Critical Chd Vietnam

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Critical

Congenital HeartDisease in Neonates :

Early detection & Initial Treatment Sukman T Putra, MD, FACC, FESC

Senior Lecturer, Chairman of CardiologyDepartment of Pediatrics, University of

Indonesia,Integrated Cardiovascular Center, Dr.CiptoMangunkusumo National General Hospital, Jakarta,

INDONESIAE-mail : [email protected]

19th Vietnam Congress of Pediatrics,HCMC ,27-28 Dec 2008


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C h ildre n a r

e n o t

li t t le a

d u l t s


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THE REGIONAL HEART CENTER ININDONESIA

Population : 230 million

46.000 CHD babies born/year

19.000 islands 2


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THE PURPOSE

Early detection &

Recognitionof CCHD in NeonatesThe initial treatment /

Management byPrim.PhysicianTiming of referral of theNeonates with CCHD 5

19th Vietnam Congress of Pediatrics,

HCMC, 27-28 December 2008


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OUTLINE

Backgrounds (Facts & Figures)Diagnosis, Early detection

&Recognition CCHD in NeonatesInitial Treatment & ManagementTiming of Referral of the Neonates

with CCHDConclusion

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HCMC, 27-28 December 2008


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Issues of CHD inDeveloping

Countries

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The magnitude of the problemsType of CHD at birth andsurvival patterns

CHD as a contributor to IMRResources for CHD treatment

Congenital CardiologyToday-

B a c k g

r o u n

d


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Congenital Heart Disease

in the Developing World

U n f o r t u n a t e l y..

.... v e r y f e w h i g h - q u

a l i t y

I n s t i t u t i o n s w i t h c o m p r

e h e n s i v e f a c i l i t i e s

t o t a k e c a r e o f c h i l d r e n w i t h

C o n g e n i t a l H e

a r t D i s e a s e e x i s t o u

t s i d e o f t h e

D e v e l o p e d W o

r l d

Kumar, K. CongenitalCardiology Today,Vol 3, April


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Congenital Heart Disease

TES

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DEFINITION OF CHDA gross abnormality of the heart o

intrathoraxic great vessels that isctually or potentially of functional

significance

Mitchel et al , Circulation1971;43: 323-3219th Vietnam Congress of Pediatrics,HCMC 27-28 Dec.2008


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CONGENITAL HEARTDISEASE

The global prevalence of CHD

4-5/1000 live birth 12-14 / livebirth Constant in different geographic &

ethnics backgrounds Contributor to IMR7% of neonatal death : major

congenital malformation ( of 10 Lancet-


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Facts & Figures of CHDin Early life

One in three infants (30%)with

a potentially life threateningcardiovascular malformatleft hospital undiagnosed !!

Prenatal diagnosis improvespost-natal outcome

(TGA,HLHSCoarctation, etc.) 11


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Facts & Figures of CHDin Early Life

Routine Neonatal Examinationfails to detect > 50% of CHD in

neonatesFails to detect >1/3 by 6 weeksNormal findings examination

does not exclude heart diseaseBabies with murmur at neonatalor 6 weeks should be referred forcardiac evaluation

Arch Dis Child Fetal Neonatal Ed 12


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229Full-term

Infant

CCHD

13Acta Paediatrica 2006; 95:407-13


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Of 669 infants with life-threateningcardiovascular malformation :

55 (8%) had an antenatal diagnosis 416 (62%) had postnatal diagnosisbefore

discharged from hospital

168 (25%) was diagnosed in livinginfantafter discharged

30 (5%) were diagnosed at autopsy 14


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DIAGNOSISEARLY DETECTION &

RECOGNITIONCritical Congenital Heart

Diseasein Neonates19th Vietnam Congress of Pediatrics,HCMC, 27-28 December 2008


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Critical Congenital HeartDisease (CCHD)in Neonates

