Challenges Regarding the Value of Routine Perioperative Transesophageal Echocardiography in Mitral Valve Surgery.

BACKGROUND AND OBJECTIVES: Transesophageal echocardiography (TEE) is considered an indispensable tool for perioperative evaluation in mitral valve (MV) surgery. TEE is routinely performed by anesthesiologists competent in TEE; however, in certain situations, the expertise of a senior cardiologist specializing in TEE is required, which incurs additional costs. The purpose of this study is to determine the indications for specialized perioperative TEE based on its utility and the correlation between intraoperative TEE diagnoses and surgical findings, compared with routine TEE performed by an anesthesiologist. MATERIALS AND METHODS: We conducted a three-year prospective study involving 499 patients with MV disease undergoing cardiac surgery. Patients underwent intraoperative and early postoperative TEE and at least one other perioperative echocardiographic evaluation. A computer application was dedicated to calculating the utility of each type of specialized TEE indication depending on the type of MV disease and surgical intervention. RESULTS: The indications for performing specialized perioperative TEE identified in our study can be categorized into three groups: standard, relative, and uncertain. Standard indications for specialized intraoperative TEE included establishing the mechanism and severity of MR (mitral regurgitation), guiding MV valvuloplasty, diagnosing associated valvular lesions post MVR (mitral valve replacement), routine evaluations in triple-valve replacements, and identifying the causes of acute, intraoperative, life-threatening hemodynamic dysfunction. Early postoperative specialized TEE in the intensive care unit (ICU) is indicated for the suspicion of pericardial or pleural effusions, establishing the etiology of acute hemodynamic dysfunction, and assessing the severity of residual MR post valvuloplasty. CONCLUSIONS: Perioperative TEE in MV surgery can generally be performed by a trained anesthesiologist for standard measurements and evaluations. In certain cases, however, a specialized TEE examination by a trained senior cardiologist is necessary, as it is indirectly associated with a decrease in postoperative complications and early postoperative mortality rates, as well as an improvement in immediate and long-term prognoses. Also, for standard indications, the correlation between surgical and TEE diagnoses was superior when specialized TEE was used.

Genotype-Phenotype Correlations in Alport Syndrome-A Single-Center Experience.

BACKGROUND: Alport syndrome (AS) is a common and heterogeneous genetic kidney disease, that often leads to end-stage kidney disease (ESKD). METHODS: This is a single-center, retrospective study that included 36 adults with type IV collagen (COL4) mutations. Our main scope was to describe how genetic features influence renal survival. RESULTS: A total of 24 different mutations were identified, of which eight had not been previously described. Mutations affecting each of the type IV collagen α chains were equally prevalent (33.3%). Most of the patients had pathogenic variants (61.1%). Most patients had a family history of kidney disease (71%). The most prevalent clinical picture was nephritic syndrome (64%). One-third of the subjects had extrarenal manifestations, 41.6% of patients had ESKD at referral, and another 8.3% developed ESKD during follow-up. The median renal survival was 42 years (95% CI, 29.98-54.01). The COL4A4 group displayed better renal survival than the COL4A3 group (p = 0.027). Patients with missense variants had higher renal survival (p = 0.023). Hearing loss was associated with lower renal survival (p < 0.001). CONCLUSIONS: Patients with COL4A4 variants and those with missense mutations had significantly better renal survival, whereas those with COL4A3 variants and those with hearing loss had worse prognoses.

Hantavirus infections in the South­Eastern European countries: A study of two cases and literature review.

Hantavirus infection is a rare zoonosis in South-Eastern Europe. Depending on the serotype involved, the virus can cause hemorrhagic fever with renal syndrome which is also known as endemic nephropathy, and cardiopulmonary syndrome. Prompt diagnosis of the disease is essential for reducing the risk of severe manifestations and complications like chronic kidney disease, secondary hypertension or even death because there is no specific treatment or vaccine approved. The present study reported two cases of hemorrhagic fever with renal syndrome diagnosed in the Department of Nephrology of The Fundeni Clinical Institute (Romania). In both patients, kidney needle biopsy played a major role in establishing the diagnosis. The difficulties encountered in diagnosing this disease were also emphasized, taking into consideration the rarity of this infection in South-Eastern Europe. The key literature data on the epidemiology, pathogenesis and management of this infection were further reviewed.

