The temporospatial epidemiology of rheumatic heart disease in Far North Queensland, tropical Australia 1997-2017; impact of socioeconomic status on disease burden, severity and access to care.

BACKGROUND: The incidence of rheumatic heart disease (RHD) among Indigenous Australians remains one of the highest in the world. Many studies have highlighted the relationship between the social determinants of health and RHD, but few have used registry data to link socioeconomic disadvantage to the delivery of patient care and long-term outcomes. METHODS: A retrospective study of individuals living with RHD in Far North Queensland (FNQ), Australia between 1997 and 2017. Patients were identified using the Queensland state RHD register. The Socio-Economic Indexes for Areas (SEIFA) Score-a measure of socioeconomic disadvantage-was correlated with RHD prevalence, disease severity and measures of RHD care. RESULTS: Of the 686 individuals, 622 (90.7%) were Indigenous Australians. RHD incidence increased in the region from 4.7/100,000/year in 1997 to 49.4/100,000/year in 2017 (p<0.001). In 2017, the prevalence of RHD was 12/1000 in the Indigenous population and 2/1000 in the non-Indigenous population (p<0.001). There was an inverse correlation between an area's SEIFA score and its RHD prevalence (rho = -0.77, p = 0.005). 249 (36.2%) individuals in the cohort had 593 RHD-related hospitalisations; the number of RHD-related hospitalisations increased during the study period (p<0.001). In 2017, 293 (42.7%) patients met criteria for secondary prophylaxis, but only 73 (24.9%) had good adherence. Overall, 119/686 (17.3%) required valve surgery; the number of individuals having surgery increased over the study period (p = 0.02). During the study 39/686 (5.7%) died. Non-Indigenous patients were more likely to die than Indigenous patients (9/64 (14%) versus 30/622 (5%), p = 0.002), but Indigenous patients died at a younger age (median (IQR): 52 (35-67) versus 73 (62-77) p = 0.013). RHD-related deaths occurred at a younger age in Indigenous individuals than non-Indigenous individuals (median (IQR) age: 29 (12-58) versus 77 (64-78), p = 0.007). CONCLUSIONS: The incidence of RHD, RHD-related hospitalisations and RHD-related surgery continues to rise in FNQ. Whilst this is partly explained by increased disease recognition and improved delivery of care, the burden of RHD remains unacceptably high and is disproportionately borne by the socioeconomically disadvantaged Indigenous population.

Adult and Paediatric Cardiac Intervention in Timor-Leste: Disease Burden, Demographics and Clinical Outcomes.

BACKGROUND: The East Timor Hearts Fund (ETHF) is a charitable organisation of Australian cardiologists providing outreach screening in Timor-Leste. For patients requiring intervention, ETHF arranges logistics, procedures, and postoperative care. The aim of this project is to evaluate outcomes of patients requiring intervention. METHODS: The ETHF database of all patients was utilised to identify patients with disease warranting surgical or percutaneous intervention. Both patients who underwent intervention and those who did not proceed to intervention were included in this study. Patients who had intervention arranged by other organisations but have then had follow-up with ETHF were also included. Overall demographics and pre and postoperative factors were assessed, with sub-group analysis of adult and paediatric patients to identify any differences in care. RESULTS: Of 221 patients requiring intervention, 101 patients underwent intervention, receiving 22 different operations or procedures. Patients were predominantly young (median age 17.5 years) and female (64.7%), with rheumatic heart disease (63.8%). Twenty-four (24) (33.3%) women aged 15-45 years old with cardiac disease warranting intervention were documented as pregnant or breastfeeding at time of clinic assessment. Of patients who did not proceed to intervention, adults were more likely to be lost to follow-up (42.4% vs 18.5%) while paediatric patients were more likely to experience progression of disease (18.5% vs 7.5%, p=0.005). Median waitlist time was 5 months, with no significant difference between adults and children, correlating with a preoperative mortality rate of 5.4%. For patients who underwent intervention, post-procedure mortality was extremely low (0.9%) and attendance of at least one post-procedure review was excellent (99.0%). Eleven (11) (10.9%) patients have required repeat intervention, with no difference in rates between adult and paediatric patients. Length of follow-up extends up to 20 years for some patients. CONCLUSION: The Timor-Leste interventional cohort was predominantly a young female population with rheumatic and congenital cardiac disease. There were also high rates of pregnancy amongst female patients with severe cardiac disease. Delayed access to intervention may result in preoperative adverse events and mortality, and is a key target for improvement. Patients who undergo intervention have very low post-procedural mortality, good adherence to early medical follow-up and good long-term outcomes.

