Tetralogy of Fallot

Tetralogy of Fallot

Description of tetralogy of Fallot

It is a complex congenital defect of the heart and major blood vessels procedure. Plains but especially in childhood, when it is diagnosed and surgically corrected subsequently. Very rarely can detect up to adulthood. In such cases, it is a lighter form that the patient burden moderately.

As the name suggests, it is a combination of four defects: narrowing of the pulmonary artery (pulmonary artery truncus pulmonalis), ventricular septal defect (a hole in the partition between the left and right ventricle) overlying the aorta (aorta or aorta begins at the opening in the interventricular partition) and right ventricular hypertrophy (enlargement of the heart muscle around the right ventricle). It is important to mention that always necessarily present all four defects, but more often find a combination of only three.

Unfortunately, the tetralogy of Fallot often accompanied by other heart and vascular defects, for example, can indicate an open arterial dučej (ductus artetiosus ductus Botalli) or right sided heart arc (15 to 20 percent of holders of tetralogy of Fallot, typically cardiac arch left hand) hole in the atrial septum , malformations of the arteries that nourish the heart (coronary arteries).

Individual defect Tetralogy of Fallot

Stenosis (narrowing) of the pulmonary artery

Complains or preventing blood flow to the lungs, where it is aerated. Must therefore lacking oxygen blood flow through the hole in the bulkhead into the interventricular aorta (aorta). Much depends on the size of the narrowing of the pulmonary artery, because according to his grades are then present different symptoms in different serious.

Interventricular hole in the bulkhead

Is mixed, deoxygenated blood from the right ventricle and oxygenated blood from the left ventricle.

Overlying aorta (aorta)

This artery after divorcing his body mixed oxygenated and deoxygenated blood. The body and its organs have a lack of oxygen, which leads to most of the symptoms.

Increased muscle of the right ventricle

Since the right ventricle is overloaded, as the compensation is increased, resulting in insufficient nourish the heart muscle and other diseases that can result in failure.

Risk factors Tetralogy of Fallot

Tetralogy of Fallot is a congenital defect, which is caused by many factors. It is a combination of genetic factors and environmental factors. Unfortunately, often we do not find any specific cause, that we could have avoided.

Genetic factors can not influence much, because we are set to inherit (the gene defect was discovered at the 22nd chromosome, or it may be part of tetralogy of Fallot a genetic syndrome –DiGeorge syndrome, for example, or a well-known Down syndrome).

For risk factors external environment is clearly considered alcohol use by the mother during pregnancy. Therefore it is very important abstinence mother.

A risk to the fetus is also smoking during pregnancy. It is important to remember that hazardous active smoking, but also passive. Therefore not recommended for mothers staying in smoky areas.

There was also found a higher frequency of the defects in mothers during pregnancy were taking anti-seizure disorders (such as some medicines for epilepsy).

Some connection was found between frequency of tetralogy of Fallot and maternal phenylketonuria (= metabolic disease, an inability to metabolize the amino acid phenylalanine).

Prevention Tetralogy of Fallot

As we have already mentioned, we can clearly identify the risk factors of birth defects.Therefore, the prevention very difficult or impossible.

However, it is very important to avoid drinking alcohol during pregnancy especially in the first eight weeks, which begins to develop circulatory system including the heart.

It is also recommended not to smoke throughout pregnancy and how actively and passively. It is important to avoid smoky spaces, smoky restaurant facilities and the like.

If the mother is taking medication against seizures and suffers from phenylketonuria, it is recommended more frequent medical examinations of the mother but also the fetus especially ultrasound, which is able to detect very early congenital defects in the fetus.

Finally, it is possible genetic testing, for example in cases of suspected DiGeorge syndrome, or mutation of the 22nd chromosome. These tests but not uncommon, especially for their work and financial performance.

Signs and symptoms of tetralogy of Fallot

Symptoms are often noticeable at birth or soon after. The main symptoms of counts cyanosis(bluish skin, lips, oral mucosa and nail beds) because of their insufficient oxygenation and mixing of oxygenated and deoxygenated blood, which is distributed throughout the body. Cyanosis often worsens with exercise patient (intense crying babies, breastfeeding newborns etc.) where it may be accompanied by dizziness, weakness, and occasionally even loss of consciousness. Unfortunately, some babies may not suffer cyanosis, especially if the defect is milder.

Often Patients may dyspnea, typically after exertion (increased respiratory activity because of lack of oxygen in the body), which is very typical for a cardiac defect.

Other symptoms include heart murmurs that are caused, for example, blood flow through the hole in the partition between the chambers or the flow of blood through the pulmonary artery constriction and many others.

Cyanosis and heart murmurs are the first and most obvious symptoms, which after further investigation led to the diagnosis of tetralogy of Fallot and its subsequent treatment.

Less common symptoms especially with less disability and subsequent late diagnosis, the overall failure to thrive (retarded physical growth and slow increase in weight) Clubbing (extended last phalanges).

Diagnosis of Tetralogy of Fallot

Initial suspicion arises after the discovery of cyanosis (bluish skin, lips, mouth and nail beds) and heart murmurs. Followed by numerous tests. Most of them echocardiography (ultrasound examination of the heart and great vessels, acronym ECHO).

Further investigating the ECG (electrocardiography), which can show us enlarge and overloading of certain parts of the heart.

As a routine investigation takes a chest X-ray (heart and lungs).

When invasive cardiac catheterization is introduced into the heart and great vessels of the peripheral vascular catheter, usually from the thigh. This test illustrates very valuable information about the shape of the vascular bed around the heart, the size of the blood pressure in it and also the amount of oxygen in the blood. This information is essential when deciding on further treatment.

Recently, examination using magnetic resonance imaging, showing anatomical features of the heart and great vessels. Its indisputable advantage is non-invasive.

Treatment of Tetralogy of Fallot

The only effective treatment is cardiac surgery (surgery of the heart and surrounding blood vessels). Very often, the operation may be more than one. There are many types of surgical procedures, which are selected according to the age and condition of the patient.

It is also important to support the patient through medication, to facilitate the work of the heart and lungs and calms the patient.

Patient’s prognosis depends on the success of the operation and the level of their own defects. The patient is then monitored by a physician throughout life. Extremely important is the preventive use of antibiotics before any even the slightest surgery, including dental performance. It is the prevention against inflammations of the heart valves, and heart (endocarditis), which are very hazardous to heart damage and very poorly treated.

How can I help facilitate treatment?

Unfortunately, the only treatment is surgery on the heart and large vessels. Therefore, the only means of compliance when treating the advice and recommendations of doctors.

Complications of Tetralogy of Fallot

A complicating factor is the total failure to thrive (retarded physical growth and slow increase in weight).

A very serious complication is endocarditis when bacteria or other infectious agents attacked inner lining of heart valves and heart. Infective endocarditis greatly complicates patient management and can worsen the condition of the patient. Often can lead to cardiac reoperation. Therefore it is extremely important preventive antibiotics during slightest surgery, including dental surgery.

Another complication may be a fault in the speed and regularity of heartbeat (cardiac arrhythmia) that often accompany heart disease.

Other names: Defect membranous part of the ventricular septum, tetralogy of Fallot, pulmonary artery stenosis, the narrowing of the pulmonary artery, aorta overlying, overlying aorta, hypertrophy of the right ventricle, enlarged right ventricle, congenital heart disease

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