Every now and then his heart and blood vessels all at birth not capable enoughto keep circulation moving. Congenital heart disease with serious consequencescomes in at less than 1% of the Netherlands for newborn children.
Treatment of congenital heart disease is greatly improved in the last forty years.Successful above all new surgical techniques, preferably performed shortly afterbirth as the heart damage is still limited. Formerly deceased people with severe congenital heart disease is often at a young age, nowadays they lead after an early operation a virtually normal life. They are just old, creating a new group ofheart patients has arisen: adults with congenital heart disease. The consequences can be for adults differ from person to person, depending on the nature of theirheart defect. The one quickly becomes tired and short of breath, another with the same abnormality in a mild form is nothing more than sports.
Congenital heart disease occur during the embryonic stage, as heart and blood vessels are formed. In this early stage can easily something go wrong. The cause of congenital heart disease is wanted in a combination of factors from inside the body, such as in the case of a hereditary predisposition, and factors from outside, such as toxins or infections.
A small mistake in this formative phase can have a big impact. What is the most common by far in Netherlands is a hole in the wall between the left and right half of the heart, allowing the low oxygen and the oxygen rich blood flows mingle. A hole in the partition of the rooms on the first place of the list of congenital heart disease in newborn children in Netherlands (42 percent). In second place comesbetween a hole in the wall of the atria (9 percent). But because the hole in theroom wall itself in childhood spontaneously connect, the hole in the atrial wallamong adults more for.
Other congenital heart diseases are quite rare. We can discuss here any morethan three. The first is the tetralogy of Fallot: a hole in the room wall incombination with three other serious defects. The second and third are related tothe aorta, the largest artery with oxygenated blood. The aorta may be narrowed(coarctation of the aorta) or widened with danger of schism (Marfan syndrome).
From apple seed to shrimp
Congenital heart disease often occur in the first weeks of a lifetime, to be in the sixth to the tenth week when the embryo is a growth spurt exactly. In one month the embryo grows explosively from a shapeless lump the size of an apple seed to a person with limbs and a head the size of a shrimp.
In this phase, also creates the basic shape of the heart. This begins with the formation of specialized cardiac muscle cells, which then merge in the form of a tube. Then this tube folds in half and melt together, making the cavities of the heart form, a process that is reminiscent of glassblowing. In the tenth week, the heart broadly its final form. The recipe for the growth process is in the DNA, the genetic material in the nucleus of the cells.
Heart and circulation before and after birth
The circulatory system is very different from before birth after birth. Before birth, the lungs are collapsed and the baby receives oxygen through the placenta. The resistance in the collapsed lung is high and the blood flows through it piecemeal. Because the lungs are not doing their job, the circulation to the lungs are not yet separated from the rest. Through a hole in the wall between the atria, the foramen ovale, are the two halves of the heart in connection with each other. A large artery also connects the pulmonary artery to the aorta, the ductus arteriosus.
After birth, the umbilical cord is cut and the connection to the placenta. The newborn sucks air into his lungs, showing them unfold and will release oxygen to the blood. In the first days after birth, the lung develops further and adjusts itself to the circulatory system. There occur pressure differences between the left and right halves of the heart, causing a flap against the foramen ovale is pressed and the hole, after a period of time closes. The ductus arteriosus itself closes.
Cause is often unknown
We know that occasionally something goes wrong during the growth spurt in the first weeks, but we know in most cases do not exactly know why. The process is so complex that it is actually just a miracle that so often goes well. In no more than one out of ten cases, it is possible to cause a - or a part of the cause - to designate with certainty.
For example, the cause is a toxic substance that the placenta can not stop (drug, alcohol), an infectious disease (rubella) or an abnormality in the DNA. The future will tell whether it is possible to determine the exact cause. Of all congenital heart disease The growth of the heart in the first weeks is so complex that it is really a wonder that so often goes well.
Hereditary heart disease
Is the cause of heart disease hereditary predisposition, it will usually appear from the family history. A DNA test can confirm the genetic predisposition, in certain cases, but not always. In a hereditary disease, you are at the hospital referred to a specialized department, the department of clinical genetics. An example of a hereditary disease, Marfan's syndrome, which is discussed below. Other examples of hereditary heart diseases, certain forms of cardiomyopathy or heart arrhythmias.
An abnormality in the DNA does not always you inherit from your parents, incidentally. Such a deviation can also occur spontaneously, without the existence of genetic predisposition.
