Giant renal arteriovenous malformation: a suspicious bruit

Arteriovenous (AV) malformations remain relatively rare clinical lesions consisting in abnormal shunts between the arterial and venous vascular systems. Both,congenital or acquired fistulas, are unusual causes of renal or heart failure. Congenital AV fistulas usually present with hematuria, while acquired ones are more likely to present with hemodynamic changes, such as hypertension, cardiomegaly and congestive heart failure. Here we present a 61 year-old woman studied before by probable pulmonary arteriovenous fistula and referred to nephrologist with a six-month history of dyspnea and peripheral edema. Physical examination was remarkable for an apical systolic ejective murmur as well as another bruit over the right pulmonary base. Blood tests showed a sCr of 1.7 mg/dl with normal urinalysis. On imaging, renal duplex ultrasound showed a pulsatile hiliar mass on the right kidney with an arterial flow of 300 cm/sec that was identified as a giant AV fistula on MRA. An echocardiogram revealed severe pulmonary hypertension with a cardiopulmonary output of 12,9 l/min that doubled the systemic one (6,49 l/min). In view of the large size of the AV fistula occupying the majority of the parenchyma, endovascular approach was turned down and a laparoscopic nephrectomy was performed successfully. There was an immediate clinical improvement and 45 days after the procedure, a control-echocardiogram showed normalization of the cardiopulmonary parameters with minimal changes in glomerular filtration rate.     

Cardiac effects of persistent hemodialysis arteriovenous access in recipients of renal allograft

In hemodialysis patients, large arteriovenous (AV) fistulas for vascular access may cause ventricular hypertrophy and high-output cardiac failure. The long-term cardiac consequences of functional AV fistulas in renal transplant patients are unclear. A precise knowledge of these consequences is important to decide if and when such fistulas should be closed in successfully transplanted patients. In this retrospective study including 61 stable renal transplant patients with adequate renal function (serum creatinine <2.0 mg/100 ml), echocardiography was performed in 39 patients with a functional AV fistula (group 1) and in 22 whose fistulas had been closed, for esthetic reasons, within 2 months postoperatively (group 2). The volume flow of the fistulas, measured in 22 randomly selected individuals of group 1, was 900 +/- 350 ml/min (range 500-1,600). Patients of group 1 were older (40 +/- 12 vs. 33 +/- 12 years, p < 0.05), had longer duration of the fistula (62 +/- 31 vs. 36 +/- 30 months, p < 0.05), higher body mass index (24 +/- 4 vs. 22 +/- 3 kg/m2, p < 0.05), systolic (154 +/- 24 vs. 138 +/- 18 mm Hg, p < 0.05) and diastolic (96 +/- 12 vs. 89 +/- 11 mm Hg, p < 0.05) blood pressure and increased left ventricular (LV) end-diastolic dimension (53 +/- 5 vs. 49 +/- 5 mm, p < 0.01). LV mass, cardiac index, ejection fraction and the proportion of patients with LV hypertrophy were comparable in the two groups. LV end-diastolic dimension was positively and independently influenced only by the presence of the AV fistula (p < 0.01) after adjusting for age, duration of the fistula, body mass index, systolic and diastolic blood pressure and the nature of the antihypertensive drugs used. In conclusion, the persistence of large, high-flow AV fistulas for prolonged periods of time had little impact on cardiac morphology and function of stable renal transplant patients with adequate renal function. The data do not support routine closure of these fistulas in all renal transplant patients.     

Diagnostic implications of MR imaging for mucinous adenocarcinoma arising from fistula in ano

Mucinous adenocarcinoma associated with chronic fistula in ano is rare, and diagnosis is often difficult. Two cases of mucinous adenocarcinoma arising from fistula in ano occur in a patient with longlasting fistulas, persisting for more than 10 years, are presented. In two cases, T2-weighted MR images revealed symptomatic features; that is, the hyperintense heterogeneous content looked like a gathering of various sizes of granules. This is due to the fact that mucinous adenocarcinomas usually consist of the gathering of many small mucous lakes. This finding has the implications in diagnosing mucinous adenocarcinoma arising from carcinoma in ano.     

