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1.
Circ Cardiovasc Imaging ; : CIRCIMAGING120011136, 2021 Mar 16.
Artigo em Inglês | MEDLINE | ID: mdl-33722068

RESUMO

BACKGROUND: Left ventricular dysfunction in Ebstein's anomaly (EA) is associated with higher mortality. The health of the left ventricular myocardium in children and adolescents with EA has not been investigated in detail. METHODS: Patients with unrepaired EA who had undergone cardiac magnetic resonance imaging including T1 mapping were retrospectively reviewed. Patients were compared with age- and sex-matched controls. EA severity index was calculated using volumetric measurements at end diastole ([right atrial+atrialized right ventricular volumes]/[functional right ventricular+left atrial+left ventricular volumes]). Global circumferential and radial strain and as well as strain rate were examined using cardiac magnetic resonance feature tracking. RESULTS: Twelve EA patients and an equal number of controls were included. Functional and atrialized right ventricular end-diastolic volumes were 84±15 and 21±13 mL/m2, respectively. Late gadolinium enhancement, confined to the right ventricle, was found in 2 patients (16%). Left ventricular native T1 values and extracellular volume fractions were higher in patients compared with controls (1026±47 versus 956±40 ms, P=0.0004 and 28.5±3.4% versus 22.5±2.6%, P<0.001, respectively). Native T1 times correlated inversely with patients' age, body surface area, and O2 saturations (r=-0.63, -0.62, and -0.91, respectively; P=0.02, P=0.02, and P<0.0001, respectively). EA severity index ranged between 0.15 and 0.94 and correlated with T1 values (r=0.76, P=0.003). Native T1 correlated with global circumferential strain (r=0.58, P=0.04) but not ejection fraction (EF). EA patients had reduced maximum oxygen uptake (Vo2max). Vo2max correlated inversely with T1 values (r=-0.79, P=0.01). CONCLUSIONS: Children and adolescents with EA experience an abnormal degree of diffuse myocardial fibrosis. Its association with O2 saturation points toward a role of hypoxemia in the pathogenesis of fibrosis. Larger and prospective studies are needed to evaluate the value of T1 mapping for risk stratification and monitoring in EA.

2.
J Cardiovasc Magn Reson ; 23(1): 28, 2021 Mar 18.
Artigo em Inglês | MEDLINE | ID: mdl-33731161

RESUMO

BACKGROUND: Cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) requires identification of the normal myocardial nulling time using inversion time (TI)-scout imaging sequence. Although TI-scout images are not primarily used for myocardial assessment, they provide information regarding different signal recovery patterns of normal and abnormal myocardium facilitating identification of LGE in instances where standard LGE images alone are not diagnostic. We aimed to assess the diagnostic performance of TI-scout as compared to that of standard LGE images. METHODS: CMR studies with LGE imaging in 519 patients (345 males, 1-17 years) were reviewed to assess the diagnostic performance of LGE imaging in terms of the location of LGE and the pathologic entities. The diagnostic performance of the TI-scout and standard LGE imaging was classified into four categories: (1) equally diagnostic, (2) TI-scout superior to standard LGE, (3) standard LGE superior to TI-scout, and (4) complementary, by the consensus of the two observers. RESULTS: The study cohort consisted of 440 patients with negative LGE and 79 with evidence for LGE. For a negative diagnosis of LGE, TI-scout and standard LGE images were equally diagnostic in 75% of the cases and were complementary in 12%. For patients with LGE, TI-scout images were superior to standard LGE images in 52% of the cases and were complementary in 19%. The diagnostic performance of TI-scout images was superior to that of standard LGE images in all locations. TI-scout images were superior to standard LGE images in 11 of 12 (92%) cases with LGE involving the papillary muscles, in 7 /12 (58%) cases with subendocardial LGE, and in 4/7 (57%) cases with transmural LGE. TI-scout images were particularly useful assessing the presence and extent of LGE in hypertrophic cardiomyopathy (HCM). TI-scout was superior to standard LGE in 6/10 (60%) and was complementary in 3/10 (30%) of the positive cases with HCM. CONCLUSIONS: TI-scout images enhance the diagnostic performance of LGE imaging in children.

