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Unroofed coronary sinus newly diagnosed in adult patients after corrected congenital heart disease.

Pérez Matos AJ, Planken RN, Bouma BJ, Groenink M, Backx AP, de Winter RJ, Koolbergen DR, Mulder BJ, Boekholdt SM - Neth Heart J (2014)

Bottom Line: Patients with congenital heart disease corrected in early childhood may later in life present with cardiac symptoms caused by other associated congenital anomalies that were initially not diagnosed.Nowadays, several noninvasive imaging modalities are available for the visualisation of cardiac anatomy in great detail.We describe two patients with an unroofed coronary sinus, a rare congenital anomaly which could be diagnosed using a combination of modalities including echocardiography, cardiac CT and cardiac MRI.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, the Netherlands.

ABSTRACT
Patients with congenital heart disease corrected in early childhood may later in life present with cardiac symptoms caused by other associated congenital anomalies that were initially not diagnosed. Nowadays, several noninvasive imaging modalities are available for the visualisation of cardiac anatomy in great detail. We describe two patients with an unroofed coronary sinus, a rare congenital anomaly which could be diagnosed using a combination of modalities including echocardiography, cardiac CT and cardiac MRI.

No MeSH data available.


Related in: MedlinePlus

Computed tomography angiography (CTA) images after intravenous contrast administration of patient B. Coronal (a&d) and axial (b&c) oblique multiplanar reconstructions of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). Note the persistent left superior vena cava (c&d). RA right atrium, LA left atrium, Ao aorta
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Fig5: Computed tomography angiography (CTA) images after intravenous contrast administration of patient B. Coronal (a&d) and axial (b&c) oblique multiplanar reconstructions of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). Note the persistent left superior vena cava (c&d). RA right atrium, LA left atrium, Ao aorta

Mentions: At physical examination, her saturation was 92 % at rest, which dropped to 72 % after mild exercise. Transthoracic echocardiography revealed a moderately dilated right ventricle with a normal systolic function and severe tricuspid valve insufficiency due to tricuspid annular dilation in combination with retraction of the septal leaflet caused by the pacemaker lead. The systolic PAP was estimated at 30 mmHg using the tricuspid insufficiency signal. Colour Doppler showed a flow signal from the left to the right atrium suggesting an atrial septum defect (ASD) (Fig. 4). After infusion of agitated saline contrast, a right-to-left shunt at atrial level was seen, suggesting a patent foramen ovale or an ASD with right-to-left shunting. Transoesophageal echocardiography confirmed an 8 mm defect in the posterior part of the interatrial septum with bidirectional flow matching the diagnosis of ASD type UCS. Cardiac CT showed a PLVCS draining into a dilated coronary sinus, which was partially unroofed causing communication with the left atrium (Fig. 5).Fig. 4


Unroofed coronary sinus newly diagnosed in adult patients after corrected congenital heart disease.

Pérez Matos AJ, Planken RN, Bouma BJ, Groenink M, Backx AP, de Winter RJ, Koolbergen DR, Mulder BJ, Boekholdt SM - Neth Heart J (2014)

Computed tomography angiography (CTA) images after intravenous contrast administration of patient B. Coronal (a&d) and axial (b&c) oblique multiplanar reconstructions of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). Note the persistent left superior vena cava (c&d). RA right atrium, LA left atrium, Ao aorta
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4016326&req=5

Fig5: Computed tomography angiography (CTA) images after intravenous contrast administration of patient B. Coronal (a&d) and axial (b&c) oblique multiplanar reconstructions of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). Note the persistent left superior vena cava (c&d). RA right atrium, LA left atrium, Ao aorta
Mentions: At physical examination, her saturation was 92 % at rest, which dropped to 72 % after mild exercise. Transthoracic echocardiography revealed a moderately dilated right ventricle with a normal systolic function and severe tricuspid valve insufficiency due to tricuspid annular dilation in combination with retraction of the septal leaflet caused by the pacemaker lead. The systolic PAP was estimated at 30 mmHg using the tricuspid insufficiency signal. Colour Doppler showed a flow signal from the left to the right atrium suggesting an atrial septum defect (ASD) (Fig. 4). After infusion of agitated saline contrast, a right-to-left shunt at atrial level was seen, suggesting a patent foramen ovale or an ASD with right-to-left shunting. Transoesophageal echocardiography confirmed an 8 mm defect in the posterior part of the interatrial septum with bidirectional flow matching the diagnosis of ASD type UCS. Cardiac CT showed a PLVCS draining into a dilated coronary sinus, which was partially unroofed causing communication with the left atrium (Fig. 5).Fig. 4

Bottom Line: Patients with congenital heart disease corrected in early childhood may later in life present with cardiac symptoms caused by other associated congenital anomalies that were initially not diagnosed.Nowadays, several noninvasive imaging modalities are available for the visualisation of cardiac anatomy in great detail.We describe two patients with an unroofed coronary sinus, a rare congenital anomaly which could be diagnosed using a combination of modalities including echocardiography, cardiac CT and cardiac MRI.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, St. Antonius Hospital, Koekoekslaan 1, 3435 CM, Nieuwegein, the Netherlands.

ABSTRACT
Patients with congenital heart disease corrected in early childhood may later in life present with cardiac symptoms caused by other associated congenital anomalies that were initially not diagnosed. Nowadays, several noninvasive imaging modalities are available for the visualisation of cardiac anatomy in great detail. We describe two patients with an unroofed coronary sinus, a rare congenital anomaly which could be diagnosed using a combination of modalities including echocardiography, cardiac CT and cardiac MRI.

No MeSH data available.


Related in: MedlinePlus