Css Tetralogy of Fallot Doddy

TETRALOGY OF FALLOT

DEFINISI - EPIDEMIOLOGI

• Definisi :

• a congenital heart defect which is classically understood to involve four anatomical abnormalities.

• Epidemiologi :

• the most common cyanotic heart defect, and the most common cause blue baby syndrome. 

• tetralogy of Fallot (TOF) represents approximately 10% of cases of congenital heart disease (CHD), occurs in 3-6 infants for every 10,000 births, and is the most common cause of cyanotic CHD.

Etiologi

1. Genetik : chromosome 22 deletions and DiGeorge syndrome Children with Down syndrome fetal hydantoin syndrome or fetal

carbamazepine syndrome

1. Environment :

Factors that increase the risk for this condition during pregnancy include: Alcoholism in the mother Diabetes Mother who is over 40 years old Poor nutrition during pregnancy Rubella or other viral illnesses during pregnancy maternal phenylketonuria (PKU) birth defects,

pathophysiology

• large ventricular septal defect (VSD), or hole, • A narrowing (stenosis) of the outflow tract (infundibular stenosis) from

the right ventricle into the pulmonary artery.• The aorta is enlarged and "overrides," or sits directly above, the

ventricular septal defect (VSD). • A thickening of the muscle wall of the right ventricle resulting in a right

ventricular hypertrophy (thickening).

Diagram skematik

Manifestasi klinis

• Blue color to the skin (cyanosis), which gets worse when the baby is upset

• Clubbing of fingers (skin or bone enlargement around the fingernails)    

• Difficulty feeding (poor feeding habits)

• Failure to gain weight

• Passing out

• Poor development

• Squatting during episodes of cyanosis

• Severe form of Tetralogy of Fallot is with pulmonary atresia where no blood can cross from the right ventricle to the pulmonary arteries and lungs.

Additional anomalies

• stenosis of the left pulmonary artery, in 40% of patients

• a bicuspid pulmonary valve, in 60% of patients

• right-sided aortic arch, in 25% of patients

• coronary artery anomalies, in 10% of patients

• a foramen ovale or atrial septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot[12]

• an atrioventricular septal defect

• partially or totally anomalous pulmonary venous return

• forked ribs and scoliosis

Gambaran radiology

Normal T.O.F

TETRALOGY OF FALLOT•Chest x-ray showing features of tetralogy of Fallot: diminished pulmonary vascularity, •concave pulm. a.rterirt aortic arch, and descending aorta. •Note oval-shaped heart w/ laterally displaced and elevated cardiac apex leading to "boot" shaped silhouette

Diagnosa

• Radiografi dada memperlihatkan adanya pembesaran dari ventrikel kanan dan penurunan ukuran segmen utama arteri pulmonalis memberikan gambaran “boot-shaped” pada jantung.

• ECG memperlihatkan hipertrofi ventrikel kanan dengan deviasi axis ke kanan.

• Echocardiography : VSD dan overriding aorta

Treatment

• Propanolol, 0,01-0,25 mg/kg (rata-rata 0.05 mg/kg) diberikan dengan dorongan intravena lambat yang bisa mengurangi denyut jantung dan meredakan “spell”

• Surgery

Prognosa

• Without surgery, mortality rates gradually increase, ranging from 30% at age 2 years to, 50% by age 6 years.

• The mortality rate is highest in the first year and then remains constant until the second decade. No more than 20% of patients can be expected to reach the age of 10 years, and fewer than 5-10% of patients are alive by the end of their second decade

• Patients who have undergone total surgical repair of Tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance (New York Heart Association Class I-II).

• .

Komplikasi

• Delayed growth  and development

• Irregular heart rhythms (arrhythmias)

• Seizures during periods when there is not enough oxygen

• Death

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