Difference between revisions of "Congenital heart disease"

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Congenital heart disease (view source)

Revision as of 19:31, 8 January 2013

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====Norwood procedure====
====Norwood procedure====
Indication:  
Indication:  
*LHHS - following birth.
*[[LHHS]] - following birth.


Details:
Details:
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Indication:
Indication:
*LHHS - following Norwood procedure, reduce work done by the heart; usu. done at age 4 to 6 months.
*[[LHHS]] - following Norwood procedure, reduce work done by the heart; usu. done at age 4 to 6 months.


Details:
Details:
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*Fontan procedure cannot be done in a newborn as the pulmonary vascular bed resistance is too high.<ref name=pmid5089489/>
*Fontan procedure cannot be done in a newborn as the pulmonary vascular bed resistance is too high.<ref name=pmid5089489/>


==Shunts - overview==
=Shunts - overview=
Most shunts are a consequence of congenital heart disease.  They can be grouped into ''left-to-right'' and ''right-to-left''.
Most shunts are a consequence of congenital heart disease.  They can be grouped into ''left-to-right'' and ''right-to-left''.


===Left-to-right===
==Left-to-right==
Mnemonic ''the Ds'':<ref name=Ref_PBoD566>{{Ref PBoD|566}}</ref>
Mnemonic ''the Ds'':<ref name=Ref_PBoD566>{{Ref PBoD|566}}</ref>
*ASD = atrial septal defect.
*ASD = atrial septal defect.
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Note: The word ''Left'' has four letters and there are four L->R shunts.
Note: The word ''Left'' has four letters and there are four L->R shunts.
===Right-to-left===
==Right-to-left==
Mnemonic ''5 Ts'':<ref name=Ref_PBoD568>{{Ref PBoD|568}}</ref>
Mnemonic ''5 Ts'':<ref name=Ref_PBoD568>{{Ref PBoD|568}}</ref>
*Tetralogy of Fallot (TOF).  
*Tetralogy of Fallot (TOF).  
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=Left-to-right=
=Left-to-right=
==Ventricular septal defect==
==Ventricular septal defect==
*Abbreviated ''VSD''.
===General===
*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>
*Often associated with other cardiac abnormalities, e.g. [[Tetralogy of Fallot]].
**20-30% of VSDs are isolated.<ref name=Ref_PCPBoD8_287>{{Ref PCPBoD8|287}}</ref>
Subtypes:
#Membranous VSD ~ 90%.
#*Near aortic valve.
#Muscular VSD ~ 10%.
Clinical:
*Significance dependent on:
**Size - large ones require an intervention, e.g. surgery.
**Concurrent/associated pathology.


==Atrial septal defect==
==Atrial septal defect==
*Abbreviated ''ASD''.
===General===
*May be seen in adults.
*May be seen in adults.
*Classically a left-to-right shunt.
**Leads to RV dilation.<ref name=pmid22893692>{{Cite journal  | last1 = Nyboe | first1 = C. | last2 = Fenger-Grøn | first2 = M. | last3 = Nielsen-Kudsk | first3 = JE. | last4 = Hjortdal | first4 = V. | title = Closure of secundum atrial septal defects in the adult and elderly patients. | journal = Eur J Cardiothorac Surg | volume =  | issue =  | pages =  | month = Aug | year = 2012 | doi = 10.1093/ejcts/ezs405 | PMID = 22893692 }}</ref>


===Clinical===
Clinical:
*Fixed S2 split.
*Fixed S2 split.


===Classification===
===Classification===
Types:<ref>[http://en.wikipedia.org/wiki/File:ASD.png http://en.wikipedia.org/wiki/File:ASD.png]</ref>
Types:<ref name=Ref_PCPBoD8|287>{{Ref PCPBoD8|287}}</ref>
*Ostium secundum (most common) - between SVC and IVC.
#Ostium secundum  
*Ostium primum - between SVC and IVC closer to RV.
#*Between SVC and IVC.
*Upper sinus venosus defect - at SVC.
#Ostium primum  
*Lower sinus venosus defect - at IVC.
#*Between SVC and IVC. Closer to RV.
*Coronary sinus defect.
#Sinus venosus.
#*May be subdivided into:
#**Upper sinus venosus defect - at SVC.
#**Lower sinus venosus defect - at IVC.


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


==Eisenmenger syndrome==
==Eisenmenger syndrome==
===General===
===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>
Definition - all of the following:<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
#[[Pulmonary arterial hypertension]].
*Right-to-left shunt (that was initially left-to-right), and
#Right-to-left shunt (that was initially left-to-right).
*Cyanosis.
#Cyanosis.


====Causes====
====Causes====
*ASD - rarely causes Eisenmenger syndrome.
*[[ASD]] - rarely causes Eisenmenger syndrome.
*VSD.
*[[VSD]].
*Extra-cardiac shunt.
*Extra-cardiac shunt.


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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>
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).
*Often characterized by fibrinoid [[necrosis]] of small pulmonary arterial vessels (arterioles & small arteries).


