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
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!
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
Klinik für Kinderherzchirurgie
Normal circulation
Q = P/R
Qp/Qs = 1
Klinik für Kinderherzchirurgie
Shunts: Direction
•
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
Klinik für Kinderherzchirurgie
Shunts: Direction
•
Left >>> right : VSD
Left >> right shunt
Qp/Qs > 2 - 3
Pressure + Volume Overload
Klinik für Kinderherzchirurgie
Shunts: Physiology
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
Klinik für Kinderherzchirurgie
Shunts: Treatment
Left >>> right:
volume restriction, diuretics, inotropes, permissive
hypercapnea ventilation (hypoventilation),
shunt closure
Right >>> left:
hydration, (transfusion), hyperventilation, increase
pulmonary blood flow +/- shunt closure
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
Klinik für Kinderherzchirurgie
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%
Klinik für Kinderherzchirurgie
•
case: blue child (10 years old) with a murmur (where?)
auscultation: holosystolic murmur at precordium
saturations: ? Cyanosis: central or peripheral?
„Hippocratic fingers“- Clubbing
Central:
intracardiac shunt + obstruction to pulmonary blood flow
Peripheral:
Chronic Pneumonia, Chronic Interstitial Lung Disease, Pulmonary
Neoplasia, Circulatory Collapse (+Peripheral Vasoconstriction)
next step ?
Klinik für Kinderherzchirurgie
x-ray:
differential diagnosis?
Klinik für Kinderherzchirurgie
x-ray:
•
•
next step ?
prominent central pulmonary markings
black peripheral lung fields
Klinik für Kinderherzchirurgie
echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
Diagnosis ?
Klinik für Kinderherzchirurgie
echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
VSD: why is the child blue?
Klinik für Kinderherzchirurgie
Right >> Left shunting = Cyanosis
>
increased cellularity (muscular and interstitial)
>> fixed pulmonary vascular resistance = Eisenmenger syndrome
Klinik für Kinderherzchirurgie
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
Klinik für Kinderherzchirurgie
Portsmann,
1967
Patent Ductus Arteriosus
(PDA)
R. Gross,
Boston,
1938
Klinik für Kinderherzchirurgie
Coarctation
(coA)
Klinik für Kinderherzchirurgie
Coarctation
(coA)
• bi-modal presentation:
newborns in cardiovascular shock: ductal-dependent (PGE1)
vs.
„asymptomatic“ hypertensive children: headaches, epistaxis
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%)
Klinik für Kinderherzchirurgie
Coarctation
(coA)
C. Crafoord,
Stockholm, 1944
End-to-end
anastomosis
Klinik für Kinderherzchirurgie
Voßschulte, 1957
Patch plasty
Coarctation
(coA)
Gross, 1951
Interposition graft
Waldhausen, 1966
Subclavian flap
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
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
Klinik für Kinderherzchirurgie
Atrial Septal Defect
(ASD)
F.J. Lewis, Minneapolis,
1952, inflow occlusion
King, 1976, device closure
Klinik für Kinderherzchirurgie
Atrial Septal Defect
(ASD)
J. Gibbon Jr.,
Rochester, father of
cardio-pulmonary
bypass, 1934-53
Klinik für Kinderherzchirurgie
Atrial Septal Defect
(ASD)
J. Gibbon Jr.,
Rochester, 1953
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%
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD)
most frequent CHD ~ 30%
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.
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
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(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
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD)
C.W. Lillehei, Minneapolis
1954: VSD
„King of Hearts: the True Story of the
Maverick Who Pioneered Open Heart
Surgery “, G.W. Miller
Cross-circulation: father as
oxygenator, but potentially 200%
mortality…
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD)
C.W. Lillehei, Minneapolis
1954: VSD
28/47 patients survived:~ 40% mortality
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD): Results
mortality ~ 1-2%
heart block > pacemaker 1-2%
long-term prognosis
excellent!
Klinik für Kinderherzchirurgie
Ventricular Septal Defect
(VSD): palliation
PA banding
• multiple VSDs
• small baby, failure to thrive
Muller / Damman, 1952
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)
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
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
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
Klinik für Kinderherzchirurgie
Tetralogy of Fallot
(TOF): complete repair
C.W. Lillehei, Minneapolis
1955: Fallot correction
Klinik für Kinderherzchirurgie
Tetralogy of Fallot
(TOF)
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
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
late REOPERATION