Rheumatic Cardiac Disease Research Articles

Unknown rheumatic cardiac disease as cause of acute onset post-partum dyspnea: a case report

BackgroundAcute post-partum dyspnea configures an obstetric challenge with multiple differential diagnosis.Case presentationWe present a case of a previous healthy woman with preeclampsia who developed severe dyspnea 30 h after delivery. She complained of cough, orthopnea, and bilateral lower extremities oedema. She denied headaches, blurry vision, nausea, vomiting, fever or chills. Auscultation revealed a diastolic murmur, and was compatible with pulmonary oedema. A timely bedside echocardiogram showed moderate dilated left atrium with severe mitral insufficiency suggestive of an unknown rheumatic disease. She was managed with noninvasive ventilation, loop diuretics, vasodilators, thromboprophylaxis, head-end elevation, and fluid restriction with progressive improving.ConclusionsHemodynamic changes in pregnant patients with previously silent cardiac disease may pose a challenge and cause post-partum dyspnea. This scenario requires a timely and multidisciplinary approach.

Acute Liver Failure Due to Hypoxic Hepatitis Successfully Treated with Plasma Exchange

Hypoxic hepatitis (HH) is an uncommon cause for acute liver failure (ALF). It is also called as shock liver, ischemic, or congestive hepatopathy. HH is usually associated with premorbid cardiac illnesses such as acute myocardial infarction, rheumatic cardiac disease, cardiomyopathies, or shock due to any etiology. It occurs when hepatocytes suffer hypoxic or ischemic injury. If the primary disease is treated, the natural history of HH is usually self-limiting. However, in up to half of the patients, it can lead to multiple organ ischemic insults such as worsening liver injury or acute renal failure, where the treatment is based on the major organ of involvement. Here, we present a patient with ALF due to hypoxic liver injury with a primary cardiac disease and a favorable outcome following plasma exchange therapy in our tertiary care center.

Improvement of Rheumatic Valvular Heart Disease in Patients Undergoing Prolonged Antibiotic Prophylaxis.

Secondary prophylaxis of rheumatic heart diseases is efficient in reducing disease recurrence, heart damage, and cardiac impairment. We aimed to monitor the clinical evolution of a large Brazilian cohort of rheumatic patients under prolonged secondary prophylaxis. From 1986 to 2018, a cohort of 593 patients with rheumatic fever was followed every 6 months by the Reference Center for the Control and Prevention of Rheumatic Fever and Rheumatic Cardiopathy (CPCFR), Paraná, Brazil. In this cohort, 243 (41%) patients did not present cardiac damage (group I), while 350 (59%) were diagnosed with rheumatic heart disease (RHD) (group II) using the latest case definition. Among group II, 233 and 15 patients had impairment of the mitral and aortic valves, respectively, while 102 patients had impairment of both valves. Lesions on the mitral and aortic valves presented a regression in 69.9 and 48.7% of the patients, respectively. Active patient recruitment in the reference center and early detection of oropharyngeal GAS were important factors for optimal adherence to the prophylactic treatment. Patients with disease progression were associated with noncompliance to secondary prophylaxis. No patients undergoing regular prophylaxis presented progression of the rheumatic cardiac disease. Eighteen valvular surgeries were performed, and four (0.7%) patients died. This study confirmed that tailored and active efforts invested in rheumatic heart disease secondary prevention allowed for significant clinical improvement.

Predictive performance of six mortality risk scores and the development of a novel model in a prospective cohort of patients undergoing valve surgery secondary to rheumatic fever.

BackgroundMortality prediction after cardiac procedures is an essential tool in clinical decision making. Although rheumatic cardiac disease remains a major cause of heart surgery in the world no previous study validated risk scores in a sample exclusively with this condition.ObjectivesDevelop a novel predictive model focused on mortality prediction among patients undergoing cardiac surgery secondary to rheumatic valve conditions.MethodsWe conducted prospective consecutive all-comers patients with rheumatic heart disease (RHD) referred for surgical treatment of valve disease between May 2010 and July of 2015. Risk scores for hospital mortality were calculated using the 2000 Bernstein-Parsonnet, EuroSCORE II, InsCor, AmblerSCORE, GuaragnaSCORE, and the New York SCORE. In addition, we developed the rheumatic heart valve surgery score (RheSCORE).ResultsA total of 2,919 RHD patients underwent heart valve surgery. After evaluating 13 different models, the top performing areas under the curve were achieved using Random Forest (0.982) and Neural Network (0.952). Most influential predictors across all models included left atrium size, high creatinine values, a tricuspid procedure, reoperation and pulmonary hypertension. Areas under the curve for previously developed scores were all below the performance for the RheSCORE model: 2000 Bernstein-Parsonnet (0.876), EuroSCORE II (0.857), InsCor (0.835), Ambler (0.831), Guaragna (0.816) and the New York score (0.834). A web application is presented where researchers and providers can calculate predicted mortality based on the RheSCORE.ConclusionsThe RheSCORE model outperformed pre-existing scores in a sample of patients with rheumatic cardiac disease.

