Tetralogy of Fallot (TOF)

TOF-Definition
A congenital heart defect (CHD) which is classically understood to involve four anatomical abnormalities (although only three of them are always present). It is the most common cyanotic heart defect, representing 55-70%, and the most common cause of blue baby syndrome .

Component
A. Pulmonary stenosis B. Overriding aorta C. Ventricular septal defect(VSD) D. Right ventricular hypertrophy (RVH)

A. Pulmonary valve stenosis

Valvular heart disease in which outflow of blood from the right ventricle of the heart is obstructed at the level of the pulmonic valve. reduction of flow of blood to the lungs. 80% of right ventricular outflow tract obstruction. causes: congenital heart disease, rheumatic heart disease or a malignant carcinoid tumor.

Symptoms
Jugular venous distension, cyanosis (usually visible in the nail beds), right ventricular hypertrophy and general symptoms of lowered oxygenation of the blood. sudden fainting or dizziness if exercised too much, an enlarged liver (hepatomegaly) and swelling in the legs (oedema).

Evaluation
echocardiography The degree of stenosis is typically determined by the peak pressure gradient across the valve. If the peak gradient across the valve is greater than 50 mmHg, intervention is generally warranted. If the peak gradient is < 25 mmHg, no intervention is generally needed. If the gradient is between 25 and 50 mmHg, it is a gray area.

Treatment
Valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option.

B. Overriding aorta
a congenital heart defect where the aorta is positioned directly over a ventricular septal defect, instead of over the left ventricle. the aorta receives some blood from the right ventricle, which reduces the amount of oxygen in the blood.

C. Ventricular septal defect

Epidemiology and Etiology

VSDs are the most common congenital cardiac anomalies. They are found in 30-60% of all newborns with a congenital heart defect, or about 2-6 per 10000 births. Congenital VSDs are frequently associated with other congenital conditions, such as Down Syndrome

 A VSD can also form a few days after a myocardial infarction (heart attack) due to mechanical tearing of the septal wall, before scar tissue forms, when macrophages start remodeling the dead heart tissue.

Diagnosis
- cardiac auscultation can be obtained by non- invasive cardiac - Ultrasound (echocardiography). - To more accurately measure ventricular pressures, cardiac catheterization , can be performed.

Symptoms
Ventricular septal defect is usually symptomless at birth. It usually manifests a few weeks after birth. VSD is an acyanotic congenital heart defect.

Treatment
conservative or surgical Smaller congenital VSDs often close on their own, as the heart grows, and in such cases may be treated conservatively surgical intervention, a heart-lung machine is required and a median sternotomy is performed Ventricular septum defect in infants is initially treated medically with cardiac glycoside (e.g., digoxin10-20mcg/kg per day), loop diuretics(e.g., furosemide-3 mg/kg per day) and ACE inhibitors (e.g., catopril 0.5-2 mg/kg per day).

D. Right Ventricular Hypertrophy (RVH)

a form of ventricular hypertrophy affecting the right ventricle, the enlargement of ventricles (lower chambers) in the heart. Blood travels through the right ventricle to the lungs . If conditions occur which decrease pulmonary circulation, meaning blood does not flow well from the heart to the lungs, extra stress can be placed on the right ventricle. This can lead to right ventricular hypertrophy.

 considered to be one of the rare diseases of the heart. Unlike the left ventricle, which tends to overwork itself when it detects abnormalities, the right ventricle dilutes itself. This is the reason why left ventricular hypertrophy is way more common than right ventricular hypertrophy.

Causes
Common causes include: Pulmonary hypertension Fallot teratology Pulmonary valve stenosis Ventricular septal defect(VSD) High altitude Cardiac fibrosis COPD

Symptoms
Mild degrees of right ventricular hypertrophy won t show any symptom at all. more severe form, patients will experience shortness of breath coupled with chest pains and pressures Palpitations, or the unusual sensation that the heart is beating rapidly and forcefully, is also felt.

 Dizziness and the eventual loss of consciousness are also expected. Feeling lightheaded from time to time is actually common to people with right ventricular hypertrophy. And aside from all of these, there could also be noticeable swelling in the lower extremities such as the legs, feet, and ankles.

Treatment
The doctors of the patients with right ventricular hypertrophy addresses all the underlying causes of the disease to eventually treat it. Drugs like beta-blockers, ACE inhibitors, angiotensin receptor blockers, diuretics, and digoxin may be prescribed. But if drugs can t treat it, there might be a need to implant a cardioverter defibrillator or a pacemaker in the patient. Which one to put depends upon what his symptoms are. An open-heart surgery may also be performed and the last resort for patients with this disease will be heart transplant.

Rheumatic Heart Disease (RHD)

formerly one of the most serious forms of heart disease of childhood and adolescence. involves damage to the entire heart and its membranes. complication of rheumatic fever and usually occurs after attacks of rheumatic fever. incidence of rheumatic heart disease has been greatly reduced by widespread use of antibiotics effective against the streptococcal bacterium that causes rheumatic fever.

Rheumatic Heart Disease Statistics

Rheumatic fever and rheumatic heart disease afflict about 1,800,000 Americans. Rheumatic fever and rheumatic heart disease killed 5,014 Americans in 1997.

What causes Rheumatic Heart Disease?

Rheumatic fever causes rheumatic heart disease. Rheumatic fever results from an untreated strep throat. Rheumatic fever can permanently damage the heart valves.

Symptoms of Rheumatic Heart Disease

Breathlessness fatigue palpitations chest pain fainting attacks.

Treatment
medication and surgery. Medication will aim to avoid overexertion. Surgery - replace the damaged valve(s). The best way to prevent rheumatic heart disease is to seek immediate medical attention to a strep throat and not let it progress to rheumatic fever.

Myocarditis
inflammation or degeneration of the heart muscle. complication during or after various viral, bacterial, or parasitic infectious diseases, such as polio, influenza, rubella, or rhuematic fever. often caused by various diseases such as syphilis, goiter, endocarditis, or hypertension, however, myocarditis may appear as a primary disease in adults or as a degenerative disease of old age.

Symptoms of Myocarditis
In acute myocarditis, individuals may experience fever, chest pains, a sensation of skipped heart beat (palpitations), dyspnea and fatigue. In chronic myocarditis, fatigue, a sensation of skipped heart beats, shortness of breath and chest pains may be experienced.

 Signs and symptoms of myocarditis include: Chest pain (often described as "stabbing" in character) Congestive heart failure(leading to edema, breathlessness and hepatic congestion) palpitation (due to arrythmias) Sudden death (in young adults, myocarditis causes up to 20% of all cases of sudden death) Fever (especially when infectious, e.g. in rhuematic fever)

 Symptoms in infants and toddlers tend to be more non-specific with generalized malaise, poor appetite, abdominal pain, chronic cough. Later stages of the illness will present with respiratory symptoms with increased work of breathing and is often mistaken for asthma

Treatment of Myocarditis
no specific treatment for myocarditis. Since myocarditis may be chronic and may be present over many years, the treatment is usually based on the patient s symptoms (chest pain, shortness of breath, palpitations). Some cases of myocarditis may progress to congestive heart failure, requiring hospitalization, heart failure medications, or cardiac transplantation.