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![Page 1: Cyanosis or Congestive Heart Failure in Children: Murmurs of Shunts, Stenosis, and Insufficiency A. Dodge-Khatami, MD, PhD Chief of Pediatric Cardiac Surgery.](https://reader038.fdocuments.in/reader038/viewer/2022102923/551439fd550346494e8b45d6/html5/thumbnails/1.jpg)
Cyanosis or Congestive Heart Failure in Children:
Murmurs of Shunts, Stenosis, and Insufficiency
A. Dodge-Khatami, MD, PhDChief of Pediatric Cardiac SurgeryHead of Program for Congenital Heart DiseaseUniversity Heart Center – UHZUniversity of Hamburg-Eppendorf School of MedicineHamburg, Germany
Klinik für Kinderherzchirurgie
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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
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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
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Normal circulation
Klinik für Kinderherzchirurgie
Qp/Qs = 1Q = P/R
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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
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Shunts: Direction
• Left >>> right : VSD
Klinik für Kinderherzchirurgie
Left >> right shuntQp/Qs > 2 - 3
Pressure + Volume Overload
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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
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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
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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
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5 most common congenital heart defects?
Klinik für Kinderherzchirurgie
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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
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• case: blue child (10 years old) with a murmur (where?)auscultation: holosystolic murmur at precordiumsaturations: ? 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 ?
Klinik für Kinderherzchirurgie
„Hippocratic fingers“- Clubbing
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x-ray:
differential diagnosis?
Klinik für Kinderherzchirurgie
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x-ray: • prominent central pulmonary markings• black peripheral lung fields
next step ?
Klinik für Kinderherzchirurgie
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echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
Diagnosis ?
Klinik für Kinderherzchirurgie
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echocardiography:
Cardiomegaly, biventricular dilatation + hypertrophy
VSD: why is the child blue?
Klinik für Kinderherzchirurgie
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Right >> Left shunting = Cyanosis
>
increased cellularity (muscular and interstitial)>> fixed pulmonary vascular resistance = Eisenmenger syndrome
Klinik für Kinderherzchirurgie
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Patent Ductus Arteriosus
(PDA)
Klinik für Kinderherzchirurgie
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Patent Ductus Arteriosus
(PDA)
Klinik für Kinderherzchirurgie
• 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
•
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Patent Ductus Arteriosus
(PDA)
Klinik für Kinderherzchirurgie
R. Gross, Boston, 1938
Portsmann, 1967
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Coarctation
(coA)
Klinik für Kinderherzchirurgie
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Coarctation
(coA)
Klinik für Kinderherzchirurgie
• bi-modal presentation:
newborns in cardiovascular shock: ductal-dependent (PGE1)
vs.
„asymptomatic“ hypertensive children: headaches, epistaxis
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Coarctation
(coA)
Klinik für Kinderherzchirurgie
• 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%)
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Coarctation
(coA)
Klinik für Kinderherzchirurgie
C. Crafoord, Stockholm, 1944
End-to-end anastomosis
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Coarctation
(coA)
Klinik für Kinderherzchirurgie
Voßschulte, 1957Patch plasty
Gross, 1951Interposition graft
Waldhausen, 1966Subclavian flap
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Coarctation
(coA) : results
Klinik für Kinderherzchirurgie
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
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Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
• 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
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Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
F.J. Lewis, Minneapolis, 1952, inflow occlusion
King, 1976, device closure
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Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
J. Gibbon Jr., Rochester, father of cardio-pulmonary bypass, 1934-53
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Atrial Septal Defect
(ASD)
Klinik für Kinderherzchirurgie
J. Gibbon Jr., Rochester, 1953
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Atrial Septal Defect
(ASD) : results
Klinik für Kinderherzchirurgie
• Gibbon (1953): first success, followed by 5 deaths, abandonned surgery and requested a 1 year moratorium on his bypass machine…
• current: mortality ~ 0%
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Ventricular Septal Defect
(VSD)
most frequent CHD ~ 30%
Klinik für Kinderherzchirurgie
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Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
• Holosystolic murmur, increased pulmonary vascularity on x-ray,
• Cardiomegaly, biventricular dilatation + hypertrophy.
• Dyspnea, sweating during feeding, failure to thrive.
• Recurrent upper respiratory tract infections.
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Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
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
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Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
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Ventricular Septal Defect
(VSD >>> VSD)
Klinik für Kinderherzchirurgie
• increased cellularity (muscular and interstitial)
• increased reactivity• fixed contraction• vascular wall sclerosis
>> fixed pulmonary vascular resistance = Eisenmenger syndrome
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Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
C.W. Lillehei, Minneapolis1954: 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…
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Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
C.W. Lillehei, Minneapolis1954: VSD28/47 patients survived:~ 40% mortality
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Ventricular Septal Defect
(VSD)
Klinik für Kinderherzchirurgie
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Ventricular Septal Defect
(VSD): Results
mortality ~ 1-2%
heart block > pacemaker 1-2%
Klinik für Kinderherzchirurgie
long-term prognosisexcellent!
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Ventricular Septal Defect
(VSD): palliation
Klinik für Kinderherzchirurgie
PA banding• multiple VSDs
• small baby, failure to thrive
Muller / Damman, 1952
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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
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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
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Tetralogy of Fallot (TOF)
Palliation
Klinik für Kinderherzchirurgie
H. Taussig A. Blalock
Baltimore, 1944, classic Blalock-Taussig Shunt = „blue baby operation“
Modified BT shunt, 1976
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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
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Tetralogy of Fallot
(TOF): complete repair
Klinik für Kinderherzchirurgie
C.W. Lillehei, Minneapolis1955: Fallot correction
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Tetralogy of Fallot
(TOF)
Klinik für Kinderherzchirurgie
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Tetralogy of Fallot
(TOF): results
Klinik für Kinderherzchirurgie
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
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Tetralogy of Fallot
(TOF): reoperations
Klinik für Kinderherzchirurgie
• residual pulmonary valve INSUFFICIENCY
right ventricular volume overload + dilatation + failurearrhythmiabetter growth of pulmonary arteries?late REOPERATION
• residual right outflow STENOSIS
right ventricular pressure overloadpulmonary artery stenosis/hypoplasialate REOPERATION