4.
Ann Thorac Surg ; 2020 Dec 09.
Artigo em Inglês | MEDLINE | ID: mdl-33307072

RESUMO

BACKGROUND: The monthly In-House Hands-On Surgical Training (HOST) program was incorporated into the congenital heart surgery(CHS) curriculum for surgical trainees within our institution. This study evaluated whether there was an improvement and retention of technical skills throughout the curriculum via objective assessment methods. METHODS: Twelve 3D-printed surgical heart models were included into the year-long curriculum. The monthly sessions were attended by all trainees and staff surgeons. Proctors demonstrated the operation on a model, which was followed by two attempts by each trainee. Attempts were recorded for objective assessment. On completion of the curriculum trainees repeated four procedures an additional two times following a delay to assess skill retention. RESULTS: Twelve sessions were completed by 7 trainees within the curriculum. Objective assessments were performed in 7 sessions. Eighty-one percent of trainees' scores improved between the two attempts with a mean improvement of 13%(Attempt 1[HOST-CHS:79],Attempt 2[HOST-CHS:89],p=<0.0001). Similarly,91% of procedural times improved by a mean of 25%(Attempt 1,1:22:00[h:mm:ss],Attempt 2,1:01:21[h:mm:ss],p=<0.0001]. During individual procedure analysis, statistical significance remained in 3-of-7 procedures[p=<0.05].Four procedures were assessed for skill retention following a delay(2-14months). Scores decreased by 4% in 47% of trainees during Attempt 3(Attempt 2[HOST-CHS: 94],Attempt 3[HOST-CHS: 91],p=0.34), but improved in 79% during Attempt 4(Attempt 3[HOST-CHS: 91],Attempt 4[HOST-CHS: 99],p=0.004) matching their previous performance. CONCLUSIONS: The monthly HOST course was successfully incorporated into a training curriculum for CHS surgeons using objective assessments to measure technical performance. Trainees demonstrated an improvement across all evaluated procedures and retained their skills when reassessed following a delay highlighting its value in CHS training.

5.
Artigo em Inglês | MEDLINE | ID: mdl-33249480

RESUMO

OBJECTIVES: The hands-on surgical training course utilizes 3-dimensional (3D)-printed heart models to simulate complex congenital heart operations. This study aimed to validate a model and assessment tool in the simulation of 2 techniques of the Norwood operation and investigate whether technical performance improves following rehearsal with or without proctor presence. METHODS: Five 'experienced' and 5 'junior' surgeons performed 2 techniques of the Norwood operation on 3D-printed models of hypoplastic left heart syndrome. Performances were retrospectively assessed by 10 raters with varying experience in congenital heart surgery (CHS) (Medical Doctorate versus non-Medical Doctorate). Assessments were made with the procedure-specific Hands-On Surgical Training-CHS tool. Results were analysed for technical performance and rater consistency. Following validation, 30 surgeons (24 with proctor guidance and 6 with training videos and objective feedback only) simulated the Norwood operation twice. RESULTS: Performance scores were consistently higher for experienced surgeons and raters discriminated clearly between the experienced and junior surgeons (P ≤ 0.001). The hands-on surgical training-CHS tool showed high inter-rater (0.86) and intra-rater (0.80) reliability among all raters. Scores for both experienced and junior surgeons were highly consistent across all raters, with no statistically significant difference (P = 0.50). All surgeons successfully performed the Norwood operation. Sixty attempts were scored in total. Eighty-seven percentage (26/30) of surgeons' scores (mean: attempt 1 = 92, attempt 2 = 104) and times [mean: attempt 1 = 1:22:00, attempt 2 = 1:08:00 (h:mm:ss)] improved between the 2 attempts by 9% and 15% respectively (P ≤ 0.001). Total scores of all surgeons in the non-proctored subgroup (6) improved by 15% on average (mean: attempt 1 = 86, attempt 2 = 105, P = 0.002). CONCLUSIONS: Procedure-specific assessment tools can be developed to evaluate technical performance for complex CHS simulation and be performed reliably by non-expert raters. Rehearsal both under supervision and independently leads to technical skill improvement further supporting its value in CHS training.

