Heart Transplantation in Arrhythmogenic Right Ventricular Dysplasia: Case Reports

Objective

Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disease of familiar, origin where the myocardium is replaced by fibrofatty tissue predominantly in the right ventricle. Herein we have presented the clinical courses of 4 patients with ARVD who underwent orthotopic heart transplantation.

Patients and Methods

Among 358 adult patients undergoing heart transplantation, 4 (1.1%) displayed ARVD. The main indication for transplantation was the progression to heart failure associated with arrhythmias. All 4 patients displayed rapid, severe courses leading to heart failure with left ventricular involvement and uncontrolled arrhythmias.

Results

In all cases the transplantation was performed using a bicaval technique with prophylactic tricuspid valve annuloplasty. One patient developed hyperacute rejection and infection, leading to death on the 7th day after surgery. The other 3 cases showed a good evolution with clinical remission of the symptoms. Pathological study of the explanted hearts confirmed the presence of the disease.

Conclusions

ARVD is a serious cardiomyopathy that can develop malignant arrhythmias, severe ventricular dysfunction with right ventricular predominance, and sudden cardiac death. Orthotopic heart transplantation must always be considered in advanced cases of ARVD with malignant arrhythmias or refractory congestive heart failure with or without uncontrolled arrhythmias, because it is the only way to remit the symptoms and the disease.     

Vascular Immunohistochemical Markers: Contributions to Hepatocellular Nodule Diagnosis in Explanted Livers

Introduction

The process of hepatic carcinogenesis involves a progression including large regenerative nodules, to dysplastic nodules, and finally to hepatocellular carcinoma. Angiogenesis is fundamental to the development of malignant tumors. Changes in sinusoidal capillarization and isolated arteries occur early in hepatic carcinogenesis. However, sometimes differentiation of hepatocellular nodules can be difficult for the general pathologist. The aim of this study was to evaluate angiogenesis by immunohistochemistry using CD34 and HHF35 antibodies for differential diagnosis of large regenerative nodules versus dysplastic nodules versus hepatocellular carcinoma using explanted cirrhotic livers.

Methods

Seventy-nine nodules obtained from 29 explanted cirrhotic livers were classified according to the International Working Party as follows: 17 large regenerative, 23 low-grade dysplastic, 23 high-grade dysplastic, and 16 hepatocellular carcinomas. These nodules were submitted to immunohistochemistry with antibodies to CD34 and HHF35 to analyze sinusoidal capillarization and arterialization, respectively.

Results

Semiquantitative analysis revealed that CD34 expression was >30% in dysplastic nodules and hepatocellular carcinoma; the staining in 93.8% of cases was diffuse, almost involving the entire sinusoidal lining in hepatocellular carcinoma. The number of isolated arteries was high in hepatocellular carcinoma (average, 4.369), which positively correlated with the other nodules (P < .005).

Conclusion

Quantification of sinusoidal capillarization and isolated arteries in hepatocellular nodules, as detected with CD34 and HHF35 antibodies, respectively provided an important tool to differentiate dysplastic nodules from hepatocellular carcinoma.     

Subtle lung cancers: impact of edge enhancement and gray scale reversal on detection with digitized chest radiographs

The authors studied the impact of edge enhancement and gray scale polarity reversal on the detection of subtle lung cancers. Three experienced readers reviewed 46 biopsy-proved subtle lung cancers and 46 normal controls on chest radiographs that had been digitized into a 1,024 X 1,536-pixel matrix 8 bits deep. Receiver-operating characteristics (ROC) analysis of 1,656 pooled observations indicated that performance was best with the unmodified images (ROC area = 0.83), degraded by moderate enhancement of medium frequencies (ROC area = 0.80), and markedly impaired by severe enhancement of low frequencies (ROC area = 0.69). Gray scale polarity reversal further degraded performance (unenhanced ROC area = 0.74; moderately enhanced ROC area = 0.76; severely enhanced ROC area = 0.76). The authors conclude that edge enhancement and gray scale polarity reversal can impair the detectability of subtle lung cancers on digitized radiographs of medium resolution.     

A left knee wound complication by non-Hodgkins lymphoma in bilateral total knee arthroplasties

A 70-year-old woman with a history of bilateral primary knee osteoarthritis presented with a left knee wound complication, a non-Hodgkins lymphoma, after bilateral total knee arthroplasties. After exploring several etiologies, the evidence in this unusual case suggests a coincidental preexisting lymphoma.

Insulin resistance is associated with circulating fibrinogen levels in nondiabetic patients receiving peritoneal dialysis.