Telemedicine for Optimizing Secondary Prevention in Coronary Artery Bypass Grafting Patients during COVID-19 Pandemic.

(1) Background: The COVID-19 pandemic has introduced a major disruption to the delivery of secondary prevention measures in patients with established atherosclerotic cardiovascular disease (CVD). It required a rapid and widespread adoption of new medical services, including the use of telemedicine. This study aimed to examine the impact of COVID-19 on secondary prevention in patients with coronary artery bypass grafting (CABG) and to evaluate the effectiveness of the telemedicine application for the implementation of lifestyle change measures, remote monitoring, and treatment regimen adjustment; (2) Methods: This prospective study on 194 CABG patients evaluated three consecutive years between 2019 and 2022 in the pre-pandemic period by face-to-face visits and during the pandemic by teleconsultations or hybrid follow-up. Variables of interest were compared between four periods: pre-pandemic-pre-P (1 March 2019-29 February 2020), lockdown-Lock (1 March-31 August 2020), restrictive-pandemic-Restr-P (1 September 2020-28 February 2021), and relaxed-pandemic-Rel-P (1 March 2021-1 March 2022). (3) Results: The average values of the lipidogram, blood sugar, and uric acid increased during Lock and Restr-P, but, through the use of teleprevention, they returned to the pre-pandemic level or even below this level. The exception was blood sugar, which remained high in Rel-P. The number of newly diagnosed patients with diabetes also increased, with most of them having moderate forms of COVID. During Lock and Res-P, the percentage of obese, smoking, or hypertensive patients increased, but, through the use of teleprevention, we managed to reduce it, although it remained slightly higher than the pre-pandemic level. Physical activity decreased in the first year of the pandemic, but, in Rel-P, CABG patients became more active than before the pandemic (4) Conclusions: The use of telemedicine for cardiovascular secondary prevention allowed us to not only continue seeing CABG patients but, also, to adjust their medication and to expand cardiovascular preventive counseling and testing with favorable results, especially during the second year of the pandemic.

Importance of Tissue Doppler Evaluation in Dilated Cardiomyopathy: The Value of Diastolic Filling Pattern as a Prognostic Predictor.

(1) Background: The presence of restrictive left ventricular diastolic filling pattern (LVDFP) is associated with an unfavorable prognosis in many cardiac diseases, but few data are available on the prognostic implications of this pattern in patients with dilated cardiomyopathy (DCM). We aimed to establish the main prognostic predictors at the 1- and 5-year follow-ups in DCM patients and the value of restrictive LVDFP in increasing morbidity and mortality. (2) Methods: A prospective study of 143 patients with DCM divided in non-restrictive LVDFP group (95 patients) and restrictive group (47 patients). The patients were evaluated at a 5-year follow-up through an in-patient visit during the pre-pandemic period and hybrid methods (face-to-face, teleconsultation and home monitoring with a telemedicine application) during the pandemic period. Statistical analysis compared the two groups in terms of NYHA class, quality of life, hospitalizations/emergency department (ED) visits due to HF exacerbation and total mortality. (3) Results: The mortality rate in the restrictive group was markedly higher than that in the non-restrictive group at 1 year (17.02% vs. 10.59%, respectively, p < 0.05) and at 5 years (68.08% vs. 50.53%, p < 0.05). In the restrictive group, hospitalizations/ED visits due to HF decompensations at 1 year were significantly higher (85.11% vs. 57.89%, p < 0.05), with hospitalizations for ventricular arrhythmia being almost three times higher (21.28% vs. 7.37%, respectively, p < 0.05). The percentage of patients with a favorable evolution (in terms of NYHA class and quality of life) at the 1- and 5-year follow-ups were higher in the non-restrictive LVDFP group. The main prognostic predictors in patients with DCM at the 1-year follow-up were: restrictive LVDFP, age > 75 years, markedly dilated LV, comorbidities (DM, COPD), 2nd-degree mitral regurgitation and severe pulmonary hypertension (p < 0.05). (4) Conclusions: At the 1- and 5-year follow-ups, the presence of the restrictive LVDFP in DCM patients was independently associated with a poor prognosis, being the best clinical predictor for unfavorable evolution, after adjustment for other well-established predictive parameters in DCM patients.

