DORV, VSD, BAV, ASD, PH,


    Double Outlet Right Ventricle (DORV) 
    Normally, a ventricle has just ONE outlet. For the left ventricle, this is the aorta. For the right ventricle it is the pulmonary artery. In DORV, both of these "outlet" blood vessels - aorta and pulmonary artery -arise from the RIGHT VENTRICLE, either totally or to a great extent. Most cases of DORV have a VSD. DORV is classified based on the relationship between the VSD and the blood vessels. If the VSD is right under the aorta, it is called DORV with Sub-Aortic VSD. If it lies under the pulmonary artery, it becomes DORV with Sub-Pulmonary VSD - also called the TAUSSIG-BING anomaly. If the VSD is under both the arteries, it is called DORV with Doubly Committed VSD. Sometimes, the VSD is farther away from the arteries, and is known as DORV with Non-Committed VSD. When in addition to this, there is narrowing of the pulmonary valve (Pulmonary Stenosis), the condition is similar to Tetralogy of Fallot (ToF). If the VSD is below the pulmonary valve, the features are just like those of Transposition of Great Arteries (TGA). When the VSD is doubly committed or non committed, clinical features are variable.





    Ventricular Septal Defect (VSD) 
    A ventricular septal defect, or VSD, is the most common kind of congenital heart defect. Normally, blood entering the right side of the heart stays on the right side (this is low-oxygen blood), and blood on the left side of the heart stays on the left side (this is oxygen-rich blood) which is then pumped to the rest of the body. When a defect or "hole" is present between the ventricles (or lower chambers), blood from the left side of the heart is forced through the defect to the right side every time the heart beats. It then goes back to the lungs even though it is already rich in oxygen. Because of this, blood that is not yet oxygen-rich can't get to the lungs. The most common signs and symptoms are trouble eating and gaining weight, breathlessness and easy fatigability. A baby with a large VSD tires quickly after not eating very much, falls asleep, wakes us in a short while quite hungry, tries to eat again, tires easily, and the cycle is repeated. Because the heart has to pump extra blood, it may enlarge. Also, because there's more blood going to the lungs, high pressure may occur in the blood vessels there. This may cause permanent damage to the walls of the blood vessels over time. Many, if not most, of all VSDs will close on their own. Those that close on their own are usually small and do so in the first year of life. Large VSDs, especially those that don't close in the first year of life, will usually need to be closed surgically. VSD closure is one of the most commonly performed congenital heart operations. The child would be expected to have virtually normal growth, development, and life expectancy following repair.




    Bicuspid aortic valve 
    The normal aortic valve has three flaps (cusps) that open and close. A bicuspid valve has only two cusps. There may be no symptoms in childhood, but by adulthood (often middle age or older), the valve can become narrowed, making it harder for blood to pass through it, or it may start allowing blood to leak backward through it. Treatment depends on how well the valve works.
    Bicuspid aortic valve is the most common congenital cardiac anomaly occurring in 1-2% of the population with males affected 4 times more frequently than females.







    Atrial Septal Defect (ASD)
    The septum is the wall that separates the right and left sides of the heart. A hole in the wall between the two upper chambers is called an atrial septal defect, or ASD. This is one of the least complex forms of congenital heart defect, and was one of the first types to be repaired surgically. Normally, low-oxygen blood entering the right side of the heart stays on the right side, and oxygen-rich blood stays on the left side of the heart, where it is then pumped to the body. When a defect or "hole" is present between the atria (or upper chambers), some oxygen-rich blood leaks back to the right side of the heart. It then goes back to the lungs even though it is already rich in oxygen. Because of this, there is a significant increase in the blood that goes to the lungs.
    There are three different kinds of ASDs. The most common form of ASD is the secundum defect which usually occurs as an isolated defect. The primum ASD is associated with a cleft in the mitral valve which may also causing the valve to leak. The third kind of ASD is the sinus venosus defect, located in the superior portion of the atrial septum and typically associated with abnormal drainage of the right upper pulmonary vein.
    Atrial septal defects can vary greatly in size. Some ASDs will close on their own and no surgery is needed. Some ASDs are closed in the catheterization lab and do not require open-heart surgery. Certain devices such as the Amplatzer Occluder, the CardioSEAL, Helex, and Clamshell Device are currently being used or have been used in the past. Some ASDs will need to be corrected with open heart surgery to restore normal blood circulation and/or to repair subsequent damage which has occurred in the heart. Many ASDs are not detected until adulthood. Left untreated for decades, potential problems include lung disease, exercise intolerance, heart rhythm abnormalities, shortened life expectancy and the increased risk of a stroke.
    Atrial septal defects occur in 5 to 10 percent of all children born with congenital heart disease. For unknown reasons, girls have atrial septal defects twice as often as boys.







