MANAGEMENT OF CYANOTIC
CONGENITAL HEART DISEASE
Dr. SANDIP GUPTA
• m/c cyanotic lesions of the newborn :
EARLY LIFE-THREATENING PRESENTATION
1.SHOCK 2.CYANOSIS 3.HEART FAILURE
• Shock is managed with initial attention to
advanced life support such as establishing
airway, maintenance of ventilation.
• Vascular acess with volume resuscitation.
• Inotropic support with the goal of improving
cardiac output & tissue perfusion.
• Normal closure of the PDA in the first days of life can
precipitate profound cyanosis in the following scenarios:
• When the PDA is the only mechanism of pulmonary blood
flow, such as in patients with critically obstructive right heart
lesions (eg, critical pulmonary stenosis/atresia,tricuspid
• When the PDA supplies the majority of systemic circulation in
critically obstructive left heart lesions (including hypoplastic
left heart syndrome and critical aortic valve stenosis). With
ductal closure, these patients will present with decreased
• When the PDA provides mixing between parallel pulmonary
and systemic circulations (transposition of the great arteries).
• The neonate who fails a hyperoxia test is likely to
have a PDA dependent systemic or pulmonary
circulation should receive PGE1 till anatomical
diagnosis is established.
• PGE1is started at a dose of 0.05-0.1u/kg/min
• May be given upto 0.4u/kg/min
• When the desired effect achieved may be reduced to
• Apnoea may occur .
Time of onset of symptoms in
CHD (HART FAILURE)
At birth: HLHS, large A-V fistula,
1st wk: TGA, TAPVR
1-4 wk: COA with associated anomalies, critical AS
4-6 wk: endocardial cushion defect
6 wk-4 mth: large VSD, large PDA
Adequate calories - human milk fortifiers,
fat modular products, low osmolarity
- 1st line of drugs
- ↓ pre-load
- Do not improve CO
- Hypokalemia and
Afterload ↓ agents
How to dizitalize the heart ?
1. Baseline ECG & Serum electrolytes
2. Calculate the oral digoxin dosage :
Age Total dizitalizing
Prematures 20 5
Newborns 30 8
< 2yrs 40-50 10-12
> 2yrs 30-40 8-10
Maintenance dose is 25% of the total dig.dose in 2 divided doses
I.V. dose is 75% of the oral dose.
3. Give one half of the TDD immediately ,then 1/4th & then the final 1/4th
at 6- to 8-hr intervals.
4. Start the maintenance dose 12hrs after the final TDD but before this do
• Low cardiac output refractory
to standard therapy
• After open heart surgery
• Adjunct to DA / Dobutamine
• Inotropic,peripheral vesodilatation,
increased renal blood flow-natriuresis
• In higher doses- peripheral vesoconstriction
•Dose is gradually increased
(depends on the type of defect)
Blalock Taussig shunt
Late Complications of CCHD
• Polycythemia & Hyperviscosity syndrome.
• CNS complications.
• Bleeding Disorders.
• Hypoxic Spells and Squatting.
• Depressed Intelligent Quotient.
• Hyperuricemia and Gout.
• Failure to thrive
• Psychosocial adjustment
SUGGESTED STEPS IN THE MANAGEMENT
• Pulse Oximetry.
• ABG : confirm or reject central cyanosis.
• Hyperoxia test : helps separate cardiac causes of cyanosis
• Chest x-ray : may reveal pulmonary causes of cyanosis and
the urgency of the problem. hint at the cardiac defect.
• ECG if cardiac origin of cyanosis is suspected.
ECHO DOPPLER STUDY
Treatment of complications
• Polycythemia& hyperviscosity: symptomatic
pt’s (having headache,dizziness,visual
disturbances) should have phlebotomy .
• Iron replacement.
• Bleeding disorder lead to plt Count&
function, PT &APTT, lower levels of
coagulation factors: plt transfusion,FFP, vit-
k, cryoprecipitate can be used.
CNS Complications: high hematocrit or iron deficient
RBC’S predispose these pt’s to brain abcess, stroke.
Rt to Lt shunt may lead to embolization ,increase
chance of infection.
Gouty arthritis & Hyperuricemia can be managed
with colchicin, probenecid& allopurinol.
FOLLOW UP: Should directed towards care of
underlying heart condition ,symptoms of
hyperviscosity, change in exercise
tolerance,saturation level, prophylaxis of IE,influenza,
In stable pt’s yearly follow up is recommended &
should include vaccination, yearly blood work
function,uricacid.)& regular echo doppler studies .
