New Directives in Cardiac Imaging: Imaging the Adult With Congenital Heart Disease

doi:10.1016/j.cjca.2013.04.020

Canadian Journal of Cardiology

Volume 29, Issue 7, July 2013, Pages 830-840

https://doi.org/10.1016/j.cjca.2013.04.020 Get rights and content

Abstract

Advances in pediatric surgical and interventional techniques and medical care over the past 50 years have revolutionized the care of children with congenital heart disease. Survival to adulthood is now expected and, as such, there is a growing population of adults which is exceeding the pediatric population with congenital heart disease. Noninvasive cardiac imaging with modalities such as echocardiography, computed tomography, and cardiac magnetic resonance imaging are integral to the care of adults with congenital heart disease. These modalities are used for diagnosis, surveillance for complications late after surgery and catheter-based interventions, and in decision-making for medical, interventional, and surgical therapies. In this review we will discuss noninvasive imaging modalities used to assess congenital cardiac lesions, imaging strategies for select congenital lesions, and comment on the future of cardiac imaging in congenital heart disease.

Résumé

Les progrès en matière de chirurgie pédiatrique, de techniques interventionnelles et de soins médicaux au cours des 50 dernières années ont révolutionné les soins aux enfants ayant une cardiopathie congénitale. On s'attend maintenant à une survie jusqu'à l'âge adulte et, à ce titre, la population adulte est supérieure à la population pédiatrique ayant une cardiopathie congénitale. L'imagerie cardiaque non effractive qui utilise des modalités comme l'échocardiographie, la tomodensitométrie et l'imagerie cardiaque par résonance magnétique fait partie intégrante des soins aux adultes ayant une cardiopathie congénitale. Ces modalités sont utilisées pour le diagnostic, la surveillance des complications tardives de la chirurgie et les interventions par cathéter, ainsi que pour la prise de décision quant aux traitements médicaux, interventionnels et chirurgicaux. Dans cette revue, nous discuterons des modalités d'imagerie non effractive utilisées pour évaluer les lésions cardiaques congénitales et des stratégies d'imagerie de certaines lésions congénitales, et émettrons des commentaires sur l'avenir de l'imagerie cardiaque dans l'évaluation de la cardiopathie congénitale.

Section snippets

Echocardiography

Echocardiography is the primary modality used for cardiac imaging in ACHD. It is safe, portable, readily available, and is easy to perform. The main limitation in the adult patient population, specifically in those with previous cardiac surgery, relates to poor acoustic windows.

Understanding cardiac structure is fundamental when imaging adults with congenital cardiac lesions and cardiac anatomy is often well visualized with echocardiography. Colour Doppler can detect small intracardiac shunts

ASDs

There are several types of ASD. The most common type is the secundum ASD located in the region of the fossa ovalis. Less common are the primum ASD, the unroofed coronary sinus, and the sinus venosus defect. An intracardiac shunt at the atrial level results in volume overload of the RV. Many patients only develop symptoms in adulthood including functional decline, atrial arrhythmias, stroke from paradoxical emboli, right heart failure, and less commonly, pulmonary arterial hypertension. When

Future Directions in Cardiac Imaging

Cardiac imaging is very important in ACHD. Noninvasive cardiac imaging helps to identify important residuae and sequelae, determine when surgery or therapy are indicated, and determine long-term prognosis. Physicians have a number of complementary imaging modalities available and typically a number of modalities are required for complete assessment of the patient (Fig. 7). TTE remains the most common imaging modality. For adults with lesions such as TOF, periodic CMR is recommended to

Acknowledgements

The authors thank Dr Andrew Crean and Dr Frederic Poulin for their assistance with image selection.

