Home • What is CHD? • Categories And Conditions • How Can I Help? • Español
Index Categories (still adding... keep in tune for more categories) While we were browsing the web to look for all the categories we found at least 43 categories. Here are some of those categories.
If any doctor, obstetrician or pediatrician is looking this information and notice that any update is needed or information need to be added just contact us at godtoday@live.com. |
|
|
Aortic stenosis is a narrowing of the aortic valve or a narrowing of the aorta directly above (supravalvar) or below (subaortic) the aortic valve. Normally, oxygen-rich blood is pumped from the left ventricle, through the aortic valve, into the aorta and then out to the body. With aortic stenosis, it makes it very hard for the heart to pump blood to the body. Depending on the severity of the stenosis, open heart surgery may be needed to correct the defect. Another option may be a balloon valvuloplasty.
Subaortic stenosis refers to a narrowing of the left ventricle just below the aortic valve, which blood passes through to go into the aorta. This stenosis limits the flow of blood out of the left ventricle. This condition may be congenital or may be due to a particular form of cardiomyopathy known as "idiopathic hypertrophic subaortic stenosis" (IHSS).
Supravalvular aortic stenosis (SVAS) is a congenital narrowing of the ascending aorta which can occur as a congenital defect itself or as one component of Williams syndrome.
Congenital aortic stenosis occurs in 3 to 6 percent of all children with congenital heart defects. Relatively few children are symptomatic in infancy, but the incidence of problems increases sharply in adulthood. Congenital aortic stenosis occurs four times more often in boys than in girls. |
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. |
| back to top | back to top |
Atrioventricular Septal Defect (also known as atrioventricular canal defect, or AV canal)
This defect is usually corrected in infancy with open heart surgery to restore normal blood circulation through the heart. Surgical repair consists of separation of the common AV valve into two valves, along with closure of the VSD and ASD. Some children, however, may have too complex a defect to correct in infancy, and would initially require a pulmonary artery banding. This will decrease blood flow and reduce the high pressure in the lungs. When the child is older, the band will be removed and the defect corrected with open heart surgery.
Atrioventricular canal occurs in two out of every 10,000 live births, and equally in boys and girls.
|
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.
|
| back to top | back to top |
Congenitally Corrected Transposition of the Great Arteries (CCTGA)
In this congenital heart defect the ventricles are switched so that the left ventricle pumps blood to the lungs, and the right ventricle pumps blood out the aorta. In this defect, the position of the two ventricles is reversed so that the right atrium enters the left ventricle, and the left atrium enters the right ventricle. With this arrangement blood is flowing through the wrong ventricles, but it is still going in the correct direction, hence the term "congenitally corrected transposition". This defect is commonly associated with ventricular septal defect, pulmonic stenosis, heart block and an Ebstein-like malformation of the tricuspid valve. The normal right ventricle pumps blood to the lungs at a low pressure (about 25 mmHg). The left ventricle, pumping blood to the body, pumps at whatever pressure your blood pressure is (about 120 mmHg). With time, since the right ventricle is not built to pump to such a high pressure as the left ventricle, it may weaken, dilate, and cause symptoms.
This is an uncommon defect occurring in less than 1% of all people with congenital heart defects. Many people with this defect may live into adulthood before the defect is diagnosed and before symptoms occur. |
Complete Heart Block (Complete AV Block)
In this defect, the heart's electrical signal doesn't pass from the heart's own natural pacemaker in the atrium to the lower chambers. When this occurs, an independent pacemaker in the lower chambers takes over. The ventricles can contract and pump blood, but at a slower rate than the atrial pacemaker. Complete heart block is most often caused in adults by heart disease or as a side effect of drug toxicity. Heart block also can be present at birth. This is called congenital heart block. It also may result from an injury to the electrical conduction system during heart surgery. When the pacemaker in the ventricles isn't fast enough or reliable enough, an artificial pacemaker is put in. Heart block can be of varying degrees:
Congenital heart block, when detected at or before birth in a structurally normal heart, is strongly associated with autoantibodies reactive with certain proteins. To read more about this condition and possibly take part in the National Research Registry for Neonatal Lupus, click here: Rheumatology Neonatal |
| back to top | back to top |
|
The aorta is the main artery that sends oxygen-rich blood from the heart to the body. Coarctation of the aorta is a constricted segment of the aorta that obstructs blood flow to the body. The left ventricle has to pump harder because the pressure is high. Because of this, the heart may enlarge. Coarctations most often occur as isolated defects, but may occur with a ventricular septal defect, subaortic stenosis, or complex congenital heart defects. Surgery may be needed to correct the defect, depending on the severity of the coarctation and the presence of other congenital defects. Another option may be a balloon angioplasty.
