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 anti- congestive 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 : /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 ?? 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 PREMATURE NEONATE fluid overload patent ductus arteriosus ventricular septal defect cor pulmonale (bronchopulmonary dysplasia) hypertension 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 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 CHILD-ADOLESCENT Rheumatic fever Acute hypertension (glomerulonephritis) viral myocarditis thyrotoxicosis hemochromatosis-hemosiderosis cancer therapy (radiation, doxorubicin) sickle cell anemia endocarditis cor pulmonale (cystic fibrosis) cardiomyopathy (hypertrophic, dilated)

Left-to-right cardiac shunts (Ventricular septal defect) 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

30-50% of small defects close spontaneously, most frequently during 1 st 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) Large systemic arteriovenous fistula ---high-output failure Reduce peripheral vascular resistance and cardiac afterload Increase myocardial contractility

Supraventricular tachycardia 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 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 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

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 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) 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 1 st 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