Challenges in Caring for People with Cardiovascular Disease through and beyond the COVID-19 Pandemic: The Advantages of Universal Access to Home Telemonitoring.

(1) Background: Cardiovascular prevention was left in second place during the COVID-19 pandemic and the use of telemedicine turned out to be very useful. We aimed to evaluate the effectiveness of a telemedicine application for remote monitoring and treatment adjustments in terms of improving cardiovascular prevention. (2) Methods: A prospective study of 3439 patients evaluated between the 1st of March 2019 and the 1st of March 2022, in the pre-pandemic period by face-to-face visits and during the pandemic by teleconsultations or hybrid follow-up. We compared four periods: pre-pandemic-Pre-P (1 March 2019-1 March 2020), lockdown-Lock (1 March-1 September 2020), restrictive-pandemic-Restr-P (1 September 2020-1 March 2021), and relaxed-pandemic-Rel-P (1 March 2021-1 March 2022). (3) Results: The average values of total cholesterol (TC), LDL cholesterol, triglycerides, uric acid, and glucose had an increasing trend during Lock and Restr-P, and they decreased close to the baseline level during the Rel-P, with the exception of glucose which remained elevated in Rel-P. The number of patients with newly discovered DM increased significantly in the Rel-P, and 79.5% of them had mild/moderate forms of COVID-19. During Lock and Res-P, the percentage of obese, smoking, or hypertensive patients increased, but probably through the use of telemedicine, we managed to reduce it, although it remained slightly higher than the pre-pandemic level. Physical activity decreased in the first year of the pandemic, but in Rel-P people became more active than before the pandemic. (4) Conclusions: The use of telemedicine for cardiovascular prevention seems to yield favorable results, especially for secondary prevention in the very high-risk group and during the second year.

Incidence and Risk Factors for Long-Term Persistence of Diastolic Dysfunction after Aortic Valve Replacement for Aortic Stenosis Compared with Aortic Regurgitation.

(1) Background: Severe left ventricular (LV) diastolic dysfunction with a restrictive diastolic pattern (LVDFP) is generally associated with a worse prognosis. Its evolution and reversibility in the short- and medium-term after aortic valve replacement (AVR) has been little-studied. We aimed to evaluate the evolution of LV remodeling and LV systolic and diastolic function after AVR in aortic stenosis (AS) patients compared to aortic regurgitation (AR). Moreover, we tried to identify the main predictive parameters for postoperative evolution (cardiovascular hospitalization or death and quality of life) and the independent predictors for the persistence of restrictive LVDFP after AVR. (2) Methods: A five-year prospective study on 397 patients undergoing AVR for AS (226 pts) or AR (171 pts), evaluated clinically and by echocardiography preoperatively and until 5 years postoperatively. (3) Results: 1. In patients with AS, early post AVR, LV dimensions decreased and diastolic filling and LV ejection fraction (LVEF) improved more rapidly compared to patients with AR. At 1 year postoperatively, persistent restrictive LVDFP was found especially in the AR group compared to the AS group (36.84% vs. 14.16%). 2. Cardiovascular event-free survival at the 5-year follow-up was lower in the AR group (64.91% vs. 87.17% in the AS group). The main independent predictors of short- and medium-term prognosis after AVR were: restrictive LVDFP, severe LV systolic dysfunction, severe pulmonary hypertension (PHT), advanced age, severe AR, and comorbidities. 3. The persistence of restrictive LVDFP after AVR was independently predicted by: preoperative AR, the E/Ea ratio > 12, the LA dimension index > 30 mm/m2, an LV endsystolic diameter (LVESD) > 55 mm, severe PHT, and associated second-degree MR (p < 0.05). (4) Conclusions: AS patients had an immediate postoperative evolution in terms of LV remodeling, and LV systolic and diastolic function were more favorable compared to those with AR. The restrictive LVDFP was reversible, especially after the AVR for AS. The main prognostic predictors were the presence of restrictive LVDFP, advanced age, preoperative AR, severe LV systolic dysfunction, and severe PHT.

Challenges for Management of Dilated Cardiomyopathy during COVID-19 Pandemic-A Telemedicine Application.

