Congenital cyanotic heart disease approach

1. Approach to Cyanotic
congenital heart disease
Dr Varsha Atul Shah

2. Incidence of CHD
The incidence of moderate to severe
structural congenital heart disease in live
born infants is 6 to 8 per 1,000 live births.
Data from the New England Regional
Infant Cardiac Program suggest that
approximately 3 per 1,000 live births have
heart disease that results in death or
requires cardiac catheterization or surgery
during the first year of life.

3. Top Five Diagnoses Presenting at Different
Ages(%)
Age on admission: 0-6 d
D-Transposition of great arteries 19
Hypoplastic left ventricle 14
Tetralogy of Fallot 8
Coarctation of aorta 7
Ventricular-septal defect 3
Others 49
Age on admission: 7-13 d
Coarctation of aorta 16
Ventricular septal defect 14
Hypoplastic left ventricle 8
D-Transposition of great arteries 7
Tetralogy of Fallot 7
Others 48
Age on admission: 14-28 d
Ventricular septal defect 16
Coarctation of aorta 12
Tetralogy of Fallot 7
D-Transposition of great arteries 7
Patent ductus arteriosus 5
Others 53

4. MAJOR CCHD CATEGORIES AND THEIR
OCCURRENCE IN SINGAPORE

5. CYANOSIS –DEFINITION AND DIAGNOSTIC
DIFFICULTIES
PRESENCE OF > 3g/dl of Deoxy Hb which
correlates with 80-85% Spo2.
Can be missed when mild, in dark races
and anemia due to decreased deoxy Hb
Can be misdiagnosed as CCHD in
acrocyanosis, non cardiac causes of
cyanosis like pulmonary causes, CNS
causes and Cyanosis with normal Po2.

6. Differential Diagnosis of Cyanosis in
the Neonate
Primary cardiac lesions
Decreased pulmonary blood flow, intracardiac right-to-left shunt
Critical pulmonary stenosis
Tricuspid atresia
Pulmonary atresia/intact ventricular septum
Tetralogy of Fallot
Ebstein anomaly
Total anomalous pulmonary venous connection with obstruction
Normal or increased pulmonary blood flow, intracardiac mixing
Hypoplastic left heart syndrome
Transposition of the great arteries
Truncus arteriosus
Complete common atrioventricular canal
Total anomalous pulmonary venous connection without obstruction
Other single-ventricle complexes

7. Pulmonary lesions (intrapulmonary right-to-left shunt)
Primary parenchymal lung disease
Aspiration syndromes (e.g., meconium and blood)
Respiratory distress syndrome
Pneumonia
Airway obstruction
Choanal stenosis or atresia
Pierre Robin syndrome
Tracheal stenosis
Pulmonary sling
Absent pulmonary valve syndrome
Extrinsic compression of the lungs
Pneumothorax
Pulmonary interstitial or lobar emphysema
Chylothorax or other pleural effusions
Congenital diaphragmatic hernia
Thoracic dystrophies or dysplasia

8. Other causes
Hypoventilation
Central nervous system lesions
Neuromuscular diseases
Sedation
Sepsis
Pulmonary arteriovenous malformations
Persistent pulmonary hypertension
Cyanosis with normal PO
2
Methemoglobinemia
Polycythemia

9. Hyperoxia Test
Most sensitive and specific tool for
evaluation of a neonate with suspected
CHD especially in the absence of ECHO.
Helps to differentiate the cardiac and non
cardiac causes of cyanosis
PGE1 can be initiated based on the findings
of this test.
Initial measurement in room air then after
10 min of 100% o2 arterial or TCOM Po2 is
measured.

10. Hyperoxia test contd…
Pulse oximetry not reliable
Both pre and post ductal sites used
Differential cyanosis can aid in diagnosis
>250mmhg-no structural cyanotic HD
< 100 mmHg –intracardiac shunting-
CCHD
100-250 mmHg-intracardiac mixing
lesions
< 100 mmHg most likely duct dependent
lesion so PGE1 can be started until
anatomic lesion defined

11. History and physical examination
Onset of cyanosis
Hypoxic spells, exercise
intolerance, squatting, frequent chest
infections, CHF, Failure to thrive
Cyanosis, clubbing, pulse, Four limb
BP, growth, dysmorphology
Cardiovisceral situs
Palpation and auscultation

12. Salient clinical findings of conditions with
decreased PBF
TOF: Cyanosis proportional to
RVOT obstruction
Cyanotic spells and its
management
RV apex, parasternal heave
, Single S2, Ejection systolic
murmur at Left upper sternal
edge
TOF with PA: Single S2 but soft
murmur sometimes continuous
from the MAPCAS. Occasionally
CCF

13. Tricuspid atresia: cyanosis, LV
impulse, S2 single, Holosystolic
murmur along left sternal edge
TGA with VSD and PS or DORV
with PS-TOF picture
Ebstein’s-depends on degree of
displacement of Tricuspid
Valve, can be mild till teenage
or severe with cyanosis in
neonate.
WPW syndrome is an
association, multiple clicks, holo
systolic TR murmur, gallop.

