Effects of Surgical Techniques on Long-Term Results in Patients with Degenerative Mitral Valve Bileaflet Prolapse.

BACKGROUND AND AIM OF THE STUDY: The study aim was to evaluate the long-term results in patients with degenerative mitral valve bileaflet prolapse (DMVBLP) undergoing mitral valve repair (MVr) or mitral valve replacement (MVR), and to compare the consequences of survival related to each technique. METHODS: Between 2001 and 2012, a total of 421 patients underwent isolated primary surgery for DMVBLP. MVr was performed in 146 patients (34.7%), and MVR in 275 (65.3%). MVR patients were allocated to two subgroups. Subgroup A were operated on in routine fashion, preserving the posterior subvalvular apparatus, and in selected cases the anterior or both apparatus (n = 119; 43.3%). In subgroup B, surgery was performed without preservation of the subvalvular apparatus (n = 156; 56.7%). RESULTS: There were no intraoperative deaths in all patient groups. The median length of follow up was 5.96 ± 3.28 years. Five patients (3.4%) in the MVr group died, while 11 in MVR subgroup A (9.2%) died, and 29 in MVR subgroup B (18.6%). Patients in the MVr group demonstrated significant and persistent postoperative decreases in left ventricular end-diastolic diameter (LVEDD) and left ventricular end-systolic diameter (LVESD) during the follow up, while the left ventricular ejection fraction (LVEF) showed a trend to improve. In MVR subgroup A, preservation of the mitral subvalvular structures resulted in a decrease in LVEDD; this resulted in a lesser worsening of the LVEF, as occurs when subvalvular structures are resected. In MVR subgroup B, the LVEDD and LVESD were each increased constantly, which resulted in a statistically significant worsening of the LVEF. CONCLUSIONS: MVr in DMVBLP patients achieved a better preservation of left ventricular systolic indices than MVR, and guaranteed better shortand long-term survivals. When MVr is not feasible, it is recommended that subvalvular preservation be performed during MVR, in order to reduce the risk of early and late mortality and to improve left ventricular function.

Early cell changes and TGFß pathway alterations in the aortopathy associated with bicuspid aortic valve stenosis.

Previous studies on BAV (bicuspid aortic valve)-related aortopathy, whose aetiology is still debated, have focused mainly on severe dilatations. In the present study, we aimed to detect earlier signs of aortopathy. Specimens were collected from the 'concavity' (lesser curvature) and the 'convexity' (greater curvature) of mildly dilated AAs (ascending aortas; diameter ≤4 cm) with stenotic TAV (tricuspid aortic valve) or BAV and from donor normal aortas. Specimens were submitted to morphometry, immunohistochemistry and differential gene-expression analysis, focusing on SMC (smooth muscle cell) phenotype, remodelling, MF (myofibroblast) differentiation and TGFß (transforming growth factor ß) pathway. Smoothelin and myocardin mRNAs decreased in all the samples from patients, with the exception of those from BAV convexity, where a change in orientation of smoothelin-positive SMCs and an increase of α-SMA (α-smooth muscle actin) mRNA occurred. Dilated aortas from BAV and TAV patients showed both shared and distinct alterations concerning the TGFß pathway, including an increased TGFß and TGFßR2 (TGFß receptor 2) expression in both groups and a decreased TGFßR1 expression in BAV samples only. Despite a decrease of the mRNA coding for the ED-A (extra domain-A) isoform of FN (fibronectin) in the BAV convexity, the onset of the expression of the corresponding protein in the media was observed in dilated aortas, whereas the normal media from donors was negative for this isoform. This discrepancy could be related to modifications in the intima, normally expressing ED-A FN and showing an altered structure in mild aortic dilatations in comparison with donor aorta. Our results suggest that changes in SMC phenotype and, likely, MF differentiation, occur early in the aortopathy associated with valve stenosis. The defective expression of TGFßR1 in BAV might be a constitutive feature, while other changes we reported could be influenced by haemodynamics.

The need for a specific risk prediction system in native valve infective endocarditis surgery.

