Management of the Aortic Root in Adult Patients With Conotruncal Anomalies

Joseph A. Dearani, Harold M. Burkhart, John M. Stulak, Thoralf M. Sundt, Hartzell V Schaff

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

Conotruncal anomalies such as tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, and transposition of the great arteries are a group of congenital heart defects with abnormalities of the outflow tracts and great vessels. It is common for the ascending aorta and aortic root to be significantly dilated following initial repair of the conotruncal anomaly, and little information is available on the management of this increasing problem. Although there are few case reports of aortic dissection and rupture in the literature, it appears to be rare in the setting of a conotruncal anomaly and may be related to the absence of hypertension and smoking in many of these patients. The timing of operation with regard to the size of the aortic root is difficult. In the absence of a family history of aortic dissection or aneurysm, or documented rapid growth of the ascending aorta, we proceed with replacement of the ascending aorta when the size is ≥ 55 mm. When the size of the ascending aorta is 5.0-5.5, treatment is individualized depending on the associated anomalies that need to be addressed, patient comorbidities, and life expectancy. In this group of patients we consider a simple reduction ascending aortoplasty. We generally proceed with root replacement and coronary reimplantation when there is effacement of the sinotubular junction, or when there is severe dilatation of the aortic root with an intact sinotubular junction. If the ascending aorta is ≥ 55 mm with an intact sinotubular junction and the sinuses are ≤ 4 cm, then we use a supracoronary tube graft.

Original languageEnglish (US)
Pages (from-to)122-129
Number of pages8
JournalSeminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual
Volume12
Issue number1
DOIs
StatePublished - 2009

Fingerprint

Aorta
Dissection
Truncus Arteriosus
Double Outlet Right Ventricle
Aortic Rupture
Transposition of Great Vessels
Tetralogy of Fallot
Congenital Heart Defects
Replantation
Life Expectancy
Aneurysm
Comorbidity
Dilatation
Smoking
Hypertension
Transplants
Growth
Therapeutics

Keywords

  • Ascending aortic aneurysm
  • Conotruncal anomaly
  • Tetralogy of Fallot

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health
  • Surgery

Cite this

Management of the Aortic Root in Adult Patients With Conotruncal Anomalies. / Dearani, Joseph A.; Burkhart, Harold M.; Stulak, John M.; Sundt, Thoralf M.; Schaff, Hartzell V.

In: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual, Vol. 12, No. 1, 2009, p. 122-129.

Research output: Contribution to journalArticle

Dearani, Joseph A. ; Burkhart, Harold M. ; Stulak, John M. ; Sundt, Thoralf M. ; Schaff, Hartzell V. / Management of the Aortic Root in Adult Patients With Conotruncal Anomalies. In: Seminars in Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual. 2009 ; Vol. 12, No. 1. pp. 122-129.
@article{76a2afac757c4c8ba2f9be6926ccb298,
title = "Management of the Aortic Root in Adult Patients With Conotruncal Anomalies",
abstract = "Conotruncal anomalies such as tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, and transposition of the great arteries are a group of congenital heart defects with abnormalities of the outflow tracts and great vessels. It is common for the ascending aorta and aortic root to be significantly dilated following initial repair of the conotruncal anomaly, and little information is available on the management of this increasing problem. Although there are few case reports of aortic dissection and rupture in the literature, it appears to be rare in the setting of a conotruncal anomaly and may be related to the absence of hypertension and smoking in many of these patients. The timing of operation with regard to the size of the aortic root is difficult. In the absence of a family history of aortic dissection or aneurysm, or documented rapid growth of the ascending aorta, we proceed with replacement of the ascending aorta when the size is ≥ 55 mm. When the size of the ascending aorta is 5.0-5.5, treatment is individualized depending on the associated anomalies that need to be addressed, patient comorbidities, and life expectancy. In this group of patients we consider a simple reduction ascending aortoplasty. We generally proceed with root replacement and coronary reimplantation when there is effacement of the sinotubular junction, or when there is severe dilatation of the aortic root with an intact sinotubular junction. If the ascending aorta is ≥ 55 mm with an intact sinotubular junction and the sinuses are ≤ 4 cm, then we use a supracoronary tube graft.",
keywords = "Ascending aortic aneurysm, Conotruncal anomaly, Tetralogy of Fallot",
author = "Dearani, {Joseph A.} and Burkhart, {Harold M.} and Stulak, {John M.} and Sundt, {Thoralf M.} and Schaff, {Hartzell V}",
year = "2009",
doi = "10.1053/j.pcsu.2009.01.013",
language = "English (US)",
volume = "12",
pages = "122--129",
journal = "Pediatric Cardiac Surgery Annual",
issn = "1092-9126",
publisher = "W.B. Saunders Ltd",
number = "1",

