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MEDICINE (CARDIOLOGY)

Topic: Congenital Heart Disease

Lecturer: Dr. Jerelyn Adviento Reference: Harrisons’s Principles of Internal Medicine

CONGENITAL HEART DISEASE IN THE ADULT

Cardiac Development

CHD is generally the result of aberrant embryonic development of a normal structure or failure of such structure to progress beyond an early stage of embryonic or fetal development.

Cardiogenesisfinely tuned process with transcriptional control of a complex group of regulatory proteins that activate or inhibit their gene targets in a location- and time-dependent manner

Specific Cardiac Defects Simple defects= single lesions with a shunt or a valvular malformation

MEDICINE (CARDIOLOGY) Topic: Congenital Heart Disease Lecturer: Dr. Jerelyn Adviento Reference: Harrisons’s Principles of Internal Medicine

Intermediate defects= have two or more simple defects

MEDICINE (CARDIOLOGY) Topic: Congenital Heart Disease Lecturer: Dr. Jerelyn Adviento Reference: Harrisons’s Principles of Internal Medicine

Complex defects= intermediate defect plus more complex cardiac and vascular anatomy, often with cyanosis, and frequently with transposition complexes

MEDICINE (CARDIOLOGY) Topic: Congenital Heart Disease Lecturer: Dr. Jerelyn Adviento Reference: Harrisons’s Principles of Internal Medicine

Atrial Septal Defect

Common cardiac anomaly

Occurs frequently in females

Sinus venosus ASD

o

occurs high in the atrial septum near the entry

of the superior vena cava into the right atrium

o

associated frequently with anomalous pulmonary venous connection from the right lung to the superior vena cava or right atrium

Ostium primum ASD

 

o

lie adjacent to the atrioventricular valves, either of which may be deformed and regurgitant

o

common in Down’s syndrome, often as part of complex AV septal defects with a common AV valve and a posterior defect of the basal portion of the interventricular septum

Ostium segundum ASD

o

Most commonly involves the fossa ovalis and is midseptal in location

ASD denotes a true deficiency of the atrial septum and implies functional and anatomic patency  Factors affecting left-to-right shunt

 

o

ASD size

o

Ventricular diastolic properties

o

Relative impedance

Left-to-right shunt causes diastolic overloading of the RV and increased pulmonary blood flow

Patients are asymptomatic in early life

Cardiorespiratory symptoms occur in many older patients

Beyond 4 th decadeatrial arrhythmias, pulmonary arterial hypertension, right heart failure

In older patients, left-to-right shunting across the ASD increases as progressive systemic hypertension and/or coronary artery disease (CAD) result in reduced compliance of the LV

Physical Examination

prominent RV impulse

palpable pulmonary artery pulsation

First heart sound

 

o

normal or split, with accentuation of the tricuspid valve closure sound

o

midsystolic pulmonary outflow murmur Increased flow across the pulmonic valve

Second heart sound

 

o

widely split and is fixed in relation to respiration

o

mid-diastolic rumbling murmur, loudest at the fourth intercostal space and along the left sternal border reflects increased flow across the tricuspid valve

In ostium primum ASD

 

o

an apical holosystolic murmur indicates associated mitral or tricuspid regurgitation or a ventricular septal defect (VSD)

Increased pulmonary vascular resistance causes diminution of the left-to-right shunt

 

o

pulmonary outflow and tricuspid inflow murmurs decrease in intensity

o

pulmonic component of the second heart sound and a systolic ejection sound are accentuated

o

diastolic murmur of pulmonic regurgitation appears

Cyanosis and clubbing accompany the development

of a right-to-left shunt adults with an ASD and atrial fibrillation

 

o

findings may be confused with mitral stenosis with pulmonary hypertension

o

tricuspid diastolic flow murmur and widely

split second heart sound may be mistakenly thought to represent the diastolic murmur of mitral stenosis and the mitral “opening snap,” respectively

Electrocardiogram

Ostium segundum ASD

o

Shows right-axis deviation

o

rSr´ pattern in the right precordial leads representing enlargement of the RV outflow tract

Sinus venous ASD

o

Ectopic atrial pacemaker or first-degree heart block

Ostium primum ASD

o

o

Left superior axis deviation

Counterclockwise rotation of the frontal plane QRS loop

Varying

degrees

of

RV

and

right

atrial

(RA)

enlargement or hypertrophy may occur with each

type

of

defect,

depending

on

presence

the

and

degree of pulmonary hypertension

CXR: enlarged RA and RV and pulmonary artery and its branches

Echocardiogram

reveals pulmonary arterial and RV and RA dilatation with abnormal (paradoxical) ventricular septal motion in

the presence of a significant right heart volume overload Visualization of the ASD