The need for a specific risk score system for infective endocarditis (IE) surgery has been previously claimed. In a single-center pilot study, preliminary to future multicentric development and validation, bivariate and multivariate (logistic regression) analysis of early postoperative mortality predictors in 440 native valve IE patients were performed. Mathematical procedures assigned scores to the independent predictors emerged (AUC of the ROC curve: 0.88). Overall mortality was 9.1%. Six predictors were identified and assigned scores, including age (5-13 points), renal failure (5), NYHA class IV (9), critical preoperative state (11), lack of preoperative attainment of blood culture negativity (5), perivalvular involvement (5). Four risk classes were drawn ranging from "very low risk" (≤5 points, mean predicted mortality 1%), and to "very high risk" (≥20 points, 43% mortality). IE-specific risk stratification models are both needed, as disease-specific factors (e.g., cultures, abscess), beside the generic ones (e.g., age, renal impairment) affect mortality, and feasible.

LOWERing the INtensity of oral anticoaGulant Therapy in patients with bileaflet mechanical aortic valve replacement: results from the "LOWERING-IT" Trial.

BACKGROUND: Moderate anticoagulation after mechanical heart valve replacement has been proposed to reduce the risk of bleeding related to lifelong anticoagulation. However, the efficacy of such reduced antithrombotic regimens is still unknown. The present prospective open-label, single-center, randomized controlled trial aimed to evaluate the safety and feasibility of reduced oral anticoagulation after isolated mechanical aortic valve replacement. METHODS: Low-risk patients undergoing bileaflet mechanical aortic valve replacement were randomized to a low International normalized ratio (INR) target (1.5-2.5; LOW-INR group) or to the standard currently recommended INR (2.0-3.0; CONVENTIONAL-INR group) through daily coumarine oral therapy. No aspirin was added. Median follow-up was 5.6 years. The primary outcome was assessment of noninferiority of the low over the standard anticoagulation regimen on thromboembolic events. Secondary end point was the superiority of the reduced INR target strategy on bleeding events. RESULTS: We analyzed 396 patients (197 in the LOW-INR group and 199 in the CONVENTIONAL-INR group). The mean of INR was 1.94 +/- 0.21 and 2.61 +/- 0.25 in the LOW-INR and CONVENTIONAL-INR groups, respectively (P < .001). One versus three thromboembolic events occurred in the LOW-INR and CONVENTIONAL-INR, respectively, meeting the noninferiority criterion (P = .62). Total hemorrhagic events occurred in 6 patients in the LOW-INR group and in 16 patients in the CONVENTIONAL-INR group (P = .04). CONCLUSIONS: LOWERING-IT trial established that the proposed LOW-INR target is safe and feasible in low-risk patients after bileaflet aortic mechanical valve replacement. It results in similar thrombotic events and in a significant reduction of bleeding occurrence when compared to the conventional anticoagulation regimen.

RIFLE criteria for acute kidney injury in valvular surgery.

BACKGROUND AND AIM OF THE STUDY: The RIFLE classification, which defines three grades of increasing severity of acute kidney injury--risk (RIFLE R), injury (RIFLE I) and failure (RIFLE F), and two outcome classes (L, loss) and E (end-stage kidney disease)--represents a valuable method for evaluating acute renal failure. Risk factors for acute kidney injury (AKI) according to the RIFLE criteria and for operative mortality were identified in patients undergoing valvular procedures. METHODS: A single-center prospective cohort study of 1424 patients who were not receiving renal replacement therapy preoperatively was conducted between January 2004 and December 2007. A total of 100 variables was collected from each patient. RESULTS: The main features were: mean age 61.9 +/- 12.9 years (range: 15-88 years), 47% females, 6% endocarditis, 11% redo surgery, 8% urgent/emergent surgery, 30% combined procedures, 5% complex, and 16% associated coronary artery bypass grafting (CABG). The overall AKI prevalence was 10%, with RIFLE scores of I or F being detected in 8% and continuous veno-venous hemofiltration being required in 5%. Risk factors for AKI were age (OR 1.03; 95% CI 1.14-4.15), time of extracorporeal circulation (ECC) (OR 1.09; 95% CI 1.005-1.013), redo procedure (OR 2.35; 95% CI 1.42-3.8), chronic kidney disease (OR 3.2; 95% CI 1.6-6.1), and blood transfusion (OR 3.8; 95% CI 2.5-6.5). The transfusion of leukodepleted blood exerted a protective effect on AKI development (OR 0.6; 95% CI 0.4-0.9). The average overall hospital mortality was 4.8%. Risk factors for operative mortality included: ECC time (OR 1; 95% CI 1.002-1.014), age (OR 1.043; 95% CI 1.01-1.07), chronic kidney disease (OR 4.8; 95% CI 2.2-10.6), blood transfusion (OR 6.43; 95% CI 2.8-14.7), surgical priority (OR 6.5; 95% CI 2.8-14.7), RIFLE class I (OR 11.9; 95% CI 5.5-25.7), and RIFLE class F (OR 30; 95% CI 8.1-111.7). Mortality increased with each RIFLE stratification (Normal 1.7%, RIFLE R = 4.1%, RR = 2.5; RIFLE I = 27.6%, RR = 16.2; and RIFLE F = 43.8% RR = 25.8). CONCLUSION: AKI is a highly prevalent and prognostically important complication, for which the majority of risk factors that have been identified are not modifiable. The transfusion of leukodepleted blood products was seen to exert a preventive effect.

