A case presentation and relevant discussion on Co-arctation of aorta

1. Admission rounds
19-04-2011
DR SAPTHARISHI LG

2. INDEX CASE
B/o M
 28 day old neonate
Admitted in NICU
DOA – 6/4/2011 (Day 15)

3. Demographic details
Term / 2.5 kg / AFD/ M ch
Resident of Panchkula
Admitted to PGI NICU on Day 15 OL
Admitted outside on Day 9 of life with chief
complaints of:
 Rapid breathing x 2 days
 Poor feeding x 2 days
 Grunting x 1 day

4. History of Presenting illness
ANTENATAL HISTORY
 Born to G 2 mother ; One previous spontaneous abortion
 Unbooked but adequately supervised during the current
pregnancy
 I & II trimesters apparently uncomplicated
 III Trimester – developed fever and cough – dry cough lasting
20-25 days- received some oral antibiotics – did not require
hospitalization
 Spontaneous onset of labor; Normal vaginal delivery at term
 Cried immediately after birth; No resuscitation required
 Discharged on Post natal day 1

5. Course in GMCH 32 Hospital
Duration of stay – 24 hrs
Day of life – Day 10
ECHO evaluation
VSD ( 5 mm)
ASD (7 mm)
Manual IPPR
Intubated i/v/o
Gasping efforts
Dopa Dobuta
Shock
D-9 D-10

6. Course in Children’s hospital, PKL
Duration of stay – 4 days
Day of life – Day 11-15
Vanco/ Mero
LONS
Phototherapy
NNJ
HFOV (MAP – 18/ Ampl-60) Right
SIMV Pneumo
Resp thorax
Hepatomegaly Rt ICDT
Sacral edema
Shock hypotension Dobuta/ lasix 2 mg/kg/day
D 11 D 12 D 13 D 14

7. At presentation to PGIMER NICU
APPEARANCE WORK OF
BREATHING
Abnormal
Intubated /IPPR
UNSTABLE
LIFE
THREATENING
CIRCULATION
Normal

8. On Examination
Pulses – weak in bilateral lower limbs
Four limb BP:
 RUL - 81/41 (63)
 LUL – 69/39 (52)
 LLL – 70/37 (50)
 RLL – 73/34 (47)
Four Limb Saturation – No significant difference
No obvious congenital malformation
Rt sided ICDT in situ

9. On further Examination
p/A –
 soft, no distension
 LIVER : 3 cm under RCM, smooth surface, regular margin
 No splenomegaly ; No free fluid
CVS
 Precordial bulge, thrill +
 Apex beat normal position
 Grade III systolic murmur over precordium
RS
 Air entry reduced on Rt side
 Bilateral crepts
 Sp O 2- 99 %

10. DATABASE
Term / 2.5 kg /AFD / Mch with apparently
uneventful antenatal/perinatal period presenting on
DAY 10 of life with Respiratory failure and shock (?
Cardiogenic)

11. DIAGNOSTIC POSSIBILITIES
Congenital Heart Disease – Duct dependent
Systemic Circulation. Eg: Left sided obstructive
lesions
Acyanotic Congenital Heart Disease with Large LR
shunts
Late Onset Neonatal Sepsis with septic shock
Inborn errors of Metabolism
Endocrine disorders

12. Differential diagnosis
Duct dependent systemic Acyanotic CHD with large
circulation (LVOTO) L R shunt
 POINTS FOR:
 Decompensation in 1-2 weeks POINTS FOR:
of life  Timing of Presentation
 Differential pulses and blood
pressure
 Presentation with CCF
 Term baby / exclusively without cyanosis
breastfed POINTS AGAINST:
 no setting for sepsis
 Respiratory failure & shock
 Relatively rare
without cyanosis  Differential pulse volume
 POINTS AGAINST: and BP
 Elevated counts (?
Nosocomial sepsis)

13. Differential Diagnosis
Inborn errors of
LONS with septic shock
metabolism
POINTS FOR: POINTS AGAINST
 High leucocyte counts  No hypoglycemia
POINTS AGAINST  Acidosis improved with
management of shock
 Term / AFD
 No organomegaly
 Exclusively breastfed
 No family history
 Non-consanguinous

14. Chest X-ray

15. How Diagnosis was established??
Acute deterioration on D 8-10 of life
Admitted outside with Cardiogenic shock /
Respiratory Failure
No settings for sepsis & No h/o cyanosis
On examination, differential pulses and blood
pressure with e/o congestive cardiac failure
Most likely to be DUCT DEPENDENT SYSTEMIC
LESION
PG E1 infusion started – Response noted
ECHO revealed CO-ARCTATION OF AORTA

16. Co-arctation of Aorta
INDEX CASE
DIAGNOSIS DISCUSSION

17. Prevalence
8 – 10% of all congenital heart defect
M:F ratio :: 2:1
ASSOCIATIONS:
 Patients with Turner’s, 30% have Co Ao
 85% patients with Co Ao  BICUSPID AORTIC VALVE

18. BONNETT Classification
ALMOST ALWAYS JUXTA-DUCTAL

19. Surgical Classification
TYPE I: Isolated
TYPE II: Co Ao associated with VSD
TYPE III: Co Ao a/w complex cardiac anamolies

20. Hemodynamics

21. Computational Fluid dynamics

22. PATHOPHYSIOLOGY

23. Presentation
Bimodal distribution
 Symptomatic in First/ second week of life
 Asymptomatic children / infants
History
 Poor feeding / dyspnea / poor weight gain
 NOTE: NEWBORN DISCHARGE examination could have been
normal

24. Presentation in Newborn period
Archives of Disease in Childhood 1994; 71: F179-
F183

25. PHYSICAL EXAMINATION
Differential pulses / Blood pressure
Differential cyanosis
Features of CCF / Cardiogenic shock
NOTE
NOTE
Blood pressure differential – only after
Blood pressure differential – only after
improvement of cardiac function
improvement of cardiac function
S 2 single and loud. Loud S 3 gallop
Non specific ejection systolic murmur

26. Investigations
Chest X ray – CARDIOMEGALY
 REVERSE ‘E’ sign ( rarely seen before 10 years of age)
 Pulmonary edema / pulmonary venous congestion
ECG
 RVH / RBBB up to infancy. LVH subsequently
Echocardiography
Cardiac catheterization

27. Neonatal Vs Pediatric Co Ao
NEONATAL PEDIATRIC
SYMPTOMS Heart failure Hypertensive Urgency /
Emergency
MURMUR Negative Positive
DISCREPANT PULSES + / - (PDA) +
DIFFERENTIAL +/ - (PDA) -
CYANOSIS
CXR Cardiomegaly Rib Notching
ECG Dominant RV LV dominance
IMAGING Echo MRI

28. Echocardiography – INDEX CASE

29. Management
Medical
 PGE 1 infusion
 Ionotropes (as needed)
 Diuretics / Digoxin
 Anti-hypertensives (in older children)
Surgical
 Type I – Surgery (Role of Balloon angioplasty- controversial)
 Type II – Single stage repair (VSD + Co Ao)
Pulmonary artery banding
 Type III – Individual approach

30. Resection & end to end anastamosis

31. Waldhausen subclavian patch aortoplasty

32. Balloon Angioplasty
Especially useful for Re-coarctation

33. Outcome of Surgery
Therapeutics, Pharmacology and Clinical Toxicology
2009;13(4):409-13

34. Plan for index case
Wean from the ventilator support
Treatment of Nosocomial sepsis
Definitive surgical repair (end to end anastamosis)

35. THANK YOU