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Open access

Sergio Barros-Gomes, Niyada Naksuk, Dragan Jevremovic and Hector R Villarraga

Cardiac amyloidosis (CA) is relatively rare and frequently misdiagnosed. Other disorders presenting with increased left ventricular (LV) mass can mimic its diagnosis. This case illustrates unique findings of primary light chain (AL) amyloidosis in a patient with remarkable signs of CA. Here, we report a 49-year-old male with prior diagnosis of hypertrophic cardiomyopathy (HCM) based on an echocardiogram performed 1 year earlier, which presented with 8 weeks of periorbital rash. The patient had numbness in the past 3 years. More recently, the patient presented with shortness of breath. Physical examination was remarkable for periorbital purpura, macroglossia and orthostatic hypotension. Cardiac auscultation showed S3 and S4. Electrocardiography showed diffuse low-voltage QRS complexes. Echocardiography revealed severe diastolic impairment; granular ‘sparkling’ pattern of the myocardium with thickened walls, interatrial septum and valves; and pericardial effusion. Diastolic dysfunction and thick walls with low ECG voltage are compelling diagnostic findings. Laboratory workup showed increased free light chain-differential (FLC-diff), N-terminal fragment of brain natriuretic peptide (NT-BNP) and cardiac Troponin T (cTnT). Bone marrow biopsy confirmed AL amyloidosis. A diagnosis of AL amyloidosis with cardiac involvement mimicking HCM was made. The patient died during hospitalization due to sudden cardiac death. This case illustrates the importance of the combination of clinical, serological, and electro- and echocardiographic findings to establish the diagnosis of CA.

Learning points:

  • Several disorders presenting with increased LV mass can mimic CA.
  • Echocardiography is one of the most important methods to diagnose CA and HCM.
  • Signs of CA include LV wall thickness; thickening of interatrial septum, valves and right ventricular free wall; and pericardial effusion. Diastolic dysfunction and thick walls on echocardiography with low ECG voltage are the hallmark of disease.
  • CA is a major prognostic factor in AL amyloidosis.
  • Signs of HCM on echocardiography include several patterns of LV hypertrophy, such as sigmoidal, reverse curve, neutral and apical morphologies; LV outflow tract or mid-cavity obstruction; systolic anterior motion of mitral leaflets; mitral regurgitation and diastolic dysfunction.
  • The combination of clinical and serological features, along morphological and functional structures, has an important role for establishing diagnosis and predicting prognosis.
Open access

Antonis Pantazis, Annina S Vischer, Maria Carrillo Perez-Tome and Silvia Castelletti

The clinical spectrum of hypertrophic cardiomyopathy (HCM) is complex and includes a variety of phenotypes, which leads to different types of manifestations. Although most of the patients are asymptomatic, a significant proportion of them will develop symptoms or risk of arrhythmias and sudden cardiac death (SCD). Therefore, the objectives of HCM diagnosis and management are to relieve the patients' symptoms (chest pain, heart failure, syncope, palpitations, etc.), prevent disease progression and major cardiovascular complications and SCD. The heterogeneity of HCM patterns, their symptoms and assessment is a challenge for the cardiologist.

Open access

Thomas Mathew, Lynne Williams, Govardhan Navaratnam, Bushra Rana, Richard Wheeler, Katherine Collins, Allan Harkness, Richard Jones, Dan Knight, Kevin O'Gallagher, David Oxborough, Liam Ring, Julie Sandoval, Martin Stout, Vishal Sharma, Richard P Steeds and on behalf of the British Society of Echocardiography Education Committee

Heart failure (HF) is a debilitating and life-threatening condition, with 5-year survival rate lower than breast or prostate cancer. It is the leading cause of hospital admission in over 65s, and these admissions are projected to rise by more than 50% over the next 25 years. Transthoracic echocardiography (TTE) is the first-line step in diagnosis in acute and chronic HF and provides immediate information on chamber volumes, ventricular systolic and diastolic function, wall thickness, valve function and the presence of pericardial effusion, while contributing to information on aetiology. Dilated cardiomyopathy (DCM) is the third most common cause of HF and is the most common cardiomyopathy. It is defined by the presence of left ventricular dilatation and left ventricular systolic dysfunction in the absence of abnormal loading conditions (hypertension and valve disease) or coronary artery disease sufficient to cause global systolic impairment. This document provides a practical approach to diagnosis and assessment of dilated cardiomyopathy that is aimed at the practising sonographer.