Echocardiographic effect of successful balloon mitral valvuloplasty on right ventricular function

Abstract Background Rheumatic cardiac disease is an immunologic phenomenon that may affect any of the heart valves and is by far the most common cause of mitral stenosis. Aim of the work To evaluate impact of successful percutaneous balloon mitral valvuloplasty on right ventricular function. Subjects and methods 30 consecutive patients who underwent balloon mitral valvuloplasty for rheumatic mitral stenosis between September 2010 and July 2011 at Ain Shams University hospital were included. All patients were subjected to transthoracic echo Doppler study, tissue Doppler imaging and TEE for: (A) Assessment of severity of mitral stenosis. (B) Peak myocardial velocities during systole, early and late diastole. (C) Assessment of RV function by measurement of TAPSE and Tie index. Results The study revealed a significant drop in TAPSE (before and 24 h after, P 0.008; before and 3 ms after, P 0.001) respectively. There was no significant change 24 h and 3 ms after the procedure ( P 0.220) .There was no significant change in IVC flow before and after 24 h ( P 0.221). There was no significant drop between before and after 3 ms ( P 0.062). There was no significant change between 24 h and 3 ms after the procedure ( P 0.264). There was a significant drop in Tie index (before and 24 h after, P 0.008; before and 3 ms after, P 0.009) respectively but no significant drop was found 24 h and 3 ms after the procedure ( P 0.373). Conclusion The current study showed a significant improvement in both systolic and diastolic functions of RV as observed by different echocardiographic parameters post BMV in patients without organic TV disease.

Disturbance of intracardiac hemodynamics in children with chronic rheumatic cardiac disease.

By means of Doppler echocardiography there have been studied disturbances of intracardiac hemodynamics in 44 children aged 8-17 years with chronic rheumatic cardiac disease and developed mitral aortal and combined heart defects, as well as in chronic rheumatic cardiac disease without developed valvar defect. Differential approach has been defined to administration of inhibitors of angiotensin-converting factor in rheumatic heart defects: developed insufficiency of mitral and/or aortal valves II-III stage leads to remodeling of the left heart portions with developing chronic insufficiency of blood circulation, being an index for prolonged, not less than a year usage of the angiotensin-converting factor. In the presence of isolated mitral regurgitation, I stage in children with chronic rheumatic cardiac disease usage of the angiotensin-converting factor may be cancelled due to insignificant disturbances of valvar hemodynamics and a small risk of developing blood circulation insufficiency. Timely sanation of chronic infection foci in nasopharynx (conservative and surgical treatment of chronic tonsillitis, adenoid vegetations, therapy of hemolytic streptococcus presence) decreases risk of developing rheumatic heart defect in children suffered acute rheumatic fever.

A pendular prosthetic valve: severe paravalvular aortic leak

A 54-year old man with rheumatic cardiac disease was hospitalized for chest pain 9 years after combined aortic and mitral valve replacement (CL-III tilting disc valve prosthesis, Lanzhou, China). Physical examination and echocardiography indicated severe paravalvular aortic leak. The pendular prosthetic valve was recorded by coronary angiography (Fig. 1A and B; Supplementary Video S1). Supplementary material is available at EJCTS online. Supplementary Video 1: Coronary angiography of left anterior descending coronary artery in right anterior oblique caudal view showed severe paravalvular aortic leak and pendular flap of aortic prosthetic valve in cardiac cycle.

Clinical analysis of 16 cases of venous thromboembolism in pregnancy and puerperium

Objective To investigate the high risk factors,etiology,diagnosis,treatment and precaution of venous thromboembolism (VTE) in pregnancy and puerperium. Methods Data of 16 cases of VTE admitted in Department of Obstetrics and Gynecology,Peking Union Medical College Hospital from January 1992 to April 2011 were analyzed retrospectively.The differences of blood routine test and coagulation function between VTE patients (study group) and normal pregnant women (control group) were compared by paired t test in this case-control study. Results All patients were diagnosed by color Doppler ultrasound or CT pulmonary artery angiography.Five cases (31.2 ％) occurred before delivery and 11 (68.8 ％) occurred during postpartum.Two cases (12.5 ％)complicated by pulmonary thromboembolism,and one of them died.Six cases (37.5％) were older than or equal to 35 years old,and 12 cases (75.0％) had gestational complications.The hematocrit in study group were lower than that in control group (0.29±0.06 vs 0.39±0.02,t=4.56,P=0.01).There was no statistical difference between the coagulation function of the two groups.After anticoagulant therapy or surgery,15 cases recovered.One pulmonary thromboembolism patients complicated with rheumatic cardiac disease remained shock after anti-coagulants was administered,then died of hemorrhage of respiratory tract after inferior vena cava filter placement. Conclusions VTE is likcly to happen in puerperium than in pregnancy.There are no good screening indicators for thromboembolism during pregnancy and puerperium.Anticoagulation is recommended to be the first line therapy.For women with high risk of thromboembolism,it is suggested to prevent actively and treat early to decrease the complications and long-term sequelea. Key words: Venous thromboembolism; Pregnancy complications; Postpartum period

