PEDIATRIC HEART MURMURS

Manish Bansal, MD Clinical Assistant Professor Division of Pediatric Cardiology University of Iowa Murmur

murmur. (n.d.). Dictionary.com Unabridged. Retrieved March 9, 2018 from Dictionary.com website http://www.dictionary.com/browse/murmur Murmur

• Sound or noises made by blood • Caused by abnormal flow patterns – Abnormalities of the heart valves – Holes within the heart – Abnormal communications between blood vessels or between blood vessels and the heart. • Often innocent and result from the normal patterns of blood flow through the heart and vessels Incidence and prevalence

• Revealed in over 50% of children and adolescents, with a peak incidence between 8 to 12 years • CHD - 50-100/1000 live births • CHD may occur in presence of absence of heart murmur • 6 cases per 1000 were moderate or severe

• Pediatr Cardiol. 1994 Nov-Dec; 15(6):282-7 History History

• Feeding difficulties – appr 1/3 with CHD • Acute heart Failure – Dyspnea (74%) – Nausea and vomiting (60%) – Fatigue (56%) – Cough (40%) • Exercise intolerance – age appropriate history – Ability to play and the duration and vigor of feeding – Ability to participate in team sports with that of peers

History

• Chest pain is rarely a symptom of cardiac disease in children • Chest pain or syncope prompted consultation in approximately 10 percent of children; only 11 percent of those with chest pain and 5 percent of those with syncope had cardiac disease.

• Pediatrics. 2004;114(4):e409-e417 History Physical Examination

• Vitals compared with age appropriate norms. http://www.cc.nih.gov/ccc/pedweb/pedsstaff/age.html • Focused examination of respiratory, cardiovascular and GI system. • General appearance, activity level, color and respiratory effort • Neck: prominent vessels, abnormal pulsations and bruits. • Chest: abnormalities of sternum, abnormal cardiac impulse or thrills. Physical examination

• Lungs – Abnormal breath sounds • Abdomen – Liver location, enlargement or ascites • Pulse: rate, rhythm, volume, character and capillary refill time

Examination of heart Properties of a Murmur

• Timing - systolic vs diastolic • Duration-length in systole or diastole • Location—where in the heart they may originate • Quality or pitch—how they sound. This is important in differentiating normal flow murmurs from the abnormal. • Intensity or loudness—does not necessarily define the severity, but changes in intensity may help determine the type of murmur being heard. • Presence of an extra sound called “a click” Grading My grading

• Grade 1: I can hear it (very faint) • Grade 2: resident can hear it easily (easily heard) • Grade 3: Medical student can hear it (can’t miss it!!) • Grade 4: Thrill • Grade 5: Stethoscope half of the chest • Grade 6: Stethoscope over the chest barely touching Murmur (frequency or pitch) Red flags (odds ratio OR) for pathologic murmur

• Holosystolic murmur (OR = 54) • Grade 3 or higher (OR = 4.8) • Harsh quality (OR = 2.4) • An abnormal S2 (OR = 4.1) • Maximal intensity at the upper left sternal border (OR = 4.2) • Systolic click (OR = 8.3), • Diastolic murmur, or increased murmur intensity with standing Innocent murmurs

• May be heard in virtually anyone • Most often heard in childhood. • AKA – Functional murmur – Flow murmur – Benign murmur – Normal murmur – Non pathologic murmur – In organic murmur

Innocent murmurs (seven S’s)

• Sensitive (changes with child’s position or with respiration) • Short duration (not holosystolic) • Single (no associated clicks or gallops) • Small (murmur limited to a small area and non radiating) • Soft (low amplitude) • Sweet (not harsh sounding) • Systolic (occurs during and is limited to systole) Still murmur

• Still murmur – Grade 1 to 3 – Early systolic murmur – Low to medium pitch with a vibratory or musical quality – Best heard at lower left sternal border – Loudest when patient is supine and decreases when patient stands – Infancy to adolescence, often 2 to 6 years – Can sound like Ventricular septal defect or hypertrophic cardiomyopathy

Aortic Flow murmur

• Systolic ejection murmur best heard over the aortic valve • Older childhood into adulthood • Usually result of increased flow velocity from the larger stroke volume passing through relatively narrow LVOT and aortic valve annulus

