P.G. Herbst

Clinical features and outcome of lupus myocarditis in the Western Cape, South Africa

Background African American ethnicity is independently associated with lupus myocarditis compared with other ethnic groups. In the mixed racial population of the Western Cape, South Africa, no data exists on the clinical features/outcome of lupus myocarditis. Objectives The objective of this study was to give a comprehensive description of the clinical features and outcome of acute lupus myocarditis in a mixed racial population. Methods Clinical records (between 2008 and 2014) of adult systemic lupus erythematosus (SLE) patients at a tertiary referral centre were retrospectively screened for a clinical and echocardiographic diagnosis of lupus myocarditis. Clinical features, laboratory results, management and outcome were described. Echocardiographic images stored in a digital archive were reanalysed including global and regional left ventricular function. A poor outcome was defined as lupus myocarditis related mortality or final left ventricular ejection fraction (LVEF) <40%. Results Twenty-eight of 457 lupus patients (6.1%) met inclusion criteria: 92.9% were female and 89.3% were of mixed racial origin. Fifty-three per cent of patients presented within three months after being diagnosed with SLE. Seventy-five per cent had severely active disease (SLE disease activity index ≥ 12) and 67.9% of patients had concomitant lupus nephritis. Laboratory results included: lymphopenia (69%) and an increased aRNP (61.5%). Treatment included corticosteroids (96%) and cyclophosphamide (75%); 14% of patients required additional immunosuppression including rituximab. Diastolic dysfunction and regional wall motion abnormalities occurred in > 90% of patients. LVEF improved from 35% to 47% (p = 0.023) and wall motion score from 1.88 to 1.5 (p = 0.017) following treatment. Overall mortality was high (12/28): five patients (17.9%) died due to lupus myocarditis (bimodal pattern). Patients who died of lupus myocarditis had a longer duration of SLE (p = 0.045) and a lower absolute lymphocyte count (p = 0.041) at diagnosis. LVEF at diagnosis was lower in patients who died of lupus myocarditis (p = 0.099) and in those with a persistent LVEF < 40% (n = 5; p = 0.046). Conclusions This is the largest reported series on lupus myocarditis. The mixed racial population had a similar prevalence, but higher mortality compared with other ethnic groups (internationally published literature). Patients typically presented with high SLE disease activity and the majority had concomitant lupus nephritis. Lymphopenia and low LVEF at presentation were of prognostic significance, associated with lupus myocarditis related mortality or a persistent LVEF < 40%.

Screening for rheumatic heart disease: is a paradigm shift required?

This focused review presents a critical appraisal of the World Heart Federation criteria for the echocardiographic diagnosis of rheumatic heart disease (RHD) and its performance in African RHD screening programmes. It identifies various logistical and methodological problems that negatively influence the current guideline’s performance. The authors explore novel RHD screening methodology that could address some of these shortcomings and if proven to be of merit, would require a paradigm shift in the approach to the echocardiographic diagnosis of subclinical RHD.

Inter-scallop separations of the posterior leaflet of the mitral valve: an important cause of ‘pathological’ mitral regurgitation in rheumatic heart disease screening

Summary The 2012 World Heart Federation (WHF) criteria for echocardiographic diagnosis of rheumatic heart disease (RHD) identify that the finding of ‘pathological’ mitral regurgitation (MR) in a screened individual increases the likelihood of detecting underlying RHD. Cases of isolated ‘pathological MR’ are thus identified as ‘borderline RHD’. A large-scale echocardiographic screening program (Echo in Africa) in South Africa has identified that inter-scallop separations of the posterior mitral valve leaflet (PMVL) can give rise to ‘pathological’ MR. The authors propose that this entity in isolation should be identified and excluded from the WHF ‘borderline RHD’ category. In this case report, we present two examples of ‘pathological’ MR related to inter-scallop separation from the Echo in Africa image database. We further provide screening tips for the accurate identification of this entity.

3D image of Inoue balloon inflation during mitral valvuloplasty

Mitral balloon valvuloplasty is an important intervention in the armory of the cardiologist, offering a safe and effective percutaneous option in the treatment of patients with mitral stenosis.

