Cor Triatriatum


Article Author:
Binish Ather


Article Editor:
Waqas Siddiqui


Editors In Chief:
Eric Flake


Managing Editors:
Carrie Smith
Abdul Waheed
Frank Smeeks
Kristina Soman-Faulkner
Scott Dulebohn
Sobhan Daneshfar
William Gossman
Pritesh Sheth
John Shell
Matthew Varacallo
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Richard Ciresi
Hajira Basit
Phillip Hynes


Updated:
11/18/2018 3:09:28 PM

Introduction

Cor triatriatum is a rare condition occurring when a child is born with a thin, fibro-muscular membrane subdividing either the left or the right atrium into 3 chambers. The condition is also classified as a congenital heart defect. Cor triatriatum sinister is the most common form. The left atrium divides via an atrial appendage into an upper and a lower chamber. The upper chamber receives blood from the pulmonary veins, while the lower chamber is attached to the left atrial appendage blocking the mitral valve orifice creating a significant left ventricular inflow obstruction. The presence of the left atrial appendage differentiates cor triatriatum from another congenital heart defect, supravalvular mitral stenosis.[1]

Another, rarer form of cor triatriatum is cor triatriatum dextrum. With cor triatriatum dextrum the right valve of the sinus venosus persists dividing right atrium into 2 chambers. This form presents similarly to Ebstein's anomaly and is difficult to differentiate.

Etiology

Cor triatriatum results from the incomplete absorption of the common pulmonary vein, which is normally reabsorbed during the development of a fetus and becomes a part of the left atrium. The incomplete absorption results in the formation of an appendage which subdivides the left atrium into 2 chambers. No known genetic mutations or risk factors are found to be associated with the development of this condition.

Epidemiology

Cor triatriatum presents in association with other congenital cardiac defects such as tetralogy of Fallot, atrial septal defect, ventricular septal defect partial anomalous pulmonary venous connection, and represents only 0.1% to 0.4% of all congenital abnormalities.[2] The membrane may be complete or may contain one or more fenestrations of differing size.

Pathophysiology

A malincorporation theory[3] presented by Dr. Clifford G. Parsons explains how cor triatriatum sinister occurs. During normal fetal development, the pulmonary vein incorporates into the left atrium. If it fails to do so, the common pulmonary venous ostium remains narrow, resulting in a septum-like structure called the atrial appendage. The appendage then further divides the left atrium into 2 compartments. Although widely accepted, this theory fails to explain how fossa ovalis and atrial muscle fibers are also present in the proximal atrial chamber.[4]

Two other theories, malseptation theory and entrapment theory, also explain the pathophysiology of cor triatriatum. The malseptation theory states that the fibro-muscular membrane is an abnormal growth of the septum primum; whereas, entrapment theory emphasizes the entrapment of the common pulmonary vein in the embryonic sinus venosus, thereby preventing its incorporation into the left atrium. The malincorporation theory remains the most widely accepted theory.

History and Physical

Classically, cor triatriatum presents in infancy with signs and symptom of pulmonary hypertension and pulmonary venous obstruction. Due to low cardiac output, children can show poor growth and weight gain, feeding difficulties, respiratory distress, and tet spells. In childhood and adulthood, the signs and symptoms of pulmonary venous hypertension and right heart failure dominate as the membrane calcifies, and the opening becomes smaller and smaller decreasing cardiac output even further. Mitral valve regurgitation and atrial fibrillation impose serious dangers. Most patients present with the following features[5]:

  • Dyspnea and orthopnea
  • Easy fatigability
  • Hemoptysis
  • Exercise intolerance and shortness of breath
  • Palpitations 

Atrial fibrillation can cause systemic thromboembolism and present as pulmonary embolism or stroke. The left atrial enlarges due to backing up of blood and can present as life-threatening arrhythmias.

Cor triatriatum sinister that presents for the first time in adulthood is rare but possible. It presents similarly to mitral stenosis, but the absence of loud S1 and an opening snap helps to distinguish between the 2. Chest x-ray showing pulmonary congestion with an absence of left atrial enlargement is characteristic of cor triatriatum. A continuous gradient on Doppler echocardiography confirms the diagnosis.[4][6]

Physical Exam

The physical exam findings are due to the right heart failure and pulmonary congestion.