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DEFINITION OF CCHDCongenital Heart Disease thatare ductal dependent or may

quired surgical or invasive interventio

or resulted in death in the first30 days of life

Pediatrics,

2008;121:751-757 19th Vietnam Congress of Pediatrics,HCMC 27-28 Dec.2008


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Early identification of theinfant with serious or life

threateningheart disease) is essential for the

OPTIMAL OUTCOMEEvaluation shouldfocus on

3 cardinal signs

CyanosisDecreased systemic

perfusionTachypnea ( due to

excessivepulmonary blood flow )


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Diagnostic TOOLSof Congenital Heart Disease

History of illness Physical Examination

Electrocardiogram (ECG) Chest X-Ray

Echocardiogram Cardiac Catheterization

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Chest X-Ray

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HCMC, 27-28 Dec 2008


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Four-Chamber Views

20Imaging planes


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CRITICAL CHD

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Birth/Delivery

Surgical/Interventi

on

Hemodynamic Instability


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Unrecognized CCHD

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Profound metabolic acidosisHypoxic-ischemic

encephalopathy,Intracranial haemmorhageEntrocolitis,Cardiac Arrest even Death(when ductus constricts )

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Early Detection of CCHD

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(1) Prenatal diagnosis ( Fetal Echo )

(2) Post natal : PE,ECG,Echo, CXR

3 (Three) Cardinal Sign of CCHD

CyanosisDecreased systemic


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CCHD in Neonates

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Decreased systemic perfusion 1. Coarctation of the Aorta

2. Hypoplastic LH syndrome

3. Cardiomyopathy Cyanosis1. Decreased pulmonary blood flow2. Norma/increased PBF (TGA)

Tachypnea (excessive PBF)Left to right shunt


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CRITICAL CONGENITAL HEARTDISEASE in Neonates

HEART MURMUR Murmur detected : 54% due

to cardiovascular problems Hars murmur in neonates:

stenotic lesions (AS, PS) J Pediatr Child

Health 2001;37:331-36

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CRITICAL CONGENITAL HEARTDISEASE in NEONATES

Shock syndrome

Severe clinical state (cyanosis,weak pulses, hepatomegaly,oliguria)

Blood pressure : 4 extremities( Symtomatic: duct dependent

lesions ) 26 19th Vietnam Congress

of Pediatrics,HCMC, 27-28 Dec 2008


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Congenital Heart Diseasein Neonates

Distribution of CHD based on Age atDiagnosis0-6 days : Transposition Great

Arteries (19%)Hypoplastic Left

HSyndrome(14%)Tetralogy of Fallot (8%)Coarctation of aorta (7%)

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Congenital Heart Diseasein Neonates

Distribution of CHD Based on Age atDIAGNOSIS

7-13 days : Coarctation of Aorta (16%)Ventricular Septal Defect

(14%)

HLHS (8%)TGA (7%)Tetralogy of Fallot (7%)

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Critical Congenital HeartDisease

in NeonatesDistribution of CHD Based on

Age atDiagnosis14- 28 days : VSD (16%) Coarctation of the aorta

(12%)Tetralogy of Fallot (7%)TGA (7%)

Patent Ductus 29 Marion BS at.al :Clinics in Perinatology2001 :


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TRICUSPIDATRESIA

Hypoplastic RVR-L shunt atria level

B-T shuntand finally FONTAN


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Hypoplastic Left HeartSyndrome (HLHS)

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DUCT DEPENDENT

SYSTEMICCIRCULATION Clinical

manifestations:Day > 6 Norwood Operation Unfavour Prognosis



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Coarctation of the Aorta(CoA)



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Post Natal & FetalCirculation

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CONGENITAL HEART DISEASE

in NeonatesTransitional Circulation soon After

BIRTH

Increasing pulmonary blood flow (20 xof PBF in fetal circulation) Significant changes of central

circulation to be a serial circulation (closing of ducts, foramen ovale).

