Ventricular Septal Defect - Universitätsklinikum Hamburg

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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
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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
Shunts: Direction
•
Left >>> right : VSD
Left >> right shunt
Qp/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
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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
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
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Patent Ductus Arteriosus
(PDA)
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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
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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
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Voßschulte, 1957
Patch plasty
Coarctation
(coA)
Gross, 1951
Interposition graft
Waldhausen, 1966
Subclavian flap
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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
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Atrial Septal Defect
(ASD)
J. Gibbon Jr.,
Rochester, father of
cardio-pulmonary
bypass, 1934-53
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Atrial Septal Defect
(ASD)
J. Gibbon Jr.,
Rochester, 1953
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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
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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
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
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Tetralogy of Fallot
(TOF): complete repair
C.W. Lillehei, Minneapolis
1955: Fallot correction
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Tetralogy of Fallot
(TOF)
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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
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