LIFE

THREATENING CONGENITAL
HEART DISEASE

Dr. Heny Martini, SPJP(K)

 Structural cardiac malformations in which cardiovascular collapse is
likely and compromised if not treated early

Critical CHD lesion requiring surgery or catheter based intervention

in the first year of life
INTRODUCTION
Congenital cardiac defect 8-10/1000 live born infants
1/3-1/4 are fatal during newborn period
Prompt recognition is essential for survival
Advances in diagnosis, catheter intervention, intensive care and
surgical technique, also intensive post operative management
salvage many critically ill infant
Age on admission Diagnosis
newborn PAIVS
Severe TOF
HLHS, Critical CoA
Triscupid Atresia
1st week of life TGA
CoA
VSD/PA
TAPVD
1 month of life TGA
TOF
TAPVD
PRESENTATION OF NEONATE WITH CONGENITAL HEART
DISEASE
Timing of presentation and severity depends on
1. Nature and severity of defect
2. The alteration in cardiovascular physiology secondary to effect of
the transitional circulation as
- Closure of ductus arteriosus /restriction of patent foramen ovale
- Fall in pulmonary vascular resistance (PVR
 1. Ductal dependent
circulation

STRUCTURAL HEART DISEASE IN 2. Restriction at foramen

NEONATAL PERIOD ovale

3. Obstructed total
anomalous pulmonary
venous connection
 1. Ductal dependent
circulation

STRUCTURAL HEART DISEASE IN 2. Restriction at foramen

NEONATAL PERIOD ovale

3. Obstructed total
anomalous pulmonary
venous connection
1. Ductal Dependent Circulations
Maintain adequate systemic blood flow in left sided obstructive lesions
Critical aortic stenosis
Severe coarctation of aorta/interrupted aortic arch
Hypoplastic left heart syndrome
Maintain adequate systemic blood flow in right sided obstructive lesions
Critical pulmonary stenosis/ pulmonary atresia with intact ventricular septum
Pulmonary atresia with VSD
Tricuspid atresia with pulmonary atresia
Univentricular heart with pulmonary atresia
Severe ebsteins anomaly
Ensure adequate mixing as in conditions with parallel circulation
TGA
 1. Ductal dependent
circulation

STRUCTURAL HEART DISEASE IN 2. Restriction at foramen

NEONATAL PERIOD ovale

3. Obstructed total
anomalous pulmonary
venous connection
2. Restriction at Foramen Ovale

Relieve pulmonary congestion

Hypoplastic left heart syndrome
Mitral stenosis/atresia
Relief right sided congestion and maintain systemic blood flow
Pulmonary atresia with intact septum
Tricuspid atresia
Total anomalous pulmonary venous connection
 1. Cyanosis

2. Circulatory
collapse / Shock
LIFE THREATENING
PRESENTATION
3. Heart failure

4. Arrhythmia
Circulation is dependent on the patency of the ductus arteriosus for
survival

Ductus arteriosus close/constrict

Systemic hypoperfusion
Critical Coarctation

Critical Aortic Stenosis

Aortic Arch Interruption

Hipoplastic Left Heart Syndrome

Diagnosis
HF or shock in first 2 weeks of life
Physical examination
Progressive dyspneu
Poor perfusion
Cold and clammy mottled skin
Reduced peripheral pulses all limbs critical aortic stenosis,
hypoplastic left heart syndrome (HLHS)
Reduced femoral pulses coarctation/ interruption
Mild cyanosis HLHS (single ventricle physiology)
Single second heart sound
PRACTICAL RECOGNITON OF SHOCK SIGN IN NEONATE

