Familial atrial fibrillation: Difference between revisions

	Atrial Fibrillation Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Atrial Fibrillation from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Special Groups Postoperative AF Acute Myocardial Infarction Wolff-Parkinson-White Preexcitation Syndrome Hypertrophic Cardiomyopathy Hyperthyroidism Pulmonary Diseases Pregnancy ACS and/or PCI or valve intervention Heart failure
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram EKG Examples A-Fib with LBBB
Chest X Ray
Echocardiography
Holter Monitoring and Exercise Stress Testing
Cardiac MRI
Treatment
Rate and Rhythm Control
Cardioversion Overview Electrical Cardioversion Pharmacological Cardioversion
Anticoagulation Overview Warfarin Converting from or to Warfarin Converting from or to Parenteral Anticoagulants Dabigatran
Maintenance of Sinus Rhythm
Surgery Catheter Ablation AV Nodal Ablation Surgical Ablation Cardiac Surgery
Specific Patient Groups
Primary Prevention
Secondary Prevention
Supportive Trial Data
Cost-Effectiveness of Therapy
Case Studies
Case #1
Familial atrial fibrillation On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Familial atrial fibrillation
CDC on Familial atrial fibrillation
Familial atrial fibrillation in the news
Blogs on Familial atrial fibrillation
Directions to Hospitals Treating Familial atrial fibrillation
Risk calculators and risk factors for Familial atrial fibrillation

	Familial atrial fibrillation;
OMIM	608583
DiseasesDB	34356

VisualWikitext

Latest revision as of 02:47, 16 October 2011

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Familial atrial fibrillation is an inherited condition that disrupts the heart's normal rhythm. This condition is characterized by uncoordinated electrical activity in the heart's upper chambers (the atria), which causes the heartbeat to become fast and irregular.

Epidemiology and Demographics

Atrial fibrillation is the most common type of sustained abnormal heart rhythm (arrhythmia), affecting more than 3 million people in the United States.

The risk of developing this irregular heart rhythm increases with age.

The incidence of the familial form of atrial fibrillation is unknown; however, recent studies suggest that up to 30 percent of all people with atrial fibrillation may have a history of the condition in their family.

Cause

Familial atrial fibrillation has an autosomal dominant pattern of inheritance.

It is associated with multiple genes:

Type	OMIM	Gene	Locus
ATFB1	608583	?	10q22-q24
ATFB2	608988	?	6q
ATFB3	607554	KCNQ1	11
ATFB4	611493	KCNE2	21
ATFB5	611494	?	4q25
ATFB6	612201	NPPA	1p36-p35
ATFB7	612240	KCNA5	12p13
ATFB8	613055	?	16q22

Mutations in the KCNQ1 gene cause familial atrial fibrillation. The KCNE2 and KCNJ2 genes are associated with familial atrial fibrillation. A small percentage of all cases of familial atrial fibrillation are associated with changes in the KCNE2, KCNJ2, and KCNQ1 genes. These genes provide instructions for making proteins that act as channels across the cell membrane. These channels transport positively charged potassium ions into and out of cells. In heart muscle, the ion channels produced from the KCNE2, KCNJ2, and KCNQ1 genes play critical roles in maintaining the heart's normal rhythm. Mutations in these genes have been identified in only a few families worldwide. These mutations increase the activity of the channels, which changes the flow of potassium ions between cells. This disruption in ion transport alters the way the heart beats, increasing the risk of syncope, stroke, and sudden death.

Most cases of atrial fibrillation are not caused by mutations in a single gene. This condition is often related to structural abnormalities of the heart or underlying heart disease. Additional risk factors for atrial fibrillation include high blood pressure (hypertension), diabetes mellitus, a previous stroke, or an accumulation of fatty deposits and scar-like tissue in the lining of the arteries (atherosclerosis). Although most cases of atrial fibrillation are not known to run in families, studies suggest that they may arise partly from genetic risk factors. Researchers are working to determine which genetic changes may influence the risk of atrial fibrillation.

Familial atrial fibrillation appears to be inherited in an autosomal dominant pattern, which means the defective gene is located on an autosome, and only one copy of the defective gene - inherited from one parent - is sufficient to cause the disorder.

Diagnosis

Symptoms

The symptoms of familial atrial fibrillation include:

Dizziness,
Chest pain,
a sensation of fluttering or pounding in the chest (palpitations),
Shortness of breath,
fainting (Syncope).

Natural History

Atrial fibrillation also increases the risk of stroke and sudden death. Complications of familial atrial fibrillation can occur at any age, although some people with this heart condition never experience any health problems associated with the disorder.

