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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”

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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 heartdisease should be judged individuallyconsidering age, size, type of lesion andassociated syndrome

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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 BurchCurr Cardiol Rev 2011

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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 2015

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TETRALOGY OF FALLOT WITH CARDIOMYOPATHY

•Results from:• RV pressure overload•Chronic hypoxia•Polycythemia

POSTOPERATIVE TETRALOGY OF FALLOTWITH 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)

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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

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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 56-104 ml

ESV 87 ml 19-49 ml

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RV ANALYSIS

RVEF 9%ESV 97.6 mlEDV 107 ml

TDI PATIENT NORMAL VALUES

Myocardial Performance

Index

0.88 <0.55

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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} + 0.16, 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 isprevalent in DTGA with non restrictive VSD orLarge PDA. Grade 3 or 4 Heath & Edwardshistologic changes are found in about 20% ininfants before 2 months of age andapproximately 80% of patients after 1 year ofage

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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%

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Echo Parameter

Patient Normal Values

LVEF (mmode) 30.3% >55%LV STUDY (3D)

EFEDVESV

35%83.9 ml54 ml

>55%

LONGITUDINAL STRAIN (SYSTEMIC VENTRICLE)

STRAIN = -12 (NORMAL -20 +/-2)

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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 Patient Normal

ValuesMean

Pulmonary artery pressure

mPAP = 79 – 0.45 (RVOT AT)

51 mmHg <25 mmHg

Pulmonary Artery Pressure

by TR jet 108mmHg <30 mmHg

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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 IIIPATIENTS PROFILE

•18 years old •Male•Dx: CHD, Single Ventricle of Left ventricle morphology

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LV EJECTION FRACTION

MALE NORMAL MILD MODERATE SEVERELVEF 52-72 41-51 30-40 <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 56-104 ml

ESV 174 ml 19-49 ml

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Strain = -16.6 (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 pressuremPAP = 79 – 0.45 (RVOT AT) 57 mmHg by PAT <25mmHg

Pulmonary Vascular ResistancePVR (woods/unit) = 10. (VTR/VTIRVOT) + .16

18 woods/unit < 3 wood units

Mean Transpulmonary GradientTPGm = mPAP -mLAP

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 thePVRI , Panama 2011 Pedia Circulation 2011Bossone Eduardo MD. Et, al. Echocardiography in Pulmonary Hypertension : From diagnosis to prognosis , Journal Of the American Society of Echocardiography, January 2011.

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CONCLUSION

• Echocardiogram is a useful tool to identify operablelesions but may require additional modalities toassess those with borderline parameters forintervention

THANK YOU!