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Tetralogy of Fallot

Tetralogy of Fallot Definition A congenital malformation of the heart characterized by: A ventricular septal defect pulmonary stenosis overriding of the aorta the right ventricle develops hypertrophy Tetralogy of Fallot is one of the most common congenital heart disorders. is classified as a cyanotic heart disorder because the condition results in an inadequate flow of oxygenated blood to the systemic circulation. Patients with TOF initially present with cyanosis shortly after

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513 views24 pages

Tetralogy of Fallot

Tetralogy of Fallot Definition A congenital malformation of the heart characterized by: A ventricular septal defect pulmonary stenosis overriding of the aorta the right ventricle develops hypertrophy Tetralogy of Fallot is one of the most common congenital heart disorders. is classified as a cyanotic heart disorder because the condition results in an inadequate flow of oxygenated blood to the systemic circulation. Patients with TOF initially present with cyanosis shortly after

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justinahorro
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Tetralogy of Fallot

Definition

A congenital malformation of the heart


characterized by:
 A ventricular septal defect
 pulmonary stenosis
  overriding of the aorta
 the right ventricle develops hypertrophy
 Tetralogy of Fallot is one of the most
common congenital heart disorders.
 is classified as a cyanotic heart disorder
because the condition results in an
inadequate flow of oxygenated blood to
the systemic circulation.
 Patients with TOF initially present with
cyanosis shortly after birth, thereby
attracting early medical attention.
Cause/Etiology

 The cause is unknown, although genetic


studies suggest a multifactor etiology.
Signs and Symptoms
 Cyanosis-bluish tint of skin, lips, and nail beds.
 detectable heart murmurs
 Activity such as crying may exacerbate the condition
and babies may have shortness of breath or may faint.
Babies also may experience a "tetralogy spell" in which
oxygen levels drop suddenly leading to irritability and
then sleepiness or unresponsiveness.
 Rapid breathing
 Cool and clammy skin
 Poor gain weight
 Most adults with tetralogy of Fallot have had surgery
during childhood. The adult with unrepaired tetralogy
usually has milder cyanosis and a heart murmur or
extra heart sound.
Risk factors
 factors associated with a higher incidence of
TOF include maternal rubella (or other viral
illnesses) during pregnancy
 poor prenatal nutrition
 maternal alcohol use
 Diabetes
 maternal age older than 40 years, Children
with Down syndrome have a higher incidence
of TOF.
Diagnostic Tests
Cardiac catheterization

 provides angiographic visualization of


ventricular and pulmonary artery size.
Catheterization also helps obtain pressure
and oxygen saturation measurements in
different chambers and identifies any
possible shunts. In the presence of
preexisting shunts, angiograms should be
obtained before complete surgical repair.
 Physical Examination
Electrocardiography
 To diagnose certain cardiovascular diseases
 To evaluate heart murmurs
 Electrocardiography is a transducer that emits
and receives ultrasound waves is placed in
four positions on the chest above the heart.
Nursing responsibilities
 Place patient in a supine position on left side
facing the equipment
 Instruct family and patient about the procedure
and sensation.
Magnetic resonance imaging (MRI)
 provides good delineation of the aorta, right
ventricular outflow tract, VSDs, right
ventricular hypertrophy, and the pulmonary
artery and its branches.
 MRI can be used to measure intracardiac
pressures, gradients, and blood flows.
 Drawbacks to MRI include the need for
prolonged imaging times and the
requirement for sedation in small children
to prevent motion artifacts. Additionally,
sick infants cannot be observed when
enclosed in an MRI tunnel.
Management
Analgesics
 These agents reduce ventilator drive. Pain
control ensures patient comfort and promotes
pulmonary toilet (attempts to clear mucus and
secretions from the trachea and bronchial tree
by deep breathing, incentive spiratomy,
postural drainage, and percussion.)
 Most analgesics have sedating properties,
which are beneficial for patients who are
having hypercyanotic episodes
Morphin Sulfate (duramorph,
astromorph, MS Contin)

 Drug of choice for narcotic analgesia


because of its reliable and predictable
effects, safety profile, and easy of
reversibility with naloxone
 Administered IV, may be dosed in number
of ways and commonly titrated until
desired effect obtained.
Metoprolol and Propranolol
 Classification: Beta-Blockers

