Critical Congenital Heart Disease in Neonates

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Early detection & Initial Treatment
Sukman T Putra, MD, FACC, FESC
Senior Lecturer, Chairman of Cardiology Department of Pediatrics, University of Indonesia,Integrated Cardiovascular Center, Dr.Cipto Mangunkusumo National General Hospital, Jakarta, INDONESIA E-mail : stpfika@dnet.net.id
19th Vietnam Congress of Pediatrics, HCMC ,27-28 Dec 2008

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THE REGIONAL HEART CENTER IN INDONESIA
Population : 230 million 46.000 CHD babies born/year

19.000 islands

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THE PURPOSE
 Early detection & Recognition of CCHD in Neonates  The initial treatment / Management by Prim.Physician  Timing of referralPediatrics, of the 19th Vietnam Congress of HCMC, 27-28 December 2008 Neonates with CCHD

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OUTLINE
 Backgrounds (Facts & Figures)  Diagnosis, Early detection &Recognition CCHD in Neonates  Initial Treatment & Management Timing of Referral of the Neonates with CCHD  Conclusion
19th Vietnam Congress of Pediatrics, HCMC, 27-28 December 2008
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Issues of CHD in Developing Countries

 The magnitude of the problems Type of CHD at birth and survival patterns  CHD as a contributor to IMR  Resources for CHD treatment
Congenital CardiologyToday

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Congenital Heart Disease in the Developing World

Kumar, K. CongenitalCardiology Today,Vol 3, April 2

Congenital Heart Disease

DEFINITION OF CHD TES “ A gross abnormality of the heart or intrathoraxic great vessels that is actually or potentially of functional significance”
Mitchel et al , Circulation
19th Vietnam Congress 1971;43: 323-32 of Pediatrics,HCMC 27-28 Dec.2008
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CONGENITAL HEART DISEASE
• The global prevalence of CHD 4-5/1000 live birth – 12-14 / live birth
Constant in different geographic & ethnics backgrounds

• Contributor to IMR 7% of neonatal death : major congenital malformation ( of Lancet

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Facts & Figures of CHD in Early life
 One in three infants (30%)

with a potentially life threatening cardiovascular malformat left hospital undiagnosed !! post-natal outcome (TGA,HLHS Coarctation, etc.)

 Prenatal diagnosis improves
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Facts & Figures of CHD in Early Life
 Routine Neonatal Examination

fails to detect > 50% of CHD in neonates  Fails to detect >1/3 by 6 weeks  Normal findings examination does not exclude heart disease  Babies with murmur at neonatal or 6 weeks should be referred for cardiac evaluation
Arch Dis Child Fetal Neonatal Ed
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Facts & Figures of CHD in Early Life

Full-term Infant

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CCHD

Acta Paediatrica 2006; 95:407-13

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Facts & Figures of CHD in Early Life
Of 669 infants with life-threatening cardiovascular malformation :  55 (8%) had an antenatal diagnosis  416 (62%) had postnatal diagnosis before discharged from hospital  168 (25%) was diagnosed in living infant after discharged  30 (5%) were diagnosed at autopsy
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DIAGNOSIS EARLY DETECTION & RECOGNITION Critical Congenital Heart Disease in Neonates 19th Vietnam Congress of Pediatrics,
HCMC, 27-28 December 2008
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Critical Congenital Heart Disease (CCHD)in Neonates

DEFINITION OF CCHD Congenital Heart Disease that are ductal dependent or may equired surgical or invasive intervention or resulted in death in the first 30 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 the infant with serious or life threatening heart disease) is essential for the OPTIMAL OUTCOME Evaluation should  Cyanosis  Decreased systemic perfusion  Tachypnea (due to excessive pulmonary blood flow )
focus on 3 cardinal signs

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Diagnostic TOOLS of Congenital Heart Disease

• History of illness • Physical Examination • Electrocardiogram (ECG) • Chest X-Ray • Echocardiogram • Cardiac Catheterization
19th Vietnam Congress of Pediatrics, HCMC, 27-28 December 2008
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Chest X-Ray