Plexus network between internal mammary graft and pulmonary vasculature after coronary bypass grafting

Left internal mammary artery to pulmonary vasculature fistulas are rare complications following coronary artery bypass grafting surgery. In symptomatic cases, management may either be conservative, or involve coil embolisation or surgical ligation of the fistula. We describe a 52-year-old male patient who suffered from an acute coronary syndrome due to significant stenosis of the right coronary artery 12 years after coronary artery bypass grafting surgery. Coronary angiography accidentally revealed a fistulous connection between the left internal mammary artery and the left pulmonary vasculature. The patient underwent percutaneous coronary intervention for his right coronary artery and was managed conservatively for his fistula.     

Left ventricular intracavitary mass and pericarditis secondary to metastatic renal cell carcinoma--a case report

Cardiac metastases from renal cell carcinoma are a well-recognized entity. However, this phenomenon is extremely rare in the absence of vena caval extension. The authors report a patient who after successful resection of renal cell carcinoma presented with left ventricular mass causing left ventricular outflow tract obstruction. There was also metastatic pericardial and intramyocardial involvement. Such a unique combination of cardiac metastasis, in the same patient, has not been reported previously.     

Spontaneous idiopathic subclavian arteriovenous fistula: a case report.

Systemic arteriovenous (AV) fistulas are a rare but correctable cause of hyperkinetic circulation and congestive heart failure. They are generally due to catheterization procedures, surgery, trauma, or aneurysms. A case of truly spontaneous AV fistula between left subclavian artery and left innominate vein is described. This patient presented with symptoms that might clinically be mistaken for a carpal tunnel syndrome. The discovery of a continuous to-and-fro murmur on her left upper sternal border led to the correct diagnosis. Surgical ligature of the fistula provided complete relief of the woman's symptoms.     

Percutaneous intervention for restoration of patency of occluded lower limb arteriovenous dialysis access

BackgroundArteriovenous (AV) fistula thrombosis is a serious complication in patients undergoing hemodialysis, often presenting with symptoms of venous hypertension, failure to dialysis and uremic symptoms. Treatment is aimed to provide symptomatic relief and to maintain hemodialysis access site patency.AimTo describe our initial experience in the endovascular treatment of lower limb AV dialysis access (AV fistula) thrombosis and/or obstruction in patients undergoing hemodialysis.Settings and designThis was a retrospective study carried out in a tertiary care center. Study duration was 24 months. Follow-up was variable.Materials and methodsTwo patients with chronic kidney disease with stage 5 renal failure undergoing hemodialysis presented with lower limb arteriovenous dialysis access (arteriovenous fistula) failure between July 2014 and September 2016. Both the patients underwent endovascular treatment and were analyzed retrospectively.Results and conclusionBoth the patient underwent successful endovascular treatment for the failure of the lower limb AV dialysis access thrombosis and/or obstruction. One patient had minimal dye extravasation during manipulation of the guide wire, which ceased spontaneously. On follow-up, both patients maintained patency of the dialysis access and are undergoing successful hemodialysis. One patient had a recurrence of the thrombosis of the fistula at 9^th month of the follow-up. Endovascular treatment was tried but we could not succeed. However, we found endovascular treatment safe and effective in treating AV fistula failures.     

