Mitral annular disjunction and mitral valve prolapse extrapolating adult data to an adolescent cohort?

PURPOSE OF REVIEW: The aim of this study is to provide an update on mitral valve prolapse (MVP) and mitral annular disjunction (MAD) and who may be at risk for ventricular arrhythmias and sudden cardiac death. RECENT FINDINGS: MVP is generally considered a benign condition. However, a small subset of patients may be at risk for life-threatening ventricular arrhythmias. Among the risk factors identified in adults include patients with bileaflet mitral valves, myxomatous changes, myocardial fibrosis, and the presence of MAD. Advances in multimodal imaging have allowed for improved identification of fibrosis, anatomical valve derangements, and the amount of MAD. Recent guidelines have suggested that patients with MVP with or without MAD may be at risk for life-threatening arrhythmias if they have had prior ventricular arrhythmias, ventricular dysfunction, or unexplained syncope. Yet, extrapolation of adult data to a pediatric cohort with similar MVP and MAD at this juncture is challenging. There is, however, early evidence that some pediatric patients with significant myocardial fibrosis or abnormal tissue Doppler may be at risk for ventricular tachycardia. SUMMARY: Mitral valve prolapse and mitral annular disjunction at times coexist and at other times can be seen as isolated entities. While the incidence of arrhythmic MVP is quite rare, there is increasing evidence that certain select adults with MVP may be at risk for ventricular tachycardia and sudden cardiac death. Future multicenter studies are needed to better understand the natural history of arrhythmic mitral valve disease and how early disease manifestation in children may impact findings now being reported in young adults.

Arrhythmogenic right ventricular cardiomyopathy in the pediatric population.

PURPOSE OF REVIEW: Review the current state of the art of arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis and risk stratification in the pediatric population. RECENT FINDINGS: ARVC is an inherited cardiomyopathy characterized by progressive myocyte loss and fibrofatty replacement of predominantly the right ventricle and high risk of ventricular arrhythmias and sudden cardiac death (SCD). ARVC is one of the leading causes of arrhythmic cardiac arrest in young people. Early diagnosis and accurate risk assessment are challenging, especially in children who often exhibit little to no phenotype, even if genotype positive. Multimodal imaging provides more detailed assessment of the right ventricle and has been shown in pediatric patients to identify earlier preclinical disease expression. Identification of patients with ARVC allows the clinician to intervene early with appropriate exercise restrictions, even if genotype positive only without phenotypic expression. Emphasis should be placed on stratifying the patient's risk of ventricular arrhythmias and SCD. SUMMARY: ARVC is a challenging diagnosis to make in adolescents who often do not exhibit clinical symptoms. Newer multimodal imaging techniques and improvements in genetic testing and biomarkers should help improve early diagnosis. Exercise restriction for children with ARVC has been shown to reduce disease advancement and decreases the risk of a life-threatening event.

Reduction in radiation exposure in cardiovascular computed tomography imaging: results from the PROspective multicenter registry on radiaTion dose Estimates of cardiac CT angIOgraphy iN daily practice in 2017 (PROTECTION VI).

Aims: Advances of cardiac computed tomography angiography (CTA) have been developed for dose reduction, but their efficacy in clinical practice is largely unknown. This study was designed to evaluate radiation dose exposure and utilization of dose-saving strategies for contrast-enhanced cardiac CTA in daily practice. Methods and results: Sixty one hospitals from 32 countries prospectively enrolled 4502 patients undergoing cardiac CTA during one calendar month in 2017. Computed tomography angiography scan data and images were analysed in a central core lab and compared with a similar dose survey performed in 2007. Linear regression analysis was performed to identify independent predictors associated with dose. The most frequent indication for cardiac CTA was the evaluation of coronary artery disease in 89% of patients. The median dose-length product (DLP) of coronary CTA was 195 mGy*cm (interquartile range 110-338 mGy*cm). When compared with 2007, the DLP was reduced by 78% (P < 0.001) without an increase in non-diagnostic coronary CTAs (1.7% in 2007 vs. 1.9% in 2017 surveys, P = 0.55). A 37-fold variability in median DLP was observed between the hospitals with lowest and highest DLP (range of median DLP 57-2090 mGy*cm). Independent predictors for radiation dose of coronary CTA were: body weight, heart rate, sinus rhythm, tube voltage, iterative image reconstruction, and the selection of scan protocols. Conclusion: This large international radiation dose survey demonstrates considerable reduction of radiation exposure in coronary CTA during the last decade. However, the large inter-site variability in radiation exposure underlines the need for further site-specific training and adaptation of contemporary cardiac scan protocols.

An unusual presentation of a large cardiac mass.

We present a case of a large left ventricular capillary haemangioma incidentally discovered in a pre-adolescent patient.

Prevalence of Venovenous Shunting and High-Output State Quantified with 4D Flow MRI in Patients with Fontan Circulation.

