Dr. RSK - Cyanotic Congenital Heart Disease

APPROACH TO CYANOTIC
CONGENITAL HEART DISEASE
Dr. R. Suresh Kumar

Head, Department of Pediatric Cardiology
Cyanosis
 Bluish discolouration of skin & mucous

membranes due to increased concentration of
reduced Hb >3g% ABG; >5g%CBG
 Causes— CNS, Musculoskeletal system, Airways,
Alveolar-capillary membrane, Hb AND CVS.
 Central vs peripheral.
 Cardiac vs Respiratory. Peaceful cyanosis is
cardiac
Evaluation of the cyanotic baby
 Is the baby cyanosed? SpO2 will confirm.
 Is the cyanosis central/peripheral? Keep the baby
warm.
 Is cyanosis cardiac?
1)Respiratory distress
2)Response to oxygen
 ABG - low pH, low pO2, Low pCo2
 CxR-heart size & configuration, vascularity
 ECG Axis RV/LV dominance
Cyanotic CHD-classification
 Reduced Pulmonary blood flow

– Tetralogy of Fallot/pulmonary atresia
– TOF-like physiology (complex congenital heart
disease, large VSD PS/PA)
– Critical PS/ pul.atresia intact IVS
– Ebstein’s anomaly
 Low pAO2, oligaemic lungs, neonates respond to

PGE1.
Classification - Cyanotic CHD,
Increased PBF
 Transposition of Great Arteries

 Total Anomalous Pulmonary Venous connection
 Single Ventricle
 Truncus Arteriosus
 DORV
Clinical findings
 CCHD with decreased pulmonary blood flow

– Quiet precordium
– Single S2
– Murmurs
 Ejection systolic murmur at the base
 No murmur
 Continuous murmur of PDA / collaterals
 ECG – RAD, RVH
– LV dominance in tricuspid atresia
– LAD in tricuspid atresia, AVCD with PS, Single
ventricle with PS
Clinical findings - 2
 CCHD with increased pulmonary blood flow
– Mild cyanosis
– Active precordium
– Epigastric pulsations
– Congestive heart failure
– Single S2 (except in TAPVC)
 TGA
– Profound cyanosis
– Single S2
– No murmur
– Normal / mildly increased lung vascularity on CXR
Tetralogy of Fallot/P.Atresia
 Large VSD/ RVOT obstruction/atresia

 Cyanosis depends on degree of Obstruction,
PDA/collaterals
 Neonates may need PGE1 to maintain saturation –
needs urgent BT shunt.
 Progressive cyanosis/spells
 Total correction-around 1 yr.
Cyanotic Spells
 Rapid decrease in systemic oxygen saturation with

hyperventilation  Convulsions, coma, death.
 Age : 6mon – 6 yrs.
 Cyanosis on crying is not a spell.
 Crying/exercise may trigger spells.
 Drop in SVR/ Inf.spasm  R to L shunt 
acidosis  hyperventilation :vicious cycle
Cyanotic Spell- Treatment
 Knee chest position , Oxygen
 Morphine 0.1 mg/kg iv (sedation,resp dep.);Soda
bicarb iv 1-2 ml/kg
 Increase SVR : Propranolol 0.1 mg/kg iv;
Methoxamine 0.1 mg/kg iv; Phenylephrine 1-
5mic.g/kg /min iv infusion.
 Ventilation. Paralyse
 Emergency surgery
 Propranolol for prophylaxis
Complex CHD,TOF-like physiology
 DORV PS/PA Presents like TOF. Needs

neonatal BT shunt, if ductus-dependent. Total
correction around 1 year, may need a homograft.
 Single Ventricle PS/PA. Presents like TOF. But 2-
ventricle Repair not possible.Neonatal BT shunt, if
needed. SVC to RPA anastomosis (Bidirectional
Glenn) at 3-6months. IVC to PA connection
(Lateral tunnel or Extra cardiac Fontan) at 2-5 yrs.
Tricuspid Atresia
 Pulmonary blood flow depends on ASD, VSD,

