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Transcript
Evaluation for Congenital Heart Diseases Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery Heart Diseases in Children Congenital heart diseases Rheumatic heart disease : Rheumatic fever Other acquired diseases: Kawasaki Cardiomyopathy Arrhythmia Effects of CHD • No effect on a child • Decreased function when stressed • Decreased cardiopulmonary function • Other organ/system manifestation Presentation of CHD • • • • • Shock like symptoms Cyanosis Congestive symptoms Exercise intolerance Asymptomatic heart murmur • • • • Abnormality in routine chest PA Chest pain Syncope/ seizure/ fainting Airway obstruction/ dysphagia Shock like Symptoms • Non-specific: collapse, pallor, cold clammy skin, hypotension, oligouria, acidosis • HLHS, critical AS, IAA, COA unable to maintain systemic output during transitional circulation • Most common in neonate / infancy • Very important to recognize Cyanosis • Cyanosis: more than 5.0g% of reduced Hb. • Central versus Peripheral cyanosis • Central cyanosis: CNS, heart, lung • Pathophysiology: TGA, TOF, CML, DDPC Congestion • Symptoms: dyspnea on feeding, sweating, poor weight gain, frequent respiratory infection • Most common mode in infancy • Obligatory shunt lesions/ Left to right shunt lesions/ CML with no PS/ Myocarditis/ Arrhythmia Asymptomatic Murmur • Heart murmur noted in routine examination • ASD, VSD, PS, AS, Bicuspid AV • Common mode in childhood • Murmur does not mean there is a CHD • No murmur does not mean there is no CHD Abnormality in Chest PA Corrected TGA/ Pericardial Defect/ Cardiac Tumor Chest Pain * Anomalous origin of left coronary artery from pulmonary artery * Aortic stenosis(severe) / Hypertrophic cardiomyopathy Syncope/ Seizure/ Fainting • Anoxic spell of acyanotic TOF • Arrhythmia : VT, SVT, long QT syndrome • Syncope on exercise : AS, SAS, IHSS Airway Obstruction/ Dysphagia Airway Obstruction / Dysphagia Abnormal PA Branching Mode of / Age at Presentation • Collapse : within 3-4 days • Cyanosis : usually within a month • Congestion : 1-5 months • Murmur : any time Diagnosis of Cardiac Diseases • Anatomic Diagnosis • Hemodynamic Evaluation • Total assessment / Prognosis • Etiologic Diagnosis Diagnostic Tools • History and physical examination • Chest X ray / EKG / Blood study • Echocardiography/ Other imaging tools • Catheterization/ Other invasive tools Accuracy of Tools • History/PE : important, rarely specific • X-ray/EKG : not-confirmative • Echocardiography: confirmative, but non-invasive • Cardiac catheterization : confirmative, but invasive History Taking • Cyanosis: onset, progression, anoxic spell • Congestion: feeding, wt. gain, respiratory infection, sweating, tachy-dyspnea • Exercise tolerance: easy fatigability, DOE • Possible Causes: maternal diseases, drug, infection, family history Physical Examination • Inspection : general appearance, nutrition, syndrome?, facial morphology, jugular venous pulse, respiratory pattern, rate, chest retraction, alae nasi flaring, dyspnea, precordial bulging, cyanosis, clubbing • Palpation: apical pulse, precordial activity, thrill, arterial pulse, location and size of liver and spleen • Auscultation: S1, S2, abnormal sounds, murmur • Please do not pull out stethoscope before you observe patients carefully Chest X-ray Heart size, shape, pulmonary vascularity Chest X-ray Electrocardiography Purposes of Imaging • Anatomic-pathologic diagnosis • Hemodynamic assessment (velocity, flow, pressure, stress-strain) • Volume, function, wall motion, torsion • Coronary perfusion / Metabolism • Tissue characterization Echocardiography • Easy, non-invasive, accurate, real-time • Anatomic and physiologic information • Changed practice of pediatric cardiology Echocardiograhy - Modalities • M-mode / 2-D / 3-D • Doppler / color Doppler • Trans-thoracic, trans-esophageal, trans-abdominal, trans-vaginal, intra-cardiac, intra-vascular Echocardiograhy Echocardiograhy M-mode Echocardiograhy 3-D Echocardiograhy New Development in Echo • Imaging: edge detection/auto-measurement • Doppler: 3-D flow / stress-strain • Contrast echo: coronary perfusion Other Imaging Tools • Magnetic Resonance Imaging (MRI) • CT / Electron-beam CT (EBCT) • Radionuclide / SPECT • Positron Emission Tomography Magnetic Resonance Imaging Spin echo Gradient echo Velocity encoded Magnetic Resonance Imaging • Sectional still image/ cine image/ 3-D • Flow information / volume flow • Less window dependant / post-op study, older age / functional evaluation Magnetic Resonance Imaging Magnetic Resonance Imaging Computerized Tomography Computerized Tomography Radionuclide Study Radionuclide Study Positron Emission Tomography Purposes of Catheterization • Anatomic diagnosis • Hemodynamic assessment • Interventional procedure Equipment • Biplane monitor / Cine with digital subtraction • Patient monitoring : EKG, BP, pulse oximeter • Physiologic signal amplifier and recording device • Blood gas, O2 consumption, Dye/ Thermodilution • Emergency treatment tools : • Room for Others : anesthesia, echo, exercise Catheterization Room Fluoroscopic Monitor Physiologic Signal - Display & Recording Catheterization vs Echocardiograpy • How accurate non-invasive tests are • Risk of cardiac catheterization • How to obtain necessary information during catheterization or surgery • Nature of surgical correction • Risk of possible undiagnosed diseases Electrophysiologic Study How to Approach to CHD - as a Clinician Does this baby have a CHD? – Which baby should be referred to pediatric cardiologist – The urgency with which that referral should be made – If not, what tests and in what order to make diagnosis Babies with Suspected CHD • Clinical assessment – Mode of / age at presentation – Physical examination • Laboratory test – CBC, ABGA, hyperoxic test – Chest PA, EKG – Echocardiography if available Suspected CHD without Confirmation Any magic bullet for all? – IV inotropics – PGE 1 – Decrease oxygen consumption – General supportive care Incidence of CHD • Incidence of total CHD among races: – the same in all races ; about 5 - 8 /1,000 • Ethnic difference in incidences of individual anomalies and subtypes • Associated non-cardiac malformation Racial Difference of CHD • Left sided lesions seem to be lower in Asians • Right isomerism seems to be higher in Asians • Subpulmonic VSD is higher in Asians Classification of CHD • Why classify : the sameness, differences • How to classify : view point, purpose Classification of CHD • Clinical viewpoint cyanotic versus acyanotic shunts/obstruction/regurgitation • Pathology viewpoint normal vs abnormal connection segmental approach • Developmental viewpoint Classification of CHD Developmental viewpoint • Ectomesenchymal tissue migration abnormalities • Abnormal intra-cardiac flow • Cell death abnormalities • Extra-cellular matrix abnormalities • Abnormal targeted growth • Abnormal situs and looping Naming of CHD • Unambiguous, accurate, and succinct • Capable of describing any combination of defects • Allows for precise classification of malformations to be made during patient’s life • Useful both for anatomical, clinical, and etiologic studies Naming : Unambiguous? • Different names for the same thing • The same name for different things Ambiguity Different names for the same thing – – – – – L-transposition ventricular inversion {S,L,L} corrected transposition (physiologically) corrected transposition Atrial solitus, discordant AV/ VA connection Ambiguity The same names for different things D- transposition – a term for GA relationship – a term for VA connection – a term for specific diagnosis Naming : Simple, Specific ? • In most, simple : VSD, ASD, PDA, TOF • In a few, not simple or specific : TA • Rarely, complex : isomerism Tricuspid Atresia Right Isomerism • Dextrocardia • Bilateral SVC • Separate hepatic vein • TAPVR • Complete AVSD • LV hypoplasia • Transposition • PS or pul. Atresia Fetal Circulation • Is adapted to a special situation • Depends on placenta for O2/nutrients • Is rarely overloaded, but if overloaded little reserve Flow Pathway & Distribution (% indicates the proportion of combined output ) SVC-RV-MPA-Duct-Des. Ao IVC-PFO-LA-LV-Asc. Ao * ductus arteriosus, ductus venosus, atrial communication Oxygen Saturation % Fetal Circulation • Parallel circulation (combined output) • Communications between R & L heart • Pulmonary circulation is redundant Congenital Heart Diseases in Fetus • Often silent : – TGA : has little effect – HLHS : RV is slightly overloaded – PA + IVS : no effect at all • When CHD causes volume overload, heart fails and hydrops ensues Transitional Circulation Dramatic changes in circulation at the moment of birth and onwards : – Air breadth - lung expansion - Rp ↓ – Qp ↑ - LA pressure ↑ - PFO ↓ – P O2 ↑ - ductus arteriosus and venosus ↓ – Obliteration of placental circulation - Rs ↑ – IVC pressure ↓ - PFO ↓ Transitional Circulation & CHD • As circulation separates, TGA can not supply enough oxygen to the body • Obstructed pathway in either side hardly tolerate – right : PA or critical PS in any CHD – left : Aortic atresia or critical AS, IAA, COA – mitral atresia + small PFO; obstructed TAPVR Neonatal Circulation & CHD • Neonatal circulation – potential of increased Rp – potential of atrial communication – compliance of two ventricles is nearly equal • CHD and neonatal circulation – VSD, PDA : usually not symptomatic – ASD : usually not symptomatic