scholarly journals Right and left ventricle native T1 mapping in systolic phase in patients with congenital heart disease

2020 ◽  
pp. 028418512092456 ◽  
Author(s):  
Francesco Secchi ◽  
Marco Alì ◽  
Caterina B Monti ◽  
Andreas Greiser ◽  
Francesca R Pluchinotta ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Ejection Fraction ◽  
Negative Correlation ◽  
Congenital Heart ◽  
T1 Mapping ◽  
Diffuse Fibrosis ◽  
Native T1 ◽  
Systolic Phase

Background T1 mapping is emerging as a powerful tool in cardiac magnetic resonance (CMR) to evaluate diffuse fibrosis. However, right ventricular (RV) T1 mapping proves difficult due to the limited wall thickness in diastolic phase. Several studies focused on systolic T1 mapping, albeit only on the left ventricle (LV). Purpose To estimate intra- and inter-observer variability of native T1 (nT1) mapping of the RV, and its correlations with biventricular and pulmonary function in patients with congenital heart disease (CHD). Material and Methods In this retrospective, observational, cross-sectional study we evaluated 36 patients with CHD, having undergone CMR on a 1.5-T scanner. LV and RV functional evaluations were performed. A native modified look-locker inversion recovery short-axis sequence was acquired in the systolic phase. Intra- and inter-reader reproducibility were reported as complement to 100% of the ratio between coefficient of reproducibility and mean. Spearman ρ and Mann–Whitney U-test were used to compare distributions. Results Intra- and inter-reader reproducibility was 84% and 82%, respectively. Median nT1 was 1022 ms (interquartile range [IQR] 1108–972) for the RV and 947 ms (IQR 986–914) for the LV. Median RV–nT1 was 1016 ms (IQR 1090–1016) in patients with EDVI ≤100 mL/m2 and 1100 ms (IQR 1113–1100) in patients with EDVI >100 mL/m2 ( P = 0.049). A significant negative correlation was found between RV ejection fraction and RV–nT1 (ρ = −0.284, P = 0.046). Conclusion Systolic RV-nT1 showed a high reproducibility and a negative correlation with RV ejection fraction, potentially reflecting an adaptation of the RV myocardium to pulmonary valve/conduit (dys)-function.

Thrombin generation in paediatric patients with congenital heart disease

2008 ◽  
Vol 28 (S 01) ◽  
pp. S61-S66 ◽  
Author(s):  
G. Cvirn ◽  
A. Rosenkranz ◽  
B. Leschnik ◽  
W. Raith ◽  
W. Muntean ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Negative Correlation ◽  
Congenital Heart ◽  
Thrombin Generation ◽  
Significant Negative Correlation ◽  
Peak Height ◽  
Global Test ◽  
Paediatric Patients ◽  
Age Range

SummaryThrombin generation was studied in paediatric patients with congenital heart disease (CHD) undergoing cardiac surgery using the calibrated automated thrombography (CAT) in terms of the lag time until the onset of thrombin formation, time to thrombin peak maximum (TTP), endogenous thrombin potential (ETP), and thrombin peak height. The suitability to determine the coagulation status of these patients was investigated. Patients, material, methods: CAT data of 40 patients with CHD (age range from newborn to 18 years) were compared to data using standard coagulation parameters such as prothrombin (FII), antithrombin (AT), tissue factor pathway inhibitor (TFPI), prothrombin fragment 1.2 (F 1.2), thrombin-antithrombin (TAT), activated partial thromboplastin time (aPTT), and prothrombin time (PT). Results: A significant positive correlation was seen between ETP and FII (p < 0.01; r = 0.369), as well as between peak height and F II (p < 0.01; r = 0.483). A significant negative correlation was seen between ETP and TFPI values (p < 0.05; r = –0.225) while no significant correlation was seen between peak height and TFPI. A significant negative correlation was seen between F 1.2 generation and ETP (p < 0.05; r = –0.254) and between F 1.2 generation and peak height (p < 0.05; r = –0.236). No correlation was seen between AT and ETP or peak. Conclusions: CAT is a good global test reflecting procoagulatory and inhibitory factors of the haemostatic system in paediatric patients with CHD.

