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ZusammenfassungEine 69-jährige Patientin und ein 70-jähriger Patient wurden mit rezidivierenden, schweren Hypoglykämien und klinischer Whipple-Trias aufgenommen. Bei der Patientin ließen erhöhte Spiegel an Insulin und C‑Peptid, ein pathologischer insulinogener Index und ein Fastentest an ein Insulinom denken, welches im 68Ga-DOTATOC-PET-CT im Pankreasschwanz detektiert wurde. Es bestand eine Koinzidenz mit einem neuroendokrinen Merkel-Zell-Karzinom. Bei dem Patienten zeigten sich hingegen supprimierte Spiegel an Insulin und C‑Peptid und es konnte eine tumorassoziierte, paraneoplastische Hypoglykämie infolge IGF-2-Sekretion gesichert werden, mit Nachweis eines erhöhten Glukoseverbrauchs in der Skelettmuskulatur (18F‑FDG-PET-CT).

The combination of riociguat and interventional balloon pulmonary angioplasty (BPA) is currently used to treat patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). The aim of the present study was to evaluate the impact of this combination therapy on the prognosis of inoperable CTEPH patients by comparing the long-term survival rates of patients undergoing combination therapy with riociguat and BPA with those of inoperable patients from the first international CTEPH registry who did not receive specific treatment.Between March 2014 and August 2019, 138 technically inoperable patients were included in the present prospective, observational cohort study when they were treated with riociguat and BPA at a single CTEPH referral center. Long-term survival of this cohort was compared using propensity score matching with that of inoperable patients recruited between 2007 and 2009 in the first international CTEPH registry. Kaplan-Meier methods were used to evaluate differences in outcomes.Whereas the survival rate in the historical group was 84.6% in the first year, 76.6% in the second, 68.5% in the third, and 58.5% in the fifth year after diagnosis, implementation of riociguat/BPA led to survival rates of 100%, 96.7%, 92.9%, and 90% in the respective follow-up periods. In a comparison of 83 well-matched pairs from the 2 cohorts, survival was markedly better in the group treated with riociguat and BPA than in the historical cohort (HR = 0.145, 95% CI 0.05, 0.421).The combination of riociguat and BPA for the treatment of inoperable CTEPH is associated with excellent 5-year survival rates.

Balloon pulmonary angioplasty (BPA) is an emerging interventional treatment for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) that targets subsegmental branches of the pulmonary artery. As the reported complication rates are high, the aim of the present study was to evaluate the effects of certain complications on the outcome after treatment.From March 2014 to December 2019, a total of 235 patients with inoperable CTEPH underwent BPA. Of these patients, 140 were included who completed a follow-up examination 6 months after the last intervention; another 2 patients deceased due to complications of BPA.A high baseline pulmonary vascular resistance (PVR)6.6 WU correlated with a higher rate of complications (mostly pulmonary artery perforations). Wire perforation during BPA did not correlate with worse outcome in terms of PVR reduction. The complication rate per intervention decreased from 21% to 14% during the 5 year period of the study.Complications are frequently observed in BPA, but the mortality rate is very low in expert centers. Importantly, the occurrence of complications does not portend a worse outcome.

Purpose Trans-hiatal herniation after esophago-gastric surgery is a potentially severe complication due to the risk of bowel incarceration and cardiac or respiratory complaints. However, measures for prevention and treatment options are based on a single surgeon´s experiences and small case series in the literature. Methods Retrospective single-center analysis on patients who underwent surgical repair of trans-hiatal hernia following gastrectomy or esophagectomy from 01/2003 to 07/2020 regarding clinical symptoms, hernia characteristics, pre-operative imaging, hernia repair technique and perioperative outcome. Results Trans-hiatal hernia repair was performed in 9 patients following abdomino-thoracic esophagectomy (40.9%), in 8 patients following trans-hiatal esophagectomy (36.4%) and in 5 patients following conventional gastrectomy (22.7%). Gastrointestinal symptoms with bowel obstruction and pain were mostly prevalent (63.6 and 59.1%, respectively), two patients were asymptomatic. Transverse colon (54.5%) and small intestine (77.3%) most frequently prolapsed into the left chest after esophagectomy (88.2%) and into the dorsal mediastinum after gastrectomy (60.0%). Half of the patients had signs of incarceration in pre-operative imaging, 10 patients underwent emergency surgery. However, bowel resection was only necessary in one patient. Hernia repair was performed by suture cruroplasty without (n = 12) or with mesh reinforcement (n = 5) or tension-free mesh interposition (n = 5). Postoperative pleural complications were most frequently observed, especially in patients who underwent any kind of mesh repair. Three patients developed recurrency, of whom two underwent again surgical repair. Conclusion Trans-hiatal herniation after esophago-gastric surgery is rare but relevant. The role of surgical repair in asymptomatic patients is disputed. However, early hernia repair prevents patients from severe complications. Measures for prevention and adequate closure techniques are not yet defined.

This study examined sST2, GDF-15, and galectin-3 as indicators of disease severity and therapy response in chronic thromboembolic pulmonary hypertension (CTEPH).This study included 57 inoperable CTEPH patients who underwent balloon pulmonary angioplasty and 25 controls without cardiovascular disease. Biomarker levels were examined in relation to advanced hemodynamic impairment [tertile with worst right atrial pressure (RAP) and cardiac index], hemodynamic therapy response [normalized hemodynamics (meanPAP ≤25 mmHg, PVR ≤3 WU and RAP ≤6 mmHg) or a reduction of meanPAP ≥25%; PVR ≥ 35%, RAP ≥25%].GDF-15 [820 (556-1315) pg/ml vs. 370 (314-516) pg/ml;GDF-15 and sST2 levels are higher in CTEPH and identified patients with advanced hemodynamic impairment. Further, decreased GDF-15 levels at follow-up were associated with hemodynamic therapy response. The diagnostic strength was not superior to NT-proBNP.

