Congenital Heart Diseases (summary) .pdf

Congenital Heart Diseases Objectives: 1. Describe the clinical features that point to the presence of a congenital heart malformation. 2. Describe the general classification of heart diseases in pediatrics. 3. Differentiate cyanotic from non-c...

Congenital Heart Diseases Objectives: 1. Describe the clinical features that point to the presence of a congenital heart malformation. 2. Describe the general classification of heart diseases in pediatrics. 3. Differentiate cyanotic from non-cyanotic heart disease. 4. Understand the anatomy and physiology of common congenital cardiac defects. 5. Discusses the clinical presentation and outline the management of acyanotic and cyanotic heart diseases E Done by Maha Albarrak Blue: Main Category me Orange: Subcategory. Black: Original slides content. Red: Important information. Green: Doctor notes. Blue highlight: Info that came in to previous batches questions.  CHD (summary) __________________________________________________________ The most common CHD syndromes - Left-to-right shunts (breathless) - Right-to-left shunts (blue) - Common mixing (breathless and blue) - Outflow obstruction in a well child (asymptomatic with a murmur) - Outflow obstruction in a sick neonate (collapsed with shock) Presentation 1- Antenatal cardiac ultrasound diagnosis - Checking the anatomy of the fetal heart has become a routine part of the fetal anomaly between 18 weeks’ and 20 weeks’ gestation. - If an abnormality is detected, detailed fetal echocardiography is performed by a paediatric cardiologist. - Any fetus at increased risk, e.g. suspected Down syndrome, where the parents have had a previous child with heart disease or where the mother has congenital heart disease is also checked. - Mothers of infants with duct-dependent lesions likely to need treatment within the first 2 days of life may be offered delivery at or close to the cardiac center 2- Detection of heart murmur - The most common presentation of congenital heart disease is with a heart murmur. Even so, the vast majority of children with murmurs have a normal heart. - They have an ‘innocent murmur’, which can be heard at some time in almost 30% of children - Hallmarks of an innocent ejection murmur are (all have an ‘S’, ‘innoSent’) aSymptomatic, Soft blowing murmur, Systolic murmur only, not diastolic, left Sternal edge 3- Heart failure Symptoms - Breathlessness (particularly on feeding or exertion) Signs - Sweating - Poor weight gain - Poor feeding - Tachypnoea, tachycardia - Recurrent chest infections - Heart murmur, gallop rhythm - Elnarged heart, hepatomegaly Causes of heart failure 1. Neonates – obstructed (duct-dependent) systemic circulation - Hypoplastic left heart syndrome - Critical aortic valve stenosis - Severe coarctation of the aorta - Interruption of the aortic arch 2. Infants (high pulmonary blood flow) - Ventricular septal defect - Atrioventricular septal defect - Large persistent ductus arteriosus 3- Older children & adolescents (right or left heart failure) - Cradiomyopathis - Rhumatic heart diseases - Eisenmenger syndromy (right heart failure only) 4- Cyanosis 1- Peripheral cyanosis (blueness of the hands & feet) 2- Central cyanosis (seen on the tongue as a slate blue colour) - Associated with a fall in arterial blood oxygen tension - It can only be recognized clinically if the concentration of reduced haemoglobin in the blood exceeds 50 g/L, so it is less pronounced if the child is anaemic - Check with a pulse oximeter that an infant’s oxygen saturation is normal (≥94%). Persistent cyanosis in an otherwise well infant is nearly always a sign of structural heart disease #1 __________________________________________________________ Left to Right shunts Types Definition Clinical featurs Management 1- Atrial Has 2 types: - Asymptomatic - For secundum ASDs, this is Septal defects 1- primum/AVSD - Recurrent chest infections/wheeze by cardiac catheterization - Arrhythmias (fourth decade onwards) with insertion of an occlusion 2- secundum ASD device - For partial AVSD surgical correction is required - Treatment is usually ⭐ undertaken at about 3 years to 5 years of age in order to prevent right heart failure and arrhythmias in later life 2- Ventricular - Common, accouts for Small VSDS Small VSDS - These are smaller than the aortic valve - These lesions will close septal defects 30% of all cases of Asymptomatic spontaneously. congenital heart disease - Physical signs: - Prevention of bacterial - There is a defect - Loud pansystolic murmur at lower left endocarditis is by maintaining anywhere in the sternal edge good dental hygiene and ventricular septum - (loud murmur implies smaller defect) prophylactic antibiotic. 