Difference between revisions of "Congenital heart disease"

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'''Congenital [[heart]] disease''', abbreviated '''CHD''', 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=
====Norwood procedure====
====Norwood procedure====
Indication:  
Indication:  
Line 76: Line 76:
*TOF is the classic cause of "blue babies".
*TOF is the classic cause of "blue babies".


=Left-to-right=
==Ventricular septal defect==
==Ventricular septal defect==
*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>


==Atrial septal defect==
*May be seen in adults.
===Clinical===
*Fixed S2 split.
===Classification===
Types:<ref>[http://en.wikipedia.org/wiki/File:ASD.png http://en.wikipedia.org/wiki/File:ASD.png]</ref>
*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.
==Eisenmenger syndrome==
===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>
*[[Pulmonary arterial hypertension]] with a
*Right-to-left shunt (that was initially left-to-right), and
*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 (TOF)==
==Tetralogy of Fallot (TOF)==
===General===
===General===
Line 98: Line 135:
*Right ventricular outflow tract obstruction is usually subpulmonic stenosis.
*Right ventricular outflow tract obstruction is usually subpulmonic stenosis.


==Atrial septal defect==
==Transposition of great vessels==
*May be seen in adults.
*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.
===Clinical===
**When the ''ductus arteriosus'' closes they are in trouble.
*Fixed S2 split.
 
===Classification===
Types:<ref>[http://en.wikipedia.org/wiki/File:ASD.png http://en.wikipedia.org/wiki/File:ASD.png]</ref>
*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.


=Other=
==Left hypoplastic heart syndrome==
==Left hypoplastic heart syndrome==
Defintion:<ref>Moore. TDH. P.361</ref>
Defintion:<ref>Moore. TDH. P.361</ref>
Line 131: Line 158:
*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==
Line 143: Line 166:
For completeness... polyspenia associations:<ref name=pmid1191445/>
For completeness... polyspenia associations:<ref name=pmid1191445/>
*Polyspenia = '''G'''irls, '''G'''ood prognosis, '''G'''astrointestinal situs inversus.
*Polyspenia = '''G'''irls, '''G'''ood prognosis, '''G'''astrointestinal situs inversus.
==Eisenmenger syndrome==
===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>
*[[Pulmonary arterial hypertension]] with a
*Right-to-left shunt (that was initially left-to-right), and
*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).


==Cor pulmonale==
==Cor pulmonale==
Pulmonary hypertension due to heart disease.
[[Pulmonary hypertension]] due to heart disease.


===Pulmonary hypertension===
===Pulmonary hypertension===
Line 172: Line 176:
*Mean pulm. arterial pressure >25 mmHg at rest.
*Mean pulm. arterial pressure >25 mmHg at rest.


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


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


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

Revision as of 17:52, 10 March 2011

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

  • Common serious congenital heart defect.
    • Most common congenital defect = bicuspid aortic valve.[9]

Atrial septal defect

  • May be seen in adults.

Clinical

  • Fixed S2 split.

Classification

Types:[10]

  • 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.

Eisenmenger syndrome

General

Definition:[11]

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:[12]

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


Right-to-left

Tetralogy of Fallot (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 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.

Other

Left hypoplastic heart syndrome

Defintion:[13]

  • Physiologically inadequate left ventricle.

Key characteristic:[14]

  • Atrial septal defect (ASD) -- left-to-right shunt.

Causality:

  • Mitral stenosis.
  • Left ventricular hypoplasia.

Associations:[14]

  • Turner syndrome.
  • Noonan syndrome - sometimes called "male version of Turner syndrome".
  • Smith-Lemli-Opitz syndrome.
  • Holt-Oram syndrome.[15]


Splenic abnormalities

Asplenia is associated with cardiac abnormalities:[16]

  • 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:[16]

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

Cor pulmonale

Pulmonary hypertension due to heart disease.

Pulmonary hypertension

Main article: Pulmonary hypertension

Pressure - definition:[11]

  • Mean pulm. 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.
  6. ↑ 6.0 6.1 Fontan F, Baudet E (May 1971). "Surgical repair of tricuspid atresia". Thorax 26 (3): 240–8. PMC 1019078. PMID 5089489. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1019078/.
  7. ↑ 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. 566. ISBN 0-7216-0187-1.
  8. ↑ 8.0 8.1 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.
  9. ↑ 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.
  10. ↑ http://en.wikipedia.org/wiki/File:ASD.png
  11. ↑ 11.0 11.1 Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskr. Laeg. 171 (15): 1270-5. PMID 19416617.
  12. ↑ Daliento L, Rebellato L, Angelini A, et al. (2002). "Fatal outcome in Eisenmenger syndrome". Cardiovasc. Pathol. 11 (4): 221-8. PMID 12140128.
  13. ↑ Moore. TDH. P.361
  14. ↑ 14.0 14.1 http://emedicine.medscape.com/article/890196-overview
  15. ↑ 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.
  16. ↑ 16.0 16.1 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.
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