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Transthoracic echocardiography (TTE) with Doppler technology enables comprehensive and noninvasive assessment of cardiac morphology, function, and hemodynamics in most ACHD patients. However, due to their unique anatomies and repeated cardiac surgeries, TTE alone is not sufficient for a conclusive evaluation in patients with ACHD, especially in patients with complex ACHD, and a multimodality approach is therefore strongly recommended [5–7]. Owing to its outstanding spatial resolution, contrast-enhanced multidetector-computed tomography (MDCT) enables extensive anatomical analyses to be performed even in CHD with great complexity and it has therefore become an indispensable imaging tool for assessment of ACHD.
Noninvasive assessment of stenotic lesions in patients with complex adult congenital heart disease (ACHD) is challenging due to its complex morphology. The simultaneous two-screen display of multidetector-computed tomography (MDCT) and real-time echogram (STDME) technology can display a virtual multi-planar reconstruction from MDCT corresponding to the same cross-sectional image from transthoracic echocardiography (TTE). We investigated the usefulness of the STDME technology for stenosis severity assessment in complex ACHD patients.
Twenty-four complex ACHD patients with stenotic lesions were enrolled in this study. All patients underwent TTE and the STDME technology within a week after MDCT. Peak velocity and pressure gradient (PG) across the stenotic site were measured using continuous wave Doppler. Cardiac catheterization was performed in 17 patients.
Nine out of the twenty-four patients had undergone repair with a conduit. Peak velocity and PG from the STDME technology were higher than those from TTE (peak velocity: 3.1 ± 1.1 vs. 2.8 ± 1.0 m/s; peak PG: 43 ± 28 vs. 34 ± 21 mmHg; both p < 0.01). Peak PG from the STDME technology showed significant correlations with those from catheterization in patients with a conduit (n=7) and those without a conduit (n=10) (r = 0.795 and 0.880, respectively; both p < 0.05), while peak PG from TTE was correlated with catheterization measurements only in patients without a conduit (r = 0.850, p < 0.05).
The STDME technology enables more accurate assessment of conduit stenosis severity than does TTE in complex ACHD patients.
Ablation of Postoperative Atrial Tachycardia in Congenital Heart Disease
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This chapter reviews the etiologies, mechanisms, and therapeutic approaches used for treatment of atrial tachycardias in patients with congenital heart disease (CHD). Intraatrial reentrant tachycardia (IART) is a prevalent long-term consequence of CHD. IART may manifest as typical atrial flutter, atypical macroreentrant atrial tachycardia, or atrial fibrillation. In addition to causing symptoms, IART appears to be associated with heart failure, thrombosis and thromboembolism, and reduced survival. Although prophylactic antiarrhythmic drugs and pacing strategies may be useful in selected patients, modification of the atrial substrate using advanced ablation techniques and surgical intervention in some cases are more likely to succeed as definitive therapy. Future challenges include increasing long-term success rates and development of strategies for definitive management of atrial fibrillation in these complex patients.
PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: Developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD)
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Preprocedural evaluation should include documentation and analysis of all arrhythmias. Reports from previous surgical and catheter ablation procedures should be reviewed, and thorough knowledge of 3-dimensional cardiac anatomy obtained by echocardiography, MRI and/or CT scan.7,218,219 Vascular access may be hampered by vascular anomalies or prior interventions such that venography can be considered.
PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease
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Citation Excerpt :
Preprocedural evaluation should include documentation and analysis of all arrhythmias. Reports from previous surgical and catheter ablation procedures should be reviewed, and thorough knowledge of 3-dimensional cardiac anatomy obtained by echocardiography, MRI and/or CT scan.7,218,219 Vascular access may be hampered by vascular anomalies or prior interventions such that venography can be considered.
Evolving Trends in Interventional Cardiology: Endovascular Options for Congenital Disease in Adults
