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Document Details

FriendlyNirvana7570

Uploaded by FriendlyNirvana7570

Universitatea de Stat de Medicină și Farmacie „Nicolae Testimițanu”

2022

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cardiology congenital heart defects pediatrics

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Pediatrics DR. AHMED YASINI Cardiology Sign of cardiac & Respiratory disease Easy fatigue Sweating while feeding Feeding difficulties Rapid respirations Fetal circulation Ventricular septal defect Most common congenital cardiac lesion Perimembrano...

Pediatrics DR. AHMED YASINI Cardiology Sign of cardiac & Respiratory disease Easy fatigue Sweating while feeding Feeding difficulties Rapid respirations Fetal circulation Ventricular septal defect Most common congenital cardiac lesion Perimembranous VSDs are the most common of all VSDs (67%) 33% of all VSDs close spontaneously. Small VSDs usually close spontaneously Large VSDs can be closed by cardiac catheterization. Dx. Holosystolic murmur - Chest X-ray (large heart, pulmonary edema) - ECG- LVH - echocardiogram is definitive. Complications - Pulmonary hypertension - Endocarditis - Large defects lead to heart failure, failure to thrive indications for Surgery in first year - Failure to thrive or unable to be corrected medically - Infants at 6–12 months with large defects and pulmonary artery hypertension Question Eisenmenger syndrome shunt becomes right to left Uncorrected left-to-right shunt (VSD, ASD, PDA)→increase pulmonary blood flow→ pathologic remodeling of vasculaur→ pulmonary arterial hypertension Causes late cyanosis, clubbing , polycythemia 02.2022 Artrial septal defect - Defect in interatrial septum. - May lead to paradoxical emboli (systemic venous emboli use ASD to bypass lungs and become systemic arterial emboli). Ostium secundum defects is most common Dx. ECG, CXR, Echocardiogram Wide fixed splitting of S2 (pulmonary valve needs more time to close) Complications:- - Dysrhythmia - Low-flow lesion ( does not require endocarditis prophylaxis) TX. Usually no treatment needed. If a significant shunt is still present at around 3 years of age, closure is usually recommended. Question Endocardial cushion defect (atrioventricular canal defect) - ASD + VSD + abnormal valves Most common cardiac anomaly in DOWN syndrome (usually complete) DX. Chest x-ray: heart enlargement , increased pulmonary vessel markings edema - ECG: Right-axis deviation and RVH - Echocardiogram definitive - TX. diuretics (± digoxin) and afterload reduction for HF Surgical repair of the defect ultimately is required. - Without surgery death from heart failure. - With surgery ;arrhythmias, congenital heart block Patent ductus arteriosus (PDA) - The ductus arteriosus a lows blood to flow from the pulmonary artery to the aorta during fetal life. - PDA left-to-right shunting of blood and increased pulmonary blood flow Risk Factors More in girls. Associated with maternal rubella infection. premature infants - Machinery sound , wide pulse pressure -Tx. Indromethacin to close it, Prostaglandins to keep it open. - If PDA bursitis beyond the first week, it is unlikely to close spontaneously. -Can be closed by catheterization (coli embolization). -Ventilation and O2 supplementation can cause acute decompensation -In newborns with PDA- dependent defects. O2 stimulate PDA constriction (closure). Complications Congestive heart failure Infective endocarditis Pulmonary stenosis Vs Aoritic stenosis Pulmonary stenosis Systolic murmur Incrased blood flow through PFO RV hypertrophy- signs of RV failure CXR showing decreased pulmonary vasculaur Tx. Balloon valvuloplasty Neonate with critical pulmonary stenosis - emergent surgery Aoritic stenosis - Most are bicuspid aortic valve - Tx. Balloon valvulopathy Coarctation of the aorta BP in lower extremities (femoral) is lower than upper extremities (radial), Brachial femoral del Risk factor : Turner syndrome. Dx. Nothching of inferior borders of ribs. -Preductal:- infantile, upper body pink , loweris cyanotic. Tx. prostaglandin E1 , surgery Complications : cerebral hemorrhage (berry aneurysms) aortic rupture (aneurysms) endocarditis. HF Cyanosis Tetralogy of Fallot - The most common cyanotic congenital heart defect Abnormal septation of the truncus arteriosus into the aorta and pulmonary artery. PROVa 1. Pulmonary stenosis 2. Right ventricle hypertrophy 3. Overriding aorta 4. VSD The child will squat to decrease the RL shunt, increasing pulmonary blood flow. Tet spells:- paroxysmal hypercyanotic attacks- they can lead to convulsions and LOC. Treated by oxygen and Knee- chest position. Gasping causes syncope. Complications: Brain Abscess , cerebral hemorrhage. CXR: boot-shapped heart boot-shapped heart Question Tricuspid atresia Pulmonary blood flow will depend on the size of ASD Hypoplastic RV Increased left ventricular impulse (contrast to most others with right ventricular impulse) holosystolic murmurs along left sternal border. Tx. PGE1, surgical correction (redirect systemic VR directly to the pulmonary arteries). 1. Subclavian artery- to- pulmonary shunt (Blalock Taussig procedure) 2. Bidirectional cavopulmonary shunt (bidirectional Glenn) 3. Fontan procedure 02.2022 TOTAL ANOMALOUS PULMONARY VENOUS RETURN All of the pulmonary veins fail to connect to the left atrium and return abnormally via the right side of the heart. Dx. ECG, Echo Xray. “snowman” appearance Tx. PGE , surgery. HYPOPLASTIC LEFT HEART SYNDROME It is the most common cause of death from cardiac defects in the first month of life. A small LV which is unable to provide blood to systemic circulation-> diminished cardiac output. 1. Norwood procedure 2. Bidirectional Glenn 3. Fontan procedure Ebstein anomaly Associated with lithium More blood through ASD CXR: decreased pulmonary markings murmur of tricuspid regurgitation (Systolic Murmur in pulmonic area) and middiastolic murmur at the lower left sternum Transposition of great arteries TGA Cyanosis in newborn”24h of life” More common in infant with diabetic mothers. It needs PFO and PDA PGE1 to keep PDA Balloon atrial septostomy improves mixing between the two circulations. The arterial switch usually is performed within 2 week. Egg on a string sign. 02.2022 06.2021 01.2021 Truncus arteriosus Failure of septation of truncus Associated with CATCH-syndrome (Di George) Both ventricles have same pressure Di- George syndrome:- - Cardiac anomalies - Abnormal faces - Thymic hypoplasia - Cleft palate - Hypocalcemia - Chromosome 22 Hypertension - Renal stenosis Fibromuscular dysplasia:- most common cause in children Dx. Renal ultrasound, angiography. Renin, angiotensis. -Neurofibromatosis causes renal hypertension (pigmented brown skin) Kawasaki Mucocutaneous lymph node syndrome - Fever - Conjunctivitis - Rash genital, lips, palms - Cervical lymphadenopathy - Strawberry tongue - Sore throat/ diarrhea Leads to coronary artery aneurysm, MI Tx. High dose aspirin, IVIG Acute Rheumatic Fever Diagnosis of RHD is made with a history of recent streptococcal infection and either the presence of 2 major criteria or 1 major with 2 minor criteria Acute RF treatment: 10 days of oral amoxicillin/ penicillin or 1 dose of IM benzathine penicillin. If allergic 10 days of erythromycin, 5 days of azithromycin, or 10 days of clindamycin. Carditis – Corticosteroids for 2–3 weeks, then taper start aspirin for 6 weeks Aspirin in patients with arthritis/carditis without CHF Chorea - Phenobarbital then Haloperidol/ chlorpromazine RHD Rheumatic heart disease remains the most common form of acquired heart disease. The most common manifestation of ARF is arthritis. The most common affected valve is Mitral valve (MR/MS) then aortic Chemoprophylaxis Long QT syndrome 06.2021

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