[Caseous calcification of the mitral annulus: case report and review of the literature]. / Calcificazione caseosa dell'anulus mitralico: caso clinico e revisione della letteratura.

Caseous calcification of the mitral annulus is an uncommon variant of mitral annular calcification. It appears as a round echodense mass containing central areas of echolucencies resembling liquefaction and with no flow in the central zone on color Doppler. In most cases it involves the posterior mitral annulus region, particularly in female subjects. The pathogenesis remains unclear: hypercholesterolemia and the dissolution of lipid-rich macrophages may be implicated in liquefaction necrosis. Transthoracic and transesophageal echocardiography represents the most reliable technique for diagnosis, whereas cardiac magnetic resonance imaging is the choice in doubtful cases. We report the case of an 82-year-old female patient describing different aspects of this particular clinical condition.

Transaxillary versus transaortic approach for transcatheter aortic valve implantation with CoreValve Revalving System: insights from multicenter experience.

BACKGROUND: About one-third of candidates for transcatheter aortic valve implantation (TAVI) suffer from severe peripheral artery disease, making the routine femoral approach difficult or impossible. The aim was to compare the transaxillary (TAx) and the transaortic (TAo) route for TAVI with Medtronic CoreValve Revalving System (CRS). METHODS: Out of 1049 consecutive TAVI with 18F CRS, 242 (23%) were treated in 4 high-volume Italian Centre through TAx (61%) and TAo (39%). RESULTS: The devices success was similar (P=0.16) with a trend to a lower incidence of significant paravalvular leak (6% vs. 14%, P=0.07) and a significant reduction of permanent pacemaker (PPM) implantation (13% vs. 34%, P=0.017) in the TAo. However, this route showed a higher incidence of acute kidney injury (P=0.016) and a longer hospital stay after the index procedure (days 10 [8-14] vs. 8 [7-12], P=0.001). By a multivariate analysis the vascular access is an independent predictor for a longer hospital stay (TAo route; OR=0.37, 95% CI: 0.18-0.75; P=0.006) and for PPM implantation (TAx route; OR=3.7, 95% CI: 1.2-10.8; P=0.017). CONCLUSIONS: Although the higher clinical risk profile of transaortic population, the TAo approach showed an equally high device success with similar 30-day safety and 1year efficacy, compared to TAx route. However, due to non-procedure-specific complications post-TAo TAVI, this route requires a specialized postoperative care and the treatment of patients in highly specialized and experienced centers.

One-Year Outcomes of Transcatheter Aortic Valve Implantation Using the Direct Aortic Approach.

BACKGROUND: The direct aortic (DA) approach allows for transcatheter aortic valve implantation (TAVI) in patients with difficult peripheral vascular anatomy. The CoreValve ADVANCE Direct Aortic (ADVANCE DA) study was performed to assess the outcomes of DA TAVI with the CoreValve System (Medtronic, Minneapolis, MN) in routine practice. METHODS: Patients were selected for the DA approach by local cardiac surgical teams, and TAVI was performed with patients under general anesthesia. Safety events were adjudicated according to the Valve Academic Research Consortium-2 definitions by an independent clinical events committee. All imaging data, including that from multislice computed tomography and follow-up echocardiography, were analyzed by an independent core laboratory. RESULTS: From September 2012 to February 2014, 100 patients were enrolled (52.0% male, age 81.9 ± 5.9 years, The Society of Thoracic Surgeons Score 5.9 ± 3.2%) at 9 centers in Europe. Peripheral vascular disease was present in 51.0% of patients, and 38.0% had diabetes. Of the 100 patients enrolled, 92 underwent TAVI. At 30 days after TAVI, 98.1% were free of moderate or severe paravalvular leak. At 1 year, 16 patients had died (Kaplan-Meier rate 17.9%), 1 (1.1%) patient had had a stroke, classified as nondisabling, and 15 (17.0%) patients had received a permanent pacemaker. Most patients experienced improved quality of life as measured by the Kansas City Cardiomyopathy Questionnaire overall summary score (mean change from baseline to 1 year, 39.6 ± 26.3; p < 0.01). CONCLUSIONS: The DA approach provides a feasible alternative for patients with challenging anatomic features that may otherwise preclude use of the TAVI procedure.

Venoarterial Extracorporeal Membrane Oxygenation for Acute Fulminant Myocarditis in Adult Patients: A 5-Year Multi-Institutional Experience.

