Category Archives: Cardiology

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Diagnosis of occult diastolic dysfunction late after the Fontan procedure using a rapid volume expansion technique

Objectives

Diastolic dysfunction (DD), a key driver of long-term Fontan outcomes, may be concealed during standard haemodynamic evaluation. We sought to identify Fontan patients with occult DD using ‘ventricular stress testing’ with rapid volume expansion (RVE).

Methods

Cardiac catheterisation with RVE was performed routinely in Fontan patients between 11/2012 and 4/2015. Baseline and post-stress haemodynamic data were compared using t test, Mann–Whitney U test, 2 and Fisher’s exact tests. A post-stress ventricular end diastolic pressure (EDP) threshold of 15 mm Hg defined occult DD.

Results

Forty-six Fontan patients (48% female, median age 14.1 (IQR 9.1 to 21.3) years) were included. The median Fontan duration was 10.8 (IQR 5.1 to 17.8) years and dominant left ventricular morphology was present in 63% of patients. Volume expansion increased mean Fontan pressure (15.2±2.5 vs 12.4±2.2 mm Hg, p<0.001), pulmonary capillary wedge pressure (11.3±2.6 vs 7.9±2 mm Hg, p<0.001) and EDP (12.7±3.3 vs 8.5±2.1 mm Hg, p<0.001). Sixteen patients (35%) had occult DD, demonstrating higher baseline EDP (10.3±1.9 vs 7.6±1.5 mm Hg, p<0.001) and greater increase in EDP (6.3±2.4 vs 3.1±1.4 mm Hg, p<0.001) compared with patients without DD. Higher baseline EDP, lower baseline cardiac index and longer duration of Fontan circulation were associated with higher post-stress EDP. There were no complications related to RVE.

Conclusions

Ventricular stress testing by RVE is feasible, safe and identifies a subgroup of Fontan patients with occult DD. Higher baseline EDP and longer duration of Fontan circulation are associated with worse diastolic function. Future work is necessary to better understand the aetiology, associations and clinical implications of occult DD in Fontan survivors.

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Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review

Objective

A standardised diagnostic definition of protein-losing enteropathy (PLE) in Fontan patients serves both patient care and research. The present study determined whether a diagnostic definition of PLE was routinely used in published clinical Fontan studies, and to identify potentially relevant diagnostic criteria for composing a uniform PLE definition.

Methods

A systematic review was conducted in adherence to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations. Published clinical Fontan studies that were written in English and included at least four patients with PLE were selected. PLE definitions were quantitatively analysed using a lateral thinking tool in which definitions were fractionated into constituent pieces of information (building blocks or diagnostic criteria).

Results

We identified 364 papers. In the final analysis, data from 62 published articles were extracted. A diagnostic definition of PLE was used in only 27/62 (43.5%) of selected studies, and definitions were very heterogeneous. We identified eight major diagnostic criteria. Hypoalbuminaemia (n=23 studies, 85.2%), clinical presentation (n=18, 66.7%), documentation of enteric protein loss (n=16, 59.3%) and exclusion of other causes of hypoproteinaemia (n=17, 63.0%), were the most frequently used diagnostic criteria. Most studies used three diagnostic variables (n=13/27, 48.1%). Cut-off values for laboratory parameters (serum albumin, protein or faecal α-1-antitrypsin) were frequently incorporated in the PLE definition (n=16, 59.3%).

Conclusions

Establishment of a universally accepted PLE definition for routine use in clinical research and daily practice is required. The diagnostic criteria may help constitute a diagnostic PLE definition.

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Heart transplantation in Fontan patients across Australia and New Zealand

Objective

Patients with Fontan physiology may eventually require heart transplantation (HT). We determined the rates and outcomes of HT in a national, population-based multicentre study.

Methods

From 1990 to 2015, 1369 patients underwent the Fontan procedure as recorded in the Australia and New Zealand Fontan Registry. We identified those who underwent HT and analysed their outcomes. We compared rates of HT between two catchment areas. In area 1 (n=721), patients were referred to the national paediatric HT programme or its associated adult programme. In area 2 (n=648), patients were referred to the national paediatric HT programme or one of the other adult HT programmes.

Results

Mean follow-up time post-Fontan was 11±8 years. Freedom from Fontan failure was 74%±3.9% at 20 years. HT was performed in 34 patients. Patients living in area 1 were more likely to have HT (4.0%, 29/721 vs 0.8%, 5/648, p<0.001) with a cumulative proportion of 3.4% vs 0.7% at 10 years and 6.8% vs 1.2% at 20 years (p=0.002). Area 1 patients were more likely to undergo HT (hazard ratio 4.7, 95% CI 1.7 to 13.5, p=0.003) on multivariable regression. Post-HT survival at 1, 5 and 10 years was 91%, 78% and 71%, respectively. Compared with other patients with congenital heart disease (n=87), Fontan patients had similar in-hospital outcomes and long-term survival.

Conclusions

Although HT after the Fontan procedure can be achieved with excellent outcomes, most patients with Fontan failure do not undergo HT. Significant regional differences in rates of HT in Fontan patients exist.

