Cyanotic Heart Disease Flashcards by Rencevie Cate Bumanglag

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

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best recognized in the tongue and mucus membrane

Cyanosis

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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)

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use of the patient’s atrial septum in intraatrial baffle repair

Senning-

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Complications of Transposition
of Great
Vessels / Arteries

Lack of oxygen to tissues.
Heart failure.
Reduced heart pumping function.
Complete heart block.
Heart valve disease.

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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

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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

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right ventricle
of the heart becomes enlarged and thickened
due to the increased workload caused by the
other three defects.

Right ventricular hypertrophy:

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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

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a hole in the
wall that separates the two ventricles of the
heart.

Ventricular septal defect (VSD):

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–if prosthetic material is used in intraatrial baffle repair

Mustard

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medicine to improve cardiac function

digitalis

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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)

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Discharge and home care

medications
activity restrictions
diet and nutrition
follow-up appointment

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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

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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

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a good diagnostic cue for TOF

Squatting

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Position for acyanotic

orthopneic position

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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

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Associated defects in HLHS

ASD, PDA

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reduces the cardiac output by decreasing the venous return from the lower extremities and by
increasing the systemic vascular resistance.

knee-chest position

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no communication between RA and RV

Tricuspid
Atresia / Pulmonary
Atresia

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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.

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Nonsurgical Management in HLHS

Prostaglandin E1 infusion
Medicine
Nutrition

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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

1. Pulmonary Stenosis 2. VSD 3. Overriding aorta 4. 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

1. Improve tissue oxygenation and decrease oxygen consumption 2. Decrease cardiac demands 3. Improve cardiac function 4. Remove accumulated fluid and Na 5. Maintain hydration and nutrition 6. 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

Cyanotic Heart Disease Flashcards

(77 cards)