 

o

Two-dimensional imaging

o

Color-flow imaging

o

Echocontrast

Echocardiography and Doppler examination

 

supplanted cardiac catheterization Transesophageal echocardiography

 

o

indicated if the transthoracic echocardiogram is ambiguous - the case with sinus venosus defects, or

for guiding catheter device closure Cardiac catheterization

 
 

o

Criteria:

 

inconsistencies exist in the clinical data,

significant pulmonary hypertension or associated malformations are suspected,

CAD is a possibility

when attempting transcatheter closure of the ASD

Treatme

ATRIAL SEPTAL DEFECT

 

nt

Operative Repair

usually with a patch of pericardium or of prosthetic

material or percutaneous transcatheter device closure Pulmonary vasodilators

reduction in pulmonary artery pressure and resistance may allow closure of ASD in patients with

 

pulmonary vascular disease

 

Patients with sius venosus or ostium secundum

o

Medical management should include prompt treatment of respiratory tract infections; antiarrhythmic medications for atrial fibrillation or supraventricular tachycardia; and the usual measures for hypertension, coronary disease, or heart failure

 reveals pulmonary arterial and RV and RA dilatation with abnormal (paradoxical) ventricular septal motion in

Ventricular Septal Defect

One of the most common of all cardiac birth defects

Usually single or situated in the membranous or midmuscular portion of the septum

Functional disturbance depends on its size and on the status of the pulmonary vascular bed

Small-or moderate-size VSDs adulthood

Large VSDearly in life

VSD Spectrum

o

Spontaneous closure

More common in patients born with a small VSD

occurs in early childhood in most

o

Congestive cardiac failure

o

Death

o

Development of pulmonary vascular obstruction

Principal determinant of the clinical manifestations and course of a given VSD and feasibility of surgical repair

o

RV outflow tract obstruction

Results to increased pulmonary arterial pressure

Result of obstructive, obliterative structural changes within the pulmonary vascular bed

develops in ~5–10% of patients who present in infancy with a moderate to large left-to-right shunt

o

Aortic regurgitation

~5% of patients, results from insufficient cusp tissue or prolapse of the cusp through the

interventricular defect

o

Infective endocarditis

Eisenmenger’s syndrome

 
 

o

patients with a large communication between the two circulations at the aortopulmonary, ventricular,

o

or atrial levels and bidirectional or predominantly right-to-left shunts because of high resistance and obstructive pulmonary hypertension exertional dyspnea, chest pain, syncope and hemoptysis

o

pregnancy is contraindicated

large VSDs and pulmonary hypertension greatest risk for developing pulmonary vascular disease

right-to-left shunt leads to cyanosis, clubbing and erythrocytosis

Echocardiography with spectral and color Doppler examination

defines the number and location of defects in the ventricular septum and associated anomalies and the

 

hemodynamic physiology of the defects

 

Hemodynamic and angiographic study

may be occasionally required to assess the status of the pulmonary vascular bed and clarify details of the

 

altered anatomy

 

computed tomography (CT) or magnetic resonance imaging (MRI)

useful in delineating complex anatomy and assessing extracardiac structures

Treatme

VENTRICULAR SEPTAL DEFECT

 

nt

Closure

 

Not recommended for patient with normal pulmonary arterial pressures with small shunts (pulmonary-to-

 

systemic flow ratios of <1.5:1)

 

Operative correction or transcatheter closure

indicated when there is a moderate to large left-to-right shunt with a pulmonary-to-systemic flow ratio

 

>1.5:1

 

in the absence of prohibitively high levels of pulmonary vascular resistance (pulmonary arterial

resistance is less than two-thirds of systemic arterial resistance)

Patent Ductus Arteriosus (PDA)

Ductus Arteriosus: a vessel leading from the bifurcation of the pulmonary artery to the aorta just distal to

the left subclavian artery Vascular channel is open in the fetus but closes immediately after birth

