Extending the reach of expert amyloidosis care: A feasibility study exploring the staged implementation of a UK amyloidosis network.

There has been an exponential increase in the diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CA). In response, the Midlands Amyloidosis Service was launched with the aim of providing patients with a timely diagnosis, remote expertise from the National Amyloidosis Centre and access to emerging transthyretin (TTR)-directed therapies. This was a descriptive study of a pilot hub-and-spoke model of delivering specialist amyloidosis care. Patients with suspected amyloidosis were referred from the wider Midlands region, and seen in a consultant-led multidisciplinary clinic. The diagnosis of ATTR-CA was established according to either the validated non-biopsy criteria or histological confirmation of ATTR deposits with imaging evidence of amyloid. Study endpoints were the volume of service provision and the time to diagnosis from the receipt of referral. Patients (n=173, age 75±2 years; male 72 %) were referred between 2019 and 2021. Eighty patients (46 %) were found to have cardiac amyloidosis, of whom 68 (85 %) had ATTR-CA. The median time from referral to diagnosis was 43 days. By removing the need for patients to travel to London, an average of 187 patient-miles was saved. Fifteen (9 %) patients with wild-type ATTR-CA received tafamidis under the Early Access to Medicine scheme; 10 (6 %) were enrolled into phase 3 clinical trials of RNA interference or antisense oligonucleotide therapies. Our results suggest that implementing a UK amyloidosis network appears feasible and would enhance equity of access to specialised amyloidosis healthcare for the increasing numbers of older patients found to have ATTR-CA.

Severe aortic stenosis management in heart valve centres compared with primary/secondary care centres.

OBJECTIVE: Current guidelines recommend use of heart valve centres (HVCs) to deliver optimal quality of care for patients with valve disease but there is no evidence to support this. The hypothesis of this study is that patient care with severe aortic stenosis (AS) will differ in HVCs compared with satellite centres. We aimed to compare the treatment of patients with AS at HVCs (tertiary care hospitals with full access to AS interventions) to satellites (hospitals without such access). METHODS: IMPULSE enhanced is a European, observational, prospective registry enrolling consecutive patients with newly diagnosed severe AS at four HVCs and 10 satellites. Clinical characteristics, interventions performed and outcomes up to 1 year by site-type were examined. RESULTS: Among 790 patients, 594 were recruited in HVCs and 196 in satellites. At baseline, patients in HVCs had more severe valve disease (higher peak aortic velocity (4.3 vs 4.1 m/s; p=0.008)) and greater comorbidity (coronary artery disease (CAD) (44% vs 27%; p<0.001) prior myocardial infarction (MI) (11% vs 5.1%; p=0.011) and chronic pulmonary disease (17% vs 8.9%; p=0.007)) than those presenting in satellites. An aortic valve replacement was performed more often by month 3 in HVCs than satellites in the overall population (52.6% of vs 31.3%; p<0.001) and in symptomatic patients (66.7% vs 43.2%, p<0.001). One-year survival rate was higher for patients in HVCs than satellites (HR2.19; 95% CI 1.28 to 3.73 total population and 2.89 (95%CI 1.64 to 5.11) for symptomatic patients. CONCLUSIONS: Our data support the implementation of referral pathways that direct patients to HVCs performing both surgery and transcatheter interventions. TRIAL REGISTRATION NUMBER: NCT03112629.

Tipping the balance: Chronic superior vena cava thrombosis resulting in an aberrant and inefficient venous circuit.

Given there was no direct flow into the right atrium from the SVC, we deemed this inefficient venous circuit responsible for her breathlessness, with a small reduction in cardiac function 'tipping the balance' and producing symptoms this late in life. The patient opted for conservative management with the risk of intervention outweighing her currently manageable symptoms.

Caseload management and outcome of patients with aortic stenosis in primary/secondary versus tertiary care settings-design of the IMPULSE enhanced registry.

