Cyanotic Heart Disease Flashcards
Management of transposition of great vessels:
* procedure of choice in the 1st weeks of life
* reestablishes normal circulation with the L ventricle
acting out as the systemic pump
* transecting great arteries and anastomosing the main
pulmonary artery just above the aortic valve and
anastomosing the ascending aorta to the proximal
pulmonary artery
* coronary arteries switched from proximal aorta to the
proximal pulmonary artery
ARTERIAL SWITCH PROCEDURE
best recognized in the tongue and mucus membrane
Cyanosis
Management of Tricuspid
Atresia:
▪ Uses an intracardiac right atrial baffle, directing blood from
the inferior and superior vena cava to the pulmonary
arteries bypassing the right atrium and right ventricle.
▪ The TCP is used for patients with heart chambers which
allow recirculation and stagnation to occur
TOTAL CAVOPULMONARY REPAIR (TCR)
use of the patient’s atrial septum in intraatrial baffle repair
Senning-
Complications of Transposition
of Great
Vessels / Arteries
- Lack of oxygen to tissues.
- Heart failure.
- Reduced heart pumping function.
- Complete heart block.
- Heart valve disease.
Management of HLHS:
performed when an infant is 4 to 6 months of
age. This procedure creates a direct connection between the
pulmonary artery and the vessel (the superior vena cava)
returning oxygen-poor blood from the upper part of the
body to the heart. This reduces the work the right ventricle
has to do by allowing blood returning from the body to flow
directly to the lungs.
Bi-directional Glenn shunt Procedure
Management of total anomalous pulmonary venous connection
depends on how sick the child is
and the specific structure of the abnormal connections between the
pulmonary veins and the right atrium.
Surgery
right ventricle
of the heart becomes enlarged and thickened
due to the increased workload caused by the
other three defects.
Right ventricular hypertrophy:
Management of HLHS:
➢ performed soon after birth
➢ anastomosis of the main pulmonary artery to the aorta, to
create a new aorta,shunting to provide pulmonary blood flow
and the creation of a large ASD
➢ This surgery usually is done within the first 2 weeks of a baby’s life.
Norwood
a hole in the
wall that separates the two ventricles of the
heart.
Ventricular septal defect (VSD):
–if prosthetic material is used in intraatrial baffle repair
Mustard
medicine to improve cardiac function
digitalis
Management of transposition of great vessels: Performed during cardiac catheterization to
increase mixing and maintain cardiac output over
longer period of time
BALLOON ATRIAL SEPTOSTOMY
(RASHKINDPROCEDURE)
Discharge and home care
medications
activity restrictions
diet and nutrition
follow-up appointment
Management of transposition of great vessels:
* Created to divert venous blood to the mitral valve and
pulmonary blood to the tricuspid valve
- Continuing role of the right ventricle as the systemic
pump and the late development of right ventricular
failure
INTRAATRIAL BAFFLE REPAIR
Management of transposition of great vessels:
* Operative choice in infants with TGA, VSD and severe PS
* Involves: (1)closure of the VSD with a baffle directing L
ventricular blood through the VSD into the aorta and,
(2)closure of the pulmonic valve and placement of a
conduit from the R ventricle to the pulmonary artery
requires multiple conduit replacements as the child
grows
* An operation for repair of complete transposition of the great
arteries in association with a large VSD and pulmonic stenosis,
RASTELLI PROCEDURE
a good diagnostic cue for TOF
Squatting
Position for acyanotic
orthopneic position
Effect of Tricuspid
Atresia / Pulmonary
Atresia
- decreased blood flow to the lungs
- Complete mixing of oxygenated & unoxygenated
blood in the left side of the heart
Associated defects in HLHS
ASD, PDA
reduces the cardiac output by decreasing the venous return from the lower extremities and by
increasing the systemic vascular resistance.
knee-chest position
no communication between RA and RV
Tricuspid
Atresia / Pulmonary
Atresia
e changes in the structure
of the heart due can disrupt the electrical signals
that tell the heart to beat. It is when all signals are blocked
Complete heart block.