• Pediatr Cardiol 27:19–24, 2006

Mammary artery soufflé

• High-pitched systolic murmur that can extend into diastole • Best heard along the anterior chest wall over the breast • Rare in adolescence • Arteriovenous anastomoses or patent ductus arteriosus

Pulmonary flow murmur

• Grade 2 or 3 • Crescendo-decrescendo • Early- to mid-systolic murmur peaking in mid-systole • Best heard at the left sternal border between the second and third intercostal spaces • Characterized by a rough, dissonant quality • Loudest when patient is supine and decreases when patient is upright and holding breath • Heard in all ages • Can be confused with Atrial septal defect or pulmonary valve stenosis

Supraclavicular/ brachiocephalic systolic murmur

• Brief, low-pitched, crescendo-decrescendo murmur heard in the first two-thirds of systole • Best heard above clavicles • Radiates to neck • Diminishes when patient hyperextends shoulders • Childhood to young adulthood • D/D Bicuspid/stenotic aortic valve, pulmonary valve stenosis, or coarctation of the aorta

Venous hum

• Grade 1 to 6 continuous murmur • Accentuated in diastole • Whining, roaring, or whirring quality • Best heard over low anterior neck, lateral to the sternocleidomastoid • Louder on right • Resolves or changes when patient is supine • 3 to 8 years • D/D Cervical arteriovenous fistulas or patent ductus arteriosus Distribution of normal murmurs

Acta Informatica Medica. 2016;24(2):94-98 Causes of pathologic murmur

Acta Informatica Medica. 2016;24(2):94-98 Common Pathologic heart murmurs

Lesion Prevalence amongst Clinical features Murmur children with CHD characteristic (%) VSD 20-25 Small defects: usually Small: loud holosystolic asymptomatic murmur at LLSB Medium or large defects: Medium and large defects: CHF, symptoms of prominent left ventricular bronchial obstruction, impulses; thrill at LLSB; frequent respiratory split or loud single S2; SEM infections to holosystolic murmur at LLSB without radiation; may also hear a grade 1 or 2 mid-diastolic rumble ASD 8-13 Usually asymptomatic 2 or 3 SEM best heard at ULSB; wide split fixed S2; may have a grade 1 or 2 diastolic flow rumble at LLSB

Common Pathologic heart murmurs

Lesion Prevalence amongst Clinical features Murmur children with CHD characteristic (%) PDA 6-11 May be asymptomatic; can Continuous murmur cause easy fatigue, CHF, normal S1; S2 may be and respiratory symptoms “buried” in the murmur; thrill or hyperdynamic left ventricular impulse may be present Tetralogy of Fallot 10 Depends on the severity of Central cyanosis; clubbing ; PS grade 3 or 4 long systolic ejection murmur heard at ULSB; increased S1; single S2 Pulmonary stenosis 7.5-9 Usually asymptomatic Systolic ejection murmur (grade 2 to 5); at ULSB radiating to infraclavicular regions, axillae, and back; normal or loud S1; variable S2; systolic ejection click Common Pathologic heart murmurs

Lesion Prevalence amongst Clinical features Murmur children with CHD characteristic (%) Coarctation of Aorta 5.1-8.1 Newborns and infants: CHF SEM at back, decreased Older children: femoral pulses. BP lower in hypertension, leg pain legs than arms Aortic stenosis 5-6 Usually asymptomatic SEM at RUSB radiating to Moderate to severe: chest carotids pain with exertion, dyspnea TGA 5 Variable presentation Cyanosis. Murmur may be absent TAPVR 2-3 Onset of CHF at 4-6 weeks Grade 2-3 systolic ejection murmur at ULSB; grade 1 or 2 mid-diastolic flow rumble at LLSB; wide split fixed S2 HLHS Rare May be asymptomatic at Hyperdynamic precordium; birth but cyanosis and CHG single S2; nonspecific grade develop within first 2 1 or 2 systolic ejection weeks murmur along left sternal border Physiologic interventions