A Starr-Edwards mitral prosthesis after 44 years of good performance.

The Starr-Edwards caged-ball prosthesis has been widely used to replace cardiac valves. The Model 6120 mitral prosthesis was introduced on the market in 1965 to reduce the high incidence of ball variance and thromboembolism of the previous model. We report the case of a Starr-Edwards Model 6120 which had been in place for 44 years and was still well functioning with no apparent structural damage.

Body perfusion in surgery of the aortic arch.

We propose a new cannulation and perfusion technique for aortic arch surgery, in order to achieve continuous antegrade total-body perfusion under moderate hypothermia. The heart and the aortic arch are exposed through a median sternotomy. Cardiopulmonary bypass is established from the right atrium to the right axillary artery. At 26 degrees C of body temperature, the supra-aortic vessels are clamped, the ascending aorta and the aortic arch are incised, and a cuffed endotracheal cannula, connected to an arterial line geared by a separate roller pump, is inserted into the descending thoracic aorta. Perfusion is started in the distal body, while the brain is perfused through the right axillary artery. Once the aortic arch has been replaced with a Dacron graft and the supra-aortic vessels have been reimplanted on the graft, the arterial line in the descending thoracic aorta is clamped and removed. The supra-aortic vessel clamps are removed, the proximal part of the Dacron graft is clamped, and systemic cardiopulmonary bypass is resumed via the right axillary artery.From January 2002 through December 2005, this technique was used in 12 consecutive patients on an emergency basis, due to acute aortic dissection that required total arch replacement. Within the first 30 postoperative days, 1 patient (8.3%) died, and no patient had permanent neurologic deficits. This simple technique ensures a full-flow antegrade total-body perfusion during all phases of the surgical procedure, thereby eliminating ischemia-reperfusion syndrome and yielding excellent clinical results.

A Very Late Life-Threatening Complication After Percutaneous Closure of an Atrial Septal Defect.

Percutaneous closure is widely recognized as the first therapeutic option in the majority of cases of secundum atrial septal defect (ASD) because of its high effectiveness and safety. Nonetheless, with the progressive increase of implanted devices and follow-up duration, several adverse events, some of them potentially life-threatening, have been reported. We report the case of an asymptomatic aortic erosion that occurred 13 years after the procedure. The main feature of our case is the very late occurrence of a life-threatening asymptomatic complication of ASD percutaneous closure, which should prompt lifelong surveillance in this population.

Biotechnological traps for the reduction of inflammation due to cardiopulmonary bypass operations.

