Diane E Spicer

University of Florida, Gainesville, Florida, United States

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Publications (34)42.35 Total impact

  • Cardiology in the Young 04/2015; 25(4):1. DOI:10.1017/S1047951114002649 · 0.86 Impact Factor
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    ABSTRACT: In the normal individual, the parietal components of the body are mirror-imaged and appropriately described as isomeric. The thoraco-abdominal organs, in contrast, are lateralized. However, in "visceral heterotaxy," the thoraco-abdominal organs also show some degree of isomerism, best seen in the arrangement of the bronchial tree. Whether isomerism can be found within the heart remains controversial. One of two recent publications in this journal emphasized the crucial features of bronchial isomerism; the other, in contrast, confused the situation of isomerism within the heart. In this review, we show how the topic of cardiac isomerism is clarified by concentrating on the anatomical features of the cardiac components and determining how best they can be described. Appropriate manipulation of developing mice produces unequivocal evidence of isomerism of the atrial appendages, but with no evidence of ventricular isomerism. In hearts from patients with so-called "heterotaxy," only the atrial appendages, distinguished on the basis of the pectinate muscles lining their walls, are uniformly isomeric, permitting the syndrome to be differentiated into the subsets of left as opposed to right atrial appendage isomerism. Thus, controversies are defused by simply describing the isomerism of the atrial appendages rather than "atrial isomerism," recognizing the frequency of abnormal venoatrial connections in these settings. Any suggestion of ambiguity is removed by the equally simple expedient of describing all the variable cardiac features, describing the arrangements of the thoracic and abdominal organs separately should there be discordances. Clin. Anat., 2015. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.
    Clinical Anatomy 03/2015; 28(4). DOI:10.1002/ca.22517 · 1.16 Impact Factor
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    ABSTRACT: We have re-investigated an unusual cardiac specimen with juxtaposition of the atrial appendages. The original description dates to 1962, when the autopsy was performed at the Children's Memorial Hospital in Chicago, now Ann & Robert H. Lurie Children's Hospital of Chicago. The heart was subsequently stored in the Farouk S. Idriss Cardiac Registry at the same institution. The specimen shows usual atrial arrangement, but with the morphologically left appendage juxtaposed in a rightward manner, passing behind the heart rather than through the transverse sinus so as to reach its location inferior to the morphologically right appendage. The heart also demonstrated an inter-atrial communication between the cavities of the juxtaposed left appendage and the morphologically right atrium. We provide a detailed description of the morphology, and provide images of this lesion, which to the best of our knowledge has not previously been described.
    Cardiology in the Young 03/2015; DOI:10.1017/S1047951115000165 · 0.86 Impact Factor
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    ABSTRACT: Background Ventricular septal defects are the commonest congenital cardiac malformations. They can exist in isolation, but are also found as integral components of other cardiac anomalies, such as tetralogy of Fallot, double outlet right ventricle, or common arterial trunk. As yet, there is no agreement on how best to classify such defects, nor even on the curved surface that is taken to represent the defect.Methods Based on our previous pathological and clinical experiences, we have reviewed the history of classification of holes between the ventricles. We proposed that the defects are best defined as representing the area of deficient ventricular septation. This then permits the recognition of clinically significant variants according to the anatomic borders, and the way the curved surface representing the area of deficient septation opens into the morphologically right ventricle.ResultsClinical manifestation depends on the size of the defect, and on the relationship between systemic and pulmonary vascular resistances. Symptoms include failure to thrive, along with the manifestations of the increase in flow of blood to the lungs. Diagnosis can be made by physical examination, but is confirmed by echocardiographic interrogation, which delineates the precise anatomy, and also provides the physiologic information required for optimal clinical decision-making. Cardiac catheterization offers additional information regarding hemodynamics, particularly if there is a concern regarding an increase in pulmonary vascular resistance. Hemodynamic assessment is rarely necessary to make decisions regarding management, although it can be helpful if assessing symptomatic adults with hemodynamically restrictive defects. In infants with defects producing large shunts, surgical closure is now recommended in most instances as soon as symptoms manifest. Only in rare cases is palliative banding of the pulmonary trunk now recommended. Closure with devices inserted on catheters is now the preferred approach for many patients with muscular defects, often using a hybrid procedure. Therapeutic closure should now be anticipated with virtually zero mortality, and with excellent anticipated long-term survival.Conclusion Ventricular septal defects are best defined as representing the borders of the area of deficient ventricular septation. An approach on this basis permits recognition of the clinically significant phenotypic variants.
