Review Article

Comprehensive Review on Imaging in Adult Congenital Heart Disease: Current Status and Future Development

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Abstract

Congenital heart disease manifests as structural cardiac anomalies present at birth, with a subset persisting into adulthood as adult congenital heart disease (ACHD). These conditions necessitate lifelong imaging surveillance to guide clinical management. Echocardiography serves as the primary imaging modality, offering detailed assessment of cardiac anatomy and function. In cases of greater complexity, cardiac MRI and CT provide complementary data, particularly when integrated with echocardiographic findings. These imaging techniques are critical for diagnosis, surgical planning and longitudinal monitoring, tailored to the distinct requirements of ACHD patients. This review delineates the current applications of principal imaging modalities, evaluating their respective strengths and limitations. Additionally, the utility of these modalities in prevalent ACHD conditions, including atrial septal defect, ventricular septal defect, tetralogy of Fallot and Fontan palliation, is examined. Finally, the emerging role of artificial intelligence and machine learning in enhancing cardiac imaging for ACHD is explored.

Keywords

Adult congenital heart disease, cardiac imaging, echocardiography, cardiac magnetic resonance imaging, cardiac computed tomography,

Received:

Accepted:

Published online:

DOI: https://doi.org/10.15420/japsc.2025.28

Disclosure: TT has received honoraria and travel support from Bayer. PTL has no conflicts of interest to declare.

Correspondence: Phong Teck Lee, Department of Cardiology, National Heart Centre Singapore, 5 Hospital Drive, 169609, Singapore. E: lee.phong.teck@singhealth.com.sg

Copyright:

© The Author(s). This work is open access and is licensed under CC-BY-NC 4.0. Users may copy, redistribute and make derivative works for non-commercial purposes, provided the original work is cited correctly.

Adult congenital heart disease (ACHD) encompasses a spectrum of cardiac defects present at birth that persist or manifest significant effects into adulthood. With advances in medical and surgical interventions, the ACHD population is growing, estimated at approximately 1.4 million in the US alone, necessitating robust imaging strategies for lifelong care.1 This review aims to elucidate the current status of imaging modalities in ACHD and explore future developments, addressing the complexities and challenges in this field.

The prevalence of ACHD, operated and non-operated, has increased due to improved survival rates, with common defects in adults including atrial septal defects (ASD), ventricular septal defects (VSD), coarctation of the aorta and tetralogy of Fallot (TOF), transposition of great arteries and Ebstein’s anomaly.2 Imaging is crucial for diagnosing these conditions, planning interventions and monitoring long-term outcomes, given the altered anatomy and potential for late complications.3 Historically, imaging evolved from basic chest X-rays to advanced modalities, such as echocardiography, cardiac MRI (CMR) and CT, transforming the management of ACHD patients.4

Current Imaging Modalities in Adult Congenital Heart Disease

Imaging is fundamental to the management of ACHD, with multiple modalities complementing each other to address clinical needs. Below, we detail each modality, drawing from recent literature and guidelines.

Echocardiography

Echocardiography, particularly transthoracic (TTE) and transoesophageal (TOE), remains the first-line imaging tool due to its accessibility, portability and lack of ionising radiation. It is crucial for assessing cardiac anatomy, physiology and haemodynamics, such as right ventricular (RV) and pulmonary artery (PA) pressure gradients. Advanced techniques, such as 3D and 4D echocardiography, provide en face views for defects, such as ASDs, aiding in device closure decisions, with studies showing its utility in some conditions, such as Ebstein’s anomaly and repaired TOF.5

Types of Echocardiography

TTE is ideal for initial assessments due to its non-invasive nature and widespread availability. In contrast, TOE is minimally invasive, but yields higher resolution images – particularly of posterior structures, such as the atria and valves – proving especially useful in patients with poor TTE windows, such as those with obesity or lung disease.6 3D and 4D echocardiography enhances this further by providing volumetric data, improving the evaluation of complex cardiac structures and congenital defects, which is crucial for planning surgical or catheter-based interventions.7 Doppler echocardiography, another key modality, employs the Doppler effect to measure blood flow velocities, facilitating the assessment of shunts (including pulmonary:systemic flow ratio; Qp:Qs), valve function and haemodynamic gradients, such as in aortic stenosis or pulmonary hypertension. Finally, contrast echocardiography uses microbubble contrast agents to enhance endocardial border detection and assess myocardial perfusion, significantly improving visualisation in challenging cases, such as in patients with poor acoustic windows or suspected intracardiac shunts.8

