Long-term outcome of bicuspid aortic valve disease.

AIMS: Bicuspid aortic valve (BAV) is a common congenital condition that is frequently associated with aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic aneurysms, but specific outcome data are scarce. The present study sought to assess outcomes in a large cohort of consecutive patients with BAV. METHODS AND RESULTS: A total of 581 consecutive patients (median age 29 years, 157 female) with BAV were included in the study and followed prospectively in a heart valve clinic follow-up programme. The overall survival rate after 10 years was 94.5%. During follow-up, 158 patients developed an indication for surgery. Event-free survival rates were 97%, 94%, 87%, and 73% at 1, 2, 5, and 10 years, respectively. In the multivariable analysis, event rates were independently predicted by AS [subdistribution hazard ratio (SHR) 2.3 per degree of severity], AR (SHR 1.5 per degree of severity), baseline aortic dilatation ≥ 40 mm (SHR 1.9), and age (SHR 1.3) (P < 0.001). CONCLUSION: BAV disease is associated with a high rate of cardiac events, but state-of-the-art care results in good survival with low rates of infective endocarditis, aortic dissection, and sudden death. Incremental degrees of AS and regurgitation, the presence of aortic dilatation, and age are predictive of cardiac events.

Early Right Heart Chambers Reverse Remodeling in Patients Operated in Adulthood for Congenital Lesions Associated with Right Heart Chambers Enlargement.

BACKGROUND: Progressive right heart chambers dilatation is frequent in the adult congenital heart disease (ACHD) population. We evaluated the immediate and mid-term response of right heart chambers to surgery performed in adulthood for lesions associated with right heart chambers enlargement. METHODS: Thirty-six adult patients with lesions associated with right heart chambers enlargement submitted to surgery were studied. We collected echocardiographic data of right ventricle (RV) mid-diameter, right atrial volume indexed, RV systolic pressure, and tricuspid annular plane systolic excursion (TAPSE) prior to surgery (T0), at 2 to 5 days (T1), and 3 to 6 months (T2) after surgery. RESULTS: At T1, we observed a significant decrease of RV mid-diameter (47.2 ± 8.4 vs. 39.6 ± 7.4 mm, P < .001), right atrial volume indexed (45.6 ± 26.6 vs. 27.2 ± 11 ml/m2, P < .001), and RV systolic pressure (39 ± 14.8 vs. 32.8 ± 11.3 mm Hg, P = .03). At T2, a further significant deviation in the rate of RV diameter (39.6 ± 7.4 vs. 34.5 ± 5.1 mm, P < .001), in RV systolic pressure (32.8 ± 11.3 vs. 25.3 ± 5 mm Hg, P = .03) and TAPSE (13.9 ± 3.2 vs. 15.8 ± 2.6 mm, P < .001) was observed. CONCLUSIONS: Positive right heart chambers remodeling occurs as early as in the immediate post-operative period in most ACHD patients operated for lesions associated with right heart chambers enlargement.

Unexpected diagnosis following screening breast ultrasound.

Any instrumental examination may lead to unexpected diagnosis that in turn can radically change the clinical pathway of a patient.

Psychological Effects of Skin Incision Size in Minimally Invasive Valve Surgery Patients.

OBJECTIVE: Clinical benefits of minimally invasive cardiac valve surgery (MIVS) have been reported. Improved postoperative mental status was never analyzed with dedicated psychological tests. In the present study we intend to investigate potential benefits of MIVS for patient psychological well-being, with special attention to the relevance of the patient perception of the chest surgical scar, of the self body image and cosmetic aspects. METHODS: Between 2016 and 2017, 87 eligible patients, age 66.5 ± 14.5 years, operated on for heart valve surgery, underwent either conventional full sternotomy (CS; n = 48) or MIVS by V-shape hemi-sternotomy approach (n = 39). Before selection of the surgical approach, patients had undergone preoperative evaluation of their psychological status using Beck Depression Inventory-II (BDI-II), State-Trait Anxiety Inventory Form Y (STAI-Y), and EuroQol-5D (EQ-5D) psychological tests. Six months postoperatively, patients filled in dedicated questionnaires to assess their psychological status, quality of life, and subjective perception, thus repeating the above-mentioned tests and adding the Body Image Questionnaire (BIQ) and Patient and Observer Scar Assessment Scale (POSAS) v2.0 tests for scar-healing process evaluation. RESULTS: No patient died during the study.The 4 post-test scales of psychological well-being (BDI-II P = 0.04, STAI-Y P = 0.04, 2 indices of EQ-5D P = 0.03, P = 0.01) showed significant differences between the MIVS group and CS group, with MIVS-small incision patients having lower level of depression and anxiety symptoms and better quality of life. Mean score differences of scar perception (BIQ and POSAS v2.0) were significant, with MIVS patients having evaluated the scar quality significantly better than CS patients. CONCLUSIONS: MIVS appears associated with significant esthetical and related psychological benefits, as documented by technical tests. These findings should be considered when selecting the most appropriate technique for heart valve surgery.

