Design and implementation of multicenter pediatric and congenital studies with cardiovascular magnetic resonance: Big data in smaller bodies.

Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.

Impact of Oral Nutritional Supplementation in Chronic Kidney Disease Patients on Maintenance Hemodialysis: An Open-label, Single-arm Study among Indian Patients.

BACKGROUND: Protein-energy wasting (PEW) affects about 50-75% of patients with chronic kidney disease (CKD), particularly those who are on maintenance hemodialysis (MHD). This study evaluated the efficacy and tolerability of an oral nutritional supplement in Indian patients receiving MHD. MATERIALS AND METHODS: This was a 3-month, prospective, open-label, and single-centered study. Eligible participants supplemented their regular diet with one sachet (40 gm) of oral nutritional supplement powder twice daily for 90 days. The study efficacy endpoints were mean change in acute phase proteins (albumin and prealbumin), anthropometric measurements [weight, body mass index (BMI), and triceps skin fold thickness], handgrip strength, hemoglobin, total iron binding capacity (TIBC), potassium, and phosphorus levels, malnutrition score (MS)-modified subjective global assessment (modified SGA), malnutrition inflammation score (MIS), and nutritional status. RESULTS: The study population comprised 36 (42.9%) men and 48 (57.1%) women with a mean age of 54.85 ± 15.50 years. A paired sample t-test was used to compare the baseline with end-of-study values for continuous variables. Serum albumin, prealbumin, hemoglobin, and phosphorus levels remained stable throughout the study period. The mean change in weight, BMI, triceps skin fold thickness, handgrip strength, and TIBC for the overall study population was 1.11 kg (1.82%, p < 0.0001), 0.46 kg/m2 (1.98%, p < 0.0001), 3.47 mm (30.78%, p < 0.0001), 6.05 kg (44.98%, p < 0.0001) and 11.80 µg/dL (6.06%, p < 0.0001), respectively. At the end of the study period, there was a significant (p < 0.0001) improvement in the SGA and MIS scores. Further, there was a significant improvement in nutritional status as demonstrated by the overall intake of calories (p < 0.001), proteins (p < 0.0001), carbohydrates (p = 0.003, and fats (p < 0.0001). CONCLUSION: Protein-energy malnutrition is a strong predictor of morbidity, mortality, and poor outcomes in CKD patients. A scientifically designed formula in accordance with KDOQI standards was able to improve the nutritional status, overall body composition, sarcopenia, and quality of life in CKD patients on MHD.

Ventricular dyssynchrony late after the Fontan operation is associated with decreased survival.

BACKGROUND: Ventricular dyssynchrony and its relationship to clinical outcomes is not well characterized in patients following Fontan palliation. METHODS: Single-center retrospective analysis of cardiac magnetic resonance (CMR) imaging of patients with a Fontan circulation and an age-matched healthy comparison cohort as controls. Feature tracking was performed on all slices of a ventricular short-axis cine stack. Circumferential and radial strain, strain rate, and displacement were measured; and multiple dyssynchrony metrics were calculated based on timing of these measurements (including standard deviation of time-to-peak, maximum opposing wall delay, and maximum base-to-apex delay). Primary endpoint was a composite measure including time to death, heart transplant or heart transplant listing (D/HTx). RESULTS: A total of 503 cases (15 y; IQR 10, 21) and 42 controls (16 y; IQR 11, 20) were analyzed. Compared to controls, Fontan patients had increased dyssynchrony metrics, longer QRS duration, larger ventricular volumes, and worse systolic function. Dyssynchrony metrics were higher in patients with right ventricular (RV) or mixed morphology compared to those with LV morphology. At median follow-up of 4.3 years, 11% had D/HTx. Multiple risk factors for D/HTx were identified, including RV morphology, ventricular dilation, dysfunction, QRS prolongation, and dyssynchrony. Ventricular dilation and RV morphology were independently associated with D/HTx. CONCLUSIONS: Compared to control LVs, single right and mixed morphology ventricles in the Fontan circulation exhibit a higher degree of mechanical dyssynchrony as evaluated by CMR-FT. Dyssynchrony indices correlate with ventricular size and function and are associated with death or need for heart transplantation. These data add to the growing understanding regarding factors that can be used to risk-stratify patients with the Fontan circulation.

