ABC Associazione Bambino Cardiopatico, Parma.

CONGENITAL HEART DISEASES

Clinical features
A heart problem can manifest with different symptoms that alarm the parents and induce them to take a medical advice. The physician will check for some signs, for example a cardiac murmur, that will help him in the diagnosis.

Exercise intolerance: it is the most frequent symptom presented by a child with heart difficulties. In infants it appears with tiredness during feeding. In fact feeding is the heaviest activity in term of muscular work for infants, if there is an impairment in the cardiac function the infant is obliged to rest frequently during feeding. Often he doesn¹t manage to take adequate quantity of feed with the consequent slowing down or stop of growth. An other common symptom is respiratory distress associated with excessive sweating. In older children the exercise intolerance comes out during play, when they have difficulties in keeping up with peers and are obliged to rest because they are tired, breathless, pale and on sweating.

Cyanosis: is another common symptom which concern children with particular types of heart diseases that are defined cyanotic. The cyanosis consist in blue-purple color of skin and mucous membrane (lips, tongue) due to the fact that blood that t reached the organs and tissues through the arteries is not sufficiently oxygenated. Blood adequately oxygenated has a red color where as the less is the content in oxygen the more the color becomes blue. There are two major causes of cyanosis: heart causes in which venous blood because of a cardiac defect by-passes the lungs and goes down the aorta and the arteries without being oxygenated; lung causes in which the lung functions are impaired. There are numerous congenital cyanotic heart diseases the most frequent of which are Tetralogy of Fallot, complete Transposition of the Great Arteries and pulmonary atresia. Cyanosis determines a lack of oxygen for organs and tissues with the consequences that the child will have a reduce tolerance to exercise, growth failure, clubbing of the fingers and toes (swelling of ending phalanx associated with nails more convex than normal). The physiologic reaction to cyanosis is an increase in the red blood cells production, phenomenon called polycythemia and it is useful because with an increased number of red blood cells the blood capacity to carry oxygen is improved. In case of severe or long lasting cyanosis the rd blood cells number becomes excessive, blood becomes thicker with difficulties to flow, that can cause trouble on brain circulation. Children with blood thicker than normal are more susceptible to dehydration that may contribute to turn blood thicker; they should avoid hot weather and dispose of sufficient amount of liquid. Even exposure to cold is poorly tolerated because it increases tissues oxygen consumption. About exercise the child must feel free to find his own limit and should never be obliged to do physical activities he doesn¹t want to do.

Sometimes cyanosis represent a normal phenomenon. New-born have acrocyanosis (cyanosis of the extremities) due to immaturity of the peripheral circulation in the neonate. Even when they cry vigorously holding the breath new-born can develop cyanosis; there can be a suspicion of cyanosis in children who present a blue staining around the mouth after exposure to cold o a bath with cold water ; this is frequent in fair-haired children with pale skin because it is easy to see the color of the venous blood through a skin without pigment.

Heart failure is a suite of symptoms and clinical manifestations deriving from the incapacity of the heart to accomplish his normal function. The cardiac output is reduced, and organs do not receive an adequate amount of blood and their function is decreased. For examples muscles cannot work properly and child will get tired easily, kidney will have a decreased function and the child will urinate less and as a consequence he will keep liquid in blood and tissues. Another consequence of heart function impairment is the increase of blood pressure in veins that get to the heart. At lung level the increase in venous pressure can provoke a range of event which goes from dyspnea to pulmonary edema. The rise of pressure in heart right side and together with liquid storage provoke liver enlargement (hepatomegaly). The increased veinous pressure and the liquid retention are the cause of peripheral edema which is the swelling of legs tissues.

Heart fluttering is the sensation of a heart beating to much swiftly or irregularly. The increase in heart rate occurs normally during an exertion or an emotion, but some people can have an increase o irregularities of heart rate without warning and without an apparent reason. This generates the sensation of fluttering which is due in the majority of cases to an impairment of cardiac rhythm o arrhythmia. Generally fluttering is not dangerous, even if it can provoke anguish, because it is unpleasant to feel the proper heart beating swiftly without reason. If fluttering is short-lasting there is no problem for the child, but if they last more than half an hour, it is advisable to call on a physician or an hospital to make an examination and an ECG. Generally arrhythmia¹s that cause fluttering are characterized by an heart rate superior to normal (tachycardia) or irregularities of heart rhythm. Other arrhythmia¹s induce an heart rate inferior to normal and are called bradycardia. Arrhythmia¹s can appear in children with o without cardiac malformation or children who underwent surgery for heart defects. The majority of arrhythmia¹s are benign, but in some cases if heart rate is too high or too slow for a sufficient time cardiac function can be compromised.

Cardiac murmurs are sound that are appreciated by the physician during heart auscultation. Murmurs are present even in children with normal heart, thus the presence of a murmur does not necessarily mean presence of heart problem. Murmurs present in child with normal heart are called innocent or functional murmur to distinguish them from the pathologic murmurs which sign an heart disease, for example a valvular problem or abnormal communication between cardiac chambers. More than 50% of the children have an innocent murmur which is recognized on its auscultatory characteristics and on the basis of laboratory examination performed with ECG, chest x-ray and echocardiography. A child with an innocent murmur has an healthy heart and can do every kind of physical activity. Innocent murmur generally appear during puberty.

Respiratory infections are more frequent in children with heart diseases that cause an increase in pulmonary blood flow 4atrial septal defect, ventricular septal defect, endocardial cushion defect, patent ductus arteriosus). This is due to the fact that increased pulmonary blood flow obstruct the system that normally defends bronchi and lungs from infections. Once settled, respiratory infections usually last more than in children without heart disease.

Chest pain is rarely connected with a cardiac problem in children. It can occur in children with severe aortic stenosis and few other situations.

