Congenital heart disease

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

Ventricular septal defect

  • Common serious congenital heart defect.
    • Most common congenital defect = bicuspid aortic valve.[1]

Tetralogy of Fallot (TOF)

General

  • Most common cause of a blue baby.

Etiology

  • Abnormal septation of the truncus arteriosus.

Definition

Features:[2]

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

Notes:

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

Atrial septal defect

  • May be seen in adults.

Clinical

  • Fixed S2 split.

Classification

Types:[3]

  • 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

Defintion:[4]

  • Physiologically inadequate left ventricle.

Key characteristic:[5]

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

Causality:

  • Mitral stenosis.
  • Left ventricular hypoplasia.

Associations:[5]

  • Turner syndrome.
  • Noonan syndrome - sometimes called "male version of Turner syndrome".
  • Smith-Lemli-Opitz syndrome.
  • Holt-Oram syndrome.[6]

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:[7]

  • 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:[7]

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


Eisenmenger syndrome

Definition:[8][9]

  • 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.

Causes:

  • 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).[10]

Cor pulmonale

Pulmonary hypertension due to heart disease.

Pulmonary hypertension

Main article: Pulmonary hypertension

Pressure - definition:[8]

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

See also

References

  1. ↑ Siu SC, Silversides CK (June 2010). "Bicuspid aortic valve disease". J. Am. Coll. Cardiol. 55 (25): 2789–800. doi:10.1016/j.jacc.2009.12.068. PMID 20579534.
  2. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 568. ISBN 0-7216-0187-1.
  3. ↑ http://en.wikipedia.org/wiki/File:ASD.png
  4. ↑ Moore. TDH. P.361
  5. ↑ 5.0 5.1 http://emedicine.medscape.com/article/890196-overview
  6. ↑ Ekure EN, Okoromah CN, Briggs E, Ajenifuja OA (September 2004). "Holt-Oram syndrome with hypoplastic left heart syndrome in an African child". Niger Postgrad Med J 11 (3): 190–2. PMID 15505648.
  7. ↑ 7.0 7.1 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. http://www.pubmedcentral.nih.gov/pagerender.fcgi?artid=482884&pageindex=1#page.
  8. ↑ 8.0 8.1 Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskr. Laeg. 171 (15): 1270-5. PMID 19416617.
  9. ↑ [1]
  10. ↑ Daliento L, Rebellato L, Angelini A, et al. (2002). "Fatal outcome in Eisenmenger syndrome". Cardiovasc. Pathol. 11 (4): 221-8. PMID 12140128.
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