Delayed Diagnosis of Pulmonary Artery Thrombosis in a Patient Undergoing Mitral Valve Replacement.

ABSTRACT: The occurrence of pulmonary artery thrombus in association with rheumatic mitral stenosis is a rare complication. Pulmonary artery thrombus formation may worsen pulmonary artery pressures, and this may precipitate acute right heart failure. The possible mechanisms behind pulmonary artery thrombus formation during mitral valve replacement surgery could be acute coagulopathy following surgery, the presence of chronic pulmonary thromboembolism, or chronic atrial fibrillation. We report an unusual case of pulmonary artery thrombus in a patient with rheumatic MS which was diagnosed with transoesophageal echocardiography after MVR.

Heart Transplantation in a Patient With Rheumatic Heart Disease and Severe Left Atrial Calcification.

A 62-year-old woman who had undergone mitral valve replacement 24 years ago was admitted to the hospital with congestive heart failure. She needed heart transplantation for stage D heart failure. Preoperative cardiac computed tomographic scans showed a severely calcified left atrium and a large right atrium. Given that the left atrium's calcification was too severe to suture, the calcified left atrial wall was broadly resected, and the resected left atrial wall was reconstructed with a bovine pericardial patch for anastomosis with the donor's left atrial wall. The operation was completed without heavy bleeding, and the patient was discharged from the hospital with no complications.

Adult-onset acute rheumatic fever with chorea and carditis.

Rheumatic fever is a major cause of cardiovascular morbidity and mortality in low-income and middle-income countries, and it usually occurs at a young age. Adult-onset acute rheumatic fever is a rare condition and usually represents a recurrence of childhood-onset disease. We report a case of an elderly man presenting with rheumatic carditis and rheumatic chorea subsequently diagnosed with adult-onset rheumatic fever.

Left Atrial Enlargement as a Predictor of Atrial Fibrillation in Rheumatic Mitral Valve Disease: An Echocardiography-based Retrospective Cross-sectional Study.

BACKGROUND: Rheumatic heart disease is a major cause of cardiovascular morbidity and mortality in developing nations and is a leading cause of hospital admission due to cardiac problems in our country. This study will evaluate the association between left atrial size and the occurrence of atrial fibrillation and describe the clinical characteristics along with complications related to Rheumatic Mitral valve disease. METHODS: A retrospective cross-sectional study was conducted at a tertiary care center from January 2018 to December 2019. Reports of 207 patients admitted to medical and/or surgical wards with echocardiographic diagnosis of rheumatic mitral valve disease with or without atrial fibrillation were reviewed. Data were collected, entered, and analyzed using the Statistical Package for the Social Science version 25.0. RESULTS: Among 207 patients, atrial fibrillation was present in 90 (43.5%) patients. Atrial fibrillation was higher in patients with mixed mitral valvular lesions compared to isolated mitral stenosis or mitral regurgitation. Univariate and multivariate analysis revealed left atrial size [aOR=1.067, 95% CI: 1.023 - 1.113, P= 0.002] and age [aOR = 1.073, 95% CI: 1.042 - 1.105, P<0.001] as an independent predictor of atrial fibrillation. CONCLUSIONS: Larger left atrium was an independent predictor of atrial fibrillation. Besides this, atrial fibrillation was associated with increasing age, mixed mitral valvular lesion, and moderately reduced left ventricular ejection fraction, but not associated with gender and mitral stenosis severity. Left atrial clot was significantly higher in patients with atrial fibrillation than in sinus rhythm.

Echocardiographic Parameters and Complication Profiles Among Adult Patients with Rheumatic Heart Disease at Jimma Medical Center.