Public Health Costs for Northern Territory and South Australian Cardiac Surgery Patients.

BACKGROUND: The cost of performing cardiac surgery in the public health system in Australia is unclear. This paper analyses the cost of cardiac surgery performed at Flinders Medical Centre (FMC), South Australia, comparing cost by procedure, rheumatic valvular heart disease status, Aboriginality and location. METHODS: This study is a retrospective, population-based analysis of cardiac surgery data held in the Cardiac Surgery Registry cross-referenced to cost data provided by the FMC Department of Finance and Patient Travel, Accommodation and Transport Services at the Royal Darwin Hospital. Seven hundred ninety-five (795) patients who underwent cardiac surgery at FMC from 1 July 2014 to 30 June 2016 were included. RESULTS: Across all procedures, Northern Territory (NT) Aboriginal patients had a mean total cost of $78,506 which was $24,113 more than NT non-Aboriginal, $28,443 more than South Australian (SA) Aboriginal and $22,955 more than SA non-Aboriginal patients. The total cost of a patient undergoing a repeat sternotomy (reoperative procedure) was found to be significantly higher than a primary procedure ($85,797 versus $59,097). In patients undergoing valve surgery procedures, those identified with rheumatic heart disease had a higher mean total cost than those without (a difference of $25,094). Significantly, the rheumatic patient group showed a higher proportion of reoperative procedures (19% versus 5%). CONCLUSIONS: The cost of treating NT Aboriginal cardiac surgical patients remotely has a significant financial impact upon the health care delivery system, as does the impact of rheumatic heart disease. This study found that the cost for the NT Aboriginal patient group was substantially higher than the NT non-Aboriginal, SA Aboriginal and SA non-Aboriginal patient groups. The additional cost to family and dislocation of social structures is not able to be calculated, but would also clearly weigh heavily on both patient groups. These findings suggest that future health funding models should recognise Aboriginality, remoteness and rheumatic heart disease.

Ten-year clinical experience of humanitarian cardiothoracic surgery in Rwanda: Building a platform for ultimate sustainability in a resource-limited setting.

OBJECTIVE: Despite its near complete eradication in resource-rich countries, rheumatic heart disease remains the most common acquired cardiovascular disease in sub-Saharan Africa. With a ratio of physicians/population of 1 per 10,500, including only 4 cardiologists for a population of 11.4 million, Rwanda represents a resource-limited setting lacking the local capacity to detect and treat early cases of strep throat and perform lifesaving operations for advanced rheumatic heart disease. Humanitarian surgical outreach in this region can improve the delivery of cardiovascular care by providing sustainability through mentorship, medical expertise, training, and knowledge transfer, and ultimately the creation of a cardiac center. METHODS: We describe the experience of consecutive annual visits to Rwanda since 2008 and report the outcomes of a collaborative approach to enable sustainable cardiac surgery in the region. The Ferrans and Powers Quality of Life Index tool's Cardiac Version (http://www.uic.edu/orgs/qli/) was administered to assess the postoperative quality of life. RESULTS: Ten visits have been completed, performing 149 open procedures, including 200 valve implantations, New York Heart Association class III or IV, with 4.7% 30-day mortality. All procedures were performed with the participation of local Rwandan personnel, expatriate physicians, nurses, residents, and support staff. Early complications included cerebrovascular accident (n = 4), hemorrhage requiring reoperation (n = 6), and death (n = 7). Quality of life was assessed to further understand challenges encountered after cardiac surgery in this resource-limited setting. Four major domains were considered: health and functioning, social and economic, psychologic/spiritual, and family. The mean total quality of life index was 20.79 ± 4.07 on a scale from 0 to 30, for which higher scores indicated higher quality of life. Women had significantly lower "social and economic" subscores (16.81 ± 4.17) than men (18.64 ± 4.10) (P < .05). Patients who reported receiving their follow-up care in rural health centers also had significantly lower "social and economic" subscores (15.67 ± 3.81) when compared with those receiving follow-up care in urban health facilities (18.28 ± 4.16) (P < .005). Value afforded to family and psychologic factors remained high among all groups. Major postsurgical challenges faced included barriers to follow-up and systemic anticoagulation. CONCLUSIONS: This report represents the first account of a long-term humanitarian effort to develop sustainability in cardiac surgery in a resource-limited setting, Rwanda. With the use of volunteer teams to deliver care, transfer knowledge, and mentor local personnel, the results demonstrate superior outcomes and favorable indices of quality of life. The credibility gained over a decade of effort has created the opportunity for a partnership with Rwanda to establish a dedicated center of cardiac care to assist in mitigating the burden of cardiovascular disease throughout sub-Saharan Africa.

