Right ventricular outflow tract obstruction pathophysiology: Difference between revisions

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==Pathophysiology==
==Pathophysiology==


Almost all cases of pulmonic stenosis are due to disease in the heart secondary to [[rheumatic fever]] and the consequent [[rheumatic heart disease]] (a condition that may develop after [[strep throat]] or [[scarlet fever]]). Around 90% of cases of rheumatic heart disease are associated with pulmonic stenosis. Pulmonic stenosis is a progressive valvular disease which progression depends mainly on the degree of the narrowing of the pulmonic valve as well as on the maladaptive ventricular wall response. <ref name="pmid3750250">{{cite journal| author=Scarlat A, Bodner G, Liron M| title=Massive haemoptysis as the presenting symptom in mitral stenosis. | journal=Thorax | year= 1986 | volume= 41 | issue= 5 | pages= 413-4 | pmid=3750250 | doi= | pmc=1020641 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3750250  }} </ref> <ref name="pmid7720297">{{cite journal| author=Waller BF, Howard J, Fess S| title=Pathology of tricuspid valve stenosis and pure tricuspid regurgitation--Part I. | journal=Clin Cardiol | year= 1995 | volume= 18 | issue= 2 | pages= 97-102 | pmid=7720297 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7720297  }} </ref><ref name="pmid3696315">{{cite journal| author=Maher ER, Pazianas M, Curtis JR| title=Calcific aortic stenosis: a complication of chronic uraemia. | journal=Nephron | year= 1987 | volume= 47 | issue= 2 | pages= 119-22 | pmid=3696315 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3696315  }} </ref> <ref name="pmid14657067">{{cite journal| author=Hull MC, Morris CG, Pepine CJ, Mendenhall NP| title=Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy. | journal=JAMA | year= 2003 | volume= 290 | issue= 21 | pages= 2831-7 | pmid=14657067 | doi=10.1001/jama.290.21.2831 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14657067  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </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="pmid14657067">{{cite journal| author=Hull MC, Morris CG, Pepine CJ, Mendenhall NP| title=Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy. | journal=JAMA | year= 2003 | volume= 290 | issue= 21 | pages= 2831-7 | pmid=14657067 | doi=10.1001/jama.290.21.2831 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14657067  }} </ref> <ref name="pmid12633546">{{cite journal |author=Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J |title=The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis |journal=[[European Heart Journal]] |volume=24 |issue=5 |pages=442–63 |year=2003 |month=March |pmid=12633546 |doi= |url=http://eurheartj.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12633546 |accessdate=2012-04-11}}</ref> <ref name="pmid9060903">{{cite journal |author=Stewart BF, Siscovick D, Lind BK, Gardin JM, Gottdiener JS, Smith VE, Kitzman DW, Otto CM |title=Clinical factors associated with calcific aortic valve disease. Cardiovascular Health Study |journal=[[Journal of the American College of Cardiology]] |volume=29 |issue=3 |pages=630–4 |year=1997 |month=March |pmid=9060903 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0735109796005633 |accessdate=2012-04-11}}</ref> <ref name="pmid27866029">{{cite journal| author=Galli D, Manuguerra R, Monaco R, Manotti L, Goldoni M, Becchi G et al.| title=Understanding the structural features of symptomatic calcific aortic valve stenosis: A broad-spectrum clinicopathologic study in 236 consecutive surgical cases. | journal=Int J Cardiol | year= 2016 | volume= 228 | issue=  | pages= 364-374 | pmid=27866029 | doi=10.1016/j.ijcard.2016.11.180 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27866029  }} </ref> <ref name="pmid27810479">{{cite journal| author=Joseph J, Naqvi SY, Giri J, Goldberg S| title=Aortic stenosis: pathophysiology, diagnosis and therapy. | journal=Am J Med | year= 2016 | volume=  | issue=  | pages=  | pmid=27810479 | doi=10.1016/j.amjmed.2016.10.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27810479  }} </ref> <ref name="pmid25140960">{{cite journal| author=Otto CM, Prendergast B| title=Aortic-valve stenosis--from patients at risk to severe valve obstruction. | journal=N Engl J Med | year= 2014 | volume= 371 | issue= 8 | pages= 744-56 | pmid=25140960 | doi=10.1056/NEJMra1313875 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25140960  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref><ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref> <ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191  }} </ref> <ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191  }} </ref> <ref name="pmid15470217">{{cite journal| author=Enriquez-Sarano M, Tajik AJ| title=Clinical practice. Aortic regurgitation. | journal=N Engl J Med | year= 2004 | volume= 351 | issue= 15 | pages= 1539-46 | pmid=15470217 | doi=10.1056/NEJMcp030912 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15470217  }} </ref>
