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Decreased heart rate variability

MedGen UID:
1639159
Concept ID:
C4703580
Finding
Synonym: Reduced heart rate variability
 
HPO: HP:0031861

Definition

Reduced variation of beat-to-beat intervals of the heart that occurs in conjunction with the respiratory cycle. [from HPO]

Term Hierarchy

Conditions with this feature

Finnish type amyloidosis
MedGen UID:
301243
Concept ID:
C1622345
Disease or Syndrome
The Finnish type of systemic amyloidosis is characterized clinically by a unique constellation of features including lattice corneal dystrophy, and cranial neuropathy, bulbar signs, and skin changes. Some patients may develop peripheral neuropathy and renal failure. The disorder is usually inherited in an autosomal dominant pattern; however, homozygotes with a more severe phenotype have also been reported (Meretoja, 1973). Finnish hereditary amyloidosis, also known as Meretoja syndrome or AGel amyloidosis, is one of the most common diseases in the Finnish disease heritage. Symptoms commonly appear by age 40, with the first finding usually corneal lattice dystrophy (CLD), diagnosed by an ophthalmologist. Impaired vision, polyneuropathy, facial nerve paresis, and cutis laxa follow. These symptoms may develop slowly and simultaneously, since amyloid accumulates systemically at a constant rate (summary by Nikoskinen et al., 2015). For a discussion of genetic heterogeneity of hereditary systemic amyloidosis, see AMYLD1 (105210).
Deeah syndrome
MedGen UID:
1756624
Concept ID:
C5436579
Disease or Syndrome
DEEAH syndrome is an autosomal recessive multisystemic disorder with onset in early infancy. Affected individuals usually present in the perinatal period with respiratory insufficiency, apneic episodes, and generalized hypotonia. The patients have failure to thrive and severely impaired global development with poor acquisition of motor, cognitive, and language skills. Other common features include endocrine, pancreatic exocrine, and autonomic dysfunction, as well as hematologic disturbances, mainly low hemoglobin. Patients also have dysmorphic and myopathic facial features. Additional more variable features include seizures, undescended testes, and distal skeletal anomalies. Death in early childhood may occur (summary by Schneeberger et al., 2020).
Neurodevelopmental disorder with dysmorphic facies, impaired speech, and hypotonia
MedGen UID:
1776912
Concept ID:
C5436585
Disease or Syndrome
Neurodevelopmental disorder with dysmorphic facies, impaired speech, and hypotonia (NEDDISH) is an autosomal recessive disorder characterized by global developmental delay and mildly to severely impaired intellectual development with poor speech and language acquisition. Some patients may have early normal development with onset of the disorder in the first years of life. More variable neurologic abnormalities include hypotonia, seizures, apnea, mild signs of autonomic or peripheral neuropathy, and autism. Aside from dysmorphic facial features and occasional findings such as scoliosis or undescended testes, other organ systems are not involved (summary by Schneeberger et al., 2020).
Central hypoventilation syndrome, congenital, 1, with or without Hirschsprung disease
MedGen UID:
1794285
Concept ID:
C5562075
Disease or Syndrome
Congenital central hypoventilation syndrome (CCHS) represents the extreme manifestation of autonomic nervous system dysregulation (ANSD) with the hallmark of disordered respiratory control. The age of initial recognition of CCHS ranges from neonatal onset (i.e., in the first 30 days of life) to (less commonly) later onset (from 1 month to adulthood). Neonatal-onset CCHS is characterized by apparent hypoventilation with monotonous respiratory rates and shallow breathing either during sleep only or while awake as well as asleep; ANSD including decreased heart rate beat-to-beat variability and sinus pauses; altered temperature regulation; and altered pupillary response to light. Some children have altered development of neural crest-derived structures (i.e., Hirschsprung disease, altered esophageal motility/dysphagia, and severe constipation even in the absence of Hirschsprung disease) and/or tumors of neural crest origin (neuroblastoma, ganglioneuroma, and ganglioneuroblastoma). Neurocognitive delay is variable, and possibly influenced by cyanotic breath holding, prolonged sinus pauses, need for 24-hour/day artificial ventilation, and seizures. Later-onset CCHS is characterized by alveolar hypoventilation during sleep and attenuated manifestations of ANSD.

