Aortic intramural hematoma: Difference between revisions
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{{Infobox_Disease | | |||
Name = {{PAGENAME}} | | |||
Image = Pathogenesis-of-aortic-intramural-haematoma-illustration.png| | |||
Caption = <ref>Case courtesy of Dr Vincent Tatco, Radiopaedia.org, rID: 48454</ref>| | |||
}} | |||
{{SI}} | {{SI}} | ||
{{CMG}} | {{CMG}} '''Associate Editor-In-Chief:''' {{CZ}} | ||
'''Associate Editor-In-Chief:''' {{CZ}} | {{SK}}: IMH | ||
==Overview== | ==Overview== | ||
Aortic intramural hematoma is classically abbreviated as IMH. It may occur as a primary event in [[hypertensive]] patients in whom there is [[spontaneous bleeding]] from [[vasa vasorum]] into the media or may be caused by a penetrating [[Atherosclerotic|atherosclerotic ulcer.]] Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury. Thought to begin with the rupture of the [[vasa vasorum]], the blood vessels that penetrate the outer half of the aortic media from the [[adventitia]] and arborize within the media to supply the aortic wall. The [[hematoma]] propagates along the | Aortic intramural hematoma is classically abbreviated as IMH. It may occur as a primary event in [[hypertensive]] [[patients]] in whom there is [[spontaneous bleeding]] from [[vasa vasorum]] into the media or may be caused by a penetrating [[Atherosclerotic|atherosclerotic ulcer.]] Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury. Thought to begin with the rupture of the [[vasa vasorum]], the blood vessels that penetrate the outer half of the aortic media from the [[adventitia]] and arborize within the media to supply the aortic wall. The [[hematoma]] propagates along with the medial layer of the aorta. Consequently, intramural [[hematoma]] weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the [[intima]], the latter leading to communicating [[aortic dissection]]. Unlike aortic dissection, no [[intimal]] flap is present. If it involves the [[ascending aorta]], treatment is surgical to prevent rupture or progression to a classic [[aortic dissection]]. Conservative management is indicated for aortic intramural hematomas of the [[descending aorta]]. | ||
==Historical Perspective== | ==Historical Perspective== | ||
*The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was | *The [[diagnosis]] of aortic intramural hematoma dates back to early 1980s when the choice of [[diagnosis]] was [[transesophageal echocardiography]].<ref name="pmid9327708">{{cite journal| author=Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA et al.| title=Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection. | journal=Am Heart J | year= 1997 | volume= 134 | issue= 3 | pages= 495-507 | pmid=9327708 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9327708 }} </ref> | ||
==Classification== | ==Classification== | ||
IMH is classified into two types on the basis of Standford classification.<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref> | IMH is classified into two types on the basis of Standford [[classification]].<ref name="pmid24617732">{{cite journal| author=Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA et al.| title=Aortic arch dissection: a controversy of classification. | journal=Radiology | year= 2014 | volume= 271 | issue= 3 | pages= 848-55 | pmid=24617732 | doi=10.1148/radiol.14131457 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24617732 }} </ref> | ||
* Type A IMH: Involves the [[ascending aorta]], and may or may not involve [[descending aorta]] | * Type A IMH: Involves the [[ascending aorta]], and may or may not involve [[descending aorta]] | ||
* Type B IMH: Involves the [[descending aorta]] only distal to [[left subclavian artery]] | * Type B IMH: Involves the [[descending aorta]] only distal to [[left subclavian artery]] | ||
==Pathophysiology== | ==Pathophysiology== | ||
*The pathogenesis of IMH is characterized by either rupture of [[vasa vasorum]] or tear in the [[Intima|intimal layer]]. | *The [[pathogenesis]] of IMH is characterized by either [[rupture]] of [[vasa vasorum]] or tear in the [[Intima|intimal layer]]. | ||
*Rupture of [[vasa vasorum]] is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The [[hematoma]] is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref> | *[[Rupture]] of [[vasa vasorum]] is seen in the majority of cases, which separates [[medial]] wall of the [[aorta]] leading to a tear but there is no continuous flow. The [[hematoma]] is contained within the wall.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref> | ||
*Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with [[atherosclerotic]] changes of the aortic wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref> | *Small microscopic tear in the [[penetrating aortic ulcer]] is another mechanism leading to formation of IMH. These [[ulcers]] are mostly associated with [[atherosclerotic]] changes of the [[aortic]] wall as well.<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref> | ||
