Rest perfusion abnormalities in hypertrophic cardiomyopathy: correlation with myocardial fibrosis and risk factors for sudden cardiac death.

AIM: To measure the prevalence of abnormal rest perfusion in a population of consecutive patients with known hypertrophic cardiomyopathy (HCM) referred for cardiovascular MRI (CMR), and to assess any associations between abnormal rest perfusion and the presence, pattern, and severity of myocardial scar and the presence of risk factors for sudden death. MATERIALS AND METHODS: Eighty consecutive patients with known HCM referred for CMR underwent functional imaging, rest first-pass perfusion, and late gadolinium enhancement (LGE). RESULTS: Thirty percent of the patients had abnormal rest perfusion, all of them corresponding to areas of mid-myocardial LGE and to a higher degree of segmental hypertrophy. Rest perfusion abnormalities correlated with more extensive and confluent LGE. The subgroup of patients with myocardial fibrosis and rest perfusion abnormalities (fibrosis+/perfusion+) had more than twice the incidence of episodes of non-sustained ventricular tachycardia on Holter monitoring in comparison to patients with myocardial fibrosis and normal rest perfusion (fibrosis+/perfusion-) and patients with no fibrosis and normal rest perfusion (fibrosis-/perfusion-). CONCLUSIONS: First-pass perfusion CMR identifies abnormal rest perfusion in a significant proportion of patients with HCM. These abnormalities are associated with the presence and distribution of myocardial scar and the degree of hypertrophy. Rest perfusion abnormalities identify patients with increased incidence of episodes of non-sustained ventricular tachycardia on Holter monitoring, independently from the presence of myocardial fibrosis.

Dosimetric and image quality comparison of two digital mammography units with different target/filter combinations: Mo/Mo, Mo/Rh, W/Rh, W/Ag.

PURPOSE: Our aim was to allocate a digital mammography unit to the screening programme on the basis of the ALARA (as low as reasonably achievable) radiation protection principle. MATERIALS AND METHODS: Two Hologic Selenia mammography units were studied: one with a molybdenum anode and the other with a tungsten anode. After optimisation of the image production chain, we evaluated doses in a phantom under standard conditions. In this phase, we exposed a polymethyl-methacrylate (PMMA) phantom to the two mammography units and recorded the exposure parameters used by them. The phantom was subsequently replaced by a dedicated Radcal ionisation chamber on which preliminary dose assessments were conducted. Image quality of the two systems was compared by exposing a phantom containing geometrical inserts and setting the exposure parameters used for the dose assessments on each mammography unit. Dosimetric assessments of exposure data were recorded from the mammographic examinations of approximately 400 women (1,600 exposures). RESULTS AND CONCLUSIONS: The unit with the tungsten anode achieved a lower patient dose. As a result, the Selenia-W device was allocated to the breast screening programme.

Primary synovial sarcoma of the kidney. A case report with pathologic appraisal investigation and literature review.

Synovial sarcoma (SS) is a soft tissue neoplasm with clearly defined histologic, immunohistochemical and molecular features that usually arises in the extremities of young adults. The occurrence of these tumors in the kidney is extremely rare and have been prevalently described in case reports. The objectives of this work were to evaluate the frequency of primary renal synovial sarcomas and the pathologic progression in recognition of this possibly under-diagnosed entity. A comprehensive review of the literature has also been performed with a focus on survival. We report the clinico-pathological features of an intrarenal SS occurring in a 67-year-old man. The tumour, measuring 4 cm in its greatest diameter, completely replaced the cortex and the medulla of the inferior region of the left kidney compressing the iliopsoas muscle. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumour was composed of atypical monotonous vimentin+, CD99+, bcl-2+ spindle cells exhibiting a haphazard fascicular growth pattern and a high mitotic rate (3 to 5 mitoses per HPF). The diagnosis was supported by reverse transcription-polymerase chain reaction which demonstrated SYT-SSX2 gene fusion. The patient was alive with local recurrence of disease 24 months after surgery. Synovial sarcomas occurring in the kidney, in analogy to other sites, tend to have an aggressive biologic behaviour. Despite being extremely uncommon, with only 44 cases reported to date, they should be included in the differential diagnosis of benign and malignant spindle cell tumours of the kidney. This study also emphasizes the importance of a correct pathologic diagnosis for prognostic and therapeutic implications.

The role of early radiological studies after gastric bariatric surgery.