Reference

National Library of Medicine. Familial atrial fibrillation

Template:WH Template:WikiDoc Sources

@@ Line 1: / Line 1: @@
-{{SI}}
+{{Infobox_Disease |
-{{WikiDoc Cardiology Network Infobox}}
+  Name           = Familial atrial fibrillation |
+  Image          = |
+  Caption        = |
+  DiseasesDB     = 34356 |
+  ICD10          = |
+  ICD9           = |
+  ICDO           = |
+  OMIM           = 608583 |
+  MedlinePlus    = |
+  eMedicineSubj  = |
+  eMedicineTopic = |
+  MeshID         = |
+}}
+{{Atrial fibrillation}}
 {{CMG}}
-{{EH}}
 ==Overview==
@@ Line 14: / Line 26: @@
 The incidence of the familial form of atrial fibrillation is unknown; however, recent studies suggest that up to 30 percent of all people with atrial fibrillation may have a history of the condition in their family.
+==Cause==
+[[Image:autodominant.jpg|thumb|right|Familial atrial fibrillation has an autosomal dominant pattern of inheritance.]]
+It is associated with multiple genes:
+{| class="wikitable"
+|-
+! Type
+! [[OMIM]]
+! Gene
+! Locus
+|-
+| ATFB1
+| {{OMIM2|608583}}
+| ?
+| 10q22-q24
+|-
+| ATFB2
+| {{OMIM2|608988}}
+| ?
+| 6q
+|-
+| ATFB3
+| {{OMIM2|607554}}
+| ''[[KCNQ1]]''
+| 11
+|-
+| ATFB4
+| {{OMIM2|611493}}
+| ''[[KCNE2]]''
+| 21
+|-
+| ATFB5
+| {{OMIM2|611494}}
+| ?
+| 4q25
+|-
+| ATFB6
+| {{OMIM2|612201}}
+| ''[[NPPA]]''
+| 1p36-p35
+|-
+| ATFB7
+| {{OMIM2|612240}}
+| ''[[KCNA5]]''
+| 12p13
+|-
+| ATFB8
+| {{OMIM2|613055}}
+| ?
+| 16q22
+|}
+Mutations in the ''[[KCNQ1]]'' [[gene]] cause familial atrial fibrillation. The ''[[KCNE2]]'' and ''[[KCNJ2]]'' genes are associated with familial atrial fibrillation. A small percentage of all cases of familial atrial fibrillation are associated with changes in the ''KCNE2'', ''KCNJ2'', and ''KCNQ1'' genes. These genes provide instructions for making proteins that act as channels across the cell membrane. These channels transport positively charged [[potassium]] ions into and out of cells. In heart muscle, the ion channels produced from the ''KCNE2'', ''KCNJ2'', and ''KCNQ1'' genes play critical roles in maintaining the heart's normal rhythm. Mutations in these genes have been identified in only a few families worldwide. These mutations increase the activity of the channels, which changes the flow of potassium ions between cells. This disruption in ion transport alters the way the heart beats, increasing the risk of syncope, stroke, and sudden death.
+Most cases of atrial fibrillation are not caused by mutations in a single gene. This condition is often related to structural abnormalities of the heart or underlying heart disease. Additional risk factors for atrial fibrillation include high blood pressure ([[hypertension]]), [[diabetes mellitus]], a previous stroke, or an accumulation of fatty deposits and scar-like tissue in the lining of the arteries ([[atherosclerosis]]). Although most cases of atrial fibrillation are not known to run in families, studies suggest that they may arise partly from genetic risk factors. Researchers are working to determine which genetic changes may influence the risk of atrial fibrillation.
+Familial atrial fibrillation appears to be inherited in an [[autosomal dominant]] pattern, which means the defective gene is located on an [[autosome]], and only one copy of the defective gene - inherited from one parent - is sufficient to cause the disorder.
 ==Diagnosis==
@@ Line 28: / Line 100: @@
 ==Natural History==
 Atrial fibrillation also increases the risk of [[stroke]] and sudden death. Complications of familial atrial fibrillation can occur at any age, although some people with this heart condition never experience any health problems associated with the disorder.
-==Genetics==
-Mutations in the ''KCNQ1'' [[gene]] cause familial atrial fibrillation. The ''KCNE2'' and ''KCNJ2'' genes are associated with familial atrial fibrillation. A small percentage of all cases of familial atrial fibrillation are associated with changes in the ''KCNE2'', ''KCNJ2'', and ''KCNQ1'' genes.
-These genes provide instructions for making proteins that act as channels across the cell membrane. These channels transport positively charged [[potassium]] ions into and out of cells. In heart muscle, the ion channels produced from the ''KCNE2'', ''KCNJ2'', and ''KCNQ1'' genes play critical roles in maintaining the heart's normal rhythm. Mutations in these genes have been identified in only a few families worldwide.
-These mutations increase the activity of the channels, which changes the flow of potassium ions between cells. This disruption in ion transport alters the way the heart beats, increasing the risk of syncope, stroke, and sudden death.
-Most cases of atrial fibrillation are not caused by mutations in a single gene. This condition is often related to structural abnormalities of the heart or underlying heart disease.
-Additional risk factors for atrial fibrillation include high blood pressure ([[hypertension]]), [[diabetes mellitus]], a previous stroke, or an accumulation of fatty deposits and scar-like tissue in the lining of the arteries ([[atherosclerosis]]).
-Although most cases of atrial fibrillation are not known to run in families, studies suggest that they may arise partly from genetic risk factors. Researchers are working to determine which genetic changes may influence the risk of atrial fibrillation.
-Familial atrial fibrillation appears to be inherited in an [[autosomal dominant]] pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.
 ==Reference==
@@ Line 48: / Line 105: @@
 {{Electrocardiography}}
-{{Circulatory system pathology}}
-{{SIB}}
 [[category:genetic disorders]]

