Heart - Pathology Congenital Heart Disease MC type of heart

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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

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Heart - Pathology

• Congenital Heart Disease
• Clinical Features
• Malformations causing
• left-to-right shunt
• right-to-left shunt
• Obstruction
• Mechanisms - left-to-right shunt

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Heart - Pathology

• Congenital Heart Disease
• Mechanisms - right-to-left shunt

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Heart - Pathology

• Congenital Heart Disease
• Mechanisms - others

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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

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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

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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

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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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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

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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

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Heart - Pathology