The functional relevance of right atrial (RA) function in pulmonary hypertension (PH) remains incompletely understood. The purpose of this study was to explore the correlation of cardiac magnetic resonance (CMR) feature tracking-derived RA phasic function with invasively measured pressure-volume (P-V) loop-derived right ventricular (RV) end-diastolic elastance ( Eed) and RV-arterial coupling [ratio of end-systolic elastance to arterial elastance ( Ees/ Ea)]. In 54 patients with severe PH, CMR was performed within 24 h of diagnostic right heart catheterization and P-V measurements. RA phasic function was assessed by CMR imaging of RA reservoir, passive, and active strain. The association of RA phasic function with indexes of RV function was evaluated by Spearman’s rank correlation and linear regression analyses. Median [interquartile range] RA reservoir strain, passive strain, and active strain were 19.5% [11.0–24.5], 7.0% [4.0–12.0], and 13.0% [7.0–18.5], respectively. Ees/ Ea was 0.73 [0.48–1.08], and Eed was 0.14 mmHg/mL [0.05–0.22]. RV diastolic impairment [RV end-diastolic pressure (EDP) and Eed] was correlated with RA phasic function, but Ea and Ees were not. In addition, RA phasic function was correlated with inferior vena cava diameter. In multivariate linear regression analysis, adjusting for key P-V loop indexes, Eed and EDP remained significantly associated with RA phasic function. We conclude that RA phasic function is altered in relation to impaired diastolic function of the chronically overloaded right ventricle and contributes to backward venous flow and systemic congestion. These results call for more attention to RA function in the management of patients with PH. NEW & NOTEWORTHY There is growing awareness of the importance of the right atrial (RA)-right ventricular (RV) axis in pulmonary hypertension (PH). Our results uncover alterations in RA phasic function that are related to depressed RV lusitropic function and contribute to backward venous return and systemic congestion in chronic RV overload. Assessment of RA function should be part of the management and follow-up of patients with PH.

BackgroundVirtual surgical planning (VSP) for jaw reconstruction with free fibula flap (FFF) became a routine procedure and requires computed tomography angiography (CTA) for preoperative evaluation of the lower limbs vascular system and the bone. The aim of the study was to assess whether the distribution and density of periosteal branches (PB) and septo-cutaneous perforators (SCP) of the fibular artery have an impact on flap success.MethodThis retrospective clinical study assessed preoperative CTA of the infra-popliteal vasculature and the small vessel system of 72 patients who underwent FFF surgery. Surgical outcome of flap transfer includes wound healing, subtotal, and total flap loss were matched with the segmental vascular supply.ResultA total of 72 patients (28 females, 38.9 %; 44 males, 61.1 %) fulfilled the study inclusion criteria. The mean age was 58.5 (± 15.3 years). Stenoses of the lower limbs’ vessel (n = 14) were mostly detected in the fibular artery (n = 11). Flap success was recorded in n = 59 (82.0%), partial flap failure in n = 4 (5.5%) and total flap loss in n = 9 (12.5%). The study found a mean number (± SD) of 2.53 ± 1.60 PBs and 1.39 ± 1.03 SCPs of the FA at the donor-site. The proximal FFF segment of poly-segmental jaw reconstruction showed a higher rate of PB per flap segment than in the distal segments. Based on the total number of prepared segments (n = 121), 46.7% (n = 7) of mono-, 40.4% (n = 21) of bi-, and 31.5 % (n = 17) of tri-segmental fibula flaps were at least supplied by one PB in the success group. Overall, this corresponds to 37.2% (45 out of 121) of all successful FFF. For total flap loss (n = 14), a relative number of 42.9% (n = 6) of distinct supplied segments was recorded. Wound healing disorder of the donor site was not statistically significant influenced by the detected rate of SCP.ConclusionIn general, a correlation between higher rates of PB and SCP and the flap success could not be statistically proved by the study sample. We conclude, that preoperative PB and SCP mapping based on routine CTA imaging is not suitable for prediction of flap outcome.

The purpose of this study was to assess the diagnostic accuracy of computed tomography pulmonary angiogram (CTPA) including dual energy and reconstruction of iodine maps for diagnosing CTEPH. This method for detecting embolisms and perfusion failures was compared with V/Q-SPECT. An additional purpose was to compare the applied radiation dose of both techniques. 71 patients (49 women) with suspected CTEPH were included in this prospective study. The patients received a V/Q-SPECT and a dual-energy CTPA. Iodine maps were reconstructed from the data set. CTPA and the iodine maps were read by an experienced radiologist unaware of the clinical information as well as the results of the V/Q-SPECT. Results were compared to the V/Q-SPECT. DLP and the applied amount of radionuclides (MAA, Technegas) were obtained for comparison of radiation dose. For the diagnosis of CTEPH, the sensitivity of DECT was 1.000, specificity 0.966, PPV 0.867 and NPV 1.000, respectively. There was not a considerable difference in the x-ray exposure between the DECT examination and the V/Q-SPECT (1.892 mSv vs. 1.911 mSv; p = 0.6115). Both examination modalities were highly consistent regarding the classification of pathological segments (1177/1278 segments, 92,09 %, κ = 0,5938). This study presents the DECT, in combination with reconstructed iodine maps, as a potential alternative to the current imaging technique of first choice, V/Q-SPECT. For creating future prospective diagnostic algorithms, the implementation of DECT screening with iodine maps should be considered.· DECT correctly identified all CTEPH patients.. · There is substantial agreement between DECT and V/Q-SPECT in the classification of pathological segments.. · There is no significant difference in radiation exposure during DECT examination and V/Q-SPECT examination.. · Reduced radiation dose does not negatively impact image quality..· Schüßler A, Richter M, Tello K et al. Evaluation der diagnostischen Genauigkeit und der Strahlendosis der Dual-Energy-Computertomografie (DECT) bei chronisch thromboembolischer pulmonaler Hypertonie (CTEPH). Fortschr Röntgenstr 2021; 193: 1318 - 1326.ZIEL: Ziel der Studie war es herauszufinden, wie exakt die diagnostische Genauigkeit der computertomografischen Pulmonalisangiografie (CTPA) unter Hinzunahme von Dual-Energy und Rekonstruktionen von Jodkarten bei der Diagnosestellung einer CTEPH ist. Zudem wurde die Übereinstimmung der CTPA mit der Ventilations-/Perfusionsszintigrafie (V/Q-SPECT) bei der Detektion von Embolien und Perfusionsausfällen untersucht. Ein weiteres Ziel war es, die applizierte Strahlenexposition beider Techniken miteinander zu vergleichen. 71 Patienten (49 Frauen) mit dem Verdacht auf eine CTEPH wurden in diese prospektive Studie eingeschlossen. Die Patienten erhielten eine V/Q-SPECT und eine Dual-Energy-CTPA und hieraus rekonstruierte Jodkarten. Die Analyse der CTPA und der Jodkarten erfolgte durch einen erfahrenen Radiologen, verblindet zu den klinischen Angaben und in Unkenntnis des V/Q-SPECT-Befundes. Die Ergebnisse wurden mit dem Befund der V/Q-SPECT verglichen. DLP und die applizierten Radionuklidmengen (MAA, Technegas) wurden zur Ermittlung der Strahlenexposition erfasst. Die DECT erzielte eine Sensitivität von 1,000, eine Spezifität von 0,966, einen PPV von 0,867 und einen NPV von 1,000. Zwischen den beiden Untersuchungsmodalitäten gab es, in Bezug auf die Strahlenexposition, nahezu keinen Unterschied (1,911 mSv vs. 1,892 mSv). Die Untersuchungsmodalitäten zeigten im Hinblick auf Perfusionsdefizite eine hohe Übereinstimmung (1177/1278 Segmente, 92,09 %; κ = 0,5938). Die DECT präsentierte sich, in Kombination mit den Jodkarten, als potenzielle Alternative zum gegenwärtigen bildgebenden Verfahren der ersten Wahl, der V/Q-SPECT. Bei der Erstellung zukünftiger diagnostischer CTEPH-Untersuchungsalgorithmen sollte die Implementierung der DECT erwogen werden. · Die DECT stufte alle CTEPH-Patienten als richtig positiv ein.. · Hohe Übereinstimmung zwischen DECT und V/Q-SPECT bei der Einteilung pathologischer Segmente.. · Strahlenexposition DECT-Untersuchung und V/Q SPECT kein signifikanter Unterschied.. · Trotz eingesparter Strahlendosis keine Einbußen der Bildqualität..· Schüßler A, Richter M, Tello K et al. Evaluation of Diagnostic Accuracy and X-Ray Exposure of Dual-Energy Computed Tomography (DECT) in the Course of Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Fortschr Röntgenstr 2021; 193: 1318 – 1326.

