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7. The Cardiac Genome Clinic: implementing genome sequencing in pediatric heart disease

Author

Reuter, Miriam S., Chaturvedi, Rajiv R., Liston, Eriskay, Manshaei, Roozbeh, Aul, Ritu B., Bowdin, Sarah, Cohn, Iris, Curtis, Meredith, Dhir, Priya, Hayeems, Robin Z., Hosseini, S. Mohsen, Khan, Reem, Ly, Linh G., Marshall, Christian R., Mertens, Luc, Okello, John B.A., Pereira, Sergio L., Raajkumar, Akshaya, Seed, Mike, Thiruvahindrapuram, Bhooma, Scherer, Stephen W., Kim, Raymond H., and Jobling, Rebekah K.

Published
2020
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8. Haploinsufficiency of vascular endothelial growth factor related signaling genes is associated with tetralogy of Fallot

Author

Reuter, Miriam S., Jobling, Rebekah, Chaturvedi, Rajiv R., Manshaei, Roozbeh, Costain, Gregory, Heung, Tracy, Curtis, Meredith, Hosseini, S. Mohsen, Liston, Eriskay, Lowther, Chelsea, Oechslin, Erwin, Sticht, Heinrich, Thiruvahindrapuram, Bhooma, Mil, Spencer van, Wald, Rachel M., Walker, Susan, Marshall, Christian R., Silversides, Candice K., Scherer, Stephen W., Kim, Raymond H., and Bassett, Anne S.

Published
2019
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14. MRI Phase-Contrast Blood Flow in Fasting Pediatric Patients with Fontan Circulation Correlates with Exercise Capacity

Author

Lam, Christopher Z., primary, David, Dawn, additional, Acosta Izquierdo, Laura, additional, Pezeshkpour, Parneyan, additional, Dipchand, Anne I., additional, Jean-St-Michel, Emilie, additional, Chaturvedi, Rajiv R., additional, Ling, Simon C., additional, Wald, Rachel M., additional, Chavhan, Govind B., additional, Seed, Michael, additional, and Yoo, Shi-Joon, additional

Published
2022
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17. Clinical Genetic Risk Variants Inform a Functional Protein Interaction Network for Tetralogy of Fallot

Author

Reuter, Miriam S., primary, Chaturvedi, Rajiv R., additional, Jobling, Rebekah K., additional, Pellecchia, Giovanna, additional, Hamdan, Omar, additional, Sung, Wilson W.L., additional, Nalpathamkalam, Thomas, additional, Attaluri, Pratyusha, additional, Silversides, Candice K., additional, Wald, Rachel M., additional, Marshall, Christian R., additional, Williams, Simon G., additional, Keavney, Bernard D., additional, Thiruvahindrapuram, Bhooma, additional, Scherer, Stephen W., additional, and Bassett, Anne S., additional

Published
2021
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25. BEST ORAL ABSTRACTS1575Extracellular volume associates with outcomes more strongly than native or post-contrast myocardial T11507Cardiac Magnetic Resonance measured Extracellular Volume Independently Predicts Adverse Outcome in Heart Failure with Preserved Ejection Fraction1457Cardiac MRI Under Percussive Ventilation: A New Promising Technique1644Histological Validation of Cardiac Magnetic Resonance for the Evaluation of Myocardial Fibrosis after Heart Transplantation in Children1493First Pass Perfusion Reserve Index in Paediatric Patients with Arterial Switch for Transposition of Great Arteries1652Myocardial Fibrosis is Prevalent in Obstructive Sleep Apnea and Associated with Hospitalization for Heart Failure or Death