Left Ventricular Noncompaction: Cause or Consequence of Myocardial Disease? A Case Report and Literature Review.

A 57-year-old woman presented to the Emergency Department with symptoms of worsening heart failure (HF). She had a past medical history of breast cancer treated with surgery and chemotherapy with anthracyclines and no family history of cardiomyopathy (CMP). In the last year, she received a diagnosis of HF with normal coronary arteries, during hospitalization for acute onset of dyspnea and was treated with medical therapy. After several months, few days before admission to our hospital, an echocardiography (ECHO) showed features of left ventricular noncompaction (LVNC), not described in previous ECHO and further confirmed by cardiac magnetic resonance. This case highlights the current uncertainties regarding the pathogenesis of LVNC and the clinical challenge of cardiologists facing LVNC morphology to decide if they are observing a genetic CMP, a phenotype overlapping with dilated or hypertrophic CMP, or a variant of the left ventricular (LV) wall anatomy. No consensus exists among scientific communities regarding diagnostic criteria of LVNC and in most cases; the key element in the diagnostic decision is not the LVNC by itself, but the associated LV dilation and/or dysfunction, hypertrophy, arrhythmias, and embolic events.

Total Surgical Plication of Left Ventricular Aneurysm Using the BioVentrix Revivent Myocardial Anchoring System.

Surgical ventricular reconstruction (SVR) is the therapy of choice for patients with left ventricular dilatation, apical and anterolateral transmural scar, and low ejection fraction. STICH trial did not show that SVR led to improved survival but several observational studies did. However, because of the considerable operative risk, open heart surgery is considered risky in debilitated patients and clinical results are controversial. Alternative less invasive strategies for left ventricular aneurysm repair have been proposed. We present a case of a left ventricular aneurysm repair using the less invasive ventricular enhancement technique (LIVE) with the Revivent TC system (BioVentrix Inc., San Ramon, CA) in a totally surgical approach, instead of a hybrid interventional-surgical one, as previously described.

The "Heart Valve Clinic" Pathway for the Management of Frail Patients With Valvular Heart Disease: From "One for All" to "All for One".

Valvular heart disease (VHD) is frequently diagnosed in old patients with clinical evidence of heart failure. This elderly population typically presents a high prevalence of frailty and comorbidities, which are associated with increased operative risk for surgical and percutaneous procedures. Recently, the Euro Heart Survey reported a clear gap between treatment guidelines and their application in the "real world". A more realistic approach to the treatment of older VHD patients treatment, mostly if associated with heart failure, is advocated. A multidisciplinary approach, as obtained with the Heart Valve Clinic methodology (intended to put the patient in the "center" of the scene and the specialists "around him"), has been applied in a group of 79 patients, aged >70 years, with symptomatic VHD, divided in 2 groups according to their frailty status (58 robust and 21 frail). No in-hospital mortality and no difference in late mortality and complications were observed. Infections were more frequent (14.3 vs. 1.7 %; P = 0.02) in frail patients. In patients with postoperative complications, serum levels of interleukin 6 (67.6 vs. 49.6; P = 0.01) and of CAF (C-terminal agrin fragment) as sarcopenia marker (67.9 vs. 62.0; P = 0.04) were higher than that in uncomplicated patients. This study was designed to determine the outcomes of the multidimensional geriatric assessment in the management of older patients with heart failure eligible for heart valve surgery. Geriatric assessment and measurement of inflammatory and sarcopenia markers may represent valid tools for a more realistic evaluation of elderly patients with VHD.

Percutaneous Valve-in-Valve Treatment of a (Very Old and Fluoroscopy Invisible) Degenerated Tricuspid Prosthesis Through the Right Jugular Vein Approach.