This study sought to compare cardiac magnetic resonance (CMR) imaging-derived right ventricular (RV) strain and invasively measured pressure-volume loop-derived RV contractility, stiffness, and afterload and RV-arterial coupling in pulmonary hypertension (PH).In chronic RV pressure overload, RV-arterial uncoupling is considered the driving cause of RV maladaptation and eventual RV failure. The pathophysiological and clinical value of CMR-derived RV strain relative to that of invasive pressure-volume loop-derived measurements in PH remains incompletely understood.In 38 patients with PH, global RV CMR strain was measured within 24 h of diagnostic right heart catheterization and conductance (pressure-volume) catheterization. Associations were evaluated by correlation, multivariate logistic binary regression, and receiver operating characteristic analyses.Long-axis RV longitudinal and radial strain and short-axis RV radial and circumferential strain were -18.0 ± 7.0%, 28.9% [interquartile range (IQR): 17.4% to 46.6%]; 15.6 ± 6.2%; and -9.8 ± 3.5%, respectively. RV-arterial coupling (end-systolic [Eds]/arterial elastance [Ea]) was 0.76 (IQR: 0.47 to 1.07). Peak RV strain correlated with Ees/Ea, afterload (Ea), RV diastolic dysfunction (Tau), and stiffness (end-diastolic elastance [Eed]) but not with contractility (Ees). In multivariate analysis, long-axis RV radial strain was associated with RV-arterial uncoupling (Ees/Ea: 0.805; odds ratio [OR]: 5.50; 95% confidence interval [CI]: 1.50 to 20.18), whereas long-axis RV longitudinal strain was associated with increased RV diastolic stiffness (Eed: ≥0.124 mm Hg/ml; OR: 1.23; 95% CI: 1.10 to 1.51). The long-axis RV longitudinal strain-to-RV end-diastolic volume/body surface area ratio strongly predicted RV diastolic stiffness (area under receiver operating characteristic curve: 0.908).In chronic RV overload, CMR-determined RV strain is associated with RV-arterial uncoupling and RV end-diastolic stiffness and represents a promising noninvasive alternative to current invasive methods for assessment of RV-arterial coupling and end-diastolic stiffness in patients with PH. (Right Ventricular Haemodynamic Evaluation and Response to Treatment [Rightheart I]; NCT03403868).