=Right-to-left=
=Right-to-left=
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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==
==Transposition of the great vessels==
*[[AKA]] ''transposition of great arteries''.
*[[AKA]] ''transposition of the great arteries''.
===General===
===General===
*Aorta and pulmonary trunk hooked-up to the wrong ventricle.
*Aorta and pulmonary trunk hooked-up to the wrong ventricle.
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*Pulmonary veins do not attach to left atrium.
*Pulmonary veins do not attach to left atrium.
*Classified by where the veins attach - which is predictive of outcome.<ref>URL: [http://emedicine.medscape.com/article/899491-overview http://emedicine.medscape.com/article/899491-overview]. Accessed on: 10 March 2011.</ref><ref>{{cite journal |author=Karamlou T, Gurofsky R, Al Sukhni E, ''et al.'' |title=Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection |journal=Circulation |volume=115 |issue=12 |pages=1591–8 |year=2007 |month=March |pmid=17353446 |doi=10.1161/CIRCULATIONAHA.106.635441 |url=}}</ref>
*Classified by where the veins attach - which is predictive of outcome.<ref>URL: [http://emedicine.medscape.com/article/899491-overview http://emedicine.medscape.com/article/899491-overview]. Accessed on: 10 March 2011.</ref><ref>{{cite journal |author=Karamlou T, Gurofsky R, Al Sukhni E, ''et al.'' |title=Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection |journal=Circulation |volume=115 |issue=12 |pages=1591–8 |year=2007 |month=March |pmid=17353446 |doi=10.1161/CIRCULATIONAHA.106.635441 |url=}}</ref>
*Treatment: surgical repair (''Coles procedure''<ref>URL: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807798/ http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807798/]. Accessed on: 15 March 2011.</ref>
*Treatment: surgical repair (''Coles procedure'').<ref>URL: [http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807798/ http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807798/]. Accessed on: 15 March 2011.</ref> (???)


=Other=
=Other=
==Ebstein anomaly==
===General===
*Often right-to-left shunt - as it is seen with an [[atrial septal defect]] (ASD).
*Tricuspid incompetence -> right atrial enlargement.<ref>URL: [http://www.chop.edu/service/cardiac-center/heart-conditions/ebsteins-anomaly.html http://www.chop.edu/service/cardiac-center/heart-conditions/ebsteins-anomaly.html]. Accessed on: 27 November 2011.</ref>
*Weak association with maternal lithium use.<ref name=pmid16611133>{{Cite journal  | last1 = Giles | first1 = JJ. | last2 = Bannigan | first2 = JG. | title = Teratogenic and developmental effects of lithium. | journal = Curr Pharm Des | volume = 12 | issue = 12 | pages = 1531-41 | month =  | year = 2006 | doi =  | PMID = 16611133 }}</ref>
==Left hypoplastic heart syndrome==
==Left hypoplastic heart syndrome==
*Abbreviated ''LHHS''.
===General===
Defintion:<ref>Moore. TDH. P.361</ref>
Defintion:<ref>Moore. TDH. P.361</ref>
*Physiologically inadequate left ventricle.
*Physiologically inadequate left ventricle.


Key characteristic:<ref name=emed_hlhs>[http://emedicine.medscape.com/article/890196-overview http://emedicine.medscape.com/article/890196-overview]</ref>
Key characteristic:<ref name=emed_hlhs>[http://emedicine.medscape.com/article/890196-overview http://emedicine.medscape.com/article/890196-overview]</ref>
*Atrial septal defect (ASD) -- left-to-right shunt.
*[[Atrial septal defect]] (ASD) -- left-to-right shunt.


Causality:
Causality:
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Associations:<ref name=emed_hlhs/>
Associations:<ref name=emed_hlhs/>
*Turner syndrome.
*[[Turner syndrome]].
*Noonan syndrome - sometimes called "male version of Turner syndrome".
*Noonan syndrome - sometimes called "male version of Turner syndrome".
*Smith-Lemli-Opitz syndrome.
*Smith-Lemli-Opitz syndrome.
*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==
{{Main|Spleen}}
Asplenia is associated with cardiac abnormalities:<ref name=pmid1191445>{{cite journal |author=Rose V, Izukawa T, Moes CA |title=Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis |journal=Br Heart J |volume=37 |issue=8 |pages=840-52 |year=1975 |month=August |pmid=1191445 |pmc=482884 |doi= |url=http://www.pubmedcentral.nih.gov/pagerender.fcgi?artid=482884&pageindex=1#page}}</ref>
Asplenia is associated with cardiac abnormalities:<ref name=pmid1191445>{{cite journal |author=Rose V, Izukawa T, Moes CA |title=Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis |journal=Br Heart J |volume=37 |issue=8 |pages=840-52 |year=1975 |month=August |pmid=1191445 |pmc=482884 |doi= |url=http://www.pubmedcentral.nih.gov/pagerender.fcgi?artid=482884&pageindex=1#page}}</ref>
*Aspenia = '''B'''oys, '''B'''ad congenital malformations (transposition of great vessels, pulmonary atresia/stenosis, totally anomalous pulmonary return).
*Aspenia = '''B'''oys, '''B'''ad congenital malformations ([[transposition of the great vessels]], pulmonary atresia/stenosis, [[total anomalous pulmonary venous return]]).
**Interesting is that these are all right-to-left shunts.
**Interesting is that these are all right-to-left shunts.


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==Cor pulmonale==
==Cor pulmonale==
[[Pulmonary hypertension]] due to heart disease.
Heart disease due to pulmonary disease.
 
Causes - incomplete list:<ref>URL: [http://medsources.blogspot.ca/2011/09/cor-pulmonale.html http://medsources.blogspot.ca/2011/09/cor-pulmonale.html]. Accessed on: 2 May 2012.</ref>
*[[Primary pulmonary hypertension]].
*Chronic thromboembolic disease.
*[[Chronic obstructive pulmonary disease]].
*[[Interstitial lung disease]].
**[[Idiopathic pulmonary fibrosis]].
*[[Cystic fibrosis]].


===Pulmonary hypertension===
==Pulmonary hypertension==
{{Main|Pulmonary hypertension}}
{{Main|Pulmonary hypertension}}
*May cause ''[[cor pulmonale]]''.


Pressure - definition:<ref name=pmid19416617/>
Pressure - definition:<ref name=pmid19416617/>
*Mean pulm. arterial pressure >25 mmHg at rest.
*Mean pulmonary arterial pressure >25 mmHg at rest.


=See also=
=See also=
Michael
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