6.
J Am Heart Assoc ; 9(22): e018275, 2020 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-33170057

RESUMO

Various congenital heart diseases are associated with malalignment of a part of the ventricular septum. Most commonly, the outlet septum is malaligned toward the right or left ventricle. Less commonly, the whole or a major part of the ventricular septum is malaligned in relation to the atrial septal plane. Although the pathological conditions associated with ventricular septal malalignment have been well recognized, the descriptions are often confusing and sometimes incorrect. In this pictorial essay, we introduce our systematic approach to the assessment of malalignment type ventricular septal defects with typical case examples. The systematic approach comprises description of the essential features of malalignment, including the following: (1) the malaligned part of the ventricular septum, (2) the reference structure, (3) the mechanism of malalignment, (4) the direction of malalignment, and (5) the severity of malalignment.

7.
Heart ; 2020 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-32748799

RESUMO

OBJECTIVES: Although a life-preserving surgery for children with single ventricle physiology, the Fontan palliation is associated with striking morbidity and mortality with advancing age. Our primary objective was to evaluate the impact of non-invasive, external, thoraco-abdominal ventilation on pulmonary blood flow (PBF) and cardiac output (CO) as measured by cardiovascular magnetic resonance (CMR) imaging in adult Fontan subjects. METHODS: Adults with a dominant left ventricle post-Fontan palliation (lateral tunnel or extracardiac connections) and healthy controls matched by sex and age were studied. We evaluated vascular flows using phase-contrast CMR imaging during unassisted breathing, negative pressure ventilation (NPV) and biphasic ventilation (BPV). Measurements were made within target vessels (aorta, pulmonary arteries, vena cavae and Fontan circuit) at baseline and during each ventilation mode. RESULTS: Ten Fontan subjects (50% male, 24.5 years (IQR 20.8-34.0)) and 10 matched controls were studied. Changes in PBF and CO, respectively, were greater following BPV as compared with NPV. In subjects during NPV, PBF increased by 8% (Δ0.20 L/min/m2 (0.10-0.53), p=0.011) while CO did not change significantly (Δ0.17 L/min/m2 (-0.11-0.23), p=0.432); during BPV, PBF increased by 25% (Δ0.61 L/min/m2 (0.20-0.84), p=0.002) and CO increased by 16% (Δ0.47 L/min/m2 (0.21-0.71), p=0.010). Following BPV, change in PBF and CO were both significantly higher in subjects versus controls (0.61 L/min/m2 (0.2-0.84) vs -0.27 L/min/m2 (-0.55-0.13), p=0.001; and 0.47 L/min/m2 (0.21-0.71) vs 0.07 L/min/m2 (-0.47-0.33), p=0.034, respectively). CONCLUSION: External ventilation acutely augments PBF and CO in adult Fontan subjects. Confirmation of these findings in larger populations with longer duration of ventilation and extended follow-up will be required to determine sustainability of haemodynamic effects.

8.
J Surg Educ ; 2020 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-32646811

RESUMO

OBJECTIVE: Over the last decade medical students' interest in pursuing surgery as a career has declined. This is more apparent in high-specialized specialities such as congenital heart surgery (CHS). Early hands-on simulation has shown to have a positive impact on medical students' interest in pursuing surgery, however, its incorporation into medical school curricula is lacking. This study aimed to evaluate the impact of incorporating medical students as surgical assistants during the Hands On Surgical Training course in CHS. METHODS: Local preclinical medical students were invited to participate as surgical assistants during the 5th annual Hands On Surgical Training course in CHS. Among those who responded to the invitation, students were randomly selected and allocated to assist a congenital heart surgeon. All selected students attended an assistants' session prior to the course to familiarize themselves with assisting and to practice basic surgical skills. At the end of both courses students completed a questionnaire based on Likert 5-point scale to evaluate the courses' usefulness. RESULTS: Fifteen medical students completed the questionnaires. All reported a beginner level of understanding of congenital heart disease. All students were highly satisfied with using 3D-printed models to help their understanding of congenital heart disease (4.80 ± 0.41) and agreed that the sessions improved their assisting skills (4.93 ± 0.26). All expressed a desire to attend similar sessions in the future and agreed that surgical simulation inclusion into medical school curricula would enhance learning (5.00 ± 0.00). Interest in pursuing a career in CHS increased from 33% (5) to 87% (13) by the end of the course. CONCLUSIONS: Integration of preclinical medical students into surgical simulation increases interest in pursuing highly specialised surgical specialities such as CHS. Early exposure and the incorporation of such simulation programs into medical school curricula will likely improve surgical skill acquisition and may enable students to be better informed when selecting future career choices.

