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�::.m�������Tj%%%%l�%,j�2R�%�n'n'n'n'n'n'n'K2M2M2M2M2M2M2$B4h�6�q29�)n'n'�)�)q2n'n'�2�0�0�0�)n'n'K2�0�)K2�0�0�0n'�%�q0p�%�-��0�1t�20�2�0j7�.�j7�0�0j7�08n'h�'J�0 (<\(Yn'n'n'q2q2u0n'n'n'�2�)�)�)�)jj�	��djj	�jjj����Multidetector computer tomography in congenital heart disease

Claudia Pujol Salvador 1, Julia Lemmer, MD 1, Farid Pouralikhan, MD 1, Michael P�rner, MD 2, Vasiliki Trigas, MD 1, Siegrun Mebus, MD 1, Stefan Martinoff, MD 2, Harald Kaemmerer, MD 1

1Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum M�nchen, Technische Universit�t M�nchen, M�nchen, Germany
2Institut f�r Radiologie und Nuklearmedizin, Deutsches Herzzentrum M�nchen, Technische Universit�t M�nchen, M�nchen, Germany

Short title: Computed tomography in congenital heart disease
Original research

Correspondence: 
Prof. Dr. Dr. Harald Kaemmerer, 
Deutsches Herzzentrum M�nchen, Department of Pediatric Cardiology and Congenital Heart Disease 
Phone:	+49-89-1218-3011
Fax:	+49-89-1218-3013
E-mail:	Kaemmerer@dhm.mhn.de

Total word count: 2763.


Objectives: Extracardiac pathology is common in patients with congenital heart disease and multidetector computer tomography can be a useful tool for detection. To date, there are only scarce reports regarding �incidental� non-cardiac findings during a cardiac computer tomography. The objectives of this study were to analyze indications, implementation, and timing of multidetector computer tomography in a congenital heart disease population, and to underline the necessity of a good cooperation between congenital cardiologists and radiologists. 
Materials and methods: All patients who underwent a multidetector computer tomography in a 32 month-period were enrolled in this single centre study. Type of congenital heart disease, timing of the procedure (preoperative, postoperative, follow-up, native), explored areas and indications were registered. 
Results: During 32 months, 250 scans were done in 195 patients. Mean age was 23,0 � 17,1 years (minimum 3 days, maximum 73 years); 48,2% were younger than 20 years. Almost 71% had undergone reparative cardiovascular surgery. Complex congenital heart disease (26,5%) and left-ventricular heart obstructions (22,5%) were the most common defects referred to computer tomography. Around 20% of patients underwent more than one scan. Postoperative/postinterventional (36%) and follow-up periods (30,4%) were most frequent timing for the scan. Most indications focused on the thorax (55,5%), particularly when cardiovascular complications were suspected. The assessment of cerebral pathology was the second most frequent indication in 25% of patients. Final diagnosis was achieved in 94% of cases. 
Conclusions: Extracardiac pathology is frequent in patients with congenital heart disease, especially in younger patients and with complex cardiac pathology. Multidetector computer tomography is a very useful tool when suspecting cardiovascular complications or cerebral pathology. Therefore, a good cooperation between radiologists and congenital cardiologists is mandatory. 
Key words: multidetector computer tomography, congenital heart disease, extracardiac pathology.
Abbreviations: CHD: congenital heart disease; CT: computer tomography; MDCT: multidetector computer tomography; MRI: magnetic resonance imaging

