Cor triatriatum with mitral regurgitation in a 52-year-old male

The clinical, echocardiographic, and cineangiographic features of the oldest known living case of cor triatriatum are presented. Mitral regurgitation, also found in this patient, has been reported on only two previous occasions to coexist with otherwise uncomplicated cor triatriatum. The value of two-dimensional echocardiography in the identification of this congenital anomaly is confirmed.     

Cor triatriatum: a cause of hemoptysis

Hemoptysis can be caused by either pulmonary or extrapulmonary causes. Congenital heart disease should be considered as a possible cause in patients who have no obvious evidence of pulmonary disease. We report on an 8-year-old girl who presented with recurrent hemoptysis without other cardiopulmonary signs, except for mild tachypnea and a prominent pulmonic component of the second heart sound, suggesting pulmonary hypertension. A chest X-ray revealed pulmonary venous congestion without other parenchymal disease. An echocardiogram revealed classical cor triatriatum, with a 6-mm orifice in the anomalous septum. Cardiac evaluation should be considered in patients with hemoptysis unexplained by pulmonary causes, even in the absence of overt cardiac symptoms.     

The dilated coronary sinus: utility of coronary sinus cross-sectional area and eccentricity index in differentiating right atrial pressure overload from persistent left superior vena cava

Background: Pulmonary hypertension and right atrial pressure overload (RAPO) cause dilation of the coronary sinus (CS). Persistent connection of the left superior vena cava (LSVC) to the CS is another cause of CS dilation. The purpose of this study was to evaluate the usefulness of coronary sinus cross‐sectional area (CSA) and eccentricity index (EI) in differentiating persistent LSVC from right heart overload and RAPO in patients with dilated CS. Methods: We identified 15 patients with a dilated CS by echocardiography. Offline analysis was used to measure CS‐CSA and CS‐EI at end‐diastole in the parasternal long axis plane. EI was defined as B/A, where A is the widest diameter and major axis of the CS, and B is the diameter of the minor axis (perpendicular to and bisecting A at its midpoint). Persistent LSVC was confirmed by either computed tomography or injection of agitated saline in the left antecubital vein. Results: CS‐CSA was significantly larger in PLSVC group than in group with RAPO. Also, CS‐EI was lower in PLSVC than in RAPO group (P = 0.0003). EI was the most sensitive and specific discriminator between patients with persistent LSVC vs. RAPO. CS‐EI was <0.8 in all PLSVC patients and >0.8 in all RAPO patients (sensitivity and specificity = 100%). Conclusion: Patients with persistent LSVC have a significantly higher CS‐CSA than those with elevated RA pressure. When dilated CS is present, a CS‐EI <0.8 is 100% sensitive and specific for persistent LSVC. Thus, the CS‐EI can be used in cases of dilated CS to diagnose the presence of persistent LSVC with a very high degree of certainty, and can help differentiate this congenital anomaly from RAPO. (Echocardiography 2011;28:829‐832)     

Coronary Sinus Orifice Atresia Associated with Persistent Left Superior Vena Cava: A Case Report with Literature Review

Coronary sinus (CS) orifice atresia is a rare malformation, usually associated with persistent left superior vena cava (PLSVC). The preoperative diagnosis is very challenging. The only clue for diagnosis is the reverse direction of flow through PLSVC. We present the case of a 69‐year‐old woman with CS orifice atresia and PLSVC. Then, we retrospectively study 99 cases of this malformation, including ours. This malformation is usually associated with the “enlarged” CS (>12 mm) and “small” PLSVC (≤5 mm), with the incidence of 81 and 87%, respectively, from the literature review.     

Cor triatriatum dexter assessed by three-dimensional echocardiography reconstruction in two adult patients

Cor triatriatum dexter is a rare congenital anomaly caused by persistence of the right valve of the embryonic sinus venosus, and this results in septation of the right atrium. Three-dimensional echocardiography is a novel technique that can be used to identify and characterize this entity. Here, we report two women with cor triatriatum dexter.     

Occlusion of the coronary sinus ostium: a danger for cavopulmonary anastomosis.

Coronary sinus (CS) ostial obstruction decompressing through a left superior vena cava is a rare finding and presents unique risks to patients requiring a Glenn anastomosis for single-ventricle palliation. Preoperative diagnosis is imperative in order to avoid inadvertent ligation or coil occlusion. We report three cases with single-ventricle physiology and CS obstruction identified at cardiac catheterization. Balloon occlusion angiography proved valuable in making the diagnosis.     

Cor triatriatum: the utility of cardiovascular imaging

A 44-year-old man with no known cardiac history presented with worsening dyspnea on minimal exertion. During follow-up, computed tomography angiography and echocardiography confirmed the incidental finding of cor triatriatum. As improvements in spatial and temporal resolution continue, cardiac computed tomography may become better suited to the dynamic imaging of anatomical defects in the heart, including, but not limited to, coronary artery disease.     

Aneurysmal coronary sinus associated with severe valvular heart disease

We report a case of aneurysmal coronary sinus complicating severe valvular disease with severe tricuspid regurgitation. Injection in the left superior vena cava outlines the aneurysmal coronary sinus with stasis of the contrast. As this anomaly carries special surgical implication in patients requiring open heart surgery, a correct preoperative diagnosis is mandatory.     

Persistent Left Superior Vena Cava and Absent Right Superior Vena Cava: Not Only an Anatomic Variant

Introduction:

A 71 year old asymptomatic woman came for an echocardiogram because of a left bundle branch block. A much dilated coronary sinus (CS) with an entering large vessel was found along with a mild left ventricular systolic dysfunction. Cardiac Magnetic Resonance (CMR) showed a persistent left superior vena cava (PLSVC), and an absent right superior vena cava (ARSVC). PLSVC drained into the dilated CS. No other cardiac abnormalities were found. Any late Gadolinium enhancement was also not seen. PLSVC and ARSVC are associated with sinus node and conduction tissue maldevelopment and atrial arrhythmias, and thus clinical follow up is indicated.

Conclusion:

CMR is a useful addition to echocardiogram to search for further cardiac abnormalities, and outline the anatomy with precision in doubtful cases.     

Cor triatriatum sinister: assessment by live/real time three-dimensional transthoracic echocardiography

We report a 49-year-old morbidly obese female with a poor acoustic window in whom live/real time three-dimensional transthoracic echocardiography was able to make a confident diagnosis of cor triatriatum sinister. En face views of the membrane facilitated accurate assessment of the size and shape of the large nonobstructing opening in the membrane. Maximum dimensions of the opening were 3.06 × 1.03 cm and area was 2.3 cm².     

Cor triatriatum dextro iatrogenica: an unusual complication of atrial septal defect closure device

We present a case of a rare complication of atrial septal defect (ASD) device closure causing cor triatriatum dextro iatrogenica. A 29-year-old female presented with sudden onset dysarthria and ataxia and was found to have basilar and thalamic infarcts. Further evaluation using transthoracic echocardiography revealed an ASD which was repaired using the Gore HELEX septal occluder. Transesophageal echocardiography done after 2 months of ASD closure revealed an interesting finding termed cor triatriatum dextro iatrogenica. We briefly describe the case and discuss the relevant literature.