9/14/2016 1 Benjamin Jacob, PA-C, MPAS Texas Children’s Hospital Pediatric Cardiology for the PA in primary care. What Not to Miss. PAGE 1 • To help Physician Assistants in primary care identify signs and symptoms that may indicate significant cardiac pathology in the pediatric population. • To understand some of the significant sequelae of missed cardiac diagnosis. Goals of this presentation. PAGE 2 • Syncope with exertion. • Diastolic murmur. • Failure to thrive in infants. • Chest pain with exertion. The Short List of Red Flags
Transcript
9/14/2016
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PAGE 0
Benjamin Jacob, PA-C, MPAS
Texas Children’s Hospital
Pediatric Cardiology for the PA in primary care.
What Not to Miss.
PAGE 1
• To help Physician Assistants in primary care identify
signs and symptoms that may indicate significant cardiac
pathology in the pediatric population.
• To understand some of the significant sequelae of
missed cardiac diagnosis.
Goals of this presentation.
PAGE 2
• Syncope with exertion.
• Diastolic murmur.
• Failure to thrive in
infants.
• Chest pain with exertion.
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.
• History of arrhythmias.
• Infants with SIDS
• Heart Disease or heart failure under the age of 50.
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 #1
VitalsRR 65 HR 158 BP 85/55 Sats 98% Temp 98.5Physical exam 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?
Large VSD
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• Signs
• S1 coincident
HOLOSYSTOLIC murmur
• Large 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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• Eisenmenger’s Syndrome
• Irreversible Pulmonary
Hypertension due to
muscularization of pulmonary
vasculature.
• Significant RVH
• Clubbing of digits
• Cyanosis
What happens if we miss VSDs?
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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 consciousnessin 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 grandmother only.
Clinical Vignette
Physical exam
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.
• Vigorous Precordial thrust
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.
HCM
• 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 Vignette
Physical 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
• Cervical lymphadenopathy
• Oral mucosa erythema
• Rash
• Peeling of hands and feet
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• Acute Phase Symptoms
• 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
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• 2 or 3 of the major
criteria.
• Watchful waiting when
appropriate.
• Labs
• ESR, CRP, WBC
Incomplete KD
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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.
KD- Coronary Aneurysm
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• Risk stratification for ischemia with coronary aneurysm is difficult.
• Longitudinal follow up with stress testing (Sestimibi) can help determine need for intervention either surgically or by cath.
Coronary aneurysms
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• Ongoing myocardial
ischemia 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 in discomfort, he gets pale and sweaty. But 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.
• 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
• 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.
