Good afternoon

SEMINAR ON

CYANOTIC HEART

DISEASES

Presented by:

B.Kalpana

M.Sc (N) 2nd yeara

Cyanotic heartdiseases

EMBRYOLOGY OF HEART

LOCATION OF CARDIOGENIC AREA

1. Neural plate2. Primitive node3. Primitive streak4. Cut edge of amnion5. Angiogenetic cell clusters

 

19 daysCardiogenic area

Formation of endocardial tubes - 20 days

Formation of primitive tube-21 days

Development of regions in primitive heart Tube -22 days

FORMATION OF CARDIAC LOOP -23 DAYS

BENDING OF PRIMITIVE HEART TUBE-24 DAYS

NORMAL : Loop to the RIGHT: Levocardia!

ABNORMAL: Loop to the LEFT: Dextrocardia!

VENTRICULAR LOOPING END WEEK 4

FROM TUBE TO FOUR CHAMBERSINTERNAL VIEW

ENDOCARDIAL CUSHION: 80 DAYS

HEART FORMATION CON’T Weeks 5-8

SVC, IVC formRight and Left Atrium divideVentricles start to formAorta and pulmonary arteries

Weeks 8-12Ventricles and mitral and tricuspid

valvesAorta and pulmonary artery, aortic

arch Week 12:Fetal Circulation begins

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

CYANOTIC HEART DISEASE

Cyanotic heart disease is a heart

defect, present at birth

(congenital), that results in low

blood oxygen levels. There may be

more than one defect.

CYANOSIS IN CHILDREN

Central cyanosis:

Cyanosis of the tongue,mucous membranes and peripheral skin, it is necessary to have >3g/dl of reduced Hb to have it.

Peripheral cyanosis:

It is visible only in the skin of the extremities with normal arterial saturation due to vasomotor instability,ex. cold environment.

CAUSES OF CENTRAL CYANOSIS B) LUNG DISEASE D) CNS DEPRESSION a)      RDS a) IVHb)      Pneumonia b) Perinatal asphyxiac)      Pneumothorax c) Heavy maternal sedationd)      Pleural effusion e)      Diaphragmatic herniaf)       T.E.Fistula

 C) PERSISTENT PULMONARY E) MISCELLANOUS HYPERTENSION a) shock & sepsis b) Hypoglycemia c) Methemoglobinemia d) Neuromuscular conditions ( Werdnig – Hoffman)

RADIOLOGICAL FEATURES

CXR may exclude non cardiac causes of cyanosis e.g. RDS. . Meconium aspiration, Diaphgramatic hernia, Pneumothorax

Pulmonary Vascular Markings

Decreased Increased

Heart Size Heart Size

Normal Increased Increased( “Boot shaped”) (“ Wall-to-Wall”) TOF Ebstein (“ egg-on-end”) D-TGA Aortic Arch \ Mediastinum

Abdominal Situs

AUSCULTATION

HEART SOUNDS: 

S1 is normally accentuated in newborns

 S2 split is normally heard as a slurring

rather than a distinct split.S2 is single in many cyanotic lesions

especially in, D-TGA, TOF.

 S2 is widely split in TAPVR, Critical PS.

 

MURMURS SYSTOLIC EJECTION MURMURS May be heard in the first hours of life. Usually due to ventricular obstruction e.g. AS., PS., TOF  DIASTOLIC MURMURS: Rarely heard in newborns Early diastolic murmurs heard in Truncus arteriosus , TOF with absent pulmonary valve  Continuous: Continuous murmurs are caused by AV fistulas (not PDA) ABSENT: Silent hearts often characteristic of Tricuspid atresia ,

Pulmonary atresia & D-TGA. 

1.Look for cyanosis and dysmorphology

Examination of CVS in the newborn

Feel the pulsesespecially brachial and femoral

Look at the respiratory pattern and for evidence of recession

Feel the precordium for hyperactivityand for thrills

Locate the apex beat

Feel for hepatomegally

Listen carefully

Do what is necessary to calm the baby down!

