Multimodality Imaging for Diagnosis and Characterization of a Cardiac Hydatid Cyst.

Here, we report the case of a young patient admitted to the emergency department because of abdominal pain. Computed tomography revealed a mass within her right heart. Through serial multimodality imaging testing, including computed tomography, three-dimensional (2D)- and three-dimensional echocardiography, as well as cardiac magnetic resonance, the diagnosis of cardiac involvement in the course of Echinococcus granulosus infection was hypothesized.

Early systemic sclerosis: analysis of the disease course in patients with marker autoantibody and/or capillaroscopic positivity.

OBJECTIVE: To investigate whether patients affected by 1 of the 3 subsets of early systemic sclerosis (SSc; scleroderma), i.e., subset I, Raynaud's phenomenon with SSc marker autoantibodies and typical capillaroscopic findings; subset II, autoantibody positive only; and subset III, capillaroscopy positive only and not satisfying the 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for SSc at admission, differ from each other in the time to satisfy the criteria. METHODS: Early SSc patients subdivided into the 3 subsets indicated above consecutively admitted to a rheumatology/angiology center were monitored for 12-102 months (median 36 months). Patients were reevaluated twice yearly to assess whether and when each patient satisfied the new ACR/EULAR classification criteria for SSc. Patients with undifferentiated connective tissue disease (UCTD) served as the comparator group. RESULTS: During followup, 11 (52.3%) of 21 subset I, 10 (66.6%) of 15 subset II, 0 of 24 subset III, and 0 of 44 UCTD patients satisfied the criteria (P = 0.0001). The difference was significant between early SSc and UCTD patients (P = 0.0001) and, within the group of early SSc patients, between each of the 2 autoantibody-positive subsets (subsets I and II) and the capillaroscopic-positive/autoantibody-negative subset (subset I versus III: P = 0.0001; subset II versus III: P = 0.0009). There was no difference between the 2 autoantibody-positive subsets (P = 0.454). In addition to marker autoantibody positivity, preclinical lung or heart involvement was associated with an increased risk to satisfy the criteria during followup. CONCLUSION: Our data demonstrated faster progression of SSc in autoantibody-positive patients, particularly in those with preclinical internal organ involvement at baseline, than in autoantibody-negative patients.

Early systemic sclerosis: marker autoantibodies and videocapillaroscopy patterns are each associated with distinct clinical, functional and cellular activation markers.

INTRODUCTION: Early systemic sclerosis (SSc) is characterized by Raynaud's phenomenon together with scleroderma marker autoantibodies and/or a scleroderma pattern at capillaroscopy and no other distinctive feature of SSc. Patients presenting with marker autoantibodies plus a capillaroscopic scleroderma pattern seem to evolve into definite SSc more frequently than patients with either feature. Whether early SSc patients with only marker autoantibodies or capillaroscopic positivity differ in any aspect at presentation is unclear. METHODS: Seventy-one consecutive early SSc patients were investigated for preclinical cardiopulmonary alterations. Out of these, 44 patients and 25 controls affected by osteoarthritis or primary fibromyalgia syndrome were also investigated for serum markers of fibroblast (carboxyterminal propeptide of collagen I), endothelial (soluble E-selectin) and T-cell (soluble IL-2 receptor alpha) activation. RESULTS: Thirty-two of the 71 patients (45.1%) had both a marker autoantibody and a capillaroscopic scleroderma pattern (subset 1), 16 patients (22.5%) had only a marker autoantibody (subset 2), and 23 patients (32.4%) had only a capillaroscopic scleroderma pattern (subset 3). Patients with marker autoantibodies (n = 48, 67.6%) had a higher prevalence of impaired diffusing lung capacity for carbon monoxide (P = 0.0217) and increased serum levels of carboxyterminal propeptide of collagen I (P = 0.0037), regardless of capillaroscopic alterations. Patients with a capillaroscopic scleroderma pattern (n = 55, 77.5%) had a higher prevalence of puffy fingers (P = 0.0001) and increased serum levels of soluble E-selectin (P = 0.0003) regardless of marker autoantibodies. CONCLUSION: These results suggest that the autoantibody and microvascular patterns in early SSc may each be related to different clinical-preclinical features and circulating activation markers at presentation. Longitudinal studies are warranted to investigate whether these subsets undergo a different disease course over time.

[An atypical case of tako-tsubo syndrome presenting with symptomatic bradycardia]. / Sindrome di tako-tsubo: descrizione di un caso atipico con bradicardia sintomatica all'esordio.