    Orphanet Journal of Rare Diseases 12/2014; 9(1):144. DOI:10.1186/s13023-014-0144-2 · 3.96 Impact Factor
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    ABSTRACT: Advances made in the understanding of the molecular biology of the cardiac valves have been truly spectacular. Not all of those investigating these aspects, however, have an appropriate understanding of the underlying anatomy. Partly, this reflects problems in describing the components of the various valves, a difficulty also emphasised by surgeons who repair or replace the valves. In this review, we describe briefly the overall anatomy of the cardiac valves, pointing to their similarities and differences. We then suggest that uniform terms can be developed to account for the components of the valves, treating them as complexes that guard the atrioventricular and ventriculo-arterial junctions. The atrioventricular valvar complex is made up of an annulus, leaflets, tendinous cords, and papillary muscles. The tension apparatus is required to hold the leaflets together against the force of ventricular systole. The ventriculo-arterial complex is also based on the leaflets, but supported within the valvar sinuses, and limited distally by the sinutubular junction. It is the semilunar nature of the leaflets that underscores their snug closure during ventricular diastole. The complexes thus defined can be separated to produce paired valves in the normal arrangement, or to produce common valves in the congenitally malformed hearts. Knowledge of development now permits accurate inferences to be made regarding the origin of the various components, and their relevance to valvar disease. The valvar leaflets are developed from the endocardial cushions formed in the atrioventricular canal and the outflow tract by a process of endothelial-to-mesenchymal transformation. The papillary muscles of the atrioventricular valves are then derived from the trabecular layer of the developing ventricular walls, whereas the sinuses of the ventriculo-arterial valves are formed by additional growth of the non-myocardial tissues, concomitant with excavation of the outflow cushions to form the leaflets.
    Cardiology in the Young 12/2014; 24(6):1008-22. DOI:10.1017/S1047951114001942 · 0.86 Impact Factor
  • Thora S Steffensen, Diane E Spicer
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    ABSTRACT: Congenital coronary artery anomalies are rare. Pathologists are exposed to those in mainly two settings; in association with sudden death and usually extreme exercise in young adults, and in association with complex congenital heart disease in the pediatric and perinatal population. Pediatric pathologists, other pathologists and pathologists' assistants performing pediatric or forensic autopsies therefore need to be familiar with coronary artery anomalies.
    Fetal and Pediatric Pathology 10/2014; DOI:10.3109/15513815.2014.966182 · 0.40 Impact Factor
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    ABSTRACT: Although holes, or channels, between the ventricles are the commonest congenital cardiac malformations, there is still no consensus as to how they can best be described and categorised. So as to assess whether it is possible to produce a potentially universally acceptable system, we have analysed the hearts categorised as having ventricular septal defects in a large archive held at Birmingham Children's Hospital. Materials and methods We analysed all the hearts categorised as having isolated ventricular septal defects, or those associated with aortic coarctation or interruption in the setting of concordant ventriculo-arterial connections, in the archive of autopsied hearts held at Birmingham Children's Hospital, United Kingdom.
    Cardiology in the Young 09/2014; DOI:10.1017/S104795111400170X · 0.86 Impact Factor
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    ABSTRACT: Coronary arteries have been extensively described and recognized by gross anatomic studies. However, in the clinical setting, the recognition of the conal artery is essential during coronary angiography, as well as certain congenital heart conditions such as tetralogy of Fallot. In order to provide a complete anatomic and physiologic correlation of the actual incidence and distribution of the conal artery we examined 300 formalin fixed hearts with gross dissections and 300 coronary angiograms. The conal artery was identified in all hearts examined and five main patterns were recognized. In Type A (193, 32.1%), the conal artery arose as a branch of the right coronary artery (RCA); in Type B (96, 16%), the conal artery arose from the common coronary ostium with the RCA; in Type C (242, 40.3%), the conal artery took origin from the right aortic sinus as an independent artery; in Type D (48, 8%), multiple conal arteries were present and arose from the RCA as separate branches (32, 66.6%), from a common ostium with the RCA (8, 16.6%) or from the aortic sinus (8, 16.6%); in Type E (22, 3.6%), the conal artery arose as a branch of the right ventricular branch (17, 2.8%) or acute marginal artery (5, 0.8%). The relative prevalence of the five patterns as well as the morphology and the topography of the conal artery varied significantly with the degree of coronary luminal stenosis (as observed during angiography) and also with the degree of hypertrophied ventricular wall (as observed during gross dissections). Clin. Anat., 2014. © 2014 Wiley Periodicals, Inc.