Assessment of trans-aortic pressure gradient using a coronary pressure wire in patients with mechanical aortic and mitral valve prostheses.

Accurate evaluation of trans-aortic valvular pressure gradients is challenging in cases where dual mechanical aortic and mitral valve prostheses are present. Non-invasive Doppler echocardiographic imaging has its limitations due to multiple geometric assumptions. Invasive measurement of trans-valvular gradients with cardiac catheterization can provide further information in patients with two mechanical valves, where simultaneous pressure measurements in the left ventricle and ascending aorta must be obtained. Obtaining access to the left ventricle via the mitral valve after a trans-septal puncture is not feasible in the case of a concomitant mechanical mitral valve, whereas left ventricular apical puncture technique is associated with high procedural risks. Retrograde crossing of a bileaflet mechanical aortic prosthesis with standard catheters is associated with the risk of catheter entrapment and acute valvular regurgitation. In these cases, the assessment of trans-valvular gradients using a 0.014˝ diameter coronary pressure wire technique has been described in a few case reports. We present the case of a 76-year-old female with rheumatic valvular heart disease who underwent mechanical aortic and mitral valve replacement in the past. She presented with decompensated heart failure and echocardiographic findings suggestive of elevated pressure gradient across the mechanical aortic valve prosthesis. The use of a high-fidelity 0.014˝ diameter coronary pressure guidewire resulted in the detection of a normal trans-valvular pressure gradient across the mechanical aortic valve. This avoided a high-risk third redo valve surgery in our patient. © 2017 Wiley Periodicals, Inc.

Thrombocytopenia as a Preoperative Risk Assessment Tool in Patients With Rheumatic Heart Disease Undergoing Valve Replacement Surgery.

BACKGROUND: Postoperative thrombocytopenia has been reported to be correlated with adverse events, but the prognostic value of baseline thrombocytopenia is unclear. This study was undertaken to evaluate the relationship between preoperative thrombocytopenia and adverse outcomes in patients with rheumatic heart disease who underwent valve replacement surgery. METHODS AND RESULTS: A total of 1789 patients with rheumatic heart disease undergoing valve replacement surgery were consecutively enrolled and postoperatively followed up for 1 year. Patients were stratified on the basis of presence (n=495) or absence (n=1294) of thrombocytopenia (platelet count, <150×109/L), according to hospital admission platelet counts. During the hospitalization period, 69 patients (3.9%) died. The in-hospital all-cause mortality rate was significantly higher in the thrombocytopenic group (6.9% versus 2.7%; P<0.001). Multivariate analyses revealed that thrombocytopenia was independently associated with in-hospital all-cause mortality (odds ratio, 2.21; 95% confidence interval, 1.29-3.80; P=0.004). Platelet counts could predict in-hospital all-cause mortality for patients both with and without previous atrial fibrillation (areas under the curve, 0.708 [P<0.001] and 0.610 [P=0.025], respectively). One-year survival was significantly lower in patients with thrombocytopenia compared with controls (91.3% versus 96.1%; log-rank=14.65; P<0.001). In addition, thrombocytopenia was an independent predictor for postoperative 1-year all-cause mortality in multivariate Cox regression analysis. CONCLUSIONS: Platelet counts, as simple and inexpensive indexes, were reliable to be used as a preoperative risk assessment tool for patients with rheumatic heart disease undergoing valve replacement surgery.

A Cost-Effectiveness Tool to Guide the Prioritization of Interventions for Rheumatic Fever and Rheumatic Heart Disease Control in African Nations.

BACKGROUND: Rheumatic heart disease (RHD) prevalence and mortality rates remain especially high in many parts of Africa. While effective prevention and treatment exist, coverage rates of the various interventions are low. Little is known about the comparative cost-effectiveness of different RHD interventions in limited resource settings. We developed an economic evaluation tool to assist ministries of health in allocating resources and planning RHD control programs. METHODOLOGY/PRINCIPAL FINDINGS: We constructed a Markov model of the natural history of acute rheumatic fever (ARF) and RHD, taking transition probabilities and intervention effectiveness data from previously published studies and expert opinion. Our model estimates the incremental cost-effectiveness of scaling up coverage of primary prevention (PP), secondary prevention (SP) and heart valve surgery (VS) interventions for RHD. We take a healthcare system perspective on costs and measure outcomes as disability-adjusted life-years (DALYs), discounting both at 3%. Univariate and probabilistic sensitivity analyses are also built into the modeling tool. We illustrate the use of this model in a hypothetical low-income African country, drawing on available disease burden and cost data. We found that, in our hypothetical country, PP would be cost saving and SP would be very cost-effective. International referral for VS (e.g., to a country like India that has existing surgical capacity) would be cost-effective, but building in-country VS services would not be cost-effective at typical low-income country thresholds. CONCLUSIONS/SIGNIFICANCE: Our cost-effectiveness analysis tool is designed to inform priorities for ARF/RHD control programs in Africa at the national or subnational level. In contrast to previous literature, our preliminary findings suggest PP could be the most efficient and cheapest approach in poor countries. We provide our model for public use in the form of a Supplementary File. Our research has immediate policy relevance and calls for renewed efforts to scale up RHD prevention.

