Download Recognition of Cardiovascular Disorders

Recognition of Cardiovascular Disorders
Daniel J. Murphy, Jr., M.D.
Professor of Pediatrics (Cardiology)
Stanford University
Stanford, CA
OBJECTIVES
At the end of this presentation the participant will be able to:
1.
2.
3.
4.
5.
Describe the characteristics of innocent cardiac murmurs
List the causes of chest pain in children
Evaluate the cyanotic newborn
Identify causes, symptoms and treatment of congestive
heart failure and cardiogenic shock in infants and children
Recognize and treat hypercyanotic spells
HEART MURMURS IN CHILDREN
Auscultation Basics
• Stethoscope
• Environment
• Examine all areas
• Changes with respiration and position
HEART MURMURS IN CHILDREN
HEART MURMURS IN CHILDREN
Innocent Murmurs
• Vibratory (Still's) murmur
• Pulmonary flow murmur
• Peripheral pulmonary arterial stenosis murmur
• Aortic systolic murmur
• Venous hum
INNOCENT MURMURS IN INFANTS AND CHILDREN
NEONATAL(PPS)
STILL’S
VENOUS HUM
PULMONARY
FLOW
Medium to high
Low
High
Medium
I-II / VI
I-III / VI
I-III / VI
I-III / VI
Time in Cycle
Mid-systolic
Mid-systolic
Continuous
Mid-systolic
Quality
Pitch
Intensity
Soft ejection
Vibratory, Musical
Buzzing
Soft blowing
Soft blowing
Location
1st & 2nd ICS
RSB, LSB, back
LLSB
R&L
Infraclavicular areas
2nd LICS
Increased by
Increased
Cardiac Output
Supine, fever,
exercise
Sitting
Standing
Supine,
Expiration, C. O.
Decreased by
Decreased
Cardiac Output
Standing,
Valsalva
Supine, head turn,
Jugular compression
Upright,
Inspiration, C.O.
Age Appears
Birth - One week
1 – 10 years
2 – 5 years
7 – 10 years
3 – 4 months
Puberty
7 – 10 years
Persists in adults
Produced by
Relatively small
pulmonary arteries
Vibration in LVOT
Flow in jugular veins
Flow in RVOT
Confused with
Pulmonary Branch
stenosis
Hypertrophic
Cardiomyopathy
PDA
ASD or
Mild PS
Age Disappears
HEART MURMURS IN CHILDREN
Pulmonary Branch Stenosis
• Systolic ejection murmur, grade 1-2/6
• Heard best in axillae and back
• Birth to 2-3 months
• Produced by relatively small size of pulm. arteries
• Confused with peripheral pulm stenosis or PDA
HEART MURMURS IN CHILDREN
Still’s Murmur
• Systolic ejection murmur, grade 1-3/6
• “Musical”, “Buzzing”, vibratory
• Heard best at the mid to lower left sternal border
• One year to adolescence (also in infants)
• Increased by supine position, fever, or exercise
• Vibration in the left ventricular outflow tract
• Confused with hypertrophic cardiomyopathy
HEART MURMURS IN CHILDREN
Venous Hum
• Continuous murmur, grade 1-3/6
• Heard best in the right or left infraclavicular area
• Two to ten years
• Increased in the upright position
• Abolished by jugular compression or head turn
• Produced by turbulent flow in the jugular veins
• Confused with patent ductus arteriosus
1. A loud systolic murmur is heard in a term
newborn at 2 hours of age. Possibilities include
all EXCEPT:
A. Aortic stenosis
B. Pulmonary stenosis
C. Tricuspid regurgitation
D. Large ventricular septal defect
E. Mitral regurgitation
HEART MURMURS IN CHILDREN
“Innocent” Murmurs in the Newborn Infant
• Tricuspid regurgitation
• Closing patent ductus arteriosus
• Peripheral pulmonary stenosis
• Pulmonary flow
• Vibratory (Still’s)
HEART MURMURS IN CHILDREN
Six Cardinal Clinical Signs
• Pansystolic murmurs (VSD, TR, MR)
• Harsh murmurs (VSD, valve stenosis, outflow tract
obstruction)
• Very loud murmurs (≥ grade 3)
• Murmurs heard at the upper left sternal border
• Systolic clicks (aortic or pulmonary stenosis, MVP)
• Abnormal S2 (split-ASD, loud & single-pulm htn)
McCrindle, et al.