Markers of susceptibility to acute rheumatic fever: the B-cell antigen D8/17 is not robust as a marker in South Africa

Acute rheumatic fever and its chronic sequelae, rheumatic cardiac disease, and neuropsychiatric movement disorders, remain major public health problems in South Africa. Early identification and treatment of streptococcal pharyngitis in susceptible individuals would prevent rheumatic cardiac disease. The B-cell antigen D8/17 is a marker of susceptibility to rheumatic fever in some populations. We assessed the significance of the D8/17 marker in a group of South Africans. Blood was collected from 107 individuals; 40 patients had previous confirmed rheumatic fever, 20 were first-degree relatives, and 47 were controls. The expression of D8/17 in each sample was analysed by flow cytometry. The mean proportion of B-cells that were D8/17 positive was 0.5% in the index cases, 0.47% in their relatives, and 0.27% in the controls. There was a significant difference between the index cases and the controls, p = 0.03, but the mean percentage positive in each group was very low. Patients with a history of rheumatic fever had statistically increased expression of the D8/17 marker. However, the actual percentages in this observational study were markedly lower than in other populations, ranging from 0.14%-1.53% compared to 11.6%-39.3%. The D8/17 marker would be an impractical screening tool in the South African population.

Intracellular Cardiac Amyloid - like Deposition in a Young Girl

Herein we report a significant observation in 12 year old female, clinically diagnosed as a case of restrictive cardiomyopathy. During the histological study of postmortem examination of explanted heart, our attention was diverted to histochemical reaction that demonstrated the presence of a substance within cardiomyocytes of both the atria. Ultra structurally and immunohistochemically it was suggestive of amyloidosis, but in the absence of a positive Congo red stain (CR) and apple-green birefringence, the diagnosis remains unconfirmed. Although rare, atypical intracellular cardiac amyloidosis such as the familial amyloidotic polyneuropathy of the Portuguese type has been documented. Amyloidosis acquired secondary to chronic inflammatory disease like rheumatic cardiac disease is also well known. Herein we report a rare intracellular cardiac amyloid-like deposit in both atria. This is unlike normal Periodic Acid Schiff’s (PAS) positive glycoprotein deposition present in cardiac conducting tissue.

Oxidative stress in chronic cardiopathy associated with Chagas disease

Background Chagas disease is characterized by chronic inflammation similar to autoimmune diseases. The imbalance between prooxidant and antioxidant defenses is known as oxidative stress, and is often associated with inflammatory processes of many diseases. Methods The present work measured some components of the antioxidant system present in the blood of four groups of patients in different stages of chronic Chagas heart disease ( n = 10 each group), according to the modified Los Andes clinical-hemodynamic classification. Each group of chagasic patients was compared with four groups of healthy subjects, and chagasic group IV was compared to group V, characterized by rheumatic cardiac disease, giving a total of 90 subjects. Antioxidant enzymes including superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), glutathione reductase (GR), glutathione S-transferase (GST), and the contents of reduced (GSH), oxidized (GSSG) and total glutathione (TG) were assayed. Results SOD, CAT and GR activities showed essentially no differences among the four groups, however, intraerythrocytic GSH concentrations showed decreased values parallel to the progression of the disease. In addition, the glutathione peroxidase (GPx) activities were higher in groups III and V and lower in groups I, II and IV, also suggesting an antioxidant depletion with progression of the disease. In contrast, except for SOD and CAT activities, patients from group V showed a generally higher antioxidant capacity compared to the chagasic group IV. Conclusion The results indicate a general increase of oxidative stress parallel to the progression of Chagas disease.