Almost all cases of pulmonic stenosis are due to disease in the heart secondary to [[rheumatic fever]] and the consequent [[rheumatic heart disease]] (a condition that may develop after [[strep throat]] or [[scarlet fever]]). Around 90% of cases of rheumatic heart disease are associated with pulmonic stenosis. Pulmonic stenosis is a progressive valvular disease which progression depends mainly on the degree of the narrowing of the pulmonic valve as well as on the maladaptive ventricular wall response. <ref name="pmid3750250">{{cite journal| author=Scarlat A, Bodner G, Liron M| title=Massive haemoptysis as the presenting symptom in mitral stenosis. | journal=Thorax | year= 1986 | volume= 41 | issue= 5 | pages= 413-4 | pmid=3750250 | doi= | pmc=1020641 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3750250  }} </ref> <ref name="pmid7720297">{{cite journal| author=Waller BF, Howard J, Fess S| title=Pathology of tricuspid valve stenosis and pure tricuspid regurgitation--Part I. | journal=Clin Cardiol | year= 1995 | volume= 18 | issue= 2 | pages= 97-102 | pmid=7720297 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7720297  }} </ref><ref name="pmid3696315">{{cite journal| author=Maher ER, Pazianas M, Curtis JR| title=Calcific aortic stenosis: a complication of chronic uraemia. | journal=Nephron | year= 1987 | volume= 47 | issue= 2 | pages= 119-22 | pmid=3696315 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3696315  }} </ref> <ref name="pmid14657067">{{cite journal| author=Hull MC, Morris CG, Pepine CJ, Mendenhall NP| title=Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy. | journal=JAMA | year= 2003 | volume= 290 | issue= 21 | pages= 2831-7 | pmid=14657067 | doi=10.1001/jama.290.21.2831 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14657067  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </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="pmid14657067">{{cite journal| author=Hull MC, Morris CG, Pepine CJ, Mendenhall NP| title=Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy. | journal=JAMA | year= 2003 | volume= 290 | issue= 21 | pages= 2831-7 | pmid=14657067 | doi=10.1001/jama.290.21.2831 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14657067  }} </ref> <ref name="pmid12633546">{{cite journal |author=Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J |title=The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis |journal=[[European Heart Journal]] |volume=24 |issue=5 |pages=442–63 |year=2003 |month=March |pmid=12633546 |doi= |url=http://eurheartj.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=12633546 |accessdate=2012-04-11}}</ref> <ref name="pmid9060903">{{cite journal |author=Stewart BF, Siscovick D, Lind BK, Gardin JM, Gottdiener JS, Smith VE, Kitzman DW, Otto CM |title=Clinical factors associated with calcific aortic valve disease. Cardiovascular Health Study |journal=[[Journal of the American College of Cardiology]] |volume=29 |issue=3 |pages=630–4 |year=1997 |month=March |pmid=9060903 |doi= |url=http://linkinghub.elsevier.com/retrieve/pii/S0735109796005633 |accessdate=2012-04-11}}</ref> <ref name="pmid27866029">{{cite journal| author=Galli D, Manuguerra R, Monaco R, Manotti L, Goldoni M, Becchi G et al.| title=Understanding the structural features of symptomatic calcific aortic valve stenosis: A broad-spectrum clinicopathologic study in 236 consecutive surgical cases. | journal=Int J Cardiol | year= 2016 | volume= 228 | issue=  | pages= 364-374 | pmid=27866029 | doi=10.1016/j.ijcard.2016.11.180 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27866029  }} </ref> <ref name="pmid27810479">{{cite journal| author=Joseph J, Naqvi SY, Giri J, Goldberg S| title=Aortic stenosis: pathophysiology, diagnosis and therapy. | journal=Am J Med | year= 2016 | volume=  | issue=  | pages=  | pmid=27810479 | doi=10.1016/j.amjmed.2016.10.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27810479  }} </ref> <ref name="pmid25140960">{{cite journal| author=Otto CM, Prendergast B| title=Aortic-valve stenosis--from patients at risk to severe valve obstruction. | journal=N Engl J Med | year= 2014 | volume= 371 | issue= 8 | pages= 744-56 | pmid=25140960 | doi=10.1056/NEJMra1313875 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25140960  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref> <ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref><ref name="pmid23062541">{{cite journal| author=Dweck MR, Boon NA, Newby DE| title=Calcific aortic stenosis: a disease of the valve and the myocardium. | journal=J Am Coll Cardiol | year= 2012 | volume= 60 | issue= 19 | pages= 1854-63 | pmid=23062541 | doi=10.1016/j.jacc.2012.02.093 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23062541  }} </ref> <ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191  }} </ref> <ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191  }} </ref> <ref name="pmid15470217">{{cite journal| author=Enriquez-Sarano M, Tajik AJ| title=Clinical practice. Aortic regurgitation. | journal=N Engl J Med | year= 2004 | volume= 351 | issue= 15 | pages= 1539-46 | pmid=15470217 | doi=10.1056/NEJMcp030912 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15470217 }} </ref> </ref> <ref name="pmid24603191">{{cite journal| author=Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA et al.| title=2014 AHA/ACC guideline 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. | journal=J Am Coll Cardiol | year= 2014 | volume= 63 | issue= 22 | pages= e57-185 | pmid=24603191 | doi=10.1016/j.jacc.2014.02.536 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24603191 }} </ref>