Discovery of congenital heart disease
The majority of congenital heart disease is detected in the first year of life, sometimes shortly after birth because the baby is striking blue and short of breath. But beware: not all blue and wheezy babies have congenital heart disease. Newborn babies do not have strong lungs and are often some blue and short of breath, even if it has nothing to do with heart disease.
It also appears that an adult has long tired and short of breath and feel confronted in the hospital with congenital heart disease. Congenital heart disease can often be relatively easily detected on an ultrasound of the heart. Adulthood
Risk of ignition
Almost all people with congenital heart disease have an increased risk of endocarditis. This disease, an inflammation of the lining of the heart, is very dangerous. People with a congenital heart disease therefore be given in certain cases, in order to keep preventive antibiotics. The risk of endocarditis as small as possible,
We all have a hole in the septum
We are all born with a hole in the wall between the atria (foramen ovale). Before birth hole that had a function (see above), but after the birth, when the oxygen-poor blood to be separated on their way to the lungs of oxygen-rich blood gives the correct hole problems. In three out of four people saying the hole closes shortly after birth, and one in four of the passage remains open. Evil may be that in most cases do not, because the hole is then sealed by a flap. Blood can only under special circumstances - flowing from one breast to the other - for example, if the flap is pushed away by a hard sneeze.
Sometimes a patent foramen ovale shows some problems. There are then in the turbulence around the hole blood clots that are carried to the brains where they can cause a stroke. Via the bloodstream At an increased risk of stroke coagulation-inflammatory drugs, there are prescribed, such as aspirin. If the medications do not help, the hole can be closed by catheterization surgically. A catheter with an expandable prosthesis is brought to the heart through the circulatory system to close the gap. This is a little invasive surgery.
Hole in the chamber wall: VSD
A hole in the wall (septum) between the chambers (ventricles) is called a ventricular septal defect or VSD. A VSD is the most common congenital heart defect at birth. In adulthood is much less common, because the hole in most people closing up. Or the hole to cause problems in adulthood, is dependent on the size and location. The size may vary from a few millimeters to a few centimeters, and the hole can be located on a place difficult to treat, for example, in a heart valve.
Hole in the atrial wall: ASD
A hole in the wall (septum) between the atria called an atrial septal defect or ASD (atrial = chest). An ASD is the most common congenital heart disease in adulthood. Often, an ASD until adulthood symptoms, such as fatigue, shortness of breath, or abnormal heart rhythms. A large ASD is an extra burden on the heart muscle, which in time can have more than the right ventricle to the left ventricle serious consequences. It is better if the ASD already discovered at a young age because if it is treated early, the outlook is better. An ASD can be closed. In a minor surgery.
Tetralogy of Fallot
Tetralogy is a series in four parts, including a trilogy series in three parts. The nineteenth-century physician Etienne Fallot described a syndrome in which he distinguished four congenital heart defects: a hole in the wall between the rooms (VSD), a narrowing of the pulmonary artery of the heart valve, an abnormally constructed beginning of the aorta and a thickened heart muscle. Fallot was not the first to describe the syndrome, but it got its name: tetralogy of Fallot.
Actually, it is not four heart defects, but a single abnormality that affects four parts of the heart. The one exception is the wrong landscaped divider. The abnormalities of the pulmonary artery and the aorta flow from it, the thickened heart muscle caused by a prolonged extra effort from the right ventricle.
By the narrowing in the pulmonary artery, the right ventricle the blood can not properly direction lungs and pumps the oxygen depleted blood is mixed through the hole in the chamber wall with the oxygenated blood in the left ventricle. That means that someone with tetralogy of Fallot is a mixture of oxygen and oxygen-rich blood in his arteries. How oxygen poorer the blood, the more bluish skin color. So you can sometimes read the severity of the clinical picture in a baby skin color. Is the narrowing in the pulmonary artery severe, then the color blue. Is the narrowing less severe than the baby pink color. Etienne Fallot described the disease as the blue disease.
Nowadays, tetralogy of Fallot treated by open heart surgery, in which the narrowing in the pulmonary artery is made wider and the hole in the chamber wall filled. The operation is preferably carried out in the first year of life, when the damage is still limited and the heart muscle has not had much to endure. After surgery, there is an almost normal life expectancy, although treatment remains necessary and there is an increased risk of endocarditis and valvular lesions.
