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Pulmonary artery aneurysm

MedGen UID:
505727
Concept ID:
CN004380
Finding
 
HPO: HP:0004937

Definition

An aneurysm (localized balloon-like outward bulging) in the pulmonary artery. [from HPO]

Term Hierarchy

Conditions with this feature

Loeys-Dietz syndrome 2
MedGen UID:
390653
Concept ID:
C2674876
Disease or Syndrome
Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections) and skeletal manifestations (pectus excavatum or pectus carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus). Approximately 75% of affected individuals have LDS type I with craniofacial manifestations (widely spaced eyes, bifid uvula/cleft palate, craniosynostosis); approximately 25% have LDS type II with systemic manifestations of LDSI but minimal or absent craniofacial features. LDSI and LDSII form a clinical continuum. The natural history of LDS is characterized by aggressive arterial aneurysms (mean age at death 26.1 years) and a high incidence of pregnancy-related complications, including death and uterine rupture.
Loeys-Dietz syndrome 1
MedGen UID:
395828
Concept ID:
C2697933
Disease or Syndrome
Loeys-Dietz syndrome (LDS) is characterized by vascular findings (cerebral, thoracic, and abdominal arterial aneurysms and/or dissections) and skeletal manifestations (pectus excavatum or pectus carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus). Approximately 75% of affected individuals have LDS type I with craniofacial manifestations (widely spaced eyes, bifid uvula/cleft palate, craniosynostosis); approximately 25% have LDS type II with systemic manifestations of LDSI but minimal or absent craniofacial features. LDSI and LDSII form a clinical continuum. The natural history of LDS is characterized by aggressive arterial aneurysms (mean age at death 26.1 years) and a high incidence of pregnancy-related complications, including death and uterine rupture.
Autosomal recessive cutis laxa type 1B
MedGen UID:
482428
Concept ID:
C3280798
Disease or Syndrome
EFEMP2-related cutis laxa (autosomal recessive cutis laxa type 1B [ARCL1B]) is characterized by cutis laxa and systemic involvement, most commonly arterial tortuosity, aneurysms and stenosis; retrognathia; joint laxity; and arachnodactyly. Severity ranges from perinatal lethality as a result of cardiopulmonary failure to manifestations limited to the vascular and craniofacial systems.

Recent clinical studies

Etiology

Lerner AD, Riker DR
Ann Thorac Surg 2014 May;97(5):e139-41. doi: 10.1016/j.athoracsur.2013.12.083. PMID: 24792302
Unlu M, Demirkol S, Balta S, Bozlar U, Kucuk U
Kardiol Pol 2013;71(11):1180-2. doi: 10.5603/KP.2013.0300. PMID: 24297717
Derk G, Laks H, Aboulhosn J
Catheter Cardiovasc Interv 2013 Nov 1;82(5):828-32. Epub 2013 Mar 19 doi: 10.1002/ccd.24829. [Epub ahead of print] PMID: 23404953
Patnaik AN, Barik R, Babu S, Gullati AS
Pediatr Cardiol 2013 Mar;34(3):748-51. Epub 2012 May 12 doi: 10.1007/s00246-012-0338-0. [Epub ahead of print] PMID: 22580773
Shayan H, Sareyyupoglu B, Shigemura N, Thacker J, Bermudez C, Toyoda Y
Ann Thorac Surg 2012 Jan;93(1):e3-5. doi: 10.1016/j.athoracsur.2011.07.067. PMID: 22186486

Diagnosis

Lerner AD, Riker DR
Ann Thorac Surg 2014 May;97(5):e139-41. doi: 10.1016/j.athoracsur.2013.12.083. PMID: 24792302
Pande S, Tewari P, Agarwal SK, Jain M, Phadke RV
Ann Thorac Surg 2014 Feb;97(2):e33-5. doi: 10.1016/j.athoracsur.2013.10.055. PMID: 24484839
Bezgin T, Elveran A, Karagöz A, Doğan C, Esen AM
Herz 2014 Aug;39(5):657-60. Epub 2013 Jul 24 doi: 10.1007/s00059-013-3887-y. [Epub ahead of print] PMID: 23989764
Tefera E, Teodori M
World J Pediatr Congenit Heart Surg 2013 Oct;4(4):427-9. doi: 10.1177/2150135113494403. PMID: 24327639
Arango Tomás E, Cerezo Madueño F, Salvatierra Velázquez A
Interact Cardiovasc Thorac Surg 2013 Jul;17(1):176-8. Epub 2013 Feb 26 doi: 10.1093/icvts/ivt064. [Epub ahead of print] PMID: 23442938Free PMC Article

