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Elevated pulmonary artery pressure

MedGen UID:
812247
Concept ID:
C3805917
Finding
Synonym: Increased pulmonary artery pressure
 
HPO: HP:0004890

Definition

An abnormally elevated blood pressure in the circulation of the pulmonary artery. [from HPO]

Conditions with this feature

Pulmonary hypertension due to chronic exposure to high altitude
MedGen UID:
83314
Concept ID:
C0340552
Disease or Syndrome
Pulmonary hypertension, primary, 3
MedGen UID:
815522
Concept ID:
C3809192
Disease or Syndrome
Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Pulmonary arterial hypertension is one form of a broader condition known as pulmonary hypertension. Pulmonary hypertension occurs when most of the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. To overcome the increased resistance, blood pressure increases in the pulmonary artery and in the right ventricle of the heart, which is the chamber that pumps blood into the pulmonary artery. Ultimately, the increased blood pressure can damage the right ventricle of the heart.\n\nSigns and symptoms of pulmonary arterial hypertension occur when increased blood pressure cannot fully overcome the elevated resistance. As a result, the flow of oxygenated blood from the lungs to the rest of the body is insufficient. Shortness of breath (dyspnea) during exertion and fainting spells are the most common symptoms of pulmonary arterial hypertension. People with this disorder may experience additional symptoms, particularly as the condition worsens. Other symptoms include dizziness, swelling (edema) of the ankles or legs, chest pain, and a rapid heart rate.
Pulmonary hypertension, primary, 4
MedGen UID:
815528
Concept ID:
C3809198
Disease or Syndrome
Primary pulmonary hypertension is a rare progressive disease characterized by increased pulmonary artery pressure in the absence of common causes of pulmonary hypertension, such as chronic heart, lung, or thromboembolic disease. There is often vascular remodeling. The clinical presentation can be nonspecific, and patients often receive a diagnosis late in their clinical course (summary by Ma et al., 2013). For a general phenotypic description and a discussion of genetic heterogeneity of primary pulmonary hypertension, see PPH1 (178600).
Megacystis-microcolon-intestinal hypoperistalsis syndrome 2
MedGen UID:
1788773
Concept ID:
C5543476
Disease or Syndrome
Megacystis-microcolon-intestinal hypoperistalsis syndrome-2 (MMIHS2) is characterized by prenatal bladder enlargement, neonatal functional gastrointestinal obstruction, and chronic dependence on total parenteral nutrition and urinary catheterization. The majority of cases have a fatal outcome due to malnutrition and sepsis, followed by multiorgan failure (summary by Wang et al., 2019). For a discussion of genetic heterogeneity of MMIHS, see 249210.

Professional guidelines

PubMed

Mukherjee D, Konduri GG
Compr Physiol 2021 Jun 30;11(3):2135-2190. doi: 10.1002/cphy.c200023. PMID: 34190343Free PMC Article
Aryal SR, Moussa H, Sinkey R, Dhungana R, Tallaj JA, Pamboukian SV, Patarroyo-Aponte M, Acharya D, Bajaj NS, Bhattarai S, Lenneman A, Joly JM, Sibai BM, Rajapreyar IN
Am J Obstet Gynecol MFM 2020 May;2(2):100087. Epub 2020 Jan 23 doi: 10.1016/j.ajogmf.2020.100087. PMID: 33345958
Barnett CF, Alvarez P, Park MH
Cardiol Clin 2016 Aug;34(3):375-89. doi: 10.1016/j.ccl.2016.04.006. PMID: 27443135

Recent clinical studies

Etiology

Park E, Safdar Z
Methodist Debakey Cardiovasc J 2024;20(2):70-80. Epub 2024 Mar 14 doi: 10.14797/mdcvj.1308. PMID: 38495664Free PMC Article
Jankowich M, Maron BA, Choudhary G
Lancet Respir Med 2021 Oct;9(10):1185-1191. Epub 2021 Jul 21 doi: 10.1016/S2213-2600(21)00072-2. PMID: 34270923Free PMC Article
Maron BA, Brittain EL, Hess E, Waldo SW, Barón AE, Huang S, Goldstein RH, Assad T, Wertheim BM, Alba GA, Leopold JA, Olschewski H, Galiè N, Simonneau G, Kovacs G, Tedford RJ, Humbert M, Choudhary G
Lancet Respir Med 2020 Sep;8(9):873-884. Epub 2020 Jul 27 doi: 10.1016/S2213-2600(20)30317-9. PMID: 32730752Free PMC Article
Barnes H, Brown Z, Burns A, Williams T
Cochrane Database Syst Rev 2019 Jan 31;1(1):CD012621. doi: 10.1002/14651858.CD012621.pub2. PMID: 30701543Free PMC Article
Shah SJ
JAMA 2012 Oct 3;308(13):1366-74. doi: 10.1001/jama.2012.12347. PMID: 23032553

