymptomatic, diagnosed by a murmur and echocardiogram. Aortic Stenosis: Narrowing of the aortic outflow tract causes increased workload on left ventricle, may be asymptomatic and only diagnosed later in life as exercise intolerance, syncopal episodes, epigastric pain.

Acquired Cardiovascular Disorders

Childhood obesity: Risk factors: Race, socioeconomic status, and lack of health insurance High-fat, high sodium diet, sedentary activities (TV, computer use, etc.) Association with parental obesity Places child at risk for asthma, sleep apnea, hypertension, type 2 diabetes, dyslipidemia, cardiovascular disease Social and economic consequences

Clinical Manifestations of Heart Defects in an Infant: NOTES

Clinical signs of congestive heart failure (acquired) Poor feeding and sucking; leads to failure to thrive Dyspnea, tachypnea, diaphoresis, retractions, grunting, nasal flaring Wheezing, coughing, rales are rare (even with significant heart failure) Skin changes, such as pallor or mottling Hepatomegaly *Peripheral edema is rare in infants and young children usually points to renal disease

What Else Could Go Wrong?

Coarctation of the Aorta: "Pinching" of aorta causes increased workload on the left ventricle, newborns may present with CHF. Once the ductus arteriosis closes, the child's condition may rapidly deteriorate including hypotension, acidosis and shock. Older children my have hypertension in upper extremities, decreased or absent pulses in lower extremities, cool, mottled skin, shiny, hairless skin, leg cramps during exercise This child may also be asymptomatic.

Clinical Manifestations of Heart Defects in an Infant

First is a heart murmur. Diaphoretic with sucking as an infant. Poor weight gain - May be diagnosed "Failure to Thrive" (FTT) Cyanosis shortly after birth not responding to supplemental oxygen. Hypercyanotic spells---suddenly which are relieved with knee chest position.

Sudden Cardiac Death: Pediatrics

Hypertrophic cardiomyopathy and coronary artery anomalies are most frequent causes. Most cardiovascular abnormalities go unnoticed until the time of death. Study by Fuller et al, (2009) of 5,615 screening athletic physicals found: Cardiac history: detected 0 athletes Auscultation/inspection: 1/6000 BP evaluation: 1/1,000 ECG evaluation: 1/350 athletes

Heart Failure: Pediatrics

Infants have greater risk of HF because of immature heart which is more sensitive to volume or pressure overload. Children respond to hypoxemia with bradycardia. Congenital heart disease is the leading cause of death in first year of life.

Oxygenation

Newborns have a high oxygen demand. Heart rate of a newborn ranges from 100-180 beats per minute. Anything that interrupts oxygenation to the pregnant mother, to the fetus, or to the newborn can endanger the normal transition of a newborn's circulation after birth.

Basic Facts about Congenital Heart Defects

Leading cause of death (except for prematurity) in first year of life. The etiology (cause) is known in only 10% of defects.

Heart Failure: Pediatrics - Teaching:

Make sure teacher and class understand symptoms when not repaired: Cyanosis Increased fatigue Increased susceptibility to chest infections Small size

Good Prenatal Care

Many fetal anomalies, including some congenital cardiac defects, are discovered by ultrasound during routine prenatal care visits. A little information can go a long way: Parents and families can determine their birth plan based on known issues. Infants needing immediate heart surgery upon birth may be born in tertiary hospitals where this level of care is available. Coping with the anomaly can be improved when families have knowledge in advance of the birth.

Basic Facts about Congenital Heart Defects: Risk Factors

Multiple risk factors are known: Genetics, environment, chromosomal aberrations, age of parents Maternal rubella Insulin-dependent diabetes Alcohol use during pregnancy Smoking Drugs

Heart Failure: Pediatrics - Parent Education:

Parent Education: Digoxin Symptoms of toxicity: Bradycardia Follow through with frequent labs, especially electrolyte levels and renal and hepatic function Monitor for hypokalemia

Clinical signs of acquired congenital CHF

Poor feeding and sucking; leads to failure to thrive Dyspnea, tachypnea, diaphoresis, retractions, grunting, nasal flaring Wheezing, coughing, rales are rare (even with significant heart failure) Skin changes, such as pallor or mottling Hepatomegaly Peripheral edema is rare in infants and young children and instead of CHF usually points to renal disease

Basic Facts about Congenital Heart Defects: NOTES

Some heart defects are repaired shortly after birth. Good prenatal care can result in birth in a hospital prepared for this level of care.

Family Education: When Infant has Tetralogy of Fallot but is sent home to gain weight

Surgery may be planned once the baby has gained sufficient weight, but what do we tell families who are bringing this baby home? Educate about cyanosis and TET Spell and knee-chest positioning. Position infant at a 45-degree angle in car seat for circulation. Allow longer time to eat and include high-calorie formula if bottle-feeding. Provide additional education as appropriate if child receiving tube-feedings.

Acquired Cardiovascular Disorders: Pediatrics

Systemic Hypertension: In preadolescent children, HTN is often secondary to another problem. Over 70% are due to renal disease. In adolescents, over 80% are due to primary HTN, with common risk factors like obesity and clinical manifestations like sleep disorders Children with HTN are commonly asymptomatic.