CASE REPORT

Niger J Paed 2012; 40 (1): 88 –90

Chinawa JM

Obu HA

Eze JC

Cor triatriatum sinistrium in a 10

year old Nigerian: A case report

Agwu S

Ani OS

Okorowo CJ

Omeke J

DOI:http://dx.doi.org/10.4314/njp.v40i1.18

Accepted: 15th May 2012

Abstract We present a rare and

first case of Cor triatriatum sinis-

trum (CT) in a patient who presents

with dyspnoea, easy fatigability,

chest pain, murmurs and typical

ECG and 2D-echo findings. The

purpose of presenting this case

report is to highlight the distinctive

manifestation of Cor triatriatum

sinistrum and to provide a concise

report of this disease with the hope

that such information will help

identify patients earlier in the

course of their disease.

Surgical correction offers good and

long term results for both classic

and atypical types. In a resource

poor country like ours, high index

of suspicion, early diagnosis and

timely referral are warranted so as

to avert death.

Chinawa JM (

) Obu HA

Department of Paediatrics College of

Medicine University of Nigeria

Teaching Hospital.

Ituku-Ozalla - Enugu Nigeria.

Email: josephat.chinawa@unn.edu.ng.

Eze JC, Omeke J, Agwu S, Ani OS

Okorowo CJ

Department of Paediatrics Enugu state

University of Technology Teaching

Hospital Park Lane, Enugu, Nigeria.

Key words: Cor triatriatum sinis-

trium, typical presentation, Nigeria.

Introduction

Examination revealed a malnourished child (mastoid

prominence, and loss of muscle bulk ), with an asym-

metrical left precordial bulge ; in obvious respiratory

distress, (evident by flaring of ala nasi, intercostal and

subcoastal recessions), mildly pale ,not cyanosed with

bilateral pitting pedal oedema up to the midshaft and no

digital clubbing. Pulse rate was 108 beat per minute, full

volume and regular, non collapsible, with radio-radial

synchrony and without radio-femoral delay. Precordium

was hyperactive. Apex beat was displaced to 6th inter-

costal space lateral to mid clavicular line with a pansys-

tolic murmur at lower left sternal border and a loud P2.

Respiratory rate was 48 cycles per minute, with diffuse

bilateral fine crepitations. There was tender hepa-

tomegaly.

Cor Triatriatum Sinistrum (CT) is a rare but surgically

correctable congenital cardiac anomaly accountin₁ g for

0

.1-0.4% of all congenital cardiac malformation. Cor

Triatriatum Sinistrum is characterized by the presence

of a fibro -muscular membrane that subdivides the left

atrium into 2 chambers in the classical form. Most pa-

tients with classical CT present during the neonatal pe-

riod or early infancy and adult cases of CT are very

2

rare. It could be associated with other₂ congenital car-

diac anomalies in 24% to 80% of cases.

Case presentation

A 10 year old Igbo female, presented in the Children

out patient of the Enugu State University Teaching

Hospital with a history of chest pain, leg swelling and

fast breathing of one year duration. Child was apparently

well until one year ago when she had repeated episodes

of chest pain which progressively increased in severity

and localised over the precordial region, and does not

radiate to any part of the body, the pain often interferes

with physical activity which also worsens it. This was

followed immediately by fast breathing that was insidi-

ous in onset, recurrent, worse at night, associated with

dyspnoea on mild exertion, increasing fatigue, or-

thopnoea and paraxosymal nocturnal dyspnoea. There

was no history of cyanosis or squatting. Leg swelling

had been recurrent and does not get resolved as the day

goes by.

Chest X-ray (CXR) showed situs solitus , levocardia

cardiomegaly and pulmonary plethora.(Fig 1).

Electrocardiogram (ECG) showed normal sinus rhythm,

right atrial enlargement, right axis deviation and right

ventricular hypertrophy ( Figs 2 and 3) and 2D-Echo

results showed five chambered heart, dilated right cham-

ber and grade two mitral regurgitation (Fig 4). Child was

nursed in cardiac position, placed on humidified oxygen,

intravenous frusemide and oral Digoxin . Patient mark-

edly improved for about two months now.

8

9

Fig 2: Left atrial enlargement (Q waves in lead V1 and V2)

Fig 3: Right axis deviation (dominant S in lead 1 and R in lead aVF, tall P

waves -Right atrial enlargement)

have TAPVD. Cor Triatriatum Sinistrium can present in

a myriad of ways: from accidental discovery to present-

ing with heart failure, to presenting in sudden death.