BACKGROUND: Insulin resistance (IR) and inflammation are associated with increased risk of cardiovascular disease in the general population. Continuous glucose absorption in peritoneal dialysis (PD) may induce hyperglycemia and hyperinsulinemia. METHODS: We evaluated IR in nondiabetic patients receiving PD, and analyzed the association between IR and systemic inflammation biomarkers by performing a cross-sectional study on ambulatory dialysis. A total of 25 nondiabetic patients receiving PD and 25 healthy individuals, matched for gender, age, and body mass index (BMI), were included. The PD group was composed of 11 men and 14 women, with a mean age of 47 +/- 14 years and mean BMI of 25.5 +/- 4.7 kg/m(2). The control group was composed of 10 men and 15 women, with a mean age of 45 +/- 12 years and BMI of 24.0 +/- 2.8 kg/m(2). RESULTS: IR was evaluated by the homeostasis model assessment method (HOMA-IR). Inflammation was assessed through high-sensitivity C-reactive protein (CRP) and fibrinogen. Body composition and truncal fat were evaluated by dual energy x-ray absorptiometry. HOMA-IR was significantly higher (P < .0001) in subjects receiving PD (4.9, range: 2.3-9.3 mmol/L x muU/mL) compared with healthy subjects (1.2, range: 0.4-4.8 mmol/L x muU/mL). As expected, compared with controls, patients receiving PD had significantly higher levels of insulin (26.5 +/- 7.5 muU/mL vs 6.3 +/- 3.4 muU/mL; P < .0001), CRP (6.3, range: 0.3-61.1 mg/L vs 2.4, range: 0.6-5.9 mg/L; P = .001), and fibrinogen (379 +/- 101 mg/dL vs 268 +/- 66 mg/dL; P < .0001). However, there were no significant differences in body and truncal fat mass between the groups. A significant correlation between HOMA-IR and fibrinogen (Rho = 0.48; P = .01) was observed. However, no correlation was found between HOMA-IR and CRP. Also, no significant correlations were found between HOMA-IR and body fat mass (Rho = 0.11), and between HOMA-IR and truncal fat mass (Rho = 0.19). CONCLUSIONS: Patients receiving PD demonstrate a state of IR that is associated with high circulating levels of fibrinogen. This suggests that hyperfibrinogenemia may be involved in the pathogenesis of IR in this setting.     

Bacterial colonization in hemodialysis temporary dual lumen catheters: a prospective study

AIMS: The use of hemodialysis temporary dual-lumen catheters is often complicated by infections, which may be a significant cause of death among patients with end stage renal disease (ESRD). The aim of this study was to assess the incidence of bacteremia and bacterial colonization related to non-tunneled, non-cuffed, dual-lumen temporary catheters in patients with ESRD submitted to hemodialysis. METHODS: This study included 29 patients with ESRD. After catheter implantation, patients were monitored throughout the period of catheter permanence by means of blood samples collected weekly from a peripheral vein. Bacteria were isolated and identified according to CLSI recommendations. When catheters were removed for any reason, their tips were evaluated microbiologically. RESULTS: A total of 194 blood samples from the 29 patients implanted with 55 catheters were analyzed. Of these, 15.5% (30 samples) demonstrated bacterial growth, principally Staphylococcus epidermidis (64.5%). Twenty patients (68.9%) presented at least one positive blood culture during follow-up. The median time for catheter colonization was 18.5 days (95% CI: 16.8-30.3). Of the 55 catheters implanted, 28 (50.9%) showed bacterial colonization, corresponding to 23.4 episodes/1000 catheter/days and 9.2 episodes of bacteremia /1000 catheter/days. Fifteen of 28 catheter tips analyzed showed bacterial growth (53.5%). In 14 of these (93.3%), there was agreement between the isolates from the catheter tip and blood cultures. Of 24 episodes of positive blood cultures from 20 different patients in 17 episodes (70.8%), the patients showed no clinical signs or symptoms of bacteremia. CONCLUSIONS: The high incidence of catheter colonization, the correlation between blood and catheter tip cultures, and the occurrence of frequent cases of asymptomatic bacteremia justify the proposal of routine peripheral blood collections to monitor patients undergoing hemodialysis with temporary dual-lumen catheters.     

Is radiotherapy important for low-grade soft tissue sarcoma of the extremity?

Radiotherapy and limb-preserving surgery has replaced amputation and compartmental resection for treatment of soft tissue sarcomas. However, the role of radiotherapy in low-grade tumors remains unclear. This study reviews the outcomes of 132 patients who received multimodality treatment for low-grade soft tissue sarcoma. Large primary tumors (> 5 cm) and the absence of radiotherapy correlated with local recurrence. Radiotherapy was most effective in patients operated on with marginal margins. Patients who were treated with wide surgical margins or had small tumors (< or =5 cm) showed no benefit with adjuvant radiotherapy. Size greater than 5 cm and local recurrence correlated with metastasis. Radiotherapy appears to be important in the management of low-grade soft tissue sarcoma. The principles of local treatment for low-grade soft tissue sarcoma should be the same as for high-grade tumors with a combination of surgery and adjuvant radiotherapy. In a subset of patients with small and widely excised tumors, consideration may be given to withholding radiotherapy. Local recurrence and metastasis from low-grade soft tissue sarcoma may occur as long as 1 decade after primary tumor resection. Long-term review of patients with low-grade tumors may be indicated.     

Myofibroblastoma and solitary fibrous tumour of the breast: histopathologic and immunohistochemical studies

Thirteen mesenchymal tumours of the breast were reviewed histologically and immunohistochemically. Nine tumours (male:female ratio 5:4, average age 64 years) were classified as myofibroblastomas and presented as a single nodule (four) or a multilobular mass (five). They were composed of elongated cells with vesicular nuclei showing grooves, intranuclear inclusion, and small but conspicuous nucleoli resembling myofibroblasts seen in scar tissue. In six tumours, hypercellular zones alternated with paucicellular, hyalinized areas. The collagen pattern was dense, diffuse, and brightly eosinophilic in all neoplasms. The tumour cells were positive for desmin, actin, and vimentin in all nine lesions, and in six tumours a focal CD 34 positivity was also demonstrated. Four tumours (four female patients, average age 75 years) were classified as solitary fibrous tumours and consisted of well-circumscribed nodules. They were characterized by a monomorphic population of thin, spindled to ovoid cells with finely dispersed chromatin and inconspicuous nucleoli. The pattern of the collagen in these tumours was one of thick, brightly eosinophilic bands. These four tumours were negative for all markers tested except vimentin and CD 34. Although myofibroblastomas and solitary fibrous tumours of the breast share many morphologic features, there are enough differences in their cytologic composition and, most importantly, in their immunohistochemical profile to consider them as distinct entities.