Postinflammatory rupture of right coronary artery ostium complicating Behçet disease.

INTRODUCTION: Behçet disease (BD) is a chronic relapsing inflammatory disease of unknown etiology, characterized by variable clinical manifestations. METHODS: A 28-year-old woman with BD Cardiovascular and Bipolar Disorder underwent emergency surgical treatment of ascending aortic pseudoaneurysm for ruptured right coronary ostium.A modified Bentall procedure with tricuspid annuloplasty, surgical thrombectomy, and a single coronary artery bypass using a saphenous vein graft was performed. A biological prosthesis was used initially but subsequently deteriorated. We then successfully performed a valve-in-valve (ViV) transcatheter aortic valve implantation (TAVI). RESULTS: The postoperative recovery was uneventful and at follow-up, two years post valve procedure, the patient was completely symptom-free. She remains under long-term medical surveillance. CONCLUSION: The management of BD with cardiac involvement must be led by a multidisciplinary team.

Heart Transplantation, Either Alone or Combined With Liver and Kidney, a Viable Treatment Option for Selected Patients With Severe Cardiac Amyloidosis.

Heart transplantation in cardiac amyloidosis (CA) patients is possible and generally considered for transplantation if other organs are not affected. In this study, we aimed to describe and assess outcome in patients following heart transplantations at our CA referral center. Methods: We assessed all CA patients that had heart transplantations at our center between 2005 and 2018. Patients with New York Heart Association status 3 out of 4, with poor short-term prognosis due to heart failure, despite treatment, and without multiple myeloma, systemic disease, severe neuropathic/digestive comorbidities, cancer, or worsening infections were eligible for transplantation. Hearts were transplanted by bicaval technique. Standard induction and immunosuppressive therapies were used. Survival outcome of CA patients after transplantation was compared with recipients with nonamyloid pathologies in France. Results: Between 2005 and 2018, 23 CA patients had heart transplants: 17 (74%) had light chain (light chain amyloidosis [AL]) and 6 (26%) had hereditary transthyretin (hereditary transthyretin amyloidosis [ATTRv]) CA. Also, 13 (57%) were male, and the mean age at diagnosis was 56.5 y (range, 47.7-62.8). Among AL patients, 13 had heart-only and 5 had heart-kidney transplantations. Among ATTRv patients, 1 had heart-only and 5 had heart-liver transplantations. The 1-y survival rate after transplantation was 78%, 70% with AL, and 100% with ATTRv. At 2 y, 74% were alive: 65% with AL and 100% with ATTRv. Conclusion: After heart transplantation, French CA and nonamyloid patients have similar survival outcomes. Among CA patients, ATTRv patients have better prognosis than those with AL, possibly due to the combined heart-liver transplantation. Selected CA patients should be considered for heart transplantations.

Aortic valve repair versus mechanical valve replacement for root aneurysm: the CAVIAAR multicentric study.

OBJECTIVES: Despite growing evidence that aortic valve repair improves long-term patient outcomes and quality of life, aortic valves are mostly replaced. We evaluate the effect of aortic valve repair versus replacement in patients with dystrophic aortic root aneurysm up to 4 years. METHODS: The multicentric CAVIAAR (Conservation Aortique Valvulaire dans les Insuffisances Aortiques et les Anévrismes de la Racine aortique) prospective cohort study enrolled 261 patients: 130 underwent standardized aortic valve repair (REPAIR) consisting of remodelling root repair with expansible aortic ring annuloplasty, and 131 received mechanical composite valve and graft replacement (REPLACE). Primary outcome was a composite criterion of mortality, reoperation, thromboembolic or major bleeding events, endocarditis or operating site infections, pacemaker implantation and heart failure, analysed with propensity score-weighted Cox model analysis. Secondary outcomes included major adverse valve-related events and components of primary outcome. RESULTS: The mean age was 56.1 years, and valve was bicuspid in 115 patients (44.7%). Up to 4 years, REPAIR did not significantly differ from REPLACE in terms of primary outcome [Hazard Ratio (HR) 0.66 (0.39; 1.12)] but showed significantly less valve-related deaths (HR 0.09 [0.02; 0.34]) and major bleeding events (HR 0.37 [0.16; 0.85]) without an increased risk of valve-related reoperation (HR 2.10 [0.64; 6.96]). When accounting for the occurrence of multiple events in a single patient, the REPAIR group had half the occurrence of major adverse valve-related events (HR 0.51 [0.31; 0.86]). CONCLUSIONS: Although the primary outcome did not significantly differ between the REPAIR and REPLACE groups, the trend is in favour of REPAIR by a significant reduction of valve-related deaths and major bleeding events. Long-term follow-up beyond 4 years is needed to confirm these findings.