*On microscopic histopathological analysis, [[hematoma]] is seen in the media layer of the aorta. | *On microscopic [[histopathological]] analysis, [[hematoma]] is seen in the media layer of the [[aorta]]. | ||
==Clinical Features== | ==Clinical Features== | ||
* Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH. | * Pain is the most common presentation of patients with IMH. The location of [[pain]] depends on the location of IMH. | ||
* [[Chest pain]] is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), and abdominal pain (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref> | * [[Chest pain]] is the most common presenting symptoms in 82.5 % of the [[patients]], followed by back pain (41%), and [[abdominal pain]] (13.1%).<ref name="pmid24515957">{{cite journal| author=Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V| title=Aortic intramural hematoma and its complications. | journal=Circulation | year= 2014 | volume= 129 | issue= 6 | pages= 711-6 | pmid=24515957 | doi=10.1161/CIRCULATIONAHA.113.001809 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24515957 }} </ref> | ||
* Pain is reportedly severe on onset in almost all the cases, abrupt in onset. | *[[Pain]] is reportedly severe on onset in almost all the cases, abrupt in onset. | ||
* Radiation of pain is seen 45.9% of patients commonly radiating to the back and shoulders. | *[[Radiation]] of [[pain]] is seen 45.9% of patients commonly radiating to the back and shoulders. | ||
* Up to 32% of these patients can present with [[hypertension]], whereas a minority (11.9%) present with [[hypotension]]. | * Up to 32% of these [[Patient|patients]] can present with [[hypertension]], whereas a minority (11.9%) present with [[hypotension]]. | ||
* Patient with Type A IMH can present with murmur of [[aortic regurgitation]] (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients. | * Patient with Type A IMH can present with murmur of [[aortic regurgitation]] (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients. | ||
==Differentiating Aortic Intramural Hematoma from other Diseases== | ==Differentiating Aortic Intramural Hematoma from other Diseases== | ||
*IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, | *IMH must be differentiated from other diseases that cause sudden onset [[chest pain]], [[back pain]], [[abdominal pain]] with or without [[radiation]] such as [[Aortic Dissection|aortic dissection]], [[acute coronary syndrome]], [[pulmonary embolism]], [[renal infarct]], [[Mesenteric ischemia|meseneteric ischemia]], and trauma to the back. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
* The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref> | * The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.<ref name="pmid6702637">{{cite journal| author=Larson EW, Edwards WD| title=Risk factors for aortic dissection: a necropsy study of 161 cases. | journal=Am J Cardiol | year= 1984 | volume= 53 | issue= 6 | pages= 849-55 | pmid=6702637 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6702637 }} </ref> | ||
*IMH is more commonly observed among patients age in the range of 60-80 years old. | *IMH is more commonly observed among patients age in the range of 60-80 years old. | ||
*Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref> | *Mean age for presentation is 69 years.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref> | ||
*IMH is more common in men with 50-81% of cases | *IMH is more common in men with 50-81% of cases occurring commonly in men.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref> | ||
* There is no racial predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref> | * There is no [[racial]] predilection for IMH.<ref name="pmid27533160">{{cite journal| author=Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA| title=Acute Aortic Dissection and Intramural Hematoma: A Systematic Review. | journal=JAMA | year= 2016 | volume= 316 | issue= 7 | pages= 754-63 | pmid=27533160 | doi=10.1001/jama.2016.10026 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27533160 }} </ref> | ||
==Risk Factors== | ==Risk Factors== | ||
* Common risk factors in the development of IMH are [[hypertension]], smoking, connective tissue disorder ([[Marfan's syndrome]], [[Loeys-Dietz syndrome]]), [[bicuspid aortic valve]], prior aortic surgery, [[aortic aneurysm]] and [[atherosclerosis]].<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref> | * Common risk factors in the development of IMH are [[hypertension]], [[smoking]], [[Connective tissue disease|connective tissue disorder]] ([[Marfan's syndrome]], [[Loeys-Dietz syndrome]]), [[bicuspid aortic valve]], prior aortic surgery, [[aortic aneurysm]] and [[atherosclerosis]].<ref name="pmid10685714">{{cite journal| author=Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL et al.| title=The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease. | journal=JAMA | year= 2000 | volume= 283 | issue= 7 | pages= 897-903 | pmid=10685714 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10685714 }} </ref> | ||
