Improving Pediatric Care

From the 36th Annual UCLA Family Practice Refresher Course, sponsored by the David Geffen School of Medicine at the University of California, Los Angeles

Educational Objectives

The goal of this program is to improve management of heart murmurs and failure to thrive (FTT) in children. After hearing and assimilating this program, the clinician will be better able to:

1.   Use  characteristics such as volume and pitch to identify murmurs.

2.   Recognize murmurs and conditions that warrant referral to a pediatric cardiologist.

3.   Work up and identify causes of chest pain.

4.   Evaluate and monitor weight and height in children with FTT.

5.   Describe syndromes associated with growth impairment.

Faculty Disclosure

In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty and members of the planning committee to disclose relevant financial relationships within the past 12 months that might cre­ate any personal conflicts of interest. Any identified conflicts were resolved to ensure that this educational ac­tivity promotes quality in health care and not a proprietary business or commercial interest. For this program, the faculty and planning committee reported nothing to disclose.

Acknowledgements

Drs. Levi and Berkowitz spoke in Los Angeles, CA, on May 28, 2009, at the 36th Annual UCLA Family Prac­tice Refresher Course, presented by the David Geffen School of Medicine at the University of California, Los Angeles. The Audio-Digest Foundation thanks the speakers and the David Geffen School of Medicine for their cooperation in the production of this program.

Heart Murmurs and Chest Pain in Children

Daniel S. Levi, MD, Assistant Professor of Pediatrics, Division of Pediatric Cardiology, David Geffen School of Medicine at the University of California, Los Angeles

Introduction: improve audibility of murmurs in infants by removing clothing and assessing in quiet room; thorough patient history important; children who have murmurs and difficulty breathing, cyanosis, failure to thrive, or high blood pressure (BP; palpate femoral pulse) should be referred to pediatric cardiologist

Listening to murmurs: listen for S₁, S₂, and split in S₂ (difficult in children), then listen for murmur; increasing or decreasing venous return (eg, by standing child up or positioning in Valsalva’s maneuver) helps distinguish be­nign murmur from pathologic murmur; ventricular septal defect (VSD)  —  murmur changes “very little” with de­creased venous return and maneuvering; hypertrophic cardiomyopathy (HCM)  —important to identify; murmur becomes louder with decreased venous return or with standing child up; obstructive murmur (occurs at end of systole); benign murmurs  —  eg, flow murmurs and Still’s murmurs; most become softer with decreased venous return due to lower cardiac output; with increased venous return (due to, eg, squatting, exercise, lying down), be­nign murmurs become louder; right-sided murmurs  —  often flow murmurs; become louder with inhalation due to turbulent flow through right ventricular outflow tract (RVOT) and pulmonary valve (slight to no change in left-sided murmurs); children and adolescents with murmurs and fever, anemia, pregnancy, hyperthyroidism, or other conditions that increase cardiac output often referred to pediatric cardiologist; atrial septal defect (ASD)  —left-to-right shunt at atrial level; turbulent flow in RVOT and pulmonary valve; listen for fixed split S₂ heart sound (difficult in infants); monitoring small ASDs reasonable, since some spontaneously resolve; innocent murmurs  —  almost all children have slight murmur (commonly, grade 2/6 “midpitch, midsystolic” murmur); flow murmurs typically midpitch, heard at left upper sternal border

Pitch: important for pathologic murmurs; represents velocity; velocity high in small VSD; as murmur becomes larger, pressure in right ventricle becomes higher; pitch indicates size of VSD (“big hole, low pitch; small hole, high pitch”)

Benign murmurs: palpate femoral pulse; take thorough history; listen and evaluate chest (eg, feel for thrill, perfu­sion, and pulses); Still’s murmur  —  “musical”; midsystolic; does not go through S₂ like VSD; may be due to false tendons across left ventricle or vibration of mitral valve; reasonable to refer to cardiologist; typically in children 2 to 5 yr of age; venous hum murmur  —communication between high-pressure aorta and low-pressure pulmonary ar­tery or atrium (as in, eg, patent ductus arteriosus [PDA], aortopulmonary [AP] window, coronary artery fistula, Blalock-Taussig [BT] shunt) results in continuous varied murmur; continuous murmurs not normal; venous hum murmurs distinguished from PDA by lower pitch, and disappearing when child supine; differential diagnosis in­cludes arteriovenous malformation (AVM); pulmonary flow murmur  —turbulent flow; heard at left upper sternal border; decreases with decreased venous return; peripheral pulmonic stenosis (PPS)  —benign murmur; seen on echocardiography; occurs in infants; “pretty loud” murmur; high gradients and turbulence in first arcade branches of pulmonary artery; resolves by 1 yr of age; watch for Williams or Alagille syndromes

