congenital heart disease in neonates 在新生儿和紫绀型先天性心脏病课件

APPROACH TO CYANOTIC CONGENITAL HEART DISEASE Dr. R. Suresh Kumar Head, Department of Pediatric Cardiology Cyanosis lBluish discolouration of skin 5g%CBG lCauses CNS, Musculoskeletal system, Airways, Alveolar-capillary membrane, Hb AND CVS. lCentral vs peripheral. lCardiac vs Respiratory. Peaceful cyanosis is cardiac Evaluation of the cyanotic baby lIs the baby cyanosed? SpO2 will confirm. lIs the cyanosis central/peripheral? Keep the baby warm. lIs cyanosis cardiac? 1)Respiratory distress 2)Response to oxygen lABG - low pH, low pO2, Low pCo2 lCxR-heart size Soda bicarb iv 1-2 ml/kg lIncrease SVR : Propranolol 0.1 mg/kg iv; Methoxamine 0.1 mg/kg iv; Phenylephrine 1- 5mic.g/kg /min iv infusion. lVentilation. Paralyse lEmergency surgery lPropranolol for prophylaxis Complex CHD,TOF-like physiology lDORV PS/PA Presents like TOF. Needs neonatal BT shunt, if ductus-dependent. Total correction around 1 year, may need a homograft. lSingle Ventricle PS/PA. Presents like TOF. But 2- ventricle Repair not possible.Neonatal BT shunt, if needed. SVC to RPA anastomosis (Bidirectional Glenn) at 3-6months. IVC to PA connection (Lateral tunnel or Extra cardiac Fontan) at 2-5 yrs. Tricuspid Atresia lPulmonary blood flow depends on ASD, VSD, Pulmonary stenosis. May be increased / decreased / balanced. Cyanosis or heart failure predominant presentation accordingly. lLV dominance and left axis in ECG helpful. lManagement in Fontan track.Neonatal BT shunt (for severe cyanosis) or PA banding (for heart failure) may be needed. Critical PS PAIVS lCyanosis on day 1. Pulmonary circulation ductus dependent. RV normal or in a range of hypoplasia. lIf RV is adequate,catheter or surgical pulmonary valvotomy is the treatment. If RV is small, BT shunt. May need both. lFurther management may be - Glenn + Pulm. Valvotomy (1 ventricle) or staged Fontan, when 2-ventricle repair not possible. lMyocardial sinusoids / RV dependent coronary circulation - adverse prognosis. Ebsteins anomaly lInferior displacement of septal and inferior tricuspid leaflets. Thin RV. May have PS. lNeonatal cyanosis may be severe. High PVRTR R to L atrial shunt. Usually transient. lCourse:May be asymptomatic. Cyanosis, Rt heart failure and SVT may be problems. lSurgery for severe cyanosis/heart failure. Ebsteins anomaly lHistory of SVT lGenerally good effort tolerance lCardiomegaly lMultiple heart sounds (split S1, split S2, S3, S4) lScratchy systolic murmur at LLSB lCardiomegaly with huge RA on CxR. lECG-abnormal axis, RAE, polyphasic QRS. May have WPW syndrome Cyanotic CHD, increased PBF. Transposition of GA lCommonest cyanotic CHD on day 1. lAorta from RV, PA from LV. Presentation depends on Intact IVS, VSD, PS. lTGA intact IVS. Cyanosis on Day1. No murmur. CxR- egg on side heart,normal vascularity. Untreated 90% die in first month, if no ASD. lManagement : PGE1 till Balloon Atrial Septostomy. Arterial Switch Operation in first month. Good long-term result. TGA with VSD lNo PS: Cyanosis minimal. CHF in a few weeks. Needs Arterial Switch operation in 3 months. Rapid progression of PVD, if untreated. lTGA/VSD/PS: Cyanosis depends on degree of PS. BT shunt if cyanosis is severe. Rastelli operation at 3-5 yrs. TAPVC lAll 4 pulm veins join a common chamber which eventually reaches RA. lSupracardiac- common chamber drains to innominate vein or to SVC. lCardiac- drains to coronary sinus or RA lInfracardiac (Infradiaphragmatic). Descending vertical vein portal vein ductus venosus IVC. Obstructed. TAPVC-2 lObstructed venous return: Presents with pulmonary oedema. Infradiaphragmatic on Day1, supracardiac in a fewdays. Emergency surgery life saving. Normal life span. lUnobstructed: Cardiac, some supracardiac. Slow onset of CHF, minimal cyanosis. Clinical features of ASD Figure of 8 cardiac silhouette on CxR. Good surgical results. Persistent Truncus Arteriosus lA single arterial trunk leaves the heart above a VSD. PA arises with a common trunk or as separate branches. lCHF in a few weeks of life, mild cyanosis. Bounding pulses,systolic click and murmur. EDM, if truncal valve is incompetent. CxR - CE, High origin of PA branches,increased vascularity. lSurgical Repair in first month preferred. Cyanotic CHD in older children and adults lTetralogy of Fallot Commonest CCHD with reduced pulmonary blood flow surviving into adulthood Exertional dyspnoea and squatting Varying degrees of cyanosis lEisenmenger syndrome History of recurrent respiratory infections in early childhood Good effort tolerance till late in the nature history Mild cyanosis Findings of severe pulmonary artery hypertension Eisenmenger VSD lPresents in second decade lSingle S2 lNormal heart size on CXR lDilated central pulmonary arteries with peripheral pruning Eisenmenger PDA lPresents in second or third decade lDiff