3/14/2011 MANAGEMENT OF NEWBORNS CARDIAC INTENSIVE CARE CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 WITH HEART DEFECTS

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1 CONFERENCE FOR HEALTH PROFESSIONALS IRVINE, CA. MARCH 7, 2011 MANAGEMENT OF NEWBORNS WITH HEART DEFECTS A NTHONY C. CHANG, MD, MBA, MPH M E D I C AL D I RE C T OR, HEART I N S T I T U T E C H I LDRE N S H OS P I TAL OF O RANGE C OU N T Y AC H C H OC. ORG HISTORICAL BACKGROUND Alfred Blalock Helen Taussig Vivien Thomas Johns Hopkins

2 PERSONAL BACKGROUND William Rashkind William Norwood Philadelphia Aldo Castaneda Boston Michael DeBakey Houston CHD FACTOIDS - INCIDENCE IS ABOUT 1/100 - MOST COMMON CONGENITAL ANOMALY - MOST COMMON LESION IS VSD - MULTIFACTORIAL IN 90% - CYANOSIS, HEART MURMUR, AND SHOCK ARE MOST COMMON PRESENTATIONS - PULSE OXIMETRY SCREENING KEY TO EARLY DIAGNOSIS 2

3 CASE NEONATE WITH HEART MURMUR AT 24 HOURS OF LIFE OXYGEN SATURATION 98% IN RA NO RESPIRATORY DISTRESS SHUNT LESIONS CARDIAC PHYSIOLOGY ATRIAL SEPTAL DEFECT Preoperative Pathophysiology Left to Right Shunt Increased Pulmonary Flow RV Volume overload 3

4 ATRIAL SEPTAL DEFECT Preoperative Pathophysiology Left to Right Shunt Increased Pulmonary Flow RV Volume overload Partial Anomalous PV Return LV-RA Shunt (Gerbode Defect) CA-RA Fistula Cerebral AVM VENTRICULAR SEPTAL DEFECT Preoperative Pathophysiology Left to Right Shunt Increased Pulmonary flow and Pressure LV Volume Overload VENTRICULAR SEPTAL DEFECT Preoperative Pathophysiology Left to Right Shunt Increased Pulmonary flow and Pressure LV Volume Overload Patent Ductus Arteriosus Aortopulmonary Window 4

5 COMMON ATRIOVENTRICULAR CANAL Preoperative Pathophysiology Common Mixing Increased Pulmonary Blood Flow and Pressure AV Valve Regurgitation Biventricular Volume Overload Cyanosis COMMON ATRIOVENTRICULAR CANAL COMMON ATRIOVENTRICULAR CANAL POSTOPERATIVE ISSUES PULMONARY HYPERTENSION RESIDUAL AV VALVE REGURGITATION JUNCTIONAL ECTOPIC TACHYCARDIA HEART BLOCK AV VALVE STENOSIS 5

6 OBSTRUCTIVE LESIONS TETRALOGY OF FALLOT TETRALOGY OF FALLOT POSTOPERATIVE ISSUES RESTRICTIVE RV PATHOPHYSIOLOGY PULMONARY INSUFFICIENCY RESIDUAL RVOTO JUNCTIONAL ECTOPIC TACHYCARDIA 6

7 AORTIC STENOSIS PREOPERATIVE PATHOPHYSIOLOGY LV PRESSURE OVERLOAD LV CONCENTRIC HYPERTROPHY SUBENDOCARDIAL ISCHEMIA COEXISTING LESIONS AORTIC STENOSIS POSTOPERATIVE ISSUES CORONARY ISCHEMIA LV DYSFUNCTION NEOAORTIC INSUFFICIENCY ROSS RV DYSFUNCTION RESIDUAL VSD RVOT NARROWING ROSS-KONNO COARCTATION OF THE AORTA PREOPERATIVE PATHOPHYSIOLOGY LV PRESSURE OVERLOAD LV HYPERTROPHY MYOCARDIAL ISCHEMIA CVA 7

8 COARCTATION OF THE AORTA POSTOPERATIVE ISSUES POSTCOARCTECTOMY SYNDROME EARLY- CATECHOLAMINES LATE- RENIN ANGIOTENSIN RESIDUAL ARCH OBSTRUCTION PARALYSIS VOCAL CORD PARALYSIS PARALYZED DIAPHRAGM DUCTAL DEPENDENT LESIONS 8

9 CARDIAC PHYSIOLOGY FETAL PHYSIOLOGY CARDIAC PHYSIOLOGY NEONATAL PHYSIOLOGY CASE NEONATE WITH CYANOSIS AT 6 HOURS OF AGE PULSE OXIMETRY 72% IN RA AND 79% ON OXYGEN NO RESPIRATORY DISTRESS 9

10 DUCTAL DEPENDENT LESIONS PULMONARY BLOOD FLOW CRITICAL PS PULMONARY ATRESIA (PAT) TOF WITH PS OR PAT SV W/PS OR PAT TRICUSPID ATRESIA EBSTEIN S ANOMALY TRICUSPID ATRESIA EBSTEIN S ANOMALY 10

11 EBSTEIN S ANOMALY PREOPERATIVE PATHOPHYSIOLOGY SEVERE TRICUSPID INSUFFICIENCY FUNCTIONAL AND ANATOMIC PULMONARY ATRESIA RV-LV INTERACTION PULMONARY HYPOPLASIA CARDIOPULMONARY COMPROMISE TETRALOGY OF FALLOT/PULMONARY ATRESIA HETEROTAXY SYNDROME Heterotaxy 11

