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Chronic atrial and intestinal dysrhythmia
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Chronic atrial and intestinal dysrhythmia

Chronic atrial and intestinal dysrhythmia (CAID) is a rare genetic disorder that simultaneously disrupts the heart's rhythm and the digestive system's ability to move food.

Overview

Chronic atrial and intestinal dysrhythmia (CAID) is a disorder affecting the heart and the digestive system. CAID disrupts the heart's rhythm; affected individuals have a heart rhythm abnormality called sick sinus syndrome. The disorder also impairs the rhythmic muscle contractions that propel food through the intestines (peristalsis), causing a digestive condition called intestinal pseudo-obstruction. The heart and digestive issues develop at the same time, usually by age 20.

Symptoms

Heart Rhythm Abnormalities

Sick sinus syndrome (also known as sinus node dysfunction) is an abnormality of the sinoatrial (SA) node, the heart's natural pacemaker. Symptoms related to abnormal heartbeats can include:

  • Dizziness and light-headedness
  • Fainting (syncope)
  • A sensation of fluttering or pounding in the chest (palpitations)
  • Confusion or memory problems
  • Chest pain, difficulty breathing, or excessive tiredness (fatigue) during exercise

Digestive Issues

In intestinal pseudo-obstruction, impairment of peristalsis leads to a buildup of partially digested food in the intestines. Symptoms include:

  • Abdominal swelling (distention) and pain
  • Nausea and vomiting
  • Constipation or diarrhea
  • Loss of appetite and impaired ability to absorb nutrients, which may lead to malnutrition

Causes

CAID is caused by mutations in the SGO1 gene. This gene provides instructions for making part of a protein complex called cohesin, which helps control the placement of chromosomes during cell division. Researchers suggest that SGO1 gene mutations may result in a cohesin complex that is less able to hold sister chromatids together, resulting in decreased chromosomal stability. This instability is thought to cause early aging (senescence) of cells in the intestinal muscle and in the SA node, leading to the signs and symptoms of CAID.

Inheritance

This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

Frequently Asked Questions

Because sick sinus syndrome in CAID causes progressive, severe bradycardia (slow heart rate) and sinus pauses, most patients require the surgical implantation of a permanent cardiac pacemaker early in life, often before adulthood. Medications like antiarrhythmics must be used with extreme caution, as drugs that slow the heart rate can worsen sinus node failure, while anticholinergics used for the heart can severely worsen gut hypomotility.
Gastrointestinal management focuses on maintaining hydration and nutritional intake while minimizing intestinal stagnation. Initial strategies include consuming small, frequent, low-fat, and low-fiber meals, along with prokinetic agents to stimulate motility. If oral nutrition fails to sustain weight or triggers severe pain and vomiting, enteral tube feeding via a jejunostomy or total parenteral nutrition (TPN) through a central line becomes necessary. Decompression gastrostomy tubes are also frequently placed to vent trapped gas and fluid.
Immediate medical care is required for sudden episodes of fainting (syncope), rapid flutter or pounding in the chest, or severe lightheadedness, which can indicate pacemaker malfunction or dangerous atrial tachyarrhythmias. From a digestive standpoint, acute worsening of abdominal distension accompanied by intractable vomiting, cessation of gas passage, localized abdominal tenderness, or fever warrants urgent evaluation to rule out bowel ischemia, perforation, or central venous catheter-related sepsis.
CAID is caused by specific pathogenic variants in the SGO1 gene, which encodes a cohesin-associated protein essential for chromosomal stability. Because intestinal pseudo-obstruction can also occur in systemic sclerosis, mitochondrial disorders, or neurogenic intestinal dysmotilities, molecular genetic testing with targeted gene sequencing confirms the CAID diagnosis and directs clinicians to screen proactively for asymptomatic cardiac rhythm disturbances.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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