Pathology. Congenital heart disease. congenital heart diseases. congenital heart diseases - etiology. lecture 7

Transcription

1 Pathology lecture 7 prof hab. n. med. Andrzej Marszałek Congenital heart disease congenital heart diseases Def.: anatomical anomalies present at delivery ambnormal embryogenesis 3. and 8. week of gestation in 11-18% cases there abnormalities in other organs congenital heart diseases - etiology 90% cases unknown embryonal factors: genetic (5%) chromosomal abnormalities : Turner s s. Down s s. metabolic disorders: Hurler s s., Marfan s s.

2 congenital heart diseases - etiology Placental factors: problems with placenation Early pregnancy bleedings abnormal vessels in placenta or unblical cord congenital heart diseases - etiology other: irradiation drugs (eg. Thalidomid) fever malnutrition hormonal abnormalities hypoxia infections (rubella) DM in mother tobacco smoking congenital heart diseases - etiology found in 3 to 6 (even 8)/1000 born alive higher in stillbirths and preterm deliveries without treatment moratlity up to 50% congenital heart diseases - etiology the most common in newborns: 28-33% VSD (ventricular septal defect) [80% early death] 10-12% PDA (patent ductus arteriosus) [30% early death] 5-10% ASD (atria septal defect) [30% early death] 5-9% great vessels transposition [80% early death] 8% aortic stenosis [50% early death] 7-9% Fallot s tetralogy [25% wczesny zgon] 7-10% PS (pulmonary stenosis)

3 congenital heart diseases M:F [VSD] most common 1:1 [ASD] 1:2 [PDA] 1:2 Fallot s tetralogy most common najczęstsza sinicza 1:1 Aortic valve stenosis 3:1 Aortic coartation 2:1 Great vessels transposition 2:1 congenital heart diseases - clinic might be lethal Born alive then symptoms related to cahnges in circulation Some are clinical silent till late adulthood congenital heart diseases - clinic increased risk of endocarditis Abnormal coagulation Risk during pregnancy (woman) Increase risk of arrythmias ASD Could be clinically silent till age of 30 Irreversible pulmonary hypertension less then 10% of cases Low mortality dyspnea cyanosis

4 VSD PDA 90% septum BŁONIASTA 10% muscular septum (could heal spontaneously) Surgical correction usually before 1 year of age 90% cases a single abnormality Could be accompanied by VSD, coarctation, aortic stenosis In some cases is beneficial Complete transposition of great vessels in 35% together with other abnormality ( VSD) in 65% with formane ovale and PDA Without surgical correction lead to death Tetralogy of Fallot the most common (75%) cyanotic heart anomalyin kids above age of 2 described by Fallot (1888): Stenosis of pumonary trunk VSD Shift of pulmonary artery to the right right venricle hypertrophy In some cases: foramen ovale or ASD

5 other endocarditis PERICARDITIS PERICARDITIS In pericardial sac normally about 50 ml of fluid but it could raised up to 2000 ml, Rapid increase of volume ( ml) my lead to cardiac arrest haemopericardium Most common becouse of heart rupture (MI), injury, dissecting aneurysm, rupture of vessels in neoplasm, bleeding diathesis heart tamponade

6 HEART TUMORS HEART TUMORS primary secondary (metastatic) 30 times more common then primary lipoma benign hemangioma MALIGNANT angiosarcoma fibrosarcoma myxoma rhabdomyosarcoma (in kids) rhabdomyoma Inflammatory tumor leiomyosarcoma hemangiopericytoma myxoma Most common primary (~40%) Could be familial (Carneya s.) 90% in atria (80% left) 50 y.o.a clinic: Tumor balloting emboli fever Clinically malignant Heart tumors secondary metastatic; Most common from: MM Breast ca Lung ca RCC choriocarcinoma rhabdomyosarcoma (kids)

7 Heart tumors secondary Invading by veins: Adrenal cortex ca mesothelioma osteosarcoma (pelvis) RCC Malignant thymoma Vascular lesions Hemangioma beingn Like normal vessels Congenital or acquired most common in infants and kids H&N, and liver

8 Angiosarcoma malignant several localizations: skin, Soft tissues, breast, liver, spleen irregular chanels & endothelia (CD31+) angiosarcoma prognosis: POOR 5-yrs survival 10-20% meta: lungs liver spleen Lymph nodes Kaposi sarcoma lymphangiomas Low grade malignant tu Described by Kaposi in 1872 developed from mesenchymal cell types: classic and HIV-related

9 Kaposi sarcoma classical, rare, related to immunocompromised status (but not HIV) In Europeans (90% males from East Europe; Ashkenazi Jews) Endemic in Africa (young patieints) slow progression (ussually only in the skin) in elderly recurrencies AIDS-related(epidemic), Kaposi sarcoma Poor prognosis (if over 50yrs) Kaposi sarcoma Transplants receipients