From January 1968 to July 1983, 38 patients with an intracardiac myxoma underwent surgical excision of the tumor at our Institution. There were 15 males and 23 females, ranging in age from 17 to 68 years (mean 47.7). In all but 2 patients, the presence of an intracardiac myxoma was documented preoperatively by angiographic and/or echocardiographic study. The first 2 patients of this series were referred with the diagnosis of mitral stenosis, and the myxoma was an unexpected surgical finding. In 32 cases (84.2%) the myxoma was located in the left atrium, in 4 (10.5%) in the right atrium and in 2 (5.3%) in the right ventricle. Surgery was performed on an emergency basis in 36 patients. Two patients (5.2%) both with a left atrial myxoma, died after surgery: one died intraoperatively of hemorrhage and the other after one month of bowel infarction. Follow-up of the 36 survivors ranges from 3 months to 14.5 years (average 5.2 yr); all were controlled by means of clinical and echocardiographic investigations, and no evidence of tumor recurrence was detected. Surgery for intracardiac myxoma, which should be undertaken on an emergency basis, carries a low operative risk; excision of the tumor appears to be curative with no recurrences at long-term follow-up; non-invasive tools are of the utmost importance in both the preoperative assessment and follow-up of these patients.

Resistance to enzyme replacement therapy (ERT) is a major therapeutic challenge in Fabry disease (FD). Recent reports attribute to immune-mediated inflammation a main role in promoting disease progression and resistance to ERT. Aim of the study is to report a Gb3-induced auto-reactive panmyocarditis causing inefficacy of ERT and severe electrical instability, which required cardiac transplantation. Examining the explanted heart from a 57-year-old man with FD cardiomyopathy (CM) on 3-year ERT presenting incoming ventricular fibrillation, we documented a severe virus-negative myocarditis extended to cardiomyocytes, intramural coronary vessels, conduction tissue, and subepicardial ganglia. Serology was positive for anti-Gb3, anti-heart, and anti-myosin antibodies. In vitro Gb3 stimulation of patient's peripheral blood mononuclear cells (PBMC) induced high amount production of inflammatory cytokine IL1-β, IL-6, IL-8, and TNF-α. PBMC were stained using the monoclonal antibodies CD3-V500, CD4-V450, CD8-APCcy7, CD45RO-PerCPcy5.5 and CD27-FITC from BD Biosciences and CD56-PC7 from Bekman Coulter. The phenotypic analysis of PBMC showed a lower frequency of CD8 (9.2%) vs. 19.3% and NKT cells (1.6% vs. 2.4%) in Fabry patient respect to healthy donor, suggesting a possible homing to peripheral tissues. A Gb3-induced auto-reactive myocarditis is suggested as a possible cause of FDCM progression and ERT resistance. Immune-mediated inflammation of systemic Fabry cells may coexist and be controlled by implemental immunosuppressive therapy.A. Frustaci et al. Figure 1 Histological and ultrastructural changes of an explanted Fabry heart requiring transplantation because of an incoming ventricular fibrillation. (A) Macroscopic examination of explanted heart showing by sequential cuts severe biventricular hypertrophy. (B) Extensive myocarditis associated to remarkably hypertrophied and vacuolated cardiomyocytes affected by FDCM (H&E 200×). (C) Inflammatory cells being mainly represented by C-activated T lymphocytes (immunohistochemistry with CD45Ro, 200×). (D) Ultrastructural examination showing cardiomyocyte vacuoles consisting of myelin bodies massively secreted (arrows) in the interstitium. (E) Vasculitis of an intramural coronary artery (vessel diameter 180 μm) which wall is infiltrated by glycosphingolipid vacuoles (200×). (F) It shows a section of the vacuolized conduction tissue (ct) infiltrated and damaged by CD45Ro + T lymphocytes (immunohistochemistry 200×). (G) Subepicardial neuron ganglion extensively infiltrated and damaged by T lymphocytes.Fabry cardiomyopathy: Gb3-induced auto-reactive panmyocarditis requiring heart transplantation

The present report reviews our experience with 12 cases of delayed cardiac tamponade following open heart surgery, which occurred at various times after the seventh postoperative day (average 19 days). In each case the diagnosis was made on clinical grounds, supported by the radiographic findings in all, and confirmed by echocardiography in 4. Pericardial effusion was serous in 5 patients, sero-sanguineous in 3, sanguineous in 3, and purulent in one; it was most frequently caused by a postpericardiotomy syndrome (7 cases). A single pericardiocentesis was curative in 6 instances, while repeat procedures were required in 2 because of recurrence. A repeat median sternotomy was performed in 5 patients associated with pericardiocentesis, and a pericardiectomy in 2. Three patients died within one month from the initial operation; no recurrences have been noted so far in the long-term survivors. According to the results of the present investigation, early clinical recognition is considered the clue to a successful outcome. Sole pericardiocentesis is followed by immediate improvement of the patient's state, and is often curative, but recurrences may require other maneuvers for further fluid evacuation. Awareness of this possible complication may contribute to a decrease in the still high mortality rate associated with this condition.

No abstract

Between January 1970 and December 1981, a total of 21 reoperations for periprosthetic leak were performed on 20 patients out of 999 with previously implanted prosthetic mitral valves. In most of them reoperations were performed within the first year, since the initial procedure and the leading indications were intractable congestive heart failure or infection of the mitral prosthesis. The mortality rate was 30% and was related to the preoperative cardiac functional status. The preoperative variables significantly related to an increased incidence of dehiscence of the mitral prosthesis necessitating reoperation were a degenerative disease (P = 0.016) or an infective endocarditis (P = 0.0006) of the native valve, both causing mitral regurgitation. Rheumatic disease, type of prosthesis, supra- or subannular insertion, age of the patient, and operative year, were not significant, neither were calcifications that are probably neutralized by the routine use of special surgical techniques. It is suggested that the use of techniques specifically designed to eliminate periprosthetic leak in patients affected by mitral regurgitation due to degenerative or infective disease of the native valve, might lead to a further reduction of reoperations for this complication.