[Changes in biochemical parameters of blood in patients with rheumatic heart failure following surgical intervention].

We analysed changes of biochemical parameters of blood in patients treated for chronic rheumatic heart problems. It was shown that biochemical parameters of blood changed within 12 days after surgical intervention; the changes were especially pronounced on days 1-3 whereas normalization occurs by days 9-12. Pathogenetically sound time periods for changes of biochemical characteristics of blood serum were established in the patients treated for chronic rheumatic heart problems. On the one hand, it will allow more accurate monitoring of development of complications in the postoperative period and on the other hand to reduce expenses by decreasing the number of unnecessary analyses.

Partnership for sustainability in cardiac surgery to address critical rheumatic heart disease in sub-Saharan Africa: the experience from Rwanda.

IMPORTANCE: Rheumatic heart disease (RHD) in the developing world results in critical disability among children, adolescents, and young adults-marginalizing a key population at its peak age of productivity. Few regions in sub-Saharan Africa have independently created an effective strategy to detect and treat streptococcal infection and mitigate its progression to RHD. OBJECTIVE: We describe a unique collaboration, where the Rwanda Ministry of Health, the Rwanda Heart Foundation, and an expatriate humanitarian cardiac surgery program have together leveraged an innovative partnership as a means to expand Rwanda's current capacity to address screening and primary prevention, as well as provide life-saving cardiac surgery for patients with critical RHD. EVIDENCE REVIEW: Interviews with key personnel and review of administrative records were conducted to obtain qualitative and quantitative data on the recruitment of clinical personnel, procurement of equipment, and program finances. The number of surgical cases completed and the resultant clinical outcomes are reviewed. FINDINGS: From 2008 to 2013, six annual visits were completed. A total of 128 prosthetic valves have been implanted in 86 complex patients in New York Heart Association (NYHA) class III or IV heart failure, with excellent clinical outcomes (5 % 30-day mortality). Postoperative complications included a cerebrovascular accident (n = 1) and hemorrhage, requiring reoperation (n = 2). All procedures were performed with participation of local personnel. CONCLUSIONS AND RELEVANCE: This strategy provides a reliable and consistent model of sophisticated specialty care delivery; inclusive of patient-centered cardiac surgery, mentorship, didactics, skill transfer, and investment in a sustainable cardiac program to address critical RHD in sub-Saharan Africa.

The challenges of managing rheumatic disease of the mitral valve in Jamaica.

Between January, 2009 and December, 2013, 84 patients were identified who underwent isolated mitral valve surgery in Jamaica at The University Hospital of the West Indies and The Bustamante Hospital for Children. The most common pathology requiring surgery was rheumatic heart disease, accounting for 84% of the procedures performed. The majority of patients had regurgitation of the mitral valve (67%), stenosis of the mitral valve (22%), and mixed mitral valve disease (11%). The most common procedure performed was replacement of the mitral valve (69%), followed by mitral valve repair (29%). Among the patients, one underwent closed mitral commissurotomy. The choice of procedure differed between age groups. In the paediatric population (<18 years of age), the majority of patients underwent repair of the mitral valve (89%). In the adult population (18 years and above), the majority of patients underwent mitral valve replacement (93%). Overall, of all the patients undergoing replacement of the mitral valve, 89% received a mechanical valve prosthesis, whereas 11% received a bioprosthetic valve prosthesis. Of the group of patients who underwent mitral valve repair for rheumatic heart disease, 19% required re-operation. The average time between initial surgery and re-operation was 1.2 years. Rheumatic fever and rheumatic heart disease remain significant public health challenges in Jamaica and other developing countries. Focus must remain on primary and secondary prevention strategies in order to limit the burden of rheumatic valvulopathies. Attention should also be directed towards improving access to surgical treatment for young adults.

Comprehensive hemodynamic assessment of 368 normal St. Jude Medical mechanical mitral valve prostheses based on early postimplantation echocardiographic studies.

