Page |1
NR602 I HUMAN CASE SARAH JAMIESON WHO
PRESENTS FOR MOOD ASSIGNMENT CHAMBERLAIN
UNIVERSITY 2025
• Cyanosis in cases with mild RVOT obstruction may be so slight it is not initially
evident or may be present at birth (with severe obstruction). Cyanosis is usually
present by 6 months of age.
• Dyspnea and cyanosis (including hypercyanotic episodes, or "TET spells")
increase by 2 to 4 months old, especially with crying, feeding, and/or defecation.
The infant may also have a history of poor weight gain.
Physical findings of TOF - ans-Cyanosis of the mucous membranes and dyspnea
• A grade III to V/VI, harsh systolic ejection murmur at the left mid- to upper
sternal border (VSD murmur and symptoms of a large VSD). There may be a
palpable thrill and a holosystolic murmur at the LLSB.
• Sternal lift secondary to right ventricular hypertrophy.
Diagnosis findings of TOF - ans-Chest radiography may show a boot-shaped heart
with decreased pulmonary vascular markings.
• ECG shows right ventricular hypertrophy and right axis deviation and may show
a conduction delay in V1.
• An echocardiogram shows the extent of the pulmonary obstruction and
demonstrates the anatomy of the overriding aorta and VSD.
• Pulse oximetry values decrease over time, with resultant increase in hemoglobin
and hematocrit values.
• Cardiac catheterization may be performed to delineate location of coronary
arteries prior to surgical correction
Management of TOF - ans-Management
, Page |2
• In neonates with severe pulmonary obstruction, the ductus arteriosus is
maintained or reopened with prostaglandin E1 (PGE1) until more definitive repair
or palliation is possible.
• For hypercyanotic episodes, the child should be cradled in a knee-chest position,
soothed, and given oxygen and perhaps morphine sulfate subcutaneously until
the spell subsides. The knee-chest maneuver increases systemic resistance,
decreases right-to-left shunting, and increases pulmonary blood flow, alleviating
the symptoms. Immediate intervention is required for infants who are "spelling,"
especially if the previously mentioned maneuvers do not end the spell. Most
children are surgically repaired before hypercyanotic spells begin.
• Complete repair with open-heart surgery is usually performed in infancy by
closing the VSD and relieving the RVOT obstruction.
• Long-term complications after repair include pulmonary valve regurgitation and
atrial and ventricular arrhythmias and require lifelong cardiology follow-up.
Recent studies indicate progressive right ventricular dilation leads to increasing
QRS duration on ECG. QRS duration of 180 mm significantly increases the risk of
ventricular tachycardia and sudden death. A cardiology consult is indicated before
clearing for sports participation (Park, 2016).
• SBE prophylaxis is indicated for 6 months after repair. Patients who have
pulmonary valve replacement required prophylaxis for their entire life
Transposition of great of the great arteries - ans-results from incomplete
septation and migration of the truncus arteriosus during fetal development. In d-
TGA, the aorta arises from the right ventricle and the pulmonary artery arises
from the left ventricle. The aorta receives the deoxygenated systemic venous
blood and returns it to the systemic arteries. The pulmonary artery receives
oxygenated pulmonary venous blood and returns it to the pulmonary circulation
Signs and symptoms of transposition of great arteries - ans-Cyanosis is
immediately evident by 1 hour of birth (approximately half) or within the first day
, Page |3
after birth. Because d-TGA allows mixing of oxygenated and unoxygenated blood,
occasionally less cyanotic infants may present as late as 3 months old.
• CHF symptoms may be present.
• Affected infants are often large for gestational age with retardation of growth
and development after the neonatal period.
Management of Transposition of great arteries - ans-Intravenous PGE1 is given to
delay closure or reopen the ductus arteriosus.
• A balloon atrial septostomy may be performed to promote mixing of
oxygenated and unoxygenated blood in the atria.
• The arterial switch (Jatene procedure) is usually performed in the first few days
of life. If this is not possible, a number of other operations may be performed,
such as the Nakaidoh, Damus-Kaye-Stansel, or réparation à l'étage
ventriculaire(REV) procedures.