==Genetics==
==Genetics==

Revision as of 16:28, 8 April 2020

Right ventricular outflow tract obstruction Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

pulmonic stenosis is most commonly secondary to acute rheumatic fever. Generally, the initial valvulitis is associated with valvular regurgitation but over a period of 2 or more years, the commissures fuse and the valves thicken and calcify. The chordal supporting structure also calcifies and retracts. The result is the typical “fish mouth deformity”. 70% of the time; the mitral valve is involved in isolation, and 25% of the time; the aortic valve is involved as well. The tricuspid and pulmonic valves are involved less commonly. Patients develop symptoms when the mitral vavle area is 2 to 2.5 cm2.

Pathophysiology

Almost all cases of pulmonic stenosis are due to disease in the heart secondary to rheumatic fever and the consequent rheumatic heart disease (a condition that may develop after strep throat or scarlet fever). Around 90% of cases of rheumatic heart disease are associated with pulmonic stenosis. Pulmonic stenosis is a progressive valvular disease which progression depends mainly on the degree of the narrowing of the pulmonic valve as well as on the maladaptive ventricular wall response. [1] [2][3] [4] [5] [5] [6] [4] [7] [8] [9] [10] [11] [5] [5][5] [12] [12] [13] </ref> [12]

Genetics

[Disease name] is transmitted in [mode of genetic transmission] pattern.