Coarctation of the aorta occurs in about 6 to 8 percent of all children with congenital heart disease. Boys have the defect twice as often as girls do. |
Left coronary artery attached to the right side of the heart along with the right coronary artery. For more information see this site at the University of Washington. |
| back to top | back to top |
DiGeorge Syndrome DiGeorge syndrome is a complex birth defect. In most cases there is a chromosomal defect on chromosome 22. DiGeorge consists of a particular group of symptoms frequently occurring together, including the following:
Statistically, patients with DiGeorge have the following:
|
A very rare congenital heart defect in which both the pulmonary artery and the aorta arise from the right ventricle. Normally, only the pulmonary artery that carries blood to the lungs for oxygen arises from the right ventricle. The aorta, which carries oxygenated blood from the heart to the body, normally arises from the left ventricle. This defect almost always coincides with a ventricular septal defect (VSD) an abnormal opening in the septum.
In the case of DORV, a ventricular septal defect is helpful because it allows the oxygen rich blood of the left ventricle passage to the right ventricle, which pumps it to the aorta and out of the heart to the body. If didn't exist, there would be no way for oxygen-rich blood to get to the aorta. Still, this oxygen rich blood mixes with oxygen poor blood, so oxygen levels in the blood are not optimal, and the heart must work extra hard to maintain circulation.
There are four variations dependitn in the position of the aorta:
The DORV will develop at the 8th week during the fetal stages. Some congenital defects can be genetically transmited but most of the time is by chance. No gene is involved.
Some of the symptosn may include (but may differs in each children):
If a VSD is present the symptons may vary.
If you noticed you child get bluish (Cyanotic), call your doctor. This condition is part of the cyanotic defects, considered one of the most severe categories for CHD. There several procedures to diagnose DORV which include:
|
| back to top | back to top |
|
Dextrocardia litterally means "heart on the right". If the developing heart tube bends to the left instead of the right, then the heart is displaced to the right and develops in a mirror image of its normal state. It is interesting to note that identical twins are sometimes "mirror images" of each other, one having organs in the normal positions and one having them on the opposite side of the body. This is a condition called situs inversus.
Having dextrocardia does not mean the heart is defective, it just means that it is on the right instead of the left side of the body. Assuming there are no associated vascular abnormalities, then the heart functions normally.
In cases where the heart is the only organ which is transposed, known as isolated dextrocardia, there are usually other severe cardiac abnormalities. Dextrocardia can complicate heart defect treatments in that it can make surgery even more difficult, and heart transplants for patients with dextrocardia are more difficult since the anatomy of the donor and recipient don't match. |
Double (or Dual) Chamber Right Ventricle (DCRV) DCRV is a rare defect (0.5 - 2% CHD patients have this one) where there is, basically, extra muscle in the right ventricle that causes it to function as 2 ventricles. It almost always presents with a VSD.
