or triatriatum sinistrum (CTS) is a rare congenital heart disease with an incidence of approximate- ly 0.1% in adults.

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or triatriatum sinistrum (CTS) is a rare congenital heart disease with an incidence of approximate- ly 0.1% in adults.

In this disease, the left atrium is

divided into two VSDFHV E\ D ¿EUR- muscular mem- brane, and these spaces are com- posed of pulmonary veins proximally and the left atrial appendix distally. In most cases, these spaces are linked to each other through one or PRUH RUL¿FHV7KH PDMRULW\ RI SDWLHQWV SUHVHQW ZLWK

clinical signs depending on the size of the opening on WKH¿EURPXVFXODUPHPEUDQH+RZHYHUPRVWRIWKHVH

cases (75%) are lost in infancy.

In this article, we present a case of a 20-year-old male having fenestrated CTS.

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A 20-year-old male patient was admitted to our car- diology clinic with dyspnea on exertion, fatigue, and SDOSLWDWLRQ +H KDG EHHQ VXIIHULQJ WKHVH V\PSWRPV

for several years, while his medical history was un- UHPDUNDEOH$FFRUGLQJWRWKH1HZ<RUN+HDUW$VVR- FLDWLRQ1<+$FODVVL¿FDWLRQKLVIXQFWLRQDOFDSDFLW\

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88 bpm with a regular rhythm, and his respiratory rate ZDVPLQRQSK\VLFDOH[DPLQDWLRQ%RWKOXQJVZHUH

equally participating in breathing and his respiratory sounds were normal. An apical early to mid-systolic

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CTS Cor triatriatum sinistrum MR Magnetic resonance NYHA New York Heart Association TEE Transesophageal echocardiography TTE Transthoracic echocardiography

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late systole, was heard on cardiac auscultation. ECG showed signs of an incomplete right bundle branch block with normal sinus rhythm. Transthoracic echo- FDUGLRJUDSK\ 77( 9LYLG  *( 0HGLFDO 6\VWHP showed a membrane dividing the left atrium (Fig.

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the inter-atrial or inter-ventricular septum. For further anatomical diagnosis, transesophageal echocardiogra- SK\7((ZDVSHUIRUPHG9LYLG*(0HGLFDO6\V- tem). Anatomic localization of the pulmonary veins was normal, and the inter-atrial septum was intact. The membrane originating from the left upper pulmonary vein, extending to the interatrial septum, and dividing the left atrium into two compartments as proximal and distal was seen. Fenestrations were observed in this membrane where it adheres to the interatrial septum )LJ%VHHVXSSOHPHQWDU\YLGHR¿OH&RORUÀRZ

transition between the proximal and distal portions in this region was imaged by color-Doppler echocardiog- raphy. We did not obtain a pressure gradient from this UHJLRQ &DUGLDF PDJQHWLF UHVRQDQFH 05 LPDJLQJ

was performed as a more advanced imaging method, with which we were able to see fenestrations of the membrane dividing the left atrium (see supplementary YLGHR¿OH7KHSDWLHQWZDVIROORZHGPHGLFDOO\

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In this very rarely seen congenital malformation, the left atrium is divided by an abnor-

PDO ¿EURPXVFXODU PHPEUDQH LQWR D SRVWHURVXSH- rior chamber receiving the pulmonary veins and an antero-inferior chamber giving rise to the left atrial appendage. The incidence of this malformation is between 0.1% and 0.4%.

The severity of clinical symptoms is associated with the size of the defect on WKH ¿EURPXVFXODU PHPEUDQH WKH UHVLVWDQFH WR SXO- monary venous return, and pulmonary hypertension.

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defects without any difference in pressure gradients between the proximal and distal spaces have been de- scribed. Associated abnormalities such as atrial sep- tal defect, patent foramen ovale, partial anomalous venous return, and persistent left superior vena cava have been reported.

Typical symptoms of mitral stenosis may be seen. Either a systolic or diastolic murmur may be heard or the patient may have a com- pletely normal physical examination. A functional FDSDFLW\RI1<+$FODVVDQGDQDSLFDOV\VWROLFPXU- mur were present in our case. The mechanism of low- JUDGH V\VWROLF PXUPXU PD\ EH GXH WR ÀRZ WKURXJK

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possible to diagnose the disease by TTE, but in most FDVHV7((LVUHTXLUHGWRFRQ¿UPDQGWRHYDOXDWHDG- ditional cardiac anomalies.

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TEE. It is important to note that the presence of fenes- trations may be misdiagnosed as. To rule out the very UDUHDQRPDO\RIFRUSRO\DWULDWXPZHSHUIRUPHG05

imaging on our patient,

but this anomaly was not

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