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Resumen El termino «tecnicas de imagen hibridas en cardiopatia isquemica» se refiere a la fusion de la informacion obtenida con varias tecnicas de imagen en dicho contexto clinico, lo que permite evaluar la presencia, la extension y la gravedad de la enfermedad coronaria ateroesclerotica junto con la importancia hemodinamica de las lesiones o la funcion miocardica. Se ha adoptado en varios centros la fusion de las imagenes de la coronariografia no invasiva obtenidas por tomografia computarizada con las de perfusion miocardica obtenidas por tomografia computarizada por emision monofotonica o tomografia por emision de positrones, y su implementacion se recoge en las guias internacionales de enfermedad arterial coronaria. Las nuevas modalidades hibridas que incorporan la estimacion de la reserva fraccional de flujo y la tomografia computarizada de perfusion han ganado terreno, lo cual resulta muy prometedor para la evaluacion diagnostica y terapeutica de los pacientes con enfermedad arterial coronaria. En esta revision se comentan las tecnicas de imagen hibridas no invasivas disponibles actualmente en la practica clinica y las futuras y prometedoras innovaciones tecnologicas en este campo.

Purpose To compare the diagnostic accuracy of different computed tomographic (CT) fractional flow reserve (FFR) algorithms for vessels with intermediate stenosis. Materials and Methods This cross-sectional HIPAA-compliant and human research committee-approved study applied a four-step CT FFR algorithm in 61 patients (mean age, 69 years ± 10; age range, 29-89 years) with a lesion of intermediate-diameter stenosis (25%-69%) at CT angiography who underwent FFR measurement within 90 days. The per-lesion diagnostic performance of CT FFR was tested for three different approaches to estimate blood flow distribution for CT FFR calculation. The first two, the Murray law and the Huo-Kassab rule, used coronary anatomy; the third used contrast material opacification gradients. CT FFR algorithms and CT angiography percentage diameter stenosis (DS) measurements were compared by using the area under the receiver operating characteristic curve (AUC) to detect FFRs of 0.8 or lower. Results Twenty-five lesions (41%) had FFRs of 0.8 or lower. The AUC of CT FFR determination by using contrast material gradients (AUC = 0.953) was significantly higher than that of the Huo-Kassab (AUC = 0.882, P = .043) and Murray law models (AUC = 0.871, P = .033). All three AUCs were higher than that for 50% or greater DS at CT angiography (AUC = 0.596, P < .001). Correlation of CT FFR with FFR was highest for gradients (Spearman ρ = 0.80), followed by the Huo-Kassab rule (ρ = 0.68) and Murray law (ρ = 0.67) models. All CT FFR algorithms had small biases, ranging from -0.015 (Murray) to -0.049 (Huo-Kassab). Limits of agreement were narrowest for gradients (-0.182, 0.147), followed by the Huo-Kassab rule (-0.246, 0.149) and the Murray law (-0.285, 0.256) models. Conclusion Clinicians can perform CT FFR by using a four-step approach on site to accurately detect hemodynamically significant intermediate-stenosis lesions. Estimating blood flow distribution by using coronary contrast opacification variations may improve CT FFR accuracy. © RSNA, 2017 Online supplemental material is available for this article.

Aims Fractional flow reserve (FFR) estimated from coronary computed tomography angiography (CT-FFR) offers non-invasive detection of lesion-specific ischaemia. We aimed to develop and validate a fast CT-FFR algorithm utilising the Lattice Boltzmann method for blood flow simulation (LBM CT-FFR). Methods and results Sixty-four patients with clinically indicated CTA and invasive FFR measurement from three institutions were retrospectively analysed. CT-FFR was performed using an onsite tool interfacing with a commercial Lattice Boltzmann fluid dynamics cloud-based platform. Diagnostic accuracy of LBM CT-FFR ≤0.8 and percent diameter stenosis >50% by CTA to detect invasive FFR ≤0.8 were compared using area under the receiver operating characteristic curve (AUC). Sixty patients successfully underwent LBM CT-FFR analysis; 29 of 73 lesions in 69 vessels had invasive FFR ≤0.8. Total time to perform LBM CT-FFR was 40±10 min. Compared to invasive FFR, LBM CT-FFR had good correlation (r=0.64), small bias (0.009) and good limits of agreement (-0.223 to 0.206). The AUC of LBM CT-FFR (AUC=0.894, 95% confidence interval [CI]: 0.792-0.996) was significantly higher than CTA (AUC=0.685, 95% CI: 0.576-0.794) to detect FFR ≤0.8 (p=0.0021). Per-lesion specificity, sensitivity, and accuracy of LBM CT-FFR were 97.7%, 79.3%, and 90.4%, respectively. Conclusions LBM CT-FFR has very good diagnostic accuracy to detect lesion-specific ischaemia (FFR ≤0.8) and can be performed in less than one hour.

AIMS: Positron emission tomography (PET) myocardial perfusion imaging (MPI) can non-invasively measure myocardial blood flow reserve (MBFR). We aimed to examine whether MBFR identifies patients with a survival benefit after revascularization, helping to guide post-test management. METHODS AND RESULTS: We examined all-cause mortality in 12 594 consecutive patients undergoing Rb(82) rest/stress PET MPI from January 2010 to December 2016, after excluding those with cardiomyopathy, prior coronary artery bypass surgery (CABG), and missing MBFR. Myocardial blood flow reserve was calculated as the ratio of stress to rest absolute myocardial blood flow. A Cox model adjusted for patient and test characteristics, early revascularization (percutaneous coronary intervention or CABG ≤90 days of MPI), and the interaction between MBFR and early revascularization was developed to identify predictors of all-cause mortality. After a median follow-up of 3.2 years, 897 patients (7.1%) underwent early revascularization and 1699 patients (13.5%) died. Ischaemia was present in 4051 (32.3%) patients, with 1413 (11.2%) having ≥10% ischaemia. Mean MBFR was 2.0 ± 1.3, with MBFR

Objectives: Recommendations regarding sports restriction are lacking for middle-aged athletes with anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). Methods: Sixty-three patients with ACAOS were subdivided into ACAOS with (n = 38) or without (n = 25) an interarterial course (IAC). Sports behavior, either competitive (COMP) or recreational (REC), was evaluated at the time of diagnosis and after a median follow-up of 4.2 years. Results: Mean age was 56 ± 11 years and 48 (76.2%) patients were engaged in sports. Three individuals (4.8%) were surgically corrected after diagnosis. Thirty-eight (60.3%) patients were aware of their diagnosis at follow-up and 12 (19.0%) were counseled by their physician about sports restrictions. Sports behavior at the time of diagnosis and at follow-up did not differ significantly, neither in patients engaged in COMP (17.5 vs. 12.7%, p = 0.619) nor those engaged in REC (58.7 vs. 61.9%, p = 0.856). Sport-related symptoms were not significantly different between ACAOS patients with and without IAC. No athlete had died at follow-up. Conclusions: The majority of middle-aged individuals with ACAOS were involved in sports activities at the time of diagnosis and at follow-up. Awareness and counseling about ACAOS diagnosis had no significant effect on sports behavior. IAC did not have an impact on sport-related symptoms, and outcomes were favorable in all athletes, regardless of surgical correction.

Background Risk assessment in the expanding population of adult patients with congenital heart disease (ACHD) is of high importance. Aim To report clinical characteristics, complications, mortality rate and causes of death in a single-centre ACHD cohort. Methods Data were prospectively collected from an ACHD outpatient clinic from January 2012 until December 2019. Results Of 356 patients (median age 34 years, 52.5% female), 116 (32.6%) were classified as simple, 182 (51.1%) as moderate and 58 (16.3%) as complex ACHD. At baseline assessment, more than half (n ​= ​184) were symptomatic, while 69 patients (19.4%) had a history of arrhythmia and 21 (5.9%) a history of heart failure (HF) hospitalisation. Over a median follow-up of 4 years (1–7 years), 29 (8.1%) patients died, mainly due to HF. In multivariate Cox regression analysis, age [hazard ratio (HR) 1.03 (confidence interval (CI) 1.00–1.05), p ​= ​0.034], Bethesda classification [HR 1.77 (CI 1.05–3.02), p ​= ​0.034], NYHA class III/IV [HR 3.28 (CI 1.19–9.04), p ​= ​0.022] and history of HF hospitalisation [HR 3.79 (CI 1.27–11.30), p ​= ​0.017] predicted all-cause death. A total of 16.3% of patients developed complications, which were independently predicted by age [HR 1.03 (CI 1.01–1.05), p ​= ​0.004], history of surgical procedure [HR 1.45 (CI 1.12–1.86), p ​= ​0.004] and history of HF hospitalisation [HR 3.38 (CI 1.56–7.29), p ​= ​0.002]. Conclusion HF was the leading cause of death in this ACHD cohort and HF hospitalisation was a predictor of morbidity and mortality. These findings highlight the need for prevention of HF hospital admissions and strategies to improve postdischarge outcomes.

The aim of this study was to summarize the published reports about neurological manifestations of Kawasaki disease and underlying mechanisms. PubMed was used as the source for medical database. The prevalence of neurological complications is estimated to range from 1.1 to 3.7% and can include strokes, inflammation of central nervous system, dysfunction of cranial nerves, behavioral disorders as well as syndrome of inappropriate antidiuretic hormone secretion. Their incidence is usually associated with an increased risk for coronary artery aneurysms. In particular, the involvement of facial nerve seems to be a marker of disease severity. Although prognosis is generally remarkable, serious complications may arise in some cases. The early initiation of appropriate therapy prevents the appearance of neurological manifestations in children with Kawasaki disease.

A handheld model derived from medical images represents a natural progression from its 3D visualization. 3D printing technologies are only one component of the highly technically demanding aspects of creating 3D-printed medical models. A number of additive manufacturing technologies are available today and are collectively called as 3D printing or rapid prototyping technologies. This chapter will review the technical aspects of those 3D printing technologies that are widely used in a present-day clinical environment. 3D printing technologies that are now emerging lie between anatomic models printed for surgical planning and functional models that can replicate tissue characteristics. Furthermore, we will discuss techniques used to establish the reproducibility and accuracy of 3D-printed models, thereby establishing their safety and the quality necessary for the implementation of the technology into every-day clinical practice.

Coronary computed tomography angiography (CCTA) provides critical prognostic information on plaque burden and stenosis severity of coronary arteries. We aimed to investigate the long-term prognostic value of coronary artery volume per myocardial mass as a potential new imaging parameter. Consecutive patients with suspected coronary artery disease (CAD) were included. Coronary artery volume index (CAVi) was defined as volume over myocardial mass. Additionally, obstructive CAD (≥ 70% stenosis) and segment severity score (SSS: sum of all segments scored according to lesion severity with 0 = no lesion, 1 = narrowing

Background The feasibility of assessing endothelial shear stress (ESS) in coronary lesions by non-invasive imaging and its potential role in different clinical settings has been recently explored. However, the relationship of ESS with functional indices derived by computed tomography coronary angiography (CTCA) and its value in predicting perfusion changes by quantitative positron emission tomography (PET) downstream stenotic vessels has not been assessed. Purpose To investigate the feasibility of calculating local ESS, its relationship with stenosis severity as well as with virtual functional assessment index (vFAI), and the comparative performance of the two parameters for predicting impaired coronary vasodilating capability in terms both of stress myocardial blood flow (MBF) and myocardial flow reserve (MFR) in patients submitted to CTCA. Methods Thirty-two patients (23 male-9 female, mean age 65.6±7.2 years) with intermediate pre-test likelihood of coronary artery disease (CAD), who were enrolled in the EVINCI and SMARTool projects, and had undergone CTCA with vFAI and PET myocardial perfusion imaging with 15 O-water or 13 N-ammonia were included in the study. PET was considered positive when >1 contiguous segments showed both stress MBF ≤2.3 mL/g/min and MFR ≤2.5 for 15 O-water or ≤1.79 mL/g/min and ≤2.0, for 13 N-ammonia respectively. A vFAI threshold of 0.85 was used as predictor of impaired vasodilating capability. ESS computation was based on a mean aortic pressure of 100 mmHg for the inlet and a mean blood flow at rest of 0.00105 kg/s for the outlet. ESS was calculated (Pa) in the full length of the stenosis and the mean value was obtained. Results Hybrid imaging analysis was performed in CTCA and PET datasets. 51 coronary segments were assessed. There were 27 lesions with stenosis 31–50% and 24 lesions with stenosis 51–70%. ESS was higher in the latter (20.4, IQ: 11.4–32.1 vs. 10.4, IQ: 5.5–15.7, p=0.04). Similarly, ESS was higher in stenoses with impaired vasodilating capacity compared to those without, although this difference did not reach statistical significance (22.8, IQ: 13.2–35.1 vs. 10.6, IQ: 5.7–22.1, p=0.10). The ROC curve analysis for prediction of both abnormal stress MBF and MFR followed the same pattern (AUC=0.668, 95% confidence interval (CI): 0.490–0.810, p=0.11).On the other hand, there was a moderate negative correlation (r=−0.41, p=0.004) between ESS and vFAI and the former was lower in stenoses with vFAI >0.85 compared to those below this threshold (7.35, IQ: 3.2–13.9 vs. 19.1, IQ: 14.1–32.8, p=0.012). vFAI was a good predictor of coronary flow capacity (AUC=0.737, CI: 0.58–0.85, p=0.02). Conclusion Calculation of ESS is feasible in CTCA datasets. ESS was related with stenosis severity and there was a trend to be higher in lesions with impaired coronary vasodilating capability. ESS is modestly related with vFAI and may also be an additional predictor of impaired regional myocardial flow obtained by PET imaging. Acknowledgement/Funding This study has received funding from the EU H2020 research and innovation programme under grant agreement No 689068 and from p-Med GR 5002802

A surface 12-lead electrocardiogram (ECG) is widely available, fast, inexpensive, and safe. However, its value to predict a true myocardial scar in patients with ischemic cardiomyopathy (ICM) has not been studied extensively yet. This study was conducted to assess whether Q waves on resting surface 12-lead ECG are predictive of non-viable myocardium in patients with ICM.We analyzed resting ECGs of 149 patients with ICM undergoing cardiac positron emission tomography (PET) with 13N-ammonia (NHPathological Q waves had a sensitivity and specificity of 70% and 40%, respectively, and a PPV and NPV of 37% and 73%, respectively, to detect myocardial scar on FDG PET. For QS complexes, sensitivity and specificity were 46% and 59%, respectively, and PPV and NPV were 36% and 68%, respectively. Sensitivity was lower, but specificity was significantly higher in both the LCX and RCA compared to the LAD territory (p0.001), particularly for QS complexes.Pathological Q waves on resting 12-lead ECG have poor or at best moderate sensitivity and specificity to detect myocardial scar on FDG PET. These findings support the use of more advanced imaging techniques to assess myocardial viability in ICM.

Aim Amongst patients with coronary artery disease (CAD), women experience relatively worse outcomes as compared to men. Evidence to date has failed to explore unique female imaging targets as major determinants of cardiovascular risk. We sought to assess the prognostic value of epicardial (EFV) and intrathoracic fat volume (IFV) quantification in women and men with suspected and known CAD. Methods and results Intrathoracic fat volume and EFV were calculated from non-contrast CT and analyzed in a propensity-matched cohort of 190 patients (95 women, mean age 62.5 ± 11.3 years) undergoing myocardial perfusion imaging (MPI) and coronary computed tomography angiography (CCTA) for evaluation of CAD. IFV and EFV were significantly lower in women as compared to men (198.2 ± 78.4 vs 293.2 ± 114.7 cm3 and 105.6 ± 48.9 vs 135.8 ± 60.9 cm3, p Conclusion Quantification of IFV provides incremental prognostic value for MACE in women, beyond that provided by traditional risk factors and imaging findings.

OBJECTIVE. We report the development and use of MRI-compatible and MRI-visible 3D printed models in conjunction with advanced visualization software models to plan and simulate safe access routes to achieve a theoretic zone of cryoablation for percutaneous image-guided treatment of a C7 pedicle osteoid osteoma and an L1 lamina osteoblastoma. Both models altered procedural planning and patient care. CONCLUSION. Patient-specific MRI-visible models can be helpful in planning complex percutaneous image-guided cryoablation procedures.

3D printing applications for cardiovascular care range from models for education to planning and simulation of interventions and the generation of implantable devices. This Review summarizes the current cardiovascular 3D printing strategies and applications, including the workflow from image acquisition to the generation of a hand-held model, and highlights the future perspectives of cardiovascular 3D printing. 3D-printed models fabricated from CT, MRI, or echocardiography data provide the advantage of haptic feedback, direct manipulation, and enhanced understanding of cardiovascular anatomy and underlying pathologies. Reported applications of cardiovascular 3D printing span from diagnostic assistance and optimization of management algorithms in complex cardiovascular diseases, to planning and simulating surgical and interventional procedures. The technology has been used in practically the entire range of structural, valvular, and congenital heart diseases, and the added-value of 3D printing is established. Patient-specific implants and custom-made devices can be designed, produced, and tested, thus opening new horizons in personalized patient care and cardiovascular research. Physicians and trainees can better elucidate anatomical abnormalities with the use of 3D-printed models, and communication with patients is markedly improved. Cardiovascular 3D bioprinting and molecular 3D printing, although currently not translated into clinical practice, hold revolutionary potential. 3D printing is expected to have a broad influence in cardiovascular care, and will prove pivotal for the future generation of cardiovascular imagers and care providers. In this Review, we summarize the cardiovascular 3D printing workflow, from image acquisition to the generation of a hand-held model, and discuss the cardiovascular applications and the current status and future perspectives of cardiovascular 3D printing.

Medical 3-dimensional (3D) printing is emerging as a clinically relevant imaging tool in directing preoperative and intraoperative planning in many surgical specialties and will therefore likely lead to interdisciplinary collaboration between engineers, radiologists, and surgeons. Data from standard imaging modalities such as computed tomography, magnetic resonance imaging, echocardiography, and rotational angiography can be used to fabricate life-sized models of human anatomy and pathology, as well as patient-specific implants and surgical guides. Cardiovascular 3D-printed models can improve diagnosis and allow for advanced preoperative planning. The majority of applications reported involve congenital heart diseases and valvular and great vessels pathologies. Printed models are suitable for planning both surgical and minimally invasive procedures. Added value has been reported toward improving outcomes, minimizing perioperative risk, and developing new procedures such as transcatheter mitral valve replacements. Similarly, thoracic surgeons are using 3D printing to assess invasion of vital structures by tumors and to assist in diagnosis and treatment of upper and lower airway diseases. Anatomic models enable surgeons to assimilate information more quickly than image review, choose the optimal surgical approach, and achieve surgery in a shorter time. Patient-specific 3D-printed implants are beginning to appear and may have significant impact on cosmetic and life-saving procedures in the future. In summary, cardiothoracic 3D printing is rapidly evolving and may be a potential game-changer for surgeons. The imager who is equipped with the tools to apply this new imaging science to cardiothoracic care is thus ideally positioned to innovate in this new emerging imaging modality.

Aims The association of low endothelial shear stress (ESS) with high-risk plaque (HRP) has not been thoroughly investigated in humans. We investigated the local ESS and lumen remodelling patterns in HRPs using optical coherence tomography (OCT), developed the shear stress score , and explored its association with the prevalence of HRPs and clinical outcomes. Methods and results A total of 35 coronary arteries from 30 patients with stable angina or acute coronary syndrome (ACS) were reconstructed with three dimensional (3D) OCT. ESS was calculated using computational fluid dynamics and classified into low, moderate, and high in 3-mm-long subsegments. In each subsegment, (i) fibroatheromas (FAs) were classified into HRPs and non-HRPs based on fibrous cap (FC) thickness and lipid pool size, and (ii) lumen remodelling was classified into constrictive, compensatory, and expansive. In each artery the shear stress score was calculated as metric of the extent and severity of low ESS. FAs in low ESS subsegments had thinner FC compared with high ESS (89 ± 84 vs.138 ± 83 µm, P < 0.05). Low ESS subsegments predominantly co-localized with HRPs vs. non-HRPs (29 vs. 9%, P < 0.05) and high ESS subsegments predominantly with non-HRPs (9 vs. 24%, P < 0.05). Compensatory and expansive lumen remodelling were the predominant responses within subsegments with low ESS and HRPs. In non-stenotic FAs, low ESS was associated with HRPs vs. non-HRPs (29 vs. 3%, P < 0.05). Arteries with increased shear stress score had increased frequency of HRPs and were associated with ACS vs. stable angina. Conclusion Local low ESS and expansive lumen remodelling are associated with HRP. Arteries with increased shear stress score have increased frequency of HRPs and propensity to present with ACS.

Refractory angina refers to a group of patients with stable coronary atherosclerotic disease and angina symptoms, unresponsive to traditional medical management, while considered to be suboptimal candidates for revascularization procedures. Up to 15% of angina patients are considered to have refractory angina and, taking into account the aging population and the improvements in the treatment of stable coronary artery disease, the incidence of this entity is expected to increase. This review describes traditional and novel pharmacotherapies for symptoms relief and for long-term management of refractory angina. Mechanisms of action and relevant clinical trials are discussed and current recommendations from major European and US cardiovascular societies are reported.

BackgroundIf undetected, infrarenal Abdominal Aortic Aneurysm (AAA) growth can lead to rupture, a high-mortality complication. Some AAA patients exhibit inhomogeneous luminal contrast attenuation at first-pass CT angiography (CTA). This study assesses the association between this observation and aneurysm growth.MethodsSixty-seven consecutive pre-repair AAA CTAs were included in this retrospective study. The "Gravitational Gradient" (GG), defined as the ratio of the mean attenuation in a region-of-interest placed posteriorly to that in a region-of-interest placed anteriorly within the lumen of the aortic aneurysm on a single axial slice, and the maximum aneurysm diameter were measured from each CT data set. "AAA Contrast Inhomogeneity" was defined as the absolute value of the difference between the GG and 1.0. Univariate and multivariate logistic regression was used to assess the association of aneurysm growth >0.4 and>1.0cm/year to AAA Contrast Inhomogeneity, aneurysm diameter, patient characteristics and cardiovascular co-morbidities.ResultsAAA Contrast Inhomogeneity was not correlated to aneurysm diameter (p=0.325). In multivariable analysis that included initial aneurysm diameter and AAA Contrast Inhomogeneity, both factors were significantly associated with rapid aneurysm growth (initial diameter: p=0.029 and 0.011, and, AAA Contrast Inhomogeneity: p=0.045 and 0.048 for growth >0.4cm/year and >1cm/year respectively).ConclusionsAAA Contrast Inhomogeneity is a common observation in first-pass CTA. It is associated with rapid aneurysm growth, independent of aneurysm diameter.

We report the case of an infant who presented with respiratory distress at the Emergency Department. A chest radiograph showed interposition of colon loops between the right hemidiaphragm and liver, while abdominal and thoracic ultrasound examinations were normal. The aforementioned radiological finding was considered to be Chilaiditi’s sign. This sign usually presents as an incidental radiological finding and may be mistaken for pneumoperitoneum or diaphragmatic hernia. Clinicians’ familiarity with rare radiological signs is necessary.

Objectives Medical three-dimensional (3D) printing, the fabrication of handheld models from medical images, has the potential to become an integral part of otolaryngology-head and neck surgery (Oto-HNS) with broad impact across its subspecialties. We review the basic principles of this technology and provide a comprehensive summary of reported clinical applications in the field. Methods Standard bibliographic databases (MEDLINE, Embase, Cumulative Index to Nursing and Allied Health Literature, Web of Science, and The Cochrane Central Registry for Randomized Trials) were searched from their inception to May 2018 for the terms: "3D printing," "three-dimensional printing," "rapid prototyping," "additive manufacturing," "computer-aided design," "bioprinting," and "biofabrication" in various combinations with the terms: "ptolaryngology," "head and neck surgery," and "otology." Additional articles were identified from the references of retrieved articles. Only studies describing clinical applications of 3D printing were included. Results Of 5,532 records identified through database searching, 87 articles were included for qualitative synthesis. Widespread implementation of 3D printing in Oto-HNS is still at its infancy. Nonetheless, it is increasingly being utilized across all subspecialties from preoperative planning to design and fabrication of patient-specific implants and surgical guides. An emerging application considered highly valuable is its use as a teaching tool for medical education and surgical training. Conclusions As technology and training standards evolve and as healthcare moves toward personalized medicine, 3D printing is emerging as a key technology in patient care in Oto-HNS. Treating physicians and surgeons who wish to stay abreast of these developments will benefit from a fundamental understanding of the principles and applications of this technology. Laryngoscope, 129:2045-2052, 2019.

Microduplication 22q11.2 syndrome has been recently characterized as a new chromosomal syndrome having overlapping features with the classic DiGeorge syndrome Phenotypic variability in microduplication 22q11.2 syndrome ranges from normal or near normal phenotype to multiple congenital defects, including heart defects, velopharyngeal insufficiency with or without cleft palate, or hyper nasal speech, urogenital abnormalities and autoimmunity

Positron emission tomography (PET) integrating assessment of perfusion withIn 180 consecutive patients with ischemic cardiomyopathy, myocardium was categorized on a segment-level into normal, ischemic, hibernating, and scar. From dynamic images, stress MBF, rest MBF, and k2 were derived and myocardial flow reserve (MFR) and volume of distribution (VD) were calculated.Across myocardial tissues, all parameters differed significantly. The area under the curve (AUC) was 0.564 (95% CI 0.527-0.601), 0.635 (0.599-0.671), 0.553 (0.516-0.591), 0.520 (0.482-0.559), and 0.560 (0.522-0.597) for stress MBF, rest MBF, MFR, k2, and VD. The generalized linear mixed model correctly classified 81% of scar as viable, hibernating myocardium. If the threshold of rest MBF to predict viability was set to 0.45 mL·minQuantitative NH3 PET parameters have low to moderate diagnostic performance to predict viability in ischemic cardiomyopathy. However, if rest MBF falls below 0.45 mL·min

Aims To determine if tube-adapted thresholds for coronary artery calcium (CAC) scoring by computed tomography at 80 kilovolt-peak (kVp) tube voltage and 70-kVp yield comparable results to the standard 120-kVp protocol. Methods and results We prospectively included 103 patients who underwent standard scanning with 120-kVp tube voltage and additional scans with 80 kVp and 70 kVp. Mean body mass index (BMI) was 27.9 ± 5.1 kg/m2. For the lowered tube voltages, we applied novel kVp-adapted thresholds for calculation of CAC scores and compared them with standard 120-kVp scans using intraclass correlation and Bland-Altman (BA) analysis. Furthermore, risk-class (CAC score 0/1-10/11-100/101-400/>400) changes were assessed. Median CAC score from 120-kVp scans was 212 (interquartile range 25-901). Thirteen (12.6%) patients had zero CAC. Using the novel kVp-adapted thresholds, CAC scores derived from 80-kVp scans showed excellent correlation (r = 0.994, P

Enhanced sympathetic nervous system activity is associated with increased mortality in many cardiac conditions including heart failure and coronary artery disease (CAD). To ensure adequate image quality of coronary CT angiography (CCTA), pre-scan β-adrenergic blockers (BB) are routinely administered. It is currently unknown whether sensitivity to sympathicolytic compounds is associated with severity of CAD. A total of 2633 consecutive patients (1733 [65.8%] men and 900 [34.2%] women, mean age 56.7 ± 11.5 years) undergoing CCTA for exclusion of significant CAD at our department between 06/2013 and 12/2016 were evaluated. Acute heart rate (HR) responses to BB administration were recorded in all patients. Coronary plaque burden as indicated by segment severity score (SSS), segment involvement score (SIS), and significant CAD (i.e. > 50% luminal narrowing) was higher in weak responders to BB as compared to strong responders to BB (p = 0.001 for SSS and SIS, and p = 0.021 for significant CAD). Accordingly, in a multiple linear regression model adjusted for known risk factors of CAD such as smoking, hypertension, diabetes and dyslipidaemia, as well as age, sex, body mass index (BMI), glomerular filtration rate, and HR during CCTA scan, a strong response to BB was selected as a significant independent negative predictor of coronary plaque burden (beta coefficient - 0.08, p = 0.001). We demonstrate that individuals with a weak acute response to BB administration encounter an increased risk of severe CAD. Taking into account sensitivity to sympatho-inhibition may add complementary information in patients undergoing CCTA for evaluation of CAD.

Introduction: Polypharmacy is increasing among hospitalized children and is considered as a major risk factor for potential drug-drug interactions. Aims and Objectives: Our aim is to investigate prevalence and risk factors of polypharmacy among children hospitalized due to respiratory problems. Methods: We reviewed medical records of 163 children (median age 17 months) hospitalized in a university pediatric clinic of a tertiary hospital during the years 2017-2018. Results: Diagnoses included pneumonia (40 children), bronchiolitis (71 children), asthma (12 children), non-specific lower respiratory infections (23 children), laryngotracheobronchitis (10 children) and upper respiratory infections (7 children). Children were exposed to 3.8±1.4 distinct drugs each hospital day and to 5.1±1.96 drugs cumulatively during their hospitalization. The most frequently used drugs were nebulized salbutamol (85.2%), macrolides (78.5%), nebulized ipratropium (61.9%), penicillins (61.9%), antipyretics (49.6%). Cumulative drug exposure presented a positive correlation with length of hospital stay (p=0.003) and tended to be positively correlated with the presence of fever (p=0.08). Polypharmacy was defined as the 90th percentile of the cumulative number of distinct drugs during hospitalization (=7 drugs). Polypharmacy was observed in 34 children (20.8%) and was significantly associated with the length of hospital stay. Age, abnormal chest x-ray, leukocytosis or specific diagnoses were not significant predictors of polypharmacy. Conclusions: Drug exposure increases with long hospital stay and fever. The effect of other factors (e.g. physicians9 experience or skills) on polypharmacy needs investigation.

We describe the case of a 5-year-old girl with severe congenital neutropenia presenting with recurrent skin and respiratory infections. Sequence analysis of ELANE and HAX1 genes identified a mutation in heterozygous state in exon 2 of the ELANE gene: c.157C > G (p.His53Asp), not previously described in the literature at the exon coding level. Given the autosomal dominant inheritance and the location of the mutation within a “hotspot,” this mutation was considered as clinically relevant. ELANE should be screened in patients with congenital neutropenia of no obvious etiology. A detailed medical history and clinical evaluation can prevent unnecessary investigations allowing for a targeted diagnostic strategy.

Evidence to date has failed to adequately explore determinants of cardiovascular risk in women with coronary microvascular dysfunction (CMVD). Heart rate responses to adenosine mirror autonomic activity and may carry important prognostic information for the diagnosis of CMVD. Hemodynamic changes during adenosine stress were analyzed in a propensity-matched cohort of 404 patients (202 women, mean age 65.9 ± 11.0) who underwent clinically indicated myocardial perfusion 13N-ammonia Positron-Emission-Tomography (PET) at our institution between September 2013 and May 2017. Baseline heart rate (HR) was significantly higher in patients with abnormal coronary flow reserve (CFR, p

BACKGROUND:Sexual dimorphism in cardiac sympathetic outflow has recently gained attention in the context of Takotsubo cardiomyopathy. Previous studies suggest that there are sex- and age-dependent differences in peripheral autonomic control, however, data on cardiac-specific sympathetic activation in aged women and men are lacking. METHODS AND RESULTS:Regional quantitative analysis of cardiac fluorine-18 (18F)- Dihydroxyphenylalanine (DOPA) uptake was retrospectively performed in 133 patients (69 females, mean age 52.4±17.7 years) referred for assessment of neuroendocrine tumours (NET) by Positron-Emission-Tomography. Cardiac 18F-DOPA uptake was significantly higher in women as compared to men (1.33±0.21 vs. 1.18±0.24, p55 years of age (1.39±0.25 in women vs. 1.09±0.24 in men, p

It is unclear whether non-invasive screening of asymptomatic diabetic patients for coronary artery disease (CAD) may improve cardiac outcomes. Thus, we performed a systematic literature review and meta-analysis of randomised controlled trials (RCT's) on this topic. We searched appropriate RCT's in five online databases (PubMed/MEDLINE, Cochrane Library, Embase, Scopus, and Web of Science) from January 2000 to November 2017 and in 41 recent reviews. Two investigators independently extracted and assessed study data using standardised forms. Additional unpublished data were obtained from trial authors. The primary endpoint 'any cardiac event' was a composite of cardiac death, non-fatal myocardial infarction (MI), unstable angina (UA), or heart failure (HF) hospitalisation. We performed a meta-analysis of relative risks (RRs) with 95% confidence intervals (CI) using the Mantel-Haenszel method. We included five RCT's with 3299 patients, of which 189 (5.7%) experienced any cardiac event on follow-up (weighted mean 4.1 years). Non-invasive CAD screening significantly reduced any cardiac event by 27% [RR 0.73 (95% CI 0.55-0.97), P = 0.028, number needed to screen 56]. This result was driven by important, albeit non-significant decreases in non-fatal MI [RR 0.65 (95% CI 0.41-1.02), P = 0.062] and HF hospitalisation [RR 0.61 (95% CI 0.33-1.10), P = 0.100]. Non-invasive CAD screening did not significantly affect cardiac death [RR 0.92 (95% CI 0.53-1.60), P = 0.77] and UA [RR 0.73 (95% CI 0.41-1.31), P = 0.29]. Compared with the standard care, non-invasive CAD screening reduced cardiac events by 27% in asymptomatic diabetic patients, largely through reductions in non-fatal MIs, and HF hospitalisations. The present results justify larger, appropriately powered trials to potentially revisit current recommendations.

Coronary computed tomography angiography (CCTA) holds a prime role in noninvasive assessment of atherosclerosis by enabling plaque characterization and serial imaging. The napkin-ring sign has been described in plaques that are characterized at CCTA by a low attenuation center, covered by a higher attenuation ring-like periphery. Such atherosclerotic plaques are closely linked with future acute coronary syndromes, independent of other high-risk CCTA plaque features, such as low attenuation plaque and positive remodeling.1 Medical treatment, primarily with the use of statins, that promote plaque stabilization, has been associated with resolution of inflammation and favorable histological changes in coronary plaques, leading to plaque regression.2 We present a 62-year-old male patient with history of hypertension and dyslipidemia who was assessed with CCTA because of atypical chest pain (Figure 1). A high-risk coronary plaque was identified at the mid left anterior descending artery (LAD) resulting in moderate luminal stenosis (≈50%; Figure 1, left). The upstream/proximal (Figure 1i, …

Acute right ventricular myocardial infarction (RVMI) is observed in 30-50% of patients presenting with inferior wall myocardial infarction (MI) and, occasionally, with anterior wall MI. The clinical consequences vary from no hemodynamic compromise to severe hypotension and cardiogenic shock depending on the extent of RV ischemia. Areas covered: The pathophysiological mechanisms, diagnostic steps, and novel therapeutic approaches of acute RVMI are described. Expert commentary: Diagnosis of acute RVMI is based on physical examination, cardiac biomarkers, electrocardiography, and coronary angiography, whereas noninvasive imaging modalities (echocardiography, cardiac magnetic resonance imaging) play a complementary role. Early revascularization, percutaneous or pharmacological, represents key step in the management of RMVI. Maintenance of reasonable heart rate and atrioventricular synchrony is essential to sustain adequate cardiac output in these patients. When conventional treatment is not successful, mechanical circulatory support, including right ventricle assist devices, percutaneous cardiopulmonary support, and intra-aortic balloon pump, might be considered. The prognosis associated with RVMI is worse in the short term, compared to non-RVMI, but those patients who survive hospitalization have a relatively good long-term prognosis.

