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| [[Category: (name of the system)]] | | [[Category: (name of the system)]] |
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| ==[[Pulmonary atresia overview|Overview]]==
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| Pulmonary atresia is a congenital malformation of the pulmonary valve in which the outflow of the blood from the right side of the heart to the pulmonary artery is obstructed due to the valve orifice fails to develop. In most instances, the condition is almost always fatal without any intervention. Atresia means "no opening". In a normal healthy functioning heart, the valve consist of three flaps that opens on ventricular systole leading to emptying of blood from the right ventricle to the pulmonary artery and ultimately to the lungs for proper oxygenation.
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| This condition often is accompanied by a shunt/foreman ( ''''Pulmonary atresia with Ventricular septum defect''' ) that transports the Oxygen-poor blood directly from the right ventricle to the left side of the heart through a shunt. This oxygen-poor blood is then pumped through the aorta to the rest of the body, making fingers, toes, and lips appear blue or cyanotic. [1] In other instance ( ''''Pulmonary atresia with intact Ventricular ventricular septum'''' ), the blood can not flow from the right side to the left side via shunt and due to this reason, the size of the right ventricle is comparatively smaller than the former one. Early intervention is required in these cases and with the immediate intervention, the 5-year survival is 80 percent.
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| The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, open-heart surgery can be performed to make blood flow through the heart in a normal pattern. If the right ventricle is small and unable to act as a pump, doctors may perform an operation called the Fontan procedure. In this procedure, the right atrium is connected directly to the pulmonary artery. Many children with Pulmonary Atresia will go on to lead 'normal' lives.
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| ==[[Pulmonary atresia pathophysiology|Pathophysiology]]==
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| The pulmonary valve is located on the right side of the heart between the right ventricle and the pulmonary artery.<ref name="pmid19271591">{{cite journal| author=McIntyre A| title=Career opportunities in the smaller medical specialties. | journal=Clin Med (Lond) | year= 2009 | volume= 9 | issue= 1 | pages= 10-1 | pmid=19271591 | doi= | pmc=5922622 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19271591 }} </ref> In a normally 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 | display-authors=etal| 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>. 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="pmid17519398">{{cite journal| author=Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E | display-authors=etal| 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="pmid12878741">{{cite journal| author=Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F | display-authors=etal| 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 | display-authors=etal| 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 | display-authors=etal| 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=https://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=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11870246 }} </ref><ref name="pmid8480616">{{cite journal| author=Vaslef SN, Roberts WC| title=Early descriptions of aortic valve stenosis. | journal=Am Heart J | year= 1993 | volume= 125 | issue= 5 Pt 1 | pages= 1465-74 | pmid=8480616 | doi=10.1016/0002-8703(93)91036-e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8480616 }} </ref><ref name="pmid7925504">{{cite journal| author=Lindroos M, Kupari M, Valvanne J, Strandberg T, Heikkilä J, Tilvis R| title=Factors associated with calcific aortic valve degeneration in the elderly. | journal=Eur Heart J | year= 1994 | volume= 15 | issue= 7 | pages= 865-70 | pmid=7925504 | doi=10.1093/oxfordjournals.eurheartj.a060602 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7925504 }} </ref><ref name="pmid3565291">{{cite journal| author=Aronow WS, Schwartz KS, Koenigsberg M| title=Correlation of serum lipids, calcium, and phosphorus, diabetes mellitus and history of systemic hypertension with presence or absence of calcified or thickened aortic cusps or root in elderly patients. | journal=Am J Cardiol | year= 1987 | volume= 59 | issue= 9 | pages= 998-9 | pmid=3565291 | doi=10.1016/0002-9149(87)91144-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3565291 }} </ref><ref name="pmid26227196">{{cite journal| author=Pawade TA, Newby DE, Dweck MR| title=Calcification in Aortic Stenosis: The Skeleton Key. | journal=J Am Coll Cardiol | year= 2015 | volume= 66 | issue= 5 | pages= 561-77 | pmid=26227196 | doi=10.1016/j.jacc.2015.05.066 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26227196 }} </ref><ref name="pmid24153092">{{cite journal| author=Lugiano CA| title=Aortic stenosis. | journal=JAAPA | year= 2013 | volume= 26 | issue= 11 | pages= 46-7 | pmid=24153092 | doi=10.1097/01.JAA.0000436518.69169.8e | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24153092 }} </ref><ref name="pmid11794152">{{cite journal| author=Mylonakis E, Calderwood SB| title=Infective