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Transcript
Pathological physiology of cardiovascular system 3. Congenital heart diseases Rácz Oliver, Sedláková Eva Institute of Pathological Physiology, Medical School, P.J. Šafárik University © Oliver Rácz 2011 16.2.2012 kvs3e12.ppt 1 Occurence & clinical significance of congenital heart defects 0,6 – 0,7 % live births ( 300/year) Prenatal and/or very early diagnostics Early or postponed surgical intervention Two thirds live up to adult age (sometimes with residual abnormalities) Sometimes (ASD) discovered in adult age* In Slovakia 10 000 people 16.2.2012 *foramen ovale is not closed in 25 % of healthy people – kvs3e12.ppt 2 without consequences Classification (Cyanotic & noncyanotic) Defects with shunts (left to right, late cyanosis) defects of atrial or ventricular septum, ductus Botalli apertus (ASD, VSD, DBA) Defects with stenoses aortal & pulmonal stenosis, coarctation of aorta Defects with dyslocation dextrocardia, transposition big vessels Combined – Fallot’s tetralogy and others 16.2.2012 kvs3e12.ppt 3 Classification 1. Defects with shunts (left to right, late cyanosis) defects of atrial or ventricular septum, ductus Botalli apertus (ASD, VSD, DBA) 2. Combined – Fallot’s tetralogy and others There are congenital and (mostly NOT) hereditary conditions But there are also hereditary heart pathologies: Some arrhytmias Hypertrophic and dilated cardiomyopathies 16.2.2012 kvs3e12.ppt 4 Embryological development of the heart and the intrauterine circulation 4th week: 5 segments of the embryonal tube: sinus venosus, common atrium, common ventricle, bulbus cordis and truncus arteriosus 5th – 8th week: septum formation between the left and right side, valves, endocardium – a very sensitive period of time ... Through pulmonary circulation only 5 % of blood 16.2.2012 kvs3e12.ppt 5 Embryological development & intrauterine circulation 16.2.2012 kvs3e12.ppt 6 Embryological development & intrauterine circulation Both ventricles pump blood into systemic circulation Foramen ovale Ductus arteriosus Oxygen through placenta and vena umbilicalis W. Harvey, 1578 - 1657 16.2.2012 kvs3e12.ppt 7 Embryological development & intrauterine circulation 16.2.2012 kvs3e12.ppt 8 Foramen ovale persistens 16.2.2012 kvs3e12.ppt 9 Rubella and not only the heart Togaviridiaes, Rubivirus 0,6 % of exposed women develop abnormalities 1st trimester infections lead to fetal damage. Delayed growth of tissues and Immune disturbances 16.2.2012 kvs3e12.ppt 10 Rubella and not only the heart Congenital defects Sensorineural deafness Congenital heart defects Cataract, choroidoretinitis Growth retardation Microcephaly, mental retardation Urogenital abnormalities 16.2.2012 kvs3e12.ppt 11 Rubella and not only the heart Transient abnormalities Thrombocytopenic purpura Bone lesions Pneumonitis Hepatosplenomegaly Late consequences ???? Diabetes mellitus Thyroid dysfunction Autism Panencephalitis 16.2.2012 kvs3e12.ppt 12 Etiology of congenital heart defects Viral infection in 5th – 8th gestational week (rubella and other). Chemical: alcohol, smoking, immunosuppresive drugs, thalidomid, antimetabolites and other. Hereditary (also – arrythmias, cardiomyopathies, valvular malformatioms) As a part of chromosomal aberrations and hereditary diseases m. Down, sy. Turner, Marfan etc. It is theory – the cause is clear only in 10% cases 16.2.2012 kvs3e12.ppt 13 Incidency (106 births), 2002 Malformation Ventricular septum defect Atrial septum defect Incidence 4482 1043 % 42 10 Pulmonal stenosis Ductus Botalli Fallot tetralogy 836 781 577 8 7 5 Coarctation of aorta AV defect Aortic stenosis Complete transposition 492 396 388 388 5 4 4 4 Other 374 3 Ebstein: 1/20 000 or 0,5 % of cong. Heart defects 