Rare Case of an Unroofed Coronary Sinus

Thangaroopan, Molly; Truong, Quynh A.; Kalra, Mannudeep K.; Yared, Kibar; Abbara, Suhny

doi:10.1161/circulationaha.107.707018

Cited by 43 publications

(18 citation statements)

References 2 publications

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“…Previous classification of CS anomaly was suggested by Mantini and et al 1 as following: “1- Enlargement of the CS with or without left-to-right shunt, 2- Absent of CS, 3- Atresia of coronary sinus ostium, 4- Hypoplastic CS”. The morphological types have been divided into four groups: type I, completely unroofed with persistent LSVC; type II, completely unroofed without persistent LSVC; type III, partially unroofed mid portion (as illustrated in our case); and type IV, partially unroofed terminal portion ( 3 ).…”

Section: Discussionmentioning

confidence: 95%

Partially unroofed coronary sinus with intact atrial septum in an elderly patient

et al. 2012

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Section: Discussionmentioning

confidence: 95%

Partially unroofed coronary sinus with intact atrial septum in an elderly patient

et al. 2012

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“…This lesion was considered as the rarest type of atrial septal defect ( 1 ). Unroofed CS is often seen associated with other congenital heart disease, particularly persistent left superior vena cava (LSVC) that drains directly into the coronary sinus ( 1 , 2 ); however, other types of congenital heart disease such as Cor triatriatum, pulmonary atresia, tetralogy of Fallot, and anomalous pulmonary venous drainage are reported in patients with coronary sinus defects ( 3 ). It is most commonly diagnosed in childhood.…”

Section: Discussionmentioning

confidence: 99%

“…Accordingly, TEE that is able to assess these posterior structures more accurately, particularly in mid-esophageal long-axis views, often confirms the anomaly ( 5 ). Recently, multidetector computed tomography (MDCT) and cardiac magnetic resonance imaging have provided promising results ( 2 , 6 - 8 ). In addition, several arrhythmias have been also reported in patients with unroofed CS.…”

Section: Discussionmentioning

confidence: 99%

“…Unroofed CS is often associated with other congenital abnormalities. Patients may present with dyspnea, positional or transient cyanosis, hypoxia, paradoxical embolus, cerebral abscess or right-sided heart enlargement and failure or they may be completely asymptomatic ( 1 , 2 ). The case presented here is a 48-year-old patient with a history of surgical closure of an atrial septal defect (ASD) in whom an unroofed coronary sinus was discovered during work up for palpitation.…”

Section: Introductionmentioning

confidence: 99%

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Partially unroofed coronary sinus, persistent left superior vena cava and cortriatriatum: A rare combination of interruption in normal embryogenesis

Peighambari¹,

Esmaeilzadeh²,

Alizadehasl³

et al. 2014

Res Cardiovasc Med

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“…11,12) However, it should be kept in mind that the usual timing of CT image acquisition is set to obtain the maximum enhancement of arterial structure. Therefore, when the contrast media is injected through the right arm in patients with unroofed CS and PLSVC, if there is no left-to-right shunt through an unroofed CS, the contrast enhancement cannot be observed in the CS.…”

Section: )mentioning

confidence: 99%

Imaging of Right-to-Left Shunt in an Adult Patient with Unroofed Coronary Sinus with Persistent Left Superior Vena Cava

et al. 2017

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SummaryA 54-year-old woman with a history of multiple cardiac surgeries suffered from hypoxemia caused by a right-to-left intra-cardiac shunt due to coronary sinus (CS) anomaly with persistent left superior vena cava (PLSVC). Both the contrast echocardiography and enhanced computed tomography (CT) provided conclusive diagnosis of this rare congenital anomaly, which was overlooked for a long time. However, an important diagnostic clue was left-arm injection of the contrast media. In the present case, previously performed enhanced CT with its routine manner, i.e., contrast through the right arm, missed this anomaly. It is crucial to note that the unusual type of unroofed CS with PLSVC, presenting with an entirely right-to-left intra-cardiac shunt, cannot be delineated on an enhanced routine chest CT if the contrast media is injected through the right arm.(Int Heart J 2017; 58: 1008-1011) Key words: Echocardiography, Computed tomography, Diagnosis U nroofed coronary sinus (CS) is a rare congenital cardiac anomaly with a reported prevalence of only 0.1% among all congenital heart diseases. 1) In this pathology, there is a significant communication between the CS and the left atrium (LA) due to complete or partial absence of the roof or septum between the CS and the LA. Other than persistent left superior vena cava (PLSVC), which is complicated with as much as 75% of the cases of this pathology, 1) other complicated malformations could critically affect the clinical manifestations of unroofed CS. 2) Here, we describe an adult case who suffered from hypoxemia due to a variant form of this pathology overlooked for a long time; then, we reemphasize "classical" diagnostic clues and pitfalls of this pathology that we learned from the present case. Case ReportA 54-year-old woman was referred to our department for evaluation of hypoxemia. She had a history of multiple cardiac surgeries: repair of pulmonary artery stenosis during childhood, repair of ruptured sinus of Valsalva at 25 years of age, and aortic valve replacement at 35 years of age. She underwent aortic valve re-replacement for prosthetic valve regurgitation 13 months ago. She had taken medications for hypertension after the latest surgery. Although hypoxemia was noted before she underwent the latest cardiac surgery, the etiology was considered as transient pulmonary congestion caused by a prosthetic valve regurgitation. Following successful surgery, arterial oxygen saturation of less than 90% at rest on room air was finally recognized as hypoxemia of unknown etiology. Her physical examination was unremarkable, without grade 2/6 systolic ejection murmur heard at the aortic area. A chest radiogram did not show pulmonary congestion. Transthoracic echocardiography revealed abnormal ventricular septal motion, presumably due to postoperative pericardial adhesions. However, there was neither evidence of elevated left ventricular filling pressure nor abnormalities in the postsurgical lesions. Moreover, the CS did not appear dilated on the transthoracic echocardiography....

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Rare Case of an Unroofed Coronary Sinus

Cited by 43 publications

References 2 publications

Partially unroofed coronary sinus with intact atrial septum in an elderly patient

Partially unroofed coronary sinus with intact atrial septum in an elderly patient

Partially unroofed coronary sinus, persistent left superior vena cava and cortriatriatum: A rare combination of interruption in normal embryogenesis

Imaging of Right-to-Left Shunt in an Adult Patient with Unroofed Coronary Sinus with Persistent Left Superior Vena Cava

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