Background: Despite the fact that patients with rheumatic heart disease (RHD) need early medical attention and follow-up, most patients in developing countries tend to present with debilitating complications. The objective of this study was to evaluate the echocardiographic features of adult individuals diagnosed with RHD and examine the associated complications among patients who started follow-up at Jimma Medical Center's (JMC) cardiac follow-up clinic. Methods: A prospective cross-sectional study was conducted at JMC between January 5 and April 15, 2023. Echocardiographic patterns were taken by senior cardiologists; socio-demographic variables, anthropometric measurements, and behavioral factors were collected through a structured questioner. Results: The study recruited a total of 115 participants, of whom 86 (74.8%) were female and 29 (25.2%) were male. The mean age of the patients was 32.31 (SD± 12.16) years. The mitral valve was affected in 98.26% of cases, while the aortic and tricuspid valve abnormalities were diagnosed in 49.5% and 21.7%, respectively. The most frequent combinations of valve lesions were mitral regurgitation (MR) + mitral stenosis (MS) + aortic regurgitation (AR) (15.7%), followed by MR + AR + TR (8.7%). The occurrence of MR+MS+AR was higher in females (17.4%) compared to males (10.3%), whereas the occurrence of MS+MR was higher in males (24.1%) compared to females (20.9%). Females have a severely reduced ejection fraction compared to males (84.8% vs 15.2%, P = 0.044). Nearly two-thirds (63.5%) of individuals experienced RHD-related complications; the most commonly encountered complications were pulmonary hypertension (26.1%) and atrial fibrillation (19.1%). Conclusion: RHD predominantly affects individuals in their active and productive years, particularly females. Most patients have multiple-valve lesions.

Comparative efficacy and safety of mitral valve repair versus mitral valve replacement in Rheumatic heart disease: A high-value care systematic review and meta-analysis.

Rheumatic Heart Disease (RHD) remains a leading cause of cardiovascular death (CVD) globally. Mitral Valve repair (MVP) and mitral valve replacement (MVR) are the two most commonly and successfully used techniques to treat the disease. MVP is associated with reduced post-operative complications compared to MVR; however, it carries the risk of valvular fibrosis and scarring. Given the lack of recommendations, inconsistent findings, and paucity of pathophysiological evidence at present, we aimed to conduct a meta-analysis and systematically review the available literature to determine the efficacy and safety of MVP compared to MVR in improving clinical outcomes among patients with RHD. A comprehensive literature search was conducted on MEDLINE (PubMed), Cochrane Central and Scopus from inception till September 2023. The primary objective was early mortality defined as any cause-related death occurring 30 days following surgery. Secondary outcomes included long-term survival defined as the time duration between hospital discharge and all-cause death. Infectious endocarditis, thromboembolic events (including stroke, brain infarction, peripheral embolism, valve thrombosis, and transient ischemic attack), and haemorrhagic events (any serious bleeding event that required hospitalisation, resulted in death, resulted in permanent injury, or required blood transfusion) were all considered as post- operative complications. Additionally aggregated Kaplan-Meier curves were reconstructed for long term survival, freedom from reoperation, and freedom from valve-related adverse events by merging the reconstructed individual patient data (IPD) from each individual study. A significant decrease in early mortality with MV repair strategy versus MV replacement [RR 0.63; P = 0.003) irrespective of mechanical or bioprosthetic valves was noted. The results reported significantly higher long-term survival in patients undergoing MVP versus MVR (HR 0.53; P = 0.0009). Reconstructed Kaplan-Meier curves showed that the long term survival rates at 4, 8, and 12 years were 88.6, 82.0, 74.6 %, in the MVR group and 91.7, 86.8, 81.0 %, in the MVP group, respectively. MVP showed statistically significant reduction in early mortality, adverse vascular events, and better long-term survival outcomes compared to the MVR strategy in this analysis.

Outcome following acute and recurrent rheumatic fever.