Coarctation of the aorta
Coarctation means narrowing of an artery, not by silting up from the inside as in artery disease, but by a constriction from the outside. Think of the shape of an hourglass. The result is the same, namely that there is less blood can flow through the tube. The aorta is the main branch for the oxygen-rich blood, which is directly connected. On the left chamber of the heart A narrowing therefore has serious implications, not only for the oxygen supply to the body but also for the left ventricle of the heart, which may be overloaded because the blood must be pumped. Through a narrow opening
A coarctation of the aorta is usually arises at the place where an artery is running for the birth between the pulmonary artery and the aorta, the ductus arteriosus. This artery should be opened only for the birth after birth comes unexpectedly close the vessel (see the beginning of this chapter). Apparently there is a coarctation something wrong with the construction of this particular artery, but the exact cause of congenital heart disease is not known.
This accident is not alone. In about half the cases, the coarctation is associated with other congenital heart defects. Especially valve disorders are common, in particular, an abnormal aortic valve with two slip instead of three. Such a bicuspid aortic valve (bicuspid =) called aortic valve. Not infrequently there is also a hole in the wall between the rooms (VSD) or other heart abnormalities.
Marfan's syndrome
Marfan syndrome is named after the French pediatrician Antoine Marfan, who described the syndrome at the end of the nineteenth century for the first time. It is in most cases, a hereditary disease, but there are also cases of Marfan are known in which the deviation in the DNA occur spontaneously.
The abnormal DNA causes a problem with the synthesis of connective tissue cells. As a result, the connective tissue is weakened, and thereby also the aorta.
A normal aorta is packed into connective tissue which provides firmness as a kind of corset. When Marfan syndrome losses layers in the aorta wall in the course of the years, their binding effect and weakens the aortic wall. The aorta is gradually widening and parts of the inner let go, which ultimately may result. Fatal tear in the aorta
The treatment of Marfan syndrome is aimed to prevent. Such a fatal rupture of the aortic wall The best way to do that is an open heart surgery, in which the weak and dilated portion of the aorta is replaced. The operation is generally carried out only at a time when the diameter of the aorta has been broadened to 50 to 60 mm - that is to say more than twice the normal width - and the danger of a tear will be too large. That moment can vary greatly from person to person. After surgery, life expectancy in the short term excellent, but there is still relatively little known about the long term.
Treatment of congenital heart disease is greatly improved in the last forty years.Successful above all new surgical techniques, preferably performed shortly afterbirth as the heart damage is still limited. Formerly deceased people with severe congenital heart disease is often at a young age, nowadays they lead after an early operation a virtually normal life. They are just old, creating a new group ofheart patients has arisen: adults with congenital heart disease. The consequences can be for adults differ from person to person, depending on the nature of theirheart defect. The one quickly becomes tired and short of breath, another with the same abnormality in a mild form is nothing more than sports.
Congenital heart disease occur during the embryonic stage, as heart and blood vessels are formed. In this early stage can easily something go wrong. The cause of congenital heart disease is wanted in a combination of factors from inside the body, such as in the case of a hereditary predisposition, and factors from outside, such as toxins or infections.
A small mistake in this formative phase can have a big impact. What is the most common by far in Netherlands is a hole in the wall between the left and right half of the heart, allowing the low oxygen and the oxygen rich blood flows mingle. A hole in the partition of the rooms on the first place of the list of congenital heart disease in newborn children in Netherlands (42 percent). In second place comesbetween a hole in the wall of the atria (9 percent). But because the hole in theroom wall itself in childhood spontaneously connect, the hole in the atrial wallamong adults more for.
Other congenital heart diseases are quite rare. We can discuss here any morethan three. The first is the tetralogy of Fallot: a hole in the room wall incombination with three other serious defects. The second and third are related tothe aorta, the largest artery with oxygenated blood. The aorta may be narrowed(coarctation of the aorta) or widened with danger of schism (Marfan syndrome).
From apple seed to shrimp
Congenital heart disease often occur in the first weeks of a lifetime, to be in the sixth to the tenth week when the embryo is a growth spurt exactly. In one month the embryo grows explosively from a shapeless lump the size of an apple seed to a person with limbs and a head the size of a shrimp.
In this phase, also creates the basic shape of the heart. This begins with the formation of specialized cardiac muscle cells, which then merge in the form of a tube. Then this tube folds in half and melt together, making the cavities of the heart form, a process that is reminiscent of glassblowing. In the tenth week, the heart broadly its final form. The recipe for the growth process is in the DNA, the genetic material in the nucleus of the cells.
