The scintigraphic diagnosis of cardiac amyloidosis. An expert opinion endorsed by the Section of Nuclear Medicine of the Polish Cardiac Society and the Polish Nuclear Medicine Society

Amyloid transthyretin cardiomyopathy is a progressive disease that confers significant mortality. While it is relatively rare, the frequency of diagnoses has risen with the increased contribution of novel diagnostic approach over the last decade. Traditionally tissue biopsy was considered to be a gold standard for amyloidosis diagnosis. However, there are significant limitations in the wide application of this approach. A noninvasive imaging-based diagnostic algorithm has been substantially developed in recent years. Establishing radionuclide imaging standards may translate into a further enhancement of disease detection and improving prognosis in the group of patients. Therefore we present in the following document current evidence on the scintigraphic diagnosis of cardiac transthyretin amyloidosis. Moreover, we present standardized protocol for the acquisition and interpretation criteria in the scintigraphic evaluation of cardiac amyloidosis

Current practice of care for adolescent and adult patients after Fontan surgery in Poland

Background: The growing number of adults patients after the Fontan operation requires regular surveillance tests in the specialized centers. Aims: Evaluation of current practice of care for Fontan patients in Poland based on a multicenter survey. Methods: Eight centers were included in the study-5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To aim for a comparison between the centers and facilitate the interpretation of the results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. Results: The number of 398 Fontan patients (243 adults and 155 adolescents [age 14-18 years]) was included in the study. The median FSS was 13 points with variability between the centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs 12 points, p< 0.001). Most of the patients, both in the ACHD (82.3%) and in pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, unsatisfactory utilization of tests identifying the early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy) was observed. Conclusions: The results of the study showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents

Influence of atorvastatin on coronary calcifications and myocardial perfusion defects in systemic lupus erythematosus patients: a prospective, randomized, double-masked, placebo-controlled study

INTRODUCTION: Mortality in systemic lupus erythematosus (SLE) patients is influenced by an increased occurrence of severe cardiovascular complications. Statins have been proven to protect a wide spectrum of SLE patients from these complications. This study was conducted to determine the possible efficacy of atorvastatin in SLE patients as assessed by multi-detector computed tomography (MDCT)-based coronary calcium scoring and single photon emission computed tomography (SPECT) of the myocardium. METHODS: Sixty SLE patients in stable clinical conditions were randomized to receive either atorvastatin (40 mg daily; n = 28) or placebo (n = 32). Clinical and biochemical evaluation together with MDCT-based coronary calcium scoring and SPECT studies (Tc-99 m sestamibi) were performed at the time of randomization and after 1 year of treatment. RESULTS: At randomization, SPECT revealed perfusion defects at rest in 22 (36.7%) patients and exercise-induced defects in 8 (13.3%), whereas MDCT revealed coronary calcifications in 15 subjects (25%). Coronary calcium deposits increased after 1 year in the placebo group (plaque volume change from 35.2 ± 44.9 to 62.9 ± 72.4, P < 0.05; calcium score from 32.1 ± 39.1 to 59.5 ± 64.4; P < 0.05), but not in the atorvastatin group (plaque volume 54.5 ± 62.4 vs. 51.0 ± 47.6, P not significant; calcium score 44.8 ± 50.6 vs. 54.9 ± 62.5, P not significant). The atorvastatin group showed a decrease in total serum cholesterol (from 5.1 ± 1.2 to 4.4 ± 0.7 mmol/L, P < 0.05), LDL cholesterol (2.9 ± 1.0 to 2.3 ± 0.6 mmol/L, P < 0.05), triglycerides (1.6 ± 0.6 to 1.2 ± 0.5 mmol/L, P < 0.05), and C-reactive protein (CRP) (4.4 ± 4.1 to 2.7 ± 1.7 mg/L, P < 0.05). There was no change in the mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score in patients from both groups. Perfusion defects observed at randomization showed no change after one year treatment with atorvastatin. CONCLUSIONS: In SLE patients 40 mg of atorvastatin daily for 1 year led to a decrease in serum lipids and CRP levels. Additionally the progression of atherosclerosis, as assessed by MDCT-based coronary calcium scoring, is restrained by atorvastatin treatment. The value of statin treatment in patients with SLE free from cardiovascular disease clinical symptoms should be addressed in large, prospective clinical trials

An adult patient with tetralogy of Fallot and anomalous left anterior descending artery after conduit type of repair

We report a case of 21 – year – old patient with tetralogy of Fallot and with coronary artery abnormality after staged cardiac surgeries. Due to an anomalous coronary artery, crossing the right ventricule outflow tract, the patient needed to conduit type of repair.<br>We discuss patient management. About 3% of patientswith tetralogy of Fallot have anomalous coronary artery. An anomalous coronary artery, crossing the right ventricule outflow tract, makes the reconstruction of RVOT by using a trans – annular patch impossible and it may necessitate a conduit type of repai

Past and current cause-specific mortality in Eisenmenger syndrome

Aims Eisenmenger syndrome (ES) is associated with considerable morbidity and mortality. Therapeutic strategies have changed during the 2000s in conjunction with an emphasis on specialist follow-up. The aim of this study was to determine the cause-specific mortality in ES and evaluate any relevant changes between 1977 and 2015. Methods and results This is a retrospective, descriptive multicentre study. A total of 1546 patients (mean age 38.7 +/- 15.4 years; 36% male) from 13 countries were included. Cause-specific mortality was examined before and after July 2006, 'early' and 'late', respectively. Over a median follow-up of 6.1 years (interquartile range 2.1-21.5 years) 558 deaths were recorded; cause-specific mortality was identified in 411 (74%) cases. Leading causes of death were heart failure (34%), infection (26%), sudden cardiac death (10%), thromboembolism (8%), haemorrhage (7%), and peri-procedural (7%). Heart failure deaths increased in the 'late' relative to the 'early' era (P = 0.032), whereas death from thromboembolic events and death in relation to cardiac and non-cardiac procedures decreased (P = 0.014, P = 0.014, P = 0.004, respectively). There was an increase in longevity in the 'late' vs. 'early' era (median survival 52.3 vs. 35.2 years, P <0.001). Conclusion The study shows that despite changes in therapy, care, and follow-up of ES in tertiary care centres, all-cause mortality including cardiac remains high. Patients from the 'late' era, however, die later and from chronic rather than acute cardiac causes, primarily heart failure, whereas peri-procedural and deaths due to haemoptysis have become less common. Lifelong vigilance in tertiary centres and further research for ES are clearly neede