fbpx
Wikipedia

Cor triatriatum

April 14, 2024

Cor triatriatum (or triatrial heart)[1] is a congenital heart defect where the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is subdivided by a thin membrane, resulting in three atrial chambers (hence the name).

Cor triatriatum
Other namesTriatrial heart
SpecialtyCardiology

Cor triatriatum represents 0.1% of all congenital cardiac malformations and may be associated with other cardiac defects in as many as 50% of cases. The membrane may be complete or may contain one or more fenestrations of varying size.

Cor triatriatum sinistrum is more common.[2] In this defect, there is typically a proximal chamber that receives the pulmonic veins and a distal (true) chamber located more anteriorly where it empties into the mitral valve. The membrane that separates the atrium into two parts varies significantly in size and shape. It may appear similar to a diaphragm or be funnel-shaped, band-like, entirely intact (imperforate) or contain one or more openings (fenestrations) ranging from small, restrictive-type to large and widely open.

In the pediatric population, this anomaly may be associated with major congenital cardiac lesions such as tetralogy of Fallot, double outlet right ventricle, coarctation of the aorta, partial anomalous pulmonary venous connection, persistent left superior vena cava with unroofed coronary sinus, ventricular septal defect, atrioventricular septal (endocardial cushion) defect, and common atrioventricular canal. Rarely, asplenia or polysplenia has been reported in these patients. In the adult, cor triatriatum is frequently an isolated finding.

Cause edit

Cor triatriatum dextrum is extremely rare and results from the complete persistence of the right sinus valve of the embryonic heart. The membrane divides the right atrium into a proximal (upper) and a distal (lower) chamber. The upper chamber receives the venous blood from both vena cavae and the lower chamber is in contact with the tricuspid valve and the right atrial appendage.

Mechanism and Symptoms edit

The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chambers. If the communicating orifice is small, the patient is critically ill and may succumb at a young age (usually during infancy) to congestive heart failure and pulmonary edema.[3] If the connection is larger, patients may present in childhood or young adulthood with a clinical picture similar to that of mitral stenosis. As the malformed membrane calcifies with age, thus further narrowing such opening, decreased cardiac output produces features of pulmonary venous hypertension and right heart failure—including symptoms of dyspnea and orthopnea, easy fatigability, palpitations and shortness of breath, among others.[3] Cor triatriatum may also be an incidental finding when it is nonobstructive.

Diagnosis edit

Primarily diagnosed with imaging, such as echocardiogram (ultrasound of the heart), CT, and/or MRI.[4]

Treatment edit

Treatment of Cor triatriatum varies among cases and is dependent upon presentation of symptoms—incidental finding of the condition in asymptomatic patients does not typically require immediate medical management, but for those exhibiting dyspnea and pulmonary congestion, surgical intervention is required. The disorder can be treated surgically by removing the membrane dividing the atrium. The surgery, which usually occurs by first excising the diaphragm and then closing the atrial septum, has a reported survival of 90% at five years, with almost all patients becoming asymptomatic post-surgery.