Listen at the back for radiation of murmurs

3 months old- severe failure to thrive

Special tests-echocardiography

PULMONARY VEINS - COLOUR DOPPLER

Subcostal view

Apical view

TETRALOGY OF FALLOT

TETRALOGY OF FALLOT (TOF)

Named by Etienne-Louis Arthur Fallot in 1888

Approximately 10% of all complex CHD Single developmental error of the terminal

portion of the spiral truncoconal septum Four distinct components: subpulmonic

stenosis, VSD, overriding aorta, and RV hypertrophy

Often accompanied by other anomalies

DEFINITION

Tetralogy of Fallot is the result of unequal division of the conus. Four alterations result: Narrower right ventricle outflow region Defect in the interventricular septum An aorta that overrides directly above the septal

defect The right ventricular wall hypertrophies.

FOUR MALFORMATIONS IN TOF

A.Pulmonic stenosis

B.Overriding aorta

C.Ventricular septal defect (VSD)

D.Right ventricular hypertrophy

CAUSES

UnknownMethylene tetrahydrofolate

reductase gene (Portugal study)Maternal rubella during pregnancyDown syndromeCATCH 22(cardiac defects, thymic

hypoplasia,cleft palate, hypocalcemia)

Right ventricular out flow tract obstruction

Deletion of chromosome band 22q11

SIGNS AND SYMPTOMS

Cyanosis-blue color to the skin, which gets worse when the baby is upset

Squating position

Clubbing of fingers

CONT…………..

Failure to gain weightPoor developmentDifficulty in feeding

PATHOPHYSIOLOGY

Pulmonary stenosisA narrowing of the right ventricular

outflow tract and occur at the pulmonary valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis).

Caused by overgrowth of the heart muscle wall (hypertrophy of the septo parietal trabeculae).

These events finally leads to overriding of aorta.

OVERRIDING OF AORTA

An aortic valve with biventricular connection,

that is, it is situated above the ventricular

septal defect and connected to both the right

and the left ventricle.

The degree to which the aorta is attached to

the right ventricle is referred to as its degree

of "override."

The aortic root can be displaced toward the

front (anteriorly) or directly above the septal

defect, but it is always abnormally located to

the right of the root of the pulmonary artery.

VENTRICULAR SEPTAL DEFECT

A hole between the two bottom chambers (ventricles) of the heart.

The defect is centered around the most superior aspect of the ventricular septum (the outlet septum), and in the majority of cases is single and large.

In some cases thickening of the septum (septal hypertrophy) can narrow the margins of the defect.[

RIGHT VENTRICULAR HYPERTROPHY

The right ventricle is more muscular than normal, causing a characteristic boot-shaped (coeur-en-sabot) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increases in size to deal with the increased obstruction to the right outflow tract.

This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy generally increases with age.

ADDITIONAL ANAMOLIES

stenosis of the left pulmonary artery, 40% of patients a bicuspid pulmonary valve, 40% of patients right-sided aortic arch, 25% of patients coronary artery anomalies, 10% of patients a foramen ovale or atrial

septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot,

atrioventricular septal defectpartially or totally anomalous pulmonary venous returnforked ribs and scoliosis.

DIAGNOSIS OF TETRALOGY OF FALLOT

History –cyanosis,feeding Physical examination:

Single accent. S2 ESM

  CXR:

boot shaped heart (coeur en sabot)

ECG: RVH Hematocrit-elevated ABG and Oximetry

CONTD…

Echocardiography-ductus arteriosus, VSD,ASD

MRI- belineation of Aorta, RVOT,VSD RV hypertrophy

Cardiac catheterization: -assessment of pulmonary

annulus size&pulmonary artery -RVOT,VSD -coronary artery abnormalities

SOME PICTURES OF TETRALOGY OF FALLOT

CYANOTIC OR TET SPELL

AnxietyOver sweating (hyperventilation)

Sudden increase in cyanosis

CYANOTIC SPELLS Spasm of decrease SVR crying

RVOT

 Increase R…..L shunting

Increase systemic venous return DecreaseO2 Increase CO2 Decrease pH

Tachypneea 

CYANOTIC SPELLS

Increase systemic vascular resistance Squat/Knee chest position Ketamine 1-2mg/kg IV Neosynephrine 0.02mg/kg IV Tachycardia Propranolol 0.1mg/ Kg IV Release of infundibular spasm   Irritability Morphine 0.2mg/ Kg   S.C or IM

Hypoxia Oxygen  Dehydration Volume

Acidosis NaHco3 1mEq/ Kg IV

TOF MANAGEMENT

Medical :Correct iron deficiency anemiaCorrect polycythemiaB-BlockerAnalgesics-to reduce the ventilatory drive.