Arch Pediatric Adol Med 1996; 150:169
HEART MURMURS IN CHILDREN
Circulation 1994; 90:2180-8
HEART MURMURS IN CHILDREN
When to Refer a Child with a Heart Murmur
• All diastolic murmurs
• All pansystolic murmurs
• Late systolic murmurs
• Very loud murmurs (≥ grade 3)
• Continuous murmurs except venous hums
• Associated cardiac abnormalities
NEONATAL CYANOSIS
CENTRAL
PERIPHERAL
hyperoxia
 pO2
ventilation
 pO2
echocardiogram
NORMAL
PULMONARY
DISEASE
 pO2
PERSISTENT
PULMONARY
HYPERTENSION
ABNORMAL
CYANOTIC
CONGENITAL
HEART DISEASE
COLD
SEPSIS
SHOCK
HYPOGLYCEMIA
POLYCYTHEMIA
3. Arterial blood gas results from an infant with a
cyanotic congenital heart defect should include:
A. Increased PO2 with crying
B. Increased PO2 with increased FI02
C. Elevated PCO2
D. All of the above
E. None of the above
PULSE OXIMETRY NEWBORN SCREENING
 The screening is targeted toward healthy newborn
infants in the newborn nursery.
 Screening should not be undertaken until 24
hours of life
 Oxygen saturations should be obtained in the
right hand and one foot.
PULSE OXIMETRY NEWBORN SCREENING
 Pass: pulse oximetry reading of ≥95% in either
extremity with a ≤3% absolute difference between
the upper and lower extremity
 Immediate evaluation: saturations <90%
 In the event of a positive screening result, CCHD
needs to be excluded with a diagnostic
echocardiogram. Infectious and pulmonary causes
of hypoxemia should also be excluded.
The proposed pulse-oximetry monitoring protocol based on
results from the right hand (RH) and either foot (F).
Kemper A R et al. Pediatrics 2011;128:e1259-e1267
©2011 by American Academy of Pediatrics
SYNDROMES AND CHD
• Down (40-50%)
AV Canal Defect, VSD, TOF
• Turner (35%)
Coarctation, bicuspid aortic valve, AS
• Noonan (80-90%)
Pulmonary stenosis, ASD, HCM
• Williams (60%)
Supravalvar aortic stenosis, coarctation
(del 7q11.23)
• DiGeorge (35%)
(del 22q11)
• Alagille (95%)
Conotruncal malformations (Interrupted
aortic arch, truncus arteriosus, TOF)
Pulm art stenosis, TOF, pulm stenosis
SYNDROMES AND CHD
• Alagille (arteriohepatic dysplasia)
Peripheral pulmonary stenosis, PS, TOF
• Asplenia
Complex cyanotic CHD
• Carpenter
PDA, VSD
• CHARGE association
VSD, ASD
• Cri-du-chat (5p-)
Various congenital cardiac defects
• De Lange
Tetralogy of Fallot, VSD
• Ellis van Creveld
Common atrium (ASD)
• Fanconi
PDA, VSD
• Fetal alcohol
VSD, ASD, tetralogy of Fallot
• Fetal hydantoin
ASD, VSD, coarctation
SYNDROMES AND CHD
• Goldenhar
Tetralogy of Fallot
• Holt-Oram
ASD, VSD
• Infant of a diabetic mother
Hypertrophic cardiomyopathy, VSD, TGA
• Laurence-Moon-Biedl
Tetralogy of Fallot, VSD
• Marfan
Aortic root aneurysm, mitral prolapse
• Multiple lentigenes (leopard)
Pulmonary stenosis
• Polyspenia
Complex CHD
• Rubella
PDA, peripheral pulmonary stenosis
• Rubinstein-Taybi
PDA
SYNDROMES AND CHD
• Marfan
Aortic root aneurysm, mitral prolapse
• Multiple lentigenes (leopard)
Pulmonary stenosis
• Polyspenia
Complex CHD
• Rubella
PDA, peripheral pulmonary stenosis
• Rubinstein-Taybi
PDA
• Smith-Lemli-Opitz
VSD, PDA
• Thrombocytopenia-absent radius
ASD, tetralogy of Fallot
• Trisomy D
VSD, PDA
• Trisomy E
VSD, PDA
• Wolf
ASD, VSD
FAMILY HISTORY AND CV DISEASE
• Dilated Cardiomyopathy
• Hypertrophic Cardiomyopathy
• Marfan syndrome
• Muscular dystrophy
• Long QT syndrome (sudden death)
CARDIOVASCULAR PREPARTICIPATION
SPORTS SCREENING
 Objective: Identification of “silent” cardiovascular abnormalities that
can progress or cause sudden cardiac death.
