Pulmonary atresia pathophysiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
The [[pulmonary valve]] is located on the right side of the heart between the [[right ventricle]] and [[pulmonary artery]]. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref> In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close like one way doors. As these flaps open and close they force blood to flow forward into the [[pulmonary artery]] and backward into the [[right ventricle]] then forward again to the lungs where the blood becomes oxygenated. With the disease pulmonary atresia, the flap-like openings are completely covered by a layer of tissue, thus preventing the ability of blood flow to the lungs to become oxygenated. The body requires oxygenated blood for survival.<ref name="pmid17519398">{{cite journal| author=Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E et al.| title=Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. | journal=Circulation | year= 2007 | volume= 115 | issue= 23 | pages= 3015-38 | pmid=17519398 | doi=10.1161/CIRCULATIONAHA.106.183056 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17519398 }} </ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref> Pulmonary atresia is not threatening to a developing fetus however, because the mother's placenta provides the needed oxygen since the baby's lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with Pulmonary atresia there is no opening on the [[pulmonary valve]] for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref> .<ref name="pmid12878741">{{cite journal| author=Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F et al.| title=Acquired von Willebrand syndrome in aortic stenosis. | journal=N Engl J Med | year= 2003 | volume= 349 | issue= 4 | pages= 343-9 | pmid=12878741 | doi=10.1056/NEJMoa022831 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12878741 }} </ref><ref name="pmid26269004">{{cite journal| author=Tamura T, Horiuchi H, Imai M, Tada T, Shiomi H, Kuroda M et al.| title=Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. | journal=J Atheroscler Thromb | year= 2015 | volume= 22 | issue= 11 | pages= 1115-23 | pmid=26269004 | doi=10.5551/jat.30809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26269004 }} </ref> <ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172 }} </ref> | The [[pulmonary valve]] is located on the right side of the heart between the [[right ventricle]] and [[pulmonary artery]]. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref> In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close like one way doors. As these flaps open and close they force blood to flow forward into the [[pulmonary artery]] and backward into the [[right ventricle]] then forward again to the lungs where the blood becomes oxygenated. With the disease pulmonary atresia, the flap-like openings are completely covered by a layer of tissue, thus preventing the ability of blood flow to the lungs to become oxygenated. The body requires oxygenated blood for survival.<ref name="pmid17519398">{{cite journal| author=Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E et al.| title=Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. | journal=Circulation | year= 2007 | volume= 115 | issue= 23 | pages= 3015-38 | pmid=17519398 | doi=10.1161/CIRCULATIONAHA.106.183056 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17519398 }} </ref> <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref> Pulmonary atresia is not threatening to a developing fetus however, because the mother's placenta provides the needed oxygen since the baby's lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with Pulmonary atresia there is no opening on the [[pulmonary valve]] for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth. <ref name="pmid10">{{cite journal |vauthors=Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT |title=Digitoxin metabolism by rat liver microsomes |journal=Biochem. Pharmacol. |volume=24 |issue=17 |pages=1639–41 |date=September 1975 |pmid=10 |pmc=5922622 |doi=10.1136/bmj.1.6001.93-a |url=}}</ref> .<ref name="pmid12878741">{{cite journal| author=Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F et al.| title=Acquired von Willebrand syndrome in aortic stenosis. | journal=N Engl J Med | year= 2003 | volume= 349 | issue= 4 | pages= 343-9 | pmid=12878741 | doi=10.1056/NEJMoa022831 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12878741 }} </ref><ref name="pmid26269004">{{cite journal| author=Tamura T, Horiuchi H, Imai M, Tada T, Shiomi H, Kuroda M et al.| title=Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index. | journal=J Atheroscler Thromb | year= 2015 | volume= 22 | issue= 11 | pages= 1115-23 | pmid=26269004 | doi=10.5551/jat.30809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26269004 }} </ref> <ref name="pmid18820172">{{cite journal| author=Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD et al.| title=2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. | journal=Circulation | year= 2008 | volume= 118 | issue= 15 | pages= e523-661 | pmid=18820172 | doi=10.1161/CIRCULATIONAHA.108.190748 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18820172 }} </ref> <ref name="pmid11870246">{{cite journal| author=Carabello BA| title=Clinical practice. Aortic stenosis. | journal=N Engl J Med | year= 2002 | volume= 346 | issue= 9 | pages= 677-82 | pmid=11870246 | doi=10.1056/NEJMcp010846 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11870246 }} </ref> | ||
[[Image:Pulmonary-artery-atresia.jpg|400px|Only an aorta can be seen originating from this pathology specimen. No pulmonary artery is present.]] | [[Image:Pulmonary-artery-atresia.jpg|400px|Only an aorta can be seen originating from this pathology specimen. No pulmonary artery is present.]] | ||
Revision as of 14:56, 26 February 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [2], Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [4]
Overview
In a normal heart, the opening in the pulmonary valve has three flaps to open and close. In a patient with pulmonary atresia, the flap-like openings are covered by a layer of tissue. This significantly impacts the ability for the heart to shunt blood to the lungs for oxygenation.
Pathophysiology
The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. [1] In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close like one way doors. As these flaps open and close they force blood to flow forward into the pulmonary artery and backward into the right ventricle then forward again to the lungs where the blood becomes oxygenated. With the disease pulmonary atresia, the flap-like openings are completely covered by a layer of tissue, thus preventing the ability of blood flow to the lungs to become oxygenated. The body requires oxygenated blood for survival.[2] [1] Pulmonary atresia is not threatening to a developing fetus however, because the mother's placenta provides the needed oxygen since the baby's lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with Pulmonary atresia there is no opening on the pulmonary valve for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth. [1] .[3][4] [5] [6]
References
- ↑ 1.0 1.1 1.2 Schmoldt A, Benthe HF, Haberland G, Raffle A, Gray J, MacDonald HR, Ehrhart IC, Parker PE, Weidner WJ, Dabney JM, Scott JB, Haddy FJ, Gatzy JT (September 1975). "Digitoxin metabolism by rat liver microsomes". Biochem. Pharmacol. 24 (17): 1639–41. doi:10.1136/bmj.1.6001.93-a. PMC 5922622. PMID 10.
- ↑ Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E; et al. (2007). "Genetic basis for congenital heart defects: current knowledge: a scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics". Circulation. 115 (23): 3015–38. doi:10.1161/CIRCULATIONAHA.106.183056. PMID 17519398.
- ↑ Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F; et al. (2003). "Acquired von Willebrand syndrome in aortic stenosis". N Engl J Med. 349 (4): 343–9. doi:10.1056/NEJMoa022831. PMID 12878741.
- ↑ Tamura T, Horiuchi H, Imai M, Tada T, Shiomi H, Kuroda M; et al. (2015). "Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index". J Atheroscler Thromb. 22 (11): 1115–23. doi:10.5551/jat.30809. PMID 26269004.
- ↑ Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD; et al. (2008). "2008 Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". Circulation. 118 (15): e523–661. doi:10.1161/CIRCULATIONAHA.108.190748. PMID 18820172.
- ↑ Carabello BA (2002). "Clinical practice. Aortic stenosis". N Engl J Med. 346 (9): 677–82. doi:10.1056/NEJMcp010846. PMID 11870246.