Tricuspid atresia - ans-results in a small right ventricle without access from the
right atrium. Blood returning from the systemic circulation must pass over an ASD
to the left atrium and then left ventricle before being pumped to either the lungs
or the body
Pulmonic stenosis - ans-there is narrowing at the subpulmonic, valvular, or
supravalvular area. Right-sided pressure is increased as the ventricle pumps
against the obstruction. Right ventricular hypertrophy occurs as a result of this
increased load.
Clinical findings of pulmonic stenosis - ans-Exertional dyspnea and fatigue are
noticeable as stenosis progresses.
• Cyanosis from right-to-left shunting over the foramen ovalemay be evident with
critical pulmonic stenosis in the newborn.
, Page |4
• Growth and development are usually normal except in cases of Turner or
Noonan syndrome in which short stature is common
Management of pulmonic stenosis - ans-• Balloon valvuloplasty in neonates and
older children with stenosis greater than 50 mm Hg are performed. If
unsuccessful, surgical valvuloplasty or replacement may be indicated. Stents and
balloons are also used for branch stenosis.
• With mild stenosis, families must be encouraged to treat their children normally
and not limit their activity. Moderate stenosis can progress to severe narrowing
during periods of rapid growth, such as during infancy or adolescence
Clinical findings of COA - ans-Upper extremity HTN with lower extremity
hypotension are present, although milder cases may cause only a minimal
discrepancy between upper and lower extremity BPs. In severe cases, poor lower
extremity perfusion may be noticed with lower body mottling or pallor.
• Delayed timing and absent or weak arterial and other distal arterial pulses may
occur.
• Bounding brachial, radial, and carotid pulses may occur.
• Signs of CHF may be evident.
• A systolic ejection murmur may be detected in the left infraclavicular region
with transmission to the back.
• A ventricular heave at the apex may be palpated.
• A gallop rhythm may occur in infants with CHF.
Symptoms of COA - ans-symptoms include tachypnea, poor feeding, and possibly
cool lower extremities. In children 3 to 5 years old, coarctation may go unnoticed
until HTN or a murmur is detected. Retrospectively, children with coarctation may
have had complaints of headaches or leg pain with exercise
NR602 I HUMAN CASE SARAH JAMIESON WHO
PRESENTS FOR MOOD ASSIGNMENT CHAMBERLAIN
UNIVERSITY 2025
• Cyanosis in cases with mild RVOT obstruction may be so slight it is not initially
evident or may be present at birth (with severe obstruction). Cyanosis is usually
present by 6 months of age.
• Dyspnea and cyanosis (including hypercyanotic episodes, or "TET spells")
increase by 2 to 4 months old, especially with crying, feeding, and/or defecation.
The infant may also have a history of poor weight gain.
Physical findings of TOF - ans-Cyanosis of the mucous membranes and dyspnea
• A grade III to V/VI, harsh systolic ejection murmur at the left mid- to upper
sternal border (VSD murmur and symptoms of a large VSD). There may be a
palpable thrill and a holosystolic murmur at the LLSB.
• Sternal lift secondary to right ventricular hypertrophy.
Diagnosis findings of TOF - ans-Chest radiography may show a boot-shaped heart
with decreased pulmonary vascular markings.
• ECG shows right ventricular hypertrophy and right axis deviation and may show
a conduction delay in V1.
• An echocardiogram shows the extent of the pulmonary obstruction and
demonstrates the anatomy of the overriding aorta and VSD.
• Pulse oximetry values decrease over time, with resultant increase in hemoglobin
and hematocrit values.
• Cardiac catheterization may be performed to delineate location of coronary
arteries prior to surgical correction
Management of TOF - ans-Management
, Page |2
• In neonates with severe pulmonary obstruction, the ductus arteriosus is
maintained or reopened with prostaglandin E1 (PGE1) until more definitive repair
or palliation is possible.
• For hypercyanotic episodes, the child should be cradled in a knee-chest position,
soothed, and given oxygen and perhaps morphine sulfate subcutaneously until
the spell subsides. The knee-chest maneuver increases systemic resistance,
decreases right-to-left shunting, and increases pulmonary blood flow, alleviating
the symptoms. Immediate intervention is required for infants who are "spelling,"
especially if the previously mentioned maneuvers do not end the spell. Most
children are surgically repaired before hypercyanotic spells begin.