PURPOSE: To assess the ability of four-dimensional (4D) flow MRI to quantify flow volume of the Fontan circuit, including the frequency and hemodynamic contribution of systemic-to-pulmonary venovenous collateral vessels. MATERIALS AND METHODS: In this retrospective study, patients with Fontan circulation were included from three institutions (2017-2021). Flow measurements were performed at several locations along the circuit by two readers, and collateral shunt volumes were quantified. The frequency of venovenous collaterals and structural defects were tabulated from concurrent MR angiography, contemporaneous CT, or catheter angiography and related to Fontan clinical status. Statistical analysis included Pearson and Spearman correlation and Bland-Altman analysis. RESULTS: Seventy-five patients (mean age, 20 years; range, 5-58 years; 46 female and 29 male patients) were included. Interobserver agreement was high for aortic output, pulmonary arteries, pulmonary veins, superior vena cava (Glenn shunt), and inferior vena cava (Fontan conduit) (range, ρ = 0.913-0.975). Calculated shunt volume also showed strong agreement, on the basis of the difference between aortic and pulmonary flow (ρ = 0.935). A total of 37 of 75 (49%) of the patients exhibited shunts exceeding 1.00 L/min, 81% (30 of 37) of whom had pulmonary venous or atrial flow volume step-ups and corresponding venovenous collaterals. A total of 12% of patients (nine of 75) exhibited a high-output state (>4 L/min/m2), most of whom had venovenous shunts exceeding 30% of cardiac output. CONCLUSION: Fontan flow and venovenous shunting can be reliably quantified at 4D flow MRI; high-output states were found in a higher proportion of patients than expected, among whom venovenous collaterals were common and constituted a substantial proportion of cardiac output.Keywords: Pediatrics, MR Angiography, Cardiac, Technology Assessment, Hemodynamics/Flow Dynamics, Congenital Supplemental material is available for this article. © RSNA, 2021.

Left Ventricular Aneurysm in a Child After Recovering From Dilated Cardiomyopathy.

Left ventricular aneurysms are extremely rare in children. A child developed an aneurysm a year after recovering from idiopathic dilated cardiomyopathy. The initial management was conservative. After several years and due to aneurysm enlargement and other complications, the patient underwent successful aneurysm surgical repair with left ventricular aneurysmorrhaphy. We describe our experience treating this child during the course of this disease.

Clinical role of F-18 fluorodeoxyglucose positron emission tomography imaging in patients with lung cancer and suspected malignant pleural effusion.

STUDY OBJECTIVES: The goals of this study were to determine the sensitivity, specificity, and predictive accuracy of F-18 fluorodeoxyglucose positron emission tomography (PET-FDG) imaging in detecting metastatic disease involvement of pleura and/or presence of malignant pleural effusion in patients with proven lung cancer. We wanted to compare efficacy of PET-FDG imaging to CT scanning in differentiating benign pleural effusion from malignant effusion and/or pleural involvement in patients with lung cancer. METHODS: We studied 35 patients with biopsy-proven lung cancer and abnormal findings on CT scanning for presence of pleural effusion (n = 34) and/or pleural thickening or nodular involvement (n = 4). The results of positron emission tomography and CT scanning were compared to pleural cytology (n = 31), histologic findings of pleural biopsy (n = 3), and/or clinical follow-up (n = 3) for at least 1 year for presence or absence of malignant pleural effusion. RESULTS: PET-FDG imaging correctly detected the presence of malignant pleural effusion and malignant pleural involvement in 16 of 18 patients and excluded malignant effusion or pleural metastatic involvement in 16 of 17 patients (sensitivity, specificity, and accuracy of 88.8%, 94.1%, and 91.4% respectively). CONCLUSION: PET-FDG imaging is a highly accurate and reliable noninvasive test to differentiate malignant from benign pleural effusion and/or pleural involvement in patients with lung cancer and findings of suspected malignant pleural effusion on CT scanning.

Pulmonary artery size as a predictor of pulmonary hypertension and outcomes in patients with chronic obstructive pulmonary disease.

RATIONALE: The relationship between pulmonary artery size with underlying pulmonary hypertension and mortality remains to be determined in COPD. We sought to evaluate the relationships in a cohort of patients with advanced COPD. METHODS: A retrospective study of advanced COPD patients evaluated between 1998 and 2012 was conducted at a tertiary care center. Patients with chest computed tomography images and right heart catheterizations formed the study cohort. The diameters of the pulmonary artery and ascending aorta were measured by independent observers and compared to pulmonary artery pressures. Intermediate-term mortality was evaluated for the 24-month period subsequent to the respective studies. Cox proportional hazards model was used to determine independent effects of variables on survival. RESULTS: There were 65 subjects identified, of whom 38 (58%) had pulmonary hypertension. Patients with and without pulmonary hypertension had mean pulmonary artery diameters of 34.4 mm and 29.1 mm, respectively (p = 0.0003). The mean PA:A ratio for those with and without pulmonary hypertension was 1.05 and 0.87, respectively (p = 0.0003). The PA:A ratio was an independent predictor of mortality with a reduced survival in those with a PA:A >1 (p = 0.008). CONCLUSIONS: The PA:A ratio is associated with underlying pulmonary hypertension in patients with COPD and is an independent predictor of mortality. This readily available measurement may be a valuable non-invasive screening tool for underlying pulmonary hypertension in COPD patients and appears to impart important independent prognostic information.