Evaluation of the dose values of a polytrauma whole-body CT examination used in clinical practice with regard to the 2016 updated diagnostic reference levels and reduction of the mean exposure levels using simple optimization steps. In each case, 100 exposure values before and after dose optimization were compared with the old and new diagnostic reference levels. The grayscale values and the signal-to-noise ratio (SNR) were determined for the lung, the aortic arch and the liver. A visual assessment of the image quality was performed by two radiologists on the basis of a Likert scale (0 - non-diagnostic, 1 - poor visualization, 2 - moderate visualization, 3 - good visualization, 4 - excellent visualization) for CT examinations both before and after optimization. The acquired exposure values after dose optimization were below the old and new diagnostic reference levels (1319.98 ± 463.16 mGy · cm) while the mean value of the exposure values before optimization (1774.96 ± 608.78 mGy · cm) exceeded the current diagnostic reference levels. The measured grayscale values (HU) were (before versus after optimization): lung - 833 HU vs. - 827 HU (p = 0.43), aortic arch 341 HU vs. 343 HU (p = 0.70) and liver 68 HU vs. 67 HU (p = 0.35). After dose optimization the SNR in the lung was minimally higher, while it was minimally lower in the two other regions than before the optimization. Visual assessment of the image quality showed almost identical values with 3.85 evaluation points before and 3.82 evaluation points after dose optimization (p = 0.57). Due to the updating of the diagnostic reference levels, an analysis of the own exposure values is necessary in order to be able to detect high values promptly and to initiate appropriate measures for dose reduction. Appropriate adaptation of the examination parameters with consideration of the necessary image quality allows a significant reduction of the radiation exposure in most cases, also on CT devices of older generations.· In many cases a dose reduction below the DRLs is already possible by optimizing the examination technique.. · In order to ensure a diagnostic image quality, the control of the image quality is unavoidable in a dose reduction.. · Through suitable parameter adjustments a compliance with the DRLs is also possible, using CT devices of older generation without iterative image reconstruction..· Schäfer SB, Rudolph C, Kolodziej M et al. Optimization of Whole-Body CT Examinations of Polytrauma Patients in Comparison with the Current Diagnostic Reference Levels. Fortschr Röntgenstr 2019; 191: 1015 - 1025.ZIEL: Die Überprüfung der Dosiswerte an einem in der klinischen Routine betriebenen Polytrauma-Ganzkörper-CT hinsichtlich der 2016 aktualisierten diagnostischen Referenzwerte (DRW) und Reduktion der mittleren Expositionswerte durch einfache Optimierungsschritte. Jeweils 100 Expositionswerte vor und nach der Dosisoptimierung wurden mit den alten und den neuen DRW verglichen. Die Graustufenwerte und das Signal-Rausch-Verhältnis (SNR) wurden für die Lunge, den Aortenbogen und die Leber bestimmt. Durch 2 Radiologen erfolgte eine visuelle Beurteilung der Bildqualität anhand einer Likert-Skala (0 – nicht diagnostisch, 1 – schlecht sichtbar, 2 – moderat sichtbar, 3 – gut sichtbar, 4 – exzellent sichtbar) sowohl für die CT-Untersuchungen vor als auch nach der Optimierung. Die ermittelten Expositionswerte nach der Dosisoptimierung lagen unterhalb der alten und neuen DRW (1319,98 ± 463,16 mGy · cm), während der Mittelwert der Expositionswerte vor der Optimierung (1774,96 ± 608,78 mGy · cm) die aktuellen DRW überschritt. Die gemessenen Graustufenwerte (HE) betrugen (vor gg. nach der Optimierung) Lunge – 833 HE gg. – 827 HE (p = 0,43), Aortenbogen 341 HE gg. 343 HE (p = 0,70) und Leber 68 HE gg. 67 HE (p = 0,35) (gg = gegenüber). Das SNR war nach der Dosisoptimierung in der Lunge minimal höher, in den beiden anderen Regionen minimal geringer als vor der Optimierung. Die visuelle Beurteilung der Bildqualität wies mit 3,85 Bewertungspunkten vor und 3,82 Bewertungspunkten nach der Dosisoptimierung (p = 0,57) relativ identische Werte auf. Anlässlich der Aktualisierung der DRW ist eine Analyse der eigenen Expositionswerte unumgänglich, um Überschreitungen zeitnah erkennen und geeignete Maßnahmen zur Dosisreduktion einleiten zu können. Bereits durch geeignete Anpassung der Untersuchungsparameter unter Berücksichtigung der erforderlichen Bildqualität ist in den meisten Fällen eine deutliche Reduktion der Strahlenexposition, auch bei CT-Geräten der älteren Generationen, möglich. · Eine Reduktion der Strahlenexposition unter die DRW ist in vielen Fällen bereits durch Optimierung der Untersuchungstechnik möglich.. · Die Kontrolle der Bildqualität zur Gewährleistung einer diagnostischen Bildqualität trotz Dosisreduktion ist unumgänglich.. · Die Einhaltung der DRW ist auch bei CT-Geräten der älteren Generation auch ohne iterative Bildrekonstruktion durch geeignete Anpassungen möglich.. · Weiteres Optimierungspotenzial zur aktiven Dosisreduktion ist vorhanden..· Schäfer SB, Rudolph C, Kolodziej M et al. Optimization of Whole-Body CT Examinations of Polytrauma Patients in Comparison with the Current Diagnostic Reference Levels. Fortschr Röntgenstr 2019; 191: 1015 – 1025.

Balloon pulmonary angioplasty (BPA), for chronic thromboembolic pulmonary hypertension, improves pulmonary and systemic hemodynamics. The kidney might benefit from this effect. However, staged BPA therapy comes along with repetitive administration of contrast agent. This study examined the overall effect of BPA therapy on renal function. This study included consecutive patients who underwent BPA treatment and completed a 6-month follow-up between March 2014 and March 2017. Biomarker-based evaluation of renal function was performed at baseline, consecutively prior to and after each BPA and at 6-month follow-up. The 51 patients underwent an average of 5 (±2) BPA sessions. In this course, patients received 133 (±48; 21-300) mL of contrast agent per session and 691 (±24; 240-1410) mL during the whole sequence. Acute kidney injury occurred after 6 (2.3%) procedures. The creatinine [80.1 (IQR 67.8-96.8) µmol/L vs. 77.4 (IQR 66.9-91.5) µmol/L, p = .02] and urea level [13.7 (IQR10.7-16.6) mmol/L vs. 12.5 (IQR 10.0-15.5) mmol/L, p = .02] decreased from baseline to the 6-month follow-up. The estimated glomerular filtration rate (eGFR) [79 (IQR 59-94) mL/min/m