Peak incidence of 2-4 months
Hyperapnea (Rapid and deep
Irritability and prolonged
Decreased heart murmur
Hypoxic Spell/ Hypercyanotic Spells/Blue Spells
Lower SVR or inc resistance of RVOT can
increase the R-L shunt
Stimulates the respiratory
center to produce hyperapnea
Results in an increase in systemic
In turn, increases R-L shunt
TOF - treatment
• Recognition & treatment of cyanotic spells.
• Ballon dilation of RVOT (used in the past).
• IE prophylaxis.
• Detection of relative Iron deficiency, iron deficient are more
suscetible for CVA
Surgery for TOF
• Shunt operation to increase PBF.
• Many one prefers primary repair.
• Shunts preferred in –
1. Neonates with TOF & PA.
2. Infants with hypoplastic Pulmonary annulus.
3. Children with hypoplastic PA.
4. Medically un-managable hypoxic spells.
5. Infants <2.5kg.
Shunts – various locations
Modified ballock-taussing is the preferred one
Definitive repair :
• Early surgery generally preferred.
• Saturation <75% , occurrence of cyanotic spell considered
indication for surgery.
• Symptomatic with favourable RVOT & PA have repair after3-
• Mildly cyanotic who had shunt surgery may have repair after 1-2
• Patch closure of VSD, RVOT widening, PA vulvotomy(avoiding
palcement of patch)
Defect to permit mixing of 2 circulations- ASD, VSD, PDA.
1. PGE1 – to keep Patent ductus , untill time of surgery.
2. CHF Rx
3. Before surgery, cardiac catheterization and a balloon atrial
septostomy (i.e., the Rashkind procedure) often carried out to
have some flexibility in planning surgery
Palliative: Balloon Artrial Septostomy delay in surgery necessary
Def: Arterial Switch operation
A. Atrial baffle operation – Mustard and Senning operations.
B. Ventricular level(VSD +PS) – Rastelli
C. Gr. Art level – (Physiological + Anatomical Correction)
Jantene oper done usually in 1st 2wks of life.
Tricuspid Atresia – T/t
• PGE IV infusion to maintain patent ductus.
• Most require a palliative shunt before definitive FONTAN
• Blalock-Taussig shunt in infancy
– systemic to pulmonary arterial shunt
– Provide stable blood flow to the lungs
– A gortex tube is sewen between the subclavian artery and
the right pulmonary artery
• BIDERECTIONAL GLENN SHUNT : SVC is connected to the
RPA ( end to side anastomosis )
• IVC continues to be connected to the heart.
• HEMI FONTAN operation :
• Modified fontan operation is the definitive choice usually
done at 1.5-3 yr – directs the whole systemic venous blood
to pulmonary arteries without intervening heart chambers.
Management of PTA
In1st few wks most of infants can be managed with
anticongestive medication;as PVR falls HF worsens & surgery
is indicated in1st few months
• surgical : various modification of Rastelli operation.
• For all types VSD is closed in such a way that LV ejects into
truncus.PA are separated from truncus & continuity is
established betwn PA &RV.
Total Anomalous Pulmonary
• The pulmonary veins drain into the RA or its venous tributaries
rather than the LA
• A interatrial communication (ASD or PFO) is necessary for survival
• Pulmonary venous return reaches the RA
– Systemic and pulmonary venous blood are completely mixed
Common pulmonary vein drains into the SVC
via the left SVC and left innominate vein.
• The common PV drains into the coronary
• The common PV drains into the portal
vein, ductous venosus, hepatic vein, or IVC.
:combination of other
Clinical Signs for Obstructed Veins
• Profound desaturation
• PGE1 administration does not improve oxygenation because
elevated pulmonary pressures in the right side of the heart
(due to obstructed pulmonary outflow) will result in right to
left shunting across an open ductus further decreasing arterial
• Digitalis and diuretics to treat heart failure
• Intubation and inc PEEP for those with severe pulm over load
• Corrective surgery: pulmonary venous confluence is
aanastomosed directly to LA, & ASD is closed
• All infants with PV obstruction should be operated soon after
diagnosis.If surgery cannot be done immediately ECMO may
be required to maintain oxygenation.
• With out obstruction, but with HF not managable , operation
4-6 month age
• RV size? Coronary sinusoids present or not.
• Severe cyanosis/ tachypnea
• S2 single
• No murmur
• Decrease PVM
• RAE, LVH
Pulmonary atresia with intact septum
• Medical :
– PGE1 – Radiofrequency perforation of PV.
– Open pulmonary valvotomy+ palliative Blallock-shunt
– (in the neonatal period).
– If good RV size : Biventricular repair (at 1 year of age).
– If small RV size ± sinusoids + RVDCC :
– BT- shunt in neonatal period
– Bidirectinol Glenn (at 6 months of age).
– Total Cavo Pulmonary Connection (TCPC)
– (at 4 years of age)