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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

Schematic drawing of an unroofed coronary sinus. SVC  superior vena cava, IVC inferior vena cava, LA left atrium, RA right atrium, RV right ventricle, LV left ventricle
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Fig1: Schematic drawing of an unroofed coronary sinus. SVC  superior vena cava, IVC inferior vena cava, LA left atrium, RA right atrium, RV right ventricle, LV left ventricle

Mentions: An unroofed coronary sinus (UCS) is a rare congenital heart anomaly, first described in 1965 by Raghib et al. [1] An UCS is often associated with a persistent left vena cava superior (PLVCS) and other congenital heart defects including ventricular septum defect, atrioventricular septal defect, cor triatum and tetralogy of Fallot [1–3]. Kirklin and Barratt-Boyes classified the anatomical variants of UCS as follows: type I: completely unroofed coronary sinus with a PLVCS; type II: completely unroofed coronary sinus without PLVCS; type III: partially unroofed coronary sinus in the mid-portion; and type IV: partially unroofed coronary sinus in the terminal portion [4]. Under normal circumstances, the coronary sinus drains blood from the cardiac veins into the right atrium. An UCS is caused by partial or complete absence of the partition between the coronary sinus and the left atrium, which leads to communication between the left and right atrium and interatrial shunt. In addition, the communication between the coronary sinus and the left atrium causes aberrant drainage of the cardiac venous system into the left atrium (see Fig. 1 for a schematic drawing). If an associated PLVCS exists, the systemic venous return via the PLVCS is also drained aberrantly into the left atrium. As a result of the right-to-left communication, there is mild desaturation as well as an increased risk of cerebral emboli and brain abscess [1]. On the other hand, the left-to-right shunt causes volume overload of the right ventricle which can cause right-sided heart failure.Fig. 1


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)

Schematic drawing of an unroofed coronary sinus. SVC  superior vena cava, IVC inferior vena cava, LA left atrium, RA right atrium, RV right ventricle, LV left ventricle
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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

Fig1: Schematic drawing of an unroofed coronary sinus. SVC  superior vena cava, IVC inferior vena cava, LA left atrium, RA right atrium, RV right ventricle, LV left ventricle
Mentions: An unroofed coronary sinus (UCS) is a rare congenital heart anomaly, first described in 1965 by Raghib et al. [1] An UCS is often associated with a persistent left vena cava superior (PLVCS) and other congenital heart defects including ventricular septum defect, atrioventricular septal defect, cor triatum and tetralogy of Fallot [1–3]. Kirklin and Barratt-Boyes classified the anatomical variants of UCS as follows: type I: completely unroofed coronary sinus with a PLVCS; type II: completely unroofed coronary sinus without PLVCS; type III: partially unroofed coronary sinus in the mid-portion; and type IV: partially unroofed coronary sinus in the terminal portion [4]. Under normal circumstances, the coronary sinus drains blood from the cardiac veins into the right atrium. An UCS is caused by partial or complete absence of the partition between the coronary sinus and the left atrium, which leads to communication between the left and right atrium and interatrial shunt. In addition, the communication between the coronary sinus and the left atrium causes aberrant drainage of the cardiac venous system into the left atrium (see Fig. 1 for a schematic drawing). If an associated PLVCS exists, the systemic venous return via the PLVCS is also drained aberrantly into the left atrium. As a result of the right-to-left communication, there is mild desaturation as well as an increased risk of cerebral emboli and brain abscess [1]. On the other hand, the left-to-right shunt causes volume overload of the right ventricle which can cause right-sided heart failure.Fig. 1

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