发绀型先天性心脏病课件

Cyanotic Heart LesionsCyanotic Heart Lesions Neonatal Intensive Care Nursery Night Curriculum Series CyanosisCyanosis Arterial saturation less than 90% and a PO2 less than 60 torr In all cyanotic heart lesions the amount of cyanosis seen is dependent on the amount of pulm blood flow oDecreased PBF- increased cyanosis oIncreased PBF- minimal cyanosis but CHF may develop With 100% oxygen oPO2 250 is not congenital heart disease oPO2 100 is cardiac disease 5 “Ts”5 “Ts” Most common cyanotic lesions of the newborn oTetralogy of Fallot oTransposition of the Great Arteries oTruncus Arteriosus oTotal Anomalous Venous Return oTricuspid Atresia General Sources of Arterial General Sources of Arterial SaturationSaturation Decreased Pulmonary Blood Flow Admixture LesionsIncreased Pulmonary Blood Flow Tetralogy of FallotTransposition of Great Vessels Truncious Arteriosus Tricuspid AtresiaAnomalous pulmonary venous return Pulmonary Atresia PGEPGE For cyanotic heart lesions with reduced blood flow RE-opens PDA and prevents it from closing Allows partially desaturated systemic arterial blood to enter the pulmonary artery and be oxygenated Initial dose 0.1mg/kg/min Side effects- apnea, fever, hypotension Complete Transposition Complete Transposition of the Great Arteriesof the Great Arteries 5% of all CHD Boys 3:1 Most common cyanotic condition that requires hospitalization in the first two weeks of life Complete Transposition of Complete Transposition of the Great Arteriesthe Great Arteries Aorta arises from the right ventricle Pulmonary artery arises from the left ventricle Complete Transposition of Complete Transposition of the Great Arteriesthe Great Arteries Complete separation of the 2 circuits o Hypoxemic blood circulating in the body o Hyperoxemic blood circulating in the pulmonary circuit Complete Transposition of Complete Transposition of the Great Arteriesthe Great Arteries Defect to permit mixing of 2 circulations - ASD, VSD, PDA. oVSD is present in 40% of cases Necessary for survival Clinical SymptomsClinical Symptoms Depend on anatomy present No mixing lesion and restrictive PFO oProfound hypoxia oRapid deterioration oDeath in first hours of life oAbsent respiratory symptoms or limited to tachypnea oSingle second heart sound, no murmurs Clinical SymptomsClinical Symptoms Mixing lesion present (VSD or large PDA) oLarge vigorous infant oCyanotic oLittle to no resp distress oMost likely to develop CHF in first 3-4 months of life excessive sweating (a cold, clammy sweat often noted during feeding); poor feeding, slow weight gain, irritability or lethargy, and/or rapid breathing CXRCXR Egg shaped cardiac silhouette Narrow superior mediastinum ManagementManagement Prostaglandin to establish patency of the ductus arteriosus o Increases shunting from aorta into the pulmonary artery o Increases pulmonary venous return distending the left atrium o Facilitates shunting from the left to the right atrium of fully saturated blood across the foramen ovale. ManagementManagement Therapeutic balloon atrial septostomy (Rashkind Procedure) if surgery is not going to be performed immediately Improves mixing and pulmonary venous return at the atrial level TreatmentTreatment Surgery consists of switching the right and left sided structures at the atrial level, at the ventricular level, or at the great artery level. Tetraology of FallotTetraology of Fallot 1.VSD 2.RVOT Obstruction 3.RVH 4.Overriding aorta Two Important Two Important AbnormalitiesAbnormalities NonRestrictive VSD oLarge enough to equalize pressures in both ventricles Degree of RVOT Clinical Presentation of Clinical Presentation of Cyanotic TOFCyanotic TOF Cyanosis, clubbing, dyspnea on exertion, squatting, hypoxic spells. Loud systolic ejection murmur, systolic thrill at middle LSB Soft murmurs are associated with less blood flow and more hypoxia CXR- boot shapedCXR- boot shaped Hypoxic Spell Hypoxic Spell (“TET Spell”)(“TET Spell”) Peak incidence of 2-4 months Characterized by: oHyperapnea (Rapid and deep respirations) oIrritability and prolonged crying oInc cyanosis oDecreased heart murmur PathophysiologyPathophysiology o Lower SVR or inc resistance of RVOT can increase the R- L shunt Stimulates the respiratory center to produce hyperapnea Results in an increase in systemic venous return In turn, increases R-L shunt through VSD TET Spell TreatmentTET Spell Treatment 1.Hold infant in knee-chest position 2.Morphine 3.Sodium bicarbonate to treat acidosis- decreases resp stimulating effect of acidosis 4.Vasoconstrictor (phenylephrine) 5.Propranolol TreatmentTreatment Early surgical repair depending on pts weight VSD is closed and obstructing ventricular muscle is removed Total Anomalous Pulmonary Total Anomalous Pulmonary Venous ReturnVenous Return The pulmonary veins drain into the RA or its venous tributaries rather than the LA A interatrial communication (ASD or PFO) is necessary for survival Pulmonary venous return reaches the RA oSystemic and pulmonary venous blood are completely mixed 4 Types 4 Types 1. Supracardiac 1. Supracardiac Common pulmonary vein drains into the SVC via the left SVC and left innominate vein. 2. Cardiac2. Cardiac The common PV drains into the coronary sinus 3. Infracardiac3. Infracardiac The common PV drains into the portal vein, ductous venosus, hepatic vein, or IVC. Infracardiac-type TAPVC. Pulmonary venous blood draining through the liver to reach the IVC and right atrium. 4. Mixed4. Mixed A combination of the other types Clinical Signs for Clinical Signs for Unobstructed VeinsUnobstructed Veins Mild cyanosis, signs of CHF in infancy, history of pneumonia Widely split S2, Grade 2-3/6 systolic murmur heard at the ULSB CXR- marked cardiomegaly Clinical Signs for Clinical Signs for Obstructed VeinsObstructed Veins Profound desaturation Acidosis PGE1 administration does not improve oxygenation because elevated pulmonary pressures in the right side of the heart (due to obstructed pulmonary outflow) will result in right to left shunting across an open ductus further decreasing arterial saturation. TreatmentTreatment Digitalis and diuretics to treat heart failure Intubation and inc PEEP for those with severe pulm over load Corrective surgery Tricuspid AtresiaTricuspid Atresia Tricuspid valve is absent RV and PA are hypoplastic Associated defects- ASD, VSD, or PDA (necessary for survival) Dilation of LA and LV Essentially single ventricle physiology Clinical SignsClinical Signs Severe cyanosis, poor feeding, tachypnea Single S2, grade 3/6 systolic murmur at LLSB if VSD is present CXR- boot shaped heart TreatmentTreatment PGE IV infusion Blalock-Taussig shunt in infancy osystemic to pulmonary arterial shunt oProvide stable blood flow to the lungs oA gortex tube is sewen between the subclavian artery and the right pulmonary artery