An unusual case of infective endocarditis involving a right coronary artery to superior vena cava fistula

Coronary artery fistulas (CAFs) are rare and mostly congenital anomalous connections between a coronary artery and a cardiac chamber or great vessel. Most CAFs are small, asymptomatic, and found incidentally during cardiac imaging. However, they can lead to serious complications including myocardial infarction, congestive heart failure, arrhythmias, or fistula rupture. CAFs have been associated with infective endocarditis, but to our knowledge, this complication has never been reported involving an isolated CAF to an otherwise anatomically normal great vessel. We report the first case of this complication in a 49‐year‐old man with a presumed streptococcus vegetation found within an isolated large, tortuous CAF connecting the right coronary artery to the superior vena cava. After completing antibiotic treatment, transcatheter closure of the CAF was performed. Since then, the patient has remained symptom‐free. This case demonstrates that CAF closure is feasible following CAF‐associated endocarditis, and that closure may represent a viable strategy for reducing risk of recurrent infection. © 2014 Wiley Periodicals, Inc.     

A case of absent right superior vena cava with persistent left superior vena cava: Cross-sectional echocardiographic diagnosis

Summary We report a case of absence of the right superior vena cava. Hitherto, the findings in this condition have only been obtained using invasive methods; this report is the first in which the diagnosis was made by means of echocardiography. The findings include a markedly enlarged coronary sinus draining into the right atrium and a specific finding of contrast echocardiography in which contrast material injected into the right antecubital vein can be seen in the enlarged coronary sinus. The contrast echo flows into the right atrium from the coronary sinusonly. The size of the coronary sinus was far beyond the range (91±38 mm²/m²) in patients with persistent left superior vena cava with the presence of the right superior vena cava.     

Persistent left superior vena cava,absent right superior vena cava and coronary-bronchial fistula: The good,the bad and the ugly (case report and review of literature)

A 62-year-old Caucasian male presented with syncope during casual daily activity without preceding prodromes. During ECG Holter monitoring, we observed numerous asystolic pauses lasting >4 s due to sino-atrial blockade and sinus bradycardia. During pacemaker implantation, persistent left superior vena cava with agenesis of the right superior vena cava was diagnosed. Unproblematic placement of atrial lead was followed by challenging placement of the right ventricular lead. Anterior position with a sharp angulation to the right ventricular wall was achieved with excellent stimulation parameters. Transesophageal echocardiography confirmed the diagnosis of persistent left superior vena cava with agenesis of right superior vena cava. Moreover, selective coronary angiography showed connection between right coronary artery branch and bronchial vessel. To the best of our knowledge, we are the first to describe a combination of persistent left superior vena cava with absent right superior vena cava, coronary-bronchial fistula and conduction abnormality with the necessity of device implantation.     

Aortocaval fistula complicating abdominal aortic aneurysm: Case report and literature review

Aortocaval fistula is a rare complication of abdominal aortic aneurysm involving less than 1% of all abdominal aortic aneurysms. The diagnosis is difficult, and preoperative identification can be challenging. We describe an interesting case of a 72-year-old man presenting with acute-onset dyspnea who was found to have an aortocaval fistula secondary to a large abdominal aortic aneurysm. The clinical features that exemplify aortocaval fistula are discussed and the literature reviewed. © 1996 Wiley-Liss, Inc.     

Large left circumflex coronary artery with a fistula to superior vena cava: Diagnosis by echocardiography

The left circumflex coronary artery associated with a fistula to superior vena cava is a rare entity. We describe a 7‐year‐old girl who presented with a cardiac murmur and was diagnosed with a coronary artery fistula between the left circumflex artery and superior vena cava by echocardiography. The surgical occlusion of the fistula was successful.     

Congenital aphallia associated with congenital urethrorectal fistula: A rare case report

Rationale:Aphallia is an extremely rare congenital malformation of unknown cause, with few reports in the literature. It is usually associated with other urogenital and gastrointestinal anomalies and is believed to be a result of either the absence of a genital tubercle or chromosome polymorphism. Herein, we describe an extremely rare case of congenital aphallia with congenital urethrorectal fistula and describe our treatment for this patient.Patient concerns:An 8-year-old boy was brought to our hospital by his parents because of congenital absence of the penis. The child was male per karyotype and had excess heterochromatin on chromosome 9 (46 XY with 9 qh+). No urethral orifice was identified, and urine passed rectally since birth; thus, urinary tract outlet obstruction led to urine reflux from the anus to the epididymis for a long time. The boy had to be placed on prophylactic antibiotics because he developed urinary tract infection and epididymitis almost every day.Diagnosis:Congenital aphallia (46 XY normal male karyotype) associated with congenital urethroretal fistula.Interventions:We performed urethral exteriorization via perineal urethroplasty and urethrorectal fistula repair. The parents approved for phallic reconstruction when the boy reached puberty.Outcome:A new external urethral orifice was created on the lower scrotum. The urinary reflux was corrected, and the epididymitis symptoms disappeared. The urethral fistula was then closed. At 8 months follow up, the patient was no longer on antibiotics and had no symptoms of urinary tract infection or epididymitis.Conclusions:Compatible treatment should be adopted to address urinary tract drainage and infection. Management requires a stepwise approach to address needs as they arise. Neophalloplasty should be performed by an experienced team in early adolescence.     

