Asian Annals
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 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):
Tulga A Ulus
Oguz Tasdemir
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Birincioglu, C. L.
Right arrow Articles by Tasdemir, O.
Right arrow Search for Related Content
PubMed
Right arrow Articles by Birincioglu, C. L.
Right arrow Articles by Tasdemir, O.
Asian Cardiovasc Thorac Ann 2000;8:161-163
© 2000 Asia Publishing EXchange Pte Ltd

CASE STUDY

Repair of Isolated Atrial Inversion With Venous Return Abnormality

Cemal Levent Birincioglu, MD, Hakan Tikiz, MD1,, Hasmet Bardakçi, MD, Yücel Balbay, MD1,, Tulga A Ulus, MD, Siber Göksel, MD1,, Oguz Tasdemir, MD

Department of Cardiovascular Surgery
1 Department of Cardiology
Türkiye Yüksek Ihtisas Hospital
Ankara, Turkey
For reprint information contact: Cemal Levent Birincioglu, MD Tel: 90 532 227 9246 Fax: 90 312 312 4120 email: b_levent{at}hotmail.com Hekimkoy, 9 Cadde, 20 Sokak No. 5, Umitkoy, Ankara, Turkey.

   Abstract
TOP
 Abstract
Introduction
 Case Report
 Discussion
 References
 
A 33-year-old woman was found to have atrial inversion (atrioventricular discordance), right-hand ventricular morphology, ventriculoarterial concordance associated with a primum atrial septal defect, and abnormal systemic and pulmonary venous return. The pulmonary veins, inferior vena cava, and left superior vena cava drained into the left-sided morphologically right atrium. Only the right superior vena cava drained into the right-sided morphologically left atrium. Intraatrial baffle repair was performed successfully.


   Introduction
TOP
 Abstract
 Introduction
Case Report
 Discussion
 References
 
Isolated atrial inversion is a rare cardiac anomaly in which the atria are inverted in mirror image (atrial situs inversus) and there is normal ventricular topography (right-hand ventricular morphology, ventricular d loop) with discordant atrioventricular and concordant ventriculoarterial connection.1 The physiology resembles transposition of the great vessels. This rare congenital anomaly has been successfully repaired by atrial switch operations such as the mirror-image Mustard or Senning procedures through the left-sided morphologically right atrium.1–3 Sometimes, the left-sided morphologically right atrium is too posterior for such a surgical approach and the association of a pulmonary and systemic venous return anomaly may cause some difficulties in performing these kinds of procedures. An intraatrial baffle repair technique in a patient with atrial inversion together with a primum atrial septal defect is described.


   Case Report
TOP
 Abstract
 Introduction
 Case Report
Discussion
 References
 
A 33-year-old woman was admitted to hospital because of mild cyanosis since childhood, weakness, and easy fatigability. She had finger clubbing and cyanosis of the lips, tongue, and nails. Her heart rate was 86 beats•min–1 with a regular rhythm and her blood pressure was 100/60 mm Hg. The cardiac apex pulsation was palpated at the 5th intercostal space on the left midclavicular line. Auscultation revealed a 2/6 systolic ejection murmur and fixed split of the second heart sound at the pulmonic area and a 2/6 pansystolic murmur radiating to the left axilla at the apex. Among the routine laboratory findings, hemoglobin and hematocrit values were 172 g•L–1 and 52.7%, respectively. Electrocardiography showed sinus rhythm, P waves were negative in lead I and positive in leads II, III, aVF, and V1. The mean P-axis in the frontal plane was +120°. Radiography showed a mirror-image bronchial arrangement (long bronchus to the right side and short bronchus to the left side) and normal visceral situs (right-sided bulk of the liver and left-sided fundus of the stomach). On echocardiography, especially in apical 4-chamber view, an absence of tissue in the inferior portion of the atrial septum and left-sided atrial dominance was detected. In subcostal 4-chamber view during systole, the septal attachments of the right and left atrioventricular valve leaflets were at the same level. Color Doppler examination showed moderate mitral regurgitation and increased turbulence in the left-sided atrium, so contrast echocardiographic techniques were performed for precise definition of the anomalous systemic venous return. Injection of contrast medium from the left brachial and right femoral vein unexpectedly showed initial filling of the left-sided atrium and then the right-sided atrium via an atrial septal defect. Repeating this procedure from the right brachial vein, it was seen that the contrast medium filled the right-sided atrium first and then the left-sided atrium, as expected.

During cardiac catheterization, injection of opaque material from the right femoral vein revealed that the inferior vena cava (IVC) entered the left-sided atrium together with the hepatic vein (Figure 1Go). Radiopaque injection into the left-sided atrium revealed that it had the morphology of a right atrial appendage (Figure 2Go). This atrium also received blood from a left superior vena cava (SVC). Radiopaque injection into the left SVC clearly demonstrated the connection between the left and right SVC and the left-sided atrium (Figure 3Go). The right-sided atrium and right ventricle were opacified by way of an atrial septal defect during all injections. The aorta arose from the left ventricle and the pulmonary artery arose from the right ventricle (concordant ventriculoarterial relation). Systemic and pulmonary oxygen saturation were 56% and 38%, respectively. Coronary angiography revealed that the left anterior descending coronary artery originated from the right sinus of Valsalva with a distinct orifice.



