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Ann Thorac Surg 1995;59:1491-1494
© 1995 The Society of Thoracic Surgeons

Nature of Coarctation in Hypoplastic Left Heart Syndrome

Department of Cardiovascular Pathology, University of Amsterdam, Academic Medical Center, Amsterdam, the Netherlands

Accepted for publication February 21, 1995.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
The study was designed to establish the underlying pathology of aortic coarctation in hypoplastic left heart syndrome. Aortic coarctation in patients with hypoplastic left heart syndrome is a significant problem when a Norwood procedure or heart transplantation is performed. Previous reports have claimed that the obstruction was merely a branch-point phenomenon and that ductal tissue was absent. Five heart specimens with hypoplastic left heart syndrome and aortic coarctation were examined histologically. The distal segment of the aortic arch, together with the arterial duct, were dissected and processed en bloc. The blocks were sectioned parallel to the longitudinal axis of the aortic arch. In 4 hearts the coarctation was in the preductal position. Ductal tissue encircled the aortic lumen and extended into the aorta both proximally and distally from the ductal orifice. The fifth case showed medial thickening of the aortic wall in the paraductal position, but without ductal tissue. The arterial duct showed histologic characteristics of persistent patency. Our observations suggest that aortic coarctation in the preductal position in hypoplastic left heart syndrome is caused by the extension of ductal tissue. The findings may have an impact on clinical strategies for hypoplastic left heart syndrome.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
The hypoplastic left heart syndrome (HLHS) often is associated with aortic coarctation in juxtaposition with the arterial duct [1–3]. Analysis of the outcome of palliative operation with the classic Norwood procedure revealed that aortic coarctation had no decremental effect on early (less than 30 days) mortality [4]. However, the longitudinal results after first-stage palliation revealed that obstruction in the aortic arch was a significant problem, despite the modification of the original procedure by patch augmentation of the reconstructed arch [5]. At the same time, it was demonstrated that balloon dilation angioplasty of the postoperative aortic arch obstruction yielded good results [6, 7]. These observations suggest that residual tissues are responsible for the obstruction and, therefore, it is tempting to relate the phenomenon to the presence of ductal tissue as part of the coexistent coarctation. This assumption, however, is in striking contrast with a report by Elzenga and Gittenberger-de Groot [8], who report on 3 cases of hypoplastic left heart with aortic coarctation and absent ductal tissue. These investigators concluded that the obstruction was not a ``true'' coarctation, but rather a branch-point phenomenon.

The apparent controversy has gained additional clinical relevance as one of the present options for patients with HLHS is heart transplantation. Maintenance of ductal patency is a prerequisite, which may be achieved by stenting [9, 10]. In case the condition is further complicated by aortic coarctation, balloon dilation may be considered as an additional alternative procedure to bridge the waiting period. In view of the less than optimal results with balloon dilation angioplasty of native aortic coarctation in babies, attributed to the presence of ductal tissue, it is worthwhile to know whether or not aortic coarctation in HLHS also is associated with ductal tissue.

For these reasons we studied histologically the site of aortic coarctation in 5 heart specimens from patients with HLHS.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Five hearts with hypoplastic left ventricle, hypoplastic ascending aorta, aortic valve atresia, and coarctation of the aorta were selected from formalin-fixed specimens in the Cardiovascular Registry. Aortic coarctation was defined as a localized narrowing in the distal part of the aortic arch of 50% or more [4]. Three specimens had hypoplasia of the mitral orifice; 2 had mitral atresia. The specimens ranged from 2 to 8 days of age.

The distal segment of the aortic arch, together with the arterial duct, was dissected and processed en bloc. The blocks were embedded in paraffin and serially sectioned at 5 µm, parallel to the longitudinal axis of the aortic arch. At 250-µm intervals, sections were stained with hematoxylin and eosin and an elastic tissue stain. Additional sections were mounted when deemed necessary to verify the presence and extension of ductal tissue.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Histologic examination showed an infolding of the media, albeit to varying degrees, in all 5 specimens. In 4 specimens the coarctation was in preductal position; the fifth specimen showed the coarctation in the paraductal location.

Ductal tissue extended into the aorta and completely encircled the lumen in all 4 specimens with a preductal coarctation (Figs 1 and 2). In each of these specimens ductal tissue extended both proximally into the aortic isthmus, and distally into the descending aorta; however, the distances varied considerably.

View larger version (142K): [in this window] [in a new window]   Fig 1. . Histologic section of a specimen with ductal tissue encircling the lumen. Ductal tissue stains light. (A) A prominent ridge (asterisk), composed of an infolding of the media with ductal tissue on its crest, is present opposite the arterial duct. Ductal tissue also extends onto the adjacent aortic wall at both sides of the coarctation (arrows). (B) A close-up view of ductal tissue on the crest of the ridge. (DA = arterial duct; DAO = descending aorta; IST = aortic isthmus.) (Elastic van Gieson stain; A, x2.5; B, x10 before 33% reduction.)