== Natural History, Complications and Prognosis== | == Natural History, Complications and Prognosis== | ||
* IMH resolve spontaneously in about 10% of patients.<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref> | * IMH resolve spontaneously in about 10% of [[patients]].<ref name="pmid20625143">{{cite journal| author=Braverman AC| title=Acute aortic dissection: clinician update. | journal=Circulation | year= 2010 | volume= 122 | issue= 2 | pages= 184-8 | pmid=20625143 | doi=10.1161/CIRCULATIONAHA.110.958975 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20625143 }} </ref> | ||
*Early clinical features include sudden onset chest pain, back pain and hypertensive emergency. | *Early clinical features include sudden onset [[chest pain]], [[back pain]] and [[hypertensive emergency]]. | ||
*If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref> | *If left untreated, 16-47% of [[patients]] with IMH may progress to develop [[aortic dissection]] which can be life threatening.<ref name="pmid12119238">{{cite journal| author=Nienaber CA, Sievers HH| title=Intramural hematoma in acute aortic syndrome: more than one variant of dissection? | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 284-5 | pmid=12119238 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119238 }} </ref> | ||
* Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref> | * Common [[complications]] of IMH include progression to [[aortic dissection]], formation of periaortic hematoma, [[pericardial effusion]], [[pericardial tamponade]], [[pseudoaneurysm]], and [[aortic regurgitation]].<ref name="pmid12119251">{{cite journal| author=Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H et al.| title=Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis. | journal=Circulation | year= 2002 | volume= 106 | issue= 3 | pages= 342-8 | pmid=12119251 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12119251 }} </ref> | ||
* Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical) | *[[Prognosis]] is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical). | ||
== Diagnosis == | == Diagnosis == | ||
===Diagnostic Criteria=== | ===Diagnostic Criteria=== | ||
* The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref> | * The [[diagnosis]] of IMH is made with clinical examination in combination of [[radiological]] or [[echocardiographic]] findings. Although, there is no specific [[diagnostic criteria]] set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref> | ||
=== Symptoms === | === History and Symptoms === | ||
* Symptoms of IMH may include | * Symptoms of IMH may include sudden onset [[chest pain]], [[back pain]], radiation of pain from chest to back, [[abdominal pain]], and [[sweating]].<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref> | ||
=== Physical Examination === | === Physical Examination === | ||
* Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref> | * Patients with IMH usually appear distressed and it depends on the stage of their presentation.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref> | ||
* Physical examination may be remarkable for | * Physical examination may be remarkable for [[sweating]], [[tachycardia]], [[hypertension]], [[aortic regurgitation]] murmur, and radial-radial pulse deficit or radial-femoral pulse deficit depending on the location of the IMH. | ||
=== Laboratory Findings === | === Laboratory Findings === | ||
* There are no specific laboratory findings associated with IMH. | * There are no specific laboratory findings associated with IMH. | ||
* Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection. | * Occasionally, a drop in [[hemoglobin]] or [[hematocrit]] can be seen in [[patients]] where IMH is progressing to [[aortic dissection]]. | ||
{| align="right" | |||
|[[File:Aortic-intramural-haematoma-6 (1).jpg|thumb|none|250px|contrast-enhanced CT<ref>Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 50557</ref>]] | |||
|} | |||
{| align="right" | |||
|[[ File:Aortic-intramural-haematoma-6.jpg|thumb|none|200px|contrast-enhanced CT<ref>Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 50557</ref>]] | |||
|} | |||
===Imaging Findings=== | ===Imaging Findings=== | ||
* Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR | *[[Transthoracic echocardiography]], [[transesophageal echocardiography]], [[CT angiography]], [[MR angiography]] are the modality of [[imaging]] which can be used.<ref name="pmid15020502">{{cite journal| author=Song JK| title=Diagnosis of aortic intramural haematoma. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 368-71 | pmid=15020502 | doi= | pmc=1768152 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020502 }} </ref> | ||
* MR | *[[MR angiography]] is the imaging modality of choice for IMH. However, as it requires 25-35 minutes; thus in an emergency situation [[CT angiography]] or [[echocardiogram]] is the test of choice in the emergency situation. | ||
* When CT or | * When [[CT]] or [[echocardiorgraphy]] is equivocal, [[MR]] should be the next step. | ||
* On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in diagnosis of IMH. | * On echocardiography, an echolucent crescent can be seen in the [[aorta]]. This is seen in 70-80% of the patients. M-mode [[echocardiography]] is more useful in the diagnosis of IMH. | ||
* CT shows attenuation by the aortic wall and intimal flap can be seen. | *[[CT]] shows attenuation by the aortic wall and intimal flap can be seen. Crescentic aortic wall thickening can be seen as well. | ||
* MR is superior to CT to CT and echocardiography for diagnosis of IMH. | *[[MR]] is superior to CT to [[CT]] and [[echocardiography]] for [[diagnosis]] of IMH. | ||
== Treatment == | == Treatment == | ||
=== Medical Therapy === | === Medical Therapy === | ||
* The mainstay of therapy for IMH is control of hypertension as the hypertensive emergency. In patients with IMH, the target blood pressure should be lower than 130/80 mm of Hg. Labetalol IV or infusion can be a treatment of choice as it controls blood pressure as well as heart rate. Drugs causing reflex tachycardia should be avoided.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref><ref name="pmid14677748">{{cite journal| author=Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR| title=Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 4 | pages= 325-7 | pmid=14677748 | doi= | pmc=307723 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14677748 }} </ref> | * The mainstay of therapy for IMH is control of hypertension as the hypertensive emergency. In [[patients]] with IMH, the target [[blood pressure]] should be lower than 130/80 mm of Hg. [[Labetalol]] [[Intravenous therapy|IV]] or [[infusion]] can be a treatment of choice as it controls [[blood pressure]] as well as heart rate. Drugs causing reflex [[tachycardia]] should be avoided.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref><ref name="pmid14677748">{{cite journal| author=Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR| title=Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 4 | pages= 325-7 | pmid=14677748 | doi= | pmc=307723 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14677748 }} </ref> | ||
* Heart rate should be controlled prior to initiating blood pressure medications. | *[[Heart rate]] should be controlled prior to initiating [[blood pressure]] medications. | ||
* Anti-hypertensive medication acts by decreasing the stress in the media thereby preventing the extension of IMH. | *[[Anti-hypertensive]] medication acts by decreasing the stress in the media thereby preventing the extension of IMH. | ||
* Response to anti-hypertensive can be monitored with a change in blood pressure and heart rate every 30 minutes. | * Response to [[anti-hypertensive]] can be monitored with a change in blood pressure and [[heart rate]] every 30 minutes. | ||
* When patients are clinically stable, they can be closely monitored without proceeding to surgery. | * When [[patients]] are clinically stable, they can be closely monitored without proceeding to [[surgery]]. | ||
=== Surgery === | === Surgery === | ||
* Surgery is preferred options in patients who are at high risk to proceed to aortic dissection.<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref> | *[[Surgery]] is preferred options in [[patients]] who are at high risk to proceed to [[aortic dissection]].<ref name="pmid15020506">{{cite journal| author=Dake MD| title=Aortic intramural haematoma: current therapeutic strategy. | journal=Heart | year= 2004 | volume= 90 | issue= 4 | pages= 375-8 | pmid=15020506 | doi= | pmc=1768168 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15020506 }} </ref> | ||
* Surgical option depends on the location of IMH. Replacement of aortic root is advised for type A IMH whereas minimal invasive surgery can be performed.<ref name="pmid14677748">{{cite journal| author=Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR| title=Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 4 | pages= 325-7 | pmid=14677748 | doi= | pmc=307723 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14677748 }} </ref> | * Surgical option depends on the location of IMH. Replacement of [[aortic root]] is advised for type A IMH whereas minimal invasive surgery can be performed.<ref name="pmid14677748">{{cite journal| author=Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR| title=Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery. | journal=Tex Heart Inst J | year= 2003 | volume= 30 | issue= 4 | pages= 325-7 | pmid=14677748 | doi= | pmc=307723 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14677748 }} </ref> | ||
