Congenital heart disease

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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.

Clinical:

  • Fixed S2 split.

Classification

Types:[11]

  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:[12]

  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:[13]

  • 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.[14][15]
  • Treatment: surgical repair (Coles procedure).[16] (???)

Other

Ebstein anomaly

General

  • Often right-to-left shunt - as it is seen with an atrial septal defect (ASD).
  • Tricuspid incompetence -> right atrial enlargement.[17]
  • Weak association with maternal lithium use.[18]

Left hypoplastic heart syndrome

Defintion:[19]

  • Physiologically inadequate left ventricle.

Key characteristic:[20]

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

Causality:

  • Mitral stenosis.
  • Left ventricular hypoplasia.

Associations:[20]

  • Turner syndrome.
  • Noonan syndrome - sometimes called "male version of Turner syndrome".
  • Smith-Lemli-Opitz syndrome.
  • Holt-Oram syndrome.[21]


Splenic abnormalities

Asplenia is associated with cardiac abnormalities:[22]

  • 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:[22]

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

Cor pulmonale

Pulmonary hypertension due to heart disease.

Pulmonary hypertension

Main article: Pulmonary hypertension

Pressure - definition:[12]

  • 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. ↑ 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.
  11. ↑ 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.
  12. ↑ 12.0 12.1 Jensen AS, Iversen K, Vejlstrup NG, Hansen PB, Sondergaard L (April 2009). "[Eisenmenger syndrome]" (in Danish). Ugeskr. Laeg. 171 (15): 1270-5. PMID 19416617.
  13. ↑ Daliento L, Rebellato L, Angelini A, et al. (2002). "Fatal outcome in Eisenmenger syndrome". Cardiovasc. Pathol. 11 (4): 221-8. PMID 12140128.
  14. ↑ URL: http://emedicine.medscape.com/article/899491-overview. Accessed on: 10 March 2011.
  15. ↑ 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.
  16. ↑ URL: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2807798/. Accessed on: 15 March 2011.
  17. ↑ URL: http://www.chop.edu/service/cardiac-center/heart-conditions/ebsteins-anomaly.html. Accessed on: 27 November 2011.
  18. ↑ Giles, JJ.; Bannigan, JG. (2006). "Teratogenic and developmental effects of lithium.". Curr Pharm Des 12 (12): 1531-41. PMID 16611133.
  19. ↑ Moore. TDH. P.361
  20. ↑ 20.0 20.1 http://emedicine.medscape.com/article/890196-overview
  21. ↑ 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.
  22. ↑ 22.0 22.1 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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