Reverse Takotsubo Cardiomyopathy and Cardiogenic Shock Associated With Methamphetamine Consumption.

BACKGROUND: Reverse Takotsubo cardiomyopathy is characterized by transient myocardial hypokinesia affecting predominantly the basal myocardial wall. It is a rare variant of Takotsubo cardiomyopathy affecting younger patients. CASE REPORT: We report a case of a young man who having consumed methamphetamines presented with cardiogenic shock and severe left ventricular systolic dysfunction, affecting predominantly the basal segments with sparing of the apex. After inotropic support, the left ventricular ejection fraction improved. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: It is important that emergency physicians are aware of the danger of methamphetamine consumption, and how it can lead to potentially fatal acute cardiac syndromes, including reverse Takotsubo cardiomyopathy and cardiogenic shock.

The transthyretin amyloidoses: advances in therapy.

There are two forms of transthyretin (TTR) amyloidosis: non-hereditary and hereditary. The non-hereditary form (ATTRwt) is caused by native or wild-type TTR and was previously referred to as senile systemic amyloidosis. The hereditary form (ATTRm) is caused by variant TTR which results from a genetic mutation of TTR. The predominant effect of ATTRwt amyloidosis is on the heart, with patients having a greater left ventricular wall thickness at presentation than the devastating form which is light chain (AL) amyloidosis. ATTRm amyloidosis is broadly split into two categories: a type that predominantly affects the nervous system (often called familial amyloid polyneuropathy (FAP)) and one with a predilection for the heart (often called familial amyloid cardiomyopathy (FAC)). Approximately half of all TTR mutations known to express a clinical phenotype cause a cardiomyopathy. Since the introduction of orthotopic liver transplantation for ATTRm amyloidosis in 1991, several additional therapies have been developed. These therapies aim to provide a reduction or elimination of TTR from the plasma (through genetic approaches), stabilisation of the TTR molecule (to prevent deposition) and dissolution of the amyloid matrix. We describe the latest developments in these approaches to management, many of which are also applicable to wild-type amyloidosis.

Lesson of the month 2: Cough and right hypochondrial discomfort.

Cystic echinococcosis, commonly known as hydatid disease, is caused by the larval stage of the tapeworm Echinococcus granulosus. Humans are an accidental host to this rare disease in the UK, with around 10-20 new cases reported each year. Once suspected, the diagnosis is confirmed through a combination of relevant history, imaging studies and serological testing. This lesson presents a case of hydatid disease and outlines the significant management issues when cysts rupture and the disease becomes disseminated.

Lyme disease in the United Kingdom.

Lyme disease, while still an uncommon disease in the UK, is on the increase. Case numbers have increased by 3.6-fold since 2001, with over 950 cases reported by the Health Protection Agency (HPA) in 2011, compared with less than 500 cases annually pre-2004. HPA indications of the true incidence are suggested to be closer to 3000 cases/year, of which around 82% of cases are indigenously acquired. Three genospecies, Borrelia burgdorferi sensu stricto, Borrelia afzelli and Borrelia garinii, represent the predominant pathogenic variants in the UK. Erythema migrans is the commonest manifestation, occurring in 60%-91% of cases. In the UK, neuroborelliosis is the most common complication, while myocarditis is unusual, and death from either conduction disease or carditis is extremely rare. The role of Borrelia infection in chronic dilated cardiomyopathy in the UK remains unproven. Controversy over the existence of either 'chronic Lyme disease' and/or 'post-Lyme disease syndrome' continues unabated. National medical societies, patient advocacy groups, insurance companies, lawyers, doctors, the private health medical sector and scientific journals have all become embroiled in this bitter controversy. New developments include diagnostic tests able to detect Lyme disease at an earlier stage, shorter durations of antibiotic therapy and potential advances in vaccines against Borrelia.

Sarcoidosis: the links between epidemiology and aetiology.