    Clinical Anatomy 09/2014; DOI:10.1002/ca.22469 · 1.16 Impact Factor
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    ABSTRACT: The past decades have seen immense progress in the understanding of cardiac development. Appreciation of precise details of cardiac anatomy, however, has yet to be fully translated into the more general understanding of the changing structure of the developing heart, particularly with regard to formation of the septal structures. In this review, using images obtained with episcopic microscopy together with scanning electron microscopy, we show that the newly acquired information concerning the anatomic changes occurring during separation of the cardiac chambers in the mouse is able to provide a basis for understanding the morphogenesis of septal defects in the human heart. It is now established that as part of the changes seen when the heart tube changes from a short linear structure to the looped arrangement presaging formation of the ventricles, new material is added at both its venous and arterial poles. The details of these early changes, however, are beyond the scope of our current review. It is during E10.5 in the mouse that the first anatomic features of septation are seen, with formation of the primary atrial septum. This muscular structure grows toward the cushions formed within the atrioventricular canal, carrying on its leading edge a mesenchymal cap. Its cranial attachment breaks down to form the secondary foramen by the time the mesenchymal cap has used with the atrioventricular endocardial cushions, the latter fusion obliterating the primary foramen. Then the cap, along with a mesenchymal protrusion that grows from the mediastinal mesenchyme, muscularizes to form the base of the definitive atrial septum, the primary septum itself forming the floor of the oval foramen. The cranial margin of the foramen is a fold between the attachments of the pulmonary veins to the left atrium and the roof of the right atrium. The apical muscular ventricular septum develops concomitant with the ballooning of the apical components from the inlet and outlet of the ventricular loop. Its apical part is initially trabeculated. The membranous part of the septum is derived from the rightward margins of the atrioventricular cushions, with the muscularizing proximal outflow cushions fusing with the muscular septum and becoming the subpulmonary infundibulum as the aorta is committed to the left ventricle. Perturbations of these processes explain well the phenotypic variants of deficient atrial and ventricular septation. Anat Rec, 2014. © 2014 Wiley Periodicals, Inc.
    The Anatomical Record Advances in Integrative Anatomy and Evolutionary Biology 08/2014; 297(8). DOI:10.1002/ar.22949 · 1.53 Impact Factor
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    ABSTRACT: Background: Disagreement currently exists regarding the definition of aortic dextroposition. It is suggested that the term be used interchangeably with aortic overriding, along with suggestions that the aortic valve overrides in the normal heart. The dextroposed aorta, however, does not always override the crest of the muscular ventricular septum. It is incorrect to argue that the normal aortic valve overrides. It is the cavity of the right aortic valvar sinus, rather than the valvar orifice, that sits above the muscular septum when the septum itself is intact. Therefore, to circumvent these difficulties, those using the term "dextroposition" find it necessary to distinguish "true" as opposed to "false" categories. The problems arise because "dextroposition" is remarkably ill-suited as an alternative term for aortic valvar overriding. Methods and Results: In this review, combining developmental, morphologic, and clinical data, we show how aortic overriding is best considered on the basis of biventricular connection of the aortic root in the setting of deficient ventricular septation. When analysed in this manner, it becomes an easy matter to distinguish between one-to-one and double outlet ventriculo-arterial connections. Appreciation of these features emphasises the different spatial alignments of interventricular communications as opposed to the plane of deficient ventricular septation. The concept of overriding is applicable not only to biventricular connection of the aortic root, but also the pulmonary and common arterial roots. Conclusions: The diagnostic techniques now available to the paediatric cardiologist illustrate the features of arterial valvar overriding with exquisite accuracy, informing the discussions now required for optimal decision making.
    Cardiology in the Young 07/2014; 25(04):1-14. DOI:10.1017/S1047951114001139 · 0.86 Impact Factor
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    ABSTRACT: Although steps are being taken to produce a universally acceptable coding system for categorisation of the congenitally malformed hearts, obstacles remain in the search for consensus. One of the groups of lesions continuing to produce the greatest problems is those that permit interventricular shunting. The difficulties relate partly to the words used to describe the group itself, as those using Germanic languages describe the holes as ventricular septal defects, whereas those using Romance languages consider them to represent interventricular communications. The two terms, however, are not necessarily synonymous. Further disagreements relate to whether the lesions placed within the group should be sub-categorised on the basis of their geographical location within the ventricular mass, as opposed to the anatomic nature of their borders. In reality, attention to both the features is necessary if we are to recognise the full extent of phenotypic variability. In this review, we first review the evolution and theories of analysis naming the channels that permit interventricular shunting. We then demonstrate that embryologic techniques provide evidence that the changing morphology of the developing murine heart parallels the anatomy of the different lesions encountered in the congenitally malformed human heart. We suggest that, with attention paid to the temporal development of the normal murine heart, combined with a strict definition of the plane of separation between the right and left ventricular cavities, it will be feasible to produce a categorisation that is acceptable to all.
