Papillary fibroelastoma prolapsing into the left ventricular outflow tract: diagnosis using three-dimensional TEE.

A 72-year-old woman was admitted to our hospital with exertional dyspnea and atrial tachycardia due to atypical atrial flutter. Comprehensive three-dimensional transesophageal echocardiography (3D-TEE) with offline reconstruction of the dataset (Siemens Foursight TEE, Erlangen, Germany) allowed visualization of the origin of the tumor and gave important additional information about the tumor: the tumor surface was floppy, it had the appearance of a "sea anemone", and it was attached to the endocardium of the head of the papillary muscle by a short stalk. The tumor was assessed to be 1.9 × 1.4 × 0.9 cm, and a prolapsing of the tumor into the left ventricular outflow tract during systole was demonstrated.A tumor size of 8 × 10 mm and an infiltration of the head of the anterior papillary muscle were found during surgery. The histopathological findings were typical for a papillary fibroelastoma (PFE) with involvement of the head of the papillary muscle.Our case indicates that a PFE might be difficult to distinguish from myxoma by echocardiography. Thus, the multimodal imaging approach and the three-dimensional visualization of the surface, the localization, and the attachment of the tumor to the head of a papillary muscle were very helpful for the identification of a fibroelastoma in this particular case.

Addison's disease due to bilateral adrenal malignancy in a dog.

A 12-year-old Rottweiler cross Labrador was presented with anorexia and weakness. Adrenal insufficiency was diagnosed with hyponatraemia, hyperkalaemia and undetectable resting and post-ACTH cortisol and aldosterone concentrations. The only abnormal diagnostic imaging result was bilateral adrenomegaly. Cytologic findings of liver, spleen and peripheral lymph nodes were normal. The dog responded initially to standard replacement therapy but relapsed shortly afterwards. The owners opted for euthanasia and allowed only removal of both adrenal glands. Microscopically, infiltrative polymorphic proliferations of densely packed tumour cells arranged as nests, intermingled with multifocal areas of necrosis and inflammatory cells were found. Silver staining revealed a few non-neoplastic adrenomedullary cells, whereas neoplastic cells did not stain. Immunohistochemistry was negative for neuron-specific enolase, vimentin, cytokeratin, synaptophysin, chromogranin A, S-100 protein, CD 56, 79 and 3. The final diagnosis was highly anaplastic bilateral adrenal neoplasia. This is the first report of bilateral adrenal malignancy presenting as clinical hypoadrenocorticism in a dog.

[Purple glove syndrome - a case report]. / Purple Glove Syndrom nach peripherer venöser Phenytoin Gabe - Ein Fallbericht.

The purple glove syndrome (PGS) is a soft tissue injury after peripheral intravenous phenytoin administration or oral overdosage. The incidence of PGS is described with 0-6%. Typical symptoms are purple discoloration, oedema, pain, and a decrease of range of motion. In severe cases PGS may lead to abscess, skin loss and compartment syndrome. The established treatment of PGS is immediate interruption of phenytoin injections, splinting, elevation, and close observation. In cases of severe complications (e. g., compartment syndrome), surgical intervention is necessary. The case of a 40-year-old female patient is reported who was transferred to our department 4 days after intravenous phenytoin administration and who underwent successful surgical revision.

[Heterotopic pancreatic tissue in the bifurcation of the bile duct : Rare diagnosis mimicking a Klatskin tumour]. / Heterotopes Pankreasgewebe in der Hepatikusgabel : Seltene Differenzialdiagnose zum Klatskin-Tumor.

Heterotopic tissue of the pancreas can be found in 1-2% of autopsies. The main locations are stomach, duodenum, jejunum, Meckel's diverticulum, and ileum. Less frequently it is observed in the liver, gallbladder, common bile duct, and papilla of Vateri. We report the first case of heterotopic pancreatic tissue in the bifurcation of the bile duct mimicking a Klatskin's tumour. The case is discussed based on the current literature.

