A survey-based triage tool to identify patients potentially eligible for referral to an advanced heart failure centre.

AIMS: Accurate prevalence data for ambulatory advanced heart failure (HF) in European countries remains limited. This study was designed to identify the population of patients potentially eligible for referral for assessment for advanced surgical HF therapies to a National advanced HF and cardiac transplant centre. METHODS AND RESULTS: A survey comprising 13 potential clinical markers of advanced HF was developed, modified from the 'I NEED HELP' tool from the 2018 position statement of the Heart Failure Association of the European Society of Cardiology, and distributed to all HF clinic services (secondary and tertiary units) nationwide. Each HF clinic unit was asked to complete the survey on consecutive patients over a 3 month period fulfilling the following three criteria: (i) age <65 years; (ii) ejection fraction <40% and (iii) HF of >3 months duration. As a comparison, the number of actual referrals to the advanced HF clinic were also audited over a 9 month period. In all, 21 of 26 HF clinic units participated in the survey. Across the period of inclusion, 4950 all-comer HF patients were seen across all sites. Of these, 375 (7.5%) fulfilled the inclusion criteria and were surveyed (74.4% male, median age 57 years [IQR: 11 years]). In total, 246 (66%) of the surveyed patients had ≥1 potential markers for advanced HF, representing just under 5% of the total all-comer HF population seen across the same time period. Of these, 67 patients (27%) had ≥2, 48 (20%) had 3 and 40 (16%) had ≥4 potential markers. The most frequently noted markers were ≥1 HF hospitalization or unscheduled clinic review (56%), intolerance to renin-angiotensin-aldosterone system inhibitors due to hypotension or renal dysfunction (29%) and intolerance to beta-blockers due to hypotension (27%). Almost one-quarter of patients reported NYHA Class III or IV symptoms. During the advanced HF clinic audit, the number of patients actually referred to the advanced HF clinic during the same time period was <5% of this potentially eligible cohort. CONCLUSIONS: In this index prospective National survey, approximately 5% of an all-comer routine HF clinic population and two-thirds of a pre-selected HF with reduced EF under 65 years cohort were found to have at least one clinical or biochemical marker suggesting advanced or impending advanced HF. Almost one-quarter of patients in this chronic outpatient 'snapshot' population have NYHA III-IV symptoms. This simple one-page triage survey-modified from the 'I NEED HELP' tool-is useful to identify a population potentially eligible for referral to an advanced HF centre for assessment for advanced surgical therapies, thereby aiding resource and service planning.

Effect of tafamidis on global longitudinal strain and myocardial work in transthyretin cardiac amyloidosis.

AIMS: In patients with transthyretin amyloid cardiomyopathy (ATTR-CM), the effect of tafamidis on myocardial function using serial speckle tracking echocardiography has not been reported. The purpose of this study was to describe the natural history of myocardial function in untreated ATTR-CM and determine the effect of tafamidis on myocardial functional parameters over 12 months of treatment. METHODS AND RESULTS: A total of 45 subjects with ATTR-CM were retrospectively studied: 23 treated with tafamidis and 22 untreated. Two-dimensional speckle tracking echocardiography was analysed at baseline and 1 year. Serial longitudinal, circumferential, and radial strain, twist, torsion, and myocardial work were measured. Over 1 year, absolute global longitudinal strain (GLS) deteriorated more in the untreated group by a median of 1.1% [inter-quartile range (IQR) 0.95] compared with 0.3% (IQR 1) in the tafamidis group (P = 0.02). Myocardial work index and efficiency also deteriorated to a greater degree: 142.5 mmHg% (IQR 197) and 4% (IQR 8), respectively, in the untreated group compared with 61.5 mmHg% (IQR 210) and 1% (IQR 7) in the tafamidis group (P = 0.04). There were no significant between group differences in left ventricular ejection fraction (LVEF), tissue Doppler velocities, circumferential or radial strain, LV twist or torsion at 1 year. The stabilization effect of tafamidis on myocardial function at 1 year did not differ according to baseline GLS, LVEF, or National Amyloidosis Centre disease stage. CONCLUSIONS: In ATTR-CM, tafamidis resulted in a lesser deterioration in GLS, myocardial work index, and efficiency over a 12-month period compared with a cohort not treated with tafamidis.

