Aortopathy and aortic valve surgery in patients with bicuspid aortic valve with and without raphe.

AIM: To evaluate the association between raphe in bicuspid aortic valve (BAV) patients and valve dysfunction, aortopathy and aortic valve surgery in the REBECCA registry [REgistro della valvola aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging (SIECVI)]. METHODS: Prevalence of aortic valve dysfunction and aortopathy was investigated in BAV patients with and without raphe. Aortic valve dysfunction (regurgitation or stenosis) was categorized as mild, moderate and severe. Aortopathy was defined as annulus ≥14 mm/m2; root ≥20 mm/m2; sinotubular junction ≥16 mm/m2; ascending aorta ≥17 mm/m2, and classified in Type A, isolated ascending aorta dilatation; Type B, aortic root and ascending aorta dilatation; and Type C, isolated aortic root dilatation. RESULTS: Overall, 695 patients with BAV were enrolled; 520 (74.8%) with raphe and 175 (25.2%) without raphe. BAV patients with raphe presented more frequently with moderate or severe aortic stenosis than BAV patients without raphe (183 [35.2%] vs 34 [19.4%], p < 0.001). A higher prevalence of aortopathy, particularly Type B, was observed in patients with vs without raphe. At multivariable analysis, raphe was a predictor of aortic valve surgery at three-year follow-up (odds ratio 2.19, 95% confidence interval 1.08-4.44, p < 0.001). CONCLUSIONS: Patients with BAV and raphe have a higher prevalence of significant aortic stenosis, aortopathy, especially Type B, and a higher risk of undergoing aortic valve surgery at three-year follow-up.

Infective Endocarditis in People Who Inject Drugs: Report from the Italian Registry of Infective Endocarditis.

Intravenous drug use is a predisposing condition for infective endocarditis (IE). We report the clinical features of IE, taken from the Italian Registry of IE, in people who inject drugs (PWIDs). The registry prospectively collected epidemiological, clinical, in-hospital, and follow-up data on patients with IE from 17 Italian centers. A total of 677 patients were enrolled, and 61 (9%) were intravenous drug users (IDUs). Most PWIDs were male (78.6%), and aged between 41 and 50 years old (50%). The most frequent comorbidities were HIV (34.4%) and chronic liver disease (32%). Predisposing factors for IE were present in 6.5% of the patients, and 10% had minor valvular abnormalities. IE had occurred previously in 16.4% of the patients, and 50% of them had undergone heart surgery. Overall mortality was 9.8% in IDUs and 20% in patients with recurrent IE. IE in PWIDs mostly affected the native valves (90%). The echocardiographic diagnosis of IE was based on the detection of vegetation in 91.82% of cases. Staphylococcus aureus was the main microorganism isolated (70%) from blood cultures. Thirty patients (49%) underwent heart surgery: thirteen had aortic valves, eleven had mitral valves, and six had tricuspid valve interventions. IE in PWIDs was relatively common, and patients with native valve right-sided IE had a better prognosis, with a low rate of surgical interventions.

Antiplatelet Therapy with Cangrelor in Patients Undergoing Surgery after Coronary Stent Implantation: A Real-World Bridging Protocol Experience.

Objective The aim of the study is to describe the real-world use of the P2Y 12 inhibitor cangrelor as a bridging strategy in patients at high thrombotic risk after percutaneous coronary intervention (PCI) and referred to surgery requiring perioperative withdrawal of dual antiplatelet therapy (DAPT). Materials and Methods We collected data from nine Italian centers on patients with previous PCI who were still on DAPT and undergoing nondeferrable surgery requiring DAPT discontinuation. A perioperative standardized bridging protocol with cangrelor was used. Results Between December 2017 and April 2019, 24 patients (mean age 72 years; male 79%) were enrolled. All patients were at high thrombotic risk after PCI and required nondeferrable intermediate to high bleeding risk surgery requiring DAPT discontinuation (4.6 ± 1.7 days). Cangrelor infusion was started at a bridging dose (0.75 µg/kg/min) 3 days before planned surgery and was discontinued 6.6 ± 1.5 hours prior to surgical incision. In 55% of patients, cangrelor was resumed at 9 ± 6 hours following surgery for a mean of 39 ± 38 hours. One cardiac death was reported after 3 hours of cangrelor discontinuation prior to surgery. No ischemic outcomes occurred after surgery and up to 30-days follow-up. The mean hemoglobin drop was <2 g/dL; nine patients received blood transfusions consistent with the type of surgery, but no life-threatening or fatal bleeding occurred. Conclusion Perioperative bridging therapy with cangrelor is a feasible approach for stented patients at high thrombotic risk and referred to surgery requiring DAPT discontinuation. Larger studies are warranted to support the safety of this strategy.