[Correction of finger clubbing in primary hypertrophic osteoarthropathy (Touraine-Solente-Gole syndrome)]. / Korrektur der Trommelschlegelfinger unter ästhetischen Aspekten bei hereditärer hypertropher Osteoarthropathie (Touraine-Solente-Gole-Syndrom).

Finger clubbing can be a single physical finding. In Touraine-Solente-Gole syndrome, the primary form of hypertrophic osteoarthropathy, it is mostly associated with bone pain, hyperhydrosis, pachydermy and wrinkling of the forehead. In other cases, the presence of clubbing is associated with neoplastic, pulmonary, cardiac or other diseases and is then called Bamberger-Pierre-Marie syndrome, the secondary type of hypertrophic osteoarthropathy. The patient's history and careful physical examination, sometimes accompanied by laboratory and imaging studies, leads to the diagnosis. A patient with hereditary hypertrophic osteoarthropathy and its clinical symptoms is presented. Surgical correction of the clubbing fingers is demonstrated in the paper with bilateral resection and shortening of the nail bed, nail matrix and resection of soft tissue. Clubbing fingers are rare, but they might be part of a syndrome or a symptom of other diseases. Reconstructive surgery for aesthetic reasons can be performed.

[Malignant eccrine poroma]. / Das maligne ekkrine Porom.

INTRODUCTION: Malignant eccrine poroma is a very rare tumour of the sweat glands with high malignancy and presenting with a polymorph clinical and histological picture. CASE REPORT: We describe the case of a 99-year-old patient with a malignant poroma on the buttock. Despite the large size of the tumour, no metastasis was found with standard examination techniques. Radical excision and defect closure with a Limberg flap was performed. RESULT: The healing course was uneventful and without complications. CONCLUSION: The malignant poroma is a tumour of high malignancy which can easily be misdiagnosed because of its different forms of presentation. Radical surgical therapy is the only known effective treatment.

Unusual case of an 18-year-old heart transplant recipient with endocardial fibroelastosis.

An 18-year-old female Japanese patient who suffered from heart failure and severe pulmonary hypertension was referred to our clinic. The etiology of her cardiomyopathy was unclear. Inhaled prostacyclin therapy resulted in an improvement of pulmonary arterial pressure and allowed us to avoid lung transplantation. Heart transplantation resulted in a complete remission of her respiratory function. Autopsies of the explanted heart revealed massive endomyocardial fibroelastosis. We concluded that endomyocardial fibroelastosis has to be considered a cause of heart failure in young adults with unclear cardiomyopathy.

Aortic dissection after previous coronary artery bypass grafting.

Aortic dissection after coronary artery bypass grafting (CABG) is a rare but potentially fatal complication. The aim of this study was to identify the reasons. Between 1991 and 2000 in our institution CABG was performed on 22,732 patients. In the same time interval 12 (0.5 degree/00) patients presented with an aortic dissection after previous CABG. Age: 59.1 +/- 5.9 years, gender: 10/2, only Stanford A dissections, 4 chronic and 8 acute dissections, mortality: 3, all acute. 2 died of cardiac complications (left heart failure), 1 of other complications (gastrointestinal ischemia). The time interval between CABG and dissection was 2.5 +/- 3.6 years. Two dissections were intraoperative, another 5 were within the first year; the longest time interval was 10 years. In 5 cases the entry originated from a central anastomosis, 1 originated from the aortic cannulation site, and 1 from the site of the cross clamping. In 5 cases the entry was not directly related to the previous operation (1 was located in close proximity to the left coronary ostium, 2 between aortic valve annulus and the coronary ostia and 2 between the distal coronary arteries in the ascending aorta). Pathological changes of the aorta were not described at the time of CABG; only in 1 case a mild aortic regurgitation and dilatation (47 mm) at the time of the first operation was described. As our results suggest an aortic dissection presenting after CABG must be considered to be a rare complication of the previous operation. Considering the severity of this complication satisfying results can be achieved.

Transcoronary ablation of septal hypertrophy (TASH): a new treatment option for hypertrophic obstructive cardiomyopathy.