Magnetic resonance three-dimensional steady-state free precession imaging of the thoracic duct in patients with Fontan circulation and its relationship to outcomes.

BACKGROUND: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes. METHODS: This was a retrospective, single-center study of patients with Fontan circulation who underwent CMR. Frequency matching of age at CMR was used to construct a comparison group of patients with repaired tetralogy of Fallot (rTOF). TD characteristics included maximum diameter and a qualitative assessment of tortuosity. Clinical outcomes included protein-losing enteropathy (PLE), plastic bronchitis, listing for heart transplantation, and death. A composite outcome was defined as presence of any of these events. RESULTS: The study included 189 Fontan patients (median age 16.1 years, IQR 11.0-23.2 years) and 36 rTOF patients (median age 15.7 years, IQR 11.1-23.7 years). The TD diameter was larger (median 2.50 vs. 1.95 mm, p = 0.002) and more often well visualized (65% vs. 22%, p < 0.001) in Fontan patients vs. rTOF patients. TD dimension increased mildly with age in Fontan patients, R = 0.19, p = 0.01. In Fontan patients, the TD diameter was larger in those with PLE vs. without PLE (age-adjusted mean 4.11 vs. 2.72, p = 0.005), and was more tortuous in those with NYHA class ≥ II vs. class I (moderate or greater tortuosity 75% vs. 28.5%, p = 0.02). Larger TD diameter was associated with a lower ventricular ejection fraction that was independent of age (partial correlation = - 0.22, p = 0.02). More tortuous TDs had a higher end-systolic volume (mean 70.0 mL/m2 vs. 57.3 mL/m2, p = 0.03), lower creatinine (mean 0.61 mg/dL vs. 0.70 mg/dL, p = 0.04), and a higher absolute lymphocyte count (mean 1.80 K cells/µL vs. 0.76 K cells/µL, p = 0.003). The composite outcome was present in 6% of Fontan patients and was not associated with TD diameter (p = 0.50) or tortuosity (p = 0.09). CONCLUSIONS: The TD is well visualized in two-thirds of patients with Fontan circulation on 3D-bSSFP images. Larger TD diameter is associated with PLE and increased TD tortuosity is associated with an NYHA class ≥ II.

Cardiac Magnetic Resonance Imaging to Determine Single Ventricle Function in a Pediatric Population is Feasible in a Large Trial Setting: Experience from the Single Ventricle Reconstruction Trial Longitudinal Follow up.

The Single Ventricle Reconstruction (SVR) Trial was a randomized prospective trial designed to determine survival advantage of the modified Blalock-Taussig-Thomas shunt (BTTS) vs the right ventricle to pulmonary artery conduit (RVPAS) for patients with hypoplastic left heart syndrome. The primary aim of the long-term follow-up (SVRIII) was to determine the impact of shunt type on RV function. In this work, we describe the use of CMR in a large cohort follow up from the SVR Trial as a focused study of single ventricle function. The SVRIII protocol included short axis steady-state free precession imaging to assess single ventricle systolic function and flow quantification. There were 313 eligible SVRIII participants and 237 enrolled, ages ranging from 10 to 12.5 years. 177/237 (75%) participants underwent CMR. The most common reasons for not undergoing CMR exam were requirement for anesthesia (n = 14) or ICD/pacemaker (n = 11). A total of 168/177 (94%) CMR studies were diagnostic for RVEF. Median exam time was 54 [IQR 40-74] minutes, cine function exam time 20 [IQR 14-27] minutes, and flow quantification time 18 [IQR 12-25] minutes. There were 69/177 (39%) studies noted to have intra-thoracic artifacts, most common being susceptibility artifact from intra-thoracic metal. Not all artifacts resulted in non-diagnostic exams. These data describe the use and limitations of CMR for the assessment of cardiac function in a prospective trial setting in a grade-school-aged pediatric population with congenital heart disease. Many of the limitations are expected to decrease with the continued advancement of CMR technology.

