congenital heart disease - web2.aabu.edu.jo · congenital heart disease ... congenital heart...
TRANSCRIPT
![Page 1: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/1.jpg)
Cardiology review lecture 1
Congenital heart disease
![Page 2: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/2.jpg)
Congenital Heart Disease
• Commonest group of life
threatening anomalies
• 8/1000 live births
– VSD 30-50%, PDA 10%, ASD 7%.
– PS 7%
– Coarctation 6%
![Page 3: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/3.jpg)
Principle differences in fetal
circulation compared to
post-natal circulation:
• Combined ventricular output
• Three critical anatomic communications– PFO (from RA to LA)
– PDA (from PA to AO)
– Ductus venosus (from UV to IVC)
• Organ responsible for oxygenation is Placenta
![Page 4: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/4.jpg)
Transition From the Fetal
Circulation• Pulmonary vascular resistance falls
• Ductus venosus and ductus
arteriosus close
• Right-to-left shunting through
foramen ovale ceases
Timing of these events determines
the timing of presentation of
congenital heart defects
![Page 5: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/5.jpg)
Structural heart disease
Acyanotic
with shunt•ASD
•VSD
•PDA
Cyanotic•TOF
•Tricuspid Atresia
Non Shunt lesions
Obstruction•Aortic stenosis AS
•Supravalvar AS
•Coarctation
•Mitral Stenosis
•Pulmonary Stenosis
Regurgitation
•Aortic regurgitation
•Mitral regurgitation
•Pulmonary reg.
![Page 6: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/6.jpg)
How will this
baby look at
12 hours of
age?
![Page 7: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/7.jpg)
Presentation First 24
Hours
• Critically ill” - like
asphyxia
• Cyanosis (may be mild)
• Pure heart failure is
uncommon
• Murmur
![Page 8: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/8.jpg)
Symptoms of cardiac failure in
the infant
• Poor feeding
• Failure to thrive
• Fatigue
![Page 9: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/9.jpg)
Left to Right shunt
• Portion of fully oxygenated pulmonary
venous blood bypassing the systemic
flow and going back to the lungs
• In-effective pulmonary blood flow
• S&S of Increased pulmonary blood
flow
![Page 10: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/10.jpg)
Left to right shunts
Physiologic effect of the shunt is
dependent on three factors:
1) Location of the shunt
2) Size of the defect
3) Relative pulmonary and systemic
vascular resistance (or ventricular
compliance in case of atrial level shunts)
![Page 11: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/11.jpg)
Left Atrium
Left Ventricle
Aorta
Right Atrium
Right Ventricle
Pulmonary Artery
Atrial level shunts
![Page 12: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/12.jpg)
Atrial Level shunt: Anatomy
![Page 13: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/13.jpg)
Atrial Level Shunt: Physiology
•ASD causes volume
load on the right
atrium, and right
ventricle leading to
dilation of these
structure
•Majority of the shunt
occurs during diastole
(ventricular filling)
•Cardiac output is well
maintained even in
large ASD’s
![Page 14: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/14.jpg)
Symptoms: ASD• Usually asymptomatic
• Symptoms of CHF, tachypnia, or
decreased exercise tolerance are rare
• Most ASD’s are discovered incidentally
or due to a heart murmur
![Page 15: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/15.jpg)
Examination
• Normal in young infants
• Wide, fixed S2
• Ejection systolic murmur
![Page 16: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/16.jpg)
Diagnostic studies
• ECG: Usually Normal, but may reflect right atrial and right ventricular dilatation
• CXR: May show increased pulmonary vascular markings and dilated pulmonary trunk
• Echocardiography is diagnostic, and important to determine the type and physiologic effect of the defect
![Page 17: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/17.jpg)
Management
• No restriction from activity
• No medications
• Observation for spontaneous closure if
secundum type and no significant
volume overload on the right ventricle
![Page 18: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/18.jpg)
Left Atrium
Left Ventricle
Aorta
Right Atrium
Right Ventricle
Pulmonary Artery
Ventricular level shunt
![Page 19: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/19.jpg)
Ventricular Level Shunt:
Physiology
•VSD causes Pressure
load on the right ventricle
causing RVH, and Volume
load on the left atrium and
ventricle leading to dilation
•Shunt occurs during
Systole (ventricular
emptying)
•Cardiac output is well
maintained even in large
VSD’s
![Page 20: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/20.jpg)
Symptoms: VSD• Newborns with VSD are usually well
• Mod-Large VSD in infants may cause
CHF symptoms once Pulmonary
vascular resistances
• Decreased feeding, diaphoresis and
