Non-cyanotic Atrial Septal defects Defect Often doesnt show up - PDF document

10/8/2009 Hot Topics in Pediatric Cardiology • Review of Major Defects • Fetal Diagnosis of Congenital Heart Disease • Neonatal Diagnosis of CHD g • Treatment in Cath Lab • Heart Surgery Outcomes • Primary Care Issues after Heart Surgery • Adults with CHD • Pregnant women with CHD Non-cyanotic Atrial Septal defects Defect • Often doesn’t show up for 1 • Don’t get blue, yr. • May have trouble gaining weight • Pulmonary • May have congestive heart failure • May have congestive heart failure Vascular DZ risk Vascular DZ risk 10% if not and pulmonary overcirculation corrected • May have exercise/ exertion • Usually difficulties corrected at age 3-4 Ventricular Septal Treatment in Cath Lab Defect ASD Device • Effect of VSD depends on Size, Closure Location, PVR • Moderate to Moderately severe Sub Art . often no S/S until 6-8 wks of age because of high PVR of newborn 1

10/8/2009 Atrioventricular VSD Closure Canal • Often have Downs Syndrome • Partial or Complete Types • Usually have large Left to Right shunts Coarctation of the AV Canal Repair Aorta • Severe coarctation is dependant on ductus for systemic y blood flow—shock when DA closes • Can be diagnosed much later in less severe cases Interruption of Coarctation Repair the Aortic Arch • Resection of narrowing with • Severe form of patch augmentation in some coarctation with no cases for initial coarct continuation of the • For restenosis balloon aortic arch to the aortic arch to the aortoplasty (cath lab) descending aorta. • Hypertension not uncommon • If the ductus closes after and before repair the child has no flow to • Side effects of surgery r/t the lower extremities cross-clamp time (below and may become clamp ischemia) severely ill and die. 2

10/8/2009 Patent Ductus Aortic Stenosis Arteriosus • Aortic valve is often Flow from small as well as the Aorta • Hard on little stenotic, may be leaky to Branch premies (regurgitant). PA’s, floods • Big DA (term lungs lungs • The LV has to work Th LV h t k baby) big progressively harder and failure, can be becomes hypertrophied. disastrous • At risk for ventricular • Endocarditis risk arrhythmias Aortic Valve Cyanotic Defects Repair • Are blue, get bluer with cry, exertion • Can grow ok sometimes Can grow ok sometimes • If not repaired-At greater risk of – endocarditis – Stokes/abcesses – Immune problems Balloon Valvuloplasty Ross Procedure Tetrology of D-Transposition of Fallot Great Arteries • Often not • Great arteries cyanotic at birth, reversed, not becomes cyanotic compatable Overiding Overiding over time ti with life ith lif Aorta especially with • May have VSD, crying. PFO, DA for PS VSD • Usually repaired mixing surgically 3-6 months of age RVH 3

10/8/2009 Hypoplastic Left Arterial Switch Heart Syndrome • Often the LA, mitral valve, aortic valve, and aortic arch are ti h also small. • Dependant on the Ductus to maintain perfusion, shocky and acidotic when closes Glenn Anastamosis HLHS Paliation (Stage II ~6mos of age) Fontan Procedure Total Anomalous Pulmonary (Stage III ~ 2-4 years of Venous Return age) • Can have supra- diaphramatic or infra-diaphragmatic d drainage, if i if obstructed quite cyanotic 4

10/8/2009 Pulmonary Atresia Tricuspid Atresia with VSD • Most severe end of • Hypoplastic TOF spectrum right heart • Cyanotic @ birth • 50% have 50% have • Ductal Dependant for transposition of pulmonary blood flow great arteries • Often poor pulmonary • Cyanotic early branch tree development Tricuspid Atresia Double Outlet Palliation Right Ventricle Truncus Truncus repair Arteriosus • Fetal truncal artery never completed dividing into the aorta and pulmonic artery. • Various types • If pulmonary artery takeoff narrow then lungs protected • Otherwise-pulmonary overflow and vasc disease 5

