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Copy of Pediatric Cardiology for the General Practitioner

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Frances Woodard

on 15 February 2017

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Transcript of Copy of Pediatric Cardiology for the General Practitioner

Primary Care Guide to Pediatric Cardiology
The incidence of congenital heart defects is 1 % in live births in the United States (~40,000 births/year).
Most are caused by genetic-environmental interaction (multifactorial inheritance)
Fetal Circulation
Down Syndrome (Trisomy 21)
: Most common chromosome abnormality with 40% having a cardiac defect

DiGeorge Syndrome (Deletion 22q11, velocardiofacial)
: 75% will have cardiac defects including IAA, truncus arteriosus, or TOF

Turner Syndrome
: Cardiac anomalies occur 35%-55% of the time and are mainly left-sided.

Marfan Syndrome
: Connective tissue disorder and aortic root dilation (high risk for aortic dissection)

Williams Syndrome
: Cardiac defect is supravalvar aortic stenosis and coronary artery abnormalities

Fetal

Circulation
Syndromes with Associated Congenital Heart Defects
Tests Interpretation
Obtaining the History...
Vital signs
Oxygen saturations
Heart rate and respiratory rate (look at trends)
Upper and lower extremity BP
CXR (cardiac size, pulmonary vascular markings)
EKG (to be read by a pediatric cardiologist)
Most important clues are going to be from history, physical exam and vital signs
MURMURS
Grade/Intensity
Quality
Where is cardiac cycle does it take place (systole or diastole)
Location
Transmission
Age when first detected
Innocent vs Pathologic Murmurs
Innocent
Usually systolic
Low pitch, vibratory
Loudest at LLSB or 2nd/3rd ICS
No louder than a grade III
Changes intensity with positioning
Normal vital signs and development
Pathologic
Systolic and/or diastolic
Harsh
Heard throughout all auscultation areas
Grade I-VI
Doesn't change with positioning
Concern with vital signs and clinical appearance
Congenital Heart Defects

Atrial Septal Defect (ASD)
Ventricular Septal Defect (VSD)
Atrioventricular Septal Defect (AVSD)
Patent Ductus Arteriosus (PDA)
Aortic Stenosis (AS)
Pulmonary Stenosis (PS)
Coarctation of the aorta (CoA)
Vascular Rings/Slings
Partial/Total anomalous pulmonary venous return (PAPVR/TAPVR)
Tetrology of Fallot (TOF)
Anomalous origin of left coronary artery to pulmonary artery (ALCAPA)
Tricuspid Atresia (TA)
Pulmonary Atresia/intact ventriucular septum (PA/IVS)
Transposition of the great arteries
Interrupted aortic arch (d-TGA)
Truncus arteriosus
Ebstein's of tricuspid valve
Double outlet right ventricle (DORV)
Double inlet left ventricle (DILV)
Hypoplastic Left Heart Syndrome (HLHS)
Murmur work-up in all ages
Newborns
Physical Examination (overall appearance, vital signs, respiratory and cardiac exam)
Respiratory (retractions, rate, accessory muscle use)
Cardiac (pulses, murmurs, split s2)
Toddlers
Vital signs and physical exam
Growth and development (how do they plot on growth curve?)
ACTIVITY TOLERANCE

Adolescents
Vital signs and physical exam
Activity tolerance
Symptoms during activity (syncope, chest pain??)
Toddler and School Age
In addition to obtaining a good history and performing a thorough physical exam........LOOK for the following:
The Adolescent and the Athlete
Kawasaki Disease (Treatment)
Red Flags on the History and Physical
Syncope or chest pain during exercise
Murmur that sounds pathological
Decrease exercise tolerance
Acquired Heart Disease
Kawasaki Disease
Myocarditis
Endocarditis

Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)
Etiology is essentially unknown
Diagnostic Criteria:
Fever of at least 4-5 days
4 out of the 5 following:
Bilateral conjunctivitis
Changes in lips and oral cavity
Cervical lymphadenopathy (usually unilateral)
Erythematous rash (trunk and extremities)
Changes in extremities (swollen, erythematous, and tender palms/soles)
Treating with IVIG (intravenous immunglobulin) in the 1st 10 days of the illness in combination with aspirin reduces prevalence of coronary abnormalities from 40% to <4%

