Scimitar Syndrome in Adulthood: Challenges in Management and Individualized Approaches.

Scimitar syndrome is a congenital disorder characterized by partial anomalous pulmonary venous return to the inferior vena cava (IVC). Clinical manifestation in adulthood is infrequent. The management approach has not been universally accepted and may be challenging. Individually tailored and multidisciplinary team-based decisions are often necessary. We present the case of a symptomatic patient diagnosed with complex congenital heart disease, including scimitar syndrome and atrial septal defect at the age of 50 years. Surgical repair, involving scimitar vein implantation in the left atrium using a pericardial patch, was performed. Despite surgical correction, dyspnea persisted, and hemoptysis developed. A diagnostic workup revealed a critical stenosis of the re-inserted vein. This was successfully treated by percutaneous intervention with stent implantation. The patient has remained asymptomatic since the procedure. Scimitar syndrome can be first diagnosed in adulthood, and clinical manifestations can vary. Diagnostic workup necessitates a CT angiogram, magnetic resonance scan, and catheterization in selected cases. Stenoses of re-implanted pulmonary veins (PVs) can develop years after surgical correction, and hemoptysis may serve as a warning symptom prompting further PV imaging. Percutaneous vascular intervention using a stent is warranted in symptomatic cases and can lead to long-term success.

Temporal Analysis in Outcomes of Long-Term Mechanical Circulatory Support: Retrospective Study.

BACKGROUND: Mechanical assist device indications have changed in recent years. Reduced incidence of complications, better survival, and the third generation of mechanical support devices contributed to this change. In this single-center study, we focused on two time periods that are characterized by the use of different types of mechanical support devices, different patient characteristics, and change in the indications. METHODS: The data were processed from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). We retrospectively defined two time intervals to reflect changes in ventricular assist device technology (period 1: 2007-2015; period 2: 2016-20222). A total of 181 patients underwent left ventricular assist device implantation. Device utilization was the following: HeartMate II = 52 (76.4%) and HeartWare = 16 (23.6%) in period 1 and HeartMate II = 2 (1.8%), HeartMate 3 = 70 (61:9%), HeartWare = 29 (25.7%), SynCardia TAH = 10 (8.8%), and BerlinHeart EXCOR = 2 (1.8%) in period 2. The outcomes of the time intervals were analyzed and evaluated. RESULTS: Survival was significantly higher during the second time period. Multivariate analysis revealed that age and bypass pump time are independent predictors of mortality. Idiopathic cardiomyopathy, bypass time, and the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) score are independent predictors of adverse events. Furthermore, the first period was noted to be at an increased risk of the following adverse events: pump thrombosis, gastrointestinal bleeding, and bleeding events. CONCLUSION: Despite the higher risk profile of the patients and persistent challenges, during the second period, there was a significant decrease in mortality and morbidity. The use of the HeartMate 3 device may have contributed to this result.

An Unexpected Diagnostic Twist in an Elderly Patient: No Heart Failure With Preserved Ejection Fraction.

Heart failure with preserved ejection fraction (HFpEF) is considered to be the dominant cause of dyspnea and pulmonary hypertension (PH) in elderly patients with preserved left ventricular systolic function and cardiovascular comorbidities. However, it is important to keep in mind that left ventricular diastolic dysfunction is not the only possible cause of PH in cases of late-onset clinical manifestation. A multiparametric approach is essential for accurate diagnosis and therapeutic decision-making. A 74-year-old patient was admitted due to progressive dyspnea and suspicion of PH. Given the patient's risk profile, HFpEF and concomitant post-capillary PH were anticipated. Despite negative findings on CT angiography and transesophageal echocardiography, right heart catheterization was performed, revealing discrepant oxygen saturations in the superior vena cava and right atrium. A partial anomalous pulmonary venous return and an atrial septal defect were identified through cardiac magnetic resonance imaging.

Impact of prehospital IV furosemide or nitrate application on hospital outcome in acute heart failure patients.