eg:morphine sulfateAlpha-adrenergic agonists-to improve hemodynamic

status by improving the myocardial contractility and increasing heart rate resulting in increased cardiac output.

eg:phenylephrine

FACTORS THAT INCREASE THE RISK FOR SURGERY

Low birth weight Pulmonary artery atresia Major associated anomalies Multiple previous surgeries Absent pulmonary valve syndrome Young or old age Severe annular hypoplasia Small pulmonary arteries High peak RV–to–left ventricular pressure ratio Multiple VSDs Coexisting cardiac anomalies

CONTRAINDICATIONS FOR SURGERY

The presence of an anomalous coronary artery

Very low birth weight Small pulmonary arteries Multiple VSDs Multiple coexisting intracardiac

malformations

POTTS SHUNT

A side to side anastomosis of pulmonary artery with aorta is created

The Potts shunt has been abandoned because of a tendency toward increased pulmonary blood flow and increasing difficulty with takedown at the time of corrective surgery.

It consists of constructing a shunt between the ascending aorta and right pulmonary artery

The Waterston shunt is sometimes used, but it also increases pulmonary artery blood flow. This shunt is more related to pulmonary artery stenosis, which generally requires reconstruction.

SURGICAL MANAGEMENT

Surgical:

Palliative = Blalock-Taussig shunt

for small PA’s Definitive= Total correction

GOAL OF PALLIATIVE SURGERY

The goals of palliation for tetralogy of Fallot (TOF) are to increase pulmonary blood flow independent of ductal patency and to allow pulmonary artery growth and even total correction.

ADVANTAGES OF MODIFIEDB-T SHUNT

preservation of the subclavian artery, suitability for use on either side, good relief of cyanosis, easier control and closure at time of primary

repair, excellent patency rate, and decreased incidence of iatrogenic

pulmonary/systemic artery trauma.

CORRECTIVE SURGERY

Primary correction is the ideal operation for treatment of tetralogy of Fallot (TOF) and is usually performed under cardiopulmonary bypass (CPB). The aims of the surgery are to close the ventricular septal defect (VSD), resect the area of infundibular stenosis, and relieve the right ventricular (RV) outflow tract obstruction (RVOTO).

CORRECTIVE SURGERY

Before cardiopulmonary bypass is

initiated, previously placed systemic-to-

pulmonary artery shunts are isolated

and taken down. Patients then undergo

cardiopulmonary bypass. Associated

anomalies, such as atrial septal defect

(ASD) or patent foramen ovale, are

closed.

SURGICAL COMPLICATIONS

Early postoperative complications following repair of tetralogy of Fallot (TOF) include the creation of heart block and residual ventricular septal defects (VSDs). Ventricular arrhythmias are more common and are reportedly the most frequent cause of late postoperative death. Sudden death from ventricular arrhythmias has been reported in 0.5% of individuals within 10 years of repair.

TRANSPOSITION OF GREAT VESSELS

The hallmark of tranposition of

great arteries is ventriculoarterial

discordance, in which the aorta

arises from the morphologic right

ventricle and pulmonary artery

arises from the morphologic left

ventricle

TRANSPOSITION OF THEGREAT ARTERIES (TGA)

Complete TGA or D-TGAEmbryological inversion of the great

arteriesVentriculoarterial discordance

Congenitally corrected TGA or L-TGAEmbryological inversion of the ventriclesAtrioventricular & ventriculoarterial

discordance (double discordance)

CAUSESUnknownAbnormal persistence of the subaorticconus with resorption or underdevelopment of the subpulmonaryconus(infundibulum)

PATHOPHYSIOLOGYAtrial septal defectVentricular septal defectPatent ductus arteriosus

DIAGNOSIS OF TGA Transposition of the great arteries with

intact ventricular septum: Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants.

Transposition of the great arteries with large ventricular septal defect: Infants may not initially manifest symptoms of heart disease, although mild cyanosis (particularly when crying) is often noted. Signs of congestive heart failure (tachypnea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.