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	CONGENITAL HEART DISEASES Clinical features A heart problem can manifest with different symptoms that alarm the parents and induce them to take a medical advice. The physician will check for some signs, for example a cardiac murmur, that will help him in the diagnosis. Exercise intolerance: it is the most frequent symptom presented by a child with heart difficulties. In infants it appears with tiredness during feeding. In fact feeding is the heaviest activity in term of muscular work for infants, if there is an impairment in the cardiac function the infant is obliged to rest frequently during feeding. Often he doesn¹t manage to take adequate quantity of feed with the consequent slowing down or stop of growth. An other common symptom is respiratory distress associated with excessive sweating. In older children the exercise intolerance comes out during play, when they have difficulties in keeping up with peers and are obliged to rest because they are tired, breathless, pale and on sweating. Cyanosis: is another common symptom which concern children with particular types of heart diseases that are defined cyanotic. The cyanosis consist in blue-purple color of skin and mucous membrane (lips, tongue) due to the fact that blood that t reached the organs and tissues through the arteries is not sufficiently oxygenated. Blood adequately oxygenated has a red color where as the less is the content in oxygen the more the color becomes blue. There are two major causes of cyanosis: heart causes in which venous blood because of a cardiac defect by-passes the lungs and goes down the aorta and the arteries without being oxygenated; lung causes in which the lung functions are impaired. There are numerous congenital cyanotic heart diseases the most frequent of which are Tetralogy of Fallot, complete Transposition of the Great Arteries and pulmonary atresia. Cyanosis determines a lack of oxygen for organs and tissues with the consequences that the child will have a reduce tolerance to exercise, growth failure, clubbing of the fingers and toes (swelling of ending phalanx associated with nails more convex than normal). The physiologic reaction to cyanosis is an increase in the red blood cells production, phenomenon called polycythemia and it is useful because with an increased number of red blood cells the blood capacity to carry oxygen is improved. In case of severe or long lasting cyanosis the rd blood cells number becomes excessive, blood becomes thicker with difficulties to flow, that can cause trouble on brain circulation. Children with blood thicker than normal are more susceptible to dehydration that may contribute to turn blood thicker; they should avoid hot weather and dispose of sufficient amount of liquid. Even exposure to cold is poorly tolerated because it increases tissues oxygen consumption. About exercise the child must feel free to find his own limit and should never be obliged to do physical activities he doesn¹t want to do. Sometimes cyanosis represent a normal phenomenon. New-born have acrocyanosis (cyanosis of the extremities) due to immaturity of the peripheral circulation in the neonate. Even when they cry vigorously holding the breath new-born can develop cyanosis; there can be a suspicion of cyanosis in children who present a blue staining around the mouth after exposure to cold o a bath with cold water ; this is frequent in fair-haired children with pale skin because it is easy to see the color of the venous blood through a skin without pigment. Heart failure is a suite of symptoms and clinical manifestations deriving from the incapacity of the heart to accomplish his normal function. The cardiac output is reduced, and organs do not receive an adequate amount of blood and their function is decreased. For examples muscles cannot work properly and child will get tired easily, kidney will have a decreased function and the child will urinate less and as a consequence he will keep liquid in blood and tissues. Another consequence of heart function impairment is the increase of blood pressure in veins that get to the heart. At lung level the increase in venous pressure can provoke a range of event which goes from dyspnea to pulmonary edema. The rise of pressure in heart right side and together with liquid storage provoke liver enlargement (hepatomegaly). The increased veinous pressure and the liquid retention are the cause of peripheral edema which is the swelling of legs tissues. Heart fluttering is the sensation of a heart beating to much swiftly or irregularly. The increase in heart rate occurs normally during an exertion or an emotion, but some people can have an increase o irregularities of heart rate without warning and without an apparent reason. This generates the sensation of fluttering which is due in the majority of cases to an impairment of cardiac rhythm o arrhythmia. Generally fluttering is not dangerous, even if it can provoke anguish, because it is unpleasant to feel the proper heart beating swiftly without reason. If fluttering is short-lasting there is no problem for the child, but if they last more than half an hour, it is advisable to call on a physician or an hospital to make an examination and an ECG. Generally arrhythmia¹s that cause fluttering are characterized by an heart rate superior to normal (tachycardia) or irregularities of heart rhythm. Other arrhythmia¹s induce an heart rate inferior to normal and are called bradycardia. Arrhythmia¹s can appear in children with o without cardiac malformation or children who underwent surgery for heart defects. The majority of arrhythmia¹s are benign, but in some cases if heart rate is too high or too slow for a sufficient time cardiac function can be compromised. Cardiac murmurs are sound that are appreciated by the physician during heart auscultation. Murmurs are present even in children with normal heart, thus the presence of a murmur does not necessarily mean presence of heart problem. Murmurs present in child with normal heart are called innocent or functional murmur to distinguish them from the pathologic murmurs which sign an heart disease, for example a valvular problem or abnormal communication between cardiac chambers. More than 50% of the children have an innocent murmur which is recognized on its auscultatory characteristics and on the basis of laboratory examination performed with ECG, chest x-ray and echocardiography. A child with an innocent murmur has an healthy heart and can do every kind of physical activity. Innocent murmur generally appear during puberty. Respiratory infections are more frequent in children with heart diseases that cause an increase in pulmonary blood flow 4atrial septal defect, ventricular septal defect, endocardial cushion defect, patent ductus arteriosus). This is due to the fact that increased pulmonary blood flow obstruct the system that normally defends bronchi and lungs from infections. Once settled, respiratory infections usually last more than in children without heart disease. Chest pain is rarely connected with a cardiac problem in children. It can occur in children with severe aortic stenosis and few other situations. << back