Double-chambered left ventricle with complex congenital heart disease in an infant: an extremely rare pathology

2019 ◽  
Vol 16 (3) ◽  
pp. 187-191
Author(s):  
T.V. Rogova ◽  
A.I. Kim ◽  
A.V. Sobolev ◽  
S.A. Aleksandrova ◽  
E.V. Kholmanskaya ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Complex Congenital Heart Disease

scholarly journals Evolution of biventricular T1 values in patients with right-sided congenital heart disease

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
O Burdet ◽  
AG Pavon ◽  
J Bouchardy ◽  
C Blanche ◽  
P Monney ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Interstitial Fibrosis ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Native T1 ◽  
Funding Sources ◽  
Basal Slice ◽  
Relationship Of

Abstract Funding Acknowledgements Type of funding sources: None. Background Conflicting reports exist on the prevalence and clinical impact of interstitial fibrosis in right ventricular (RV) congenital heart disease (CHD). This study evaluates the longitudinal evolution of native myocardial T1 relaxation time (T1) in RV CHD. Methods On a 1.5T scanner, an ECG-triggered modified Look-Locker inversion recovery sequence (scheme 3(3)3(3)5) was acquired on a short-axis basal slice covering the RV and left ventricle (LV) on two consecutive CMR exams. Global and segmental (LV = 6, RV = 4) RV and LV T1 values  were calculated (Figure). Results Mean time between CMR exams for 36 included patients (age 34 ± 2y) was 22 ± 2 months. All LV segments and 81/88% of RV segments of first and second CMR could be analyzed, respectively.  T1 increased mildly but not significantly (table). There was no relationship of T1 to pulmonary regurgitation fraction, pulmonary stenosis or RV enddiastolic volume (p &gt; 0.05). Global RV T1 of the second CMR was related to RV ejection fraction (RVEF): r = 0.353, 3.0 ± 1.4, p = 0.038. T1 of the infero-septal LV segment of first and second CMR, global LV T1 of second CMR and increase of T1 of global LV, anterior, antero-lateral and –septal LV segments, were related to age at CMR: r = 0.333 - 0.463, p &lt; 0.05, respectively. Conclusions Native T1 values increased mildly in patients with stable RV CHD, which was not statistically significant probably due to the short to median follow-up. Global RV T1 appears to be related to RVEF which could be sign of increasing interstitial fibrosis whereas the relationship of LV T1 to age might be a physiological finding. First CMR native T1 (ms) Second CMR native T1 (ms) p LV Global 1007 ± 37 1014 ± 39 0.413 LV Anterior 994 ± 53 999 ± 54 0.710 LV Antero-lateral 965 ± 63 981 ± 58 0.186 LV Infero-lateral 1000 ± 52 1004 ± 63 0.695 LV Inferior 1035 42 1037 ± 50 0.744 LV Infero-septal 1028 ± 35 1036 ± 43 0.282 LV Antero-septal 1016 ± 38 1024 ± 48 0.347 RV Global 1091 ± 90 1096 ± 85 0.410 RV Inferior 1112 ± 104 1115 ± 118 0.696 RV Infero-lateral 1061 ± 130 1077 ± 115 0.425 RV Antero-lateral 1046 ± 127 1080 ± 109 0.088 RV Anterior 1088 ± 156 1108 ± 154 0.410 Abstract Figure. Determination of biventricular T1 values

scholarly journals Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

2017 ◽  
Vol 5 (1) ◽  
pp. 53-56
Author(s):  
Rahul Regi Abraham ◽  
Rahul Regi Abraham
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Left Ventricle ◽  
Congenital Heart ◽  
Pulmonary Stenosis ◽  
Eisenmenger Syndrome ◽  
Tertiary Center ◽  
History Of ◽  
Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

Quality of life following paediatric heart transplant: are age and activity level factors?