The tako-tsubo syndrome (transient left ventricular apical ballooning with normal coronary arteries), initially described in Japanese patients, is now being increasingly observed worldwide and should be considered in the differential diagnosis of acute coronary syndromes. Angina-like chest pain, electrocardiographic changes and an increase in myocardial markers are often present, as well as history of acute stressful events preceding symptom onset. We report the case of an Asiatic woman in whom typical, reversible abnormalities in left ventricular motion were associated with symptomatic junctional bradycardia. Nevertheless, the patient was completely free from angina and excluded acute pain or emotions in the previous weeks. Coronary angiography showed absence of significant disease and left ventricular function was found to be unremarkable 1 month after the acute event. Although infrequent, atypical presentations of tako-tsubo syndrome have occasionally been reported and, in our opinion, they could provide interesting insights into the ill-defined pathophysiology of the disease.

Analysis of valve motion after the reimplantation type of valve-sparing procedure (David I) with a new aortic root conduit.

BACKGROUND: The reimplantation type of valve-sparing procedure does not allow proper reconstruction of the sinuses of Valsalva. We assessed the valve motion after a reimplantation type (David I) of valve-sparing procedure using a new Dacron conduit that incorporates sinuses of Valsalva. METHODS: Nine consecutive patients undergoing an aortic valve-sparing procedure using the new conduit were studied using two-dimensional transesophageal echocardiography shortly (2 +/- 1 months) after operation to determine root distensibility, expressed as percent change in radius and as pressure strain of the elastic modulus. Next, monodimensional view was used to assess valve motion in its various phases (rapid valve opening velocity, slow closing leaflet displacement, rapid valve closing velocity, maximal leaflet displacement, and leaflet displacement before valve closure). Seven healthy individuals served as control subjects. RESULTS: Root distensibility was reduced at the level of the annulus and sinotubular junction but was similar to control subjects at the level of the sinuses (percent change in radius, 4.1% +/- 0.8% versus 4.5% +/- 1.2%; pressure strain of the elastic modulus, 1,286 +/- 674 g/cm2 versus 1,195 +/- 628 g/cm2). Rapid valve opening (69 +/- 34.4 cm/s versus 51 +/- 11.9 cm/s) and closing (47.6 +/- 16 cm/s versus 36.4 +/- 9 cm/s) velocity as well as slow closing leaflet displacement (24% +/- 4.7% versus 22.1% +/- 7.9%), maximal leaflet displacement (20.1 +/- 4 mm versus 22.7 +/- 1.9 mm), and leaflet displacement before valve closure (15.2 +/- 3 mm versus 17.6 +/- 0.8 mm) were similar to control subjects. CONCLUSIONS: The new aortic root conduit used in a reimplantation type of valve-sparing procedure allows the anatomic reconstruction of the aortic root with leaflet motion similar to that of normal subjects.

One-year appraisal of a new aortic root conduit with sinuses of Valsalva.

OBJECTIVES: We evaluate the clinical results 1 year after an anatomic reconstruction of the aortic root in which we used a specifically designed aortic root prosthesis that incorporates the sinuses of Valsalva. MATERIAL AND METHODS: The new aortic Dacron prosthesis has a proximal portion in the Dacron conduit that expands on implantation, creating pseudosinuses. During a 12-month period, 28 patients (mean age 59 +/- 14 years) underwent a Bentall operation (12 cases), a remodeling procedure (7 cases), and a reimplantation procedure (9 cases) with the use of a new aortic root conduit. All patients had aortic root aneurysm with an anatomically normal (for the valve-sparing procedure) or diseased aortic valve (for the Bentall operation). Five patients had aortic dissection and 5 had Marfan disease. The mean follow-up was 6 +/- 3 months. All patients underwent postoperative transesophageal and transthoracic echocardiographic studies. RESULTS: All patients survived and were in good clinical condition at the latest follow-up. Postoperative echocardiography showed a marked reduction in ventricular volumes in all patient groups (P <.0005). In the Bentall group the new prosthesis appeared to reduce the tension on the coronary ostial sutures. In patients undergoing both types of valve-sparing procedures a similar normal anatomy of the aortic root was reconstructed. In the reimplantation group the anulus was smaller than in the remodeling group (P =.01). Patients undergoing the reimplantation procedure had less bleeding and a lower incidence of residual valve insufficiency. CONCLUSIONS: The new aortic root prosthesis allowed the reconstruction of the aortic root anatomy in all types of surgical techniques with low postoperative morbidity.