Specific Uses in Adult Congenital Heart Disease

In ACHD, echocardiography serves several specific purposes. It is vital for shunt assessment, detecting and quantifying intracardiac shunts, such as ASDs and VSDs, using colour Doppler to visualise flow direction and continuous wave Doppler to measure flow velocities, aiding in the determination of shunt significance. Echocardiography also evaluates valve function, assessing both native and prosthetic valves for stenosis or regurgitation, which is essential for managing valve-related complications common in ACHD, such as in patients with repaired defects or prosthetic replacements. Additionally, 3D and 4D echocardiograms provide critical information on ventricular function, detailing left ventricular (LV) and RV size and performance, which is particularly important for patients with volume or pressure overload, such as those with TOF, where RV dysfunction is a key concern.9 Finally, echocardiography facilitates haemodynamic assessment by estimating pulmonary artery pressures, a crucial measurement in some conditions, such as pulmonary hypertension associated with ACHD, helping guide therapy in these complex cases.

Challenges

Echocardiography in ACHD faces several challenges that can complicate its application. Poor acoustic windows pose a significant issue, particularly in adults who are obese or have had previous surgeries, as these factors can obscure adequate imaging, often necessitating TOE to achieve diagnostic clarity.6 Additionally, complex anatomy in postsurgical patients – common in ACHD due to repairs, such as those for tetralogy of Fallot or Fontan procedures – alters standard cardiac landmarks, making image interpretation difficult and requiring operators with specialised experience to accurately assess the altered structures.10 Furthermore, quantification accuracy is a limitation, as echocardiography may underestimate RV volumes and function compared with CMR, the reference standard, potentially impacting management decisions in conditions where precise RV assessment is critical, such as repaired TOF.1,11

Recent Advances

Recent advances in echocardiography have significantly enhanced its utility in ACHD. Improved transducer technology, including higher frequency transducers and harmonic imaging, has elevated image quality by reducing noise and improving resolution, thereby increasing diagnostic accuracy across a range of cardiac conditions.12 3D and 4D echocardiography represents another leap forward, offering more accurate assessment of complex defects and ventricular volumes compared with traditional 2D imaging, which facilitates precise planning for surgical or catheter-based interventions, particularly in ACHD patients with intricate anatomy.13 Additionally, speckle tracking echocardiography has emerged as a powerful tool, enabling the assessment of myocardial strain and strain rate to detect early ventricular dysfunction before overt changes in ejection fraction occur, thus aiding in risk stratification and guiding clinical management in ACHD patients.14

Cardiac MRI

CMR is considered the reference standard for quantifying RV and LV volumes, mass, and function, offering comprehensive anatomical and functional evaluation without ionising radiation.11 It is particularly valuable for assessing valvar dysfunction, shunts and tissue characterisation, such as myocardial fibrosis detected via late gadolinium enhancement (LGE), as seen in postoperative cases, including repaired TOF and Fontan operations.15–17

Sequences Used in Cardiac MRI

CMR employs several key sequences to evaluate cardiac health comprehensively. Cine imaging assesses cardiac function and anatomy with high temporal resolution, making it essential for evaluating ventricular function, such as ejection fraction and wall motion.11 Phase contrast imaging measures blood flow velocities and volumes, proving valuable for quantifying shunts and valvar regurgitation while providing critical haemodynamic data, such as in congenital heart disease or valve dysfunction.18 LGE detects myocardial fibrosis, playing a significant role in identifying arrhythmogenic substrates and guiding decisions for ICD implantation, particularly in some conditions, such as ischaemic cardiomyopathy or repaired tetralogy of Fallot.16 Additionally, T1 and T2 mapping, as emerging techniques, quantify myocardial tissue characteristics, such as oedema or fibrosis, enhancing diagnostic capabilities in complex cases, such as myocarditis or diffuse fibrosis.19

