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Congenital heart disease (view source)

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'''Congenital [[heart]] disease''' is a niche area of a niche area.
'''Congenital [[heart]] disease''', abbreviated '''CHD''', is a niche area of [[cardiac pathology]], which is a subset of [[cardiovascular pathology]].


==Paediatric cardiac surgery==
=Paediatric cardiac surgery=
===Operations for LHHS===
====Norwood procedure====
====Norwood procedure====
Indication:
*[[LHHS]] - following birth.
Details:
*Pulmonary artery is attached to the aorta.
*Pulmonary artery is attached to the aorta.
*The lungs are perfused by a Blalock-Taussig shunt (subclavian artery -> pulmonary artery) or a Sano shunt (single ventricle -> synthetic conduit -> pulmonary artery).
*The lungs are perfused by a Blalock-Taussig shunt (subclavian artery -> pulmonary artery) or a Sano shunt (single ventricle -> synthetic conduit -> pulmonary artery).
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*[http://hlhs-awareness-uk.com/norwood.aspx LHHS (hlhs-awareness-uk.com)].
*[http://hlhs-awareness-uk.com/norwood.aspx LHHS (hlhs-awareness-uk.com)].
*[http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp Single ventricle defects (heart.org)].
*[http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp Single ventricle defects (heart.org)].
=====Hybrid procedure=====
*Is an alternative to the ''Norwood procedure''.
*It is call ''hybrid procedure'' as it is a mix of a surgery ''and'' a minimally invasive interventional procedure.<ref>URL: [http://www.childrenshospital.org/views/august05/cardiacsurgery.html http://www.childrenshospital.org/views/august05/cardiacsurgery.html]. Accessed on: 14 January 2011.</ref>
Key elements:<ref>URL: [http://www.childrenshospital.org/views/august05/cardiacsurgery.html http://www.childrenshospital.org/views/august05/cardiacsurgery.html]. Accessed on: 14 January 2011.</ref>
#Stent the ductus arteriosus (interventional cardiology/interventional radiology).
#Band the pulmonary artery - to reduce the pulmonary pressure (cardiac surgery).
Notes:
*It appears to be associated with more GI complications.<ref name=pmid21188371>{{cite journal |author=Weiss SL, Gossett JG, Kaushal S, Wang D, Backer CL, Wald EL |title=Comparison of Gastrointestinal Morbidity After Norwood and Hybrid Palliation for Complex Heart Defects |journal=Pediatr Cardiol |volume= |issue= |pages= |year=2010 |month=December |pmid=21188371 |doi=10.1007/s00246-010-9864-9 |url=}}</ref>
====Bidirectional Glenn Shunt====
*[[AKA]] bidirectional cavopulmonary shunt (BCPS), [[AKA]] hemi-Fontan procedure, [[AKA]] bidirectional cavopulmonary anastomosis (BDCPA).<ref name=pmid17200277>{{cite journal |author=Alsoufi B, Bennetts J, Verma S, Caldarone CA |title=New developments in the treatment of hypoplastic left heart syndrome |journal=Pediatrics |volume=119 |issue=1 |pages=109–17 |year=2007 |month=January |pmid=17200277 |doi=10.1542/peds.2006-1592 |url=http://pediatrics.aappublications.org/cgi/content/full/119/1/109}}</ref>
Indication:
*[[LHHS]] - following Norwood procedure, reduce work done by the heart; usu. done at age 4 to 6 months.
Details:
*The superior vena cava (SVC) is hooked-up to the right pulmonary artery.<ref name=pmid13566431>{{cite journal |author=GLENN WW |title=Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery; report of clinical application |journal=N. Engl. J. Med. |volume=259 |issue=3 |pages=117–20 |year=1958 |month=July |pmid=13566431 |doi=10.1056/NEJM195807172590304 |url=}}</ref>
*The conduit connecting the ventricle and lungs (Blalock-Taussig shunt ''or'' Sano shunt) is disconnected.
Image:
*[http://www.chitexas.org/hlh-surgical.asp Bidirectional Glenn Shunt (chitexas.org)].


====Fontan procedure====
====Fontan procedure====
*Blood from right atrium goes directly to the lungs.
*De-oxygenated blood bypasses the heart en route to the lungs.
*Usually done at age 2-4 years.<ref name=pmid17200277/>


There is the original Fontan procedure and two variants:
There is the original Fontan procedure and two variants:
- Atriopulmonary connection (the original).
* Atriopulmonary connection (the original) - right atrial appendage connected to (proximal) right pulmonary artery.<ref name=pmid5089489>{{cite journal |author=Fontan F, Baudet E |title=Surgical repair of tricuspid atresia |journal=Thorax |volume=26 |issue=3 |pages=240–8 |year=1971 |month=May |pmid=5089489 |pmc=1019078 |doi= |url=}}</ref>
- Intracardiac total cavopulmonary connection (lateral tunnel).
* Intracardiac total cavopulmonary connection (lateral tunnel).
- Extracardiac total cavopulmonary connection.
* Extracardiac total cavopulmonary connection.


