Management of congenital heart disease in the peripartum period: An illustrative case series

More women with complex congenital heart disease (CHD) reach adulthood resulting in a cohort of patients who are at high risk for adverse events during pregnancy. The haemodynamic changes usual to pregnancy may be poorly tolerated in patients with poor systemic ventricular function, cyanosis, left-sided obstructive lesions and pulmonary hypertension. Complex CHD patients are best managed by a multi-disciplinary team at a high-risk centre. Pre-conception counselling aims at risk stratifying by means of a clinical evaluation, electrocardiogram and echocardiography. Echocardiography plays a vital role in delineating the initial lesions and residual lesions with its haemodynamic complications. The modified WHO (mWHO) classification provides a helpful tool to stratify anatomical and physiological lesions by maternal and foetal event rates and is recommended by the European Society of Cardiology (ESC). Patients with cyanosis, severe aortopathy and severe pulmonary hypertension fall into Class IV and termination of pregnancy is advised. Patients may however choose to continue their pregnancy. We present 3 such cases of complex CHD (Fontan circulation, severe aortopathy and severe pulmonary hypertension) and illustrate some pertinent management principles in the peripartum period

Prevalence, characteristics and additional stroke risk stratification: an analysis of the Atrial Fibrillation cohort within the REMEDY study

Background: Atrial fibrillation (AF) is the most common arrhythmia and may be complicated by embolic stroke. It is also associated with a significant risk of heart failure and mortality. The burden of rheumatic heart disease remains great in the developing world. The prevalence of AF in those with rheumatic heart disease is in the order of 20% with a resultant 17-fold increased risk of embolic stroke. Over time, many other risk factors for stroke in the AF population have been described. Stroke risk stratification tools such as the CHADS₂ (Congestive heart failure, hypertension, age of 75 or older, diabetes mellitus or stroke/TIA) and CHA₂DS₂VASc (with the addition of a second age category, female gender, and peripheral artery disease) scores have been developed. These are used to assess the need for anticoagulation and have been well validated. These scores have traditionally excluded those patients with valvular AF. Valvular AF has not been studied extensively in the contemporary era. Oral anticoagulation had previously been advised in all patients with valvular AF. Little is known however about outcomes for stroke and mortality in this cohort of patients. Furthermore, the utilization of the CHADS₂ and CHA₂DS₂VASc scores may provide incremental benefit in prognostication and resultantly, both more diligent prescription of anticoagulation and improved outcomes. Objectives: The objectives of this study were as follows - 1. To determine the prevalence of AF in the Global Rheumatic Heart Disease Registry (the REMEDY study) and in the Groote Schuur Hospital (GSH) cohort. 2. To assess the demographic, social and clinical characteristics of patients with AF in the REMEDY study and in the GSH cohort. 3. To assess the frequency of CHADS₂ and CHA₂DS₂VASc risk factors in the GSH cohort and to calculate a CHADS₂ and CHA₂DS₂VASc score on each of the patients with AF. 4. To establish whether CHADS₂ and CHA₂DS₂VASc scores further increase the risk of stroke and death in this cohort of patients with valvular AF. Methods: This is a substudy of the Global Rheumatic Heart Disease Registry (the REMEDY study). We assessed those with AF from the entire cohort for prevalence and outcome data. Patients with ECG or Holter proven AF from the GSH cohort were further risk stratified using the CHADS₂ and CHA₂DS₂Vasc scores. Clinical data was obtained from folder reviews and telephonic interviews. The CHADS₂ and CHA₂DS₂Vasc scores for each patient in the GSH cohort were calculated. Patients were followed up for 2 years and information pertaining to death and stroke were obtained from folder reviews. These were then correlated with the CHADS₂ and CHA₂DS₂Vasc scores. Results: A total of 2624 REMEDY patients were analysed. Of these, 22% in the total cohort (586 of 2684 patients) and 38.2% in the GSH cohort (187 of 489 patients) had AF. These patients were older (35 years vs. 25 years, p<0.0001), more likely to be female (73.1% vs. 65.6%, p=0.001) and more frequently had a history of congestive heart disease (41.0% vs. 33.3%, p=0.001) when compared to those in sinus rhythm. They also had significantly more strokes (13.8% vs. 5%, p<0.0001) and a poorer NYHA class (NYHA III& IV 30.8% vs. 25.2%, p=0.002). The cohort with AF had more severely impaired left ventricular (LV) function compared to those in sinus rhythm (Ejection fraction (EF) 57% vs. 61%. P<0.0001). The presence of a larger left atrial (LA) size, spontaneous echo contrast and LA thrombus was much greater in the AF cohort. Of those patients in AF, only 68% had received a prescription for warfarin. The GSH cohort was risk stratified using the CHADS₂ and CHA₂DS₂VASc scores. Twenty-three percent of patients had a CHADS₂ score of 0 and 27.7% of 1. When the same cohort was scored using the CHA₂DS₂VASc score, only 5.4% had a score of 0; this difference was mainly driven by the additional category of female gender. The patients in our cohort were young (median age 28 years) and had few comorbidities. Despite this, patients with AF did significantly worse than those in sinus rhythm, with a stroke rate of 4.6% and a mortality rate of 13.1% observed at 2 years (compared to a 1.5% stroke rate and 5.5% mortality rate for those in sinus rhythm). The presence of any additional comorbidities significantly reduced survival in both the short and long term. Greater CHA₂DS₂VASc score categories (CHA₂DS₂VASc 1 and CHA₂DS₂VASc 2 or more) conferred an incrementally higher risk of death. Conclusion: In a contemporary cohort of patients with rheumatic heart disease, AF is common with a prevalence of 22-39%. These patients were older and exhibited features of more advanced disease both clinically and on echo, compared to their sinus rhythm counterparts. The mortality and stroke rates in the AF group were high despite the relatively young age of this cohort. Mortality and stroke increased significantly and incrementally with each greater CHA₂DS₂VASc score category. Given the differences in chronicity between RHD in the developed world (i.e., disease of older people) and RHD in developing countries (i.e., disease of the young), these results cannot be extrapolated to those living in the first world