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

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Four-Chamber Views

Imaging planes
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CRITICAL CHD
Birth/ Delive ry Surgical/ Interventi on

Hemodynamic Instability
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Unrecognized CCHD
 Profound metabolic acidosis  Hypoxic-ischemic encephalopathy,  Intracranial haemmorhage  Entrocolitis,  Cardiac Arrest even Death (when ductus constricts)
19th Vietnam Congress of Pediatrics, Ho Chi Minh City 27-28 Dec 2008
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Early Detection of CCHD
(1) Prenatal diagnosis (Fetal Echo) (2) Post natal : PE, ECG,Echo, CXR 3 (Three) Cardinal Sign of CCHD Cyanosis Decreased systemic

 

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CCHD in Neonates
Decreased systemic perfusion 1. Coarctation of the Aorta 2. Hypoplastic LH syndrome 3. Cardiomyopathy  Cyanosis 1. Decreased pulmonary blood flow 2. Norma/increased PBF (TGA)  Tachypnea (excessive PBF) Left to right shunt 
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CRITICAL CONGENITAL HEART DISEASE 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
19th Vietnam Congress of Pediatrics,
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CRITICAL CONGENITAL HEART DISEASE in NEONATES

Shock syndrome
Severe clinical state (cyanosis, weak pulses, hepatomegaly, oliguria) Blood pressure : 4 extremities
( Symtomatic: “duct dependent 19th Vietnam Congress of lesions’’ ) Pediatrics,
HCMC, 27-28 Dec 2008
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Congenital Heart Disease in Neonates
Distribution of CHD based on Age at Diagnosis 0-6 days : Transposition Great Arteries (19%) Hypoplastic Left HSyndrome(14%) Tetralogy of Fallot (8%) Coarctation of aorta (7%)
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Congenital Heart Disease in Neonates
Distribution of CHD Based on Age at DIAGNOSIS 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 Heart Disease in Neonates
Distribution of CHD Based on Age at Diagnosis
14- 28 days : VSD (16%) Coarctation of the aorta (12%) Tetralogy of Fallot (7%) TGA (7%) Marion BS at.al :Clinics in Perinatology 29 Patent 2001 : Ductus

TRICUSPID ATRESIA
•Hypoplastic RV •R-L shunt atria level •B-T shunt and finally FONTAN
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Hypoplastic Left Heart Syndrome (HLHS)
• DUCT DEPENDENT SYSTEMIC CIRCULATION • Clinical manifestations: Day > 6 •19th Vietnam Congress of Pediatrics, Norwood Operation HCMC, 27-28 December 2008 • Unfavour Prognosis
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Coarctation of the Aorta (CoA)

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

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Post Natal & Fetal Circulation

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

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CONGENITAL HEART DISEASE in Neonates
Transitional Circulation soon After BIRTH • Increasing pulmonary blood flow (20 x of PBF in fetal circulation) • Significant changes of central circulation to be a “serial circulation” ( closing of ducts, foramen ovale). • Increased ventricular output for respiratory efforts and thermoregulati
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Interventional Cardiology Procedures in Neonates with CCHD
19th Vietnam Congress of Pediatrics City 27-28 Dec 2008

Sirkulasi Janin (Fetal Circulation)

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RV-graphy before Ballooning

BALLOONING PULMONARY VALVE

RV GRAPHY AFTER BALLONING

INITIAL TREATMENT OF CRITICAL CONGENITAL HEART DISEASE
19th Vietnam National Pediatric Congress, Ho Chi Minh City 27-28 Dec 2008

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Patent Ductus Arteriosus
Echocardiogram of 50 normal Neonates :  42% closed in 24 hours  78% closed in 40 hours  90% closed in 48 hours  Undetected at the age of 96 hrs
1981;98:443-48 J Pediatr

TIMING OF REFERRAL CRITICAL CONGENITAL HEART DISEASE
19th Vietnam National Pediatric Congress, Ho Chi Minh City 27-28 Dec 2008