Summary Background Healthcare-associated infections (HCAIs) are associated with increased morbidity and mortality and with excess costs. Central line-associated bloodstream infections (CLABSIs) are the most common HCAIs in neonates and children. Aim To establish national benchmark data for rates of CLABSI in neonatal and paediatric intensive care units (NICUs and PICUs) and paediatric oncology units (ONCs). Methods Active surveillance for CLABSI was conducted from June 2016 to February 2017. A collaborative of 14 NICUs, four PICUs, and six ONCs participated in the programme. Surveillance definitions of central line (CL), central line utilization (CLU) ratio, CLABSI event, and CLABSI rate were based on the Centers for Disease Control and Prevention's 2014 National Healthcare Safety Network criteria. Medical records were assessed daily for calculating CL-days, patient-days, and susceptibility of isolated organisms. Findings A total of 111 CLABSI episodes were recorded. The overall mean CLABSI rate was 4.41 infections per 1000 CL-days, and the CLU ratio was 0.31. CLABSI rates were 6.02 in NICUs, 6.09 in PICUs, and 2.78 per 1000 CL-days in ONCs. A total of 123 pathogens were isolated. The most common pathogens were Enterobacteriaceae (36%), followed by Gram-positive cocci (29%), non-fermenting Gram-negative bacteria (16%), and fungi (16%). Overall, 37% of Gram-negative pathogens were resistant to third-generation cephalosporins and 37% to carbapenems. Conclusion Nationally representative CLABSI rates were determined for paediatric patients. These data could be used to benchmark and serve as baseline data for the design and evaluation of infection control and antimicrobial stewardship interventions.

OBJECTIVE: Results of the use of the transluminal attenuation gradient (TAG) at coronary CT angiography (CCTA) to predict hemodynamically significant disease vary widely. This study tested whether diagnostic performance of TAG to predict fractional flow reserve (FFR) ≤ 0.8 is improved when applied separately to subsets of coronary arteries that carry similar physiological flow. METHODS: 28 patients with 64 × 0.5 mm CCTA and invasive FFR in ≥1 major coronary artery were retrospectively evaluated. Two readers assessed TAG in each artery. The receiver operating characteristic (ROC) area under the curve (AUC) was used to assess the diagnostic performance of TAG to detect hemodynamically significant disease following a clinical use rule [negative: FFR > 0.8 or ≤ 25% diameter stenosis (DS) at invasive catheter angiography; positive: FFR ≤ 0.8 or ≥ 90% DS at invasive catheter angiography]. ROC AUC was compared for all arteries pooled together, vs separately for arteries carrying similar physiological flow (Group 1: all left anterior descending plus right-dominant left circumflex; Group 2: right-dominant RCA plus left/co-dominant left circumflex). RESULTS: Of the 84 arteries, 30 had FFR measurements, 30 had ≤25% DS and 13 had ≥90% DS. 11 arteries with 26–89% DS and no FFR measurement were excluded. TAG interobserver reproducibility was excellent (Pearson r = 0.954, Bland–Altman bias: 0.224 Hounsfield unit cm(–1)). ROC AUC to detect hemodynamically significant disease was higher when considering arteries separately (Group 1 AUC = 0.841, p = 0.039; Group 2 AUC = 0.840, p = 0.188), than when pooling all arteries together (AUC = 0.661). CONCLUSION: Incorporating information on the physiology of coronary flow via the particular vessel interrogated and coronary dominance may improve the accuracy of TAG, a simple measurement that can be quickly performed at the time of CCTA interpretation to detect hemodynamically significant stenosis in individual coronary arteries. ADVANCES IN KNOWLEDGE: The interpretation of TAG may benefit by incorporating information regarding which coronary artery is being interrogated.

A reduction in left ventricular apical tracer uptake (apical thinning) is frequently observed in myocardial perfusion imaging (MPI), yet its cause remains a matter of debate, particularly in perfusion emission tomography (PET). This analysis sought to determine whether apical thinning in PET-MPI is attributable to true anatomical thinning of the left ventricular apical myocardium.We retrospectively analyzed 57 patients without any history or signs of apical myocardial infarction who underwent rest PET-MPI with 13N-ammonia and contrast-enhanced cardiac computed tomography (CT). Semi-quantitative normalized percent apical 13N-ammonia uptake at rest, myocardial blood flow (MBF), and k2 wash-out rate constants were compared to apical myocardial wall thickness measurements derived from CT and base-to-apex gradients were calculated. Apical thinning was found in 93% of patients and in 74% when analysis of normalized apical tracer uptake was confined to end-systole. No significant correlation was found between apical myocardial thickness and apical tracer uptake (r = - 0.080, P = .553), MBF (r = - 0.211, P = .115), or k2 wash-out rate (r = - 0.023, P = .872), nor between apical myocardial thickness and any gradients. A statistically significant but small difference in apical myocardial thickness was observed in patients with moderately to severely reduced apical tracer uptake vs patients with normal to mildly reduced uptake (4.3 ± 0.7 mm vs 4.7 ± 0.7 mm; P = .043).Apical thinning is a highly prevalent finding during 13N-ammonia PET-MPI that is not solely attributable to true anatomical apical wall thickness or the partial volume effect. Other factors that yet need to be identified seem to have a more prominent impact.