U.S. flag

An official website of the United States government


Send to:

Choose Destination

Chronic atrial and intestinal dysrhythmia(CAID)

MedGen UID:
Concept ID:
Disease or Syndrome
Synonym: CAID
SNOMED CT: Chronic atrial and intestinal dysrhythmia (720507006); CAID (chronic atrial and intestinal dysrhythmia) syndrome (720507006)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
Concept ID:
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
Gene (location): SGO1 (3p24.3)
Monarch Initiative: MONDO:0014528
OMIM®: 616201
Orphanet: ORPHA435988


Syndrome with characteristics of sick sinus syndrome and intestinal pseudo-obstruction. The heart and digestive issues develop at the same time, usually by age 20. The syndrome is caused by mutations in the SGO1 gene. This gene provides instructions for making part of a protein complex cohesin. This protein complex helps control the placement of chromosomes during cell division. Research suggests that SGO1 gene mutations may result in a cohesin complex that is less able to hold sister chromatids together, resulting in decreased chromosomal stability during cell division. This instability is thought to cause senescence of cells in the intestinal muscle and in the sinoatrial node, resulting in problems maintaining proper rhythmic movements of the heart and intestines. [from SNOMEDCT_US]

Clinical features

From HPO
Atrial fibrillation
MedGen UID:
Concept ID:
An atrial arrhythmia characterized by disorganized atrial activity without discrete P waves on the surface EKG, but instead by an undulating baseline or more sharply circumscribed atrial deflections of varying amplitude an frequency ranging from 350 to 600 per minute.
Atrial flutter
MedGen UID:
Concept ID:
Pathologic Function
A type of atrial arrhythmia characterized by atrial rates of between 240 and 400 beats per minute and some degree of atrioventricular node conduction block. Typically, the ventricular rate is half the atrial rate. In the EKG; atrial flutter waves are observed as sawtooth-like atrial activity. Pathophysiologically, atrial flutter is a form of atrial reentry in which there is a premature electrical impulse creates a self-propagating circuit.
Mitral regurgitation
MedGen UID:
Concept ID:
Disease or Syndrome
An abnormality of the mitral valve characterized by insufficiency or incompetence of the mitral valve resulting in retrograde leaking of blood through the mitral valve upon ventricular contraction.
Sick sinus syndrome
MedGen UID:
Concept ID:
Disease or Syndrome
An abnormality involving the generation of the action potential by the sinus node and is characterized by an atrial rate inappropriate for physiological requirements. Manifestations include severe sinus bradycardia, sinus pauses or arrest, sinus node exit block, chronic atrial tachyarrhythmias, alternating periods of atrial bradyarrhythmias and tachyarrhythmias, and inappropriate responses of heart rate during exercise or stress.
Bicuspid aortic valve
MedGen UID:
Concept ID:
Congenital Abnormality
Aortic valve disease-2 (AOVD2) is characterized by bicuspid aortic valve (BAV) and dilation of the ascending aorta. Calcification of the valve and the aorta has been observed, and some patients exhibit coarctation of the aorta (Tan et al., 2012; Luyckx et al., 2019; Park et al., 2019). For a general phenotypic description and a discussion of genetic heterogeneity of aortic valve disease, see AOVD1 (109730).
MedGen UID:
Concept ID:
A slower than normal heart rate (in adults, slower than 60 beats per minute).
Pulmonic stenosis
MedGen UID:
Concept ID:
Disease or Syndrome
A narrowing of the right ventricular outflow tract that can occur at the pulmonary valve (valvular stenosis), below the pulmonary valve (infundibular stenosis), or above the pulmonary valve (supravalvar stenosis).
Decreased body weight
MedGen UID:
Concept ID:
Abnormally low body weight.
Failure to thrive
MedGen UID:
Concept ID:
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChronic atrial and intestinal dysrhythmia

Recent clinical studies

Clinical prediction guides

Piché J, Gosset N, Legault LM, Pacis A, Oneglia A, Caron M, Chetaille P, Barreiro L, Liu D, Qi X, Nattel S, Leclerc S, Breton-Larrivée M; CoHEART Consortium, McGraw S, Andelfinger G
Cell Mol Gastroenterol Hepatol 2019;7(2):411-431. Epub 2018 Oct 24 doi: 10.1016/j.jcmgh.2018.10.011. PMID: 30739867Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...