Sarcoidosis is a multisystem inflammatory disease, the aetiology of which has still to be resolved. The proposed mechanism is that a susceptible genotype is exposed to one or more potential antigens. A sustained inflammatory response follows, which ultimately results in pathognomonic granuloma formation. Various clinical phenotypes exist with specific genetic associations influencing disease susceptibility, protection, and clinical progression. Occupational and environmental factors, including microbial elements, may then effect the development of this disease. Sarcoidosis is a heterogeneous disease, showing geographic and racial variation in clinical presentation. It demonstrates a familial tendency and clear genotype associations. Additionally, it appears to cluster within closely associated populations (eg, work colleagues) and appears to be related to selected occupations and environmental exposures. Frequently occult, but occasionally fatal, this disease has a very variable prognosis. It is also unusual in having no specific biomarker. The epidemiology and multiple factors that appear to influence the aetiology of sarcoidosis illustrate why this disease state is frequently described as a clinical enigma.

Amyloid heart disease: a brief review of treatment options.

Heart involvement by amyloid deposition remains the most challenging of all organ sytems that may become involved, in what is generally a systemic disease. The correct diagnosis of amyloid type is critical to selection of the appropriate and wide range of therapies. The treatment of amyloid heart disease comprises two strategies: conventional management of a restrictive cardiomyopathy, and varied therapies aimed at the underlying amyloidogenic process. In light chain (AL) amyloidosis, many of the most efficacious therapies involve chemotherapeutic agents with their own inherent toxicities to the heart and bone marrow. In the case of the hereditary amyloidosis, major surgery in the form of liver transplantation is usually required. Moreover, consideration should be given to screening of family members for a potentially hereditary disease. Several types of amyloidosis may require one or more, of heart, liver and/or kidney transplantation, sometimes in addition to high-dose chemotherapy. The objective is to provide a schematic overview of available therapies in the management of AL, hereditary, senile systemic, isolated atrial and secondary forms of amyloidosis.

Delayed pericardial effusions: life-threatening complication presenting up to 100 days after chest trauma.

Stab wounds are often managed conservatively with simple wound assessment and closure. However, even apparently minor thoracic wounds can cause delayed pericardial effusions presenting as life-threatening tamponade sometimes days, weeks or months later. Patients suffering stab wounds to the chest should receive echocardiographic follow-up to exclude delayed pericardial effusions.

Lesson of the month (1). Myotonic dystrophy and out-of-hospital arrest.

Myotonic dystrophy (MD) is an autosomal dominant disorder which affects both smooth and skeletal muscles. The incidence is approximately 1 in 8,000 births. It is the most common muscular dystrophy to manifest in adulthood, and the second most common skeletal muscle disorder after Duchenne MD. Cardiac rhythm disturbances are a common cause of death in these patients. This lesson describes a case in which a previously undiagnosed case of MD presented with an episode of ventricular fibrillation.

Discussing deactivation of implantable cardiac defibrillators.

Implantable cardiac defibrillators are a key component in preventing sudden cardiac death for patients with life-threatening arrhythmias. Through ageing, frailty and the progression of cardiac and non-cardiac morbidity, many will develop a 'life-limiting' condition. This raises the challenge of how to approach making decisions to deactivate the defibrillator function. This article discusses the background to deactivation of implantable cardioverter defibrillators and the practical considerations for different circumstances.

A randomized trial of home telemonitoring in a typical elderly heart failure population in North West London: results of the Home-HF study.

AIMS: Heart failure chiefly affects the elderly, with frequent emergency admissions. Telemonitoring can identify worsening heart failure but previous randomized trials have enrolled selected patient populations. The Home-HF study examined the impact of home telemonitoring on typical heart failure patients discharged from three acute hospitals in North West London, UK. METHODS AND RESULTS: Patients hospitalized with heart failure were randomized to telemonitoring or usual specialist care. Primary outcome measures were days alive and out of hospital. Secondary outcome measures were number and duration of heart failure hospitalizations, clinic visits, and quality of life. We recruited 182 patients. There was no difference in the primary outcome measure in the two groups, but there were significantly fewer unplanned hospitalizations for heart failure decompensation, and a reduction in clinic and emergency room visits in the telemonitoring group. There was no statistically significant difference in the mean direct health service costs. CONCLUSION: Home telemonitoring in a typical elderly population of heart failure patients produces a similar outcome to 'usual' specialist care, but reduces clinic and emergency room visits and unplanned heart failure rehospitalizations at little additional cost. This method of disease monitoring may allow specialist services to increase the number of patients under their care.

Mode of death in patients with newly diagnosed heart failure in the general population.