10/8/2009 Ebsteins Anomaly Fetal Diagnosis Fetal Echocardiograms • Tricuspid valve • Some CHD easier to diagnose via fetal echo displaced low in RV – HLH,HRH, other single ventricles – Ebstein’s anomaly • Very regurgitant – AV canal • Shunting R → L thru Sh ti R L th • More difficult to diagnose Foramen Ovale – TGA, TAPVR, COARCT, VSD, ASD, TETS • Sometimes inadequate • Impossible— pulm flow without – Ductus, small VSD, ASD, mild valve problem ductus – In Oregon ~ 60% of children who need neonatal surgery were diagnosed in utero • SVT Neonatal Diagnosis Flow from RV to Ductal flow descending reverses, Branch Aorta via ductus, PA’s open up PA’s tiny Ductus closes , Nursery is the ideal time to diagnose congenital heart disease in order to assure early appropriate care (if not prenatally diagnosed) Then PVR Th PVR • Many problems very subtle in early NB drops period (2mos) – Some present after ductus closes (8-48? Hours) – Some present when PulmonaryVascular Fetal Circulation 8 Hours old 24 hrs Resistance drops (2-6 weeks) – Some very minor findings won ’t be obvious for years (minor coarct, ASD, bicusp AV) Neonatal Diagnosis Neonatal Diagnosis • 50 % of babies with murmur in first When to get consult on a newborn few days of life have CHD • Pathological Murmur • 25 % of babies with murmur at 6 weeks have CHD • Cyanosis (sats less than 95) • Cyanosis (sats less than 95) • Diagnoses most likely to lead to death • Poor pulses/perfusion soon after discharge: HLH, IAA, Coarctation (they look pink until ductus closes) • Some get irreversible pulmonary vascular disease and can’t be repaired- shortened life 6

10/8/2009 Case present • Term Baby born in southern Oregon, went • Went to pcp, he was noted to home day 2. On day 5 mom noted fussiness, have poor perfusion (cool appearing pale and cold and decreased extremities & faint pulses) and p ) feeding. Mom states that his stools on day of f g. m y f cyanosis (saturation 84). presentation were "frothy" in appearance, a decreasing PO intake, • Sent to ED nearby tele echo • Mom also describes increased work of showed hypoplastic left heart breathing for two days leading to presentation. syndrome Scenario HLHS continued • What caused symptoms to appear? • Child started on prostaglandin, • Transported to DCH • Had Norwood procedure and was • Had Norwood procedure and was eventually discharged home with Interstage Monitoring Interstage Primary Care Issues Monitoring • For children with single ventricle • Immediate post-op or very severe palliated anatomy complications • Mortality between initial surgery Mortality between initial surgery • vaccines i and 2 nd stage ~ 16% • Synagis • To reduce this we send home with • Endocarditis prophylaxis – OXYGEN SATURATION MONITOR • Dental care – BABY SCALE • Neuro-developmental issues – GUIDELINES TO CALL US 7

10/8/2009 Primary Care Issues Post-op Medical Visit • Assess wound healing, pain, feeding • Assess medications compliance/complications (Digoxin, li / li ti (Di i Lasix, Aldactone, Enalapril, sildenafil, propranol,). • Assess ability to obtain medications • Assess for arrhythmias, post- cardiotomy syndrome. Vaccines postop Synagis/Numax Palivizumab (Synagis) Recommendations • Can restart regular vaccines • Give to children under 2 years of age with except: serious congenital heart disease (Nov-May) • No live virus 6 mo after surgery No live virus 6 mo after surgery – Cyanotic heart disease Cyanotic heart disease if received blood. – Acyanotic disease requiring medications • Should get flu vax if older than 6 – Administer next dose when medically stable following surgery months • Adjust monthly timetable accordingly Endocarditis Endocarditis prophylaxis prophylaxis Procedures with Endocarditis Prophylaxis Risks for Endocarditis Recommended High- --Need prophylaxis for life • Dental extraction • Prosthetic Valves • Cleaning • Complex cyanotic CHD (Tet, TGA, single C l ti CHD (T t TGA i l • T &A, Bronch w\ rigid ventricle) Not Recommended • Shunts & Conduits • Vaginal Delivery • Epicardial pacers • C-section, Hysterectomy Moderate —need prophylaxis for 6 months after • Ear tubes, intubation surgery • All other congenital heart surgery 8