Nonsurgical Management in HLHS
Prostaglandin E1 infusion
Medicine
Nutrition
surgical Management of Transposition
of Great
Vessels / Arteries
ARTERIAL SWITCH PROCEDURE
INTRAATRIAL BAFFLE REPAIR
RASTELLI PROCEDURE
Tetralogy of fallot management: Complete repair in the first few years of life
BROCK PROCEDURE
Manifestations of Tricuspid
Atresia / Pulmonary
Atresia
- Cyanosis
- Shortness of breath
- Difficulty in feeding
- Clubbing of fingers
Manifestations in HLHS
- Problems breathing,
- Pounding heart,
- Weak pulse, or
- Cyanosis
- Heart murmur
management of tetralogy of fallot
BLALOCK TAUSSIG/MODIFIED BLALOCK TAUSSIG:
BROCK PROCEDURE
Tetralogy of fallot management: low and reduce right to left shunting in tetralogy
of Fallot. It involved resection of part of the RV
infundibulum using a punch or biopsy-like
instrument introduced through the right ventricle
so as to reduce RV outflow tract obstruction,
without VSD closure
BROCK PROCEDURE
Manifestations of Truncus arteriosus
- cyanosis
- Problems breathing
- Pounding heart
- Weak pulse
Tetralogy of fallot management: nvolves closure of the VSD
and resection of the infindibular stenosis with a pericardial
patch to enlarge the R ventricular outflow tract
BROCK PROCEDURE –
condition in which
the heart can’t pump enough blood to meet the
body’s needs. It may develop over time because
the right lower heart chamber (ventricle) is
pumping under higher pressure than usual. The
strain may make the muscle of the right ventricle
stiff or weak.
Heart failure
Management of Tricuspid
Atresia:
➢ The IVC is connected to the pulmonary artery.
➢ Usually done when the patient is between 2-3 years
old
FONTAN PROCEDURE
Manifestations of Transposition
of Great
Vessels / Arteries
- cyanosis)
- Shortness of breath
- Weak pulse
- Poor weight gain
increased risk of thromboembolism → chronic hypoxemia leads to polycythemia in an attempt
to increase the Hgb available to carry O2.
Brain infarctions, Blood clots
o Closure of VSD and excision of pulmonary artery from aorta and
attaching it to right ventricle via homograft
o Requires additional procedures to replace conduit as its size
becomes inadequate in relation to child growth
MODIFIED BASTELLI PROCEDURE
Manifestations of tetralogy of fallot
*difficulty in breathing
*rapid breathing
*heart murmur
*clubbing of fingers and toes
Nonsurgical Management of Transposition
of Great
Vessels / Arteries
PROSTAGLANDIN E1 IV
BALLOON ATRIAL SEPTOSTOMY
(RASHKINDPROCEDURE)
Management of HLHS:
This procedure usually is done sometime during the period
when an infant is 18 months to 3 years of age. Doctors
connect the pulmonary artery and the vessel (the inferior
vena cava) returning oxygen-poor blood from the lower part
of the body to the heart, allowing the rest of the blood
coming back from the body to go to the lungs. Once this
procedure is complete, oxygen-rich and oxygen-poor blood
no longer mix in the heart and an infant’s skin will no longer
look bluish.
Fontan procedure
Management of Tricuspid
Atresia:
▪ Uses an extracardiac conduit, taking blood from the inferior
vena cava directly to the pulmonary arteries,thus bypassing
the right ventricle.
▪ This allows the patient to still utilize the pumping power of
the right atrium.
ATRIOPULMONARY SHUNT (AP)
Actions to support child and family
*educate about the disorder
*prepare for surgery
*provide post-operatice care
*plan for discharge and home care
Position of aorta and PA are reversed. The PA
leaves the L ventricle and the aorta exists from the
R ventricle.
Transposition
of Great
Vessels / Arteries
postoperative care
monitor
VS
fluid
respiratory status
provide comfort
nursing care for tet spells
a. place in knee chest position for infants and squat for older children
b. Oxygen
c. Morphine sulfate SC or IV as prescribed-
d. IV fluids as prescribed
e. Calm, comforting approach
f. Prevent respiratory infection
Tetralogy of fallot management: provides
blood flow to the pulmonary artery from the L or R
subclavian artery
BLALOCK TAUSSIG/MODIFIED BLALOCK TAUSSIG
NURSING DIAGNOSES FOR CYANOTIC HEART DEFECTS
- Decreased CO related to developing CHF
- Altered nutrition (less then body requirement) related to dyspnea and fatigue
- Activity intolerance related to cyanosis and dyspnea on exertion
- Altered growth and development related to hypoxemia
Management of Tricuspid
Atresia:
➢ Preferred in very small babies - below 2 years of age - in
whom the lung vessel resistance is still quite high, and in
borderline cases with abnormal pulmonary arteries.
➢ This is a shunt from SVC to the pulmonary artery (the first
shunt is removed at the second operation).
BIDIRECTIONAL GLENN
SHUNT/BIDIRECTIONAL CAVO- PULMONARY SHUNT
(BCPS)/PARTIAL FONTAN
Complications of Truncus arteriosus
- Breathing problems.
- pulmonary hypertension.
- Enlargement of the heart.
- Heart failure.
- regurgitation.
- arrhythmias.
