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{{Right ventricular outflow tract obstruction}}
{{Right ventricular outflow tract obstruction}}
{{CMG}}
{{CMG}}; '''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu] [[User:Mohammed Salih|Mohammed Salih, MD.]] [mailto:Mohammed.Salih@stjoeshealth.org]
 
'''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
 
==Overview==
==Overview==
'''Right ventricular outflow tract''' (''RVOT'') and/or '''pulmonary artery obstruction''' are present in some form in 25 % of all congenital heart defects. The right ventricular outflow tract obstruction includes stenosis or narrowing of the [[pulmonary valve]], the tissue above the valve (supravalvar obstruction) and below it(subvalvar obstruction). Congenital subvalvar and supravalvar right ventricular outflow tract stenosis usually occurs with other congenital heart defects such as [[Ventricular Septal Defect]] (VSD) or [[Tetralogy of Fallot]]. The timing and type of surgery will vary from patient to patient depending upon the severity of the obstruction and the associated congenital defect.
'''Right ventricular outflow tract''' (''RVOT'') and/or '''pulmonary artery obstruction''' are present in some form in 25 % of all congenital heart defects. The right ventricular outflow tract obstruction includes stenosis or narrowing of the [[pulmonary valve]], the tissue above the valve (supravalvar obstruction) and below it(subvalvar obstruction). Congenital subvalvar and supravalvar right ventricular outflow tract stenosis usually occurs with other congenital heart defects such as [[Ventricular Septal Defect]] (VSD) or [[Tetralogy of Fallot]]. The timing and type of surgery will vary from patient to patient depending upon the severity of the obstruction and the associated congenital defect.


If right ventricular outflow tract obstruction is present and the ventricular septum is intact, usually the right ventricle will adapt better given that no right to left shunting is present. This absence of shunting may result in sufficient pulmonary blood flow to allow the patient to remain asymptomatic longer.
If right ventricular outflow tract obstruction is present and the ventricular septum is intact, usually the right ventricle will adapt better given that no right to left shunting is present. This absence of shunting may result in sufficient pulmonary blood flow to allow the patient to remain asymptomatic longer.
 
Pulmonary stenosis is one of the most common RVOT obstructions. The oxygen-depleted blood is coming from the body to the heart through the superior and inferior vena cava, arriving into the right atrium, right ventricle, passing through the RVOT and pulmonary valve to the pulmonary artery and lungs to pick up oxygen. Normally the pulmonary valve has three leaflets. These leaflets can be malformed due to congenital defects, producing pulmonary valve narrowing (stenotic) or leaky (insufficient). If the pulmonary valve is tight (stenosis), the right side of the heart needs to work harder to push blood through the valve to the lungs. The stenosis, insufficiency or both can be mild to severe. Therefore the symptoms will depend upon the grades of pulmonary stenosis.
Usually therapy is directed to increase pulmonary blood flow and decompressing the right ventricle (RV) and it will depend on the severity and location of the defect. According to the anatomic features of the pulmonary valve stenosis, the physician may use balloon dilatation (a minimally invasive transvenous procedure) to dilate the obstruction during cardiac catheterization, but if the pulmonary artery is the structure involved, stenting (insertion of a small tube) within the narrowed region can be done following balloon angioplasty. Standard treatment of right ventricular outflow tract or pulmonary artery obstruction involves open chest surgery. <ref>Surgery for congenital heart defects. J.Stark and M. de leval. Grune & Stratton.</ref>


Usually therapy is directed to increase pulmonary blood flow and decompressing the right ventricle (RV) and it will depend on the severity and location of the defect. According to the anatomic features of the pulmonary valve stenosis, the physician may use balloon dilatation (a minimally invasive transvenous procedure) to dilate the obstruction during cardiac catheterization, but if the pulmonary artery is the structure involved, stenting (insertion of a small tube) within the narrowed region can be done following balloon angioplasty. Standard treatment of right ventricular outflow tract or pulmonary artery obstruction involves open chest surgery. <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>


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


[[Category: Cardiology]]
[[Category:Disease]]
 
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Pediatrics]]
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Latest revision as of 02:43, 27 June 2020

Right ventricular outflow tract obstruction Microchapters

Home

Patient Information

Overview

Anatomy of Pulmonary Valve

Classification

Pulmonary valve stenosis
Pulmonary subvalvular stenosis
Pulmonary supravalvular stenosis
Pulmonary atresia

Pathophysiology

Causes

Differentiating Right ventricular outflow tract obstruction from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Electrocardiogram

Chest X Ray

Echocardiography

Cardiac Catheterization

Pulmonary Angiography

Treatment

Indications For Surgery

Surgery

Pre-Operative A/P

Post-Operative A/P

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Special Scenarios

Pulmonary artery conduits/Prosthetic Valves

Double-Chambered Right Ventricle

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3] Mohammed Salih, MD. [4]

Overview

Right ventricular outflow tract (RVOT) and/or pulmonary artery obstruction are present in some form in 25 % of all congenital heart defects. The right ventricular outflow tract obstruction includes stenosis or narrowing of the pulmonary valve, the tissue above the valve (supravalvar obstruction) and below it(subvalvar obstruction). Congenital subvalvar and supravalvar right ventricular outflow tract stenosis usually occurs with other congenital heart defects such as Ventricular Septal Defect (VSD) or Tetralogy of Fallot. The timing and type of surgery will vary from patient to patient depending upon the severity of the obstruction and the associated congenital defect.

If right ventricular outflow tract obstruction is present and the ventricular septum is intact, usually the right ventricle will adapt better given that no right to left shunting is present. This absence of shunting may result in sufficient pulmonary blood flow to allow the patient to remain asymptomatic longer.

Usually therapy is directed to increase pulmonary blood flow and decompressing the right ventricle (RV) and it will depend on the severity and location of the defect. According to the anatomic features of the pulmonary valve stenosis, the physician may use balloon dilatation (a minimally invasive transvenous procedure) to dilate the obstruction during cardiac catheterization, but if the pulmonary artery is the structure involved, stenting (insertion of a small tube) within the narrowed region can be done following balloon angioplasty. Standard treatment of right ventricular outflow tract or pulmonary artery obstruction involves open chest surgery. [1] [2][3] [4] [5]

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. 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. 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.

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