BACKGROUND: Rheumatic carditis is the leading cause of permanent disability caused by damage of the cardiac valve. This study aimed to determine the outcome and predictors of valve surgery in patients with acute rheumatic fever (ARF) and recurrent rheumatic fever (RRF). METHODS: This was a retrospective study of patients diagnosed with ARF and RRF between 2006 and 2021. The predictors of valve surgery were analysed using multivariable Cox proportional regression. RESULTS: The median age of patients with ARF and RRF (n=92) was 11 years (range 5-18). Seventeen patients (18%) were diagnosed with RRF. The most common presenting symptoms included clinical carditis (87%), heart failure (HF) (63%), fever (49%) and polyarthralgia (24%). Patients with moderate-to-severe rheumatic carditis (88%) were given prednisolone. After treatment, the severity of valvular regurgitation was reduced in 52 patients (59%). Twenty-three patients (25%) underwent valve surgery. The incidence of HF, RRF, severe mitral regurgitation on presentation, left ventricular enlargement and pulmonary hypertension was greater in the surgical group than in the non-surgical group. Recurrent rheumatic fever (hazard ratio 7.9, 95% CI 1.9-33.1), tricuspid regurgitation (TR) gradient ≥ 42 mmHg (HR 6.3, 95%CI 1.1-38.7) and left ventricular end-diastolic dimension (LVEDD) ≥6 cm (HR 8.7, 95% CI 2.1-35.9) were predictors of valve surgery (multivariable Cox proportional regression analysis). CONCLUSION: Clinical carditis was the most common presenting symptom in patients with ARF and RRF. The majority of patients responded positively to prednisolone. These findings highlight the predictors of valve surgery following ARF, including RRF, TR gradient ≥ 42 mmHg and LVEDD ≥ 6 cm.Abbreviations: ARF: acute rheumatic fever; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GAS: group A beta-haemolytic Streptococcus; HF: heart failure; HR: hazard ratio; LVEDD: left ventricular end-diastolic dimension; MR: mitral regurgitation; RHD: rheumatic heart disease; RRF: recurrent rheumatic fever; TR: tricuspid regurgitation.

Outcomes of rheumatic fever in Uganda: a prospective cohort study.

BACKGROUND: Rheumatic heart disease is the largest contributor to cardiac-related mortality in children worldwide. Outcomes in endemic settings after its antecedent illness, acute rheumatic fever, are not well understood. We aimed to describe 3-5 year mortality, acute rheumatic fever recurrence, changes in carditis, and correlates of mortality after acute rheumatic fever. METHODS: We conducted a prospective cohort study of Ugandan patients aged 4-23 years who were diagnosed with definite acute rheumatic fever using the modified 2015 Jones criteria from July 1, 2017, to March 31, 2020, enrolled at three rheumatic heart disease registry sites in Uganda (in Mbarara, Mulago, or Lira), and followed up for at least 1 year after diagnosis. Patients with congenital heart disease were excluded. Patients underwent annual review, most recently in August, 2022. We calculated rates of mortality and acute rheumatic fever recurrence, tabulated changes in carditis, performed Kaplan-Meier survival analyses, and used Cox regression models to identify correlates of mortality. FINDINGS: Data were collected between Sept 1 and Sept 30, 2022. Of 182 patients diagnosed with definite acute rheumatic fever, 156 patients were included in the analysis. Of these 156 patients (77 [49%] male and 79 (51%) female; data on ethnicity not collected), 25 (16%) died, 21 (13%) had a cardiac-related death, and 17 (11%) had recurrent acute rheumatic fever over a median of 4·3 (IQR 3·0-4·8) years. 16 (24%) of the 25 deaths occurred within 1 year. Among 131 (84%) of 156 survivors, one had carditis progression by echo. Moderate-to-severe carditis (hazard ratio 12·7 [95% CI 3·9-40·9]) and prolonged PR interval (hazard ratio 4·4 [95% CI 1·7-11·2]) at acute rheumatic fever diagnosis were associated with increased cardiac-related mortality. INTERPRETATION: These are the first contemporary data from sub-Saharan Africa on medium-term acute rheumatic fever outcomes. Mortality rates exceeded those reported elsewhere. Most decedents already had chronic carditis at initial acute rheumatic fever diagnosis, suggesting previous undiagnosed episodes that had already compounded into rheumatic heart disease. Our data highlight the large burden of undetected acute rheumatic fever in these settings and the need for improved awareness of and diagnostics for acute rheumatic fever to allow earlier detection. FUNDING: Strauss Award at Cincinnati Children's Hospital, American Heart Association, and Wellcome Trust.

Manifestations of Rheumatic Carditis, Regression of Valvular Regurgitation, and Independent Predictors of Mitral Regurgitation Improvement After Rheumatic Carditis in Thai Children.