Increased ventricular output forrespiratory efforts and thermoregulati

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Interventional CardiologyProcedures in Neonates

with CCHD19th Vietnam Congress of Pediatrics

City 27-28 Dec 2008

Si k l i J i (F l


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Sirkulasi Janin (FetalCirculation)

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RV-graphy before Ballooning


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BALLOONING PULMONARY VALVE


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RV GRAPHY AFTER BALLONING


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Patent Ductus

Arteriosus Echocardiogram of 50 normalNeonates :

42% closed in 24 hours78% closed in 40 hours90% closed in 48 hoursUndetected at the age of 96 hrs

J Pediatr1981;98:443-48


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TIMING OF REFERRALCRITICAL CONGENITAL

HEART DISEASE19th Vietnam National Pediatric Congress,

Ho Chi Minh City 27-28 Dec 2008


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URGENT REFERRAL of CCHD

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Potentially lethal CHD (15%CHD)

CyanosisShockPulmonary edema


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PALLIATIVEPROCEDURESfor increasingPulmonary Circulation

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19th Vietnam Congress Pediatrics,HCMC, 27-28 Dec 2008

HLHS


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INITIAL TREATMENT

To prevent deterioration Should follow the general

approach guidelines forcritically ill neonates. Should be initiated as soon as

the diagnosis established 48


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INITIAL TREATMENT

Basic advanced life support Maintaining the ductus &stable airway (PGE1 orStenting PDA)

Blood gas & monitoring blood

pressure 49


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TREATMENTPrimary treatment CONSERVATIVES (O2,mecahnical

ventilation )

PALLIATIVES : Ballooon atrialseptostomy in TGA

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TREATMENTThe second step SURGERY (BT shunt / Repair) PDA Stenting ( before surgery)

an alternative to surgical shunt Alwi at.al JACC

2004;44:438-4551


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Prostaglandin E1Administered for duct

dependent lesions: 10-20nanogram/min.

Side effects: apnea 10-15%cases Additional : diuretic &inotropic

Effective : age less than 2 52


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Oxygen : consider the goal of therapy and adverse effects(maintain O2 sat & PaO2)

Fluids : fluid status & urineoutput. Day 1 & 2 same fluid andglucose requirement as normal(depend on the type of defectsfor the next days)

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Case Illustration (Real Case)

Fullterm baby : Born by S,C , weight 3200 gram, AS 9/1012 hours after birth : mild cyanosis , tachypnoe

PE: central cyanosis, RR 48x/m,

HR 144x/m, no murmur , normal pulses ECG : RAD, no hypertrophy, normal CXR : normal pulm vasc .egg on side Blood Gas Analysis : pH : 7.35 , PaO2 : 66 mmHg

O2 Sat 79%Consulted to Pediatric Cardiologis t ECHO : Transposition Great Arteries, PDA, small ASD

Surgery : 3 weeks of age

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TGA

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CRITICAL CONGENITAL HEARTDISEAS in NEONATES

TIMING OF REFERRAL

A professional decisionmaking Symtomatic neonates Asymtomatic neonates

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CRITICAL CONGENITAL HEARTDISEAS in NEONATES

TIMING OF REFERRAL A professional decision

making Symtomatic neonates Asymtomatic neonates

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CONGENITAL HEART DISEASEin NEONATES

TIMING OF REFERRAL Should be a professional decision

Symtomatic neonatesStart initial treatmentimmediately.PGE1 started before referral

As soon as the baby stable

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Hypercyanotic SpellsRare in the newborn period,beginWith irritability & crying. Increased cyanosis Placing knee-chest (>> syst.vasc

resist) Morphine 0.1 mg/kg i.v If unresponsive: start vasopressor

(phenyleprine) to decrease R-L

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CRITICAL CONGENITAL HEART


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CONCLUSION Early detection of Critical CHD in

neonates is very important for theoptimal outcome. Initial evaluation of neonates

suspected CHD should include :history, physical exam, ECG, CXR,echocardiogram and Hyperoxictest

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CONCLUSION More than 50% of CHD fails to

detect on routine neonatalexamination.

Hyperoxic test is important todifferentiate cyanosis due tocardiac and non cardiac origin.

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CONCLUSION PGE1 should be started immediately in

neonates with critical CHD

Initial treatment consist of: PGE1, fluidsand medication The timing of referral should be a

professional decision which muchdepend on the diagnostic and initialtreatment

Pediatricians : should be able to detectearly signs and symptom of Critical CHDin neonates.

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Ho Chi Minh City 27-28 Dec 2008


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Critical Chd Vietnam