Mark Zittergruen, MD, MBA Mercy Pediatric Cardiology

February 3, 2018

AGENDA Heart murmurs and structural heart

disease Pediatric chest discomfort Pediatric syncope Pediatric arrhythmias in the office setting

Fluid Dynamics 101 Q α ΔP/R Differences between flow and velocity Magnitude of shunt vs. velocity of flow

Fluid flow follows a pressure gradient No gradient = no flow Think newborn – no murmur if RVP=LVP

Influence of resistance – diameter of orifice R α 1/r4 ›››› Q α ΔPr4

Double the radius, resistance decreases by 16X – flow increases by 16X

Applies to airways as well

Physics of the Murmur Murmurs generally related to turbulence which is

proportional to velocity (Reynolds number) and inversely proportional to diameter and viscosity

Heart sounds related to deceleration of blood with abrupt valve closure and its effect on surrounding structures

Children’s chests vs. adults

How many murmurs are out there? Prevalence in infancy estimated at 60% Prevalence in school-age children ranges

from 75-90% at some time CHD – approximately 8/1000 at birth and

4/1000 in school age children Therefore – most murmurs (99%) are

benign

Importance of the Diagnosis Obviously important to diagnose CHD and react

appropriately May be asymptomatic in childhood with dire

consequences later in life (Eisenmenger’s syndrome)

Innocent murmur is not a disease Psychological issues – school, reproduction Socioeconomic issues

Clues to the Significance History and Physical are VERY IMPORTANT!!!

Growth and development Feeding pattern ? Symptoms: dyspnea/tachypnea, activity, cyanosis

(central vs. peripheral), chest discomfort, syncope Infant can have significant congenital heart disease

and have no murmur Transposition rarely has murmur unless VSD is

present HLHS rarely has a murmur

Physical Exam “Across the room” assessment Height and weight plotted Complete vitals including BPs in UE and LE; use of

pulse oximetry Inspection of the chest and neck Palpation of chest Lungs, abdomen, and pulses Clubbing and cyanosis

Precordial Thrill

Palpable manifestation of a loud murmur Often best felt with palm rather than finger

tips Related to large pressure gradients When might a thrill be a good thing? When might a thrill be a bad thing?

Makes the murmur an automatic grade 4

Dr. Z’s Guide to Listening! Practice makes perfect Room has to be quiet Differentiate from respirations – “pinch the

nose” technique Supine and upright Don’t rush the exam “See the murmur”

First Heart Sound S1 associated with closure of mitral and

tricuspid valves May hear splitting of S1 in normal children Especially if very trim and athletic with

slow heart rates Split S1 also heart frequently in RBBB and

Ebstein’s anomaly

S2 – Critical Sound S2 represents closure of pulmonic and aortic

valves Normal splitting – A2 followed by P2 With inspiration – splitting increases PRACTICE LISTENING FOR THIS

Single S2 or widely split S2 may be only physical clue for pathology!

S2 and Pathology Fixed split S2 – very common with ASD and

RBBB Single S2 found in pulmonary hypertension

(in which case P2 will be very loud) or if only one semilunar valve is present

Paradoxical splitting of S2 – when LV ejection is delayed such as LBBB or severe AS

Cardiac Auscultation - Timing What happens during systole?

Cardiac contraction – VSD murmur AV valves closed – Tricuspid and mitral regurgitation Semilunar valves open – Aortic and pulmonic stenosis

What happens during diastole? AV valves open – Tricuspid and mitral stenosis Semilunar valves closed – Aortic and pulmonic

regurgitation Some murmur are continuous

PDA, coarctation

Murmurs Must classify in terms of:

Intensity - Grades 1-6 Grade 1 - Barely audible Grade 2 - Soft, but easily heard Grade 3 - Moderately loud, no thrill Grade 4 - Moderately loud with thrill Grade 5 - Audible with stethoscope barely on chest Grade 6 – Audible with stethoscope off the chest