Tricuspid valve dysfunction adversely affects prognosis and may cause severe symptoms. Among the different opportunity offered by transcatheter techniques, the valve in valve represents an emerging strategy to treat patients with degenerated surgical biological prosthesis. We describe a case report of a percutaneous valve in valve treatment of a very old and fluoroscopy invisible tricuspid degenerated bioprosthesis. In the reported case, pivotal issue for percutaneous valve in valve procedure success was the achievement of perfect alignment between transcatheter valve and degenerated bioprosthesis despite the horizontal right chamber axis and the poor valve visibility. Of note, the combination of jugular vein approach, transapical delivery system rotation, right ventricle guidewire placement, and right atrium angiography made the valve in valve procedure safely.

Aortic Valve Replacement in Elderly Patients With Small Aortic Annulus: Results With Three Different Bioprostheses.

OBJECTIVES: Aortic valve replacement (AVR) in patients with small aortic annulus (diameter ≤21 mm) is considered a challenging scenario because of technical aspects and the high risk of patient-prosthesis mismatch (PPM). The choice of the appropriate prosthesis is crucial, and at the moment, an ideal device has yet to be identified. We compare clinical and hemodynamic results after AVR with three bioprostheses with different design and characteristics. METHODS: We retrospectively evaluated 76 consecutive patients from two cardiac surgery centers who underwent AVR (Trifecta = 24; Edwards INTUITY Elite valve system = 26, and Perceval = 26) for severe aortic stenosis between 2013 and 2017. Patients selected were older than 75 years and with an annulus diameter ≤21 mm at preoperative echocardiogram. Reinterventions and combined procedures were excluded. Minimally invasive AVR was performed in 44 (57.8%) patients. Telephonic interview was obtained at 2.9 ± 0.5 years and echocardiographic follow-up at 2.2 ± 0.8 years. RESULTS: Clinical outcome was similar in the three groups. At follow-up, Trifecta patients presented significantly higher peak and mean transprosthetic pressure gradients ( P = 0.04 and 0.01). Effective orifice area and left ventricular mass regression were comparable, although an advantage was observed in Perceval patients without reaching the statistical significance. Incidence of moderate ( P = 0.2) and severe PPM ( P = 0.7) was comparable. CONCLUSIONS: Despite higher postoperative pressure gradients observed with the Trifecta valve, all three prostheses (Trifecta, Edwards INTUITY Elite, and Perceval) have proven to be reliable when implanted in small aortic annuli, with good clinical outcome and favorable left ventricular mass regression.

Treatment of cardiac synovial sarcoma: experience of two cases.

BACKGROUND: Primary heart sarcomas are exceedingly rare tumors. Among primary cardiac sarcomas, synovial sarcoma is one of the rarest, involving cardiac cavities or pericardium. CASE PRESENTATION: Two cases of synovial sarcoma are presented with the clinical course and therapy. Both cases were treated with surgery and chemo/radiotherapy. Interestingly, one of the patient, a 52-year-old male with an intracardiac synovial sarcoma, undergone a SynCardia total artificial heart implantation, but died for multiple pulmonary metastases waiting for transplantation. CONCLUSION: Complete surgical resection of cardiac synovial sarcoma is the gold standard of therapy, though rarely possible. Although guidelines for the treatment are not well established, due to limited number of cases reported, chemotherapy and radiotherapy are frequently administered and seem to prolong mean patient's survival. Cardiac transplantation could be considered in selected cases.

Acute heart failure related to a large left atrial myxoma.

An association between atrial myxoma and left ventricular failure is rarely described, is not completely understood, and may have multiple etiologies. We present a 49-year-old man with no history of cardiovascular disease who was admitted to our hospital with pulmonary edema. He was in atrial fibrillation with rapid ventricular response. Echocardiography showed a 10.5-cm left atrial myxoma, which had been asymptomatic until the onset of congestive heart failure in the presence of severe left ventricular systolic dysfunction. Left ventricular inflow obstruction associated with the giant atrial mass could not be the only cause for acute heart failure.

Dynamic right ventricular outflow obstruction: A rare cause of hypotension during anestesia induction.