OR

Genes involved in the pathogenesis of [disease name] include:

  • [Gene1]
  • [Gene2]
  • [Gene3]

OR

The development of [disease name] is the result of multiple genetic mutations such as:

  • [Mutation 1]
  • [Mutation 2]
  • [Mutation 3]

Associated Conditions

Conditions associated with [disease name] include:

  • [Condition 1]
  • [Condition 2]
  • [Condition 3]

Gross Pathology

On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

References

  1. ↑ Scarlat A, Bodner G, Liron M (1986). "Massive haemoptysis as the presenting symptom in mitral stenosis". Thorax. 41 (5): 413–4. PMC 1020641. PMID 3750250.
  2. ↑ Waller BF, Howard J, Fess S (1995). "Pathology of tricuspid valve stenosis and pure tricuspid regurgitation--Part I." Clin Cardiol. 18 (2): 97–102. PMID 7720297.
  3. ↑ Maher ER, Pazianas M, Curtis JR (1987). "Calcific aortic stenosis: a complication of chronic uraemia". Nephron. 47 (2): 119–22. PMID 3696315.
  4. ↑ 4.0 4.1 Hull MC, Morris CG, Pepine CJ, Mendenhall NP (2003). "Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of hodgkin lymphoma treated with radiation therapy". JAMA. 290 (21): 2831–7. doi:10.1001/jama.290.21.2831. PMID 14657067.
  5. ↑ 5.0 5.1 5.2 5.3 5.4 Dweck MR, Boon NA, Newby DE (2012). "Calcific aortic stenosis: a disease of the valve and the myocardium". J Am Coll Cardiol. 60 (19): 1854–63. doi:10.1016/j.jacc.2012.02.093. PMID 23062541.
  6. ↑ 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.
  7. ↑ Cleland JG, Swedberg K, Follath F, Komajda M, Cohen-Solal A, Aguilar JC, Dietz R, Gavazzi A, Hobbs R, Korewicki J, Madeira HC, Moiseyev VS, Preda I, van Gilst WH, Widimsky J, Freemantle N, Eastaugh J, Mason J (2003). "The EuroHeart Failure survey programme-- a survey on the quality of care among patients with heart failure in Europe. Part 1: patient characteristics and diagnosis". European Heart Journal. 24 (5): 442–63. PMID 12633546. Retrieved 2012-04-11. Unknown parameter |month= ignored (help)
  8. ↑ Stewart BF, Siscovick D, Lind BK, Gardin JM, Gottdiener JS, Smith VE, Kitzman DW, Otto CM (1997). "Clinical factors associated with calcific aortic valve disease. Cardiovascular Health Study". Journal of the American College of Cardiology. 29 (3): 630–4. PMID 9060903. Retrieved 2012-04-11. Unknown parameter |month= ignored (help)
  9. ↑ Galli D, Manuguerra R, Monaco R, Manotti L, Goldoni M, Becchi G; et al. (2016). "Understanding the structural features of symptomatic calcific aortic valve stenosis: A broad-spectrum clinicopathologic study in 236 consecutive surgical cases". Int J Cardiol. 228: 364–374. doi:10.1016/j.ijcard.2016.11.180. PMID 27866029.
  10. ↑ Joseph J, Naqvi SY, Giri J, Goldberg S (2016). "Aortic stenosis: pathophysiology, diagnosis and therapy". Am J Med. doi:10.1016/j.amjmed.2016.10.005. PMID 27810479.
  11. ↑ Otto CM, Prendergast B (2014). "Aortic-valve stenosis--from patients at risk to severe valve obstruction". N Engl J Med. 371 (8): 744–56. doi:10.1056/NEJMra1313875. PMID 25140960.
  12. ↑ 12.0 12.1 12.2 Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP, Guyton RA; et al. (2014). "2014 AHA/ACC guideline 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". J Am Coll Cardiol. 63 (22): e57–185. doi:10.1016/j.jacc.2014.02.536. PMID 24603191.
  13. ↑ Enriquez-Sarano M, Tajik AJ (2004). "Clinical practice. Aortic regurgitation". N Engl J Med. 351 (15): 1539–46. doi:10.1056/NEJMcp030912. PMID 15470217.

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