Cardiopulmonary bypass induces a systemic inflammatory response (SIR), characterized by the activation of cellular and humoral elements, with concomitant release of neutrophil elastase and matrix-metallo proteinases. In the present study, the protease release during extracorporeal circulation in 28 patients undergoing cardiac surgical operations was monitored using casein zymography. A peak in protease activity was found in all patients at the end of cardiopulmonary bypass. Plasma samples of patients were allowed to interact with different traps obtained by immobilizing different protease inhibitors on specific carriers. alpha1-Antitrypsin, Bovine Pancreatic Trypsin Inhibitor, Elastatinal or Leupeptin were used as inhibitors and were covalently immobilized by diazotization or by condensation. A reduction in the proteolytic activity of the plasma samples was observed after interaction with the different traps. The most efficient traps, i.e. the ones displaying greatest power to inhibit protease activity, were those obtained by immobilizing Bovine Pancreatic Trypsin Inhibitor and Leupeptin. The biocompatibility of traps was also tested. Results show that protease activity in blood can be decreased by our protease traps.

Cardiac metastases in malignant fibrous histiocytoma. A case report.

Malignant fibrous histiocytoma metastasizing to the left ventricle is an uncommon form of cardiac malignancy. This report describes a rare case of left ventricular metastases from a malignant fibrous histiocytoma of the posterior compartment of the right thigh, recurring five years after treatment with surgery, hyperthermic perfusion of the limb and radiotherapy. As the patient presented symptoms of cardiac tamponade, open heart surgery was performed through a median sternotomy; however, the tumor was not resectable and only a biopsy was performed. A partial response was obtained with standard and high-dose chemotherapy with peripheral blood progenitor cell transplantation. The response continued to improve with immunotherapy. The patient returned to normal physical activity. He died four years later due to a ventricular arrhythmia.

Cerebral perfusion issues in acute type A aortic dissection without preoperative malperfusion: how do surgical factors affect outcomes?

OBJECTIVES: Both preoperative (disease-related) and operative (management-related) variables make the assessment of the outcomes of acute type A aortic dissection (ATAAD) surgery a difficult task. Our aim was to evaluate the impact of operative factors, including arterial cannulation site, route of cerebral perfusion and surgeon's specific experience with ATAAD ('aortic surgeon'), on the early results of surgical management, with particular attention to neurological injury. METHODS: Penn classification was used to identify clinically homogeneous risk groups of ATAAD patients undergoing surgery. Between January 2007 and June 2014, 111 of 183 ATAAD patients treated with open surgery in a single centre were in Penn Class Aa (no ischaemic complications at presentation). They were divided in two groups depending on the arterial cannulation site: femoral artery (FemA; 56 patients) or right axillary artery (RAxA; 55 patients). Study outcomes included: 30-day mortality, major adverse cardiac and cerebrovascular events at 30 days, neurological complications and in particular, patterns of stroke as defined by Bamford classification. RESULTS: No significant differences in preoperative variables were observed between cannulation-site groups, except for myocardial ischaemic time (60.9 ± 30.4 min in the RAxA group vs 81.7 ± 52.3 in the FemA group, P = 0.014) and cerebral perfusion time (42.1 ± 25.5 min in the RAxA group vs 52.9 ± 32.6 in the FemA group, P = 0.048). Outcomes in terms of mortality and neurological injury did not differ except for a higher incidence of lacunar cerebral infarction (LACI) in the RAxA group (14.5 vs 3.6%, P = 0.043), mainly but not exclusively explained by a higher incidence of LACI in unilateral (17.2%) than in bilateral cerebral perfusion (6.9%) within the RAxA group. The 'non-aortic surgeon' was associated instead with 30-day mortality and composite outcome in multivariable analysis (respectively, OR 6.40, P = 0.002 and OR 4.68, P = 0.001). CONCLUSIONS: The RAxA cannulation and FemA cannulation are associated with comparable 30-day mortality following surgery for aortic dissection. However, the possible higher risk of LACI-type strokes in the RAxA group, especially when associated with unilateral brain perfusion, should be considered when RAxA cannulation is performed in ATAAD. The hypothesis that more experienced surgeons may produce better earlier outcomes warrants further investigation.

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.

Perioperative myocardial injury after adult heart transplant: determinants and prognostic value.

