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Objectives To define and compare growth rates of the distal aorta in Marfan patients with and without aortic root replacement using serial MR angiography (MRA). Methods We retrospectively included 136 Marfan patients with a total of 645 MRAs who underwent a median of five MRAs (range: 2–13) at 1.5 T and 3 T in annual intervals. Of these, 41 patients (34.8 ± 12 years) had undergone aortic root replacement. The remaining 95 patients (29.0 ± 17 years) still had a native aorta and served as the control group. Thoracic aortic diameters were independently measured at eleven predefined levels. Estimated growth rates were calculated using a mixed effects model adjusted for sex, age, BMI, and medication. Results Marfan patients with aortic root replacement revealed the highest mean estimated growth rate in the proximal descending aorta (0.77 mm/year, CI: 0.31–1.21). Mean growth rates at all levels of the distal thoracic aorta were significantly higher in patients with aortic root replacement (0.28–0.77 mm/year) when compared to patients without aortic root replacement (0.03–0.07 mm/year) (all p Conclusion Distal thoracic aortic diameters increase at a significantly higher rate in Marfan patients with aortic root replacement compared to Marfan patients without aortic root replacement. Further studies are warranted to investigate if the increased growth rate of the distal thoracic aorta after aortic root replacement is caused by altered hemodynamics due to the rigid aortic root graft or due to the general genetic disposition of post-operative Marfan patients. Clinical relevance statement High growth rates of the distal aorta after aortic root replacement underline the need for careful life-long aortic imaging of Marfan patients after aortic root replacement. Key Points • Aortic growth rates in Marfan patients with aortic root replacement are highest in the mid-aortic arch, the proximal- and mid-descending aorta. • Growth rates of the distal thoracic aorta are significantly higher in Marfan patients with aortic root replacement compared to Marfan patients without aortic root replacement. • Antihypertensive medication, gender, and BMI have no significant impact on distal aortic growth rates in Marfan patients.

To evaluate the sensitivity, specificity, and interobserver reliability of high-pitch dual-source computed tomography angiography (CTA) in the detection of anomalous pulmonary venous connection (APVC) in infants with congenital heart defects and to assess the associated radiation exposure. 78 pulmonary veins in 17 consecutively enrolled patients with congenital heart defects (6 females; 11 males; median age: 6 days; range: 1-299 days) were retrospectively included in this study. All patients underwent high-pitch dual-source CTA of the chest at low tube voltages (70 kV). APVC was evaluated independently by two radiologists. Sensitivity, specificity, positive (PPV) and negative predictive values (NPV), and interobserver agreement were determined. For standard of reference, one additional observer reviewed CT scans, echocardiography reports, clinical reports as well as surgical reports. In cases of disagreement the additional observer made the final decision based on all available information. Detection of APVC with high-pitch dual-source CTA revealed a good sensitivity (91 %) and specificity (99 %), with PPV and NPV of 98 % and 97 %. Interobserver agreement was almost perfect (Kappa = 0.84). The median DLP was 3.8 mGy*cm (IQR 3.3-4.7 mGy*cm) and the median radiation dose was 0.33 mSv (IQR 0.26-0.39 mSv). High-pitch dual-source CTA in infants with congenital heart defects allows for accurate and reliable assessment of APVC at a low radiation dose. · High-pitch dual-source CTA enables detection of anomalous pulmonary vein connection with high sensitivity in infants.. · Interrater reliability in the detection of anomalous pulmonary vein connection with high-pitch dual-source CTA is almost perfect.. · Radiation dose of high-pitch dual-source CTA in the cardiac examination of infants is low..