}

TY - JOUR

T1 - Management of the Aortic Root in Adult Patients With Conotruncal Anomalies

AU - Dearani, Joseph A.

AU - Burkhart, Harold M.

AU - Stulak, John M.

AU - Sundt, Thoralf M.

AU - Schaff, Hartzell V

PY - 2009

Y1 - 2009

N2 - Conotruncal anomalies such as tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, and transposition of the great arteries are a group of congenital heart defects with abnormalities of the outflow tracts and great vessels. It is common for the ascending aorta and aortic root to be significantly dilated following initial repair of the conotruncal anomaly, and little information is available on the management of this increasing problem. Although there are few case reports of aortic dissection and rupture in the literature, it appears to be rare in the setting of a conotruncal anomaly and may be related to the absence of hypertension and smoking in many of these patients. The timing of operation with regard to the size of the aortic root is difficult. In the absence of a family history of aortic dissection or aneurysm, or documented rapid growth of the ascending aorta, we proceed with replacement of the ascending aorta when the size is ≥ 55 mm. When the size of the ascending aorta is 5.0-5.5, treatment is individualized depending on the associated anomalies that need to be addressed, patient comorbidities, and life expectancy. In this group of patients we consider a simple reduction ascending aortoplasty. We generally proceed with root replacement and coronary reimplantation when there is effacement of the sinotubular junction, or when there is severe dilatation of the aortic root with an intact sinotubular junction. If the ascending aorta is ≥ 55 mm with an intact sinotubular junction and the sinuses are ≤ 4 cm, then we use a supracoronary tube graft.

AB - Conotruncal anomalies such as tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, and transposition of the great arteries are a group of congenital heart defects with abnormalities of the outflow tracts and great vessels. It is common for the ascending aorta and aortic root to be significantly dilated following initial repair of the conotruncal anomaly, and little information is available on the management of this increasing problem. Although there are few case reports of aortic dissection and rupture in the literature, it appears to be rare in the setting of a conotruncal anomaly and may be related to the absence of hypertension and smoking in many of these patients. The timing of operation with regard to the size of the aortic root is difficult. In the absence of a family history of aortic dissection or aneurysm, or documented rapid growth of the ascending aorta, we proceed with replacement of the ascending aorta when the size is ≥ 55 mm. When the size of the ascending aorta is 5.0-5.5, treatment is individualized depending on the associated anomalies that need to be addressed, patient comorbidities, and life expectancy. In this group of patients we consider a simple reduction ascending aortoplasty. We generally proceed with root replacement and coronary reimplantation when there is effacement of the sinotubular junction, or when there is severe dilatation of the aortic root with an intact sinotubular junction. If the ascending aorta is ≥ 55 mm with an intact sinotubular junction and the sinuses are ≤ 4 cm, then we use a supracoronary tube graft.

KW - Ascending aortic aneurysm

KW - Conotruncal anomaly

KW - Tetralogy of Fallot

UR - http://www.scopus.com/inward/record.url?scp=63749132446&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=63749132446&partnerID=8YFLogxK

U2 - 10.1053/j.pcsu.2009.01.013

DO - 10.1053/j.pcsu.2009.01.013

M3 - Article

C2 - 19349026

AN - SCOPUS:63749132446

VL - 12

SP - 122

EP - 129

JO - Pediatric Cardiac Surgery Annual

JF - Pediatric Cardiac Surgery Annual

SN - 1092-9126

IS - 1

ER -