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2. heart pathology; congenital disorders


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2. heart pathology; congenital disorders

  1. 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 1 Dr. Krishna Tadepalli, MD,
  2. 2. Heart - Pathology Congenital Heart Disease  Clinical Features  Malformations causing left-to-right shunt right-to-left shunt Obstruction  Mechanisms - left-to-right shunt Type of Defect Mechanism Ventricular Septal Defect (VSD) hole within the membranous or muscular portions of the interventricular septum Atrial Septal Defect (ASD) A hole from a septum secundum or septum primum defect in the interatrial septum Patent Ductus Arteriosus (PDA) Ductus Arteriosus remains open (normally closes Soon after birth) 2 Dr. Krishna Tadepalli, MD,
  3. 3. Heart - Pathology Congenital Heart Disease  Mechanisms - right-to-left shunt Tetrology of Fallot Pulmonic stenosis  right ventricular hypertrophy , VSD& overriding aorta Transposition of Great Vessels Aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A VSD, or ASD with PDA, is needed for survival Truncus Arteriosus Incomplete separation of the aortic and pulmonary outflows 3 Dr. Krishna Tadepalli, MD,
  4. 4. Heart - Pathology Congenital Heart Disease  Mechanisms - others Hypoplastic Left Heart Syndrome hypoplasia or atresia of the aortic and mitral valves, with a small to absent left ventricle Coarctation of Aorta outflow obstructive narrowing of the aortic lumen, proximal (infantile form) or distal (adult form) to the ductus Total Anomalous Pulmonary Venous Return (TAPVR) pulmonary veins drain into left innominate vein, coronary sinus, or some other site 4 Dr. Krishna Tadepalli, MD,
  5. 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 5 Dr. Krishna Tadepalli, MD,
  6. 6. Heart - Pathology Congenital Heart Disease VSD   Most common congenital cardiac anomaly (42%) Spontaneously close in half of patients  ⅔ 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 6 Dr. Krishna Tadepalli, MD,
  7. 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 7 Dr. Krishna Tadepalli, MD,
  8. 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 8 Dr. Krishna Tadepalli, MD,
  9. 9. Heart - Pathology Tetralogy of Fallot  Tetra = 4  sub pulmonary stenosis  Right ventricular hypertrophy  VSD  Aorta that overrides the VSD  Morphology  Boot-shaped Heart (due to right ventricular hypertrophy)  Aortic valve insufficiency or ASD (Pentology)  Pink Tetralogy = Mild Sub-pulmonary stenosis TGA    TGA incompatible unless a shunt exists TGA + VSD (35%) = stable shunt TGA + Patent foramen ovale or PDA (65%)=unstable shunts 9 Dr. Krishna Tadepalli, MD,
  10. 10. Heart - Pathology            Truncus Arteriosus Single great artery that receives blood from both ventricles Early systemic cyanosis Irreversible pulmonary HTN Tricuspid Atresia High mortality in the first weeks underdevelopment (hypoplasia) of RV Congenital Cyanosis Coarctation of Aora Males are affected twice females with Turner syndrome Two classic forms – infantile, adult half of cases = associated with bicuspid aortic valve Clinical features= rib notching, pan systolic murmur & thrill  hypertension in the upper extremities  weak pulses and Hypotension in the lower extremities 10 Dr. Krishna Tadepalli, MD,
  11. 11. Heart - Pathology 11 Dr. Krishna Tadepalli, MD,