A black box congenital heart defect: Anomalous superior vena cava drainage into the left atrium.

A 25-year-old pregnant woman with low oxygen saturation due to unclear congenital heart disease was admitted. During work-up the rare anomaly of superior vena cava drainage to the left atrium was diagnosed by transthoracic bubble test with agitated saline. The pathology was later confirmed by contrast multislice computed tomography angiography after delivery.

The Prognostic Role of Polysomnography Parameters in Heart Failure Patients with Previous Decompensation.

Background: Sleep-disordered breathing (SDB) is a widespread comorbidity in patients with chronic heart failure (HF) and may have a deleterious effect on the pathogenesis of HF. We aimed to evaluate the prognostic role of polysomnography parameters in HF patients with previous decompensation. Methods: 123 patients were included in the prospective cohort study. In addition to the standard examination, all patients underwent polysomnography (PSG). Results: The Kaplan−Meier analysis showed the incidence of the combined endpoint differs between LVEF categories ≤25.5% vs. >25.5% (χ2 = 9.6, log rank p = 0.002), NTpro-BNP > 680 vs. ≤680 pg/mL (χ2 = 12.7, log rank p = 0.001), VO2peak categories <16 vs. ≥16 mL/min/kg (χ2 = 14.2, log rank p = 0.001), VE/VCO2 slope ≥38.5 vs. <38.5 (χ2 = 14.5, log rank p = 0.001), wake after sleep onset >40 min vs. ≤40 min (χ2 = 9.7, log rank p = 0.03), and sleep stage 2 (S2) <44% vs. ≥44% (χ2 = 12.4, log rank p = 0.001). Conclusion: Among the PSG parameters, WASO > 40 min and S2 < 44% were associated with a combined endpoint in patients with previous decompensation of HF. Moreover, higher NT-proBNP and VE/VCO2 slope, lower LVEF, and VO2peak were also independent factors of a poor prognosis.

Acute Cardiovascular Effects of Non-Invasive Electrical Spinal Cord Stimulation: Results from a Pilot Study in Humans.

We aimed to determine if non-invasive electrical spinal cord stimulation (NIE-SCS) is associated with acute changes in systemic and pulmonary hemodynamics and cardiac electrophysiology at rest. Nine subjects without structural heart disease referred for catheter ablation of cardiac arrhythmia were included. NIE-SCS was performed in each patient at vertebral levels T1, T7, and T11. Higher systolic BP (BPs) was detected during T1 NIE-SCS as compared with baseline (147.9 ± 22.5 vs 135.4 ± 17.4 mmHg; P = 0.02). Atrioventricular nodal effective refractory period (AVN ERP) was shorter during stimulation at T1 and T7, when compared with baseline values (baseline 303.3 ± 15.0 vs 272.0 ± 19.2 for T1 vs 278.0 ± 8.3 ms for T7; P < 0.05). NIE-SCS at the T1 level is associated with an elevation of BPs. NIE-SCS at the T1 and T7 levels shortens AVN ERP. Further studies are needed for the evaluation of chronic effects.

The Autonomic Regulation of Circulation and Adverse Events in Hypertensive Patients during Follow-Up Study.

PURPOSE: Comprehensive study of autonomic regulation assessed during follow-up could provide new detailed information about the risks stratification for hypertensive patients. Therefore, we investigated the associations of these indices with death, stroke, and revascularization during the follow-up observation of 55 patients. METHODS: All patients were with target organ damage, and 27 of them had associated clinical conditions (ACC). Mean age of patients with and without ACC was 62.6 ± 4.2 and 51.9 ± 9.9 (mean ± SD) years, respectively. Follow-up was from 66 to 95 months. At entry, autonomic regulation was assessed by the tilt test, Valsalva maneuver, hand-grip test, and cold-stress vasoconstriction. Hemodynamic parameters were measured by continuous blood pressure monitoring, occlusion plethysmography, and electrocardiography. Re-examination of patients was carried out by questioning and physical and laboratory examination. RESULTS: We found that fatal outcomes were associated with a lower Valsalva index (1.34 ± 0.16 vs. 1.69 ± 0.37, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%, P < 0.05) and depressed cold vasoconstriction (0.20 ± 0.02 vs. 0.39 ± 0.16%. CONCLUSIONS: This study shows that such autonomic regulation indices as Valsalva index, blood pressure dynamics in the tilt test, cold-stress vasomotor reactivity, and BPV are important for prognosis of hypertension course.

Ventricular tachycardia as the first manifestation of Churg-Strauss syndrome.

Life-threatening arrhythmias are often found in heart diseases, but they are rare as clinical symptoms of Churg-Strauss syndrome. We report a case of a 66-year-old woman with symptomatic monomorphic ventricular tachycardia as the first sign of Churg-Strauss syndrome. Cardiac manifestations were the main clinical symptoms of the disease, and changes in other organs were weakly expressed. Furthermore, increased serum IgG4 level was revealed. It was the reason for the differential diagnosis with IgG4-related diseases. Echocardiography, cardiac magnetic resonance imaging, and histopathological analysis of biopsies had an important role in diagnosis. .

Genetic Spectrum of Idiopathic Restrictive Cardiomyopathy Uncovered by Next-Generation Sequencing.

BACKGROUND: Cardiomyopathies represent a rare group of disorders often of genetic origin. While approximately 50% of genetic causes are known for other types of cardiomyopathies, the genetic spectrum of restrictive cardiomyopathy (RCM) is largely unknown. The aim of the present study was to identify the genetic background of idiopathic RCM and to compile the obtained genetic variants to the novel signalling pathways using in silico protein network analysis. PATIENTS AND METHODS: We used Illumina MiSeq setup to screen for 108 cardiomyopathy and arrhythmia-associated genes in 24 patients with idiopathic RCM. Pathogenicity of genetic variants was classified according to American College of Medical Genetics and Genomics classification. RESULTS: Pathogenic and likely-pathogenic variants were detected in 13 of 24 patients resulting in an overall genotype-positive rate of 54%. Half of the genotype-positive patients carried a combination of pathogenic, likely-pathogenic variants and variants of unknown significance. The most frequent combination included mutations in sarcomeric and cytoskeletal genes (38%). A bioinformatics approach underlined the mechanotransducing protein networks important for RCM pathogenesis. CONCLUSIONS: Multiple gene mutations were detected in half of the RCM cases, with a combination of sarcomeric and cytoskeletal gene mutations being the most common. Mutations of genes encoding sarcomeric, cytoskeletal, and Z-line-associated proteins appear to have a predominant role in the development of RCM.