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Benjamin Jacob, PA-C, MPASTexas Children’s HospitalCardiology

Pediatric Cardiology: You don’t want to Miss this.

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• To help Physician Assistants identify sometimes subtle signs and symptoms that may indicate significant cardiac pathology in the pediatric population.

• To help understand some of the significant sequelae of certain undetected cardiac diagnoses.

• To suggest practice modification in physical exam and history taking that may help rule in or rule out cardiac etiologies.

Goals of this presentation.

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• Syncope with exertion.• Chest pain with exertion.• Diastolic murmur.• Failure to thrive in

infants.• Family history of Sudden

Cardiac Death.

The Short List of Red Flags

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• Family History Red Flags• Sudden or unexplained death under

the age of 40.

• Family members requiring pacemaker or defibrillator under 50.

• History of arrhythmias.

• Infants with SIDS

• Heart Disease or heart failure under the age of 50.

The Cardiac History

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Key Points of the Cardiac Exam

• The precordium• Active vs Quiet, Displaced PMI

• Hepatomegaly• Auscultation

• Know Normal vs Abnormal

• Pulses• Brachiofemoral delay in infants

• Capillary refill• Respiratory

• Rate, Retractions, flaring, head-bobbing

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• Clicks, and rubs, and gallops… Oh my!

• Early systolic, holosystolic, midsystolic

• Crescendo/decrescendo, machine-like, Rumble, Radiating to the axilla.

• 1/6, 3/6, 1/4

Murmurs

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• KNOW NORMAL!!!• Systolic or Diastolic

• Diastolic murmurs are pathologic.

• Feel a pulse to differentiate.

• New murmur in a known patient.

Simplified Murmurs• Innocent vs. Pathologic

• Stills murmur- humming, musical loudest between LLSB and the apex.

• Vibratory flow murmur• Increased in high-output states (i.e. fever, exertion)

• Peripheral pulmonary stenosis- radiates to the axilla.REFER IF UNSURE!

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• Chest X-Ray• Cardiac Silhouette

• Cardiomegaly defined as heart width >50% thoracic width

• Lung Fields

• EKG• Inexpensive and available.

Initial Tests

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• Adolescent chest pain is common

Chest Pain• Is it cardiac? • Idiopathic (52%)• Musculoskeletal (36%)• Respiratory (7%)• Gastrointestinal (3%)• Cardiac (1%)

• 37 pts out of n=3700 Sabeen et al

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Chest Pain Management Algorithm

• Pain with exertion , Palpitations associated, Pain while supine improves with leaning forward

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• From History:• With Exertion.

• Syncope while supine.

• Caused by startling noise

• Family history of unexplained death at an early age.

Alarming Syncope Findings• From EKG:

• Long QTc• Deep Q waves• Brugada pattern• ST changes• Ectopy- PVCs, PACs• Heart Block• Pre-excitation- WPW

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2 month old male born to a G2P1 now 2 mother via normal vaginal birth with uncomplicated delivery. Mother brings the baby to his PCP with concerns that he is having trouble finishing feeds because he is frequently stopping. If he does finish his bottle he takes about 40 minsto 1 hour to do so. He has become sweaty with feeds or when crying.

Clinical vignette #1VitalsRR 65 HR 158 BP 85/55 Sats 98% Temp 98.5Physical exam Constitutional: Small for age (5th percentile on growth chart)HEENT: Moist mucous membranes. Mild nasal flaring. Resp: tachypnea, moderate subcostal retractions. Bilateral lung sounds have mild crackles at the bases.Cardiac: Normal S1, S2. you hear a II/VI holosystolic murmur that is loudest at the lower left sternal border. Abdomen: Liver edge is palpated 2 cm below the RCM. Non-tender. Non-distended.

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Vignette #1 Cont.

• Diagnosis?

VSD

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• Signs• S1 coincident

HOLOSYSTOLIC murmur

• Unrestrictive VSDs may not have loud murmurs.

• Cardiomegaly and pulmonary edema on CXR with Dilated Left Ventricle.

Ventricular Septal Defect (VSD)

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• Symptoms• Tachypnea

• Pulmonary Edema secondary to overcirculation.

• Poor Feeding• Frequent breaks, takes 40-60

mins to finish a bottle.

• Poor Weight Gain

Ventricular Septal Defect (VSD)

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• Irreversible pulmonary hypertension.

