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Congenital Heart Disease in
Adult
Jacqueline Chan, BSOT-4
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Congenital Heart Disease
A defect in the structure of the heart
and the great vessels which ispresent at birth.
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Etiology
Generally the result of aberrant embryonic
development of a normal structure or failure
of such a structure to progress beyond and
early stage of embryonic or fetal
development.
Malformations: due to complex multifactorial
genetic and environmental causes.
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Etiology
May be anticipated in occasional pregnancies
by detection of abnormal chromosomes in
fetal cells.
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Epidemiology
Complicates ~1% of all live births.
Occurs in 4% of offspring of women with CHD.
Adults now outnumber children with CHD.
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Shunting Lesions
Shunt - refers to an abnormal connection
allowing blood to flow directly from one side
of the cardiac circulation to the other.
Left-to-right shunt: allows the oxygenated,
pulmonary venous blood to return directly to
the lungs rather than being pumped to the
body.
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Shunting Lesions
Right-to-left shunt: allows the deoxygenated,
systemic venous return to bypass the lungs
and return to the body without becoming
oxygenated.
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Shunt Volume
The ratio of total pulmonary blood flow tototal systemic blood flow
A Qp/Qs ratio of 1:1 - normal and usually
indicates that there is no shunting. A Qp/Qs ratio of >1:1 - pulmonary flow
exceeds systemic flow and defines a net left-
to-right shunt. A Qp/Qs ratio of
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2 Types
Cyanotic results in low blood oxygen levels
Acyanotic - does not usually interfere with theamount of blood oxygen that reaches the
tissues of the body. Does not cause cyanosis.
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Pathophysiology
Progress from prenatal life to adulthood.
Malformations that are benign or escapedetection in childhood may become clinically
significant in the adult.
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Pulmonary Hypertension
Status of pulmonary vascular bed: principal
determinant of the clinical manifestations and
course of a given lesion and of the feasibility
of surgical repair.
Eisenmenger syndrome
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Erythrocytosis
Chronic hypoxemia in cyanotic CHD results in
secondary erythrocytosis due to increased
erythropoietin production
Hemostasis is abnormal in CHD
Oral contraceptives is CI in cyanotic women
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Pregnancy
Mothers health: CV lesion associated with
pulmonary vascular dx & pulmonary
hypertension or LV outflow tract obstruction.
Death malformations causing heart failure
Fetus: maternal cyanosis, heart failure, or
pulmonary hypertension
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Pregnancy
Pxs with cyanotic CHD, pulmonary
hypertension, or Marfan syndrome with a
dilated aortic root should not become
pregnant.
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Infective endocarditis
Inflammation of the inner tissue of the heart
caused by infectious agents.
Routine antimicrobial prophylaxis is
recommended
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Specific Cardiac Effects
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Complex Adult CHD
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Intermediate Complexity CHD
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Simple Adult CHD
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Acyanotic CHD with a Left-to-Right
Shunt
Atrial Septal Defect
Ventricular Septal Defect
Patent Ductus Arteriosus Aortic Root to Right Heart Shunts
Aneurysm of an aortic sinus of Valsalva
Coronary arteriovenous fistula
Anomalous origin of the L coronary artery from
the pulmonary artery
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Atrial Septal Defect (ASD)
Common cardiac anomaly. First encountered
in adult. More frequently in females.
Pathophysiology: error in developmental
process of the atrial septum resulting in a
defect in the wall separating the 2 atria
Usually asymptomatic in early life
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Atrial Septal Defect (ASD)
>4th decade: atrial arrhythmias, pulmonary
arterial hypertension, bidirectional and then
right-to-left shunting
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Atrial Septal Defect (ASD)
Types:
Sinus venosus type
Ostium primum type
Ostium secundum type
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Atrial Septal Defect
Sinus Venous Type
- Occurs high in the atrial septum near the
entry of the superior VC into the RA
Ostium primum type
- Lie adjacent to the AV valves, either of which
may be deformed and regurgitant.
- Common in Downs Syndrome
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Atrial Septal Defect
Ostium secundum type
- An abnormally large opening in the atrial
septum at the site of the foramen ovale and the
midseptal in location
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ASD: Diagnosis
PE: prominent RV impulse & palpablepulmonary artery pulsation
ECG
Chest x-ray: enlargement of the RA and RV,dilatation of the pulmonary artery and itsbranches, and increased pulmonary vascular
markings Echocardiogram: pulmonary arterial and RV
and RA dilatation
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ASD: Treatment
Operative repair or percutaneous
transcatheter device closure
Ostium primum: cleft mitral valves repair +
patch closure of the atrial defect
Ostium secundum or sinus venous types:
incidence of progressive symptoms during 5th
or 6th decade.
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ASD: Treatment
Medical mgt:
o Prompt txt of respiratory tract infections
o
Antiarrhythmic medications for atrialfibrillations or supraventricular tachycardia
o Usual measures for hypertension, coronary
heart disease, or heart failure
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Ventricular Septal Defect
Common as isolated defects or as a
component of a combination of anomalies.
Opening usually single and situated in the
membranous portion of the septum.