Author

Treibel, Thomas A., primary, Duca, Franz, primary, Schwitter, Juerg, primary, Ide, Seiko, primary, Sandrini, C., primary, Fridman, Yaron, primary, Fridman, Yaron, additional, Hackman, Brianne, additional, Kadakkal, Ajay, additional, Sayeed, Aatif, additional, Maanja, Maren, additional, Daya, Hussein Abu, additional, Moon, James C., additional, Wong, Timothy C., additional, Schelbert, Erik B., additional, Kammerlander, Andreas A., additional, Zotter-Tufaro, Caroline, additional, Aschauer, Stefan, additional, Bonderman, Diana, additional, Mascherbauer, Julia, additional, Beigelman-Aubry, Catherine, additional, Peguret, Nicolas, additional, Stuber, Matthias, additional, Delacoste, Jean, additional, Belmondo, Bastien, additional, Lovis, Alban, additional, Simons, Julien, additional, Long, Olivier, additional, Grant, Kathleen, additional, Berchier, Gregoire, additional, Rohner, Chantal, additional, Bonanno, Gabriele, additional, Coppo, Simone, additional, Ozsahin, Esat-Mahmut, additional, Qanadli, Salah, additional, Meuli, Reto, additional, Bourhis, Jean, additional, Riesenkampff, Eugenie, additional, Chiasson, David, additional, Dipchand, Anne I., additional, Kantor, Paul F., additional, Chaturvedi, Rajiv R., additional, Yoo, Shi-Joon, additional, Grosse-Wortmann, Lars, additional, Aquaro, GD, additional, De Marchi, D, additional, Ait Ali, L, additional, Khraiche, D, additional, Boddaert, N, additional, Bonnet, D, additional, Raimondi, F, additional, and Hackman, Brianne E., additional

Published
2016
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29. Surgical Palliation Strategy Does Not Affect Interstage Ventricular Dysfunction or Atrioventricular Valve Regurgitation in Children With Hypoplastic Left Heart Syndrome and Variants

Author

Chetan, Devin, primary, Kotani, Yasuhiro, additional, Jacques, Frederic, additional, Poynter, Jeffrey A., additional, Benson, Lee N., additional, Lee, Kyong-Jin, additional, Chaturvedi, Rajiv R., additional, Friedberg, Mark K., additional, Van Arsdell, Glen S., additional, Caldarone, Christopher A., additional, and Honjo, Osami, additional

Published
2013
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30. Histological validation of cardiovascular magnetic resonance T1 mapping markers of myocardial fibrosis in paediatric heart transplant recipients.

Author

Seiko Ide, Riesenkampff, Eugenie, Chiasson, David A., Dipchand, Anne I., Kantor, Paul F., Chaturvedi, Rajiv R., Shi-Joon Yoo, and Grosse-Wortmann, Lars

Subjects
MYOCARDIUM, STATISTICAL correlation, HEART transplantation, HISTOLOGICAL techniques, MAGNETIC resonance imaging, RESEARCH methodology, PHOTOGRAPHY, STATISTICAL hypothesis testing, STATISTICS, T-test (Statistics), DATA analysis, CONTROL groups, INTER-observer reliability, DATA analysis software, DESCRIPTIVE statistics, CORONARY angiography, MANN Whitney U Test, WOUNDS & injuries
Abstract

Background: Adverse fibrotic remodeling is detrimental to myocardial health and a reliable method for monitoring the development of fibrotic remodeling may be desirable during the follow-up of patients after heart transplantation (HTx). Quantification of diffuse myocardial fibrosis with cardiovascular magnetic resonance (CMR) has been increasingly applied and validated histologically in adult patients with heart disease. However, comparisons of CMR findings with histological fibrosis burden in children are lacking. This study aimed to compare native T1 times and extracellular volume fraction (ECV) derived from CMR with the degree of collagen on endomyocardial biopsy (EmBx), and to investigate the association between myocardial fibrosis and clinical as well as functional markers in children after HTx. Methods: EmBx and CMR were performed on the same day. All specimens were stained with picrosirius red. The collagen volume fraction (CVF) was calculated as ratio of stained collagen area to total myocardial area on EmBx. Native T1 values and ECV were measured by CMR on a mid-ventricular short axis slice, using a modified look-locker inversion recovery approach. Results: Twenty patients (9.9 ± 6.2 years of age; 9 girls) after HTx were prospectively enrolled, at a median of 1.3 years (0.02-12.6 years) post HTx, and compared to 24 controls (13.9 ± 2.6 years of age; 12 girls). The mean histological CVF was 10.0 ± 3.4%. Septal native T1 times and ECV were higher in HTx patients compared to controls (1008 ± 32 ms vs 979 ± 24 ms, p < 0.005 and 0.30 ± 0.03 vs 0.22 ± 0.03, p < 0.0001, respectively). CVF showed a moderate correlation with native T1 (r = 0.53, p < 0.05) as well as ECV (r = 0.46, p < 0.05). Native T1 time, but not ECV and CVF, correlated with ischemia time (r = 0.46, p < 0.05). Conclusions: CMR-derived fibrosis markers correlate with histological degree of fibrosis on EmBx in children after HTx. Further, native T1 times are associated with longer ischemia times. [ABSTRACT FROM AUTHOR]