Fabry disease (FD) is an X-linked multi-organ disorder of lysosomal metabolism with cardiac disease being the leading cause of death. Identifying early FD-specific pathologies is important in the context of maximum therapeutic benefit in these stages. Therefore, the aim of this study was to investigate the value of quantitative cardiac T1 mapping as a potential disease-specific surrogate. 16 consecutive FD patients (9 female, 7 male; median age: 54 years, IQR 17) and 16 control patients (9 female, 7 male; median age: 52 years, IQR 20) were investigated at 1.5 Tesla. Native T1 mapping was performed using a modified look locker inversion recovery sequence (MOLLI) and native T1 times were measured within the septal myocardium at the midventricular short-axis section. Also functional parameters, left ventricular morphology, presence of late-gadolinium enhancement, cTnI- and Lyso-Gb3-Levels were evaluated. The median native septal T1 time for FD was 889.0 ms and 950.6 ms for controls (p 0.003). LGE and positive cTnI values (0.26 ± 0.21) were present in 5 FD patients (31.25 %), and left ventricular hypertrophy (LVH) was present in 4 FD patients (25.00 %). The 4 cTnI and 8 Lyso-Gb3 positive FD patients had significantly lower native T1 values (p 0.05, respectively p 0.01). Assuming a T1 cut-off value of 900 ms for the identification of increased cardiac lipid deposit, 9 patients with FD (56.25 %) had pathologic values (4 patients cTnI and 8 patients Lyso-Gb3 positive). Moreover, native septal T1 showed a good negative correlation to Lyso-Gb3 (r = - 0.582; p = 0.018). A pathologic cardiac native T1 time obviously reflects cardiac involvement in the scope of FD at tissue level. In the future native T1 mapping as an imaging biomarker might allow identification of early stages of cardiac involvement in FD before morphological changes are obvious. · Native T1 values are significantly decreased in Fabry disease.. · Native T1 shows promising correlation to cardiac and Fabry-specific biomarkers.. · Native T1 mapping might have great potential for early disease detection and therapy monitoring..· Roller FC, Fuest S, Meyer M et al. Assessment of Cardiac Involvement in Fabry Disease (FD) with Native T1 Mapping. Fortschr Röntgenstr 2019; 191: 932 - 939.ZIEL: Morbus Fabry (FD) ist eine X-chromosomale Multiorganerkrankung des lysosomalen Metabolismus, wobei die kardiale Beteiligung die Haupttodesursache der Erkrankung darstellt. Deswegen ist es wichtig, möglichst frühe Erkrankungsmanifestationen zu detektieren, um einen maximalen therapeutischen Nutzen zu erzielen. Das Ziel unserer Studie war es, die Wertigkeit des nativen T1-Mappings als krankheitsspezifisches Äquivalent zu untersuchen. 16 konsekutive FD-Patienten (9 weiblich, 7 männlich; Altersmedian 54 Jahre; IQR 17) und 16 Kontrollpatienten (9 weiblich, 7 männlich; Altersmedian 52 Jahre, IQR 20) wurden mit einem 1,5-Tesla MRT-System untersucht. Das native T1-Mapping wurde als modifizierte Look-Locker-Sequenz (MOLLI) durchgeführt, die Messungen erfolgten im septalen linksventrikulären Myokard auf mittventrikulären Kurzachsenschnitten. Zudem wurden die linksventrikuläre Funktion und Morphologie, das Vorhandensein einer Kontrastmittelspätanreicherung sowie cTnI- und Lyso-Gb3-Laborwerte ausgewertet. Die mediane native septale T1-Zeit bei FD-Patienten betrug 889,0 und 950,6 für die Kontrollgruppe (p 0,003). 5 (31,25 %) Patienten hatten eine Kontrastmittelspätanreicherung und positive cTnI-Werte, 4 Patienten (25,0 %) eine linksventrikuläre Hypertrophie. Die 5 cTnI- und die 8 Lyso-Gb3-positiven Patienten hatten signifikant niedrigere native T1-Zeiten (p 0,05, respektive p 0,01). Unter der Annahme eines Grenzwertes von 900 ms für die Detektion eines erhöhten, zellulären Lipidgehalts zeigten 9 Patienten (56,25 %) pathologische Werte. Davon waren 8 Patienten Lyso-Gb3- und 4 Patienten cTnI-positiv. Zudem zeigte sich eine gute negative Korrelation der nativen T1-Zeit zu den Lyso-Gb3-Werten (r = – 0,582; p = 0,018). Offensichtlich reflektiert eine pathologische native T1-Zeit eine kardiale Beteiligung bei FD-Patienten. Zukünftig könnte natives T1-Mapping als Biomarker in der Bildgebung behilflich sein, frühe kardiale Beteiligungen im Rahmen des FD zu detektieren, bevor andere morphologische Veränderungen zu erkennen sind. · Die native T1-Zeit ist bei Patienten mit Morbus Fabry signifikant niedriger.. · Die native T1-Zeit korreliert zu kardialen und erkrankungsspezifischen Biomarkern.. · Das native T1-Mapping könnte großes Potenzial in Diagnostik und Therapie-Monitoring haben..

Background * These authors contributed equally as last authors. Balloon pulmonary angioplasty is an evolving, interventional treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary hypertension at rest as well as exercise capacity is considered to be relevant outcome parameters. The aim of the present study was to determine whether measurement of pulmonary hemodynamics during exercise before and six months after balloon pulmonary angioplasty have an added value. Methods From March 2014 to July 2018, 172 consecutive patients underwent balloon pulmonary angioplasty. Of these, 64 consecutive patients with inoperable CTEPH underwent a comprehensive diagnostic workup that included right heart catheterization at rest and during exercise before balloon pulmonary angioplasty treatments and six months after the last intervention. Results Improvements in pulmonary hemodynamics at rest and during exercise, in quality of life, and in exercise capacity were observed six months after balloon pulmonary angioplasty: WHO functional class improved in 78% of patients. The mean pulmonary arterial pressure (mPAP) at rest was reduced from 41 ± 9 to 31 ± 9 mmHg (p

Symptomatic patients with residual pulmonary perfusion defects or vascular lesions but no pulmonary hypertension at rest are diagnosed with chronic thromboembolic disease (CTED). Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but data regarding the safety and efficacy of BPA in patients with CTED are lacking. We report a prospective series of ten consecutive patients with CTED who underwent 35 BPA interventions (median of four per patient) at two German institutions. All patients underwent a comprehensive diagnostic workup at baseline and 24 weeks after their last intervention. BPA was safe, with one pulmonary vascular injury and subsequent self-limiting pulmonary bleeding as the only complication (2.9% of the interventions, 10% of the patients). After the procedures, World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, and pulmonary arterial compliance improved, and NT-proBNP concentrations declined in 9/10 patients. BPA may be a new treatment option for carefully selected patients with CTED. A larger, prospective, international registry is required to confirm these results.