Specific Applications in Adult Congenital Heart Disease

In ACHD patients, CMR offers specific applications critical for comprehensive management. It provides accurate and reproducible measurements of LV and RV volumes, mass, and ejection fraction, essential for long-term follow-up in some conditions, such as tetralogy of Fallot, where ventricular volume and function impact prognosis.20,21 CMR also assesses valvar function, evaluating stenosis and regurgitation with quantification of flow and regurgitant fractions, aiding in decisions for valve replacement, such as in pulmonary valve dysfunction.22 For shunt quantification, it delivers precise measurements of shunt size and Qp:Qs, crucial for managing shunt-related complications. Vascular imaging with CMR evaluates great vessels, such as the aorta and pulmonary arteries, for anomalies or postinterventional changes, guiding interventions, such as stent placement or surgical repair.1,23 Additionally, LGE identifies myocardial viability and scarring, detecting fibrosis that can guide arrhythmia management or decisions on ICD implantation, particularly in patients with repaired congenital defects at risk for sudden cardiac death.16

Challenges

CMR in ACHD faces several challenges that can limit its application. Cost and availability pose significant barriers, as CMR is more expensive and less widely available than echocardiography, restricting access in resource-limited regions.11 Patient tolerance is another concern, with long scan times – often 30–60 minutes – and the need to lie still proving problematic, particularly for those with claustrophobia or irregular heart rhythms, sometimes necessitating sedation to complete the procedure. Additionally, device compatibility remains an issue, as patients with certain implanted devices, such as pacemakers or ICDs, may be ineligible for CMR due to safety risks from magnetic interference, although the increasing prevalence of MRI-conditional devices is expanding its applicability in this population.24

Recent Advances

Recent advances in CMR have enhanced its diagnostic potential and patient experience. Faster imaging techniques, such as parallel imaging and compressed sensing, reduce scan times – sometimes from 60 to <30 minutes – improving patient comfort and minimising motion artefacts, which is particularly beneficial for ACHD patients with limited tolerance.25 Higher field strength, such as 3T scanners, provides higher resolution images, offering improved detail of cardiac structures, although they can introduce more artefacts, necessitating advanced postprocessing techniques to maintain image quality.26,27 Additionally, the development of new contrast agents with better safety profiles and specific targeting capabilities, such as those reducing nephrotoxicity compared with traditional gadolinium-based agents, enhances diagnostic accuracy by improving tissue characterisation and visualisation of pathology.28

CT

CT provides excellent 3D spatial resolution and rapid acquisition, making it suitable for evaluating small vessels, coronary arteries and mechanical valves. It is particularly useful in some conditions, such as TOF, for assessing conduit calcification and in coarctation for stent evaluation.29

Specific Uses in Adult Congenital Heart Disease

In ACHD, CT serves several specific purposes critical for diagnosis and management. Coronary artery assessment is vital in patients with transposition of the great arteries or those at risk for coronary artery disease, providing detailed imaging to guide revascularisation strategies, such as in cases of anomalous coronary origins. CT also evaluates vascular anatomy, assessing conditions, such as coarctation of the aorta, pulmonary artery stenosis or post-stent placement outcomes, aiding in planning vascular interventions by delineating vessel morphology.23,30,31 For pre-interventional planning, CT offers precise anatomical details for surgical or transcatheter interventions, such as transcatheter valve replacements or defect closures, enhancing procedural success rates in complex ACHD cases.32 Additionally, it detects mechanical valve thrombosis or dysfunction, which is crucial for managing valve-related complications in ACHD patients with prosthetic valves.33 Finally, CT assesses ventricular size and function when cardiac MRI is unsuitable – due to incompatible devices, such as certain pacemakers or claustrophobia – serving as an effective alternative for volumetric and functional analysis.34

Challenges

CT involves ionising radiation exposure, a significant concern for younger patients requiring multiple scans, necessitating dose minimisation strategies to reduce long-term risks.35 The use of iodinated contrast, which is nephrotoxic and can trigger allergic reactions, limits its application in patients with renal impairment, requiring careful patient selection to avoid complications.36 Image artefacts from metal implants, common in postsurgical ACHD patients, can complicate interpretation, often demanding advanced reconstruction techniques to mitigate their impact.37

