Three patients with cor triatriatum sinister detected in adulthood

    Authors

    Keywords

    cor triatriatum, transthoracic echocardiography, transesophageal echocardiography, pulmonary hypertension

    DOI

    https://doi.org/10.15836/ccar2017.158

    Full Text

    **Introduction**: Cor triatriatum is one of the rarest congenital cardiac anomalies accounting for 0.1-0-4% of all congenital heart disease. It is frequently associated with other congenital heart defects and is normally diagnosed in childhood mostly because it is symptomatic with symptoms of left heart obstruction or arrhythmia. (1-3) We present three cases of cor triatriatum sinister diagnosed in adult age with successful conservative treatment and follow up. **Case report**: First patient was a 30-year-old gravida with an asymptomatic, recently diagnosed cor tratriatum sinister. She has had a history of heart murmur since childhood, but has never been properly evaluated because she had good effort tolerance. Transthoracic (TTE) and transesophageal (TEE) 2D and 3D echocardiogram documented a fibromuscular membrane across the left atrium, dividing it into two compartments, with the proximal one receiving the pulmonary venous flow. Together with the gynecologist we decided to continue with the conservative approach. A healthy baby girl was successfully delivered at 38 weeks’ gestation, without any complications. Second patient was a 70-year-old woman that was referred to our emergency unit because of dyspnea and palpitations in the last two weeks, with more frequent episodes in the previous days and nonspecific anterior chest discomfort. TTE showed an abnormal membrane that bisects the left atrium into two chambers, an enlargement of right atrium, a mitral and tricuspid regurgitation and a reduced ejection fraction with pulmonary hypertension. On color Doppler was visualized a left-right (L-D) shunt with two jets. L-D shunt with two jets was confirmed on TEE, one jet on interatrial and membrane conjunction and second in proximal part of interatrial septum. The patient refused operative treatment and was successfully treated with pharmacological therapy. The third case was a 33-year-old men that was referred to TTE because of palpitations and a cor triatriatum without shunt was recognized. **Conclusion**: Cor triatriatum is a rare congenital heart disease with varying presentations. The golden standard in three dimensional TTE and TEE.

    Literature

    1. Naimo PS, Konstantinov IE. Cor triatriatum sinister: Is it less sinister in older patients? J Thorac Cardiovasc Surg. 2015 Nov;150(5):e77–8. https://doi.org/10.1016/j.jtcvs.2015.08.030
    2. Nassar PN, Hamdan RH. Cor Triatriatum Sinistrum: Classification and Imaging Modalities. Eur J Cardiovasc Med. 2011 Jan;1(3):84–7. https://pubmed.ncbi.nlm.nih.gov/22379596/
    3. Bassareo PP, Tumbarello R, Mercuro G. Cor triatriatum and lipomatous hypertrophy of the interatrial septum in the elderly: a case report. Cardiovasc Ultrasound. 2010 Mar 9;8:4. https://doi.org/10.1186/1476-7120-8-4
    Cardiologia Croatica
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    Three patients with cor triatriatum sinister detected in adulthood

    Extended Abstract
    Issue4
    Published
    Pages158
    PDF via DOIhttps://doi.org/10.15836/ccar2017.158
    cor triatriatum
    transthoracic echocardiography
    transesophageal echocardiography
    pulmonary hypertension

    Authors

    Ivana JurinORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Josip VinceljORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Diana RudanORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Mario UdovičićORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Sandra Jakšić JurinjakORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Jasmina ĆatićORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Petra Vitlov*ORCIDUniversity Hospital Dubrava, Zagreb, Croatia
    Tomislava Bodrožić Džakić PoljakORCIDUniversity Hospital Dubrava, Zagreb, Croatia

    *Correspondence email: petra.vitlov@gmail.com

    Full Text

    Introduction: Cor triatriatum is one of the rarest congenital cardiac anomalies accounting for 0.1-0-4% of all congenital heart disease. It is frequently associated with other congenital heart defects and is normally diagnosed in childhood mostly because it is symptomatic with symptoms of left heart obstruction or arrhythmia. (1–3) We present three cases of cor triatriatum sinister diagnosed in adult age with successful conservative treatment and follow up.

    Case report: First patient was a 30-year-old gravida with an asymptomatic, recently diagnosed cor tratriatum sinister. She has had a history of heart murmur since childhood, but has never been properly evaluated because she had good effort tolerance. Transthoracic (TTE) and transesophageal (TEE) 2D and 3D echocardiogram documented a fibromuscular membrane across the left atrium, dividing it into two compartments, with the proximal one receiving the pulmonary venous flow. Together with the gynecologist we decided to continue with the conservative approach. A healthy baby girl was successfully delivered at 38 weeks’ gestation, without any complications. Second patient was a 70-year-old woman that was referred to our emergency unit because of dyspnea and palpitations in the last two weeks, with more frequent episodes in the previous days and nonspecific anterior chest discomfort. TTE showed an abnormal membrane that bisects the left atrium into two chambers, an enlargement of right atrium, a mitral and tricuspid regurgitation and a reduced ejection fraction with pulmonary hypertension. On color Doppler was visualized a left-right (L-D) shunt with two jets. L-D shunt with two jets was confirmed on TEE, one jet on interatrial and membrane conjunction and second in proximal part of interatrial septum. The patient refused operative treatment and was successfully treated with pharmacological therapy. The third case was a 33-year-old men that was referred to TTE because of palpitations and a cor triatriatum without shunt was recognized.

    Conclusion: Cor triatriatum is a rare congenital heart disease with varying presentations. The golden standard in three dimensional TTE and TEE.

    Literature

    1. 1.
      Naimo PS, Konstantinov IE. Cor triatriatum sinister: Is it less sinister in older patients? J Thorac Cardiovasc Surg. 2015 Nov;150(5):e77–8.DOI
    2. 2.
      Nassar PN, Hamdan RH. Cor Triatriatum Sinistrum: Classification and Imaging Modalities. Eur J Cardiovasc Med. 2011 Jan;1(3):84–7.PubMed
    3. 3.
      Bassareo PP, Tumbarello R, Mercuro G. Cor triatriatum and lipomatous hypertrophy of the interatrial septum in the elderly: a case report. Cardiovasc Ultrasound. 2010 Mar 9;8:4.DOI