respiratory distress
• Compensated patients deteriorate
rapidly with infection
![Page 21: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/21.jpg)
Examination
• Newborns may not have murmur (High PVR)
• Displaced apex beat
• Pan-systolic murmur
• Small muscular defects may have short murmurs
• Loud S2
• S3
![Page 22: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/22.jpg)
Diagnostic studies
• ECG: (beyond infancy)
– Left axis deviation
– LVH
– Left atrial dilation
• CXR shows cardiomegally, and
increased pulmonary vascular markings
in significant VSD’s
![Page 23: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/23.jpg)
Management
• No restriction from activity
• No SBE prophylaxis (the newer
guidelines)
• Spontaneous closure is common in
small and moderate perimembranous
and muscular defects
• Surgical treatment is the standard
treatment for symptomatic VSD’s
![Page 24: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/24.jpg)
Left Atrium
Left Ventricle
Aorta
Right Atrium
Right Ventricle
Pulmonary Artery
PDA
![Page 25: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/25.jpg)
• Located just distal to the origin of the left subclavian artery
• 1 / 2500 to 1/ 5000 live births– Increased
incidence with prematurity
• 12% of all CHD
• Female to male ratio = 2:1
• Associations: Asphyxia,Chromosomal anomalies, Birth at high altitudes, Congenital Rubella, Drugs, Genetic?
![Page 26: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/26.jpg)
Normal postnatal closureFunctional closure
– Usually occurs within the first 24 hours
– Stimulated by:
• High pO2 (more prominent effect with increased GA)
• Interruption of Prostaglandins– Most important factor in ductal patency
– Produced by the placenta and the ductal tissue
– Metabolized by the lungs
– Ductal tissue is much more sensitive to prostaglandins at earlier gestations
Complete “anatomic” closure (fibrosis)
– Usually occurs in the first 2-3 weeks
![Page 27: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/27.jpg)
PDA physiology
• Flow across the PDA depends on– size
– Pressure gradient
– relative resistence (SVR vs. PVR)
• Cardiac consequences– Increased pulmonary blood flow and venous return
– Left ventricular dilation (volume load on LV)
– Right ventricular hypertrophy (pressure load on RV)
– Similar physiologic effect as VSD
– Unlike VSD it causes diastolic hypotension decreased myocardial coronary perfusion may worsen congestive heart failure
![Page 28: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/28.jpg)
Presentation
• In premature infants:
• Hyperdynamic circulation
– Wide pulse pressure
– Heart murmur
– Increased oxygen requirement occurs due to
pulmoanry congention, and increased respiratory
distress
• In Term infants: Usually asymptomatic in the
first few weeks of life (similar to VSD’s)
![Page 29: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/29.jpg)
Management
• Asymptomatic PDA’s require no treatment before age of 1 year, elective closure can usually be done by catheterization
• Symptomatic PDA’s– Symptomatic treatment of CHF (diuresis, inotropic
support, and vasodilators)
– Avoid lowering PVR (avoid oxygen, alkalosis, NO)
– Medical closure (Indomethacin IV, preferably before day 10 of life, Ibuprofen IV has similar effect)
– Surgical closure in refractory cases
![Page 30: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/30.jpg)
Cyanotic heart disease (right to
left shunt)
![Page 31: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/31.jpg)
General causes of Cyanosis
Pulmonary Cardiac Others
Airway diseaseIntrapulmonary
shunting
Intracardiac
shunting
Cyanosis
![Page 32: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/32.jpg)
Hyperoxia test
Administration of 100% O2 for 15 minutes
Measure arterial PO2
PO2 <150 PO2 >250PO2 150-250
Cardiac PulmonaryGray zone
![Page 33: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/33.jpg)
Tetralogy of Fallot (TOF)
1. ASD
2. VSD
3. Overriding
aorta
4. RV
hypertrophy
![Page 34: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/34.jpg)
TOF
• Most common cyanotic heart lesion
• 3rd most common overall
• TOF is approximately 10% of CHD
• In the extreme form there is complete pulmonary atresia (PA/VSD), 2% of CHD
• Exact cause of TOF is uncertain
• Generally both TOF and PA/VSD are isolated findings
• Not typically found with syndromes– De Lange, Goldenhar, Klippel-Feil
• TOF is seen with malformation assoc– VACTERL, CHARGE, Velo-cardio-facial
![Page 35: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/35.jpg)
5
5
5
2
33
3
100%
100%
50%
80%
50%
50%
![Page 36: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/36.jpg)
Clinical Features
• Asymptomatic infant with murmur is very common in the usual TOF patients
• Murmur of RVOT obstruction can be confused with VSD in infancy
• Cyanosis– Typically appears between 6wks and 6 months in
the unrepaired infant
– Nail beds and mucous membranes
