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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

Daniel X Augustine, Lindsay D Coates-Bradshaw, James Willis, Allan Harkness, Liam Ring, Julia Grapsa, Gerry Coghlan, Nikki Kaye, David Oxborough, Shaun Robinson, Julie Sandoval, Bushra S Rana, Anjana Siva, Petros Nihoyannopoulos, Luke S Howard, Kevin Fox, Sanjeev Bhattacharyya, Vishal Sharma, Richard P Steeds, Thomas Mathew and the British Society of Echocardiography Education Committee

Pulmonary hypertension is defined as a mean arterial pressure of ≥25 mmHg as confirmed on right heart catheterisation. Traditionally, the pulmonary arterial systolic pressure has been estimated on echo by utilising the simplified Bernoulli equation from the peak tricuspid regurgitant velocity and adding this to an estimate of right atrial pressure. Previous studies have demonstrated a correlation between this estimate of pulmonary arterial systolic pressure and that obtained from invasive measurement across a cohort of patients. However, for an individual patient significant overestimation and underestimation can occur and the levels of agreement between the two is poor. Recent guidance has suggested that echocardiographic assessment of pulmonary hypertension should be limited to determining the probability of pulmonary hypertension being present rather than estimating the pulmonary artery pressure. In those patients in whom the presence of pulmonary hypertension requires confirmation, this should be done with right heart catheterisation when indicated. This guideline protocol from the British Society of Echocardiography aims to outline a practical approach to assessing the probability of pulmonary hypertension using echocardiography and should be used in conjunction with the previously published minimum dataset for a standard transthoracic echocardiogram.

Open access

V S Ellensen, S Saeed, T Geisner and R Haaverstad

We present a rare complication of deep venous thrombosis with pulmonary embolism that threatened the patient with systemic embolization. A 36-year-old female was referred to the hospital after five days of progressive shortness of breath and chest pain. Preceding onset of symptoms, she had undergone surgery leading to reduced physical activity and had just returned from vacation by a long flight. Investigations with transthoracic and transesophageal echocardiography revealed a thromboembolism-in-transit across a patent foramen ovale. Thoracic CT showed submassive bilateral pulmonary embolism. Hemodynamic parameters were stable. The patient was treated surgically with extraction of the thrombus, closure of the foramen ovale and removal of the bilateral pulmonary emboli. She was discharged after an uneventful hospital stay.

Learning points:

  • Thromboembolism-in-transit across a patent foramen ovale usually occurs in the presence of deep venous thrombosis with pulmonary embolism. The abrupt rise in pulmonary arterial pressure may contribute to the migration of the thrombus across the atrial septum to the systemic circulation.
  • If any abnormal structures are seen in the left atrium by TTE in a patient with pulmonary embolism, a TEE should be performed to rule out an embolus entrapped in a patent foramen ovale.
  • When acute pulmonary hypertension cannot be assessed by conventional methods, additional parameters such as shortened right ventricular outflow tract acceleration time and a mid-systolic notching of the pulse wave Doppler profile in the right ventricular outflow tract may be useful.
  • Mortality is highest during the initial 24 h after onset of chest symptoms; thus, optimal treatment must commence urgently.
  • The choice of treatment in each individual patient must be made after a thorough discussion in a multidisciplinary heart team.
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.
Open access

Vishal Sharma, David E Newby, Ralph A H Stewart, Mildred Lee, Ruvin Gabriel, Niels Van Pelt and Andrew J Kerr

Stress echocardiography is recommended for the assessment of asymptomatic patients with severe valvular heart disease (VHD) when there is discrepancy between symptoms and resting markers of severity. The aim of this study is to determine the prognostic value of exercise stress echocardiography in patients with common valve lesions. One hundred and fifteen patients with VHD (aortic stenosis (n=28); aortic regurgitation (n=35); mitral regurgitation, (n=26); mitral stenosis (n=26)), and age- and sex-matched controls (n=39) with normal ejection fraction underwent exercise stress echocardiography. The primary endpoint was a composite of death or hospitalization for heart failure. Asymptomatic VHD patients had lower exercise capacity than controls and 37% of patients achieved <85% of their predicted metabolic equivalents (METS). There were three deaths and four hospital admissions, and 24 patients underwent surgery during follow-up. An abnormal stress echocardiogram (METS <5, blood pressure rise <20 mmHg, or pulmonary artery pressure post exercise >60 mmHg) was associated with an increased risk of death or hospital admission (14% vs 1%, P<0.0001). The assessment of contractile reserve did not offer additional predictive value. In conclusion, an abnormal stress echocardiogram is associated with death and hospitalization with heart failure at 2 years. Stress echocardiography should be considered as part of the routine follow-up of all asymptomatic patients with VHD.

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

Alice Cowley, Laura Dobson, John Kurian and Christopher Saunderson

Isolated myocardial involvement in tuberculosis is exceedingly rare but there are reports it can present with sudden cardiac death, atrioventricular block, ventricular arrhythmias or congestive cardiac failure. We report the case of a 33-year-old male, of South Asian descent, who presented with chest pain, shortness of breath and an abnormal ECG. The patient had no significant past medical history and coronary angiogram showed no evidence of coronary artery disease. Of note, the patient had recently been discharged from a local district hospital with an episode of myocarditis. The patient was found to be severely hypoxic with evidence of severe biventricular failure on echocardiography. Computed tomography of the chest demonstrated hilar lymphadenopathy, and the differential diagnosis was thought to be tuberculosis or sarcoidosis. A TB Quantiferon gold test performed at the district hospital was positive; however, fine needle aspiration was negative for acid-fast bacilli. Despite aggressive diuresis, the patient became increasingly hypoxic and suffered a cardiac arrest. Post-mortem confirmed a diagnosis of myocardial tuberculosis – a rare case of acute decompensated heart failure.

Learning points:

  • Tuberculosis myocarditis is a rare diagnosis but should be considered in at risk individuals presenting with acute fulminant myocarditis.
  • Cardiac failure can occur even in the absence of disseminated tubercular disease.
  • TB myocarditis is not just a disease of the immunocompromised.
  • Definitive diagnosis of cardiac tuberculosis during life requires a myocardial biopsy.
  • Echocardiography is a vital tool for the assessment of cardiac function, filling pressures and fluid status in the critically unwell patient.