Workshop B: Essentials of Neonatal Cardiology and CHD Anthony C. Chang, MD, MBA, MPH CARDIAC INTENSIVE CARE

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1 SHUNT LESIONS NEONATAL : CONGENITAL CARDIAC MALFORMATIONS AND CARDIAC SURGERY ANTHONY C. CHANG, MD, MBA, MPH CHILDREN S HOSPITAL OF ORANGE COUNTY ATRIAL SEPTAL DEFECT LEFT TO RIGHT SHUNT INCREASED PULMONARY FLOW RV VOLUME OVERLOAD ATRIAL DYSRHYTHMIAS ATRIAL SEPTAL DEFECT PARTIAL ANOMALOUS PV RETURN LV-RA SHUNT (GERBODE DEFECT) CA-RA FISTULA CEREBRAL AVM VENTRICULAR SEPTAL DEFECT LEFT TO RIGHT SHUNT INCREASED PULMONARY FLOW AND PRESSURE LV VOLUME OVERLOAD VENTRICULAR SEPTAL DEFECT PATENT DUCTUS ARTERIOSUS AORTOPULMONARY WINDOW 1

2 COMMON ATRIOVENTRICULAR CANAL COMMON MIXING INCREASED PULMONARY BLOOD FLOW AND PRESSURE AV VALVE REGURGITATION BIVENTRICULAR VOLUME OVERLOAD COMMON ATRIOVENTRICULAR CANAL PAP PBF PVR PAP PBF PVR Low Normal Low CDH Normal or Low PAP PBF PVR PAP PBF PVR CDH Normal or Low VSD Normal VSD in Premie Low 2

3 PAP PBF PVR VSD in Premie VSD in Neonate Low Normal OBSTRUCTIVE LESIONS PULMONARY STENOSIS TETRALOGY OF FALLOT PREOPERATIVE PHYSIOLOGY UNRESTRICTIVE VSD DYNAMIC RVOT OBSTRUCTION RV HYPERTROPHY R TO L SHUNT WITH DECREASE IN SVR TETRALOGY OF FALLOT PREOPERATIVE PHYSIOLOGY TOF AND WITH STENOTIC SHUNT DORV WITH SUBPS SV WITH SUBPS ANY SV WITH STENOTIC SHUNT TREAT WITH OXYGEN, VOLUME, AND NEOSYNEPHRINE 3

4 TETRALOGY OF FALLOT/PULMONARY ATRESIA TETRALOGY OF FALLOT/ ABSENT PULMONARY VALVE AORTIC STENOSIS LV PRESSURE OVERLOAD LV CONCENTRIC HYPERTROPHY SUBENDOCARDIAL ISCHEMIA COEXISTING LESIONS AORTIC STENOSIS CORONARY ISCHEMIA ROSS LV DYSFUNCTION NEOAORTIC INSUFFICIENCY RV DYSFUNCTION RESIDUAL VSD RVOT NARROWING ROSS-KONNO COARCTATION OF THE AORTA LV PRESSURE OVERLOAD LV HYPERTROPHY MYOCARDIAL ISCHEMIA CVA DUCTAL DEPENDENT LESIONS 4

5 DUCTAL DEPENDENT LESIONS Pulmonary Blood Flow Critical PS/ PAtresia TOF with PS or PAtresia SV with PS or Patresia Tricuspid Atresia Ebstein s Anomaly TRICUSPID ATRESIA Ebstein s Anomaly Ebstein s Anomaly Preoperative Pathophysiology Severe TR Functional and PS/PAt RV/LV Interaction Pulmonary Hypoplasia Anatomic HETEROTAXY SYNDROME Heterotaxy HETEROTAXY SYNDROME Asplenia Heterotaxy DORV/ CAVC TAPVR SubPS or PAtresia AET 5

6 HETEROTAXY SYNDROME Heterotaxy Polysplenia Primum ASD/ LVOTO Interrupted IVC Ipsilateral SVC/PVs CHB DUCTAL DEPENDENT LESIONS SYSTEMIC BLOOD FLOW CRITICAL AORTIC STENOSIS COARCTATION OF THE AORTA INTERRUPTED AORTIC ARCH HYPOPLASTIC LEFT HEART SYNDROME HYPOPLASTIC LEFT HEART SYNDROME SINGLE VENTRICLE PHYSIOLOGY INCREASED QP:QS RV VOLUME OVERLOAD EXTRACARDIAC ISSUES HYPOPLASTIC LEFT HEART SYNDROME SINGLE VENTRICLE PHYSIOLOGY INCREASED QP:QS RV VOLUME OVERLOAD EXTRACARDIAC ISSUES SINGLE VENTRICLE PHYSIOLOGY CRITERIA SYSTEMIC AND PULMONARY VENOUS RETURN TO COMMON MIXING CHAMBER SYSTEMIC AND PULMONARY BLOOD FLOW DETERMINED BY SYSTEMIC AND PULMONARY VASCULAR RESISTANCE Single Ventricle Physiology Qp:QS Calculation IF AO-MV OXYGEN% IS 25% IF PV OXYGEN% IS 95% THEN QP:QS = AO MV/ PV PA = 25 / 95 PA AND SINCE AO = PA SAT 25 / 95 POX 6