Pulmonary stenosis. May be increased / decreased
/ balanced. Cyanosis or heart failure predominant
presentation accordingly.
 LV dominance and left axis in ECG helpful.
 Management in Fontan track.Neonatal BT shunt
(for severe cyanosis) or PA banding (for heart
failure) may be needed.
Critical PS PAIVS
 Cyanosis on day 1. Pulmonary circulation ductus
dependent. RV normal or in a range of hypoplasia.
 If RV is adequate,catheter or surgical pulmonary
valvotomy is the treatment. If RV is small, BT shunt. May
need both.
 Further management may be - Glenn + Pulm. Valvotomy
(1½ ventricle) or staged Fontan, when 2-ventricle repair
not possible.
 Myocardial sinusoids / RV dependent coronary circulation
- adverse prognosis.
Ebstein’s anomaly
 Inferior displacement of septal and inferior tricuspid
leaflets. Thin RV. May have PS.
 Neonatal cyanosis may be severe. High PVRTR  R to
L atrial shunt. Usually transient.
 Course:May be asymptomatic. Cyanosis, Rt heart failure
and SVT may be problems.
 Surgery for severe cyanosis/heart failure.
Ebstein’s anomaly
 History of SVT
 Generally good effort tolerance
 Cardiomegaly
 Multiple heart sounds (split S1, split S2, S3, S4)
 Scratchy systolic murmur at LLSB
 Cardiomegaly with huge RA on CxR.
 ECG-abnormal axis, RAE, polyphasic QRS. May
have WPW syndrome
Cyanotic CHD, increased PBF.
Transposition of GA
 Commonest cyanotic CHD on day 1.
 Aorta from RV, PA from LV. Presentation depends
on Intact IVS, VSD, PS.
 TGA intact IVS. Cyanosis on Day1. No murmur.
CxR- egg on side heart,normal vascularity. Untreated
90% die in first month, if no ASD.
 Management : PGE1 till Balloon Atrial Septostomy.
Arterial Switch Operation in first month. Good long-
term result.
TGA with VSD
 No PS: Cyanosis minimal. CHF in a few
weeks. Needs Arterial Switch operation in 3
months. Rapid progression of PVD, if untreated.
 TGA/VSD/PS: Cyanosis depends on degree of
PS. BT shunt if cyanosis is severe. Rastelli
operation at 3-5 yrs.
TAPVC
 All 4 pulm veins join a common chamber which
eventually reaches RA.
 Supracardiac- common chamber drains to innominate
vein or to SVC.
 Cardiac- drains to coronary sinus or RA
 Infracardiac (Infradiaphragmatic). Descending vertical
vein  portal vein  ductus venosus  IVC.
Obstructed.
TAPVC-2
 Obstructed venous return: Presents with
pulmonary oedema. Infradiaphragmatic on Day1,
supracardiac in a fewdays. Emergency surgery life
saving. Normal life span.
 Unobstructed: Cardiac, some supracardiac. Slow
onset of CHF, minimal cyanosis. Clinical features
of ASD Figure of 8 cardiac silhouette on CxR.
Good surgical results.
Persistent Truncus Arteriosus
 A single arterial trunk leaves the heart above a

VSD. PA arises with a common trunk or as
separate branches.
 CHF in a few weeks of life, mild cyanosis.
Bounding pulses,systolic click and murmur. EDM,
if truncal valve is incompetent. CxR - CE, High
origin of PA branches,increased vascularity.
 Surgical Repair in first month preferred.
Cyanotic CHD in older children
and adults
 Tetralogy of Fallot
– Commonest CCHD with reduced pulmonary blood flow
surviving into adulthood
– Exertional dyspnoea and squatting
– Varying degrees of cyanosis
 Eisenmenger syndrome
– History of recurrent respiratory infections in early
childhood
– Good effort tolerance till late in the nature history
– Mild cyanosis
– Findings of severe pulmonary artery hypertension
Eisenmenger VSD
 Presents in second decade

 Single S2
 Normal heart size on CXR
 Dilated central pulmonary arteries with
peripheral pruning
Eisenmenger PDA
 Presents in second or third decade
 Differential cyanosis and clubbing
 Normally split S2 with loud P2
 Normal heart size on CXR
 Dilated central pulmonary arteries with
peripheral pruning
Eisenmenger ASD
 Presents in fourth or fifth decade

 Cardiomegaly
 Widely split S2
 Atrial fibrillation

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APPROACH TO CYANOTIC

CONGENITAL HEART DISEASE

Dr. R. Suresh Kumar

 Bluish discolouration of skin & mucous

 Reduced Pulmonary blood flow

 Low pAO2, oligaemic lungs, neonates respond to

 Transposition of Great Arteries

 CCHD with decreased pulmonary blood flow

 Large VSD/ RVOT obstruction/atresia

 Rapid decrease in systemic oxygen saturation with

 DORV PS/PA Presents like TOF. Needs

 Pulmonary blood flow depends on ASD, VSD,

 A single arterial trunk leaves the heart above a

 Presents in second decade

 Presents in fourth or fifth decade

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