In 1991, our group started to develop a catheter interventional therapy for hypertrophic obstructive cardiomyopathy (HOCM). The new concept was proposed in 1994. It is based on the conventional PTCA technique with the aim of inducing an artificial myocardial infarction by instillation of 96% ethanol into the most proximally situated septal branch of the left anterior descending coronary artery. This leads to a subaortic contraction disorder with subsequent decrease of the intraventricular pressure gradient, shrinkage of the hypertrophied septal bulge and widening of the outflow tract ("therapeutic remodeling"). The subaortic defect is small and well demarcated as assessed by left ventricular angiography, transesophageal echocardiography and 18 F-glucose positron emission tomography. The term transcoronary ablation of septum hypertrophy (TASH) was suggested. Our patient cohort that now comprises 215 therapeutic procedures in 187 patients underwent a large variety of prospective studies (maximum follow-up 4.5 years) including invasive controls at regular intervals, investigation of hemodynamics at rest and at exercise, transesophageal and transthoracic echocardiography, Doppler echocardiography during bicycle exercise, electrophysiologic testing, Holter monitoring and measurement of myocardial metabolism and perfusion, assessment of microembolic events by transcranial Doppler sonography and histological examinations. This article gives an overview and reports our increasing experience in applying TASH. The following post-TASH findings were obtained: significant hemodynamic and clinical improvement at rest and at exercise, decrease of septum thickness, increase of outflow tract area and decrease of induced ventricular tachycardia. There were well-demarcated, histologically atypical subaortic myocardial defects, no microembolic events, abnormal early peak of infarct related enzymes, and no change of baroreflex sensitivity. Pre-/post-TASH evaluations of the patients should be based in particular on clinical symptoms correlated to the intraventricular gradient measured by bicycle exercise Doppler echocardiography and to outflow tract area as assessed by transesophageal echocardiography. Since 1994, as a roughly estimate, worldwide 1000 patients in 20 countries have been treated. According to published articles, abstract presentations and workshops, TASH consistently leads to a pronounced clinical and hemodynamic benefit for patients with HOCM. TASH has become an established technique. At least in centers with a high level of expertise, it is no longer experimental but a routinely performed alternative to surgical treatment for HOCM, i.e., the previous gold standard of therapy. Of course, patient outcome needs further careful clinical and prognostic evaluation. With respect to complications, TASH appears to be superior to surgery (transaortic septal myectomy) for HOCM. Like surgical treatment, TASH is currently indicated in critically ill patients with typical HOCM (subaortic form), who exhibit with drug refractory symptoms, including patients, who preferred DDD pacemaker therapy as a first therapeutic step but in whom this produced no subsequent clinical benefit.

Acute and long-term results after transcoronary ablation of septal hypertrophy (TASH). Catheter interventional treatment for hypertrophic obstructive cardiomyopathy.

AIMS: To evaluate acute and long-term symptomatic, haemodynamic (at rest and during exercise) and electrophysiological results of transcoronary ablation of septal hypertrophy (TASH), a catheter interventional treatment for hypertrophic obstructive cardiomyopathy. METHODS AND RESULTS: Sixty-two transcoronary ablations of septal hypertrophy were performed by injection of 4.6+/-2.6 ml 96% ethanol into septal branches in 50 patients with hypertrophic obstructive cardiomyopathy and severe symptoms. Serial left and right heart catheterization, transoesophageal echocardiography and electrophysiological investigations were repeated 2 weeks and 7+/-1 months (n=37) after intervention. Transcoronary ablation of septal hypertrophy led to a reduction in septal thickness, sustained elimination of the outflow obstruction (51+/-41 vs 6+/-10 mmHg at rest, P<0.001; 134+/-48 vs 28+/-32 mmHg, P<0.001, post-extrasystolic), a decrease in left ventricular filling pressures at rest and during exercise and a pronounced clinical improvement. There was no evidence for the creation of an arrhythmogenic substrate as assessed by serial programmed electrical stimulation in 39 patients. However, permanent high-grade atrioventricular block occurred in 17% of the patients. There were two early, but no late deaths during a mean follow-up time of 10. 6+/-5.6 months. CONCLUSION: Transcoronary ablation of septal hypertrophy is a promising new treatment for hypertrophic obstructive cardiomyopathy in patients with severe symptoms. It should now be compared with alternative treatment strategies in prospective randomized studies.

