Cyanotic Congenital Heart

Defects:
Practical Approach for Pediatrician

Rizky Adriansyah
Causes of Central Cyanotic Neonate
1. CNS Depression : Perinatal asphyxia
Heavy maternal sedation
Intrauterine fetal distress

2. Pulmonary Disease : Respiratory distress syndrome

Pneumothorax or pleural effusion
Hernia Diaphragmatica
PPHN

3. Heart Disease : Cyanotic CHD

4. Hematology Disease : Hemoglobinopathy

Approach to a Central Cyanotic Neonate
• Clinical Findings : Respiratory problems ?
Peripheral, central or differential cyanosis ?
Cardiac murmur ?

• Blood Gas Analysis & Hyperoxia Test

• Chest X-ray : Cardiomegaly ?

Abnormal cardiac shillouette ?
Pulmonary vasculary markings ?
Pulmonary causes of cyanosis ?

• Other laboratory findings

Step by step
Cyanosis

1 Respiratory
distress (-)
Respiratory
distress (+)

2 Peripheral
Cyanosis
Central
Cyanosis
Differential
Cyanosis
Obstruction
(-)
Obstruction
(+)

Hyperoxia Hyperoxia ENT

test test evaluation

3 PaO2 < 100

PaO2 100 –
PaO2 < 100
PaO2 100 –
PaO2 > 150
150 150

4
Heart
Heart evaluation Heart Likely heart PPHN, Respiratory
evaluation evaluation disease Heart? evaluation
PPHN
Neonatal Heart Evaluation
• History Taking & Physical Examination

General Practitioner
• Chest X-ray
Pediatrician
• ECG

• Echocardiography Pediatrician +
Screening basic echocardiography training
Diagnosis
Pediatric Cardiologist
• Cardiac Catheterization
Selected Aspects of History Taking
• Gestational and Natal History
Infections (TORCH, HIV)
Medication, Alcohol, and Smooking
Maternal Conditions (Diabetes, SLE, parent with CHD)
Birth Weight
• Postnatal History
Cyanosis Characteristic
Respiratory Problems
• Family History
Hereditary Diseases
Chromosomal Abnormalities
Physical Examination for Heart
Evaluation
• Inspection General Appearance
Major Congenital Abnormalities (syndromic)
Colour (peripheral, central, differensial cyanosis ?)
Respiratory Rate, Dyspnea & Retraction

• AuscultationHeart Rate,Heart Sounds, & Heart Murmurs

• PalpationPeripheral Pulses

• Pulse Oxymetry
Peripheral Cyanosis vs. Central Cyanosis
Heart Auscultation
Rate : bpm (normal, tachycardia, or bradycardia)
regularity (reguler or irreguler)  confirm ECG

Heart Sounds
Physiologic : S1 & S2
Patologic : S3 & S4

Heart Murmurs
Grade Physiologic/innocent
Patologic: grade > II/6 or thrill
Timing Systolic (early/mid/end), Diastolic
Holosystolic, Continous
Punctum maximum area
Radiation, etc
Pulse Oxymetry for Screening CCHD
Diagnostic Physical Pulse
Test Examination Oxymetry PE + PO

Sensitivity 62.5% 62.07% 82.76%

Specificity 98.07% 99.82% 97.88%

PPV 1.35% 20.69% 2.92%

NPV 99.98% 99.97% 99.99%

BMJ. 2008;337:a3037
Algorithm for CCHD using Pulse Oxymetry
2009 AAP/AHA Scientific Statement
Conclusions
• CCHD is not detected in some newborns until after their hospital discharge, which
results in significant morbidity and occasional mortality

• Furthermore, routine pulse oxymetry performed on asymptomatic newborns after 24

hours of life, but before hospital discharge, may detect CCHD

• Routine pulse oxymetry performed after 24 hours in hospital that have on-site pediatric
cardiovascular services incurs very low cost and risk of harm

• Future studies in larger populations and across a broad range of newborn delivery
systems are needed to determine whether this practice should become standard of care
in the routine assessment of the neonate
CHD lesions divided by likelihood of being detected by pulse oximetry

