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Heart - Pathology

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Heart - Pathology

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    1. Heart - Pathology Congenital Heart Disease MC type of heart disease among children MC type= VSD Etiology and Pathogenesis Multifactorial = majority of cases Developmental Trisomy 21 (Down syndrome) MC known genetic cause Endocardial cushion defects (AVSD) & ASD Di -George syndrome with TOF Environment= infection (congenital rubella) or teratogens

    2. Heart - Pathology Congenital Heart Disease Clinical Features Malformations causing left-to-right shunt right-to-left shunt Obstruction Mechanisms - left-to-right shunt

    3. Heart - Pathology Congenital Heart Disease Mechanisms - right-to-left shunt

    4. Heart - Pathology Congenital Heart Disease Mechanisms - others

    5. Heart - Pathology Congenital Heart Disease Left-to-right shunts ASD, VSD, and Patent Ductus Arteriosus [PDA] Congenitally acyanotic Late cyanotic congenital heart disease or Eisenmenger syndrome ASD MC congenital cardiac anomaly asymptomatic until adulthood Secundum ASD =90% of all ASDs Defect is due to deficient or fenestrated oval fossa Volume hypertrophy of RA & RV Pulmonary blood flow - 2 to 4 times normal Murmur at pulmonary area Complications = uncommon Normal postoperative survival

    6. Heart - Pathology Congenital Heart Disease VSD Most common congenital cardiac anomaly (42%) Spontaneously close in half of patients ? rd are associated with other defects (TOF) clinically = Size is most critical Morphology Membranous VSD(90%) Swiss-cheese septum =Multiple muscular septum VSDs Right ventricular hypertrophy and pulmonary hypertension are present from birth Rx = Surgical closure indicated at age 1 year with large defects

    7. Heart - Pathology Congenital Heart Disease PDA Majority are isolated defects (90%) Others (10%)-associated with VSD, COA, or PS, AS No functional difficulties at birth & childhood Continuous harsh machinery-like murmur PDA is Life-threatening or life-saving prostaglandin E ? preservation of ductal patency ? Drug closes PDA AVSD (endocardial cushion defect ) Superior & inferior endocardial cushions fail to fuse ? Inadequate formation of the AV valves MC with Down syndrome

    8. Heart - Pathology Congenital Heart Disease Right to left shunts Tetralogy of Fallot - MC cyanotic congenital heart disease Transposition of the great arteries Tricuspid atresia Total anomalous pulmonary venous connection Truncus Arteriosus

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