Transcript What are the infant`s problems

CPC
林世朋 李春銘 大夫
三軍總醫院小兒部
Chief complaints

Heart murmur and moderate mitral
regurgitation when he was at age one month
 Cough with sputum, runny nose, tachypnea
and dyspnea when he was 7 months old.
Present illness (1)

The male infant was well until routine vaccination
and infant physical check-up at age 1 month when
the physician found heart murmur.
 At that time, echocardiography disclosed
increased mitral inflow rate, moderate mitral
regurgitation, normal pulmonary pressure and no
cardiomegaly.
 Then, the patient received regular OPD follow-up
at the same medical center.
Present illness (2)

At age 7 months, the patient suffered from
cough with sputum, runny nose, tachypnea,
and dyspnea.
 He was admitted to the same medical center
and received a series of examinations.
Present illness (3)

10 days after treatment with IVIG and anticongestive heart failure drugs, echocardiography
showed improved ejection fraction 65%, but still
dilated left ventricle and atrium.
 After 11-day admission, he was discharged. It
followed, then, that anti-congestive heart failure
drugs were prescribed and a series of
echocardiography was performed at the OPD.
Present illness (4)

At age 2 years, physical examination showed rapid
heart beats, no cyanosis, no clubbing finger, mild
hepatomegaly, clear breathing sound and mild
tachypnea.
 Echocardiography revealed
*dilated four chambers, especially in left ventricle
*moderate mitral regurgitation
*ejection fraction 60%.
Present illness (5)

Finally, at age 3 years, the
echocardiography disclosed:
*dilated left ventricle & atrium
*increased echogenicity of endocardium
of left ventricle
*severe mitral regurgitation
 A diagnostic study was done at that age.
Personal and past history

A male infant was born after an uneventful
pregnancy
 Birth history: G2P2A0
 Fullterm with normal Apgar scores.
Laboratory findings

At age 7 months
 WBC : 9300 /ul
; Hgb: 10.7 g/dL
Platelet : 155000 /ul ; N/L :28/58
CRP: 1.35 mg/dl
CK: 48 u/l ; CK-MB: 10 u/l
 CxR: cardiomegaly
Echocardiographic findings

At age 1 month :
1.increased mitral inflow rate
2. moderate mitral regurgitation
 At age 7 months :
1. dilated LV & LA
2. moderate mitral regurgitation
3. Ejection fraction:43 %  EF : 65% (post-treatment)
 At age 2 years :
1.dilated four chambers, especially in LV
2.moderate mitral regurgitation
3.ejection fraction 60%.
 At age 3 years :
1.dilated LV & LA
2.increased echogenicity of endocardium of LV
3.severe mitral regurgitation
What are the infant’s
problems ??
Problems

Tachypnea
 Dyspnea
 Mild hepatomegaly
 Cardiomegaly with dilated LV and LA
 Moderate/severe mitral regurgitation
 Increased echogenicity of endocardium of
LV
 Congestive heart failure
Questions ??
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EKG ?
Congenital cardiac anomaly from
echocardiography ?
BP?
Renal function ? Potassium level ? LDH ?
Fever ? MI ? Troponin I ?
Floppy, muscle tone, or muscle biopsy ?
Viral study? Endomyocardial biopsy ?
Etiology of Heart Failure

FETAL
severe anemia (hemolysis, fetal-materanal transfusion, parvovirus B19-induced anemia, hypoplastic
anemia)
supraventricular tachycardia
ventricular tachycardia
comlete heart block
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PREMATURE NEONATE
fluid overload
ventricular septal defect
hypertension

