Patologická fyziológia kardiovaskulárneho systému 3. Vrodené ...

Patologická fyziológia kardiovaskulárneho systému 3. Vrodené ...

Pathological physiology of cardiovascular system 3. Congenital heart diseases Rcz Oliver, Sedlkov Eva Institute of Pathological Physiology, Medical School, P.J. afrik University Oliver Rcz 2011 01.03.2020 kvs309e.ppt 1 Occurence & clinical significance of congenital heart defects 0,6 0,7 % live births ( 300/year)year) Prenatal and/year)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 01.03.2020 *foramen ovale is not closed in 25 % of healthy people kvs309e.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 Fallots tetralogy and others

01.03.2020 kvs309e.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 Fallots tetralogy and others There are congenital and (mostly NOT) hereditary conditions

But there are also hereditary heart pathologies: Some arrhytmias Hypertrophic and dilated cardiomyopathies 01.03.2020 kvs309e.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 01.03.2020 kvs309e.ppt 5 Embryological development & intrauterine circulation 01.03.2020

kvs309e.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 01.03.2020

kvs309e.ppt 7 Embryological development & intrauterine circulation 01.03.2020 kvs309e.ppt 8 Foramen ovale persistens

01.03.2020 kvs309e.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 01.03.2020

kvs309e.ppt 10 Rubella and not only the heart Congenital defects Sensorineural deafness Congenital heart defects Cataract, choroidoretinitis Growth retardation Microcephaly, mental retardation Urogenital abnormalities 01.03.2020 kvs309e.ppt

11 Rubella and not only the heart Transient abnormalities Thrombocytopenic purpura Bone lesions Pneumonitis Hepatosplenomegaly Late consequences ???? Diabetes mellitus Thyroid dysfunction Autism Panencephalitis 01.03.2020 kvs309e.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 01.03.2020

kvs309e.ppt 13 Incidency (106 births), 2002 Malformation Ventricular septum defect Incidence 4482 % 42 Atrial septum defect Pulmonal stenosis

1043 836 10 8 Ductus Botalli Fallot tetralogy 781 577 7 5 Coarctation of aorta

AV defect 492 396 5 4 Aortic stenosis Complete transposition 388 388 4 4

Other 374 3 Ebstein: 1/year)20 000 or 0,5 % of cong. Heart defects 01.03.2020 kvs309e.ppt 14 Atrial septum defect 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 01.03.2020 kvs309e.ppt 15

01.03.2020 RA LA RV LV kvs309e.ppt 16 01.03.2020

RA LA RV LV kvs309e.ppt 17 01.03.2020 kvs309e.ppt 18

Ventricular septum defect 80 % p. membranacea 15 % p. muscularis (m. Roger small hole, strong murmur) pulmonary circulation overload, pulmonary hypertension 01.03.2020 kvs309e.ppt 19

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 01.03.2020 kvs309e.ppt 20 01.03.2020 RA LA RV

LV S kvs309e.ppt 21 01.03.2020 kvs309e.ppt 22 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 01.03.2020 kvs309e.ppt 23 RA

LA RV LV S D 01.03.2020 kvs309e.ppt 24 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 01.03.2020 kvs309e.ppt 25 01.03.2020

kvs309e.ppt 26

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