    Pulmonary hypertension

    is a condition in which pressure in the blood vessels in the lungs is higher than normal. This is different from systemic hypertension where pressure in the blood vessels to the body is higher than normal. Systemic hypertension is a common condition especially in people as they age. It is diagnosed by taking a blood pressure in the arm. Systemic hypertension is usually successfully treated by oral medications. In contrast, pulmonary hypertension is a rare condition in both children and adults. Patients usually present to the doctor with symptoms of fatigue, chest pain, difficulty breathing with activity and episodes of fainting or nearly fainting. As you can imagine, these symptoms are difficult to get a handle on, especially in infants. Thus, pulmonary hypertension is often diagnosed late, after the patient has had vague symptoms for some time. Pulmonary hypertension can be diagnosed by echocardiography (cardiac ultrasound), although cardiac catheterization is often recommended to confirm the diagnosis and as a way to better understand the cause.
    There are two broad classes of pulmonary hypertension: those without any obvious cause (termed idiopathic) and those caused by or occurring with other diseases. Diseases that can cause pulmonary hypertension include:
      • lung disease (such as immature lungs in premature babies or airway obstruction)
      • some congenital heart diseases with excessive blood flow to the lungs or blockage of blood flow from the lungs back to the heart
      • diseases of the clotting system of the body
      • diseases of inflammation (such as systemic lupus erythematosus, SLE)
      • some drugs and toxins
      • HIV infection
      • certain blockages in the drainage of the liver (portal hypertension)
    When possible, the best treatment for pulmonary hypertension is treating the underlying disease. When this is possible, the pulmonary hypertension may resolve or at least improve. In situations where treatment of the underlying disease does not improve the pulmonary hypertension and in situations of idiopathic pulmonary hypertension, medications are available. In the past, these medications were only intravenous and often involved long-term intravenous therapy. Inhaled and oral medications have more recently become available. Although rare, lung transplantation has been used successfully in severe cases.
    For more information on pulmonary hypertension, visit the Pulmonary Hypertension Association's website.



















































    • What exactly is CHD?
      Congenital Heart Defects are when babies are born with something wrong with their hearts. When some part of the heart doesn't develop normally, the flow of blood is changed through the heart. Some CHDs, like mild pulmonary valve stenosis (narrowing of the pulmonary valve) are simple defects and usually do not require treatment. The only symptom may be a heart murmur.
      Other defects, however, can be more complex, such as tetralogy of fallot, which is the most common complex CHD. Tetralogy of fallot (TOF) consists of four different types of defects which prevents an adequate amount of blood from reaching the lungs, resulting in oxygen-poor blood flow to the body. In the case of this type of defect, open heart surgery is needed either at birth or later in infancy. Symptoms of TOF usually include cyanosis or blueness of the skin and/or mouth and fingernails.
      Whether mild or complex, there are 35 known types of defects. Heart defects may involve missing, unconnected or misplaced arteries; underdevloped or missing valves, and narrowed or blocked blood vessels.


      Treatments for CHD
      Causes of CHD

      To date, there is no known cause of CHD. Researches have speculated that heart defects are caused by a genetic abnormality or when the developing fetus is exposed to infections, toxic substances or drugs. Other factors may include:
      • Hereditary - a parent with a congenital heart defect may be more likely to have a child with CHD.
      • Genetic Disorders - Children with genetic disorders are more likely to have CHD. Half of all Down Syndrome babies have heart conditions.
      • Smoking during pregnancy - smoking has been linked to several congenital heart defects
      • Babies born prematurely have a higher chance of having a congenital heart defect.
      • Babies born to women who have chronic conditions such as diabetes have a higher chance of having a congenital heart defect.

      Symptoms of CHD

      If a baby has a mild defect, there may be no symptoms at all. There may be a heart murmur which can be heard with a stethoscope but sometimes even healthy babies and children have heart murmurs and some of them even grow out of their murmur.
      Some symptoms of CHD in babies and children include:
      • Rapid or difficulty breathing
      • Cyanosis
      • Tires easily
      • Poor circulation
      • Sweating
      • Poor weight gain
      • Sudden weight gain or puffiness or swelling of the skin
      There are some types of heart defects that cause the heart to work harder than it should. If the heart continues to work too hard it could result in heart failure. This is also known as Congestive Heart Failure or CHF.
      Symptoms of heart failure include:
      • Fatigue with physical activity
      • Shortness of breath
      • A buildup of blood and fluid in the lungs
      • A buildup of fluid in the feet, ankles, and legs
      Treatments for CHD vary depending on the severity of the heart defect and also the general health of the heart patient. As discussed earlier, mild defects might not require any treatment. While this is true, it is important to be seen by a pediatric cardiologist as he or she deems necessary. Some defects can get worse over time so it is always best to take extra caution.
      Medication
      Sometimes medication is required. Some examples of medicines used to treat CHD include:
      • Antiarrhythmics - controls irregular heartbeats
      • Cardiac glycosides - increases strength of heartbeats
      • Vasodilators - enlarges blood vessels
      • Diuretics - reduces extra fluid (usually used in cases of heart failure)
      • Prostaglandins and prostaglandin inhibitors - improves blood flow to the lungs or body
      Catherization
      Some who have CHD need to undergo a heart catherization. This is done in order to find out the details of the heart defect or to sometimes to repair it. With heart catherization, a catheter is threaded through a blood vessel, usually in the groin and into the heart. This procedure allows the doctor see how blood is flowing through the heart and heart arteries.
      Surgery
      Sometimes treatment includes surgery. The type of surgery depends on the type of heart defect. There are many different kinds of surgeries to treat several different heart defects. If the defect is life-threatening, surgery might be done right away in a newborn. For other defects, surgery is usually done within the first two years of the child's life. Sometimes a child has two, three or more surgeries within the first years of the child's life.
      There are cases where all three types of treatment - medicine, catherization and surgery - may be required. Also, it is important to know that just because a child has had corrective surgery does not mean they are cured. A congenital heart defect is something the child is going to receive ongoing care for, or at least be seen by a pediatric or a congenital adult cardiologist on a regular basis
      .






      Signs and symptoms are related to the type and severity of the heart defect. Symptoms frequently present early in life, but it's possible for some CHDs to go undetected throughout life. [3] Some children have no signs while others may exhibit shortness of breath, cyanosis, syncope, [4] heart murmur, under-developing of limbs and muscles, poor feeding or growth, or respiratory infections. Congenital heart defects cause abnormal heart structure resulting in production of certain sounds called heart murmur. These can sometimes be detected by auscultation; however, not all heart murmurs are caused by congenital heart defects.