• Eisenmenger’s Syndrome• Extreme Pulmonary Hypertension

due to muscularization of pulmonary vasculature.

• Significant RVH• Severe clubbing of digits• Cyanosis due to VSD changing

direction (now shunting R->L)

What happens if VSDs are missed?

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• An 17-year-old male, who is a active high school student with no past medical history has sudden loss of consciousness in the 3rd quarter of a Football game. CPR is administered by bystanders on the sideline. On arrival of emergency medical professional, he has regained consciousness. The family history is significant for a murmur in his father and paternal grandmother only.

Clinical VignetteCardiac exam

Normal S1, Fixed split S2. Systolic ejection murmur that increases in intensity when going from a supine to a standing position and disappears with squatting.

Hyperactive Precordium

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• CXR- Normal except for 1 broken rib

• EKG- Normal sinus rhythm, LVH, Inverted T-waves.

Tests• Echo- Parasternal Long Axis

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• Genetic cardiac disease leading to significant hypertrophy of the myocardium of the left ventricle without notable dilation or other precipitating cardiac cause.

• Prevalence thought to be as high as 1:500 of the general population. Allen et al

Hypertrophic Cardiomyopathy• Increased risk of sudden

Death. (1-2% per year) Maron et al

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• Murmur is variable dependent on dynamic LV outflow obstruction• Valsalva will increase the

murmur.

• Squatting will decrease. • Increased SVR, Increased

Preload

• Hyperdynamic Precordium

Hypertrophic Cardiomyopathy

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• Symptoms• Exertional dyspnea

• Heart failure symptoms don’t typically present until early adulthood, ages 20-40. (But they can!)

• Aborted sudden death.

HOCM• EKG- Abnormal in 90-95%

of patients with HCM. Allen et al

• No Reliable pattern but can be useful to indicate further testing.

• Amplitude of R waves (LVH) has poor correlation to degree of hypertrophy on echo.

• Inversion of T waves.

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HCM Sudden Death in Athletes• Most common cause of SCD in

Athletes. Maron et al

• Part of the hot topic of obtaining EKGs with sports physicals.

• Most recent AHA recommendation in 2014 is not to require EKG Screening

• Estimates show SCD about 1 in 80,000 to 1 in 200,000 participants nationally. Maron et al

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A previously healthy 1-year-old female was admitted to a children's hospital with a 7-day history of spiking fever up to 103°F (39.5°C). Three days after the onset of fever she developed left-sided neck swelling and became progressively fussy and irritable. She was seen at an emergency room, diagnosed with cervical adenitis, and sent home on oral antibiotics.

The mother noted continued irritability, high fever, and decreased oral intake. On subsequent admission she was extremely irritable, with a temperature of 102°F (38.9°C), heart rate of 140 beats per minute, respiratory rate of 40 breaths per minute, and blood pressure 110/54 mmHg. There were no signs of nuchal rigidity.

Clinical VignettePhysical exam

Both palpebral and bulbar conjunctivae were deep red and injected, lips were dry and crusted, the oropharynx has some areas of ulcerated mucosa, and the tongue papillae were enlarged and red. Examination of the neck revealed a mildly tender left unilateral mass, measuring 4 cm. The skin showed a generalized polymorphous, erythematous, macular, blanching rash, in addition to severely red and desquamated perineal region. Her extremities, especially palms and soles, were swollen, red, and mildly tender.

A. Epocrates online

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Diagnosis?

Kawasaki Disease

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Kawasaki Diagnosis• > 4 days of Fever + 4/5

Diagnostic features.• 5 Diagnostic Features

• Nonexudative conjunctivitis• Unilateral Cervical

lymphadenopathy• Oral mucosa erythema

(strawberry tongue)• Rash• Peeling of hands and feet

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• Acute Phase Cardiac Symptoms- Kawa-shocky• Think “Myocarditis” picture

• Tachycardia out of proportion to febrile tachycardia.

• Gallop or soft heart sounds may be present.

Kawasaki Disease• EKG

• Decreased QRS voltage• Sinus tachycardia• Flattened T-Waves

• Echo• Key test to identify and monitor

developing aneurysm.• Also can show extent of

depressed function and wall motion abnormalities

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• IVIG to help control inflammatory response in first 7-10 days. Newberger et al

• High dose aspirin until >48 hours with no fever. Then continue reduced dose for 6 weeks.