BACKGROUND AND OBJECTIVES: The 2019 coronavirus pandemic (COVID-19) represented a significant challenge for the medical community. The first aim of this study was to examine the COVID-19 impact on the follow-up of patients with dilated cardiomyopathy (DCM) and to establish the advantages of multiparametric home monitoring. Also, we tried to establish the main prognostic predictors at 2-years follow-up and the value of LV diastolic filling pattern (LVDFP) in increasing mortality and morbidity. MATERIALS AND METHODS: We conducted a prospective study of 142 patients with DCM assessed by in-patient visit in the pre-pandemic period and hybrid (face-to-face, online consultation and telemedicine home monitoring with a dedicated application) during the pandemic period. The statistical analysis compared the strategy used in the pre-pandemic with management during the pandemic, in terms of clinical assessment, hospitalizations/emergency room visits due to HF exacerbation and total mortality. RESULTS: We did not observe significant changes in blood pressure (BP), heart rate (FC), weight and symptoms or an increased rate of adverse drug events between the two periods. We successfully titrated HF medications with close monitoring of HF decompensations, which were similar in number, but were mostly managed at home during the pandemic. There was also no statistically significant difference in emergency room visits due to severe decompensated HF. Mortality in the first and second year of follow-up was between 12.0 and 13%, similar in the pre-pandemic and pandemic periods, but significantly higher in patients with restrictive LVDFP. Clinical improvement or stability after 2 years was more frequent in patients with nonrestrictive LVDFP. The main prognostic predictors at 1 and 2-years follow-up were: the restrictive LVDFP, significantly dilated LV, comorbidities (DM, COPD), older age, associated severe mitral regurgitation and pulmonary hypertension. CONCLUSIONS: The pandemic restrictions determined a marked decrease of the healthcare use, but no significant change in the clinical status of DCM patients under multiparametric home monitoring. At 2-years follow-up, the presence of the restrictive LVDFP was associated with an increased risk of death and with a worse clinical status in DCM patients.

Diastolic versus Systolic Left Ventricular Dysfunction as Independent Predictors for Unfavorable Postoperative Evolution in Patients with Aortic Regurgitation Undergoing Aortic Valve Replacement.

Background and Objectives: Chronic severe aortic valve disease is associated with important changes in left ventricle (LV) performance associated with eccentric or concentric LV hypertrophy. We aimed to assess the immediate prognostic implications of the type of the LV diastolic filling pattern (LVDFP) compared with LV systolic performance in patients with severe aortic regurgitation (AR) undergoing aortic valve replacement (AVR) and to define the independent echographic predictors for the immediate and long-term prognoses. Materials and Methods: We performed a prospective study enrolling 332 AR patients undergoing AVR, divided into two groups: Group A­201 pts with normal LV systolic function, divided into two subgroups (A1: 129 pts with a nonrestrictive LVDFP and A2: 72 pts with restrictive LVDFP), and Group B­131 pts with LV systolic dysfunction (LV ejection fraction LVEF < 50%), divided into two subgroups (B1: 83 pts with a nonrestrictive LVDFP and B2: 48 pts with restrictive LVDFP). Results: The early postoperative mortality rate was higher in patients with a restrictive LVDFP (11.12% in A2 and 12.5% in B2) compared with normal LV filling (2.32% in A1 and 7.63% in B1, p < 0.0001), regardless of the LVEF. The restrictive LVDFP­defined by at least one of the following echographic parameters: an E/A > 2 with an E wave deceleration time (EDt) < 100 ms; an isovolumetric relaxation time (IVRT) < 60 ms; or an S/D ratio < 1 in the pulmonary vein flow­was an independent predictor for early postoperative mortality, increasing the relative risk by 8.2-fold. Other independent factors associated with early poor prognosis were an LV end-systolic diameter (LVESD) > 58 mm, an age > 75 years, and the presence of comorbidities (chronic obstructive pulmonary disease-COPD or diabetes mellitus). On a medium-term, an unfavorable evolution was associated with: an age > 75 years (RR = 8.1), an LV end-systolic volume (LVESV) > 95 cm3 (RR = 6.7), a restrictive LVDFP (RR = 9.8, p < 0.0002), and pulmonary hypertension (RR = 8.2). Conclusions: The presence of a restrictive LVDFP in patients with AR undergoing AVR is associated with both increased early and medium-term mortality rates. The LV diastolic function is a more reliable parameter for prognosis than LV systolic performance (RR 9.2 versus 2.1). Other independent predictors for increased early postoperative mortality rate were: an age > 75 years, an LVESD > 58 mm, and comorbidities (diabetes mellitus, COPD), and for unfavorable evolution at 2 years postoperatively: an age > 75 years, an LVESV > 95 cm3, and severe pulmonary hypertension.

