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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

Pulmonary angiogram by contrast-enhanced magnetic resonance angiography (MRA) of patient A. Axial (a) and sagittal (c) oblique multiplanar reconstruction and coronal maximum intensity projection (b) of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). The right upper lobe drains into the right superior vena cava (dotted arrow). RA right atrium, LA left atrium, Ao aorta, SVC superior vena cava
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Fig2: Pulmonary angiogram by contrast-enhanced magnetic resonance angiography (MRA) of patient A. Axial (a) and sagittal (c) oblique multiplanar reconstruction and coronal maximum intensity projection (b) of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). The right upper lobe drains into the right superior vena cava (dotted arrow). RA right atrium, LA left atrium, Ao aorta, SVC superior vena cava

Mentions: Transthoracic echocardiography showed a dilated and hypertrophied right ventricle with a moderately decreased systolic function and a moderate pulmonary valve stenosis (peak gradient 42 mmHg) with a mild pulmonary regurgitation. The pulmonary arterial pressure (PAP) could not be estimated. He underwent MRI to quantify the extent of right ventricular (RV) dilatation and dysfunction. The right ventricle was moderately dilated (RV end-diastolic volume 304 ml versus left ventricular end-diastolic volume 198 ml) and systolic function was mildly impaired (ejection fraction 45 %). Flow through the pulmonary artery was larger than through the aorta, and there was a consistently larger stroke volume of the right compared with the left ventricle, which could not be explained by valvular regurgitation. Magnetic resonance angiography revealed a partial aberrant pulmonary venous connection (PAPVC) of the right upper lobe to the superior caval vein (Fig. 2). Subsequently, a cardiac CT was performed, which revealed an UCS type II (Fig. 3). In addition, a pseudo-aneurysm was seen at the distal anastomosis of the homograft.Fig. 2


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)

Pulmonary angiogram by contrast-enhanced magnetic resonance angiography (MRA) of patient A. Axial (a) and sagittal (c) oblique multiplanar reconstruction and coronal maximum intensity projection (b) of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). The right upper lobe drains into the right superior vena cava (dotted arrow). RA right atrium, LA left atrium, Ao aorta, SVC superior vena cava
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4016326&req=5

Fig2: Pulmonary angiogram by contrast-enhanced magnetic resonance angiography (MRA) of patient A. Axial (a) and sagittal (c) oblique multiplanar reconstruction and coronal maximum intensity projection (b) of the unroofed coronary sinus (*), the coronary sinus defect (thick arrow) and the entry of the coronary sinus into the right atrium (thin arrow). The right upper lobe drains into the right superior vena cava (dotted arrow). RA right atrium, LA left atrium, Ao aorta, SVC superior vena cava
Mentions: Transthoracic echocardiography showed a dilated and hypertrophied right ventricle with a moderately decreased systolic function and a moderate pulmonary valve stenosis (peak gradient 42 mmHg) with a mild pulmonary regurgitation. The pulmonary arterial pressure (PAP) could not be estimated. He underwent MRI to quantify the extent of right ventricular (RV) dilatation and dysfunction. The right ventricle was moderately dilated (RV end-diastolic volume 304 ml versus left ventricular end-diastolic volume 198 ml) and systolic function was mildly impaired (ejection fraction 45 %). Flow through the pulmonary artery was larger than through the aorta, and there was a consistently larger stroke volume of the right compared with the left ventricle, which could not be explained by valvular regurgitation. Magnetic resonance angiography revealed a partial aberrant pulmonary venous connection (PAPVC) of the right upper lobe to the superior caval vein (Fig. 2). Subsequently, a cardiac CT was performed, which revealed an UCS type II (Fig. 3). In addition, a pseudo-aneurysm was seen at the distal anastomosis of the homograft.Fig. 2

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