CONTD…

Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: Infants often present with extreme cyanosis at birth,

MEDICATIONS

Inotropic agentsDigoxinLoop diureticsProstaglandins-alprostadil

SURGICAL CARE

Arterial switch procedureIt represents an repair

and establishes ventriculoarterial

concordance

TGA MANAGEMENT

Medical:PGE1O2 (3L/minute)Correct :

acidosis ,hypoglycemia. electrolyte disturbances.

Transcatheter :BAS

Surgical: Arterial switch (Jatene operation) at 7-15 daysAtrial switch ( Senning operation) at 6-9 months

SURGICAL REPAIRBalloon atrial septostomy

Developed by Rashkind (1965)Enlarges the atrial communication

Atrial switchPerformed first by Senning (1958)

and later modified by Mustard (1964)

Atrial baffle is created to direct venous return to the contralateral ventricle

SURGICAL REPAIR

Arterial switch Performed first by Jatene (1976) Great arteries transected and reattached to

appropriate AV valve Coronary ostia also transplanted Surgical treatment of choice Excellent outcomes so far

Atrial Switch There are two types of atrial switch

operations - the MUSTARD operation, and the

SENNING operation. Both are similar in principle, but

differ in technique.

The atrial switch operation is an open heart

procedure and is carried out with the assistance of a

heart-lung machine. The right atrium is opened, and

the wall between the atria is fully removed.

Atrial Switch Using pericardium (Mustard) or flaps created from the atrial septum and wall (Senning), a "baffle" is constructed directing blood from the veins in the right atrium towards the left ventricle. The same baffle also directs blood from pulmonary veins to the right ventricle. The circulation is therefore restored to normal in a functional sense.

Definitive Procedures Rastelli procedure.

Pulmo artery transected distal to its valve and proximal end is oversewn. Rt. Ventricle incised high along its outflow tract. Intraventricular dacron prosthetic tunnel is created between the edge of septal defect and aortic orifice.

EBSTEIN’S ANAMOLY

Congenital defect Origins of septal or posterior leaflets, or both, are displaced downward into RV Leaflets are variably deformed Atrialization of right ventricle Anterior leaflet is enlarged and sail-like

EBSTEIN’S ANOMALY

The tricuspid valve is abnormal and inserts well down into the RV. There is often severe trisuspid regurgitation, which can lead to death in the fetus or infant. Usually also with ASD so right-to-left flow results in cyanosis.

Physical Examination Cyanosis and clubbing - Varying degrees of cyanosis at various times in life and transient worsening with arrhythmias Precordial asymmetry-Usually left parasternal prominence and occasionally right parasternal prominence Absent left parasternal ( ie , right ventricular) lift an important negative sign Jugular venous pulse May be normal Large a and v waves late in the course of the disease, with development of right heart failure

Arterial pulses Usually normal Diminished volume

Heart sounds First heart sound widely split with loud tricuspid component Mitral component may be soft or absent in the presence of prolonged PR interval. Second heart sound usually is normal widely split when the pulmonary component is delayed due to RBBB.

ADDITIONAL HEART SOUNDS AND MURMURS

Third and fourth heart sounds commonly present, even in the absence of congestive heart failure (CHF).

Summation of third and fourth heart sounds, especially with prolonged PR interval, can mimic an early diastolic murmur.

The holosystolic murmur of tricuspid regurgitation at the lower left parasternal area and sometimes at the apex murmur intensity and duration increase during inspiration.

Chest X-Ray: Normal findings Cardiomegaly ( Rounded or Box-like contour ) Small aortic root and main pulmonary artery shadow Decreased pulmonary vasculature Large right atrium

Wall to wall

heart

Ebstein’s anomaly

12-LEAD EKG:

Rhythm -Usually normal sinus findings Intermittently SVT, paroxysmal SVT, atrial flutter, atrial fibrillation, ventricular tachycardia Abnormal P waves consistent with right atrial enlargement PR interval -Most commonly prolonged May be normal or short in patients with WPW (B) syndrome QRS complex -RBBB Low voltage in many patients

ECG from a patient with Ebstein's anomaly showing huge P waves and low amplitude QRS waves. RBBB and T wave inversion are not present on this ECG.

