cor triatriatum

COR TRIATRIATUM

Dr. Obayda Mu’men DiraneyyaMBBS, SCC-Ped, CABP, MRCPCH

Assistant Consultant

Pediatric Cardiology

KACC- Riyadh

PRESENTATION

• Introduction

• Cor triatriatum sinister/dexter

• Epidemiology

• Embryogenesis

• Classification

• Clinical Presentation

• Diagnosis

• Management

• Prognosis

INTRODUCTION

Cor-

Tri-

Atiatum

INTRODUCTION

• A rare congenital cardiac anomaly in which a membrane or

diaphragm subdivides the atrium into a proximal chamber

and a distal chamber.

• Types

• Left atrium cor triatriatum sinister

• Right atrium cor triatriatum dexter

COR TRIATRIATUM SINISTRUM

HISTORY

• Church: 1st description in 1868

• Borst: the term of “cor triatriatum” in 1905

• Miller et al: angiographic diagnosis in 1964

• Ostman-Smith: description by echocardiography in 1984

• Lewis et al: 1st surgical correction in 1956

EPIDEMIOLOGY

• Incidence is 0.1-0.4% at autopsy

• Much lower in reality 0.004 in 1000 live births

• Isolated defect in 33-50% of cases

• Equal frequency in both sexes

ASSOCIATIONS

• May be associated with

• TOF, DORV, CoA, PAPVC, VSD, AVSD,

• persistent LSVC with unroofed coronary sinus,

• rarely asplenia or polysplenia has been reported

• In the adult has been reported in association with

• ostium secundum ASD,

• dilated coronary sinus due to persistent LSVC,

• BAV

Abnormal

Connection

Abnormal

Drainage

with Normal

Connection

Pulmonary venous anomalies

Abnormal

Numbers

Stenotic

Connection

Abnormal

Connection

Abnormal

Drainage

with Normal

Connection

Pulmonary venous anomalies

Abnormal

Numbers

Stenotic

Connection

Cor TriatriatumStenosis of

Individual Veins

EMBRYOGENESIS

Moss and Adam’s Heart Disease in Infants, Children and Adolescents. Hugh D. Allen and others.

EMBRYOGENESIS

Invagination from

sinoatrial regionConfluence of vessels

from pulmonary plexus

Development of Common Pulmonary Vein

Confluence of

capillaries growing

into mesocardium

EMBRYOGENESIS

• By the end of the first month of gestation, the common pulmonary

vein can be identified as a vessel draining the pulmonary plexus

and entering the sinoatrial portion of the heart

• Site of entry is cephalad to the junction of the left and right horns

of the sinus venosus and to the left of the developing septum

primum

EMBRYOGENESIS

• Imperfect development of the

common pulmonary vein

provides embryologic basis

for most anomalies of the

pulmonary veins.