Open access

Sothinathan Gurunathan and Roxy Senior

Summary

We present the case of a 32-year-old man who presented with a remote history of chest pain and was diagnosed with non-compaction cardiomyopathy on echocardiography. On presentation, he was relatively asymptomatic with normal cardiac function. Unfortunately, he presented 1 year later with a catastrophic embolic stroke.

Learning points:

  • Left ventricular non-compaction (LVNC) is a myocardial disorder characterised by prominent left ventricular (LV) trabeculae, a thin compacted layer and deep intertrabecular recesses.
  • Two-dimensional echocardiography with colour Doppler is the study of choice for diagnosis and follow-up of LVNC. CMR serves an important role where adequate echocardiographic imaging cannot be obtained.
  • LVNC is associated with high rates of mortality and morbidity in adults, including heart failure, thromboembolic events and tachyarrhythmias.
Open access

Peter H Waddingham, Sanjeev Bhattacharyya, Jet Van Zalen and Guy Lloyd

Objective

Patients with non-ischaemic systolic heart failure (HF) and idiopathic dilated cardiomyopathy (DCM) are a heterogenous group with varied morbidity and mortality. Prognostication in this group is challenging. We performed a systematic review and meta-analysis to examine the significance of the presence of contractile reserve as assessed via stress imaging on mortality and hospitalisation.

Methods

A search for studies that non-invasively assessed contractile reserve in patients with DCM or non-ischaemic HF with reduced ejection fraction, stress imaging with follow-up data comparing outcomes. A range of imaging modalities and stressors were included. We examined primary endpoints of mortality and secondary endpoints of combined cardiovascular events including HF progression or hospitalisation. Our analysis compared endpoints in patients with contractile reserve and those without it.

Results

Nine prospective cohort studies were identified describing a total of 787 patients. These studies are methodologically but not statistically heterogenous (I 2 = 31%). Using a random effect model, the presence of contractile reserve was associated with a significantly lower risk of mortality and cardiovascular events odds ratios of 0.20 (CI 0.11, 0.39) (P < 0.00001) and 0.13 (CI 0.04, 0.40) (P = 0.0004), respectively.

Conclusion

Regardless of stressor and imaging modality and despite the significant methodological heterogeneity within the current data (imaging techniques and parameters), patients with non-ischaemic cardiomyopathy and reduced EF who demonstrate contractile reserve have a lower mortality, and lower events/hospitalisations. The presence of contractile reserve therefore offers a potential positive prognostic indicator when managing these patients.

Open access

Anne Ringle, Anne Dornhorst, Michaela B Rehman, Cristina Ruisanchez and Petros Nihoyannopoulos

Background

We sought to assess the long-term evolution of left ventricular (LV) function using two-dimensional (2D) and three-dimensional (3D) speckle tracking echocardiography (STE) for the detection of preclinical diabetic cardiomyopathy, in asymptomatic type 1 diabetic patients, over a 6-year follow-up.

Design and methods

Sixty-six asymptomatic type 1 diabetic patients with no cardiovascular risk factors were compared to 26 matched healthy controls. Conventional, 2D and 3D-STE were performed at baseline. A subgroup of 14 patients underwent a 6-year follow-up evaluation.

Results

At baseline, diabetic patients had similar LV ejection fraction (60 vs 61%; P = NS), but impaired longitudinal function, as assessed by 2D-global longitudinal strain (GLS) (−18.9 ± 2 vs −20.5 ± 2; P = 0.0002) and 3D-GLS (−17.5 ± 2 vs −19 ± 2; P = 0.003). At follow-up, diabetic patients had worsened longitudinal function compared to baseline (2D-GLS: −18.4 ± 1 vs −19.2 ± 1; P = 0.03). Global circumferential (GCS) and radial (GRS) strains were unchanged at baseline and during follow-up. Metabolic status did not correlate with GLS, whereas GCS and GRS showed a good correlation, suggestive of a compensatory increase of circumferential and radial functions in advanced stages of the disease – long-term diabetes (GCS: −26 ± 3 vs −23.3 ± 3; P = 0.008) and in the presence of microvascular complications (GRS: 38.8 ± 9 vs 34.3 ± 8; P = 0.04).

Conclusions

Subclinical myocardial dysfunction can be detected by 2D and 3D-STE in type 1 diabetic patients, independently of any other cardiovascular risk factors. Diabetic cardiomyopathy progression was suggested by a mild decrease in longitudinal function at the follow-up, but did not extend to a clinical expression of the disease, as no death or over heart failure was reported.