Introduction

Congenital heart diseases (CHD) are the most common type of birth defect. The overall incidence is about 75/1,000 live births. For the moderate and severe forms, the incidence of CHD is about 6/1,000 live births(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "ISSN" : "0735-1097", "PMID" : "12084585", "abstract" : "This study was designed to determine the reasons for the variability of the incidence of congenital heart disease (CHD), estimate its true value and provide data about the incidence of specific major forms of CHD. The incidence of CHD in different studies varies from about 4/1,000 to 50/1,000 live births. The relative frequency of different major forms of CHD also differs greatly from study to study. In addition, another 20/1,000 live births have bicuspid aortic valves, isolated anomalous lobar pulmonary veins or a silent patent ductus arteriosus. The incidences reported in 62 studies published after 1955 were examined. Attention was paid to the ways in which the studies were conducted, with special reference to the increased use of echocardiography in the neonatal nursery. The total incidence of CHD was related to the relative frequency of ventricular septal defects (VSDs), the most common type of CHD. The incidences of individual major forms of CHD were determined from 44 studies. The incidence of CHD depends primarily on the number of small VSDs included in the series, and this number in turn depends upon how early the diagnosis is made. If major forms of CHD are stratified into trivial, moderate and severe categories, the variation in incidence depends mainly on the number of trivial lesions included. The incidence of moderate and severe forms of CHD is about 6/1,000 live births (19/1,000 live births if the potentially serious bicuspid aortic valve is included), and of all forms increases to 75/1,000 live births if tiny muscular VSDs present at birth and other trivial lesions are included. Given the causes of variation, there is no evidence for differences in incidence in different countries or times.", "author" : [ { "dropping-particle" : "", "family" : "Hoffman", "given" : "Julien I E", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kaplan", "given" : "Samuel", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Journal of the American College of Cardiology", "id" : "ITEM-1", "issue" : "12", "issued" : { "date-parts" : [ [ "2002", "6", "19" ] ] }, "page" : "1890-900", "title" : "The incidence of congenital heart disease.", "type" : "article-journal", "volume" : "39" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=95c568cc-b836-41ad-8bf6-f296a5f10535" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>1</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }1). Advances in medical and surgical care have improved the survival of these patients(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1093/eurheartj/ehq249", "ISSN" : "1522-9645", "PMID" : "20801927", "author" : [ { "dropping-particle" : "", "family" : "Baumgartner", "given" : "Helmut", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Bonhoeffer", "given" : "Philipp", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Groot", "given" : "Natasja M S", "non-dropping-particle" : "De", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Haan", "given" : "Fokko", "non-dropping-particle" : "de", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Deanfield", "given" : "John Erik", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Galie", "given" : "Nazzareno", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gatzoulis", "given" : "Michael a", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gohlke-Baerwolf", "given" : "Christa", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kaemmerer", "given" : "Harald", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kilner", "given" : "Philip", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Meijboom", "given" : "Folkert", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Mulder", "given" : "Barbara J M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oechslin", "given" : "Erwin", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oliver", "given" : "Jose M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Serraf", "given" : "Alain", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Szatmari", "given" : "Andras", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Thaulow", "given" : "Erik", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Vouhe", "given" : "Pascal R", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Walma", "given" : "Edmond", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "European heart journal", "id" : "ITEM-1", "issue" : "23", "issued" : { "date-parts" : [ [ "2010", "12" ] ] }, "page" : "2915-57", "title" : "ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).", "type" : "article-journal", "volume" : "31" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=c37850c3-8f49-4d40-995a-6506caf8f2bf" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>2</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }2). At present, more than 80-85% of patients reach adulthood(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "ISSN" : "1468-201X", "PMID" : "12181200", "abstract" : "This report addresses the needs and problems of grown-up congenital heart (GUCH) patients and makes recommendations on organisation of national medical care, training of specialists, and education of the profession. The size of the national population of patients with grown-up congenital heart disease (GUCH) is uncertain, but since 80-85% of patients born with congenital heart disease now survive to adulthood (age 16 years), an annual increase of 2500 can be anticipated according to birth rate. Organisation of medical care is haphazard with only three of 18 cardiac surgical centres operating on over 30 cases per annum and only two established specialised units fully equipped and staffed. Not all grown-ups with congenital heart disease require the same level of expertise; 20-25% are complex, rare, etc, and require life long expert supervision and/or intervention; a further 35-40% require access to expert consultation. The rest, about 40%, have simple or cured diseases and need little or no specialist expertise. The size of the population needing expertise is small in comparison to coronary and hypertensive disease, aging, and increasing in complexity. It requires expert cardiac surgery and specialised medical cardiology, intensive care, electrophysiology, imaging and interventions, \"at risk\" pregnancy services, connection to transplant services familiar with their basic problem, clinical nurse specialist advisors, and trained nurses. 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It is estimated that there are about 2800 CHD-adults per 1 million population, with more than half of them having moderate or high complexity defects(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1093/eurheartj/ehq249", "ISSN" : "1522-9645", "PMID" : "20801927", "author" : [ { "dropping-particle" : "", "family" : "Baumgartner", "given" : "Helmut", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Bonhoeffer", "given" : "Philipp", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Groot", "given" : "Natasja M S", "non-dropping-particle" : "De", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Haan", "given" : "Fokko", "non-dropping-particle" : "de", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Deanfield", "given" : "John Erik", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Galie", "given" : "Nazzareno", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gatzoulis", "given" : "Michael a", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gohlke-Baerwolf", "given" : "Christa", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kaemmerer", "given" : "Harald", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kilner", "given" : "Philip", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Meijboom", "given" : "Folkert", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Mulder", "given" : "Barbara J M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oechslin", "given" : "Erwin", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oliver", "given" : "Jose M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Serraf", "given" : "Alain", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Szatmari", "given" : "Andras", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Thaulow", "given" : "Erik", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Vouhe", "given" : "Pascal R", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Walma", "given" : "Edmond", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "European heart journal", "id" : "ITEM-1", "issue" : "23", "issued" : { "date-parts" : [ [ "2010", "12" ] ] }, "page" : "2915-57", "title" : "ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).", "type" : "article-journal", "volume" : "31" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=c37850c3-8f49-4d40-995a-6506caf8f2bf" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>2</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }2). Because of complications, previous corrective surgery, and palliative surgery, up to 75% of these patients will require life-long follow-up(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1093/eurheartj/ehq249", "ISSN" : "1522-9645", "PMID" : "20801927", "author" : [ { "dropping-particle" : "", "family" : "Baumgartner", "given" : "Helmut", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Bonhoeffer", "given" : "Philipp", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Groot", "given" : "Natasja M S", "non-dropping-particle" : "De", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Haan", "given" : "Fokko", "non-dropping-particle" : "de", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Deanfield", "given" : "John Erik", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Galie", "given" : "Nazzareno", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gatzoulis", "given" : "Michael a", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gohlke-Baerwolf", "given" : "Christa", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kaemmerer", "given" : "Harald", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kilner", "given" : "Philip", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Meijboom", "given" : "Folkert", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Mulder", "given" : "Barbara J M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oechslin", "given" : "Erwin", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oliver", "given" : "Jose M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Serraf", "given" : "Alain", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Szatmari", "given" : "Andras", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Thaulow", "given" : "Erik", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Vouhe", "given" : "Pascal R", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Walma", "given" : "Edmond", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "European heart journal", "id" : "ITEM-1", "issue" : "23", "issued" : { "date-parts" : [ [ "2010", "12" ] ] }, "page" : "2915-57", "title" : "ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).", "type" : "article-journal", "volume" : "31" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=c37850c3-8f49-4d40-995a-6506caf8f2bf" ] }, { "id" : "ITEM-2", "itemData" : { "ISSN" : "1468-201X", "PMID" : "12181200", "abstract" : "This report addresses the needs and problems of grown-up congenital heart (GUCH) patients and makes recommendations on organisation of national medical care, training of specialists, and education of the profession. 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It requires expert cardiac surgery and specialised medical cardiology, intensive care, electrophysiology, imaging and interventions, \"at risk\" pregnancy services, connection to transplant services familiar with their basic problem, clinical nurse specialist advisors, and trained nurses. An integrated national service is described with 4-6 specialist units established within adult cardiology, ideally in relation or proximity to university hospital/departments in appropriate geographic location, based in association with established paediatric cardiac surgical centres with designated inpatient and outpatient facilities for grown-up patients with congenital heart disease. Specialist units should accept responsibility for educating the profession, training the specialists, cooperative research, receiving patients \"out of region\", sharing particular skills between each other, and they must liaise with other services and trusts in the health service, particularly specified outpatient clinics in district and regional centres. Not every regional cardiac centre requires a full GUCH specialised service since there are too few patients. Complex patients need to be concentrated for expertise, experience, and optimal management. 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Cardiac imaging is the cornerstone for the diagnosis, pre- and postoperative evaluation and follow-up of patients with CHD. Besides echocardiography and cardiac catheterization, magnetic resonance imaging (MRI) and computer tomography (CT) are gaining importance. These techniques do not depend on acoustic window, permit the depiction of extracardiac structures and 3D reconstruction(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "author" : [ { "dropping-particle" : "", "family" : "Leschka", "given" : "Sebastian", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Oechslin", "given" : "Erwin", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Husmann", "given" : "Lars", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Desbiolles", "given" : "Lotus", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Marincek", "given" : "Borut", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Genoni", "given" : "Michele", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Pr\u00eate", "given" : "Ren\u00e9", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Jenni", "given" : "Rolf", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Wildermuth", "given" : "Simon", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Alkadhi", "given" : "Hatem", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "RadioGraphics", "id" : "ITEM-1", "issued" : { "date-parts" : [ [ "2007" ] ] }, "page" : "829-847", "title" : "Pre- and Postoperative Evaluation of Con- genital Heart Disease in Children and Adults with 64-Section CT", "type" : "article-journal" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=6379e3a4-ba7a-405b-974c-e26e0fe56679" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>4</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }4). 

