Screening tools for the detection of clinically silent cardiac sarcoidosis.

BACKGROUND: clinically silent cardiac sarcoidosis (CS) may be associated with adverse outcomes, hence the rationale for screening patients with extracardiac sarcoidosis. The optimal screening strategy has not been clearly defined. METHODS: patients with extra-cardiac sarcoidosis were prospectively included and underwent screening consisting of symptom history, electrocardiography (ECG), transthoracic echocardiogram, Holter, and signal-averaged ECG (SAECG). Cardiac magnetic resonance (CMR) was performed in all patients. Clinically silent CS was defined as CMR demonstrating late gadolinium enhancement (LGE) in a pattern compatible with CS according to a majority of independent and blinded CMR experts. Significant cardiac involvement was defined as the presence of LGE ≥6% and/or a positive fluorodeoxyglucose-positron emission tomography. RESULTS: among the 129 patients included, clinically silent CS was diagnosed in 29/129 (22.5%), and 19/129 patients (14.7%) were classified as CS with significant cardiac involvement. There was a strong association between hypertension and CS (p < 0.05). Individual screening tools provided low diagnostic yield; however, combination of tests performed better, for example, a normal Holter and a normal SAECG had negative predictive values of 91.7%. We found consistently better diagnostic accuracy for the detection of CS with significant cardiac involvement. CONCLUSION: clinically silent CS and CS with significant cardiac involvement were found in 22.5% and 14.7% of patients with extra-cardiac sarcoidosis. The association with hypertension raises the possibility that some cases of hypertensive cardiomyopathy may be mistaken for CS. Screening with readily available tools, for example Holter and SAECG, may help identifying patients without CS where additional CMR is not needed.

Mitral annular disjunction on cardiac MRI: Prevalence and association with disease severity in Loeys-Dietz syndrome.

BACKGROUND: The purpose of this study was to evaluate mitral annular disjunction (MAD) on cardiac magnetic resonance imaging (MRI) in Loeys-Dietz Syndrome (LDS) and to explore its association with adverse outcomes. METHODS: In this retrospective cohort study, adult patients with LDS who underwent cardiac MRI were evaluated for MAD, aortic dimensions, and ventricular volumetry. Aortic events were defined as aortic surgery and/or dissection and severe arrhythmic events as cardiac arrest or sustained ventricular tachycardia (VT). RESULTS: Among 46 LDS patients (52% female, 37.2 ± 14.3 years), 17 had MAD (37%). MAD and no MAD groups were similar in age, sex, aortic dimensions and left ventricular parameters. After a clinical follow-up of 4.3 years (IQR 1.5-8.4), 3 in MAD and 4 in no MAD groups required aortic valve sparing root replacement (VSRR) and 1 in MAD developed type A dissection. Over a similar imaging follow-up period [4.1 years (IQR 2.7-9.1) vs. 3.2 years (IQR 1.0-9.0), p = 0.65], compared to baseline, increase in native aortic root size was significant only in MAD (39.4 ± 4.6 mm vs. 38.1 ± 5.3 mm, p = 0.02, 19.3 ± 2.4 mm/m2 vs. 18.7 ± 2.4 mm/m2, p = 0.01) compared to those without MAD. Patients with MAD were younger at first aortic event compared to those without (26.7 ± 11.5 years vs. 45.0 ± 14.9 years, p = 0.03). MAD distance correlated with need for VSRR, r = 0.57, p = 0.02. Two patients in the MAD group developed sustained VT. No cardiac arrest or death was observed. CONCLUSION: MAD is highly prevalent in LDS, associated with progressive aortic dilatation, and aortic events at younger age. MAD may be a marker of disease severity necessitating close surveillance.

Association of Pectus Excavatum With Ventricular Remodelling and Mitral Valve Abnormalities in Marfan Syndrome.

Background: Marfan syndrome (MFS) is an inherited connective tissue disorder. Pectus excavatum (PEX) is common in MFS. The purpose was to evaluate the association of PEX with cardiovascular manifestations of MFS, biventricular size and function. Methods: MFS adults undergoing cardiac MRI were retrospectively evaluated. Exclusion criteria were incomplete cardiac MRI, significant artifacts, co-existent ischaemic or congenital heart disease. Haller Index (HI) ≥3.25 classified patients as PEX positive (PEX+) and PEX negative (PEX-). Cardiac MRI analysis included assessment of mitral valve prolapse (MVP), mitral annular disjunction (MAD), biventricular volumetry and aortic dimensions. Results: 212 MFS patients were included, 76 PEX+ and 136 PEX- (HI 8.3 ± 15.2 vs 2.3 ± 0.5, P < .001). PEX+ were younger (33.4 ± 12.0 vs 38.1 ± 14.3 years, P = .02) and similar in sex distribution (55% vs 63% male, P = .26) compared to PEX-. MVP and MAD were more frequent in PEX+ vs PEX- (43/76 [57%] vs 37/136 [27%], P < .001; 44/76 [58%] vs 50/136[37%], P = .003, respectively). PEX+ had higher right ventricular end-diastolic and end-systolic volumes (RVEDVi 92 ± 17mL/m2 vs 84 ± 22mL/m2, P = .04; RVESVi 44 ± 10 mL/m2 vs 39 ± 14 mL/m2, P = .02), lower RV ejection fraction (RVEF 52 ± 5% vs 55 ± 6%, P = .01) compared to PEX-. Left ventricular (LV) volumes, LVEF and aortic dimensions were similar. Conclusion: MFS adults with PEX have higher frequency of cardiac manifestations including MV abnormalities, increased RV volumes and lower RVEF compared to those without PEX. Awareness of this association is important for all radiologists who interpret aortic CT or MRI, where HI can be easily measured. PEX in MFS may suggest more severe disease expression necessitating careful screening for MV abnormalities and outcomes surveillance.