Bicuspid Aortic Valve Registry of the Italian Society of Echocardiography and Cardiovascular Imaging (REgistro della valvola aortica bicuspide della società italiana di ECocardiografia e CArdiovascular imaging): Rationale and Study Design.

BACKGROUND: Bicuspid aortic valve (BAV) is the most common congenital heart disease, affecting 0.5%-2% of the general population. It is associated not only with notable valvular risk (aortic stenosis and/or regurgitation, endocarditis) but also with aortopathy with a wide spectrum of unpredictable clinical presentations, including aneurysmal dilation of the aortic root and/or ascending thoracic aorta, isthmic coarctation, aortic dissection, or wall rupture. METHODS: The REgistro della Valvola Aortica Bicuspide della Società Italiana di ECocardiografia e CArdiovascular Imaging is a retrospective (from January 1, 2010)/prospective, multicenter, observational registry, expected to enroll 3000 patients with definitive diagnosis of BAV made by transthoracic and/or transesophageal echocardiography, computed tomography, cardiovascular magnetic resonance, or at surgery. Inclusion criteria were definitive diagnosis of BAV. Patients will be enrolled regardless of the presence and severity of aortic valve dysfunction or aortic vessel disease and the coexistence of other congenital cardiovascular malformations. Exclusion criteria were uncertain BAV diagnosis, impossibility of obtaining informed consent, inability to carry out the follow-up. Anamnestic, demographic, clinical, and instrumental data collected both at first evaluation and during follow-up will be integrated into dedicated software. The aim is to derive a data set of unselected BAV patients with the main purpose of assessing the current clinical presentation, management, and outcomes of BAV. CONCLUSIONS: A multicenter registry covering a large population of BAV patients could have a profound impact on the understanding of the natural history of this disease and could influence its management.

Mortality and timing of surgery in the left-sided infective endocarditis: an Italian multicentre study.

OBJECTIVES: Observational studies on early surgery in infective endocarditis have conflicting results. This study aims to compare the treatment strategies for early surgery (within 2 weeks of diagnosis) and late surgery/medical therapy in terms of survival among patients with the left-sided infective endocarditis. METHODS: This study included patients with the left-sided infective endocarditis registered between 2006 and 2010 in the Italian Registry of Infective Endocarditis (RIEI). A Cox proportional hazards model was used to estimate the effect of these treatment strategies on overall survival and included sociodemographic and clinical characteristics associated with treatment, risk factors for mortality and early surgery as a time-dependent covariate to avoid indication and immortal time biases. RESULTS: Among the 502 patients included, 184 (36.7%) underwent early surgery. Of the remaining 318 patients, 138 underwent late surgery. The early surgery group had fewer patients with comorbidities and with enterococcus as the causative microorganism, but this group had more complicated cardiac conditions. No difference in mortality risk was estimated between the treatment groups including early surgery as time-dependent variables (adjusted hazard ratio = 0.95, 95% confidence interval 0.55-1.63), while a distorted and overestimated beneficial effect of surgery was estimated considering surgery as a non-time-dependent variable (adjusted hazard ratio 0.41, 95% confidence interval 0.25-0.70). CONCLUSIONS: Our study did not confirm a better overall survival in patients undergoing early surgery. However, even with the use of statistical techniques to control biases, we could not draw definitive conclusions that early surgery is not beneficial. Our results need to be assessed by randomized trials before any changes in clinical practice can be recommended.

