HEART MURMURS THROUGHOUT CHILDHOOD Frances R. Zappalla, D.O. Nemours Cardiac Center A.I. du Pont Hospital for Children Wilmington, DE Definition: HEART MURMURS An extra abnormal heart sound usually detected while listening to the heartbeat with a stethoscope. 2 PARENTAL ANXIETY. Anxiety about need for medication (49%) Sports restrictions (41%) Cardiac surgery (29%), Cardiac risk for siblings (20%) Premature death (13%). 19% of mothers felt the murmur resulted from something they did wrong during pregnancy. After reassurance from the cardiologist, 7% of parents had persistent anxiety J Pediatr. 2002 Jun;140(6):747-52 3 1
HEART MURMURS Most systolic murmurs in otherwise healthy children are innocent and do not need a referral...however, see the previous slide. If findings are suspicious or in the present of parental anxiety. referral for a cardiology office visit is more cost effective than ordering an echocardiogram alone ECHO ONLY Slightly more than half of echocardiography studies (68%) performed in adult laboratories were technically adequate Of those 52% had the correct diagnosis 16% incorrect diagnosis Repeat echocardiography in 38% Abnormalities not diagnosed correctly in 14% 6 had important lesions Normal hearts were labeled as abnormal in 18% RA Hurwitz et al Peds 1998 DIASTOLIC MURMURS ARE PATHOLOGIC UNTIL PROVEN OTHERWISE 2
AUSCULTATION AREAS RUSB Aortic LUSB Pulmonic LLSB TV, Septum Apex - MV Fetal Circulation The colors indicate the oxygen saturation of the blood. The arrows show the course of the fetal circulation. FETAL CIRCULATION 55 % of the highly saturated umbilical venous returns via ductus venous to IVC - RA junction preferentially crosses the foramen ovale into the left atrium Highest oxygen content diverted to coronary arteries and the brain Remaining umbilical venous flow, portal venous blood, and SVC flow crosses the tricuspid valve 3
Circulation after Birth Arrows indicate the course of the neonatal circulation. TRANSITIONAL CIRCULATION Clamping of Umbilical cord Removes the low resistance placenta Decreases ductus venous flow and systemic venous return to IVC Increases systemic resistance Spontaneous respiration Decreases pulmonary vascular resistance Increases pulmonary venous return to LA TRANSITIONAL CIRCULATION Increased pulmonary venous return Increases in LA pressure Flap valve of foramen ovale closes PDA closes within 10 to 15 hours 4
PULSE OXIMETRY 20,055 newborn babies were screened 53 had major congenital heart disease (24 critical) Prevalence of 2 6 per 1000 livebirths. Sensitivity of pulse oximetry 75 00% (95% CI) for critical cases 49 06% for all major congenital heart defects. False-positive results noted for 169 (0 8%) 6 cases were significant, but not major, CHD 40 were other illnesses that required urgent medical intervention. The prevalence of major CHDs with normal pulse oximetry was 1.4 per 1000 live births Ewer et al LANCET 2011 NEONATES AND INFANTS LOW-RISK GROUP Otherwise well - no symptoms and no other signs Alert, active, feeding well, gaining weight Normal pulses, heart rate, respiratory rate and liver size. Normal blood pressures in all extremities Normal pulse oximeter saturation in upper and lower extremities in room air NEONATES AND INFANTS RED FLAGS Comfortable Tachypnea Diminished pulses Poor feeding Poor weight gain Pulse Oximetry <90% 5
BENIGN MURMURS IN THE NEONATE Physiologic peripheral pulmonary stenosis Transient patent ductus arteriosus murmur Tricuspid regurgitation murmur Flow Murmurs PERIPHERAL PULMONARYSTENOSIS Physiologic Grade 1-2/6 systolic ejection murmur Heard best in the upper left sternal border but radiates well to the axilla and back Most resolved within 6 weeks Remainder by 6 months TRANSIENT PATENT DUCTUS ARTERIOSUS Closing ductus arteriosus Usually a systolic ejection murmur of 1-2/6 intensity Heard best in the left upper sternal border Usually heard at 24-48 hours of life and.. usually has disappears by the time the cardiologist comes to listen 6
TRICUSPID REGURGITATION MURMUR Infants with fetal distress or perinatal asphyxia Holosystolic murmur due to high right ventricular systolic pressure Sounds like a small ventricular septal defect PATENT FORAMEN OVALE Normal structure Functional closure occurs after birth as left atrial pressure exceeds right atrial pressure Right to left shunting may occur in neonatal period causing peri-oral cyanosis 7
PATHOLOGICAL MURMURS Most Common Ventricular septal defect Pulmonary stenosis Aortic stenosis PATHOLOGICAL MURMURS AV valve insufficiency in Complete AV Canal defect Tetralogy of Fallot Coarctation of the aorta Ebstein s Anomaly CHD WITHOUT MURMURS Hypoplastic left heart syndrome Tricuspid Atresia Critical Coarctation Interrupted aortic arch Complete Common AV canal 8