Professional guidelines

PubMed

Salo TM, Viikari JS, Antila KJ, Voipio-Pulkki LM, Jalonen JO, Välimäki IA
J Auton Nerv Syst 1996 Aug 27;60(1-2):61-70. doi: 10.1016/0165-1838(96)00036-7. PMID: 8884697
Krone RJ
Ann Intern Med 1992 Feb 1;116(3):223-37. doi: 10.7326/0003-4819-116-3-223. PMID: 1728206

Recent clinical studies

Etiology

da Silva RB, Neves VR, Montarroyos UR, Silveira MS, Sobral Filho DC
Heart Lung 2023 May-Jun;59:33-36. Epub 2023 Jan 26 doi: 10.1016/j.hrtlng.2023.01.007. PMID: 36706686
Chang HC, Huang CJ, Yang AC, Cheng HM, Chuang SY, Yu WC, Chiang CE, Chen CH, Sung SH
J Am Heart Assoc 2021 Dec 21;10(24):e021585. Epub 2021 Dec 10 doi: 10.1161/JAHA.121.021585. PMID: 34889105Free PMC Article
do Nascimento Vinholes L, Sousa da Silva A, Marinho Tassi E, Corrêa Borges de Lacerda G
Acta Neurol Scand 2021 Jan;143(1):62-70. Epub 2020 Aug 25 doi: 10.1111/ane.13330. PMID: 32749695
Roumeliotis A, Roumeliotis S, Chan C, Pierratos A
Curr Vasc Pharmacol 2021;19(1):21-33. doi: 10.2174/1570161118666200401112106. PMID: 32234001
Pimentel RMM, Macedo H Jr, Valenti VE, Rocha FO, Abreu LC, de M Monteiro CB, Ferreira C
Respir Care 2019 Sep;64(9):1088-1095. Epub 2019 May 28 doi: 10.4187/respcare.06681. PMID: 31138737

Diagnosis

Suzuki M, Nakamura T, Hirayama M, Ueda M, Hatanaka M, Harada Y, Nakatochi M, Nakatsubo D, Maesawa S, Saito R, Fujiwara K, Katsuno M
J Neural Transm (Vienna) 2022 Oct;129(10):1299-1306. Epub 2022 Jul 14 doi: 10.1007/s00702-022-02528-y. PMID: 35835890
Thapa R, Pokorski I, Ambarchi Z, Thomas E, Demayo M, Boulton K, Matthews S, Patel S, Sedeli I, Hickie IB, Guastella AJ
Autism Res 2021 Jan;14(1):75-85. Epub 2020 Nov 22 doi: 10.1002/aur.2437. PMID: 33225622
Roumeliotis A, Roumeliotis S, Chan C, Pierratos A
Curr Vasc Pharmacol 2021;19(1):21-33. doi: 10.2174/1570161118666200401112106. PMID: 32234001
Vaillancourt M, Chia P, Sarji S, Nguyen J, Hoftman N, Ruffenach G, Eghbali M, Mahajan A, Umar S
Respir Res 2017 Dec 4;18(1):201. doi: 10.1186/s12931-017-0679-6. PMID: 29202826Free PMC Article
De Jong MJ, Randall DC
J Cardiovasc Nurs 2005 May-Jun;20(3):186-95; quiz 196-7. doi: 10.1097/00005082-200505000-00010. PMID: 15870589

Therapy

McDonnell P, Fornell P, Ponce S, Dyer L
Birth Defects Res 2023 Mar 1;115(4):474-487. Epub 2022 Dec 14 doi: 10.1002/bdr2.2135. PMID: 36515170
Zhang Q, Du X, Li H, Jiang Y, Zhu X, Zhang Y, Niu Y, Liu C, Ji J, Chillrud SN, Cai J, Chen R, Kan H
J Hazard Mater 2022 Aug 5;435:129031. Epub 2022 Apr 29 doi: 10.1016/j.jhazmat.2022.129031. PMID: 35523096
do Nascimento Vinholes L, Sousa da Silva A, Marinho Tassi E, Corrêa Borges de Lacerda G
Acta Neurol Scand 2021 Jan;143(1):62-70. Epub 2020 Aug 25 doi: 10.1111/ane.13330. PMID: 32749695
Roumeliotis A, Roumeliotis S, Chan C, Pierratos A
Curr Vasc Pharmacol 2021;19(1):21-33. doi: 10.2174/1570161118666200401112106. PMID: 32234001
Vaillancourt M, Chia P, Sarji S, Nguyen J, Hoftman N, Ruffenach G, Eghbali M, Mahajan A, Umar S
Respir Res 2017 Dec 4;18(1):201. doi: 10.1186/s12931-017-0679-6. PMID: 29202826Free PMC Article