Echocardiogram: Echocardiogram standard for diagnosis. M-mode Paradoxical septal motion Dilated right ventricle Delayed closure of tricuspid valve leaflets

more than 65 milliseconds after mitral valve closure

CONTD…

Two-dimensional Apical displacement of the septal leaflet of

greater than 8 mm/m 2 – Abnormalities in morphology and septal

attachment of the septal and anterior tricuspid leaflets

Eccentric leaflet coaptation Dilated right atrium Dilated right ventricle with decreased contractile

performance Various left heart structural abnormalities

Doppler studies

Varying degrees of tricuspid regurgitation Excludes associated shunts

ASSESSMENT OF SEVERITY AND SURGICAL OPTIONS BY ECHOCARDIOGRAPHY

Functional right ventricular area less than 35% of total right ventricular area or an atrialized to functional right ventricular ratio greater than 0.5 associated with unfavorable prognosis

Functional right ventricular size Degree of septal leaflet

displacement Amount of leaflet tethering

CONTD…

Magnitude of leaflet deformity and dysplasia

Aneurysmal dilatation of right ventricular outflow tract (right ventricular outflow tract-to-aortic root ratio of >2:1 on parasternal short axis view)

Moderate-to-severe tricuspid regurgitation

Cardiac Catheterization:

Cardiac Catheterization Rarely performed today Confirms echocardiographic findings Can reveal right ventricular electrical activity on the intracardiac ECG with simultaneous right atrial pressure and waveform when the catheter is withdrawn from the right ventricle, back across the tricuspid valve into the right atrium

Complications: Congestive heart failure Sudden cardiac death Bacterial endocarditis Brain abscess Paradoxical embolism Transient ischemic attacks Stroke

MEDICAL MANAGEMENT

Antibiotic prophylaxis for infective endocarditis Medical therapy for heart failure - Angiotensin-

converting enzyme (ACE) inhibitors, diuretics, and digoxin

Arrhythmia treatment - Medical treatments such as anti-arrhythmic drugs or radiofrequency ablation of the accessory pathways Curative therapy of SVT with radiofrequency

ablation is currently the treatment of choice.The success rate is lower than that in patients

without significant structural heart disease.

Factors associated with lower likelihood of success include the following: Accessory pathways located along the atrialized right ventricle

Multiple accessory pathwaysComplex geometry of the pathways

Abnormal morphology of the endocardial action potentials in this region

Indications for surgery are generally as follows: New York Heart Association (NYHA) class I-

II heart failure with worsening symptoms or with a cardiothoracic ratio of 0.65 or greater[6]

NYHA class III-IV heart failureHistory of paradoxical embolismSignificant cyanosis with arterial O2

saturation of 80% or less and/or polycythemia with hemoglobin of 16 g/dL or more

Arrhythmias refractory to medical and radiofrequency ablation

SURGICAL MANAGEMENT

Approaches: Approaches Tricuspid valve repair is preferred

over valve replacement Bioprosthetic valves are preferred over mechanical prosthetic valves. The atrialized portion of the right ventricle can be resected surgically, and the markedly dilated, thin-walled right atrium can be resected. Associated septal defects may be closed

Repair of TV & Closure of ASD: Repair of TV & Closure of ASD Usual

preparation for operation & anaesthesia CPB Two venous cannulae Assess atrialized portion for paradoxical movement Body temperature about 25 o C RA incised parallel to AV groove

Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt.

TOTAL ANAMOLOUS PULMONARY

VENOUS RETURN

TAPVR

Definition -No direct communication exists between

the pulmonary veins and the LA. -Instead, they drain anomalously into the systemic venous tributaries or into the

RA. Epidemiology -1% of all congenital heart defects. -There is a marked male preponderance

for the infracardiac type (male/female ratio of 4:1)

TOTALLY ANOMALOUS PULMONARY VENOUS DRAINAGE (INFRADIAPHRAGMATIC)

All four pulmonary veins drain to the right side. Below the diaphragm they are always obstructed. Infant presents in first days with cyanosis, circulatory and respiratory failure and collapse.

1)Mixing of oxygenated and deoxygenated blood before or at the level of the right atrium (total mixing lesion). -Cyanosis -Right atrial blood either passes into the right ventricle

and pulmonary artery or passes through an atrial septal defect (ASD) or patent foramen ovale into the left atrium.

-Enlarged RA,RV, PA, small or normal LA, LV2) Obstructed pulmonary venous return

-severe pulmonary congestion,pulmonary hypertension, hypoxia

3) Restriction of PFO or ASD: -diminished LV preload->low cardiac output, small left

side heart

Pathophysiology

CLINICAL MANIFESTATION

1)Severe obstruction to pulmonary venous return, -In neonatal period: Cyanosis and severe tachypnea are

prominent, but murmurs may not be present. -Severely ill and fail to respond to mechanical ventilation.