Predisposing cardiac conditions, interventional procedures, and antibiotic prophylaxis among patients with infective endocarditis.

UNLABELLED: Efficacy and safety of antibiotic prophylaxis (AP) for prevention of infective endocarditis (IE) in patients with predisposing cardiac condition (PCC) undergoing invasive procedures is still debated. We sought to assess the prevalence of PCC, the type of interventional procedures preceding the onset of symptoms, and the usefulness of AP in a large cohort of consecutive patients with definite IE. METHODS: We examined 677 (median age 65.34 years; male 492 [73%]) consecutive patients with IE enrolled from July 2007 through 2010 into the Italian Registry of Infective Endocarditis. RESULTS: Predisposing cardiac condition was present in 341 patients (50%).Thirty-two patients (4.7%) underwent dental procedures. Of 20 patients with PCC undergoing dental procedure, 13 had assumed AP. Viridans group streptococci were isolated from blood cultures in 8 of 20 patients with PCC and prior dental procedure. Nondental procedures preceded IE in 139 patients (21%). They were significantly older and had more comordibities compared with patients undergoing dental procedures. Predisposing cardiac condition was identified in 91 patients. Perioperative antimicrobial prophylaxis was administered to 67 patients. Staphylococcus aureus was the most frequent causative agent. Cardiac surgery was necessary in 85 patients (20 with prior dental and 65 with nondental procedure). Surgical mortality (12% vs 0%, P = .03) and hospital mortality (23% vs 3%, P = .001) were significantly larger among patients with nondental procedures. CONCLUSIONS: In a large unselected cohort of patients with IE, the incidence of preceding dental procedures was minimal. The number of cases potentially preventable by means of AP was negligible. Nondental procedures were more frequent than dental procedures and were correlated with poorer prognosis.

Clinical epidemiology in Italian Registry of Infective Endocarditis (RIEI): Focus on age, intravascular devices and enterococci.

INTRODUCTION: The epidemiology of infective endocarditis (IE) is changing due to a number of factors, including aging and health related comorbidities and medical procedures. The aim of this study is to describe the main clinical, epidemiologic and etiologic changes of IE from a large database in Italy. METHODS: We prospectively collected episodes of IE in 17 Italian centers from July 2007 to December 2010. RESULTS: We enrolled 677 patients with definite IE, of which 24% health-care associated. Patients were male (73%) with a median age of 62 years (IQR: 49-74) and 61% had several comorbidities. One hundred and twenty-eight (19%) patients had prosthetic left side IE, 391 (58%) native left side IE, 94 (14%) device-related IE and 54 (8%) right side IE. A predisposing cardiopathy was present in 50%, while odontoiatric and non odontoiatric procedures were reported in 5% and 21% of patients respectively. Symptoms were usually atypical and precocious. The prevalent etiology was represented by Staphylococcus aureus (27%) followed by coagulase-negative staphylococci (CNS, 21%), Streptococcus viridans (15%) and enterococci (14%). CNS and enterococci were relatively more frequent in patients with intravascular devices and prosthesis and S. viridans in left native valve. Diagnosis was made by transthoracic and transesophageal echocardiography in 62% and 94% of cases, respectively. The in-hospital mortality was 14% and 1-year mortality was 21%. CONCLUSION: The epidemiology is changing in Italy, where IE more often affects older patients with comorbidities and intravascular devices, with an acute onset and including a high frequency of enterococci. There were few preceding odontoiatric procedures.

[Multidisciplinary position paper on the management of patent foramen ovale in the presence of cryptogenic cerebral ischemia - Italian version 2013]. / Documento di posizione multidisciplinare sulla gestione del forame ovale pervio in presenza di ischemia cerebrale criptogenica - versione 2013.