12 HETEROTAXY SYNDROME Asplenia Heterotaxy DORV CAVC TAPVR SubPS or PAtresia AET HETEROTAXY SYNDROME Heterotaxy Asplenia Polysplenia Primum ASD LVOTO Interrupted IVC Ipsilateral SVC/PVs CHB CASE NEONATE WITH CYANOSIS AND SHOCK AT 18 HOURS OF AGE PULSE OXIMETRY 79% IN RA AND 81% ON OXYGEN SEVERE RESPIRATORY DISTRESS 12

13 DUCTAL DEPENDENT LESIONS SYSTEMIC BLOOD FLOW CRITICAL AORTIC STENOSIS COARCTATION OF THE AORTA INTERRUPTED AORTIC ARCH HYPOPLASTIC LEFT HEART SYNDROME HYPOPLASTIC LEFT HEART SYNDROME PREOPERATIVE PATHOPHYSIOLOGY SINGLE VENTRICLE PHYSIOLOGY INCREASED QP:QS RV VOLUME OVERLOAD EXTRACARDIAC ISSUES HYPOPLASTIC LEFT HEART SYNDROME POSTOPERATIVE ISSUES RV DYSFUNCTION (RV-LV) SINGLE VENTRICLE PHYSIOLOGY TRICUSPID REGURGITATION SHUNT STENOSIS/OBSTRUCTION RESIDUAL ARCH OBSTRUCTION RESTRICTIVE ASD NORWOOD 13

14 HYPOPLASTIC LEFT HEART SYNDROME POSTOPERATIVE ISSUES RV DYSFUNCTION (RV-LV) SINGLE VENTRICLE PHYSIOLOGY TRICUSPID REGURGITATION RVOT STENOSIS/OBSTRUCTION RESIDUAL ARCH OBSTRUCTION RESTRICTIVE ASD SANO PGE1 FACTOIDS - CAN AFFECT ALL FOUR VITAL SIGNS TEMP, HR, RR, AND BP - APNEA RISK HIGHEST IN FIRST 12 HOURS AND INCREASED IN LOW BIRTHWEIGHT AND/OR CYANOSIS - CAN OPEN PATENT DUCTUS ARTERIOSUS IN NEONATES UP TO 3 WEEKS OF AGE MISCELLANEOUS 14

15 TRANSPOSITION OF THE GREAT ARTERIES PREOPERATIVE PATHOPHYSIOLOGY MIXING ASD (MOST EFFECTIVE) VSD PDA COEXISTING PPHN (REVERSE DIFFERENTIAL CYANOSIS) CARDIAC PHYSIOLOGY TRANSPOSITION OF THE GREAT ARTERIES CARDIAC PHYSIOLOGY 15

16 TRANSPOSITION OF THE GREAT ARTERIES POSTOPERATIVE ISSUES LV PREPAREDNESS PULMONARY HYPERTENSION CORONARY ISCHEMIA TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION PREOPERATIVE PATHOPHYSIOLOGY OBSTRUCTIVE TYPE LUNG PATHOLOGY NONOBSTRUCTIVE TYPE RV VOLUME OVERLOAD 16

17 TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION POSTOPERATIVE ISSUES PULMONARY PATHOLOGY PULMONARY HYPERTENSION NONCOMPLIANT LV ATRIAL ECTOPIC TACHYCARDIA PV CONFLUENCE NARROWING TRUNCUS ARTERIOSUS PEROPERATIVE PATHOPHYSIOLOGY COMMON MIXING INCREASED PULMONARY BLOOD FLOW AND PRESSURE BIVENTRICULAR VOLUME OVERLOAD TRUNCAL STENOSIS/ INSUFFICIENCY EXTRACARDIAC ISSUES ISCHEMIC BOWEL SYNDROME MICRODELETION 22Q11 TRUNCUS ARTERIOSUS POSTOPERATIVE ISSUES PULMONARY HYPERTENSION RV RESTRICTIVE DYSFUNCTION JUNCTIONAL ECTOPIC TACHYCARDIA CORONARY ISCHEMIA TRUNCAL STENOSIS/ INSUFFIENCY RESIDUAL VSD 17

18 DOUBLE OUTLET RIGHT VENTRICLE PREOPERATIVE PATHOPHYSIOLOGY DORV/SUBAORTIC VSD (VSD) DORV/ SUBAORTIC VSD AND PS (TOF) DORV/ SUBPULMONARY VSD (TGA) DOUBLE OUTLET RIGHT VENTRICLE PREOPERATIVE PATHOPHYSIOLOGY SUMMARY ANY NEONATE WITH HEART MURMUR OR CYANOSIS SHOULD BE ASSESSED WITH PULSE OXIMETRY AND POSSIBLY ECHOCARDIOGRAPHY AND SEEN BY PEDIATRIC CARDIOLOGIST PRIOR TO DISCHARGE. NEONATES WITH DUCTAL-DEPENDENT SYSTEMIC BLOOD FLOW LESIONS LIKE HLHS ARE MORE CRITICALLY-ILL THAN THOSE WITH DUCTAL- DEPENDENT PULMONARY BLOOD FLOW LESIONS LIKE PULMONARY STENOSIS. 18

19 MULTIDISCIPLINARY TEAM 19

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