View larger version (128K):
[in this window]
[in a new window]
  		Figure 1. Radiopaque injection just below the diaphragm demonstrated that the inferior vena cava drained into the left-sided atrium together with the hepatic vein (left anterior oblique view). HV = hepatic vein, IVC = inferior vena cava, LSA = left-sided atrium, RSA = right-sided atrium.

 


View larger version (134K):
[in this window]
[in a new window]
  		Figure 2. Radiopaque injection into the left-sided atrium clearly demonstrated the triangular-shaped right atrial appendage (arrows) of the left-sided atrium (atrial situs inversus). The primum atrial septal defect is indicated by the asterisk (left anterior oblique view). LSA = left-sided atrium.

 


View larger version (123K):
[in this window]
[in a new window]
  		Figure 3. Radiopaque injection from the left superior vena cava demonstrated its drainage into the left-sided atrium and its connection to the right superior vena cava via an innominate vein (left anterior oblique view). IV = innominate vein, LSA = left-sided atrium, LSVC = left superior vena cava, RSA = right-sided atrium, RSVC = right superior vena cava.

 
At surgery, the angiographic finding that the left-sided posteriorly placed atrium had a right atrial appendage was confirmed, and the right-sided anteriorly placed atrium was found to have left atrial appendage morphology (atrial situs inversus). There were bilateral SVCs. Right-hand ventricular topology was found and the pulmonary trunk spiraled around the ascending aorta. Because the left-sided atrium was located too far posteriorly for an easy surgical approach, it was preferred initially to open the anteriorly placed right-sided atrium. When the right-sided atrium was opened, it was evident that it received blood from only the right SVC and communicated with the left-sided atrium through a primum atrial septal defect; it drained into the right ventricle through a separated valve such as that seen in atrioventricular cushion-type deformity (Figure 4AGo). This valve had 5 leaflets with an ovoid annulus and 2 separated atrioventricular orifices that were supported by 2 papillary muscles anchored on the left ventricular side and 3 papillary muscles anchored on the right ventricular side. The 5 leaflets were arranged as a trifoliate left valve and a quadrifoliate right valve by a connecting tongue of valve tissue that was adjacent to the muscular inlet of the ventricular septum. Examination of the left-sided atrium revealed that the left SVC and IVC, all pulmonary veins, and the coronary sinus drained into this dominant atrium. The IVC drained into the left-sided atrium just to the left side of the interatrial septum (Figure 4AGo). The atrial septal defect was enlarged by cutting the posteroinferior part until the IVC could be seen clearly. The atrial septum was then reorganized with a polytetrafluoroethylene patch, leaving the right SVC and the IVC at the right side. In this way, the right SVC and IVC drained into the right ventricle. There was a connection between the right and left SVC with an innominate vein that was demonstrated by angiography, so the left-sided SVC was clamped and the distal pressure was measured. The pressure did not increase compared to that of the right side, so it was thought that the right SVC was adequate for left brachiocephalic venous drainage. Thus, the left SVC was tied below the innominate vein with silk tape (Figure 4BGo). The patient left the operating room in sinus rhythm and without any problem. She was discharged from the hospital after a smooth 10-day recovery period.



View larger version (25K):
[in this window]
[in a new window]
  		Figure 4. (A) Schematic representation of the patient's malformations detected at surgery. (B) Schematic representation of the surgical procedure. CS = coronary sinus, IV = innominate vein, IVC = inferior vena cava, LSA = left-sided atrium, LSVC = left superior vena cava, PTFE = polytetrafluoroethylene, PV = pulmonary veins, RSA = right-sided atrium, RSVC = right superior vena cava.

 

   Discussion
TOP
 Abstract
 Introduction
 Case Report
 Discussion
References
 
Isolated atrial inversion may occur concomitantly with some additional cardiac anomaly.4 There were many anatomic variations in this case and some were exploited to make the operation easier, so it was possible to keep to the right of the physiological continuity between the arterial and venous circulation. It should be remembered that modifications of certain surgical techniques, as carried out in this case, might be helpful in facilitating procedures. This is believed to be the first reported case of atrial situs inversus with primum atrial septal defect and a systemic and pulmonary venous return abnormality.


   References
TOP
 Abstract
 Introduction
 Case Report
 Discussion
 References
 

  1. Clarkson PM, Brandt PW, Barratt-Boyes BG, Neutze JM. "Isolated atrial inversion". Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: diagnosis and surgical correction. Am J Cardiol 1972;29:877–81.[Medline]

  2. Leijala MA, Lincoln CR, Shinebourne EA, Nellen M. A rare congenital cardiac malformation with situs inversus and discordant atrioventricular and concordant ventriculoarterial connections: diagnosis and surgical treatment. Am Heart J 1981;101:355–6.[Medline]

  3. Santoro G, Masiello P, Farina R, Baldi C, Di Leo L, Di Benedetto G. Isolated atrial inversion in situs inversus: a rare anatomic arrangement. Ann Thorac Surg 1995; 59:1019–21.[Abstract/Free Full Text]

  4. Lev M, Rowlatt UF. The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Am J Cardiol 1961;8:216–63.





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 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):
Tulga A Ulus
Oguz Tasdemir
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Birincioglu, C. L.
Right arrow Articles by Tasdemir, O.
Right arrow Search for Related Content
PubMed
Right arrow Articles by Birincioglu, C. L.
Right arrow Articles by Tasdemir, O.

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