7 Single Ventricle Physiology Qp:QS (Pox 87%) IF AO-MV OXYGEN% IS 25% IF PV OXYGEN% IS 95% THEN QP:QS = AO MV/ PV PA = 25 / 95 PA AND SINCE AO = PA SAT 25 / = 3:1 HYPOPLASTIC LEFT HEART SYNDROME RV DYSFUNCTION (RV-LV) SINGLE VENTRICLE PHYSIOLOGY TRICUSPID REGURGITATION SHUNT STENOSIS/OBSTRUCTION RESIDUAL ARCH OBSTRUCTION RESTRICTIVE ASD NORWOOD HYPOPLASTIC LEFT HEART SYNDROME RV DYSFUNCTION (RV-LV) SINGLE VENTRICLE PHYSIOLOGY TRICUSPID REGURGITATION RVOT STENOSIS/OBSTRUCTION RESIDUAL ARCH OBSTRUCTION SANO Bidirectional Cavopulmonary Anastomosis Normal Profile PA PRESSURE MMHG LA PRESSURE 0-5 MMHG TP < 10 MMHG SATURATION 80 5% FONTAN MISCELLANEOUS 7

8 TRANSPOSITION OF THE GREAT ARTERIES MIXING ASD (MOST EFFECTIVE) VSD PDA COEXISTING PPHN (REVERSE DIFFERENTIAL CYANOSIS) TRANSPOSITION OF THE GREAT ARTERIES TRANSPOSITION OF THE GREAT ARTERIES PA saturation > Aortic saturation During systole (compliance of atria), atrial shunt is mostly L R During diastole (compliance of ventricles), mostly R L atrial shunt is TRANSPOSITION OF THE GREAT ARTERIES LV PREPAREDNESS CORONARY ISCHEMIA TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION OBSTRUCTIVE TYPE LUNG PATHOLOGY NONOBSTRUCTIVE TYPE RV VOLUME OVERLOAD TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION PULMONARY PATHOLOGY NONCOMPLIANT LV ATRIAL ECTOPIC TACHYCARDIA PV CONFLUENCE NARROWING 8

9 TRUNCUS ARTERIOSUS PEROPERATIVE PATHOPHYSIOLOGY INCREASED PULMONARY BLOOD FLOW AND PRESSURE BIVENTRICULAR VOLUME OVERLOAD TRUNCAL STENOSIS/ INSUFFICIENCY EXTRACARDIAC ISSUES TRUNCUS ARTERIOSUS RV RESTRICTIVE DYSFUNCTION JUNCTIONAL ECTOPIC TACHYCARDIA TRUNCAL STENOSIS/ INSUFFIENCY RESIDUAL VSD THE PRESSURE-VOLUME LOOP CARDIAC OUTPUT MANIPULATION CARDIAC OUTPUT MANIPULATION CARDIAC OUTPUT MANIPULATION PRELOAD 9

10 CARDIAC OUTPUT MANIPULATION AFTERLOAD CARDIAC OUTPUT MANIPULATION CONTRACTILITY VALVE STENOSIS [AORTIC] During ventricular ejection, LV pressure exceeds aortic pressure. Stroke volume is decreased; stroke work can be about the same but potential energy is increased. VALVE STENOSIS [MITRAL] During ventricular filling (diastole), LA pressure exceeds LVEDP. Both stroke volume and stroke work are decreased and the potential energy is unchanged. VALVE INSUFFICIENCY [AORTIC] During ventricular relaxation, blood flows backwards from aorta into to the LV. Pulse pressure widens. Both stroke volume and stroke work are increased but potential energy can be about the same. VALVE INSUFFICIENCY [MITRAL] During systole, LV ejects blood back into the LA as well as into the aorta. The LAP and the v-wave are increased. Both stroke volume and stroke work are increased but potential energy can be about the same. 10

11 VALVE INSUFFICIENCY [MITRAL] Acute- increased LA pressure with pulmonary edema and decreased cardiac output Chronic-increased LA size with little if any pulmonary edema and normal cardiac output Cardiac Cycle of Work/ Career Systole Diastole 11