There is no generally accepted consensus on the management of patent foramen ovale (PFO) in the presence of cryptogenic cerebral ischemia, because of the lack of conclusive evidence. The aim of this position paper was to develop and promote a joint approach based on available data that may be shared by different specialists, while waiting for definite results from randomized controlled trials. A position statement was produced involving the major national scientific societies. The task force members were nominated by the presidents and/or executive boards of each society or working group, as appropriate, based on their previous work in relevant topic areas. Specific task force working groups prepared the drafts. In order to achieve maximum agreement, these drafts were merged and distributed to the scientific societies for local evaluation. The ensuing final draft, merging all the revisions, was reviewed by the task force and finally approved by all scientific societies. The following issues were addressed: definitions of transient ischemic attack (TIA) and both symptomatic and asymptomatic cryptogenic stroke; formulation of a diagnostic workup for patients with clinical event(s) and PFO; recommendations regarding medical and interventional treatment options considering individual risk factors based on the three available randomized trials and other observational studies; recommendations regarding requirements for operators and centers in Italy; definition of a follow-up evaluation protocol. In conclusion, available data provided the basis for the first multi-society position paper on the management of cryptogenic stroke/TIA and PFO.

Management of patients with patent foramen ovale and cryptogenic stroke: a collaborative, multidisciplinary, position paper: executive summary.

OBJECTIVES: To organize a common approach on the management of patent foramen ovale (PFO) and cryptogenic stroke that may be shared by different specialists. BACKGROUND: The management of PFO related to cryptogenic stroke is controversial, despite an increase in interventional closure procedures. METHODS: A consensus statement was developed by approaching Italian national cardiological, neurological, and hematological scientific societies. Task force members were identified by the president and/or the boards of each relevant scientific society or working group, as appropriate. Drafts were outlined by specific task force working groups. To obtain a widespread consensus, these drafts were merged and distributed to the scientific societies for local evaluation and revision by as many experts as possible. The ensuing final draft, merging all the revisions, was reviewed by the task force and finally approved by scientific societies. RESULTS: Definitions of transient ischemic attack and both symptomatic and asymptomatic cryptogenic strokes were specified. A diagnostic workout was identified for patients with candidate event(s) and patient foramen ovale to define the probable pathogenesis of clinical events and to describe individual PFO characteristics. Further recommendations were provided regarding medical and interventional therapy considering individual risk factors of recurrence. Finally, follow-up evaluation was appraised. CONCLUSIONS: Available data provided the basis for a shared approach to management of cryptogenic ischemic cerebral events and PFO among different Italian scientific societies. Wider international initiatives on the topic are awaited.

The diagnostic utility of transthoracic echocardiography for the diagnosis of infective endocarditis in the real world of the Italian Registry on Infective Endocarditis.

BACKGROUND: The choice of the imaging modality (transthoracic [TTE] vs. transesophageal echocardiography [TEE]) for the diagnosis of infective endocarditis (IE) depends on different variables. Aim of the present study is to provide updated data on the diagnostic sensitivity and the clinical usefulness of TTE vs. TEE from the Italian Registry on IE (RIEI). METHODS: The RIEI has enrolled consecutive cases of IE in every participating centre, evaluating diagnostic and therapeutic data from a real world practice perspective. RESULTS: From July 2007 to October 2010, 658 consecutive cases with definite IE according to Duke criteria have been enrolled in the RIEI (483 males). The following diagnostic echocardiographic exams were performed: 616 TTE (94%) and 476 TEE (72%). A positive TTE was recorded in 399 cases (65%), an uncertain TTE in 108 cases (17%), and a negative TTE in 109 cases (18%). For TEE, a positive study was reported in 451 cases (95%), uncertain in 13 cases (2.7%), and negative in 12 cases (2.5%) (P < 0.001). This difference is not evident in patients with tricuspid valve IE or i.v. drug addiction, and in Streptococcus bovis or Streptococcus viridans IE. TTE was significantly more performed before the admission and earlier than TEE during admission (P = 0.000). TTE was mainly responsible for the initial diagnosis in 59%. TEE contributed to changing the therapeutic approach in 42%. CONCLUSIONS: In the real world, TTE is performed earlier and more commonly, and it is the major echocardiographic tool for the initial diagnosis. TEE confirms its superior diagnostic sensitivity in most cases, although it is relatively underused.