Background: Acute rheumatic fever (ARF) with carditis can lead to the development of rheumatic heart disease in children and young adults. Objective: This study aimed to investigate the manifestations of rheumatic carditis, clinically significant regression of valvular regurgitation as assessed by echocardiography, and the independent predictors of mitral regurgitation (MR) improvement after rheumatic carditis in Thai children. Method: Children diagnosed with rheumatic carditis during 2005-2020 at Siriraj Hospital (Bangkok, Thailand) were retrospectively enrolled. Trivial, and mild regurgitation were grouped as non-clinically significant (NCS) regurgitation. Valvular regression was defined moderate-severe regurgitation improving to NCS regurgitation. Results: Eighty-one patients (mean age: 10 years, range: 8-12 years) were included. At presentation, 59 (72.8%) patients had combined mitral regurgitation (MR) and aortic regurgitation (AR), 20 (24.6%) patients had MR alone, and 2 (2.4%) patients had AR alone. Concerning severity, 28 (34.6%) and 30 (37%) patients presented with severe and moderate MR, respectively. Severe and moderate AR was found in 9 (11.1%) and 16 (19.8%) patients, respectively. At the one-year follow-up, 43.4% of moderate-severe MR, and 41.7% of moderate-severe AR improved to NCS regurgitation. Multivariate analysis revealed high erythrocyte sedimentation rate (ESR) (p = 0.01) and severe carditis (p = 0.05) at presentation to be independent predictors of MR improvement. Conclusion: Thai children with rheumatic carditis had a high incidence of valvular regurgitation; however, the valvular damage was improved in most patients. High ESR and severe carditis independently predict MR improvement.

Surgical aortic valve replacement etiologies, hemodynamics, and outcomes in 1346 patients from the Malaysian heart centre.

BACKGROUND: This study examined the characteristics and outcomes of surgical aortic valve replacement (SAVR) both isolated and in combination with other cardiac surgery in Malaysia from 2015 to 2021. METHODS: This was a retrospective study of 1346 patients analyzed on the basis of medical records, echocardiograms and surgical reports. The overall sample was both considered as a whole and divided into aortic stenosis (AS)/aortic regurgitation (AR)-predominant and similar-severity subgroups. RESULTS: The most common diagnosis was severe AS (34.6%), with the 3 most common etiologies being bicuspid valve degeneration (45.3%), trileaflet valve degeneration (36.3%) and rheumatic valve disease (12.2%). The second most common diagnosis was severe AR (25.5%), with the most common etiologies being root dilatation (21.0%), infective endocarditis (IE) (16.6%) and fused prolapse (12.2%). Rheumatic valve disease was the most common mixed disease. A total of 54.5% had AS-predominant pathology (3 most common etiologies: bicuspid valve degeneration valve, degenerative trileaflet valve and rheumatic valve disease), 36.9% had AR-predominant pathology (top etiologies: root dilatation, rheumatic valve disease and IE), and 8.6% had similar severity of AS and AR. Overall, 62.9% of patients had trileaflet valve morphology, 33.3% bicuspid, 0.6% unicuspid and 0.3% quadricuspid. For AS, the majority were high-gradient severe AS (49.9%), followed by normal-flow low-gradient (LG) severe AS (10.0%), paradoxical low-flow (LF)-LG severe AS (6.4%) and classical LF-LG severe AS (6.1%). The overall in-hospital and total 1-year mortality rates were 6.4% and 14.8%, respectively. Pure severe AS had the highest mortality. For AS-predominant pathology, the etiology with the highest mortality was trileaflet valve degeneration; for AR-predominant pathology, it was dissection. The overall survival probability at 5 years was 79.5% in all patients, 75.7% in the AS-predominant subgroup, 83.3% in the AR-predominant subgroup, and 87.3% in the similar-severity subgroup. CONCLUSIONS: The 3 most common causes of AS- predominant patients undergoing SAVR is bicuspid valve degeneration, degenerative trileaflet valve and rheumatic and for AR-predominant is root dilatation, rheumatic and IE. Rheumatic valve disease is an important etiology in our SAVR patients especially in mixed aortic valve disease. Study registration IJNREC/562/2022.

Midterm Outcome of Valve Repair for Rheumatic Mitral Stenosis: 6-year Experience in a Single Mid-Volume Cardiac Center.