Auscultatory events Intervention and response Valvar aortic stenosis Louder following a pause after a premature beat HCM Louder on standing, Valsalva maneuver. Fainter with prompt squatting Mitral regurgitation Louder on sudden squatting or with isometric handgrip Mitral valve prolapse Midsystolic click moves toward S1 and late systolic murmur Starts earlier on standing; click may occur earlier on Inspiration; murmur starts later and click moves toward S2during squatting Tricuspid regurgitation Louder with inspiration VSD Louder with isometric grip Physiologic interventions

Auscultatory events Intervention and response Aortic regurgitation Louder with sitting upright and leaning forward, sudden squatting, and isometric handgrip Mitral stenosis Louder with exercise, left lateral decubitus position, coughing Continuous murmurs - Patent ductus Diastolic phase louder with isometric arteriosus handgrip Cervical venous hum Disappears with direct compression of Jugular vein Role of diagnostic testing

• CXR rarely assist with diagnosis in an asymptomatic patient. • ECG is useful depending on clinical examination and symptoms Cost effectiveness and practical implications

J Pediatr 2002;141:504-11

Is echocardiogram necessary Is Echocardiogram necessary

Indications for referral

• Innocent murmur – Absence of abnormal physical findings (except for murmur) – Negative review of symptoms – Negative history – Murmur with seven features of innocent murmur

• Not appropriate for newborns and infants younger than 1 year as there is higher rate of asymptomatic structural heart disease. Neonatal Heart Murmurs

• Incidence 23.81 per 1000 live birth • Incidence of CHD 10.75 per 1000 live births

• Bansal M, Jain H. Cardiac Murmur in Neonates. Indian Pediatrics 2005; 42:397-398

Neonatal heart murmurs

• 1 percent of newborns have a heart murmur • 31 to 86 percent of these infants have structural heart disease • Referral to a pediatric cardiologist is recommended • Sensitivity for detection of a pathologic heart murmur in newborns ranges from 80.5 to 94.9% among pediatric cardiologists, with specificity ranging from 25 to 92 percent Key recommendations

Clinical recommendation Evidence Rating Structural heart disease is more likely when C the murmur is holosystolic, diastolic, grade 3 or higher, or associated with a systolic click; when it increases in intensity with standing; or when it has a harsh quality. Chest radiography and electrocardiography B rarely assist in the diagnosis of heart murmurs in children. Family physicians should consider referral to B a pediatric cardiologist for newborns with a heart murmur, even if the child is asymptomatic, because of the higher prevalence of structural heart lesions in this population.

Am Fam Physician. 2011;84(7):793-800 References

1. Evaluation of heart murmurs in children: Cost-effectiveness and practical implications. Yi, Michael S. et al. The Journal of Pediatrics , Volume 141 , Issue 4 , 504 - 511 2. Phoon, Colin. (2001). Estimation of pressure gradients by auscultation: An innovative and accurate physical examination technique. American heart journal. 141. 500-6. 10.1067 3. Frank JE, Jacobe KM. Evaluation and Management of Heart Murmurs in Children. Am Fam Physician. 2011;84(7):793-800 4. Bansal M, Jain H. Cardiac Murmur in Neonates. Indian Pediatrics 2005; 42:397-398 5. Van Oort A, Le Blanc-Botden M, De Boo T, Van Der Werf T, Rohmer J, Daniëls O. The vibratory innocent heart murmur in schoolchildren: difference in auscultatory findings between school medical officers and a pediatric cardiologist.Pediatr Cardiol. 1994 Nov-Dec; 15(6):282-7. 6. Begic Z, Dinarevic SM, Pesto S, Begic E, Dobraca A, Masic I. Evaluation of Diagnostic Methods in the Differentiation of Heart Murmurs in Children. Acta Informatica Medica. 2016;24(2):94-98. doi:10.5455/aim.2016.24.94-98. 7. Advani, N., Menahem, S., & Wilkinson, J. (2000). The diagnosis of innocent murmurs in childhood. Cardiology in the Young, 10(4), 340-342. doi:10.1017/S104795110000963X 8. Geva, T, Hegash, J, Frand, M. Reappraisal of the approach to the child with heart murmur; is echocardiography mandatory? Int J Cardiol 1988. 107–113

Thanks Manish Bansal, MD [email protected] Ph: 319 356 3537