cyanotic heart diseases
TRANSCRIPT
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