OBJECTIVES: Previous studies have reported satisfactory long-term results of mitral valve (MV) repair for rheumatic mitral disease. However, the effects of this procedure in isolated rheumatic mitral stenosis remain unclear. In addition, protective effects of MV repair on cardiac function have not been verified in rheumatic MV disease. This study retrospectively evaluated early mortality and mid-term results of MV repair for isolated rheumatic mitral stenosis in a mid-volume cardiac centre, and explored the effects of this procedure on cardiac function. METHODS: Between January 2015 and May 2021, 360 patients with isolated rheumatic mitral stenosis and combined (concomitant) atrial fibrillation (AF) underwent MV repair (100 patients) or MV replacement (260 patients). Perioperative characteristics were compared between the two groups and a regression analysis for early mortality and mid-term left ventricular ejection fraction was conducted. In addition, mid-term survival was compared between the two groups. RESULTS: Baseline characteristics of the two groups were balanced after matching. Compared with patients in the replacement group, patients with MV repair had a lower occurrence of postoperative hypotension and AF. There was no difference in early mortality or mid-term survival between the two groups. However, MV repair was associated with a higher mid-term left ventricular ejection fraction. During follow-up, four thromboembolic events and four haemorrhagic events occurred in the replacement group. No blood coagulation-related complications occurred in the repair group. CONCLUSION: Mitral valve repair for isolated rheumatic mitral stenosis and concomitant AF was feasible in a mid-volume cardiac centre, with satisfactory perioperative results and mid-term outcomes. Furthermore, this procedure preserved mid-term left ventricular systolic function.

Tricuspid valve disease and cardiac implantable electronic devices.

The role of cardiac implantable electronic device (CIED)-related tricuspid regurgitation (TR) is increasingly recognized as an independent clinical entity. Hence, interventional TR treatment options continuously evolve, surgical risk assessment and peri-operative care improve the management of CIED-related TR, and the role of lead extraction is of high interest. Furthermore, novel surgical and interventional tricuspid valve treatment options are increasingly applied to patients suffering from TR associated with or related to CIEDs. This multidisciplinary review article developed with electrophysiologists, interventional cardiologists, imaging specialists, and cardiac surgeons aims to give an overview of the mechanisms of disease, diagnostics, and proposes treatment algorithms of patients suffering from TR associated with CIED lead(s) or leadless pacemakers.

Correlation of n-terminal pro-brain-type natriuretic peptide levels with the severity of single mitral regurgitation or accompanied by mild aorta valve dysfunction in patients with rheumatic heart disease in Sanglah general hospital.

BACKGROUND: The most frequent abnormality of the valves involved in rheumatic heart disease is mitral regurgitation. A promising supportive diagnostic tool for rheumatic heart disease is the N-terminal pro-brain natriuretic peptide, which can identify mitral valve damage. OBJECTIVE: To prove a positive correlation between N-terminal pro-brain natriuretic peptide levels and the severity of mitral regurgitation or accompanied by mild aortic valve dysfunction in children with rheumatic heart disease. MATERIAL AND METHOD: Children aged 1-18 years who had been identified as having rheumatic heart disease with a single mitral regurgitation or accompanied by mild aortic issues at Sanglah General Hospital, Denpasar, met the inclusion criteria for this cross-sectional study. Mitral regurgitation severity was determined using the parameters of vena contracta width, effective regurgitation orifice area, regurgitant jet area, and regurgitation volume. Bivariate analysis using the Spearman correlation test. RESULTS: From 36 research subjects, the mean age was 11.32 years. In this study, there was a moderate positive correlation between the levels of N-terminal pro-brain natriuretic peptide and the regurgitation jet area. N-terminal pro-brain natriuretic peptide levels also had a moderately positive correlation with the vena contracta width and a weakly positive correlation with the regurgitation volume. Effective regurgitation orifice area and N-terminal pro-brain natriuretic peptide levels did not significantly correlate. CONCLUSION: There was a moderately positive correlation between N-terminal pro-brain natriuretic peptide levels and the severity of mitral regurgitation on regurgitation jet area, a moderately positive correlation with the vena contracta width, and a weakly positive correlation with regurgitation volume in rheumatic heart disease patients.

Coronary artery disease in patients with rheumatic valvular heart disease- An analysis from eastern India.

