1. MANAGEMENT OF CYANOTIC
CONGENITAL HEART DISEASE
Dr. SANDIP GUPTA
PGT, pediatrics

2. 5 “T’s”
• m/c cyanotic lesions of the newborn :
–TOF
–TGA
–TA
–TAPVC
–Tricuspid Atresia

3. 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.

4. CYANOSIS
• 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
atresia.)
• 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
peripheral perfusion.
• When the PDA provides mixing between parallel pulmonary
and systemic circulations (transposition of the great arteries).

5. PGE1
• 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
0.025u/kg/min.
• Apnoea may occur .

6. Time of onset of symptoms in
CHD (HART FAILURE)
 At birth: HLHS, large A-V fistula,
pulmonary atresia
 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

7. General measures:
Propped up
Oxygen
Adequate calories - human milk fortifiers,
fat modular products, low osmolarity
products
Salt restriction
Bed rest

8. Medical management
Diuretics
- 1st line of drugs
- ↓ pre-load
- Do not improve CO
or myocardial
contractility
- Hypokalemia and
hypochloremic
alkalosis
Inotropic agents
-Digoxin
-Dobutamine
-Dopamine
-Amrinone
/milrinone
Afterload ↓ agents
Dilators:
Arteriolar-
Veno-
Mixed-

9. How to dizitalize the heart ?
1. Baseline ECG & Serum electrolytes
2. Calculate the oral digoxin dosage :
Age Total dizitalizing
dose(μg/kg)
Maintenance
dose(μg/kg/D)
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
ECG

10. Other ionotropes:
Phosphodiesterase
inhibitors:
Milrinone/amrinone
• Low cardiac output refractory
to standard therapy
• After open heart surgery
• Adjunct to DA / Dobutamine
• S/E-thrombocytopenia
Adrenergic agents:
Dopamine
• Inotropic,peripheral vesodilatation,
increased renal blood flow-natriuresis
• 5mcg/kg/min
• In higher doses- peripheral vesoconstriction
Dobutamine
•2.5-40mcg/kg/min
•Dose is gradually increased
Surgery:
(depends on the type of defect)
 Blalock Taussig shunt
 Balloon septoplasty
 Mustard Senning
 Jatene’s switch

11. Late Complications of CCHD
• Polycythemia & Hyperviscosity syndrome.
• Clubbing.
• CNS complications.
• Bleeding Disorders.
• Hypoxic Spells and Squatting.
• Depressed Intelligent Quotient.
• Hyperuricemia and Gout.
• Failure to thrive
• Psychosocial adjustment

12. SUGGESTED STEPS IN THE MANAGEMENT
• Pulse Oximetry.
• ABG : confirm or reject central cyanosis.
• Hyperoxia test : helps separate cardiac causes of cyanosis
from pulmonary.
• 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

13. 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.

14. 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,
pneumococcus infection.
In stable pt’s yearly follow up is recommended &
should include vaccination, yearly blood work
up(CBC,ferritin,clotting profile,renal
function,uricacid.)& regular echo doppler studies .

15. Peak incidence of 2-4 months
Characterized by:
Hyperapnea (Rapid and deep
respirations)
Irritability and prolonged
crying
Inc cyanosis
Decreased heart murmur
Hypoxic Spell/ Hypercyanotic Spells/Blue Spells
(“TET Spell”)
Pathophysiology
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
venous return
In turn, increases R-L shunt
through VSD.

16. Management: Cyanotic Spell:
• Moist O2 inhalation
• Knee Chest
• Morphin Sulphate 0.1 -0.2mg/kg IM/IV
• I.V. Propranolol (0.5 mg/kg)
• NaHCo3 : 1 mEq / kg I.V.
• Phenylephrine 0.02 mg/kg
• Ketamine 1-3 mg/kg I.V.
• Gen. Anaesthesia
• Emergency Surgical intervention

17. TOF
1. Non-restrictive VSD
2. RVOT Obstruction
3. RVH
4. Overriding aorta

18. TOF - treatment
MEDICAL :
• 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

19. 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.

20. Shunts – various locations
Modified ballock-taussing is the preferred one

21. Definitive repair :
• Early surgery generally preferred.
• Saturation <75% , occurrence of cyanotic spell considered
indication for surgery.
• Symptomatic with favourable RVOT & PA have repair after3-
4months.
• Mildly cyanotic who had shunt surgery may have repair after 1-2
yrs.
• Patch closure of VSD, RVOT widening, PA vulvotomy(avoiding
palcement of patch)

22. TGA
Defect to permit mixing of 2 circulations- ASD, VSD, PDA.

23. Medical :
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
Surgical :
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
operation(intraventricular tunnel)
C. Gr. Art level – (Physiological + Anatomical Correction)
Jantene oper done usually in 1st 2wks of life.

25. Tricuspid Atresia

26. Tricuspid Atresia – T/t
• PGE IV infusion to maintain patent ductus.
• Most require a palliative shunt before definitive FONTAN
OPERATION.
• 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

27. • 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.

28. Fontan Procedure
• Redirects IVC to lungs

29. Truncus Arteriosus

30. 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.

31. Total Anomalous Pulmonary
Venous Return
• 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

32. 4 Types
1. Supracardiac
Common pulmonary vein drains into the SVC
via the left SVC and left innominate vein.

33. 2. Cardiac
• The common PV drains into the coronary
sinus

34. 3. Infracardiac
• The common PV drains into the portal
vein, ductous venosus, hepatic vein, or IVC.
4.Mixedtype
:combination of other
types

35. Clinical Signs for Obstructed Veins
• Profound desaturation
• Acidosis
• 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
saturation.

36. Treatment
• 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

37. Pulmonary Atresia/VSD Management
• Medical: ? PDA dependent
– PGE1
• Surgical:
– Palliative=shunt
– Definitive= staged repair (Unifocalization)
– + RV-PA conduit

38. • RV size? Coronary sinusoids present or not.
• PE:
• Severe cyanosis/ tachypnea
• S2 single
• No murmur
• CXR:
• RAE
• Decrease PVM
• ECG:
• RAE, LVH

39. Pulmonary atresia with intact septum
• Medical :
– PGE1 – Radiofrequency perforation of PV.
• Surgical:
– 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)

40. Ebstein Anomaly

41. Ebstein management
• Medical :
– Mild : nothing
– CHF : Diuretics
– Severe cyanosis : PGE1
– SVT : adenosine, b-blockers are 1st line preventive drugs.
• Surgical :
– Palliative shunt
– Definitive repair :
– If good RV size : biventricular repair ( TV repair )
a) Danielson repair
b) Carpentier repair
– If small RV----- Univentricular repair strategy

43. INDICATION of operation

44. Single Ventricle
• ↑Pulm. Blood flow:
No PS
Lt-→ Rt shunt
Like large VSD
• ↓Pulm. Blood flow:
PS
Rt-→Lt shunt
Like Fallot

45. Single Ventricle Management
• With ↓pulm. Blood flow:
– Medical: PGE1
– Surgical :
–
- Blalock shunt
- Bidirectional Glenn
- Total Cavo Pulmonary Connection
(TCPC)

46. Double Outlet RV

47. Double Outlet RV
• ? position of VSD ?PS
• Sub – aortic VSD+ no PS: like large VSD
CHF
• Subaortic VSD+PS:
like Fallot
• Sub-pulmonic VSD (Tassig-Bing):
like TGA
• Doubly commited VSD

48. Thank You