BACKGROUND: Acute fulminant myocarditis (AFM) may represent a life-threatening event, characterized by rapidly progressive cardiac compromise that ultimately leads to refractory cardiogenic shock or cardiac arrest. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) provides effective cardiocirculatory support in this circumstance, but few clinical series are available about early and long-term results. Data from a multicenter study group are reported which analyzed subjects affected by AFM and treated with VA-ECMO during a 5-year period. METHOD: From hospital databases, 57 patients with diagnoses of AFM treated with VA-ECMO in the past 5 years were found and analyzed. Mean age was 37.6 ± 11.8 years; 37 patients were women. At VA-ECMO implantation, cardiogenic shock was present in 38 patients, cardiac arrest in 12, and severe hemodynamic instability in 7. A peripheral approach was used with 47 patients, whereas 10 patients had a central implantation or other access. RESULTS: Mean VA-ECMO support was 9.9 ± 19 days (range, 2 to 24 days). Cardiac recovery with ECMO weaning was achieved in 43 patients (75.5%), major complications were observed in 40 patients (70.1%), and survival to hospital discharge occurred in 41 patients (71.9%). After hospital discharge (median follow-up, 15 months) there were 2 late deaths. The 5-year actual survival was 65.2% ± 7.9%, with recurrent self-recovering myocarditis observed in 2 patients (at 6 and 12 months from the first AFM event), and 1 heart transplantation. CONCLUSIONS: Cardiopulmonary support with VA-ECMO provides an invaluable tool in the treatment of AFM, although major complications may characterize the hospital course. Long-term outcome appears favorable with rare episodes of recurrent myocarditis or cardiac-related events.

Italian multicentre study on type A acute aortic dissection: a 33-year follow-up†.

OBJECTIVES: Despite substantial progress in surgical techniques and perioperative management, the treatment and long-term follow-up of type A acute aortic dissection (AAD) still remain a major challenge. The objective of this retrospective, multicentre study was to assess in a large series of patients the early and long-term results after surgery for type A AAD. METHODS: We analysed the preoperative, intraoperative and postoperative conditions of 1.148 consecutive patients surgically treated in seven large referral centres from 1981 to 2013. We applied to each patient three different multi-parameter risk profiles (preadmission risk, admission risk and post-surgery risk) in order to compare risk factors and outcome. Long-term Kaplan-Meier survival was evaluated. RESULTS: The median age was 64 years and the male population was predominant (66%). Identified diagnosis of collagen disease was present in 9%, and Marfan syndrome in 5%. Bicuspid aortic valve was present in 69 patients (6%). Previous cardiac surgery was identified in 10% of the patients. During surgery, the native aortic valve was preserved in 72% of the cases, including leaflet resuspension in 23% and David operation in 1.2%. Considering aortic valve replacement (AVR: 28%), bioprosthesis implantation was performed in 14.7% of the subjects. Neurological impairment at discharge was shown in 23% of the cases among which 21% of patients had new neurological impairment versus preoperative conditions. The overall 30-day mortality rate was 25.7%. All risk profiles remained independently associated with in-hospital mortality. During the available follow-up of hospital survivors (median: 70 months, interquartile range: 34-113, maximum: 396), cardiac-related death occurred in 7.9% of the subjects. The cumulative survival rate for cardiac death was 95.3% at 5 years, 92.8% at 10 years and 52.8% at 20 years. Severe aortic regurgitation (AR) (grade 3-4) at the time of surgery showed to be a significant risk factor for reintervention during the follow-up (P < 0.001). Among risk profiles, only the preadmission risk was independently associated with late mortality after multivariate analysis. Unexpectedly, there was no difference in freedom from cardiac death between patients with and without AVR. CONCLUSIONS: Although surgery for type A has remained challenging over more than three decades, there is a positive trend in terms of hospital mortality and long-term follow-up. About 90% of patients were free from reoperation in the long term, although late AR remains a critical issue, suggesting that a thorough debate on surgical options, assessment and results of a conservative approach should be considered.

Role of different vascular approaches on transcatheter aortic valve implantation outcome: a single-center study.

OBJECTIVE: To compare different vascular approaches on clinical outcome of patients undergoing transcatheter aortic valve implantation (TAVI) with self-expandable bioprosthesis. METHODS: We included all the patients undergoing CoreValve implantation at our institute between September 2007 and March 2014. They were divided into four groups based on the vascular approach: percutaneous transfemoral (pTF), cut-down transfemoral (cTF), transaxillary (TAx) and transaortic (TAo). Clinical outcomes were evaluated according to Valve Academic Research Consortium-2 recommendations. RESULTS: Out of 322 consecutive patients, 170 (53%) underwent pTF, 76 (23%) cTF, 32 (10%) TAx and 44 (14%) TAo approach. Although the TAx and TAo patients had a higher risk profile, they had a similar outcome compared with the pTF and cTF groups; in particular, there were no differences regarding cardiovascular and all-cause mortality at 30 days, 1 and 2 years, as well as stroke, myocardial infarction, bleeding, major vascular complications, permanent pacemaker implantation and acute kidney injury rates. The observed device success rate was higher in the TAo than in the other approaches (88.6 versus 65.9, 68.7 and 76.3% in the pTF, cTF and TAx groups, respectively; P = 0.019). No differences occurred regarding 30-day early safety and 1-year clinical efficacy across the four groups. Fluoroscopy time, amount of contrast medium used and minor vascular complications were significantly higher in pTF patients, as well as in-hospital stay in the TAo group. Atrial fibrillation and prosthetic valve regurgitation, but not the vascular approach, were independent predictors of all-cause mortality. CONCLUSION: A more invasive vascular approach, for CoreValve implantation, even in higher risk patients, does not affect early-term, mid-term and long-term outcomes.