References edit

  1. ^ Ather, Binish; Meredith, Amanda; Siddiqui, Waqas J. (2023), "Cor Triatriatum", StatPearls, Treasure Island (FL): StatPearls Publishing, PMID 30480976, retrieved 2024-01-11
  2. ^ Ullah, Waqas; Sattar, Yasar; Rauf, Hiba; Roomi, Sohaib; Shah, Murtaza (13 December 2019). "A systematic review of a long-forgotten cause of atrial fibrillation and stroke: cor triatriatum". Cureus. 11 (12): e6371. doi:10.7759/cureus.6371. PMC 6957057. PMID 31938652.
  3. ^ a b "Cor Triatriatum - Symptoms, Causes, Treatment | NORD". rarediseases.org. Retrieved 2024-01-11.
  4. ^ "Cor Triatriatum - Symptoms, Causes, Treatment | NORD". rarediseases.org. Retrieved 2024-01-11.
  • Ather B, Meredith A, Siddiqui WJ. Cor Triatriatum. [Updated 2022 Sep 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK534243/
  • Church WS. Congenital malformation of heart: abnormal septum in left auricle. Trans Path Soz. 1868;19:188-190.
  • Griffith TW. Note on a Second Example of Division of the Cavity of the Left Auricle into Two Compartments by a Fibrous Band. J Anat Physiol. Apr 1903;37:255-7. [Medline].
  • Anderson RH. Understanding the nature of congenital division of the atrial chambers. Br Heart J. Jul 1992;68(1):1-3. [Medline].
  • Richardson JV, Doty DB, Siewers RD, et al. Cor triatriatum (subdivided left atrium). J Thorac Cardiovasc Surg. Feb 1981;81(2):232-8. [Medline].
  • Trento A, Zuberbuhler JR, Anderson RH, et al. Divided right atrium (prominence of the eustachian and thebesian valves). J Thorac Cardiovasc Surg. Sep 1988;96(3):457-63. [Medline].
  • Marin-Garcia J, Tandon R, Lucas RV Jr, et al. Cor triatriatum: study of 20 cases. Am J Cardiol. Jan 1975;35(1):59-66. [Medline].
  • Niwayama G. Cor triatriatum. Am Heart J. Feb 1960;59:291-317. [Medline].
  • Nassar PN, Hamdan RH. Cor Triatriatum Sinistrum: Classification and Imaging Modalities. Eur J Cardiovasc Med. 2011 Jan;1(3):84-87. doi: 10.5083/ejcm.20424884.21. PMID 22379596; PMCID: PMC3286827.
  • Jennings RB Jr, Innes BJ. Subtotal cor triatriatum with left partial anomalous pulmonary venous return. Successful surgical repair in an infant. J Thorac Cardiovasc Surg. Sep 1977;74(3):461-6. [Medline].
  • Tuccillo B, Stümper O, Hess J, et al. Transoesophageal echocardiographic evaluation of atrial morphology in children with congenital heart disease. Eur Heart J. Feb 1992;13(2):223-31. [Medline].
  • Wolf WJ. Diagnostic features and pitfalls in the two-dimensional echocardiographic evaluation of a child with cor triatriatum. Pediatr Cardiol. 1986;6(4):211-3. [Medline].
  • Beller B, Childers R, Eckner F, et al. Cor triatriatum in the adult. Complicated by mitral insufficiency and aortic dissection. Am J Cardiol. May 1967;19(5):749-54. [Medline].

