ATS
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
 QUICK SEARCH:   [advanced]


     


This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Vladimir V. Alexi-Meskishvili
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Alexi-Meskishvili, V. V.
Right arrow Articles by Berger, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Alexi-Meskishvili, V. V.
Right arrow Articles by Berger, F.
Related Collections
Right arrow Great vessels
Right arrowRelated Article

Ann Thorac Surg 2001;71:1006-1008
© 2001 The Society of Thoracic Surgeons


Case report

Eleven-year follow-up after descending thoracic aorta replacement in a small child

Vladimir V. Alexi-Meskishvili, MD, PhDa, Felix Berger, MDa

a Departments of Cardiothoracic and Vascular Surgery and Congenital Heart Disease, German Heart Institute Berlin, Berlin, Germany

Accepted for publication April 15, 2000.

Address reprint requests to Dr Alexi-Meskishvili, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, D-13353 Berlin, Germany
e-mail: alexi{at}dhzb.de


    Abstract
 Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 
Eleven years after the prosthetic replacement of a hypoplastic thoracic aorta in a 3.5-year-old child, there was normal growth of the aortic arch and abdominal aorta without signs of restenosis. This case illustrates that growth of the aorta can be normal after replacement of its thoracic part in a growing child.


    Introduction
 Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 
Hypoplasia or stenosis of the descending aorta in infants and small children, especially without involvement of the abdominal aorta and visceral branches, is a very rare malformation [1, 2]. Long-term results after prosthetic replacement of the thoracic aorta in a small child are unknown. Therefore, we report the 11-year follow-up of a 3.5-year-old child after the replacement of a severely hypoplastic thoracic aorta.

A 7-month-old male infant presented for the first time with clinical signs of aortic coarctation, which was confirmed only by echocardiography. The infant underwent operation in June 1985. After left thoracotomy, severe hypoplasia of the thoracic aorta between the ligamentum arteriosus and diaphragm was found and not a localized coarctation as initially suspected. The hypoplastic segment had a 3 mm diameter and 5 cm length. The aortic arch diameter was 10 mm and the aorta at the diaphragm level was 6 mm (normal 6 mm) [3]. The hypoplastic thoracic aorta was replaced with an 8 mm Dacron (C.R. Bard, Haverhill, MA) prosthesis, both ends of which were connected to the isthmus and nonhypoplastic segment of the descending aorta in an end-to-side fashion. There was no pressure gradient at the prosthesis immediately after operation. Histologic examination of the hypoplastic segment revealed a thin intima and a normally developed media and adventitia without any signs of acute or chronic inflammation. The case was reported elsewhere [4].

Follow-up observations revealed a progressive increase in the pressure gradient in the prosthesis with a maximum systolic pressure of 120 mm Hg at the upper extremity and 60 mm Hg at the femoral artery, with electrocardiographic signs of severe left ventricular hypertrophy. Because there were signs of prosthetic obstruction, the child underwent reoperation in May 1988 at the age of 3.5 years. At operation the old prosthesis, which was partially occluded because of neointimal ingrowth and thrombosis, was replaced with a 14 mm diameter and 9 cm length Dacron prosthesis. Proximal and distal anastomoses were created in an oblique fashion with the aortic isthmus and aorta at the diaphragm level to create an anastomosis of maximum diameter. The systolic pressure gradient of the prosthesis after operation dropped to 10 mm Hg and the postoperative course was uneventful.

Eleven years after the last operation and at the age of 14.5 years, the patient underwent a follow-up examination in our institution. Physical examination revealed a well-developed child (body weight, 67 kg; height, 167 cm) without exercise limitations or heart murmurs. The electrocardiograph showed slight signs of left ventricular hypertrophy. Arterial pressure in both arms and lower extremities was 130/72 and 120/87 mm Hg, respectively. Obtained from the long axis parasternal view, echocardiography revealed normal left ventricular contractility (ejection fraction, 75%; shortening fraction, 41%). Left ventricular systolic and diastolic inner dimensions were 2.7 and 4.3 cm, respectively. There were no valvular or other intracardiac abnormalities.

Spiroergometry during treadmill exercise test up to a maximum of 240 W (2.6 W/kg, ie, 90% of normal) reveald a satisfying cardiorespiratory function with a normal heart rate increase up to 187 beats/min, a maximum oxygen uptake VO2/kg of 27.8 mL · min-1 · kg-1 (60% of normal), a maximum CO2 elimination VCO2 of 2.11 L/min, and a V slope of 23. Blood pressure increased to a maximum of 180/95 mm Hg.

Cardiac catheterization revealed normal left ventricular pressure (120/0 to 12 mm Hg) with a pressure gradient between the aorta ascendens and abdominal aorta of 10 mm Hg. After intravenous injection of 0.5 mg etilefrin (Effortil, Boehringer Ingelheim Pharma KG, Ingelheim, Germany), the arterial pressure increased to 180/90 mm Hg with a maximum pressure gradient at the conduit of 20 mm Hg. Aortography revealed the absence of conduit deformation or anastomotic stenoses (Fig 1). Renal arteries and other visceral aortic branches were normally developed. The diameter of the ascending aorta was 3.5 cm, of the aortic arch 18 mm, of the aortic isthmus 15 mm, of the conduit 15 mm, and of the abdominal aorta 13 mm.



