Congenital heart disease
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]
- 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.[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:
- 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.[11]
Clinical:
- Fixed S2 split.
Classification
Types:[12]
- 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.
- May be subdivided into:
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]
- Pulmonary arterial hypertension.
- Right-to-left shunt (that was initially left-to-right).
- Cyanosis.
Causes
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]
- Atrial septal defect (ASD) -- left-to-right shunt.
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]
- Aspenia = Boys, Bad 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.
For completeness... polyspenia associations:[23]
- Polyspenia = Girls, Good prognosis, Gastrointestinal situs inversus.
Cor pulmonale
Heart disease due to pulmonary disease.
Causes - incomplete list:[24]
- Primary pulmonary hypertension.
- Chronic thromboembolic disease.
- Chronic obstructive pulmonary disease.
- Interstitial lung disease.
- Cystic fibrosis.
Pulmonary hypertension
- May cause cor pulmonale.
Pressure - definition:[13]
- Mean pulmonary arterial pressure >25 mmHg at rest.
See also
References
- ↑ URL: http://www.childrenshospital.org/views/august05/cardiacsurgery.html. Accessed on: 14 January 2011.
- ↑ URL: http://www.childrenshospital.org/views/august05/cardiacsurgery.html. Accessed on: 14 January 2011.
- ↑ 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.
- ↑ 4.0 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.
- ↑ 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.
- ↑ 6.0 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/.
- ↑ 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.
- ↑ 8.0 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.
- ↑ 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.
- ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 287. ISBN 978-1416054542.
- ↑ Nyboe, C.; Fenger-Grøn, M.; Nielsen-Kudsk, JE.; Hjortdal, V. (Aug 2012). "Closure of secundum atrial septal defects in the adult and elderly patients.". Eur J Cardiothorac Surg. doi:10.1093/ejcts/ezs405. PMID 22893692.
- ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 287. ISBN 978-1416054542.
- ↑ 13.0 13.1 Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskr. Laeg. 171 (15): 1270-5. PMID 19416617.
- ↑ Daliento L, Rebellato L, Angelini A, et al. (2002). "Fatal outcome in Eisenmenger syndrome". Cardiovasc. Pathol. 11 (4): 221-8. PMID 12140128.
- ↑ URL: http://emedicine.medscape.com/article/899491-overview. Accessed on: 10 March 2011.
- ↑ Karamlou T, Gurofsky R, Al Sukhni E, et al. (March 2007). "Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection". Circulation 115 (12): 1591–8. doi:10.1161/CIRCULATIONAHA.106.635441. PMID 17353446.
- ↑ URL: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807798/. Accessed on: 15 March 2011.
- ↑ URL: http://www.chop.edu/service/cardiac-center/heart-conditions/ebsteins-anomaly.html. Accessed on: 27 November 2011.
- ↑ Giles, JJ.; Bannigan, JG. (2006). "Teratogenic and developmental effects of lithium.". Curr Pharm Des 12 (12): 1531-41. PMID 16611133.
- ↑ Moore. TDH. P.361
- ↑ 21.0 21.1 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.
- ↑ 23.0 23.1 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.
- ↑ URL: http://medsources.blogspot.ca/2011/09/cor-pulmonale.html. Accessed on: 2 May 2012.