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+A 33 - year old woman presented to our cardiology service with signs and symptoms of congestive heart failure.
+Her medical history was unremarkable, however a year ago and soon after her third child delivery, she had been admitted in another hospital for acute pulmonary oedema after labor.
+Cor triatriatum with obstructive behavior causing pulmonary hypertension had bee diagnosed, while the left ventricle was structurally and functionally intact.
+The patient at that time denied surgey and had been discharged on medical therapy.
+At present admission the patient presented with NYHA functional class III, symptoms of heart failure and palpittions as a result of persistent atrial flutter.
+On physical examination a loud pulmonary component of the 2nd heart sound and a diastolic murmur was heard in the mitral area.
+Signs of right-sided heart failure were absent.
+A transthoracic echocardiography revealed a moderately dilated left ventricle (LV), globally hypokinetic, with severely impaired systolic function (EF estimated ≥30%).
+Left atrium (LA) was dilated, with a mobile, membrane-like echogenic structure into it.
+Transesophageal echocardiogram (TEE) documented a fibromuscular membrane across the LA, dividing it into two compartments, a proximal one receiving the pulmonary venous flow and a distal one containing the left atrial appendage (LAA).
+The two chambers communicated via a non-restrictive orifice, but the membrane prolapsed towards the mitral valve inflow causing severe obstruction.
+Mitral valve appeared normal, with mild regurgitation.
+Patent foramen ovale (PFO), atrial septal defect (ASD) and anomalous venous connections were ruled out and the diagnosis of cor triatriatum was reconfirmed.
+Magnetic resonance imaging ( MRI) of the heart also revealed the fibromuscular septum into the left atrium and the low left ventricular ejection fraction [(LVEF) 30%, cardiac index 1,6 L/min/m2, cardiac output 2,7 L/min] (figure ​1).
+Coronary angiography showed normal coronary arteries.
+With these findings the patient was scheduled for surgery.
+Anesthetic induction was achieved with standard technique including administration of sodium pentothal, sevofluorane, fentanyl and muscle relaxant.
+Invasive monitoring included the use of right radial arterial lines, a pulmonary artery catheter and a foley catheter with temperature probe to measure bladder temperature as an indicator of core body temperature.
+Transesophageal echocardiography (TEE) was also instituted.
+Surgery was performed through a median sternotomy.
+Connection to cardiopulmonary bypass (CPB) was achieved by standard ascending aorta and bicaval cannulation.
+Mildly hypothermic (32°C) CPB was established.
+Cold blood cardioplegia was administered in an antegrade fashion through the aortic root after clamping the aorta.
+The interatrial groove was developed and the common pulmonary venous chamber of the left atrium was opened through a vertical incision anterior to the right pulmonary veins, exactly as for mitral valve surgery.
+After insertion of a self-retaining retractor to facilitate exposure, the diaphragm was exposed and the central hole in it was identified.
+A preliminary incision out from the hole improved exposure for the definitive excision.
+Orifices of the pulmonary veins on both sides were located.
+Position of the atrial septum was also identified by a small opening in the right atrium and by inserting a curved clamp to displace the septum into the common pulmonary venous chamber of the left atrium.
+There was no atrial septal defect or patent foramen ovale.
+The diaphragm was then easily completely excised exposing the mitral valve (figure ​2).
+The left atrial appendage was closed from inside the left atrium using a running 3-0 polypropylene suture to prevent future thrombus formation.
+The atriotomy incisions were closed, the heart having been filled with blood before the last few sutures were placed.
+The patient was rewarmed, the aortic cross-clamp was removed and additional de-airing was carried out in the usual manner.
+CPB was terminated with minimal inotropic support, involving milrinone and levophed with good hemodynamics.
+The postoperative course was uneventful and the patient was extubated after 12 hours and discharged from the hospital on the fifth postoperative day.
+At 3 months follow-up, the patient was asymptomatic (NYHA class I), in sinus rhythm.
+TTE and MRI revealed a mildly dilated LV with great improvement in systolic function and an estimated LVEF of 50%.