BACKGROUND: The authors investigated early radiological findings after gastric surgery for morbid obesity to evaluate their usefulness in avoiding complications or facilitating treatment. MATERIAL AND METHODS: 413 patients underwent gastric bariatric surgery: 327 had vertical banded gastroplasty (VBG), 55 Roux-en-Y gastric bypass (RYGBP), 22 adjustable silicone gastric banding (ASGB), and 9 biliopancreatic diversion (BPD). A radiological upper gastrointestinal investigation employing water-soluble contrast medium was performed in each patient between the 2nd and 8th postoperative day. Several techniques were employed to assess different radiological findings related to the anatomic modifications after the bariatric surgery. RESULTS: In VBGs, delayed emptying was found in 10 patients (3%), gastric leak in 3 patients (0.9%), vertical suture breakdown in 1 patients (0.3%), and a wide pouch in 4 patients (1.2%). In RYGBP, a leak was detected in 2 patients (3.6%), delayed emptying in 2 (3.6%), and a wide pouch in 5 (9.1%). ASGB required band enlargement for stomal stenosis in 6 patients (27.2%). Temporary delayed emptying from stomal stenosis was also observed in 2 BPDs (22.2%). Overall complications were 35/413 (8.2%). Two cases of gastric leak after VBG were reoperated. Stomal stenosis after ASGB were treated by percutaneous band deflation; other cases were medically treated until complete healing. CONCLUSIONS: Early radiological study after gastric bariatric surgery is advisable, since it detected postoperative complications (gastric perforation, stomal stenosis, etc.) and modified the clinical approach. As the interpretation of these radiographs is often difficult, involving different projections or patient's positions or other technical managements, surgeons and radiologists must interact and be knowledgable.

Dramatic worsening of vascular calcifications after kidney transplantation in spite of early parathyroidectomy.

Vascular calcification is a common feature in chronic dialysis patients, but their clinical significance is debated and the role of kidney transplantation (TP) in the natural history of their development has received scanty attention. We will describe a case of dramatic worsening of vascular calcifications during TP in a young patient in spite of early and successful parathyroidectomy (PTX), and will discuss other causes which might be putatively linked to vascular damage during the time of TP. A 37-year-old man on regular dialytic treatment (RDT) for 11 years, received his first cadaveric transplantation in January 1993. He underwent PTX 6 months after TP because of the lack of decreasing in parathyroid hormone values despite normal graft function. Although PTX was effective, a dramatic worsening was evident in large as well as in medium and small-sized arteries during the following three years of TP. In February 1997, few months after starting dialysis again because of the recurrence of his primary membranoproliferative glomerulonephritis (MPGN), the patient experienced myocardial infarction followed by aorto-coronary bypass (right coronary artery and anterior descending coronary artery) and leg "claudicatio". Though a role for parathyroid hormone in vascular disease has been commonly accepted, the case here reported clearly shows that blunting parathyroid gland activity may be unable to avoid the worsening of a process of vascular disease during the time of TP. Many other factors--linked to the time of TP--may be involved in vascular diseases, such as nephrotic syndrome, dyslipidemia, hypertension and drugs. In the case of our patient, a clear cut risk factor for his progressive atherosclerosis can be designated hyperlipidema and other disturbancies secondary to a nephrotic syndrome due to relapse of MPGN, together with persistent hypertension. This is the first case report in the English literature which clearly demonstrates that TP may add fuel to the fire of vascular disease also in young people and even in the absence of parathyroid hyperactivity, perhaps on the basis of a favorable genetic background. Furthermore, the history of our patient demonstrates that vascular calcifcation heralds major cardiovascular diseases.

Sonographic appearance of aggressive angiomyxoma of the bladder.

Aggressive angiomyxoma is a rare, benign but locally aggressive mesenchymal neoplasm. We report the sonographic findings in a case of histologically proven aggressive angiomyxoma of the bladder. Sonography revealed a solid polypoid mass 2 cm in diameter with sharply demarcated borders, a heterogeneous echotexture, and a slightly hyperechoic rim.

[Tridimensional ultrasonography. First clinical experience with dedicated devices and review of the literature]. / Ecotomografia tridimensionale. Prime esperienze cliniche con apparecchiatura dedicata e revisione della letteratura.