See www.emedicine.com/ped/topic612.htm for more information. |
| back to top | back to top |
Ebstein's Anomaly This defect is a downward displacement of the tricuspid valve (located between the heart's upper and lower chambers on the right side) into the heart's right bottom chamber (or right ventricle). It's usually associated with an atrial septal defect. While there is free flow of blood forward across the tricuspid valve to the right ventricle, the deformed tricuspid valve allows a large amount of blood to flow backwards from the right ventricle to right atrium when the right ventricle contracts. About 10% of cases are associated with chronic maternal lithium use. The treatment of this disorder depends on whether or not the person with it has any symptoms. Surgery is sometimes required early in life. On the other hand, people may have a normal life expectancy. Irregular and fast heartbeats (arrhythmia) frequently accompany this condition. |
Endocardial Fibroelastosis (EF) Endocardial Fibroelastosis is a rare heart disorder that affects infants and children. It is characterized by an abnormal thickening of heart tissue, especially around the valves, causing abnormal enlargement of the heart (cardiac hypertrophy), especially affecting the left ventricle. Impaired heart and lung function can eventually lead to congestive heart failure. It can cause valve failure and sudden death. Endocardial Fibroelastosis may occur for no apparent reason (sporadic) or may be inherited as an X-linked or autosomal recessive genetic trait. |
| back to top | back to top |
Eisenmenger's Syndrome Eisenmenger's complex is a ventricular septal defect combined with pulmonary high blood pressure, the passage of blood from the right side of the heart to the left (right to left shunt) and an enlarged right ventricle. It may also include a malpositioned aorta that receives blood from both the right and left ventricles (an overriding aorta). Without early surgical correction of the underlying defect, such changes may cause progressive damage small blood vessels in lung tissue (pulmonary vascular disease). |
|
| back to top | back to top |
Hypertrophic Cardiomyopathy (HCM, HOCM, IHSS) The main feature of Hypertrophic Cardiomyopathy is an excessive thickening of the heart muscle. Heart muscle may thicken in normal individuals as a result of high blood pressure or prolonged athletic training. In Hypertrophic Cardiomyopathy (HCM), however, the muscle thickening occurs without an obvious cause. The condition has been known by a number of other names including "hypertrophic obstructive cardiomyopathy" (HOCM), "idiopathic hypertrophic sub-aortic stenosis" (IHSS) and "muscular sub-aortic stenosis" The general term "hypertrophic cardiomyopathy" is now the most widely used and recommended. Cardiomyopathy differs from many of the other disorders of the heart in several ways, including the following:
See The Cardiomyopathy Association for more information.
Hypoplastic Right Heart Disease (Pulmonary Atresia)
See this Link for more information
Interupted Aortic Arch (IAA)
Kawasaki Disease
Kawasaki disease is fairly common in the US. According to the American Heart Association, the illness is a major cause of heart disease in children. About 1,800 new cases are diagnosed in the US each year, and the incidence is on the rise. Kawasaki disease has replaced acute rheumatic fever as the leading cause of acquired heart disease in children in the US and Japan.
Kawasaki disease occurs more often in Japan than in any other country. In the US, children of Asian or Asian-American heritage are affected more often than other races, although Kawasaki disease can occur in any racial or ethnic group. The vast majority of children who develop Kawasaki disease are under age 5. The average age child seen with the illness is 2 years old. It occurs in boys twice as often as in girls. |
Hypoplastic Left Heart Syndrome (HLHS) Hypoplastic left heart syndrome, or HLHS for short, means that the left side of the heart did not develop normally. Therefore, the mitral and aortic valves are usually tiny or absent, as are the the left ventricle and the first part of the aorta. Perhaps the most critical defect in HLHS is the small, underdeveloped left ventricle. In a normal heart, this chamber is very strong and muscular so it can pump blood to the body. When the chamber is small and poorly developed, it will not function effectively and cannot provide enough bloodflow to meet the body's needs. For this reason, an infant with HLHS will not live long without surgical intervention. Parents are given a number of options depending on when the diagnosis is made potentially including -- abortion of the fetus, compassionate care (no surgical intervention which results in the baby passing away usually within the first two weeks of life) or one of a number of surgical option.
There are two, possibly three, surgical options; one is cardiac transplantation, in which the heart is replaced by a donated heart,; another is a 3-staged surgical procedure and the last surgical option is only available for a subset of HLHS patients and is called a biventricular repair.