INTRODUCTION: Dynamic obstruction of right ventricle outflow tract (RVOTO) is a rare condition that may acutely cause severe heart failure. It has been reported in some hypertrophic cardiomyopathies, after lung transplantation, and in some cases of hemodynamic instability after cardiopulmonary bypass. PRESENTATION OF CASE: We report the case of a 71-year-old man who developed severe hypotension during the induction of general anesthesia for surgical coronary revascularization. Hypotension did not respond to the initial treatment with vasoconstrictors and fluids. RVOTO was suspected during pulmonary artery catheterization because of the difficulty of the catheter tip to move from the right ventricle to the pulmonary artery and, successively, because of the finding of a large gradient between the systolic pressure in the right ventricle and in the pulmonary artery. The diagnosis was confirmed by transesophageal echocardiogram (TEE). Hemodynamics recovered after the infusion of cristalloids, 1L, and the suspension of vasoconstrictors and inotropes. DISCUSSION: This is the first case in which RVOTO was observed during the induction of general anesthesia. Although this is a rare condition, the diagnostic suspect is of outmost importance because treatment is mainly based on fluid administration, and drugs with positive inotropic properties (like most vasoconstrictors) are contraindicated. CONCLUSIONS: RVOTO is an unusual, but possible cause of severe arterial hypotension during general anesthesia induction. TEE is useful for the evaluation of severely hypotensive patients who do not respond to routine treatment with fluids and vasoconstrictors.

Angina in left main coronary artery occlusion by pulmonary artery aneurysm.

A 51-year-old woman with exercise angina and a history of pulmonary artery hypertension related to a previous pulmonary thromboembolism, was referred to our hospital. Computed tomography and coronary angiography showed a 95-mm aneurysm of the main pulmonary artery, which totally occluded the left main coronary artery. After a multidisciplinary evaluation, we recommended heart-lung transplantation, but the patient refused any kind of surgical procedure. Due to the chronic occlusion, stenting of the left main coronary artery was unfeasible. With no other options available, we could only start pulmonary antihypertensive therapy. At the 1-year follow-up, the patient reported relief of her angina.

Takotsubo Cardiomyopathy Following Cardiac Surgery.

BACKGROUND: Takotsubo cardiomyopathy syndrome, commonly occurring in postmenopausal women, is characterized by transient apical systolic dysfunction in absence of coronary lesions. The cardiomyopathy is often observed after intense stressful events such as a major surgical procedure. METHODS: A 72-year-old woman symptomatic for dyspnea at rest, chest pain, and peripheral edema successfully underwent surgery for noncoronary sinus aneurysm-right atrium fistula repair. Two days after surgery the patient developed takotsubo syndrome, diagnosed according to the Mayo Clinic criteria. We reviewed the literature on takotsubo cardiomyopathy as a complication of major cardiac surgery procedures. RESULTS: Takotsubo cardiomyopathy is confirmed as a possible early complication of cardiac surgery. Exaggerated sympathetic stimulation may cause massive endogenous catecholamine release. Hypoperfusion during cardiopulmonary bypass, inotropic drugs administration, and postoperative anxiety and pain are all factors generating stress, possible coronary artery spasm and transient cardiomyopathy, clinically simulating acute myocardial infarction. Several clinical features have been described such as acute mitral insufficiency, systolic anterior motion of the anterior mitral valve leaflet, left ventricular outflow tract obstruction, acute cardiac failure, and cardiogenic shock. Intraventricular thrombi and adverse cerebrovascular events may also be possible complications. Rare catastrophic events such as left ventricular free wall rupture and ventricular septal perforation have been also encountered. CONCLUSIONS: After cardiac surgery takotsubo cardiomyopathy should be suspected if clinical and instrumental criteria are met, and promptly differentiated from the more frequent acute myocardial infarction. Prognosis may be favorable if appropriate conservative medical treatment is promptly started.

Ventricular assistance devices as bridge to transplantation.

The authors herein review the rationale and indications for the use of ventricular assistance devices as a bridge to heart transplantation and discuss the current evidence on the subject. The potential effects of device implantation on posttransplant outcomes and the therapeutic strategies in acute and elective cases are revised and illustrated.

Entrapment of a floating thrombus in the right atrium by persistent Chiari's network: a barrier to massive pulmonary embolism.

Syncope may be a manifestation of pulmonary embolism. Occlusion of a pulmonary artery with cardiac output drop, sometimes resulting in cardiac arrest, is the main underlying mechanism. In this letter we describe the case of a patient in which the persistence of a long remnant of the embryonal Chiari's network could have worked as an anatomic barrier preventing massive pulmonary embolism and cardiac arrest.