Ventricular metastasis without atrial or caval involvement: a rare presentation

Right ventricular metastases from renal cell carcinoma without inferior vena cava (IVC) or right atrium involvement are rare. We discuss a 44-year-old male who presented with a left thigh mass, which was resected and the pathology revealed an epithelial sarcoma or a possible metastasis from another organ. His preradiotherapy cardiac evaluation showed mass in the right and left ventricles, which was confirmed by transesophagial echocardiogram. Computed tomography revealed a right renal mass in addition to the ventricular masses without any involvement of IVC or the atria. The patient underwent right radical nephrectomy without complications and systemic chemotherapy with interleukin-1 for metastatic renal cell carcinoma. He is doing well and follow-up echocardiogram showed significant reduction in the size of the cardiac metastases, even without surgical resection of these tumors. Thus, our article presents this unique case of asymptomatic ventricular metastases of renal cell carcinoma without any atrial or caval involvement and provides a new insight into the management of this syndrome.     

Enterovesical fistula caused by a bladder squamous cell carcinoma

Enterovesical fistulas are not uncommon in patients with inflammatory or malignant colonic disease, however, fistulas secondary to primary bladder carcinomas are extremely rare. We herein reported a patient presenting with intractable urinary tract infection due to enterovesical fistula formation caused by a squamous cell carcinoma of the urinary bladder. This patient underwent en bloc resection of the bladder dome and involved ileum, and recovered uneventfully without urinary complaint. To the best of our knowledge, this is the first case reported in the literature.     

Achalasia of the Esophagus in Association with Renal Cell Carcinoma

A case of secondary achalasia due to renal cell carcinoma is presented. The patient presented with typical clinical, radiologic, and manometric features of achalasia, and was found to have a renal cell carcinoma with metastases to lymph nodes in the region of the cardioesophageal junction. Direct esophageal involvement could not be demonstrated, however. Complete symptomatic remission was obtained with a single hydrostatic balloon dilatation of the cardioesophageal junction and was maintained until the patient's death 7 months later, perhaps as a result of regression of paraesophageal nodal metastases following radiotherapy, immunotherapy, and chemotherapy.     

Percutaneous occlusion with coils of coronary artery fistulas in adults

Coronary artery fistulas are one of the most common congenital anomalies of the coronary arteries. Most fistulas are small and of no clinical significance, although larger or multiple fistulas can be symptomatic and produce complications. Early percutaneous occlusion is now always recommended, and surgical closure is restricted to use for multiple or large fistulas. Here we report four cases of coronary fistula draining from the coronary arteries to the pulmonary vascular bed, which were treated with percutaneous occlusion by coils.     

Coronary arterial fistulas in childhood

We reviewed 16 patients with coronary arterial fistulas seen between 1976 and 1997, and aged 2 days to 16 years, with a median age of 3.2 years. Only four patients were symptomatic: two had heart failure, one had exertional dyspnoea, and one infective endocarditis. The fistulas originated from the right coronary artery in seven patients, from the left coronary artery in seven, from both coronary arteries in one patient, while the origin was not clearly defined in the final patient. Associated cardiac anomalies were discovered in six patients, with three of the fistulas being diagnosed at the same presentation. Cross-sectional echocardiography had revealed a dilated coronary artery in 7 out of 11 subjects. The ratio of pulmonary to systemic flows ranged between 0.9 to 3.0, with a median of 1.5. Ten patients were referred for corrective surgery without any mortality. Trans-catheter closure was successfully undertaken in one patient, while spontaneous closure of the fistula was noted in two patients. We conclude that coronary arterial fistulas, although rare and potentially serious, are generally treatable.     

Pulmonary hypertension as a presentation of hepatocarcinoma. Report of a case and brief review of the literature

A case, unique in the literature, is reported in which a primary carcinoma of the liver presented a right-sided heart failure and pulmonary hypertension. The diagnosis of hepatocarcinoma was established by needle biopsy of the liver. Later, postmortem examination demonstrated that the pulmonary arterial tree was severely compromised by multiple tumor microemboli, despite the persistent lack of characteristic roentgenographic abnormality in our patient. In reviewing the literature, we found rare cases of occult renal cell carcinoma, choriocarcinoma and one of occult hepatocarcinoma, which presented as pulmonary embolism. These were diagnosed by pulmonary embolectomy, human chorionic gonadotrophin levels or autopsy, respectively. In another small group of reported cases of known carcinoma (gastric, breast, colonic) the patients had a clinical picture of "idiopathic" pulmonary hypertension or of pulmonary hypertension with pulmonary metastases. Pulmonary hypertension in these cases resulted from carcinomatous lymphangitis and/or tumor microembolization, as in our case. We report this case to emphasize the necessity of including occult carcinoma in the differential diagnosis of pulmonary hypertension and right ventricular failure.     

