mf3 - congenital heart disease in adult

7/30/2019 MF3 - Congenital Heart Disease in Adult

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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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>4th decade: atrial arrhythmias, pulmonary

arterial hypertension, bidirectional and then

right-to-left shunting


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Types:

Sinus venosus type

Ostium primum type

Ostium secundum type


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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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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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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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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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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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- 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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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

mf3 - congenital heart disease in adult

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