 Personal history:
 History of heart disease, including Kawasaki disease
 Heart murmur
 Systemic hypertension
 Fatigue
 Syncope/near-syncope
 Excessive/unexplained exertional dyspnea
 Exertional chest pain
 Medication history
 Illicit drug use (including performance enhancing drugs)
CARDIOVASCULAR PREPARTICIPATION
SPORTS SCREENING
 Objective: Identification of “silent” cardiovascular abnormalities that
can progress or cause sudden cardiac death.
 Physical examination:
 Heart murmur (supine/standing)
 Femoral arterial pulses
 Stigmata of Marfan syndrome
 Brachial blood pressure measurement (sitting)
CARDIOVASCULAR PREPARTICIPATION
SPORTS SCREENING
!! RED FLAGS !!
• Syncope or near-syncope on exertion
• Chest pain on exertion
• Excessive dyspnea or fatigue with activity
• Family history: Marfan, cardiomyopathy, long QT syndrome, sudden death
• Irregular rhythm
• Weak or absent lower extremity pulses
• Hypertension
• Loud systolic murmur
• Any diastolic murmur
Singh, et al.
Pediatrics in Rev 2006:27:418-23
• Stigmata of genetic syndromes associated with CV disease
CONTRAINDICATIONS TO SPORTS
PARTICIPATION
• Pulmonary vascular disease with cyanosis
• Severe pulmonary hypertension
• Excessive dyspnea or fatigue with activity
• Severe aortic stenosis or regurgitation
• Severe mitral stenosis or regurgitation
• Cardiomyopathy
• Acute pericarditis or myocarditis
• Vascular form of Ehlers-Danlos Singh, et al.
Pediatrics in Rev 2006:27:418-23
2. All of the following may cause chest pain in
children except:
A. Asthma
B. Costochondritis
C. Anxiety
D. Upper respiratory infection
E. Gastroesophageal reflux
CHEST PAIN IN CHILDREN
Diagnosis
% of Children
______________________________
Idiopathic origin
21
Musculoskeletal origin
15
Cough
10
Costochondritis
9
Psychogenic origin
9
Asthma
7
Trauma
5
Pneumonia
4
Gastrointestinal disorders
4
Cardiac disease
4
Sickle cell disease
2
Miscellaneous
9
Selbst, et al.
Pediatrics 1988; 82:319
CHEST PAIN IN CHILDREN
Total
Patients
Chest Xray
Results
Normal
Abnormal
76
71
5
100
91
9
Urinalysis or HCT
28
28
0
SMAC
5
5
0
Sed rate
7
7
0
T3/T4
2
1
1
Echocardiogram
1
1
0
Pregnancy test
1
0
1
ECG
CHEST PAIN IN CHILDREN
When to Refer a Child with Chest Pain
• Acute distress present
• Significant trauma
• Pain associated with syncope, dizziness, palpitations, exertion
• History of cardiac or Kawasaki disease
• Pleural effusion or pneumothorax present
• Serious emotional problems
• Esophageal foreign body or caustic ingestion
Selbst, et al.