Acute phase treatment

2017 Kawasaki Guidelines- 72 page guide to diagnosis and

management of KD.

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• 2 or 3 of the major criteria.

• Watchful waiting when appropriate.

• Labs• ESR, CRP, WBC

Incomplete KD

Circulation. 2017;135:e927–e999. DOI: 10.1161/CIR.0000000000000484

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• Most common acquired heart disease in children in the US. Newberger et

al

• Development of coronary artery aneurysm can lead to myocardial ischemia via arterial intimal thickening.

• Coronary aneurysms are easier to miss in incomplete Kawasaki Disease.

• Echos typically with initial suspicion then at 2 and 6 weeks after onset.

Cardiac Implications

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• Risk stratification for ischemia with coronary aneurysm is difficult and has become quite complex.

• Longitudinal follow up with stress testing, CMR, exercise, or (Sestimibi) can be used to determine need for intervention either surgically or by cath.

Coronary aneurysms

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• Myocardial ischemia due to coronary thrombosis leading to sudden death.

• Heart failure secondary to infarction and ischemia.

What happens if it’s missed

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• 10 week old who presents in your office because mother states that he is having discomfort with eating. He seems hungry and wants the bottle but after a few minutes he stops and screams uncontrollably in discomfort, he sometimes gets pale and sweaty with these episodes. He may be able to resume after about 5 to 10 minutes. These events are very intermittent. He has been more tired over the last week. There has been no reported emesis with these events.

Clinical Vignette (Challenging diagnosis)

• Physical Exam• Vitals- RR 60, HR 160, BP

87/45 , Temp 97.8, Sats 97%• Cardiac- You think you year

an extra sound most consistent with a gallop.

• Respiratory- mildly tachypnic, no retractions

• Abdomen- liver felt 3 cm below the RCM.

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• CXR• Cardiomegaly

• Mild interstitial edema

Tests

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EKG

Anomalous Left Coronary Artery from the Pulmonary Artery.

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• Babies with Angina.• Present around 8-12

weeks after PVR drops.• Also called Bland-White-

Garland

Anomalous Left Coronary artery from Pulmonary Artery (ALCAPA)

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ALCAPA

• Collaterals develop to help supply hypoxic myocardium.

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• Physical Exam• Laterally displaced Point of

maximal impulse (PMI)

• Sometimes with blowing holosystolic murmur of mitral regurgitation.

• Gallop rhythm

ALCAPA

• CXR• Cardiomegaly with Dilated

left heart

• Could see pulmonary congestion secondary to left heart failure

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• This can easily be misdiagnosed as colic or reflux.• If the child is stopping in the

middle of feeds with inconsolable crying.

• Heart failure symptoms are common: Sweaty, pale, tachypneic.

• ~1 in 300,000 live births Kiethet al

Its Rare but Don’t Miss It!• Mortality

• 87% present in infancy. Neufeld et al

• 65%-85% of the infants that present die before the age of 1 due to heart failure symptoms without surgical intervention. Wesselhoeft et al

• Early detection and surgical intervention is crucial

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• Q waves in I and AVL• Q waves in V4-V6• ALCAPA needs to be

ruled out.• Anterolateral infarcts.

ALCAPA EKG

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• Inflammation of the cardiac myocytes with necrosis that is not in a pattern typical of coronary ischemia.

• Can have many causes• Causes 46% of Dilated

Cardiomyopathy (DCM) Towbin et al

Myocarditis• Many Viral causes

• Parvovirus B19, adenovirus 2 and 5, coxsackie B

• Many other viruses have been identified causes of myocarditis.

• Medications, parasites, fungi, and autoimmune causes as well

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• Flu-like symptoms • Fever

• Poor appetite

• Vomiting.

• Listlessness

Presentation

• Congestive Heart Failure• Tachypnea

• Hepatomegaly

• Tachycardia

• Pallor

• Gallop on auscultation

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• CXR• Cardiomegaly

• Increased vascular markings

• Pulmonary Edema

• Warning- May be normal if early in the clinical course

Tests• EKG

• Low voltage QRS complex• Inverted T waves• ST elevation/depression

consistent with myopericarditis

• Can present with VT, Afib, SVT, Complete AV Block and cause sudden death

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• Helpful labs• CKMB

• Troponin

• Trending BNP- helps with treatment management more than diagnosis

• Viral studies for most common viruses can aid in diagnosis.