Additional Prognostic Value of Tissue Doppler Evaluation in Patients with Aortic Stenosis and Left-Ventricular Systolic Dysfunction Undergoing Aortic Valve Replacement.

Background and Objectives: Patients with surgical aortic stenosis (AS) show impaired diastolic filling, which is a risk factor for early and late mortality after aortic valve replacement (AVR). There is a paucity of information concerning the impact of restrictive diastolic filling and the evolution of diastolic dysfunction in the early and medium terms post-AVR. We aimed to determine the prognostic value of the presence of a restrictive left-ventricular (LV) diastolic filling pattern (LVDFP) and dilated left atrium (LA) in patients with AS and LV systolic dysfunction (LVEF < 40%) who underwent AVR, and to define the independent predictors for immediate and long-term prognosis and their value for preoperative risk estimation. Materials and Methods: The study was prospective and included 197 patients with surgical AS and LVEF <40% who underwent AVR. Preoperative echocardiographic examinations were repeated at day 10, at 1, 3 and 6 months, and at 1 and 2 years after surgery, with evaluation of LVEF, diastolic function and LA dimension index (mm/m2). Depending on LV systolic performance, patients were classified as Group A (LVEF: 30−40%) or Group B (LVEF < 30%). Results: The main echographic independent parameters for early and late postoperative death were: restrictive LVDFP, significant pulmonary hypertension, LV end-systolic diameter (LVESD) >55 mm and the presence of second-degree mitral regurgitation. Restrictive LVDFP and LA dimension >30 mm/m2 were independent predictors for fatal outcome (p = 0.0017). Conclusions: Assessment of diastolic function and LA dimension are reliable parameters in predicting fatal outcome and hospitalization for heart failure, having an independent and incremental prognostic value in patients with surgical AS. Complete evaluation of LVDFP with all the echographic measurements (including TDI) should routinely be part of the preoperative assessment of patients with LV systolic dysfunction undergoing AVR.

Successful Treatment of Catastrophic Antiphospholipid Syndrome Using Rituximab: Case Report and Review of the Literature.

BACKGROUND: Kidney involvement is a frequent complication of systemic lupus erythematosus (SLE) and kidney biopsy is essential in differentiating lupus nephritis (LN) from thrombotic microangiopathy (TMA) secondary to antiphospholipid autoantibodies (aPL). Association between antiphospholipid syndrome (APS) and acquired hemophilia due to inhibitors was very rarely described in SLE patients. CASE PRESENTATION: We present the case of a 61-year-old male diagnosed with SLE who acquired deficiency of clotting factor VIII due to circulating inhibitors, admitted for acute kidney injury (AKI), microangiopathic hemolytic anemia, thrombocytopenia, and diplopia. Kidney biopsy showed TMA due to APS, but no signs of LN. Head computed tomography identified low dense areas in the white matter, suggesting small blood vessels' involvement. A diagnosis of probable catastrophic antiphospholipid syndrome (CAPS) was established and treatment with low molecular weight heparin, intravenous methylprednisolone, plasmapheresis, and rituximab was initiated, followed by resolution of AKI, diplopia, and TMA with complete depletion of CD19+B-lymphocytes (CD19+B-Ly) after one month. We further review the current knowledge regarding pathogenesis and management of CAPS in SLE patients. CONCLUSIONS: Targeted therapy was possible after kidney biopsy, improving renal and general prognosis. CD19+B-Ly repopulation preceded biological relapse, so monitoring of CD19+B-Ly may serve as a tool to predict relapses and guide rituximab therapy.

Familial Mediterranean fever-associated renal amyloidosis: case report and review of the literature.

Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease, which is diagnosed especially in Mediterranean patients, but is a rare disorder in our geographical area. Due to its rarity and symptoms consisting mainly in recurrent episodes of fever and serositis, it may be mistaken with other, more frequent diseases, especially acute abdomen and systemic rheumatic diseases. The most important life-threatening complication is secondary amyloidosis, which usually affects kidneys, with proteinuria up to nephrotic syndrome and chronic kidney disease progressing to end-stage renal disease requiring dialysis or transplantation. In patients with suspected amyloidosis, kidney biopsy or submucosal rectal biopsy are the methods of choice for diagnosis. Kidney biopsy is also useful in patients with FMF who start to develop proteinuria, since other non-amyloid glomerular involvement may appear in FMF. Colchicine is now the gold standard for treatment, not only to reduce the frequency of attacks but also to improve renal prognosis. For this reason, the sooner the diagnosis is established the better the prognosis will be since the patient will benefit from the appropriate treatment with Colchicine. We present the case of a young female patient diagnosed through kidney biopsy with amyloid A (AA) amyloidosis after 30 years of evolution of FMF and we review the present knowledge regarding the pathogenesis and management of this rare genetic disease.

Nephrotic syndrome after autologous hematopoietic stem cell transplantation: a case report.

Nephrotic syndrome (NS) is a rare complication of hematopoietic cell transplantation (HCT) and is thought to represent a renal manifestation of chronic graft-versus-host disease (cGVHD). Glomerulopathies occur less often in recipients of autologous as compared to allogeneic HCT and, in this setting, renal pathology is less well characterized. This case report describes a 54-year-old man admitted for the evaluation of a nephrotic-range proteinuria. His past medical history included a ? light-chain secreting multiple myeloma (MM) for which he underwent autologous HCT. Prior to admission, the level of proteinuria on successive check-ups was over 3.5 g÷day, while on treatment with Losartan for the past six months for mild arterial hypertension. At the time of admission, the clinical examination was unremarkable and there were not any signs of cGVHD. Initial testing showed a nephrotic-range proteinuria (5.6 g÷day) with normal renal function, while excluding secondary causes of NS. The patient underwent a kidney biopsy that revealed the classic variant of focal and segmental glomerulosclerosis (FSGS). The patient was started on Cyclosporine 5 mg÷kg÷day and, after nine months, he experienced a partial remission (proteinuria 1.2 g÷day). This is the first report of FSGS as the etiology of autologous HCT-associated NS.

Smoking-associated nodular glomerulosclerosis, a rare renal pathology resembling diabetic nephropathy: case report.

INTRODUCTION: Smoking is an important risk factor not only for cardiovascular and pulmonary diseases, but also for the progression of chronic kidney disease of different etiologies. Nodular glomerulosclerosis is a renal pathology pattern, which was described in different kidney conditions, especially diabetic nephropathy. A very rare association among smoking, hypertension and nodular mesangial glomerulosclerosis has been recently described. CASE PRESENTATION: In this paper, we present the case of a non-diabetic male patient referred to our Department for advanced chronic kidney disease and nephrotic syndrome. After excluding different causes of secondary nephrotic syndrome, a kidney biopsy was performed. The patient was diagnosed with smoking associated nodular glomerulosclerosis, with a histological aspect closely resembling diabetic nephropathy. A low protein and low salt diet was started, accompanied by smoking cessation, the administration of diuretics, of antiproteinuric treatment with angiotensin receptor blocker and antihypertensive therapy. Under this therapy, after six months, the patient evolution was good with a clear improvement of kidney function and important reduction of proteinuria. DISCUSSION: We also present the possible factors that could be involved in the deleterious effects of smoking upon kidney structure endothelial dysfunction, angiogenesis, altered intrarenal hemodynamics, nervous sympathetic system, increased oxidative stress and, very important, the generation of advanced glycation end products, which are also implicated in the development of diabetic nephropathy. CONCLUSIONS: Although a rare condition, smoking associated nodular glomerulosclerosis is a diagnosis not to be missed when dealing with a heavy smoker patient, especially when hypertensive, and sometimes associating nephrotic syndrome and this diagnosis should be considered together with much more frequent causes of nephrotic syndrome.