    Cardiology in the Young 05/2014; DOI:10.1017/S1047951114000869 · 0.86 Impact Factor
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    ABSTRACT: It is frequent, in the current era, to encounter congenital cardiac malformations described in terms of "cor triatriatum". But can hearts be truly found with three atrial chambers? The morphological method, emphasised by Van Praagh et al, states that structures within the heart should be defined on the basis of their most constant components. In the atrial chambers, it is the appendages that are the most constant components, and to the best of our knowledge, hearts can only possess two appendages, which can be of either right or left morphology. The hearts described on the basis of "cor triatriatum", nonetheless, can also be analysed on the basis of division of either the morphologically right or the morphologically left atriums. In this review, we provide a description of cardiac embryology, showing how each of the atrial chambers possesses part of the embryological body, along with an appendage, a vestibule, a venous component, and a septum that separates them. We then show how it is, indeed, the case that the hearts described in terms of "cor triatriatum" can be readily understood on the basis of division of these atrial components. In the right atrium, it is the venous valves that divide the chamber. In the left atrium, it is harder to provide an explanation for the shelf that produces atrial division. We also contrast the classic examples of the divided atrial chambers with the vestibular shelf that produces supravalvar stenosis in the morphologically left atrium, showing that this form of obstruction needs to be distinguished from the fibrous shelves producing intravalvar obstruction.
    Cardiology in the Young 05/2014; 25(02):1-15. DOI:10.1017/S1047951114000572 · 0.86 Impact Factor
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    ABSTRACT: The morphology of ventricular septal defects (VSDs) that are doubly committed and juxtaarterial places the patient at risk for aortic valvar prolapse and aortic valvar insufficiency (AI). Surgical repair of this type of defect often involves placing sutures through the base of one or more of the leaflets of the pulmonary valve, raising concern for late pulmonary valvar insufficiency (PI). The purpose of this review was to analyze the postoperative follow-up relating to potential late complications with the aortic and pulmonary valves. Between 1980 and 2012, 106 patients with doubly committed juxtaarterial VSD underwent intracardiac repair. Median age at repair was 1.1 years. Preoperative evaluation showed 69 patients (65%) had aortic valvar prolapse and 51 (48%) had AI. Operative approach was through the pulmonary trunk in 88 (83%) of the patients. In 81 patients (76%), sutures securing the VSD patch had been placed through the base of the pulmonary valvar leaflets. Operative survival was 100%. Follow-up ranges from 6 months to 17 years, with a mean of 4.9 years. No patient had heart block or residual shunting. Of the 70 patients with long-term contemporary echocardiographic follow-up, 66 (94%) had trivial or no AI and 4 (6%) had mild AI. Of these patients, 49 (70%) had trivial or no PI, and 21 (30%) had mild PI. In 1 patient having aortic valvoplasty at the time of VSD closure, the aortic valve was replaced 7 months later. No other patient had worrisome progression of their AI or PI. The incidence of aortic valvar prolapse and AI in the setting of doubly committed juxtaarterial VSD is quite high. The optimal surgical approach is through the pulmonary trunk. Sutures placed through the base of the pulmonary valvar leaflets do not predispose to clinically significant late pulmonary valvar insufficiency. Timely surgical closure of this type of defect prevents progression of AI.
    The Annals of thoracic surgery 03/2014; DOI:10.1016/j.athoracsur.2014.01.059 · 3.65 Impact Factor
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    ABSTRACT: Coronary arterial fistulas are abnormal connections between the coronary arteries and the chambers of the heart or major thoracic vessels. Although first described in 1841, the true incidence is difficult to evaluate because approximately half of the cases may be asymptomatic and clinically undetectable. This review will discuss the history and prevalence of coronary artery fistulas, their morphology, histology, presentation, diagnosis, treatment options, and complications.
