Hypertrophic obstructive cardiomyopathy caused by Fabry disease: implications for surgical myectomy.

Hypertrophic obstructive cardiomyopathy can be the phenotype of storage disorders as Fabry disease cardiomyopathy. In this instance, its recognition through GLA gene analysis and preventive administration of enzyme replacement therapy may reduce heart failure risk of surgical septal myectomy (SSM). A 59-year-old man was referred for SSM as dyspnoea and low threshold muscle fatigue associated to severe left ventricular outflow obstruction (gradient of 100 mmHg) due to both interventricular septal hypertrophy and mitral leaflet systolic anterior motion were not controlled by metoprolol 100 mg bid. Electrocardiogram showed sinus rhythm and a complete left bundle branch block. Cardiac magnetic resonance imaging showed a preserved left ventricular (LV) contractility (ejection fraction 70%) but failed to reveal reduced T1 mapping and fibrosis of postero-lateral LV wall suggesting Fabry disease cardiomyopathy. Cardiac catheterization and coronary angiography documented increased LV end-diastolic pressure but normal coronary arteries. SSM was followed by acute renal and heart failure with left ventricular ejection fraction declining to 35%. Histology of SSM showed regularly arranged severely enlarged cardiomyocytes containing extensive vacuoles that were intensely positive to immunofluorescence with anti-Gb3 antibodies and appeared at electron microscopy to consist of myelin bodies suggesting the diagnosis of FD. This entity was confirmed by low blood levels of alpha-galactosidase A (0.8 nmol/mL/h; NV > 1), high values of Lyso-Gb3 (5.85 nmol/L; NV < 2.3), and the presence of the pathogenic mutation c.644A>G in the exon 5 of GLA gene. This study emphasizes the importance of a genetic screening for FD before SSM be considered for hypertrophic obstructive cardiomyopathy.

Cardiac surgery practice during the COVID-19 outbreak: a multicentre national survey.

OBJECTIVES: Healthcare systems worldwide have been overburdened by the coronavirus disease 2019 (COVID-19) outbreak. Accordingly, hospitals had to implement strategies to profoundly reshape both non-COVID-19 medical care and surgical activities. Knowledge about the impact of the COVID-19 pandemic on cardiac surgery practice is pivotal. The goal of the present study was to describe the changes in cardiac surgery practices during the health emergency at the national level. METHODS: A 26-question web-enabled survey including all adult cardiac surgery units in Italy was conducted to assess how their clinical practice changed during the national lockdown. Data were compared to data from the corresponding period in 2019. RESULTS: All but 2 centres (94.9%) adopted specific protocols to screen patients and personnel. A significant reduction in the number of dedicated cardiac intensive care unit beds (-35.4%) and operating rooms (-29.2%), along with healthcare personnel reallocation to COVID departments (nurses -15.4%, anaesthesiologists -7.7%), was noted. Overall adult cardiac surgery volumes were dramatically reduced (1734 procedures vs 3447; P < 0.001), with a significant drop in elective procedures [580 (33.4%) vs 2420 (70.2%)]. CONCLUSIONS: This national survey found major changes in cardiac surgery practice as a response to the COVID-19 pandemic. This experience should lead to the development of permanent systems-based plans to face possible future pandemics. These data may effectively help policy decision-making in prioritizing healthcare resource reallocation during the ongoing pandemic and once the healthcare emergency is over.

The TRIBECA study: (TRI)fecta (B)ioprosthesis (E)valuation versus (C)arpentier Magna-Ease in (A)ortic position.

OBJECTIVE: To determine whether the Trifecta bioprosthetic aortic valve produces postoperative haemodynamic results comparable with or better than those of the Magna Ease aortic valve bioprosthesis. METHODS: We retrospectively reviewed the medical records of patients who had undergone aortic valve replacement with Trifecta or Magna Ease prostheses at eight European institutions between January 2011 and May 2013, and analysed early postoperative haemodynamic performance by means of echocardiography. RESULTS: A total of 791 patients underwent aortic valve replacement (469 Magna Ease, 322 Trifecta). Haemodynamic variables were evaluated on discharge and during the follow-up (minimum 6 months, maximum 12 months). The mean gradient and the indexed effective orifice area (IEOA) were as follows: 10 mmHg [interquartile range (IQR): 8-13] and 1.10 cm(2)/m(2) (IQR: 0.95-1.27) for Trifecta; 16 mmHg (IQR: 11-22) and 0.96 cm(2)/m(2) (IQR: 0.77-1.13) for Magna Ease (P < 0.001). These significant differences were maintained across all valve sizes. Similar statistically significant differences were found when patients were matched and/or stratified for preoperative characteristics: body-surface area, ejection fraction, mean gradients and valve size. Severe prosthesis-patient mismatch (IEOA: <0.65 cm(2)/m(2)) was detected in 2 patients (0.6%) with Trifecta and 40 patients (8.5%) with Magna Ease (P < 0.001). CONCLUSIONS: The haemodynamic performance of the Trifecta bioprosthesis was superior to that of the Magna Ease valve across all conventional prosthesis sizes, with almost no incidence of severe patient-prosthesis mismatch. The long-term follow-up is needed to determine whether these significant haemodynamic differences will persist, and influence clinical outcomes.