– May be present at rest or only with agitation/exercise
• Persistent cyanosis and clubbing if not repaired
![Page 37: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/37.jpg)
Diagnostic studies
• CXR “coeur en sabot”
– Normal heart size
– Upturned apex
– Concave upper left
heart border
– Normal or decreased
pulmonary vascular
markings
• Echocardiography is
diagnostic
![Page 38: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/38.jpg)
Surgical Management
• VSD closure
– transatrial access if possible
– Infundibular resection for visualization
– Patch closure
![Page 39: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/39.jpg)
Trcuspid Atresia
• Complete absence
of communication
between the right
atrium and right
ventricle
• About 3 % of
congenital heart
disease
![Page 40: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/40.jpg)
Tricuspid Atresia
• There is an obligate interatrial communication
• Usually associated with VSD
• The pulmonary blood flow is dependent on the size of the VSD
• Pulmonary blood flow can be increased or decreased causing variable presenting symptoms
• If there is no VSD ( also called Hypoplastic right ventricle) the pulmonary blood flow is dependent on the PDA
![Page 41: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/41.jpg)
Tricuspid Atresia- presentation
• The presentation will depend on the
amount of pulmonary blood flow
– cyanosis
– congestive heart failure
• CXR will also reflect the amount of
pulmonary blood flow
![Page 42: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/42.jpg)
Acyanotic-Non shunt lesions
![Page 43: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/43.jpg)
non-shunt
Acyanotic heart lesions
Obstruction•Aortic stenosis AS
•Supravalvar AS
•Coarctation
•Mitral Stenosis
•Pulmonary Stenosis
Regurgitation•Aortic regurgitation
•Mitral regurgitation
•Pulmonary regurgitation
Generally cause
pressure overload
Generally cause
Volume overload
![Page 44: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/44.jpg)
Coarctation of aorta
• Narrowing of the aortic lumen that
causes an obstruction to blood flow.
• Variable degrees of obstruction,
anatomic location, and hence clinical
manifestations and outcome after
treatment.
![Page 45: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/45.jpg)
![Page 46: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/46.jpg)
CLINICAL FEATURES
• 3 typical patterns.
• Infant with congestive heart failure.– Catastrophic illness on day 8 - 12
– Shock, CHF
• Child with a murmur – Subtle signs, pressure gradient, radiofemoral delay
on examination
• Adolescent– Systolic hypertension, Chest pain
![Page 47: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/47.jpg)
TREATMENT
• Untreated it has a a poor prognosis.
• Treatment depends on the clinical presentation:
Infants presenting with shock require medical management and immediate surgical treatment.
For patients presenting during childhood timing is more elective.
![Page 48: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/48.jpg)
Aortic stenosis
100/60
100/5
100/60
160/15
![Page 49: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/49.jpg)
Aortic stenosis
• Clinical presentation depends on the
degree of stenosis
– Ranges from asymptomatic to Chest pain
with exercise to heart failure
– May present with sudden cardiac death
![Page 50: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/50.jpg)
PresentationVolume overload results in ventricular dilation and
congestive heart failure
Atrial dilation may lead to atrial arrythmias
Examination typically shows holosystolic murmur
radiating to the axilla, with displaced apex beat and
S3
ManagementDiuresis, and afterload reduction are the mainstay of
treatment
Surgical repair of replacement of the valve in refractory
CHF
![Page 51: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/51.jpg)
Heart Failure in Children
![Page 52: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/52.jpg)
Definition
Pathophysiological state in which an abnormality of cardiac function is responsible for the failure of the heart to pump blood to meet the metabolic requirements of the body
Chronic heart failure is a clinical syndrome in which heart disease reduces cardiac output, increases venous pressures, and is accompanied by molecular abnormalities that cause progressive deterioration of the failing heart and premature death of myocardial cells
![Page 53: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/53.jpg)
How may the heart fail?
Too muchpreload
Streching of muscle fibers
• Lt-Rt shunts• Complete heart block
Too muchafterload
Increased wall stress
• Hypertension• Coarctation• Aortic
stenosis
Impairedcontractility
• Dilatedcardiomyo-pathy
• Myocarditis
Impaired filling
• Hypertrophiccardiomyo-pathy
• Constrictivepericarditis
![Page 54: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/54.jpg)
What causes heart failure in
children?
