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Difference between revisions of "Congenital heart disease"
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'''Congenital [[heart]] disease''', abbreviated '''CHD''', is a niche area of a | '''Congenital [[heart]] disease''', abbreviated '''CHD''', is a niche area of [[cardiac pathology]], which is a subset of [[cardiovascular pathology]]. | ||
=Paediatric cardiac surgery= | |||
====Norwood procedure==== | ====Norwood procedure==== | ||
Indication: | Indication: | ||
| Line 76: | Line 76: | ||
*TOF is the classic cause of "blue babies". | *TOF is the classic cause of "blue babies". | ||
=Left-to-right= | |||
==Ventricular septal defect== | ==Ventricular septal defect== | ||
*Common serious congenital heart defect. | *Common serious congenital heart defect. | ||
**Most common congenital defect = bicuspid aortic valve.<ref name=pmid20579534>{{cite journal |author=Siu SC, Silversides CK |title=Bicuspid aortic valve disease |journal=J. Am. Coll. Cardiol. |volume=55 |issue=25 |pages=2789–800 |year=2010 |month=June |pmid=20579534 |doi=10.1016/j.jacc.2009.12.068 |url=}}</ref> | **Most common congenital defect = bicuspid aortic valve.<ref name=pmid20579534>{{cite journal |author=Siu SC, Silversides CK |title=Bicuspid aortic valve disease |journal=J. Am. Coll. Cardiol. |volume=55 |issue=25 |pages=2789–800 |year=2010 |month=June |pmid=20579534 |doi=10.1016/j.jacc.2009.12.068 |url=}}</ref> | ||
==Atrial septal defect== | |||
*May be seen in adults. | |||
===Clinical=== | |||
*Fixed S2 split. | |||
===Classification=== | |||
Types:<ref>[http://en.wikipedia.org/wiki/File:ASD.png http://en.wikipedia.org/wiki/File:ASD.png]</ref> | |||
*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. | |||
==Eisenmenger syndrome== | |||
===General=== | |||
Definition:<ref name=pmid19416617>{{cite journal |author=Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L |title=[Eisenmenger syndrome] |language=Danish |journal=Ugeskr. Laeg. |volume=171 |issue=15 |pages=1270-5 |year=2009 |month=April |pmid=19416617 |doi= |url=}}</ref> | |||
*[[Pulmonary arterial hypertension]] with a | |||
*Right-to-left shunt (that was initially left-to-right), and | |||
*Cyanosis. | |||
====Causes==== | |||
*ASD - rarely causes Eisenmenger syndrome. | |||
*VSD. | |||
*Extra-cardiac shunt. | |||
Note: Eisenmenger syndrome, by definition in some sources, is (only) due to a VSD. | |||
===Microscopic=== | |||
End-stage disease:<ref name=pmid12140128>{{cite journal |author=Daliento L, Rebellato L, Angelini A, ''et al.'' |title=Fatal outcome in Eisenmenger syndrome |journal=Cardiovasc. Pathol. |volume=11 |issue=4 |pages=221-8 |year=2002 |pmid=12140128 |doi= |url=}}</ref> | |||
*Often characterized by fibrinoid [[necrosis]] of small pulmonary arterial vessels (arterioles & small arteries). | |||
=Right-to-left= | |||
==Tetralogy of Fallot (TOF)== | ==Tetralogy of Fallot (TOF)== | ||
===General=== | ===General=== | ||
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*Right ventricular outflow tract obstruction is usually subpulmonic stenosis. | *Right ventricular outflow tract obstruction is usually subpulmonic stenosis. | ||
== | ==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. | |||
* | |||
* | |||
* | |||
=Other= | |||
==Left hypoplastic heart syndrome== | ==Left hypoplastic heart syndrome== | ||
Defintion:<ref>Moore. TDH. P.361</ref> | Defintion:<ref>Moore. TDH. P.361</ref> | ||
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*Holt-Oram syndrome.<ref name=pmid15505648>{{cite journal |author=Ekure EN, Okoromah CN, Briggs E, Ajenifuja OA |title=Holt-Oram syndrome with hypoplastic left heart syndrome in an African child |journal=Niger Postgrad Med J |volume=11 |issue=3 |pages=190–2 |year=2004 |month=September |pmid=15505648 |doi= |url=}}</ref> | *Holt-Oram syndrome.<ref name=pmid15505648>{{cite journal |author=Ekure EN, Okoromah CN, Briggs E, Ajenifuja OA |title=Holt-Oram syndrome with hypoplastic left heart syndrome in an African child |journal=Niger Postgrad Med J |volume=11 |issue=3 |pages=190–2 |year=2004 |month=September |pmid=15505648 |doi= |url=}}</ref> | ||
==Splenic abnormalities== | ==Splenic abnormalities== | ||
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For completeness... polyspenia associations:<ref name=pmid1191445/> | For completeness... polyspenia associations:<ref name=pmid1191445/> | ||
*Polyspenia = '''G'''irls, '''G'''ood prognosis, '''G'''astrointestinal situs inversus. | *Polyspenia = '''G'''irls, '''G'''ood prognosis, '''G'''astrointestinal situs inversus. | ||
==Cor pulmonale== | ==Cor pulmonale== | ||
Pulmonary hypertension due to heart disease. | [[Pulmonary hypertension]] due to heart disease. | ||
===Pulmonary hypertension=== | ===Pulmonary hypertension=== | ||
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*Mean pulm. arterial pressure >25 mmHg at rest. | *Mean pulm. arterial pressure >25 mmHg at rest. | ||
=See also= | |||
*[[Cardiovascular pathology]]. | *[[Cardiovascular pathology]]. | ||
*[[Heart]]. | *[[Heart]]. | ||
*[[Pulmonary hypertension]]. | *[[Pulmonary hypertension]]. | ||
=References= | |||
{{reflist|2}} | {{reflist|2}} | ||
[[Category:Cardiovascular pathology]] | [[Category:Cardiovascular pathology]] | ||