EMBRYOLOGY

EMBRYOGENESIS

• Cor triatriatum is the result of stenosis of the common

pulmonary vein

• In the usual case, the stenosis occurs late, after collateral venous

connections have been lost

• Or else the severity of the obstruction produced by cor

triatriatum is insufficient to stimulate maintenance of the

primitive routes of venous drainage

EMBRYOGENESIS

• Occasionally, cor triatriatum may be associated with

anomalous pulmonary venous connection, implying that in

such cases, the obstruction was early enough and sufficient

to favor persistence of one of the primitive drainage

channels such as a levoatriocardinal vein

COR TRIATRIATUM

Central panel shows the normal

obliteration of the primitive

venous connections

A

Failure of CPV-LA junction

atresia of common

pulmonary vein

B

Stenosis of CPV-LA

junction cor triatriatum

Pulmonary–

systemic venous

connections still

present

EarlyPulmonary–

systemic venous

connections

absent

Pulmonary–

systemic venous

connections still

present

Pulmonary–

systemic venous

connections

absent

Late Early Late

Common Pulmonary Vein

Atresia Stenosis

TAPVC/

PAPVC

Cor

Triatriatum

Cor

Triatriatum

with APVC

Atresia of the

common

pulmonary

vein

EMBRYOGENESIS

• Malseptation theory:The membrane is an abnormal growth of

septum primum

• Entrapment theory: Right horn of the embryonic sinus venosus

entraps the CPV and thereby prevents its incorporation

• Malincorporation theory: CPV fails to incorporate the pulmonary

circulation into the LA and the CPV ostium remains narrow

Pulmonary venous chamber

receives all pulm. veins

Anatomic Classification of Cor Triatriatum

Total

No other

connection


receives some pulm. veins

Subtotal

Other anomalous

connection

Classic cor

triatriatum

Cor triatriatum

+ APVC

Direct

to RA

TAPVC

Communicate

with LA

Do not

communicate

with LA

Connects

with RA

Connects

with LA

With

normal PVC

With

PAPVC

With normal

remaining PVC




Total

No other

connection



Subtotal

Other anomalous

connection

Classic cor

triatriatum

Cor triatriatum

+ APVC

Direct

to RA

TAPVC

Communicate

with LA

Do not

communicate

with LA

Connects

with RA

Connects

with LA

With

PAPVC

With normal

remaining PVC

With normal

remaining PVC




Total

No other

connection



Subtotal

Other anomalous

connection

Classic cor

triatriatum

Cor triatriatum

+ APVC

Direct

to RA

TAPVC

Communicate

with LA

Do not

communicate

with LA

Connects

with RA

Connects

with LA

With normal

remaining PVC

With

PAPVC

With normal

remaining PVC




Total

No other

connection



Subtotal

Other anomalous

connection

Classic cor

triatriatum

Cor triatriatum

+ APVC

Direct

to RA

TAPVC

Communicate

with LA

Do not

communicate

with LA

Connects

with RA

Connects

with LA

With

PAPVC

With normal

remaining PVC

With normal

remaining PVC

THE WINDSOCK

“DO WHAT IS RIGHT, NO MATTER WHICH WAY THE

WIND BLOWS"

CLINICAL PRESENTATION

• Symptoms depend on

• Size of the opening between pulmonary venous chamber & LA

• Location and size of associated communication to the RA

• Most patients (75%) are symptomatic during infancy

• Minority might be asymptomatic till second/third decade


Symptoms

• Young child: weak pulses, pallor, SOB, frequent chest

infections, failure to thrive, rare presentations

• Adolescent may become symptomatic if:

• Fibrosis and calcification of the orifice of accessory membrane

• Development of MR

• Development of AF

• Thromboembolism (AF/restrictive orifice)


Signs

• PHN

• Tachypnea, distress, loud S2, rales, pleural effusion,

hypoxemia

• RVF

• Raised JVP, RV heave, TR, tender hepatomegaly, jaundice,

ascites, edema

DD

• Bronchial asthma

• Pulmonary vein stenosis

• Pulmonary veno-occlusive disease

• Supravalvular mitral ring

• Atrial tumors

ECHOCARDIOGRAPHY

• Dilation of the RA, RV, and PA

• PW Doppler and color Doppler should be used to determine

whether any restriction across the cor membrane is present

• Mean gradients greater that 3 mm Hg should be considered

abnormal

Supramitral ring

membraneCor triatriatum membrane

Linear echo-bright structureCurvilinear echo-bright

structure (windsock)Appearance

On the atrial surface of the

base of MV leafletsLocation

Frequently immobileMobile during cardiac cycleMobility

Moves towards MV during

diastoleMovement

Only diastolicSystolic and diastolicFlow

Leaflet mobility maybe

affectedNormal

Appearance and motion of

MV

Membrane is inferior to LA

appendage and PFO

Membrane superior to LA

appendage and PFOAnatomic Relation

Insert into proximal chamberPulmonary veins

ELECTROCARDIOGRAPHY

• The typical finding is RVH

• RAH results in tall, peaked P waves in some of the case

• Broad, notched P waves are present in some cases

presumably as a consequence of the dilated pulmonary

venous chamber, but are absent in others

RADIOLOGY

• Fine diffuse reticular markings fan out to involve lower lung fields

• Kerley B lines

• Prominent venous engorgement of the upper pulmonary veins

(staghorn sign)

• Enlargement of the MPA, RVH, and signs of “LA” enlargement,

including double density at the right cardiac border (dilated

pulmonary venous chamber)

MAGNETIC RESONANCE IMAGING/ COMPUTED TOMOGRAPHY

• Can delineate the LA membrane of cor triatriatum

• Can be helpful in cases of anomalous PVC

• May be helpful in defining the cause of PHN when

echocardiography is not diagnostic

CARDIAC CATHETERIZATION

• No longer considered necessary

• Pulmonary hypertension routinely is found

• Using oximetry one can exclude a L-R shunt

• PHN is either caused by primary pulmonary vascular disease or

secondary to pulmonary venous obstruction

• The pulmonary arterial wedge pressure is elevated, and LA

pressure is normal

TREATMENT

• Stabilize hemodynamics

• Rx hypoxemia, fluid overload, pulmonary congestion

• Anticoagulation

• Prophylactic: RV failure

• Therapeutic: AF

TREATMENT

• Surgical resection

• Urgently if obstructive (restrictive communication)

• Often necessary in first year of life

• Indicated if high PA pressure

PROGNOSIS

• Prognosis is related to the size of the orifice in the obstructing

membrane

• In Niwayama’s survey, average survival was

• 3 ⅓ months when the opening was <3 mm, and

• 16 years when the opening was >3 mm

• When pulmonary edema and right heart failure occur, survival is

usually only a matter of months

Niwayama G. Cor triatriatum. Am Heart J. 1960;59:291–317.

