1. Klinik für Kinderherzchirurgie
Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency
A. Dodge-Khatami, MD, PhD
Chief of Pediatric Cardiac Surgery
Head of Program for Congenital Heart Disease
University Heart Center – UHZ
University of Hamburg-Eppendorf School of Medicine
Hamburg, Germany

2. most important objective:
Klinik für Kinderherzchirurgie
even rare congenital heart defects will be seen once in your careers (0.8% of all births); how should you react?
most important objective:
distinguish between a blue and pink patient with a murmur and understand why!

3. Shunts: Location + Direction
Klinik für Kinderherzchirurgie
Shunts: Location + Direction
Intra or extra-cardiac?
Which heart chambers are affected?
Qp/Qs = pulmonary / systemic flow ratio
Qp = VO2 / pulm Vv O2 – PA O2
Qs = VO2 / Vv O2 – Ao O2
In the absence of a shunt, Qp/Qs = 1

4. Klinik für Kinderherzchirurgie
Normal circulation
Q = P/R
Qp/Qs = 1

5. Shunts: Direction Klinik für Kinderherzchirurgie
Left >>> right or Right >>> left?
Which is more probable? Why?
Left > right : PDA, ASD, VSD, AVSD, AP window, Truncus, PAPVD, TAPVD
Right > left : right inflow or outflow obstruction + intra-cardiac shunt: Tricuspid atresia (TA)/Tricuspid Stenosis (TS), Pulmonary Atresia/Pulmonary stenosis, TOFallot

6. Left >>> right : VSD
Klinik für Kinderherzchirurgie
Shunts: Direction
Left >>> right : VSD
Left >> right shunt
Qp/Qs > 2 - 3
Pressure + Volume Overload

7. Shunts: Physiology Klinik für Kinderherzchirurgie
Left >>> right:
LV volume overload
Increased pulmonary flow, pulmonary infections
Pulmonary Hypertension (PHN), severity and degree according to shunt size
Bacterial endocarditis
Right >>> left:
RV pressure overload + strain
Cyanosis
Polyglobulia

8. Shunts: Treatment Klinik für Kinderherzchirurgie
Left >>> right:
volume restriction, diuretics, inotropes, permissive hypercapnea ventilation (hypoventilation),
shunt closure
Right >>> left:
hydration, (transfusion), hyperventilation, increase
pulmonary blood flow +/- shunt closure

9. Shunts: Operative Indications
Klinik für Kinderherzchirurgie
Shunts: Operative Indications
L >> R:
Symptoms: tachycardia, tachypnea, hepatomegaly, sweating during feeds, failure to thrive
Qp:Qs > 1.5
Aortic valve prolapse +/- insufficiency
R >> L:
cyanosis, RVH + strain

10. 5 most common congenital heart defects?
Klinik für Kinderherzchirurgie
5 most common congenital heart defects?

11. 5 most common congenital heart defects?
Klinik für Kinderherzchirurgie
5 most common congenital heart defects?
Ventricular Septal Defect (VSD) 30%
Patent Ductus Arteriosus (PDA) 10%
Coarctation (coA) 5-8%
Atrial Septal Defect (ASD) ~ 8%
Tetralogy of Fallot (TOF) 5-10%

12. Klinik für Kinderherzchirurgie
case: blue child (10 years old) with a murmur (where?)
auscultation: holosystolic murmur at precordium
saturations: ? Cyanosis: central or peripheral?
Central:
intracardiac shunt + obstruction to pulmonary blood flow
Peripheral:
Chronic Pneumonia, Chronic Interstitial Lung Disease, Pulmonary Neoplasia, Circulatory Collapse (+Peripheral Vasoconstriction)
next step ?
„Hippocratic fingers“- Clubbing

13. x-ray: differential diagnosis?
Klinik für Kinderherzchirurgie
x-ray:
differential diagnosis?