Describing mode of death in three major cardiac amyloidosis subtypes to improve management and survival.

BACKGROUND: The three main cardiac amyloidosis (CA) types have different progression and prognosis. Little is known about the mode of death (MOD) which is commonly attributed to cardiovascular causes in CA. Improving MOD's knowledge could allow to adapt patient care. OBJECTIVE: This retrospective study describes the MOD that occurred during long-term follow-up in CA patients in light-chain (AL), transthyretin hereditary (ATTRv) or wild-type (ATTRwt). MATERIAL AND METHODS: Patients referred to and cared for, at the French referral centre for CA, Henri Mondor Hospital, Créteil between 2010 and 2016 were included. Clinical information surrounding patient deaths were investigated and centrally evaluated by two blinded clinical committees which classified MOD as cardiovascular, non-cardiovascular or unknown and sub-classified it depending on its subtype. RESULTS: From the 566 patients included, 187 had AL, 206 ATTRv and 173 ATTRwt. During the 864 patient-year follow-up, 160 (28%) deaths occurred, with median survival time of 17.3 months (interquartile range 5.1-35.4). The most frequent MOD was cardiovascular (64%) of which worsening heart failure occurred most frequently and for which, 69% were of AL subtype, 79% ATTRv and 76% ATTRwt. Sudden death also occurred more frequently in AL subtype accounting for 29% of AL deaths. Non-cardiovascular MOD occurred in 26% of patients overall. Among these, infection was the most common non-cardiovascular MOD in any type of CA (80%). CONCLUSIONS: Mortality is high during natural course of CA and differs between subtypes. The main MOD were worsening heart failure, sudden death and infection, opening room to optimise management.

Should We Quantify Valvular Calcifications on Cardiac CT in Patients with Infective Endocarditis?

BACKGROUND: Evaluate the impact of valvular calcifications measured on cardiac computed tomography (CCT) in patients with infective endocarditis (IE). METHODS: Seventy patients with native IE (36 aortic IE, 31 mitral IE, 3 bivalvular IE) were included and explored with CCT between January 2016 and April 2018. Mitral and aortic valvular calcium score (VCS) were measured on unenhanced calcium scoring images, and correlated with clinical, surgical data, and 1-year death rate. RESULTS: VCS of patients with mitral IE and no peripheral embolism was higher than those with peripheral embolism (868 (25-1725) vs. 6 (0-95), p < 0.05). Patients with high calcified mitral IE (mitral VCS > 100; n = 15) had a lower rate of surgery (40.0% vs.78.9%; p = 0.03) and a higher 1-year-death risk (53.3% vs. 10.5%, p = 0.04; OR = 8.5 (2.75-16.40) than patients with low mitral VCS (n = 19). Patients with aortic IE and high aortic calcifications (aortic VCS > 100; n = 18) present more frequently atypical bacteria on blood cultures (33.3% vs. 4.8%; p = 0.03) than patients with low aortic VCS (n = 21). CONCLUSION: The amount of valvular calcifications on CT was associated with embolism risk, rate of surgery and 1-year risk of death in patients with mitral IE, and germ's type in aortic IE raising the question of their systematic quantification in native IE.

Timing of aortic valve replacement in high-gradient severe aortic stenosis: impact of left ventricular ejection fraction.