In most adults, pulmonary pressures are normal, and a gradient and shunt from aorta to pulmonary artery

persist throughout the cardiac cycle, resulting in a characteristic thrill and a continuous “machinery” murmur with late systolic accentuation at the upper left sternal edge Adult with large left-to-right shunt through ductus arteriosus

o pulmonary vascular obstruction (Eisenmenger’s syndrome) with pulmonary hypertension, right-to-left shunting, and cyanosis have usually developed Severe pulmonary vascular disease results in reversal of flow through the ductus;

unoxygenated blood is shunted to the descending aorta;

the toes—but not the fingers—become cyanotic and clubbed, a finding termed differential cyanosis

Cardiac Failure and Infective Endocarditis leading causes of death in adults with PDA

Severe

Pulmonary

Vascular

Obstruction:

may

cause aneurysmal dilatation, calcification, and rupture of the ductus

 Principal determinant of the clinical manifestations and course of a given VSD and feasibility of

Aortic Root-to-Right Shunts Three most common causes:

(1) congenital aneurysm of an aortic sinus of Valsalva with fistula

(2) coronary arteriovenous fistula (3) anomalous origin of the left coronary artery from the pulmonary trunk Aneurysm of an aortic sinus of Valsalva

separation or lack of fusion between the media of the aorta and the annulus of the aortic valve

Rupture occurs in the third or fourth decade of life

Aorticocardiac fistula is between the right coronary cusp and the RV; but occasionally, when the noncoronary

cusp is involved, the fistula drains into the RA Abrupt rupture causes chest pain, bounding pulses, a continuous murmur accentuated in diastole, and volume overload of the heart

Diagnosis:

2-D and Doppler echocardiographic studies

Cardiac catheterization- quantifies left-to-right shunt

Thoracic aortography- visualizes fistula

Medical managementdirected at cardiac failure, arrhythmias or endocarditis

At operation, the aneurysm is closed and amputated, and the aortic wall is reunited with the heart, either by direct suture or with a patch or prosthesis

Coronary Arteriovenous Fistula

Unusual anomaly

consists of a communication between a coronary artery and another cardiac chamber, usually the coronary

if shunt is large, there may be a coronary “steal” syndrome with myocardial ischemia and possible angina or

sinus, RA, or RV shunt is usually of small magnitude, and myocardial blood flow is not usually compromised

ventricular arrhythmias PE: loud, superficial, continuous murmur at the lower or midsternal border usually prompts a further evaluation of asymptomatic patient

Diagnosis:

 

Doppler echocardiography: demonstrate site of drainage

2D echocardiography: detected if site of origin is proximal

Angiography (classic catheterization, CT, or MRI): permits identification of the size and anatomic features

of the fistulous tract, may be closed by suture or transcatheter obliteration

Anomalous origin of the LCA from the pulmonary artery

oxygenated blood from the aortic root flows via a dilated RCA and collaterals to the LCA and retrograde to the

lower pressure pulmonary artery circulation via the anomalous LMCA (which emerges from the pulmonary artery) Diagnosis: ECG findings of an anterolateral myocardial infarction and left ventricular hypertrophy (LVH)

Management: coronary artery reimplantation, coronary artery bypass with an internal mammary artery graft, or saphenous vein-coronary artery graft

Congenital Aortic Stenosis

Malformation that cause obstruction to LV outflow

 
  • (a) Congenital valvular aortic stenosis

  • (b) Discrete subaortic stenosis

  • (c) Supravalvular aortic stenosis

Bicuspid aortic valves are more common in males than in females

Congenital bicuspid aortic valveinitially function normal, most common congenital malformations of the

o

congenitally deformed, rigid aortic valve commonly lead to thickening of the cusps and, in later life, to

heart and may go undetected in early life May develop stenosis or regurgitation with time or be the site of infective endocarditis= difficult to distinguish

from acquired rheumatic or degenerative calcific aortic valve disease

calcification Obstruction = concentric hypertrophy of the LV wall

Diagnosis: Echocardiography – reveals morphology of the aortic valve and aortic root and quantitates

severity of stenosis or regurgitation

 

Treatme

VALVULAR AORTIC STENOSIS

nt

Patient with diminished cardiac reserve

Administration of Digoxin, Diuretics and Sodium restriction while awaiting operation

Dilated aortic root

 

Beta blockers, ARBs, or ACE inhibitors

Aortic valve replacement

 

Indicated in adults with critical obstruction

i.e., aortic valve area <0.45 cm2/m2, with symptoms secondary to LV dysfunction or myocardial ischemia,