• Congenital heart disease:– Volume overload
– Pressure overload
– Post-op. patients with residual lesions
– Pulmonary vascular disease
– Chronic hypoxia
• Coronary artery disease– Congenital anomalies
– Acquired disease
• Endocarditis
• Cardiomyopathies– Dilated
– Hypertrophic
– Restrictive
• Chronic anemia
• Primary pulmonary hypertension
• Rheumatic heart disease
![Page 55: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/55.jpg)
What happens when the heart fails?
(Compensatory mechanisms)
Increase in sympathetic tone
HR ContractilityVasoconstriction ( afterload)
Myocardial work, myocardial O2 req.
With time, this will lead to diastolic leak of Ca from sarcoplasmic ret.Leading to depletion of intracellular Ca
Decreased contractility
![Page 56: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/56.jpg)
(More Compensatory mechanisms)
Renin-Angiotensin-Aldosterone activation
Salt and water retentionVasoconstriction
( afterload)
Myocardial work, myocardial O2 req.
With time, systemic and pulmonary venous congestion
Decreased contractility
Frank-Starling law contractility
Renal hypo-response to natriureticpeptides
![Page 57: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/57.jpg)
(More Compensatory mechanisms)
Inflammatory response due to wall stress
Cytokines, free radicals
Decreased contractility
Apoptosis
Necrosis and Fibrosis
![Page 58: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/58.jpg)
Clinical Features
• Reflection of:
– Compensatory mechanisms
– Manifestation of decreased tissue
perfusion
– Manifestation of pulmonary and systemic
venous congestion
![Page 59: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/59.jpg)
Clinical features
• Tachycardia, cardiomegally,
• Cold extremities, weak pulses, hypotension, skin mottling
• Metabolic acidosis
• Dyspnia, tachypnia, retractions, grunting
• Poor feeding, poor growth
• Central cyanosis (pulmonary edema)
• Peripheral cyanosis (decreased perfusion)
• Hepatomegally, peripheral edema
• Exercise intelerance in older children
![Page 60: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/60.jpg)
Management goals
• Improve symptoms
• Slow progression of ventricular
remodeling
• Improve survival
Inotropes
Vasodilators
Diuretics
![Page 61: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/61.jpg)
Diuresis
• Decreases volume overload
• BUT stimulates Renin-Angiotensin-
Aldosterone axis, and sympathetic
nervous system
![Page 62: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/62.jpg)
Inotropic support• Digoxin Inhibits sarcolemmal Na-K ATPase pump
Increase intracellular Na
Inhibition of Na/Ca exchange
Increase intracellular Ca
Increase contractility
• Decrease sympathetic tone and norepinephrine
Therefore; Improves symptoms and decreases hospital stay
In the acute setting, inotropic support is usually accoplished by intravenous medications: Dopamine, Dobutamine, Epinephrine, Milrinone
Improving cardiac output will promote more effective diuresis
![Page 63: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/63.jpg)
Vasodilation• Most common approach is ACE inhibition
• Favorable ventricular remodeling
• Improvement of renal function
• Blunts hypertrophic and apoptotic effect of angiotensin II
Therefore; improves cardiac output, and decreases wedge pressure (left atrial pressure), and improves symptoms
Angiotensin receptor antagonists (e.g Losartan) has similar effect
![Page 64: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/64.jpg)
Feel the pulses
especially brachial and femoral
![Page 65: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/65.jpg)
Look at the respiratory pattern and for evidence of recession
![Page 66: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/66.jpg)
Feel the precordium for
hyperactivity
and for thrills
![Page 67: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/67.jpg)
Locate the
apex beat
![Page 68: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/68.jpg)
Feel for
hepatomegally
![Page 69: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/69.jpg)
Do what is necessary to
calm the baby down!
![Page 70: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/70.jpg)
Do what is necessary to
calm the baby down!
![Page 71: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/71.jpg)
Listen at the back for radiation of murmurs
![Page 72: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/72.jpg)
3 months old- severe failure to thrive
![Page 73: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/73.jpg)
Special tests-
echocardiography
![Page 74: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/74.jpg)
Pulmonary veins - colour
DopplerSubcostal view
Apical view
![Page 75: Congenital heart disease - web2.aabu.edu.jo · Congenital Heart Disease ... congenital heart defects. Structural heart disease Acyanotic with shunt](https://reader031.vdocuments.mx/reader031/viewer/2022021622/5b84da8f7f8b9ad34a8d09a8/html5/thumbnails/75.jpg)
Thank you