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Cor triatriatum presenting as heart failure with reduced ejection fraction: a case report
© Kokotsakis et al; licensee BioMed Central Ltd. 2011
Received: 8 October 2010
Accepted: 14 June 2011
Published: 14 June 2011
Cor triatriatum is a rare congenital cardiac malformation and it usually refers to the left atrium. We report an unusual case of cor triatriatum in a 33 - year old woman presented with congestive heart failure caused by left ventricular systolic dysfunction.
Cor triatriatum is a rare congenital cardiac malformation with an estimated incidence of 0,1% of all congenital heart disease and it usually refers to the left atrium (cor triatriatum sinister). In cor triatriatum sinister the left atrium is divided by a fibromuscular membrane into two distinct chambers: a posterior - superior chamber receiving the four pulmonary veins and an anterior - inferior chamber ( true left atrium ) that connects to the left ventricle by means of the mitral valve . In the majority of cases it is diagnosed in neonatal period or early infancy, whereas adult cases are very rare. We report an unusual case of cor triatriatum in a 33 - year old woman presented with congestive heart failure caused by left ventricular systolic dysfunction.
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.
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%.
Cor triatriatum is a rare congenital anomaly with a ratio of men to women of 1.5:1 . In cor triatriatum the right and left pulmonary veins can be considered as not joining the left atrium but rather as entering a chamber posterior and a little superior or medial to the left atrium that is analogous to the common pulmonary venous sinus found in patients with total or partial anomalous pulmonary connection. However the pulmonary veins in cor triatriatum are incorporated into the structure of the left atrium, whereas in total anomalous pulmonary venous connection the pulmonary veins connect to sites separate from the left atrium. Other associated anomalies are the unrooted coronary sinus with a left superior vena cava joining the left atrium, ventricular septal defect, coarctation of the aorta, atrioventricular septal defect, tetralogy of Fallot and rarely asplenia and polysplenia. No genetic predisposition has been linked to this particular anomaly. The clinical features on presentation can mimic those of mitral stenosis, supravalvular mitral ring, left atrial thombus or pulmonary venous stenosis, since these entities share a common haemodynamic pathophysiology of flow obstruction between the pulmonary venous system and the left ventricle. The most common presenting symptoms in adults are dyspnea, hemoptysis, orthopnea as a result of the obstructive function of the intra-atrial membrane . Several techniques have been used for diagnosis establishment such as TTE, TEE, CT, MRI. The use of CT bares the risk of radiation, while TEE the discomfort of scope intubation. MR imaging when compared with echocardiography and cardiac angiography was found to have a higher detection rate . In addition MR fast gradient-recalled echo imaging of the cardiac cycle has been shown to be of better benefit in the assessment of cardiac function and has been established as the modality of choise for the assessment of LVEF . According to Loeffler's classification of the lesion, group 3 lesions have large openings in the membrane, leading to little or no obstruction . Patients with group 3 lesions can survive into adulthood with minor or no symptoms at all, as in the case of our patient. Late clinical presentations and conversion to a symptomatic state may be due to fibrosis and calcification of the orifice of the septum, onset of atrial flutter and fibrillation with rapid ventricular response, development of mitral regurgitation. Asymptomatic patients with an incidental diagnosis and a non-restrictive opening of the intra-atrial diaphragm, can be observed and followed-up regularly by TTE or MRI . For symptomatic patients, surgical excision is the definite treatment, eventhough successful balloon catheter dilatation of the communication between the two chambers has been described .
Our patient had two previous uneventful pregnancies and experienced acute heart failure symptoms in the early postpartum period of her third normal pregnancy. The increased demands of pregnancy induce an even greater pressure gradient between the left cardiac chambers and thus a greater elevation of left atrial pressure, causing a decompensation of the patient's previously compensated cardiac function. However, severe systolic dysfunction causing symptomatic heart failure, to the best of our knowledge, has never been reported in patients with cor triatriatum.
The presence of normal coronary anatomy and the exclusion of cardiomyopathies, using CMR, combined with the rapid recovery after surgical correction, leads us to believe that there is a causal relationship among these entities. Pronounced preload mismatch due to severe membrane prolapse in the LV inflow, combined with the sequential volume changes during pregnancies, leaded to decompensation and systolic dysfunction. Membrane surgical excursion leaded to rapid recovery. Peripartum cardiomyopathy seems highly unlikely, due to late onset, and rapid postoperative recovery.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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