v t e Electrocardiography
Overview	History of the EKG • EKG interpretation basics • Normal sinus rhythm
EKG Complexes	P wave • QRS complex • ST Segment • T wave • T wave alternans • Tombstone T wave • U wave Osborn wave • H wave • K wave • Delta wave NSSTW changes
EKG Intervals	PR Interval • QRS Interval • ST Interval • QT Interval
Conduction System & Bradycardia	Cardiac pacemaker • SA node • AV node• Bundle of His • Purkinje fibers • Sinus bradycardia • First Degree AV Block • Second Degree AV Block • Complete or Third-Degree AV Block • Concealed conduction • AV Junctional Rhythms • LBBB • LAHB • LPHB • RBBB • Trifascicular block
Atrial Arrhythmias	Sinus tachycardia • Premature Atrial Contractions (PACs) • Ectopic Atrial Rhythm • Paroxysmal Atrial Tachycardia (PAT) • Paroxysmal Atrial Tachycardia (PAT) with Block • Multifocal Atrial Tachycardia (MAT) • Atrial Flutter • Atrial Fibrillation • Wandering atrial pacemaker
Ventricular Arrhythmias	Differential Diagnosis of Tachycardia with a Wide QRS Complex • Accelerated Idioventricular Rhythm • Ventricular Parasystole • Premature Ventricular Contractions (PVCs) • Ventricular tachycardia • Ventricular Fibrillation • Sudden cardiac death
EKG Abnormalities in Disease States	Hypertrophy & Dilatation • Right atrial enlargement • Left atrial enlargement • Biatrial enlargement • Left Ventricular Hypertrophy • Right Ventricular Hypertrophy • Biventricular Hypertrophy • Acute myocardial infarction • NSTEMI • STEMI • Tombstone ST elevation • Right ventricular myocardial infarction • Atrial infarction Pre-excitation Syndromes • Wolff-Parkinson-White Syndrome • Lown Ganong Levine Syndrome • Mahaim Type Preexcitation Cardiomyopathies • Arrhythmogenic Right Ventricular Dysplasia • Dilated Cardiomyopathy • Hypertrophic Cardiomyopathy Drug Effects on the EKG • Adenosine • β-blockers • Digitalis • Quinidine • Procainamide • Disopyramide • Lidocaine • Tocainide and Mexiletine • Phenytoin • Encainide, Flecainide and Propafenone • Amiodarone • Bretylium • Ca Channel Blockers • Phenothiazines • Tricyclic Antidepressants • Lithium Congenital Heart Disease • Dextrocardia • Atrial Septal Defect • Ventricular Septal Defect • Tetralogy of Fallot • Conjoined Twins or Siamese Twins • Congenital heart block Electrolyte Disturbances • Hyperkalemia • Hypokalemia • Hypercalcemia • Hypocalcemia • Nonspecific Changes Other Heart Diseases • Pericarditis • Myocarditis • Tamponade • Heart Transplantation • Sick Sinus Syndrome • Long QT Syndrome Inherited Disease • Brugada Syndrome Systemic Diseases • CNS Disease • Cardiac Tumors Heart Transplantation • EKG Changes in patient with Heart Transplantation Exogenous Effects • Hypothermia • Chest Trauma • Insect Bites • Electric Injuries
Technical Issues and Potential Errors in Interpretation	Artifacts • Lead Placement Errors • The EKG in a Patient with a Pacemaker • EKG in athletes