Synovial sarcoma in the right atrium and right ventricle

A metastatic synovial sarcoma in the right atrium and ventricle is described. A 36-year-old man was admitted to our hospital with generalized fatigue, dyspnoea, and precordial pain. Transthoracic echocardiography demonstrated a metastatic tumour in both the right atrium and right ventricle and revealed obstruction of the inflow tract of the right ventricle caused by a metastatic right atrial tumour. Thoracic computed tomography revealed a pleural-based paravertebral mass in the left intrathoracic cavity and multiple pulmonary nodules in both lungs. Cardiac surgery was performed for palliative treatment due to right cardiac failure and a risk of fatal embolization. The patient died 12 months after the cardiac surgery.     

Right lung cancer with partial anomalous pulmonary venous return and absent right upper lobe

When a vascular shunt is present, major lung resection may cause acute right heart failure. We report a case of right lung cancer with ipsilateral partial anomalous pulmonary venous return (PAPVR) and absent right upper lobe managed by pneumonectomy. A 48-year-old former smoker was diagnosed as right lung cancer; chest computed tomography (CT) revealed an anomalous right pulmonary vein draining into inferior vena cava. Bronchus of right upper lobe was not found in fiberoptic bronchoscopy. Right pneumonectomy was performed. Pathological examination revealed a T2aN2M0 squamous lung cancer. The patient tolerated well without notable descending of arterial oxygen pressure.     

Complete revascularization of total obstruction of both subclavian arteries and descending abdominal aorta by combined surgery and percutaneous transluminal angioplasty

A 48-year-old man was admitted with chest pain, intermittent claudication and right upper extremity weakness. Magnetic resonance angiography revealed total occlusion of both subclavian arteries and descending abdominal aorta below renal artery. End-to-side aorta to bifemoral graft surgery and percutaneous transluminal angioplasty of both subclavian arteries were performed. Follow-up angiography after 6 months revealed patent left subclavian artery but 70% occluded right subclavian artery. Therefore re-intervention was performed at right subclavian artery.     

Bromocriptine-responsive Cushing's disease; clinical and biochemical remission accompanied by amelioration of impaired ocular movement

A 73-year-old woman presented with disturbance of right eyelid opening with ptosis and impairment of right ocular movement, and later was found to have Cushingoid features. Endocrine examinations revealed that plasma ACTH and cortisol levels were elevated, lack of circadian rhythm, resistant to low-doses (0.5, 1, 2 mg) and high-dose (8 mg) dexamethasone, and responsive to CRH and DDAVP. Magnetic resonance imaging (MRI) revealed a large pituitary tumor invading the right cavernous sinus. After two months treated with bromocriptine (5 mg/day), she showed clinical improvement with normalization of plasma ACTH and cortisol levels, and improvement of right eyelid opening and ocular movement. MRI, however, revealed no apparent reduction in the size of pituitary tumor. This is a rare case of bromocriptine-responsive Cushing's disease.     

Atypical Mycobacterial Infection in Sequestrated Lung in an Infant Presenting with Chronic Pneumonitis and Recurrent Wheezing

An infant was referred to us for persistent cough and recurrent wheezing. Chest X‐ray showed an abnormal shadow in the right lower lobe. A volumetric contrast‐enhanced computerized tomography (CT) scan of the chest revealed an abnormal blood vessel extending from the descending aorta to the right lower lobe and anomalous venous drainage from the right lower lobe into inferior vena cava. Angiography confirmed the CT scan findings. Pulmonary sequestration associated with scimitar syndrome was subsequently diagnosed. Patient underwent right lower lobectomy and right inferior pulmonary vein was anastomosed into the left atrium. Pathological examination after surgery revealed epitheloid granuloma in the sequestrated lung parenchyma and myocobacteria in the draining lymph nodes. This is a rare case of pulmonary sequestration associated with scimitar syndrome and complicated by mycobacterial infection.     

Ruptured aneurysm of the sinus of Valsalva with Wildervanck syndrome (cervico-oculo-acoustic syndrome), blepharoptosis and short stature: case report.

A 62-year-old woman was admitted to hospital because of nausea. A grade 5/6 continuous murmur was audible near the left sternal border at the second intercostal space. Chest X-ray showed cardiomegaly and bilateral pleural effusion. She was diagnosed as heart failure and a diuretic was prescribed. After remission of the heart failure, echocardiography showed shunt flow from the right coronary cusp to the right ventricle. Aortography revealed that an aneurysm of the right coronary sinus of Valsalva had ruptured into the right ventricle. Coronary angiography revealed a single coronary artery. Chest computed tomography revealed persistent left superior vena cava. Surgical repair was carried out and the patient made an uneventful recovery. In addition to these cardiovascular abnormalities, she had Wildervanck syndrome (Klippel-Feil syndrome, Duane syndrome and sensorineural hearing disturbance), blepharoptosis and short stature. This rare combination has not been reported previously.     

Hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis

A 22-year-old man was referred to our hospital because of thrombocytopenia. Abdominal computed tomography (CT) revealed hypoplasia of the right hepatic lobe, the development of porto-systemic collateral vessels, splenomegaly and a periaortic soft-tissue mass. Laboratory tests and needle liver biopsy indicated no evidence of liver cirrhosis. Consequently, a diagnosis of hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis was established. Portal hypertension and hypersplenism was thought to be the cause of the thrombocytopenia. CT arterioportography revealed that anomalies of the portal venous system could have resulted in the hypoplasia of the right hepatic lobe. This is the first report describing hypoplasia of the right hepatic lobe accompanied by supervening idiopathic retroperitoneal fibrosis.     

Right ventricular thrombus: clinical and diagnostic features

Because of clinical symptoms and findings at physical, echocardiographic, and angiographic examination, a patient was suspected of having a tumor in the right ventricle. Results of surgical and pathological examination, however, revealed a thrombus obliterating the right ventricle. The purpose of this article is to delineate the features of a patient with right ventricular thrombus.     

Arrythmogenic right ventricular cardiomiopathy in patient with abnormal origin of right coronary artery and ventricular tachycardia during exercises

A 18-year-old patient with abnormal origin of right coronary artery and ventricular tachycardia during exercises is presented. Myocardial biopsy revealed arrhythmogenic right ventricular cardiomyopathy.     

Two cases of bi-ventricular dysplasia associated with ventricular tachycardia and familial occurrence of sudden death.

Two strikingly similar patients with arrhythmogenic right ventricular dysplasia which severely impaired not only the right ventricle but also the left ventricle are described in association with familial occurrence of sudden death. A 49-year-old man experienced syncope which was due to ventricular tachycardia. Electrocardiography revealed a first degree atrioventricular block, incomplete right bundle-branch block, T wave inversions in leads II, III, a VF and V1 to V5, and multiformal ventricular extrasystoles. Echocardiography and ventricular cineangiography showed not only the right ventricular dilatation with an aneurysm in the right ventricular apex, inflow and outflow tracts, but also mild dilatation of the left ventricle with left ventricular apical and posterior aneurysms. Radionuclide angiography also disclosed dysfunction of both ventricles, especially during exercise. His family history revealed that 3 members of his family died of sudden deaths. A 56-year-old woman experienced syncope secondary to ventricular tachycardia, with left bundle-branch block. Electrocardiography showed complete right bundle-branch block, left axis deviation, and T wave inversions in leads V1 to V4. Echocardiography and ventricular cineangiography revealed not only marked right ventricular dilatation with the "triangle of dysplasia", but also a left ventricular aneurysm in the apex and posterior portion. Her elder brother died of a sudden death, and electrocardiograms of 2 members of her family showed ventricular extrasystoles and T wave inversions. These 2 cases may well be termed "familial bi-ventricular dysplasia".     

Inexcitable right ventricle and bilateral bundle branch block in Uhl's disease

A 29-year-old female with Uhl's anomaly developed complete atrioventricular (A-V) block. His bundle studies revealed block distal to the His bundle recording site with narrow QRS complexes. Right ventricular capture could not be obtained and despite successful left ventricular epicardial pacing, the patient died. Autopsy revealed absence of myocardium in most areas of the right ventricle and the right side of the ventricular septum with a normal tricuspid valve. Conduction system examination revelaed total destruction of both bundle branches. This is the first case where bilateral bundle branch block is shown to be present in Uhl's anomaly. Narrow QRS complexes probably reflected the absence of right ventricular forces.     

Successful thoracoscopic ligation and transection of racemose hemangioma of bronchial artery]

We used thoracoscopy for the successful ligation and transection of a racemose hemangioma of bronchial artery. The patient was a 61-year-old woman who had been admitted to our hospital because of hemoptysis. Bronchoscopic examination revealed bulging lesions covered with normal bronchial mucosa in the right B5 and B8, and bronchial arteriography revealed a shunt between the right bronchial arteries and pulmonary arteries and veins. Ligation and transection of the right bronchial artery under thoracoscopy was performed. Hemoptysis has not recurred 9 months after the operation. Thoracoscopic ligation and transection of bronchial artery may be an effective and less invasive procedure for the treatment of racemose hemangioma.     

The Simultaneous Occurrence of Deep Vein Thrombosis and Pulmonary and Arterial Embolization

INTRODUCTION A 45-year-old female presented with 8 hours of right lower extremity pain and dyspnea. She was tachycardic and her right lower extremity was dusky, cold, and pulseless. DISCUSSION Computerized tomography of the venous and arterial systems revealed massive pulmonary embolism and right lower extremity arterial and left lower extremity venous thromboses. Management included intra-arterial thrombolytics, right lower extremity arterial embolectomy, and anticoagulation. Echocardiogram revealed a patent foramen ovale (PFO) with a right-to-left shunt. CONCLUSION We hypothesize that our patient developed deep venous thrombosis that led to major pulmonary embolization and increased right-sided pressures. In the setting of a patent foramen ovale, a later venous embolus transversed the PFO and lodged in the femoral artery. Our case illustrates the importance of a high index of suspicion for a paradoxical embolus in patients with arterial thrombosis. This abstract was presented at the meeting of the Southern Society of General Internal Medicine in February 2005     

Portal thrombosis due to intrahepatic cholangiocarcinoma following successful treatment for hepatocellular carcinoma