Vascular hamartoma of the left ventricle.

Primary tumors of the heart are very rare. We report a case of a 28-year old female patient in whom a tumor of the left ventricle was first diagnosed by transthoracic echocardiography. Angiography, nuclear magnetic resonance imaging and fasting positron emission tomography with 18-fluorodesoxyglucose suggested the diagnosis of a well vascularized tumor. The tumor was subtotally excised during heart surgery under total cardiopulmonary bypass and histological examination identified a predominantly vascular hamartoma.

[Emboliform sarcoma of the right pulmonary artery]. / Emboliformes Sarkom der rechten Arteria pulmonalis.

Sarcoma of the pulmonary artery is a rare tumor. The histopathologic features of these tumors differ widely, with leiomyosarcomas representing less than 20% of all pulmonary sarcomas. Diagnosis is very difficult because most of the cases have no specific clinical signs: the major differential diagnosis is that of pulmonary thromboembolism. We report the case of a 45-year-old woman, who was first treated for pulmonary thromboembolism until the diagnosis of an intravascular growing tumor was made by means of computed tomography, pulmonary angiography and nuclear magnetic resonance tomography. The patient underwent right pneumonectomy and microscopically a leiomyosarcoma of the right pulmonary artery was found.

The pharmacological effect of sodium phosphate after absorption from the peritoneal cavity.

Hyperosmolar solutions are frequently used in clinical practice for enemas. In a review of the literature we found 43 published cases of severe complications connected with the use of hyperosmolar enemas. The absorption of sodium phosphate led to a raise of the temperature up to 41.1 degrees C, massive acidosis and electrolyte disturbances with hypocalcemia and hypernatremia. The clinical picture was dominated in most of the cases by somnolence or coma. In our experiment the absorption of sodium phosphate from the peritoneal cavity of rats led to similar clinical symptoms and--depending on the amount of sodium phosphate absorbed--to the death of the animals. Hypovolemia, hypernatremia, hypocalcemia and a direct toxic effect of phosphate on the kidneys is responsible for the complex mode of intoxication. Sodium phosphate solutions should not be used in patients with inflammatory bowel disease with a high risk of laceration of the mucosa or perforation of the bowel.

[Incidence of carcinoma in follicular neoplasia of the thyroid gland]. / Carcinomincidenz bei folliculären Neoplasien der Schilddrüse.

Within four years 1376 thyroid operations were performed. In 152 = 11% a follicular or oncocytic tumor was found, 20 = 13% of these were classified as carcinoma. Seven of these were of the grossly invading type, raising suspicion of malignancy already pre- and intraoperatively while 13 encapsulated tumors were found by the pathologist only. If preoperatively a cold nodule or a nodular goitre was described, the carcinoma incidence was about 16%, while warm or hot nodes showed a carcinoma in 2%. Besides tumors of < 1 cm in diameter where we found no carcinoma the size of follicular tumors had no influence on the incidence of carcinomas.

[Metastasizing round-cell sarcoma of the right atrium]. / Metastasierendes Rundzellsarkom des rechten Vorhofs.

The chest roentgenogram in a 46-year-old woman with dyspnoea for several months revealed global cardiac enlargement. Echocardiography demonstrated a tumour in the right atrium, about 4 x 5 cm, as well as circular pericardial effusion. Septic temperatures occurred in the further course, and blood cultures grew Staphylococcus aureus. Recurrent pulmonary emboli and cerebral emboli with hemiparesis, especially of the brachiofacial region, occurred despite heparinization. Shortly after hospitalization a chest roentgenogram revealed a round shadow, about 1 cm in diameter, in the left upper lobe. The patient died in cardiogenic shock on the 16th hospital day. Autopsy showed the tumour to be a poorly differentiated round-cell sarcoma, originating from the wall of the right atrium, infiltrating the myocardium through to the epicardium and extending to the tricuspid orifice. The foramen ovale was open, making paradoxical emboli at atrial level possible.