Congestive Heart Diseases in children.pptx

congestive heart diseases in children

1. CONGENITAL HEART DISEASE D R. CHIKONDE

2. Prevalence Congenital heart disease occurs in approximately 0.8% of live births. The incidence is higher in stillborns (3-4%), spontaneous abortions (10-25%), and premature infants (about 2% excluding patent ductus arteriosus [PDA]).

3. LESION Ventricular septal defect Atrial septal defect (secundum) Patent ductus arteriosus Coarctation of aorta Tetralogy of Fallot Pulmonary valve stenosis Aortic valve stenosis d-Transposition of great arteries Hypoplastic left ventricle Hypoplastic right ventricle Truncus arteriosus Total anomalous pulmonary venous return Tricuspid atresia Single ventricle Double-outlet right ventricle Others % OF ALL LESIONS 35-30 6-8 6-8 5-7 5-7 5-7 4-7 3-5 1-3 1-3 1-2 1-2 1-2 1-2 1-2 5-10

4. CHD Most congenital defects are well tolerated in the fetus because of the parallel nature of the fetal circulation. Even the most severe cardiac defects (e.g., hypoplastic left heart syndrome) can usually be well compensated for by the fetal circulation

5. HISTORY A comprehensive cardiac history starts with details of the perinatal period including the presence of cyanosis respiratory distress, or prematurity. □ Maternal complications such as gestational diabetes, medications, systemic lupus erythematosus, or substance abuse can be associated with cardiac problems. □ If cardiac symptoms began during infancy, the timing of the initial symptoms should be noted to provide important clues about the specific cardiac condition

6. History Symptoms of heart failure in infants and children are age specific Infants Feeding difficulties are common Inquiry should be made about the frequency of feeding and either the volume of each feeding or the time spent on each breast An infant with heart failure often takes less volume per feeding and becomes dyspneic or diaphoretic while sucking After falling asleep exhausted, the baby, inadequately fed, will awaken for the next feeding after a brief time

7. HISTORY □ Older children, heart failure may be manifested as Exercise intolerance, Difficulty keeping up with peers during sports or need for a nap after coming home from school Poor growth. Eliciting a history of fatigue in an older child requires questions about age-specific activities, including stair climbing, walking, bicycle riding, physical education class, and competitive sports; information should be obtained regarding more severe manifestations such as orthopnea and nocturnal dyspnea.

8. Examination cyanosis, abnormalities in growth, and any evidence of respiratory distress. Evaluation of a murmur must always be performed in the context of other physical findings. quality of the pulses or the presence of a ventricular heave

9. Cyanosis At rest is often overlooked by parents; it may be mistaken for a normal individual variation in color. Cyanosis during crying or exercise, however, is more often noted as abnormal by observant parents. Many infants and toddlers turn “blue around the lips” when crying vigorously or during breath-holding spells; this condition must be carefully differentiated from cyanotic heart disease by inquiring about inciting factors, the length of episodes, and whether the tongue and mucous membranes also appear cyanotic. Newborns have cyanotic extremities (acrocyanosis) when undressed and cold; this response to cold must be carefully differentiated from true cyanosis.

10. Pulse A wide pulse pressure with bounding pulses may suggest an aortic runoff lesion such as patent ductus arteriosus, aortic insufficiency, an arterial-venous communication, or increased cardiac output secondary to anemia, anxiety, or conditions associated with increased catecholamine or thyroid hormone secretion Carefully evaluate the character of the pulses

11. Pulse Contn The presence of diminished pulses in all extremities is associated with pericardial tamponade, left ventricular outflow obstruction, or cardiomyopathy. The radial and femoral pulses should always be palpated simultaneously. Normally, the femoral pulse should be appreciated immediately before the radial pulse. In older children with coarctation of the aorta, blood flow to the descending aorta may channel through collateral vessels and result in the femoral pulse being delayed until after the radial pulse (radial-femoral delay).