Giant coronary sinus and absent right superior vena cava

Although persistent left superior vena cava (PLSVC) is rare, it is the most common thoracic venous anomaly. The prevalence of PLSVC is 0.3 % in a general population and 10 % in patients with congenital heart disease. Diagnosis of PLSVC is usually incidental during cardiovascular imaging or surgery. Although PLSVC is usually not associated with any negative hemodynamic effect, it is important to be aware of its existence, since it may cause problems in central venous catheterization, pacemaker implantation and cardiopulmonary bypass. The overall proportion of absent right superior vena cava in patients with PLSVC is approximately 20 %. If PLSVC is associated with absence of the right superior vena cava, the coronary sinus may become gigantic due to excessive inflow. Herein, we report a case of PLSVC complicated by the absence of a right superior vena cava resulting in a giant coronary sinus.     

Chemoradiation-induced superior vena cava syndrome: A case report

A case of a 54-year-old man who developed superior vena cava syndrome secondary to vascular fibrosis, 30 months after radical chemoradiation for stage III non-small cell lung cancer, is presented. The literature regarding the etiology, diagnosis and treatment of this rare entity is discussed.     

Bilateral absence of the superior vena cava presenting with superior vena cava syndrome: A case report

Bilateral absence of the superior vena cava (SVC) is a very rare congenital vascular anomaly that is mainly asymptomatic. In this report, we describe an adult male patient with bilateral absence of the SVC presenting with SVC syndrome. Blood from the upper body returned to the right atrium via the superficial thoracoepigastric veins, the great saphenous veins, the common femoral veins, and the inferior vena cava.     

Unroofed coronary sinus and persistent left superior vena cava.

This report describes a case of unroofed coronary sinus and persistent left superior vena cava discovered during an echocardiographic investigation for dilated pulmonary artery. An unroofed coronary sinus is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava. The latter is a usual cause of a dilated coronary sinus. The detection of a dilated coronary sinus should therefore prompt the search for abnormal coronary sinus drainage and other cardiac abnormalities. The treatment of unroofed coronary sinus and persistent left superior vena cava is undertaken only after assessing the pre- and post-treatment haemodynamics of all co-existing abnormalities.     

Aortic pseudoaneurysm as a rare cause of superior vena cava syndrome--a case report

This case is about an aortic pseudoaneurysm after Dacron graft replacement that presented as a rapidly evolving superior vena cava syndrome.     

Absent right superior vena cava with persistent left superior vena cava: Implications and management

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 in a 26 day old infant with multiple congenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals a short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well; the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.     

Atresia of the right atrial orifice of the coronary sinus with persistent left superior vena cava: a case report

A 39-year-old woman presented with atresia of the right atrial orifice of the coronary sinus with a persistent left superior vena cava detected at cardiac catheterization. She was admitted with frequent episodes of angina at rest and on exertion. Coronary angiography, including spasm provocation test, yielded normal results. However, left coronary arteriography demonstrated a dilated coronary sinus and a persistent left superior vena cava draining into the innominate vein. The contrast medium leaked slightly into the right atrial cavity through the obstructed orifice of the coronary sinus. Atresia of the coronary sinus orifice is a rare malformation usually found at autopsy. Only 3 cases have been reported in Japan. This is the first adult Japanese case detected when the patient was still alive.     

Malignant non-Hodgkin's lymphoma in the superior vena cava and the right ventricle. A case report

A 39-year old patient presented with small, sudden hemoptyses and a diagnosis of pulmonary tuberculosis was made on radiographic pleural and parenchymatous lesions but without bacteriological confirmation. Two years later, the patient was admitted to hospital following severe hemoptysis. Echocardiography revealed the presence of a tumour in the right ventricular outflow tract. At surgery, nodules were also resected from the superior vena cava. Histological examination of the surgical specimens suggested that the tumour was an angiosarcoma. Six months later, the patient returned complaining of a painful swelling in the left iliac fossa. Immuno-histochemical analysis of the cardiac and abdominal tumours finally concluded that it was in fact a malignant non-Hodgkinian lymphoma.     

Persistent left superior vena cava draining into the coronary sinus: report of 10 cases and literature review

BACKGROUND: Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly. HYPOTHESIS: This study examines the epidemiologic, clinical, and morphologic characteristics of a cohort of patients with PLSVC draining into the coronary sinus. METHODS: We examined the clinical and morphologic characteristics of patients with PLSVC draining into the coronary sinus diagnosed at a single referral hospital for a defined population in northwestern Spain. We designed a prospective study of the case records of all patients diagnosed with PLSVC draining into the coronary sinus at the echocardiography laboratory of the Hospital Xeral-Calde from January 2001 through December 2002. Patients were included if they had a PLSVC diagnosed by transthoracic echocardiogram (TTE) using an echo-contrast enhancement and confirmed by a magnetic resonance (MR) imaging. Ten patients (6 women) fulfilled the inclusion criteria described above. All patients were adults and had associated heart disease, including a congenital heart disease in three cases. RESULTS: Magnetic resonance imaging examination confirmed the presence of PLSVC and the site of drainage into the coronary sinus. Absence of the right superior vena cava was observed only in three patients, in whom the main coronary sinus size was significantly increased. Absence of the left brachiocephalic vein was diagnosed in five patients. CONCLUSION: This study describes 10 new cases of PLSVC and supports the necessity of considering PLSVC draining into the coronary sinus in the diagnosis of patients presenting with dilated coronary sinus diagnosed by TTE. It also underlines the important role of MR imaging in the evaluation of these abnormalities. An associated heart disease must always be excluded in these patients.