9.
Pediatr Radiol ; 50(11): 1610-1616, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32613358

RESUMO

Digital reality is an emerging platform for three-dimensional representation of medical imaging data. In this technical innovation paper, the authors evaluated the accuracy and utility of mixed-reality technology in the morphological evaluation of complex congenital heart disease. The authors converted CT datasets of 12 heart specimens with different subtypes of double-outlet right ventricle to stereoscopic images and interrogated them using a mixed-reality system. The morphological features identified on the stereoscopic models were compared with findings at macroscopic examination of the actual heart specimens. The results showed that the mixed-reality system provided highly accurate stereoscopic display of spatially complex congenital cardiac lesions, with interactive features that might enhance 3-D understanding of morphology. Additionally, the authors found that high-resolution digital reproduction of cardiac specimens using clinical CT scanners is feasible for preservation and educational purposes.

10.
3D Print Med ; 6(1): 12, 2020 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-32488567

RESUMO

BACKGROUND: The Hands-On Surgical Training in Congenital Heart Surgery (HOST-CHS) program using 3D printed heart models has received positive feedback from attendees. However, improvements were necessary in the simulator set up to replicate the ergonomics experienced in the operating room. This paper illustrates the development of a dynamic chest wall and operating table simulator to enhance the simulation experience. METHODS: The simulator was designed to address the limitations with the existing set up. This included a suboptimal operating position, unrealistic surgical exposure and limitations in illuminating the operative field and recording procedures. A combination of computer-aided design and various 3D-printing techniques were used to build the components. The simulator's usefulness was evaluated by surgeons who attended the 5th annual HOST course via a questionnaire. RESULTS: The simulator consists of three components; an operating table simulator which allows height adjustment and pitch-and-roll motion; a suture retraction disc, which holds sutures under tension to improve exposure; and a pediatric chest wall cavity to replicate a surgeon's access experience during surgery. Nineteen surgeons completed the questionnaire. All surgeons agreed that the addition of the simulator was acceptable for surgical simulation and that it helped replicate the ergonomics experienced in the operating room. CONCLUSIONS: The inclusion of the HOST-CHS simulator adds value to simulation in congenital heart surgery (CHS) as it replicates the view and exposure a surgeon experiences. Improvements like these will help develop high-fidelity simulation programs in CHS, which could be utilized to train surgeons globally.

12.
3D Print Med ; 6(1): 2, 2020 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-32016687

RESUMO

BACKGROUND: The structure of the valve leaflets and sinuses are crucial in supporting the proper function of the semilunar valve and ensuring leaflet durability. Therefore, an enhanced understanding of the structural characteristics of the semilunar valves is fundamental to the evaluation and staging of semilunar valve pathology, as well as the development of prosthetic or bioprosthetic valves. This paper illustrates the process of combining computer-aided design (CAD), 3D printing and flow assessment with 4-dimensional flow magnetic resonance imaging (MRI) to provide detailed assessment of the structural and hemodynamic characteristics of the normal semilunar valve. METHODS: Previously published geometric data on the aortic valve was used to model the 'normal' tricuspid aortic valve with a CAD software package and 3D printed. An MRI compatible flow pump with the capacity to mimic physiological flows was connected to the phantom. A peak flow rate of 100 mL/s and heart rate of 60 beats per minute were used. MRI measurements included cine imaging, 2D and 4D phase-contrast imaging to assess valve motion, flow velocity and complex flow patterns. RESULTS: Cine MRI data showed normal valve function and competency throughout the cardiac cycle in the 3D-printed phantom. Quantitative analysis of 4D Flow data showed net flow through 2D planes proximal and distal to the valve were very consistent (26.03 mL/s and 26.09 mL/s, respectively). Measurements of net flow value agreed closely with the flow waveform provided to the pump (27.74 mL/s), confirming 4D flow acquisition in relation to the pump output. Peak flow values proximal and distal to the valve were 78.4 mL/s and 63.3 mL/s, respectively. Particle traces of flow from 4D-phase contrast MRI data demonstrated flow through the valve into the ascending aorta and vortices within the aortic sinuses, which are expected during ventricular diastole. CONCLUSION: In this proof of concept study, we have demonstrated the ability to generate physiological 3D-printed aortic valve phantoms and evaluate their function with cine- and 4D Flow MRI. This technology can work synergistically with promising tissue engineering research to develop optimal aortic valve replacements, which closely reproduces the complex function of the normal aortic valve.

13.
J Thorac Cardiovasc Surg ; 160(1): 229-240.e1, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-31973896

RESUMO

BACKGROUND: Hands-on surgical simulation has been sought to address training limitations within congenital heart surgery (CHS). However, there is a need for objective assessment methods to measure surgeons' performance to justify its global adoption. This study aimed to validate a procedure-specific assessment tool for the simulation of the arterial switch operation on 3D-printed models and to evaluate the consistency of scoring among evaluators with different levels of experience in CHS. METHODS: Five "expert" and 5 "junior" surgeons performed the arterial switch procedure on 3D-printed models with transposition of the great arteries during 2 hands-on surgical training courses. Their performance was retrospectively assessed by 9 evaluators with varying experience in CHS (staff surgeons, resident surgeons, and non-MD raters). Assessments were done using 2 assessment tools: the Hands-On Surgical Training-Congenital Heart Surgery (HOST-CHS) assessment tool and the global rating scale (GRS). RESULTS: The HOST-CHS tool showed a higher interrater and intrarater reliability compared with the GRS. Total scores for expert surgeons were highly consistent across all evaluators. Non-MD raters' total scores for junior surgeons were slightly higher than those of residents and staff evaluators. All grades of evaluator were able to discriminate between junior and expert surgeons. CONCLUSIONS: This study demonstrates the development and validation of an objective, procedure-specific assessment tool for the arterial switch operation with consistency among evaluators with different experience. There is now a platform for quantifying and accurately evaluating performance, which will be highly beneficial in training and developing the next generation of congenital heart surgeons.


Assuntos
Transposição das Grandes Artérias/educação , Avaliação Educacional/métodos , Treinamento por Simulação/métodos , Cirurgiões/educação , Transposição dos Grandes Vasos/cirurgia , Humanos , Impressão Tridimensional , Reprodutibilidade dos Testes , Estudos Retrospectivos
14.
J Thorac Cardiovasc Surg ; 160(4): 1035-1042, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31983523

RESUMO

OBJECTIVE: Data supporting the use of hands-on simulation in congenital heart surgery are promising but primarily qualitative. This study aimed to demonstrate if there was an objective improvement in time and technical performance of the arterial switch procedure on 3-dimensional printed heart models by surgeons using a validated assessment method. METHODS: A total of 30 surgeons of varying experience performed the arterial switch procedure twice on 3-dimensional printed models with transposition of the great arteries during the Hands-on Surgical Training courses. Surgeons' performances were recorded and retrospectively assessed for both time and performance using the Hands-on Surgical Training-Congenital Heart Surgery tool, a validated procedure-specific assessment tool for the arterial switch. RESULTS: A total of 60 videos were scored. Eighty percent of surgeons (24/30) had improved from their first attempt. The mean total score of the first attempt performance compared with the second was 103 and 120, respectively, with a mean difference in score of 17 (95% confidence interval, 10-24). All surgeons were statistically significantly quicker in their second attempt. The mean time for the first attempt compared with the second was 1 hour, 28 minutes, 4 seconds and 1 hour, 5 minutes, and 45 seconds, respectively, with a mean difference of 0 hours, 22 minutes, 19 seconds (95% confidence interval, 0 hours, 15 minutes, 22 seconds to 0 hours, 25 minutes, 34 seconds). CONCLUSIONS: This is the first study to demonstrate an objective improvement in time and technical performance of the arterial switch procedure on 3-dimensional printed heart models. This supports the evidence that simulation in the form of deliberate practice with constructive, objective feedback is fundamental in the training of future congenital heart surgeons. These simulations and assessments should be incorporated to create structured, standardized training curricula within congenital heart surgery.