BACKGROUND: Two-dimensional and Doppler-derived echocardiographic data on normal St. Jude Medical mechanical mitral valve prosthesis function have been reported but remain limited. METHODS: Comprehensive retrospective two-dimensional and Doppler echocardiographic assessment of 368 normal St. Jude Medical mechanical mitral valve prostheses was performed early after implantation. The early postimplantation hemodynamic profiles of 98 patients were compared with profiles obtained by follow-up transthoracic echocardiography performed <13 months after implantation. RESULTS: Using mean ± 2 SDs to define the normal distribution of values for Doppler-derived hemodynamic variables, the calculated normal ranges of values were as follows: mean gradient, 2 to 7 mm Hg; peak early mitral diastolic velocity (E velocity), 1.1 to 2.4 m/sec; time-velocity integral of the mitral valve prosthesis (TVIMVP) 20 to 50 cm; ratio of the TVIMVP to the time-velocity integral of the left ventricular outflow tract (TVILVOT), 0.9 to 2.5; pressure half-time, 35 to 99 msec; and effective orifice area, 1.12 to 3.24 cm(2). Patients with severe prosthesis-patient mismatch (ie, indexed effective orifice area ≤ 0.9 cm(2)/m(2)) had significantly higher mean gradients, E velocity, TVIMVP, and TVIMVP/TVILVOT. There was a trend for longer pressure half-times for patients with severe prosthesis-patient mismatch than for patients without severe prosthesis-patient mismatch, but none of these patients had pressure half-times > 130 msec. Among the 98 patients with follow-up transthoracic echocardiography <1 year after implantation, no significant differences were observed between early postimplantation findings and follow-up hemodynamic profiles. CONCLUSIONS: This study establishes parameters (mean ± 2 SDs) defining the distribution of values for Doppler-derived hemodynamic data with normal St. Jude Medical mechanical mitral valve prostheses. Prostheses with hemodynamic values outside these parameters are likely dysfunctional; however, prosthesis dysfunction may be present even when hemodynamic values are within these ranges.

Valvulopathies in sub-Saharan African children: patterns, humanitarian interventions and cardiac surgical problems.

Despite the high burden of rheumatic fever in sub-Saharan African, there is currently no sustained and comprehensive strategy to control the disease. Consequently in this area the number of patients affected by rheumatic valve disease (RVD), most with a surgical indication, is 10-20 fold higher than in industrialised countries and estimates indicate that more than 50% of African RVD patients will die before age 25. In this paper, we review clinical and management issues of RVD in children in sub-Saharan Africa. Severe heart failure and undergrowth are the prevalent presentation of the illness. Severe mitral regurgitation is the commonest rheumatic valvulopathy observed in the first and second decades. Valve repair, the approach of choice, may be associated with unfavourable outcomes in patients with extreme cardiomegaly. In young people, whenever correct anticoagulation may reasonably be achieved, mechanical mitral prostheses should be preferred, even in females. The early deterioration of biologic mitral prostheses strongly suggests limiting their use to those cases in which correct anticoagulation is not feasible. In most sub-Saharan countries, socioeconomic factors strongly limit access to health services and to cardiac surgery in particular. Efforts to overcome these barriers have resulted in humanitarian projects along two patterns: creation of high tech on site health care structures or transfer of children with complex diseases to receive highly specialised cardiac surgical care abroad. We summarise the experience of our programme that followed the latter approach.

Early cardiac morbidity of rheumatic fever in children in New Zealand.

AIM: The aim of this study was to review the severity and morbidity of acute rheumatic fever (ARF) and rheumatic heart disease (RHD) for children with the most significant cardiac disease in the current era in New Zealand. METHOD: Retrospective 2-year review of children with ARF and RHD admitted to Starship Children's cardiology ward. Medical and surgical admissions were classified. Echocardiographic severity of cardiac disease and cardiac surgical data were analyzed. Using length of stay data and 2009 District Health Board costings, admission costs were calculated. RESULTS: 36 children had 49 admissions. Mean age 11.8 plus or minus 2.4 years. All but one child was of Maori or Pacific Island ethnicity. 10 children had symptoms and signs of congestive cardiac failure on admission. The average length of stay was 23 days, but the subset of children with ARF requiring cardiac surgery at the same admission had an average of 54 days (range 36-78 days) in hospital. The total hospital costs over the 2-year period was $1,918,600. CONCLUSION: Failure to prevent rheumatic fever in New Zealand means that there is significant cardiac sequelae for those children who develop severe RHD. The early morbidity includes heart failure, need for cardiac surgery, and prolonged hospital stay.