The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO2), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO2, work rate at the ventilatory anaerobic threshold (VAT), VO2 at VAT, and ventilatory efficiency (VE/VCO2) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO2, baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation.Trial Registration NCT0274115.

Dyspepsia - The Indian perspective: A cross sectional study on demographics and treatment patterns of Dyspepsia from across India (Power 1.0 study).

BACKGROUND: Dyspepsia includes a spectrum of symptoms ranging from epigastric pain and early satiety to postprandial fullness. The worldwide prevalence of dyspepsia is 20-30%. It is slightly higher in the Western population and occurs more frequently among women. While the precise prevalence of dyspepsia in India is not available, different studies estimate that it affects 7.6-49% of the Indian population. Through our current study, we wanted to understand the demographics, clinical profile, patient presentation, and management in India. We also wanted to document the pattern of use of proton pump inhibitors (PPI) and patient satisfaction with PPIs in Indian patients with dyspepsia. MATERIALS AND METHODS: This pan-India, multi-centric, cross-sectional, questionnaire-based, noninterventional, observational study was conducted between February and October 2021 in patients >18 years of age with a clinical diagnosis of any form of dyspepsia. Descriptive statistics were used for categorical variables, and between-group comparisons were made using Fischer's exact test, with p < 0.05 denoting statistical significance. RESULTS: A total of 3,739 patients from across 29 states of India participated in the study. Most of the patients were male (70.8%) and were from urban areas (56.8%). The highest percentage of patients were aged 31-40 (33.8%), and most patients (60.2%) had dyspepsia for a duration of 6-12 months. Patients with functional dyspepsia (FD) (78.5%) were significantly higher compared to organic dyspepsia (OD) (21.5%) (p < 0.001). The most frequent presenting symptoms were epigastric pain, nausea, vomiting, and heartburn. A quarter (25.6%) of the dyspepsia patients were associated with various comorbid conditions, of which diabetes mellitus, hypertension, and irritable bowel syndrome are the most common ones. A total of 619 patients in the study were on concomitant medications, of which the most common were antidiabetic drugs (271/619, 43.8%). Rabeprazole was the most frequently used PPI (2467/3739, 66.0%) among the study participants. The patient satisfaction analysis showed that, overall, patients were satisfied with PPIs, as most patients (~80%) agreed to almost all questions. The analysis for individual PPIs showed the highest "agree" responses in the rabeprazole group for almost all questions (12 of 13). Around 86.4% of patients on rabeprazole agreed with "immediate relief from acidity," 84.9% for "gives me complete relief," and 85.9% for "relief from nighttime acidity symptoms." CONCLUSION: Our study involving over 3,700 Indian patients with Dyspepsia adds to the growing knowledge of dyspepsia in India. Dyspepsia is more prevalent in males and in the 31-50 age group. FD is the most common form. Overall, patients were satisfied with PPIs in dyspepsia management in India. Patients on rabeprazole showed higher levels of medication adherence, satisfaction with symptom relief, convenience of therapy, and safety compared to patients on other PPIs. Against the backdrop of a paucity of reliable data about dyspepsia in India, our study results provide valuable insights into Dyspepsia and its management in an Indian setting.

Effectiveness of Rabeprazole and Other Proton Pump Inhibitors in Managing GERD with Varying Severity: A Retrospective, Real-world EMR-based Study (POWER GERD Study).