1- perimembranous - Quiet pulmonary second sound (P2). (adjacent to the tricuspid - Chest radiograph is Normal. valve) __________________________________ ________________________________ 2- Muscular (completely Large VSDS Large VSDS surrounded by muscle) - Are same size or bigger than aortic - Drug therapy for heart failure is valve with diuretics, often combined - They can most be divide with captopril considered according to Symptoms: the size of the lesion - heart failure with breathlessness & Additional calorie input is 1- Small VSDS faltering growth after 1 week old & chest required 2- Large VSDS infections - There is always pulmonary Physical signs hypertension in children with - (Tachypnoea, tachycardia , cardiomegaly and large VSD and left-to- right hepatomegaly) “2 Vitals, 2 Organs” shunt. This will ultimately lead to - Active precordium irreversible damage of the - Soft pansystolic murmur or no murmur pulmonary capillary vascular (implying large defect) bed. To prevent this, surgery is - Apical mid-diastolic murmur (from increased usually performed at 3 months to flow across the mitral valve after the blood 6 months of age in order to: has circulated through the lungs) 1- manage heart failure and - Loud pulmonary second sound (P2) – from faltering growth raised pulmonary arterial pressure 2- prevent permanent lung damage from pulmonary hypertension and high blood flow. 3- Persistance The ductus arteriosus - Continuous murmur beneath the left - Closure is recommended to connects the pulmonary clavicle abolish the lifelong risk of ductus - The murmur continues into diastole because artery to the descending bacterial endocarditis and of arteriosus aorta. In term infants, it the pressure in the pulmonary artery is lower pulmonary vascular disease. normally closes shortly after than that in the aorta. birth. - The pulse pressure is increased, causing a - Closure is with a coil or collapsing or bounding pulse. occlusion device introduced via a - Symptoms are unusual, but when the duct is cardiac catheter at about 1 year large there will be increased pulmonary blood of age. flow with heart failure & pulmonary hypertension. - Occasionally, surgical ligation is required. - Indomethacin medication #2 __________________________________________________________ Right to left shunts Types Features Symptoms Signs Management This is the most - Severe cyanosis, - Clubbing of the - Hypercyanotic spells are 1- Tetralogy hypercyanotic spells and fingers and toes will usually self-limiting and common cause of of Fallot cyanotic congenital squatting on exercise develop in older followed by a period of sleep. developing in late infancy. children. If prolonged (beyond about 15 heart disease. min), they should be given - Hypercyanotic spells, as prompt treatment, according to there are four they may lead to - A loud harsh need, with: cardinal anatomical myocardial infarction, ejection systolic - sedation and pain relief features: cerebrovascular accidents murmur at the left - intravenous propranolol 1- large VSD and even death if left sternal edge from day untreated. 1 of life - intravenous volume administration 2- overriding of the aorta with respect to - They are characterized by - With increasing - bicarbonate to correct acidosis a rapid increase in the ventricular cyanosis, usually right ventricular - muscle paralysis and artificial septum associated with irritability outflow tract ventilation in order to reduce or inconsolable crying obstruction, which is metabolic oxygen demand. 3- subpulmonary because of severe predominantly stenosis causing right muscular and below - Surgery at around 6 months of - hypoxia and age. (It involves closing the ventricular outflow the pulmonary valve breathlessness and pallor VSD) tract obstruction the murmur will because of tissue acidosis. - Very cyanosed Infants who are shorten and cyanosis - On auscultation, there is a will increase. require a shunt to increase 4- right ventricular very short murmur during pulmonary blood flow. Is done hypertrophy as a a spell. by surgical placement of an result. artificial tube between the subclavian artery & the pulmonary artery or sometimes by balloon dilatation of the right ventricular outflow tract. 2- Transposition - In which the two - Cyanosis is the - Cyanosis is always - The key is to improve mixing of the great main arteries predominant present - Maintaining the patency of the ductus arteriosus with a arteries leaving the heart symptom. - The 2nd heart sound prostaglandin infusion is are reversed is often loud & single mandatory. (transposed) - A balloon atrial septostomy - Usually no murmur, may be a life-saving procedure but may be a systolic murmur from - All patients will require increased flow or surgery, which is usually the stenosis within the arterial switch procedure in the left ventricular neonatal period. (pulmonary) outflow tract. Hyperoxia (nitrogen washout) test - The test is used to help determine the presence of heart disease in a cyanosed neonate. - The infant is placed in 100% oxygen (headbox or ventilator) for 10 minutes. If the right radial arterial partial pressure of oxygen (PaO2) from a blood gas remains low (