14. Salient clinical findings of conditions with
Increased PBF
D-TGA with IVS-CYANOSIS and
tachypnea, S2 single and loud, soft or
absent MURMUR.
D-TGA with VSD-presents with cardiac
failure, subtle cyanosis and holo
systolic VSD murmur.
L-TGA-physiologically corrected so can
be asymptomatic but may have
associated lesions like
VSD, EBSTEIN’S, PS, WPW syndrome
etc.

15. DORV WITHOUT PS
DORV+TGA, TAUSSIG
BING TYPE
DORV without PS is like VSD
DORV+ TGA-Sub arterial
VSD, Cardiac Failure, Loud
ESM, left sided obstructive
lesions are common

16. Salient clinical findings of conditions with
increased PBF
TAPVR-L-R
shunt with
Cardiac failure
features, varia
ble cyanosis
depending on
the
obstruction, m
ay need
emergency
surgery, no
response to
PGE1.

17. Truncus
arteriosus-in
neonates
murmur and
mild
cyanosis, later
develops
Cardiac
failure, valve
insufficiency, si
ngle S2, Loud
ESM with thrill
and MDM due
to mitral flow
murmur

18. SINGLE VENTRICLE
HLHS
Single ventricle-with PS-
TOF like, without PS-TGA
with VSD like
Hypoplastic left heart
syndrome-cyanosis and poor
perfusion and cardiac failure
with non descript murmur.

19. Tetralogy of Fallot

20. TAPVR-Snowman Appearance

21. Truncus Arteriosus

22. Tricuspid Atresia

23. Ebstein’s anomaly

24. Dextrocardia with situs inversus

25. D-TGA-EGG ON SIDE APPEARANCE

26. L-TGA

27. ECG IN CCHD
DETERMINATION OF VENTRICULAR
HYPERTROPHY AND QRS AXIS DEVIATION
AIDS IN DIAGNOSIS

30. ECG IN CCHD
CCHD RAD LAD RAE LAE RVH LVH RBBB
TOF + +
+post
repair
PA+IVS + + +
TR.ATRES
IA
+ + +
EBSTEIN’S + +
D-TGA
+VSD
+ + + +
TRUNCUS + +
TAPVR + +

32. PULMONARY ATRESIA WITH IVS

33. TRICUSPID ATRESIA

34. Ebstein’s with WPW Syndrome

35. RVH IN D-TGA

36. SHUNT SURGERIES

37. NORWOOD PROCEDURE

40. JANTENE’S ARTERIAL SWITCH

44. Medical Management
PGE1: drug used to maintain patency of ductus
arteriosus in duct dependent conditions for
systemic and pulmonary blood flow
0.01-0.4 mic./kg/min titrated according to
response. Lower doses effective and central line
is preferred not mandatory. Dilute with NS OR
5%D
Apnea, hypotension, pyrexia, flushing, diarrhea,
edema, gastric outlet obstruction, inhibition of
platelet aggregation on long term use.

45. Antifailure medications used to treat heart
failure in mixing lesions.
Frusemide , spironolactone, digoxin, and
captopril can be used.
Prognosis after Surgery
Tetralogy of Fallot: Surgical risk<5 percent and
performed as early as 3 months of age usually
between 4-6 months, post op need to look for
RV failure, PR, Conduction blocks, residual
VSD.
TOF WITH PA: BT shunt or direct complete
repair: RV-PA conduit +VSD CLOSURE
For MAPCAs -unifocalisation

46. Prognosis contd…
Tricuspid atresia-5 year survival 80% and
10 year survival 70% post op.
HLHS:25% MORTALITY stage 1, 5% for
Stage 2, 15-20% for Stage 3. Overall
survival after stage 3 55% at 4 years.
Ebstein’s : 5-20% mortality in valve repair
and ASD closure
Truncus Arteriosus: 10-30% surgical
mortality
DORV: Rastelli: 5-15% mortality

47. References
Nelson’s TB of Pediatrics 18
th
edn.
Cloherty manual of neonatal care, 6
th
edn,
Park, Paediatric cardiology for
practitioners, 5
th
edn
Nada’s text book of Paediatric cardiology,
2
nd
edn
A practical approach to Cyanotic
congenital heart disease, Yip WCL, Tay
JSH: Singapore medical journal, 1983

48. Thank you