Background: The ratios of tricuspid annular plane systolic excursion (TAPSE)/echocardiographically measured systolic pulmonary artery pressure (PASP), fractional area change/invasively measured mean pulmonary artery pressure, right ventricular (RV) area change/end-systolic area, TAPSE/pulmonary artery acceleration time, and stroke volume/end-systolic area have been proposed as surrogates of RV-arterial coupling. The relationship of these surrogates with the gold standard measure of RV-arterial coupling (invasive pressure-volume loop-derived end-systolic/arterial elastance [Ees/Ea] ratio) and RV diastolic stiffness (end-diastolic elastance) in pulmonary hypertension remains incompletely understood. We evaluated the relationship of these surrogates with invasive pressure-volume loop-derived Ees/Ea and end-diastolic elastance in pulmonary hypertension. Methods: We performed right heart echocardiography and cardiac magnetic resonance imaging 1 day before invasive measurement of pulmonary hemodynamics and single-beat RV pressure-volume loops in 52 patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. The relationships of the proposed surrogates with Ees/Ea and end-diastolic elastance were evaluated by Spearman correlation, multivariate logistic regression, and receiver operating characteristic analyses. Associations with prognosis were evaluated by Kaplan-Meier analysis. Results: TAPSE/PASP, fractional area change/mean pulmonary artery pressure, RV area change/end-systolic area, and stroke volume/end-systolic area but not TAPSE/pulmonary artery acceleration time were correlated with Ees/Ea and end-diastolic elastance. Of the surrogates, only TAPSE/PASP emerged as an independent predictor of Ees/Ea (multivariate odds ratio: 18.6; 95% CI, 0.8–96.1; P =0.08). In receiver operating characteristic analysis, a TAPSE/PASP cutoff of 0.31 mm/mm Hg (sensitivity: 87.5% and specificity: 75.9%) discriminated RV-arterial uncoupling (Ees/Ea Conclusions: Echocardiographically determined TAPSE/PASP is a straightforward noninvasive measure of RV-arterial coupling and is affected by RV diastolic stiffness in severe pulmonary hypertension. Clinical Trial Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT03403868.

Cardiopulmonary exercise testing (CPET) is an important tool for assessing functional capacity and prognosis in pulmonary arterial hypertension (PAH). However, the associations of CPET parameters with the adaptation of right ventricular (RV) function to afterload remain incompletely understood.In this study, 37 patients with PAH (idiopathic in 31 cases) underwent single-beat pressure–volume loop measurements of RV end-systolic elastance (Ees), arterial elastance (Ea) and diastolic elastance (Eed). Pulmonary arterial stiffness was assessed by magnetic resonance imaging. The results were correlated to CPET variables. The predictive relevance of RV function parameters for clinically relevant ventilatory inefficiency, defined as minute ventilation/carbon dioxide production (V′E/V′CO2) slope >48, was evaluated using logistic regression analysis.The median (interquartile range) of the V′E/V′CO2 slope was 42 (32–52) and the V′E/V′CO2 nadir was 40 (31–44). The mean±sd of peak end-tidal carbon dioxide tension (PETCO2) was 23±8 mmHg. Ea, Eed and parameters reflecting pulmonary arterial stiffness (capacitance and distensibility) correlated with the V′E/V′CO2 slope, V′E/V′CO2 nadir, PETCO2 and peak oxygen pulse. RV Ees and RV–arterial coupling as assessed by the Ees/Ea ratio showed no correlations with CPET parameters. Ea (univariate OR 7.28, 95% CI 1.20–44.04) and Eed (univariate OR 2.21, 95% CI 0.93–5.26) were significantly associated with ventilatory inefficiency (pOur data suggest that impaired RV lusitropy and increased afterload are associated with ventilatory inefficiency in PAH.

The aim of this study was to assess septal and right ventricular insertion point (RVIP) fibrosis in patients with chronic thromboembolic pulmonary hypertension (CTEPH) via native T1 mapping and extracellular volume fraction (ECV) determination and to analyze correlations with functional parameters. Imaging was performed at 1.5 Tesla in 24 patients diagnosed with CTEPH and 24 controls. T1 values were measured within the septal myocardium, the upper and lower RVIP, and the lateral wall at basal short axis section. The mean septal native T1 values were 1012.8 ms ± 50.5 in the CTEPH group and 956.9 ms ± 24.4 in controls (p

Purpose To analyze referral indications, imaging findings and diagnoses in breast sonography in a division of pediatric radiology. Materials and Methods Breast ultrasound examinations of 270 patients were analyzed for referral reasons, imaging findings and final diagnoses (152 females, 118 males). The mean age of the patients was 8.8 years (range, 6 days–18 years). Each breast was examined systematically in two orthogonal probe orientations. Pathological findings were documented on two orthogonal imaging planes. Color Doppler ultrasonography was used additionally. Images and clinical data were reviewed in all cases. Results The most frequent referral reasons in female patients were breast enlargement (104 patients), palpable mass (24 patients) and mastodynia (23 patients). The most frequent diagnoses were normal gland tissue (101 patients), cysts (9 patients), augmented adipose tissue (7 patients) and hemangiomas (7 patients). The most frequent referral reasons in male patients were breast enlargement (106 patients), palpable mass (13 patients) and mastodynia (9 patients). The most frequent diagnoses were gland tissue (79 patients), augmented adipose tissue (24 patients) and cysts (10 patients). Only 2 malignant masses were diagnosed: A Burkitt lymphoma and a relapsed ALL. Conclusion Fear of breast cancer and permanent damage to the breast leads to low-threshold medical consultations and referrals. Sensitive handling is required especially in adolescent patients. Most disorders arise due to the variability of breast development. Ultrasound serves as a means to exclude significant diseases of the breast.

Aims Balloon pulmonary angioplasty (BPA) is an interventional treatment modality for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Therapy monitoring, based on non-invasive biomarkers, is a clinical challenge. This post-hoc study aimed to assess dynamics of high-sensitivity cardiac troponin T (hs-cTnT) as a marker for myocardial damage and its relation to N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels as a marker for cardiac wall stress. Methods and results This study included 51 consecutive patients who underwent BPA treatment and completed a 6-month follow-up (6-MFU) between 3/2014 and 3/2017. Biomarker measurement was performed consecutively prior to each BPA and at 6-MFU. In total, the 51 patients underwent an average of 5 BPA procedures. The 6-month survival rate was 96.1%. The baseline (BL) meanPAP (39.5±12.1mmHg) and PVR (515.8±219.2dyn×sec×cm-5) decreased significantly within the 6-MFU (meanPAP: 32.6±12.6mmHg, P

The aim of this study was to assess native T1 mapping in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) before and 6 months after balloon pulmonary angioplasty (BPA) and compare the results with right heart function and pulmonary haemodynamics. Magnetic resonance imaging at 1.5 T and right heart catheterisation were performed in 21 consecutive inoperable CTEPH patients before and 6 months after BPA. T1 values were measured within the septal myocardium, the upper and lower right ventricular insertion points, and the lateral wall at the basal short-axis section. In addition, the area-adjusted septal native T1 time (AA-T1) was calculated and compared with right ventricular function (RVEF), mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR). The mean AA-T1 value decreased significantly after BPA (1,045.8 ± 44.3 ms to 1,012.5 ± 50.4 ms; p < 0.001). Before BPA, native T1 values showed a moderate negative correlation with RVEF (r = -0.61; p = 0.0036) and moderate positive correlations with mPAP (r = 0.59; p < 0.01) and PVR (r = 0.53; p < 0.05); after BPA correlation trends were present (r = -0.21, r = 0.30 and r = 0.35, respectively). Native T1 values in patients with inoperable CTEPH were significantly lower after BPA and showed significant correlations with RVEF and pulmonary haemodynamics before BPA. Native T1 mapping seems to be indicative of reverse myocardial tissue remodelling after BPA and might therefore have good potential for pre-procedural patient selection, non-invasive therapy monitoring and establishing a prognosis. • BPA is a promising treatment option for patients with inoperable CTEPH • Native septal T1 values significantly decrease after BPA and show good correlations with right ventricular function and haemodynamics before BPA • Prognosis and non-invasive therapy monitoring might be supported in the future by native T1 mapping

BACKGROUND Balloon pulmonary angioplasty (BPA) is an emerging interventional treatment option for chronic thromboembolic pulmonary hypertension (CTEPH). The non-invasive monitoring of CTEPH patients is a clinical challenge. In this study we examined changes in N-terminal pro-B-type natriuretic peptide (NT-proBNP) in patients undergoing BPA for inoperable CTEPH and related them to peri-procedural success. METHODS In this study we analyzed a total of 51 consecutive patients who underwent BPA treatment and completed a 6-month follow-up (6-MFU) between March 2014 and March 2017. Serum samples for NT-proBNP measurement were collected before every BPA and at 6-MFU. RESULTS The 51 patients underwent 265 interventions involving angioplasty of a total of 410 vessels. The 6-month survival rate was 96.1%. The baseline (BL) mean pulmonary artery pressure (PAP) was 39.5 ± 12.1 mm Hg, pulmonary vascular resistance (PVR) was 515.8 ± 219.2 dynes/s/cm5 and the median NT-proBNP level was 820 (153 to 1,871.5) ng/liter. At BL, World Health Organization functional class (FC) was ≥III in 96.1% of the patients, whereas, at 6-MFU, 11.8% were in WHO FC ≥III. At 6-MFU, mean PAP (32.6 ± 12.6 mm Hg; p < 0.001), PVR (396.9 ± 182.6 dynes/s/cm5; p < 0.001) and NT-proBNP (159.3 [84.4 to 464.3] ng/liter; p < 0.001) levels were reduced. The decrease in NT-proBNP levels correlated with the decrease in mean PAP (rrs = 0.43, p = 0.002) and PVR (rrs = 0.50, p = 0.001). A reduction in the NT-proBNP level of 46% indicated a decrease in mean PAP of ≥25% (area under the curve [AUC] = 0.71) and a reduction of 61% indicated a decrease in PVR of ≥35% (AUC 0.77). CONCLUSIONS Our results demonstrate that NT-proBNP levels decrease after BPA, providing valuable evidence of procedural success. NT-proBNP measurement allows identification of patients who are BPA non-responders and may thus be a valuable adjunct in therapy monitoring.

Aim To evaluate the incidence, risk factors, and prognostic implications of contrast medium-induced acute kidney injury (CI-AKI) in patients undergoing transcatheter aortic valve implantation (TAVI) evaluation. Materials and methods Datasets from 98 out of 207 consecutive patients referred for multidetector computed tomography (MDCT) for TAVI evaluation were eligible for evaluation and were analysed retrospectively. The incidence of CI-AKI was correlated to outcome and to potential risk factors: kidney function (estimated glomerular filtration rate [eGFR]), heart failure, diabetes, amount of contrast medium, and duration of examination period. Results CI-AKI occurred in 67 patients (68.4%) and mainly correlated with eGFR (p=0.01) and the amount of contrast medium as a function of eGFR (p=0.04). CI-AKI occurred before TAVI in 36 (53.7%) patients of which 13 (19.4%) did not undergo TAVI. In-hospital all-cause mortality was 21.4%, and of those 21 patients, 18 (85.7%) had CI-AKI and nine (42.9%) did not undergo TAVI. One-year all-cause mortality was 39.8%, and of those 39 patients who died within 1 year, 31 (79.5%) had CI-AKI. Conclusion CI-AKI mostly occurs already before TAVI as a consequence of pre-procedural imaging, which therefore represents the main contributor for CI-AKI in relation to TAVI. Regarding the observation that some patients will ultimately have no benefit because TAVI is not performed and the poor prognosis linked to CI-AKI should encourage improvement in patient selection when referring to pre-procedural imaging.

Purpose Patients suffering from severe blunt abdominal trauma are challenging because of their need for accurate diagnostic imaging and fast therapeutic action. Whole-body computed tomography (WBCT) is highly sensitive and represents the gold standard in the trauma room diagnostic setting. The aim of our study was to investigate the impact and therapy relevance of abdominal follow-up sonography (AFS) as part of the tertiary trauma survey (TTS) in patients without abdominal parenchymal organ lesions or free abdominal fluid in initial WBCT. Materials and Methods All adult patients without abdominal parenchymal organ lesions or free intraabdominal fluid in the initial WBCT examination, who received AFS within 24 hours after trauma, were included in this retrospective analysis between January 2008 and December 2011. Results 316 patients were analyzed (ISS 10 ± 8, NISS 13 ± 11) according to the inclusion criteria. Overall, only small amounts of free intraabdominal fluid were detected in AFS in 3 patients (0.9 %) and remained without therapeutic consequence. None of the patients died due to intraabdominal bleeding. Conclusion AFS as part of the TTS did not show additional benefits and had no impact on further treatment in patients without abdominal parenchymal organ lesions or free intraabdominal fluid in the initial WBCT examination. We conclude that AFS is not routinely required but should be performed if indicated on a clinical or laboratory basis because of its fast and less invasive character. Key points Citation Format