BACKGROUND: Patients with high-gradient (HG) severe aortic stenosis (AS) and left ventricular (LV) dysfunction are at high risk of death. The optimal timing for aortic valve replacement (AVR) is not defined by guidelines. The objective was to define the optimal timing to perform isolated AVR in patients with HG-AS and severe LV dysfunction. METHODS: We retrospectively included 233 consecutive patients admitted for severe HG-AS (aortic valve area <1cm2 and mean gradient ≥40mmHg). Severe LV dysfunction was defined by LV ejection fraction ≤35% (LVEF). All-cause mortality while waiting for AVR and after the intervention (30 days) was compared in patients with (n = 28) and without (n = 205) LVEF ≤35%. RESULTS: Patients with HG-AS and severe LV dysfunction had a higher risk profile than those with LVEF >35%. AVR was performed in 93% (218/233) of patients, 41% by surgery (SAVR) and 53% by transcatheter (TAVR). TAVR was the preferred method to treat HG-AS patients with LVEF ≤35%. All-cause mortality while waiting for AVR was higher in patients with severe LV dysfunction (22% vs. 2.0%, p < 0.001) and occurred within a shorter time (12 [8-26] days vs. 63 [58-152] days, p = 0.010) compared to those with LVEF >35%. All death in HG-AS patients with a severe LV dysfunction occurred within the first month. Postoperative mortality was low (1.3%), irrespective of LVEF. CONCLUSIONS: AVR should be performed promptly after Heart Team decision in patients with HG severe AS and LVEF ≤35% because of a very high and premature risk of death while waiting for intervention.

Valvular surgery in donor hearts before orthotopic heart transplantation.

BACKGROUND: Donor heart shortage has extended the waiting time and increased the mortality of patients on the transplant waiting list. Widening old standard donor criteria has successfully increased the number of heart transplantations, but for many years, a valve disease in a donor heart has been considered a primary contraindication for organ donation. AIMS: To analyse the results of aortic and mitral valvular surgery in marginal donor hearts with valvulopathy before orthotopic heart transplantation. METHODS: Between January 2012 and November 2015, we performed 53 heart transplantations in our department. In four donors, echocardiography performed at the time of organ procurement showed a valvular disease: three had moderate-to-severe mitral regurgitation; and one had moderately severe aortic valve stenosis. RESULTS: The mean bench mitral repair and aortic replacement time, aortic cross-clamp time and total ischaemic time were: 18 (range 7-25) minutes, 78.7 (range 57-98) minutes and 184 (range 89-255) minutes, respectively. Intraoperative transoesophageal echocardiography showed good mitral repair or aortic prosthetic valve function, and good right and left ventricular function. One patient died of infectious pneumonia after 1 month. The mean duration of follow-up for the patients discharged home was 75±13 months, and all have returned to an active unrestricted lifestyle. CONCLUSIONS: Our limited series demonstrates that conventional valvular procedures performed on otherwise healthy donor hearts with mitral and aortic valve pathology can efficaciously expand the donor pool for orthotopic cardiac transplantation and decrease the mortality rate on the waiting list.

Floating thrombus in the ascending aorta revealed by peripheral arterial embolism.

A floating thrombus in the ascending aorta is rarely found in clinical practice and is an uncommon cause of peripheral arterial embolization. When there is minimal atherosclerosis or a normal aorta, the management of such a lesion is poorly defined. Currently, there is no clear consensus concerning optimal treatment. Herein, we report 2 cases of ascending aortic thrombus that are complicated by a peripheral embolic event. Due to the risk of recurrent systemic embolism, particularly with strokes, surgical thrombectomy with ascending aortic wall replacements was performed. We believe that floating ascending aorta thrombus represents a serious source of systemic embolism and stroke. Surgical removal is easy to perform with good clinical outcomes. Conservative treatments such as anticoagulation or thromboaspiration may be considered in high-risk or inoperable patients.

The convergent procedure: a hybrid approach for long lasting persistent atrial fibrillation ablation.

