Bipin B.Mohanty,M.Ch,FACC,*Ahmed Y.Al-ansi, Anasth.&ICU specialist†,

Haifa N. Al-Malhany, MD‡, Abdulmalek Sharaf,MD§.

Department of cardiac surgery, cardiac center, Thawra Modern General Hospital,

Sana'a, Yemen.

INCIDENTAL COR - TRIATRIATUM SINISTER

WITH CONGENITAL MR IN A YOUNG

FEMALE, SURGICAL MANAGEMENT WITH

BRIEF REVIEW OF LITERATURE

CASE REPORT

• In June 3rd 2009,F.A. a 25 year old female,

• presented with history of palpitation, shortness of breath for last two years.

• No rheumatic history was present.

• PE: revealed an irregular pulse rate of 80b/min, B.P 110/74mmHg and

elevated jugular venous pressure (JVP). Auscultation, revealed accentuated

P2 and soft S1 with pansystolic murmur at the apex conducted to axilla.

ECG showed AF .

• Hematology and biochemistry were within normal limits but

liver function showed two folds rise of total and direct

Billirubin..

• Chest radiography revealed cardiomegaly with LV contour and a

prominent pulmonary vasculature and bi-atrial enlargement.

• Transthoracic echocardiography picture showed this:

• Dimensions as follow:

• left atrium 77mm

• LVIDD 72mm, LVISDS 44mm

• ejection fraction 68%

• Mitral valve area was 3.2 cm² with (grade IV/IV) MR. Also there was

trivial AR and mild TR with PA pressure 69 mmHg indicating severe

PAH.

• but a membrane across the cavity of left atrium was

not reported..

• Procedure: With above findings at evaluation, she was scheduled for mitral valve

surgery. With full heparinizations and moderate hypothermic CPB (with aortic and

two stages venous cannula into RA&IVC) aorta was clamped and antegrade blood

cardioplegia instilled.

• Left atriotomy revealed ……………oooopss where is Mitral vale ???!!!!!!!!!

• Finding: There was a fibromuscalr membrane with one central

orifice of 17mm with mild calcification around it. There was a

muscular band like papillary muscle connecting the membrane from

its base to the annulus of the mitral valve close to left atrial

appendage orifice. This finding has not been reported before though

accessory 3rd papillary muscle had been described.

• The superior chamber was very large, receiving the pulmonary

venous drainage. The inferior chamber with papillary muscle like

band contained left atrial appendage, fossa ovalis and mitral valve.

• The mitral valve annulus was dilated, anterior and posterior leaflets

were hypoplastic, retracted and there were two clefts in the posterior

mitral leaflet with gross noncoaptation. The chordae tendinae and

two papillary muscles were short, slender and appeared rudimentary.

procuder:

• The anomalous fibromuscular membrane was excised widely

keeping 1mm rim. Mitral valve replacement was performed with

posterior leaflet and chordal preservation using 33 mm bileaflet

mechanical prosthetic mitral valve (St.Jude Regent.Left atrial

appendage was closed from inside LA.

Follow up:

• She is doing well and being followed up to now with control Echo, PT, INR

and IE prophylaxis. Chest X-Ray and ECHO show remarkably reduced heart

size and no residual membrane

SO:What is cor Triatriatum?

• Cor Triatriatum, a heart with 3 atria (triatrial heart), a congenital

anomaly in which the left atrium (cor Triatriatum sinister) or right

atrium (cor Triatriatum dexter) is divided into 2 parts by a fold of

tissue, a membrane, or a fibromuscular band. Classically, the

proximal (upper or superior) portion of the corresponding

atrium receives venous blood, whereas the distal (lower or

inferior) portion is in contact with the atrioventricular valve and

contains the atrial appendage and the true atrial septum that

bears the fossa ovalis.

• The incidence of cor Triatriatum has been variously reported as 0.1-

0.4%.

• is increasingly diagnosed more accurately with improvement

in diagnostic imaging.

• Cor triatriatum with congenital Mitral Regurgitation is extremely

rare.

The age at presentation:

Patients with a restrictive opening in the >>>> infancy with

signs and symptoms of pulmonary venous obstruction and

severe congestive heart failure. In such infants 75% of

patients with such anomaly die in infancy if not operated.

Patients with a large unrestrictive opening in the anomalous

septum may remain symptom-free for many years before

developing congestive heart failure as in our patient.

Embryologic basis of this anomaly remains controversial.

The 3 main theories:

1- The malseptation hypothesis was put by Fowler in 1881.

2- The malincorporation hypothesis by Parson.

3- Van Praagh and Corsini proposed the entrapment hypothesis.

Classification :

Loeffler 1949 classified cor triatriatum into 3groups on the basis of

the number and size of the openings in the anomalous membrane :

Group I = no opening;

Group II = one or more small openings;

Group III = a wide opening as in our case.