External links edit

  • long description at emedicine

April 14, 2024
triatriatum, this, article, includes, list, general, references, lacks, sufficient, corresponding, inline, citations, please, help, improve, this, article, introducing, more, precise, citations, december, 2017, learn, when, remove, this, template, message, tri. This article includes a list of general references but it lacks sufficient corresponding inline citations Please help to improve this article by introducing more precise citations December 2017 Learn how and when to remove this template message Cor triatriatum or triatrial heart 1 is a congenital heart defect where the left atrium cor triatriatum sinistrum or right atrium cor triatriatum dextrum is subdivided by a thin membrane resulting in three atrial chambers hence the name Cor triatriatumOther namesTriatrial heartSpecialtyCardiologyCor triatriatum represents 0 1 of all congenital cardiac malformations and may be associated with other cardiac defects in as many as 50 of cases The membrane may be complete or may contain one or more fenestrations of varying size Cor triatriatum sinistrum is more common 2 In this defect there is typically a proximal chamber that receives the pulmonic veins and a distal true chamber located more anteriorly where it empties into the mitral valve The membrane that separates the atrium into two parts varies significantly in size and shape It may appear similar to a diaphragm or be funnel shaped band like entirely intact imperforate or contain one or more openings fenestrations ranging from small restrictive type to large and widely open In the pediatric population this anomaly may be associated with major congenital cardiac lesions such as tetralogy of Fallot double outlet right ventricle coarctation of the aorta partial anomalous pulmonary venous connection persistent left superior vena cava with unroofed coronary sinus ventricular septal defect atrioventricular septal endocardial cushion defect and common atrioventricular canal Rarely asplenia or polysplenia has been reported in these patients In the adult cor triatriatum is frequently an isolated finding Contents 1 Cause 2 Mechanism and Symptoms 3 Diagnosis 4 Treatment 5 References 6 External linksCause editCor triatriatum dextrum is extremely rare and results from the complete persistence of the right sinus valve of the embryonic heart The membrane divides the right atrium into a proximal upper and a distal lower chamber The upper chamber receives the venous blood from both vena cavae and the lower chamber is in contact with the tricuspid valve and the right atrial appendage Mechanism and Symptoms editThe natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chambers If the communicating orifice is small the patient is critically ill and may succumb at a young age usually during infancy to congestive heart failure and pulmonary edema 3 If the connection is larger patients may present in childhood or young adulthood with a clinical picture similar to that of mitral stenosis As the malformed membrane calcifies with age thus further narrowing such opening decreased cardiac output produces features of pulmonary venous hypertension and right heart failure including symptoms of dyspnea and orthopnea easy fatigability palpitations and shortness of breath among others 3 Cor triatriatum may also be an incidental finding when it is nonobstructive Diagnosis editPrimarily diagnosed with imaging such as echocardiogram ultrasound of the heart CT and or MRI 4 Treatment editTreatment of Cor triatriatum varies among cases and is dependent upon presentation of symptoms incidental finding of the condition in asymptomatic patients does not typically require immediate medical management but for those exhibiting dyspnea and pulmonary congestion surgical intervention is required The disorder can be treated surgically by removing the membrane dividing the atrium The surgery which usually occurs by first excising the diaphragm and then closing the atrial septum has a reported survival of 90 at five years with almost all patients becoming asymptomatic post surgery References edit Ather Binish Meredith Amanda Siddiqui Waqas J 2023 Cor Triatriatum StatPearls Treasure Island FL StatPearls Publishing PMID 30480976 retrieved 2024 01 11 Ullah Waqas Sattar Yasar Rauf Hiba Roomi Sohaib Shah Murtaza 13 December 2019 A systematic review of a long forgotten cause of atrial fibrillation and stroke cor triatriatum Cureus 11 12 e6371 doi 10 7759 cureus 6371 PMC 6957057 PMID 31938652 a b Cor Triatriatum Symptoms Causes Treatment NORD rarediseases org Retrieved 2024 01 11 Cor Triatriatum Symptoms Causes Treatment NORD rarediseases org Retrieved 2024 01 11 Ather B Meredith A Siddiqui WJ Cor Triatriatum Updated 2022 Sep 19 In StatPearls Internet Treasure Island FL StatPearls Publishing 2023 Jan Available from https www ncbi nlm nih gov books NBK534243 Church WS Congenital malformation of heart abnormal septum in left auricle Trans Path Soz 1868 19 188 190 Griffith TW Note on a Second Example of Division of the Cavity of the Left Auricle into Two Compartments by a Fibrous Band J Anat Physiol Apr 1903 37 255 7 Medline Anderson RH Understanding the nature of congenital division of the atrial chambers Br Heart J Jul 1992 68 1 1 3 Medline Richardson JV Doty DB Siewers RD et al Cor triatriatum subdivided left atrium J Thorac Cardiovasc Surg Feb 1981 81 2 232 8 Medline Trento A Zuberbuhler JR Anderson RH et al Divided right atrium prominence of the eustachian and thebesian valves J Thorac Cardiovasc Surg Sep 1988 96 3 457 63 Medline Marin Garcia J Tandon R Lucas RV Jr et al Cor triatriatum study of 20 cases Am J Cardiol Jan 1975 35 1 59 66 Medline Niwayama G Cor triatriatum Am Heart J Feb 1960 59 291 317 Medline Nassar PN Hamdan RH Cor Triatriatum Sinistrum Classification and Imaging Modalities Eur J Cardiovasc Med 2011 Jan 1 3 84 87 doi 10 5083 ejcm 20424884 21 PMID 22379596 PMCID PMC3286827 Jennings RB Jr Innes BJ Subtotal cor triatriatum with left partial anomalous pulmonary venous return Successful surgical repair in an infant J Thorac Cardiovasc Surg Sep 1977 74 3 461 6 Medline Tuccillo B Stumper O Hess J et al Transoesophageal echocardiographic evaluation of atrial morphology in children with congenital heart disease Eur Heart J Feb 1992 13 2 223 31 Medline Wolf WJ Diagnostic features and pitfalls in the two dimensional echocardiographic evaluation of a child with cor triatriatum Pediatr Cardiol 1986 6 4 211 3 Medline Beller B Childers R Eckner F et al Cor triatriatum in the adult Complicated by mitral insufficiency and aortic dissection Am J Cardiol May 1967 19 5 749 54 Medline External links editlong description at emedicine Retrieved from https en wikipedia org w index php title Cor triatriatum amp oldid 1195719074, wikipedia, wiki, book, books, library,

article

, read, download, free, free download, mp3, video, mp4, 3gp, jpg, jpeg, gif, png, picture, music, song, movie, book, game, games.