PP-227 A Very Rarely Seen Cardiac Mass ( Rosai- Dorfman Disease): Case Report

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Text of PP-227 A Very Rarely Seen Cardiac Mass ( Rosai- Dorfman Disease): Case Report

  • Multi-detector computed tomography (MDCT) angiography wasperformed to ascertain pulmonary venous anatomy and to describe othersitus abnormalities accompanying dextrocardia. MDCT showed midlinelocated liver, and no spleen was observed (Fig. 1A). Multiplanarreconstruction image showed that pulmonary veins draining both lungsformed a vertical vein which connected to the portal vein, direct hepaticvein inow into the right atrium (Fig. 1B), inferior vena cava inter-ruption with prominent azygous continuation (Fig. 1C).

    Aspleniawith cardiovascular anomalies or right atrial isomerism, alsoknown as Ivemark syndrome is an example of heterotaxy syndrome. Thissyndrome is associated with high occurrence of congenital heart defectsincluding pulmonary and systemic venous anomalies. Although cathe-terization provides comprehensive data about the pulmonary venousanatomy; in patients with severe cyanosis and obstructive type connec-tion, these procedures are too risky. Because detailed depiction of anat-omy is crucial, MDCT angiography may be a useful alternative for thisgroup of patients to evaluate pulmonary veins, extracardiac great vesselanomalies and associated visceral anomalies before urgent surgicalapproaches.

    venous connection

    - PP-226

    Multimodality Imaging of Isolated Bicuspid Pulmonary ValveLeading to Pulmonay Stenosis. Sait Demirkol1, Sevket Balta2,

    A 21-year-old man was admitted to our outpatient clinic forroutine evaluation. Pansystolic murmur was heard on the left secondintercostal space. The electrocardiogram showed normal sinus rhythm.Two-dimensional transthoracic echocardiography short axis viewrevealed a peak pressure gradient over the right ventricular outowtract of 30 mm Hg (Figure 1A). Two-dimensional transesophagealechocardiography short axis view demonstrated bicuspid pulmonaryvalve (Figure 1B, arrow). Three-dimensional transesophageal echo-cardiography full-volume acquisition also showed bicuspid pulmonaryvalve (Figure 1C, arrow). To clarify this pathology, we performedcomputed tomography (CT). Transverse view of colored threedimensional volume rendered CT angiography images also demon-strated bicuspid pulmonary valve (Figure 1D, arrow). We here report acase of isolated bicuspid pulmonary valve leading to pulmonary ste-nosis. There is a difculty in imaging pulmonary valve morphology.

    Results: After surgery, in the histopathologic examination reported

    MARCH 13e16, 2014


    ABSTRACTSFigure 1. 2D TTE revealed a peak pressure gradient over the rightventricular outow tract of 30 mm Hg (Figure 1A). 2D TEE demon-strated bicuspid pulmonary valve (Figure 1B, arrow). 3D TEE full-volume acquisition showed bicuspid pulmonary valve (Figure 1C,arrow). Transverse view of colored three dimensional volume renderedMurat Unlu1, Cengiz Ozturk2, Turgay Celik1, Atila Iyisoy1,Mustafa Demir1. 1Department of Cardiology, Gulhane MedicalFaculty, Ankara, Turkey; 2Department of Cardiology, EskisehirMilitary Hospital, Eskisehir, Turkey.

    Isolated bicuspid pulmonary valve is a rare arterial valve anomaly withvery few reports in the literature. It is usually in association with othercongenital cardiac lesions. However, the true incidence of bicuspidpulmonary valve could be underestimated because of the difculty inimaging pulmonary valve morphology with conventional two-dimen-sional transthoracic echocardiography.CT angiography images also demonstrated bicuspid pulmonary valve(Figure 1D, arrow).

    S94 The American Journal of Cardiology MARCH 13e16, 2014Rosai Dorfman Disease.Conclusions: Cases with RDD without extranodal involvement have

    a benign clinical progress usually and clinic follow up is enough inthose cases. Aggressive behavior and mortality is rarely seen.For this reason, the full spectrum of non-invasive cardiac imagingmodalities should be performed in the diagnosis of bicuspid pulmo-nary valve. Multimodality imaging can help to better diagnose thiscondition.

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    A Very Rarely Seen Cardiac Mass ( Rosai- Dorfman Disease): CaseReport. E. Ozbudak, S. Yavuz, D. Durmaz, A.A. Arkan, A. Aydn,M. Kanko, T. Berki. Department of Cardiovascular Surgery, KocaeliUniversity, Kocaeli, Turkey.

    Objective: Sinus histiocytosis (Rosai-Dorfman Disease) is a rare dis-ease, which is characterized by massive lympadenopathies with un-known etiology. It was rst dened in 1969 by Rosai and Dorfman.Thefrequency of cardiac involvement is less than 1% in Rosai-Dorfmandisease (RDD). Here, we report a case with extra nodal RDD in whichcardiac involvement was detected.Methods: A- 62-years-old male patient was referred to cardiology

    clinic with the complaints of atypical chest pain and dyspnea. Hisphysical examination was unremarkable. A cardiac mass with 2x1.8 cmdimentions attached to wall of the right atrium was observed in trans-thoracic echocardiography. Thoracic Computerised Tomography (CT)and Cardiac Magnetic Rezonans (MRI) showed a mass with 37x29 mmdimensions originating from the wall of the superior vena cava andextending to the interatrial septum, and along the lateral right atrial wallto the atrioventricular groove.Figure 1. Image of the cardiac mass.