    Cardiovascular Pathology 02/2014; DOI:10.1016/j.carpath.2014.01.010 · 2.34 Impact Factor
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    ABSTRACT: It is well recognized that the patients with the most complex cardiac malformations are those with so-called visceral heterotaxy. At present, it remains a fact that most investigators segregate these patients on the basis of their splenic anatomy, describing syndromes of so-called asplenia and polysplenia. It has also been known for quite some time, nonetheless, that the morphology of the tracheobronchial tree is usually isomeric in the setting of heterotaxy. And it has been shown that the isomerism found in terms of bronchial arrangement correlates in a better fashion with the cardiac anatomy than does the presence of multiple spleens, or the absence of any splenic tissue. In this exercise in anatomy, we use hearts from the Idriss archive of Lurie Children's Hospital in Chicago to demonstrate the isomeric features found in the hearts obtained from patients known to have had heterotaxy. We first demonstrate the normal arrangements, showing how it is the extent of the pectinate muscles in the atrial appendages relative to the atrioventricular junctions that distinguishes between morphologically right and left atrial chambers. We also show the asymmetry of the normal bronchial tree, and the relationships of the first bronchial branches to the pulmonary arteries supplying the lower lobes of the lungs. We then demonstrate that diagnosis of multiple spleens requires the finding of splenic tissue on either side of the dorsal mesogastrium. Turning to hearts obtained from patients with heterotaxy, we illustrate isomeric right and left atrial appendages. We emphasize that it is only the appendages that are universally isomeric, but point out that other features support the notion of cardiac isomerism. We then show that description also requires a full account of veno-atrial connections, since these can seemingly be mirror-imaged when the arrangement within the heart is one of isomerism of the atrial appendages. We show how failure to recognize the presence of such isomeric appendages can lead to spurious diagnoses of discordant atrioventricular connections. Overall, we show that the finding of isomeric atrial appendages, guided by the finding of bronchial isomerism, is but the prelude to full segmental sequential analysis. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
    Multimedia Manual of Cardiothoracic Surgery 01/2014; 2014. DOI:10.1093/mmcts/mmu027
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    ABSTRACT: Holes between the ventricles are the commonest congenital cardiac malformations. As yet, however, there is no consensus as to how they can best be described and categorized. In this, our third exercise in cardiac anatomy, we address the issue of classification of ventricular septal defects. We begin our demonstration by analysing the normal heart. We show that the larger part of the ventricular septum is made up of its muscular component. The membranous part accounts for only a small portion, which is located centrally within the cardiac base. This small membranous part forms a boundary between the right-sided chambers and the aortic root. Holes at this site, therefore, which account for the commonest defects closed surgically, will open centrally in the cardiac base, being located postero-inferiorly relative to the supraventricular crest. We then show that the larger part of the crest itself is a free-standing muscular sleeve, which lifts the leaflets of the pulmonary valve away from the cardiac base. Only a very small part of the muscle forming the right ventricular outlet is located in the septal position. Turning our attention to malformed hearts, we show how holes between the ventricles can open centrally at the cardiac base, open to the inlet or outlet of the right ventricle or open within the substance of the apical muscular septum. We demonstrate, however, that description of such geographical location of the defects does not paint the full picture, since lesions with markedly different phenotypic features can open in comparable geographic locations. We illustrate how it is the phenotypic features, as seen from the right ventricle, which convey the crucial information for the surgeon with regard to the location of the atrioventricular conduction axis, using hearts with holes opening to the inlet of the right ventricle with muscular as opposed to partially fibrous borders to emphasize this point. We continue by showing how holes with different phenotypes can also open to the outlet of the right ventricle, the key feature in this regard being malalignment between the apical muscular septum relative to the muscular outlet septum or its fibrous remnant. Malalignment can also be found between the apical ventricular septum and the atrial septum, this being shown in a defect opening to the inlet of the right ventricle. We conclude by emphasizing that, so as to bring together all the information of surgical significance, it is necessary to take note of the geographical location of holes between the ventricles, their phenotypic features and the presence or absence of malalignment between the septal components. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
    Multimedia Manual of Cardiothoracic Surgery 01/2014; 2014. DOI:10.1093/mmcts/mmu026
