Inter-rater and intra-rater reliability and agreement of echocardiographic diagnosis of rheumatic heart disease using the World Heart Federation evidence-based criteria.

OBJECTIVE: Different definitions have been used for screening for rheumatic heart disease (RHD). This led to the development of the 2012 evidence-based World Heart Federation (WHF) echocardiographic criteria. The objective of this study is to determine the intra-rater and inter-rater reliability and agreement in differentiating no RHD from mild RHD using the WHF echocardiographic criteria. METHODS: A standard set of 200 echocardiograms was collated from prior population-based surveys and uploaded for blinded web-based reporting. Fifteen international cardiologists reported on and categorised each echocardiogram as no RHD, borderline or definite RHD. Intra-rater and inter-rater reliability was calculated using Cohen's and Fleiss' free-marginal multirater kappa (κ) statistics, respectively. Agreement assessment was expressed as percentages. Subanalyses assessed reproducibility and agreement parameters in detecting individual components of WHF criteria. RESULTS: Sample size from a statistical standpoint was 3000, based on repeated reporting of the 200 studies. The inter-rater and intra-rater reliability of diagnosing definite RHD was substantial with a kappa of 0.65 and 0.69, respectively. The diagnosis of pathological mitral and aortic regurgitation was reliable and almost perfect, kappa of 0.79 and 0.86, respectively. Agreement for morphological changes of RHD was variable ranging from 0.54 to 0.93 κ. CONCLUSIONS: The WHF echocardiographic criteria enable reproducible categorisation of echocardiograms as definite RHD versus no or borderline RHD and hence it would be a suitable tool for screening and monitoring disease progression. The study highlights the strengths and limitations of the WHF echo criteria and provides a platform for future revisions.

Incorporating selected non-communicable diseases into facility-based surveillance systems from a resource-limited setting in Africa.

BACKGROUND: As Mozambique faces a double burden of diseases, with a rise of Non Communicable Diseases (NCD) superimposed to uncontrolled communicable diseases (CD), routine disease surveillance system does not include NCD. The objectives of our study were to i) upgrade of the current surveillance system by adapting the data collection tools to NCD; ii) describe the occurrence and profile of selected NCD using these data collection tools. METHODS: Workshops were implemented in a first referral urban hospital of Mozambique to train clinical staff, administrative workers and nurses on NCD surveillance, as well as select conditions to be prioritized. Based on the WHO Global Action Plan and Brazaville Declaration for NCD prevention and control, we selected arterial hypertension, diabetes, stroke, chronic respiratory diseases, mental illness and cancers. Data collection tools used for CD were changed to include age, gender, outcome and visit type. Between February/2014 and January/2015 we collected data at an urban hospital in Mozambique's capital. RESULTS: Over 12 months 92,018 new patients were assisted in this hospital. Data was missing or diagnosis was unreadable in 2637 (2.9%) thus only 89,381 were used for analysis; of these 6423 (median age 27 years; 58.4% female) had at least one selected NCD as their primary diagnosis: arterial hypertension (2397;37.31%), mental illness (1497;23.30%), asthma (1495;23.28%), diabetes (628;9.78%), stroke (299;4.66%), chronic obstructive pulmonary disease 61 (0.95%) and cancers 46 (0.72%). Emergency transfers were needed for 76 patients (1.2%), mainly due to hypertensive emergencies (31; 40.8%) and stroke (18;23.7%). Twenty-four patients died at entry points (0.3%); 10 of them had hypertensive emergencies. CONCLUSION: Changes in existing surveillance tools for communicable diseases provided important data on the burden and outcomes of the selected NCD helping to identify priority areas for training and health care improvement. This information can be used to design the local NCD clinics and to strengthen the health information system in resource-limited settings in a progressive and sustainable way.

Active schistosomiasis, severe hypereosinophilia and rapid progression of chronic endomyocardial fibrosis.

Endomyocardial fibrosis (EMF) is a neglected restrictive cardiomyopathy of unknown aetiology and unclear natural history, which causes premature deaths in endemic areas. We present the case of a 13-year-old boy from a highly endemic area, presenting with concurrent signs of chronic EMF and severe hypereosinophilia associated with active schistosomal cystitis. We discuss the possible role of this parasitic infection in determining the progression of EMF in endemic areas for both conditions.

Medical disease as a cause of maternal mortality: the pre-imminence of cardiovascular pathology.