Multidetector computer tomography (MDCT) provides great resolution imaging(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1093/eurheartj/ehm544", "ISSN" : "0195-668X", "PMID" : "18084017", "abstract" : "As a consequence of improved technology, there is growing clinical interest in the use of multi-detector row computed tomography (MDCT) for non-invasive coronary angiography. Indeed, the accuracy of MDCT to detect or exclude coronary artery stenoses has been high in many published studies. This report of a Writing Group deployed by the Working Group Nuclear Cardiology and Cardiac CT (WG 5) of the European Society of Cardiology and the European Council of Nuclear Cardiology summarizes the present state of cardiac CT technology, as well as the currently available data concerning its accuracy and applicability in certain clinical situations. Besides coronary CT angiography, the use of CT for the assessment of cardiac morphology and function, evaluation of perfusion and viability, and analysis of heart valves is discussed. 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MDCT provides, on one side, excellent representation of cardiovascular anatomic structures in patients with CHD(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.2214/AJR.08.2192", "ISSN" : "1546-3141", "PMID" : "19620435", "abstract" : "OBJECTIVE: The purpose of this article is to describe the spectrum of imaging findings of congenital heart disease in adults.\n\nCONCLUSION: Continued advances in CT have facilitated evaluation of two important patient populations: adults with surgically palliated congenital heart disease and adults with previously undiagnosed congenital heart disease.", "author" : [ { "dropping-particle" : "", "family" : "Wiant", "given" : "Amanda", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Nyberg", "given" : "Eric", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gilkeson", "given" : "Robert C", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "AJR. 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It enables to assess systemic and pulmonary circulation, coronary artery blood flow, collateral circulation and shunt status(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.5603/FM.2013.0032", "ISSN" : "1644-3284", "author" : [ { "dropping-particle" : "", "family" : "Bayraktutan", "given" : "U.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kantarci", "given" : "M.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Ogul", "given" : "H.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kizrak", "given" : "Y.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Pirimoglu", "given" : "B.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Genc", "given" : "B.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Yuceler", "given" : "Z.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Ceviz", "given" : "N.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Folia Morphologica", "id" : "ITEM-1", "issue" : "3", "issued" : { "date-parts" : [ [ "2013", "9", "5" ] ] }, "page" : "188-196", "title" : "The utility of multidetector computed tomography for evaluation of congenital heart disease", "type" : "article-journal", "volume" : "72" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=f5834979-6341-49ca-8318-5e365a2dea1b" ] }, { "id" : "ITEM-2", "itemData" : { "DOI" : "10.2214/AJR.07.2889", "ISSN" : "1546-3141", "PMID" : "18716119", "abstract" : "OBJECTIVE: The purpose of this article is to review the CT appearance of postoperative morphology and complications after surgical correction of congenital heart anomalies.\n\nCONCLUSION: Echocardiography is typically the initial imaging technique used for congenital heart disease; however, some thoracic regions are beyond the imaging scope of echocardiography, particularly after surgical revision. This article shows, through a series of illustrative cases, the usefulness of 64-MDCT in these patients.", "author" : [ { "dropping-particle" : "", "family" : "Spevak", "given" : "Philip J", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Johnson", "given" : "Pamela T", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Fishman", "given" : "Elliot K", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "AJR. American journal of roentgenology", "id" : "ITEM-2", "issue" : "3", "issued" : { "date-parts" : [ [ "2008", "9" ] ] }, "page" : "854-61", "title" : "Surgically corrected congenital heart disease: utility of 64-MDCT.", "type" : "article-journal", "volume" : "191" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=5a636c7a-7758-4d09-8ad1-2414a482161e" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>6,10</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }6,10). On the other side, CHD patients often suffer from extracardiac complications that can require MDCT. 