BACKGROUND: Atrial fibrillation (AF) is associated with increased risk of stroke, heart failure and all-cause mortality. The Cox-Maze procedure is the most effective approach to ablate persistent AF but presents a significant morbidity and mortality. Additionally, the classical endocardial ablation approach has limited efficacy to treat long lasting persistent AF. We described a new, minimally invasive hybrid approach, combining an endocardial and epicardial ablation named convergent procedure to treat long lasting persistent AF patients. METHODS: We studied 55 consecutive patients with long lasting persistent AF who underwent the convergent procedure in 2 French centers between 2010 and 2015. All patients had at least one previous failed endocardial ablation and were highly symptomatic. Patients with a history of thoracic surgery were excluded. A 24 hour-Holter ECG was performed systematically at 3, 6 and 12 months after the convergent procedure. All patients reached 1-year follow-up. RESULTS: No death, stroke, phrenic nerve palsy or tamponade occurred immediately after the procedure. Post-surgery average length of stay was 8±4 days. Later, 3 patients (5%) developed diaphragmatic hernia resulting in a modified surgery technique. At 12 months, 76% of patients were in sinus rhythm after an average of 1.43 ablation procedure. Finally, 91% of patients were maintained on antiarrhythmic drugs. CONCLUSIONS: Thoracoscopic hybrid epicardial-endocardial ablation technique proved to be effective and safe to treat long lasting persistent AF patients with previous failed endocardial AF ablation.

Causes and consequences of cardiac fibrosis in patients referred for surgical aortic valve replacement.

AIMS: Cardiac fibrosis is associated with left ventricular (LV) remodelling and contractile dysfunction in aortic stenosis (AS). The fibrotic process in this condition is still unclear. The aim of this study was to determine the role of both local and systemic inflammation as underlying mechanisms of LV fibrosis and contractile dysfunction. The diagnostic values of 2D-strain echocardiography and serum biomarkers in the evaluation of cardiac fibrosis in this condition were assessed through correlation analyses. METHODS AND RESULTS: Patients with AS referred for surgical valve replacement were prospectively and consecutively included. They all had a comprehensive echocardiography including 2D strain. Blood samples were collected to measure cytokines and inflammatory biomarkers using Luminex bead-based assays. A per-surgical myocardial biopsy of the basal antero-septal segment (S1) was performed. Serial sections of each biopsy were stained with Sirius red. Digital image analysis was used to quantify fibrosis. Immunostainings using specific antibodies against macrophage, glycoprotein (gp) 130, and interleukin 6 (IL-6) were also performed. Patients were divided into tertiles reflecting the severity of fibrosis: mild, moderate, and severe load (TF1 to TF3). The mean age of the 58 included patients was 73 ± 11 years. Twenty-four (43%) were in New York Heart Association III-IV. Mean aortic valve area was 0.8 ± 0.2 cm2 . Mean aortic stenosis peak velocity and mean gradient were respectively 4.5 ± 0.8 m/s and 54 ± 15 mmHg. The mean LV ejection fraction was 54 ± 12%, and the global LV longitudinal strain was -15 ± 4%. The mean S1 strain, corresponding to the biopsied region, was -10 ± 6% and was strongly correlated to fibrosis load (R = 0.83, P < 0.0001). TF3 was associated with higher mortality (P = 0.009), higher serum C-reactive protein and IL-6, and lower gp130 compared with the other tertiles (P < 0.05). IL-6 and gp130 were expressed in the heart and respectively in the plasma membrane of macrophages and in the cytoplasm of both macrophages and cardiomyocytes. During follow-up, three patients died and were all in the third fibrosis tertile. CONCLUSIONS: We found a positive correlation between elevated inflammatory markers and degree of fibrosis load. These two parameters were associated with worse outcomes in patients with severe AS. Our results may be of interest especially in patients for whom a transcatheter aortic valve implantation is indicated and myocardial biopsy is not possible. Strategies aiming at preventing inflammation might be considered to decrease or limit the progression of cardiac fibrosis in patients followed for AS.

Successful percutaneous treatment of an anomalous left circumflex coronary artery occlusion after tricuspid valve repair.

