Perforation of the atretic pulmonary valve. Long-term follow-up

OBJECTIVES: We evaluated the long-term results of perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum (PA-IVS). BACKGROUND: Interventional perforation of the pulmonary valve is considered the elective first stage treatment for PA-IVS, particularly in patients with a tripartite right ventricle (RV) and normal coronary circulation. However, the long-term results of this procedure are lacking. METHODS: Between January 1991 and December 2001, 39 newborns with a favorable form of PA-IVS underwent attempted perforation of the pulmonary valve. We evaluated the early and long-term outcomes. RESULTS: Median tricuspid and pulmonary z values were -1.2 and -2.4, respectively. Perforation was successful in 33 patients. Among them, 17 needed neonatal surgery, 13 did not need any surgery, and 3 had elective surgery after the first month of life. There were two procedure-related deaths, seven nonfatal procedural complications, and four postsurgical deaths. Compared with patients needing neonatal surgery, those having no or elective surgery had a higher incidence of a tripartite RV and a higher median tricuspid z value (92% vs. 53%, p = 0.04 and -1.7 vs. -0.5, p = 0.03). At a median follow-up of 5.5 years (range 0.5 to 11.5), survival was 85% and freedom from surgery was 35%. Five patients, four of whom had neonatal surgery, underwent a partial cavo-pulmonary connection. CONCLUSIONS: Our results show that this technique, although burdened by non-negligible mortality and morbidity, is effective in selected patients with a normal-sized RV. Preselection of patients allows interventional or surgical biventricular correction in the majority of cases.     

Vertebral hyperostosis and diabetes mellitus: a case-control study.

OBJECTIVE--To compare glucose metabolism in patients with vertebral hyperostosis (VH), with that in control patients. METHODS--We studied 50 patients aged 60 years or more who had VH according to Resnick's criteria, and 50 control patients without VH, matched for sex, age, weight and height. Plasma glucose was evaluated before and 120 minutes after ingestion of 75 g glucose. World Health Organisation criteria for diabetes mellitus (DM) were used. Radiographs of the pelvis and thoracic and lumbar spine were performed and read blind by two physicians. RESULTS--Statistical analysis showed no difference between cases and control patients for prevalence of DM, and plasma glucose at 0 and 120 minutes. CONCLUSION--These data suggest that glucoregulation in patients with VH does not differ from that in matched controls.     

慢性乙型肝炎发病机制的超微形态学基础研究

为探讨慢性乙型肝炎（乙肝）肝细胞损伤机制的超微形态学基础，对５０例乙肝患者肝穿刺进行电观察，发现淋巴细胞浸入肝组织后，可以与肝细胞发生接触、质膜融合和胞质沟通。肝细胞和淋巴细胞之间可见电子致密物，这可能是淋巴细胞产生的多种因子。枯否细胞质膜与淋巴细胞质膜融合，浆细胞质膜可与淋巴细胞质膜融合，淋巴细胞之间胞质可互相沟通，提示了免疫细胞间以及免疫细胞与肝细胞间的一些关系，为研究乙肝的细胞免疫反应机制提     

Clinical outcome following coronary angioplasty in dialysis patients: a case-control study in the era of coronary stenting

BACKGROUND—Balloon coronary angioplasty has been reported to be ineffective in patients treated for end stage renal disease because of a high restenosis rate.
OBJECTIVE—To compare the clinical outcome following coronary angioplasty with provisional stenting in dialysis versus non-dialysis patients.
DESIGN—A case-control study.
PATIENTS—Of 1428 consecutive patients who underwent coronary angioplasty, 100 (7%) were being treated for end stage renal disease. These were compared with 100 control patients matched for age, sex, coronary lesions, presence of diabetes mellitus, and rate of coronary stenting (40%).
MAIN OUTCOME MEASURES—In-hospital and one year clinical outcome.
RESULTS—The rates of procedural success (90% v 93%), in-hospital mortality (1% v 0%), stent thrombosis (0% v 0%), and Q wave myocardial infarction (0% v 1%) were similar in dialysis and non-dialysis patients. One year clinical outcome after coronary angioplasty was similar in the two groups in terms of clinical restenosis (31% v 28%) and myocardial infarction (6% v 2%), but cardiac death was more common in dialysed patients (11% v 2%, p < 0.03).
CONCLUSIONS—Dialysis does not increase the risk of clinical restenosis after coronary angioplasty with provisional stenting. Coronary angioplasty is a safe and effective therapeutic procedure in selected dialysis patients with culprit lesions accessible to stenting. However, the one year survival is reduced in this high risk population.


Keywords: renal disease; angioplasty; stents; restenosis     

Severe cardiac involvement in children with systemic sclerosis and myositis

OBJECTIVE: To assess the outcome of children with systemic sclerosis (SSc) and features of polymyositis. METHODS: The charts of 4 children who met the American College of Rheumatology criteria for SSc and had features of polymyositis, as defined by the presence of proximal muscle weakness and elevated serum creatine phosphokinase or aldolase level, were retrospectively reviewed. RESULTS: All children had multivisceral involvement including (1) myocardial perfusion defects in all cases, with mild to severe dilated cardiomyopathy in 3; (2) lung restrictive syndrome in 3; (3) mild to severe esophageal involvement in all cases; and (4) severe intestinal dysfunction in one child. Combination therapy of corticosteroids, methotrexate (MTX), and cyclosporine resulted in improved skin thickness and muscle strength scores in all cases, as well as in lung restrictive syndrome in 2, but was not effective regarding the progression of intestinal malabsorption in one patient, esophageal dysmotility in 3 patients, and dilated cardiomyopathy in 3. Endstage cardiac failure caused 2 deaths. In one child, heart transplantation was performed for the first time in this indication. CONCLUSION: Children with diffuse cutaneous SSc and features of polymyositis are prone to develop severe cardiomyopathy. Combination therapy of corticosteroids, MTX, and cyclosporine seems to be active on muscle, skin, and lung involvement but does not impair progression of esophageal or myocardial dysfunction. Heart transplantation might be considered, as an experimental treatment, in young patients with severe cardiomyopathy and no other irreversible organ damage.     

Primary cardiac angiosarcoma. A therapeutical dilemma

Cardiac angiosarcomas are malignant tumors that almost invariably have a short and fatal evolution. The therapeutic approach includes surgery, chemotherapy, and radiation therapy, alone or in combination. Heart transplantation is an attractive option in nonresectable tumors, even though the current experience is still limited. However, in most patients, the diagnosis is still established late, and survival is only slightly altered by the proposed treatments, mainly due to previously existing and undetected metastases. We report a case that illustrates the therapeutic dilemma faced with this neoplasia, and we discuss the case based on a literature review.