The purpose of this study was to investigate MDCT for the diagnosis of extracardiac pathology in patients with CHD, and to underscore the importance of cooperation between radiologists and cardiologists. It provides useful information about the frequency and type of CHD that require MDCT, the explored regions and most common indications. It also informs about technical information such as need of contrast medium and number of procedures required to achieve a final diagnosis. 


Materials and methods

Study design
We retrospectively analyzed 195 consecutive patients who underwent an MDCT in our centre. MDCT was indicated according to clinical symptoms, when other imaging techniques were not enough to achieve a diagnosis or were contraindicated (for example, MRI and pacemaker). Patients with contrast medium allergy, orthopnoea and renal failure were excluded. Informed consent was mandatory. 
CHD was classified into native, reparative surgery, palliative surgery and interventionally treated. Number of surgical or interventional procedures was also analyzed. Type of CHD was divided into 8 groups: complex heart anomalies (like transposition of the great arteries, truncus arteriosus communis; groups 1-3), outflow tract obstructions (right or left sided; groups 4-5), shunts (pretricuspid, postricuspid; groups 6-7) and others (group 8). 
Timing of MDCT (native, preoperative, postoperative, long-term follow-up) and indications were reflected. 
The explored regions were divided into 6 groups: thorax, cranium, abdomen, sternum, neck and extremities. The need of contrast medium was also noticed. 

Technical considerations
MSCT was performed with the patient in supine position, using a 128-slices CT (Dual-Source CT, SOMATOM Definition, Siemens AG, Forchheim, Germany). Iomeprol (Imeron �, Altana, Konstanz, Germany) was used as contrast medium whenever necessary. Application- and flow-rate were adjusted according to patient's age and weight. Application was made - especially in children - by hand or with a syringe pumps (Injektron CT2, Medtron, Saarbr�cken, Germany). After contrast administration, an isotonic sodium chloride solution (Delta Select �, Delta Select GmbH, Pfullingen, Germany) was applied. Prospective ECG-triggering was used to reduce X-ray time. Beta-blockers (bisoprolol) were used to reduce heart rate in tachyarrhythmic patients. The objective was to achieve a heart rate of 65 bpm. 
All source images were interpreted and postprocessed on a workstation. Axial source, multiplanar reconstruction, maximum intensity projection, and volume-rendered images were used for the assessment. The images were analysed by a radiologist and a paediatric cardiologist with training in radiology for more than 5 years.

Statistical analysis
Data are presented numerically, as percentages or as means � standard deviation (SD). Data were processed using SPSS 15.0 (SPSS Inc, Chicago, IL).


Results

During 32 months, 195 patients (111 men and 84 women) underwent 250 MDCT. Mean age was 23,0 � 17,1 years (minimum 3 days, maximum 73 years); 48,2% were younger than 20 years (figure 1). 
Up to 31(15,9%) patients had a native CHD. In 138 (70,8%) patients a reparative surgery was done, 13 (6,7%) patients underwent palliative surgery and 13 (6,7%) underwent an interventional procedure. The number of surgeries were: one in 61 (31,3%) patients, two in 34 (17,4%),  three in 25 (12,8%), four in 18 (9,2%), five in 11 (5,6%) and six ore more in 15 (7,7%) patients. 
Patients were classified into 8 diagnostic groups: 51 (25,6%) had a complex CHD (groups 1-3), 44 (22,5%) had a left heart obstruction (group 4), 36 (18,4%) a right heart obstruction (group 5), 22 (11,3%) postricuspidal shunts (group 6), 18 (9,2%) pretricuspid shunts (group 7) and 24 (12.3%) had other types of CHD (group 8). The results are given in table 1. 
Only one MDCT was required in 157 (80,5%) patients, two scans in 29 (14,9%) patients, three in four (2,1%) patients, four in two (1,0%) patients and five in three (1,5%) patients. 
Timing of MDCT is reflected in figure 2. Thorax was the most explored region (n= 172, 55,5%), followed by cranium (n= 72; 23,2%), abdomen (n= 44; 14,2%), sternum (n= 13; 4,2%), neck (n= 6; 1,9%) and extremities (n=3; 1,0%). 
Indications for MDCT were: diagnosis/exclusion of pulmonary embolism (19,3%), cerebral symptoms (26,6%), symptoms around sternotomy (8%), diagnosis/exclusion of aortic aneurysm or dissection (14,6%), inflammation (18%), choanal atresia (2%), haemoptysis (2,6%), vascular anomalies (2%) and others (6,7%). A final diagnosis was achieved in 94% of cases. 
Intravenous contrast medium was used in 208 (83,2%) scans. Four patients required oral contrast medium as well, and in one patient it was also administrated through a cutaneous fistula. No allergic reactions were reported. 