BACKGROUND AND AIM OF THE STUDY: Implications of Cardiac troponin (cTnI) release after cardiac transplantation are still unclear. This study disclosed risk factors and prognostic implication of cTnI early levels in a single centre cohort operated on between January 1999 and December 2010. METHODS: Data on 362 consecutive recipients (mean age: 47.8±13.7, 20.2% female, 18.2% diabetics, 22.1% with previous cardiac operations, 27.6% hospitalized, 84.9±29.4 ml/min preoperative glomerular filtration rate) were analyzed using multivariable logistic regression modeling. Target outcomes were determinants of troponin release, early graft failure (EGF), acute kidney injury (AKI) and operative death. RESULTS: Mean cTnI release measured 24 hours after transplant was 10.9±11.6 µg/L. Overall hospital mortality was 10.8%, EGF 10.5%, and AKI was 12.2%. cTnI release>10 µg/L proved an independent predictor of EGF (OR 2.2; 95% CI, 1.06-4.6) and AKI (OR 1.031; 95% CI, 1.001-1.064). EGF, in turn, proved a determinant of hospital mortality. Risk factors for cTnI>10 µg/L release were: status 2B (OR 0.35; 95% CI, 0.18-0.69, protective), duration of the ischemic period (OR 1.006; 95% CI, 1.001-1.011), previous cardiac operation (OR 2.9; 95% CI, 1.67-5.0), and left ventricular hypertrophy (OR 3.3; 95% CI, 1.9-5.6). CONCLUSIONS: Myocardial enzyme leakage clearly emerged as an epiphenomenon of more complicated clinical course. The complex interplay between surgical procedure features, graft characteristics and recipient end-organ function highlights cTnI release as a risk marker of graft failure and acute kidney injury. The search for optimal myocardial preservation is still an issue.

Postinfarction ventricular septal defect in a patient without coronary lesions.

A 46-year-old man with polycystic kidney disease was referred to our institution for ventricular septal defect complicating myocardial infarction. Cardiac catheterization disclosed normal coronary arteries and absence of myocardial bridging. None of the more frequent causes of thrombosis were present, and histopathology proved negative for acute myocarditis. The surgical procedure was successful and the 11-month follow-up uneventful.

Extensive retroperitoneal fibrosis with duodenal and ureteral obstruction associated with giant inflammatory aneurysm of the abdominal aorta.

We report a case of abdominal aortic aneurysm complicated by retroperitoneal fibrosis with both duodenal and bilateral ureteral obstruction. The patient underwent successful bilateral transurethral ureteral stenting, and then he was referred for surgical treatment of the aneurysm. Massive retroperitoneal fibrosis was found at surgery, and the mass was removed along with the diseased aorta, which was replaced by a bifurcated Dacron prosthesis; duodenolysis and ureterolysis were concomitantly performed. Ureteral stents were removed on the 8th postoperative day. Follow-up assessment at 1 year showed normalization of the urinary tract structure at echography and good hemodynamic performance of the vascular prosthesis at Doppler examination. To our knowledge, no other case of duodenal and bilateral ureteral stenosis secondary to massive retroperitoneal reactive fibrosis in association with abdominal aortic aneurysm has been reported.

Aortic valve dysfunction and dilated ascending aorta. A complex and controversial association.

Several pathogenetic mechanisms account for the association of the ascending aorta dilation with aortic valve dysfunction. Functional aortic insufficiency can derive from medial degeneration of the aortic wall and annuloaortic ectasia; leaflet structural disease can determine root dilation by increasing aortic wall stress in case of both regurgitation and stenosis; aortic valve disease and aortic aneurysm can however coexist due to two different intrinsic etiologies. In the attempt to best tailor the surgical correction of such conditions to the underlying causative mechanism, several technical options have already been developed including composite or separate aortic valve and root replacement, valve-sparing operations, and aortoplasty techniques. The criteria for surgical indication cannot leave the underlying pathogenesis out of consideration as well. The newly acquired knowledge in the basic research on this topic is expected to affect the approach to the individual patient in the future.

[Ways in which new surgical techniques have influenced the increasing use of cardiosurgery]. / In che modo le nuove tecniche chirurgiche hanno influenzato il crescente utilizzo della cardiochirurgia.