OBJECTIVES: This study aims to evaluate the association between prehospital intravenous therapy and clinical outcomes in the patients with acute heart failure. METHODS: We conducted a prospective, multicenter observational study of consecutive AHF patients. Univariate and logistic regression analysis were performed to determine association between prehospital furosemide or nitrates administration and hospital outcome (death, length of stay). RESULTS: Data on a total of 1239 patients were processed. The mean age in the whole cohort was 71 ± 11.8 years with a gender distribution (M/F) of 634/605 patients. By prehospital treatment whole cohort was divided into 4 groups: F+ group with prehospital IV furosemide administration of 602 patients (48.6 %), F- group without prehospital IV furosemide administration of 637 patients (51.4 %), N+ group with prehospital IV nitrates administration of 110 patients (8.9 %) and N- group without IV nitrates administration of 1129 patients (91.1 %). Group of combined F+/N+ was not created. Ninety-four patients (7.6 %) died during the index hospitalization. Hospital mortality (p = 0.138) and length of stay (p = 0.101) did not differ in F+ vs F-. The patients with prehospital nitrates administration did not differ in mortality, but a shorter length of stay in univariate analysis (p = 0.03) was recorded. After adjusting for age, systolic BP and mode of referral to hospitalization, early IV furosemide usage nor nitrates showed no impact on hospital mortality and length of stay. CONCLUSIONS: Prehospital treatment with IV furosemide or nitrates in AHF patients seemed to have no major impact on hospital mortality or length of hospitalization after adjustment for several cofounders (Tab. 2, Ref. 16).

Genomic Adaptations to an Endoparasitic Lifestyle in the Morphologically Atypical Crustacean Sacculina carcini (Cirripedia: Rhizocephala).

The endoparasitic crustacean Sacculina carcini (Cirripedia: Rhizocephala) has a much simpler morphology than conventional filter-feeding barnacles, reflecting its parasitic lifestyle. To investigate the molecular basis of its refined developmental program, we produced a draft genome sequence for comparison with the genomes of nonparasitic barnacles and characterized the transcriptomes of internal and external tissues. The comparison of clusters of orthologous genes revealed the depletion of multiple gene families but also several unanticipated expansions compared to non-parasitic crustaceans. Transcriptomic analyses comparing interna and externa tissues revealed an unexpected variation of gene expression between rootlets sampled around host midgut and thoracic ganglia. Genes associated with lipid uptake were strongly expressed by the internal tissues. We identified candidate genes probably involved in host manipulation (suppression of ecdysis and gonad development) including those encoding crustacean neurohormones and the juvenile hormone binding protein. The evolution of Rhizocephala therefore appears to have involved a rapid turnover of genes (losses and expansions) as well as the fine tuning of gene expression.

Characterization of the Complete Mitochondrial Genome of the Bromeliad Crab Metopaulias depressus (Rathbun, 1896) (Crustacea: Decapoda: Brachyura: Sesarmidae).

Metopaulias depressus is a non-marine crab endemic to Jamaica that dwells in rainforest bromeliads and exhibits elaborate active parental care behavior. Current genomic resources on M. depressus are rare, limiting the understanding of its adaptation to terrestrial life in species that evolved from marine ancestors. This study reports the complete mitochondrial genome of M. depressus assembled using Sanger sequencing. The AT-rich mitochondrial genome of M. depressus is 15,765 bp in length and comprises 13 protein-coding genes (PCGs), 2 ribosomal RNA genes, and 22 transfer RNA genes. A single 691 bp-long intergenic space is assumed to be the control region (CR) or D-loop. A set of selective pressure analyses indicate that the entirety of the PCGs experience purifying selection. Cox1, cox2, nad5, cox3, and atp6 experience strong purifying selection, and atp8 experiences weak purifying selection compared to the rest of the PCGs. The secondary structures of most tRNA genes exhibit a standard 'cloverleaf' structure, with the exception of trnS1, which lacks the dihydroxyuridine (DHU) arm but not the loop, the trnH gene, which lacks the thymine pseudouracil cytosine (T) loop but not the arm, and trnM, which exhibits an overly developed T loop. A maximum likelihood phylogenetic analysis based on all PCGs indicated that M. depressus is more closely related to the genera Clistocoeloma, Nanosesarma, and Parasesarma than to Chiromantes, Geosesarma, and Orisarma. This study contributes to deciphering the phylogenetic relationships within the family Sesarmidae and represents a new genomic resource for this iconic crab species.