Discussion

This study shows that MDCT plays a crucial role in the diagnosis and assessment of extracardiac structures and complications in patients with CHD. The most frequent CHD that require MDCT are complex CHD and left heart obstruction pathologies. More than half of indications concentrate in the thorax region, particularly in the diagnosis/exclusion of vascular complications or anomalies. Immediate postoperative/postinterventional period and long-term follow-up are the most frequent timing of MDCT. Contrast medium is required in most cases.  
Besides cardiac illness, this study shows that patients with CHD present frequently with extracardiovascualr pathology. Earlier reports concentrated on the anatomical depiction of CHD and informed about extracardiovascular lesions as incidental diagnosis. Concerning this, Bayraktutan reported 26% of extracardiovascular lesions in a cohort of 105 patients with CHD. The most common diagnosis were pneumonia and pulmonary oedema(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.5603/FM.2013.0032", "ISSN" : "1644-3284", "author" : [ { "dropping-particle" : "", "family" : "Bayraktutan", "given" : "U.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kantarci", "given" : "M.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Ogul", "given" : "H.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Kizrak", "given" : "Y.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Pirimoglu", "given" : "B.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Genc", "given" : "B.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Yuceler", "given" : "Z.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Ceviz", "given" : "N.", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Folia Morphologica", "id" : "ITEM-1", "issue" : "3", "issued" : { "date-parts" : [ [ "2013", "9", "5" ] ] }, "page" : "188-196", "title" : "The utility of multidetector computed tomography for evaluation of congenital heart disease", "type" : "article-journal", "volume" : "72" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=f5834979-6341-49ca-8318-5e365a2dea1b" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>6</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }6). 
The present study show that, even though the most explored region was the thorax in 55,5%, there were non-negligible cerebral symptoms (almost 25%) that required MDCT. Common indications were also vascular complications (suspected pulmonary embolism, aneurysm/dissection, vascular anomalies, and haemoptysis) and cerebral symptoms. Inflammation was the third cause of MDCT in 18%. Another factor contributing to the frequency of thoracic MDCT is probably the fact that 36% of the scans were done in the immediate postoperative period. 