· Well L, Weinrich JM, Meyer M et al. Sensitivity of High-Pitch Dual-Source Computed Tomography for the Detection of Anomalous Pulmonary Venous Connection in Infants. Fortschr Röntgenstr 2021; 193: 551 - 558.ZIEL: Bewertung der Sensitivität, Spezifität und Interrater-Reliabilität der High-Pitch-Dual-Source-Computertomografie-Angiografie (CTA) in der Detektion von Pulmonalvenenfehlmündungen bei Säuglingen mit angeborenen Herzfehlern und Bewertung der damit verbundenen Strahlenbelastung. In diese retrospektive Studie wurden 78 Lungenvenen aus 17 konsekutiv untersuchten Säuglingen mit angeborenen Herzfehlern eingeschlossen (6 Mädchen, 11 Jungen; Medianalter 6 Tage, Range 1–299 Tage). Bei allen Patienten wurde eine High-Pitch-Dual-Source-CTA des Thorax bei niedriger Röhrenspannung (70 kV) durchgeführt. Das Vorliegen von Pulmonalvenenfehlmündungen wurde von 2 Radiologen unabhängig voneinander bewertet. Sensitivität, Spezifität, positive (PPV) und negative prädiktive Vorhersagewerte (NPV) sowie die Interrater-Reliabilität wurden bestimmt. Als Referenzstandard diente die Kombination aus CT-Scans, Befunden der Echokardiografie, klinischen Berichten sowie Operationsberichten, die von einem erfahrenen Kinderradiologen in Zusammenschau ausgewertet wurden. Dieser entschied im Fall inkongruenter Befunde über die korrekte Interpretation. Die Detektion von Pulmonalvenenfehlmündungen mit der High-Pitch-Dual-Source-CTA gelang mit guter Sensitivität (91 %) und Spezifität (99 %), bei PPV und NPV von 98 % und 97 %. Die Interrater-Reliabilität war nahezu perfekt (Kappa = 0,84). Der Median des DLP betrug 3,8 mGy*cm (IQR 3,3–4,7 mGy*cm), die effektive Dosis betrug im Median 0,33 mSv (IQR 0,26–0,39 mSv). Die High-Pitch-Dual-Source-CTA bei Säuglingen mit angeborenen Herzfehlern ermöglicht eine genaue und zuverlässige Detektion von Lungenvenenfehlmündungen bei niedriger Strahlendosis. · Die High-Pitch-Dual-Source-CTA ermöglicht die Detektion von Pulmonalvenenfehlmündungen bei Säuglingen mit hoher Sensitivität.. · Die Interrater-Reliabilität bei der Erkennung von Pulmonalvenenfehlmündungen mit High-Pitch-Dual-Source-CTA ist hoch.. · Die Strahlendosis der High-Pitch-Dual-Source-CTA bei der kardialen Untersuchung von Säuglingen ist gering..

Aortic diameter measurements play a crucial role for the indication of aortic root surgery in Marfan patients. However, for magnetic resonance angiography (MRA)-derived measurements, there is no consensus on whether the aortic wall should be included or excluded in the aortic diameter. The purpose of this retrospective study was to compare the reliability of non-contrast bright blood MRA aortic inner-to-inner and outer-to-outer edge measurements in patients with Marfan syndrome. Forty Marfan patients underwent ECG-gated balanced steady-state free-precession MRA of the aorta at 1.5 T. Two readers independently performed inner and outer measurements at different aortic levels. They rated the image quality of the delineation of both inner and outer vessel wall edges on a four-point scale. MRA-derived diameters of the sinuses of Valsalva were compared with echocardiography-derived diameters. Aortic vessel wall delineation score was rated higher at all levels for inner than for outer vessel walls (p

Background Aortic valve repair has become a treatment option for adults with symptomatic bicuspid (BAV) or unicuspid (UAV) aortic valve insufficiency. Our aim was to demonstrate the feasibility of 4D flow cardiovascular magnetic resonance (CMR) to assess the impact of aortic valve repair on changes in blood flow dynamics in patients with symptomatic BAV or UAV. Methods Twenty patients with adult congenital heart disease (median 35 years, range 18–64; 16 male) and symptomatic aortic valve regurgitation (15 BAV, 5 UAV) were prospectively studied. All patients underwent 4D flow CMR before and after aortic valve repair. Aortic valve regurgitant fraction and systolic peak velocity were estimated. The degree of helical and vortical flow was evaluated according to a 3-point scale. Relative flow displacement and wall shear stress (WSS) were quantified at predefined levels in the thoracic aorta. Results All patients underwent successful aortic valve repair with a significant reduction of aortic valve regurgitation (16.7 ± 9.8% to 6.4 ± 4.4%, p p = 0.014). Both helical flow (1.6 ± 0.6 vs. 0.9 ± 0.5, p p = 0.002) as well as both flow displacement (0.3 ± 0.1 vs. 0.25 ± 0.1, p = 0.031) and WSS (0.8 ± 0.2 N/m2 vs. 0.5 ± 0.2 N/m2, p Conclusions 4D flow CMR allows assessment of the impact of aortic valve repair on changes in blood flow dynamics in patients with bicuspid aortic valve disease.

Patients with genetic aortic syndromes such as Marfan or Loeys-Dietz syndrome have a decreased life expectancy due to the risk of aortic dissection and rupture. Imaging plays an important role in the acute setting but also in the initial diagnosis and image-based monitoring. In this article, we provide an overview of the most common genetic aortic syndromes and recommended imaging strategies. Furthermore, we highlight modern imaging methods allowing for the quantification of hemodynamic changes in aortic disease. This is a narrative review article on genetic aortic syndromes and recommended imaging strategies, where we take into account expert opinions and standard-of-care practices from our own center. Radiological imaging plays a key role in the initial diagnosis and surveillance of patients with genetic aortic syndromes. Radiologists contribute significantly to the multi-disciplinary setting of genetic aortic syndromes with knowledge of special features and recommended imaging methods. Accurate measurement of the aorta is crucial, particularly in terms of diameter-based surgical treatment algorithms. Modern imaging methods like 4D-flow MRI and pulse wave velocity have a potential to further improve individualized risk stratification in patients with genetic aortic syndromes. · The risk for cardiovascular complications such as acute aortic syndrome is increased in patients with genetic aortic syndromes.. · Recommended time intervals between image-based monitoring depend on the underlying aortic disease.. · CT-angiography should be used only in the acute setting.. · Non-contrast MR-angiography is adequate for screening and image-based monitoring of patients with genetic aortic syndromes..· Weinrich JM, Lenz A, Girdauskas E et al. Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes. Fortschr Röntgenstr 2020; 192: 50 - 58. Genetische Aortenerkrankungen wie das Marfan- oder Loeys-Dietz-Syndrom umfassen ein relativ kleines, jedoch wichtiges kardiovaskuläres Patientenkollektiv. Die betroffenen Patienten unterscheiden sich durch diverse Phänotypen, haben jedoch alle bereits in jungem Alter ein sehr hohes Risiko für ein akutes Aortensyndrom. Sowohl in der Akutsituation als auch im Rahmen der Vorsorge ist die Schnittbildgebung unverzichtbar. In diesem Übersichtsartikel stellen wir die häufigsten genetischen Aortenerkrankungen und die für sie empfohlene Bildgebung vor. Darüber hinaus geben wir einen Ausblick auf moderne Methoden zur Erfassung der Hämodynamik bei Aortenerkrankungen. Diese Übersichtsarbeit basiert auf der Kombination publizierter Expertenmeinungen sowie den klinischen Standards unseres auf Aortenerkrankungen spezialisierten Zentrums. Die radiologische Bildgebung ist zentraler Bestandteil der initialen Diagnosestellung und Verlaufskontrolle genetischer Aortenerkrankungen und unterscheidet sich in Abhängigkeit der Grunderkrankung. Durch Kenntnis der Besonderheiten genetischer Aortenerkrankungen und der empfohlenen Bildgebungstechniken trägt der Radiologe wesentlich zur optimalen Patientenbetreuung bei. Die genaue Vermessung der Aortendiameter beeinflusst maßgeblich den interdisziplinären Beschluss eines prophylaktischen chirurgischen Aortenersatzes. Moderne Bildgebungstechniken wie die 4D-Fluss-MRT und die Pulswellengeschwindigkeit haben das Potenzial, die individualisierte Risikostratifizierung bei Patienten mit genetischen Aortenerkrankungen zu verbessern. · Patienten mit genetischen Aortenerkrankungen haben bereits in jungem Alter ein erhöhtes Risiko für vaskuläre Komplikationen wie das akute Aortensyndrom.. · Die Schnittbildgebung ist sowohl in der Akutsituation als auch im Rahmen der Vorsorge unverzichtbar.. · Die empfohlenen Zeitabstände und die Zielorgane für bildgebende Verlaufsuntersuchungen unterscheiden sich in Abhängigkeit von der Aggressivität der genetischen Aortenerkrankung.. · Die CT-Angiografie sollte nur in der Akutsituation oder in der präoperativen bzw. präinterventionellen Therapieplanung eingesetzt werden.. · Im Rahmen der Vorsorge empfiehlt sich die native MR-Angiografie der Aorta..· Weinrich JM, Lenz A, Girdauskas E et al. Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes. Fortschr Röntgenstr 2020; 192: 50 – 58.

