Difference between revisions of "Congenital heart disease"
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==Paediatric cardiac surgery== | ==Paediatric cardiac surgery== | ||
====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). | ||
| Line 10: | Line 13: | ||
*[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)]. | ||
====Bidirectional Glenn Shunt==== | |||
*[[AKA]] bidirectional cavopulmonary shunt (BCPS). | |||
Indication: | |||
*LHHS - following Norwood procedure, reduce work done by the heart. | |||
Details: | |||
*The SVC is hooked-up to the right pulmonary artery. | |||
*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==== | ||
* | *De-oxygenated blood bypasses the heart en route to the lungs. | ||
There is the original Fontan procedure and two variants: | There is the original Fontan procedure and two variants: | ||
Revision as of 17:41, 11 January 2011
Congenital heart disease is a niche area of a niche area.
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:
Bidirectional Glenn Shunt
- AKA bidirectional cavopulmonary shunt (BCPS).
Indication:
- LHHS - following Norwood procedure, reduce work done by the heart.
Details:
- The SVC is hooked-up to the right pulmonary artery.
- 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.
There is the original Fontan procedure and two variants: - Atriopulmonary connection (the original). - 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.
- Fontan procedure often preceeded by a Norwood procedure.
Ventricular septal defect
- Common serious congenital heart defect.
- Most common congenital defect = bicuspid aortic valve.[1]
Tetralogy of Fallot (TOF)
General
- Most common cause of a blue baby.
Etiology
- Abnormal septation of the truncus arteriosus.
Definition
Features:[2]
- 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.
Atrial septal defect
- May be seen in adults.
Clinical
- Fixed S2 split.
Classification
Types:[3]
- 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.
Left hypoplastic heart syndrome
Defintion:[4]
- Physiologically inadequate left ventricle.
Key characteristic:[5]
- Atrial septal defect (ASD) -- left-to-right shunt.
Causality:
- Mitral stenosis.
- Left ventricular hypoplasia.
Associations:[5]
- Turner syndrome.
- Noonan syndrome - sometimes called "male version of Turner syndrome".
- Smith-Lemli-Opitz syndrome.
- Holt-Oram syndrome.[6]
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
Asplenia is associated with cardiac abnormalities:[7]
- Aspenia = Boys, Bad congenital malformations (transposition of great vessels, pulmonary atresia/stenosis, totally anomalous pulmonary return).
- Interesting is that these are all right-to-left shunts.
For completeness... polyspenia associations:[7]
- Polyspenia = Girls, Good prognosis, Gastrointestinal situs inversus.
Eisenmenger syndrome
- Pulmonary arterial hypertension with a
- Right-to-left shunt (that was initially left-to-right), and
- Cyanosis.
Note: Eisenmenger syndrome, by definition in some sources, is (only) due to a VSD.
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).[10]
Cor pulmonale
Pulmonary hypertension due to heart disease.
Pulmonary hypertension
Main article: Pulmonary hypertension
Pressure - definition:[8]
- Mean pulm. arterial pressure >25 mmHg at rest.
See also
References
- ↑ Siu SC, Silversides CK (June 2010). "Bicuspid aortic valve disease". J. Am. Coll. Cardiol. 55 (25): 2789–800. doi:10.1016/j.jacc.2009.12.068. PMID 20579534.
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 568. ISBN 0-7216-0187-1.
- ↑ http://en.wikipedia.org/wiki/File:ASD.png
- ↑ Moore. TDH. P.361
- ↑ 5.0 5.1 http://emedicine.medscape.com/article/890196-overview
- ↑ Ekure EN, Okoromah CN, Briggs E, Ajenifuja OA (September 2004). "Holt-Oram syndrome with hypoplastic left heart syndrome in an African child". Niger Postgrad Med J 11 (3): 190–2. PMID 15505648.
- ↑ 7.0 7.1 Rose V, Izukawa T, Moes CA (August 1975). "Syndromes of asplenia and polysplenia. A review of cardiac and non-cardiac malformations in 60 cases withspecial reference to diagnosis and prognosis". Br Heart J 37 (8): 840-52. PMC 482884. PMID 1191445. http://www.pubmedcentral.nih.gov/pagerender.fcgi?artid=482884&pageindex=1#page.
- ↑ 8.0 8.1 Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskr. Laeg. 171 (15): 1270-5. PMID 19416617.
- ↑ [1]
- ↑ Daliento L, Rebellato L, Angelini A, et al. (2002). "Fatal outcome in Eisenmenger syndrome". Cardiovasc. Pathol. 11 (4): 221-8. PMID 12140128.