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    ABSTRACT: In the first of our exercises in anatomy, created for the Multimedia Manual of the European Association of Cardiothoracic Surgery, we emphasized that thorough knowledge of intracardiac anatomy was an essential part of the training for all budding cardiac surgeons, explaining how we had used the archive of congenitally malformed hearts maintained at Lurie Children's Hospital in Chicago to prepare a series of videoclips, demonstrating the salient features of tetralogy of Fallot. In this series of videoclips, we extend our analysis of the normal heart, since for our initial exercise we had concentrated exclusively on the structure of the right ventricular outflow tract. We begin our overview of normal anatomy by emphasizing the need, in the current era, to describe the heart in attitudinally appropriate fashion. Increasingly, clinicians are demonstrating the features of the heart as it is located within the body. It is no longer satisfactory, therefore, to describe these components in a 'Valentine' fashion, as continues to be the case in most textbooks of normal or cardiac anatomy. We then emphasize the importance of the so-called morphological method, which states that structures within the heart should be defined on the basis of their own intrinsic morphology, and not according to other parts, which are themselves variable. We continue by using this concept to show how it is the appendages that serve to distinguish between the atrial chambers, while the apical trabecular components provide the features to distinguish the ventricles. We then return to the cardiac chambers, emphasizing features of surgical significance, in particular the locations of the cardiac conduction tissues. We proceed by examining the cardiac valves, and conclude by providing a detailed analysis of the septal structures. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
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    ABSTRACT: It is axiomatic that those performing surgery on the congenitally malformed heart require a thorough knowledge of the lesions they will be called upon to correct. The necessary anatomical knowledge is becoming increasingly difficult to obtain at first hand, since relatively few centres now hold archives of specimens obtained in an appropriately legal fashion from the patients unfortunately dying in previous years. One centre with such an archive is Ann and Robert H. Lurie Children's Hospital in Chicago, known previously as Chicago Memorial Children's Hospital. The archive was established by Farouk S. Idriss, and was subsequently enhanced and consolidated by his son, Rachid. It is now under the care of Carl L. Backer, the current chief of paediatric cardiothoracic surgery at Lurie Children's. With the support of Carl, the archive has been triaged and catalogued by Diane E. Spicer and Robert H. Anderson. It has now been used to create a series of video presentations, illustrating the salient features of surgical anatomy of selected entities, with the videoclips being edited and prepared for publication by Anne Sarwark. This video contains the fruits of the first of these exercises in anatomy, and is devoted to tetralogy of Fallot.We begin the exercise by making comparisons between the normal heart and the arrangement seen in typical tetralogy. We emphasize the need to recognize the 'building blocks' of the normal outflow tracts, and show how they come apart in tetralogy. We then show the variations to be found in the specific morphology of the borders of the hole between the ventricles, with the crest of the apical ventricular septum being overridden by the orifice of the aortic valve such that the latter structure has a biventricular connection. We emphasize that it is the squeeze between the deviated muscular outlet septum and septoparietal trabeculations that is the essential phenotypic feature of the lesion. We then proceed to demonstrate the further variation to be found in the length of the outlet septum, which in extreme cases can be fibrous and hypoplastic rather than muscular. We also show how the ventriculo-arterial connection can vary from being concordant to becoming double outlet from the right ventricle. We conclude by emphasizing that the anatomy of tetralogy can also be recognized when the subpulmonary outflow tract is atretic rather than stenotic. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
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    ABSTRACT: It is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with "la maladie bleu", that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.
    Cardiology in the Young 12/2013; 23(6):857-865. DOI:10.1017/S1047951113001686 · 0.86 Impact Factor
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    ABSTRACT: This article combines material from three complementary overviews presented in the Symposium on Pulmonary Venous Anomalies during the Joint Meeting of the World Society for Pediatric and Congenital Heart Surgery and Sociedad Latina de Cardiologia y Cirugia Cardiovascular Pediátrica in Lima, Peru. We discuss the embryologic basis for nomenclature, the hierarchical diagnostic categories, and the important anatomic and morphologic characteristics of anomalous pulmonary venous connections. The anatomic descriptions help to guide an understandable and sensible approach to the diagnosis and surgical management of these various disorders.
    01/2013; 4(1):30-43. DOI:10.1177/2150135112458439

Publication Stats

47 Citations
42.35 Total Impact Points


  • 2009–2015
    • University of Florida
      • Department of Pediatrics
      Gainesville, Florida, United States
  • 2014
    • Florida Heart Research Institute
      Miami, Florida, United States
  • 2012–2014
    • Newcastle University
      Newcastle-on-Tyne, England, United Kingdom
  • 2010–2012
    • University of South Florida St. Petersburg
      St. Petersburg, Florida, United States
    • All Children's Hospital
      Florida City, Florida, United States
    • University College London
      • Institute of Child Health
      London, ENG, United Kingdom
  • 2007
    • University of Pennsylvania
      • Department of Medicine
      Filadelfia, Pennsylvania, United States