Pulmonary atresia: Difference between revisions
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{{CMG}}; '''Associate Editor(s)-In-Chief: | {{CMG}}; '''Associate Editor(s)-In-Chief: {{Mwaq}} | ||
==[[Pulmonary atresia overview|Overview]]== | ==[[Pulmonary atresia overview|Overview]]== | ||
Revision as of 23:59, 23 June 2020
For patient information click here
| Pulmonary atresia | ||
| ICD-10 | Q25.5 | |
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| ICD-9 | 747.3 | |
| MedlinePlus | 001091 | |
| MeSH | C14.240.670 | |
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Pulmonary atresia Microchapters |
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Diagnosis |
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Treatment |
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Pulmonary atresia On the Web |
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American Roentgen Ray Society Images of Pulmonary atresia |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Muhammad Waqas, M.D.[2]
Overview
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.
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.
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.
Pathophysiology
The pulmonary valve is located on the right side of the heart between the right ventricle and the pulmonary artery.[1] 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.[2]. 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.[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]
Epidemiology and demographics
- While there is no difference in the incidence of Pulmonary atresia in male or female, it is found that pulmonary atresia with VSD ( PA-VSD ) is slightly more prevalent in males than in females. [17]
1) The prevalence of Pulmonary Atresia with VSD is estimated to be around 0.07 per 1000 live breath. and 2.5-3.4 % among all congenital heart diseases.[3]
2) The overall incidence of PA-IVSD is under estimated as most of the fetus are spontaneously aborted due to the underlying other congenital malformations or are diagnosed on routine antenatal ultrasound and results in elective termination.
- The reported incidence is 6-8 per 100,000 live births and 1-3% of all congenital heart disease. [4][5]
Classification
Natural history, Complications, and Prognosis
Causes
Differentiating Pulmonary atresia from other Disorders
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;
1) Tetralogy of Fallot
2) Critical Pulmonary Stenosis
3) Tricuspid Atresia
Diagnosis
History and Symptoms | Physical Examination | Laboratory Tests | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy
- Medical therapy in newborns with Pulmonary atresia serves only as a bridge to surgical treatment. It is temporarily and definitive treatment is only surgical.
- 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.
- 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.
- It should be kept in mind these procedures ONLY are the short term management and eventually newborn will need surgery. [18][19][20]
Surgical: Surgery
Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
References
- ↑ McIntyre A (2009). "Career opportunities in the smaller medical specialties". Clin Med (Lond). 9 (1): 10–1. PMC 5922622. PMID 19271591.
- ↑ 2.0 2.1 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.
- ↑ Vaslef SN, Roberts WC (1993). "Early descriptions of aortic valve stenosis". Am Heart J. 125 (5 Pt 1): 1465–74. doi:10.1016/0002-8703(93)91036-e. PMID 8480616.
- ↑ Lindroos M, Kupari M, Valvanne J, Strandberg T, Heikkilä J, Tilvis R (1994). "Factors associated with calcific aortic valve degeneration in the elderly". Eur Heart J. 15 (7): 865–70. doi:10.1093/oxfordjournals.eurheartj.a060602. PMID 7925504.
- ↑ Aronow WS, Schwartz KS, Koenigsberg M (1987). "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". Am J Cardiol. 59 (9): 998–9. doi:10.1016/0002-9149(87)91144-1. PMID 3565291.
- ↑ Pawade TA, Newby DE, Dweck MR (2015). "Calcification in Aortic Stenosis: The Skeleton Key". J Am Coll Cardiol. 66 (5): 561–77. doi:10.1016/j.jacc.2015.05.066. PMID 26227196.
- ↑ Lugiano CA (2013). "Aortic stenosis". JAAPA. 26 (11): 46–7. doi:10.1097/01.JAA.0000436518.69169.8e. PMID 24153092.
- ↑ Mylonakis E, Calderwood SB (2001). "Infective endocarditis in adults". N Engl J Med. 345 (18): 1318–30. doi:10.1056/NEJMra010082. PMID 11794152.
- ↑ Siu SC, Silversides CK (2010). "Bicuspid aortic valve disease". J Am Coll Cardiol. 55 (25): 2789–800. doi:10.1016/j.jacc.2009.12.068. PMID 20579534.
- ↑ Linefsky JP, O'Brien KD, Katz R, de Boer IH, Barasch E, Jenny NS; et al. (2011). "Association of serum phosphate levels with aortic valve sclerosis and annular calcification: the cardiovascular health study". J Am Coll Cardiol. 58 (3): 291–7. doi:10.1016/j.jacc.2010.11.073. PMC 3147295. PMID 21737022.
- ↑ Gotoh T, Kuroda T, Yamasawa M, Nishinaga M, Mitsuhashi T, Seino Y; et al. (1995). "Correlation between lipoprotein(a) and aortic valve sclerosis assessed by echocardiography (the JMS Cardiac Echo and Cohort Study)". Am J Cardiol. 76 (12): 928–32. doi:10.1016/s0002-9149(99)80263-x. PMID 7484833.
- ↑ 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.
- ↑ https://www.uptodate.com/contents/pulmonary-atresia-with-intact-ventricular-septum-pa-ivs?search=pulmonary%20atresia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1#H172915282][https://emedicine.medscape.com/article/905119-overview#a5
- ↑ "Congenital Heart Defects - Facts about Pulmonary Atresia | CDC".
- ↑ "www.heart.org" (PDF).
- ↑ "Congenital Heart Defects | National Heart, Lung, and Blood Institute (NHLBI)".