The three-step procedure is called "the Norwood" because the first operation is called the Norwood Procedure. The Norwood is done very soon after birth, usually within the first few days of life. The second step is called the hemi-Fontan (which is frequently a bi-directional Glenn operation). It sends half of the blood returning from the body to the lungs, reducing the workload on the heart. This second step is part of the preparation to transform the HLHS heart into a two-chamber pumping heart which will only function to pump blood to the body (after the third operation, all of the blood will travel passively to the lungs). The third operation is called the Fontan operation. In this operation, the other half of the blood returning from the body to the heart is sent instead to the lungs. In what used to be a uniformly fatal disease, consider the following statistics from the very best facilities treating HLHS:
These statistics are not representative of EVERY facility treating HLHS, so it is imperative parents ask their children's doctors for information BEFORE a treatment facility is chosen -- if there is time. This is one of the advantages of finding out about the HLHS diagnosis in utero.
Most patients who get through the three stages do quite well. They are able to lead a fairly normal life with few restrictions. Most patients have to be on some kind of anticoagulant (like baby aspirin), many need Digoxin, some kind of diuretic (at least for some time post-operatively) and/or medications to control blood pressure. Of course they have to take antibiotics prophylactically, as do other children with severe, congenital heart defects.
The biventricular repair is only available for a small subset of HLHS infants whose left ventricle is small, but not too small. The biventricular repair is actually a series of operations which must be performed in stages, like the Norwood Procedure; however, instead of converting the heart into a two-chamber pumping heart, the bi-ventricular repair encourages growth of the small left ventricle so that ultimately the child will have a fully functional, four-chamber pumping heart. Not as many facilities have experience performing this fairly new procedure, so some research on the part of the parents may be needed to find the ideal facility for their child.
Hypoplastic left heart syndrome occurs in up to four out of every 10,000 live births. It is one of the top three heart abnormalities to cause problems in the newborn. HLHS occurs slightly more often in boys than in girls. |
| back to top | back to top |
|
An overriding aorta is a congenital heart defect where the aorta is positioned directly over a ventricular septal defect, instead of over the left ventricle. The result is that the aorta receives some blood from the right ventricle, which reduces the amount of oxygen in the blood. It is one of the four conditions of the Tetralogy of Fallot. The other conditions are Pulmonary Stenosis, RVH, and VSD (ventricular septal defect).
|
Pulmonary Stenosis (PS)
Depending on the severity of the pulmonary stenosis, open heart surgery may be needed to correct the defect. Another option may be a balloon valvuloplasty. This procedure is done in the cardiac catheterization lab.
Pulmonary stenosis is the second most common congenital heart defect, comprising 5 to 10 percent of all cases. It is a component of half of all complex congenital heart defects. |
|
This condition includes four conditions into the CHD categories:
Tetralogy means four part things, that's why we can describe Tetralogy of Fallot as Fallot's four part condition. Basically is an abnormal development and congenital malformations. No one knows exactly what promote this condition but many are sure in not a genetic passover. There's a sever variant of this condition when it's combined with other CHD condition, it's call Tetralogy Of Fallot With Pulmonary Atresia or Pseudotruncus Arteriosus.
The primary symptom of Tetralogy of Fallot is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. This occurs because of the resistance of blood flow through the pulmonary valve. Once the blood flows into the left ventricle, it is ejected into the aorta and delivers de-oxygenated blood into the body. Because there is de-oxygenated blood being delivered to the body, these babies may appear cyanotic, or "blue". Open heart surgery is needed to correct this defect. Cyanosis is the term used to describe the blue gray skin coloration provoked by the low levels of oxygen in the blood or lungs.
Tetralogy of Fallot is treated surgically. First, if the infant is too small the doctors will practice a temporary operation where positioned a shunt to provide adequate blood flow to the lungs, it's only practice is the baby is severly cyanotic. This operation will allow the baby to grow big enough to have a full repair.