Purpose: To evaluate the effectiveness of rabeprazole and other proton pump inhibitors (PPIs) in providing symptomatic relief in patients with varying severity of gastroesophageal reflux disease (GERD). Methods: In this multicenter retrospective study, electronic medical records (EMRs) of GERD patients prescribed with PPIs at two Indian clinics/hospitals were reviewed (2016-2020). Rabeprazole's effectiveness was assessed at different follow-up visits and compared with other PPIs. Results: Overall, 269 patients (moderate and severe GERD: 84.39%) were included in three groups, viz rabeprazole, pantoprazole, and esomeprazole groups. A significant proportion of patients experienced quick and complete symptomatic relief at visit 1 with rabeprazole compared to the baseline visit, which gradually increased till visit 4 for both daytime [viz heartburn (38.78-93.88%; p < 0.001)] and nocturnal symptoms [viz sleep disturbances (62.92-97.75%; p < 0.001)]. Rabeprazole provided quick relief at visit 1 when compared with pantoprazole for daytime heartburn (38.78 vs 5.56%; p = 0.01), daytime epigastric pain (66.04 vs 12.12%; p = 0.049), and nocturnal water brash (60.71 vs 16.13%; p = 0.015), and when compared with esomeprazole for nocturnal nausea (82.61 vs 20.00%; p = 0.013). Further, the proportion of patients exhibiting complete treatment response was relatively higher in the rabeprazole group (83.33%) than in the pantoprazole (62.07%) and esomeprazole (65.67%) groups at visit 4. Conclusion: Rabeprazole was effective in providing quick and sustained relief for both daytime and nocturnal GERD symptoms in patients with moderate and severe GERD. Rabeprazole also demonstrated greater effectiveness when compared with pantoprazole and esomeprazole in reducing the severity of multiple GERD symptoms. How to cite this article: Lawate P, Jilawar N, Vyas K, et al. Effectiveness of Rabeprazole and Other Proton Pump Inhibitors in Managing GERD with Varying Severity: A Retrospective, Real-world EMR-based Study (POWER GERD Study). J Assoc Physicians India 2023;71(10):37-44.

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation.

BACKGROUND: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. METHODS: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. RESULTS: Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2-9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (- 0.61%/year vs. - 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). CONCLUSIONS: Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.

Expert Opinion on Usage of Pidotimod in Adult Patients with Chronic Obstructive Pulmonary Disease: An Indian Perspective.

India has a disproportionately high burden of acute and chronic pulmonary diseases. In India, 65 million suffer from non-communicable respiratory diseases. The outbreak of the novel coronavirus disease 2019 (COVID-19) had worsened the situation. Patients affected with COVID-19 with a previous history of comorbidities, such as COPD and chronic lung diseases, had the worst prognosis, resulting in adverse outcomes, such as acute respiratory distress syndrome (ARDS) and pneumonia. Immune modulation strategies have since gained a lot of traction amongst practitioners. Modulation of the immune system with Pidotimod along with standard-of-care (SOC) treatment has proven efficacious in the past two decades in patients with recurrent respiratory tract infections (RRTIs), bronchitis, COPD, and pneumonia. In this article, we have reviewed the current unmet needs in the management of COPD in India and evaluated the usage of Pidotimod in adult COPD patients based on expert panel discussion.

Effectiveness of Nimesulide in Acute Fever Management in Adults: Retrospective Electronic Medical Records Database Study Outcome in Outpatient Department.

BACKGROUND: Various clinical trials have established anti-inflammatory and antipyretic properties of Nimesulide in a controlled setting, however, the fever management in real-world settings is quite different. OBJECTIVE: To assess the effectiveness of Nimesulide in acute fever management in real-world clinical practice. METHODOLOGY: A retrospective, multicenter study was conducted on electronic medical records (EMR) of 302 patients visiting out-patient departments at three centers between Jan 2016 and Jan 2020 and were prescribed Nimesulide for acute fever. The effectiveness of Nimesulide was analyzed as a change in fever from baseline to follow-up visit within 14 days and tolerability as the number of side effects captured post-Nimesulide ingestion. RESULTS: The provisional diagnosis at the baseline visit reported major complaints like fever, fever with abdominal pain, body-ache, cough and myalgia. The mean baseline body temperature was 103.2±1.5°F with a mean duration of 4.4±2.8 days significantly (p 0.0001) decreased to 99.7±1.8°F on the administration of Nimesulide. The liver and the renal profiles were found to be normal on records, and the side effects such as nausea and dyspepsia were reported only in 2% of patients. CONCLUSION: Nimesulide was found to be well-tolerated and effective as an antipyretic for acute fever management in adults during short-term use in real-world clinical practice.

