CONGENITAL HEART DISEASE.

1
CONGENITAL HEART DISEASE.

• SADATH ALI KHAN.

2
Epidemiology

• Prevalence0.5-0.8 of live births
  (8/1000).Leading cause ofdeath in children with
  CHD.
• EtiologyUnknown,multifactorial
  inheritance,genetic factors implicated,high
  incidence in first degree relatives.
• 3 have a single gene defect,13 have associated
  chromosomal abnormalities.
• 2-4 are associated with environmental or
  maternal conditions teratogenic influences.
• Gender differencesASD,VSD,PDA Pulmonic
  stenosis more common in girls,left sided lesions
  in boys.

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Classification

• Acyanoticaccording to the predominant
  physiologic load placed on the heart.
• Volume loadL-R shunts-ASD,VSD,PDA.
• Pressure loadVentricular outflow obstruction
• Pulmonary,aortic valve lesions,aortic coarctation
  pulmonary stenosis.

• Cyanoticbased on pathophysiology.
• Decreased pulmonary bloodflowTOF,Pulmonary
  atresia,Tricuspid atresia,Single ventricle with
  pulmonic stenosis.
• Increased pulmonary blood flowTransposition of
  great vessels,Truncus arteriosus.

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ATRIAL SEPTAL DEFECT.

• Sinus venosus defecthigh in the septum.
• Ostium secundum defectmidseptum.
• Ostium primum defectlow in the septum.
• PathophysiologyL-R shunt-increased flow across
  Rt heart-RV PA enlargement.
• Clinical featuresasymptomatic,slow wt
  gain,frequent LRTI.
• DiagnosisRt ventricular heave,systolic
  murmur,fixed wide split S2.

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Investigations

• CXRenlarged heart PA,increased vascularity.
• ECGRt axis in secundum defect,hallmark of primum
  defect is extreme Lt axis,RVH.
• ECHORVH,valve anatomy,flow direction.
• Treatmentclosure during cardiac
  cathetrization,surgical closure.

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VENTRICULAR SEPTAL DEFECT.

• Most common CHD (26),may be single or multiple.
• PathophysiologyLt-Rt shunt as long as pulmonary
  vascular resistance is lower than systemic
  resistance,if reverse shunt reverses
• Large defects lead to pul.hypertension-Eissenmenge
  r syndrome.
• Clinical features depend on size,asymptomatic,gro
  wth failure,recurrent LRTI,congestive heart
  failure,SOB,cyanosis
• Diagnosispansystolic murmur,loud p2.

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Investigations

• CXRcardiomegaly,enlarged LALV.
• ECGextreme lt axis is charecteristic,biventricula
  r hypertrophy.
• ECHOchamber size pressures.
• Cardiac catheterO2 content,PA pressure,size no
  of defects.
• TreatmentEndocarditis prophylaxis,digoxin,diureti
  cs.
• Surgical closure before pulmonary vascular
  changes become irreversible.

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PATENT DUCTUS ARTERIOSUS.

• Connection between PA descending aorta
• 10 of CHD.
• PathophysiologyLt-Rt shunt,reverses if pulmonary
  resistance increases-RV enlargement.If PDA is
  large Eissenmenger syndrome can develop.
• Clinical featuresdepend on size direction of
  flow,slow growth,LRTI,SOB,cyanosis.
• Diagnosisbounding pulse,continous murmur,loud S2.

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Investigations

• CXRcardiomegaly,increased pul vascularity.
• ECGLt or biventricular hypertrophy.
• ECHO2D visualises PDA,doppler shows turbulance.
• Cardiac catheterPA pressures O2 sats.
• TreatmentEndocardial prophylaxis as long as
  patent,Indomethacin.
• Surgicalligation is curative.