Congenital heart disease (notes) Year 4 Paediatrics Notes

Congenital heart disease (notes) Year 4 · Paediatrics Collection: 4TH YEAR ALL NOTES / PAEDS / OTHER NOTES -- Congenital Heart Disease \nMohammed Alghamdi, MD, FRCPC, FAAP, FACC\nAssociate Professor and Consultant\nPediatric Cardiology, Cardiac Science\nKing Fahad Cardiac Centre\nKing Saud University The dr didn’t give his slides and mentioned illustrated as the reference \n\n INTRODUCTION\nThe most common malformation in children\n• CHD ~ 0.8% of live births.\n• Major CHD: CHD is typically 1 in a 100.\n\n – Ventricular Septal Defect: 35% The most common\n\n – Atrial Septal Defect: 7 %\n– Patent Ductus Ateriosus: 7 %\n– Coarctation of Aorta: 6 %\n– Tetralogy of Fallot: 6 % Most common cyanotic heart disease\n\n – Pulmonary valve stenosis: 6 %\n– Aortic valve stenosis: 5 %\n– D-Transposition of great arteries: 4 % Is CHD something inherited from your parents? Nobody can answer that, it is multifactorial, and your percentage of having it with normal parents is 1% but if one of your parents have CHD then that percentage increases to 2% other diseases like (left sided obstructive lesions: coarctation, bicyspid aortic valve) the percentage will rise to 4-5% \n\n Congenital Heart Disease\nimp\n• Etiology: Mostly unknown\n• Chromosomal abnormalities can cause\n• Trisomy 21: AVSD 50% of the cases of downsyndrome have CHD, most commonly AVSD\n\n • Trisomy 18: VSD\n• Trismoy 13: PDA, VSD, ASD The last 4 are above your level\nArch obstruction\n• DiGeorge Syndrome: Arch, Conotruncal abnormalities\n• Turner syndrome: Coarctation of Aorta + Bicuspid Aortic Valve\n\n • Williams Syndrome: Supra-aortic stenosis, PA stenosis\nProbably\n\n I Bo • Noonan Syndrome: Dysplastic pulmonary valve v\nNot going\nto ask you\nabout it.\n\n Pulmonary\nAlso called male Turner ArteryBranch\nstenosis \n\n New slide\n\n 20-30% of cardiac\nlow output to lungs\nResistance\nResistance\nAll organs are HighGi system lower the resistance\nexcept the kidney when eating to increase blood ow\n\n 1 Resistance\nWe all know that fluids like to move from High resistance to low resistance What are the low resistance organs in our bodies\nThe\nlungs and Kidneys So if there is hole in the Heart the\nany\nthe lungs This is a\ngo to\nBlood will choose to\nkey to understand all the diseases 3 Duct Dependant circulation\nSome Congenital Heart disease are called Duct Dependant\ncirculation Diseases means that if the duct closes the Baby\nwill die We can divide them into \nPulmonary duct dependant The forward flow of the Blood\nfrom the Heart to the lungs is limited therefor thereshould\nvery\nbe another Pathway which is the PDA\nSystemicductdependant The forward flowfrom the left Ventricle\nto the Body if it gets significantly Reduced by a critical AorticValve\nstenosis or critical coartication and source on way for the Blood to reach\ntheBody has to be there \n\n Classification of CHD Divided into 2 major groups: Presence or absence of cyansosis\n\n – Cyanotic heart diseases.\n– Acyanotic heart diseases. Subdivided further according to:\n– Physical Finding\n– Chest X-ray finding\n– ECG finding Diagnosis is confirmed by:\n– Echo, Cardiac CT/MRI or Cardiac Catheterization.\n• Best modality to diagnose CHD is Echo (very good for detecting intra-cardiac abnormalities because it has motion)\n• Cardiac CT/ MRI is used to investigate something the echo can’t show (detects extra-cardiac abnormalities)\n(MRI is good for 1. Volume 2. Function 3. Extra-cardiac structure like veins and arteries)\nThe CT is the best when it comes to extra-cardiac structures but it has radiation. So we usually go for MRI\n• Cardiac catheter is good for hemodynamic assessment measuring the pressure gradient or for interventions \n\n HKEY CONCEPTS: \n• End result of L-R shunt is pulmonary overcirculation\n• It is the mixing of red blood with blue blood that increases the volume and that increased volume goes to the pulmonary circulation \n• R-L shunt end result is pulmonary hypoperfusion\n• The blue blood mixes with the red blood so the blue blood in the right ventricle decreases leading to pulmonary hypoperfusion reducing the\npulmonary flow. \n• The aorta should receive pure red blood, when you have R-L shunt you have mixed blood so you have a cause of cyanosis \n• in L-R shunt the aorta is receiving PURE red blood but LESS amount so it does not cause cyanosis, the right ventricle is getting more blood\nwhich will eventually go to the lung leading to heart failure \nWhat defines a L-R shunt vs. R-L Shunt (blood flow)\n1. Pressure difference ( blood goes from high pressure to low pressure)\n2. Resistance difference (the blood goes to where there is less resistance)\n• you have 2 circulations the pulmonary vs the systemic resistance. The systemic circulation has the Higher resistance unlike the pulmonary which\nhas a Lower resistance this is because of the pressure difference (systemic 120/80 vs pulmonary 20/8) so the blood will choose to go to the\nlungs \n3. Location of the defect\n4. Size of t