Table 1 Method to calculate right atrial stroke volumes and emptying fractions in each phase of right atrial motion | Herz 7 · 2015Original Article laration of Helsinki, as reflected in a priori approval by the institution's human research committee. Three-dimensional speckle-tracking echocardiography-derived right atrial volumetric measurements Two-dimensional echocardiographyAll patients underwent three-dimensional echocardiographic imaging immediately after a two-dimensional echocardiographic study using a commercially available PST-25SX matrix-array transducer (Toshiba Medical Systems, Tokyo, Japan) with three-dimensional speckletracking echocardiography capability [1][2][3]. To create full-volume three-dimensional datasets, six wedge-shaped subvolumes were acquired from an apical window over six consecutive cardiac cycles within a single breath-hold and during a constant R-R interval. The sector width was decreased as much as possible to improve the temporal and spatial resolution of the image in order to obtain a fullvolume three-dimensional dataset of the right atrium with optimal border delineation. Chamber quantification by three-dimensional speckle-tracking echocardiography was performed off-line using the 3D Wall Motion Tracking software, version 2.5 (Toshiba Medical Systems, Tokyo, Japan). Three-dimensional echocardiographic datasets were displayed in apical four-chamber and two-chamber views and three short-axis views in basal, mid-atrial, and superior right atrial regions, respectively (. Fig. 1). In the apical four-chamber and two-chamber views, the endocardial border was traced by setting multiple reference points by the user starting at the base of the right atrium at the tricuspid valve level, moving toward the lowest point of the right atrium, and excluding the right atrial appendage and venae cavae from the right atrial cavity, as demonstrated previously for the left atrium [7][8][9] (. Fig. 1). The epicardial border was adjusted manually or by setting a default thickness for the myocardium. Three-dimensional wall motion tracking, which is based on a three-dimensional block-matching algorithm, was automatically performed by the software following detection of the right atrial borders at the end-diastolic reference frame. The user could correct the shape of the right atrium if necessary throughout the entire cardiac cycle. The following volumetric calculations were performed for the right atrium respecting cardiac cycle (. Fig. 2): 1. Maximum right atrial volume (V max ) was measured at endsystole, the time at which the right atrial volume was the largest just before tricuspid valve opening 2. Minimum right atrial volume (V min ) was measured at enddiastole, the time at which the right atrial volume was the smallest before tricuspid valve closure Abstract · Zusammenfassung

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients’ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan–Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

PurposePrevious studies have shown that atherosclerosis of the descending aorta detected by transesophageal echocardiography (TEE) is a good marker of coexisting coronary artery disease. The aim of our study was to evaluate whether the presence of atherosclerosis on the descending aorta during TEE has any prognostic impact in predicting cardiovascular events.Material and MethodsThe study group consisted of 238 consecutive in-hospital patients referred for TEE testing (135 males, 103 females, mean age 58 +/- 11 years) with a follow up of 24 months. The atherosclerotic lesions of the descending aorta were scored from 0 (no atherosclerosis) to 3 (plaque >5 mm and/or "complex" plaque with ulcerated or mobile parts).ResultsAtherosclerosis was observed in 102 patients, (grade 3 in 16, and grade 2 in 86 patients) whereas 136 patients only had an intimal thickening or normal intimal surface. There were 57 cardiovascular events in the follow-up period. The number of events was higher in the 102 patients with (n = 34) than in the 136 patients without atherosclerosis (n = 23, p < 0.01). The frequency of events was in close correlation with the severity of the atherosclerosis of the descending aorta. Fifty percent of the patients with grade 3 experienced cardiovascular events. Excluding patients with subsequent revascularization, the multivariate analysis only left ventricular function with EF < 40% (HR 3.0, CI 1.3–7.1) and TEE atherosclerotic plaque >=2 (HR 2.4, CI 1.0–5.5) predicted hard cardiovascular events.ConclusionAtherosclerosis of the descending aorta observed during transesophageal echocardiography is a useful predictor of cardiovascular events.

BackgroundThree-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF).ObjectivesTo compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls.MethodsThe study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years). They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men).ResultsIncreased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls.ConclusionsSignificant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair.

Cardiac angiosarcomas are the most common primary malignant cardiac tumors in adults. The diagnosis is often delayed due to nonspecific clinical symptoms at presentation. The cornerstones of diagnosis are echocardiography and the histological evaluation of the cardiac biopsy. The knowledge on the treatment is limited; the outcomes of chemotherapy, radiotherapy, complete surgical removal, and heart transplantation are controversial. We report a 38-year-old woman with a primary heart tumor which infiltrated the right atrial wall and the pericardium and caused pericardial effusion. Angiosarcoma was verified histologically. The surgical excision could not be radical, and the patient died 3 months from diagnosis.

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Left ventricular twist results from the movement of two orthogonally oriented muscular bands of the helical myocardial structure, with a consequent clockwise rotation of the left ventricular base and counterclockwise rotation of the left ventricular apex. To the best of the authors' knowledge, this is the first time that left ventricular "rigid body rotation", the near absence of left ventricular twist in hypoplastic right-heart syndrome, has been demonstrated.

Background: In complete or dextro-transposition of the great arteries (dTGA), the aorta and the pulmonary artery are transposed. The present study was designed to examine dTGA-associated left atrial (LA) volumetric and functional abnormalities in adult patients late after repair and to compare their results to those of healthy controls. Methods: The present study consisted of 15 dTGA patients (30.3 ± 8.1 years, 9 males), the patients had Mustard (n = 8) or Senning (n = 7) procedure performed. Their results were compared to those of 36 age- and gender-matched healthy subjects (28.7 ± 1.5 years, 24 males). Results: Increased maximum LA volume and reduced LA emptying fractions respecting the cardiac cycle could be demonstrated in our dTGA patients. LA stroke volumes representing all LA functions were significantly reduced. Peak LA circumferential, longitudinal, and area strains and LA circumferential, longitudinal, and area strains measured at atrial contraction were reduced in our dTGA patients. Most LA strains were reduced in patients having Mustard surgery compared to controls and patients undergoing Senning operation. Conclusions: Significant LA volumetric and functional abnormalities could be demonstrated in adult patients with dTGA late after repair. Senning procedure seems to have more beneficial long-term effects on LA volumetric and functional features as compared to the Mustard procedure.