Spontaneous closure
Txt: operative correction or transcatheter
closure
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Patent Ductus Arteriosus
The ductus arteriosus fails to close
Adults: normal pulmonary pressures and agradient and shunt from aorta to pulmonary
artery Characteristic thrill and a continuous
machinery murmur with late systolic
accentuation at the upper left sternal edge. Death: cardiac failure and infective
endocarditis
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Patent Ductus Arteriosus: Txt
Surgically ligated or divided
Transcatheter closure
Thoracoscopic surgical approaches
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Aortic Root to Right Heart Shunts
Aneurysm of an aortic sinus of Valsalva
- Separation or lack of fusion between themedia of the aorta and the annulus of the
aortic valve.- Abrupt rupture: chest pain, bounding pulses,
continuous murmur accentuated in diastole,volume overload of the heart
- Med. Mgt.: cardiac failure, arrhythmias,endocarditis
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Aortic Root to Right Heart Shunts
Coronary AV fistula
- Communication between a coronary artery
and another cardiac chamber
- Large shunt: coronary steal syndrom
- Potential complications: infective endocarditis,
thrombus formation with occlusion or distal
embolization with myocardial infarction, rupture
of an aneurysmal fistula
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Aortic Root to Right Heart Shunts
Anomalous origin of the left coronary artery
from the pulmonary artery
- Death: myocardial infarction and fibrosis
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Acyanotic CHD w/o a shunt
Congenital aortic stenosis
Valvular aortic stenosis
Subaortic stenosis
Supravalvular stenosis
Coarctation of the aorta
Pulmonary stenosis
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Congenital aortic stenosis
Malformations that cause obstruction to LV
outflow
Valvular aortic stenosis
- One of the most common congenital
malformation of the heart & may go
undetected in early life
- Thickening of cusps and later, calcification
- Diagnosis: echocardiography
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Congenital aortic stenosis
- Txt: prohpylaxis against infective endocarditis;digoxin and diuretics; aortic valve replacement
Subaortic stenosis
- a.k.a. hypertrophic cardiomyopathy
- Membranous diaphragm or fibromuscular ringencircling the LV outflow tract just beneath thebase of the aortic valve
- Txt: complete excision of ring
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Congenital aortic stenosis
Supravalvular aortic stenosis
- Diffuse narrowing of the ascending aorta
- Coronary arteries are subjected to elevated
systolic pressures from the LV, are often
dilated and tortuous, and are susceptible to
premature atherosclerosis
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Pulmonary Stenosis
Supravalvular, subvalvular, or valvular levels
Valvular: most common form of isolated RVobstruction
Mild: generally asymptomatic, little or no progression
Moderate: systolic transvalvular pressure gradient 50-80 mmHg
Severe
Fatigue, dyspnea, RV failure, syncope Txt: cardiac catheter technique of balloon valvuloplasty
direct surgical relief of moderate and sever obstruction
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Pulmonary Stenosis
Fatigue, dyspnea, RV failure, syncope
Txt: cardiac catheter technique of balloon
valvuloplasty direct surgical relief of moderate
and severe obstruction
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Complex Congenital Heart Lesions
Tetralogy of Fallot
Complete Transposition of the Great Arteries
Single Ventricle
Tricuspid Atresia
Ebstein Anomaly
Congenitally Corrected Transposition Malpositions of the heart
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Tetralogy of Fallot
Four components:
a. Malaligned ventricular septal defect
b. Obstruction to RV outflow
c. Aortic override of the ventricular septal
defect
d. RV hypertrophy
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Tetralogy of Fallot
SSx: pulmonary blood flow markedly reduced,
large volume of desaturated systemic venous
blood is shunted from right to left across the
ventricular septal defect, severe cyanosis &erythrocytosis, symptoms of systemic hypoxemia
Chest x-ray: coeur en sabot(boot-shaped heart)
with prominent right ventricle and a concavity in
the pulmonary conus region
Txt: reoperation, interventional catheterization
C l t T iti f th G t
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Complete Transposition of the Great
Arteries
A.k.a. dextro- or D-transposition of the greatarteries
Aorta arises rightward anteriorly from the RV, andthe pulmonary artery emerges leftward and
posteriorly from the LV, resulting in 2 separateparallel circulations
More common in males
Txt: balloon or blade catheter or surgical creation
or enlargement of an interatrial communication,systemic-pulmonary artery anastomosis,rearranging venous returns
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Single Ventricle: both AV valves or a common AV
valve opening to a single ventricular chamber
- Modifications of the Fontan approach
Tricuspid Atresia: characterized by atresia of the
tricuspid valve, an interatrial communication, andhypoplasia of the RV & pulmonary artery.
Dominated by severe cyanosis.
- Txt: atrial septostomy & palliative operations to
increase blood flow, Fontan atriopulmonary or total
cavopulmonary connection
Eb t i l d d di l t f
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Ebstein anomaly: downward displacement of
the tricuspid valve into the RV due to
anomalous attachments of the tricuspid
leaflets. Results in tricuspid regurgitation.
- Txt: prosthetic replacement of the tricuspid
valve
Congenitally corrected transposition:
transposition of the ascending aorta &pulmonary trunk & inversion of the ventricles
f
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Malpositions of the heart:
a. dextocardia: cardia apex at right side of the
chestb. Mesocardia: midline
c. Isolated levocardia: normal location but
abnormal position of the viscera
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OT Management
Stress management techniques
IADLs
Coping techniques
Lifestyle modifications
Recommended