BACKGROUND: During the last decade new strategies and equipment have gained popularity in the clinical and experimental setting of cardiac surgery. Little is known about the real impact of these new approaches on the development of heart surgery. The present paper aimed to characterize, through a multicenter evaluation, the state of the art in Italy. METHODS: A survey of all active heart surgery centers in Italy (both public and private institutions) was conducted through a questionnaire. Diffusion, effectiveness and future perspective of these new surgical techniques were asked. Off-pump coronary artery surgery, myocardial revascularization in patients with low left ventricular ejection fraction, left ventricular surgical remodeling, mitral valve reconstruction, ministernotomic approach in aortic surgery and the use of laser, robots, heart port-access and endoscopy were the strategies under investigation. RESULTS: The use of laser, heart port-access and endoscopy were considered to have a poor impact due to proved ineffectiveness and/or technical complexity. Indications and long-term outcomes of off-pump coronary artery surgery are still under active debate. The introduction of new prosthetic materials has resulted in the widespread diffusion of mitral valve reconstruction approaches with concomitant widening of the indications. Similarly there is wide consensus on myocardial revascularization in patients with low ejection fraction. Ministernotomic strategies and ventricular remodeling are slowly but progressively gaining acceptance. CONCLUSIONS: Cardiac surgery in Italy is characterized by a great effort on new research field. Traditional approaches are still preferred unless new techniques have proved effective after strict scientific evaluation.

Anatomic reconstruction in degenerative mitral valve bileaflet prolapse: long-term results.

BACKGROUND: Degenerative mitral valve (MV) bileaflet prolapse (DMVBP) is recognized as one of the most complex lesions to be treated by reconstructive surgery. In the present study, we report our long-term results with reconstructive surgery for DMVBP. METHODS: From 2000 to 2011, 140 patients with MV regurgitation due to DMVBP were treated at our institution. Mean age was 56.4±14.5 years (range 16 to 84). Of the 140 study patients, 24 (17%) were in functional class I, 48 (34%) in class II, 60 (43%) in III, and 8 (6%) in class IV of the New York Heart Association. The MV leaflets were reconstructed without using prosthetic material, maintaining the normal shape and dimension of the valve ring and inter-papillary distance. The standard surgical procedure included the excision of the most elongated or ruptured chordae area of the posterior leaflet, with subsequent transposition of second-order chordae from the posterior leaflet to the most elongated or ruptured chordae area of the anterior leaflet. This procedure was performed in 123 patients. A para-commissural edge-to-edge was performed in 16 patients and a triangular resection of the anterior MV leaflet in 1 patient. The posterior leaflet was reconstructed with different techniques: a longitudinal suture of the annulus and residual scallops in 86 patients, a Z-plasty suture in 51 and a sliding suture of the residual posterior scallops in 3 patients. A posterior trygon-to-trygon annuloplasty was performed with an autologous pericardium strip in all patients. During follow-up, serial echocardiograms were obtained once a year. RESULTS: There were no hospital deaths. Mean follow-up was 6.42±3.1 years. Of the 140 study patients, 7 developed severe (3+), 10 moderate (2+) and 18 mild (1+) MV regurgitation. Two patients were reoperated within 6 months; 1 after 8 years and 1 after 10 years for recurrence of severe MV regurgitation. At 12 years after the initial surgical procedure, overall survival was 95.8%, freedom from MV re-intervention was 91.8% and freedom from late recurrence of 2+ or greater MV regurgitation was 78.7%. All patients had a satisfactory residual MV area, leaflets motion, and inter-papillary muscle distance. No patient developed left ventricular outflow tract obstruction. CONCLUSIONS: Our results confirm the effectiveness of anatomic reconstruction in the DMVBP. Survival rate after mitral valve repair is identical to that of the general population when surgery is performed in asymptomatic or pauci-symptomatic patients; the identification of this kind of patient is required followed by a therapeutic strategy for early surgery. This management approach in patients with DMVBP leads to optimal long-term results of MV repair.

Towards an individualized approach to bicuspid aortopathy: different valve types have unique determinants of aortic dilatation.

