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Difference between revisions of "Congenital heart disease"
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→Atrial septal defect
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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= | |||
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== | |||
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== | |||
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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===General=== | ===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: | ||
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==Ebstein anomaly== | ==Ebstein anomaly== | ||
===General=== | ===General=== | ||
*Often right-to-left shunt - as it is seen with an atrial septal defect (ASD). | *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> | *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> | *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, | *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== | ||
[[ | 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== | |||
{{Main|Pulmonary hypertension}} | {{Main|Pulmonary hypertension}} | ||
*May cause ''[[cor pulmonale]]''. | |||
Pressure - definition:<ref name=pmid19416617/> | Pressure - definition:<ref name=pmid19416617/> | ||
*Mean | *Mean pulmonary arterial pressure >25 mmHg at rest. | ||
=See also= | =See also= | ||