A giant coronary artery aneurysm with coronary arteriovenous fistula in asymptomatic elderly patient.

Coronary Arteriovenous Fistula (CAF) is a rare defect that occurs in 0.1-0.2% of patients undergoing coronary angiography; Coronary Artery Aneurism (CAA) also occurs in approximately 15-19% of patients with CAF. It is usually congenital, but in rare occasions it occurs after chest trauma, cardiac surgery, or coronary interventions. The case described is that of a 72-year-old woman, without previous history of cardiovascular disease, who presented a huge cardiac mass. A multimodal approach was necessary to diagnose a giant CAA with CAF responsible for compression and displacement of cardiac structures. Due to likely congenitally origin of the lesion and the absence of symptoms correlated to the CAA and to the CAF we decided to avoid invasive interventions and to treat the patient with medical therapy.

Mitral valve repair or replacement for ischemic mitral regurgitation? The Italian Study on the Treatment of Ischemic Mitral Regurgitation (ISTIMIR).

OBJECTIVE: It is uncertain whether mitral valve replacement is really inferior to mitral valve repair for the treatment of chronic ischemic mitral regurgitation. This multicenter study aimed at providing a contribution to this issue. METHODS: Of 1006 patients with chronic ischemic mitral regurgitation and impaired left ventricular function (ejection fraction < 40%) operated on at 13 Italian institutions between 1996 and 2011, 298 (29.6%) underwent mitral valve replacement whereas 708 (70.4%) received mitral valve repair. Propensity scores were calculated by a nonparsimonious multivariable logistic regression, and 244 pairs of patients were matched successfully using calipers of width 0.2 standard deviation of the logit of the propensity scores. The postmatching median standardized difference was 0.024 (range, 0-0.037) and in none of the covariates did it exceed 10%. RESULTS: Early deaths were 3.3% (n = 8) in mitral valve repair versus 5.3% (n = 13) in mitral valve replacement (P = .32). Eight-year survival was 81.6% ± 2.8% and 79.6% ± 4.8% (P = .42), respectively. Actual freedom from all-cause reoperation and valve-related reoperation were 64.3% ± 4.3% versus 80% ± 4.1%, and 71.3% ± 3.5% versus 85.5% ± 3.9 in mitral valve repair and mitral valve replacement, respectively (P < .001). Actual freedom from all valve-related complications was 68.3% ± 3.1% versus 69.9% ± 3.3% in mitral valve repair and mitral valve replacement, respectively (P = .78). Left ventricular function did not improved significantly, and it was comparable in the 2 groups postoperatively (36.9% vs 38.5%, P = .66). At competing regression analysis, mitral valve repair was a strong predictor of reoperation (hazard ratio, 2.84; P < .001). CONCLUSIONS: Mitral valve replacement is a suitable option for patients with chronic ischemic mitral regurgitation and impaired left ventricular function. It provides better results in terms of freedom from reoperation with comparable valve-related complication rates.

Emergency surgery for native mitral valve endocarditis: the impact of septic and cardiogenic shock.

BACKGROUND: Limited information exists about the real impact of the etiology of shock on early and late outcome after emergency surgery in acute native mitral valve endocarditis (ANMVE). This multicenter study analyzed the impact of the etiology of shock on early and late outcome in patients with ANMVE. METHODS: Data were collected in eight institutions. Three hundred-seventy-nine ANMVE patients undergoing surgery on an emergency basis between May 1991 and December 2009 were eligible for the study. According to current criteria used for the differential diagnosis of shock, patients were retrospectively assigned to one of three groups: group 1, no shock (n=154), group 2, cardiogenic shock (CS [n=118]), and group 3, septic shock (SS [n=107]). Median follow-up was 69.8 months. RESULTS: Early mortality was significantly higher in patients with SS (p<0.001). At multivariable logistic regression analysis, compared with patients with CS, patients with SS had more than 3.8 times higher risk of death. That rose to more than 4 times versus patients without shock. In addition, patients with SS had 4.2 times and 4.3 times higher risk of complications compared with patients with CS and without shock, respectively. Sepsis was also an independent predictor of prolonged artificial ventilation (p=0.04) and stroke (p=0.003) whereas CS was associated with a higher postoperative occurrence of low output syndrome and myocardial infarction (p<0.001). No difference was detected between groups in 18-year survival, freedom from endocarditis, and freedom from reoperation. CONCLUSIONS: Our study suggests that emergency surgery for ANMVE in patients with CS achieved satisfactory early and late results. In contrast, the presence of SS was linked to dismal early prognosis. Our findings need to be confirmed by further larger studies.