Sawfly Genomes Reveal Evolutionary Acquisitions That Fostered the Mega-Radiation of Parasitoid and Eusocial Hymenoptera.

The tremendous diversity of Hymenoptera is commonly attributed to the evolution of parasitoidism in the last common ancestor of parasitoid sawflies (Orussidae) and wasp-waisted Hymenoptera (Apocrita). However, Apocrita and Orussidae differ dramatically in their species richness, indicating that the diversification of Apocrita was promoted by additional traits. These traits have remained elusive due to a paucity of sawfly genome sequences, in particular those of parasitoid sawflies. Here, we present comparative analyses of draft genomes of the primarily phytophagous sawfly Athalia rosae and the parasitoid sawfly Orussus abietinus. Our analyses revealed that the ancestral hymenopteran genome exhibited traits that were previously considered unique to eusocial Apocrita (e.g., low transposable element content and activity) and a wider gene repertoire than previously thought (e.g., genes for CO2 detection). Moreover, we discovered that Apocrita evolved a significantly larger array of odorant receptors than sawflies, which could be relevant to the remarkable diversification of Apocrita by enabling efficient detection and reliable identification of hosts.

Ten-year survival and prognostic markers in one thousand patients with advanced heart failure. A single-centre analysis.

AIM: Patients with advanced heart failure (HF) represent a pool of candidates for heart transplantation and long-term mechanical circulatory support devices. The aim of our study was to determine simple and reliable markers of one-year mortality for selection of the most suitable patients for heart replacement therapy. METHODS AND RESULTS: One thousand consecutive patients with HF (mean age 49 ± 10.9 years; 86.8% males) referred to a single tertiary centre from January 1998 to January 2010 in order to assess the indication for heart transplantation were enrolled. Kaplan-Meier survival analysis was performed. Independent mortality predictors were established using logistic regression analysis. The mean follow-up was 4.3 ± 2.7 years (range 1-12 years). Cumulative survival was as follows: 1-year survival 83%, 3-year 63%, 5-year 50%, 7-year 39%, and 10-year 23%. Independent predictors of 1-year mortality included coronary artery disease, left ventricular diastolic diameter >79 mm, plasma sodium <135 mmol/L, the need for intravenous treatment at hospital admission (diuretics and/or inotropes), and furosemide dose at discharge >240 mg/day. CONCLUSIONS: Short-term prognosis of HF patient can be estimated based on simple parameters. Patients with signs of poor prognosis should be referred to tertiary centres to be considered for heart replacement therapy.

Telmisartan and metabolic syndrome after heart transplantation.

INTRODUCTION: Metabolic syndrome (MS) is a cardiovascular risk predictor. Prevalence of MS after heart transplantation (HTx) is high. Recent data suggest a positive metabolic effect of telmisartan. AIM: To describe the influence of telmisartan on lipid and glycide metabolism in MS after HTx. METHODS: Fifteen patients aged 55+/-12 yr, 88+/-25 months after HTx with MS receiving statins were followed. The reason for telmisartan administration was arterial hypertension with either drug intolerance or poor control. Body mass index (BMI), waist circumference, total cholesterol, low density lipoprotein LDL-cholesterol, high density lipoprotein-cholesterol, triglycerides, C-reactive protein (CRP), fasting glucose, immunoreactive insulin (IRI), C-peptide and the homeostasis model assessment (HOMA) index were determined. Ambulatory blood pressure monitoring was performed. After initial evaluation, telmisartan 80 mg was started. After 20 +/- 5 wk follow-up, identical parameters were measured. Statistical significance was evaluated using Student's t-test. RESULTS: BMI, waist circumference, systolic and diastolic blood pressures, serum lipids and CRP remained unchanged after telmisartan. Significant reduction in fasting glucose (6.7 vs. 5.6 mmol/L, p < 0.02), IRI (8.8 vs. 8.5 U/mL p = 0.05), HOMA (7.3 vs. 5.8 mmol/L x muU/mL, p < 0.05) and C-peptide (4.0 vs. 3.3 ng/mL, p < 0.02) was found. CONCLUSIONS: Telmisartan had a positive impact on insulin sensitivity parameters (fasting glucose, IRI, C-peptide and HOMA) in this population. No effect on obesity, serum lipids and systemic inflammation was observed.