1. Capillary refill time (CRT)

The accepted upper normal time is < 3 sec
CRT is more reliable when measured on the chest but not the
forehead, palm, or heel
A positive predictive value for systemic blood flow was only when
the refill time was over 6 second
2. Central peripheral temperature difference (CPTd)
Normal CPTd < 1oC during the 1st postnatal day in ELBW infants
In a thermoneutral environment (secondary to poor peripheral
perfusion in shock) peripheral vasoconstriction will skin
temperature and lead to an CPTd
CPTd depends on body temperature, enviromental temperature
and the use of vasoactive drug
No relation between CPTd and systemic blood flow or systemic
vascular resistance
3. Blood presure (BP)
Hypotension may be a relatively late sign
Mean BP < 30 mmHg with assumption that cerebral blood flow
become pressure dependent at a MAP around 30 mmHg
BP is not linearly related to systemic blood flow
The consequence of using BP to diagnose low systemic blood flow
many patients will be undertreated or overtreated risk of
adverse effects and iatrogenic damage
4. Heart Rate
Cardiac output (COP) = stroke volume (SV) X heart rate (HR)
COP dependent on HR because SV is at fixed level in neonates
HR can be influenced by many factor such as pain, temperature,
stress etc
Single HR value poorly reflects systemic perfusion
5. Urine output (UOP)
Urine output is in shock because of renal perfusion
a poor marker of circulatory failure in the absence of a direct
relationship with systemic blood flow
Normally, UOP changes during the 1st day of life with an initial
phase of low urine production (first 24 h), followed by a period of
transient polyuria (2nd and 3rd days of life), after which diuresis
stabilizes and depends on fluid intake difficult to differentiate
from oliguria due to impared renal perfusion
6. Color
To evaluate the adequacy of peripheral perfusion and/or
oxygenation
It is influenced by many factor such as oxygenation, Hb
concentration, skin temperature, skin thickness, peripheral
perfusion, race, and ambient temperature and light
Very subjective
 NO SINGLE SPESIFIC SIGN OF SHOCK

Combination of different variables improve the

predictive value for the detection of neonatal
circulatory failure/ shock
 Electrocardiography
Right ventricular hypertrophy
LVH with strain (critical AS)
Poor/absence of left ventricle
forces (HLHS)
Chest X-ray
Cardiomegaly
Pulmonary venous hypertension
Increased pulmonary blood flow
(HLHS)
CXR in critical CoA
 Echocardiography establish diagnosis, severity, and hemodynamic
effect

Neonate with severe aortic stenosis

Neonate with severe CoA
Initial Management
Treatment of shock
Stable vascular access, and airway also breathing
Correction of metabolic acidosis
Start prostaglandin E1 infusion initial dose of PGE1 is 0.05
mcg/kg/minute continuous intravenous infusion; the dose may be
increased to 0.1 mcg/kg/minute if desired effect is not obtained
within ten minutes
 IMPORTANCE CAUTION !
Cardiogenic shock should be suspected in any infant or child
with
a history of heart disease
An abnormal cardiac examination
And/or worsening clinical condition despite fluid
resuscitation
 UNTIL A
DEFINITIVE
PROCEDURE
IS PLANNED

Maintain a balance Avoid any

between systemic maneuver that
and pulmonary reduce pulmonary
blood flow vascular resistence

Injudicious use of
inotropes hyperventilation
increase SVR
1. Critical aortic stenosis
Ballon dilatation of aortic valve
2. Severe coarctation of aorta
Surgery
Patient with additional problem (sepsis, multiorgan dysfunction)
ballon dilatation
3. Arch interruption
surgery
4. HLHS
Staged surgery for norwood operation and cardiac transplantation
OVERVIEW
Central cyanosis :
a bluish purple discoloration of tissue (nail bed,tongue, mucous membran)
when systemic arterial concentration of deoxygenated hemoglobin in the
blood > 5 g/dl (3,1 mmol/L) correspond to an oxygen saturation < 85%
Clinical detection depends on % arterial blood that is desaturated and Hb
Concentration
Cyanosis severe hypoxemia, metabolic acidosis, and death
The rapidity of progression of disease is partly dependent on how soon and
severely the pulmonary blood flow is compromised with ductal closure
EFFECT OF HYPOXEMIA IN DUCT-DEPENDENT CYANOTIC CHD
 RV hypertrophy
1. Increased
pulmonary blood
flow Left or combined
hypertrophy

Cyanotic
RV hypertrophy

2. Decreased
pulmonary blood LV hypertrophy
flow

Left or combined
hypertrophy
 RV hypertrophy
1. Increased
pulmonary blood
flow Left or combined
hypertrophy

Cyanotic
RV hypertrophy

2. Decreased
pulmonary blood LV hypertrophy
flow

Left or combined
hypertrophy
1. INCREASED PULMONARY BLOOD FLOW
RV hypertrophy

Transposition of Great Arteries

Total Anomalous Pulmonary Venous Connection
Hypoplastic Left Heart Syndrome

Left or combined hypertrophy

Persistent truncus arteriosus

TGA with VSD
Single ventricle
 RV hypertrophy
1. Increased
pulmonary blood
flow Left or combined
hypertrophy