  • Accentuated pulmonary component of second heart sound
  • A soft mid-systolic murmur at the upper left sternal border with a wide and fixed splitting of an atrial septal defect.
  • Right ventricular heave
  • Rales at the lung base
  • Diminished peripheral pulses
  • Hepatomegaly and right upper quadrant tenderness
  • Ascites
  • Peripheral edema
  • Distended peripheral veins
  • Distended jugular venous and elevated jugular venous pressure
  • Pallor
  • Poor weight gain

Evaluation

The mainstay of evaluation and diagnosis includes imaging studies such as chest x-ray, ECG and echocardiography, angiography and left and right heart catheterization.[7][8]

Chest X-Ray

A chest x-ray is the initial investigation of choice. The findings include:

  • Pulmonary congestion with haziness (Kerley B-lines)
  • Ground glass appearance of acute pulmonary edema
  • Prominent pulmonary vessels
  • Pleural effusion
  • Left atrial enlargement
  • Cardiomegaly

Electrocardiography (ECG)

ECG findings are non-specific in cor triatriatum. Can range from atrial fibrillation and no specific P-wave changes to right axis deviation due to pulmonary congestion and right ventricular hypertrophy.

Echocardiography

Echocardiography is the diagnostic modality of choice as it not only allows for definitive diagnosis, but the 3-dimensional reconstruction of echocardiographic images pinpoints the exact location of the defect and appendage, helps direct the surgical approach to the disease. The left atrial appendage and its fenestrations can be easily visualized along with the presence of an atrial septal defect, pulmonary stenosis, mitral valve stenosis, or regurgitation. Pulmonary arterial and venous drainage patterns can also be seen on echocardiography. Echocardiography differentiates between cor triatriatum and supravalvular mitral stenosis by clear visualization of the left atrial appendage in the left atrium.

Transesophageal echocardiography outlines the precise nature and anatomy of the defect; especially in older patients, but if unavailable, transthoracic echocardiography can be performed. [9][10]

Angiography

Angiography helps determine the severity of obstruction and the time of surgical intervention needed.

Treatment / Management

Medical Management

Asymptomatic Patients

Asymptomatic patients need no specific treatment. Observe the patients for the development of signs and symptoms. Schedule regular medical follow-ups.

Symptomatic Patients

Treatment option for symptomatic patients includes both medical/conservative management and surgical repair. Medical treatment includes:

  • Hemodynamic stabilization of fluid overload and pulmonary edema
  • Rate and rhythm control and anticoagulation for patients with atrial fibrillation
  • Thromboembolic prophylaxis with anticoagulation against deep vein thrombosis, pulmonary embolism and stroke
  • Obtain surgical consultation

Surgical Management

Surgery is the definitive treatment. Complete surgical resection of atrial appendage/accessory membrane through a midline sternotomy under cardiopulmonary bypass and closure of atrial septum with a pericardial patch provides the optimum cure. The 10-year survival rate following surgery is 83%, while patients with coexisting congenital heart diseases have a greater risk of adverse outcomes and a lower survival rate.[11][12]

Differential Diagnosis

Many congenital heart diseases can present similarly to cor triatriatum and should be taken into consideration when making a diagnosis of this disorder. These include:

  • Supravalvular mitral stenosis
  • Mitral stenosis
  • Idiopathic pediatric pulmonary arterial hypertension
  • Pulmonary venous hypertension
  • Total anomalous pulmonary venous return
  • Partial anomalous pulmonary venous return
  • Atrial septal defect/ ventricular septal defect
  • Ventricular septal defect
  • Idiopathic pulmonary hypertension
  • Tricuspid stenosis
  • Atrial myxoma

An experienced surgical team should evaluate imaging results as high suspicion can accelerate diagnosis and delay or prevent unfavorable outcomes.

Complications

  • Right-sided failure
  • Pulmonary edema/hypertension
  • Atrial arrhythmias
  • Death

Deterrence and Patient Education

  • Pediatric cardiologist
  • Pediatric cardiac surgeon
  • Intensivist

Enhancing Healthcare Team Outcomes

Diagnosing and treating cor-triatriatum requires an interprofessional approach.