Maternal mortality ratio in low- to middle-income countries (LMIC) is 14 times higher than in high-income countries. This is partially due to lack of antenatal care, unmet needs for family planning and education, as well as low rates of birth managed by skilled attendants. While direct causes of maternal death such as complications of hypertension, obstetric haemorrhage and sepsis remain the largest cause of maternal death in LMICs, cardiovascular disease emerges as an important contributor to maternal mortality in both developing countries and the developed world, hampering the achievement of the millennium development goal 5, which aimed at reducing by three-quarters the maternal mortality ratio until the end of 2015. Systematic search for cardiac disease is usually not performed during pregnancy in LMICs despite hypertensive disease, rheumatic heart disease and cardiomyopathies being recognised as major health problems in these settings. New concern has been rising due to both the HIV/AIDS epidemic and the introduction of highly active antiretroviral therapy. Undetected or untreated congenital heart defects, undiagnosed pulmonary hypertension, uncontrolled heart failure and complications of sickle cell disease may also be important challenges. This article discusses issues related to the role of cardiovascular disease in determining a substantial portion of maternal morbidity and mortality. It also presents an algorhitm to be used for suspected and previously known cardiac disease in pregnancy in the context of LIMCs.

Development of the roadmap and guidelines for the prevention and management of high blood pressure in Africa: Proceedings of the PASCAR Hypertension Task Force meeting: Nairobi, Kenya, 27 October 2014.

Africa has one of the fastest growing economies in the world. The economic changes are associated with a health transition characterised by a rise in cardiovascular risk factors and complications, which tend to affect the African population at their age of maximum productivity. Recent data from Africa have highlighted the increasing importance of high blood pressure in this region of the world. This condition is largely underdiagnosed and poorly treated, and therefore leads to stroke, renal and heart failure, and death. Henceforth, African countries are taking steps to develop relevant policies and programmes to address the issue of blood pressure and other cardiovascular risk factors in response to a call by the World Health Organisation (WHO) to reduce premature deaths from non-communicable diseases (NCDs) by 25% by the year 2025 (25 × 25). The World Heart Federation (WHF) has developed a roadmap for global implementation of the prevention and management of raised blood pressure using a health system approach to help realise the 25 × 25 goal set by the WHO. As the leading continental organisation of cardiovascular professionals, the Pan-African Society of Cardiology (PASCAR) aims to contextualise the roadmap framework of the WHF to the African continent through the PASCAR Taskforce on Hypertension. The Taskforce held a workshop in Kenya on 27 October 2014 to discuss a process by which effective prevention and control of hypertension in Africa may be achieved. It was agreed that a set of clinical guidelines for the management of hypertension are needed in Africa. The ultimate goal of this work is to develop a roadmap for implementation of the prevention and management of hypertension in Africa under the auspices of the WHF.

Secondary intracardiac Burkitt-like lymphoma in the absence of HIV infection.

Malignant lymphoma can involve the cardiac cavity or myocardium as a mass. Since clinical symptoms of its cardiac involvement are usually absent or non-specific, they may be undetected during life. Burkitt-like lymphoma (BLL) is a highly aggressive B-cell lymphoma with a high proliferative rate. Histopathological characteristics are considered borderline between those of classic Burkitt lymphoma and diffuse large B-cell lymphoma. Extensive cardiac involvement of BLL is rare and poorly documented in the literature. We report the case of a child with BLL, presenting with extensive infiltration of the heart in the absence of HIV infection, and with right-sided heart failure and positional dyspnoea as the major clinical problems. We highlight the challenges for diagnosis and adequate treatment in poor settings like ours.

Echocardiography accurately assesses the pathological abnormalities of chronic endomyocardial fibrosis.

