COMPLEX CONGENITAL HEART DISEASE: WHEN IS IT TOO LATE TO INTERVENE?

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1 COMPLEX CONGENITAL HEART DISEASE: WHEN IS IT TOO LATE TO INTERVENE? Aurora S. Gamponia, MD, FPPS, FPCC, FPSE OBJECTIVES Identify complex congenital heart disease at high risk or too late for intervention Present echocardiographic parameters that would suggest inoperable state 1

2 INCIDENCE Estimates 1.2 million affected babies every year Worldwide: 9 per 1,000 of 135 million birth have congenital heart disease At least 300,000 (2 to 3 per 1,000) will have severe conditions - Botto LD Philippine Heart Center (Pediatric Cardiovascular Surgery) Number of cardiac surgeries in 2017: 1003 Number of unoperated service patients: 632 RECOMMENDATION: ESC, AHA Operability in complex congenital heart disease should be judged individually considering age, size, type of lesion and associated syndrome 2

3 COMMON ETIOLOGY OF HIGH RISK OR TOO LATE FOR INTERVENTION Severe Pulmonary Hypertension/ Eisenmenger Syndrome Heart Failure/ Cardiomyopathy Associated severe defects COMPLEX CONGENITAL HEART DISEASE : HIGH RISKS FOR CARDIAC SURGERY AND CONSIDERED FOR HEART TRANSPLANT Hypoplastic Left Heart with abnormalities (impaired ventricular function Severe Ebstein s Anomaly Pulmonary Atresia with intact ventricular septum associated with abnormal coronary anatomy Severe Valve Abnormalities Heterotaxy Lesions E Sian Pincott and M Burch Curr Cardiol Rev

4 TETRALOGY OF FALLOT Pulmonary hypertension due to MAPCAs Heart failure due to secondary cardiomyopathy that results from RV pressure overload, and polycythemia Post TOF repair with severe pulmonary regurgitation TETRALOGY OF FALLOT Yasuhara, et. Al. Int. Heart J

5 TETRALOGY OF FALLOT WITH CARDIOMYOPATHY Results from: RV pressure overload Chronic hypoxia Polycythemia POSTOPERATIVE TETRALOGY OF FALLOT WITH SEVERE PULMONARY REGURGITATION RV dysfunction and ventricular- ventricular interaction RV EDV > 170 ml/ m2 or RV ESV > 85 ml/ m2, none achieved normalization of right ventricular volume after pulmonary valve replacement Post operative pulmonary regurgitation with progressive RV enlargement and dysfunction: risk of sudden cardiac death and the relationship between RV size and QRS duration (risk related duration of 180 ms) 5

6 TETRALOGY OF FALLOT Predictors of poor outcome, late repair of >/= 6 years old, elevated RV EDV, reduced RV EF and LV EF, longer QRS duration 100% sensitivity for adverse clinical outcome when the RV EDD was >/= 45/m2 and 96% specific when the LVEF < 30% - Knauth AI Heart 2008 CASE 17 years old Female Dx: CHD, Tetralogy of Fallot 6

7 TOF UNREPAIRED Patient Normal values RVEF (FAC) 29% >35% TAPSE 1.4 > 1.7 LV STUDY (3D) PATIENT NORMAL VALUES EF (3D) 19% >49% EDV 107 ml ml ESV 87 ml ml 7

8 RV ANALYSIS RVEF 9% ESV 97.6 ml EDV 107 ml TDI PATIENT NORMAL VALUES Myocardial Performance Index 0.88 <0.55 8

9 PULMONARY HYPERTENSION IN CONGENITAL HEART DISEASE Surgery is contraindicated when the pulmonary vascular resistance index (PVRI) is >/= 6 WUxm2 and a PVR/SVR ratio > 0.3 have to be evaluated by vasodilator testing (ESC, AHA) Pulmonary Vascular Resistance : { TRVmax/ VTI (RVOT) x10} , value >38 specificity of 100% for PVR of > 8 wood units Tricuspid annular plane systolic excursion of,18 9n adult with pulmonary hypertension is with greater systolic dysfunction and lower survival rate TRANSPOSITION OF THE GREAT ARTERIES Accelerated pulmonary vascular disease is prevalent in DTGA with non restrictive VSD or Large PDA. Grade 3 or 4 Heath & Edwards histologic changes are found in about 20% in infants before 2 months of age and approximately 80% of patients after 1 year of age 9