Diagnosis

Singh N, Mullin CJ
R I Med J (2013) 2021 Sep 1;104(7):30-35. PMID: 34437663
Mukherjee D, Konduri GG
Compr Physiol 2021 Jun 30;11(3):2135-2190. doi: 10.1002/cphy.c200023. PMID: 34190343Free PMC Article
Huitema MP, Mathijssen H, Mager JJ, Snijder RJ, Grutters JC, Post MC
Semin Respir Crit Care Med 2020 Oct;41(5):659-672. Epub 2020 Aug 10 doi: 10.1055/s-0040-1713615. PMID: 32777851
Maron BA, Brittain EL, Hess E, Waldo SW, Barón AE, Huang S, Goldstein RH, Assad T, Wertheim BM, Alba GA, Leopold JA, Olschewski H, Galiè N, Simonneau G, Kovacs G, Tedford RJ, Humbert M, Choudhary G
Lancet Respir Med 2020 Sep;8(9):873-884. Epub 2020 Jul 27 doi: 10.1016/S2213-2600(20)30317-9. PMID: 32730752Free PMC Article
Barnes H, Brown Z, Burns A, Williams T
Cochrane Database Syst Rev 2019 Jan 31;1(1):CD012621. doi: 10.1002/14651858.CD012621.pub2. PMID: 30701543Free PMC Article

Therapy

Park E, Safdar Z
Methodist Debakey Cardiovasc J 2024;20(2):70-80. Epub 2024 Mar 14 doi: 10.14797/mdcvj.1308. PMID: 38495664Free PMC Article
Bhogal S, Khraisha O, Al Madani M, Treece J, Baumrucker SJ, Paul TK
Am J Ther 2019 Jul/Aug;26(4):e520-e526. doi: 10.1097/MJT.0000000000000766. PMID: 30946047
Barnes H, Brown Z, Burns A, Williams T
Cochrane Database Syst Rev 2019 Jan 31;1(1):CD012621. doi: 10.1002/14651858.CD012621.pub2. PMID: 30701543Free PMC Article
Ghofrani HA, Humbert M, Langleben D, Schermuly R, Stasch JP, Wilkins MR, Klinger JR
Chest 2017 Feb;151(2):468-480. Epub 2016 Jun 2 doi: 10.1016/j.chest.2016.05.024. PMID: 27263466
Shah SJ
JAMA 2012 Oct 3;308(13):1366-74. doi: 10.1001/jama.2012.12347. PMID: 23032553

Prognosis

Zi-Yang Y, Nanshan X, Dongling L, Tao H, Yigao H, Danqing Y, Caojin Z
Gene 2023 Oct 5;882:147642. Epub 2023 Jul 15 doi: 10.1016/j.gene.2023.147642. PMID: 37454747
Maron BA, Brittain EL, Hess E, Waldo SW, Barón AE, Huang S, Goldstein RH, Assad T, Wertheim BM, Alba GA, Leopold JA, Olschewski H, Galiè N, Simonneau G, Kovacs G, Tedford RJ, Humbert M, Choudhary G
Lancet Respir Med 2020 Sep;8(9):873-884. Epub 2020 Jul 27 doi: 10.1016/S2213-2600(20)30317-9. PMID: 32730752Free PMC Article
Nasr VG, Callahan R, Wichner Z, Odegard KC, DiNardo JA
Anesth Analg 2019 Jul;129(1):27-40. doi: 10.1213/ANE.0000000000003924. PMID: 30451723
Wang J, Su Y, Bai J, Wang W, Qin S, Ge J
J Interv Card Electrophysiol 2014 Aug;40(2):171-8. Epub 2014 Apr 13 doi: 10.1007/s10840-014-9890-2. PMID: 24728706
Shah SJ
JAMA 2012 Oct 3;308(13):1366-74. doi: 10.1001/jama.2012.12347. PMID: 23032553

Clinical prediction guides

Chuang ML, Sia SK, Chang KW
Ann Med 2023 Dec;55(1):2228696. doi: 10.1080/07853890.2023.2228696. PMID: 37387199Free PMC Article
Mathew R
Med Sci (Basel) 2021 Sep 22;9(4) doi: 10.3390/medsci9040058. PMID: 34698188Free PMC Article
Sun J, Weng B, Zhang X, Chu X, Cai C
BMC Cardiovasc Disord 2021 Oct 24;21(1):514. doi: 10.1186/s12872-021-02330-w. PMID: 34689755Free PMC Article
Hester J, Ventetuolo C, Lahm T
Compr Physiol 2019 Dec 18;10(1):125-170. doi: 10.1002/cphy.c190011. PMID: 31853950Free PMC Article
Nasr VG, Callahan R, Wichner Z, Odegard KC, DiNardo JA
Anesth Analg 2019 Jul;129(1):27-40. doi: 10.1213/ANE.0000000000003924. PMID: 30451723

Recent systematic reviews

Barnes H, Brown Z, Burns A, Williams T
Cochrane Database Syst Rev 2019 Jan 31;1(1):CD012621. doi: 10.1002/14651858.CD012621.pub2. PMID: 30701543Free PMC Article

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