Ann Thorac Surg 2001;71:2043-2044
© 2001 The Society of Thoracic Surgeons
Case report
Anomalous origin of innominate artery from right pulmonary artery in DiGeorge syndrome
Kagami Miyaji, MDa,
Robert L. Hannan, MDa,
Redmond P. Burke, MDa
a Department of Cardiovascular Surgery and Cardiology, Miami Children’s Hospital, Miami, Florida, USA
Accepted for publication May 1, 2000.
Address reprint requests to Dr Burke, Department of Cardiovascular Surgery and Cardiology, Miami Children’s Hospital, 3200 SW 60 Court, Suite 102, Miami, FL 33155
e-mail: redmond111{at}aol.com
 |
Abstract
|
|---|
We experienced a case of anomalous origin of innominate artery from right pulmonary artery (isolated innominate artery). This patient was a 2-month-old baby girl weighing 3.2 kg with DiGeorge syndrome, who was diagnosed with perimembranous ventricular septal defect, atrial septal defect, and patent ductus arteriosus. This type of anomaly is exceedingly rare.
|
Introduction
|
|---|
DiGeorge syndrome is commonly used to describe patients with congenital aplasia or hypoplasia of both thymus and parathyroid glands. A wide spectrum of anomalies, including cardiovascular anomalies, can result from complete or partial failure of development of the third and fourth pharyngeal pouches [1].
The patient was a 2-month-old baby girl weighing 3.2 kg with DiGeorge syndrome who had been hospitalized essentially since birth because of failure to thrive and congestive heart failure. She was diagnosed by echocardiogram with perimembranous ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), bilateral peripheral pulmonary artery stenosis, and an aberrant right subclavian artery. She was referred to our hospital for diagnostic catheterization and surgical intervention. The results of cardiac catheterization revealed large perimembranous VSD, ASD, small PDA, bilateral pulmonary artery stenosis, marked dilation of main pulmonary artery, and anomalous origin of innominate artery from right pulmonary artery (isolated innominate artery) (Fig 1). The pulmonary:systemic blood flow ratio was 4.0. The oxygen saturation of her right arm was around 90%, whereas that of her left arm was 96%. The patient needed surgical repair; however, after catheterization, she presented with sepsis and required intravenous antibiotic therapy. One week later, the patient’s blood culture turned negative and she became hemodynamically stable and ready for surgery.
View larger version (181K):
[in this window]
[in a new window]
|
Fig 1. The pulmonary angiography shows the origin of the innominate artery from the midportion of the right pulmonary artery (Rt.PA).
|
|
Under the general anesthesia, a median sternotomy was performed. There was no thymus and pericardium was opened. We inspected the anatomy and found that there was no innominate artery arising from the ascending aorta. A massively enlarged main pulmonary artery trunk had rolled over on top of the left pulmonary artery, producing a proximal kink. The right pulmonary artery was dissected and the innominate artery was found to arise from the superior aspect of the bifurcation of the branch pulmonary arteries on the right pulmonary artery side (Fig 2). The PDA was ligated. Under cardiopulmonary bypass and mild hypothermia, the aorta was cross-clamped and the heart was arrested by administration of a cardioplegic solution. The VSD was closed with a pericardial patch and the ASD was also closed. A side-clamp was placed on the right pulmonary artery and the innominate artery was detached. The innominate artery was also clamped, because there was a lot of back flow across the cerebral vessels. A 4-mm punch hole was made on the lateral side of the ascending aorta and an anastomosis of the innominate artery to the aorta was performed using a running suture. The plication of the main pulmonary artery was performed to relieve the rotational kinking of the left pulmonary artery. The patient tolerated the procedure well and returned to the unit in stable condition. The postoperative course was uneventful and, on the 13th postoperative day, she was discharged home.
View larger version (122K):
[in this window]
[in a new window]
|
Fig 2. The intraoperative picture shows the innominate artery is arising from the right pulmonary artery. (RA = right atrium; Rt.PA = right pulmonary artery.)
|
|
|
Comment
|
|---|
About 80% of DiGeorge syndrome patients have aortic arch anomalies or conotruncal anomalies, such as persistent truncus arteriosus and tetralogy of Fallot [1, 2]. Aortic arch anomalies have included interrupted aortic arch, double aortic arch, vascular ring, and aberrant subclavian artery. However, an aortic arch vessel originating from the pulmonary artery is a very rare anomaly, and only a few cases with right aortic arch and left common carotid or innominate artery arising from the pulmonary artery have been reported [3, 4]. We encountered the left aortic arch and innominate artery originating from the right pulmonary artery (isolated innominate artery). The Edwards’ hypothetical double aortic arch [5] explains aortic arch abnormalities by selective regression of various parts of either arch (Fig 3). In our case, the regression occurred at two sites: in the right posterior arch distal to the ductus arteriosus and in the right anterior arch proximal to the right common carotid artery. This resulted in an isolated innominate artery with the left aortic arch. This type of anomaly is exceedingly rare.
View larger version (10K):
[in this window]
[in a new window]
|
Fig 3. (A) The hypothetical double aortic arch of Edwards.(B) Concurrent interruption of the right posterior arch distal to the right ductus arteriosus and the right anterior arch proximal to the right common carotid artery, resulting in an isolation of innominate artery. (AA = ascending aorta; DA = descending aorta; LCCA = left common carotid artery; LDA = left ductus arteriosus; LSA = left subclavian artery; RCCA = right common carotid artery; RDA = right ductus arteriosus; RSA = right subclavian artery.)
|
|
|
References
|
|---|
-
Freedom R.M., Rosen F.S., Nadas A.S. Congenital cardiovascular disease and anomalies of the third and fourth pharyngeal pouch. Circulation 1972;46:165-172.[Abstract/Free Full Text]
-
Van Mierop L.H., Kutsche L.M. Cardiovascular anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor. Am J Cardiol 1986;58:133-137.[Medline]
-
Fong L.V., Venables A.W. Isolation of the left common carotid or left innominate artery. Br Heart J 1987;57:552-554.[Abstract/Free Full Text]
-
Huang S.F., Wu M.H. Left common carotid artery arising from the pulmonary artery in a patient with DiGeorge syndrome. Heart 1996;76:82-83.[Abstract/Free Full Text]
-
Edwards J.E. Anomalies of derivatives of aortic arch systems. Med Clin North Am 1948;19:925-949.
Top
Abstract
Introduction