We present a patient with severe tracheal stenosis resulting from a compression by the innominate artery 6 months after an arterial switch operation in a dextro-transposition of the great arteries. Segmentation and three-dimensional (3D) visualization were derived from a contrast-enhanced dual-source computed tomography and post-processing was performed using a dedicated open-source platform (3D Slicer). Post-processing allowed a comprehensible visualization of the relationship of the innominate artery to the trachea when compared to standard computer tomography reformations. Finally, the surgical approach to move the innominate artery anteriorly in order to relieve the tracheal obstruction was emphasized based on the improved 3D visualization of the actual pathology. An effective aortopexy could be performed and the postoperative result was confirmed by a second 3D visualization. About 3 months of follow-up, the patient is completely asymptomatic. Three-dimensional visualization offers excellent opportunities for diagnosis, treatment planning and follow-up in patients with a vascular-related tracheal stenosis in the context of congenital heart disease.

PurposeFeasibility of a highly reduced-dose lumbar spine CT protocol using iterative reconstruction (IR) in a human cadaver model.Materials and methodsThe lumbar spine of 20 human cadavers was repeatedly examined using three different reduced-dose protocols (RDCT) with decreasing reference tube current-exposure time products (RDCT-1: 50 mAs; RDCT-2: 30 mAs; RDCT-3: 10 mAs) at a constant tube voltage of 140 kV. A clinical standard-dose protocol (SDCT) served as the reference (reference tube current-exposure time product: 70 mAs; tube voltage: 140 kV). Images were reconstructed using filtered back projection (FBP) and two increasing levels of IR: IRL4 and IRL6. A five-point scale was used by two observers to assess the diagnostic quality of anatomical structures (cortical and trabecular bone, intervertebral foramina, pedicles and intervertebral joints, spinous and transverse processes). Objective image noise (OIN) was measured. Results were interpreted using a linear mixed-effects regression model.ResultsRDCT-2 with IRL6 (1.2 ± 0.5mSv) was the lowest reduced-dose protocol which provided diagnostically acceptable and equivalent image quality compared to the SDCT (2.3 ± 1.1mSV) with FBP (p > 0.05). All RDCT protocols achieved a significant reduction of the mean (±SD) effective radiation doses (RDCT-1: 1.7±0.9mSv; RDCT-2: 1.2±0.5mSv; RDCT-3: 0.4±0.2mSv; p < 0.05) compared to SDCT. OIN was lower in all RDCT protocols with the application of IRL4 and IRL6, compared to the SDCT with FBP (p < 0.05).ConclusionHighly reduced-dose lumbar spine CT providing diagnostically acceptable image quality is feasible using IR in this cadaver model and may be transferred into a clinical setting.

Objectives This study analyzed the presence of myocardial fibrosis detected by late gadolinium-enhancement (LGE) cardiac magnetic resonance (CMR) in correlation with the performance of competitive triathletes objectified by an exercise test and individual competition history. Background Myocardial fibrosis detected by LGE CMR has been reported to occur in 0% to 50% of asymptomatic athletes. However, the cause and mechanisms of myocardial fibrosis are unclear. Methods Eighty-three asymptomatic triathletes undergoing >10 training h per week (43 ± 10 years of age; 65% male) and 36 sedentary controls were studied by using LGE and extracellular volume (ECV) CMR. Parameters of physical fitness were measured by spiroergometry. Triathletes reported their lifetime competition results. Results LGE CMR revealed focal nonischemic myocardial fibrosis in 9 of 54 (17%) male triathletes (LGE + ) but in none of the female triathletes (p + triathletes had higher peak exercise systolic blood pressure (213 ± 24 mm Hg) than LGE − triathletes (194 ± 26 mm Hg; p + triathletes (93 ± 7 g/m 2 ) than in LGE − triathletes (84 ± 11 g/m 2 ; p 0.05). ECV in LGE − myocardium was higher in LGE + triathletes (26.3 ± 1.8%) than in LGE − triathletes (24.4 ± 2.2%; p + triathletes completed longer cumulative distances in swimming and cycling races and participated more often in middle and Iron Man distances than LGE − triathletes. A cycling race distance of >1,880 km completed during competition had the highest accuracy to predict LGE, with an area under the curve value of 0.876 (p Conclusions Myocardial fibrosis in asymptomatic triathletes seems to be associated with exercise-induced hypertension and the race distances. There appears to be a safe upper limit, beyond which exercise may result in myocardial fibrosis.