Background: Severe aortic stenosis (AS) is one of the most common and most serious valve diseases. Without timely intervention with surgical aortic valve replacement or transcatheter aortic valve replacement, patients have an estimated survival of 2-3 years. Guidelines for the treatment of AS have been developed, but studies suggest that as many as 42% of patients with AS are not treated according to these recommendations.The aims of this registry are to delineate the caseload of patients with AS, outline the management of these patients and determine appropriateness of treatments in participating centres with and without onsite access to surgery and percutaneous treatments. Methods/design: The IMPULSE enhanced registry is an international, multicentre, prospective, observational cohort registry conducted at four central full access centres (tertiary care hospitals) and at least two satellite centres per hub (primary/secondary care hospitals). An estimated 800 patients will be enrolled in the registry and patient follow-up will last for 12 months. Discussion: In addition to the primary aims determining the caseload management and outcome of patients with AS in primary, secondary and tertiary care settings, the registry will also determine a time course for the transition from asymptomatic to symptomatic status and the diagnostic steps, treatment decisions and the identification of decision-makers in tertiary versus primary/secondary care hospitals. The last patient will be enrolled in the registry in 2018 and results of the registry are anticipated in 2019. Registration number: NCT03112629.

Feasibility of performing non-contrast magnetic resonance angiography in pregnant subjects with familial aortopathies.

BACKGROUND: Pregnancy is associated with an increased risk of aortic pathology. We sought to assess the feasibility of performing non-contrast 3D steady-state free-precession (SSFP) magnetic resonance angiography (MRA) in pregnant subjects with inherited aortopathy. METHODS: Fifteen pregnant subjects (age 27±4yr) with positive genotyping for aortopathy (Marfan, Loeys-Dietz, Ehlers-Danlos) and/or a family history of aortic dissection underwent non-contrast 3D-SSFP MRA at 1.5T (Avanto, Siemens Healthcare, Erlangen, Germany) using a modified ECG-triggered orientated in a sagittal-oblique plane with a respiratory navigator at the diaphragmatic level (mean acquisition time 4.1±1.9min). Imaging was performed during the mid-trimester (21±5weeks). Image analysis was performed off-line using Cvi42 software (Circle Cardiovascular Imaging, Calgary, Canada). An assessment of image quality (score 0-3) was made before performing inner edge to inner edge measurements of the thoracic aorta at 7 levels from the multiplanar reconstructions by two independent blinded observers. RESULTS: Non-contrast 3D-MRA was successfully acquired in all 15 subjects. Image quality was deemed excellent in 87% (13/15) of cases after a mean acquisition time of 4.1±1.9min. There was a high level of agreement for aortic measurements, with low intra- and inter-observer variability (ICC ranges; 0.95-0.99 and 0.92-0.98, respectively). All pregnancies reached term (≥37/40) with a mean gestation at delivery of 38.0±0.5weeks. The mode of delivery was vaginal in 9 out of 15 subjects (60%). CONCLUSIONS: Non-contrast SSFP MRA imaging provides a quick and reproducible method of assessing the thoracic aorta in pregnancy.

The role of TTE in assessment of the patient before and following TAVI for AS.

Transcatheter aortic valve implantation is now accepted as a standard mode of treatment for an increasingly large population of patients with severe aortic stenosis. With the availability of this technique, echocardiographers need to be familiar with the imaging characteristics that can help to identify which patients are best suited to conventional surgery or transcatheter aortic valve implantation, and what parameters need to be measured. This review highlights the major features that should be assessed during transthoracic echocardiography before presentation of the patient to the 'Heart Team'. In addition, this review summarises the aspects to be considered on echocardiography during follow-up assessment after successful implantation of a transcatheter aortic valve.

Heart disease in pregnancy: ischaemic heart disease.

Coronary artery disease and in particular acute coronary syndromes in pregnancy are increasing with high risk of mortality and significant morbidity. Whilst women with atherosclerotic risk factors are at greater risk of developing problems in pregnancy, it is important to remember that women can develop problems even in the absence of atherosclerosis-secondary to thrombosis or coronary dissection. A low threshold to investigate women with chest pain is paramount, and women with raised troponin levels should be investigated seriously. Acute coronary syndromes should be managed using an invasive strategy where possible and women should not have coronary angiography withheld for fear of foetal harm. This article aims to review the limited available data of coronary artery disease in pregnancy and give practical advice on the management of stable and acute coronary disease, with particular emphasis on the latter.

An unusual cause of chest pain in a young woman.

A 25-year-old-woman presented to health-care services recurrently due to pleuritic chest pain. This increased during the first trimester of her first pregnancy. She was subsequently referred to a tertiary cardiology centre where she was seen by a cardiologist with a special interest in obstetric cardiology. After further investigations, an unusual cause of her pain was identified. Her investigations, management and her clinical course are presented here.