No live vaccines (MMR) should be given for at least 5 months after IVIG

High-dose aspirin 80-100 mg/kg/day divided every 6 hours until afebrile for 48 hours, then 3-5 mg/kg daily until next cardiology evaluation (6 weeks).
Kawasaki Disease (Pearls)
Follow-up: 2 weeks, 6 weeks, 6 months, 1 year
May be discharged one year past diagnosis if there are no coronary artery involvement (via echocardiogram
Coronary artery changes seen within 10 days of Kawasaki’s and is rarely seen past six weeks
Diagnosis of atypical Kawasaki’s disease may be made in presence of coronary artery involvement (but work up everything else prior to this!!!)
Myocarditis
Inflammation of the heart muscle
Most common causes are viral infections
Clinical presentation: flu like symptoms, symptoms from arrhythmias, signs/symptoms of congestive heart failure
Diagnostic measures: endomyocardial biopsy (potential), echocardiogram
Treatment (initial): inotropes, afterload reduction, diuresis; (long-term): possible transplant
Summary of AHA guidelines for Prevention of Infective Endocarditis
1. Cardiac conditions associated with high risk from endocarditis for which SBE prophylaxis with dental procedures is recommended:
a. Prosthetic cardiac valves
b. Previous endocarditis
c. CHD: unrepaired cyanotic CHD (including palliative shunts and conduits), completely repaired CHD with prosthetic material or device (by surgical or cath intervention) the first 6 months past procedure, repaired CHD with residual defects at site or adjacent to site of prosthetic patch or device, heart transplants who develop cardiac valvopathy (above cardiac conditions require SBE for respiratory tract, skin, or musculoskeletal tissue procedures)
Endocarditis
Infection of the lining of the heart muscle usually from Streptococcus species
Duke criteria: 2 major, 1 major + 3 minor, 5 minor
Major: + blood culture, (3 separate 12 hours apart), mass on echo, new valvar regurgitant jet on echo
Minor: h/o drug use, CHD, fever, Janeway lesions, conjunctival hemorrhage, Osler nodes, Roth spots
Treatment is 6 weeks of antibiotic therapy with surveillance blood cultures and CBCs
Myocarditis
Inflammation of the heart muscle
Most common causes are viral infections
Clinical presentation: flu like symptoms, symptoms from arrhythmias, signs/symptoms of CHF
Dx: endomyocardial biopsy
Treatment (initial): inotropes, afterload reduction, diuresis; (long-term): transplant
d. Dental procedures not requiring SBE: routine anesthetic injections thru non-infected tissue, taking dental radiographs, placement of removable prosthdontic/orthodontic appliances, adjustment of orthodontic appliances, placement of orthodontic brackets, shedding of deciduous teeth, and bleeding from trauma to lips or oral mucosa


No SBE prophylaxis needed for GU or GI tract procedures
Summery of AHA guidelines for Prevention of Infective Endocarditis... con’t
Grade
I: barely detected
II: < heart sounds
III: equal or greater than heart sounds
IV: thrill
V: thrill and stethoscope off chest
VI: no stethoscope
Quality
harsh, blowing, vibratory

Cardiac cycle
short systolic, mid-systolic, diastolic, holosystolic
Location
apex (mitral valve), LLSB (tricuspid valve) – all S1, ULSB (pulmonary artery) , URSB (aorta)
Transmission
back and axilla
***
In a neonate, what is more important than identifying a murmur is the ability to split S2
***
Describing the Murmur...
Chest Pain
History!! History!! History!!
How long has the pain been occurring?
How many times a day does it occur?
Does chest pain occur at rest or with exercise?
Describe chest pain (burning, sharp)
What makes it worse or better?