Endomyocardial fibrosis (EMF) is a cardiomyopathy with high prevalence in Sub-Saharan Africa with unclear etiology, pathogenesis and natural history. Most pathological abnormalities can potentially be diagnosed by echocardiography allowing surgery in some cases. With increasing availability of echocardiography in endemic areas for EMF we designed a study aiming at assessing its accuracy in defining EMF structural abnormalities pre-operatively, and describe pathological findings through detailed intraoperative examination and evaluation of histopathological changes in tissue obtained from excisional biopsies. Transthoracic echocardiography was performed pre-operatively to 29 patients with chronic severe EMF. All patients were submitted to excisional endomyocardial biopsies during surgery and careful standardized intra-operative evaluation was also done. Surgical and histopathological findings were compared to those obtained by echocardiography. Of the 29 patients studied, 16 had moderate lesions while 13 had severe disease. Their mean age was 12 (±4.6) years and 17 were males. All but one patient were in NYHA functional class III or IV at time of surgery. All patients had severe atrioventricular valve regurgitation with valves considered suitable for repair. We evaluated tissue from 25 left ventricles and 12 right ventricles. Endocardial thickening was the most prominent abnormality due to deposition of hyaline and cellular collagen underneath a layer of apparently normal endocardial endothelial cells. The mean endocardial thickness was 2,541 ± 1,707 µm. There was high agreement between echocardiographic and intraoperative findings, with the findings coinciding completely in 24 patients. Severe EMF assessed by echocardiography was associated with intense endocardial fibrosis on histology. In contrast, the presence of inflammation in peripheral blood was not associated with tissue inflammation. Structural abnormalities of chronic severe EMF are accurately diagnosed by transthoracic echocardiography, allowing this non-invasive technique to be used as the gold standard for diagnosis and surgical management of chronic EMF in endemic areas.

Deep-vein and intracardiac thrombosis of unclear aetiology: possible association with intermittent low-voltage electrical trauma.

Extensive deep-vein thrombosis (DVT) may result from a high-voltage electrical injury. Little is known about the effects of long-term, low-voltage electrical stimulation. We report on the unusual finding of deep-vein thrombosis in a black patient, affecting the superior vena cava and right femoral vein, in association with a right atrial mural thrombus, following low-voltage electrical stimulation of the body for analgesic purposes. The patient had slightly decreased blood levels of proteins C and S, with no other known risk factors. The diagnosis, management and prognosis are discussed. This case demonstrates the need to investigate the cardiovascular effects of chronic, low-voltage electrical stimulation.

Neglected Cardiomyopathies in Africa

"Cardiomyopathies are forms of disease in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease; hypertension; valve disease and congenital heart disease sufficient to explain he observed myocardial abnormality. They constitute the greatest challenge of all the cardiovascular diseases in Africa; due to the difficulties in diagnosing and managing them; related to the lack of access to specialised investigations or effective interventions in most endemic areas. In this continent; in addition to the ""usual"" forms of cardiomyopathy; there is an increased incidence of specific forms of muscle heart disease like Endomyocardial Fibrosis and Peripartum Cardiomyopathy which; despite affecting thousands of people; have been largely neglected. Their etiology remains unknown; the mechanisms involved are not fully nderstood; and although some improvement in management has been witnessed in recent years much still need to be done to improve the outcome. The current knowledge on epidemiology; determinants; thophysiology; diagnosis and management of Endomyocardial Fibrosis and Peripartum Cardiomyopathy is reviewed in this article; which also presents the ongoing efforts for better understanding of their pathogenesis and the recent improvements in their management."

[Geographic origin of endomyocardial fibrosis treated at the central hospital of Maputo (Mozambique) between 1987 and 1999]. / Origine géographique des cas de fibrose endomyocardique traités à l'hôpital central de Maputo (Mozambique) entre 1987 et 1999.

Endomyocardial Fibrosis (EMF) is a restrictive myocardiopathy of unknown aetiology, which is specific to tropical areas. In order to document the geographical distribution of this disease in Southern Mozambique, we reviewed the medical records of patients treated at Maputo Central Hospital between 1987 and 1999. Analysis was restricted to cases originating from the 3 provinces of Southern Mozambique. Cumulative incidences were computed for the period of 1987-1999, by age group, sex and residential district at disease onset. The geographical distribution of the disease in the 34 districts (administrative subdivisions) of the study area was mapped. Between 1987 and 1999, 118 cases of EMF originating from Maputo, Gaza and Inhambane provinces were diagnosed at Maputo Central Hospital. Seventy seven patients (65%) were living in Inhambane Province at the time of disease onset. Cumulative incidence for this province was 6.9/100,000 over the study period of 1987-1999. Comparatively, cumulative incidences were respectively 1.7/100,000 for Maputo and 1.0/100,000 for Gaza Province (chi 2 = 64.3, p < 10(-5)). All the patients were native from 22 out of the 34 districts of these 3 provinces. Affected districts were all located in the coastal area and higher cumulative incidence was computed for Inharrime district (18.3/100,000). This study confirmed that endomyocardial fibrosis is endemic in some areas of South Mozambique, but did not allow to measure the true magnitude of the disease which is probably grossly underestimated. The geographical distribution of EMF in South Mozambique reinforce the environmental hypothesis of aetiologic disease.