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Heartbeat: Focus on the Fontan patient

Effective surgical palliation for complex congenital heart disease in infancy and childhood allows these patients to live into adulthood but may be associated with adverse outcomes over the longer term. Management of adult patients with “Fontan physiology” is especially challenging. The Fontan operation was first described in 1971 so that the oldest surviving patients with this procedure are only in their 40’s, with most being much younger.The physiology of the Fontan circulation, as elucidated in an elegant review by Gewillig and Brown (see page 1081), is characterized by an obligatory increase in systemic venous pressures because blood flow is directed to the pulmonary circuit without an intervening pumping chamber (e.g. absence of a functional right ventricle). In addition, forward cardiac output is lower than normal and is not responsive to normal physiological stressors, such as exercise (figure 1). In addition, over the long term, the Fontan…

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Chest pain with diffuse coronary ischaemia

Clinical introduction

An 80-year-old male with a medical history of hypertension, diabetes and moderate calcific aortic stenosis, presented with ischaemic chest pain at rest. Cardiovascular examination revealed soft systolic murmur only. ECG (see online supplementary figure S1) demonstrated inferolateral ST segment depression with ST elevation in lead aVR, suggesting diffuse subendocardial ischaemia possibly consistent with a threatening left main or proximal left anterior descending (LAD) coronary lesion. Emergency coronary angiography demonstrated tandem smooth stenoses in the proximal left circumflex (LCx) and LAD arteries (figure 1A), which persisted throughout the cardiac cycle, and after 200 mcg bolus of intracoronary nitrate. Aortogram is shown (figure 1B).

Question

What diagnosis underlies the cause of this patient’s symptoms?

  • Congenital coronary artery anomaly.

  • Coronary artery vasospasm.

  • Myocardial bridging.

  • Obstructive coronary artery disease.

  • Unruptured left sinus of Valsalva aneurysm.

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    Coronary heart disease and sexual activity

    Human quality of life in contemporary societies is heavily influenced by sexuality. When coronary heart disease (CHD) occurs, both the affected individuals and their partners are concerned that sexual activity could exacerbate the cardiac condition, possibly causing myocardial infarction (MI) or sudden death.1 Although MI might be triggered by various factors, such as physical exertion, stressful events or heavy meals, when triggers are ranked from the highest to the lowest odds ratios (by calculating attributable fractions at the population level), traffic exposure poses at least a threefold higher risk than positive emotions or sexual activity.2 However, post-MI patients and their partners have limited access to information and/or counselling about sexual issues during the post-hospitalisation phase, despite an ascertained need for sexual counselling by healthcare professionals and the importance of sexual concerns in early, middle, and later recovery after MI.3

    Sexual activity post-MI…

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    Surgical options after Fontan failure

    Objective

    The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX.

    Methods

    A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%).

    Results

    The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0–23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04).

    Conclusions

    Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

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    Rescuing the failing Fontan

    Dr van Melle and associates from the European Congenital Heart Surgeons Association (ECHSA) are to be congratulated on their multicenter study on the treatment of failed Fontan patients.1 With the Fontan operation now being 40 years old surgeons are seeing the full spectrum of Fontan circulation failures.2 Strategies (and outcomes) for treating these patients are critically important and the review from the ECHSA is very timely and helpful. The authors have divided the failed Fontan patients into three groups: those undergoing Fontan takedown early after the Fontan operation, those undergoing the Fontan conversion procedure, and those undergoing heart transplantation. These are all critically ill patients with high mortality rates in all three groups. A summary of their results is shown in table 1 (Results of Surgical Intervention for the failing Fontan).1 The immediate conclusion looking at these numbers is that all of…

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    Serial galectin-3 and future cardiovascular disease in the general population

    Background

    Lifetime risk for cardiovascular (CV) disease is high but predicting incident events on an individual level remains difficult. Single measurements of galectin-3, a marker of tissue fibrosis, predict mortality and new-onset heart failure (HF). Persistently elevated levels may indicate a clinically silent disease process.

    Objectives

    Our aim was to establish the value of serial galectin-3 measurements to predict CV outcomes in the general population.

    Methods

    Plasma galectin-3 was measured in the Prevention of REnal and Vascular ENd-stage Disease (PREVEND) study at baseline and after ~4 years. Changes in serial galectin-3 were expressed as categorical changes or absolute change from baseline and were related to subsequent outcome.

    Results

    Serial galectin-3 was measured in 5958 subjects (mean age 49±12 years; 49% female). The median duration of follow-up was 8.3 years. Persistently elevated galectin-3 (defined as highest quartile at baseline and highest quartile during visit 2, n=757 subjects) was associated with a higher risk for new-onset HF, CV mortality, all-cause mortality, new-onset atrial fibrillation and CV events, compared with subjects with non-persistently elevated galectin-3. After multivariable adjustments for baseline characteristics, serial galectin-3 remained an independent predictor of new-onset HF (HR 1.85 (1.10–3.13); p=0.02) but not for other outcomes. Serial measurements provided more accurate prognostic value to predict new-onset HF, compared with a single baseline measurement (Harrell’s C: 0.72 (0.68–0.75) vs 0.68 (0.65–0.72); p=0.002, respectively) with significant net reclassification.

    Conclusions

    Persistently elevated galectin-3 predicts new-onset HF after adjustment for covariates, and serial measurements provide more accurate prognostic information compared with single determination of galectin-3. This may help to identify individuals who are at risk for incident HF and might provide a measure to monitor interventions.

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    Homeward Bound, not hospital rebound: how transitional palliative care can reduce readmission

    Despite therapeutic advances in the management of heart failure (HF), approximately 25% of hospitalised patients with HF are readmitted within 30 days.1 These costly episodes of care have given rise to numerous policy initiatives, such as the Medicare Hospital Readmissions Reduction Programme which fiscally penalises hospitals with ‘excess’ readmissions.2

    Transitional care programmes have emerged as a potential solution to avert HF readmissions by monitoring and supporting patients and caregivers for a limited period of time post-discharge. A recent meta-analysis of these interventions suggests that programmes which incorporate home visits are effective at reducing all-cause readmission and mortality, whereas less-intensive programmes of structured telephone support alone can reduce HF-related readmissions and mortality.3

    In their Heart publication, Wong et al report the findings from their pilot trial of a transitional palliative care programme for patients with end-stage HF (TPC-ESHF).4 They demonstrate that patients randomised…

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