Cor triatriatum: Difference between revisions
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==Background== | ==Background== | ||
*Congenital | [[File:Cor triatriatum.jpg|thumb|XC: anomalous upper chamber]] | ||
*Congenital anomaly: heart with three atria | |||
*Either LA or RA can be divided into 2 compartments (cor triatriatum sinistrum or cor triatriatum dextrum) | *Either LA or RA can be divided into 2 compartments (cor triatriatum sinistrum or cor triatriatum dextrum) | ||
*Can be associated with other congenital syndromes including tetralogy of Fallot, double outlet RV, coarctation of aorta, VSD, AV septal defect | *Can be associated with other congenital syndromes including [[tetralogy of Fallot]], double outlet RV, [[coarctation of the aorta]], [[VSD]], AV septal defect | ||
*0.1-0.4% incidence in USA | *0.1-0.4% incidence in USA | ||
==Clinical Features== | ==Clinical Features== | ||
*Sinistrum variant most associated with other congenital cardiovascular defects in symptomatic infants | *Sinistrum variant most associated with other congenital cardiovascular defects in symptomatic infants | ||
*Can be incidental finding in adults as either isolated finding or in association with PFO, ASD, persistent | *Can be incidental finding in adults as either isolated finding or in association with PFO, ASD, persistent left superior vena cava | ||
*Symptoms depend on size of opening in accessory membrane between the atria | *Symptoms depend on size of opening in accessory membrane between the atria | ||
*Symptoms include dyspnea on exertion, orthopnea, easy fatiguability, low exercise tolerance, palpitations | *Symptoms include [[dyspnea]] on exertion, orthopnea, easy fatiguability, low exercise tolerance, [[palpitations]] | ||
*Increased risk of thrombus formation in LA | *Increased risk of thrombus formation in LA | ||
*[[Murmur]] | |||
*Elevated JVD | |||
*[[Hepatomegaly]] | |||
*[[Ascites]] | |||
*Peripheral edema | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Pericarditis]] | |||
*[[Pulmonary Hypertension]] | |||
*Right [[heart failure]] | |||
*Atrial Myxoma | |||
*[[Mitral Stenosis]] | |||
*Tricuspid Stenosis | |||
== | {{Congenital heart disease DDX}} | ||
* | |||
* | ==Evaluation== | ||
* | *Initial workup may include: | ||
* | **[[ECG]]: Atrial premature complexes, Left or Right atrial abnormalities, Right axis deviation, Right heart strain | ||
* | **[[CXR]] | ||
* | **[[Echocardiography]] | ||
**Right heart cath | |||
==Management== | ==Management== | ||
*As | *As associated with major cyanotic or acyanotic congenital heart lesions, mortality can be up to 75% in untreated symptomatic infants | ||
*Treat increased pulmonary vascular resistance, [[heart failure]] | |||
*Inotropic agents (eg, [[dopamine]], [[dobutamine]]), [[diuretics]] | |||
*Manage [[hypoxia]], fluid status, rate in [[afib]] | |||
*[[DVT]]/PE prophylaxis | |||
**Cardiac surgery consultation | |||
==Disposition== | ==Disposition== | ||
==See Also== | ==See Also== | ||
*[[Congenital heart disease]] | |||
==External Links== | ==External Links== | ||
==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:Pediatrics]] | |||
[[Category:Cardiology]] | |||
Latest revision as of 12:45, 14 May 2022
Background
- Congenital anomaly: heart with three atria
- Either LA or RA can be divided into 2 compartments (cor triatriatum sinistrum or cor triatriatum dextrum)
- Can be associated with other congenital syndromes including tetralogy of Fallot, double outlet RV, coarctation of the aorta, VSD, AV septal defect
- 0.1-0.4% incidence in USA
Clinical Features
- Sinistrum variant most associated with other congenital cardiovascular defects in symptomatic infants
- Can be incidental finding in adults as either isolated finding or in association with PFO, ASD, persistent left superior vena cava
- Symptoms depend on size of opening in accessory membrane between the atria
- Symptoms include dyspnea on exertion, orthopnea, easy fatiguability, low exercise tolerance, palpitations
- Increased risk of thrombus formation in LA
- Murmur
- Elevated JVD
- Hepatomegaly
- Ascites
- Peripheral edema
Differential Diagnosis
- Pericarditis
- Pulmonary Hypertension
- Right heart failure
- Atrial Myxoma
- Mitral Stenosis
- Tricuspid Stenosis
Congenital Heart Disease Types
- Cyanotic
- Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
- Differentiation by pulmonary vascularity on CXR[1]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Evaluation
- Initial workup may include:
- ECG: Atrial premature complexes, Left or Right atrial abnormalities, Right axis deviation, Right heart strain
- CXR
- Echocardiography
- Right heart cath
Management
- As associated with major cyanotic or acyanotic congenital heart lesions, mortality can be up to 75% in untreated symptomatic infants
- Treat increased pulmonary vascular resistance, heart failure
- Inotropic agents (eg, dopamine, dobutamine), diuretics
- Manage hypoxia, fluid status, rate in afib
- DVT/PE prophylaxis
- Cardiac surgery consultation
Disposition
See Also
External Links
References
- ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease