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Elevated jugular venous pressure

MedGen UID:
636282
Concept ID:
C0520861
Finding
Synonyms: Increase in Jugular Venous Pressure; Raised jugular venous pressure
SNOMED CT: Raised jugular venous pressure (22447003)
 
HPO: HP:0030848

Definition

Increased jugular venous pressure. [from HPO]

Term Hierarchy

Conditions with this feature

Myopathy, myosin storage, autosomal recessive
MedGen UID:
340603
Concept ID:
C1850709
Disease or Syndrome
Autosomal recessive myosin storage congenital myopathy-7B (CMYP7B) is a skeletal muscle disorder characterized by the onset of scapuloperoneal muscle weakness in early childhood or young adulthood. Affected individuals have difficulty walking, steppage gait, and scapular winging due to shoulder girdle involvement. The severity and progression of the disorder is highly variable, even within families. Most patients develop respiratory insufficiency, nocturnal hypoventilation, and restrictive lung disease; some develop hypertrophic cardiomyopathy. Additional features include myopathic facies, high-arched palate, scoliosis, and muscle wasting with thin body habitus. Serum creatine kinase may be normal or elevated. Skeletal muscle biopsy shows variable findings, including myosin storage disease, type 1 fiber predominance, centralized nuclei, and multiminicore disease (Onengut et al., 2004; Tajsharghi et al., 2007; Beecroft et al., 2019). For a discussion of genetic heterogeneity of congenital myopathy, see CMYP1A (117000).
Pulmonary venoocclusive disease 1
MedGen UID:
854500
Concept ID:
C3887658
Disease or Syndrome
Pulmonary venoocclusive disease primarily affects the postcapillary venous pulmonary vessels and may involve significant pulmonary capillary dilation and/or proliferation. PVOD is an uncommon cause of pulmonary artery hypertension (PPH; see 178600), a severe condition characterized by elevated pulmonary artery pressure leading to right heart failure and death. PVOD accounts for 5 to 10% of 'idiopathic' PPH and has an estimated incidence of 0.1 to 0.2 cases per million. The pathologic hallmark of PVOD is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue, with intimal thickening present in venules and small veins in lobular septa and, rarely, larger veins. Definitive diagnosis of PVOD requires histologic analysis of a lung sample, although surgical lung biopsy is often too invasive for these frail patients. Patients with PVOD respond poorly to available therapy, therefore it is crucial to distinguish PVOD from other forms of PPH. Radiologic characteristics suggestive of PVOD on high-resolution CT of the chest include nodular ground-glass opacities, septal lines, and lymph node enlargement. In addition, because PVOD mainly affects postcapillary vasculature, it causes chronic elevation of pulmonary capillary pressure and thus promotes occult alveolar hemorrhage, which may be a characteristic feature of PVOD (summary by Montani et al., 2008). Genetic Heterogeneity of Pulmonary Venoocclusive Disease See also PVOD2 (234810), caused by mutation in the EIF2AK4 gene (609280) on chromosome 15q15.

Professional guidelines

PubMed

Wexler RK, Pleister A, Raman SV
Am Fam Physician 2017 Dec 15;96(12):784-789. PMID: 29431371
Mant J, Doust J, Roalfe A, Barton P, Cowie MR, Glasziou P, Mant D, McManus RJ, Holder R, Deeks J, Fletcher K, Qume M, Sohanpal S, Sanders S, Hobbs FD
Health Technol Assess 2009 Jul;13(32):1-207, iii. doi: 10.3310/hta13320. PMID: 19586584
Chong HH, Plotnick GD
Compr Ther 1995 Jul;21(7):378-85. PMID: 7554815

Recent clinical studies

Etiology

Wexler RK, Pleister A, Raman SV
Am Fam Physician 2017 Dec 15;96(12):784-789. PMID: 29431371
Baeza-Trinidad R, Mosquera-Lozano JD, El Bikri L
Eur J Heart Fail 2017 Jan;19(1):111-115. Epub 2016 Jul 18 doi: 10.1002/ejhf.610. PMID: 27427508
Fellowes C, Daly C, Babu-Narayan SV, Lyne J, Collinson J
Can J Cardiol 2008 Feb;24(2):e3-5. doi: 10.1016/s0828-282x(08)70574-9. PMID: 18273478Free PMC Article
Drazner MH, Rame JE, Dries DL
Am J Med 2003 Apr 15;114(6):431-7. doi: 10.1016/s0002-9343(03)00058-5. PMID: 12727575
Drazner MH, Rame JE, Stevenson LW, Dries DL
N Engl J Med 2001 Aug 23;345(8):574-81. doi: 10.1056/NEJMoa010641. PMID: 11529211