PURPOSE: We report our preliminary clinical experience with three-dimensional ultrasound (3D US) in abdominal and small parts imaging, comparing the yield of 3D versus 2D US and through a literature review. MATERIAL AND METHODS: We used a Tomtec Echo-Scan 3.1 connected to a Philips P 700 unit with a 3.5 MHz convex probe and to a Toshiba SSA-340 A (equipped with power Doppler) with a 3.5 MHz convex and a 7.5 MHz linear probes. The system consists of: a) a workstation (166 MHz Intel Pentium, 128 Mbytes RAM, 520 Mbytes hard disk, 1.3 Gbyte Magneto-Optical drive); b) a spatial location system (3D Freehand Scanning) whose sensor, attached to the probe, provides spatial coordinates for each US scan in an electromagnetic field created by a transmitter; the software can thus correctly stack 2D US images to make 3D reconstructions of anatomical structures. The technical steps are: 1) setting; 2) image acquisition; 3) image processing and 3D rendering using surface or volume modes; 4) image archiving. 2D US was performed on 50 subjects, namely 20 volunteers and 30 patients with different pathologic conditions and 3D reconstructions were obtained from the best US images. We evaluated which anatomical structures and pathologic conditions are best suited for 3D rendering. RESULTS: The best 3D images were obtained from anatomical structures and pathologic conditions with a liquid content (i.e., bladder and gallbladder; cysts), or those adjacent to them (i.e., uterus and prostate). Major limitations were encountered in the assessment of the parenchyma of liver, kidneys, pancreas, thyroid, testis and breast, due to intrinsic texture low contrast, while intraparenchymal liquid structures (i.e., vessels, urinary cavities) and structures surrounded by liquid (i.e., hydrocele, ascites) were better demonstrated. DISCUSSION: The system permits accurate spatial location, and therefore stacking, of each US scan; this provides good-quality 3D images with fewer artifacts. The system can be connected to any existing US unit and to many kinds of probes. Incorrect processing or rendering may worsen 3D image quality and thus anatomical reconstructions; other drawbacks may come from difficult stacking of reconstructed images or limited field of view. Our personal experience and the review of 3D US literature indicate that the system may be used for the following clinical applications: anatomical assessment of lesions for minimally invasive treatment; targeting areas of interest and adjacent structures during radiotherapy; lesion volume studies during therapy; 3D vascular mapping with power Doppler; 3D reconstructions by intraluminal approach; real-time 3D scanning for US guidance during minimally invasive procedures. CONCLUSIONS: Our preliminary experience suggests that technological progress will soon lead to a widespread use of 3D US and its applications.

[Vascular calcification after kidney transplantation. Preliminary results]. / Le calcificazioni vascolari dopo trapianto di rene. Risultati preliminari.

BACKGROUND: Vascular calcifications (VC) are a common feature in chronic dialysis patients, but their clinical significance is debated, and the role of kidney transplantation (TRP) in the natural history of their development has received only scanty attention. METHODS: In our study we reviewed skeletal surveys as well as clinical and biochemical records of 13 patients who started again chronic dialysis at our Centre after failure of their kidney grafts. Changes of VC (during TRP) were scored as: 1 = no substantial progression (4 patients), 2 = moderate worsening (4 patients), 3 = severe worsening (5 patients = 38.4%). RESULTS: The most interesting association with the clinical/biochemical parameters seems to be between the score 3 subgroup and highest Ca*P values and vitamin D therapy. Four out of five score 3 patients experienced overt vascular events and 4 out of 5 of the same subgroup experienced parathyroidectomy (PTX) before, during or after the TRP. CONCLUSIONS: In this preliminary study we can conclude that a) the possibility of dramatic worsening of VC during TRP is not a rare event and this feature has a strong clinical implication, b) PTX before TRP could remove at least one of the putative risk factors in patients waiting for TRP with suboptimal control with medical therapy.

Morphological comparison of mitral and aortic bioprostheses explanted from the same patient for primary tissue failure.

Host factors might influence the fate of tissue prostheses. We have analyzed the tissue failure phenomenon in mitral and aortic porcine bioprostheses explanted from the same patient so as to avoid the bias of host factors. The 50 bioprostheses of this study were explanted from 25 patients reoperated on because of malfunctioning due to primary tissue failure. Bioprostheses explanted were photographed, radiographed and observed in polarizing light. All lesions analyzed received a score on the basis of morphological criteria. No difference was observed in the occurrence of pannus, tears or cells infiltrates. Calcifications occurred more extensively in mitral position (p=0.0031). Creep of the stent was significantly greater in mitral position (p=0.0324). Since the model of our study removed other individual factors, we may conclude that there is evidence for an earlier and more extensive calcific degeneration in the mitral than in the aortic position.

Embolization of a complete prosthetic aortic valve to the abdominal aorta. Long-term survival after surgery.

We describe a case of complete embolization of an aortic valve prosthesis to the abdominal aorta. To our knowledge, this patient is the 1st asymptomatic long-term survivor of this catastrophic event. In such cases, we recommend emergency surgery on the basis of echocardiographic results alone. Delay for more complete diagnostic evaluation is not warranted.

[Systemic mastocytosis: a case with diffuse bone involvement]. / Mastocitosi sistemica: un caso con diffuso coinvolgimento osseo.