Who to refer to pediatric cardiologist: children with murmur and cyanosis, poor growth, or signs of heart failure (palpate for enlarged liver and listen for congestion in lungs); infants <4 mo of age; children with greater than grade 3/4 murmur; grading  —  1/6, if murmurs not as loud as heart sounds; 2/6, if murmurs as loud as heart sounds; 3/6, if murmurs louder than heart sounds; 4/6, if “you can feel it”; children with grades 3/6 or 4/6 should be referred; refer children with diastolic murmurs or findings on chest x-ray or electrocardiography (ECG)

Chest x-rays: look for cardiomegaly and pulmonary edema; many children present with myocarditis that resembles bronchiolitis

Diastolic murmurs: listen for S₁, S₂, and absence of silence; typically, aortic valve regurgitation only audible dia­stolic murmur in children; loud S₂ heart sounds can be sign of pulmonary hypertension; fixed split S₂ can be sign of ASD; differentiating between “holeless” systolic murmur (“something that goes right into S₂”) and long systolic murmur important

Referrals: pediatric cardiologists reassure parents and manage expectations of echocardiographic findings (all babies have holes in heart); do not refer child to adult cardiologist or adult echocardiography technician

Chest pain: <1% of chest pain in children associated with heart; chest pain mostly musculoskeletal, pulmonary, or gastrointestinal (GI); chest pain in heart identified as pericardial pain, myocardial pain, coronary (in adults), or arrhythmias (chest discomfort); anomalous coronary arteries and HCM can cause chest pain in children, usually with exercise; refer any chest pain with exercise; screen for HCM; coronary artery abnormalities can be difficult to manage and can cause chest pain with exercise (as in, eg, Kawasaki disease); cardiac chest pain uncommon, but usually due to pericarditis, myocarditis, anomalous coronary HCM (if it occurs with exercise), or arrhythmias (eg, atrioventricular nodal re-entrant tachycardia [AVRT]); thorough patient and family history important (prolonged QT syndrome and HCM strongly genetic); characterizing pain  —difficult for children to de­scribe pain (eg, stabbing or dull); children can characterize duration; ask, “does it hurt worse when you breathe in?” (if yes, cardiac pain unlikely); ask about relation to exercise, eating, deep breathing, and recent trauma; ask what improves pain (eg, ibuprofen [eg, Motrin], aspirin, antacids, bronchodilators)

Work-up: use ECG to rule out myocardial ischemia; in patients with suspected arrhythmia, look for D wave; pay at­tention to ST segments (if throughout, consider pericarditis); Q waves can represent anomalous coronary; HCM  —  look for signs of LV strain (flipped T waves) and increased left-sided forces; chest x-ray  —recognize cardiomegaly; look at lungs and heart; work-up based on patient history; perform echocardiography and ECG in any patient with chest pain on exertion; exercise stress testing for patients with exercise-induced syncope or chest pain

Failure to Thrive

Carol D. Berkowitz, MD, Executive Vice-Chair and Professor, Department of Pediatrics, David Geffen School of Medicine at the University of California, Los Angeles, and Harbor-UCLA Medical Center, Torrance, CA

Failure to thrive (FTT): symptom, not disease; child’s environment does not foster physical growth, development, emotional growth, and social skills; all children with FTT small, but not all small children have FTT

Evaluation: measure and plot height and weight on appropriate curves (eg, age, sex); consider prematurity and spe­cial genetic conditions; children do not gain weight every day (not necessary to weigh children frequently, since weight gained in “steps”); all children, except blind children, tend to grow taller in summer (light thought to have important effect on growth); medical assessment  —  consider contributing factors to growth impairment (eg, birth-related or medical problems, family conditions, physical findings); >95% of time, diagnosis based on history and physical examination

Birth history: ask whether pregnancy planned and/or wanted; ask about prenatal care (eg, “when was the first time you saw a doctor?”); consider maternal gravidity (uterus needs time to recapture nutritive ability between pregnancies; 2.5-4 yr ideal interval); ask about previous fetal loss; genetics referral indicated if mother had several miscarriages before birth of small dysmorphic infant; consider parental depression associated with previous miscarriage; ask about problems during pregnancy and exposure to cigarettes, alcohol, and drugs

Alcohol and drug exposure: maternal alcohol use number one cause of intrauterine growth restriction in United States; fetal alcohol syndrome  —  facies; flattened midface (between tip of nose and upper lip); thin upper lip; flat­tened nasal bridge and upturned nose; small palpebral fissures; prenatal growth restriction continues postnatally; neurodevelopmental disabilities (eg, attention deficit disorder, oppositional defiant behavior); alcohol exposure not always cause of growth impairment (consider attention and feeding given by parents, family, and/or caregiv­ers); cocaine exposure  —  in appropriate environments, most cocaine-exposed babies gain weight at accelerated rate; weight gain continues, then levels off; children eventually reach acceptable height; do not always blame alco­hol and/or drug exposure (important to consider environment)