A 57-year-old man, who had undergone hepatic arterial infusion chemotherapy with right portal occlusion for hepatocellular carcinoma was admitted to our hospital because of severe abdominal pain. Contrast-enhanced computed tomograms revealed that most areas of the liver were not enhanced, a finding suspicious for perfusion disturbance in the liver. Angiography revealed an interrupted right hepatic artery. Arterial portograms revealed complete obstruction of the right portal vein and a small left branch of the portal vein. Despite anticoagulant therapy with urokinase for portal vein thrombosis, the patient died from hepatorenal failure. Autopsy revealed that cholangiocarcinoma occupied almost the entire parenchyma of the right lobe, although the treated hepatocellular carcinoma lesion was completely necrotic. The right hepatic artery was obstructed due to direct invasion of tumor. There were diffuse thrombi in the left portal branches surrounded by tumor infiltrating along Glisson's sheath to the peripheral portion of the left lobe.     

A case of obturator hernia with overlooked Howship-Romberg sign and treated as sciatica

We report a case of a patient with incarcerated obturator hernia who presented with right thigh pain. An 88-year-old woman who had experienced right thigh pain for the previous 3 years was given a diagnosis of sciatica at the orthopedic department. In July 2009, she was hospitalized with the chief complaint of appetite loss. The day after admission she experienced increased right thigh pain and lower abdominal pain. Abdominal ultrasonography revealed a keyboard sign. Based on this finding, we diagnosed an ileus, which was alleviated by the insertion of an ileus tube. However, after the removal of the ileus tube, her right thigh pain recurred. Therefore, a diagnosis of hernia was considered. Contrast-enhanced computed tomography revealed an incarcerated bowel in the region between the pectineus muscle and muscle obturator. Laparotomy showed that the ileal part located about 15 cm from the terminal ileum was incarcerated in the right foramen obturatum; therefore, ileal resection and end-to-end anastomosis were performed. After the operation, her intestinal obstruction symptoms and right pain disappeared. If right pain and ileus symptoms of unknown cause occur in elderly persons, obturator hernia should be considered.     

Cardiac sarcoidosis mimicking right ventricular dysplasia.

A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.     

Right-sided myxomas with extramedullary hematopoiesis and ossification in Carney complex

The authors report the case of multiple right-sided myxomas in a 42-year-old man with Carney complex. He had previously been diagnosed as Cushing's syndrome and undergone resection of pituitary adenoma at the age of 21. After 10 years, bilateral adrenalectomy had been performed with recurrence of Cushing's syndrome. Recently, he complained of palpitation and intracardiac masses were detected. On physical examination, he had nevi on the lips and in the oral cavity. A tiny eyelid nodule was noted and histopathological analysis confirmed the diagnosis of skin myxoma. Thyroid ultrasonography revealed multiple hypoechoic nodules, which were confirmed pathologically as follicular adenomas. Scrotal ultrasonography also revealed small multiple testicular tumors. Echocardiography demonstrated intracardiac masses in right atrium and right ventricle. Right atriotomy revealed a right atrial mass attached to the annulus of the tricuspid valve and another mass arising from the interatrial septum. In right ventricle, a polypoid mass arose from the anterior leaflet of the tricuspid valve. Also, there were multiple tiny to small masses on the interatrial septum. The masses were completely excised and the tricuspid valve was repaired with annuloplasty. Histopathological analysis confirmed myxomas with foci of extramedullary hematopoiesis and ossification.     

Abdominal wall abscess secondary to subcapsular tubercular liver abscess.

We report a 22-year-old woman who presented with an abdominal wall lump in the right upper quadrant 15 days after starting antitubercular treatment for right pleural effusion. CT scan revealed a right liver lobe subcapsular abscess communicating vith subcutaneous tissue. Aspiration of pus revealed acid-fast bacilli. She responded to 9 months of antitubercular treatment.     

Exercise-induced right ventricular tachycardia and arrhythmogenic right ventricular dysplasia: electrophysiologic and therapeutic considerations

Three cases of exercise-induced ventricular tachycardia with associated right ventricular dysplasia are described. In each case, patients experienced ventricular tachycardia during treadmill tests. In all three patients, echocardiography or nuclear scintigraphy revealed a dilated right ventricle with reduced ejection fraction; cardiac catheterization with angiography also showed a dilated and hypokinetic right ventricle in each case. Two patients underwent electrophysiology studies that showed an inducible ventricular tachycardia originating from the right ventricular outflow tract. All three patients were treated with single agent antiarrhythmic drugs that controlled their ventricular tachycardia.     

Right atrial and right ventricular thrombus in a patient with hepatic carcinoma - a case report

A case of a 62-year-old male with suspected aortic dissection is presented. Echocardiography did not confirm the initial diagnosis and revealed the presence of thrombus in the inferior vena cava, right atrium and right ventricle. Computerised tomography, followed by histological examination of liver biopsy, revealed hepatic carcinoma. The patient received anticoagulation and unfortunately died 14 days later. Symptomatology of the right-heart thrombus and thrombo-embolic complications in patients with neoplastic disorders are discussed.