BACKGROUND: Early prognosis for incident (new) heart failure (HF) patients in the general population is poor. Clinical trials suggest approximately half of chronic HF patients die suddenly but mode of death for incident HF cases in the general population has not been evaluated. AIMS: To describe mode of death in the first six months after a new diagnosis in the general population. METHODS: Two-centre UK population-based study. RESULTS: 396 incident HF patients were prospectively identified. Overall mortality rates were 6% [3-8%], 11% [8-14%] and 14% [11-18%] at 1, 3 and 6months respectively. There were 59 deaths over a median follow-up of 10months; 86% (n = 51) were cardiovascular (CV) deaths. Overall, the mode of death was progressive HF in 52% (n = 31), sudden death (SD) in 22% (n = 13), other CV death in 12% (n = 7), and non-CV death in 14% (n = 8). On multivariable analysis, progressive HF deaths were associated with older age, lower serum sodium, systolic hypotension, prolonged QRS duration at baseline and absence of ACE inhibitor therapy at the time of discharge or death. CONCLUSION: Early prognosis after a new diagnosis of HF in the general population is poor and progressive HF, rather than sudden death, accounts for the majority of deaths.

Severe anaphylaxis to Gelofusine during a transthoracic echo bubble study.

We describe a severe anaphylactic reaction to Gelofusin, used as part of a transthoracic echo study on a middle-aged woman who had suffered a prior cerebral event.

Sarcoid heart disease.

To this day the aetiology of sarcoidosis continues to elude definition. Partially as a consequence of this, little in the way of new therapies has evolved. The enigma of this condition is that, unusually for a disease with the potential for devastating consequences, many patients show spontaneous resolution and recover. Cardiac involvement can affect individuals of any age, gender or race and has a predilection for the conduction system of the heart. Heart involvement can also cause a dilated cardiomyopathy with consequent progressive heart failure. The most common presentation of this systemic disease is with pulmonary infiltration, but many cases will be asymptomatic and are detected on routine chest radiography revealing lymphadenopathy. Current advances lie in the newer methods of imaging and diagnosing this unusual heart disease. This review describes the pathology and diagnosis of this condition and the newer imaging techniques that have developed for determining cardiac involvement.

Hypothyroid cardiomyopathy due to hypopituitarism: a diagnostic dilemma.

A case of hypothyroid cardiomyopathy secondary to hypopituitarism posed a diagnostic challenge. This lesson describes the patient's presentation with simultaneous acute heart failure and neurological signs; the investigations and imaging findings which initially suggested myocardial infarction (MI) and/or infiltration; and the response to treatment, with biochemical, sonographic and clinical resolution of the cardiomyopathy following thyroxine replacement therapy. This lesson illustrates the cardiovascular sequelae of severe hypothyroidism, and physicians are reminded of the difficulties involved in investigating putative coronary events in hypothyroid patients, since hypothyroidism itself may mimic MI.

Prevalence of obstructive coronary artery disease and prognosis in patients with stable symptoms and a zero-coronary calcium score.

AIMS: CT calcium scoring (CTCS) and CT cardiac angiography (CTCA) are widely used in patients with stable chest pain to exclude significant coronary artery disease (CAD). We aimed to resolve uncertainty about the prevalence of obstructive coronary artery disease and long-term outcomes in patients with a zero-calcium score (ZCS). METHODS AND RESULTS: Consecutive patients with stable cardiac symptoms referred for CTCS or CTCS and CTCA from chest pain clinics to a tertiary cardiothoracic centre were prospectively enrolled. In those with a ZCS, the prevalence of obstructive CAD on CTCA was determined. A follow-up for all-cause mortality was obtained from the NHS tracer service. A total of 3914 patients underwent CTCS of whom 2730 (69.7%) also had a CTCA. Half of the patients were men (50.3%) with a mean age of 56.9 years. Among patients who had both procedures, a ZCS was present in 52.2%, with a negative predictive value of 99.5% for excluding ≥70% stenosis on CTCA. During a mean follow-up of 5.2 years, the annual event rate was 0.3% for those with ZCS compared with 1.2% for CS ≥1. The presence of non-calcified atheroma on CTCA in patients with ZCS did not affect the prognostic value (P = 0.98). CONCLUSION: In patients with stable symptoms and a ZCS, obstructive CAD is rare, and prognosis over the long-term is excellent, regardless of whether non-calcified atheroma is identified. A ZCS could reliably be used as a 'gatekeeper' in this patient cohort, obviating the need for further more expensive tests.