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URGENT REFERRAL of CCHD

Potentially lethal CHD (15% CHD)  Cyanosis  Shock  Pulmonary edema
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CONGENITAL HEART DISEASE

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PALLIATIVE PROCEDURES for increasing Pulmonary Circulation
19th Vietnam Congress Pediatrics, HCMC, 27-28 Dec 2008

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HLHS

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

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CRITICAL CONGENITAL HEART DISEASE in NEONATES
INITIAL TREATMENT

• To prevent deterioration • Should follow the general approach guidelines for critically ill neonates. • Should be initiated as soon as the diagnosis established.

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CRITICAL CONGENITAL HEART DISEASE in Neonates
INITIAL TREATMENT

Basic advanced life support • Maintaining the ductus & stable airway (PGE1 or Stenting PDA) • Blood gas & monitoring blood pressure

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CRITICAL CONGENITAL HEART DISEASE in Neonates

TREATMENT Primary treatment • CONSERVATIVES (O2,mecahnical ventilation ) • PALLIATIVES : Ballooon atrial septostomy in TGA
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CRITICAL CONGENITAL HEART DISEASE in NEONATES
TREATMENT The second step • SURGERY (BT shunt / Repair) • PDA Stenting ( before surgery) an alternative to surgical shunt
Alwi at.al JACC 2004;44:438-45
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CRITICAL CONGENITAL HEART DISEASE in Neonates

• Prostaglandin E1 Administered for duct dependent lesions: 10-20 nanogram/min. Side effects: apnea 10-15% cases Additional : diuretic & inotropic • Effective : age less than 2

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CRITICAL CONGENITAL HEART DISEASE in Neonates
• Oxygen : consider the goal of therapy and adverse effects (maintain O2 sat & PaO2) • Fluids : fluid status & urine output. Day 1 & 2 same fluid and glucose requirement as normal (depend on the type of defects for the next days)
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Case Illustration (Real Case)
Fullterm baby : • Born by S,C , weight 3200 gram, AS 9/10 12 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 Cardiologist ECHO : Transposition Great Arteries, PDA, small ASD Surgery : 3 weeks of age
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“ Egg on Side” appearance

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TGA

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“Booth Shape” appearance

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CRITICAL CONGENITAL HEART DISEAS in NEONATES

TIMING OF REFERRAL “ A professional decision making” • Symtomatic neonates • Asymtomatic neonates
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CRITICAL CONGENITAL HEART DISEAS in NEONATES

TIMING OF REFERRAL “ A professional decision making” • Symtomatic neonates • Asymtomatic neonates
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CONGENITAL HEART DISEASE in NEONATES
TIMING OF REFERRAL • Should be a professional decision • Symtomatic neonates Start initial treatment immediately. PGE1 started before referral • As soon as the baby stable
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CRITICAL CONGENITAL HEART DISEASE in NEONATES
Hypercyanotic Spells Rare in the newborn period,begin With 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 DISEASE in NEONATES
CONCLUSION • Early detection of Critical CHD in neonates is very important for the optimal outcome. • Initial evaluation of neonates suspected CHD should include : history, physical exam, ECG, CXR, echocardiogram and Hyperoxic test 19th Vietnam Congress of Pediatrics,
HCMC ,27-28 Dec 2008
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CRITICAL CONGENITAL HEART DISEASE in NEONATES
CONCLUSION • More than 50% of CHD fails to detect on routine neonatal examination. • Hyperoxic test is important to differentiate cyanosis due to cardiac and non cardiac origin.
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CRITICAL CONGENITAL HEART DISEASE in NEONATES
CONCLUSION • PGE1 should be started immediately in neonates with critical CHD • Initial treatment consist of: PGE1, fluids and medication • The timing of referral should be a “professional decision” which much depend on the diagnostic and initial treatment • Pediatricians : should be able to detect early signs and symptom of Critical CHD in neonates. 19th Vietnam National Pediatric Congress,
Ho Chi Minh City 27-28 Dec 2008
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