MDCT has several advantages. First of all, it permits to acquire high-definition images in a very short time. Furthermore, it allows defining vascular structures of 1-2 mm(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1055/s-2000-8344", "ISSN" : "0171-6425", "PMID" : "11145399", "abstract" : "Management of patients with congenital heart disease requires detailed information on cardiac and great vessel morphology. In previous years, the diagnosis and the treatment of congenital malformations has often depended on cardiac catherization, and in many institutions, cardiac catherization still remains the gold standard against which other modalities are measured. In the past decade, however, imaging methodologies have increasingly shifted toward the use of less invasive and noninvasive techniques. Currently, echocardiography is the initial method of choice in evaluating the anatomy, especially in younger patients. Meanwhile, several newer imaging techniques like magnetic resonance imaging (MRI) and spiral or multislice computed tomography (CT) are in use. They offer extremely useful information about abnormalities of the heart and great vessels as well as the assessment of cardiac anatomy and function. Echo, angiography, MRI and CT should be seen as complimentary investigations in adult congenital heart disease.", "author" : [ { "dropping-particle" : "", "family" : "Kaemmerer", "given" : "H", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Stern", "given" : "H", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Fratz", "given" : "S", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Prokop", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Schwaiger", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Hess", "given" : "J", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "The Thoracic and cardiovascular surgeon", "id" : "ITEM-1", "issue" : "6", "issued" : { "date-parts" : [ [ "2000", "12" ] ] }, "page" : "328-35", "title" : "Imaging in adults with congenital cardiac disease (ACCD).", "type" : "article-journal", "volume" : "48" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=c39140b2-41ba-466d-8304-59c995e450a0" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>11</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }11). In cardiology, MDCT is the non-invasive method of choice for the evaluation of coronary anomalies and coronary artery disease(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1016/j.ijcard.2006.07.074", "ISSN" : "1874-1754", "PMID" : "17055084", "abstract" : "PURPOSE: Adults with congenital heart disease (CHD) form a growing population of patients requiring coronary artery assessment. In light of their increased incidence of coronary anomalies and need for reoperations, it is important to define their coronary anatomy. The purpose of this study was to define the spectrum of coronary artery anatomies and feasibility of coronary artery delineation in adults with CHD using 16- and 64-slice computed tomography angiography.\n\nMATERIALS AND METHODS: Coronary multislice computed tomography (MSCT) examinations performed in adults with CHD between 2004 and 2005 were reviewed with institutional review board approval. Informed consent was not required. In addition to image quality, the following coronary parameters were evaluated by analysis of axial, multiplanar reformatted, and volume-rendered images: (1) relationship to great arteries, (2) ostia number and location, (3) proximal course, (4) length of vessel seen, and (5) dominance.\n\nRESULTS: Eighty-five examinations in adults with CHD adults were identified. The relationship of the great vessels, number and location of the coronary ostia, and proximal course of the coronaries could be identified in all studies. Coronary anomalies were detected in 16 (19%) subjects. The entire coronary tree was visible in 77 (91%) of subjects.\n\nCONCLUSIONS: MSCT provides detailed, noninvasive assessment of complex coronary artery anatomy in adults with CHD. MSCT should be considered a useful modality in the assessment of these patients, as it does not require arterial access and provides detailed 3-dimensional anatomic information that has important implications for long-term management.", "author" : [ { "dropping-particle" : "", "family" : "Cook", "given" : "Stephen C", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "V", "family" : "Raman", "given" : "Subha", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "International journal of cardiology", "id" : "ITEM-1", "issue" : "1", "issued" : { "date-parts" : [ [ "2007", "6", "25" ] ] }, "page" : "101-6", "title" : "Unique application of multislice computed tomography in adults with congenital heart disease.", "type" : "article-journal", "volume" : "119" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=2fa4ac1b-7e21-4d86-b6b6-7149da06392c" ] }, { "id" : "ITEM-2", "itemData" : { "DOI" : "10.1016/j.crad.2005.06.011", "ISSN" : "0009-9260", "PMID" : "16291307", "abstract" : "Acute aortic syndromes (AAS) encompass a spectrum of emergencies. These include those non-traumatic disease entities of the aorta namely, penetrating atherosclerotic ulcer, intramural haematoma, dissection and aneurysm rupture. The various types of AAS cannot be reliably differentiated on clinical grounds alone. Acute thoracic aortic injury is usually included in this group even though clinical presentation is different, i.e., in the context of trauma, the imaging features are very similar. Differentiation of AAS from acute coronary syndrome (ACS) is important, however, it must be remembered that ACS may occur as a result of AAS. Now electrocardiogram (ECG)-gating technology is widely available, ECG-gated multi-detector row computed tomography (MDCT) is a powerful clinical tool in the acute emergency setting, which enables rapid and specific diagnosis of aortic pathology. ECG-gated MDCT significantly reduces motion artefact, avoids potential pitfalls in diagnosis and often provides diagnostic information about the coronary arteries. It should be used as a first-line imaging technique. This article examines the role of MDCT imaging and cardiac gating in the assessment of AAS and discusses the differentiation of this spectrum of aortic diseases with reference to the key imaging findings as obtained by experience in our institution.", "author" : [ { "dropping-particle" : "", "family" : "Manghat", "given" : "N E", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Morgan-Hughes", "given" : "G J", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Roobottom", "given" : "C a", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Clinical radiology", "id" : "ITEM-2", "issue" : "12", "issued" : { "date-parts" : [ [ "2005", "12" ] ] }, "page" : "1256-67", "title" : "Multi-detector row computed tomography: imaging in acute aortic syndrome.", "type" : "article-journal", "volume" : "60" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=3d4eee63-6016-485d-9837-850c6832d16f" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>12,13</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }12,13). Some studies pointed out that MDCT is also valid for the study of shunt-size, location and flow direction. It is a method of choice in patients with metallic devices and pacemakers(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1055/s-2000-8344", "ISSN" : "0171-6425", "PMID" : "11145399", "abstract" : "Management of patients with congenital heart disease requires detailed information on cardiac and great vessel morphology. In previous years, the diagnosis and the treatment of congenital malformations has often depended on cardiac catherization, and in many institutions, cardiac catherization still remains the gold standard against which other modalities are measured. In the past decade, however, imaging methodologies have increasingly shifted toward the use of less invasive and noninvasive techniques. Currently, echocardiography is the initial method of choice in evaluating the anatomy, especially in younger patients. Meanwhile, several newer imaging techniques like magnetic resonance imaging (MRI) and spiral or multislice computed tomography (CT) are in use. They offer extremely useful information about abnormalities of the heart and great vessels as well as the assessment of cardiac anatomy and function. Echo, angiography, MRI and CT should be seen as complimentary investigations in adult congenital heart disease.", "author" : [ { "dropping-particle" : "", "family" : "Kaemmerer", "given" : "H", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Stern", "given" : "H", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Fratz", "given" : "S", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Prokop", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Schwaiger", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Hess", "given" : "J", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "The Thoracic and cardiovascular surgeon", "id" : "ITEM-1", "issue" : "6", "issued" : { "date-parts" : [ [ "2000", "12" ] ] }, "page" : "328-35", "title" : "Imaging in adults with congenital cardiac disease (ACCD).", "type" : "article-journal", "volume" : "48" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=c39140b2-41ba-466d-8304-59c995e450a0" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>11</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }11). MDCT also allows the analyses of cardiac dimensions and function(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1136/hrt.2006.104729", "ISSN" : "1468-201X", "PMID" : "18003697", "author" : [ { "dropping-particle" : "", "family" : "Crean", "given" : "Andrew", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Heart (British Cardiac Society)", "id" : "ITEM-1", "issue" : "12", "issued" : { "date-parts" : [ [ "2007", "12" ] ] }, "page" : "1637-47", "title" : "Cardiovascular MR and CT in congenital heart