The risk of coronary artery injury during valve surgery is higher in case of anomalous coronary artery origin and trajectory. Unlike previous reports on coronary obstruction related to aortic or mitral surgery, we report the first case, to the best of our knowledge, of an anomalous left circumflex artery occlusion after tricuspid valve repair, and its successful percutaneous treatment.

A New Three-Dimensional Echocardiography Method to Quantify Aortic Valve Calcification.

BACKGROUND: Aortic valve calcification (AVC) quantification is computed from multidetector computed tomography (MDCT). The aim of this study was to test the hypothesis that three-dimensional (3D) transthoracic echocardiography can be used to provide a bedside method to assess AVC. METHODS: The study included 94 patients (mean age, 78 ± 12 years; mean aortic valve [AV] area, 1.0 ± 0.6 cm2) referred for MDCT and echocardiography for AV assessment. Apical 3D full-volume data sets focused on the AV region were acquired during transthoracic echocardiography, and a region-growing algorithm was applied offline to compute 3D transthoracic echocardiographic AVC (AVC-3DEcho). AVC-3DEcho was compared with AVC by MDCT and with calcium weight in the subgroup of patients referred for surgery, with explanted AVs analyzed by a pathologist (n = 22). RESULTS: In the explanted valve group, AVC-3DEcho score exhibited fair correlations with MDCT score (r = 0.85, P < .001), calcium load (r = 0.81, P < .001), and peak AV velocity (r = 0.64, P < .001). In the overall population, AVC-3DEcho score correlated modestly with MDCT score (r = 0.61, P < .001) but had similar accuracy to identify severe aortic stenosis (area under the curve = 0.94). AVC-3DEcho > 1,054 mm3 identified severe aortic stenosis with specificity of 100% and sensitivity of 76%. In addition, AVC-3DEcho was associated with the presence of significant paravalvular regurgitation after transcatheter aortic valve implantation. Finally, intraobserver and interobserver variability for AVC-3DEcho score was 4.2% and 8.9%, respectively. CONCLUSIONS: AVC-3DEcho correlated with calcium weight obtained from pathologic analysis and MDCT. These data suggest that a bedside method for quantifying AV calcification with ultrasound is feasible.

Spontaneous mechanical rupture of a papillary muscle effectively resolved by mitral valve repair.

We report a case of spontaneous mechanical papillary muscle rupture. In theory, the papillary muscle works as a shock absorber that compensates for geometric changes of left ventricular wall. We believe that the aetiology of papillary muscle rupture, in this case, is linked to the physical and mechanical strains exerted on the papillary. We performed a mitral valve repair with excellent short- and long-term results.

Pulmonary Artery Dissection: A Case Treated by Homograft Replacement.

Pulmonary artery dissection was diagnosed in a 32-year-old man who was admitted to the emergency department with intense chest pain. He had a history of pulmonary balloon valvuloplasty for congenital pulmonary stenosis at the age of 7 and no pulmonary hypertension. The operation was performed with cardiopulmonary bypass. The dissected pulmonary arterial trunk was removed with the distorted valve, and replaced with a pulmonary artery homograft. The postoperative course was uneventful. Histologic examination revealed medionecrosis. Pulmonary artery dissection is a rare but highly lethal pathology. In the absence of pulmonary hypertension, surgical treatment with homograft replacement can lead to excellent results.

Prognostic value of right ventricular systolic function in cardiac amyloidosis.