Diagnostic Accuracy of MRI in Evaluating Response After Neoadjuvant Systemic Therapy in Operable Breast Cancer.

Background Neoadjuvant chemotherapy (NAC) is an important step in the treatment of various types of breast cancer by downsizing the tumor to make it operable. Determining disease extent after NAC is essential for accurate surgical planning. MRI has been the gold standard for detecting tumors that are usually difficult to detect on ultrasound or mammography. However, the use of MRI after NAC is controversial. Therefore, we aimed to evaluate the diagnostic accuracy of post-NAC MRI in the detection of residual disease preoperatively and to investigate the factors associated with pathological complete response (pCR). Methodology This retrospective review study was approved by the institutional review board with waiving of the informed consent. A total of 90 charts between January 2016 and January 2019 were reviewed. Baseline lesion size was measured as the maximal diameter in a single dimension by pretreatment MRI. To assess the diagnostic accuracy of MRI in detecting residual disease, we used two different definitions of pCR in the breast. The first is the resolution of both invasive disease and ductal carcinoma in situ. The second is the resolution of the invasive disease only. As a secondary objective of the study, we assessed the association between different patients' characteristics and both MRI and pathologic response using univariate and multivariate analysis. Results A total of 52 women (mean age: 47.4 years; range: 28-74) with 56 breast masses were eligible for the study. Complete MRI response was noted in 22 (39%) masses. pCR was achieved in 14 (25%) and 25 (44.6%) masses using the first and second pCR definitions, respectively. The negative predictive value (NPV) and overall accuracy of MRI for detecting residual disease were 50% and 75%, respectively, using the first pCR definition. With the second pCR definition, NPV and accuracy were 77.3% and 76.8%, respectively. Positive axillary lymph nodes were the only significant factor associated with incomplete MRI and pathological responses. Conclusions MRI NPV for residual disease was higher with the second pCR definition; however, overall accuracy was not different. MRI accuracy in detecting residual disease after NAC is not adequate to replace pathological assessment.

Clinicians' Behavior Toward Radiology Reports: A Cross-Sectional Study.

Background The radiology report is the way of communication between the radiologists and the clinicians of different specialties. Each part of the report is important and significant in the patient management plan. Therefore, knowledge of interpretation and behavior in understanding the final report is a variable crucial skill. Methods This is a cross-sectional survey study to explore the behavior and attitude of clinicians toward radiology reports in relation to their professional clinical demographic. A total of 107 physicians participated, including consultants, specialists, and residents among different specialties. Results Among the 107 responses, 58.9% were male and 41.1% were female. The majority of the physicians (78.5%) read the radiology report for every requested study for each patient, while 21.5% of participants didn't read the radiology report for the studies they requested, instead, they only read it occasionally. Gender played a significant factor, as female practitioners were more likely to read the complete radiology report (P = 0.033). In addition, the age of the practitioner was also significant as clinicians in the age group 40-60 years old were more likely to check the requested radiology image prior to reading the report compared to age groups 20-39 and >60 years (P = 0.035). Lastly, specialists were significantly more likely to read the entire radiology report compared to consultants and residents (P = 0.006). Conclusion More emphasis and awareness should be provided to clinicians on the importance of reading the entire radiology report as some information can be missed if not being read completely.

Bowel Damage at Diagnosis Using the Lémann Index Score in Saudi Arabian Patients With Crohn's Disease.

Background Advanced bowel damage caused by Crohn's disease (CD) in the form of strictures and penetrating lesions has been associated with future surgical resection. However, in general, the degree of bowel damage in patients with CD is not examined at the time of diagnosis, and the natural history of CD may differ phenotypically between patients from Arabic countries as compared to patients from Europe and North America. Thus, we aimed to assess the degree of structural bowel damage in Saudi Arabian CD patients at diagnosis. We used the Lémann Index (LI) score, an instrument that measures cumulative digestive tissue damage by magnetic resonance enterography (MRE) and endoscopy, to establish any possible association between the duration of symptoms and the degree of bowel damage. Method This retrospective study was conducted by reviewing the data of all CD patients following up at King Abdulaziz University Hospital (KAUH) that were investigated by endoscopy and MRE at baseline. MRE-LI was calculated by scoring previous surgery, disease location and extension, and intestinal complications. A LI score of >2.0 was set as the cut-off point for bowel damage. Descriptive statistics were used to provide an overview of demographic and clinical characteristics, and hypothesis testing was applied to identify associations. Result Eighty-three patients with CD were included in this study. Fifty point six percent (50.6%) of the cohort comprised females and the median age was 27 years. With regards to CD location and extension, 34.9% showed ileal disease (L1), 9.6% showed colonic CD (L2), whereas 55.4% had ileocolonic involvement (L3). Moreover, 48.2% of patients presented with non-complicated behavior (B1), 25.3% had at least one stricture (B2), and 26.5% showed a penetrating phenotype (B3). Perianal CD was observed in 2.4% of subjects and 62.7% had undergone bowel resection. Mean LI was 2.4 (±2.6) with 34 patients (39.8%) exhibiting an LI score indicative of advanced bowel damage at the time of diagnosis. The duration of symptoms did not correlate with the degree of bowel damage according to the LI score. Conclusion A significant proportion of patients with CD presented with advanced bowel damage at the time of diagnosis, suggesting that a severe form of CD may be endemic in Saudi Arabia.