The role of advanced imaging in the diagnosis and management of scimitar syndrome in pediatric patients.

Scimitar syndrome with peripheral left pulmonary artery branch stenoses

* ALL: : acute lymphoblastic leukemia CT: : computed tomography IVC: : inferior vena cava NA: : neonatal appendicitis NEC: : necrotizing enterocolitis WBC: : white blood cell A 7-day-old boy born at term is brought to the emergency department by his foster mother for refusal to feed for ∼20 hours and increased crying, although he is intermittently consolable. When given his bottle, the infant starts to suck but then cries as though in pain. He has not vomited, and his last bowel movement was yesterday. The stool has been moderately well formed without blood or mucus, and wet diapers have continued. There is no history of respiratory symptoms, lethargy, rashes, or jaundice. The biological mother is a 27-year-old woman with a known history of previous drug abuse; she had received some prenatal care. This infant was born by scheduled cesarean delivery. Initial physical examination reveals a crying but consolable neonate. His temperature is 37.7°C, heart rate is 170 beats/minute, and respiratory rate is 56 breaths/minute; the remainder of his findings are normal. His complete blood count (white blood cell [WBC] 11.7 × 109/L, 78.5% neutrophils, 11.4% monocytes, 9.8% lymphocytes), serum concentrations of electrolytes and glucose, urinalysis, and cerebrospinal fluid studies are normal. Cultures are obtained. An abdominal radiograph is normal and abdominal ultrasonography reveals trace pelvic fluid. He is given a bolus of normal saline and is started on antibiotics to treat possible sepsis. A few hours after admission, repeat examination is concerning for inconsolability, poor suck reflex, and lethargy. His abdomen is distended, firm, and tender to palpation. Bowel sounds are normal. Further studies are performed that reveal the diagnosis. A previously healthy 10-month-old boy is seen for a 2-week history of an erythematous left eye with tearing. Earlier, he was diagnosed as having conjunctivitis at an urgent care clinic and was treated with a 10-day course of polymyxin B/trimethoprim eye drops without improvement. He has felt warm intermittently but has not had a …

Major advances in the field of pediatric cardiology and cardiac surgery over the last several decades have led to a dramatic improvement in survival rates for most forms of congenital heart disease (CHD). For example, hypoplastic left heart syndrome, a previously lethal defect, now has early survival rates up to 90% at major centers.1 These improved outcomes have produced a growing population of survivors with complex CHD who are now reaching adulthood (Figure 1). During this period, improvements in surgical and medical treatments have been accompanied by developments in diagnostic modalities. Echocardiography has replaced catheterization as the primary diagnostic modality, and it is now uncommon for newborn infants to undergo catheterization for purely diagnostic purposes. Although echocardiography remains the bedrock of noninvasive cardiac imaging, the array of diagnostic modalities and techniques available continue to grow and this has spawned the specialty of “noninvasive cardiac imaging” and the need for the “cardiac imager” to be adept in all the different modalities. Figure 1. Percentage of patients under the age of 1 year (grey bars) and over the age of 18 years (black bars) undergoing echocardiography at Children’s Hospital Boston from 1983 through 2006. Note the reverse trends of these age groups reflecting the steady increase in the proportion of adult patients with congenital heart disease. Although the absolute number of infants undergoing echocardiography during this time period has increased, their proportion has steadily declined. Echocardiography, cardiac magnetic resonance (CMR), and cardiac computed tomography (CCT) are the primary modalities used for noninvasive cardiac imaging in patients with CHD. Nuclear scintigraphy is used in selected circumstances. The Table summarizes the strengths and weaknesses of each modality. Figure 2 shows temporal trends in utilization for the various noninvasive cardiac imaging techniques at our center. It is clear that echocardiography is the most frequently …

The patient is a 41-year-old woman who was referred to our hospital because of an abnormal chest radiograph. On admission, she was asymptomatic, but her cardiac silhouette was shifted to the right on chest radiograph. From contrast-enhanced chest computed tomography (CT), we suspected that the right upper pulmonary vein connected to the inferior vena …

Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery, Scimitar Syndrome, and Aortic Coarctation

A New Surgical Approach to Scimitar Syndrome

Partial Anomalous Pulmonary Venous Return Detected Incidentally during Port Placement