Prognosis

da Silva RB, Neves VR, Montarroyos UR, Silveira MS, Sobral Filho DC
Heart Lung 2023 May-Jun;59:33-36. Epub 2023 Jan 26 doi: 10.1016/j.hrtlng.2023.01.007. PMID: 36706686
Herzog SA, Brakoulias V
Curr Med Chem 2022;29(35):5584-5594. doi: 10.2174/0929867329666211217094941. PMID: 34923935
Chang HC, Huang CJ, Yang AC, Cheng HM, Chuang SY, Yu WC, Chiang CE, Chen CH, Sung SH
J Am Heart Assoc 2021 Dec 21;10(24):e021585. Epub 2021 Dec 10 doi: 10.1161/JAHA.121.021585. PMID: 34889105Free PMC Article
Pimentel RMM, Macedo H Jr, Valenti VE, Rocha FO, Abreu LC, de M Monteiro CB, Ferreira C
Respir Care 2019 Sep;64(9):1088-1095. Epub 2019 May 28 doi: 10.4187/respcare.06681. PMID: 31138737
De Jong MJ, Randall DC
J Cardiovasc Nurs 2005 May-Jun;20(3):186-95; quiz 196-7. doi: 10.1097/00005082-200505000-00010. PMID: 15870589

Clinical prediction guides

Zhang Z, Liu M, Zhao L, Liu L, Guo W, Yu J, Yang H, Lai X, Zhang X, Yang L
Environ Pollut 2023 Aug 1;330:121760. Epub 2023 May 2 doi: 10.1016/j.envpol.2023.121760. PMID: 37142210
da Silva RB, Neves VR, Montarroyos UR, Silveira MS, Sobral Filho DC
Heart Lung 2023 May-Jun;59:33-36. Epub 2023 Jan 26 doi: 10.1016/j.hrtlng.2023.01.007. PMID: 36706686
Zhang Q, Du X, Li H, Jiang Y, Zhu X, Zhang Y, Niu Y, Liu C, Ji J, Chillrud SN, Cai J, Chen R, Kan H
J Hazard Mater 2022 Aug 5;435:129031. Epub 2022 Apr 29 doi: 10.1016/j.jhazmat.2022.129031. PMID: 35523096
Herzog SA, Brakoulias V
Curr Med Chem 2022;29(35):5584-5594. doi: 10.2174/0929867329666211217094941. PMID: 34923935
do Nascimento Vinholes L, Sousa da Silva A, Marinho Tassi E, Corrêa Borges de Lacerda G
Acta Neurol Scand 2021 Jan;143(1):62-70. Epub 2020 Aug 25 doi: 10.1111/ane.13330. PMID: 32749695

Recent systematic reviews

McDonnell P, Fornell P, Ponce S, Dyer L
Birth Defects Res 2023 Mar 1;115(4):474-487. Epub 2022 Dec 14 doi: 10.1002/bdr2.2135. PMID: 36515170
Niu Z, Liu F, Li B, Li N, Yu H, Wang Y, Tang H, Chen X, Lu Y, Cheng Z, Liu S, Chen G, Zhang Y, Xiang H
Environ Health Prev Med 2020 Dec 1;25(1):77. doi: 10.1186/s12199-020-00912-2. PMID: 33261557Free PMC Article
Speer KE, Semple S, Naumovski N, McKune AJ
Physiol Behav 2020 Apr 1;217:112806. Epub 2020 Jan 15 doi: 10.1016/j.physbeh.2020.112806. PMID: 31954147
de Looff PC, Cornet LJM, Embregts PJCM, Nijman HLI, Didden HCM
PLoS One 2018;13(10):e0205741. Epub 2018 Oct 18 doi: 10.1371/journal.pone.0205741. PMID: 30335812Free PMC Article
da Silva VP, Ramalho Oliveira BR, Tavares Mello RG, Moraes H, Deslandes AC, Laks J
Curr Alzheimer Res 2018;15(1):80-88. doi: 10.2174/1567205014666170531082352. PMID: 28558638

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