1、Pediatric Heart Disease,Infants may have one of the following: Cyanosis (cyanotic CHD or R to L shunt) Pulmonary and tricuspid atresia CHF (L to R shunt) or shock Shock from outflow obstruction Diagnostic evaluation CXR EKG,Cyanotic Heart Defects,Tetralogy of Fallot Transposition of the great vessel
2、s Truncus arteriosus Total anomalous pulmonary venous return Tricuspid valve Abnormalities Severe pulmonic stenosis,Tetralogy of Fallot,Consists of VSD, obstructed right vent. outflow tract, dextroposed overriding aorta, RVH. TeT spells-worsening obstruction of flow in the pulmonary artery leading t
3、o greater R to L shunt. Causes cyanosis and dyspnea.,Transposition of Great Vessels,Appears in 1st week of life. Aorta comes from R vent. and pulmonary art. Comes from L vent. Must have a VSD or ASD for survival If suspected, start Prostaglandin E1,Truncus Arteriosus,Large arterial trunk from the ve
4、ntricular portion of the heart. Supplies blood to systemic and pulmonary circulation. Present with increased pulm. blood flow, dyspnea and CHF,Coarctation of the Aorta,Narrowing of the aortic lumen. Present with CHF and feeding difficulty. Decreased pulse amplitude in the lower ext. Hypertension in
5、the upper ext. Older kids present with exercise intolerance and rib notching.,Ductal Dependent heart defects,Depend on a patent ductus arteriosus. Systemic blood flow depends on a R to L shunt from the pulmonary artery through the DA to aorta. Pulmonic blood flow depends on a L to R shunt from the a
6、orta through the DA to the pulmonary art.,Defects dependent on R to L shunt via the PDA include: Critical Aortic Stenosis Hypoplastic left heart Severe coarctationUsual Clinical Presentation Circulatory collapse at the end of first week of life,Treatment,Prostaglandin infusion keeps the DA open unti
7、l surgery. Start at 0.1 mcg/kg/min and titrate,CHF,Usually w/in the first 6 months when PVR has decreased allowing L to R shunt (VSD or PDA) Triad of CHF in infancy Tachypnea Tachycardia Hepatosplenomegaly,Treatment,Supportive Lasik Digoxin except in IHSS or TOF Inotropes Pressors Vasodilators After
8、load reducing drugs Head up NPO Treat infections,Atrial septal defects,RA and RV enlargement Pulmonary over-circulation High pressure Low volume ASD low morbidity and mortality Repair may be surgical or trans cath,Vent. Septal Defect,Most common CHD May occur in any septal location Hemodynamic signi
9、ficance depends on the size of the defect. Spontaneous closure in the first 6 mo. In 30-40%.,Surgical repair required if: Infant has failure to thrive Pulmonary HTN R to L shunt,Aortic Stenosis,May have a mono, bi or tricuspid valve. If severe, avoid prosthetic valve until they are old enough to rec
10、eive an adult sized one.,In critical AS, LV unable to pump adequate flow past the aortic valve. If no PDA, not compatible with life,Kawasakis Disease,Acute self limited multisystem vasculitis Clinical presentation Fever 5 or more days with 4 of 5 other clinical features Bilat. conjunctival inection
11、w/o exudates Mucous membranes changes of upper resp. tract Erythema and edema of hands/feet (early) and desquamation (subacute phase) Exantham polymorphous, truncal Acute cervical lymphadenopathy,Lab findings include high WBC, left shift, hemolytic anemia, high platelets, high CRP/ESR, pyuria, bilir
12、ubinuria On CXR infiltrates, cardiomegaly, long PR or QT, dysrhythmias Cardiac echocardiography show coronary aneurysms 80-90% of time,Treatment,Hospitalization for IVIG ASA Cardiac eval. Corticosteroids may decrease coronary aneurysms,Myocarditis,Causes Idiopathic Inflammatory Acute rheumatic fever
13、 Collagen vascular disease Lyme disease Toxins HIVViruses Adenovirus Coxsackie A and B,Often misdiagnosed Classic presentation CHF or fulminant cardiogenic shock Syncope from dysrhythmias May present with cough, wheeze/tachypnea, congestion, or fever. Consider in a child with wheezing and no history
14、 of asthma or in a febrile child whose wheezing is not responding to treatment.,Diagnostic eval CXR-cardiomegaly EKG changes Elevated troponin Definitive diagnosis-biopsy or MRI,Treatment Bedrest Oxygen Inotropes May need transplant 35% mortality,SVT,Most common dysrhythmia Present with tachycardia,
15、 poor feeding, tachypnea, pallor, lethargy, chest pain. HR usually 230 BPM Unstable sync. Cardiovert 0.5 j/kg. Lidocaine 1 mg/kg should be given prior d/t risk of V fib. Adenosine 0.1 mg/kg the 0.3 mg/kg,A fib,Usually d/t rheumatic heart disease or dilated cardiomyopathy. Unstable-cardiovert 0.5 J/k
16、g Medical therapy (dig) is usually not effective Anticoagulation is not necessary,V Tach,Usually seen with congenital heart disease, but also with myocarditis, cardiomyopathy, or prolonged QT. Unstable sync. Cardiovert 1-2 J/kg Start Lidocaine or Procainamide if pt not stable for transfer,V Fib,Initial energy for defib is 2 J/kg. Can be doubled. A trial of amiodarone 5mg/kg infusion followed by defib. If successful, Amiodarone infusion 5-15 micrograms/kg/min. After 3 defib attempts and a second 2 mg/kg bolus infusion usually indicates futility.,