Recent Advances

Recent advances address the aforementioned issues: low-dose CT protocols, such as those employing iterative reconstruction, reduce radiation exposure while maintaining image quality, enhancing patient safety for serial imaging.38 Dual-energy CT provides material-specific imaging, distinguishing tissues and reducing artefacts from contrast or metal, thereby improving diagnostic accuracy in complex cases.39 Additionally, iterative reconstruction further enhances image quality at lower radiation doses, making CT more suitable for longitudinal follow-up in ACHD patients.38

Nuclear Imaging

Nuclear imaging, including single-photon emission computed tomography (SPECT) and PET, is less commonly used, but reserved for specific applications, such as myocardial stress perfusion or assessing pulmonary blood flow when CMR is unavailable. It adds value in evaluating viability and detecting inflammatory processes, with advances in dedicated cardiac SPECT scanners and low-dose imaging techniques.40

Specific Uses and Challenges

In ACHD, nuclear imaging techniques, such as SPECT and PET, offer specific diagnostic applications, although they come with notable challenges. Myocardial perfusion imaging with SPECT or PET assesses coronary artery disease, providing functional data on MI, which is particularly valuable in patients with complex anatomy or those unable to undergo CMR due to device incompatibility or claustrophobia.41 Pulmonary blood flow imaging, often using ventilation/perfusion scans, evaluates lung blood flow distribution or detects pulmonary embolism in patients with pulmonary hypertension or shunt lesions, aiding in diagnostic clarification.42

PET also excels in viability assessment, identifying viable myocardium to guide revascularisation strategies in ischaemic ACHD patients, where distinguishing hibernating from scarred tissue can influence outcomes.43 However, radiation exposure from both SPECT and PET, involving ionising radiation, raises concerns for repeated studies, particularly in younger patients, limiting their use to minimise cumulative risk.35 Additionally, nuclear imaging’s limited resolution compared with CMR or CT hampers detailed anatomical assessments, often necessitating complementary imaging modalities for a comprehensive evaluation.44

Clinical Applications of Imaging in Adult Congenital Heart Disease

In ACHD, imaging is indispensable for initial diagnosis, preoperative planning, postoperative follow-up and complication management across a spectrum of lesions. The following sections explore these imaging applications in specific ACHD lesions, including ASDs, VSDs, TOF and Fontan surgery.

Atrial Septal Defect

ASDs require precise imaging for effective diagnosis and management due to their potential to cause right heart volume overload, pulmonary hypertension or arrhythmias if untreated. For initial diagnosis, echocardiography is the cornerstone, using 2D imaging and colour Doppler to detect the defect’s location (e.g. secundum, primum or sinus venosus) and assess shunt direction, with continuous wave Doppler quantifying flow velocities to estimate shunt significance. In select cases, TOE is subsequently required to confirm ASD size and assess suitability for percutaneous device closure (Figure 1 ). In cases with poor acoustic windows – common in obese adults or those with lung disease – CMR or CT provides detailed anatomical visualisation and shunt quantification, measuring the Qp:Qs, where a ratio >1.5 often indicates a significant shunt warranting intervention.1,45 CMR also excels in assessing RV size and function, while CT may be required in select cases to delineate associated anomalies, such as partial anomalous pulmonary venous return.32

Figure 1: Transoesophageal Echocardiogram Depicting En Face Visualisation of Secundum Atrial Septal Defect from the Right Atrium

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Postoperatively, echocardiography monitors for residual shunts, device position (e.g. after transcatheter closure) and RV remodelling, with CMR employed periodically to quantify RV volume reduction and confirm haemodynamic normalisation, especially if echocardiography is inconclusive. For late complications, such as atrial arrhythmias or pulmonary hypertension, CMR with LGE detects atrial fibrosis linked to arrhythmic risk, while CT evaluates pulmonary vascular changes and echocardiography tracks RV dysfunction or tricuspid regurgitation.46