Cor-triatriatum is a very rare congenital heart disorder. However, most infants are symptomatic at birth and need neonatal intensive care unit (NICU) care. Nurses play a vital role in the monitoring of these infants both pre and post surgery. The infants need to be monitored for arrhythmias, pulmonary edema, or right heart failure. The outcomes for untreated infants is poor. The outcomes for other infants with cor triatriatum depend on the presence of other co-existing heart defects. For those with only the cor triatriatum defect, the outcomes are excellent. The surgery is best done at centers with experience with this disorder. While it appears that only the accessory membrane needs to be resected, the small size of the left atrium and collapse of the structure during cardiopulmonary bypass can make visualization difficult.[11][13]


  • Image 7031 Not availableImage 7031 Not available
    http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2018000100101
Attributed To: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0066-782X2018000100101

Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE courses, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Cor Triatriatum - Questions

Take a quiz of the questions on this article.

Take Quiz
Cor triatriatum sinistrum can be confused with which diagnosis in adults?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Select the least likely complication of cor triatriatum sinistrum.



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Cor triatriatum sinistrum presents with an outflow tract obstruction from the left atrium into the left ventricle. What other congenital heart diseases presents similarly to cor triatriatum sinistrum?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following is not a complication of cor triatriatum sinister?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which is the definitive treatment option for a patient with cor triatriatum?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 4-year-old girl presents to the emergency department with shortness of breath, fever, and cough. She also complains of fatigue on exertion, tachypnea, and that her fingers turn blue when she runs about a mile. She has a history of recurrent hospitalizations due to respiratory tract infections. After a thorough examination, the diagnosis of congenital heart disease is suspected. Which of the following will confirm the diagnosis of cor triatriatum in this child?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Cor Triatriatum - References

References

Nassar PN,Hamdan RH, Cor Triatriatum Sinistrum: Classification and Imaging Modalities. The European journal of cardiovascular medicine. 2011 Jan     [PubMed]
Hamdan R,Mirochnik N,Celermajer D,Nassar P,Iserin L, Cor Triatriatum Sinister diagnosed in adult life with three dimensional transesophageal echocardiography. BMC cardiovascular disorders. 2010 Oct 28     [PubMed]
Slight RD,Nzewi OC,Mankad PS, Echocardiographic diagnosis of cor triatriatum sinister in the adult. Heart (British Cardiac Society). 2004 Jan     [PubMed]
Jha AK,Makhija N, Cor Triatriatum: A Review. Seminars in cardiothoracic and vascular anesthesia. 2017 Jun     [PubMed]
PARSONS CG, Cor triatriatum; concerning the nature of an anomalous septum in the left auricle. British heart journal. 1950 Oct     [PubMed]
Narayanapillai J, Cor triatriatum sinister with severe obstruction: a rare presentation in an adult. BMJ case reports. 2016 Aug 5     [PubMed]
Fuchs MM,Connolly HM,Said SM,Egbe AC, Outcomes in patients with cor triatriatum sinister. Congenital heart disease. 2018 Jul     [PubMed]
Ozyuksel A,Yildirim O,Avsar M,Hayirlioglu M,Demiroluk S,Kucukosmanoglu O,Bilal MS, Surgical correction of cor triatriatum sinister in the paediatric population: mid-term results in 15 cases. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 2015 Jan     [PubMed]
Ostman-Smith I,Silverman NH,Oldershaw P,Lincoln C,Shinebourne EA, Cor triatriatum sinistrum. Diagnostic features on cross sectional echocardiography. British heart journal. 1984 Feb     [PubMed]
Thakrar A,Shapiro MD,Jassal DS,Neilan TG,King ME,Abbara S, Cor triatriatum: the utility of cardiovascular imaging. The Canadian journal of cardiology. 2007 Feb     [PubMed]
Humpl T,Reineker K,Manlhiot C,Dipchand AI,Coles JG,McCrindle BW, Cor triatriatum sinistrum in childhood. A single institution's experience. The Canadian journal of cardiology. 2010 Aug-Sep     [PubMed]
Chen Q,Guhathakurta S,Vadalapali G,Nalladaru Z,Easthope RN,Sharma AK, Cor triatriatum in adults: three new cases and a brief review. Texas Heart Institute journal. 1999     [PubMed]
An G,Zhang H,Zheng S,Wang W,Ma L, Mid-term Outcomes of Common Congenital Heart Defects Corrected Through a Right Subaxillary Thoracotomy. Heart, lung & circulation. 2017 Apr     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Pediatric-Developmental. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Pediatric-Developmental, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Pediatric-Developmental, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Pediatric-Developmental. When it is time for the Pediatric-Developmental board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Pediatric-Developmental.