=== Prevention === | === Prevention === | ||
* There are no primary preventive measures available for IMH. | * There are no [[Primary prevention|primary preventive]] measures available for IMH. | ||
* Once diagnosed and successfully treated, patients with IMH who are medically managed are followed-up every 3-6 months by imaging. | * Once [[Diagnosis|diagnosed]] and successfully treated, patients with IMH who are medically managed are followed-up every 3-6 months by [[imaging]]. | ||
==Guideline== | ==Guideline== | ||
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'''Abbreviations''': '''CT''' = computed tomography; '''IMH''' = intramural haematoma; '''MRI''' = magnetic resonance imaging; '''TEVAR''' = thoracic endovascular aortic repair. | '''Abbreviations''': '''CT''' = computed tomography; '''IMH''' = intramural haematoma; '''MRI''' = magnetic resonance imaging; '''TEVAR''' = thoracic endovascular aortic repair. | ||
</small></small> | </small></small> | ||
==See also== | ==See also== | ||
*[[Acute aortic syndrome]] | *[[Acute aortic syndrome]] | ||
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==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
{{WikiDoc Help Menu}} | {{WikiDoc Help Menu}} | ||
{{WikiDoc Sources}} | {{WikiDoc Sources}} | ||
[[CME Category::Cardiology]] | [[CME Category::Cardiology]] | ||
[[Category:Cardiology]] | [[Category:Cardiology]] |
Latest revision as of 19:05, 23 September 2020
Aortic intramural hematoma | |
[1] |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Synonyms and keywords:: IMH
Overview
Aortic intramural hematoma is classically abbreviated as IMH. It may occur as a primary event in hypertensive patients in whom there is spontaneous bleeding from vasa vasorum into the media or may be caused by a penetrating atherosclerotic ulcer. Intramural hematoma may also develop as a result of blunt chest trauma with aortic wall injury. Thought to begin with the rupture of the vasa vasorum, the blood vessels that penetrate the outer half of the aortic media from the adventitia and arborize within the media to supply the aortic wall. The hematoma propagates along with the medial layer of the aorta. Consequently, intramural hematoma weakens the aorta and may progress either to outward rupture of the aortic wall or to inward disruption of the intima, the latter leading to communicating aortic dissection. Unlike aortic dissection, no intimal flap is present. If it involves the ascending aorta, treatment is surgical to prevent rupture or progression to a classic aortic dissection. Conservative management is indicated for aortic intramural hematomas of the descending aorta.
Historical Perspective
- The diagnosis of aortic intramural hematoma dates back to early 1980s when the choice of diagnosis was transesophageal echocardiography.[2]
Classification
IMH is classified into two types on the basis of Standford classification.[3]
- Type A IMH: Involves the ascending aorta, and may or may not involve descending aorta
- Type B IMH: Involves the descending aorta only distal to left subclavian artery
Pathophysiology
- The pathogenesis of IMH is characterized by either rupture of vasa vasorum or tear in the intimal layer.
- Rupture of vasa vasorum is seen in the majority of cases, which separates medial wall of the aorta leading to a tear but there is no continuous flow. The hematoma is contained within the wall.[4]
- Small microscopic tear in the penetrating aortic ulcer is another mechanism leading to formation of IMH. These ulcers are mostly associated with atherosclerotic changes of the aortic wall as well.[4]
- On microscopic histopathological analysis, hematoma is seen in the media layer of the aorta.
Clinical Features
- Pain is the most common presentation of patients with IMH. The location of pain depends on the location of IMH.
- Chest pain is the most common presenting symptoms in 82.5 % of the patients, followed by back pain (41%), and abdominal pain (13.1%).[4]
- Pain is reportedly severe on onset in almost all the cases, abrupt in onset.
- Radiation of pain is seen 45.9% of patients commonly radiating to the back and shoulders.
- Up to 32% of these patients can present with hypertension, whereas a minority (11.9%) present with hypotension.
- Patient with Type A IMH can present with murmur of aortic regurgitation (35.2%) and pulse deficit is seen in a minority(15.1%) of the patients.