Image:
Image:
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Notes:
Notes:
*Fontan procedure cannot be done in a newborn as the pulmonary vascular bed resistance is too high.
*Fontan procedure cannot be done in a newborn as the pulmonary vascular bed resistance is too high.<ref name=pmid5089489/>
*Fontan procedure often preceeded by a Norwood procedure.
 
=Shunts - overview=
Most shunts are a consequence of congenital heart disease.  They can be grouped into ''left-to-right'' and ''right-to-left''.
 
==Left-to-right==
Mnemonic ''the Ds'':<ref name=Ref_PBoD566>{{Ref PBoD|566}}</ref>
*ASD = atrial septal defect.
*VSD = ventricular septal defect.
*AVSD = atrioventricular defect.
*PDA = patent ductus arteriosus.
 
Note: The word ''Left'' has four letters and there are four L->R shunts.
==Right-to-left==
Mnemonic ''5 Ts'':<ref name=Ref_PBoD568>{{Ref PBoD|568}}</ref>
*Tetralogy of Fallot (TOF).
*Transposition of great arteries.
*Truncus arteriosus.
*Tricuspid valve atresia.
*Total anomalous pulmonary venous return.
 
Clinical:
*These babies are blue, as the blood partially bypasses the lung and/or oxygenated blood is mixed with de-oxygenated blood.
*TOF is the classic cause of "blue babies".


=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>


==Tetralogy of Fallot (TOF)==
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==
*Abbreviated ''ASD''.
===General===
*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:
*Fixed S2 split.
 
===Classification===
Types:<ref name=Ref_PCPBoD8|287>{{Ref PCPBoD8|287}}</ref>
#Ostium secundum
#*Between SVC and IVC.
#Ostium primum
#*Between SVC and IVC. Closer to RV.
#Sinus venosus.
#*May be subdivided into:
#**Upper sinus venosus defect - at SVC.
#**Lower sinus venosus defect - at IVC.
 
Notes:
*The ostium secundum are the most common and usually found in isolation, i.e. there are not other associated abnormalities.
 
==Eisenmenger syndrome==
===General===
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]].
#Right-to-left shunt (that was initially left-to-right).
#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==
*Abbreviated ''TOF''.
===General===
===General===
*Most common cause of a ''blue baby''.
*Most common cause of a ''blue baby''.


===Etiology===
====Etiology====
*Abnormal septation of the truncus arteriosus.
*Abnormal septation of the truncus arteriosus.


===Definition===
====Definition====
Features:<ref name=Ref_PBoD568>{{Ref PBoD|568}}</ref>
Features:<ref name=Ref_PBoD568>{{Ref PBoD|568}}</ref>
*Right ventricular hypertrophy.  
*Right ventricular hypertrophy.  
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*Right ventricular outflow tract obstruction is usually subpulmonic stenosis.
*Right ventricular outflow tract obstruction is usually subpulmonic stenosis.


==Atrial septal defect==
==Transposition of the great vessels==
*May be seen in adults.
*[[AKA]] ''transposition of the great arteries''.
===General===
*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.


===Clinical===
==Total anomalous pulmonary venous return==
*Fixed S2 split.
*[[AKA]] ''total anomalous pulmonary venous drainage'' (TAPVD).
*Abbreviated ''TAPVR''.
===General===
*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>
*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> (???)


===Classification===
=Other=
Types:<ref>[http://en.wikipedia.org/wiki/File:ASD.png http://en.wikipedia.org/wiki/File:ASD.png]</ref>
==Ebstein anomaly==
*Ostium secundum (most common) - between SVC and IVC.
===General===
*Ostium primum - between SVC and IVC closer to RV.
*Often right-to-left shunt - as it is seen with an [[atrial septal defect]] (ASD).
*Upper sinus venosus defect - at SVC.
*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>
*Lower sinus venosus defect - at IVC.
*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>
*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==
*Abbreviated ''LHHS''.


==Left hypoplastic heart syndrome==
===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>
==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==
==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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*Polyspenia = '''G'''irls, '''G'''ood prognosis, '''G'''astrointestinal situs inversus.
*Polyspenia = '''G'''irls, '''G'''ood prognosis, '''G'''astrointestinal situs inversus.


==Cor pulmonale==
Heart disease due to pulmonary disease.


==Eisenmenger syndrome==
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>
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><ref>[http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/nine/000952281.htm]</ref>
*[[Primary pulmonary hypertension]].
*Pulmonary arterial hypertension with a
*Chronic thromboembolic disease.
*Right-to-left shunt (that was initially left-to-right), and
*[[Chronic obstructive pulmonary disease]].
*Cyanosis.
*[[Interstitial lung disease]].
 
**[[Idiopathic pulmonary fibrosis]].
Note: Eisenmenger syndrome, by definition in some sources, is (only) due to a VSD.
*[[Cystic fibrosis]].
 
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).<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>
 
==Cor pulmonale==
Pulmonary hypertension due to heart disease.


===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=
*[[Cardiovascular pathology]].
*[[Cardiovascular pathology]].
*[[Heart]].
*[[Pulmonary hypertension]].


==References==
=References=
{{reflist|2}}
{{reflist|2}}


[[Category:Cardiovascular pathology]]
[[Category:Cardiovascular pathology]]
Michael
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