Pediatric Rev 1986; 8:56
CHEST PAIN IN CHILDREN
Summary
• Generally benign and of noncardiac origin
• In adolescents, CP may be recurrent and may
interfere with activities of daily life
• During evaluation of recurrent CP, a new
etiology is frequently offered
Lam JC and Tobias JD
South Med J 94:921-924, 2001
3. Arterial blood gas results from an infant with a
cyanotic congenital heart defect should include:
A. Increased PO2 with crying
B. Increased PO2 with increased FI02
C. Elevated PCO2
D. All of the above
E. None of the above
NEONATAL CYANOSIS
CENTRAL
PERIPHERAL
hyperoxia
 pO2
ventilation
 pO2
PULMONARY
DISEASE
 pO2
echocardiogram
NORMAL
ABNORMAL
PERSISTENT
PULMONARY
HYPERTENSION
CYANOTIC
CONGENITAL
HEART DISEASE
COLD
SEPSIS
SHOCK
HYPOGLYCEMIA
POLYCYTHEMIA
CONGESTIVE HEART FAILURE
DEFINITION
CHF is a clinical syndrome in which the heart is unable to
pump enough blood to the body to meet its needs, to
dispose of venous return, or a combination of the two.
CONGESTIVE HEART FAILURE
ETIOLOGY
Congenital heart disease
• Birth – first week: Hypoplastic left heart, large AV fistula
Critical AS or PS, TAPVR
PDA (prematures)
• 1 – 4 weeks:
Coarctation, critical AS,
Large left to right shunts (prematures)
Truncus arteriosus
• 6 weeks – 4 mo
VSD, AV canal defect, large PDA
Anomalous L coronary artery
4. All of the following cardiac conditions can
present with heart failure in the first four months
of life except:
A. Hypoplastic left heart syndrome
B. Coarctation of the aorta
C. Complete AV canal defect
D. Large ventricular septal defect
E. Moderately severe pulmonary stenosis
CONGESTIVE HEART FAILURE
ETIOLOGY
• Congenital heart disease
• Acquired heart disease (acute rheumatic carditis, myocarditis)
• Myocardial dysfunction (metabolic abnormalities, dilated
cardiomyopathy
• Miscellaneous: chronic tachycardia, complete AV block, severe
anemia, acute hypertension
5. In infants, signs of congestive heart failure
include all of the following except:
A. Tachypnea
B. Tachycardia
C. Hepatomegaly
D. Pedal edema
E. Poor feeding
CONGESTIVE HEART FAILURE
CLINICAL MANIFESTATIONS
• Poor weight gain, poor feeding (in infants), anorexia, nausea
• Dyspnea on exertion or with feeding, exercise intolerance
• Diaphoresis (cold sweat on forehead) during feeding in infants
• Tachycardia
• Tachypnea, retractions
• Wheezing (râles are rare in CHF), cough
• Hepatomegaly, puffy face, esp. eyelids
• Gallop rhythm, cool extremities
6. Which is most beneficial in treating CHF?
A. K+ supplement
B. Na+ supplement
C. Furosemide
D. Neosynephrine
E. Morphine
CONGESTIVE HEART FAILURE
MANAGEMENT
• Oxygen (unless CHF is caused by excessive pulmonary flow)
• Diuretics – Lasix 1 mg/kg/dose
• Digoxin 8 – 10 mcg/kg/day (5 mcg/kg/day in prematures)
• ACE inhibitors
 captopril 0.5 – 6.0 mg/kg/day
 enalapril 0.1 mg/kg once or twice daily
• Misc: reduce energy expenditure, ± fluid restriction
• Beta-blockers: carvedilol (not yet standard therapy)
7. The following are true of furosemide EXCEPT:
A. Should be given rapidly IV
B. Usual dosage is 0.5-2.0 mg/kg/dose
C. Can cause hypokalemia
D. Can cause hyperostosis and nephrocalcinosis in
newborns
E. Can cause hypochloremic alkalosis
8. Digoxin levels are increased by all except:
A. Quinidine
B. Amiodarone
C. Hypokalemia
D. Carvedilol
E. Erythromycin
9. An ACE inhibitor would be indicated for:
A. Pulmonary stenosis
B. Aortic stenosis
C. Atrial septal defect
D. Hypertrophic cardiomyopathy
E. Dilated cardiomyopathy
CARDIOGENIC SHOCK
DEFINITION
Failure of the circulatory system to
supply oxygen and nutrients to meet
cellular metabolic demands
CARDIOGENIC SHOCK
ETIOLOGY
Newborns
• Left heart obstructive lesions (HLHS, AS, COA)
• Myocarditis
• Tachyarrhythmia
• Sepsis
Infants and
Older Children
• Sepsis
• Myocardial infarction
• Myocarditis
CARDIOGENIC SHOCK
CLINICAL MANIFESTATIONS
• Pallor
• Tachycardia
• Tachypnea
• Hypotension, narrow pulse pressure
• Oliguria
• Metabolic acidosis
CARDIOGENIC SHOCK
EVALUATION
• Chest X-ray
• Electrocardiogram
• Echocardiogram
• Cardiology consultation
CARDIOGENIC SHOCK
MANAGEMENT
• Intubation and mechanical ventilation
• Positive inotropic agents (epinephrine, dopamine, dobutamine)
• Afterload reducing agents (milrinone, nitroprusside)
• Diuretics – Lasix 1 mg/kg/dose
• Judicious fluid replacement as indicated clinically
CARDIOGENIC SHOCK
Dopamine
1-5 mcg/kg/min: dopaminergic
5-15 mcg/kg/min: more beta-1
10-20 mcg/kg/min: more alpha-1
Dobutamine
2.5-20 mcg/kg/min: mostly beta-1, some beta-2
Epinephrine
0.05-0.1 mcg/kg/min: mostly beta-1, some beta-2
0.1 to 0.2 mcg/kg/min: alpha-1
Milrinone
50mcg/kg load then 0.375-0.75mcg/kg/min: phosphodiesterase
inhibitor; increased inotropy and peripheral vasodilation
10. Long term complications of cyanotic congenital
heart disease include:
A. Brain abscess
B. Stroke
C. Erythrocytosis
D. Bleeding disorders
E. All of the above
COMPLICATIONS OF CYANOTIC
CONGENITAL HEART DISEASE
• Erythrocytosis: symptoms rare with Hct < 65%
• “Relative” anemia secondary to iron deficiency
• Stroke: associated with Fe++ deficiency or paradoxical embolism
• Brain abscess – intracardiac shunting
• Bleeding disorders and thrombosis
• Hypercyanotic spells
11. A 3-month-old girl with known congenital heart disease
has been having brief intermittent episodes of cyanosis.
Today, a few minutes after feeding, she developed
tachypnea, cyanosis, and persistent irritability.
The MOST likely diagnosis is:
A. Breathholding
B.
C.
D.
E.