Ventricular Septal Defect

TTE is the primary diagnostic tool, using 2D imaging and colour Doppler to determine the defect’s location (e.g. perimembranous, muscular or inlet), size and shunt direction, while continuous wave Doppler quantifies flow velocities to assess shunt severity (Figure 2). TOE provides superior resolution for evaluating defect margins and can help guide surgical management. In patients with suboptimal echocardiographic windows, CMR offers precise anatomical visualisation and quantifies the Qp:Qs, with a ratio >1.5 often indicating a significant shunt requiring intervention (Figure 2).1,45 CMR also accurately assesses LV and RV volumes and function, essential for guiding management.1,45 Cardiac CT is particularly useful for identifying VSD anatomy and associated anomalies, such as coronary artery abnormalities. Postoperatively, echocardiography monitors residual shunts, device positioning and ventricular remodelling, while CMR, with LGE, evaluates long-term ventricular function and detects myocardial fibrosis, aiding in the assessment of complications, such as heart failure or arrhythmias.16

Figure 2: Moderate-sized Perimembranous Ventricular Septal Defect

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Tetralogy of Fallot

Preoperative Imaging

TOF is the most common cyanotic congenital heart disease. If left untreated, the mortality rate after age 40 years is 95%.47 However, unrepaired TOF is still found in developing countries due to limited healthcare access. Preoperative cardiac evaluation includes complete cardiac anatomy evaluation, the level and degree of right ventricular outflow tract (RVOT) obstruction, the size of pulmonic valve annulus, pulmonic valve anatomy and function, PA branches, ventricular functions, aortic valve, the ascending aorta size, systemic-to-pulmonary collaterals, and coronary artery anomaly.48 Although echocardiography is the first-line imaging modality for cyanotic patients, the PA branches, collaterals and coronary arteries are better evaluated by either cardiac CT or cardiac MRI (Figure 3).

Figure 3: Case of Uncorrected Tetralogy of Fallot

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Postoperative Imaging

Surgical repair of TOF involves the relief of RVOT obstruction. Therefore, RVOT aneurysm and pulmonary regurgitation (PR) are often found decades after surgery. In addition, residual RVOT or branch PA stenosis, residual VSDs, aortic dilatation with aortic regurgitation, RV dilatation and RV dysfunction with tricuspid regurgitation are cardiac abnormalities commonly found in patients after TOF repair. Cardiac imaging needs to explore all of these abnormalities. Although multimodality imaging is recommended in patients with repaired TOF, echocardiography is an excellent first-line imaging for screening.10,49 The 2018 American College of Cardiology/American Heart Association guidelines for management of ACHD patients have recommended routine TTE every 6 months or 2 years, depending on the physiological stage of the patients.1

PR severity, residual VSDs, degree of aortic regurgitation and the RV dilatation can be demonstrated by echocardiography. However, CMR has been recommended as the gold standard for RV size and function quantification. PR can also be graded by CMR, and the pulmonary valve replacement has been recommended as a class 1 indication in asymptomatic patients with severe PR with either the RV end-diastolic volume index >160 ml/m2 or the RV end-systolic volume index >80 ml/m2.45 The residual branch PA stenosis can be well appreciated using contrast-enhanced magnetic resonance angiography or CT angiography. Although the peak gradient across stenosis cannot be measured by CMR, the flow distribution in the left and right lungs can be quantified by CMR flow analysis. In addition, the RVOT scar, as demonstrated by LGE-CMR, is one of the additional risk factors for sudden cardiac death in patients with repaired TOF (Figure 4 ).1,45

Figure 4: Late Gadolinium Enhancement on Cardiac MRI

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In patients with metallic artefacts or patients with cardiac implant devices, cardiac CT may be used if CMR is not feasible due to metallic artefacts. However, due to radiation concern and iodine contrast exposure, the risk and benefit of cardiac CT for follow-up should be weighed cautiously, especially in young patients. Currently, transcatheter pulmonic valve replacement has been recommended as a class 1 indication for patients with severe PR.45 Cardiac CT has emerged as an important imaging modality for intervention determination. Details of the technical approach and reporting cardiac CT have been described in the recent practice guidelines.50

Fontan Surgery

Patients with Fontan surgery have long-term consequences related to the elevated central venous pressure and reduced cardiac output. Multimodality imaging is necessary for assessing the Fontan circulation due to its complex haemodynamics.51 The comprehensive assessment involves the ventricular and valve function, Fontan pathway patency, and the presence of collateral flows. Other extra-cardiac findings, especially liver cirrhosis or hepatic tumour and lymphatic disorders, are also important targets of imaging surveillance.

