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Download Definition: An extra abnormal heart sound usually detected while
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4/19/2012 HEART MURMURS THROUGHOUT CHILDHOOD Frances R. Zappalla, D.O. Nemours Cardiac Center A.I. du Pont Hospital for Children Wilmington, DE HEART MURMURS Definition: 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 4/19/2012 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 4/19/2012 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 4/19/2012 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 4/19/2012 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 4/19/2012 BENIGN MURMURS IN THE NEONATE • Physiologic peripheral pulmonary stenosis • Transient patent ductus arteriosus murmur • Tricuspid regurgitation murmur • Flow Murmurs PERIPHERAL PULMONARY STENOSIS • 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 4/19/2012 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 4/19/2012 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 4/19/2012 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 PaO2 in room air • Administer 100% FiO2 for 10 minutes • Repeat PaO2 in 100 % O2 PaO2 > 300 suggests intrapulmonary shunt PaO2 < 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 4/19/2012 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%) PV Subpulmonary TV Subaortic 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 4/19/2012 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 4/19/2012 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 S1 S2 S1 12 4/19/2012 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 4/19/2012 ASD PULMONARY FLOW MURMUR • Systolic flow murmur • Systolic flow murmur • Widely split S2 • Split S2 • Increased P2 • Normal P2 • rsR’ in V1 • May have rsR’ in V1 • Right Axis deviation • Normal Axis deviation • Right Arial Enlargement • Normal P waves CLASSIC ASD ECG ASD CLOSURE 14 4/19/2012 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 4/19/2012 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 4/19/2012 Histopathology of heart sections showing significant myofibre disarray and interstitial fibrosis in HCM Cell Research (2003) 13, 9– 20 17 4/19/2012 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 4/19/2012 JONES CRITERIA • Evidence of recent Group A hemolytic Strep infection • Plus 2 major or one minor criteria MAJOR MINOR Carditis Athralgia Polyarthritis Syndenham’s chorea Fever Erythema marginatum Prolonged PR interval Elevated acute phase reactants Subcutaneous nodules 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 4/19/2012 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…. 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