BACKGROUND: Right ventricular (RV) dysfunction is a strong predictor of poor outcomes in heart failure. Its prognostic meaning in cardiac amyloidosis (CA) is under-investigated. METHODS: Hundred and twenty nine patients with suspected CA and an interventricular septum thickness (IVST) ≥ 12 mm underwent echocardiography with measurement of left ventricular (LV) and RV longitudinal strain (LS), late gadolinium-enhancement (LGE) cardiac MRI, and standard evaluation. RESULTS: Among 82 confirmed CA, types were immunoglobulin light chain (AL, n = 26), hereditary transthyretin (m-TTR, n = 37) and senile (WT-TTR, n = 19). Compared to those without, CA patients had significantly lower RV fractional shortening (RV-FS), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler systolic velocity, and global RV-LS, without any difference among the CA types. RV-LGE, observed in 62% of CA patients, was associated with lower global and basal RV-FS. Median follow-up was 8(2; 16) months. Using multivariate analysis, NYHA-class and low TAPSE independently predicted major adverse cardiac event (MACE) defined as death, heart transplantation and acute heart failure. Independent determinants of TAPSE < 14 mm, the best cut-off value, were LV ejection fraction (LVEF), estimated filling pressure (E/E'), NT-proBNP and pulmonary artery pressure, but not RV-LGE. CONCLUSIONS: RV dysfunction is common in CA. Its routine evaluation by a simple TAPSE may be an aid in assessing the prognosis of CA patients.

Risk factors for valve-related complications after mechanical heart valve replacement in 505 patients with long-term follow up.

BACKGROUND AND AIM OF THE STUDY: Currently, valve thrombosis, thromboembolic events and bleeding events account for 75% of all complications that occur after mechanical heart valve replacement. The study aim was to determine the main risk factors for valve-related complications in patients undergoing mechanical heart valve replacement. METHODS: Data were available from the systematic follow up of patients who had received a CarboMedics bileaflet mechanical heart valve replacement at the authors' institution. Follow up examinations were conducted prospectively at two-year intervals, via questionnaires sent to the patients' general practitioners, or by telephone calls. RESULTS: Between January 1988 and December 2005, a total of 505 consecutive patients (300 males, 205 females; mean age 52 years; range: 5 to 77 years) underwent heart valve replacement using a CarboMedics mechanical prosthesis. Aortic valve replacement (AVR) was performed in 308 patients, mitral valve replacement (MVR) in 134 patients, and double-valve replacement (DVR) in 62 patients. The follow up was 95.4% complete; the mean follow up was 7.5 years, and the total follow up 3,718 patient-years. Thromboembolic and bleeding complications represented the leading cause of valve-related events (104/195), of valve-related mortality (15/25), and of the need for repeat surgery (9/16). Valve thrombosis occurred in 12 patients. Implantation in the mitral position was identified as a risk factor (HR = 15.07; CI: 8.41-23.07; p 0.0001). Thromboembolism occurred in 32 patients; the use of antiplatelet agents was found to be a protective factor (HR = 0.23; CI: 0.08-0.70; p = 0.01). Bleeding events occurred in 52 patients; risk factors for bleeding events included a history of thromboembolic or bleeding complications (HR = 2.70; CI: 1.33-5.26; p = 0.006) and an unstable International Normalized Ratio (INR) (HR = 2.86; CI: 1.01-8.08; p = 0.05). CONCLUSION: After mechanical heart valve replacement, the only risk factors for bleeding complications were an unstable INR and a history of thromboembolic or bleeding events. The use of antiplatelet agents proved to be a protective factor against thromboembolic events.

A new technique for interrupted aortic arch repair: the Neville tube.

We have developed a new technique for interrupted aortic arch repair in which the pulmonary artery anterior wall is cut off and tailored so as to re-establish aortic continuity with an autologous tube. We are describing this method herein, with an 8-year follow-up of the first patient.

Localized constrictive pericarditis after Gore-Tex pericardial substitution.

A 65-year-old patient presented with recurrent cardiac decompensation 12 years after aortic prosthesis replacement and expanded polytetrafluoroethylene (ePTFE) membrane pericardial substitution. Diagnosis of pericardial constriction was difficult. Only one cardiac imaging method, radionuclide ventriculography, was helpful. Upon re-operation, an epicardial fibrous strap which restricted right ventricle (RV) diastolic expansion was found between the anterior free wall and diaphragmatic portion of the RV. Clinical status dramatically improved after surgical removal of this bridle, as did ventricular filling curves in radionuclide imaging. This case shows that delayed cardiac constriction is possible after ePTFE pericardial substitution, especially if the membrane is applied to both anterior and diaphragmatic aspects of the heart.