Extended replacement of a calcified ascending aorta in adulthood sporadic, diffuse type, supravalvular aortic stenosis.

The diffuse variant of supravalvular aortic stenosis (SVAS) is one of the most rare congenital vascular pathological conditions of adulthood. Due to its rarity, surgical treatment of this clinical entity is still a matter of debate because of the variable degree of aortic narrowing, presence of multilevel obstruction, and age at presentation, all factors that strongly influence the disease prognosis. We report a case of an adult patient with an extremely calcified diffuse SVAS who underwent successful replacement of the ascending aorta through the interposition of a tubular prosthesis. Six months' follow-up showed complete relief of the aortic gradient and an improvement in clinical performance.

Aortic valve re-implantation technique with Gelweave Valsalva prosthesis: safety, reproducibility and pitfalls of a geometric reconstruction.

BACKGROUND: To evaluate the safety, reproducibility and pitfalls of an aortic valve re-implantation (AVRei) technique. METHODS: From June 2005 to December 2008, 30 patients underwent aortic valve-sparing re-implantation with Gelweave Valsalva prosthesis. Mean age was 66 +/- 7 years (range 47-81). Mean aortic root diameter was 49 +/- 6 mm (range 37-70) and 12 patients had an aortic insufficiency more than 2+. All the patients were elective, except three who underwent surgery for type A aortic dissection. Two patients had Marfan syndrome and one had a bicuspid aortic valve. Isolated aortic root replacement was performed in 26 patients, whereas hemiarch extension was required in four. All the survivors underwent serial echocardiographic assessment for functional results and multi-detector computed tomography (MDCT) for aortic root morphology evaluation. RESULTS: There was one early death and one re-exploration for bleeding. Two patients suffered from a perioperative stroke and four required a pacemaker implantation because of a complete atrio-ventricular block. Mean follow-up was 12 +/- 10 months (range 1-42) with no late deaths, whereas freedom from reoperation was 100% and freedom from aortic insufficiency 2+ or more was 96.5%. MDCT aortic root reconstruction showed a pseudo-normalization of the neo-sinuses of Valsalva mimicking the human normal aortic root morphology. CONCLUSION: AVRei with Valsalva conduit is a well-tolerated procedure both in elective and emergency situations. In well-selected patients, good functional and clinical results can be achieved, regardless of the cause of the aortic root disease. Application of simple surgical manoeuvres allows durable clinical efficacy to be obtained without the risk of major complications.

Importance of surgical timing on postoperative outcome in patients with native valve acute endocarditis.

BACKGROUND: The present study was undertaken to establish whether surgical outcome could be influenced by surgical timing in patients affected by native valve endocarditis (NVE). METHODS: From March 2002 to December 2004, 19 patients underwent surgical operation for NVE. Aortic valve replacement (AVR) was performed in ten patients (53%), mitral valve repair (MVRep) was performed in five patients (26%) and multivalvular procedures were performed in the remaining four patients (21%). In three patients (15.5%), emergency surgery was required for refractory congestive heart failure, urgent surgery was necessary in ten patients [in six patients (31%) for paravalvular abscess, in three patients (15.5%) for macrovegetations and in one patient (6%) for systemic embolism, respectively], five patients (26.3%) with isolated valve incompetence underwent elective surgery, whereas delayed surgery was reserved for one patient (6%) because of pre-operative embolic stroke. RESULTS: There were no surgical procedure, cardiac or infectious related deaths at 30 days in the entire group. One patient died from an intravenous overdose. Follow-up was 100% complete in the 18 hospital survivors and ranged from 4 to 37 months (mean 14.2 +/- 10 months). There were no late death, recurrence of endocarditis, or re-operation at follow-up. CONCLUSIONS: The surgical results for NVE are excellent if surgical timing criteria are correctly applied during the acute phase of the infectious process. Immediate surgical correction is required when rapid hemodynamic deterioration occurs whereas a more aggressive surgical approach appears to be advisable in the case of paravalvular abscess, macrovegetations or systemic embolism. Delayed surgery is recommended when pre-operative stroke develops.