小儿先心病

Pediatric Heart Disease lInfants may have one of the following: n Cyanosis (cyanotic CHD or R to L shunt) lPulmonary and tricuspid atresia n CHF (L to R shunt) or shock n Shock from outflow obstruction n Diagnostic evaluation lCXR lEKG Cyanotic Heart Defects lTetralogy of Fallot lTransposition of the great vessels lTruncus arteriosus lTotal anomalous pulmonary venous return lTricuspid valve Abnormalities lSevere pulmonic stenosis Tetralogy of Fallot lConsists of VSD, obstructed right vent. outflow tract, dextroposed overriding aorta, RVH. lTeT spells-worsening obstruction of flow in the pulmonary artery leading to greater R to L shunt. Causes cyanosis and dyspnea. Transposition of Great Vessels lAppears in 1st week of life. lAorta comes from R vent. and pulmonary art. Comes from L vent. lMust have a VSD or ASD for survival lIf suspected, start Prostaglandin E1 Truncus Arteriosus lLarge arterial trunk from the ventricular portion of the heart. lSupplies blood to systemic and pulmonary circulation. lPresent with increased pulm. blood flow, dyspnea and CHF Coarctation of the Aorta lNarrowing of the aortic lumen. lPresent with CHF and feeding difficulty. lDecreased pulse amplitude in the lower ext. lHypertension in the upper ext. lOlder kids present with exercise intolerance and rib notching. Ductal Dependent heart defects lDepend on a patent ductus arteriosus. n Systemic blood flow depends on a R to L shunt from the pulmonary artery through the DA to aorta. n Pulmonic blood flow depends on a L to R shunt from the aorta through the DA to the pulmonary art. lDefects dependent on R to L shunt via the PDA include: n Critical Aortic Stenosis n Hypoplastic left heart n Severe coarctation lUsual Clinical Presentation n Circulatory collapse at the end of first week of life Treatment lProstaglandin infusion keeps the DA open until surgery. lStart at 0.1 mcg/kg/min and titrate CHF lUsually w/in the first 6 months when PVR has decreased allowing L to R shunt (VSD or PDA) lTriad of CHF in infancy n Tachypnea n Tachycardia n Hepatosplenomegaly Treatment lSupportive lLasik lDigoxin except in IHSS or TOF lInotropes lPressors lVasodilators lAfterload reducing drugs lHead up lNPO lTreat infections Atrial septal defects lRA and RV enlargement lPulmonary over-circulation n High pressure n Low volume lASD low morbidity and mortality lRepair may be surgical or trans cath Vent. Septal Defect lMost common CHD lMay occur in any septal location lHemodynamic significance depends on the size of the defect. lSpontaneous closure in the first 6 mo. In 30 -40%. l Surgical repair required if: n Infant has failure to thrive n Pulmonary HTN n R to L shunt Aortic Stenosis lMay have a mono, bi or tricuspid valve. lIf severe, avoid prosthetic valve until they are old enough to receive an adult sized one. lIn critical AS, LV unable to pump adequate flow past the aortic valve. n If no PDA, not compatible with life Kawasakis Disease lAcute self limited multisystem vasculitis lClinical presentation n Fever 5 or more days with 4 of 5 other clinical features lBilat. conjunctival inection w/o exudates lMucous membranes changes of upper resp. tract Erythema and edema of hands/feet (early) and desquamation (subacute phase) lExantham polymorphous, truncal lAcute cervical lymphadenopathy Lab findings include high WBC, left shift, hemolytic anemia, high platelets, high CRP/ESR, pyuria, bilirubinuria lOn CXR infiltrates, cardiomegaly, long PR or QT, dysrhythmias lCardiac echocardiography show coronary aneurysms 80-90% of time Treatment lHospitalization for IVIG lASA lCardiac eval. lCorticosteroids may decrease coronary aneurysms Myocarditis lCauses n Idiopathic n Inflammatory lAcute rheumatic fever lCollagen vascular disease lLyme disease lToxins lHIV n Viruses lAdenovirus lCoxsackie A and B lOften misdiagnosed lClassic presentation n CHF or fulminant cardiogenic shock n Syncope from dysrhythmias May present with cough, wheeze/tachypnea, congestion, or fever. Consider in a child with wheezing and no history of asthma or in a febrile child whose wheezing is not responding to treatment. lDiagnostic eval n CXR-cardiomegaly n EKG changes n Elevated troponin n Definitive diagnosis-biopsy or MRI lTreatment n Bedrest n Oxygen n Inotropes n May need transplant 35% mortality SVT lMost common dysrhythmia lPresent with tachycardia, poor feeding, tachypnea, pallor, lethargy, chest pain. lHR usually 230 BPM lUnstable sync. Cardiovert 0.5 j/kg. Lidocaine 1 mg/kg should be given prior d/t risk of V fib. lAdenosine 0.1 mg/kg the 0.3 mg/kg A fib lUsually d/t rheumatic heart disease or dilated cardiomyopathy. lUnstable-cardiovert 0.5 J/kg lMedical therapy (dig) is usually not effective lAnticoagulation is not necessary V Tach lUsually seen with congenital heart disease, but also with myocarditis, cardiomyopathy, or prolonged QT. lUnstable sync. Cardiovert 1-2 J/kg lStart Lidocaine or Procainamide if pt not stable for transfer V Fi