 

or with hemodynamic evidence of LV dysfunction

Aortic balloon valvuloplasty

 
 

asymptomatic children or adolescents or young adults with critical aortic stenosis without valvular

calcification or these features If surgery is contraindicated in older patients because of a complicating medical problem such as

malignancy or renal or hepatic failure, balloon valvuloplasty may provide short-term improvement Serve as bridge to aortic valve replacement in patients with severe HF

Transcatheter aortic valve replacement

Potential alternative to surgery

Subaortic Stenosis

discrete form is consists of a membranous diaphragm or fibromuscular ring encircling the LV outflow tract just

beneath the base of the aortic valve. The jet impact from the subaortic stenotic jet on the underside of the aortic valve often begets progressive aortic valve fibrosis and valvular regurgitation.

Diagnosis:

 

Echocardiography demonstrates the anatomy of the subaortic obstruction

Doppler studies show turbulence proximal to the aortic valve and can quantitate the pressure gradient and

severity of aortic regurgitation Treatment: complete excision of the membrane or fibromuscular ring Supravalvular Aortic Stenosis

localized or diffuse narrowing of the ascending aorta originating just above the level of the coronary arteries at

the superior margin of the sinuses of Valsalva the coronary arteries are subjected to elevated systolic pressures from the LV, are often dilated and tortuous,

and are susceptible to premature atherosclerosis. The coronary ostia may also become obstructed by the aortic valve leaflets

a genetic defect is located in chromosome region as elastin on chromosome 7

most commonly associated cardiac defect in Williams-Beuren syndrome

“elfin” facies

low nasal bridge

cheerful demeanor

mental retardation with retained language skills and love of music

supravalvular aortic stenosis, and

transient hypercalcemia

Coarctation of the Aorta

Narrowing or constriction of the lumen of the aorta

may occur anywhere along its length but is most common distal to the origin of the left subclavian artery near

the insertion of the ligamentum arteriosum occurs in ~7% of patients with CHD

more common in males than females,

particularly frequent in patients with gonadal dysgenesis (e.g., Turner’s syndrome).

Clinical manifestations depend on: (1) site and (2) extent of obstruction and (3) the presence of associated

cardiac anomalies, most commonly a bicuspid aortic valve. Circle of Willis aneurysms may occur in up to 10%

Headache, epistaxis, chest pressure, and claudication with exercise may occur

Physical Examination

             attention is usually
attention is usually directed to the cardiovascular system when a heart murmur or hypertension in the upper
extremities and absence, marked diminution, or delayed pulsations in the femoral arteries are detected on
physical examination
Enlarged and pulsatile collateral vessels may be palpated in the intercostal spaces anteriorly, in the axillae, or
posteriorly in the interscapular area
A midsystolic murmur over the left interscapular space may become continuous if the lumen is narrowed
sufficiently to result in a high-velocity jet across the lesion throughout the cardiac cycle
Additional systolic and continuous murmurs over the lateral thoracic wall may reflect increased flow through
dilated and tortuous collateral vessels
ECG  LV hypertrophy
Chest X-ray dilated left subclavian artery high on the left mediastinal border and a dilated ascending aorta
Indentation of the aorta at the site of coarctation and pre- and poststenotic dilatation (the “3” sign) along the
left paramediastinal shadow are essentially pathognomonic
Notching of the third to ninth ribs- radiographic sign-due to inferior rib erosion by dilated collateral vessels
2D-echocardiography from suprasternal windows identifies the site of coarctation
Doppler quantitates the pressure gradient
Transesophageal echocardiography and MRI or CT allow visualization of the length and severity of the
obstruction and associated collateral arteries.
Cardiac catheterization: indicated primarily to evaluate the coronary arteries or to perform catheter-based
intervention (angioplasty and stent of the coarctation) in adults
Chief hazards of proximal aortic severe hypertension
  • (a) cerebral aneurysms and hemorrhage

  • (b) aortic dissection and rupture

  • (c) premature coronary arteriosclerosis

  • (d) aortic valve failure

  • (e) LV failure;

infective

endarteritis

may

occur

on

the

coarctation

site

or

endocarditis may settle on an associated bicuspid aortic valve (~50% of patients)

 

