Dataset Information

Patient-Specific 3-Dimensional Printing of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

ABSTRACT: A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary collaterals. The virtual and solid 3-dimensional heart models provided intuitive representation of such complex and highly individual pathologies and allowed comprehensive spatial conceptualization of the cardiac structures. (Level of Difficulty: Advanced.).

SUBMITTER: Elmaghraby A

PROVIDER: S-EPMC8288583 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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Patient-Specific 3-Dimensional Printing of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

Elmaghraby Ahmed A Ghareep Abdel Naser AN Alkuwari Maryam M Hijazi Ziyad M ZM Salustri Alessandro A

JACC. Case reports 20191023 4

A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary collaterals. The virtual and solid 3-dimensional heart models provided intuitive representation of such complex and highly individual pathologies and allowed comprehensive spatial conceptualization of the cardiac structures. (<b>Level of Difficulty: Advanced.</b>). ...[more]

PMID: 34316872

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Project description:ObjectivesThe aim of this study was to characterize the anatomy of aortopulmonary collateral (APC) arteries in tetralogy of Fallot and pulmonary stenosis and to determine whether APC density identified on preoperative multidetector cardiac computed tomography predicts in-hospital outcome.MethodsThe retrospective single-centre study includes consecutive 135 (2015-2019) patients who underwent one-stage repair. Preoperative multidetector cardiac computed tomography, echocardiography and clinical outcomes were reviewed. The cut-off value of indexed total distal APC cross-sectional area (APC-CSA) was identified by receiver operating characteristic curve. Logistic regression was used for predictors analysis.ResultsThe median age and body weight were 19.7 (10.1-89.7) months and 10 (8.3-18) kg. A total of 337 APCs were detected with only one demonstrating severe stenosis. There was a strong and significant correlation between mean APC diameter per patient and age (r = 0.70, P < 0.001). APCs were imaged but mainly received no interventions. In-hospital mortality was similar between patients with high (indexed APC-CSA ≥3.0 mm2/m2) and low (<3.0 mm2/m2) APC density (P = 0.642). Significantly greater patients with high indexed APC-CSA experienced the in-hospital composite outcome of death, arrest, renal/hepatic injury, lactic acidosis or extracorporeal membrane oxygenation (P = 0.007). High APC density was associated with greater dosing (P = 0.008) and longer (P = 0.01) use of inotropic support, prolonged pleural drainage (P = 0.013), longer ventilation (P = 0.042), intensive care unit (P = 0.014) and hospital (P = 0.027) duration. No reintervention and death occurred in the follow-up with the median duration of 24.4 (11-36.6) months. Multivariable analysis showed the Nakata index (P = 0.05) and high APC density (P = 0.02) independently predicted the composite outcome.ConclusionsIn tetralogy of Fallot and pulmonary stenosis, APCs are likely to be dilated bronchial arteries. Preoperative multidetector cardiac computed tomography-derived APC density was as important as Nakata index in predicting the occurrence of in-hospital composite outcome. The APC-CSA of 3.0 mm2/m2 maybe considered as a threshold for risk stratification.

Project description:Background Tetralogy of Fallot with major aortopulmonary collateral arteries is a heterogeneous form of pulmonary artery (PA) stenosis that requires multiple forms of intervention. We present a patient-specific in vitro platform capable of sustained flow that can be used to train proceduralists and surgical teams in current interventions, as well as in developing novel therapeutic approaches to treat various vascular anomalies. Our objective is to develop an in vitro model of PA stenosis based on patient data that can be used as an in vitro phantom to model cardiovascular disease and explore potential interventions. Methods and Results From patient-specific scans obtained via computer tomography or 3-dimensional (3D) rotational angiography, we generated digital 3D models of the arteries. Subsequently, in vitro models of tetralogy of Fallot with major aortopulmonary collateral arteries were first 3D printed using biocompatible resins and next bioprinted using gelatin methacrylate hydrogel to simulate neonatal vasculature or second-order branches of an older patient with tetralogy of Fallot with major aortopulmonary collateral arteries. Printed models were used to study creation of extraluminal connection between an atretic PA and a major aortopulmonary collateral artery using a catheter-based interventional method. Following the recanalization, engineered PA constructs were perfused and flow was visualized using contrast agents and x-ray angiography. Further, computational fluid dynamics modeling was used to analyze flow in the recanalized model. Conclusions New 3D-printed and computational fluid dynamics models for vascular atresia were successfully created. We demonstrated the unique capability of a printed model to develop a novel technique for establishing blood flow in atretic vessels using clinical imaging, together with 3D bioprinting-based tissue engineering techniques. Additive biomanufacturing technologies can enable fabrication of functional vascular phantoms to model PA stenosis conditions that can help develop novel clinical applications.

Project description:RATIONALE:Familial recurrence studies provide strong evidence for a genetic component to the predisposition to sporadic, nonsyndromic Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease phenotype. Rare genetic variants have been identified as important contributors to the risk of congenital heart disease, but relatively small numbers of TOF cases have been studied to date. OBJECTIVE:We used whole exome sequencing to assess the prevalence of unique, deleterious variants in the largest cohort of nonsyndromic TOF patients reported to date. METHODS AND RESULTS:Eight hundred twenty-nine TOF patients underwent whole exome sequencing. The presence of unique, deleterious variants was determined; defined by their absence in the Genome Aggregation Database and a scaled combined annotation-dependent depletion score of ?20. The clustering of variants in 2 genes, NOTCH1 and FLT4, surpassed thresholds for genome-wide significance (assigned as P<5×10-8) after correction for multiple comparisons. NOTCH1 was most frequently found to harbor unique, deleterious variants. Thirty-one changes were observed in 37 probands (4.5%; 95% CI, 3.2%-6.1%) and included 7 loss-of-function variants 22 missense variants and 2 in-frame indels. Sanger sequencing of the unaffected parents of 7 cases identified 5 de novo variants. Three NOTCH1 variants (p.G200R, p.C607Y, and p.N1875S) were subjected to functional evaluation, and 2 showed a reduction in Jagged1-induced NOTCH signaling. FLT4 variants were found in 2.4% (95% CI, 1.6%-3.8%) of TOF patients, with 21 patients harboring 22 unique, deleterious variants. The variants identified were distinct to those that cause the congenital lymphoedema syndrome Milroy disease. In addition to NOTCH1, FLT4 and the well-established TOF gene, TBX1, we identified potential association with variants in several other candidates, including RYR1, ZFPM1, CAMTA2, DLX6, and PCM1. CONCLUSIONS:The NOTCH1 locus is the most frequent site of genetic variants predisposing to nonsyndromic TOF, followed by FLT4. Together, variants in these genes are found in almost 7% of TOF patients.

Dataset Information

Patient-Specific 3-Dimensional Printing of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

Publications

Patient-Specific 3-Dimensional Printing of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

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