Open access

Martin R Cowie

Heart failure is appropriately described as an epidemic, with 1–2% of health care expenditure being directed at its management. In England, the National Institute for Health and Care Excellence (NICE) has issued guidance on the best practice for the diagnosis and treatment of acute and chronic heart failure. Echocardiography is key to the diagnosis of the underlying cardiac abnormalities, and access to this (with our without biochemical testing using natriuretic peptides) is key to high-quality and speedy diagnosis. New models of care aim to speed up access to echocardiography, but a shortage of technically trained staff remains a limiting factor in improving standards of care. The NHS audits the quality of care and outcome for patients admitted to hospital with heart failure, and this continues to show wide variation in practice, particularly, where patients are not reviewed by the local heart failure multidisciplinary team. Recently, the All Party Parliamentary Group on Cardiac Disease issued 10 suggestions for improvement in care for patients with heart failure – access to echocardiography being one of the key suggestions. Time will tell as to whether this support from law makers will assist in the implementation of NICE-recommended standards of care consistently across the country.

Open access

Hitesh C Patel and Justin A Mariani

This case describes an iatrogenic cause of heart failure: the pacemaker syndrome. The diagnosis was initially overlooked but in retrospect could have easily been made by reviewing the rhythm strip recorded during the echocardiogram. The patient eventually received the correct treatment to restore atrioventricular synchrony and experienced rapid resolution to her disabling symptoms.

Learning points:

  • New-onset heart failure following a pacemaker implant should be evaluated with an echocardiogram.
  • Alongside pacing-induced left ventricular systolic dysfunction and pacing wire-related cardiac valve disruption, pacemaker syndrome should be considered.
  • Interpreting a good-quality (showing both P waves and QRS complexes) rhythm strip can greatly aid in the diagnosis of pacemaker syndrome.
Open access

Francesca Tedoldi, Maximilian Krisper, Clemens Köhncke and Burkert Pieske

Summary

We present a very rare example of chronic right heart failure caused by torrent tricuspid regurgitation. Massive right heart dilatation and severe tricuspid regurgitation due to avulsion of the tricuspid valve apparatus occurred as a result of a blunt chest trauma following the explosion of a gas bottle 20 years before admission, when the patient was a young man in Vietnam. After this incident, the patient went through a phase of severe illness, which can retrospectively be identified as an acute right heart decompensation with malaise, ankle edema, and dyspnea. Blunt chest trauma caused by explosives leading to valvular dysfunction has not been reported in the literature so far. It is remarkable that the patient not only survived this trauma, but had been managing his chronic heart failure well without medication for over 20 years.

Learning points

  • Thorough clinical and physical examination remains the key to identifying patients with relevant valvulopathies.
  • With good acoustic windows, TTE is superior to TEE in visualizing the right heart.
  • Traumatic avulsion of valve apparatus is a rare but potentially life-threatening complication of blunt chest trauma and must be actively sought for. Transthoracic echocardiography remains the method of choice in these patients.

Open access

Daniel Hammersley, Aamir Shamsi, Mohammad Murtaza Zaman, Philip Berry and Lydia Sturridge

A 63-year-old female presented to hospital with progressive exertional dyspnoea over a 6-month period. In the year preceding her admission, she reported an intercurrent history of abdominal pain, diarrhoea and weight loss. She was found to be hypoxic, the cause for which was initially unclear. A ventilation–perfusion scan identified a right-to-left shunt. Transoesophageal echocardiography (TOE) demonstrated a significant right-to-left intracardiac shunt through a patent foramen ovale (PFO); additionally severe tricuspid regurgitation was noted through a highly abnormal tricuspid valve. The findings were consistent with carcinoid heart disease with a haemodynamically significant shunt, resulting in profound systemic hypoxia. 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) and imaging were consistent with a terminal ileal primary carcinoid cancer with hepatic metastasis. Liver biopsy confirmed a tissue diagnosis. The patient was commenced on medical therapy for carcinoid syndrome. She subsequently passed away while undergoing anaesthetic induction for valvular surgery to treat her carcinoid heart disease and PFO.

Learning points:

  • Carcinoid syndrome is a rare condition, which presents a significant diagnostic challenge due to its insidious presentation and symptoms. This frequently results in a marked delay in diagnosis.
  • Carcinoid heart disease is characterised by distortion and fixation of right-sided heart valves, which cause valvular regurgitation, stenosis or both. Valvular abnormalities are often found in association with right ventricular failure.
  • In the case described, carcinoid heart disease was found in association with a significant right-to-left intracardiac shunt, created through a PFO due to right atrial volume overload. This prevented right ventricular failure at the expense of creating a state of severe induced systemic hypoxia.
  • This physiological adaptation resulted in an unusual presentation of this condition, due to symptoms resulting from hypoxia, rather than the classical symptoms of carcinoid syndrome or right ventricular failure.