Background To determine the type and frequency of vascular and organ malformations in adults with thalidomide embryopathy (TE) using non-contrast magnetic resonance angiography (MRA) and to assess the effect of the observed malformations on renal function. Methods and Results: The institutional ethics committee approved this prospective study and written informed consent was given by all 78 subjects (50 females) with TE (mean age: 55±1.1 years), who were examined by non-contrast MRA at 3T. ECG-triggered balanced turbo field echo images of the chest, abdomen and pelvis were obtained in coronal and sagittal orientations. Two observers assessed the frequency of vascular and organ malformations. Serum creatinine and estimated glomerular filtration rate (eGFR) were obtained to assess renal function. In 58 subjects, 99 vascular anomalies were observed, including 68 arterial (69%) and 31 venous anomalies (31%); 15 patients had 16 abdominal organ malformations including 12 kidney anomalies and 4 cases of gallbladder agenesis. Most vascular anomalies affected the renal vessels (n=66, 67%) or supraaortic arteries (n=28, 28%). Serum creatinine and eGFR revealed normal renal function in all subjects. Conclusions Vascular and organ anomalies occurred in a high number of subjects with TE without evidence of renal dysfunction. Information about the presence of malformations may be important for future surgical interventions in subjects with TE.

Gadoxetate disodium is an intracellular contrast agent for magnetic resonance imaging (MRI) of the liver. Recent publications revealed that injection of gadoxetate disodium can lead to imaging artifacts due to transient severe motion (TSM) in the arterial phase of contrast-enhanced liver MRI. In this review we present and discuss published frequencies of TSM, contrast injection and image acquisition protocols, potential risk factors, and proposed strategies to avoid or minimize the effects of TSM.Two reviewers independently searched the PubMed search engine for "transient severe motion artifact" and related terms. Reference lists of retrieved articles were also searched. The two reviewers selected in consensus nine studies that reported both frequencies of TSM and potential risk factors. Study data were extracted by both reviewers, and disagreement was resolved by consensus.TSM is caused by impaired breath-hold ability after gadoxetate disodium injection and occurs in 5 - 22 % of patients. The dose of applied contrast agent, repeated exposure to gadoxetate disodium, high BMI and pulmonary disease have been described as potential risk factors for TSM. However, there are only few concordant results on this topic and the pathophysiology of TSM has not been identified. Proposed strategies for the prevention of TSM are slow injection rates and low doses of diluted gadoxetate disodium. Accelerated and free-breathing MRI sequence protocols and breath-hold training may minimize the effects of TSM. Further prospective studies are needed to confirm these strategies and to identify the underlying mechanism of TSM.· TSM occurs in 5 - 22 % of patients after gadoxetate disodium injection.. · Potential risk factors of TSM are dose, repeated exposure, BMI, pulmonary disease.. · The underlying mechanism for TSM has not been identified.. · Slow injection rates and diluted gadoxetate disodium may prevent TSM.. · Accelerated image acquisition or free-breathing sequences may mitigate the effects of TSM..