Management of patients with patent foramen ovale and cryptogenic stroke: a collaborative, multidisciplinary, position paper.

OBJECTIVES: To organize a common approach on the management of patent foramen ovale (PFO) and cryptogenic stroke that may be shared by different specialists. BACKGROUND: The management of PFO related to cryptogenic stroke is controversial, despite an increase in interventional closure procedures. METHODS: A consensus statement was developed by approaching Italian national cardiological, neurological, and hematological scientific societies. Task force members were identified by the president and/or the boards of each relevant scientific society or working group, as appropriate. Drafts were outlined by specific task force working groups. To obtain a widespread consensus, these drafts were merged and distributed to the scientific societies for local evaluation and revision by as many experts as possible. The ensuing final draft, merging all the revisions, was reviewed by the task force and finally approved by scientific societies. RESULTS: Definitions of transient ischemic attack and both symptomatic and asymptomatic cryptogenic strokes were specified. A diagnostic workout was identified for patients with candidate event(s) and patient foramen ovale to define the probable pathogenesis of clinical events and to describe individual PFO characteristics. Further recommendations were provided regarding medical and interventional therapy considering individual risk factors of recurrence. Finally, follow-up evaluation was appraised. CONCLUSIONS: Available data provided the basis for a shared approach to management of cryptogenic ischemic cerebral events and PFO among different Italian scientific societies. Wider international initiatives on the topic are awaited.

The role of transthoracic echocardiography in the diagnosis and management of acute type A aortic syndrome.

BACKGROUND: Transthoracic echocardiography (TTE) has been traditionally considered inadequate for the diagnosis of acute type A aortic syndrome (AAAS). In the last decade, high-resolution probes and harmonic imaging have been implemented in new echocardiographic systems. However, studies assessing the diagnostic accuracy of TTE for the identification of AAAS in large populations using modern ultrasound technology are lacking. METHODS: The diagnostic value of harmonic imaging TTE was assessed in 270 consecutive patients with suspected AAAS in whom TTE was the initial diagnostic test. RESULTS: Acute type A aortic syndrome was diagnosed in 67 patients and excluded in 203 patients (disease prevalence 25%). Sixty-two patients had a classic acute type A aortic dissection, and 5, an acute type A intramural hematoma. Image quality achieved was considered optimal in 244 patients (90%). In the whole study population, TTE had sensitivity, specificity, positive predictive value, and negative predictive value for the diagnosis of AAAS of 87%, 91%, 75%, and 95%, respectively. When evaluating only patients with optimal image quality, these values increased to 97%, 100%, 100%, and 99%, respectively. Forty-seven patients with clear-cut evidence of AAAS were transferred immediately to the operative room, where transesophageal echocardiography confirmed the diagnosis obtained by TTE in all patients. CONCLUSIONS: Transthoracic echocardiography is a useful imaging modality for the diagnosis of classic acute type A aortic dissection. It cannot be used as the sole screening technique for detecting AAAS, but in the light of the predictive values observed, patients with optimal image quality and clear-cut diagnosis of AAAS should proceed to the operative room, whereas in patients with negative or indeterminate studies, other imaging techniques are needed to refine the diagnosis.

Infective endocarditis in the real world: the Italian Registry of Infective Endocarditis (Registro Italiano Endocardite Infettiva - RIEI).