16.2.2012 kvs3e12.ppt 14 Etiology of congenital heart defects congenital or genetic? Hereditary Holt-Oram sy. = ASD, disturbances of upper extremity development ?! – thalidomid ?! Gene for a transcriptional factor, TBX5 Mutation of another transcription factor NKX2-5 Heterozygotes: ASD, risk of sudden death Homozygote drosophila = tinman, no heart 16.2.2012 kvs3e12.ppt 15 Atrial septum defect Not ! The most common, women > men 2 basic types with left to right shunt ostium secundum ostium primum (+ abnormalities of AV valves) and abnormal position of pulmonary venes Increased blood flow through pulmonary circulation, later pulmonary hypertension Dg sometimes in adult life – dyspnoe, fatigue, supraventricular tachyarrhytmias 16.2.2012 kvs3e12.ppt 16 16.2.2012 RA LA RV LV kvs3e12.ppt 17 16.2.2012 RA LA RV LV kvs3e12.ppt 18 16.2.2012 kvs3e12.ppt 19 Ventricular septum defect 80 % p. membranacea 15 % p. muscularis (m. Roger – small hole, strong murmur) pulmonary circulation overload, pulmonary hypertension 16.2.2012 kvs3e12.ppt 20 25 % of congenital heart malformations 25 % died before age 20 years but 66% live up to 60 Most small defects close spontaneously before age 10 16.2.2012 kvs3e12.ppt 21 RA RV 16.2.2012 LA S kvs3e12.ppt LV 22 16.2.2012 kvs3e12.ppt 23 Open ductus Botalli Closing in full-term newborns in 24 h DBA often in premature newborns Pulmonary circulation overload Big shunt can cause heart failure Risk of bacterial endocarditis 16.2.2012 kvs3e12.ppt 24 RA LA RV LV S D 16.2.2012 kvs3e12.ppt 25 Eisenmenger syndrome ASD, VSD, DBA with pulmonary hypertension and right to left shunt Cyanosis, polyglobulia Dyspnoe, fatigue, syncopa, oedema Too late for surgery 16.2.2012 kvs3e12.ppt 26 Fallot tetralogy Pulmonary stenosis subaortal VSD riddling aorta right ventricular hypertrophy strong cyanosis, hypoxia growth retardation Ht, Hb, Er – high, high blood viscosity Blalock and Taussig and the lesson from Fallot pentalogy 16.2.2012 kvs3e12.ppt 27 16.2.2012 kvs3e12.ppt 28 Transposition of aorta/a. pulmonalis Two parallel circulations! RV – aorta – systemic circulation – v. cava – RA Deoxygenated blood LV – a. pulmonalis – pulmonary circulation – vv. pulmonales – LA Oxygenated blood Limited life due to shunts 16.2.2012 kvs3e12.ppt 29 Transposition of aorta/a. pulmonalis Two parallel circulations! Solution: Exchange the venous parts, too! Complete transposition but one circulation RV – system – LA – LV – lungs – RA… 16.2.2012 kvs3e12.ppt 30 16.2.2012 RA LA RV LV kvs3e12.ppt 31 16.2.2012 RA LA RV LV kvs3e12.ppt 32 16.2.2012 RA LA RV LV kvs3e12.ppt 33 Correction – „transtransposition“ 10 year survival is good Later problems Physical exercise Failure of the systemic right ventricle Late coplications, arrythmias. SK – 80-100 young people 16.2.2012 kvs3e12.ppt 34 16.2.2012 RA LA RV LV kvs3e12.ppt 35 Nezlučiteľná so životom 20-20/100 000 SK – 15 ročne Senning, 1959 Mustard, 1964 Prekríženie predsiení! Kaldarová a spol., Kardiológia pre prax 2008, 6, 219 – 223 Detské kardiocentrum, BA 16.2.2012 kvs3e12.ppt 36 Ebstein „Endocardial cushion defects“ Important for the development of AV region, lower part of atrial and upper part of ventricular septum Abnormal developent is responsible for cca 5% of congenital heart defects, in m. Down even in 50 % - some ASD, VSD, valvular abnormalities Ebstein – abnormal tricuspidal valve deep in the ventricle 16.2.2012 kvs3e12.ppt 37 16.2.2012 kvs3e12.ppt 38 Ebstein Ebstein – abnormal tricuspidal valve deep in the ventricle Atrialisation of the right ventricle, but contraction together with the other parts of the ventricle Regurgitation, worsened by the contraction of the ventricular part Often combined with WPW syndrome, ASD 16.2.2012 kvs3e12.ppt 39 16.2.2012 kvs3e12.ppt 40