Due to complex anatomy, either cardiac MRI or cardiac CT show better comprehensive evaluation than echocardiography. For cardiac CT, technical issues regarding the contrast dispersal through the pulmonary vasculature should be considered. Nevertheless, cardiac CT is the gold standard imaging for collateral vessels evaluation due to its high spatial resolution. CMR is an excellent imaging tool for Fontan pathway flow quantification. Previous studies have demonstrated that the CMR parameters, such as collateral flow volume quantified by cardiac MRI and the end-diastolic ventricular volume index, are associated with worse clinical outcomes in Fontan surgery patients.17,52 Recently, 4D flow CMR is an emerging technology for Fontan pathway flow evaluation (Figure 5).53 The CMR-derived energetic flow has been associated with functional capacity in Fontan surgery patients.54,55

Figure 5: 4D Flow Cardiac MRI Sequence of a Patient who Underwent Fontan Surgery

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Future Developments in Imaging for Adult Congenital Heart Disease

Role of Artificial Intelligence and Machine Learning

Artificial intelligence (AI) and machine learning (ML) are revolutionising imaging in ACHD, enhancing the current status of diagnostic and management strategies while paving the way for future developments. In the evaluation of ACHD lesions, such as ASDs, VSDs and TOF, AI/ML leverages large datasets from echocardiography, CMR and CT to improve diagnostic precision and efficiency. Currently, ML algorithms can analyse echocardiographic images to automatically detect and classify congenital defects, such as ASD or VSD, improving diagnostic accuracy and reducing time, which is particularly beneficial in initial assessments for ACHD.56

In preoperative planning, AI-driven segmentation of CMR images accurately quantifies ventricular volumes and shunt ratios (e.g. Qp:Qs), aiding in timing interventions, such as pulmonary valve replacement in TOF, with studies showing improved reproducibility over manual methods.57 Postoperatively, ML models predict outcomes by integrating serial imaging data, such as RV remodelling after ASD closure, enhancing follow-up care through risk stratification.58 For complication management, AI applied to CMR LGE can detect and quantify myocardial fibrosis, a key arrhythmic risk factor in TOF, potentially guiding ICD implantation with greater accuracy.59

Looking to the future, AI promises real-time image optimisation (e.g. reducing CMR scan times via compressed sensing), integration of multimodal imaging data for personalised treatment plans and predictive analytics for long-term ACHD outcomes, although challenges, such as data standardisation and validation, remain.60 These advances position AI/ML as a transformative tool in ACHD imaging, bridging current practices with innovative future applications.

Personalised Medicine and 3D Printing

The integration of 3D printing and advanced imaging technologies is advancing personalised medicine in ACHD, addressing the heterogeneous nature of lesions, such as ASDs, VSDs and TOF, through tailored diagnostic and therapeutic approaches. 3D printing creates patient-specific models from detailed CMR or CT imaging data, enabling surgeons to visualise and plan complex repairs – such as pulmonary artery reconstruction in TOF or device closure in ASD – with enhanced precision, leading to improved procedural outcomes and reduced operative risks.61,62 Virtual reality complements this by leveraging imaging datasets to simulate procedures and visualise intricate anatomy in 3D, enhancing preoperative planning and training.63

Conclusion

Advances in imaging in ACHD have evolved into a cornerstone of clinical management, with echocardiography, CMR, CT and nuclear imaging providing critical insights into the diagnosis, preoperative planning, postoperative follow-up and complication management. Currently, these modalities offer a robust framework for assessing anatomical and functional abnormalities, guiding interventions, and monitoring long-term outcomes. The integration of newer technologies, such as faster imaging techniques, AI and ML, promise to further refine ACHD care. Together, these developments underscore the pivotal role of imaging in bridging current ACHD management with a future of precision and innovation.

Clinical Perspective

  • Multimodality imaging plays an important role in the lifetime management of adult congenital heart disease patients.
  • Echocardiography is the first-line tool for adult congenital heart disease diagnosis and monitoring.
  • Cardiac MRI provides gold standard ventricular volumetric and shunt assessment.
  • CT offers high-resolution vascular and coronary imaging, ideal for preprocedural planning.
  • Artificial intelligence, machine learning and 3D printing enhance diagnostic precision and personalised treatment planning for adult congenital heart disease care.

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