Timing – Systolic, diastolic, continuous Location Transmission Quality – Musical, vibratory, harsh, blowing

Red Flags in Auscultation IMPORTANT SLIDE #1 Murmur is associated with a thrill Murmur is diastolic Murmur is continuous and not suppressible Murmur radiates to back or axillae (unless

patient is a baby) Murmur preceded by click or there is an

extra heart sound (gallop)

The Still’s Murmur Most common benign murmur of childhood The vibratory, low frequency murmur heard

at the LLSB, Grade 1-3 Loudest when supine Little radiation Louder with activity, anemia, or illness Softer with Valsalva

Pulmonic Outflow Murmur 15% of all innocent murmurs Very common in children with chest wall

abnormalities (pectus excavatum) Loudest at ULSB Blowing low frequency murmur, NO CLICK,

NORMAL S2, softer in inspiration May only be audible with anemia,

thyrotoxicosis, pregnancy, fever

Peripheral Pulmonic Stenosis Very common murmur in infants –

especially premature infants Related to increasing cardiac output and

small peripheral branch pulmonary arteries LUSB with radiation to axillae Usually gone by 1 year of age

Benign Venous Hum Related to flow in the superior vena cava Common in toddlers/preschoolers Continuous murmur heard under the right

clavicle Very positional – usually disappears when

child is supine Can be suppressed with neck pressure or

changes in child’s head position

VSD Often high-pitched systolic murmur Huge VSD may have lower frequency Frequency/harshness of murmur related to

velocity of blood across the septum Harsher is usually better – signifies a nice

pressure gradient between LV and RV

Echocardiogram

VSD Echocardiogram - Color

ASD You do NOT hear the blood as it crosses the septum –

WHY? What are we hearing? Murmur is a pulmonic murmur heard best at ULSB

related to large volume flow Usually a split S2 is key to the diagnosis PFO should not cause a murmur

ASD ECHOCARDIOGRAM 1

ASD ECHOCARDIOGRAM 2

PFO

Pulmonic Stenosis Same murmur as ASD May have an associated

thrill Usually preceded by a

systolic click Harsher is NOT better with

PS (or AS) Implies a larger gradient

across the valve

PDA Classically the machinery continuous

murmur heard over upper left chest ?Why continuous

Sounds “extra-cardiac” May be tricky in infancy – often systolic only Pulse pressure should be wider

PDA - Echocardiogram

PDA - Echocardiogram

Strategy In general – not a crisis May be very stressful to family Suddenly child is lethargic or dyspneic!

Confidence in your management will assist family

Importance of discussing murmur with family – my perspective

Defending your colleagues’ ears!

Strategy Continued In otherwise asymptomatic child Observation usually very acceptable to

family ? Prudent suggestion for SBE precautions

until definitive diagnosis; certainly not usually needed

An EKG and CXR may be very beneficial – especially if murmur persists

My Strategy If exam suggests benign murmur and

EKG/CXR demonstrate no concerns…. Educate family Follow up in 2 years unless there are

other issues (such as small child, symptoms that are likely unrelated, or subtle dysmorphic features)

Smart Shopping Visit to Dr. Z Level 3 new consult - $452.20 EKG - $228.30 CXR - $78.00 – $235.50 TOTAL: $758.50 - $916.00 ECHO: $2,973.30

Potential savings on benign murmur: $2,057.5 - $2,214.8

The Horrors of the ECHO-only Expensive and Non-Exact Test I only see what the technician shows me

Best echocardiograms are done in my clinic Difficult to know what I’m looking for given no

clinical information Missed diagnoses Certainly not defensible Not a fishing tool Often causes “problems” – PFO, TR, silent PDA

However ….