10 TRANSPOSITION OF THE GREAT ARTERIES Assessment of operability in DTGA with VSD: an practical method by Pankaj Bajpai Patients with pulmonary hypertension were given heated and humidified oxygen at 10 L/min by mask for 48 hours (intermittent) Echo parameter: color Doppler showed increased pulmonary blood flow to the LA, dilation of the LA and LV and right to left shunting Am Pediatr Cardiol 2011 Echo parameter PATIENT NORMAL VALUES RVEF (FAC) 26.2% >35% 10

11 Echo Patient Normal Values Parameter LVEF (mmode) 30.3% >55% LV STUDY (3D) EF EDV ESV 35% 83.9 ml 54 ml >55% LONGITUDINAL STRAIN (SYSTEMIC VENTRICLE) STRAIN = -12 (NORMAL -20 +/-2) 11

12 STRAIN = -10 (NORMAL -20 +/-2) Jashari, Haki et al. Normal ranges of Left ventricular strain in children: a meta analysis. Cardiovascular ultrasound 2015, 13:37. Echo parameter Mean Pulmonary artery pressure mpap = (RVOT AT) Patient Normal Values 51 mmhg <25 mmhg Pulmonary Artery Pressure by TR jet 108mmHg <30 mmhg 12

13 SINGLE VENTRICLE PHYSIOLOGY In children/ adolescent with single ventricle physiology, the hemodynamic threshold for operability pre-fontan surgery is probably a mean transpulmonary gradient </= 6 mmhg; pulmonary vascular resistance index of </= 3 WU x m2 Mean Transpulmonary gradient + mpap mean LAP LAP = SBP 4 (MR Vmax)2 Goscan J et al Am Heart J 1991 CASE III PATIENTS PROFILE 18 years old Male Dx: CHD, Single Ventricle of Left ventricle morphology 13

14 LV EJECTION FRACTION MALE NORMAL MILD MODERATE SEVERE LVEF <30 Lang, et. al. ASE Recommendations for cardiac chambers: Quantification by Echocardiography in adults: J Am Soc Echocardiogr 2015;28:1-39. LV STUDY (3D) Patient Normal Values EF 32.5% >45% EDV 258. l ml ESV 174 ml ml 14

15 Strain = (normal -20 +/-2) Jashari, Haki et al. Normal ranges of Left ventricular strain in children: a meta analysis. Cardiovascular ultrasound 2015, 13:37. ESTIMATED PULMONARY ARTERY PRESSURE BY ECHO Patient Normal values Mean Pulmonary artery pressure mpap = (RVOT AT) 57 mmhg by PAT <25mmHg Pulmonary Vascular Resistance PVR (woods/unit) = 10. (VTR/VTIRVOT) +.16 Mean Transpulmonary Gradient TPGm = mpap -mlap 18 woods/unit < 3 wood units 41 mmhg < 6 mmhg S wave velocity by DTI 0.9 cm/sec < 10 cm/sec Cero MJD et.al,. A consensus approach to the Classification of pediatric PAH vascular disease:report for the PVRI, Panama 2011 Pedia Circulation 2011 Bossone Eduardo MD. Et, al. Echocardiography in Pulmonary Hypertension : From diagnosis to prognosis, Journal Of the American Society of Echocardiography, January

16 CONCLUSION Echocardiogram is a useful tool to identify operable lesions but may require additional modalities to assess those with borderline parameters for intervention THANK YOU! 16