Diagnosis

Rahman A, Saraswat A
Aust Fam Physician 2017 Nov;46(11):810-814. PMID: 29101915
Fellowes C, Daly C, Babu-Narayan SV, Lyne J, Collinson J
Can J Cardiol 2008 Feb;24(2):e3-5. doi: 10.1016/s0828-282x(08)70574-9. PMID: 18273478Free PMC Article
Roy CL, Minor MA, Brookhart MA, Choudhry NK
JAMA 2007 Apr 25;297(16):1810-8. doi: 10.1001/jama.297.16.1810. PMID: 17456823
van't Laar A
Neth J Med 2003 Jul;61(7):268-72. PMID: 14567526
Tsang TS, Oh JK, Seward JB, Tajik AJ
Herz 2000 Dec;25(8):734-40. doi: 10.1007/pl00001991. PMID: 11200121

Therapy

Renier W, Winckelmann KH, Verbakel JY, Aertgeerts B, Buntinx F
Eur J Emerg Med 2018 Feb;25(1):3-11. doi: 10.1097/MEJ.0000000000000429. PMID: 29252938
Rahman A, Saraswat A
Aust Fam Physician 2017 Nov;46(11):810-814. PMID: 29101915
Fellowes C, Daly C, Babu-Narayan SV, Lyne J, Collinson J
Can J Cardiol 2008 Feb;24(2):e3-5. doi: 10.1016/s0828-282x(08)70574-9. PMID: 18273478Free PMC Article
Drazner MH, Rame JE, Dries DL
Am J Med 2003 Apr 15;114(6):431-7. doi: 10.1016/s0002-9343(03)00058-5. PMID: 12727575
Drazner MH, Rame JE, Stevenson LW, Dries DL
N Engl J Med 2001 Aug 23;345(8):574-81. doi: 10.1056/NEJMoa010641. PMID: 11529211

Prognosis

Renier W, Winckelmann KH, Verbakel JY, Aertgeerts B, Buntinx F
Eur J Emerg Med 2018 Feb;25(1):3-11. doi: 10.1097/MEJ.0000000000000429. PMID: 29252938
Baeza-Trinidad R, Mosquera-Lozano JD, El Bikri L
Eur J Heart Fail 2017 Jan;19(1):111-115. Epub 2016 Jul 18 doi: 10.1002/ejhf.610. PMID: 27427508
Drazner MH, Rame JE, Dries DL
Am J Med 2003 Apr 15;114(6):431-7. doi: 10.1016/s0002-9343(03)00058-5. PMID: 12727575
Drazner MH, Rame JE, Stevenson LW, Dries DL
N Engl J Med 2001 Aug 23;345(8):574-81. doi: 10.1056/NEJMoa010641. PMID: 11529211
Tsang TS, Oh JK, Seward JB, Tajik AJ
Herz 2000 Dec;25(8):734-40. doi: 10.1007/pl00001991. PMID: 11200121

Clinical prediction guides

Joshi SS, Hamra M, Newby DE
Heart 2020 Jan;106(2):126-163. doi: 10.1136/heartjnl-2019-315821. PMID: 31874903
Renier W, Winckelmann KH, Verbakel JY, Aertgeerts B, Buntinx F
Eur J Emerg Med 2018 Feb;25(1):3-11. doi: 10.1097/MEJ.0000000000000429. PMID: 29252938
Mant J, Doust J, Roalfe A, Barton P, Cowie MR, Glasziou P, Mant D, McManus RJ, Holder R, Deeks J, Fletcher K, Qume M, Sohanpal S, Sanders S, Hobbs FD
Health Technol Assess 2009 Jul;13(32):1-207, iii. doi: 10.3310/hta13320. PMID: 19586584
Meyer P, Ekundayo OJ, Adamopoulos C, Mujib M, Aban I, White M, Aronow WS, Ahmed A
Am J Cardiol 2009 Mar 15;103(6):839-44. doi: 10.1016/j.amjcard.2008.11.045. PMID: 19268742Free PMC Article
Tsang TS, Oh JK, Seward JB, Tajik AJ
Herz 2000 Dec;25(8):734-40. doi: 10.1007/pl00001991. PMID: 11200121

Recent systematic reviews

Pranata R, Yonas E, Chintya V, Alkatiri AA, Budi Siswanto B
Indian Heart J 2019 May-Jun;71(3):277-283. Epub 2019 Jun 3 doi: 10.1016/j.ihj.2019.05.001. PMID: 31543202Free PMC Article
Renier W, Winckelmann KH, Verbakel JY, Aertgeerts B, Buntinx F
Eur J Emerg Med 2018 Feb;25(1):3-11. doi: 10.1097/MEJ.0000000000000429. PMID: 29252938
Mant J, Doust J, Roalfe A, Barton P, Cowie MR, Glasziou P, Mant D, McManus RJ, Holder R, Deeks J, Fletcher K, Qume M, Sohanpal S, Sanders S, Hobbs FD
Health Technol Assess 2009 Jul;13(32):1-207, iii. doi: 10.3310/hta13320. PMID: 19586584

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