View larger version (84K): [in this window] [in a new window]   Fig 2. . Composite of the histologic appearance of ductal tissue, encircling the lumen of the aortic arch at the site of junction with the arterial duct (DA), in a heart specimen with only minimal medial infolding. The level of the sections shifts from ventral to dorsal (A to C). Ductal tissue extends (light staining tissue) far into the descending aorta (DAO), but also somewhat proximally into the isthmus (IST). (Elastic van Gieson stain; x2.5 before 33% reduction.)

View larger version (125K): [in this window] [in a new window]   Fig 3. . Histologic appearance of the 1 specimen with abnormal histology of the arterial duct (DA). (A) Note mucoid change of the muscular media of the arterial duct, but without bulky cushion tissue as anticipated. The luminal site is lined by a distinct internal elastic lamina. There is no extension of ductal tissue into the isthmus (IST) or the descending aorta (DAO) or onto the crest of the medial infolding (asterisk). The latter is shown in detail in (B). (Elastic van Gieson stain; A, x2.5; B, x14 before 33% reduction.)

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

The observation of ductal tissue extending onto the coarctation is at variance with a previous report on the histologic characteristics of aortic coarctation in HLHS [8]. However, the same group of investigators also published a histologic study of coarctation and other obstructive arch anomalies in relation to the arterial duct that contained 3 specimens with HLHS [13]. As far as we can judge from their data, each of these 3 cases showed ductal tissue extending onto the ridge of the coarctation, as well as extension of ductal tissue onto the wall of the descending aorta. It is unclear from their presentations whether the 3 cases in both publications are the same.

The present observation of extension of ductal tissue from the arterial duct into the aortic arch is in favor of a concept of abnormal extension of ductal tissue downstream, once abnormalities of flow through the arterial duct occur during embryonic and fetal life [14].

The present observations are relevant clinically for a number of reasons. In the majority (4 of 5 specimens) ductal tissue formed a sling that encircled the lumen of the aorta at the site of the junction with the arterial duct. In fact, 1 specimen with only minimal infolding of the media also showed this slinglike ductal extension. The fact that in each of these 4 specimens the coarctation was found in the preductal position may be more than coincidental. Anderson and colleagues [15] previously mentioned that preductal coarctation in these cases is related intimately to the arterial duct, although their statement was based only on gross inspection. The present observations are the more interesting, as the one specimen without extension of ductal tissue appeared to represent a case of persistent patency of the arterial duct. The aortic ridge, interpreted grossly as coarctation, was located in a juxtaductal position, but the arterial duct did not contain the bulky cushionlike tissue as anticipated. Instead, there was a distinct internal elastic lamina with only mild intimal thickening. It is tempting, therefore, to speculate that persistent patency of the ductus, associated with structural abnormalities, also prevents extension of ductal tissue onto the aorta. Thus far we have not been able to retrieve data from the literature relevant to this point.

Our observations emphasize the significance of augmentation of the aortic arch as part of the initial palliative procedure. At the same time, they may explain the relatively high incidence of aortic arch obstruction after the procedure. We hypothesize that ductal tissue remnants produce the obstruction, although-as far as we are aware-as yet this has not been verified histologically. It is also important to reemphasize that ductal tissue spreads out onto the inner wall of the aorta, particularly in the distal direction into the descending aorta, for variable distances. Hence, arch obstruction after the first palliative procedure is not necessarily confined to the precise site of the initial coarctation ridge, but may well appear at some distance either proximally or distally. Finally, the observations are important in light of attempts to relieve obstruction of aortic coarctation in patients with HLHS awaiting cardiac transplantation. In analogy with the relatively poor results with balloon dilation angioplasty of native coarctation in babies, considered to be attributable to the proliferative tendency of ductal tissue, we hypothesize that also in case of HLHS early recurrence of stenosis is due to occur. Indeed, the problem of recurrent coarctation after heart transplantation in patients with HLHS has recently been highlighted [16].

	Acknowledgments

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
During the course of this study Dr Machii was a Research Fellow from the Kitasato University School of Medicine, Kanagawa, Japan. Wilfried Meun took the photographs and Marsha Schenker provided secretarial assistance.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 
Present address: Department of Thoracic and Cardiovascular Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Sagamihara, Kanagawa 228, Japan.

Address reprint requests to Dr Becker, Department of Cardiovascular Pathology, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam-ZO, the Netherlands.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment Acknowledgments References

 

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5. Meliones JN, Snider AR, Bove EL, Rosental A, Rosen DA.Longitudinal results after first-stage palliation for hypoplastic left heart syndrome. Circulation 1990;82(Suppl 4):151–6.
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9. Rosenthal E, Qureshi SA, Kakadekar AP, Tabatabaie AH, Baker EJ, Tynan M. Comparison of stent implantation with balloon dilation for maintenance of ductus arteriosus patency [Abstract]. J Am Coll Cardiol 1992;19(Suppl A):25A.
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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Machii, M. Articles by Becker, A. E. Search for Related Content PubMed PubMed Citation Articles by Machii, M. Articles by Becker, A. E.