Kidney Function in Patients With Neuromuscular Disease: Creatinine Versus Cystatin C

Background: Accurate measurement of kidney function in patients with neuromuscular disorders is challenging. Cystatin C, a marker not influenced by skeletal muscle degradation, might be of clinical value in these patients.Methods: We consecutively enrolled 39 patients with neuromuscular disorders. We investigated the association of the eGFR, based on plasma creatinine and Cystatin C, with clinical and biochemical variables associated with kidney function, namely age and galectin-3.Results: Creatinine-based eGFR was 242 (±80) and Cystatin C-based eGFR was 110 (±23) mL/min/1.73 m2. Cystatin C-based eGFR was associated with age (β −0.63 p < 0.0001) and galectin-3 levels (β −0.43 p < 0.01), while creatinine-based eGFR was not (β −0.22 p = 0.20; β −0.28 p = 0.10). Sensitivity analyses in Duchenne and Becker patients revealed the same results: Cystatin C-based eGFR was associated with age (β −0.61 p < 0.01) and galectin-3 levels (β −0.43 p = 0.05), while creatinine-based eGFR was not (β −0.32 p = 0.13; β −0.34 p = 0.14).Conclusions: These data indicate that estimation of renal function in patients with neuromuscular disorders cannot reliably be achieved with creatinine, while Cystatin C appears a reasonable alternative. Since a large proportion of patients with neuromuscular disorders develops heart failure, and requires heart failure medication, adequate monitoring of renal function is warranted.

The Challenges of Tachycardia Induced Cardiomyopathy - a Case Report

Tachycardia-induced cardiomyopathy (TIC) is characterized by reversible left ventricular dysfunction caused by long-standing tachycardia. Treatment options for tachyarrhythmias causing TIC have evolved, especially the rhythm control strategies, ensuring a better and more sustainable control of the arrhythmia. We report the case of a 46-year-old male presenting with acute heart failure, atrial fi brillation (AF) of unknown duration and severe left ventricular dysfunction, as well as left ventricular dilation. His medical history was relevant for atrial fl utter treated with catheter ablation, hypertension, and frequent atrial ectopy for which he had been prescribed amiodarone. Coronary artery disease and other potential causes for left ventricular dysfunction were excluded with coronary angiography and cardiac magnetic resonance. Thus, the patient had a high suspicion of TIC. We opted for a rhythm control strategy, however, after a successful initial electrical cardioversion, he had AF recurrence a few days later, under classic heart failure medication and antiarrhythmics. Pulmonary vein isolation was then performed, with no complications. At the one-month follow-up visit the patient was arrhythmia-free and had a normal left ventricular ejection fraction, with a slightly enlarged left ventricle. We opted to continue the heart failure medication. This case illustrates a typical case of AF induced TIC and the limited pharmacological options that exist for rhythm control, as well as the high efficacy of catheter ablation and value of imaging.

Heart Failure Patients with type II diabetes still require more focus

Funding Acknowledgements Type of funding sources: None. Background A bidirectional relationship exists between heart failure and diabetes; heart failure is a risk factor for type II diabetes and type II diabetes is a risk factor for heart failure. The prevalence of both chronic conditions is on a continuous upward rise. Heart failure has a poorer prognosis than diabetes and therefore heart failure patients with diabetes should be also managed at a heart failure centre. It has been shown that heart failure medication is of benefit to patients with or without diabetes. Purpose With new advances in heart failure medication this retrospective and prospective analysis examines if patients with or without type II diabetes receiving similar care at a heart failure centre will have similar outcomes. Methods A retrospective and prospective cohort analyses was performed on 50 patients attending a single centre heart failure clinic over a 12-month period. LVEF, NT Pro BNP and NYHA was examined pre- and post-receiving similar heart failure care. Results 50 patients were recruited for this study. There were 25 patients in the type II diabetes cohort (10 female: 15 male. Average age 77 years old) and 25 patients in the non-diabetes cohort (7 female:18 male. Average age 79 years old). In the type II diabetes cohort average LVEF pre care 27%: post care 33% (difference 6%), average NT Pro BNP pre care 3558 pg/ml: post care 2564 pg/ml (difference 994 pg/ml), average NYHA pre care II: post care I-II (difference of 0.5). In the non-diabetic cohort average LVEF pre care 26%: post care 37% (difference 11%), average NT Pro BNP pre care 1679 pg/ml: post care 1135 pg/ml (difference 544 pg/ml), average NYHA pre care II: post care I-II (difference 0.5). Conclusion Both cohorts of patients had similar NYHA functional class outcomes. The patients in the type II diabetes cohort had higher NT Pro BNP levels pre care and higher reduction in NT Pro BNP post care than the non-diabetic cohort. Both cohorts had similar LVEF pre care, but the non-diabetic cohort had better improvement post care, with possible avoidance of need for implantable cardiac devices compared to the type II diabetes cohort. This study shows that heart failure nurses should be aware that heart failure patients with type II diabetes carry a higher risk profile and should be actively identified as part of an individualised, person-centred care approach. Heart failure patients with type II diabetes need to have vigilant scrutiny of their care to optimise their outcomes.

