Tetralogy of Fallot

Ns. Nurhusna, S.kep

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

  

   

CyanosisCyanosis-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
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.
 To

Magnetic resonance imaging (MRI)

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.
 provides

Management

Analgesics
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
 These

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:

BetaBeta-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
ClassificationClassification- Decongestant Brand names: Ah-Chew D, Lusonal, Nasop, NeoAhNeoSyneprine 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

surgical repair involves closure of the VSD and relief of RVOTO  A median sternotomy approach is used with cardiopulmonary bypass  Blalock-Taussig procedure
 Complete

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

Prognosis


In the present era of cardiac surgery, children with simple forms of tetralogy of Fallot (TOF) enjoy good long-term survival with an longexcellent 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 20;104(21):2565NKX2.5 mutations in patients with tetralogy of fallot Goldmuntz E, Geiger E, Benson DW. Division in cardiology, the childrens 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 genotypegenotype-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 conformationsequencing 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] [PubMed-

References
http://www.daviddarling.info/encyotra/T/tetral ogy 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