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Download Paediatric Cardiology - Dr. Herchel Rosenberg
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Transcript
Paediatric Cardiology: An Outline of Congenital Heart Disease Dr. H.C. Rosenberg [email protected] Objectives To provide an outline of congenital heart disease List criteria for Kawasaki syndrome Describe the common innocent murmurs of childhood An Outline of Congenital Heart Disease Pink (Acyanotic) Blue (Cyanotic) Resistance= ? Acyanotic Congenital Heart Disease Normal Pulmonary Blood Flow ↑ Pulmonary Blood Flow Acyanotic Congenital Heart Disease Normal Valve Not Pulmonary Blood Flow Lesions fundamentally different from adults Acyanotic Congenital Heart Disease ↑ Pulmonary Blood Flow Shunt Lesions Atrial Level Shunt ASD Physiology Left to Right shunt because of greater compliance of right ventricle Loads right ventricle and right atrium Increased pulmonary blood flow at normal pressure Low resistance ASD History Usually asymptomatic in childhood Occasionally Presentation frequent respiratory tract infections with murmur as pre-schooler or older ASD Physical Examination Right ventricular “lift” Wide fixed S2 Blowing SEM in pulmonic area ASD ASD ASD Natural History Generally do well through childhood Major complication atrial fibrillation Can develop pulmonary hypertension / RV failure but not before third or fourth decade of life ASD Management Device closure around three years of age or when found Surgery for very large defects or outside fossa ovalis (eg. sinus venosus defect) ASD Shunt Lesions Ventricular Level Shunt VSD Physiology Left to Right shunt from high pressure left ventricle to low pressure right ventricle Loads left atrium and left ventricle (right ventricle may see pressure load) VSD History Small defects Presentation Large with murmur in newborn period defects Failure to thrive (6 wks to 3 months) Tachypnea, poor feeding, diaphoresis VSD Physical Examination Active left ventricle Small defect Pansystolic Large murmur, normal split S2 defect SEM, narrow split S2, diastolic murmur at apex from high flow across mitral valve VSD BVH VSD VSD Natural History Small defect Often close No real significance beyond endocarditis risk Large defect Failure to thrive Progression to pulmonary hypertension as early as 1 year VSD Management Small defect Large defect Semi-elective closure if growth failure or evidence of increased pulmonary hypertension Occasionally elective closure if persistent cardiomegally beyond 3 years of age Shunt Lesions Great Artery Level Shunt PDA Physiology Left to Right shunt from high pressure aorta to low pressure pulmonary artery Loads left atrium and left ventricle (right ventricle may see pressure load) PDA History Premature Failure Older duct to wean from ventilator +/- murmur infant Usually murmur from early infancy Occasionally signs of heart failure PDA Physical Examination Active left ventricle Hyperdynamic pulses Premature duct SEM Older with diastolic spill infant Continuous murmur PDA Management Premature Duct Trial of indomethacin Surgical ligation Older infant Leave till 1 year of age unless symptomatic Coil / device closure Rarely surgical ligation Truncus Arterisosus Cyanotic Congenital Heart Disease “Blue” blood (deoxygenated hemoglobin” enters the arterial circulation Systemic oxygen saturation is reduced Cyanosis may or may not be clinically evident Causes of Cyanosis Respiratory Cardiac Hematologic Polycythemia Hemoglobins with decreased affinity Neurologic Decreased Respiratory drive Cyanosis Respiratory Cardiac Hyperoxic Place test infant in 100% 02 Lung disease should respond to 02 Failure of saturation to rise to > 85% suggest cardiac disease Cyanotic Congenital Heart Disease ↓Pulmonary Blood Flow ↑Pulmonary Blood Flow Cyanotic Congenital Heart Disease Decreased Pulmonary Blood Flow Cyanotic Congenital Heart Disease - ↓ Pulmonary Flow = RVOT Obstruction + Shunt Tetralogy of Fallot VSD Over-riding aorta Pulmonary stenosis RVH Tetralogy of Fallot History Presentation Severe depends on severity of PS stenosis Cyanosis shortly after birth (as duct closes) Mild stenosis May present as heart murmur (from shortly after birth) Tetralogy of Fallot Physical Examination Variable cyanosis (remember the 50g/l rule) Right ventricular “tap” Decreased P2 +/- ejection click “Tearing” SEM Tetralogy of Fallot Management Outside the newborn period, surgical repair if symptomatic Elective repair at 6 months Role for beta blockers to palliate hypercyanotic spells Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells) Episodes of profound cyanosis Most frequently after waking up or exercise Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells) Fall in P02 Increased R to L shunt Hyperventilation Increased Return of deeply desaturated venous blood Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells Treatment Tuck knees to chest (pinches off femoral veins) In hospital O2 Bicarbonate Phenylephrine Morphine IV beta blocker Tetralogy of Fallot Tetralogy of Fallot Decreased Pulmonary Blood Flow Duct Dependent Congenital Heart Disease 1. 