Evaluation of the SUNHEART Cardiology Outreach Programme

Introduction: The demand for advanced cardiac care and specialised interventions is on the increase and this results in bottlenecks and increased waiting times for patients who require advanced cardiac care. By decentralising cardiac care, and using a hub-and-spoke model, the SUNHEART Outreach Programme of cardiovascular care aims to improve access to advanced cardiac care in the Western Cape. Tygerberg Hospital is the central hub, with the fi rst spoke being Paarl Hospital. Objective: To determine the value of the SUNHEART Outreach Programme to the public health care system. Methods: An audit of patients accessing the Outreach Programme was performed for the period May 2013 - May 2014 and consequently compared to a historical cohort of patients accessing the health care system during the preceding 6 months, from October 2012 - April 2013. Access to advanced cardiac care was measured in time to initial evaluation, time to defi nitive diagnosis or intervention and patient compliance with appointments. The value to the health care system was also assessed by performing a cost analysis of transport of patients and health care workers, as well as compliance with appointments. We documented the spectrum of disease requiring advanced cardiac care to guide future interventions. Results: Data of 185 patients were included in the audit. Sixty four patients were referred to tertiary care from October 2012 - April 2013 and 121 patients were referred to the outreach facility from May 2013 - May 2014. There was a signifi cant reduction in waiting times with the median days to appointment of the historical cohort being 85 days compared to 18 days in the Outreach Programme cohort (p<0.01). Patient compliance with appointments was signifi cantly superior in the Outreach Programme cohort (90% vs. 56%: p<0.01). Valvular (36.5%) and ischaemic heart disease (35.5%) were the major pathologies requiring access to cardiac care services. Transport costs per patient treated was signifi cantly reduced in the outreach programme cohort (R118,09 vs. R308,77). Conclusion: Decentralisation of services in the form of an Outreach Programme, with a central hub, improves access to advanced cardiac care by decreasing waiting time, improving compliance with appointments and decreasing travel costs.

Post-traumatic, intrapulmonary arteriovenous fi stula: Diagnosis by trans-oesophageal echocardiography

CITATION: Van der Bijl, P., Herbst, P. G., Doubell, A. F. & Pecoraro, A. J. 2015. Post-traumatic, intrapulmonary arteriovenous fistula : diagnosis by trans-oesophageal echocardiography. SA Heart, 12(1):26-27, doi:10.24170/12-1-1731.

Patient profi le of a tertiary obstetric-cardiac clinic

Background: Cardiac disease is the most important medical cause of maternal mortality in South Africa. Management of women with cardiac disease in pregnancy is highly specialised and they should ideally be evaluated early in pregnancy and in a multidisciplinary fashion with the aim of formulating a perinatal management plan. In order to facilitate the effi cient management of these patients in the context of a large tertiary hospital in South Africa a combined obstetric-cardiac (O-C) clinic was established at Tygerberg Academic Hospital (TBH) in 2010. Objective: The purpose of this review is to describe the patient profi le of an obstetric-cardiac clinic in South Africa, specifi cally the TBH O-C clinic and to share the lessons learnt from establishing this clinic. Methods: Retrospective review performed at TBH, a referral centre in the Western Cape Province of South Africa. All women evaluated and/or managed at the Obstetric-Cardiac clinic between 10 August 2010 and 4 December 2012 were included. Results: There were 231 women, rheumatic heart disease (n=79; 34.2%) was the predominant cardiac disease followed by congenital heart disease (n=78; 33.8%), medical conditions (n=38; 16.4%) and previous peripartum cardiomyopathy (n=9; 3.9%). Eighty-two women (35.5%) were perceived to be extremely high risk and their entire pregnancies were managed in the Obstetric-Cardiac clinic. The most common RHD lesion was mitral regurgitation (34.2%) and mixed mitral valve disease (24.1%). The most frequent CHD was ventricular septal defects (n=27; 35%). Conclusions: The cardiac disease profi le of patients seen at this obstetric-cardiac clinic in a South African tertiary hospital refl ects a transition from the disease profi le of a typical developing country (high burden of rheumatic heart disease) to the disease profi le seen in a more developed country (high burden of congenital heart disease). This could indicate improved quality of socio-economic development and the health care system. The increasing complexity of cardiac pathology that has to be dealt with in pregnant patients presenting to a tertiary hospital requires close collaboration between the obstetrician, cardiologist, cardiac surgeon and anesthetist caring for these patients. A dedicated obstetric-cardiac clinic is a good model to utilise in a tertiary hospital when aiming to optimise the care of patients with cardiac disease in pregnancy.

Constrictive pericarditis: Haemodynamics in a nutshell

A 41-year-old male presented with predominantly right heart failure symptoms due to suspected constrictive pericarditis (CP).

