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Disorders
Fetal Circulation
3. Obstructive Defects
ACYANOTIC HEART DISEASE
1. Atrial Septal Defect
• Opening between atria
• late childhood/early
adulthood
• S/sx: cyanosis(CHF),
dyspnea, fatigue, failure
to thrive, split S2
• Mx: Abx, surgery 1-3 yo
• Cx: endocarditis, heart
failure
• Postop: monitor arrhythmia,
administer antibiotics
ACYANOTIC HEART DISEASE
2. Ventricular Septal Defect
• Opening in ventricular
septum
• Most common
• S/sx: respi infections,
failure to thrive, dyspnea,
fatigue, pansystolic murmur
• Mx: close spontaneously
otherwise surgery <2 yo
• Cx: pulmonary HPN,
endocarditis, heart failure
• Postop: monitor arrhythmia,
administer antibiotics
ACYANOTIC HEART DISEASE
3. Patent Ductus Arteriosus
• Aorta to pulmonary
artery
• Common in prematurity,
high altitude, maternal
rubella
• females
• S/sx:clubbing, dyspnea,
“machinery murmur”
(2nd-3rd ICS)
• Cx: heart failure,
endocarditis, pulmonary
artery stasis/HPN
• Mx: Indomethacin,
surgery
CYANOTIC HEART DISEASE
1. Tetralogy of Fallot (TOF)
• Aorta from R
ventricle
• Pulmonary a. from L
ventricle
• Males
• S/sx:cyanosis,
murmurs
• Mx: PGE for PDA,
Balloon catheter to
create ASD, definitive
surgery 1 wk-3 mos
CYANOTIC HEART DISEASE
3. Total Anomalous Pulmonary
Venous Return
• Pulmonary vein
drain to SVC or R
atrium
• PDA or foramen
ovale essential
• S/sx: cyanosis,
fatigue
• CX: R heart
failure
• Mx: PGE, surgery
OBSTRUCTIVE DEFECT
Coarctation of the Aorta
• Constriction of aorta
• S/sx: asymptomatic
HPN, irritability,
headache, epistaxis,
dyspnea, claudication,
higher BP in upper
extremities, dec
femoral and distal
pulses,systolic
murmur
• Cx:chronic HPN
• Mx:surgery 2 yo
• Postop: monitor
abdominal pain,
antihypertensives
COARCTATION OF THE
AORTA
Ndx:
Ineffective cardiopulmonary and peripheral
tissue perfusion related to impaired
cardiac function
• Proper positioning to maintain
respiration
• Conserve energy to promote rest
• Quiet activities and stimulation
• Anticipate needs
• Administer prescribed drugs
COARCTATION OF THE AORTA
• Rest periods
• Adequate nutrition
small frequent feedings
iron supplementation
Obstructive Defect
2. PULMONIC STENOSIS
Obstruction of blood flow from the right ventricle to
the pulmonary artery
PULMONIC VALVE
Backflow of blood towards the right ventricle
• Dx: clinical
Mx: Salicylates and Immunoglobulins
KAWASAKI DISEASE
KAWASAKI
• Risk for ineffective peripheral tissue
perfusion related to inflammation of
blood vessels
- Observe for chest pain, color
changes, vomiting
Nursing Care
• Monitor vital signs
• Provide adequate nutrition
• Promote safety to prevent
chorea related injuries
Genitourinary
Disorders
Urinary Tract Infection
• Females
• E coli
• Ascending infection
• S/Sx:infants – mimic GI d/o; dysuria,
frequency, hematuria, low grade fever,
abdominal pain and bedwetting
• Dx: urine culture
suprapubic any amount
clean catch > 100,000/ml
• Mx: antibiotic
hydration
Acute Glomerulonephritis
• Inflammation of glomeruli or
kidney
• Follows infection with strep10-14
days
• 5-10 yo
• Males
S/sx:sudden onset of edema and
hematuria, proteinuria,
hypertension
Nsg Care:
quiet play activities
diet – normal CHON, mod salt
restriction, fluid restriction
daily weight and output
Nephrotic Syndrome
• Altered glomerular
permeability(autoimmune);
inc permeability to albumin
• 3 yo
• Males
• Minimal change syndrome
• S/sx: proteinuria, edema-
periorbital area,
hypoalbuminemia,
hyperlipidemia
• Dx: urinalysis and 24 hr
CHON, inc ESR
Mx:steroids, immunosupressant
Nsg care:
Adequate nutrition, proper diet – decrease
salt
Weigh daily, monitor I and O
Protect edematous areas
Administer prescribed drugs
Health teaching
Wilm’s Tumor
• Malignant tumor of the kidney
• Associated with other anomalies
• 6 mos-5 yo, peaks 3-4 yo
• Good prognosis
• S/sx: abdominal mass, hematuria, low
grade fever, anemia, wt loss
• Dx: CT scan
• Mx: Nephrectomy, radiotherapy
avoid abdominal palpation
Hypospadia/Epispadi
a
A. Hypospadia B. Epispadia
C. Hypospadia w/ chordee
UROGENITAL DEFECTS
Hypospadia - common
- chordee - fibrous band
Mx: surgery
Nsg care:
Post op – pain relief
assist parents in coping
Cryptorchidism
(Undescended Testis)
• Failure of one or both testes to descend
• Descend up to 6 weeks at birth
• May be d/t dec testosterone