• Echo• Standard imaging modality to determine

Ejection fraction and wall motion abnormalities.

Diagnosis

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• Management of heart failure.• Diuretics for symptoms

• ACE inhibitor for reverse remodeling and afterload reduction

• B blocker for rate control and mortality benefit.

• Treatment with steroids, IVIG can be considered if acute presentation.

Treatment• May require mechanical support

with VA ECMO, LVAD as bridge to transplant or bridge to recovery.

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• A 2 year old female is following up in your clinic for a well child check. You have been worried that her BP has been elevated, but have not yet decided on medical therapy. You blame some of this on her agitation and anxiety while in your office. On this visit her BP documented by the clinic RN measures 132/78. You decide to have the nurse repeat BP measurement in the other arm and get 129/76.

Last Vignette • Physical Exam

• Vitals- RR 60, HR 160, BP 87/45 , Temp 97.8, Sats 97%

• Cardiac- Normal S1, intermittent split S2, soft. Radial Pulses 2+ bilaterally, Femoral pulses are difficult to find but are 1+.

• Respiratory- Clear to auscultations bilaterally, but you notice a continuous murmur while listening to the back

• Abdomen- No distention or organomegaly noted.

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• CXR- Normal• ECG

• Normal sinus rhythm, LVH

• 4 extremity BPs• RUE- 132/78

• RLE- 100/60

• LUE- 129/76

• LLE- 102/62

Work-up?

• Echo Pedecho.org

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• 3 Typical presentations• Infant with heart failure

• 8-10 days of life

• Child with murmur

• Child/Adolescent with hypertension

Coarctation of the Aorta

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• Brachio-femoral delay• Diminished lower extremity

pulses.• 4 ext Blood pressure with a

gradient of >20 mmHg systolic.

• Continuous or systolic murmur heard at the scapular border.

Coarctation• EKG

• Can have LVH due to fixed obstruction.

• Left atrial enlargement

• Echo is standard • Establishes anatomic and hemodynamic

markers

• CT angio• Catheterization

• Diagnostic and Therapeutic

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• Secondary to arterio-venous connections with large left to right shunt

• Vein of Galen malformation

• Liver Hemangiomas

• Cutaneous AVMs

• Leads to Right heart dilation, cardiac insufficiency and pulmonary hypertension.

• Looks like the heart, but this time the problem isn’t the heart!

High Output Heart failure

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• Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult. 8th ed. Vol. 1,2. Philadelphia: Wolters Kluwer Lippincott Williams & WIlkins, 2013.

• Saleeb SF, Li WY, Warren SZ, Lock JE. Effectiveness of screening for life-threatening chest pain in children.. Pediatrics. 2011 Nov; 128(5):e1062-8.

• Friedman KG, Alexander ME. Chest Pain and Syncope in Children: A Practical Approach to the Diagnosis of Cardiac Disease. The Journal of pediatrics. 2013;163(3):896-901.e1-3. doi:10.1016/j.jpeds.2013.05.001.

• Maron BJ, Towbin JA, Tiene G, et al. Contemporary definitions and classification of cardiomyopathies: an American Heart Association Statement from Council on Clinical Cardiology, Heart Failure and Transplantation committee; Quality Care and outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; Council on Epidemiology and Prevention. Circulation 2006 113:1807-1816

• Newberger JW, Takahashi M, Gerber MA, Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Theumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young. Ciculation 2004; 110: 2747-2771

References• Neufeld HN, Scheeweiss A. Coronary artery Disease in Infants and

Children. Philadelphia, PA: Lea & Febiger, 1983

• Wesselhoeft H. Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 casses with seven further cases. Circulation 1968; 38:403-425

• Towbin JA, Et al, Incidence, causes, and outcomes of dilated cardiomyopathy in children. JAMA 2006; 296:1867-1876

• Maron BJ, Levine BD, Washington RL, Baggish AL, Kovacs RJ, Maron MS. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 2: PreparticipationScreening for Cardiovascular Disease in Competitive Athletes. Circulation. 2015;132:e267-e272

• https://online.epocrates.com/diseases/23622/Kawasaki-disease/Common-Vignette

• Newberger JW, Takahashi M, Gerber MA, Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Theumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young. Ciculation 2004; 114:1708-1733.

• Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J. 1959;21:149–61.

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Questions and Discussion