disease.", "type" : "article-journal", "volume" : "93" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=016c7c73-8e7e-4976-9739-70f2bf79613f" ] }, { "id" : "ITEM-2", "itemData" : { "DOI" : "10.1016/j.crad.2007.01.003", "ISSN" : "0009-9260", "PMID" : "17467388", "abstract" : "Adult congenital heart disease is an increasingly prevalent condition with more than 135,000 patients affected in England alone. With this increased patient population and an increase in interventional procedures being performed on them, traditional imaging techniques such as cardiac magnetic resonance (CMR) may be unavailable locally or contra-indicated. Cardiac multidetector computed tomography (MDCT) is rapidly emerging as an alternative imaging method for the investigation of these patients and this review highlights the broad application of cardiac MDCT to this population and makes recommendations on the standardized reporting of complex congenital heart disease.", "author" : [ { "dropping-particle" : "", "family" : "Nicol", "given" : "E D", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gatzoulis", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Padley", "given" : "S P G", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Rubens", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Clinical radiology", "id" : "ITEM-2", "issue" : "6", "issued" : { "date-parts" : [ [ "2007", "6" ] ] }, "page" : "518-27", "title" : "Assessment of adult congenital heart disease with multi-detector computed tomography: beyond coronary lumenography.", "type" : "article-journal", "volume" : "62" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=1473a30e-d21f-4d49-a1f3-0e4682f03bd0" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>14,15</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }14,15), with less definition for segmental contraction compared to MRI. Function of prosthetic valves can also be assessed(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1016/j.crad.2007.01.003", "ISSN" : "0009-9260", "PMID" : "17467388", "abstract" : "Adult congenital heart disease is an increasingly prevalent condition with more than 135,000 patients affected in England alone. With this increased patient population and an increase in interventional procedures being performed on them, traditional imaging techniques such as cardiac magnetic resonance (CMR) may be unavailable locally or contra-indicated. Cardiac multidetector computed tomography (MDCT) is rapidly emerging as an alternative imaging method for the investigation of these patients and this review highlights the broad application of cardiac MDCT to this population and makes recommendations on the standardized reporting of complex congenital heart disease.", "author" : [ { "dropping-particle" : "", "family" : "Nicol", "given" : "E D", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Gatzoulis", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Padley", "given" : "S P G", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Rubens", "given" : "M", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Clinical radiology", "id" : "ITEM-1", "issue" : "6", "issued" : { "date-parts" : [ [ "2007", "6" ] ] }, "page" : "518-27", "title" : "Assessment of adult congenital heart disease with multi-detector computed tomography: beyond coronary lumenography.", "type" : "article-journal", "volume" : "62" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=1473a30e-d21f-4d49-a1f3-0e4682f03bd0" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>15</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }15). 
However, MDCT present some disadvantages. The most important are radiation and use of contrast medium. In patients with CHD, in which frequently imaging techniques have to be used, can be an important drawback. In this study, almost 20% of patients underwent 2 or more procedures. 
Regarding radiation, a dose of 1 mSv per year leads to a lifetime probability of fatal cancer of 4�5 new cases among 100,000 patients exposed. Hoffman et al.(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1016/j.ijcard.2006.07.012", "ISSN" : "1874-1754", "PMID" : "16997400", "abstract" : "BACKGROUND: Adult patients with congenital heart disease under follow-up often need to undergo diagnostic procedures which expose them to radiation.\n\nAIM: To evaluate radiation doses in adult patients with congenital heart disease during follow-up.\n\nMETHODS: Data on diagnostic procedures were used from the European Heart Survey on adult congenital heart disease, a multicenter retrospective cohort study. Lesions included in the survey were Atrial Septal Defect, Ventricular Septal Defect, Fallot, Fontan, Coarctation, Transposition of the Great Arteries, Marfan, and Cyanotic lesions. A total of 4110 patients (52% female) with a mean age of 32 years (range 17-85) were included. The follow-up time ranged from 0 to 72 months, with a median of 61 months. There were a total of 18,403 patient-years of follow-up. During this time, a mean of 4.5 visits per patient took place. Radiation doses were calculated using the number of examinations in each patient's data file. Effective radiation doses are given in millisievert (mSv).\n\nRESULTS: The average cumulative annual effective dose per patient was 0.46 mSv. The relative contributions to these doses were 3% by chest X-rays, 39% by computed tomography scans, 42% by angiography, and 16% by nuclear scans. Effective doses were higher in patients with Fontan, Coarctation, Marfan and Cyanotic lesions, as well as in patients with Atrial Septal Defects.\n\nCONCLUSIONS: Exposure to radiation during follow-up of patients with adult congenital heart disease mainly stems from computed tomography scans and angiography. Patients with Fontan, Coarctation, Marfan and Cyanotic lesions are more likely to get high doses from computed tomography. In these lesions, therefore, particular care should be taken to use non-ionizing imaging procedures whenever possible.", "author" : [ { "dropping-particle" : "", "family" : "Hoffmann", "given" : "Andreas", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Engelfriet", "given" : "Peter", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Mulder", "given" : "Barbara", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "International journal of cardiology", "id" : "ITEM-1", "issue" : "2", "issued" : { "date-parts" : [ [ "2007", "5", "31" ] ] }, "page" : "151-3", "title" : "Radiation exposure during follow-up of adults with congenital heart disease.", "type" : "article-journal", "volume" : "118" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=7ad14755-569f-4d02-a898-3fe5aecfde2d" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>16</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }16) analysed, in the multicentric study �European Heart Survey on adult congenital heart disease�, the cumulative radiation dose in patients with CHD. The average cumulative effective dose per patient was 0,46 mSv. CT-scans contributed in 39% to radiation, being the second source of radiation behind angiography. Furthermore, patients with higher radiation were those who more often needed controls of the aorta, like coarctation of the aorta and Marfan syndrome(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1016/j.ijcard.2006.07.012", "ISSN" : "1874-1754", "PMID" : "16997400", "abstract" : "BACKGROUND: Adult patients with congenital heart disease under follow-up often need to undergo diagnostic procedures which expose them to radiation.\n\nAIM: To evaluate radiation doses in adult patients with congenital heart disease during follow-up.\n\nMETHODS: Data on diagnostic procedures were used from the European Heart Survey on adult congenital heart disease, a multicenter retrospective cohort study. Lesions included in the survey were Atrial Septal Defect, Ventricular Septal Defect, Fallot, Fontan, Coarctation, Transposition of the Great Arteries, Marfan, and Cyanotic lesions. A total of 4110 patients (52% female) with a mean age of 32 years (range 17-85) were included. The follow-up time ranged from 0 to 72 months, with a median of 61 months. There were a total of 18,403 patient-years of follow-up. During this time, a mean of 4.5 visits per patient took place. Radiation doses were calculated using the number of examinations in each patient's data file. Effective radiation doses are given in millisievert (mSv).\n\nRESULTS: The average cumulative annual effective dose per patient was 0.46 mSv. The relative contributions to these doses were 3% by chest X-rays, 39% by computed tomography scans, 42% by angiography, and 16% by nuclear scans. Effective doses were higher in patients with Fontan, Coarctation, Marfan and Cyanotic lesions, as well as in patients with Atrial Septal Defects.\n\nCONCLUSIONS: Exposure to radiation during follow-up of patients with adult congenital heart disease mainly stems from computed tomography scans and angiography. Patients with Fontan, Coarctation, Marfan and Cyanotic lesions are more likely to get high doses from computed tomography. In these lesions, therefore, particular care should be taken to use non-ionizing imaging procedures whenever possible.", "author" : [ { "dropping-particle" : "", "family" : "Hoffmann", "given" : "Andreas", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Engelfriet", "given" : "Peter", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Mulder", "given" : "Barbara", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "International journal of cardiology", "id" : "ITEM-1", "issue" : "2", "issued" : { "date-parts" : [ [ "2007", "5", "31" ] ] }, "page" : "151-3", "title" : "Radiation exposure during follow-up of adults with congenital heart disease.", "type" : "article-journal", "volume" : "118" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=7ad14755-569f-4d02-a898-3fe5aecfde2d" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>16</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }16). Taking into account that almost half of the patients of this study were younger than 20 years, optimisation of the procedure (like ECG trigger) and the lowest required radiation should be used. 