Heart and circulation before and after birth
The circulatory system is very different from before birth after birth. Before birth, the lungs are collapsed and the baby receives oxygen through the placenta. The resistance in the collapsed lung is high and the blood flows through it piecemeal. Because the lungs are not doing their job, the circulation to the lungs are not yet separated from the rest. Through a hole in the wall between the atria, the foramen ovale, are the two halves of the heart in connection with each other. A large artery also connects the pulmonary artery to the aorta, the ductus arteriosus.
After birth, the umbilical cord is cut and the connection to the placenta. The newborn sucks air into his lungs, showing them unfold and will release oxygen to the blood. In the first days after birth, the lung develops further and adjusts itself to the circulatory system. There occur pressure differences between the left and right halves of the heart, causing a flap against the foramen ovale is pressed and the hole, after a period of time closes. The ductus arteriosus itself closes.
Cause is often unknown
We know that occasionally something goes wrong during the growth spurt in the first weeks, but we know in most cases do not exactly know why. The process is so complex that it is actually just a miracle that so often goes well. In no more than one out of ten cases, it is possible to cause a - or a part of the cause - to designate with certainty.
For example, the cause is a toxic substance that the placenta can not stop (drug, alcohol), an infectious disease (rubella) or an abnormality in the DNA. The future will tell whether it is possible to determine the exact cause. Of all congenital heart disease The growth of the heart in the first weeks is so complex that it is really a wonder that so often goes well.
Hereditary heart disease
Is the cause of heart disease hereditary predisposition, it will usually appear from the family history. A DNA test can confirm the genetic predisposition, in certain cases, but not always. In a hereditary disease, you are at the hospital referred to a specialized department, the department of clinical genetics. An example of a hereditary disease, Marfan's syndrome, which is discussed below. Other examples of hereditary heart diseases, certain forms of cardiomyopathy or heart arrhythmias.
An abnormality in the DNA does not always you inherit from your parents, incidentally. Such a deviation can also occur spontaneously, without the existence of genetic predisposition.
Discovery of congenital heart disease
The majority of congenital heart disease is detected in the first year of life, sometimes shortly after birth because the baby is striking blue and short of breath. But beware: not all blue and wheezy babies have congenital heart disease. Newborn babies do not have strong lungs and are often some blue and short of breath, even if it has nothing to do with heart disease.
It also appears that an adult has long tired and short of breath and feel confronted in the hospital with congenital heart disease. Congenital heart disease can often be relatively easily detected on an ultrasound of the heart. Adulthood
Risk of ignition
Almost all people with congenital heart disease have an increased risk of endocarditis. This disease, an inflammation of the lining of the heart, is very dangerous. People with a congenital heart disease therefore be given in certain cases, in order to keep preventive antibiotics. The risk of endocarditis as small as possible,
We all have a hole in the septum
We are all born with a hole in the wall between the atria (foramen ovale). Before birth hole that had a function (see above), but after the birth, when the oxygen-poor blood to be separated on their way to the lungs of oxygen-rich blood gives the correct hole problems. In three out of four people saying the hole closes shortly after birth, and one in four of the passage remains open. Evil may be that in most cases do not, because the hole is then sealed by a flap. Blood can only under special circumstances - flowing from one breast to the other - for example, if the flap is pushed away by a hard sneeze.
Sometimes a patent foramen ovale shows some problems. There are then in the turbulence around the hole blood clots that are carried to the brains where they can cause a stroke. Via the bloodstream At an increased risk of stroke coagulation-inflammatory drugs, there are prescribed, such as aspirin. If the medications do not help, the hole can be closed by catheterization surgically. A catheter with an expandable prosthesis is brought to the heart through the circulatory system to close the gap. This is a little invasive surgery.
Hole in the chamber wall: VSD
A hole in the wall (septum) between the chambers (ventricles) is called a ventricular septal defect or VSD. A VSD is the most common congenital heart defect at birth. In adulthood is much less common, because the hole in most people closing up. Or the hole to cause problems in adulthood, is dependent on the size and location. The size may vary from a few millimeters to a few centimeters, and the hole can be located on a place difficult to treat, for example, in a heart valve.