In the last 30 years, major improvements have been made in understanding the pathogenesis, diagnosis and treatment of infective endocarditis. Nevertheless, mortality still remains high, close to 30-40% at 1 year, and its reduction remains the main challenge. Moreover, important epidemiological changes have been recorded. Social changes in Western countries have led to an increase in the mean length of life, and thus in degenerative valvular diseases, whereas rheumatic heart disease has almost disappeared. Increasing medicalization has led to a rise in complications and diseases related to longer hospital stay, surgical therapies and other invasive interventions. At the same time, there is an increase in immunosuppressive therapies, diseases such as cancer, diabetes mellitus and renal insufficiency that may enhance the disease. Further knowledge is needed for specific subgroups to improve both treatment and prognosis. Nevertheless, randomized trials are lacking to guide the management of the disease, and the role and indications of antibiotic prophylaxis are still the subject of debate. International multicenter studies are providing new important findings based on the experience of tertiary centers; these results may reflect referral biases. The proposal of an Italian national registry on infective endocarditis (RIEI) will overcome these limitations and provide a wide picture of the national presentation of the disease. The aim of the registry is to improve the management of infective endocarditis, through a better understanding of demographic, clinical, therapeutic and prognostic features of the disease in the real world. The background, rationale, aims and expected results of the registry are reviewed.

Follow-up and physical activity in postoperative congenital heart disease.

During the past three decades, interventional cardiology and cardiac surgery have found solutions even for the most complex congenital heart malformations with an overall low operative mortality. A careful clinical and instrumental follow-up of postoperative congenital heart disease patients is fundamental not only to prevent complications and/or to treat eventual residua and sequelae, but also to modify future surgical strategies on the basis of long-term results. To be able to give a correct prognostic meaning to the data collected during the follow-up, the cardiologist should have an excellent knowledge of the native defect, the surgical technique and the post-surgical anatomy and physiology. Major cardiological concerns during a follow-up after corrective surgery are: arrhythmias; heart failure; cyanosis and erythrocytosis; and infective endocarditis. Psychosocial needs, such as employment, contraception, pregnancy and physical exercise, are very important to enable a 'normal' life, complying with the postoperative hemodynamic situation of the patients.

Delayed systolic anterior motion after mitral valve repair.

A rare case of systolic anterior motion (SAM) after mitral valve repair is described. A temporary postoperative left ventricular (LV) dysfunction protected against this complication during the early postoperative period. Only on day 9 postoperatively did the left ventricle recover and SAM develop, with significant obstruction of the LV outflow tract. Subsequently, mitral valve replacement was required. This case demonstrates the importance of the LV geometrical relationships that predispose to this complication.

Late cardiac perforation after transcatheter closure of patent foramen ovale.

Cardiac perforation after transcatheter closure of a patent foramen ovale is a very rare complication. We report a case of left atrial wall perforation and aortic root erosion occurring 16 months after implantation of an Amplatzer patent foramen ovale occluder that required emergent surgery.

Aortic dilatation in patients with bicuspid aortic valve.

The association of a bicuspid aortic valve (BAV) with abnormalities of the proximal thoracic aorta, including dilatation, aneurysm and dissection, has been previously described, leading to the hypothesis of a common underlying developmental defect involving the aortic valve and the aortic wall. Consequently, any patient with BAV should receive a careful assessment not only of the valve function, but also of the aortic root and the ascending aorta. Dilatation of the proximal thoracic aorta is a common finding in patients with BAV and is believed to be related to aortic rupture and dissection. Because progressive dilatation can occur, careful long-term surveillance of the aortic dimensions is required. Prophylactic surgical repair of the dilated aorta should be recommended more aggressively for patients with BAV than for those with a tricuspid aortic valve. However, the optimal timing of aortic surgery in BAV patients remains uncertain because of the limited data available on the natural history of asymptomatic aortic dilatation.

Aortic dimensions in patients with bicuspid aortic valve without significant valve dysfunction.

The dimensions of the entire aorta at different anatomic levels were measured by transthoracic 2-dimensional echocardiography in 162 consecutive patients with isolated bicuspid aortic valves (BAVs) without significant aortic valve dysfunction. Aortic dilation involved the aortic root and the ascending aorta but was not present in the descending and abdominal aorta. A significant increase in the dimensions of the aortic arch was found in patients with BAVs aged >40 years. Ascending aortic diameter and the extension of aortic dilation were significantly correlated with age, but no correlation was found between aortic dimensions and aortic valve morphology.