Since non-invasive techniques provide great anatomical and functional accuracy, their use has increased in the last decades. Prakash et al. pointed out that these techniques are growing while diagnostic catheterisation decreases. On the other side, interventional catheterisation shows a steady increase and surgery increases modestly(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1161/CIRCIMAGING.109.875021", "ISSN" : "1942-0080", "PMID" : "20086225", "author" : [ { "dropping-particle" : "", "family" : "Prakash", "given" : "Ashwin", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Powell", "given" : "Andrew J", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Geva", "given" : "Tal", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Circulation. Cardiovascular imaging", "id" : "ITEM-1", "issue" : "1", "issued" : { "date-parts" : [ [ "2010", "1" ] ] }, "page" : "112-25", "title" : "Multimodality noninvasive imaging for assessment of congenital heart disease.", "type" : "article-journal", "volume" : "3" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=16b61690-51ff-4c1c-b9a8-cf7a6395d801" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>17</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }17). 

The results of this study show that MDCT should be available in tertiary centres with a specific department for CHD. Because of the often non cardiac diagnosis in CHD, a strong cooperation between congenital cardiologists and radiologists is mandatory. While the first ones focus on cardiac anatomy(ADDIN CSL_CITATION { "citationItems" : [ { "id" : "ITEM-1", "itemData" : { "DOI" : "10.1002/ccd.20551", "ISSN" : "1522-1946", "PMID" : "16342271", "abstract" : "The objective of our study was to determine the contrast attenuation level that yields high quality cardiac three-dimensional (3-D) images and to predict the contrast injection rate (IR), from body weight, to reach this attenuation level. Enhanced electron beam computerized tomography (EBCT) with 3-D reconstruction is useful in delineating cardiac anatomy in complex congenital heart disease (CHD). The current experience of using electron beam angiography (EBA) in pediatric CHD is limited. Well-defined contrast injection protocols, specifically the contrast IR, have not been standardized when compared to those for adults. Establishing the contrast IR is essential in obtaining high quality 3-D images. We retrospectively analyzed the studies of 115 pediatric patients with CHD. EBA images were divided into group 1 with good quality 3-D images and group 2 with poor quality. The mean of measured enhancement level, expressed in Hounsfield units (HU), and contrast IR were analyzed in both groups. Spearman correlation was used to examine the relationship between weight and IR. The IR was predicted from weight using simple linear regression analysis. The mean level of enhancement was 344 +/- 91 and 174 +/- 31 HU for group 1 and group 2, respectively. Group 1 consisted of 103 patients (90%) and the IR strongly correlated with weight (rho = 0.861, P < 0.01). The IR was estimated from the linear regression equation IR = 0.59 + 0.056 x weight. Necessary contrast enhancement level for quality 3-D reconstruction should be greater than 250 HU, and the IR can be estimated from patient's weight.", "author" : [ { "dropping-particle" : "", "family" : "Jelnin", "given" : "Vladimir", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Co", "given" : "Jennifer", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Muneer", "given" : "Basharat", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Swaminathan", "given" : "Balasubramanian", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Toska", "given" : "Suzanna", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" }, { "dropping-particle" : "", "family" : "Ruiz", "given" : "Carlos E", "non-dropping-particle" : "", "parse-names" : false, "suffix" : "" } ], "container-title" : "Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions", "id" : "ITEM-1", "issue" : "1", "issued" : { "date-parts" : [ [ "2006", "1" ] ] }, "page" : "120-6", "title" : "Three dimensional CT angiography for patients with congenital heart disease: scanning protocol for pediatric patients.", "type" : "article-journal", "volume" : "67" }, "uris" : [ "http://www.mendeley.com/documents/?uuid=394d42ef-9fa3-4955-8a74-2203f2cf5dc9" ] } ], "mendeley" : { "previouslyFormattedCitation" : "<sup>18</sup>" }, "properties" : { "noteIndex" : 0 }, "schema" : "https://github.com/citation-style-language/schema/raw/master/csl-citation.json" }18), the seconds will provide a meaningful overview of extracardiac structures. Radiologists should also have a background in CHD in order to avoid misdiagnoses. 

Limitations

This study has several limitations that must be taken into account. First of all, it should be interpreted in light of the limitations imposed by the retrospective and descriptive nature of the study design; hence, selection of the study patients was not random. We did not compare the results with other techniques such as cardiac catheterisation or MRI. Furthermore, most indications for MDCT were based on clinical findings. We cannot exclude extracardiac diagnosis in asymptomatic patients. 
On the other hand, final diagnosis management after doing an MDCT was not reflected and the therapeutic consequences of MDCT are not clarified.  


Conclusions

Extracardiac pathology is frequent in patients with CHD, especially in younger patients and with complex cardiac pathology. MDCT is a fundamental tool when suspecting vascular complications or cerebral pathology and should be available in all tertiary centres with a department of congenital heart disease. 
Because extracardiac findings are frequent in CHD, a strong cooperation between radiologists and congenital cardiologists is mandatory.

AKNOWLEDGEMENT: 
Claudia Pujol received a grant of the Spanish Society of Cardiology for investigation in foreign countries. 

BIBLIOGRAPHY

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Table 1: Diagnostic subgroups and number of patients included

Figure 1: Distribution of MDCT according to age and gender.

Figure 2: Timing and demands of MDCT-studies in the course of the congenital heart anomaly
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