Non-compliant left atrium masquerading as severe mitral regurgitation on cardiac catheterisation haemodynamics

A prominent v wave on a capillary wedge or left atrial pressure tracings classically signify significant mitral regurgitation. However, infiltrative disease involving the left atrium and left atrial scar from prior catheter ablation or Maze procedures, may produce a similar waveform due to noncompliance of the left atrium

COVID-19 and cardiovascular imaging: a guide for the practising clinician

In the ongoing COVID-19 pandemic, patients with cardiac disease have been the worst afflicted with a high mortality. Cardiac imaging forms an integral part of the armamentarium in the management of these patients. This review focuses on providing a general guide to cardiac imaging in the COVID-19 context for the practising clinician in Africa. These recommendations are likely to be modified as further data emerge on the effect of the SARS-CoV-2 virus on the cardiovascular system

An approach to the diagnosis and management of valvular heart disease

Valvular heart disease poses a common yet difficult problem in everyday clinical practice. A thorough clinical evaluation with basic commoninvestigations such as an electrocardiogram (ECG) and a chest radiograph (CXR) remains the cornerstone of diagnosis. Echocardiographyand more invasive testing, if needed, are usually performed at specialist level to confirm the diagnosis, assess severity and assist in definitivedecision-making.The causes and clinical, ECG and CXR features of the common valve lesions are described. Patients with symptomatic valve lesions shouldbe referred for specialist assessment. In most cases, medical therapy serves as a bridge to definitive mechanical or surgical therapy

Clinical Outcomes in 3343 Children and Adults with Rheumatic Heart Disease from 14 Low and Middle Income Countries: 2-Year Follow-up of the Global Rheumatic Heart Disease Registry (the REMEDY study)