Speckle tracking echocardiography in acute lupus myocarditis: comparison to conventional echocardiography

Aims Lupus myocarditis occurs in 5–10% of patients with systemic lupus erythematosus (SLE). No single feature is diagnostic of lupus myocarditis. Speckle tracking echocardiography (STE) can detect subclinical left ventricular dysfunction in SLE patients, with limited research on its utility in clinical lupus myocarditis. We report on STE in comparison to conventional echocardiography in patients with clinical lupus myocarditis. Methods and results A retrospective study was done at a tertiary referral hospital in South Africa. SLE patients with lupus myocarditis were included and compared to healthy controls. Echocardiographic images were reanalyzed, including global longitudinal strain through STE. A poor echocardiographic outcome was defined as final left ventricular ejection fraction (LVEF) <40%. 28 SLE patients fulfilled the criteria. Global longitudinal strain correlated with global (LVEF: r = −0.808; P = 0.001) and regional (wall motion score: r = 0.715; P < 0.001) function. In patients presenting with a LVEF ≥50%, global longitudinal strain (P = 0.023), wall motion score (P = 0.005) and diastolic function (P = 0.004) were significantly impaired vs controls. Following treatment, LVEF (35–47% (P = 0.023)) and wall motion score (1.88–1.5 (P = 0.017)) improved but not global longitudinal strain. Initial LVEF (34%; P = 0.046) and global longitudinal strain (−9.5%; P = 0.095) were lower in patients with a final LVEF <40%. Conclusions This is the first known report on STE in a series of patients with clinical lupus myocarditis. Global longitudinal strain correlated with regional and global left ventricular function. Global longitudinal strain, wall motion score and diastolic parameters may be more sensitive markers of lupus myocarditis in patients presenting with a preserved LVEF ≥50%. A poor initial LVEF and global longitudinal strain were associated with a persistent LVEF <40%. Echocardiography is a non-invasive tool with diagnostic and prognostic value in lupus myocarditis.

AB0606 Lupus Myocarditis in the Western Cape, South Africa: Analysis of Clinical and Echocardiographic Features

Background Lupus myocarditis (LM) is a serious manifestation of systemic lupus erythematosus (SLE). LM in patients of African American ethnicity has an increased prevalence and higher mortality compared to other ethnic groups. In the mixed racial population of the Western Cape, South Africa, no data exists on the clinical features and outcome of LM. Echocardiography is frequently used to support the diagnosis of LM. Speckle tracking (ST) is more sensitive than standard imaging in the detection of left ventricular (LV) dysfunction. Literature on the use of ST in patients with clinically evident LM is limited. Objectives To give a comprehensive description of the clinical and echocardiographic features of acute LM in a mixed racial population. Methods Clinical records (over 6 years) of adult SLE patients at a tertiary referral centre were retrospectively screened for a clinical and echocardiographic diagnosis of LM. Clinical features, laboratory results, management and outcome were described. Echocardiographic images stored in a digital archive were reanalysed (where views allowed), including LV regional wall motion abnormalities (RWMA) and longitudinal strain through ST. Results 28 patients (6.1%) met inclusion criteria: 92.9% were female and 89% were of mixed racial origin. 54% of patients presented with LM within 3 months after being diagnosed with SLE. Median SLE disease activity index was 17.5 (IQR:12.3-24) and 50% of patients had concomitant lupus nephritis. Laboratory results included: low complement (92.3%); urinary protein >0.5g/day (83%); increased aRNP (62%). Initial (at time of diagnosis) and most recent echocardiographic data are summarised in table 1. Treatment included corticosteroids (96%) and cyclophosphamide (75%); 14% of patients required additional immunosuppression. Clinical improvement occurred in 67% of patients (563 days, median); 2 patients relapsed. Though the median LV ejection fraction (LVEF) improved from 35 to 47%, reduced longitudinal strain and RWMA persisted in most patients (Table 1). Overall mortality was high (12/28): 5/28 (17.9%) died due to LM compared to 2/24 (8.3%) in another case series. Mortality due to LM and/or treatment related complications were 35.7% (10/28).Table 1 Initial echocardiogram (n=28) Most recent echocardiogram (n=19) Median Ratio of test done Median Ratio of test done (IQR) (%) (IQR) (%) Time (days) 0 390 (93–799) Increased LVIDa (cm) 5.2 (4.4–5.6) 11/28 (39) 4.8 (4–5.6) 5/19 (26) LVEFb 35% (26–46) 47% (37–50) RWMA present 24/24 (100) 16/18 (89) Decreased longitudinal strain 13/13 (100) 8/8 (100) IQR: interquartile range; LVID: left ventricular internal diameter; LVEF: left ventricular ejection fraction; RWMA: regional wall motion abnormalities.a Increased LVID >5.3cm;b LVEF: Mild impairment: 45–54%; moderate: 36–44%; severe: ≤35%. Conclusions This is the largest reported case series on LM. The mixed racial population had a similar prevalence, but higher mortality compared to other ethnic groups (published literature). An increased awareness towards an early diagnosis is essential, especially in recently diagnosed SLE patients with concomitant lupus nephritis. ST (not previously described in acute LM) and RWMA showed persistent LV dysfunction despite an improved LVEF and could be utilised as a sensitive diagnostic tool in LM. Disclosure of Interest None declared