Treatme

COARCTATION OF THE AORTA

     

nt

Surgical or involves percutaneous catheter balloon

 

dilatation with stent placement high-quality MRI or CT procedure in follow-up

Pulmonary Stenosis with Intact Ventricular Septum

Obstruction to RV outflow may be localized to the supravalvular, valvular, or subvalvular levels or occur at

a combination of these sites Multiple sites of narrowing of the peripheral pulmonary arteries are a feature of rubella embryopathy and may

occur with both the familial and sporadic forms of supravalvular aortic stenosis Valvular pulmonic stenosis (PS): most common form of isolated RV obstruction

Severity of the obstructing lesionmost important determinant of the clinical course

In the presence of a normal cardiac output

o

<30 mmHg indicates mild PS generally asymptomatic and demonstrate little or no progression in the severity of obstruction

o

with age >50 mmHg indicates severe PS

Symptoms vary with the degree of obstruction Fatigue, dyspnea, RV failure, and syncope may limit the activity of older patientsmoderate or severe

o

o

obstruction may prevent an augmentation of cardiac output with exercise patients with severe obstructionthe systolic pressure in the RV may exceed that in the LV, because

o

the ventricular septum is intact moderate or severe stenosis

RV ejection is prolonged

the sound of pulmonary valve closure is delayed and soft

o

RV hypertrophy reduces the compliance of that chamber, and a forceful RA contraction is necessary to

o

o

o

augment RV filling A fourth heart sound

prominent a waves in the jugular venous pulse

occasionally, presystolic pulsations of the liver reflect vigorous atrial contraction

Clinical diagnosis

o

o

left parasternal lift and harsh systolic crescendo-decrescendo murmur

thrill at the upper left sternal border, typically preceded by a systolic ejection sound if the obstruction

o

is due to a mobile nondysplastic pulmonary valve holosystolic murmur of tricuspid regurgitation may accompany severe PS, especially in the presence of

o

o

o

congestive heart failure Cyanosisright-to-left shunting through a patent foramen ovale or ASD

Supravalvular or peripheral pulmonary arterial stenosismurmur is systolic or continuous and is best

heard over the area of narrowing, with radiation to the peripheral lung fields In mild cases,

o the ECG is normal, whereas moderate and severe stenoses are associated with RV hypertrophy chest x-ray with mild or moderate PS shows a heart of normal size with normal lung vascularity.

Pulmonary valvular stenosis, dilatation of the main and left pulmonary arteries occurs in part due to the direction of the PS jet and in

o

part due to intrinsic tissue weakness

severe obstructionRV hypertrophy

The pulmonary vascularity may be reduced with…

o

o

o

severe stenosis,

RV failure, and/or

right-to-left shunt at the atrial level.

2D/3D echocardiographyvisualizes pulmonary valve morphology

Doppler echocardiographyquantify the outflow tract pressure gradient (Fig. 282-4)

Treatme PULMONARY STENOSIS nt Cardiac catheter technique of balloon valvuloplasty  effective, and the surgery is

Treatme

PULMONARY STENOSIS

nt

Cardiac catheter technique of balloon valvuloplasty

effective, and the surgery is rarely necessary

Transcatheter Angioplasty or Stenting

Treats effectively the Multiple stenoses of the peripheral pulmonary arteries

TETRALOGY OF FALLOT

Four components:

  • 1. Malaligned VSD

  • 2. Obstruction to RV outflow

o

Severity Determines the clinical presentation

o

Severity of hypoplasia of the tract varies from mild to complete (pulmonary atresia)

  • 3. Aortic override of the VSD

  • 4. RV hypertrophy

o

due to the RV’s response to aortic pressure via the large VSD

Pulmonary valve stenosis and supravalvular and peripheral pulmonary arterial obstruction may coexist

Unilateral absence of a pulmonary artery -usually the left(RARE)

Severe RV outflow obstruction

o

o

Right-sided aortic arch and descending thoracic aorta occur in ~25%

pulmonary blood flow is reduced

large volume of desaturated systemic venous blood shunts right-to-left across the VSD

o

Severe cyanosis and erythrocytosis

o

symptoms of systemic hypoxemia are prominent

In many infants and children, the obstruction is mild but progressive

Diagnosis:

 

ECG showsRV hypertrophy

Chest x-ray shows a normal-sized, boot-shaped heart (coeur en sabot) with a prominent RV and a concavity in the

o

o

region of the pulmonary conus Pulmonary vascular markings are typically diminished, and the aortic arch and knob may be on the right

 

side

Echocardiography

o

o

demonstrates the malaligned VSD with the overriding aorta

the site and severity of PS, which may be subpulmonic (fixed or dynamic), at the pulmonary valve or in the

 

main or branch pulmonary arteries

Classic contrast angiography may provide details regarding the RV outflow tract, pulmonary valve and annulus, and caliber of the main

o

 

branches of the pulmonary artery, as well as about possible associated aortopulmonary collaterals.