Percutaneous transcatheter closure of a large systemic to pulmonary venous fistulae in an adult patient after extracardiac fontan

Vascular fistulae are frequent complications in patients who have undergone a Fontan operation for palliation of single ventricle physiology. Fistulae involving the pulmonary vasculature may result in progressive hypoxemia, pulmonary hemorrhage, and clinical symptoms. These are commonly managed by percutaneous transcatheter embolization utilizing coils, and more recently, vascular plugs and septal occluders. We present a clinical case of an adult patient who underwent an extracardiac Fontan procedure in childhood for univentricular physiology and presented with symptoms of systemic desaturation 10 years after his surgery. The patient was found to have a large fistula from the inferior vena cava to the right inferior pulmonary vein. The fistula was attempted to be closed with a 12 mm Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN). Angiography showed continued flow across the fistula, which was then successfully occluded with an 18 mm Amplatzer “Cribriform” Septal Occluder (St. Jude Medical, St. Paul, MN). The patient experienced immediate improvement in his systemic saturation, and demonstrates continued resolution of his symptomatic hypoxia on follow‐up. This case illustrates an uncommon systemic to pulmonary vein fistula after Fontan, and a unique, successful embolization with two septal occluders, resulting in sustained symptomatic improvement. © 2015 Wiley Periodicals, Inc.     

Vascular Access in Patients Treated With Chronic Hemodialysis for 30 Years or More

The aim of our study was to evaluate vascular access in patients treated with chronic hemodialysis for 30 years or more. Patients who had started dialysis in 1978 or earlier were identified from the Slovenian Renal Replacement Therapy Registry. The data on vascular access on April 2008 are presented. Sixteen patients were still alive, seven men and nine women aged 62 ± 12 years (46–84), and they had been treated for 32 ± 1.7 years (30–35), mainly with chronic HD. They had started HD at the age of 30 ± 12 years (13–50), and none had diabetes. The vascular access in nine was a native arteriovenous (AV) fistula, on the forearm in eight patients, and a brachiobasilic fistula in one patient. Four patients had their primary AV fistulas still in use (maximum 35 years). In the remaining five patients, multiple salvage procedures had been performed or new AV fistulas created. The vascular access in four patients was the polytetrafluoroethylene (PTFE) graft, functioning for 1–8 years. In three patients, a non‐cuffed, single‐lumen hemodialysis catheter (a precurved jugular in two patients and a subclavian in one) locked with 30% citrate, with mupirocin at the exit site, was used for 5–12 years. The catheters were exchanged approximately once every two years over a guide‐wire because of mechanical damage. None of these three patients had had catheter‐related sepsis or exit‐site infection. Before catheters, these patients had had multiple AV fistulas and PTFE grafts. Although native AV fistula is the predominant type of vascular access, a greater than 30‐year survival on hemodialysis is possible with the combined use of AV fistula, PTFE graft, and a non‐cuffed hemodialysis catheter locked with citrate.     