Medication in adults after atrial switch for transposition of the great arteries: clinical practice and recommendations

Aims Heart failure is the main threat to long-term health in adults with transposition of the great arteries (TGA) corrected by an atrial switch operation (AtrSO). Current guidelines refrain from recommending heart failure medication in TGA-AtrSO, as there is insufficient data to support the hypothesis that it is beneficial. Medication is therefore prescribed based on personal judgements. We aimed to evaluate medication use in TGA-AtrSO patients and examine the association of use of renin–angiotensin–aldosterone system (RAAS) inhibitors and β-blockers with long-term survival. Methods and results We identified 150 TGA-AtrSO patients [median age 30 years (interquartile range 25–35), 63% male] included in the CONCOR registry from five tertiary medical centres with subsequent linkage to the Dutch Dispensed Drug Register for the years 2006–2014. Use of RAAS inhibitors, β-blockers, and diuretics increased with age, from, respectively, 21% [95% confidence interval (CI) 14–40], 12% (95% CI 7–21), and 3% (95% CI 2–7) at age 25, to 49% (95% CI 38–60), 51% (95% CI 38–63), and 41% (95% CI 29–54) at age 45. Time-varying Cox marginal structural models that adjusted for confounding medication showed a lower mortality risk with use of RAAS inhibitors and β-blockers in symptomatic patients [hazard ratio (HR) = 0.13 (95% CI 0.03–0.73); P = 0.020 and HR = 0.12 (95% CI 0.02–0.17); P = 0.019, respectively]. However, in the overall cohort, no benefit of RAAS inhibitors and β-blockers was seen [HR = 0.93 (95% CI 0.24–3.63); P = 0.92 and HR = 0.98 (0.23–4.17); P = 0.98, respectively]. Conclusion The use of heart failure medication is high in TGA-AtrSO patients, although evidence of its benefit is limited. This study showed lower risk of mortality with use of RAAS inhibitors and β-blockers in symptomatic patients only. These findings can direct future guidelines, supporting use of RAAS inhibitors and β-blockers in symptomatic, but not asymptomatic patients.

Conflicting Perspectives on the Value of Neprilysin Inhibition in Heart Failure Revealed During Development of a Decision Aid Focusing on Patient Costs for Sacubitril/Valsartan

Background: Despite concerns about rising costs in health care, cost is rarely an issue discussed by patients and clinicians when making treatment decisions in a clinical setting. This study aimed to understand stakeholder perspectives on a patient decision aid (PtDA) meant to help patients with heart failure choose between a generic and relatively low-cost heart failure medication (ACE [angiotensin-converting enzyme] inhibitor or angiotensin II receptor blocker) and a newer, but more expensive, heart failure medication (angiotensin II receptor blocker neprilysin inhibitor). Methods and Results: Feedback on the PtDA was solicited from 26 stakeholders including patients, clinicians, and the manufacturer. Feedback was recorded and discussed among development team members until consensus regarding both the interpretation of the data and the appropriate changes to the PtDA was reached. Stakeholders found the PtDA sufficient in clarifying the different treatment options for heart failure. However, patients, physicians, and the manufacturer had different opinions on the importance of highlighting cost in a PtDA. Patients indicated issues of cost were crucial to the decision while physicians and manufacturers expressed that the cost issue was secondary and should be de-emphasized. Conclusions: The stratified perspectives on the role of cost in medical decision-making expressed by our participants underscore the importance and challenge of having clear, frank discussions during clinic visits about treatment cost and perceived value.