Management of Duodeno-Jejunal Flexure Transection after Blunt Trauma - A Modified Approach.

Background: Small bowel injuries are infrequent after blunt trauma and typically affect fixed segment. Untimely management of such injuries, results in high-output entero-cutaneous fistula which increases morbidity and mortality. Treatment of duodeno-jejunal flexure transection has been traditionally done by pyloric exclusion with gastrojejunostomy, but more recent evidence suggests that end-to-end anastomosis or primary closure may be equally effective in which duodeno-jejunal anastomosis is protected via an external tube duodenostomy. Objective: The objective of the study is to provide a modification to the technique of management of duodeno-jejunal flexure injury, avoiding external tube duodenostomy. Material and Methods: Patients admitted from July 1, 2015 to June 1, 2018 were identified and examined for duodeno-jejunal flexure transection. Non-accidental injury cases were excluded. Results: In the study period, a total of 10 patients were admitted with duodeno-jejunal flexure transection. All cases were admitted 24 hours after the injury and presented with shock. After fluid resuscitation and investigations, they were taken for urgent laparotomy. The whole of duodenum was mobilised, the transected ends were debrided and end-to-end duodenojejunal anastomosis was performed in two-layer fashion. An 18-French Nasojejunal (NJ) tube was placed beyond the anastomosis, and an 18-French nasogastric (NG) tube was placed in the stomach for gastric decompression. A feeding jejunostomy was performed in all cases. Both NG and NJ tubes were removed after bowel movements started and FJ was removed on first follow up. There was no incidence of duodenum related complications, and all were doing well on follow up. Discussion and conclusion: Placing the nasojejunal and nasogastric tube eliminates the need for duodenostomy and gastrostomy, respectively. This method protects the duodeno-jejunal anastomosis and decreases the incidence of duodenum-related complications.

Speckle tracking echocardiographically-based analysis of ventricular strain in children: an intervendor comparison.

BACKGROUND: Strain and synchrony can be calculated from a variety of software packages, but there is a paucity of data with inter-vendor comparisons in children. To test the hypothesis that different packages may affect results, independent of acquisition, we compared values obtained using two commercially available analysis tool (QLAB and TomTec), with several different settings. METHODS: The study population included 108 children; patients were divided into three groups: (1) normal cardiac structure and conduction; (2) ventricular paced rhythm; and (3) flattened ventricular septum (reflecting right ventricular pressure or volume load lesions). We analyzed the same image acquired from the apical 4-chamber (AP4) and short-axis at the mid-papillary level (SAXM) views in both QLAB (versions 10.5 and 10.8) and TomTec (version 1.2). In QLAB version 10.8, low, medium, and high quantification smoothness settings were employed. In TomTec, images were analyzed with both low and high frame rates. Tracking quality for each package was graded. AP4 and SAXM strain and synchrony values were recorded. A mixed-effects linear regression model was used, with main effect considered significant if the p-value was < 0.05. RESULTS: Tracking scores were high for all packages except QLAB 10.5 in the SAXM view. AP4 and SAXM strain values varied significantly between QLAB 10.5 and the other packages. Synchrony values varied widely for all strain values (p < 0.001 for both) in all packages. Quantification smoothness changes in QLAB 10.8 did not impact strain significantly in any patient group; temporal resolution changes in TomTec resulted in strain differences in children with flat ventricular septums, but not those with normal or ventricular paced hearts. CONCLUSION: Synchrony values varied substantially among all packages in children. Strain values varied widely between QLAB 10.5 and all other software packages, recommending avoidance of QLAB 10.5 for future studies. Quantification smoothness settings in QLAB 10.8 resulted in minimal strain differences. In TomTec, low and high frame rate strain values differed only in a subset of patients (flattened septum). These data suggest that reliable comparisons between strain values derived from QLAB and TomTec is possible in certain cases, but that caution should be used especially in different hemodynamics conditions.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity.

BACKGROUND: Maldistribution of pulmonary artery blood flow (MPBF) is a potential complication in patients who have undergone single ventricle palliation culminating in the Fontan procedure. Cardiovascular magnetic resonance (CMR) is the best modality that can evaluate MPBF in this population. The purpose of this study is to identify the prevalence and associations of MPBF and to determine the impact of MPBF on exercise capacity after the Fontan operation. METHODS: This retrospective single-center study included all patients after Fontan operation who had maximal cardiopulmonary exercise test (CPET) and CMR with flow measurements of the branch pulmonary arteries. MPBF was defined as > 20% difference in branch pulmonary artery flow. Exercise capacity was measured as percent of predicted oxygen consumption at peak exercise (% predicted VO2). Linear and logistic regression models were used to determine univariate and multivariable predictors of exercise capacity and correlates of MPBF, respectively. RESULTS: A total of 147 patients who had CMR between 1999 and 2017 were included (median age at CMR 21.8 years [interquartile range (IQR) 16.5-30.6]) and the median time between CMR and CPET was 2.8 months [IQR 0-13.8]. Fifty-three patients (36%) had MPBF (95% CI 29-45%). The mean % predicted VO2 was 63 ± 16%. Patients with MPBF had lower mean % predicted VO2 compared to patients without MPBF (60 ± 14% versus 65 ± 16%, p = 0.04). On multivariable analysis, a lower % predicted VO2 was independently associated with longer time since Fontan, higher ventricular mass-to-volume ratio, and MPBF. On multivariable analysis, only compression of the branch pulmonary arteries by the ascending aorta or aortic root was associated with MPBF (OR 6.5, 95% CI 5.6-7.4, p < 0.001). CONCLUSION: In patients after the Fontan operation, MPBF is common and is independently associated with lower exercise capacity. MPBF was most likely to be caused by pulmonary artery compression by the aortic root or the ascending aorta. This study identifies MPBF as an important risk factor and as a potential target for therapeutic interventions in this fragile patient population.

Inefficient Ventriculoarterial Coupling in Fontan Patients: A Cardiac Magnetic Resonance Study.

The ventriculoarterial coupling (VAC) ratio, the ratio of arterial elastance (Ea) to ventricular end-systolic elastance (Ees), reflects cardiovascular efficiency. Little is known about this ratio in patients who have undergone the Fontan procedure. Our aim was to assess the VAC ratio in a cohort of Fontan patients using a cardiac magnetic resonance (CMR) method, and to examine its relation to outcomes. We retrospectively assessed VAC from CMR data on 195 Fontan patients (age 19.6 ± 10.7 years) and 42 controls (age 15.2 ± 2.2 years). The VAC ratio was calculated as Ea/Ees (Ea = mean arterial blood pressure (MBP)/ventricular stroke volume; Ees = MBP/end-systolic volume). Compared with controls, Fontan patients had lower body surface area-adjusted median Ees (1.54 vs. 2.4, p < 0.001) and Ea (1.35 vs. 1.48, p = 0.01), and a higher median VAC ratio (0.88 vs. 0.62, p < 0.001). After a median follow-up of 4 years (range 1-10), 20 patients reached a composite endpoint of death or heart transplant listing. On multivariable modeling, being in the lowest tertile of the VAC ratio was independently associated with the composite endpoint (odds ratio 11.39, p = 0.02), and inclusion of the VAC ratio in the model improved prediction compared to traditional risk factors. In patients without ventricular dilation, the VAC ratio was the only factor predictive of the composite endpoint (p = 0.02). In conclusion, we found evidence for inefficient ventriculoarterial coupling in Fontan patients. The VAC ratio improved prediction of outcomes and was especially useful in patients without ventricular dilation. Further investigation into the clinical significance of ventriculoarterial coupling in this patient population is warranted.