Coronary artery fistulas: clinical and therapeutic considerations

Coronary artery fistulas vary widely in their morphological appearance and presentation. These fistulas are congenital or acquired coronary artery abnormalities in which blood is shunted into a cardiac chamber, great vessel, or other structure, bypassing the myocardial capillary network. The majority of these fistulas arise from the right coronary artery and the left anterior descending coronary artery; the circumflex coronary artery is rarely involved. Clinical manifestations vary considerably and the long-term outcome is not fully known. The patients with coronary fistulas may present with dyspnea, congestive heart failure, angina, endocarditis, arrhythmias, or myocardial infarction. A continuous murmur is often present and is highly suggestive of a coronary artery fistula. Differential diagnosis includes persistent ductus arteriosus, pulmonary arteriovenous fistula, ruptured sinus of Valsalva aneurysm, aortopulmonary window, prolapse of the right aortic cusp with a supracristal ventricular septal defect, internal mammary artery to pulmonary artery fistula, and systemic arteriovenous fistula. Although noninvasive imaging may facilitate the diagnosis and identification of the origin and insertion of coronary artery fistulas, cardiac catheterization and coronary angiography is necessary for the precise delineation of coronary anatomy, for assessment of hemodynamics, and to show the presence of concomitant atherosclerosis and other structural anomalies. Treatment is advocated for symptomatic patients and for those asymptomatic patients who are at risk for future complications. Possible therapeutic options include surgical correction and transcatheter embolization. Historical perspectives, demographics, clinical presentations, diagnostic evaluation, and management of coronary artery fistula are elaborated.     

Pulmonary vein compression by tumor: an unusual Doppler flow pattern

Pulmonary venous compression caused by a large lung mass was diagnosed in a 50-year-old female with metastatic renal cell carcinoma. The pulmonary venous flow pattern on Doppler revealed high flow velocity as well as the unusual finding of continued antegrade flow throughout the cardiac cycle (without reversal during atrial contraction). Extracardiac tumors can compress pulmonary veins, mimicking pulmonary vein stenosis. This may cause dyspnea due to elevated pulmonary venous pressures. This report describes an unusual pulmonary vein blood flow pattern in a patient with lung metastases.     

Pulmonary atresia, VSD in association with coronary-pulmonary artery fistula

Congenital coronary-pulmonary artery fistula is rare in patients with pulmonary atresia and ventricular septal defect. The nomenclature, physiological, clinical, and surgical implications of these fistulas are yet to be defined. We report a one-year-old child with pulmonary atresia, ventricular septal defect, and a right coronary-pulmonary artery fistula who also had a diminutive, disconnected left pulmonary artery in addition to aortopulmonary collaterals. The patient underwent corrective surgery. However, the fate of diminutive pulmonary arteries is unknown. The literature was reviewed to explore the clinical or surgical implications of such fistulas for improved understanding and management in the future.     

Ameloblastoma of the mandible with pulmonary metastases 45 years after initial diagnosis

Ameloblastoma of the mandible is a rare odontogenic tumour that rarely metastasizes. We report a patient with a slowly progressing ameloblastoma of the mandible diagnosed at the age of seven and resected multiple times due to tumour recurrence. Multiple pulmonary metastatic nodules were resected at thoracotomy 27 years after the initial diagnosis; however, further pulmonary disease was discovered. The patient was admitted with chest pain due to pulmonary metastases 45 years after the initial diagnosis. The metastases were intraluminal and could be attributed to tumour cell aspiration during the surgical procedures on the mandible. The patient also suffered from hypercalcaemia which was attributed to a parathormone-like substance secreted by the tumour.     

Metastatic renal cell carcinoma presenting as multiple pleural tumours

A 66-year-old man was admitted with dyspnoea. Chest X-ray and chest computed tomography (CT) demonstrated a left-sided pleural effusion and multiple tumours, suggesting malignant mesothelioma in the left pleural space, but there were no pulmonary lesions. However, abdominal CT revealed a right renal tumour. An ultrasonography-guided needle biopsy of the pleural mass provided evidence of metastatic renal cell carcinoma (RCC). The pleural lesions dramatically decreased in size following right radical nephrectomy and subsequent interferon-alpha treatment. While the thorax is a frequently affected site of RCC, sole pleural metastases are rare and are often secondary to lung involvement. Batson's plexus, a network of vertebral valve-less veins with multiple connections, is likely responsible for the contralateral pleural metastases of RCC.