Differentiating Aortic Intramural Hematoma from other Diseases
- IMH must be differentiated from other diseases that cause sudden onset chest pain, back pain, abdominal pain with or without radiation such as aortic dissection, acute coronary syndrome, pulmonary embolism, renal infarct, meseneteric ischemia, and trauma to the back.
Epidemiology and Demographics
- The prevalence of IMH is approximately 2 to 4 cases per 100,000 individuals worldwide.[5]
- IMH is more commonly observed among patients age in the range of 60-80 years old.
- Mean age for presentation is 69 years.[6]
- IMH is more common in men with 50-81% of cases occurring commonly in men.[6]
- There is no racial predilection for IMH.[6]
Risk Factors
- Common risk factors in the development of IMH are hypertension, smoking, connective tissue disorder (Marfan's syndrome, Loeys-Dietz syndrome), bicuspid aortic valve, prior aortic surgery, aortic aneurysm and atherosclerosis.[7]
Natural History, Complications and Prognosis
- IMH resolve spontaneously in about 10% of patients.[8]
- Early clinical features include sudden onset chest pain, back pain and hypertensive emergency.
- If left untreated, 16-47% of patients with IMH may progress to develop aortic dissection which can be life threatening.[9]
- Common complications of IMH include progression to aortic dissection, formation of periaortic hematoma, pericardial effusion, pericardial tamponade, pseudoaneurysm, and aortic regurgitation.[10]
- Prognosis is generally poor, and depends on multitude of factors like location of IMH, presentation, mode of management (medical vs surgical).
Diagnosis
Diagnostic Criteria
- The diagnosis of IMH is made with clinical examination in combination of radiological or echocardiographic findings. Although, there is no specific diagnostic criteria set for IMH. A study has suggested mean thickness of Type A IMH was in the range of 5-40 mm, and type B IMH was reported in the range of 5-23 mm.[11]
History and Symptoms
- Symptoms of IMH may include sudden onset chest pain, back pain, radiation of pain from chest to back, abdominal pain, and sweating.[11]
Physical Examination
- Patients with IMH usually appear distressed and it depends on the stage of their presentation.[11]
- Physical examination may be remarkable for sweating, tachycardia, hypertension, aortic regurgitation murmur, and radial-radial pulse deficit or radial-femoral pulse deficit depending on the location of the IMH.
Laboratory Findings
- There are no specific laboratory findings associated with IMH.
- Occasionally, a drop in hemoglobin or hematocrit can be seen in patients where IMH is progressing to aortic dissection.
Imaging Findings
- Transthoracic echocardiography, transesophageal echocardiography, CT angiography, MR angiography are the modality of imaging which can be used.[11]
- MR angiography is the imaging modality of choice for IMH. However, as it requires 25-35 minutes; thus in an emergency situation CT angiography or echocardiogram is the test of choice in the emergency situation.
- When CT or echocardiorgraphy is equivocal, MR should be the next step.
- On echocardiography, an echolucent crescent can be seen in the aorta. This is seen in 70-80% of the patients. M-mode echocardiography is more useful in the diagnosis of IMH.
- CT shows attenuation by the aortic wall and intimal flap can be seen. Crescentic aortic wall thickening can be seen as well.
- MR is superior to CT to CT and echocardiography for diagnosis of IMH.
Treatment
Medical Therapy
- The mainstay of therapy for IMH is control of hypertension as the hypertensive emergency. In patients with IMH, the target blood pressure should be lower than 130/80 mm of Hg. Labetalol IV or infusion can be a treatment of choice as it controls blood pressure as well as heart rate. Drugs causing reflex tachycardia should be avoided.[14][15]
- Heart rate should be controlled prior to initiating blood pressure medications.
- Anti-hypertensive medication acts by decreasing the stress in the media thereby preventing the extension of IMH.
- Response to anti-hypertensive can be monitored with a change in blood pressure and heart rate every 30 minutes.
- When patients are clinically stable, they can be closely monitored without proceeding to surgery.
Surgery
- Surgery is preferred options in patients who are at high risk to proceed to aortic dissection.[14]
- Surgical option depends on the location of IMH. Replacement of aortic root is advised for type A IMH whereas minimal invasive surgery can be performed.[15]
Prevention
- There are no primary preventive measures available for IMH.