Antioxidant activity and polyphenolic compound stability of lentil-orange peel powder blend in an extrusion process.

Lentil contains substantial amount of protein, carbohydrate, fibre and other nutrients and orange peels powder rich in carbohydrate and fiber content The present study was aimed to investigate the effects of extrusion processing parameter on the level of total phenolic content (TPC), total flavonoid content (TFC), total tannin content and antioxidant activity of lentil-orange peel powder blend, also to investigate the possibility of blend as a candidate for production of protein rich extruded product by using response surface methodology. It was observed that, the physicochemical properties and sensory characteristics of lentil-orange peel based extrudate were highly dependent on process variables. The blend of lentil and orange peel powder has a huge potential for extrusion to produce ready-to-eat extruded with good acceptance. The overall best quality product was optimized and obtained at 16% moisture, 150 °C die temperature and 200 rpm screw speed. Extrusion process increased nutritional value of extruded product with TPC and TFC of 70.4 and 67.62% respectively and antioxidant activity of 60.6%. It showed higher stability at 150 °C with intermediate feed moisture content and despite the use of high temperatures in the extrusion-cooking is possible to minimize the loss of bioactive compounds to achieve products. Thus, results indicated that blend of lentil and orange peel may be used as raw material for the production of extruded snacks with great nutritional value.

Myocardial Fibrosis in Congenital Heart Disease.

Myocardial fibrosis is common in patients with congenital heart disease (CHD) and has been associated with arrhythmias, decreased functional status, and adverse ventricular mechanics. There are multiple types of myocardial fibrosis that occur in response to different pathophysiologic stimuli. Recent advances in imaging technology have made detection and quantification of the types of myocardial fibrosis possible. In this review, we describe the pathophysiology of myocardial fibrosis, examine the imaging techniques used to evaluate fibrosis, and discuss the relationship between myocardial fibrosis and clinical outcomes in CHD. (Circ J 2016; 80: 1300-1307).

Left Ventricular Dysfunction Following Neonatal Pulmonary Valve Balloon Dilation for Pulmonary Atresia or Critical Pulmonary Stenosis.

Pulmonary valve (PV) balloon dilation (BD) is the primary therapy for infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS). We observed left ventricular (LV) dysfunction in patients following BD and sought to determine its incidence, clinical course and associated risk factors. Clinical, echocardiographic and catheterization data for all patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS between January 2000 and February 2014 were retrospectively analyzed (n = 129). Post-procedure LV dysfunction was defined as ejection fraction (EF) <54 %. Median age at PV BD was 1 day. Most (71 %) patients had critical PS. Median PV diameter pre-BD was 6.0 mm with PV z-scores -4.1 to 0.9, median LV EF pre-BD was 58 %. Post-BD LV dysfunction developed in 45 patients (35 %); 15 patients had LV EF ≤40 %. Median time to normalization of LV EF was 10 days (range 2-72). In univariate analysis, diagnosis (critical PS or PAIVS), right ventricle to LV pressure ratio pre-BD, acute procedural complication and post-BD inotropic support were not associated with post-BD LV dysfunction. In multivariable analysis, the predictors of post-procedure LV dysfunction were lower PV z-score (OR 1.81, p 0.04), tricuspid regurgitation pre-BD ≥ moderate (OR 3.73, p 0.008) and larger right ventricular apical area (OR 1.99, p 0.04). LV dysfunction post-neonatal PV BD develops in a significant number of patients (35 %) and can be severe, but resolves. The risk of developing LV dysfunction post-PV BD is highest in patients with larger right ventricles.

Transposition of the great arteries and sinus venosus defect with partially anomalous pulmonary venous return: physiological and anatomic considerations.

Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.