Cyanotic
RV hypertrophy

2. Decreased
pulmonary blood LV hypertrophy
flow

Left or combined
hypertrophy
2. DECREASED PULMONARY BLOOD FLOW
RV hypertrophy

Tetralogy of Fallot
Ebstein Anomaly

LV hypertrophy

Tricuspid atresia
Pulmonary atresia with hypoplastic right ventricle

Left or combined hypertrophy

Persistent truncus arteriosus with hypoplastic pulmonary artery

TGA with pulmonary stenosis
Single ventricle with pulmonary stenosis
Diagnosis
Chest X-ray
Absence of cardiomegaly
except valvar regurgitation in
dysplastic tricuspid valve and
ebstein anomaly
Dark lung field
Absence of main pulmonary
artery segment
Infant with pulmonary atresia, large ventricular septal defect
and duct dependent pulmonary blood flow. X-ray showed no
cardiomegaly, concavity at the site of main pulmonary artery
segment pulmonary bay (arrow) and decreased pulmonary
blood flow
 Tetralogy of Fallot : Coeur en sabot (boot-shaped heart)
secondary to uplifting of the cardiac apex from RVH and
the absence of a normal main pulmonary artery segment

Transposition of Great Arteries :

Egg on side and Narrow
mediastinum
 TAPVR- Snowman Appearance

1 month old baby, severely cyanosed with Ebsteins anomaly of

tricuspid valve, severe tricuspid regurgitation, functional
pulmonary atresia, atrial septal defect with right to left shunt and
PDA. X-ray showed moderate cardiomegaly, and decreased
pulmonary blood flow
Truncus Arteriosus
NEWBORN PULSE OXIMETRY SCREENING FOR CRITICAL CONGENITAL HEART DISEASE
 HYPEROXIC TEST

Helps to differentiate between cardiac and non

cardiac cause of cyanosis

Initial ABG on Right Arm

100% O2 for 10 20 min
Repeat ABG at Right Arm
INTERPRETATION OF HYPEROXIA TEST
 FAILURE OF HYPEROXIC TEST
1. Failure to raise PO2 despite normal heart
- Massive intrapulmonary shunting AVM
- shunt at PDA level in normal Heart PPHN

2. Cyanotic heart disease with large Pulmonary blood

flow may rise the PO2 with O2
- TAPVD, Truncus arteriosus
1. History and physical examination
2. Investigations ABG, CXR, ECG
3. SPO2 and Hyperoxic test

ECHOCARDIOGRAPHY
Echocardiography

Neonate with large VSD, pulmonary atresia with duct dependent pulmonary blood
flow. a. Parasternal short axis view showing large
VSD (arrow), pulmonary atresia (*). b. Suprasternal short axis view showing
tortousduct (arrow) filling pulmonary artery (*)
 If Clinically suggestive of
DUCT DEPENDENT LESIONS
TOF/PA, PAIVS, SEVERE PS, IAA, Critical CoA, PA,TA

START IV PROSTAGLANDIN
( BEFORE ECHO )
REFER PEDIATRIC CARDIOLOGIST
MANAGEMENT

To establish the diagnosis after initial stabilization, resuscitation

with or without ventilator support
Establish airway, ensure oxygenation, and adequate ventilation
Intubation is indicated when baby presents with apnea, shock or
circulatory collapse, severe metabolic acidosis
Identify the co-morbid conditions
Minimize the hypoxemia
MANAGEMENT TO MINIMIZE HIPOXEMIA
PGE1 infusion to Open/Maintain Duct
Starting dose: 0.050.1 mcg/kg/min
Maintenance: 0.0020.05 mcg/kg/min
In doubt start PGE1 till definitive ECHO diagnosis
Increase the fraction of inspired oxygen (FiO2) once ductal patency is
maintained optimize PCO2 levels between 35 to 45 mm Hg to
achieve the balance between Qp : Qs
Not try to achieve complete abolition of cyanosis Aim for
percutaneous saturation of 75 85%
MANAGEMENT TO MINIMIZE HIPOXEMIA (CONTD)
Intravenous fluid resuscitation, 10 ml/kg isotonic saline or colloid and
then optimization of fluid therapy according to the status
Blood transfusion If hemoglobin is less than 12 gm%
Correct metabolic acidosis deteriorates the oxygen uptake and its
release at tissue level
Baby with transposition physiology severely hypoxemic and PO2
< 20 mm Hg, high PCO2 in the absence of lung causes and severe
metabolic acidosis emergency balloon atrial septostomy and PGE1
infusion
Depends on the etiology :

PDA Stenting PAVSD, PAIVS, PA

Balloon valvuloplasty severe PS, critical AS
BT shunt severe ebsteins, TOF/PS, PA,TA
Corrective surgery TGA, Truncus, TAPVD