Hole in the atrial wall: ASD
A hole in the wall (septum) between the atria called an atrial septal defect or ASD (atrial = chest). An ASD is the most common congenital heart disease in adulthood. Often, an ASD until adulthood symptoms, such as fatigue, shortness of breath, or abnormal heart rhythms. A large ASD is an extra burden on the heart muscle, which in time can have more than the right ventricle to the left ventricle serious consequences. It is better if the ASD already discovered at a young age because if it is treated early, the outlook is better. An ASD can be closed. In a minor surgery.
Tetralogy of Fallot
Tetralogy is a series in four parts, including a trilogy series in three parts. The nineteenth-century physician Etienne Fallot described a syndrome in which he distinguished four congenital heart defects: a hole in the wall between the rooms (VSD), a narrowing of the pulmonary artery of the heart valve, an abnormally constructed beginning of the aorta and a thickened heart muscle. Fallot was not the first to describe the syndrome, but it got its name: tetralogy of Fallot.
Actually, it is not four heart defects, but a single abnormality that affects four parts of the heart. The one exception is the wrong landscaped divider. The abnormalities of the pulmonary artery and the aorta flow from it, the thickened heart muscle caused by a prolonged extra effort from the right ventricle.
By the narrowing in the pulmonary artery, the right ventricle the blood can not properly direction lungs and pumps the oxygen depleted blood is mixed through the hole in the chamber wall with the oxygenated blood in the left ventricle. That means that someone with tetralogy of Fallot is a mixture of oxygen and oxygen-rich blood in his arteries. How oxygen poorer the blood, the more bluish skin color. So you can sometimes read the severity of the clinical picture in a baby skin color. Is the narrowing in the pulmonary artery severe, then the color blue. Is the narrowing less severe than the baby pink color. Etienne Fallot described the disease as the blue disease.
Nowadays, tetralogy of Fallot treated by open heart surgery, in which the narrowing in the pulmonary artery is made wider and the hole in the chamber wall filled. The operation is preferably carried out in the first year of life, when the damage is still limited and the heart muscle has not had much to endure. After surgery, there is an almost normal life expectancy, although treatment remains necessary and there is an increased risk of endocarditis and valvular lesions.
Coarctation of the aorta
Coarctation means narrowing of an artery, not by silting up from the inside as in artery disease, but by a constriction from the outside. Think of the shape of an hourglass. The result is the same, namely that there is less blood can flow through the tube. The aorta is the main branch for the oxygen-rich blood, which is directly connected. On the left chamber of the heart A narrowing therefore has serious implications, not only for the oxygen supply to the body but also for the left ventricle of the heart, which may be overloaded because the blood must be pumped. Through a narrow opening
A coarctation of the aorta is usually arises at the place where an artery is running for the birth between the pulmonary artery and the aorta, the ductus arteriosus. This artery should be opened only for the birth after birth comes unexpectedly close the vessel (see the beginning of this chapter). Apparently there is a coarctation something wrong with the construction of this particular artery, but the exact cause of congenital heart disease is not known.
This accident is not alone. In about half the cases, the coarctation is associated with other congenital heart defects. Especially valve disorders are common, in particular, an abnormal aortic valve with two slip instead of three. Such a bicuspid aortic valve (bicuspid =) called aortic valve. Not infrequently there is also a hole in the wall between the rooms (VSD) or other heart abnormalities.
Marfan's syndrome
Marfan syndrome is named after the French pediatrician Antoine Marfan, who described the syndrome at the end of the nineteenth century for the first time. It is in most cases, a hereditary disease, but there are also cases of Marfan are known in which the deviation in the DNA occur spontaneously.
The abnormal DNA causes a problem with the synthesis of connective tissue cells. As a result, the connective tissue is weakened, and thereby also the aorta.
A normal aorta is packed into connective tissue which provides firmness as a kind of corset. When Marfan syndrome losses layers in the aorta wall in the course of the years, their binding effect and weakens the aortic wall. The aorta is gradually widening and parts of the inner let go, which ultimately may result. Fatal tear in the aorta
The treatment of Marfan syndrome is aimed to prevent. Such a fatal rupture of the aortic wall The best way to do that is an open heart surgery, in which the weak and dilated portion of the aorta is replaced. The operation is generally carried out only at a time when the diameter of the aorta has been broadened to 50 to 60 mm - that is to say more than twice the normal width - and the danger of a tear will be too large. That moment can vary greatly from person to person. After surgery, life expectancy in the short term excellent, but there is still relatively little known about the long term.