A 25-year-old man with no significant medical history was found to have a continuous murmur along the left sternal border on routine physical examination performed in preparation for running a marathon. Echocardiography showed a dilated left ventricle with normal function and continuous high-velocity flow in an aberrant vessel in close vicinity to the abdominal aorta (Movie I in the online-only Data Supplement). Chest x-ray (posterior-anterior and lateral projections, Figure 1) disclosed conglomeration of tubular densities in a scimitar pattern in the right lower lobe suggestive of anomalous pulmonary venous return of right lower lobe (scimitar syndrome; anomalous drainage of right lung pulmonary veins into the inferior vena cava). Cardiac magnetic resonance imaging (MRI) not only demonstrated normal drainage of all 4 pulmonary veins into the left atrium but also revealed an aberrant vessel (Movie II in the online-only Data Supplement) extending between the abdominal aorta and the right inferior pulmonary vein with an elevated systemic-to-pulmonary flow ratio of 1.8, consistent with a large left-to-left shunt. Cardiac computed tomography (CT) confirmed the presence of a large fistulous connection between the abdominal aorta and the right inferior …

PERIOPERATIVE myocardial complications after noncardiac surgery affect more than 1 million operations each year and are leading causes of morbidity and mortality, especially among patients undergoing vascular surgery. Myocardial complications such as perioperative myocardial infarction (MI) are common and are the most likely cause for perioperative death in all surgical populations.1In general surgery, the risk for perioperative MI is 0.8% in men older than 50 yr2and varies with the cardiovascular status, comorbidities, and the extent of the procedure, reaching more than 20% among patients undergoing vascular surgery.3As patients become older and sicker and procedures become more aggressive and extensive, physicians must find novel approaches to evaluate and prepare cardiac patients for noncardiac procedures and reduce perioperative myocardial events.The American Heart Association–American College of Cardiology guidelines for cardiac risk evaluation use the patient's history, physical examination, and functional capacity, and taking into account the expected surgery, one may recommend further assessment with noninvasive testing or coronary angiography.4The purpose of this review is to provide an overview of available imaging tools that could potentially be used for perioperative evaluation of cardiac patients before noncardiac surgery, in accord with recent guidelines and with focus on the recent progress made with cardiac computed tomography (CT).Traditionally, preoperative evaluation has relied on the patient's history, physical examination, and functional capacity. After preoperative evaluation, the physician should determine the clinical predictors as major, intermediate, or minor4and evaluate the functional capacity of the patient. New York Heart Association class II heart failure, which equals 4 metabolic equivalents,4has also been found to be an important predictor of perioperative cardiac complications after major noncardiac surgery. The cutoff value of 4 or more metabolic equivalents determines an adequate cardiac functional capacity and reserve and predicts perioperative cardiac events in patients treated with high-risk noncardiac surgery.5Finally, the extent and risk associated with the procedure should be categorized as major, intermediate, or minor. The cardiac risk is the combined incidence of cardiac death and nonfatal MI and is greater than 5% for high-risk procedures, whereas intermediate-risk procedures have less than 5% cardiac risk (1–5%), and low-risk procedures have less than 1% cardiac risk.4Indeed, based on these criteria, the flowchart of Eagle et al. 4suggests when a noninvasive test or coronary angiography may be appropriate to evaluate cardiac function, reserve, or ability to withstand surgical stress. Pertinent to the utility of evaluating cardiac structure and function, new imaging techniques for evaluation of the heart have emerged in the past couple of decades. These techniques may provide more comprehensive information regarding the structure and function of the heart and coronary arteries, and evaluate adequately patients who have mechanical restrictions to perform exercise-induced stress. Such developments in cardiac imaging may eventually provide an opportunity to revise and refine the steps involving the evaluation of cardiac patients.6,7This approach should be evaluated cautiously and should take into consideration recent recommendations and guidelines. For example, the current American Heart Association–American College of Cardiology guidelines recommend the use of perioperative β-blocker therapy as an alternative approach to decrease cardiovascular risk.8β-Blockers initiated at least 1 week before major vascular surgery and continued for 30 days postoperatively reduced significantly the perioperative incidence of nonfatal MI and death from cardiac causes in high-risk patients.9In addition to beta blockers, statins may reduce perioperative mortality in patients undergoing major vascular surgery and may have an additive effect to β-blockers.10,11The strategy of preoperative coronary artery revascularization before elective major vascular surgery to reduce perioperative cardiac morbidity and mortality was investigated in the Coronary Artery Revascularization Prophylaxis trial, a multicenter study involving patients with vascular disease and significant coronary artery disease (CAD), but no unstable angina. Cardiac revascularization did not result in increased survival, either perioperatively or in long-term follow-up, in patients who needed elective vascular surgery.12However, although the Coronary Artery Revascularization Prophylaxis study is a cornerstone trial in the perioperative care of cardiac patients for noncardiac surgery, it had some limitations. In particular, only 9% of the patients scheduled to undergo vascular operations were eligible for the study. The main reasons for exclusion were insufficient cardiac risk, an urgent vascular surgery, previous revascularization without ischemia, severe coexisting illnesses, left main coronary artery stenosis of at least 50%, left ventricular ejection fraction less than 20%, and severe aortic stenosis. The outcome of these patients has not been adequately evaluated.Indeed, taking into consideration the patient's perioperative cardiac risk according to clinical predictors, functional capacity, and the extent of the future surgery, the anesthesiologist must decide whether further cardiac assessment or perioperative medical management are indicated. Patients whose functional capacity is difficult to establish, who underwent previous coronary revascularization, who have unstable or changed cardiac status, or who have severe comorbidities may need further evaluation. Several imaging techniques are available for evaluation of cardiac