14. Klinik für Kinderherzchirurgie
x-ray:
prominent central pulmonary markings
black peripheral lung fields
next step ?

15. Klinik für Kinderherzchirurgie
echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
Diagnosis ?

16. Klinik für Kinderherzchirurgie
echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
VSD: why is the child blue?

17. Right >> Left shunting = Cyanosis
Klinik für Kinderherzchirurgie
Right >> Left shunting = Cyanosis
>
increased cellularity (muscular and interstitial)
>> fixed pulmonary vascular resistance = Eisenmenger syndrome

18. Patent Ductus Arteriosus (PDA)
Klinik für Kinderherzchirurgie
Patent Ductus Arteriosus (PDA)

19. Patent Ductus Arteriosus (PDA)
Klinik für Kinderherzchirurgie
Patent Ductus Arteriosus (PDA)
continuous „machinery“ murmur
LV hypertrophy + LA dilatation
Increased pulmonary vascular markings, interstitial pulmonary edema
failure to thrive
recurrent upper respiratory infections
fatigue with exertion
tachypnea, tachycardia, heart failure

20. Patent Ductus Arteriosus (PDA)
Klinik für Kinderherzchirurgie
Portsmann, 1967
Patent Ductus Arteriosus (PDA)
R. Gross, Boston, 1938

21. Klinik für Kinderherzchirurgie
Coarctation (coA)

22. Klinik für Kinderherzchirurgie
Coarctation (coA)
bi-modal presentation:
newborns in cardiovascular shock: ductal-dependent (PGE1)
vs.
„asymptomatic“ hypertensive children: headaches, epistaxis

23. Klinik für Kinderherzchirurgie
Coarctation (coA)
mid-systolic murmur in the back, systolic or continuous murmurs on the lateral chest walls (collaterals), diminished femoral pulses
Left Ventricular hypertrophy, myocardial infarction
circle of Willis aneurysms, aortic aneurysms, aortic dissection, aortic rupture
average age at death ~ 35 years if untreated : congestive heart failure (1/4), bacterial endocarditis (1/4), spontaneous rupture of the aorta (20%), intracranial hemorrhage (13%)

24. Klinik für Kinderherzchirurgie
Coarctation (coA)
C. Crafoord, Stockholm, 1944
End-to-end anastomosis

25. Klinik für Kinderherzchirurgie
Voßschulte, 1957
Patch plasty
Coarctation (coA)
Gross, 1951
Interposition graft
Waldhausen, 1966
Subclavian flap

26. Coarctation (coA) : results
Klinik für Kinderherzchirurgie
Coarctation (coA) : results
Mortality: 4-14%, age-dependent
Complications:
hypertension, chylothorax, recurrent nerve paresis (stridor)
recurrent coA ~ 10-15% if surgery in the newborn period, >> balloon dilatation
paraplegia
aneurysm

27. Atrial Septal Defect (ASD)
Klinik für Kinderherzchirurgie
Atrial Septal Defect (ASD)
systolic murmur, fixed split second heart sound (prolonged flow time on the right – delayed closure of the pulmonary valve)
Dilated right atrium + ventricle
Pulmonary hypertension recurrent upper respiratory infections
atrial arrhythmia (flutter, fibrillation)
congestive heart failure
no risk of bacterial endocarditis

28. Atrial Septal Defect (ASD)
Klinik für Kinderherzchirurgie
Atrial Septal Defect (ASD)
King, 1976, device closure
F.J. Lewis, Minneapolis, 1952, inflow occlusion

29. Atrial Septal Defect (ASD)
Klinik für Kinderherzchirurgie
Atrial Septal Defect (ASD)
J. Gibbon Jr., Rochester, father of cardio-pulmonary bypass,

30. Atrial Septal Defect (ASD)
Klinik für Kinderherzchirurgie
Atrial Septal Defect (ASD)
J. Gibbon Jr., Rochester, 1953

31. Atrial Septal Defect (ASD) : results
Klinik für Kinderherzchirurgie
Atrial Septal Defect (ASD) : results
Gibbon (1953): first success, followed by 5 deaths, abandonned surgery and requested a 1 year moratorium on his bypass machine…
current: mortality ~ 0%

32. Ventricular Septal Defect (VSD) most frequent CHD ~ 30%
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD) most frequent CHD ~ 30%

33. Ventricular Septal Defect (VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD)
Holosystolic murmur, increased pulmonary vascularity on x-ray,
Cardiomegaly, biventricular dilatation + hypertrophy.
Dyspnea, sweating during feeding, failure to thrive.
Recurrent upper respiratory tract infections.