Published
2017
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32. Hybrid Versus Norwood Strategies for Single-Ventricle Palliation

Author

Baba, Kenji, primary, Kotani, Yasuhiro, additional, Chetan, Devin, additional, Chaturvedi, Rajiv R., additional, Lee, Kyong-Jin, additional, Benson, Lee N., additional, Grosse-Wortmann, Lars, additional, Van Arsdell, Glen S., additional, Caldarone, Christopher A., additional, and Honjo, Osami, additional

Published
2012
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42. A simple and fast technique for radiofrequency-assisted perforation of the atrial septum in congenital heart disease.

Author

Sandoval, Juan Pablo and Chaturvedi, Rajiv R.

Subjects
*TRANSPOSITION of great vessels, *HYPOPLASTIC left heart syndrome, *CONGENITAL heart disease, *ATRIAL septum, *CATHETERS
Abstract

Radiofrequency (RF) assisted perforation of the atrial septum was performed successfully in three infants using a 0.035″ RF wire deployed through a Williams right posterior catheter. Balloon atrial septoplasty was performed over the 0.035″ RF wire in two of them, shortening the procedural time. [ABSTRACT FROM AUTHOR]

Published
2016
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43. Abstract 15729: A Severe Sustained Decrease in Left Heart Flow Results in Left Ventricular Hypoplasia in Mid-Gestation Fetal Lambs

Author

Chaturvedi, Rajiv R, Keunen, Johannes, de Vrijer, Barbra, Ryan, Greg, and Jaeggi, Edgar T

Abstract

Introduction:The etiology of hypoplastic left heart syndrome (HLHS) is unclear. Supravalvar aortic banding in fetal lambs produced conflicting results as to whether LV hypoplasia or hypertrophy occurred. We hypothesized low flow in the fetal left heart results in hypoplasiaMethods:LV inflow obstruction created in 75day fetal lambs by percutaneous implantation of LA coils and harvest at 125 days.Results:19 coiled fetuses and 1 control died prior to harvest. 15 coiled fetuses reached 125 days, 1 had anasarca (10.6kg) due to severe mitral regurgitation. Effective coiling resulted in tissue growth into the coil scaffold leading to severe acquired mitral stenosis and a slit-like LV. LV inflow and antegrade aortic valve (AoV) flow was so low in 9 that there was retrograde flow in the ascending aorta from the duct. 4 of 9 had no antegrade AoV flow, with the brain and coronaries wholly perfused from the duct. These low-flow LV had the most severe left heart hypoplasia and the LV was no longer apex forming. Due to the variation in fetal size (non-hydropic weight 1.74-4.45kg), each fetus?s left heart measurements were normalized to the corresponding right-heart structure and these were all smaller in the low-flow LV hearts as compared to controls (Table 1).Conclusion:Long after cardiogenesis, a severe, prolonged (1/3 gestation) decrease in left heart flow is sufficient to produce LV and ascending aortic hypoplasia and retrograde perfusion of the brain and coronaries, similar to human HLHS. This model will allow investigation of HLHS development and therapies.graphic_{3CF29FE0-3EAF-40EA-AAA1-AE98366853C0}

Published
2019
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