- Once diagnosed and successfully treated, patients with IMH who are medically managed are followed-up every 3-6 months by imaging.
Guideline
2014 ESC Guidelines on the Diagnosis and Treatment of Aortic Diseases (DO NOT EDIT)[16]
Recommendations | Class | Level |
---|---|---|
|
I | C |
I | C | |
|
I | C |
I | C | |
|
IIa | C |
|
IIb | C |
Abbreviations: CT = computed tomography; IMH = intramural haematoma; MRI = magnetic resonance imaging; TEVAR = thoracic endovascular aortic repair.
See also
External Links
References
- ↑ Case courtesy of Dr Vincent Tatco, Radiopaedia.org, rID: 48454
- ↑ Vilacosta I, San Román JA, Ferreirós J, Aragoncillo P, Méndez R, Castillo JA; et al. (1997). "Natural history and serial morphology of aortic intramural hematoma: a novel variant of aortic dissection". Am Heart J. 134 (3): 495–507. PMID 9327708.
- ↑ Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA; et al. (2014). "Aortic arch dissection: a controversy of classification". Radiology. 271 (3): 848–55. doi:10.1148/radiol.14131457. PMID 24617732.
- ↑ 4.0 4.1 4.2 Alomari IB, Hamirani YS, Madera G, Tabe C, Akhtar N, Raizada V (2014). "Aortic intramural hematoma and its complications". Circulation. 129 (6): 711–6. doi:10.1161/CIRCULATIONAHA.113.001809. PMID 24515957.
- ↑ Larson EW, Edwards WD (1984). "Risk factors for aortic dissection: a necropsy study of 161 cases". Am J Cardiol. 53 (6): 849–55. PMID 6702637.
- ↑ 6.0 6.1 6.2 Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA (2016). "Acute Aortic Dissection and Intramural Hematoma: A Systematic Review". JAMA. 316 (7): 754–63. doi:10.1001/jama.2016.10026. PMID 27533160.
- ↑ Hagan PG, Nienaber CA, Isselbacher EM, Bruckman D, Karavite DJ, Russman PL; et al. (2000). "The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease". JAMA. 283 (7): 897–903. PMID 10685714.
- ↑ Braverman AC (2010). "Acute aortic dissection: clinician update". Circulation. 122 (2): 184–8. doi:10.1161/CIRCULATIONAHA.110.958975. PMID 20625143.
- ↑ Nienaber CA, Sievers HH (2002). "Intramural hematoma in acute aortic syndrome: more than one variant of dissection?". Circulation. 106 (3): 284–5. PMID 12119238.
- ↑ Ganaha F, Miller DC, Sugimoto K, Do YS, Minamiguchi H, Saito H; et al. (2002). "Prognosis of aortic intramural hematoma with and without penetrating atherosclerotic ulcer: a clinical and radiological analysis". Circulation. 106 (3): 342–8. PMID 12119251.
- ↑ 11.0 11.1 11.2 11.3 Song JK (2004). "Diagnosis of aortic intramural haematoma". Heart. 90 (4): 368–71. PMC 1768152. PMID 15020502.
- ↑ Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 50557
- ↑ Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 50557
- ↑ 14.0 14.1 Dake MD (2004). "Aortic intramural haematoma: current therapeutic strategy". Heart. 90 (4): 375–8. PMC 1768168. PMID 15020506.
- ↑ 15.0 15.1 Saborio DV, Sadeghi A, Burack JH, Lowery RC, Genovesi MH, Brevetti GR (2003). "Management of intramural hematoma of the ascending aorta and aortic arch: the risks of limited surgery". Tex Heart Inst J. 30 (4): 325–7. PMC 307723. PMID 14677748.
- ↑ Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, Evangelista A, Falk V, Frank H, Gaemperli O, Grabenwöger M, Haverich A, Iung B, Manolis AJ, Meijboom F, Nienaber CA, Roffi M, Rousseau H, Sechtem U, Sirnes PA, Allmen RS, Vrints CJ (November 2014). "2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC)". Eur. Heart J. 35 (41): 2873–926. doi:10.1093/eurheartj/ehu281. PMID 25173340.