Congenital heart disease

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Congenital heart disease is a niche area of a niche area.

Ventricular septal defect

  • Most common congenital heart defect.

Tetralogy of Fallot (TOF)


  • Most common cause of a blue baby.


  • Abnormal septation of the truncus arteriosus.



  • Right ventricular hypertrophy,
  • Right ventricular outflow tract obstruction,
  • overriding aorta,
  • VSD (ventricular septal defect).


  • Overriding aorta = aorta has a biventricular connection --takes blood from the right ventricle.
  • Tight ventricular outflow tract obstruction is usually subpulmonic stenosis.

Atrial septal defect

  • May be seen in adults.


  • Fixed S2 split.



  • Ostium secundum (most common) - between SVC and IVC.
  • Ostium primum - between SVC and IVC closer to RV.
  • Upper sinus venosus defect - at SVC.
  • Lower sinus venosus defect - at IVC.
  • Coronary sinus defect.

The ostium secundum are the most common and usually found in isolation, i.e. there are not other associated abnormalities.

Left hypoplastic heart syndrome


  • Physiologically inadequate left ventricle.

Key characteristic:[4]

  • Atrial septal defect (ASD) -- left-to-right shunt.


  • Mitral stenosis.
  • Left ventricular hypoplasia.

Associations:Cite error: Closing </ref> missing for <ref> tag

Transposition of great vessels

  • Aorta and pulmonary trunk hooked-up to the wrong ventricle.
    • Survivable after birth only if there is a functional shunt, e.g. (persistent) patent ductus arteriosus and/or atrial septal defect.
    • When the ductus arteriosus closes they are in trouble.

Splenic abnormalities

Asplenia is associated with cardiac abnormalities:[5]

  • Aspenia = Boys, Bad congenital malformations (transposition of great vessels, pulmonary atresia/stenosis, totally anomalous pulmonary return).
    • Interesting is that these are all right-to-left shunts.

For completeness... polyspenia associations:[5]

  • Polyspenia = Girls, Good prognosis, Gastrointestinal situs inversus.

Eisenmenger syndrome


  • Pulmonary arterial hypertension with a
  • Right-to-left shunt (that was initially left-to-right), and
  • Cyanosis.

Note: Eisenmenger syndrome, by definition in some sources, is (only) due to a VSD.


  • ASD - rarely causes Eisenmenger syndrome.
  • VSD.
  • Extra-cardiac shunt.
  • End-stage disease often characterized by fibrinoid necrosis of small pulmonary arterial vessels (arterioles & small arteries).[8]

Cor pulmonale

Pulmonary hypertension due to heart disease.

Pulmonary hypertension

Pressure - definition:[6]

  • Mean pulm. arterial pressure >25 mmHg at rest.

See also


  1. Robbins p.568.
  3. Moore. TDH. P.361
  5. 5.0 5.1 Rose V, Izukawa T, Moes CA (August 1975). "Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis". Br Heart J 37 (8): 840-52. PMC 482884. PMID 1191445.
  6. 6.0 6.1 Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskr. Laeg. 171 (15): 1270-5. PMID 19416617.
  7. [1]
  8. Daliento L, Rebellato L, Angelini A, et al. (2002). "Fatal outcome in Eisenmenger syndrome". Cardiovasc. Pathol. 11 (4): 221-8. PMID 12140128.