Aortic dissection and other acute aortic syndromes in the emergency department.

Acute aortic syndromes are emergent life-threatening conditions affecting the aorta, which actual incidence is difficult to determine. Mortality of untreated patients increases steadily over time, so early diagnosis and initiation of therapy are crucial. Management of patients in Czech Republic follow, similar as in other European countries, the European Society of Cardiology guidelines from 2014, which were updated in 2018. The basis for diagnosis consists of history, physical examination, ECG, determination of vital signs, hemodynamic status and stratification of dia-gnosis probability by ADD-RS (aortic dissection detection risk score). This is followed by a series of laboratory and imaging examinations, of which the D-dimer, CT aortography and echocardiography are the most important. Recent studies show the benefit of combination of ADD-RS with D-dimer or measurement of ascendant aorta diameter by echocardiography. New emerging biomarkers are currently under investigation. Thanks to advances in technology, magnetic resonance imaging could take place as emergent diagnostic tool in the future. Initial therapy depends on the hemodynamic status of the patient. It must be followed by definitive therapy. In this publication we summarize the approach to a patient with acute aortic syndrome in the emergency department focusing on aortic dissection as its most common type.

Myocardial injury after carbon monoxide intoxication in suicide attempt, with features of both toxic and tako-tsubo cardiomyopathy: case report.

Severe carbon monoxide intoxication may cause tissue injury by hypoxemia and histotoxicity. The affection of the heart muscle often leads to transient global or regional systolic dysfunction of left ventricle or both ventricles and increased occurences of malignant arrhytmias. On the contrary, stress-induced cardiomyopathy is described as temporary segmental loss of contractility, mostly in apical segments of the left ventricle with mid- and basal sparing and less common hypokinesias in mid- or basal parts, or affection of both ventricles. This case report is dedicated to a 34-years old male, who was admitted to the department of emergency medicine after suicide attempt by carbon monoxide poisoning. Echocardiography at admission showed akinesias of midsegments of left ventricle and severe hypokinesias of apical and basal segments. Despite severe cardiogenic shock, adequate therapeutic management including mechanical ventilation, normobaric oxygenotherapy and catecholamine treatment led to a complete somatic recovery after 2 weeks, and without any permanent hypoxemic brain injury. Our case might be a coincidence of toxic cardiomyopathy, caused by carbon monoxide poisoning, and takotsubo cardiomyopathy as a result of long term exposition to combined stress factors that may lead even to a suicide attempt. Both types of cardiomyopathies often occure simultaneously due to similar pathophysiologic mechanisms. Both tako-tsubo and toxic cardiomypathy have good prognosis after overcoming the acute phase. Key words: carbon monoxide - cardiogennic shock - cardiomyopathy - suicide - tako-tsubo cardiomypathy.

Nationwide screening of Fabry disease in patients with hypertrophic cardiomyopathy in Czech Republic.

AIMS: Fabry disease (FD) is a rare X-linked genetic disorder caused by α-galactosidase A (AGALA) deficiency. Whereas 'classic' variant has multisystemic manifestation, the more recently described 'later-onset' variant is characterized by predominant cardiac involvement that often mimics hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: Consecutive unrelated patients with HCM were screened for FD in 16 (out of 17) cardiac centres in the Czech Republic covering specialized cardiology care from June 2017 to December 2018. AGALA activity and globotriaosylsphingosine (lyso-Gb3 ) levels were measured in all subjects using the dry blood spot method. FD was suspected in male patients with AGALA activity <1.2 µmol/h/L and in females with either low AGALA activity or lyso-Gb3  > 3.5 ng/mL. Positive screening results were confirmed by genetic testing. We evaluated 589 patients (390 males, 66%) with HCM (mean maximal myocardial thickness 19.1 ± 4.3 mm). The average age was 58.4 ± 14.7 years. In total, 17 patients (11 males, 6 females) had a positive screening result, and subsequently, six of them (four males and two females) had a genetically confirmed pathogenic GLA mutation (total prevalence of 1.02%). Five of these patients were carrying the p.N215S mutation known to cause a typical later-onset cardiac FD. CONCLUSIONS: We confirmed the prevalence of FD repeatedly reported in previous screening programmes (approximately 1% irrespective of gender) in a non-selected HCM population in Central Europe. Our findings advocate a routine screening for FD in all adult patients with HCM phenotype including both genders. The dry blood spot method used led to identification of clearly pathogenic variants.

Myocardial fibrosis detected by magnetic resonance in systemic sclerosis patients - Relationship with biochemical and echocardiography parameters.

OBJECTIVES: Systemic scleroderma (SSc) is a rare connective tissue disease presenting with fibrosis affecting skin and internal organs. Cardiovascular magnetic resonance (CMR) with quantification of extracellular volume (ECV) and T1 mapping might help to detect heart involvement. We aimed to evaluate whether myocardial involvement correlates with functional and laboratory parameters. METHODS: Thirty-three asymptomatic SSc patients (29 women, aged 56.6±12.2years) and 20 controls (10 women, 53.7±13.1years) were examined using CMR, echocardiography, functional pulmonary test and laboratory assessment. RESULTS: SSc patients had higher ECV (27.5±2.8 vs. 22.8±1.9%, P<0.0001) and native T1 values (1258.9±51.2 vs. 1192.2±32.6, P<0.0001) compared to controls. Plasma level of growth differentiation factor 15 (GDF-15) and galectin-3 correlated with ECV (r=0.35; P=0.0076 and r=0.38; P=0.0081) and native T1 (r=0.31; P=0.023 and r=0.35; P=0.012). GDF-15 was also negatively correlated with diffusing capacity of the lung for carbon monoxide (r=-0.58; P=0.0004) and positively correlated with modified Rodnan skin score (r=0.59; P=0.0003). Conventional echocardiography parameters were similar in SSc patients and controls. However, the global longitudinal peak systolic strain (GLPS) was lower in SSc patients compared to controls (18.6±1.6 vs. 21.1±1.2%; P<0.0001). GLPS also negatively correlated with native T1 (r=-0.35; P=0.0097), ECV (r=-0.33; P=0.014), GDF 15 (r=-0.31; P=0.022), and galectin-3 (r=-0.37; P=0.0076). CONCLUSIONS: Asymptomatic heart involvement is common in SSc patients and includes focal and diffuse myocardial fibrosis. GDF-15 and galectin-3 were positively correlated with myocardial fibrosis parameters. Future outcome studies must show whether measurement of GDF-15 and galectin-3 in SSC patients might be may be useful in clinical practice.