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Other types of ASDs, including ostium primum and sinus venosus defects, remain unsuitable for device closure. Patients with coexisting secundum defects and anomalous pulmonary veins might be carefully evaluated using cardiac MRI10 to define the relative flow from the anomalous vein vs the intracardiac shunt; in adults, especially of older age, closure of the defect without addressing the anomalous venous drainage might provide symptomatic relief (likely at the expense of mild residual right ventricle [RV] enlargement). Improvements in the ASD occluder devices, delivery systems, and techniques have improved safety and efficacy of the procedure and have allowed for closure of larger, more complex defects.
As increasing numbers of patients with congenital heart disease enter adulthood, there is a growing need for minimally invasive percutaneous interventions, primarily to minimize the number of repeated surgeries required by these patients. The use of percutaneous devices is commonplace for the treatment of simple lesions, such as atrial septal defect, patent foramen ovale, patent duct arteriosus, and abnormal vascular connections. There is also substantial experience with device closure of membranous and muscular ventricular septal defects, as well as more complex shunts such as baffle leaks after atrial switch repair and ventricular pseudoaneurysms. An increasing use of covered stents has improved the safety of aortic coarctation, conduit, and branch pulmonary stenosis interventions. Percutaneous pulmonary valve implantation now has an established role in the setting of dysfunctional right ventricle-pulmonary artery conduits or failing bioprosthetic pulmonary valves. Many patients remain unsuitable for percutaneous pulmonary valve implantation because of large diameter “native” outflow tracts, however, various techniques have emerged and multiple devices are in development to provide solutions for these unique anatomic challenges. Hybrid approaches involving use of surgical and transcatheter techniques are increasingly common, serving to optimize efficacy and safety of certain procedures; they depend on a collaborative and collegial relationship between cardiac surgeons and interventionalists that is primarily patient-centred.
Alors que de plus en plus de patients ayant une cardiopathie congénitale arrivent à l'âge adulte, le besoin d'interventions percutanées minimalement invasives se fait croissant, principalement pour minimiser le nombre de chirurgies répétées souvent requises par ces patients. L'utilisation de dispositifs percutanés est devenue fréquente pour le traitement des lésions simples, comme la communication interauriculaire, la persistance du foramen ovale, la persistance du canal artériel et les anastomoses vasculaires anormales. L'expérience concernant la fermeture des anomalies du septum membraneux et du septum musculaire par un dispositif, ainsi que les shunts plus complexes comme les fuites de la cloison après la détransposition auriculaire et les pseudoanévrismes ventriculaires est également non négligeable. L'utilisation croissante des endoprothèses recouvertes a amélioré l'innocuité des interventions liées à la coarctation de l'aorte, des sténoses du conduit et des branches pulmonaires. L'implantation percutanée d'une valvule pulmonaire a maintenant un rôle bien établi dans le cadre des conduits dysfonctionnels du ventricule droit et de l'artère pulmonaire défaillantes, ou des bioprothèses valvulaires pulmonaires dé faillantes. L'implantation percutanée d'une valvule pulmonaire demeure inappropriée pour plusieurs patients en raison des voies d'éjection « natives » de grand diamètre. Cependant, des techniques variées sont apparues, et de nombreux dispositifs sont en développement pour offrir des solutions à ces particularités anatomiques. Les approches hybrides avec l'utilisation de techniques chirurgicales et par cathéter sont de plus en plus fréquentes, et servent à optimiser l'efficacité et l'innocuité de certaines interventions; elles dépendent d'une relation collégiale et de collaboration entre les chirurgiens cardiaques et les cathétériseurs interventionnels, qui est principalement axée sur le patient.
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ReviewNew Directives in Cardiac Imaging: Imaging the Adult With Congenital Heart Disease

Abstract

Résumé

Section snippets

Echocardiography

ASDs

Future Directions in Cardiac Imaging

Acknowledgements

Can J Cardiol

J Heart Lung Transplant

Am J Cardiol

Am J Cardiol

J Am Soc Echocardiogr

J Cardiovasc Magn Reson

J Am Soc Echocardiogr

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Cardiovasc Magn Reson

J Thorac Cardiovasc Surg

JACC Cardiovasc Imaging

Can J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Am Coll Cardiol

J Am Coll Cardiol

Br Heart J

Am Heart J

J Cardiovasc Magn Reson

J Am Coll Cardiol

J Am Coll Cardiol

Chest

J Am Coll Cardiol

Am J Cardiol

J Am Coll Cardiol

Int J Cardiol

J Thorac Cardiovasc Surg

Review
New Directives in Cardiac Imaging: Imaging the Adult With Congenital Heart Disease