A Rare Association of Sinus Venosus-Type Atrial Septal Defect and Persistent Left Superior Vena Cava Detected by Transthoracic Echocardiography and Cardiac Magnetic Resonance Imaging

Abstract

Patient: Male, 60Final Diagnosis: Persistent left superior vena cavaSymptoms: —Medication: —Clinical Procedure: Transthoracic echocardiogram and cardiac magnetic resonance imagingSpecialty: CardiologyObjective:Congenital defects/diseasesBackground:Association of persistent left superior vena cava (PLSVC) and sinus venosus-type atrial septal defect (SVASD) is rare. We describe a patient with dilated coronary sinus (CS) found to have PLSVC and SVASD.Case Report:The patient is a 60-year-old man with history of stroke who underwent a transthoracic echocardiogram (TTE) for evaluation of shortness of breath. TTE demonstrated a markedly dilated CS. Agitated saline was injected into the left antecubital vein to further assess CS. The parasternal long axis view demonstrated immediate filling of the CS and confirmed the presence of a PLSVC. Apical 4-chamber view with injection of agitated saline into the right antecubital vein demonstrated immediate contrast opacification of both atria, consistent with a right to left cardiac shunt. Cardiac magnetic resonance (CMR) was performed, which confirmed the TTE findings of PLSVC and defined the cardiac shunt as SVASD.Conclusions:PLSVC should be suspected in a patient with an abnormally dilated CS. In this case we identified a rare association of PLSVC with a SVASD. TTE with agitated saline contrast injection and CMR are useful diagnostic tools for PLSVC and associated cardiac congenital anomalies, respectively.

Delayed Diagnosis of Right Heart Failure Due to Sinus Venosus Atrial Septal Defect, Persistent Left Superior Vena Cava and Partial Anomalous Pulmonary Venous Return

“…1,5 TTE has poor sensitivity (about 10%) in diagnosing SVASD, highlighting the importance of multimodality imaging in such cases. 3,6 Furthermore, multimodality imaging allows for detection of associated congenital anomalies, both common ones like PAPVR, and somewhat rarer associations like PLSVC, as seen in the present case. 2,6,7 Few case reports have been published on co-existing SVASD and PLSVC.…”

“…3,6 Furthermore, multimodality imaging allows for detection of associated congenital anomalies, both common ones like PAPVR, and somewhat rarer associations like PLSVC, as seen in the present case. 2,6,7 Few case reports have been published on co-existing SVASD and PLSVC. 6,8,9 The majority of PLSVC (92%) drain into the right atrium, via the coronary sinus, which further contributes to right ventricular volume overload.…”

Cardiac CT and Transesophageal Echocardiogram Evaluation of a Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return and a Persistent Left Superior Vena Cava

“…The prevalence of superior SVASDs and associated PAPVR co-occurring with a PLSVC is unknown. The cooccurrence of SVASD and PLSVC has been described several times in literature [3][4][5]. A patient presenting with symptoms of heart failure should receive a complete evaluation to exclude an undiagnosed SVASD.…”

“…The cooccurrence of SVASD and PLSVC has occurred several times in literature. The prevalence in the population was not found, but it is thought to be rare [3][4][5]. Further analysis of such co-occurring cases could provide additional information regarding the clinical course, particularly whether or not a duplication increases the rate at which SVASD patients develop right heart failure.…”

Multifactorial genesis of a seeming case of pulmonary hypertension

“…The association between PLSVC and sinus venous type ASD is very rare. [ 2 ] Sinus venous type ASD, if left unrepaired, may eventually lead to right heart volume overload. Herein, we describe a case of PLSVC identified in a patient with markedly dilated coronary sinus (CS) and a rare association with sinus venous type ASD.…”