“Echocardiography is the greatest invention of the modern world”

ECHOCARDIOGRAPHY Used to confirm diagnosis or evaluate

hemodynamics My bias – should have cardiologist

involved before the echo if done for murmur Has essentially made diagnostic cardiac

catheterization in childhood unnecessary

KEY POINTS REGARDING MURMURS Important slide #2 Most murmurs in an otherwise healthy child

are benign Not usually a crisis – exams over time very

helpful You will not miss something urgently

important if perfusion/saturations are normal and child is thriving

Benign murmurs are benign Practice listening!! My number 319-560-9288; my pager 0705

Primum ASD - Color

ASD – Primum defect

PEDIATRIC CHEST DISCOMFORT

Notice the wording!! Very common complaint in clinic and ER Most important thing to know: Almost

ALL pediatric chest discomfort has a non-cardiac etiology

Rare cases get a lot of media attention Parental anxiety often drives the evaluation

Chest Discomfort Etiologies

Idiopathic (40%) Musculoskeletal (35%) Costochondritis/Tietze syndrome Muscle strain/trauma

Pulmonary – asthma, infection, pleuritis (10%) Gastrointestinal/Esophageal (5%) Psychogenic – conversion, relative with recent MI

(10%) Cardiac - rare

Chest Discomfort Evaluation

Thorough history including family history Physical Exam – Don’t forget to palpate

chest wall! An EKG and CXR are reasonable tests

Chest Pain Red Flags Important slide #3 History of Kawasaki disease, CTD, or Turner syndrome History of d-TGA or Ross procedure - coronaries Chest pain only during exertion Chest pain associated withsyncope/palpitations Abnormal cardiac examination The very common sharp, stabbing, and random

midsternal or left sided pain which lasts for a few seconds-to-minutes is not too worrisome (precordial catch syndrome)

Pain associated with a host of other symptoms is usually not too worrisome (CNS component)

Cardiac Chest Discomfort Pericarditis – usually ill with fever; friction rub on

exam, pain constant and worse when supine Tachycardias may be reported as “pain” LV outflow tract obstruction – aortic stenosis,

hypertrophic cardiomyopathy History of Kawasaki disease – RED FLAG Coronary artery anomalies – exertional angina if lucky

enough to have symptoms Coronary vasospasm – think drug use (cocaine) Dissecting aortic aneurysm in CTD or Turner

syndrome

Treatment of Chest Discomfort Education and reassurance that the discomfort is not

related to the heart Point out recurrent/chronic nature of these symptoms Treat underlying problem if known Scheduled ibuprofen/naprosyn Ice/heat Tight sports bra (for the female adolescents) Usually no physical restrictions needed but patient should

be aware that exercise may exacerbate the discomfort Rarely – pain clinic, chiropractic care, psychologic care to

deal with the discomfort

Fetal Echocardiography

Fetal Echocardiography - TGA

Fetal - AVC

Syncope Very common in adolescence 15.5%-22.3% of teenagers have at least one

episode Often a familial tendency 70-80% of cases are secondary to a neurally

mediated cause (“vaso-vagal”)

Syncope – Mechanisms Decrease in perfusion of blood to the brain Drop in arteriolar resistance: neurally mediated

vasodepression, autonomic neuropathy Drop in preload: hypovolemia, autonomic

neuropathy Decrease in cerebrovascular tone: migraine,

hyperventilation Change in cardiac function: arrhythmia,

bradycardia, output issue

Syncope Etiologies - Frequencies Autonomic-mediated 73.0% Unknown origin 18.9% Cardiac 2.9% Psychiatric 2.3% Neurologic 2.1% Metabolic 0.6% Hyperventilation 0.2%

Zhang, Acta Paediatric 2009:98:882

Syncope – Usual Scenarios Most syncope occurs with standing for

prolonged periods or a sudden change to an upright position

Shower, hair grooming, choir, urination, warm environment, stressful situations, lunch lines, church

