Tetralogy of Fallot

DEFINITION OF TERMS
Tetralogy of Fallot is a congenital heart defect. a problem with the heart's structure that's present at birth. Tetralogy of Fallot is a rare, complex heart defect. It occurs in about 5 out of every 10,000 babies. The defect affects boys and girls equally.

a French physician and cardiologist Ettienne Louis Arthur Fallot

described for the first time the socalled "maladie bleue", or "blue disease"

4 defects :
Ventricular Septal Defect The heart has an inner wall that separates the two chambers on its left side from the two chambers on its right side. This wall is called a septum. The septum prevents blood from mixing between the two sides of the heart. A VSD is a hole in the septum between the heart's two lower chambers, the ventricles. The hole allows oxygen-rich blood from the left ventricle to mix with oxygen-poor blood from the right ventricle.

 Pulmonary Stenosis -narrowing of the pulmonary valve and the passage from the right ventricle to the pulmonary artery. In pulmonary stenosis, the pulmonary valve cannot fully open. Thus, the heart has to work harder to pump blood through the valve. As a result, not enough blood reaches the lungs.

 Overriding Aorta
In tetralogy of Fallot, the aorta is located between the left and right ventricles, directly over the VSD. As a result, oxygen-poor blood from the right ventricle flows directly into the aorta instead of into the pulmonary artery.

 Right Ventricular Hypertrophy

with this defect, the muscle of the right ventricle is thicker than usual. This occurs because the heart has to work harder than normal to move blood through the narrowed pulmonary valve.

ASSESSMENT
Activity/Rest Inability to carry on normal activities exertion-related dyspnea Circulation Tachycardia, palpitations; severe dysrhythmia. History of congenital/organic heart disease, rheumatic fever. Upward displacement of the diaphragm Cardiac enlargement; loud systolic murmur, associated with a thrill. BP may be elevated or may be decreased Clubbing of toes and fingers may be present, with symmetric cyanosis in surgically untreated tetralogy of Fallot.

 Elimination Urine output may be decreased.

Food/Fluid May have edema of the lower extremities Pain/Discomfort May report chest pain with/without activity Respiration Cough; may or may not be productive. Respiratory rate may be increased. Dyspnea/shortness of breath, orthopnea may be reported.

ANATOMY AND PHYSIOLOGY

A heart is a small organ located in the middle and slightly to the left of mediastinum, where it is partially overlapped the lungs. The heart is wider at the top (base) than at the bottom (apex) and is position in the chest o that the blunt tip of the apex projects forward and to the left. The lower border of the heart rests on the diaphragm.

Three layers of cardiac tissue: Epicardium: outer layer of the heart, same structure as the visceral pericardium Myocardium: Middle layer of the heart, composed of striated muscle fibers, responsible for the heart contractile force. Endocardium: inner layer of the heart, consist of endothelial tissue, lines the inside of the chambers and covers the heart valves.

Chambers Heart is divided into two halves by a muscular wall (septum). Each half has an upper collecting chamber (atrium) and lower pumping chamber (ventricle), for a total of four chambers.

Valves The four cardiac valves are flaplike structures that function to maintain unidirectional (forward) blood flow through the heart chambers. These valves open and close in response to pressure and volume changes within the cardiac chambers. 2 types of valves: Atrioventricular valves (AV) which separate the atria from the ventricles. Semilunar valves which separate the pulmonary artery and the aorta from their respective ventricles.

Oxygen-poor venous blood enters the right atrium, flows from the right atrium to the right ventricle when the tricuspid valves is opened, and is pumped to the lungs through the pulmonary artery. Oxygen rich blood returns from the lungs to the left atrium, enters the ventricle when the mitral valve is opened, and is ejected into the aorta for the distribution to the peripheral tissues.

Pathophysiology

DIAGNOSTICS
Echocardiography MRI
Ventricular septal defect with overriding aorta as seen in the parasternal (above) and apical (below) transducer positions

Chest Radiography
pulmonary circulation is poor so that lung areas are hypertransparent and heart image is typical(shoe aspect), for right ventricular hypertrophy.

Cardiac catheterization
This exploration gives a anatomic and hemodynamic assessment of the heart and appreciate very specific the oxygen saturation of the cardiac chambers.

SURGICAL MANAGEMENT
Palliative Blalock-Thomas-Taussig procedure-used in cyanotic heart defects. Placement of a blalocktaussig shunt alleviate symptoms of poor oxygenation (e.g. cyanosis) which is done by anastomosing the subclavian artery to the pulmonary artery (bypassing the stenosed pulmonary artery) so that part of hypoxemic blood in the aorta will be oxygenated in the lungs.

 Total Heart repair/ Open heart surgery

Medical management
During Tet spell attacks pt. can be given ketamine 0.5 to 3 mg/kg IV or 2 to 3 mg/kg IM Propranolol starting at 0.02 to 0.05 mg/kg IV

NURSING CARE PLAN

Impaired gas exchange R/T: Alveolar- capillary membranes changes Decreased oxygen carrying blood Impaired delivery of oxygenated blood to the tissues

 Ineffective breathing pattern R/T hyperventilation Ineffective tissue perfusion R/T inadequate oxygenated blood supplying the tissues

End