patients, including cardiac CT, coronary angiography, stress echocardiography, cardiac magnetic resonance imaging (CMRI), and myocardial nuclear studies.Computed tomography is widely available in large medical centers and is routinely used in clinical practice. The basic principle of CT is that a fan-shaped, thin x-ray beam passes through the body at many angles to allow for cross-sectional images. After collimation of the beam (i.e. , achieving a definitive slice thickness using a collimator) to reduce scatter, the photons are recorded on a corresponding detector array, and the transmission data are digitized. A “filtered back projection” reconstruction algorithm, which takes into account the attenuation of the x-ray beam along its path, allows for reconstruction of the grayscale values of each picture element (pixel) with reference to the value for water and air, to depict cross-sectional images. Reconstruction algorithms and multirow detectors applied in current scanners enable three-dimensional volumetric imaging and multiple high-quality reconstructions of various volumes of interest.13Current clinical scanners used for cardiovascular imaging employ either a rotating x-ray source with a circular, stationary detector array (e.g. , helical CT) or electromagnetic deflection of an electron beam to replace mechanical motion (electron beam computed tomography [EBCT]).14Multidetector computed tomography (MDCT) is a helical CT with a large array of detectors, which allow it to acquire a large number of slices simultaneously (4–256) and greatly increase its resolution. However, to obtain quantitative measurements of tissue opacity within a specific cardiac phase (e.g. , for measurement of perfusion), the scanning time should generally be less than 100 ms.15Sufficiently high temporal resolution (the time required to acquire the data for one image) is currently offered only by the EBCT (50 ms/image) and the novel Dual-Source CT (SOMATOM® Defi-nition; Siemens Medical Systems, Forchheim, Germany) (83 ms/image), a recently released model of MDCT that has two x-ray tubes (rather than one) positioned at 90° to each other, thereby doubling temporal resolution.16However, this technique remains to be validated for such measurements.Electron beam computed tomography was developed in the 1980s and hailed as an ultimate cardiac scanner. It allows almost simultaneous data acquisition from up to eight parallel slices (7–8 mm thick) by rapid sweeping of an electron beam along target rings in as little as 50 ms per scan. Because it does not involve moving parts (and therefore decreases the need for cooling), the speed of acquisition (temporal resolution) with EBCT is faster than with MDCT (table 1) and usually does not require slowing the heart rate pharmacologically. The high speed of EBCT is offset by moderate image quality and relatively restricted power for acquisition of a large number of images, which decreased its popularity for comprehensive cardiac studies. Consequently, its availability is limited, resulting in declining use of this technology.Multidetector computed tomography is a relatively ubiquitous and newer scanner that has temporal resolution of 330–400 ms/image, which enables many studies of cardiac anatomy and global function (e.g. , ejection fraction and cardiac output). Spatial resolution can be achieved with 64-slice scanners using isotropic voxels (consistent three-dimensional image quality in any reconstruction plane) of 0.4 × 0.4 × 0.4 mm. The technique involves continuous rotation of the x-ray tube and detectors and simultaneous translation of the patient through the gantry opening, and can acquire multiple simultaneous sections of variable widths using prospective electrocardiographic triggering.17Alternatively, retrospective electrocardiographic gating enables reconstruction of images at any desired time in the cardiac cycle. Data can be used from multiple slices to reconstruct other imaging planes. The images are of best quality when the resting heart rate (HR) is less than 70 beats/min. At faster heart rates, motion artifacts may become more prominent, and therefore HR may need to be pharmacologically decreased before scanning. The temporal resolution of MDCT determines the overall scan time. As gantry rotation speeds increase, the minimum slice thickness decreases, with submillimeter sections throughout the heart acquired during a single breath hold. The overall CT scan time is approximately 12 s, and the mean total time for the examination is less than 13 min with 64-slice technology.The improvement in MDCT technology enabled the assessment of significant luminal stenosis and identification of nonstenotic atherosclerotic plaques. Virtual noninvasive angiography, with three-dimensional reconstruction of coronary anatomy from cardiac CT images, can provide information about coronary luminal obstruction, calcium scoring, and composition of the plaque.18Furthermore, it has the added benefit of offering fine details of the examined vessels (fig. 1).Excellent sensitivity and specificity were found for evaluation of proximal, middle and distal left anterior descending, first diagonal, proximal and distal circumflex, obtuse marginal and proximal mid and distal right coronary artery.19A meta-analysis of diagnostic performance of MDCT compared with invasive coronary angiography showed a sensitivity of 85% and a specificity of 95% for identification of CAD. On average, 87% of segments had diagnostic image quality, with a significant increase from 78% with 4-slice systems to 96% with the more recent 16-slice systems.6Multidetector computed tomography could provide a clinically useful tool in the workup of symptomatic patients before angiography.20A patient with chronic chest pain indicative of CAD could undergo CT angiography, and if low calcium score and no circumferential calcifications (or other test results indicative of ischemia) are found, invasive angiography may not be indicated, because MDCT has excellent negative predictive value to rule out CAD. By this approach, patients with primarily microvessel disease (which may cause angina and abnormal stress test results) may be identified and not required to go through unnecessary fluoroscopic angiography, and aggressive medical therapy would be indicated before surgery.20MDCT also has high diagnostic accuracy in detecting bypass graft stenosis and occlusions in symptomatic patients after coronary artery bypass grafting, which might potentially reduce dramatically the number of unnecessary invasive angiographies performed in these patients.21However, although the MDCT technique has been extensively evaluated for its accuracy for detection of CAD compared with standard coronary angiography,6,19,20,22it has not been applied for the routine evaluation of cardiac patients for noncardiac surgery and, to date, is not a recommended technique for perioperative risk stratification.The main technical limitations of cardiac CT for evaluation of the coronary arteries include the difficulty in handling cardiac motion, arrhythmia, severe calcifications, vessel size less than 1.5 mm, breathing, the presence of stents, and poor enhancement.22Lesions with extensive calcified components and implanted coronary stents compromise the accuracy of MDCT coronary angiography by causing artifacts.23Stent type and diameter influence evaluability of in-stent restenosis by MDCT, but in evaluable stents, sensitivity is still 86% and specificity is 98%.24When HR is reduced below 70 beats/min, image quality is improved, especially in terms of the visualization of the right coronary and left circumflex arteries, which are both significantly prone to motion artifacts (particularly at higher HR) because of their close proximity to the atrium, which is reactivated during the early diastolic phase.25For evaluation of coronary artery calcium volume with MDCT, thin-slice retrospective spiral electrocardiographic–gated scanning is desirable.26Most studies on coronary calcification have been performed using EBCT, which is still considered the “gold standard” (table 1). To image the coronary arteries with EBCT, 30–40 axial images are obtained with 3-mm slice thickness, using single-slice prospective electrocardiographic triggering in the craniocaudal direction along the full length of the heart. Rapid image acquisition at 100 ms allows accurate measurement of calcium deposits in the coronary arteries.27Quantification of coronary artery calcifications was found to be independently associated with cardiac events in a 3-yr follow-up of thousands of initially asymptomatic patients.28Noninvasive characterization and quantification of atherosclerotic plaque burden may also have important implications for the prevention of CAD progression and its complications.18For the past several years, CMRI has been considered the reference standard for assessment of left ventricular (LV) functions. However, cardiac CT is playing an increasingly important role in this evaluation. Both CT and CMRI surpass two-dimensional imaging techniques, such as standard two-dimensional echocardiography, for cardiac quantification because of their ability to generate contiguous short axis cine images, allowing for three-dimensional measurements without the use of geometric assumptions.29Postprocessing tools allow fast and semiautomatic determination of LV function parameters from MDCT data in analogy to known CMRI evaluation approaches.30Studies have demonstrated excellent correlation between cardiac CT and CMRI for LV ejection fraction, end-diastolic volume, end-systolic volume, stroke volume, and myocardial mass.30Furthermore, global and regional LV functions agree well with echocardiography, with correlation coefficients ranging from 0.91 to 0.97 for MDCT and from 0.93 to 0.98 for CMRI.7,30,31Although MDCT is not considered to be the first-line modality for assessment of LV function, it can provide a combined assessment of cardiac morphology and function without the need for additional radiation exposure in patients undergoing MDCT coronary angiography. The acquisition of images is performed according to the R–R interval. For MDCT, the image data are gated with the electrocardiogram to allow reconstruction at various times throughout the cardiac cycle. CT allows quantitative analysis of regional and global systolic function in normal and pathologic conditions by using short axis slices from the base to the apex of the heart. End-diastole and end-systole are defined as maximal and minimal LV volume, and LV ejection fraction is the difference between them.30Diastolic function can also be assessed from the rate of change of the LV volume during diastole.13Also, because temporal resolution for electrocardiographic-gated cardiac CT scans (down to 165 ms) is poorer in comparison to cine CMRI (30–40 ms), CT imaging may miss peak ejection rate or peak filling rate. However, advances in MDCT imaging that will improve temporal resolution may correct this problem.16Regional wall motion abnormalities, myocardial thinning, ventricular aneurysm, and mural thrombi in the infarcted area can all be detected by cardiac CT. Dual-phase contrast CT can detect acute MI characterized by an initial filling defect and late enhancement at the site of the damaged myocardium. Late enhancement may have the potential to distinguish viable from nonviable myocardium, and has significant prognostic value for the recovery of the myocardial wall motion and thickness after ischemia and in response to therapeutic revascularization.32In patients with previous MI, MDCT permits accurate, noninvasive assessment of coronary artery stenosis, LV function, and perfusion, assessed from a single data set.33If the myocardium supplied by the stenotic or occluded vessel is still viable, medical treatment or revascularization can be considered to reduce the risk of perioperative ischemia in the affected myocardium.Experimental studies in animals and humans demonstrated that cardiac CT could be used to assess microvascular function, although this technique is not used clinically, partially because of high radiation exposure. Evaluation of myocardial perfusion at rest and after infusion of vasodilators imposing cardiac challenge can reveal the presence of otherwise undetectable limited myocardial flow reserve, which might have a significant value for detection of borderline or very early alterations in cardiac microvascular function, as well as detecting increases in microvascular permeability that may reflect endothelial dysfunction or ischemic changes.34The use of CT scanning is limited by the need for contrast media and radiation exposure (table 2). The risks associated with contrast media administration include extravasation at the contrast injection site, allergic contrast reaction, and a decline in function, as well as and vascular In patients with administration of contrast media is media has been reduced and is than that used during angiography potential of alternative contrast such as might further decrease the various radiation are with MDCT, and scanning may allow a decrease in radiation of cardiac function and volumes may from to potentially any based on gated that data several cardiac limitations include the need to image quality in patients and the need for and physicians for acquisition and of cardiac CT data of the limitations of MDCT is the need to decrease heart rate at time of which may be in some It further the need for CT technology to acquisition time a greater volume of developed scanners such as the Dual-Source MDCT may this to temporal the number of that can be from axial CT images is limited only by gantry rotation speed and patient heart decreased ejection fraction in is associated with decreased overall postoperatively and with increased incidence of heart failure, but no has been found with main associated with cardiac events is the presence of wall motion has a low predictive is appropriate in patients who American Heart Association–American College of Cardiology clinical guidelines and who would require if no surgery were as well as in with aortic sensitivity of can be by the heart either or pharmacologically. stress test with administration of can provide information about cardiac function, ventricular and function, and a is in to 85% of maximal HR or or as wall motion abnormalities, whereas a in ejection fraction in response to administration is a of more severe CAD. has excellent negative predictive values and sensitivity but moderate specificity and is significantly and long-term cardiac risk using initial cardiac risk assessment and noninvasive testing with may the perioperative and long-term treatment of patients undergoing major vascular comparison with other noninvasive a diagnostic new of may improve cardiac risk compared with standard in patients undergoing noncardiac surgery whose functional capacity be evaluated by stress stress has a relatively low and can assess cardiac structure and function, wall thickness, aortic and the presence of However, has sensitivity for detection of single vessel disease and is to distinguish between microvascular and CAD (table because wall motion can be found in progress has been made during the past in which is used to evaluate a of heart and including CAD. CMRI is based on the of which and to an magnetic CMRI a causes deflection of the from the direction of the main magnetic The is during the of the back their in the magnetic are and data are used for image many image are for assessment of global ventricular and function, ejection fraction, stroke volume, and detection of and acute and chronic LV can be and using analysis of may find use in the clinical magnetic resonance imaging has higher temporal resolution but resolution (the ability to distinguish between two on the image) than cardiac resolution of 1.5 × 1.5 may have an CMRI for detection of because its overall accuracy in the detection of coronary artery stenosis is higher (table 2). The axial image of MDCT allows more vascular as compared with CMRI that may artifacts to sensitivity and specificity of CMRI for detection of coronary artery stenosis are and whereas of coronary segments are is noninvasive and high-quality images of the heart (table 2). However, CMRI examination be applied in patients who have a or and the presence of and stents can CMRI images and to is the of the test than an during which the patient must be CMRI is an important noninvasive tool and does not the use of potential contrast are needed to evaluate its use as a perioperative tool and its correlation with perioperative cardiac use of myocardial computed tomography and tomography has in the past two because of their in useful information about myocardial perfusion and function based on administration of an unstable with has been used for many for preoperative cardiac evaluation. the coronary response associated with testing not the HR and has been used in patients scheduled to undergo vascular surgery. However, has low and negative predictive and was not significantly associated with the incidence of perioperative MI, ischemia, or other the other the of this test was when used in patients undergoing aortic surgery whose risk could not have been on the of clinical value of imaging for preoperative cardiac assessment in high-risk has been demonstrated in patients before and vascular of myocardial with is important in patients with LV function to and the potential of revascularization can be These studies showed excellent correlation of with cardiac both perioperatively within 30 days and at long-term has high sensitivity in coronary disease but limited sensitivity for detecting significant disease and CAD (table 2). It also little functional data other than myocardial perfusion and has relatively low resolution that the quality of scanners have a maximal of approximately is required for before the performance of myocardial perfusion because the coronary effect of or is by with can be used to functional recovery of viable myocardium after coronary artery a is from a a in and with an results in of both the and the with to in the of electromagnetic radiation of two which in from each and are detected by a of radiation detectors at The difference in time the photons each radiation detector is used to detect the source of the of radiation detectors are in the gantry with million of per the imaging is useful in noninvasive quantification of myocardial flow and coronary flow reserve, and is also of detecting early disease in high-risk asymptomatic and the progression or of is to especially in patients and in undergoing remains a significant its high and limited availability currently its use as a technology of coronary angiography is well and involves injection of contrast media into the coronary artery by imaging in multiple planes. is by comparison of diameter at a stenotic site with that of a normal reference can evaluate coronary stenosis, LV function, and (table coronary angiography is an invasive technique and has a major rate of including mortality in of it may involve for the patient and is associated with other such as vascular complications for diagnostic and for complications and complications the risk of coronary angiography with that of MDCT, some risks are common to both procedures, such as an allergic contrast reaction, and exposure to whereas are to contrast media injection causes severe allergic in of risks of a of for MDCT angiography and for coronary angiography. However, the and risks and a overall risk of mortality from coronary that for MDCT angiography use of a power in a vascular with cardiac CT an additional risk of which in of the relatively significant number of negative invasive angiographies performed each the risks to this procedure by using noninvasive can greatly to the morbidity and mortality of coronary angiography. the number of negative angiographies might to significant because the of cardiac may be as as times that of cardiac evaluation of patients with or known cardiac disease for noncardiac surgery has been in the of of and The preoperative evaluation on assessment of clinical predictors, functional capacity, and the extent of the surgical The first-line to reduce perioperative morbidity and mortality include medical therapy with with HR and However, the strategy may also include of noninvasive imaging techniques that provide comprehensive and assessment of cardiac structure and In particular, in patients with restricted physical in assessment of the functional capacity is obtained using imaging with evaluation of the heart may potentially to treatment Cardiac CT is can provide coronary angiography for plaque assessment and scoring, and can assess LV function, and myocardial in a fast and relatively CT can in out significant CAD and determination of the need for invasive angiography. cardiac CT might potentially be the tool for overall evaluation of cardiac patients for noncardiac surgery. at this is no that preoperative cardiac CT scanning is to reduce perioperative studies are needed to assess the role of cardiac CT in the evaluation of patients at risk for cardiovascular disease before noncardiac surgery.

To systematically review and evaluate the methodological quality of studies using magnetic resonance imaging (MRI) and computed tomography (CT) radiomics for cardiac applications. Multiple medical literature archives (PubMed, Web of Science, and EMBASE) were systematically searched to retrieve original studies focused on cardiac MRI and CT radiomics applications. Two researchers in consensus assessed each investigation using the radiomics quality score (RQS). Subgroup analyses were performed to assess whether the total RQS varied according to study aim, journal quartile, imaging modality, and first author category. From a total of 1961 items, 53 articles were finally included in the analysis. Overall, the studies reached a median total RQS of 7 (IQR, 4-12), corresponding to a percentage score of 19.4% (IQR, 11.1-33.3%). Item scores were particularly low due to lack of prospective design, cost-effectiveness analysis, and open science. Median RQS percentage score was significantly higher in papers where the first author was a medical doctor and in those published on first quartile journals. The overall methodological quality of radiomics studies in cardiac MRI and CT is still lacking. A higher degree of standardization of the radiomics workflow and higher publication standards for studies are required to allow its translation into clinical practice. • RQS has been recently proposed for the overall assessment of the methodological quality of radiomics-based studies. • The 53 included studies on cardiac MRI and CT radiomics applications reached a median total RQS of 7 (IQR, 4-12), corresponding to a percentage of 19.4% (IQR, 11.1-33.3%). • A more standardized methodology in the radiomics workflow is needed, especially in terms of study design, validation, and open science, in order to translate the results to clinical applications.

Scimitar syndrome is a rare, complex congenital anomaly characterized by partial anomalous pulmonary venous drainage into the inferior vena cava with hypoplasia of the right-sided lung, pulmonary artery and bronchial tree. Frequently aortopulmonary collaterals co-occur incrising pulmonary blood flow and hence the risk of pulmonary arterial hypertension. Surgical correction remains the gold-standard therapy. The autors present the case of a 23-days-old newborn after food aspiration, presenting respiratory symptoms: tachypnoe, tachycardia, respiratory effort with the use of accessory muscles, cough and crackles. The chest X-ray revealed the right lung consolidation. Laboratory markers of inflammatory process were negative. Aspiration pneumonia was diagnosed and the treatment was introduced. Despite the newborn presented respiratory distress with radiological changes. The echocardiography was performer and scimitar syndrome was suspected. The diagnosis was confirmed during cardiac catheterization in which aortopulmonary collateral artery running to the right lower lobe was demonstrated. The vessel was embolized with Amplatzer device.

Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): The research fund of the Heart Center, Rigshospitalet, Denmark Background Assessment of left ventricular (LV) volumes and function is crucial in managing patients. New imaging modalities are becoming more common. It is therefore important to compare them with the standard echocardiographic method that most treatments rely on and to determine if they are suitable for all LV geometries. Purpose The purpose was to compare end-diastolic volume (EDV), end-systolic volume (ESV) and LV ejection fraction (LVEF) for the three most common imaging modalities; echocardiography, cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT). Methods We included 85 subjects with various LV geometries; no cardiac disease (n = 44) and various cardiac disorders (n = 41). Cardiac assessment was performed using echocardiography followed immediately by CMR; re-examination after median 6 days, interquartile range 3-18 days using echocardiography followed immediately by CCT. We compared EDV, ESV and LVEF by three-dimensional echocardiography (echo-3D), CMR and CCT to echocardiographic biplane method of discs (echo-BP). The population was divided in four LV geometry profiles (normal, dilatation, hypertrophy, dilatation and hypertrophy) according to gender, age and indexed CMR-values of EDV and LV mass. We calculated inter-modality-ratios by dividing the values from echo-3D, CMR and CCT with echo-BP, to evaluate variances between the LV geometries. Results The figure demonstrates the agreement to echo-BP divided by geometry. Echo-3D had overall best agreement to EDV, ESV and LVEF. CMR overestimated both EDV and ESV. CCT overestimated EDV but not ESV. CCT overestimated LVEF by 4-16% in absolute values, whereas CMR and echo-3D had better agreement for LVEF. The correlation between echo-BP and echo-3D, CMR, and CCT, respectively was; EDV 0.91, 0.94, 0.90, ESV 0.86, 0.86, 0.79, and LVEF 0.40, 0.46, 0.38, all p < 0.001. CMR especially overestimated EDV and ESV in "hypertrophy and dilatation" whereas CCT especially underestimated EDV and ESV in solely "hypertrophy", with larger overestimation of LVEF. ANOVA-analysis of inter-modality-ratios between LV geometries indicated significant variation for EDV but not ESV by echo-3D (F = 2.9, p < 0.05 and F = 1.6, NS), no significant variation for EDV or ESV by CMR (F = 0.01 and 2.4, both NS), and significant variation for both EDV and ESV by CCT (F = 5.4, p < 0.01 and 7.2, p < 0.001). No significant variation for LVEF by echo-3D (F = 1.0, NS), but significant variation for CMR and CCT (CMR: F = 4.5, p < 0.01 and CCT: F = 8.6, p < 0.001) with slightly higher variation for CCT. Conclusions Echo-3D had the overall best agreement of volumes and LVEF, compared to echo-BP as a reference. CMR overestimated EDV and ESV whereas CCT overestimated EDV but not ESV, resulting in overestimation of LVEF by CCT but not CMR. In hypertrophic non-dilated LVs; CCT underestimated both EDV and especially ESV, with larger overestimation of LVEF. In general, CMR appears to be less dependent on LV geometry compared to echo-3D and CCT. Abstract Figure.