2014 ◽  
Vol 25 (3) ◽  
pp. 476-480 ◽  
Author(s):  
John J. Parent ◽  
Lauren Sterrett ◽  
Randall Caldwell ◽  
Robert Darragh ◽  
Marcus Schamberger ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Heart Transplantation ◽  
Negative Correlation ◽  
Congenital Heart ◽  
Activity Level ◽  
Cognitive Limitations ◽  
School Function

AbstractBackground: We evaluated whether quality of life correlates to age and activity in children following heart transplantation. In addition, quality of life in children following heart transplantation was compared with previously reported values in children with congenital heart disease. Quality of life remains an important aspect of therapy. Methods: The Pediatric Quality of Life Inventory Generic Core Scales and Cardiac Module were administered to 14 children who had previously undergone heart transplantation. Patients wore a pedometer for 7 days to assess daily activity. Results: The age at assessment was 13.1±1.9 years. The patients were 7.1±5.7 years post heart transplantation. There was a negative correlation between age at first heart transplantation and emotional (r=−0.64; p<0.05) and school function (r=−0.57; p<0.05). A negative correlation between patient’s age at assessment and perceived physical appearance existed (r=−0.53; p<0.05). Daily steps negatively correlated with cognitive (r=−0.58; p<0.05), physical (r=−0.63; p<0.05), emotional (r=−0.62; p<0.05), and school function (r=−0.66; p<0.01). Heart transplantation patients reported better scores for treatment and symptoms (p<0.05) but lower physical health scores (p<0.01) than those with moderate congenital heart disease. Conclusions: Paediatric heart transplantation patients reported overall similar quality of life as patients with moderate congenital heart disease. Children receiving heart transplants at an older age may require additional emotional and educational support. Heart transplantation patients with higher activity levels may be more aware of their physical, emotional, and cognitive limitations, and thus score lower on these quality of life indicators.

scholarly journals Myocardial T1 Mapping in Pediatric and Congenital Heart Disease

2015 ◽  
Vol 8 (2) ◽  
Author(s):  
Eugénie Riesenkampff ◽  
Daniel R. Messroghli ◽  
Andrew N. Redington ◽  
Lars Grosse-Wortmann
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
T1 Mapping ◽  
Myocardial T1

Abstract 2974: Midterm Results Of Resynchronization Therapy In Pediatrics: Do We Need New Guidelines?

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Anne M Dubin ◽  
Kathryn K Collins ◽  
George F Van Hare ◽  
V. M Reddy ◽  
Debra Hanisch ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ejection Fraction ◽  
Dilated Cardiomyopathy ◽  
Heart Block ◽  
Congenital Heart ◽  
Complete Heart Block ◽  
Resynchronization Therapy ◽  
Congenital Complete Heart Block

Cardiac resynchronization therapy (CRT) is an accepted therapy for adults with dilated cardiomyopathy (DCM) and has been shown to improve ejection fraction and functional capacity. CRT has been used in children for over 4 years with a variety of indications which differ substantially from those commonly used for adult patients. We reviewed the results of CRT in the combined Stanford-UCSF pediatric electrophysiology program over the past 4 years, in order to evaluate efficacy of CRT in different clinical scenarios. We have provided CRT to 31 patients (15 female) between 2003 and 2007. The mean age at implantation was 7.3 ± 6.6 years. 21 patients had congenital heart disease (CHD) of whom 4 had single ventricle physiology. 5 patients had DCM and 5 had congenital complete heart block with reduced LV systolic function (CCHB). All patients with DCM met criteria for CRT in adults (QRS duration < 120 msec and EF > 35%). Implantation in the other patient groups was performed at the discretion of the physician. Mean follow-up was 26 ± 15 months. Ejection Fraction (EF), by echo, was 30 ± 11% at baseline which improved to 41 ± 19% at last follow-up (p < 0.05). EF as well as LV end diastolic volume and LV end systolic volume showed immediate improvement followed by additional continued improvement over the study period. CHD and CCHB patients reported improved symptoms and had improved cardiac indices by echo. DCM patients, however, showed no improvement and instead had a gradual decrease in ejection fraction over the study period (30 ± 11% to 18 ± 11%, p< 0.01). Two of the 5 DCM patients were transplanted, and one died of pump failure. Conclusions: CRT is efficacious in selected patients with congenital heart disease, or those with congenital complete heart block. The role of CRT in pediatric patients with dilated cardiomyopathy is less clear, but adult selection criteria may not be applicable to children. Comparison of EF preCRT and at last follow-up

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