A simple chest x-ray is enough to diagnose the presence of TOF. The heart may look as "boot-like" appearance, rather than the symmetric appearance of a normal heart. The most common method to diagnose this condition, or any other congenital heart defect, is with echocardiography. It is quick, involves no radiation and is very specific. And it can be practice even in the fetal stage. Ask your obstetrician about it.
Tetralogy of Fallot occurs in about five out of every 10,000 live births. Tetralogy of Fallot occurs equally in boys and in girls. |
In tricuspid atresia, there's no tricuspid valve so no blood can flow from the right atrium to the right ventricle. As a result, the right ventricle is small and not fully developed. Survival depends on there being an opening in the wall between the atria (atrial septal defect) and usually an opening in the wall between the two ventricles (ventricular septal defect). As a result, the venous (bluish) blood that returns to the right atrium flows through the atrial septal defect and into the left atrium. There it mixes with oxygen-rich (red) blood from the lungs. Most of this poorly oxygenated mixture goes from the left ventricle into the aorta and on to the body.
The rest flows through the ventricular septal defect into the small right ventricle, through the pulmonary artery and back to the lungs. Often in these children it's necessary to do a surgical shunting procedure to increase blood flow to the lungs. Some children with tricuspid atresia have too much blood flowing to the lungs. They may need a procedure (pulmonary artery banding) to decrease blood flow to the lungs. Other children with tricuspid atresia may have a Fontan procedure. In this, a connection is created between the right atrium and pulmonary artery. The atrial defect is also closed. This eliminates the cyanosis but, without a right ventricle that works normally, the heart can't work totally as it should.
Tricuspid atresia occurs in two out of every 10,000 live births. It makes up 1 to 2 percent of all cases of congenital heart disease. TA occurs equally in boys and girls. |
|
In this defect, only one artery originates from the heart and forms both the aorta and the pulmonary artery. The truncus arises above a VSD that is almost always associated with this defect. The truncus receives low-oxygen blood from the right ventricle and oxygen-rich blood from the left ventricle. This mix of high and low-oxygen blood is sent out to the body and to the lungs. Open heart surgery in infancy is needed to correct this defect. The surgery involves closure of the VSD and removal of the pulmonary arteries from the truncus. The pulmonary arteries are then connected to the right ventricle with a prosthetic tube. This prosthetic tube usually needs to be replaced as the infant grows.
Truncus arteriosus occurs in less than one out of every 10,000 live births. It makes up 1 percent of all cases of congenital heart disease. |
Ventricular Septal Defect (VSD) Is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes. The membranous portion, which is close to the atrioventricular node, is most commonly affected in adults and older children. Congenital VSDs are collectively the most common congenital heart defects.
A cardiac auscultation is generally considered sufficient for detecting a significant VSD. Confirmation of cardiac auscultation can be obtained by non-invasive cardiac ultrasound (echocardiography). To more accurately measure ventricular pressures, cardiac catheterization, can be performed.
Treatment is either conservative or surgical. Smaller congenital VSDs often close on their own, as the heart grows, and in such cases may be treated conservatively. In cases necessitating surgical intervention, a heart-lung machine is required and a median sternotomy is performed. Percutaneous endovascular procedures are less invasive and can be done on a beating heart, but are only suitable for certain patients. Repair of most VSDs is complicated by the fact that the conducting system of the heart is in the immediate vicinity. VSD in infants is initially treated medically with digoxin(10-20ug/kg per day) and furosemide (1-3 mg/kg per day) and captopril (0.5-2 mg / kg per day). |
| back to top | back to top |
Information provided by CHD Awareness in PR (Christine Méndez) |
Last Review June 2008 |
This website is designed by Revolution Visual Arts ©2008 by Media-Ministry.org. All images are registered trademarks of Media-Ministry.org. Unathorized duplication of all or partial information will be prosecuted by law. Revolution Visual Arts is Rafael Vila, Senior Designer • Estella Vila, Marketing and Public Relations • Elsa Tapia • Media Advisor. All Rights Reserved ®2008
• God Today Free Newsletter • Revolution Visual Arts • NuestroMySpace.com •