Scimitar Syndrome: The Role of Cardiac Magnetic Resonance

Scimitar, or pulmonary venolobar, syndrome is a rare but well-known congenital cardiovascular defect that includes a hypoplastic right pulmonary artery and right lung, which leads to displacement of cardiac structures into the right hemithorax, anomalous systemic arterial supply to the right lung, and a characteristically curved anomalous right pulmonary vein that drains into the inferior vena cava and resembles the curved Middle Eastern sword “scimitar.”1,2 A variety of congenital thoracic abnormalities are associated with this specific type of partial anomalous pulmonary venous return.3 Imaging, and specifically findings from magnetic resonance imaging, in an 18-month-old male (11 kg body weight) with known congenital right pulmonary venolobar syndrome with increasingly frequent cyanotic episodes are presented. Findings on chest radiography and contrast-enhanced computed tomography of the chest performed when the patient was 4 days old included right lung hypoplasia and partial anomalous pulmonary venous return with scimitar vein to the supradiaphragmatic inferior vena cava (Figure 1). Echocardiography identified the scimitar vein and an atrial septal defect. Cardiac magnetic resonance imaging, including 4-dimensional flow-sensitive magnetic resonance imaging, confirmed these findings but also identified additional cardiovascular abnormalities, including an additional partial anomalous pulmonary venous return from the upper right lung to the superior vena cava and an anomalous systemic artery from the upper abdominal aorta to the lower right lung (Figure 2). Further comprehensive analysis …

Cardiac computed tomography (CT) produces high-quality anatomical images of the cardiac valves and associated structures. Cardiac magnetic resonance imaging (MRI) provides images of valve morphology, and allows quantitative evaluation of valvular dysfunction and determination of the impact of valvular lesions on cardiovascular structures. Recent studies have demonstrated that cardiac CT and MRI are important adjuncts to echocardiography for the evaluation of aortic and mitral valvular heart diseases (VHDs). Radiologists should be aware of the technical aspects of cardiac CT and MRI that allow comprehensive assessment of aortic and mitral VHDs, as well as the typical imaging features of common and important aortic and mitral VHDs on cardiac CT and MRI.