Therapy

Arango Tomás E, Cerezo Madueño F, Salvatierra Velázquez A
Interact Cardiovasc Thorac Surg 2013 Jul;17(1):176-8. Epub 2013 Feb 26 doi: 10.1093/icvts/ivt064. [Epub ahead of print] PMID: 23442938Free PMC Article
Elqatni M, Sekkach Y, Abouzahir A, Ghafir D
Intern Med 2011;50(3):263-4. Epub 2011 Feb 1 PMID: 21297333
Shankarappa RK, Moorthy N, Chandrasekaran D, Nanjappa MC
Tex Heart Inst J 2010;37(2):244-5. PMID: 20401307Free PMC Article
Lee SW, Lee SY, Kim KN, Jung JK, Chung WT
Clin Rheumatol 2010 Jan;29(1):91-3. Epub 2009 Oct 9 doi: 10.1007/s10067-009-1272-2. [Epub ahead of print] PMID: 19816754
Takahama M, Yamamoto R, Nakajima R, Tada H
Interact Cardiovasc Thorac Surg 2009 Mar;8(3):390-2. Epub 2008 Dec 8 doi: 10.1510/icvts.2008.194647. [Epub ahead of print] PMID: 19064583

Prognosis

Shah R, Shriki J, Shinbane JS
Tex Heart Inst J 2014 Jun;41(3):349-50. Epub 2014 Jun 1 doi: 10.14503/THIJ-12-2464. PMID: 24955063Free PMC Article
Lerner AD, Riker DR
Ann Thorac Surg 2014 May;97(5):e139-41. doi: 10.1016/j.athoracsur.2013.12.083. PMID: 24792302
Pati PK, George PV, Jose JV
J Am Coll Cardiol 2013 Feb 12;61(6):685. doi: 10.1016/j.jacc.2012.07.077. PMID: 23391202
Patnaik AN, Barik R, Babu S, Gullati AS
Pediatr Cardiol 2013 Mar;34(3):748-51. Epub 2012 May 12 doi: 10.1007/s00246-012-0338-0. [Epub ahead of print] PMID: 22580773
Shayan H, Sareyyupoglu B, Shigemura N, Thacker J, Bermudez C, Toyoda Y
Ann Thorac Surg 2012 Jan;93(1):e3-5. doi: 10.1016/j.athoracsur.2011.07.067. PMID: 22186486

Clinical prediction guides

Shah R, Shriki J, Shinbane JS
Tex Heart Inst J 2014 Jun;41(3):349-50. Epub 2014 Jun 1 doi: 10.14503/THIJ-12-2464. PMID: 24955063Free PMC Article
Rupprecht H, Ghidau M, Ditterich D
Thorac Cardiovasc Surg 2012 Oct;60(7):491-2. Epub 2011 Jul 20 doi: 10.1055/s-0031-1280070. [Epub ahead of print] PMID: 21776585
Hirohata S, Kikuchi H
Clin Exp Rheumatol 2009 Mar-Apr;27(2 Suppl 53):S91-5. PMID: 19796542
Durak D, Eren B, Turkmen N, Fedakar R
Bratisl Lek Listy 2008;109(12):582-3. PMID: 19348384
Coard KC, Martin MP
Arch Pathol Lab Med 1992 Feb;116(2):159-61. PMID: 1733410

Recent systematic reviews

Soysal T, Salihoğlu A, Esatoğlu SN, Gültürk E, Eşkazan AE, Hatemi G, Hatemi I, Öngören Aydın Ş, Erzin YZ, Başlar Z, Tüzüner N, Ferhanoğlu B, Çelik AF
Rheumatology (Oxford) 2014 Jun;53(6):1136-41. Epub 2014 Feb 6 doi: 10.1093/rheumatology/ket479. [Epub ahead of print] PMID: 24505123

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