• Complete repair with open-heart surgery is usually performed in infancy by
closing the VSD and relieving the RVOT obstruction.
• Long-term complications after repair include pulmonary valve regurgitation and
atrial and ventricular arrhythmias and require lifelong cardiology follow-up.
Recent studies indicate progressive right ventricular dilation leads to increasing
QRS duration on ECG. QRS duration of 180 mm significantly increases the risk of
ventricular tachycardia and sudden death. A cardiology consult is indicated before
clearing for sports participation (Park, 2016).
• SBE prophylaxis is indicated for 6 months after repair. Patients who have
pulmonary valve replacement required prophylaxis for their entire life
Transposition of great of the great arteries - ans-results from incomplete
septation and migration of the truncus arteriosus during fetal development. In d-
TGA, the aorta arises from the right ventricle and the pulmonary artery arises
from the left ventricle. The aorta receives the deoxygenated systemic venous
blood and returns it to the systemic arteries. The pulmonary artery receives
oxygenated pulmonary venous blood and returns it to the pulmonary circulation
Signs and symptoms of transposition of great arteries - ans-Cyanosis is
immediately evident by 1 hour of birth (approximately half) or within the first day
, Page |3
after birth. Because d-TGA allows mixing of oxygenated and unoxygenated blood,
occasionally less cyanotic infants may present as late as 3 months old.
• CHF symptoms may be present.
• Affected infants are often large for gestational age with retardation of growth
and development after the neonatal period.
Management of Transposition of great arteries - ans-Intravenous PGE1 is given to
delay closure or reopen the ductus arteriosus.
• A balloon atrial septostomy may be performed to promote mixing of
oxygenated and unoxygenated blood in the atria.
• The arterial switch (Jatene procedure) is usually performed in the first few days
of life. If this is not possible, a number of other operations may be performed,
such as the Nakaidoh, Damus-Kaye-Stansel, or réparation à l'étage
ventriculaire(REV) procedures.
Tricuspid atresia - ans-results in a small right ventricle without access from the
right atrium. Blood returning from the systemic circulation must pass over an ASD
to the left atrium and then left ventricle before being pumped to either the lungs
or the body
Pulmonic stenosis - ans-there is narrowing at the subpulmonic, valvular, or
supravalvular area. Right-sided pressure is increased as the ventricle pumps
against the obstruction. Right ventricular hypertrophy occurs as a result of this
increased load.
Clinical findings of pulmonic stenosis - ans-Exertional dyspnea and fatigue are
noticeable as stenosis progresses.
• Cyanosis from right-to-left shunting over the foramen ovalemay be evident with
critical pulmonic stenosis in the newborn.
, Page |4
• Growth and development are usually normal except in cases of Turner or
Noonan syndrome in which short stature is common
Management of pulmonic stenosis - ans-• Balloon valvuloplasty in neonates and
older children with stenosis greater than 50 mm Hg are performed. If
unsuccessful, surgical valvuloplasty or replacement may be indicated. Stents and
balloons are also used for branch stenosis.
• With mild stenosis, families must be encouraged to treat their children normally
and not limit their activity. Moderate stenosis can progress to severe narrowing
during periods of rapid growth, such as during infancy or adolescence
Clinical findings of COA - ans-Upper extremity HTN with lower extremity
hypotension are present, although milder cases may cause only a minimal
discrepancy between upper and lower extremity BPs. In severe cases, poor lower
extremity perfusion may be noticed with lower body mottling or pallor.
• Delayed timing and absent or weak arterial and other distal arterial pulses may
occur.
• Bounding brachial, radial, and carotid pulses may occur.
• Signs of CHF may be evident.
• A systolic ejection murmur may be detected in the left infraclavicular region
with transmission to the back.
• A ventricular heave at the apex may be palpated.
• A gallop rhythm may occur in infants with CHF.
Symptoms of COA - ans-symptoms include tachypnea, poor feeding, and possibly
cool lower extremities. In children 3 to 5 years old, coarctation may go unnoticed
until HTN or a murmur is detected. Retrospectively, children with coarctation may
have had complaints of headaches or leg pain with exercise