2)Mild or moderate obstruction to pulmonary venous return and a large left-to-right shunt. - Heart failure in early life - Pulmonary artery hypertension->severely ill. - Systolic murmurs :the left sternal border, gallop rhythm -Cyanosis: mild.

3)No pulmonary venous obstruction -No pulmonary hypertension these patients -less likely to be severely symptomatic during infancy. -Clinical cyanosis: mild.

SUPRACARDIAC TAPVD

DIAGNOSISX-RAY

-

In most cases without obstruction :the heart is enlarged,

the pulmonary artery and right ventricle are prominent, and

pulmonary vascularity is increased.

In neonates with marked pulmonary venous obstruction

:a perihilar pattern of pulmonary edema and a small heart

ECHOCARDIOGRAPHY:ESSENTIAL FOR DIAGNOSIS

A large right ventricle :the pattern of abnormal pulmonary venous connections. A vessel in the abdomen with Doppler venous flow away from the heart is pathognomonic of TAPVR below the diaphragm. Shunting occurs almost exclusively from right to left at the atrial level

TREATMENT Medical1.Intensive anticongestive measures with digitalis and

diuretics ->in patient without pulmonary venous obstruction2.Metabolic acidosis should be corrected3.In infants with severe pulmonary edema(in infracardiac

type and other type) ->ventilator support with oxygen and positive end-

expiratory pressure4.PGE1; In patient with PHT (controversial), ->PGE1:increase systemic flow by keeping ductus open. ->In the infacardiac type, PGE1->maintaining the ductus

venosus open5.If the size of the interatrial communication appears small

and immediate surgery is not indicated ->balloon atrial septostomy or blade septostomy may be

performed

NATURAL HISTORY

1.CHF occurs in both types of TAPVR with growth retardation and repeated pneumonias

2.Without surgical repair, two thirds of the infants without obstruction die before reaching 1 years of age.(d/t superimposed pneumonia)

3.Patients with infracardiac type rarely survive for longer than a few weeks without surgery. Most die before 2months of age.

TRICUSPID ATRESIA

Types of tricuspid atresia

Type 1

• The great arteries are related normally

Type2 • The

great arteries are d-transposed

Type 3

• The great arteries are l-transposed

History

Physical examination

Investigations

Diagnosis of Tricuspidatresia

HISTORy

CyanosisGrowth retardationNasal flaringMuscle retraction

*Brain abscess and bacterial endocarditis-headache, seizures and neurologic deficit

P

H

Y

S

I

C

A

L

Cyanosis Digital clubbing JVP distension Peripheral pulses Apical impulse displaced First heart sound increased Second heart sound –split Continuous cardiac murmur-

80%cases

examination

INVESTIGATIONSLab studies-CBP-polycythemia

Chest X-rayCardiomegaly,right

atrial enlargement

EchoPresence of ASD

ECGTall T waves-atrial

enlargement,first degree AV block

CARDIAC CATHETERIZATIONStastus of ductus arteriosusASD if present ATRIAL

SEPTOSTOMY

Severe hypoxemi

a-prostaglan

din E

Severe congestive heart failure-digitalis

and diuretic therapy

Surgical procedure

s

Infants with decreased pulmonary blood flow

Infants with increased pulmonary blood flow

Palliative procedures

SURGICAL CARE

Blalock-Taussig ShuntDefinition:

A subclavian artery to pulmonary artery anastamosis.

Advantages:Predictability of flow, shunt may grow with the child, post-op congestive heart failure is less common than with other procedures, graft thrombosis is uncommon.

Disadvantages:Inadequate flow is possible, stenosis of the anastomosis is possible due to increased tension on the vessel, possible injury to the phrenic nerve, pulmonary hypertension is possible, small diameter of the subclavian may result in worsened hypoximia.Uses:Tetralogy of Fallot - can restore partial blood flow in the obstruction of the pulmonary circulation.Tricuspid Atresia - improves oxygenation of the desaturated blood due to the the shunt to the pulmonary circulation.Pulmonary Atresia - creates a shunt between systemic and pulmonary circulations

GLENN PROCEDURE Definition:

A superior vena cava (SVC) to pulmonary artery anastomosis. This is an end to side anastomosis where the right pulmonary artery is divided at the distal end and is attached to the side of the SVC.