2. 3. Which of the following are examples of duct dependent CHD? Pulmonary atresia Patent ductus arteriosus Transposition of the great arteries Cyanotic Congenital Heart Disease With ↑Pulmonary Blood Flow Cyanotic Congenital Heart Disease With ↑Pulmonary Blood Flow Transposition of the great arteries Total anomalous pulmonary venous drainage d-Transposition Normal Heart Body RA RV PA AO LV LA Lungs Circulation is in “series” d-Transposition Circulation is in “parallel” Need for mixing Transposition History Presentation Profound cyanosis shortly after birth (as duct closes) Minimal or no murmur Tetralogy of Fallot Physical Examination Profound cyanosis Right ventricular “tap” Loud single S2 Little or no murmur Tetralogy of Fallot Management Prostaglandins to maintain mixing Balloon atrial septostomy Arterial switch repair in first week Total Anomalous Pulmonary Venous Return Pulmonary veins communicate with systemic vein Pulmonary veins fail to connect to left atrium Total Anomalous Pulmonary Venous Return - Supracardiac Pulmonary veins communicate with systemic vein Pulmonary veins fail to connect to left atrium Total Anomalous Pulmonary Venous Return - Infracardiac Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein TAPVD History Presentation depends on presence or absence of obstruction to venous return Infradiaphragmatic Almost always obstructed Cyanosis and respiratory distress shortly after birth Cardiac or supracardiac Rarely obstructed Can present like big ASD TAPVD Physical Examination Variable cyanosis (again depends on obstruction) Right ventricular “tap” Wide split S2 Blowing systolic ejection murmur TAPVD TAPVD Management If severe cyanosis in newborn Emergency surgical repair Unobstructed Semi-elective surgical repair when discovered Coarctation of the aorta Coarctation of the Aorta History Presentation varies with severity Severe coarct Failure Mild (shock) in early infancy coarct Murmur (in back) Hypertension Coarctation Physical Examination Absent femoral pulses Arm leg gradient +/- hypertension Left ventricular “tap” Bruit over back Coarctation Management Newborn with CHF Infant Emergency surgical repair Semi-elective repair in uncontrolled hypertension Older child Balloon arterioplasty Surgery on occasion Failure to repair prior to adolescence recipe for life long hypertension! “Grey” Heart Disease Critical LVOT obstruction Left Ventricular Outflow Tract Obstruction Critical Aortic Stenosis “Critical” shock Critical Aortic Stenosis Management Prostaglandins to provide source of systemic blood flow Balloon valvuloplasty Rarely surgery Hypoplastic Left Heart Syndrome “Duct dependent “ congenital heart disease Ductus arteriosus is the only source of systemic blood flow Hypoplastic left heart Management Prostaglandins Norwood procedure Kawasaki Syndrome Small artery arteritis Coronary arteries most seriously effected Dilatation/aneurysms progressing to (normal) stenosis Kawasaki Syndrome 5 days of fever plus 4 of Rash Cervical lymphadenopathy (at least 1.5 cm in diameter) Bilateral conjuctival injection Oral mucosal changes Peripheral extremity changes Swelling Peeling (often late) Kawasaki Syndrome Associated Sterile Findings pyuria Hydrops of the gallbladder Irritability!!! Kawasaki Syndrome Epidemiology Generally children < 5 years Male > Female Asian > Black > White Kawasaki Syndrome Management Gamma globulin 2g/kg 80 mg/kg ASA until afebrile then 5 mg/kg for 6 weeks Innocent Murmurs Characteristics Always Grade III or less Always systolic (or continuous) Blowing or musical quality Not best heard in back Innocent Murmurs Types Still’s Pulmonary Flow murmur Blowing SEM best heard in PA Venous Hum Vibratory SEM best heard mid-left sternal border Continuous murmur best heard in R infraclavicular Decreases lying flat or occlusion of neck veins Physiologic peripheral pulmonary artery stenosis Blowing SEM best heard in PA radiating out to both axillae Questions?