PROGNOSIS

• Patients who survive operative correction excellent prognosis

• A recently published report from Boston Children’s Hospital

included 65 patients with cor triatriatum sinister (CTS).

• The 30-day survival for this cohort was 97%, with only one

noncardiac death reported on long-term follow-up.

• The severe pulmonary arterial changes that result in pulmonary

hypertension have been reversible in the patients studied

postoperatively

Yaroglu Kazanci S, Emani S, McElhinney DB. Outcome after repair of cor triatriatum. Am J Cardiol. 2012. 109:412-6.

COR TRIATRIATUM DEXTRUM

COR TRIATRIATUM DEXTER

• Definition

• Persistence of the right valve of the sinus venosus, subdividing the

right atrium into two chambers

• Synonyms

• RA flap, RA spinnaker, RA sail, RA windsock, persistent eustachian

valve, persistent thebesian valve, IVC connecting to LA, atresia of the

RA ostium of the coronary sinus, supravalvular TS, TV “stopper,” IVC

obstruction, and others.

COR TRIATRIATUM DEXTER

• History

• Rokitansky: 1st description in 1875

• Incidence

• Extremely rare

• Association

• Can be seen in isolation

• And in association with HRHS and ventriculo-coronary artery

communications, Ebstein malformation, and TA

EMBRYOGENESIS

• Remnants of sinus venosus valves

• Eustachian valve

• Thebesian valve

• Crista terminalis • Minor persistence

• Larger than usual eustachian valve

• Larger than usual thebesian valve

• Chiari network

EMBRYOGENESIS

• 21 days

• The sinus venosus is external to the primitive RA

• The right horn of the sinus venosus receives

• the hepatic vein (precursor to the IVC) and

• the anterior cardinal vein (precursor to the SVC)

• The left horn of the sinus venosus is the embryologic precursor of

the coronary sinus.

EMBRYOGENESIS

• 90 days

• The right valve of the sinus venosus almost completely

divides the RA into

• the sinus portion (sinus venosus) receiving the SVC, IVC,

coronary sinus, and foramen ovale, and

• the muscular portion of the RA communicating with the

tricuspid valve and the RAA

EMBRYOGENESIS

EMBRYOGENESIS

• In the majority of humans, the right valve of the sinus

venosus almost completely regresses by the time of birth, a

small remnant persists as the crista terminalis

• Normally, crista terminalis separates the anterior, muscular

portion of the RA from the posterior, sinus venosus, or sinus

portion of the RA

EMBRYOGENESIS

• The superior portion of the right valve of the sinus venosus

plus a portion of the sinus venosus septum persists as the

eustachian valve guarding the inferior vena caval orifice

• The inferior portion of the right valve of the sinus venosus

plus a portion of the sinus venosus septum persists as the

thebesian valve guarding the orifice of the coronary sinus


• Unlike cor triatriatum sinister, which carries a high mortality

rate if not repaired

• Clinical manifestations depends on the degree of

partitioning or septation of the right atrium

• Asymptomatic in vast majority


• More severe septation can cause right-sided heart failure

and elevated CVP due to obstruction of TV, RVOT, or IVC

• Cyanosis can appear if there is ASD

DIAGNOSIS

• Echocardiography, contrast echocardiography, or MRI

• In a study in which MRI was compared with echocardiography and

cardiac angiography in the evaluation of pulmonary venous anomalies,

which included cases of cor triatriatum, MRI had a higher detection

rate (95%) than the other modalities (69% for angiography and 38%

for echocardiography)

MANAGEMENT

• Asymptomatic patients are generally not treated unless they are

undergoing cardiac surgery for other reasons

• Symptomatic patients has been treated traditionally by surgical

resection of the dividing membrane

• Recently, percutaneous catheter disruption of the membrane has

been reported and has been suggested as a preferred

alternative to open heart surgery

REFERENCES

• Hugh D. Allen, Moss & Adams’ Heart Disease in Infants, Children, and Adolescents. 9th ed. Wolters Kluwer. 2016.

• Myung K. Park, Park’s Pediatric Cardiology for Practitioners, 6th ed. Elsevier. 2014.

• Robert H. Anderson, Paediatric Cardiology, 3rd ed. Churchill Livingstone. 2010.

• Jamshid Shirani, Cor Triatriatum, Medscape article, WebMD Health Professional Network. Dec 18, 2014.

• David L Morales, Cor Triatriatum Surgery in the Pediatric Patient, Medscape article. Feb 06, 2015.

• Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery, Cor Triatriatum, a

presentation uploaded by Kera Carrigan. Aug 9, 2012.

• Ahmad Rustam et al, Cor triatriatum dexter: A rare cause of childhood cyanosis. Ann Pediatr Cardiol. 2012 Jan-Jun;

5(1): 92–94.

THANK YOU

cor triatriatum

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