· Well L, Weinrich JM, Adam G et al. Transient Severe Respiratory Motion Artifacts After Application of Gadoxetate Disodium: What We Currently Know. Fortschr Röntgenstr 2018; 190: 20 - 30.Dinatriumgadoxetat ist ein intrazelluläres Kontrastmittel für die Leberbildgebung in der Magnetresonanztomografie (MRT). In aktuellen Publikationen wurde das Auftreten transienter schwerer Atemartefakte (TSA) nach Gabe von Dinatriumgadoxetat beschrieben, die zur Reduktion der Bildqualität in der arteriellen Konstrastmittelphase führen. In diesem Übersichtsartikel vergleichen wir den Einfluss publizierter Untersuchungsprotokolle und potentieller Risikofaktoren auf die Häufigkeit des Auftretens der TSA. Zudem diskutieren wir vorgeschlagene Strategien zur Vermeidung oder Minimierung der Effekte der TSA.Diese Übersichtsarbeit basiert auf einer Literaturrecherche der PubMed Datenbank, welche nach „transient severe motion artifact“ und verwandten Begriffen unabhängig voneinander von zwei Autoren durchsucht wurde. Die Literaturverzeichnisse der identifizierten Arbeiten wurden ebenfalls durchsucht. Zwei Autoren wählten gemeinsam neun Arbeiten aus, in denen sowohl die Frequenz der TSA als auch potentielle Risikofaktoren untersucht wurden. Relevante Studiendaten wurden von den Autoren extrahiert, Diskrepanzen der extrahierten Daten wurden im Konsens gelöst.Die TSA werden durch eine Beeinträchtigung der Atemanhaltefähigkeit nach Gabe von Dinatriumgadoxetat verursacht und treten in 5 – 22 % aller Dinatriumgadoxetat-verstärkten Leber-MRTs auf. Die Kontrastmitteldosis, wiederholte Dinatriumgadoxetat-Exposition, hoher Body Mass Index sowie pulmonale Erkrankungen werden als potentielle Risikofaktoren für das Auftreten der TSA beschrieben. Allerdings finden sich nur wenig übereinstimmende Resultate hinsichtlich der Risikofaktoren, und die ursächliche Pathophysiologie der TSA ist bis heute ungeklärt. Vorgeschlagene Strategien zur Vermeidung der TSA sind niedrige Injektionsraten und Gabe von verdünntem, niedrig dosiertem Dinatriumgadoxetat. Durch kürzere Sequenzen, Aufnahmen ohne Atemstillstand und Atemtraining der Patienten soll der Effekt der TSA minimiert werden. Zukünftige prospektive Studien müssen diese Strategien bestätigen und den zugrundeliegenden Mechanismus für das Auftreten der TSA ermitteln.· Die Dinatriumgadoxetat-verstärkte Leber-MRT führt in 5 – 22 % zu transienten schweren Atemartefakten.. · Potentielle Risikofaktoren für TSA sind Kontrastmitteldosis, wiederholte Dinatriumgadoxetat-Gabe, BMI sowie Lungenerkrankungen.. · Die Ursache für das Auftreten von TSA ist bislang ungeklärt.. · Niedrige Injektionsraten und verdünntes Dinatriumgadoxetat sollen das Auftreten von TSA vermeiden.. · Kürzere oder durchgeatmete MRT-Sequenzen sollen den Effekt der TSA minimieren..

Objective To quantify standard values of the discus interpubicus in healthy subjects and to determine reliability and repeatability using T2 relaxation time measurements at 3T. Methods 20 asymptomatic participants (10 male, 10 female; mean age: 27.3 years ±4.1, BMI: 22.2 ±1.8) underwent a 3T Magnetic Resonance Imaging (MRI) of the pelvic region in a supine position. We included sagittal and para-axial T2w sequences centred over the pubic symphysis in order to identify the complete discus interpubicus. For quantitative analysis, a multi-echo Turbo Spin Echo (TSE) sequence (including 12 echo times between 6.4 and 76.8 ms) was acquired and analysed by using an in-house developed quantification plugin tool (qMapIt) extending ImageJ. Two readers in consensus defined three central slices of the pubic symphysis with the greatest length. For each slice, both readers separately placed three regions-of-interest (ROI) covering the whole discus interpubicus. Both readers repeated the ROI placements in identical fashion after a four-week interval on the original MRI images. Statistical analysis included intraclass correlation coefficient (ICC), nonparametric Wilcoxon test, Fisher exact test and mean relaxation time in ms and 95% confidence intervals. Results T2 relaxation time analysis was performed for all 20 participants. In total, a mean relaxation time of all analysed segments for both observers was 48.6 (±6.3 ms), with a mean relaxation time for observer 1 of 48.7 (±6.0 ms) and for observer 2 of 48.5 ms (±6.6ms). The calculated ICC comparing inter- and intrarater reproducibility was excellent in all segments (≥0.75). Conclusion T2 mapping of the discus interpubicus demonstrates good inter- and intrarater repeatability as well as reliability. Mean relaxation times were calculated with 48.6ms in healthy volunteers.