Purpose: Cardiac MRI (CMRI) is an effective method for imaging of the heart. The aim of our study was to assess whether an axial chest sequence in addition to the standard CMR examination setting has advantages in the detection of potentially significant extracardiac findings (PSEF). Materials and Methods: 400 consecutive patients were imaged at 1.5 T for clinical reasons. In addition to the standard long and short-axis views, an axial SSFP sequence was obtained covering the thorax from the lung apex to the diaphragm. All sequences were separately evaluated for PSEF. Results: A total of 25 PSEF were diagnosed in 400 patients, including 16 pleural effusions, a pulmonary fibrosis, a spondylodiscitis, ascites, lymphadenopathies, relapse of a mamma carcinoma, growth of adrenal glands metastases and diaphragmatic elevation. All 25 PSEF were detected by reading survey sequences. 24 of the 25 PSEF were detected by the additional SSFP chest sequence as well as the CINE sequences. Conclusion: In our study the additional axial SSFP chest sequence didn’t show a benefit in the detection of PSEF. With the survey sequences we were able to detect all PSEF. We conclude that survey images should be assessed for additional findings. Citation Format

Background An accurate preoperative assessment of cervical lymph node status is a prerequisite for individually tailored cancer therapies in patients with oral squamous cell carcinoma. The detection of malignant spread and its treatment crucially influence the prognosis. The aim of the present study was to analyze the different staging modalities used among patients with a diagnosis of primary oral squamous cell carcinoma between 2008 and 2015. Material and methods An analysis of preoperative staging findings, collected by clinical palpation, ultrasound, and computed tomography (CT), was performed. The results obtained were compared with the results of the final histopathological findings of the neck dissection specimens. A statistical analysis using McNemar's test was performed. Results The sensitivity of CT for the detection of malignant cervical tumor spread was 74.5%. The ultrasound obtained a sensitivity of 60.8%. Both CT and ultrasound demonstrated significantly enhanced sensitivity compared to the clinical palpation with a sensitivity of 37.1%. No significant difference was observed between CT and ultrasound. A combination of different staging modalities increased the sensitivity significantly compared with ultrasound staging alone. No significant difference in sensitivity was found between the combined use of different staging modalities and CT staging alone. The highest sensitivity, of 80.0%, was obtained by a combination of all three staging modalities: clinical palpation, ultrasound and CT. Conclusions The present study indicates that CT has an essential role in the preoperative staging of patients with oral squamous cell carcinoma. Its use not only significantly increases the sensitivity of cervical lymph node metastasis detection but also offers a preoperative assessment of local tumor spread and resection borders. An additional non-invasive cervical lymph node examination increases the sensitivity of the tumor staging process and reduces the risk of occult metastasis.

Analyzing occluded segments with computed tomography angiography (CTA) prior to percutaneous coronary intervention (PCI) increased revascularization success in chronic total occlusions (CTO). The aim of our study was to develop a scoring system for the prediction of PCI success in CTO.41 consecutive CTO patients (30 male; 63.1 years +/- 8.3 standard deviation) underwent CTA prior to PCI. All CTOs were categorized by two radiologists in consensus regarding the presence of special features and without knowledge of PCI outcome. All outcome criteria were evaluated. Afterwards one point was assigned for each unequally distributed outcome criteria per CTO and all points were added up to a single score.Severe calcifications (failure group 68.8 % vs. success group 28.0 %; p 0.02) and intraluminal calcifications (68.8 %; 40.0 %), tortuosity (25.0 %; 0 %; p 0.02), linear intrathrombus enhancement (37.5 %; 60.0 %) and distal vessel disease (68.8 %; 44.0 %) were unequally distributed. By adopting a threshold of 4 points or higher (maximum 5 points), the results were: sensitivity 31.3 %, specificity 100 %, negative predictive value (NPV) 69.4 % and positive predictive value (PPV) 100 %. The PCI complication rate was 9.8 % and the mean contrast media amount was 234.4 ml.With the suggested scoring system, based on five CTA criteria, PCI failure could be predicted with high PPV and specificity in our group of patients, but the NPV and sensitivity are low. However, 5 unsuccessful PCIs (13.2 %) could have been avoided and none would have been wrongly omitted. Regarding the complication rate during PCI and the high amounts of contrast media needed, a prediction system appears to be desirable and should be the object of large-scale trials.Single predictors of revascularization success in CTO have been identified. Success rates are improved by analyzing CTA data sets prior to revascularization approaches. Prediction of revascularization success via a scoring system based on five CTA criteria seems promising. Patient selection for the right treatment options might be improved in the future due to application of the scoring system. Also risks, complications, contrast media amounts and radiation doses might be reduced.

Cardiac magnetic resonance imaging (CMRI) is a versatile diagnostic tool. One of its main advantages is the possibility of tissue characterization. T1-weighted images for scar and T2-weighted images for edema visualization are key methods for tissue characterization. Otherwise these sequences are strongly limited for the detection of diffuse myocardial pathologies. Recently, rapid technical innovations have generated new techniques. T1, T2 mapping and evaluation of the extracellular volume fraction (ECV) allow quantification of diffuse myocardial pathologies and showed great potential in the visualization of fibrosis, edema, amyloid, iron overload and lipid. In the future these techniques might enable the detection of early cardiac involvement, even act as a prognosticator. Moreover, therapy monitoring and follow-up might be possible due to versatile parameter quantification with these new techniques. Key points: • CMR allows for tissue characterization via T1- and T2-weighted sequences. • In cases of diffuse, global myocardial pathologies, correct image interpretation with traditional CMR sequences might be difficult. • T1, T2 mapping and ECV can quantify diffuse, global myocardial pathologies. • Alterations of myocardial T1 and T2 relaxation times occur in various myocardial diseases (e. g. acute myocarditis). • In the future mapping might act as a prognosticator or therapy monitoring tool. Citation Format: • Roller FC, Harth S, Schneider C et al. T1, T2 Mapping and Extracellular Volume Fraction (ECV): Application, Value and Further Perspectives in Myocardial Inflammation and Cardiomyopathies. Fortschr Rontgenstr 2015; 187: 760 – 770