4 defects in tetralogy of fallot
- Pulmonary Stenosis
- VSD
- Overriding aorta
- R Ventricular hypertrophy
Position for cyanotic
squatting/knee-chest position
Allows unoxygenated blood to flow into the systemic circulation or conditions that result to obstructive pulmonary blood flow
CYANOTIC HEART DEFECT
- pulmonary veins are abnormally connected to
systemic venous circuit R ventricular
hypertrophy and L atrium remains small - Associated ASD allows venous blood to be shunted
from the R atrium to left ventricle - Failure of pulmonary vein to join the left atrium
Total
Anomalous
Pulmonary
Venous
Connection
Decreased pulmonary blood flow
- Tetralogy of Fallot (TOF)
- Tricuspid atresia
Management of Tricuspid
Atresia: (pulmonary-to- systemic artery
anastomosis) to increase blood flow to the lungs
shunt placement
Mixed blood flow
- Transposition of great arteries/vessels
- Truncus arteriosus
- Total anomalous pulmonary venous connection (TAPVC/TAPVR(return)/TAPVD(drainage)
- Hypoplastic left heart syndrome (HLHS)
Assessment
▪ Cyanosis
▪ Poor, difficult feeding
▪ Retarded physical growth
▪ Squatting
▪ Clubbing of fingers and toes
▪ Polycythemia
▪ Tachycardia, tachypnea
▪ Elevated Hct
Extra fluid and blood into the
lungs can make it difficult to breathe.
Breathing problems.
- The left ventricle is underdeveloped and too
small. - The mitral valves is not formed or is very small.
- The aortic valve is not formed or is very small.
- The ascending portion of the aorta is
underdeveloped or is too small. - Underdevelopment ofthe leftside ofthe heart
resulting in hypoplastic left ventricle and aortic
atresia
Hypoplastic Left Heart Syndrome
medicine to remove accumulated fluid and Na
diuretics
surgical management in HLHS
Norwood
Bidirectional glenn shunt procedure
Fontan Procedure
Heart transplantation
Combination of 4 defects
Tetralogy of fallot
Management of Tricuspid
Atresia / Pulmonary
Atresia
*continuous infusion of prostaglandin E1
*!st: palliative: shunt placement, Atrial septostomy (Rashkind procedure)
*2nd: BIDIRECTIONAL GLENN
SHUNT/BIDIRECTIONAL CAVO- PULMONARY SHUNT
(BCPS)/PARTIAL FONTAN
*3rd: Fontan procedure
-TOTAL CAVOPULMONARY REPAIR (TCR)
-ATRIOPULMONARY SHUNT (AP)
Goal of surgery in total anomalous pulmonary venous connection
restore normal blood flow through the heart.
Nursing Care
- Improve tissue oxygenation and decrease oxygen consumption
- Decrease cardiac demands
- Improve cardiac function
- Remove accumulated fluid and Na
- Maintain hydration and nutrition
- Support child and family
Absence/Failure of tricuspid or pulmonary valve to
develop
Tricuspid
Atresia / Pulmonary
Atresia
Tetralogy of fallot management (for infants who can’t undergo primary repair): done
to increase pulmonary blood flow and increase oxygen saturation
Palliative Shunt
(BLALOCK TAUSSIG/MODIFIED BLALOCK TAUSSIG)
PDA must be present to permit blood to enter the
systemic circulation or pulmonary circulation for
mixing of blood
Transposition
of Great
Vessels / Arteries
Manifestations of Total
Anomalous
Pulmonary
Venous
Connection
- Cyanosis
- Problems breathing
- Pounding heart
- Weak pulse
Failure of normal septation and division of the
embryonic bulbar trunk into the pulmonary artery
and the aorta, resulting in a
Truncus
Arteriosus
Complications of of cyanotic heart disease
*Cerebral abscess
*Brain infraction, blood clots
*L-sided heart failure
most common complication of cyanotic heart disease; bacteria in the blood returning from the systemic circulation are usually filtered out by the capillaries in the lungs. When unO2 blood enters the systemic circulation through the R-L Shunt, bacteria can travel directly to the
brain
Cerebral abscess
Management of Tricuspid
Atresia: during cardiac
catheterization if ASD is small
Atrial septostomy (Rashkind procedure)
Management of truncus arteriosus
MODIFIED BASTELLI PROCEDURE
a narrowing of the
pulmonary valve and artery that reduces blood
flow to the lungs.
Pulmonary stenosis:
aorta, which carries
oxygen-rich blood to the body, is positioned
over the ventricular septal defect, allowing
blood from both ventricles to mix
Overriding aorta:
When is the corrective repair done in total anomalous pulmonary venous connection
early infancy