Assuntos
Transposição das Grandes Artérias/educação , Educação de Pós-Graduação em Medicina , Cardiopatias Congênitas/cirurgia , Modelos Anatômicos , Impressão Tridimensional , Cirurgiões/educação , Transposição das Grandes Artérias/efeitos adversos , Competência Clínica , Cardiopatias Congênitas/patologia , Humanos , Curva de Aprendizado , Duração da Cirurgia , Estudos Retrospectivos , Análise e Desempenho de Tarefas , Fatores de Tempo , Gravação em Vídeo
15.
Korean J Radiol ; 21(2): 133-145, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31997589

RESUMO

Three-dimensional (3D) imaging and image reconstruction play a prominent role in the diagnosis, treatment planning, and post-therapeutic monitoring of patients with congenital heart disease. More interactive and realistic medical experiences take advantage of advanced visualization techniques like augmented, mixed, and virtual reality. Further, 3D printing is now used in medicine. All these technologies improve the understanding of the complex morphologies of congenital heart disease. In this review article, we describe the technical advantages and disadvantages of various advanced visualization techniques and their medical applications in the field of congenital heart disease. In addition, unresolved issues and future perspectives of these evolving techniques are described.


Assuntos
Realidade Aumentada , Cardiopatias Congênitas/diagnóstico , Imageamento Tridimensional/métodos , Realidade Virtual , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Processamento de Imagem Assistida por Computador , Imagem por Ressonância Magnética , Impressão Tridimensional
16.
Semin Thorac Cardiovasc Surg ; 32(1): 98-105, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31220532

RESUMO

Congenital heart surgery is a technically demanding specialty resulting in a prolonged training period. With the growing expectation of perfect patient outcomes, there is a need for improved training methods by implementing simulation. We assess the utilization of simulation in the training of congenital heart surgeons and discuss its future implications. A keyword-based PubMed literature search was conducted for hands-on surgical simulation in congenital heart surgery. The abstracts/titles of the search were reviewed and papers using simulation specific to congenital cardiac surgery were selected. Studies that did not include surgeons operating on the simulator, or did not incorporate assessment methods were excluded. Analysis included the problem addressed, simulator-type, methodology, assessment methods, results, benefits/limitations, and reproducibility. Five papers fulfilled our selection criteria of hands-on surgical simulation in congenital heart surgery with an assessment of the simulator or procedural performance. One simulation used animal models and 4 utilized 3D-printed models. Simulators covered either single or multiple complex procedures. All studies highlight usefulness of simulation; however, only 1 study has been replicated with >10 participants. The studies demonstrate how hands-on surgical simulation is possible within congenital heart surgery. Although primarily proof of concept studies, the next step would involve using a greater number of participants and demonstrate how repetition and deliberate practice will improve outcomes. Congenital heart surgery is one of the most technically demanding surgical specialties; therefore, we should lead the way in utilizing simulation to complement the training of our surgeons as we face the challenges ahead.


Assuntos
Procedimentos Cirúrgicos Cardíacos/educação , Educação de Pós-Graduação em Medicina , Cardiopatias Congênitas/cirurgia , Internato e Residência , Treinamento por Simulação , Cirurgiões/educação , Animais , Competência Clínica , Simulação por Computador , Currículo , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Modelos Animais , Modelos Cardiovasculares , Impressão Tridimensional
17.
Abdom Radiol (NY) ; 45(1): 188-202, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31471707

RESUMO

PURPOSE: To describe liver imaging findings and complications on computed tomography (CT) or magnetic resonance imaging (MRI) in adults with Fontan palliation and investigate whether imaging features show correlations with clinical and physiological parameters. METHODS: Our Institutional Review Board approved this retrospective study. Two blinded abdominal radiologists reviewed abdominal CT (n = 21) and MRI (n = 16) images between September 2011 and October 2017 in 37 adults (median age 27 years, interquartile range 21-36 years, 14 males [38%]) with a Fontan palliation (median post-Fontan duration 22 years, interquartile range 19-28 years). Correlation between CT/MRI findings and clinical parameters including laboratory results within 6 months of CT/MRI examinations was assessed by Spearman's rank correlation coefficient. RESULTS: Lobulated hepatic surface and blunt hepatic edge were seen in 92% (34/37) and 95% (35/37) of patients, respectively. Surface nodularity was noted in 32% (12/37). In 7 patients, there were 11 hepatic nodules which showed arterial-phase hyperenhancement and washout. Among them, 2 were biopsy-proven hepatocellular carcinomas (HCCs), and the remaining 9 were focal nodular hyperplasia (FNH)-like nodules. Suprahepatic inferior vena cava (IVC) diameter showed positive correlations with post-Fontan duration (p < 0.01), serum gamma-glutamyl transferase (p < 0.01), and total bilirubin (p < 0.01). CONCLUSION: The livers in post-Fontan adults show a unique morphology of blunt edge and lobulating surface with occasional nodularity. There is a diagnostic challenge in distinguishing HCCs from FNH-like nodules in post-Fontan population due to overlapping imaging findings. Suprahepatic IVC diameter is a potentially useful imaging marker that reflects hepatic dysfunction in Fontan palliation.

18.
Congenit Heart Dis ; 14(6): 901-923, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31532081

RESUMO

Left main coronary artery ostial atresia (LMCAOA) is a rare congenital anomaly of the coronary arteries. The published literature regarding the current diagnostic and management recommendations are limited. We present three case series of LMCAOA from our institution, including one with a unique association with anomalous origin of left coronary artery (LCA) from pulmonary artery. In addition, this report includes a review of 50 pediatric and 43 adult cases from literature. The majority of the patients were symptomatic. Sudden cardiac death occurred in 10% of pediatric patients and 7% of adult patients. Almost half of pediatric patients had additional cardiac lesions. At the time of diagnosis, 82% of patients had abnormal exercise stress test and 73% had abnormal myocardial perfusion imaging (MPI). The diagnosis of LMCAOA was suspected by echocardiography in 47% of pediatric patients, while 26% were initially misdiagnosed as anomalous origin of LCA from pulmonary artery. Coronary angiography confirmed the diagnosis in most cases and 70.5% of pediatric patients had small collaterals, while 80.5% of adult patients had large collaterals. Nine pediatric patients had no revascularization surgery with five deaths. Revascularization surgery was performed in 39 pediatric patients with four deaths. After 2005, there is a gradual shift toward performing coronary osteoplasty rather than coronary artery bypass grafting. Eighteen adult patients had revascularization surgery and all survived. Fifteen adult patients had no revascularization surgery, of which there were five deaths. In patients with LMCAOA, revascularization surgery is currently recommended in the presence of symptoms, ischemic changes on electrocardiogram or exercise stress test, myocardial perfusion defect on MPI, global left ventricular systolic dysfunction on echocardiogram, severe mitral regurgitation, or small-sized collaterals in coronary angiography. Short-term and mid-term outcomes are encouraging.


Assuntos
Circulação Colateral , Circulação Coronária , Anomalias dos Vasos Coronários , Vasos Coronários , Revascularização Miocárdica , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Técnicas de Imagem Cardíaca , Criança , Pré-Escolar , Anomalias dos Vasos Coronários/diagnóstico , Anomalias dos Vasos Coronários/mortalidade , Anomalias dos Vasos Coronários/fisiopatologia , Anomalias dos Vasos Coronários/cirurgia , Vasos Coronários/diagnóstico por imagem , Vasos Coronários/fisiopatologia , Vasos Coronários/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Revascularização Miocárdica/efeitos adversos , Revascularização Miocárdica/mortalidade , Valor Preditivo dos Testes , Fatores de Risco , Resultado do Tratamento , Adulto Jovem
19.
J Cardiovasc Magn Reson ; 21(1): 39, 2019 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-31303178

RESUMO

BACKGROUND: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion. METHODS: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls. RESULTS: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan. CONCLUSION: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures. TRIAL REGISTRATION: Retrospectively registered data.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cirrose Hepática/diagnóstico por imagem , Imagem Cinética por Ressonância Magnética , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Estudos de Viabilidade , Feminino , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/fisiopatologia , Hemodinâmica , Humanos , Lactente , Cirrose Hepática/etiologia , Cirrose Hepática/fisiopatologia , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
20.
Korean J Radiol ; 20(7): 1186-1194, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31270982

RESUMO

OBJECTIVE: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI). MATERIALS AND METHODS: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3-17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls. RESULTS: In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m², p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m², p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m², p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m², p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m², p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs. -0.08 ± 0.29 L/min/m², p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3. CONCLUSION: Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation.


Assuntos
Abdome/irrigação sanguínea , Técnica de Fontan/métodos , Imagem Cinética por Ressonância Magnética/métodos , Fluxo Sanguíneo Regional/fisiologia , Adolescente , Velocidade do Fluxo Sanguíneo/fisiologia , Criança , Pré-Escolar , Circulação Colateral , Feminino , Hemodinâmica , Artéria Hepática/fisiologia , Humanos , Lactente , Masculino , Veias Mesentéricas/fisiologia , Veia Porta/fisiologia
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