patent ductus arteriosus
cor pulmonale (bronchopulmonary dysplasia)
FULL-TERM NEONATE
asphyxial cardiomyopathy
arteriovenous malformation (vein of Galen, hepatic)
left-sided obstructive lesions (coarctation of aorta, hypoplastic left heart syndrome)
large mixing cardiac defects (single ventricle, truncus arteriosus)
viral myocarditis
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INFANT-TODDLER
left-to-right cardiac shunts (ventricular septal defect)
hemangioma (arteriovenous malformation)
Anomalous left coronary artery
metabolic cardiomyopathy
Acute hypertension (hemolytic-uremic syndrome)
supraventricular tachycardia
Kawasaki disease
Viral myocarditis
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CHILD-ADOLESCENT
Rheumatic fever
viral myocarditis
hemochromatosis-hemosiderosis
sickle cell anemia
cor pulmonale (cystic fibrosis)
Acute hypertension (glomerulonephritis)
thyrotoxicosis
cancer therapy (radiation, doxorubicin)
endocarditis
cardiomyopathy (hypertrophic, dilated)
Left-to-right cardiac shunts
(Ventricular septal defect)
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The most common cardiac malformation (25% of CHD)
Membranous, muscular, supracristal and infracristal types
The magnitude of intracardiac shunts : Qp/Qs ratio
Clinical manifestations vary according to the size of defect
and pulmonary blood flow and pressure.
Large VSD---dyspnea, feeding difficulty, poor growth,
profuse perspiration, recurrent pulmonary infection and
cardiac failure in early infancy.
In large VSD :
CxR---cardiomegaly with prominence of both ventricles,
the left atrium and the pulmonary artery
EKG---biventricular hypertrophy; p waves may be notched
or peaked
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30-50% of small defects close spontaneously, most
frequently during 1st 2 year of life.
 Small muscular VSD are more likely to close(up to 80%)
than membranous VSD( up to 35%).
 More commonly, infants with large defects have repeated
episodes of respiratory infection and heart failure despite
optimal medical management.
 Heart failure may be manifested primarily as failure to
thrive.
Hemangioma (arteriovenous malformation)
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Large systemic arteriovenous fistula
---high-output failure
 Reduce peripheral vascular resistance and cardiac
afterload
 Increase myocardial contractility
Supraventricular tachycardia
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Involve the components of the conduction system within or
above the bundle of His
 1.Re-entrant tachycardia with accessory pathway
2.Re-entrant tachycardia without accessory pathway
3.ectopic or automatic tachycardias
 Infant with SVT are often initially seen in heart failure
(because tachycardia goes unrecognized for a long time)
 Tachypnea and hepatomegaly are the prominent signs of
cardiac failure; fever and leukocytosis may be present.
 The heart rate during paroxysms is frequently in the range
of 200-300 beats/min.
 If the attack lasts 6-24 hr or more with an extreme fast
heart rate, the infant may become acutely ill, have an ashen
color, and be restless and irritable.
Kawasaki disease
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Diagnostic criteria :
1.fever lasting for at least 5 days
2.presence of at least 4 of the following 5 signs:
* bilateral bulbar conjunctival injection, generally nonpurulent
* changes in the mucosa of the oropharynx, including injected
* changes of the peripheral extremities, such as edema or
erythema of the hands or feet in the acute phase; or periungual
desquamation in the subacute phase.
* rash, primarily truncal; polymorphous but nonvesicular
* cervical adenopathy, >=1.5cm, usually unilateral
lymphadenopathy illness not explained by other known
disease process
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Mucocutaneous lymph node syndrome or infantile
polyarteritis nodosa
A severe vasculitis of all blood vessels but predominantly
affecting medium-sized arteries.
Approximately 20% of untreated patients develop coronary
artery abnormalities including aneurysm, thrombosis or
stenosis, myocardial infarction, aneurysm rupture and
sudden death.
Myocarditis manifested by tachycardia and
decreased ventricular function occurs in at least
50% of patients.
Metabolic Cardiomyopathy
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Glycogenosis II (Pompe disease)
1.Autosomal recessive(17q23), deficiency of lysosomal acid maltase
2.Infantile form : severe generalized myopathy and cardiomyopathy
---cardiomegaly, hepatomegaly,diffusely hypotonic
---serum CK level is greatly elevated
---muscle biopsy reveals a vacuolar myopathy
---EKG :
prominent P waves; a short P-R interval; massive QRS voltage;
signs of isolated left or biventricular hypertrophy; and
interventricular conduction delay
---CxR : striking cardiomegaly with prominence of LV
---Echocardiogram : severe ventricular hypertrophy
---Poor prognosis
3.Late childhood or adult form :
---a much milder myopathy without cardiac or hepatic enlargement
---myopathic weakness and hypotonia even in early infancy
--- serum CK level is greatly elevated
4.Diagnostic---quantitative assay of acid maltase activity in muscle or liver biopsy
Acute hypertension
(hemolytic-uremic syndrome)

The most common cause of acute renal failure in young
children (<age 4 years)
 Characterized by microangiopathic hemolytic anemia,
thrombocytopenia and uremia.
 E. coli o157:H7 (Shiga toxin--verotoxin)
 Also associated with other bacterial (Shigella, Salmonella,
Campylobacter, Strep. Pneumoniae,Bartonella) and viral (coxsackievirus,
echovirus, influenza, varicella, HIV, EBV) infections.
 Complications include anemia, acidosis, hyperkalemia,
fluid overload, heart failure, hypertension, and uremia.
 Extrarenal manifestations of CNS, GI tract, heart, and
skeletal muscles may be life threatening.
 Cardiac involvement--- pericarditis, myocardial
dysfunction, and arrhythmias.
Myocarditis (1)
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Viral myocarditis:
1.most common causative agents---coxsackievirus B
and adenovirus
2.S/S depend on the patient’s age and the acute or
chronic nature of the infection.
3.Neonate---fever, severe heart failure, respiratory
distress, cyanosis, distant heart sound, a gallop
rhythm, acidosis and shock.
4.evidence of viral hepatitis, aseptic meningitis and
an associated rash may be present.
5.CxR---enlarged heart and pulmonary edema
EKG---sinus tachycardia, reduced QRS complex
voltage, and ST segment and T-wave abnormality.
Myocarditis (2)
6.Older patient---a gradual onset of congestive heart
failure or a sudden onset of ventricular arrhythmia.
7.ESR, CK & LDH may be elevated in acute or
chronic myocarditis.
8.Echocardiogram---poor ventricular function and
often a pericardial effusion, MR and absence of
coronary artery or other congenital heart lesions.
9.Can be confirmed by endomyocardial biopsy.
Anomalous origin of the left coronary
artery from the pulmonary artery

Inadequate perfusion to the LCA, myocardial ischemia,
infarction and fibrosis result.
 Clinical manifestations :
1.Evidence of heart failure becomes apparent within the 1st few months of
life, and it is often precipitated by respiratory infection.
2.Cardiac enlargement is moderate to massive.
3.Gallop rhythm is common (mitral insufficiency)
 Diagnosis :
1.CxR : cardiomegaly
2.EKG :
lead I & AVL---QR pattern followed by inverted T waves
V5 &V6---deep Q waves and exhibit elevated ST segments and
inverted T waves.
3.Cardiac catheterization ---diagnostic
4.Aortography shows immediate opacification of the right coronary artery
only.

Diagnostic procedure :
cardiac catheterization
endomyocardial biopsy
Final diagnosis :
1.Anomalous origin of the left coronary
artery from the pulmonary artery
2. Myocarditis
3. Congenital mitral stenosis