Prematurity: correct head circumference for 18 mo, weight for 2 yr, and height for slightly >3 yr; “by age »2 yr, you don’t have to continue to correct”; differentiating prematurity from in utero growth restriction  —  check estimated date of confinement and how determined; determine level of maturity

Rickets: skeletal deformities associated with misdiagnosis of child abuse due to metaphyseal changes that resemble fractures; may cause impaired growth in breastfed babies

Medical history: previous growth parameters; fever and recurrent infections  —  babies expend extra energy and have decreased appetite; consider HIV infection; in babies with fatigability after feeding, consider congenital heart dis­ease; with daytime incontinence, consider kidney disease or nephrogenic diabetes insipidus; diarrhea  —  consider malabsorption, celiac disease, or parasites

Family history: “short parents have short kids”; use midparental height (sum of mother’s height and father’s height, divided by 2) curves; measure parents’ heights; foster parents often do not know parental heights

Physical examination: plot weight and height; calculate body mass index (BMI) to check nutritional status; check for dysmorphic features (as in, eg, Cornelia de Lange syndrome); cleft lip and palate  —  associated with 300 differ­ent syndromes (<10% of clefts associated with syndrome); feeding and bonding problems; Down syndrome  —  children do not follow normal growth curve; use growth curves for Down syndrome (available on Internet); Turner syndrome  —  growth impairment; small stature; use growth curves for Turner syndrome (available on Internet); chil­dren with Down syndrome and other genetic syndromes grow at their own rate (plot on appropriate curves); other findings  —  poor dentition (eg, rampant caries); cataracts (consider inborn errors of metabolism); chorioretinitis (consider congenital infection); in baby with cataracts, growth impairment, and mild jaundice, consider galactose­mia (remove lactose from diet; check neonatal screening test); important to tell parents what to do, not just what not to do; Williams syndrome  —associated with supravalvular aortic stenosis; limited cardiac conditions (that cause, eg, pulmonary hypertension, heart failure, persistent cyanosis) lead to growth impairment; increased abdominal girth  —eg, girl, 11 yr of age in less than fifth percentile for height, 75th to 90th percentile for weight, with increas­ing abdominal girth; “always cold”; thyrotropin >500 μIU/mL

Prader-Willi syndrome: genital examination important; eg, boy, 18 mo of age (size of child 11 mo of age) with un­descended testes; boy had hypogonadism, hypotonia, and FTT; decreased fetal movements manifestation of hypo­tonia

Neurodevelopmental delay: parents concerned about gastrostomy tubes; even children who cannot chew and swal­low can receive adequate growth-sustaining nutrition

Laboratory assessment: guided by history and physical examination; bone age  —  perform if child short and midpa­rental height curve appears inappropriate; normal children and children with familial short stature have bone age same as chronologic age; in children with constitutional delay, height age and bone age same (growth hormone or testosterone may be helpful); in environmental FTT, weight “falls off” before height (in constitutional delay, weight and height parallel each other)

Hospitalization: if child needs change of environment, foster home may be better than hospital, unless medical or so­cial conditions warrant hospitalization

Summary: low birth weight  —  if child premature, consider whether weight should have caught up by time of exami­nation (if yes, consider neurodevelopmental disability, eg, intraventricular hemorrhage, germinal matrix hemor­rhage, cerebral palsy [CP]; perform developmental assessment]); if child not premature, consider in utero growth restriction (due to, eg, alcohol exposure); regardless of birth weight  —  if BMI low, child needs more calories; if BMI normal and nutrition adequate, consider short stature of parents or constitutional delay (check bone age)

Suggested Reading

Bergman P et al: An approach to "failure to thrive". Aust Fam Physician 34:725, 2005; Berkowitz CD et al: Charac­teristics of mother-infant interactions in nonorganic failure to thrive. J Fam Pract 25:377, 1987; Berkowitz CD et al: Environmental failure to thrive: the need for intervention. Am Fam Physician 29:191, 1984; Guntheroth WG: Musi­cal murmurs. Am J Cardiol 69:840, 1992; Kenney K: Heart murmurs in children. A systematic approach to cardiac evaluation. Adv Nurse Pract 8:26, 2000; Killeen P: Practical evaluation of pediatric heart murmurs. JAAPA 15:24, 2002; Leung AK et al: Assessment of the child with failure to thrive. Am Fam Physician 48:1432, 1993; Pelech AN: The physiology of cardiac auscultation. Pediatr Clin North Am 51:1515, 2004; Singer LT et al: Stress and depression in mothers of failure-to-thrive children. J Pediatr Psychol 15:711, 1990; Sissman NJ: Cardinal clinical signs in the differentiation of heart murmurs in children. Arch Pediatr Adolesc Med 150:771, 1996.