Cardiac Sarcoidosis: When and How to Treat Inflammation.

Sarcoidosis is a complex, multisystem inflammatory disease with a heterogeneous clinical spectrum. Approximately 25% of patients with systemic sarcoidosis will have cardiac involvement that portends a poorer outcome. The diagnosis, particularly of isolated cardiac sarcoidosis, can be challenging. A paucity of randomised data exist on who, when and how to treat myocardial inflammation in cardiac sarcoidosis. Despite this, corticosteroids continue to be the mainstay of therapy for the inflammatory phase, with an evolving role for steroid-sparing and biological agents. This review explores the immunopathogenesis of inflammation in sarcoidosis, current evidence-based treatment indications and commonly used immunosuppression agents. It explores a multidisciplinary treatment and monitoring approach to myocardial inflammation and outlines current gaps in our understanding of this condition, emerging research and future directions in this field.

Multimodality Imaging in Cardiac Amyloidosis.

PURPOSE OF REVIEW: Cardiac amyloidosis is an increasingly recognized condition with a growing range of targeted therapies, but diagnosis requires a high index of suspicion and multimodality imaging expertise. Early diagnosis remains key to improving quality of life and survival. This article reviews the multimodality imaging approach to the diagnosis, differentiation, and prognosis of cardiac amyloidosis. RECENT FINDINGS: Recent advances in multimodality cardiac imaging have allowed for earlier diagnosis of cardiac amyloidosis resulting in earlier initiation of life-saving therapy in cases of light chain amyloidosis and life-prolonging therapy in transthyretin amyloidosis. With these advances in multimodality imaging, it is important for cardiologists and cardiac imagers to be aware of the subtleties of early disease, the appropriate diagnostic approach as well as understanding the practicalities and pitfalls that are encountered with each modality.

Clinical Significance of Partial Anomalous Pulmonary Venous Connections (Isolated and Atrial Septal Defect Associated) Determined by Cardiovascular Magnetic Resonance.

BACKGROUND: Partial anomalous venous connections (PAPVC) are associated with left to right shunting and right heart dilatation. Identification of PAPVC has increased with widespread use of cross-sectional imaging modalities. However, management strategies are mostly based on expert opinion given the scarcity of data from large series. We aimed to define types and significance of isolated and atrial septal defect (ASD) associated PAPVC detected by cardiovascular magnetic resonance. METHODS: We retrospectively reviewed our cardiovascular magnetic resonance database from 2002 to 2018 to identify isolated or ASD-associated PAPVC cases. RESULTS: A total of 215 patients (median age 46 years; range, 6-83) with isolated or ASD-associated PAPVC were identified among 102 135 clinical cardiovascular magnetic resonance studies. Of these, 104 were isolated and 111 were associated with an ASD. Anomalous connection of right upper pulmonary vein was the most common single venous anomaly (99/215), but in the isolated PAPVC group there were more anomalous left than right upper pulmonary veins (39 versus 34). The Qp/Qs was significantly higher for isolated anomalous single right upper pulmonary vein than left upper pulmonary vein (1.6 versus 1.4 respectively; P=0.01) as were right ventricular end-diastolic volumes (113.7±30.9 versus 90 [57-157] mL/m2, P=0.004). In the PAPVC with an ASD group, sinus venosus ASDs (82%) were associated with right-sided PAPVCs while both right and left-sided venous anomalies were seen in secundum ASDs (18%). In a substantial number of patients (30 out of 91) with sinus venosus ASDs, PAPVCs were more complex and involved more than a single anomalous right upper pulmonary vein; and in 5 patients with ASD, PAPVC was identified only after the ASD closure. CONCLUSIONS: This large series provides descriptive and hemodynamic features for isolated and ASD-associated PAPVCs. Anomalous isolated right upper pulmonary vein may cause a significant shunt (Qp/Qs >1.5). PAPVC associated with sinus venosus and secundum ASDs might be more complex than a single anomalous pulmonary vein and missed before ASD correction.