Advantages:Pulmonary hypertension is rare (blood is being shunted under low venous pressure), does not increase the volume of work on the heart (amount of blood returned to the heart is unchanged).

GLENN PROCEDUREDisadvantages:

Cyanosis may increase by the decreased perfusion to bothlungs, may call for a second palliative procedure such as Blalock-Taussig. Polycythemia secondary to hypoximia may increase blood viscosity and decrease flow through the pulmonary vascular bed thus decreasing oxygen saturations. Shunt is only effective if the child weighs over 8 kg.

Uses:Used to bypass the right heart, therefore becomes useful in anomalies where right side obstruction occurs, such as tricuspid atresia or tricuspid stenosis.

FONTAN OPERATION

The right atrium is connected to the pulmonary artery directly. Used when it is not possible to have a two ventricle repair (this case has tricuspid atresia). Pulmonary vascular resistance must be low. It is done at age 4 years or so.

INTERRUPTED AORTIC ARCH

Interrupted Aortic Arch

TYPES OF INTERRUPTED AORTIC ARCH

Type A

TYPE B

TYPE C

TYPE A IAA

The arch interruption occurs distal to the

origin of the left subclavian artery.

TYPE B IAA

The interruption occurs distal to the origin of

the left common carotid artery.

TYPE C IAA

The interruption occurs proximal to the origin of the left common carotid

artery

PATHOPHYSIOLOGY

With an interrupted aortic arch, venous blood returning to the heart goes to the lungs and returns to the left side of the heart as oxygenated blood, so it can go to the body through the aorta.

However, oxygenated blood leaving the left ventricle to the Aorta, only goes to vessels proximal to the interruption. These vessels usually go to the upper body and the head.

CONTD…

Arteries that come off the Aorta distal

to the obstruction get deoxygenated

blood from the right ventricle. This

blood gets to the descending Aorta

through a PDA.

Repair of this lesion entails connecting

the ascending and descending Aorta

and ligating the PDA.

INTERRUPTED AORTIC ARCH/COARCTATION

Heart failure/collapse

RASTELLI OPERATION

An example of a repair of complex CHD with two ventricles. This one had a big VSD, TGA and PS. The severe sub-pulmonary stenosis precluded an arterial switch (it would become the neo-aortic valve), so a conduit joins RV to PA, and the VSD is patched.

SEVERAL PARAMETERS SHOULD BE MET TO ENSURE A SUCCESSFUL OUTCOME.

The candidate should be aged 4 years or older.

A right atrium of normal volume and normal caval drainage should be present.

Sinus rhythm should be present.Mean pulmonary artery pressure should

be low (ie, < 15 mm Hg), as should mean pulmonary arteriolar resistance.

The pulmonary artery-to-aorta diameter ratio should be greater than 0.75.

Nursing management of client with cyanotic heart disease

Post operative assessment and management

Helping family memebers to adjust

Providing post oprative care

Proiding pre operative care

Preoprative teaching

Preoperative assessment

Nursing care

NURSING DIAGNOSIS

Impaired gas exchange R/T altered pulmonary blood flow

Altered cardiac output R/T specific anatomic defect

Activity intolerance R/T O2 in blood & tissues Fluid volume excess with CHF

CONT……….

Altered nutrition : Less than body requirement R/T excessive energy demands Increased

Potential for infection R/TCHF Anxiety related to procedures & hospitalization

Developmental delay R/T energy , inadequate nutrition Alterations in parenting

USEFUL HINTS Large male baby with rapid, shallow abdominal breathing:

D-TGA Upper body blue, lower body pink; seen in : D-GA+PDA.COA  Only cyanotic newborn who has a thrill: Tricuspid atresia.  Ejection click is often heard in : Severe PS, HLHS Systolic ejection murmurs in first hours of life: TOF, PS, AS  Silent heart characteristic of : D-TGA, Pulmonary atresia.  Pulse oximetry& ABG should be obtained from the RIGHT

arm. ECG showing LEFT axis deviation: Tricuspid atresia

Nursing management of

client with cyanotic heart

disease

Thank you