Colic
Congestive heart failure
Hypercyanotic spell
Seizure
CONGENITAL HEART DISEAE
HYPERCYANOTIC SPELLS
• Most frequent in tetralogy of Fallot
• Peak incidence, 2 to 4 months
• Early morning, after feeding, exercise, crying or defecation
• Cyanosis, increased respiration, fussy, syncope
• Treatment: knee-chest, O2, sedation, fluids, phenylephrine
• Prevention: avoid dehydration, treat Fe++ deficiency, β blocker
12. Hypercyanotic spells can be provoked by any of
the following EXCEPT:
A. Pain
B. Induction of anesthesia
C. Dehydration
D. Iron deficiency
E. Squatting
13.Treatment of hypercyanotic spells includes:
A. Isoproterenol
B. Adenosine
C. Digoxin
D. Oxygen
E. Furosemide
True or False
14. Nocturnal hypoxia can cause cor pulmonale
15. Cor pulmonale is irreversible
COR PULMONALE
• Definition: right ventricular hypertrophy or dilation secondary
to pulmonary hypertension
• Causes: Congenital heart disease, alveolar hypoxia, pulmonary
venous hypertension, primary pulmonary hypertension
• Clinical manifestations: dyspnea, fatigue, syncope, loud single
S2, hepatomegaly, venous distension
• ECG: right axis deviation, RVH
• Management: relieve airway obstruction, O2, diuretics,
ventilation, cardiac surgery, pulmonary vasodilators
ANSWERS FOR RECOGNITION OF
CARDIOVASCULAR DISORDERS
1.
2.
3.
4.
5.
D
D
E
E
D
6.
7.
8.
9.
10.
C
A
C
E
E
11.
12.
13.
14.
15.
D
E
D
True
False
SUGGESTED READINGS
Artman M, Parrish MD, Graham TP. Congestive heart failure in childhood and
adolescence: recognition and management. Amer Heart J 1983;105:471-480.
Cava JR, Sayger PL. Chest pain in children and adolescents. Pediatr Clin N
Am 2004;51:1553-1568.
Danford DA, Nasir A, Gumbiner C. Cost assessment of the evaluation of
heart murmurs in children. Pediatr 1993;91:365-368.
Danford DA. Sorting through the haystack-Decision analysis and the search
for heart disease among children with murmur. J Pediatr
2002;141:465467.
Diwarkaran A. Index of suspicion. Case 1. Cyanotic congenital heart
disease. Pediatr Rev 1999;20:246-247.
Frommelt MA. Differential diagnosis and approach to a heart murmur in term
infants. Pediatr Clin N Am 2004;51:1023-1032.
SUGGESTED READINGS
Maron BJ, et al. Recommendations and considerations related to
preparticipation screening for cardiovascular abnormalities in competitive
athletes: 2007 update. Circulation 2007;115:1643-1655.
McCrindle BW, Shaffer KM, Kan JS, et al. Cardinal clinical signs in the
differentiation of heart murmurs in children. Arch Pediatr Adol Med
1996;150:169-174.
McKiernan CA, Lieberman SA. Circulatory shock in children: an overview.
Pediatr Rev 2005;12:451-460.
Owens TR. Chest pain in the adolescent. Adol Med 2001;12:95-104.
Pelech AN. Evaluation of the pediatric patient with a cardiac murmur. Pediatr
Clin North Am 1999;167-88.
Pelech AN. The physiology of cardiac auscultation. Pediatr Clin North Am
2004;51:1515-1535.
Pierpont ME, et al. Genetic basis for congenital heart defects: current
knowledge. Circ 2007;115:3015-3038
Reich JD, Miller S, Brogdon B, et al. The use of pulse oximetry to detect
congenital heart disease. J Pediatr 2003;142:268-72.
SUGGESTED READINGS
Selbst SM. Consultation with the specialist. Chest pain in children. Pediatr Rev
1997;18:169-173.
Selbst SM. Pediatric chest pain: a prospective study. Clin Pediatr 1990;29:615618.
Selbst SM. Pediatric chest pain: a prospective study. Pediatr 1988;82:319-323.
Silberbach M, Hannon D. Presentation of congenital heart disease in the
neonate and young infant. Pediatr Rev 2007;28:123-130.
Singh A, Silberbach M. Cardiovascular preparticipation sports screening.
Pediatr Rev 2006;27:418-423.
Swenson JM, Fischer DR, Miller SA, et al. Are chest radiographs and
electrocardiograms still valuable in evaluating new pediatric patients with
heart murmurs or chest pain? Pediatr 1997;99:1-3.
Yi MS, Kimball TR, Tsevat J, et al. Evaluation of heart murmurs in children:
Cost-effectiveness and practical implications. J Pediatr 2002;141:504-511.