12. Types of Congenital heart diseases and evaluation Acyanotic Cyanotic The two groups can be further subdivided according to whether the chest radiograph shows evidence of increased, normal, or decreased pulmonary vascular markings Electrocardiogram can be used to determine whether right, left, or biventricular hypertrophy exists. The character of the heart sounds and the presence and character of any murmurs further narrow the differential diagnosis. The final diagnosis is then confirmed by echocardiography, CT or MRI, or cardiac catheterization.

13. Acyanotic CHD The most common lesions are those that produce a volume load, and the most common of these are left-to- right shunt lesions. VSD,PDA, AV septal defects Communication between the systemic and pulmonary sides of the circulation, which results in shunting of fully oxygenated blood back into the lungs .

14. Acyanotic CHD The increased volume of blood in the lungs decreases pulmonary compliance and increases the work of breathing. Fluid leaks into the interstitial space and alveoli and causes pulmonary edema. The infant acquires the symptoms we refer to as heart failure, such as tachypnea, chest retractions, nasal flaring, and wheezing. The term heart failure is a misnomer

15. Ventricular Septal Defect (VSD) VSD is the most common cardiac malformation and accounts for 25% of congenital heart disease. Defects may occur in any portion of the ventricular septum, but most are of the membranous type

16. Presentation (VSD) Presentation vary according to the size of the defect and pulmonary blood flow and pressure. Small VSDs with trivial left-to-right shunts and normal pulmonary arterial pressure are the most common. Patients are asymptomatic, and the cardiac lesion is usually found during routine physical examination. Characteristically, a loud, harsh, or blowing holosystolic murmur is present and heard best over the lower left sternal border, and it is frequently accompanied by a thrill.

17. CXR □ Usually normal, although minimal cardiomegaly and a borderline increase in pulmonary vasculature may be observed

18. Patent Ductus Arteriosus (PDA) Functional closure of the ductus normally occurs soon after birth, but if the ductus remains patent when pulmonary vascular resistance falls, aortic blood is shunted into the pulmonary artery Female patients with PDA outnumber males 2 : 1. Associated with maternal rubella infection during early pregnancy. It is a common problem in premature infants, where it can cause severe hemodynamic derangements and several major sequelae

19. Presentation (PDA) A small patent ductus does not usually have any symptoms associated with it. A large PDA will result in heart failure similar to that encountered in infants with a large VSD. Retardation of physical growth may be a major manifestation in infants with large shunts. Wide pulse pressure, most prominently, bounding peripheral arterial pulses

20. Presentation (PDA) The apical impulse is prominent and, with cardiac enlargement, is heaving Thrill, maximal in the 2nd left interspace, is often present and may radiate toward the left clavicle Classic continuous murmur is described as being like machinery or rolling thunder in quality May be localized to the 2nd left intercostal space or radiate down the left sternal border or to the left clavicle

21. CXR Patients with a large PDA show a prominent pulmonary artery with increased intrapulmonary vascular markings. Cardiac size depends on the degree of left-to-right shunting; it may be normal or moderately to markedly enlarged

22. Atrial septal defects (ASDs) Can occur in any portion of the atrial septum (secundum, primum, or sinus venosus), depending on which embryonic septal structure has failed to develop normally. Less commonly, the atrial septum may be nearly absent, with the creation of a functional single atrium. Isolated secundum ASDs account for ≈7% of congenital heart defects. The majority of cases of ASD are sporadic; autosomal dominant inheritance does occur as part of the Holt-Oram syndrome (hypoplastic or absent radii, 1st-degree heart block, ASD) or in families with secundum ASD and heart block.

