CYANOTIC DISEASES Flashcards by ARELLA

Cyanotic Heart Diseases

• Coarctation of Aorta
• Tetralogy of Fallot
• Transposition of Great
• Tricuspid Atresia
• Truncus Arteriosus
• Total Anomalous Venous Return

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_ _, a condition that affects only females, results when one of the X chromosomes (sex chromosomes) is missing or partially missing.

Turner syndrome

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Turner syndrome can cause a variety of medical and developmental problems, including

short height, failure of the ovaries to develop and heart defects.\

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assessment for coarctation of aorta

cyanosis
High BP in the arms
bounding pulses
cool and low VP in lower extremities
decreased or absence of femoral pulse
SOB

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complications for coarctation of aorta

cardiomegaly (left-sided heart enlargement)
heart failure

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surgical management for coarctation of aorta

• Resection and end-to-end anastomosis
• Subclavian aortoplasty
• balloon Angioplasty

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_ is a congenital condition whereby the aorta is narrow, usually in the area where the ductus arteriosus inserts.

Coarctation of the aorta

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medical management for coarctation of aorta

digoxin - digitalis
furosemide - diuretics
prostaglandin E1

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responsible for strengthening the pumping action of the heart

digoxin

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Treat the fluid accumulation in the lungs

furosemide

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furosemide may cause

Possible swelling to the upper extremities

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_ is used in infants with coarctation of the aorta to maintain patency of the ductus arteriosus, allowing for blood flow to the lower body and improving systemic perfusion, especially when the ductus is constricted or closed.

prostaglandin E1

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_ is a congenital heart defect characterized by four specific cardiac defects.

Tetralogy of Fallot

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Tetralogy of fallot is a common type of heart defect. It may be seen more commonly in children with _ or DiGeorge syndrome.

Down syndrome

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4 Abnormalities in Tetralogy of Fallot

Stenosis in right ventricular Outflow
Right Ventricular Septal Defect
Ventricular Septal Defect
Aortic Override of Septal Defect

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PROV

Pulmonic Stenosis
Right Ventricular Hypertrophy
Overriding of the aorta
Ventricular septal defect

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Assessment for tetralogy of Fallot

clubbed fingers and toes, failure to thrive, harsh systolic murmur, dyspnea, tet spell, boot shaped heart

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a sudden episode of profound cyanosis (bluish skin) in children with Tetralogy of Fallot, a congenital heart defect, caused by a drop in oxygen levels in the blood.

tet spell

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Children with _ exhibit blush skin during episodes of crying or feeding

tetralogy of Fallot

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symptoms of tetralogy of fallot

• Cyanosis
• Hypoxemia: oxygen sat= 65-85%
• Tet spell
• tachypnea
• altered level of consciousness

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tet spell is precipitated by

crying, feeding, defecation

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complications of tetralogy of fallot

arrhythmias, heart failure, stroke

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manage tet spell episodes by

Put the child in a knee chest position traps blood in the lower extremities to reduce venous flow of blood from the lower extremities. It will then decrease the volume of blood being shunted through the VSD and overriding aorta. The blood that enters the systemic circulation has a higher O2 content which will reduce dyspnea.

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nursing management for tetralogy of fallot

knee chest position
Oxygen administration

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to prevent tet spells:

1. Do not interrupt sleep and provide a calm quiet environment upon waking up 2. Offer a pacifier during crying 3. Small and frequent feedings 4. Swaddle or hold the infant during procedures

medical management for tetralogy of fallot

• Morphine sulfate • PGE1

_ can also be given as a sedative during a tet spell, alleviating pain and anxiety and helping decrease heart and respiratory rates.

Morphine sulfate

morphine sulfate classification

narcotic (opioid) agonist

tetralogy of fallot surgical management

- brock procedure - blalock-taussig procedure

a surgical procedure that creates a connection between the subclavian artery and the pulmonary artery to increase blood flow to the lungs

blalock-taussig procedure

a cardiac surgery used to relieve pulmonary stenosis, a narrowing of the pulmonary valve, in patients with Tetralogy of Fallot, a congenital heart defect

brock procedure

a surgical procedure where a diseased or damaged section of tissue (like bowel, artery, or trachea) is removed (resected) and the remaining healthy ends are directly joined together (anastomosed) to restore continuity.

resection with end-to-end anastomosis

a surgical procedure, primarily used in infants, to treat coarctation of the aorta (a narrowing of the aorta) by using a flap of the left subclavian artery to widen the narrowed area.

subclavian flap aortoplasty

a minimally invasive procedure used to open narrowed or blocked arteries, typically to treat conditions like coronary artery disease, by using a catheter with a balloon to widen the artery and improve blood flow, often followed by stent placement

balloon angioplasty

_ is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta.

Transposition of the great vessels

Transposition of the great vessels is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels:

superior and/or inferior vena cava, pulmonary artery, pulmonary arteries, and aorta.

assessment after birth for transposition of great vessels

• cyanosis is unchanged with supplemental oxygen (less severe if VSD present) • Hypoxemia- low oxygen saturation • no murmur present • tachypnea • metabolic acidosis

assessment of transposition of the great vessels on x-ray

• Heart appears as egg on its side/"egg on a string” appearance • Lung congestion • Cardiomegaly (both on x-ray and echo)

A condition where heart appears as egg on its side/"egg on a string” appearance

transposition of the great vessels

A condition where there is no gas exchange

transposition of the great vessels

pathophysiology of transposition of the great vessels

- RV - Ao - body - RA (right side - blood is never oxygenated - LV - PA - lungs - PV - LA (left side - blood never deoxygenated)

medical management for transposition of the great vessels

Prostaglandin E

surgical management for transposition of the great vessels

• Balloon Atrial Septostomy • Surgically switch great vessels • Arterial switch procedure

reestablishes normal circulation with the left ventricle acting as the systemic pump and creation of new aorta

arterial switch procedure

Transposition of the great vessels - Five-year survival rate > _ - No treatment one year survival rate _

80% 10%

Cyanotic Heart Diseases

• Coarctation of Aorta • Tetralogy of Fallot • Transposition of Great • Tricuspid Atresia • Truncus Arteriosus • Total Anomalous Venous Return

pulse assessment

Bounding - +3 Normal - +2 Weak - +1 Absent - 0

a narrowing of the valve between the lower right heart chamber and the lung arteries.

pulmonic stenosis

a condition defined by an abnormal enlargement of the cardiac muscle surrounding the right ventricle.

Right ventricular hypertrophy

An _ is a congenital heart defect where the aorta is positioned directly over a ventricular septal defect, instead of over the left ventricle.

overriding aorta

A _ is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle.

ventricular septal defect

murmur in coarctation of aorta

Soft or moderately loud systolic murmur prominent at the base of the heart

murmur in tetralogy of fallot

Harsh systolic murmur

murmur in transposition of the great vessels

no murmur present

It is the back up of blood vessels ; causes extra circulation to bypass the narrowing network of arteries

Collateral circulation

What age is brock procedure applicable

2-6 mos (may be 1 month)

Bluish discoloration around the lips

Circumoval pallor