Used to treat tremors, angina, HPN, heart


rhythm disorders, and other heart or
circulatory conditions. It also used to treat
or prevent heart attack, and reduce the
severity of migraine and headaches.
Phenylephrine
Classification- Decongestant

Brand names: Ah-Chew D, Lusonal, Nasop, Neo-


Syneprine
It works by constricting (shrinking ) blood vessels.
Constriction of blood vessels in the sinuse, nose,
and chest allows drainage of these areas,
which decreases congestion. Constriction of
blood vessels also affects blood pressure
Surgical management

Types of surgery
Complete Intracardiac repair
 Complete surgical repair involves closure
of the VSD and relief of RVOTO
 A median sternotomy approach is used
with cardiopulmonary bypass
 Blalock-Taussig procedure
Temporary or Palliative Surgery

 Creation of a systematic to pulmonary


artery shunt can be performed from the
midline by means of a sternotomy or
thoracotomy
Nursing diagnosis

 Impaired gas exchange related to


ventilation perfusion imbalanced

Nursing interventions
 Discuss preventing exposure to cold,
dressing warmly
Nursing Dx.
 Ineffective tissue perfusion related to
impaired transport of oxygen across the
alveolar and capillary membrane.

Nursing interventions
 Instruct the use of relaxation, stress
reduction technique as appropriate.
Nursing Dx
 Failure to thrive related to cardiac illness

Nursing interventions
 Health teaching providing an opportunity for
children and family to express fear about the
child's illness and treatment plan
 Providing physiologic and psychological
support as comfort measures after surgery and
caring for child in cardiac failure
Nursing Outcomes
Prognosis
 In the present era of cardiac surgery, children
with simple forms of tetralogy of Fallot (TOF)
enjoy good long-term survival with an excellent
quality of life. Late outcome data suggest that
most survivors are in New York Heart
Association classification I, although maximal
exercise capability is reduced in some. The
surgical procedures are still palliative, and
continued cardiac monitoring into adult life is
necessary.
Research
Circulation. 2001Nov 20;104(21):2565-8

NKX2.5 mutations in patients with tetralogy of fallot

Goldmuntz E, Geiger E, Benson DW.

Division in cardiology, the children’s hospital of Philadelphia, Philadelphia, PA, USA.goldmuntz@email.chop.edu

Background:Recent reports have implicated mutations in the prescription factor NKX2.5 as a cause of tetralogy of
fallot (TOF). To estimate the frequency of NKX2.5 mutations in TOF patients and to further investigate the
genotype-phenotype correlation of NKZ2.5 mutations, we genotype 114 TOF patients.
METHODS AND RESULTS:Patients were recruited prospectively (November 1992 through June 1999) and tested
for
22q11deletion; those with 22q11deletion or recognized chromosomal alteration were excluded from the present
study. Patients were screened for NKX2.5 alterations by conformation-sensitive gel electrophoresis and
sequencing of fragments with aberrant mobility. Four heterozygous mutations were identified in 6 unrelated
patients with cases of TOF, including 3 with pulmonary atresia and 5 with right aortic arch; none had ECG
evidence of PR interval prolongation. Three of 4 mutations (Glu21GIn, Arg216Cys, and Ala219Val) altered
highly conserved amino acids, of which two mapped in the conserved NK2 domain. The fourth mutation
(Arg25Cys) was identified in 3 unrelated probands in the present study and has been previously reported. No.
homeodomain mutations were identified.
CONCLUSIONS:NKX2.5 mutations are the first gene defects identified in nonsyndromic TOF patients. NKX2.5
mutation is present in >/=4% of TOF patients. Mutations were identified in the present study mapped outsideof
the homeodomain, were not fully penetrate, in contrast to mutations previously reported that impair
homeodomain function.

PMID: 11714651 [PubMed- indexed for MEDLINE]


References

 http://www.daviddarling.info/encyotra/T/te
tralogy of fallot.html
 http://www.ncbi.nlm.nih.gov/pubmed/and
PMC2651859/
 http://www.ncbi.nih.gov/11714651
 http://wrong diagnosis.com/f/falllot
syndrome/intro.html
Submitted By:

Charie May A. Ili


BSN3-2

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