23. Atrial septal defect (ASD) Secundum □ ASD is most often asymptomatic; Maybe discovered inadvertently during physical examination. Even an extremely large secundum ASD rarely produces clinically evident heart failure in childhood. In younger children, subtle failure to thrive may be present; in older children, varying degrees of exercise intolerance may be noted. Often, the degree of limitation may go unnoticed by the family until after surgical repair, when the child's growth or activity level increases markedly

24. Examination ASD secundum Mild left precordial bulge 2 nd heart sound widely split and fixed in its splitting in all phases of respiration Systolic ejection murmur is heard; it is medium pitched, without harsh qualities, seldom accompanied by a thrill, and best heard at the left middle and upper sternal border Short, rumbling mid-diastolic murmur produced by the increased volume of blood flow across the tricuspid valve is often audible at the lower left sternal border

25. Atrioventricular (AV) Septal Defects Common in children with Down syndrome and may occasionally occur with pulmonary stenosis Present with H/O □ Exercise intolerance, easy fatigability, and recurrent pneumonia especially in infants with large left-to-right shunts and severe mitral insufficiency

26. Cyanotic HD Can be divided according to pathophysiology: Whether pulmonary blood flow Is decreased (tetralogy of Fallot, pulmonary atresia with an intact septum, tricuspid atresia, total anomalous pulmonary venous return with obstruction) Or increased (transposition of the great vessels, single ventricle, truncus arteriosus, total anomalous pulmonary venous return without obstruction). The chest radiograph is a valuable tool for initial differentiation between these two categories

27. Cyanotic CHD with decreased Pulmonary blood flow An obstruction to pulmonary blood flow (at the tricuspid valve or right ventricular or pulmonary valve level) and a pathway by which systemic venous blood can shunt from right to left and enter the systemic circulation (via a patent foramen ovale, atrial septal defect, or VSD). Common lesions in this group include tricuspid atresia, tetralogy of Fallot, and various forms of single ventricle with pulmonary stenosis.

28. Cyanotic CHD with decreased Pulmonary blood flow In these lesions, the degree of cyanosis depends on the degree of obstruction to pulmonary blood flow. If the obstruction is mild, cyanosis may be absent at rest. These patients may have hypercyanotic (“tet”) spells during conditions of stress. In contrast, if the obstruction is severe, pulmonary blood flow may be dependent on patency of the ductus arteriosus. When the ductus closes in the 1st few days of life, the neonate experiences profound hypoxemia and shock

29. Cyanotic CHD with increased Pulmonary blood flow There is obstruction to pulmonary blood flow. Cyanosis is caused by either abnormal ventricular-arterial connections or total mixing of systemic venous and pulmonary venous blood within the heart Transposition of the great vessels is the most common of the former group of lesions.

30. Cyanotic CHD with increased Pulmonary blood flow The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. Systemic venous blood returning to the right atrium is pumped directly back to the body, and oxygenated blood returning from the lungs to the left atrium is pumped back into the lungs. The persistence of fetal pathways (foramen ovale and ductus arteriosus) allows for a small degree of mixing in the immediate newborn period; when the ductus begins to close, these infants become extremely cyanotic.

31. Tetralogy of Fallot 1) obstruction to right ventricular outflow (pulmonary stenosis), (2) ventricular septal defect (VSD), (3) dextroposition of the aorta with override of the ventricular septum (4) right ventricular hypertrophy

32. Presentation □ Paroxysmal hypercyanotic attacks (hypoxic, “blue,” or “tet” spells) are a particular problem during the 1st 2 yr of life. Infant becomes hyperpneic and restless, cyanosis increases, gasping respirations ensue, and syncope may follow. Spells occur most frequently in the morning on initially awakening or after episodes of vigorous crying. Temporary disappearance or a decrease in intensity of the systolic murmur is usual as flow across the right ventricular outflow tract diminishes. The spells may last from a few minutes to a few hours but are rarely fatal. Short episodes are followed by generalized weakness and sleep

33. CXR □ The cardiac silhouette has been likened to that of a boot or wooden shoe (“coeur en sabot”)

34. Investigations CHD CXR ECG ECHO CT Scan and MRI cardiac catherterisation

35. □ Thank you