endocarditis in adults. | journal=N Engl J Med | year= 2001 | volume= 345 | issue= 18 | pages= 1318-30 | pmid=11794152 | doi=10.1056/NEJMra010082 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11794152 }} </ref><ref name="pmid20579534">{{cite journal| author=Siu SC, Silversides CK| title=Bicuspid aortic valve disease. | journal=J Am Coll Cardiol | year= 2010 | volume= 55 | issue= 25 | pages= 2789-800 | pmid=20579534 | doi=10.1016/j.jacc.2009.12.068 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20579534 }} </ref><ref name="pmid21737022">{{cite journal| author=Linefsky JP, O'Brien KD, Katz R, de Boer IH, Barasch E, Jenny NS | display-authors=etal| title=Association of serum phosphate levels with aortic valve sclerosis and annular calcification: the cardiovascular health study. | journal=J Am Coll Cardiol | year= 2011 | volume= 58 | issue= 3 | pages= 291-7 | pmid=21737022 | doi=10.1016/j.jacc.2010.11.073 | pmc=3147295 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21737022 }} </ref><ref name="pmid7484833">{{cite journal| author=Gotoh T, Kuroda T, Yamasawa M, Nishinaga M, Mitsuhashi T, Seino Y | display-authors=etal| title=Correlation between lipoprotein(a) and aortic valve sclerosis assessed by echocardiography (the JMS Cardiac Echo and Cohort Study). | journal=Am J Cardiol | year= 1995 | volume= 76 | issue= 12 | pages= 928-32 | pmid=7484833 | doi=10.1016/s0002-9149(99)80263-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7484833 }} </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=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14657067 }} </ref>
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| ==[[Pulmonary atresia epidemiology and demographics|Epidemiology and demographics]]== | | ==[[Pulmonary atresia epidemiology and demographics|Epidemiology and demographics]]== |
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| ==[[Pulmonary atresia causes|Causes]]== | | ==[[Pulmonary atresia causes|Causes]]== |
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| ==[[Pulmonary atresia differential diagnosis|Differentiating Pulmonary atresia from other Disorders]]==
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| As Pulmonic valva atresia presents with the signs and symptoms of right ventricular outflow obstruction, it can be confused with the disease with similar presentation. Diagnosis can be made on the basis of Echocardiographic findings. Conditions sharing the pulmonic outflow obstructions are;
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| 1) Tetralogy of Fallot
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| 2) Critical Pulmonary Stenosis
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| 3) Tricuspid Atresia
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| ==Diagnosis== | | ==Diagnosis== |
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy===
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| * Medical therapy in newborns with Pulmonary atresia serves only as a bridge to surgical treatment. It is temporarily and definitive treatment is only surgical.
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| * It is targeted to keep the ductus arteriosus open that otherwise will close in 48 hr. An IV medication called prostaglandin E1 is used to keep the ductus arteriosus open and gives time while the newborn will be prepared for the definitive treatment. Blood circulates from the aorta to the pulmonary artery via this ductus and gets oxygenated in the lungs.
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| * Another short term strategy is keeping the opening between the right and left atrium patent ( foramen ovale ). This process is called "Balloon atrial septostomy" done via Cardiac Catheterization. A guided wire with the tip of the balloon on it is inserted via vein and advanced to the right side of the heart. reaching the foramen ovale. The balloon tip is inflated and adjusted at the opening and the wire is removed This opening will shunt the blood from the right atrium to the left, to the aorta, and via ductus arterosus to the pulmonary artery and then to the lungs for proper oxygenation.
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| * It should be kept in mind these procedures ONLY are the short term management and eventually newborn will need surgery. <ref>{{cite web |url=https://www.cdc.gov/ncbddd/heartdefects/pulmonaryatresia.html |title=Congenital Heart Defects - Facts about Pulmonary Atresia | CDC |format= |work= |accessdate=}}</ref><ref>{{cite web |url=http://www.heart.org/idc/groups/heart-public/@wcm/@hcm/documents/downloadable/ucm_307665.pdf |title=www.heart.org |format= |work= |accessdate=}}</ref><ref>{{cite web |url=https://www.nhlbi.nih.gov/health-topics/congenital-heart-defects |title=Congenital Heart Defects | National Heart, Lung, and Blood Institute (NHLBI) |format= |work= |accessdate=}}</ref>
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| '''Surgical:''' [[Pulmonary atresia surgery|Surgery]]
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| [[Pulmonary atresia primary prevention|Primary Prevention]] | [[Pulmonary atresia secondary prevention|Secondary Prevention]] | [[Pulmonary atresia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Pulmonary atresia future or investigational therapies|Future or Investigational Therapies]]
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| ==References== | | ==References== |
| {{reflist|2}} | | {{reflist|2}} |