OBJECTIVES: Bicuspid aortic valve (BAV)-related aortopathy is increasingly recognized to be a heterogeneous disease entity, although the surgical approach, from indications to techniques, is still standard rather than individualized. We aimed to define the determinants of aortic dilatation in BAV patients stratified according to the valve morphotype. METHODS: A consecutive echocardiographic series of 622 BAV patients was analysed. Among demographic (age, sex), anthropometric (height, weight, body surface area, body mass index), clinical (associated diseases) and echocardiographic variables (valve function, ventricular parameters), the determinants of aortic root and ascending tract diameter were assessed by multivariate regression models, as well as the predictors of aortic dilatation (size index >2.1 cm/m(2)) both in the overall population and separately in groups of different valve morphotypes (RL, right-left fusion; RN, right-non-coronary fusion). RESULTS: Independent determinants of aortic root diameter (at sinuses) were age (P < 0.001), significant aortic regurgitation (P < 0.001), sex (female protective, P < 0.001) and valve morphotype (RN protective, P < 0.001). Independent determinants of ascending aortic diameter (tubular tract) were age (P < 0.001), RN morphotype (P < 0.001), body mass index (P = 0.005) and chronic obstructive pulmonary disease (P < 0.001). In univariate analysis, the RL morphotype was associated with dilatation (ASI > 2.1 cm/m(2)) at sinuses in 41% cases vs 22% for RN (P < 0.001), and the RN morphotype was associated with dilatation at the tubular tract in 68 vs 56% for RL (P = 0.007). The presence of root dilatation was predicted by age and absence of significant stenosis in the RL morphotype subgroup, and by severe regurgitation in the RN subgroup. In the RL-type subgroup, non-regurgitant aortic valve and chronic lung disease predicted dilatation at the ascending level; and in the RN-type subgroup, age and obesity. CONCLUSIONS: The two most common BAV morphotypes are associated with aortic dilatation at two different tracts (RL at the root; RN at the tubular ascending tract) independently of valve function. Moreover, the determinants of aortic dilatation were at least in part different between the two morphotypes: this may provide stratification criteria for individualized methods of follow-up and treatment.

The ascending aorta with bicuspid aortic valve: a phenotypic classification with potential prognostic significance.

OBJECTIVES: Different methods to classify the anatomical configurations of the aorta with bicuspid aortic valve (BAV) have been proposed. We aimed to test them in terms of descriptive power (i.e. capability to identify different clusters of patients with unique associations of anatomo-clinical features) and possible prognostic significance. METHODS: A consecutive echocardiographic series of 696 BAV patients (mean age 48 ± 16 years, male:female ratio 3:1) was analysed. Three possible schemes for classification of the patterns of aortic dimensions were compared. One defined the aortic shape as 'N' (ascending < sinuses > sinotubular junction (STJ)), 'A' (ascending > sinuses > STJ) or 'E' (sinotubular ≥ sinuses), the second as 'non-dilated', 'ascending phenotype' (dilated, with ascending > sinuses) or 'root phenotype' (dilated, with sinuses > ascending) and the third as normal, 'type I' (dilated only at the ascending tract), 'type II' (dilated at both ascending and sinus levels) or 'type III' (dilated only at the sinuses). We evaluated the correlation with valve morphotypes (right-left fusion, right-non-coronary fusion) and patient characteristics. In a smaller longitudinal study (n = 150), the occurrence of fast growth of the aorta (fifth quintile: ≥1 mm/year) during follow-up (5 ± 3 years) in the various phenotypes was assessed. RESULTS: The three classification methods proved meaningful in terms of association with valve morphotypes: significant associations were found between right-left-coronary BAV and the root phenotype (P < 0.001) and between the right-non-coronary BAV and the shapes A and E (P<0.001) as well as type I aortic configuration (P < 0.001). The aortic shape showed significant association with five of the other tested clinical variables, the phenotype and the type of dilatation with eight. In the longitudinal study, the root phenotype showed the most significant association with fast growth (>1 mm/year) of the ascending diameter (50% root phenotype patients; P = 0.005). The association with the N type was weaker (P = 0.055); no association was found with types from the other classification scheme (P = 0.42). CONCLUSIONS: When tested on a large population, three previously suggested phenotypic classifications of the BAV aorta proved to categorize patients into significantly different clusters, but only the classification system distinguishing between ascending phenotype and root phenotype showed a potential prognostic value. Phenotypic class of the aorta could be a factor to integrate in future comprehensive models for risk stratification of BAV aortopathy.