General practitioner
diagnosis with a pediatric cardiology consult!!!
pedcard.musc.edu
General Pediatric Cardiology Guide
Frances Woodard, PNP
Pediatric Cardiology
MUSC
Case Study #1
You are assessing a neonate 4 hours after birth for the first time in the nursery.
Describe your physical exam.
What do you want to know regarding prenatal history?
The baby's vital signs are as follows: heart rate 150, blood pressure (right arm) 60/30, respiratory rate 65, oxygen saturations: 88% room air
What are some red flags and what additional information would you like?
Based on the above information what are you going to do next?
Case study #2
You are seeing a 10 year old for an initial visit. His family just moved into town and they are establishing care.
He complains of chest pain when he runs during soccer.
What key questions are you going to ask?
His vital signs are as follows: afebrile, heart rate of 120, blood pressure 85/40, respiratory rate of 16, oxygen saturations 97% on room air
You hear a murmur on exam.
What other diagnostic tests are you going to order?
Based on the information that you obtain what are your next steps?
The Athlete and Sudden Cardiac Death
Most frequent causes of chest pain in children:
Musculoskeletal (costochondritis)
Acid Reflux
Lung disease (asthma)
Most common cause of sudden cardiac death in athletes is hypertrophic cardiomyopathy (HCM) (1/3 of all cases).
The next common causes of sudden cardiac death in athletes are coronary abnormalities and arrhythmias
Diagnostic tests to work up the above concerns are EKG and echocardiogram
The Athlete and Sudden Cardiac Death...con't
If the United States screened every athlete with an EKG and echocardiogram, the average cost per year would be $2 billion.

AHA recommends a thorough physical exam and complete family and personal history (12 key AHA recommended elements to use for screening) because of the substantial size of the athlete cohort, the low prevalence of sudden cardiac death in sport related activities, and the limited resources.
Key elements for Sports Clearance
Personal History (4)
Chest pain
Unexpected pre-syncope/syncope
Unexplained dyspnea/fatigue with exercise
Elevated systolic blood pressure

Family History (3)
Premature sudden death
Heart disease in relative <50 years
Family members with HCM, long QT syndrome or Marfan features

Physical Exam (4)
Murmur
Femoral pulses
Physical features of Marfan
Blood pressure


Cardiac Findings in Kawasaki Disease
Major sequelae of Kawasaki disease is related to the coronary arteries
Rarely aneurysms form prior to 10 days of illness but echocardiographic findings may include ventricular dysfunction, coronary artery dilation, or mitral valve regurgitation
Patient may present with tachycardia
Also, may hear mitral regurgitation murmur or gallop on exam
Case Study #3
You are seeing a two year old in clinic for a routine well-child check whom you have been following since birth. You detect a new murmur on exam.
What questions are you going to ask her parents?

Her vital signs are as follows: heart rate 110 (she has been running around the room), blood pressure 85/40, respiratory rate 20s, oxygen saturations: 100% room air
Based on the above information what are you going to do next?
You are seeing a one month old in clinic. His parents are concerned that he has been more tachypnic and appears blue on occasion. You detect a new murmur on exam.
Describe your physical exam.
What questions are you going to ask his parents?
His vital signs are as follows: heart rate 130, blood pressure 80/40, respiratory rate 70s, oxygen saturations: 80% room air
What are some red flags and what additional information would you like?
Based on the above information what are you going to do next?
Case Study #4
You are seeing a three year old in clinic for a fever for 4 days. You detect a new murmur on exam.
What questions are you going to ask her parents?

Her vital signs are as follows: heart rate 130s, blood pressure 90/45, respiratory rate 20s, oxygen saturations: 100% room air
Describe what you will evaluate for on
physical exam.What happens next??
Case Study #5
*
Prenatal/Pregnancy history: advanced maternal age, teratogens, maternal diabetes (uncontrolled), medications (Dilantin), smoking, alcohol use, fetal distress (either prenatal or perinatal)
*
Birth/Infant history: birth weight, gestational age, complications during/after delivery, when murmur first identified, genetics, feeding regimen including volume at each feed, duration, or any symptoms (diaphoresis, cyanosis)
*

Family history: family members with CHD, h/o SIDS, or sudden death at a young age
Syncope
Most common is neurogenic vasovagal syncope (church fainting)
Treatment includes: recognition, anti-gravity measures, supra-hydration, minimizing caffeine intake
If above fails, then electrophysiologist evaluation is indicated
POTS (postural orthostatic tachycardia syndrome) is a thing of the past!!!!

My Heart is Beeping..........
Most common causes: high caffeine intake, stimulants, dehydration
Screening tests: EKG
Monitors for intermittent palpitations: Holter, ACT monitor, looping event monitor
Usually activity is not restricted (unless having other symptoms such as syncope)
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