Coronary arteriography identifies the anatomy and course of the coronary arteries, which may be anomalous

o

Cardiac MRI and CT complement echocardiography

o

o

provide much of the information gathered by angiography as well as additional functional information.

MRI is considered the clinical gold standard for quantification of RV volume and function as well as

quantification of the pulmonary regurgitation severity

Treatme

Tetralogy of Fallot

nt

Reoperation in adults is most commonly for

severe pulmonary regurgitation or pulmonary stenosis Ventricular and atrial arrhythmias occur in

15% and 25% of adults, respectively, and require medical treatment

  • 1. electrophysiologic study and ablation

  • 2. defibrillator placement, or

  • 3. transcatheter or surgical intervention

  • 4. pulmonary valve replacement

Transcatheter pulmonary valve replacement is widely used in patients meeting anatomic criteria

COMPLETE TRANSPOSITION OF THE GREAT ARTERIES

commonly called dextro- or D-transposition of the great arteries

 

aorta arises rightward anteriorly from the RV, and

 

the pulmonary artery emerges leftward and posteriorly from the LV, which results in two separate parallel circulations Most patients have an interatrial communication

 
 

o

two-thirds have a patent ductus arteriosus

 

o

about one-third have an associated VSD

More common in males and accounts for ~10% of cyanotic heart disease

 

course is determined by:

  • 1. the degree of tissue hypoxemia

 
  • 2. the ability of each ventricle to sustain an increased workload in the presence of reduced coronary arterial

 

oxygenation

 
 
  • 3. the nature of the associated cardiovascular anomalies

 
  • 4. the status of the pulmonary vascular bed

 

Patients who do not undergo surgical palliation generally do not survive to reach adulthood

 

By

the

third

decade

of

life,

~30%

of

patients

with

“atrial switch” operations will have developed decreased RV function and progressive tricuspid regurgitation, which may lead to congestive heart failure

Pulmonary

vascular

obstruction

develops

by

1–2

years

of age in patients with an associated large VSD or large patent ductus arteriosus in the absence of obstruction

to LV outflow

 

Treatme

 

Transposition of the Great Arteries

nt

 

Balloon or blade catheter or surgical creation or enlargement of an interatrial communication in the neonate simplest procedure for providing increased intracardiac mixing of systemic and pulmonary venous blood Systemic pulmonary artery anastomosis may

 

be indicated in the patient with severe obstruction to LV outflow and diminished pulmonary blood flow

 

Intracardiac repair accomplished by rearranging the venous

 

returns

(intraatrial

switch,

i.e.,

Mustard

or

 

Senning operation) the systemic venous blood is directed to the

 

mitral valve and, thence, to

the

LV and

 

pulmonary artery, while the pulmonary venous blood is diverted through the tricuspid valve and RV to the aorta late survival after repairs is good

arrhythmias (e.g., atrial flutter) or conduction

 

defects (e.g., sick sinus syndrome) occur in

~50%

of patients

by

30

years

after the

intraatrial

switch surgery

 

SINGLE VENTRICLE

a family of complex lesions with both atrioventricular valves or a common atrioventricular valve opening to a single

ventricular chamber Associated anomalies

 

o

abnormal great artery positional relationships

o

pulmonic valvular or subvalvular stenosis subaortic stenosis

o Modifications of the Fontan approach are generally applied to carefully selected patients with creation of a pathway(s) from

the systemic veins to the pulmonary arteries

TRICUSPID ATRESIA

Characterized by atresia of the tricuspid valve; an interatrial communication; and, frequently, hypoplasia of the RV and pulmonary artery Severe cyanosis due to obligatory admixture of systemic and pulmonary venous blood in the LV ECG: (1) RA enlargement, (2) left-axis deviation, and (3) LV hypertrophy Atrial septostomy and palliative operations to increase pulmonary blood flow, often by anastomosis of a systemic artery or vein to a pulmonary artery, may allow survival to the second or third decade Fontan atriopulmonary or total cavopulmonary connection: allow functional correction in patients with normal or low pulmonary arterial resistance pressure and good LV function Cosiderations:

o

  • 1. development of arrhythmias

  • 2. progressive liver dysfunction

  • 3. thromboembolic complication

  • 4. potential long-term need for heart or heart and liver transplantation

EBSTEIN’S ANOMALY

downward displacement of the tricuspid valve into the RV, due to anomalous attachment of the tricuspid leaflets, the Ebstein

tricuspid valve tissue is dysplastic and results in tricuspid regurgitation Clinical manifestations are variable:

(1)

progressive cyanosis from right-to-left atrial shunting

(2)

symptoms due to tricuspid regurgitation and RV dysfunction

(3) paroxysmal atrial tachyarrhythmias with or without atrioventricular bypass tracts (Wolff-Parkinson-White [WPW]

 

syndrome)

Diagnostic Findings two-dimensional echocardiography: abnormal positional relation between the tricuspid and mitral valves with

o

 

o

abnormally increased apical displacement of the septal tricuspid leaflet Doppler examination: Tricuspid regurgitation is quantitated

Surgical approaches include prosthetic replacement of the tricuspid valve when the leaflets are tethered or repair of the

 

native valve

Two fundamental anatomic abnormalities Transposition of the ascending aorta and pulmonary trunk

o

 

o

Inversion of the ventricles

This arrangement results in desaturated systemic venous blood passing from the RA through the mitral valve to the LV and into the pulmonary trunk, whereas oxygenated pulmonary venous blood flows from the left atrium through the tricuspid valve to the RV and into the aorta

Progressive RV dysfunction and tricuspid regurgitation may also develop in one-third of patients by age 30

 

VSD or PS due to obstruction to outflow from the right-sided subpulmonary (anatomic left) ventricle may coexist

Complete

heart

block

occurs

at

a

rate

of

2–10%

per

decade

Diagnosis

of

the

malformation

and

associated

lesions

can

be established by comprehensive two-dimensional echocardiography and Doppler examination.

 

MALPOSITIONS OF THE HEART

 

Dextrocardia: is in the right side of the chest Mesocardiac: cardiac apex is at the midline Isolated levocardia: there is a normal location of the heart in the left side of the chest but abnormal position of the viscera

 

SURGICALLY MODIFIED CONGENITAL HEART DISEASE

 

Proper care of the survivor of an operation for CHD

 

Requires that the clinician understand the details of the malformation before operation

 

Pay

meticulous

attention

to

the

details

of

the

operative

procedure

Recognize

the

postoperative

residua

(conditions

left

totally or partially uncorrected), the sequelae (conditions caused by surgery), and the complications that may have resulted from the operation

Cardiac operations involving the atria:

May be followed

  • a. closure of ASD

years later by

  • b. repair of total or partial anomalous pulmonary venous return

sinus node or atrioventricular

  • c. venous switch corrections of complete transposition of the great arteries (the Mustard or Senning operations),

node dysfunction and/or by atrial arrhythmias (especially atrial

flutter)

Intraventricular surgery may also result in electrophysiologic consequencescomplete heart block necessitating pacemaker

insertion to avoid sudden death After repair of the ostium primum ASDcleft mitral valve may become progressively regurgitant

Postoperative patient with tetralogy of FallotTricuspid regurgitation may also be progressive if RV outflow tract obstruction

was

not

relieved

adequately

at

initial

surgery

 

Use of prosthetic valves, patches, or conduits in the operative repair Special risks: infective endocarditis, thrombus formation, and premature degeneration and calcification of the

o

prosthetic materials The effect of pregnancy in postoperative patients depends on the outcome of the repair, including the presence and severity of residua, sequelae, or complications

Tubal

ligation

should

be

considered

in

those

in

whom

pregnancy is strictly contraindicated

Endocarditis Prophylaxis

Two major predisposing causes:

  • 1. cardiovascular substrate

  • 2. source of bacteremia

Prophylaxis includes both antimicrobial and hygienic measures

Routine antimicrobial prophylaxis is recommended for bacteremic dental procedures or instrumentation through an infected site

in most patients with operated CHD, particularly if foreign material, such as a prosthetic valve, conduit, or surgically constructed shunt, is in place

In

the

case

of

patches,

in

the

absence

of

a

high-pressure

patch

leak, or transcatheter devices, prophylaxis is usually recommended for 6 months until there is endothelialization

Individuals with unrepaired cyanotic heart disease are also generally recommended to receive prophylaxis