Background: There are few contemporary data on the mortality and morbidity associated with rheumatic heart disease or information on their predictors. We report the 2-year follow-up of individuals with rheumatic heart disease from 14 low- and middle-income countries in Africa and Asia. Methods: Between January 2010 and November 2012, we enrolled 3343 patients from 25 centers in 14 countries and followed them for 2 years to assess mortality, congestive heart failure, stroke or transient ischemic attack, recurrent acute rheumatic fever, and infective endocarditis. Results: Vital status at 24 months was known for 2960 (88.5%) patients. Two-thirds were female. Although patients were young (median age, 28 years; interquartile range, 18–40), the 2-year case fatality rate was high (500 deaths, 16.9%). Mortality rate was 116.3/1000 patient-years in the first year and 65.4/1000 patient-years in the second year. Median age at death was 28.7 years. Independent predictors of death were severe valve disease (hazard ratio [HR], 2.36; 95% confidence interval [CI], 1.80–3.11), congestive heart failure (HR, 2.16; 95% CI, 1.70–2.72), New York Heart Association functional class III/IV (HR, 1.67; 95% CI, 1.32–2.10), atrial fibrillation (HR, 1.40; 95% CI, 1.10–1.78), and older age (HR, 1.02; 95% CI, 1.01–1.02 per year increase) at enrollment. Postprimary education (HR, 0.67; 95% CI, 0.54–0.85) and female sex (HR, 0.65; 95% CI, 0.52–0.80) were associated with lower risk of death. Two hundred and four (6.9%) patients had new congestive heart failure (incidence, 38.42/1000 patient-years), 46 (1.6%) had a stroke or transient ischemic attack (8.45/1000 patient-years), 19 (0.6%) had recurrent acute rheumatic fever (3.49/1000 patient-years), and 20 (0.7%) had infective endocarditis (3.65/1000 patient-years). Previous stroke and older age were independent predictors of stroke/transient ischemic attack or systemic embolism. Patients from low- and lower-middle–income countries had significantly higher age- and sex-adjusted mortality than patients from upper-middle–income countries. Valve surgery was significantly more common in upper-middle–income than in lower-middle– or low-income countries. Conclusions: Patients with clinical rheumatic heart disease have high mortality and morbidity despite being young; those from low- and lower-middle–income countries had a poorer prognosis associated with advanced disease and low education. Programs focused on early detection and the treatment of clinical rheumatic heart disease are required to improve outcomes. </jats:sec

Detailed valve assessment in the adult

Recording of presentation given by Blanche Cupido with the title 'Detailed valve assessment in the adult', presented on Friday, 15 March 2019 as part of the Inaugural PROTEA (Partnerships for Children with Heart Disease in Africa) Workshop in Cape Town, South Africa.The 13th-16th March 2019 marked the Inaugural PROTEA (Partnerships for Children with Heart Disease in Africa) Workshop hosted by the Children’s Heart Disease Research Unit under the directorship of A/Prof Liesl Zuhlke and in conjunction with the Paediatric Cardiology Service of the Western Cape. A first in Africa, this workshop combined four events: a research methods workshop, a basic echocardiography (echo) workshop, two days of advanced echo as well as a rheumatic heart disease research think-tank. 130 delegates from 19 different countries representing all six continents attended the event, making it truly global and giving attendees the opportunity to meet and network with experts in the fields of rheumatic and congenital heart disease.</div

Echo assessment of the GUCH patient

Recording of presentation given by Blanche Cupido with the title 'Echo assessment of the GUCH patient', presented on Thursday, 14 March 2019 as part of the Inaugural PROTEA (Partnerships for Children with Heart Disease in Africa) Workshop in Cape Town, South Africa.The 13th-16th March 2019 marked the Inaugural PROTEA (Partnerships for Children with Heart Disease in Africa) Workshop hosted by the Children’s Heart Disease Research Unit under the directorship of A/Prof Liesl Zuhlke and in conjunction with the Paediatric Cardiology Service of the Western Cape. A first in Africa, this workshop combined four events: a research methods workshop, a basic echocardiography (echo) workshop, two days of advanced echo as well as a rheumatic heart disease research think-tank. 130 delegates from 19 different countries representing all six continents attended the event, making it truly global and giving attendees the opportunity to meet and network with experts in the fields of rheumatic and congenital heart disease.</div