Cor triatriatum dextrum is another rare congenital anom-

a₅ly caused by remnants of the right sinus venosus valve.

Failure in the regression process of the cranial part of

this sinus venosus valve leads to membranes attached to

the crista terminalis. Malformations of the lower em-

bryologic valve parts result in a Chiari network or

prominent eustachian or thebesian valves, which might

eventually become large enough that they can even

cause right ventricular outflow₆_,₇obstructionby prolapsing

through the tricuspid valve.

Natural history of CT

Fig 4: 2-D ECHO of patient showing ASD (Lower

picture) and a right atrium (arrow) and two left atria

depends on the effective size of the opening in the mem-

brane and the presence and location of an atrial septal

1

defect (ASD) . If the opening in the membrane is small

and the ASD is absent or restrictive, the patient presents

early in infancy and without treatment 75% will die.

When the opening in the diaphragm is large with the

proximal pulmonary veins chamber communicating with

the right atrium through ASD, patient will present late as

is the case of our patient.

Discussion

Cor Triatriatum Sinistrum is a rare congenital abnormal-

ity, usually diagnosed in childhood; few cas₃es remain

asymptomatic and are diagnosed in adulthood.

The clinical picture and survival of patients with cor

triatriatum in adult depends on the degree of obstruction

to pulmonary₁_,v₄enous flow and the associated intracar-

3

The first case₄ of CT was reported by Church in 1868 .

Ladipo et al in Nigeria noted CT in an adult as an inci-

dental finding masquerading as left atrial mass it is noted

diac defects.

It has been reported that ASD or patent

4

foramen ovale was present in 70-80% of patients with

that less than 250 cases have been reported in literature.

8

cor triatriatum .When the ASD exists between the left

This rarity is also noted in our setting as this is the very

first known case. Total anomalous pulmonary venous

drainage (TAPVD) is the most associated anomaly re-

atrial accessory chamber and the right atrium, the pa-

tients present to hospital with symptoms of associated

elevated pulmonary venous and arterial₈pressures, be-

cause blood is shunted from left to right. In our report,

1

ported in a third of patient with CT. Our patient did not

9

0

the patient had history of dyspnoea on exertion which is

possibly due to elevated pulmonary venous pressure and

intracardiac shunting. Our patient had an ASD. The

main focus of investigation is 2D echocardiography and

ECG. Although cross-sectional echocardiography with

Doppler has been shown to be of great value in diagno-

sis of patients with cor triatriatum sinistrium; biplanar

Trans Oesophageal Echocardiogram(TEE) provides a

m₉ore complete and detailed data of the anatomy of CT.

The result of 2D Echo in this case showed mitral and

tricuspid regurgitation with resultant left ventricular

hypertrophy. Rhythm abnormalities may have precipi-

tated this patient into heart failure. The sudden symp-

toms in our patient could be attributed to the possibility

of development of arrhythmia, progressively worsening

interference with the systemic venous return and possi-

ble pulmonary congestion.

Treatment is primarily surgical. A right atrial, transsep-

tal approach to the common pulmonary chamber and

excision of the left atrial membrane (Atriotomy) was

found to be the treatment of choice. Transaccessory

chamber approach for type I and IIb provides ample

exposu₁₀re for complete excision of the obstructing mem-

brane.

Conclusion

CT is indeed a rare congenital cardiac anomaly. Surgical

correction offers good and long term results for both

classic and atypical types. In a resource poor country

like ours, high index of suspicion, early diagnosis and

timely referral are warranted so as to avert death.

Consent: An informed written consent was received for

publication of the article.

Contribution to Authors

All the authors made substantial intellectual contribu-

tions to this case report CJM was involved in the prepa-

ration of the manuscript, revision of the article at various

stages and preparation of the final draft. Other authors

made substantial contributions preparation of the manu-

script, revision and preparation of the final draft.

Conflict of interest: None

Funding: None

Acknowledgements

Only two reports₁o₀f successful balloon catheter dilata-

tion were noted.

Medical treatment with antifailure

Our gratitude extends to the care giver and patient who

were very cooperative.

Finally we thank the almighty God whose assistance and

ideas through the course of this work is priceless.

regimen (Digioxin) and High ceiling diuretic when in

cardiac failure may help stabilize patient while surgery

is being awaited.

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