Difference between revisions of "Congenital heart disease"

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'''Congential 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=
====Norwood procedure====
Indication:
*[[LHHS]] - following birth.
Details:
*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).
Images:
*[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)].
=====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====
*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:
* 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).
* Extracardiac total cavopulmonary connection.
Image:
*[http://www.radrounds.com/photo/fontan-procedure-in Fontan procedure (radrounds.com)].
Notes:
*Fontan procedure cannot be done in a newborn as the pulmonary vascular bed resistance is too high.<ref name=pmid5089489/>
=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==
*Most common congenital heart defect.
*Abbreviated ''VSD''.
===General===
*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>
*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>Robbins p.568.</ref>
Features:<ref name=Ref_PBoD568>{{Ref PBoD|568}}</ref>
*Right ventricular hypertrophy,
*Right ventricular hypertrophy.
*Right ventricular outflow tract obstruction,
*Right ventricular outflow tract obstruction.
*overriding aorta,
*Overriding aorta.
*VSD (ventricular septal defect).
*VSD (ventricular septal defect).


Notes:
Notes:
*Overriding aorta = aorta has a biventricular connection --takes blood from the right ventricle.
*Overriding aorta = aorta has a biventricular connection --takes blood from the right ventricle.
*Tight 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:
Line 49: Line 193:
*Left ventricular hypoplasia.
*Left ventricular hypoplasia.


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.


Line 68: Line 208:
*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==
Pressure - definition:<ref name=pmid19416617>
{{Main|Pulmonary hypertension}}
*Mean pulm. arterial pressure >25 mmHg at rest.
*May cause ''[[cor pulmonale]]''.


Pressure - definition:<ref name=pmid19416617/>
*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]]

Latest revision as of 19:31, 8 January 2013

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

Paediatric cardiac surgery

Norwood procedure

Indication:

  • LHHS - following birth.

Details:

  • 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).

Images:

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.[1]

Key elements:[2]

  1. Stent the ductus arteriosus (interventional cardiology/interventional radiology).
  2. Band the pulmonary artery - to reduce the pulmonary pressure (cardiac surgery).

Notes:

  • It appears to be associated with more GI complications.[3]

Bidirectional Glenn Shunt

  • AKA bidirectional cavopulmonary shunt (BCPS), AKA hemi-Fontan procedure, AKA bidirectional cavopulmonary anastomosis (BDCPA).[4]

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.[5]
  • The conduit connecting the ventricle and lungs (Blalock-Taussig shunt or Sano shunt) is disconnected.

Image:

Fontan procedure

  • De-oxygenated blood bypasses the heart en route to the lungs.
  • Usually done at age 2-4 years.[4]

There is the original Fontan procedure and two variants:

  • Atriopulmonary connection (the original) - right atrial appendage connected to (proximal) right pulmonary artery.[6]
  • Intracardiac total cavopulmonary connection (lateral tunnel).
  • Extracardiac total cavopulmonary connection.

Image:

Notes:

  • Fontan procedure cannot be done in a newborn as the pulmonary vascular bed resistance is too high.[6]

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:[7]

  • 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:[8]

  • 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

  • Abbreviated VSD.

General

  • Common serious congenital heart defect.
    • Most common congenital defect = bicuspid aortic valve.[9]
  • Often associated with other cardiac abnormalities, e.g. Tetralogy of Fallot.
    • 20-30% of VSDs are isolated.[10]

Subtypes:

  1. Membranous VSD ~ 90%.
    • Near aortic valve.
  2. 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.[11]

Clinical:

  • Fixed S2 split.

Classification

Types:[12]

  1. Ostium secundum
    • Between SVC and IVC.
  2. Ostium primum
    • Between SVC and IVC. Closer to RV.
  3. 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:[13]

  1. Pulmonary arterial hypertension.
  2. Right-to-left shunt (that was initially left-to-right).
  3. 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:[14]

  • Often characterized by fibrinoid necrosis of small pulmonary arterial vessels (arterioles & small arteries).

Right-to-left

Tetralogy of Fallot

  • Abbreviated TOF.

General

  • Most common cause of a blue baby.

Etiology

  • Abnormal septation of the truncus arteriosus.

Definition

Features:[8]

  • 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.

Transposition of the great vessels

  • 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.

Total anomalous pulmonary venous return

  • 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.[15][16]
  • Treatment: surgical repair (Coles procedure).[17] (???)

Other

Ebstein anomaly

General

  • Often right-to-left shunt - as it is seen with an atrial septal defect (ASD).
  • Tricuspid incompetence -> right atrial enlargement.[18]
  • Weak association with maternal lithium use.[19]

Left hypoplastic heart syndrome

  • Abbreviated LHHS.

General

Defintion:[20]

  • Physiologically inadequate left ventricle.

Key characteristic:[21]

Causality:

  • Mitral stenosis.
  • Left ventricular hypoplasia.

Associations:[21]

  • Turner syndrome.
  • Noonan syndrome - sometimes called "male version of Turner syndrome".
  • Smith-Lemli-Opitz syndrome.
  • Holt-Oram syndrome.[22]

Splenic abnormalities

Asplenia is associated with cardiac abnormalities:[23]

For completeness... polyspenia associations:[23]

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

Cor pulmonale

Heart disease due to pulmonary disease.

Causes - incomplete list:[24]

Pulmonary hypertension

Pressure - definition:[13]

  • Mean pulmonary arterial pressure >25 mmHg at rest.

See also

References

  1. URL: http://www.childrenshospital.org/views/august05/cardiacsurgery.html. Accessed on: 14 January 2011.
  2. URL: http://www.childrenshospital.org/views/august05/cardiacsurgery.html. Accessed on: 14 January 2011.
  3. Weiss SL, Gossett JG, Kaushal S, Wang D, Backer CL, Wald EL (December 2010). "Comparison of Gastrointestinal Morbidity After Norwood and Hybrid Palliation for Complex Heart Defects". Pediatr Cardiol. doi:10.1007/s00246-010-9864-9. PMID 21188371.
  4. 4.0 4.1 Alsoufi B, Bennetts J, Verma S, Caldarone CA (January 2007). "New developments in the treatment of hypoplastic left heart syndrome". Pediatrics 119 (1): 109–17. doi:10.1542/peds.2006-1592. PMID 17200277. http://pediatrics.aappublications.org/cgi/content/full/119/1/109.
  5. GLENN WW (July 1958). "Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery; report of clinical application". N. Engl. J. Med. 259 (3): 117–20. doi:10.1056/NEJM195807172590304. PMID 13566431.
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