Surgical pathology of rheumatic cardiac disease in children

Surgical pathology of rheumatic cardiac disease in children

CYTOTOXIC LYMPHOCYTE ACTIVITY IN RHEUMATIC HEART DISEASE (RHD)

Decreased peripheral blood lymphoproliferative response due to dysfunctional non-T lymphocytes and the presence of unusual antigenie structures on peripheral blood non-T cells are among the alterations in the immune system found in patients with rheumatic cardiac valvular disease. The present study examined natural killer cell activity and generation of cytotoxic lymphocytes after exposure to bklastogen A in patients with RHD and age- and socioeconomic-matched controls with recurrent tonsillitis but no rheumatic history, signs or symptoms. Peripheral blood mononuclear cells (PBMNC) were obtained by isopycnic centrifugation. Fresh PBMNC were incubated with 4 concentrations of K562 erythroleukemia cell targets. Rates of target cell lysis were measured by assay of lactate dehydrogenase using a spectrophotometric assay. Natural cytotoxic activity of freshly isolated PBMNC did not differ between RHD and controls. However, the PBMNC from RHD showed significantly less cytotoxicity after they were cultured alone in media for 6 days. In marked contrast, when cultured for 6 days in the presence of streptococcal blastogen A, PBMNCs from RHD showed greater rates of target cell lysis than controls at all target cell concentrations (P<.01 at 8 x 104 target cells). Thus, PBMNCs from RHD patients after exposure to a purified group A streptococcal product generate markedly greater than normal cytotoxicity against a target that displays differentiation antigens but not major histocompatibility antigens on its surface.

Severe rheumatic cardiac disease and pregnancy: The ultimate prognosis

All of 134 women classified as having “functionally severe” rheumatic cardiac disease and surviving pregnancies in 1931 through 1943 have been traced to 1975. Ninety-three percent have died at the exponential rate of 6.3% per year. The women with and without pregnancies following admittance to the series were similar in respect to age at entry, ages in which they lived, and proportionate representation in all but one of five subgroups with extraordinarily high death rates in the first five years of follow-up. Analyses of the data by life tables, average annual death rates, lengths of survival, and average ages at death failed to uncover any evidence for a delayed adverse effect of pregnancy in the whole series or in any of the subgroups. Provided that the patient survives the gestation, the life expectancy is not shortened by pregnancy.

Clinical use of a calcium antagonistic agent (nifedipine) in acute pulmonary edema

Nifedipine induces vascular smooth muscle relaxation through a calcium antagonistic action. The possibility of clinical use of the drug as a ventricular unloading agent has been explored in this study. In patients with hypertensive (seven cases), primary (seven cases) or rheumatic (aortic insufficiency five cases, mitral regurgitation five cases) cardiac disease, nifedipine, administered in a single sublingual dose (10 mg), relieved acute pulmonary edema. Circulatory variations from control were the following: decrease of systemic and pulmonary arterial pressures, and of vascular resistances, of pulmonary wedge pressure, of left ventricular diastolic and systolic dimensions (echocardiographyhy); increase of cardiac and stroke index, of left ventricular mean rate of circumferential fiber shortening, of left and right mean pre-ejection ΔP/Δt and mean rate of ejection; improvement of forward output in primary and rheumatic disease. Nifedipine benefits acute congestive heart failure by sustained fall of both preload and afterload and, possibly, by an enhanced contractility. It seems to have an appropriate indication in cases in which left ventricular afterload reduction is desirable.

The remote prognosis for pregnant women with rheumatic cardiac disease

The remote prognosis for pregnant women with rheumatic cardiac disease

Acute rheumatic fever: a study of 132 cases in young adults.

Excerpt The changing lights cast by new diagnostic aids and better therapy require that the classic concepts of the clinical picture of a disease be reexamined from time to time. The knowledge that...

MURMURS IN CHILDREN: A CLINICAL AND GRAPHIC STUDY IN 500 CHILDREN OF SCHOOL AGE

Excerpt A century ago, auscultation of the heart led to recognition of adventitious sounds and murmurs which were explained as the result of heart disease. Medical opinion has differed widely since...

Aschoff bodies in left auricular appendages of patients with mitral stenosis; clinicopathologic study, including postoperative follow-up.

IN RECENT years surgical valvulotomy for mitral stenosis has made it possible to examine microscopically the left auricular appendage and observe histologically the rheumatic process in a part of the heart during life. Since this is a phase in the natural history of rheumatic fever, it may be possible to correlate the incidence and degree of activity with the clinical events of rheumatic cardiac disease. Although the lesions of rheumatic fever have been described in the left auricle proper,* the auricular appendage has escaped more critical examination. Von Glahn, 8 in a study of rheumatic auricular endocarditis, concluded that the changes observed may extend into the first portion of the auricular appendage. He did not, however, record Aschoff bodies in this location. Similarly, Gross, 9 in an exhaustive review of rheumatic auricular lesions, made no reference to the auricular appendage. Unusual circumstances have on occasion prompted the making of sections of this

NEWS AND ANNOUNCEMENTS

The Committee on Rheumatic Fever and Cardiac Disease of the American Academy of Pediatrics has assisted in the organization of short courses in rheumatic fever and rheumatic heart disease for pediatricians. In response to this effort 2 special courses will be offered in the spring of 1953. Major emphasis will be given to the clinical aspects of the disease, a minimum of didactic lectures and a maximum opportunity for case studies, auscultation, fluoroscopy, interpretation of electrocardiograms, etc.