Marin-Garcia and colleagues classification: on the basis of the

anatomic shape of the accessory left atrial chamber: such as

diaphragmatic, hour-glass, and tubular types.

• Andral 1829was the first observer to mention a heart "with three auricles.

• Church published the first full account of a patient in theyear 1868 with this condition in a woman aged 38 years.

• Fowler in 1881 described the embryologic basis of thisanomaly.

• The name “cor triatriatum” was applied to themalformation by Borst in 1905.

• Parson in the year 1950 reviewed in detail the theories.

• The first angiographic diagnosis was done at the MayoClinic and published by Miller in 1964.

• Finally, first surgical correction is believed to have beenperformed by Vineberg and Galloreto.

• Only three cases of cor triatriatum with congenital

mitral regurgitation has been published, which includes

case report by Timothy etal in the year 1978 in a 52

year old man.

• We report the 4th case of cor triatriatum sinister in a 25

year old female with congenital MR which is similar to

the case operated by M.Fuster-siebert in1974 in a 16

year old boy.

Summery:

• More than 700 cases of cor triatriatum have been reported in

literature but only 3 cases of cor triatriatum with congenital MR in

adults. We report the 4th case because of its rarity.

• With experienced Echo cardiographer an accurate diagnosis can

be made and surgery planned.

• If not diagnosed pre operatively this rare anomaly can be a

surgical dilemma for junior surgeons who are not aware of this

rare anomaly.

• Surgical correction of this rare condition is achieved with high

success rate by any well informed cardiac surgeon even though

this anomalous pathology is an accidental finding at operation.

• References:• Andral, G. (1829). Precis d'Anatomie path., Paris.

• Church WS. Congenital malformation of the heart: Abnormal septum in left auricle. Trans Pathol Soc Lond

1867/1868; 19: 188.

• Fowler JK. Membranous band in the left auricle. TransPathol Sco London 1881;33:77-94.

• Borst H. Ein cor triatriatum. Zentralbe Allg Pathol 1905; 16: 812.

• Parsons CG. Cor triatriatum: concerning the nature of an anomalous septum in the left auricle. Br Heart J1950;

12:327-38.

• Aris Lâcis,et al , Cor triatriatum associated with complex heart defects ACTA MEDICA LITUANICA. 2004. 11. (4).. 59–

63

• Kouchoucos NT, Blackstone EH, Doty DB, Stanley FL, Karp RB. Kirklin / Barratt-Boyes Cardiac Surgery,3rd edition.

Elsevier Science, USA, 2003: 781–9.

• Jorgensen CR, Ferlic RM, Varco RL, Lillehei CW, Eliot RS.Cor triatriatum. Review of the surgical aspects with a

followup report on the first patient successfully treated with surgery.Circulation 1967;36:101-7.

• References:• Kirklin JW, Barratt-Boyes BG, editors. Cardiac surgery:morphology, diagnostic criteria, natural history,

techniques,results, and indications. 2nd ed. New York: Churchill Livingstone 1993:678.

• Qiang Chen, MD,Soma Guhathakurta, MCh,et al CorTriatriatum in Adults,

• Tex Heart Inst J 1999; 26:206-10.

• Van Praagh R, Corsini I. Cor triatriatum: pathologic anatomy and a consideration of morphogenesis based on 13

postmortem cases and a study of normal development of the pulmonary vein and atrial septum in 83 human

embryos.Am Heart J 1969;78:379-405.

• Gharagozloo F, Bulkley BH, Hutchins GM. A proposed pathogenesis of cor triatriatum: impingement of the left

superior vena cava on the developing left atrium. Am Heart J 1977;94:618-26.

• Loeffler E. Unusual malformation of the left atrium: pulmonary sinus. Arch Pathol 1949;48:371-6.

• W Keeble, E Lundmark and H J Dargie, The paradoxical finding of mitral valve incompetence and cor triatriatum: a

mechanism revealed? Heart 2004;90;125.

• Dr. Timothy W. Leavitt, MD, J. V. Nixon, MDet , Cor triatriatum with mitral regurgitation in a 52-year-old male,

catheterization and cardiovascular diagnosis 2005,(5),75-84.

• Marin-GarciaJ, Tandom R, Lucas RVJr, Edwards JE. Cor triatriatum: Study of 20 cases. Am J Cardiol 1975; 35:59.

• M. Fuster-Siebert, R. Llorens, R. Arcas-Meca, J. Rubio-Alvarez et al, Cor Triatriatum with Mitral Valve Disease in

Adults: Texas Heart Institute J.1982(9)(3) 363-366

• Davachi F, MollerJH, EdwardsJE. Diseases of the mitral valve in infancy: An anatomic analysis of 55 cases.

Circulation 1971; 43:565.