The authors report the case of a 54-year-old woman with systemic mastocytosis with cutaneous, gastroenteric and skeletal involvement. The patient, who had presented wine-coloured skin lesions for years diagnosed as urticaria pigmentosa on the basis of the skin biopsy, was admitted to the Institute of Internal Medicine owing to the radiological findings of severe osteopenia. Instrumental and laboratory tests led to the correct diagnosis of type 1 systemic mastocytosis. The peculiarity of the case in question prompts the authors to recall the classification of mastocytosis, namely those pathological syndromes characterised by tissular infiltration by mast-cells, and to outline elements regarding its differential diagnosis in relation to disorders such as various forms of carcinoids and osteopenia, and in particular osteoporosis and osteolysis secondary to metastatic processes.

[The reliability of echotomographic diagnosis in assessing thyroid lesions. A comparison with cytology and histology]. / Attendibilità della diagnosi ecotomografica nella valutazione delle lesioni della tiroide. Confronto con la citologia e l'istologia.

The authors report their personal experience in 160 patients affected with thyroid conditions examined with US and fine needle aspiration (FNA). Benign nodular hyperplasia, cystic-colloid nodular hyperplasia, colloid cyst, hemorrhagic cyst, adenoma, thyroiditis, follicular and malignant lesions were studied. The latter consisted of 5 papillary carcinomas, 6 follicular carcinomas, 1 medullary carcinoma, 1 Hürthle cell carcinoma, 1 lymphoma and 1 metastatic lesion. The following US patterns were observed: 69.2% of the malignant lesions were hypoechoic, 15.4% were hyperechoic and 7.7% isoechoic. Most of the malignant lesions (84.6%) had inhomogeneous echo texture; 46.1% of them exhibited peripheral microcalcifications. US diagnoses were compared with cytologic and/or histologic findings to assess US accuracy. In the surgical patients US accuracy reached 78.6% in benign lesions and 61.5% in malignant lesions. Since US alone fails to yield enough pieces of information for an accurate diagnosis to be made, especially when malignant lesions are concerned, FNA should be used, while trying to define the actual role of color-Doppler US.

[Magnetic resonance in the study of patients of short stature of the hypothalamo-hypophyseal origin. Report on 29 cases]. / La risonanza magnetica nello studio dei pazienti con bassa statura di origine ipotalamo-ipofisaria. Rapporto su 29 casi.

Although growth hormone (GH) deficiency is a very common cause of short stature, many cases are still diagnosed as idiopathic. Magnetic Resonance Imaging (MRI), more clearly than CT, reveals the anatomy of the hypothalamic-hypophyseal region and of the possible alterations (pituitary hypoplasia, interruption of the stalk) causing hormonal deficit. Twenty-nine patients with short stature underwent MRI examinations of the hypothalamic-pituitary region to assess the significance of the correlation between hormonal test and MR patterns. Five patients had normal variants of short stature (NVSS), 7 had multiple pituitary hormone defects (MPHD) and 17 had isolated growth hormone deficiency (IGHD). In patients with MPHD or with severe isolated growth hormone deficit MRI shows interruption of the pituitary stalk with ectopy of the neurohypophysis or a mass. In patients with less severe IGHD and in NVSS, MRI demonstrates a normal pituitary region or a slightly hypoplastic gland, the neurohypophysis being normally situated. MRI may provide an ethiological classification in short stature patients. Typical MR patterns can be demonstrated in cases of dwarfism secondary to a mass in the hypothalamic-pituitary region or to morphological changes of the pituitary stalk, while in transient GH deficit no anatomical abnormalities are observed.

[Suspected spontaneous rupture of the ureter. Report of a clinical case]. / Sospetta rottura spontanea dell'uretere. Presentazione di un caso clinico.

A 57 year old man was admitted with left abdominal pain; radiological investigation showed a rupture of the left ureter. The authors discuss the diagnosis and the pathogenesis of ureteral rupture.

[Interventional radiology in the treatment of the complications from interventions on the lower urinary excretory tract]. / La radiologia interventistica nel trattamento delle complicanze degli interventi sulle basse vie escretrici urinarie.

Ureteral diversions may be complicated by strictures, hydronephrosis, pyelonephritis, lithiasis, fistulas, etc. In the last 10 years, 103 patients with 133 urological postoperative complications underwent percutaneous treatment. Most of the patients had been treated by percutaneous antegrade drainage; afterwards, stricture dilatation, ureteral stenting and stone extraction were performed. In one case a Strecker metallic stent was employed. We have successfully treated 101 of 133 complications (75.9%), with only 27 failures (20.3%). Five cases (3.8%) of lithiasis did not require percutaneous treatment. The good results obtained, the absence of major complications, the low cost and the little discomfort for the patients confirm the leading role of percutaneous treatment in complicated ureteral diversions.