Primary Targets Secondary Targets Possibly Screenable Not Screenable

Transposition of the Double Outlet Right Pulmonary Stenosis (PS) Left to Right Shunt (ASD,
Great Arteries (TGA) Ventricle (DORV) VSD, PDA)
Interupted Aortic Arch Aortic Stenosis without
Tetralogy of Fallot (TOF) Aortic Stenosis with PDA
(IAA) PDA
Tricuspid Atresia Coarctation Aorta with Coarctation Aorta without
PDA PDA
Single Ventricle Complete Atrioventricular
Truncus Arteriosus
Physiology Septal Defect (CAVSD)
Total Anomalous
Eibstein Anomaly with Eibstein Anomaly without
Pulmonary Venous Right to Left Shunt Right to Left Shunt
Drainage (TAPVD)
Pulmonary Atresia
Hypoplastic Left Heart
Syndrome (HLHS)
Heart Interpretation from Chest X-ray
• Heart Size and Silhouette
Normal, Cardiomegaly, or Abnormal Silhouette

• Pulmonary Vascular Markings

Normal, Increased, or Decreased

• Evaluation of Cardiac Chambers and Great Arteries

Normal, chamber enlargement, prominent PA segment, Ao dilatation

• Others
Location of the liver and stomach gas bubble (heterotaxia ?)
Normal Chest X-ray vs. Cardiomegaly

Normal CT Ratio < 0.6 Formula : (a+b)/c

Pulmonary Vascular Markings
• Normal or increased
Transposition of the great Arteries (TGA)
Total Anomalous Pulmonary Venous Drainage (TAPVD)
Hypoplastic Left Heart Syndrome (HLHS)
DORV without pulmonal stenosis

• Decreased
Tetralogy of Fallot (TOF)
Tricuspid Atresia
Pulmonary Atresia
DORV with pulmonal stenosis
Abnormal Heart Shillouette
• Boot shape : TOF
• Egg shape : TGA
• Eight shape / snowman : TAPVD
• Box shape : Eibstein Anomaly
Heterotaxia (Asplenic or Polisplenic
Syndrome)
Duct Dependent Lesion
Classification
• Duct Dependent pulmonary circulation
Tetralogy of Fallot with severe PS or PA
Pulmonary Atresia
Tricuspid Atresia

• Duct Dependent systemic circulation

Hypoplastic Left Heart Syndrome (HLHS)
Mitral Atresia

• Duct Dependent mixing circulation

Transposition of the Great Arteries (TGA)
Early management of Cyanotic CHD
• Early Recognition

• Categorizing into type & severity

• Timely initiation of proper medical treatment

• Timely referral for interventional/surgical procedure

Early management of Cyanotic CHD :
TOF
• Early Recognition : excelent physical growth, accept SaO2 > 70% room air, CHF is rare, prevent dehidration (no
furosemid !), prevent anemia, and prevent endocarditis.

• Categorizing into type & severity : confirm by echocardiography, to evaluate of pulmonal stenotic gradation &
associated lesions.

• Timely initiation of proper medical treatment :

if cyanotic spell (oxygen therapy, knee chest position, propranolol inj., morphin or sedation iv, correction of
metabolic acidosis, referral to PICU
if TOF with severe PS or PA (< 1 month) : Prostaglandin infusion therapy

• Timely referral for interventional/surgical procedure

Palliative procedure (infant period) : PDA stenting, RVOT stenting, or BT Shunt
Definitive procedure (> 6 months or 1 – 2 years) : total correction
Early management of Cyanotic CHD :
TGA-IVS and other duct dependent lesions
• Early Recognition : severe acute cyanosis (SaO2 < 70% room air) in the first week of life. The PFO and
PDA provides the needed mixing circulation. Avoid infection. Avoid fluid overload. Aggresive correction
of metabolic acidosis.

• Categorizing into type & severity : confirm by echocardiography. Evaluate of PFO and PDA.

• Timely initiation of proper medical treatment :

If CHF sign or cardiogenic shock : dobutamin inj and other inotropics
Maintance SaO2 > 75% : Prostaglandin infusion therapy (start 10 ng/kg/minute)

• Timely referral for interventional/surgical procedure

Palliative procedure (< 14 days) : ballon atrial septostomy (BAS), if no ASD
Definitive procedure (1 – 2 month) : arterial switch operation (ASO).
Thank You & Any Questions ?