INTRODUCTION: There is insufficient information on the angiographic characteristics of individuals with rheumatic valvular heart disease (VHD) from eastern India. The objective of this research is to gather important data in this area to aid the best surgical outcomes for patients with rheumatic VHD. MATERIALS AND METHODS: 978 consecutive patients with rheumatic VHD, scheduled for surgical intervention, were recruited. RESULT AND CONCLUSION: Mitral valve involvement was observed in 66.1 %, aortic valve in 7.3 % and both valves in 26.6 %. Patients with CAD had significantly higher proportions of severe aortic stenosis (AS). Therefore, addressing the risk factors for CAD is crucial in patients with rheumatic VHD.

[Rheumatic cardiopathies and its risk factors: about 50 cases]. / Les complications cardiaques du rhumatisme articulaire aigu et ses facteurs de risque : étude de 50 observations.

INTRODUCTION: Acute rheumatic fever (ARF) is a multi-systemic disease, in which cardiac involvement is the most serious major manifestation of disease. The aim of this study was to analyse cardiac involvement in children with ARF and his risk factors. MATERIALS AND METHODS: It were a retrospective study including all children under the age of 14 years who were hospitalized for ARF in the pediatric department of the CHU Hédi Chaker of Sfax, during a period of twelve years (2010-2022). RESULTS: We collected 50 cases (31 boys and 19 girls). Twenty-two patients (44%) developed cardiac lesions. The mean age at diagnosis was 9.6 years [5-14 years]. A pathological heart murmur was detected in 14 cases (n = 14/22) was classified as mild carditis in 15 cases, moderate carditis in 5 cases and severe in 2 cases. The median follow-up time was 3,3 years. Nineteen patients developed valvular sequelae Risk factors of cardiac lesions was: age more than 8 years, heart murmur, allonged PR, CRP > 100 mg/l and VS > 100 mm. CONCLUSION: CR is still a public health problem in Tunisia. It is a serious pathology that can cause serious increases in morbidity rates. Thus, we must strengthen preventive strategies.

Comparison between interventional versus medical therapy in patients with rheumatic mitral valve stenosis in Tanzania.

BACKGROUND: Rheumatic heart disease remains the most common cardiovascular disease in children and young adults. The outcome of interventional versus medical therapy on the long term is not fully elucidated yet. This study provides contemporary data on the clinical profile, treatment and follow up of patients with rheumatic mitral stenosis (MS) in Tanzania. METHODS: Patients' medical information, investigations and treatment data were recorded in this prospective cohort study. They were followed up for 6-24 months to determine the long-term outcome. Interventional therapy was defined as a combination of surgery and percutaneous balloon mitral valvuloplasty. Kaplan-Meier curves and Cox proportional hazards model were used in analyses. p-Value < 0.05 was considered statistically significant. RESULTS: We enrolled 290 consecutive patients. Interventions were done in half of the patients. Median follow up was 23.5 months. Mortality was higher in the medical than interventional treatment (10.4% vs. 4%, log-rank p = 0.001). Median age was 36 years, females (68.3%) and low income (55.5%). Multivalvular disease was found in 116 (40%) patients, atrial fibrillation (31.4%), stroke/transient ischaemic attack (18.9%) and heart failure class III-IV (44.1%). Median (IQR) duration of disease was 3 (4) years, secondary prophylaxis (27.7%) and oral anticoagulants use (62.3%). In multivariable analysis, the risk of death among patients on medical was 3.07 times higher than those on interventional treatment (crude HR 3.07, 95% CI 1.43-6.56, p = 0.004), 2.44 times higher among patients with arrhythmias versus without arrhythmias (crude HR 2.44, 95% CI 1.19-4.49, p = 0.015) and 2.13 times higher among patients with multivalvular than single valve disease (crude HR 2.13, 95% CI 1.09-4.16, p = 0.026). CONCLUSIONS: Intervention is carrying low mortality compared to medical treatment. Arrhythmias and multivalvular disease are associated with a high mortality. Rheumatic MS is more prevalent in young people, females and individuals with low income. There is a late hospital presentation and a low use of both secondary prophylactic antibiotics and anticoagulants.