Usually poor fluid intake associated Patient often has warning or aura

Syncope – First Responder ABCs of resuscitation Check pulse and BP (orthostatic) if

possible – helpful in determining cause Get student flat and prop up legs Get student out of harm’s way and check for

injuries DO NOT get up walking or drinking until

back to normal sensorium DO NOT let student go off alone

Syncope – Red Flags Important Slide #4 Sudden and without any warning Association with tachycardia/palpitations During exercise Must differentiate syncope during exercise

from generalized collapse (exhaustion) following exercise

Injury Seizure Psychiatric causes

Syncope Evaluation Assessment of incident as soon as possible – the

details are important Talk to by-standers, parents, coaches Ideal if witness (school nurse) could write

down a narrative of event including physical assessment/vital signs

H&P with orthostatic vital signs Significant if BP drops 15-20 mmHg or if pulse

increases >20 bpm My opinion - patients with syncope should have an

EKG

Syncope Treatment The Zittergruen Trinity

INCREASE FLUID INTAKE SIGNIFICANTLY INCREASE SALT INTAKE VITAMIN WITH IRON

Beverages during the school day Need to use restroom frequently

Awareness Chronicity and ownership Exercise – legs/core and wall standing to “retrain

baroreceptor reflexes” Medications

Midodrine Florinef Beta blockers

Coarctation

Rhythm Issues in the Office Biggest question: who is concerned? Many children have an intermittent

sensation of their heart beating fast or irregular Some children find sinus tachycardia

bothersome Must distinguish pathological rhythms

from physiologic rhythms and then formulate a treatment plan

Couple Things to Consider…. Children/adolescents have a wide variation

in pulse rate throughout the day Maximum heart rate is usually 220-age Gym teachers (enough said)

Sinus arrhythmia is common Heart rate speeds up during inspiration and

slows down during expiration P-waves do not change

Sinus Arrhythmia

A Few More Things Many anxious children/adolescents have

bothersome tachycardia Panic attack (If the shoe fits….)

Medication and drugs I see a lot of patients who receive ADHD/psychiatric

drugs with sinus tachycardia

Elevated average heart rate Caffeine Symptoms often occur at bedtime

Tachycardia/Palpitations Assessment of rate and rhythm during

symptoms is the best way to arrive at diagnosis

Palpation and counting “Too fast to count”

How does student look/act during symptoms

Diagnostic Tests EKG – look for any conduction

abnormalities, measure QTc, look for pre-excitation Event Monitor – best if trying to

capture an infrequent event Holter Monitor – best if symptoms are

daily or to quantitate number of ectopic events

Irregular Heart Rhythms in Infants Usually due to premature ventricular contractions

(PVCs) or premature atrial contractions (PACs) Often transient and related to high circulating

catecholamines in infant after birth Document with rhythm strip or EKG If infant appears well, usually re-evaluate at 2

weeks and refer to cardiology if persisting at that time

Supraventricular Tachycardia

SVT SVT very common in first few days of life and

adolescence Usually secondary to a bypass tract Acute treatment: Ice water to face in infants Vagal maneuvers Adenosine – 50 micrograms per kilogram to

start Cardioversion – First choice if unstable patient

Chronic treatment: usually beta-blockers or digoxin

EKG, echocardiogram

Atrial Flutter

Tachycardia/Palpitations Treatment depends on etiology Often the problem “resolves” after diagnosis Beta blockers for tachycardia Implication for gym/activities

Fluid and rest Avoidance of caffeine/stimulants Assessment and treatment of stress/anxiety

Significant Bradycardia - Infants Term baby may have dips in heart rate to 85-90 bpm

range when asleep but usually over 100 bpm If concerned, document that rhythm is sinus by EKG Heart block may be 2nd or 3rd degree Neonatal heart block needs further evaluation

Associated with l-TGA and more complex lesions Can be associated with a systemic maternal illness (SLE)

3rd degree HB usually requires pacer for normal growth and development of infant

Significant Bradycardia - Adolescent May be physiologic in well-conditioned athlete Heart rate should be > 30 bpm – especially when

awake Holter monitor to assess minimum rate, average

rate, and to look for any heart block Consider anorexia if average heart rate is slow Wenckebach rhythm is usually benign but I would

suggest a referral – few hoops to jump through New heart block – what diagnosis to exclude?

QUESTIONS???