Platypnea and orthodeoxia in a patient with pulmonary embolism.

The platypnea-orthodeoxia syndrome is a rare clinical manifestation. Platypnea is defined as shortness of breath that worsens in the upright position, and orthodeoxia represents hypoxemia that aggravates in the upright position. We report a case of a patient with platypnea-orthodeoxia syndrome associated with pulmonary embolism and patent foramen ovale.

Triple-orifice valve repair in severe Barlow disease with multiple-jet mitral regurgitation: report of mid-term experience.

OBJECTIVES: Barlow disease represents a surgical challenge for mitral valve repair (MR) in the presence of mitral insufficiency (MI) with multiple regurgitant jets. We hereby present our mid-term experience using a modified edge-to-edge technique to address this peculiar MI. METHODS: From March 2003 till December 2010, 25 consecutive patients (mean age 54 ± 7 years, 14 males) affected by severe Barlow disease with multiple regurgitant jets were submitted to MR. Preoperative transesophageal echo (TEE) in all the cases showed at least 2 regurgitant jets, involving one or both leaflets in more than one segment. In all the patients, a triple orifice valve (TOV) repair with annuloplasty was performed. Intra-operative TEE and postoperative transthoracic echocardiography (TTE) were carried out to evaluate results of the TOV repair. RESULTS: There was no in-hospital death and one late death (non-cardiac related). At intra-operative TEE, the three orifices showed a mean total valve area of 2.9 ± 0.1cm(2) (range 2.5-3.3 cm(2)) with no residual regurgitation (2 cases of trivial MI) and no sign of valve stenosis (mean transvalvular gradient 4.6 ± 1.5 mmHg). At follow up (mean 38 ± 22 months), TTE showed favourable MR and no recurrence of significant MI (6 cases of trivial and 1 of mild MI). Stress TTE was performed in 5 cases showing persistent effective valve function (2 cases of trivial MI at peak exercise). All the patients showed significant NYHA functional class improvement. CONCLUSIONS: This report indicates that the TOV technique is effective in correcting complex Barlow mitral valves with multiple jets. Further studies are required to confirm long-term applicability and durability in more numerous clinical cases.

Edge-to-edge mitral valve repair for isolated prolapse of the anterior leaflet caused by degenerative disease.

OBJECTIVE: The use of new techniques to correct mitral regurgitation due to prolapse of the anterior leaflet has been shown to expand the original surgical armamentarium and to improve postoperative outcome. We retrospectively analysed our experience with isolated prolapse of the anterior mitral leaflet repaired using the edge-to-edge technique. METHODS: From October 1986 to June 2004, 790 patients underwent mitral valve repair for mitral regurgitation at our institution. Isolated pathology of the anterior mitral leaflet, due to degenerative disease, was the cause of mitral regurgitation in 84 patients and, from 1991, 68 underwent edge-to-edge repair. RESULTS: There was no intraoperative death and one in-hospital death. Three patients died in the late follow-up period for a cumulative 13-year survival rate of 90 +/- 1.4%. No patient underwent early reoperation. Four patients underwent reoperation during the follow-up for a cumulative 13-year freedom from reoperation of 92.3 +/- 3.2%. At echocardiographic evaluation, mitral valve repair failure was associated with severe mitral regurgitation in four patients. Of the remaining 60 patients, 40 had no residual regurgitation, 18 had trivial residual regurgitation, and two had mild regurgitation. At follow-up, 49 patients are still in New York Heart Association (NYHA) class I, 14 in NYHA class II and only one in NYHA class III. CONCLUSIONS: Our study demonstrates that the 'edge-to-edge' technique is a reliable procedure to correct prolapsing leaflets. The addition of this technique to the surgical armamentarium has neutralized prolapse of the anterior leaflet as an incremental risk factor for reoperation.

The triple-orifice repair: a new technique for the treatment of mitral regurgitation in severe Barlow's disease.

We report a case of severe Barlow's disease with a very complex pathology, in which we applied the "edge-to-edge" technique, creating a triple-orifice mitral valve. Different techniques should be used to correct a similar valve defect; the combination of different surgical procedures and the valve pathology may influence the post-repair recurrence of regurgitation. We believe that it is better to perform a simple and reproducible repair than to carry on with combined complex procedures that could increase the risk of a suboptimal outcome.