View larger version (64K):
[in this window]
[in a new window]
 
Fig 1. Aortogram in the anterior-posterior position with injection into the aorta ascendens. There is no narrowing at the proximal (A) or distal (B) anastomoses (arrowheads). The descending aorta is normally developed.

 

    Comment
 Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 
There are few reports describing isolated hypoplasia of the thoracic aorta in children [1, 2, 4]. When both the thoracic and abdominal aortas are hypoplastic, stenoses of the renal arteries and other visceral branches are common [5, 6].

The cause of thoracic or thoracoabdominal aortic hypoplasia or coarctation may be the result of different inflammatory aortic diseases, and it predominantly occurs in older children and adults. Nonatherosclerotic noninflammatory coarctation and tubular stenosis of the abdominal aorta, with branch stenotic involvement and arterial hypertension, may occur equally in boys and girls, and most of them become symptomatic during the second decade of life [7]. A congenital origin of aortic hypoplasia is established by histologic examination of the aortic wall, which mostly reveals intact vessel wall architecture without any signs of inflammation [7], as in our case, which is one of the youngest reported in the literature [1]. Replacing the thoracic aorta in the first operation resulted in the normalization of arterial pressure, but neointimal ingrowth and partial thrombosis of the prosthesis resulted in the recurrence of hypertension and the necessity for prosthetic replacement. We suggest that end-to-end replacement of the thoracic aorta in a small child is preferable to prosthetic bypass between the ascendens and descendens aorta [1], because replacement of the outgrown prosthesis, which is mandatory because of the natural growth of the child, later may be technically more difficult.

In our patient the arterial pressure remained normal after 11 years together with the significant regression of left ventricular hypertrophy, normal left ventricular contractility, and normal development of the abdominal aorta, which had a normal caliber for his age [3]. This case illustrates that longitudinal and transverse growth of the aorta can be normal after prosthetic replacement of a hypoplastic thoracic aorta in a small child.


    Acknowledgments
 Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 
We thank Tonie Derwent for assistance in the preparation of the manuscript.


    References
 Top
 Abstract
 Introduction
 Comment
 Acknowledgments
 References
 

  1. Brochhagen H.G., Benx-Bohm G., Lackner K.J., Mennicken U., Schickendantz S., Borowski A. Spiral CT angiography in an infant with severe hypoplasia of a long segment of the descending aorta. Pediatr Radiol 1997;27:181-183.[Medline]
  2. Wozniak G., Bauer J., Bohle R.M., Dapper F. Coarctation of the thoraco-abdominal aorta: operative treatment with a cryopreserved arterial homograft in a seven-year-old boy. J Cardiovasc Surg 1998;39:483-488.[Medline]
  3. Moss A.J., Adams F.H., O’Loughlan B.J., Dixon W.J. The growth of the normal aorta and of the anastomotic site in infants following surgical resection of coarctation of the aorta. Circulation 1959;19:338-349.[Abstract/Free Full Text]
  4. Alexi-Meskishvili V.V., Nikolyuk A.P., Korsak V.V., Golosovska M.A. Case of the successful surgical treatment of hypoplasia of the descending thoracic aorta in an infant. Grudnaia Khir 1987;3:92-93.
  5. Mickley V., Fleiter T. Coarctation of descending and abdominal aorta: long-term results of surgical therapy. J Vasc Surg 1998;28:206-214.[Medline]
  6. Reiher L, Sandmann W. Die coarctatio der thoracoabdominalen Aorta. Chirurg 1998;69:753–8.
  7. Graham L.M., Zelenock G.B., Erlandson E.E., Goran A.G., Lindenauer S.M., Stanley J.C. Abdominal aortic coarctation and segmental hypoplasia. Surgery 1979;86:519-529.[Medline]

Related Article

Invited commentary
Markus K. Heinemann and Gerhard Ziemer
Ann. Thorac. Surg. 2001 71: 1008. [Extract] [Full Text] [PDF]



This article has been cited by other articles:


Home page
Ann. Thorac. Surg.Home page
O. Honjo, K. Ishino, M. Kawada, S.-I. Ohtsuki, and S. Sano
Coarctation of the Thoraco-Abdominal Aorta Associated With Mucopolysaccharidosis VII in a Child
Ann. Thorac. Surg., August 1, 2005; 80(2): 729 - 731.
[Abstract] [Full Text] [PDF]


This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Vladimir V. Alexi-Meskishvili
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Alexi-Meskishvili, V. V.
Right arrow Articles by Berger, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Alexi-Meskishvili, V. V.
Right arrow Articles by Berger, F.
Related Collections
Right arrow Great vessels
Right arrowRelated Article


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS