1
Multi-detector computed tomography (MDCT) angiography was performed to ascertain pulmonary venous anatomy and to describe other situs abnormalities accompanying dextrocardia. MDCT showed midline located liver, and no spleen was observed (Fig. 1A). Multiplanar reconstruction image showed that pulmonary veins draining both lungs formed a vertical vein which connected to the portal vein, direct hepatic vein inow into the right atrium (Fig. 1B), inferior vena cava inter- ruption with prominent azygous continuation (Fig. 1C). Asplenia with cardiovascular anomalies or right atrial isomerism, also known as Ivemark syndrome is an example of heterotaxy syndrome. This syndrome is associated with high occurrence of congenital heart defects including pulmonary and systemic venous anomalies. Although cathe- terization provides comprehensive data about the pulmonary venous anatomy; 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 this group of patients to evaluate pulmonary veins, extracardiac great vessel anomalies and associated visceral anomalies before urgent surgical approaches. venous connection - PP-226 Multimodality Imaging of Isolated Bicuspid Pulmonary Valve Leading to Pulmonay Stenosis. Sait Demirkol 1 , Sevket Balta 2 , Murat Unlu 1 , Cengiz Ozturk 2 , Turgay Celik 1 , Atila Iyisoy 1 , Mustafa Demir 1 . 1 Department of Cardiology, Gulhane Medical Faculty, Ankara, Turkey; 2 Department of Cardiology, Eskisehir Military Hospital, Eskisehir, Turkey. Isolated bicuspid pulmonary valve is a rare arterial valve anomaly with very few reports in the literature. It is usually in association with other congenital cardiac lesions. However, the true incidence of bicuspid pulmonary valve could be underestimated because of the difculty in imaging pulmonary valve morphology with conventional two-dimen- sional transthoracic echocardiography. A 21-year-old man was admitted to our outpatient clinic for routine evaluation. Pansystolic murmur was heard on the left second intercostal space. The electrocardiogram showed normal sinus rhythm. Two-dimensional transthoracic echocardiography short axis view revealed a peak pressure gradient over the right ventricular outow tract of 30 mm Hg (Figure 1A). Two-dimensional transesophageal echocardiography short axis view demonstrated bicuspid pulmonary valve (Figure 1B, arrow). Three-dimensional transesophageal echo- cardiography full-volume acquisition also showed bicuspid pulmonary valve (Figure 1C, arrow). To clarify this pathology, we performed computed tomography (CT). Transverse view of colored three dimensional volume rendered CT angiography images also demon- strated bicuspid pulmonary valve (Figure 1D, arrow). We here report a case of isolated bicuspid pulmonary valve leading to pulmonary ste- nosis. There is a difculty in imaging pulmonary valve morphology. For this reason, the full spectrum of non-invasive cardiac imaging modalities should be performed in the diagnosis of bicuspid pulmo- nary valve. Multimodality imaging can help to better diagnose this condition. - PP-227 A Very Rarely Seen Cardiac Mass ( Rosai- Dorfman Disease): Case Report. E. Ozbudak , S. Yavuz, D. Durmaz, A.A. Arıkan, A. Aydın, M. Kanko, T. Berki. Department of Cardiovascular Surgery, Kocaeli University, 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.The frequency of cardiac involvement is less than 1% in Rosai-Dorfman disease (RDD). Here, we report a case with extra nodal RDD in which cardiac involvement was detected. Methods: A- 62-years-old male patient was referred to cardiology clinic with the complaints of atypical chest pain and dyspnea. His physical examination was unremarkable. A cardiac mass with 2x1.8 cm dimentions 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 mm dimensions originating from the wall of the superior vena cava and extending to the interatrial septum, and along the lateral right atrial wall to the atrioventricular groove. Results: After surgery, in the histopathologic examination reported Rosai Dorfman Disease. Conclusions: Cases with RDD without extranodal involvement have a benign clinical progress usually and clinic follow up is enough in those cases. Aggressive behavior and mortality is rarely seen. Figure 1. 2D TTE revealed a peak pressure gradient over the right ventricular 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 rendered CT angiography images also demonstrated bicuspid pulmonary valve (Figure 1D, arrow). Figure 1. Image of the cardiac mass. MARCH 13e16, 2014 P O S T E R A B S T R A C T S S94 The American Journal of Cardiology â MARCH 13e16, 2014 10 th INTERNATIONAL CONGRESS OF UPDATE IN CARDIOLOGY AND CARDIOVASCULAR SURGERY ABSTRACTS / Poster

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

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MARCH 13e16, 2014

POSTER

ABSTRACTS

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 inflow 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,Murat 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 difficulty inimaging pulmonary valve morphology with conventional two-dimen-sional transthoracic echocardiography.

Figure 1. 2D TTE revealed a peak pressure gradient over the rightventricular outflow 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 renderedCT angiography images also demonstrated bicuspid pulmonary valve(Figure 1D, arrow).

S94 The American Journal of Cardiology� MARCH 13e16, 2014

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 outflowtract 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 difficulty in imaging pulmonary valve morphology.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.

- PP-227

A Very Rarely Seen Cardiac Mass ( Rosai- Dorfman Disease): CaseReport. E. Ozbudak, S. Yavuz, D. Durmaz, A.A. Arıkan, A. Aydın,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 first defined 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 cardiologyclinic 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.

Results: After surgery, in the histopathologic examination reportedRosai Dorfman Disease.

Conclusions: Cases with RDD without extranodal involvement havea benign clinical progress usually and clinic follow up is enough inthose cases. Aggressive behavior and mortality is rarely seen.

Figure 1. Image of the cardiac mass.

10th INTERNATIONAL CONGRESS OF UPDATE IN CARDIOLOGYAND CARDIOVASCULAR SURGERY ABSTRACTS / Poster