APPROACH TO THE CYANOTIC CHILD Pre- and post-ductal saturations measured on right arm and either leg detects shunting across the ductus arteriosus Arterial Blood Gas(es) Chest X-ray 12-lead ECG APPROACH TO THE CYANOTIC CHILD Hyperoxia test: Document PaO 2 in room air Administer 100% FiO 2 for 10 minutes Repeat PaO 2 in 100 % O 2 PaO 2 > 300 suggests intrapulmonary shunt PaO 2 < 150 suggests intracardiac shunt APPROACH TO THE CYANOTIC NEONATE Suspected Congenital Heart Disease Referral for pediatric cardiology evaluation in-house consult transport to tertiary center Telemedicine Echocardiography for detailed anatomic diagnosis Medical management and surgical strategies based on anatomic diagnosis 9
VENTRICULAR SEPTAL DEFECTS Most common form of CHD 1.5 to 3.5 per 1000 term infants Slightly more common in females Most common defect in chromosomal syndromes however 95% of VSD not associated with chromosomal anomaly LOCATION OF VSD Perimembranous - most common (80%) Muscular (5-20%) Subpulmonary PV Subaortic TV Anterior Inlet AV canal type VSD (5-8%) Apex VENTRICULAR SEPTAL DEFECT Small ventricular septal defects Large ventricular septal defect NO murmur until pulmonary vascular resistance drops 10
AORTIC AND PULMONARY STENOSIS Murmurs may be heard soon after birth and persist Usually preceded by an ejection click Constant with aortic stenosis Louder during expiration with pulmonic stenosis PULMONARY VALVE STENOSIS Usually asymptomatic Variable systolic ejection click at LUSB Grade 2-5/6 SEM at LUSB to lung fields Soft, delayed pulmonary component of S2 May have increased RV impulse and thrill ECG: RVH BALLOON VALVULOPLASTY 11
BENIGN MURMURS IN CHILDREN Stills Murmur Venous Hum Pulmonary Flow Murmur STILL'S MURMUR Grade 1-2/6, musical or vibratory in nature Heard best at the lower left sternal border Murmurs are accentuated by anemia fever increased cardiac output VENOUS HUM Varies with neck position and compression Diff Dx of Continuous murmurs : PDA, shunt, AV fistula S 1 S2 S 1 12
PATHOLOGIC HEART MURMURS IN THE ADOLESCENT Atrial Septal Defects (ASD) Mitral valve prolapse (MVP) Hypertrophic Cardiomyopathy (HCM) Rheumatic heart disease (RHD) ATRIAL SEPTAL DEFECTS SVC Secundum ASD and PFO Sinus Venosus ASD Primum ASD IVC 13
ASD PULMONARY FLOW MURMUR Systolic flow murmur Widely split S2 Increased P2 rsr in V1 Right Axis deviation Systolic flow murmur Split S2 Normal P2 May have rsr in V1 Normal Axis deviation Right Arial Enlargement Normal P waves CLASSIC ASD ECG ASD CLOSURE 14
MITRAL VALVE PROLAPSE Mid-systolic click at LLSB, maximal in upright position + late systolic regurgitant murmur at apex Look for associated skeletal abnormalities slender stature, scoliosis, pectus excavatum Marfan syndrome MITRAL VALVE PROLAPSE Diagnosis often over-called Prognosis generally excellent Potential complications: Mitral valve regurgitation SBE Arrhythmia Central neurologic ischemic events High risk subset of patients: Males, age > 45 Presence of MR & leaflet thickening 15
HYPERTROPHIC CARDIOMYOPATHY Most common cause of sudden death in US (35%) Sudden death usually occurs in teens and young adults < 35 years of age Occurs in about 2% (1:500) of general population Autosomal Dominant with > 400 mutations on 12 genes Variable expression and clinic course Competitive golf is permitted under guidelines HYPERTROPHIC CARDIOMYOPATHY Hyperdynamic precordium Murmur varies with maneuvers SEM at the LSB Softer with Hand-grip/ Louder with release Standing increases murmur /Squatting decreases murmur History of SOB or chest pain WITH exertion Family history of sudden death in a young relative HYPERTROPHIC CARDIOMYOPATHY Often don t develop hypertrophy until 14-17 years old Absence of hypertrophy does NOT rule out HCM 90% have ECG changes which often precede echo findings Commercially available Genetic testing (50-60% yield) 16
Histopathology of heart sections showing significant myofibre disarray and interstitial fibrosis in HCM Cell Research (2003) 13, 9 20 17
HYPERTROPHIC CARDIOMYOPATHY ACUTE RHEUMATIC FEVER New murmur or new onset of heart failure (tachypnea, S3 gallop, pulmonary edema) Murmur of mitral regurgitation at apex Diastolic decrescendo murmur of aortic regurgitation Pancarditis- can involve pericardium, myocardium and/or valves ACUTE RHEUMATIC FEVER 18
JONES CRITERIA Evidence of recent Group A hemolytic Strep infection Plus 2 major or one minor criteria MAJOR Carditis Polyarthritis Syndenham s chorea Erythema marginatum Subcutaneous nodules MINOR Athralgia Fever Elevated acute phase reactants Prolonged PR interval 55 13 YEAR OLD WITH SEVERE MITRAL REGURGITATION NEW ENDOCARDITIS RECOMMENDATIONS Prosthetic material used for cardiac valve repair Previous endocarditis Unrepaired cyanotic CHD, including palliative shunts and conduits Completely repaired congenital heart defect with prosthetic material or device(surgical or catheter intervention ) for the first 6 months after the procedure Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device Cardiac transplantation recipients who develop cardiac valvulopathy 19
SUMMARY Most murmurs in childhood are innocent In sick patients the ECG and CXR will help differentiate a flow murmur due to fever versus a pathologic murmur When in doubt. Feel free to call for help 20