34. Ventricular Septal Defect (VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD)
Untreated:
25-40% spontaneous closure > 2-3 years
endocarditis (0.3% per year)
pulmonary hypertension > pulmonary arteriolar wall thickening
increased PVR, reversal of shunt
= Eisenmenger syndrome
cyanosis (by 1-2 years of age)
death

35. Ventricular Septal Defect (VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD)

36. Ventricular Septal Defect (VSD >>> VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD >>> VSD)
increased cellularity (muscular and interstitial)
increased reactivity
fixed contraction
vascular wall sclerosis
>> fixed pulmonary vascular resistance = Eisenmenger syndrome

37. Ventricular Septal Defect (VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD)
Cross-circulation: father as oxygenator, but potentially 200% mortality…
C.W. Lillehei, Minneapolis
1954: VSD
„King of Hearts: the True Story of the Maverick Who Pioneered Open Heart Surgery “, G.W. Miller

38. Ventricular Septal Defect (VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD)
C.W. Lillehei, Minneapolis
1954: VSD
28/47 patients survived:~ 40% mortality

39. Ventricular Septal Defect (VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD)

40. Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD): Results mortality ~ 1-2% heart block > pacemaker 1-2%
long-term prognosis
excellent!

41. Ventricular Septal Defect (VSD): palliation
Klinik für Kinderherzchirurgie
Ventricular Septal Defect (VSD): palliation
PA banding
multiple VSDs
small baby, failure to thrive
Muller / Damman, 1952

42. Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF) most frequent cyanotic CHD ~ 10% 1. Overriding Aorta 2. Ventricular Septal Defect 3. Right ventricular hypertrophy 4. Right Ventricular Outflow Tract Obstruction (RVOTO)

43. Klinik für Kinderherzchirurgie
Tetralogy of Fallot
systolic murmur
right aortic arch (25%), „boot shape“ heart
right ventricular hypertrophy
cyanosis, tet „spells“: dynamic RVOT contraction
clubbing (after 6 months), dyspnea, exercise intolerance
brain abscess
polycythemia > pulmonary + cerebral thrombosis

44. Tetralogy of Fallot (TOF) Palliation
Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF) Palliation
H. Taussig A. Blalock
Baltimore, 1944, classic Blalock-Taussig Shunt = „blue baby operation“
Modified BT shunt, 1976

45. Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF) Right Ventricular Outflow Tract Obstruction (RVOTO): - Suprapulmonary (Pulmonary Arteries) - Pulmonary Valve - Subpulmonary (Right Ventricle) Central Importance of the Pulmonary Valve distally: Pulmonary Artery growth proximally: protect the Right Ventricle

46. Tetralogy of Fallot (TOF): complete repair
Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF): complete repair
C.W. Lillehei, Minneapolis
1955: Fallot correction

47. Tetralogy of Fallot (TOF)
Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF)

48. Tetralogy of Fallot (TOF): results
Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF): results
Mortality 3-5 %
Heart Block < 3%, seldom requires a pacemaker
Post-operative arrhythmia frequent
Reoperations required for:
residual VSD (seldom)
residual pulmonary valve insufficiency
residual right outflow obstruction

49. Tetralogy of Fallot (TOF): reoperations
Klinik für Kinderherzchirurgie
Tetralogy of Fallot (TOF): reoperations
residual pulmonary valve INSUFFICIENCY
right ventricular volume overload + dilatation + failure
arrhythmia
better growth of pulmonary arteries?
late REOPERATION
residual right outflow STENOSIS
right ventricular pressure overload
pulmonary artery stenosis/hypoplasia