Asian Cardiovasc Thorac Ann 2001;9:106-110
© 2001 Asia Publishing EXchange Pte Ltd
Cor Triatriatum: Clinical Presentation of 18 Cases
Ryszard W Lupinski, MD, PhD,
Sriram Shankar, MBBS,
Wong Keng Yean, MBBS1,,
Chan Yoke Hwee, MBBS2,,
Susan M Vosloo, MBChB3,,
Jacek J Moll, MD, PhD4,
Department of Pediatric Cardiothoracic Surgery
1 Department of Pediatric Cardiology Service
2 Department of Pediatric Medicine
Kandang Kerbau Women's and Children's Hospital
Singapore, Republic of Singapore
3 Department of Cardiothoracic Surgery
Red Cross Memorial Children's Hospital
Cape Town, South Africa
4 Department of Pediatric Cardiovascular Surgery
WOK Hospital
Zabrze, Poland
|
|
|
For reprint information contact: Ryszard W Lupinski, MD, PhD Tel: 65 394 1132 Fax: 65 291 0161 email: richardl{at}kkh.com.sg Department of Pediatric Cardiothoracic Surgery, Kandang Kerbau Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Republic of Singapore.
|
 |
Abstract
|
|---|
From 1985 to 1999, 18 patients with cor triatriatum underwent surgical correction. Their ages ranged from 3 months to 9 years (mean, 32.5 months). Eight patients were cyanosed; the other 10 presented with congestive heart failure. Recurrent chest infection was observed in 10 cases, and failure to thrive in 7. One patient had isolated cor triatriatum, and 17 had an associated atrial septal defect. Other associated anomalies included patent ductus arteriosus, ventricular septal defect, and partial and total anomalous pulmonary venous connection. Two deaths occurred perioperatively in patients with associated severe heart defects. Follow-up ranged from 1 month to 10 years. No late events occurred among the survivors, and all were in New York Heart Association functional class I. Their most recent echocardiograms showed no residual obstruction or shunt and good development of the left cardiac chambers. Echocardiography is recommended as the diagnostic modality of choice. Cor triatriatum can be corrected surgically with low mortality and good long-term results.
|
Introduction
|
|---|
Cor triatriatum is a rare cardiac malformation accounting for only 0.1% of all cardiac defects. Clinical presentation depends on the degree of restriction of blood flow from the upper chamber to the lower chamber of the left atrium through a fibromuscular membrane containing one or more fenestrations.1,2 The aim of this study was to describe the clinical presentation of cor triatriatum sinistrum, surgical and anatomical findings, and outcome after surgical correction.
|
Patients and Methods
|
|---|
Of 18 patients with cor triatriatum, 8 were treated in South Africa, 9 in Poland, and 1 in Singapore, between 1985 and 1999. Their case notes were reviewed retrospec-tively. Demographic features, clinical presentation, results of investigations (chest radiographs, electrocardiograms), and surgical or autopsy findings were studied. Echo-cardiography and cardiac catheterization were performed to assess the heart defect. The demographic variables are listed in Table 1
. There were 5 females and 13 males; their mean age was 32.5 months (range, 3 months to 9 years), and 6 patients were younger than one year. Ten patients had a history of recurrent chest infection and 7 presented with failure to thrive. The clinical findings are summarized in Table 2. Eight children were cyanosed and 10 presented with congestive heart failure. Right ventricular hypertrophy on an electrocardiogram was observed in all cases. On chest radiography, cardiomegaly was observed in 17 patients and plethora in 10.
Excision of the cor triatriatum membrane was ac-complished through a right atrial approach (Figure 1), with the aid of cardiopulmonary bypass in all patients except one in whom an additional left atrial incision was made to clarify anatomy. One patient had cor triatriatum alone. In 17 patients, there was an associated atrial septal defect (ASD). Other associated defects are listed in Table 1
.
View larger version (278K):
[in this window]
[in a new window]
|
Figure 1. Surgery for cor triatriatum. (A) Surgical forceps inside the obstructing membrane. (B) Left atrium after the obstructing membrane has been excised (arrow).
|
|
|
Results
|
|---|
All patients required inotropic support in the perioperative period. Two deaths occurred postoperatively due to low cardiac output in patients with associated complex heart defects. Other complications included reoperation for left atrial-to-inferior vena cava fistula, and left hemiparesis due to unrelated pathology. Follow-up ranged from 1 month to 10 years. No late events occurred among the survivors, and all were in New York Heart Association functional class I. Their most recent echocardiograms showed no residual obstruction or shunt and good development of the left cardiac chambers.
Depending on the presence and localization of an ASD, there are 4 types of cor triatriatum (Figure 2). Type I (intact interatrial septum) was found in 1 patient, type II (ASD to upper left atrial chamber) was observed in 8, type III (ASD to lower left atrial chamber) was noted in 3, and 6 had type IV (ASD to both chambers).
|
Discussion
|
|---|
In cor triatriatum, the remnants of the common pulmonary vein (the upper left atrial chamber) and primitive left atrium (the lower left atrial chamber) are separated by a fibromuscular diaphragm containing one or more fenestrations that allow some passage of blood from the upper to the lower chamber. The left atrial appendage usually arises from the lower chamber.1 Patients with this anomaly are usually symptomatic from birth.1,2 A few reports have described adults diagnosed with cor triatriatum.3,4 Clinical manifestations depend on the size of the opening and the degree of obstruction of venous return. When this is severe, signs and symptoms occur.1,2 Congestive heart failure, recurrent chest infections, failure to thrive, and cyanosis are typical.1,2 Pulmonary hyper-tension is usually present.1,5 Electrocardiographic features include right atrial dilation and right ventricular hyper-trophy. Radiographically, the heart is moderately enlarged and the lungs may reveal some degree of congestion with fluid.1,2 The diagnosis is usually confirmed by echo-cardiography.3,6–10 M-mode and 2-dimensional echocar-diography demonstrate a membrane in the mid left atrium. Doppler echocardiography is useful to assess the degree of flow obstruction within the left atrium.8,11–13 Cineangiography usually delineates clearly the membrane and associated lesions.
An ASD or foramen ovale connects the right atrium to the lower chamber in approximately 80% of cases. Often, additional malformations may be associated.2 Absent communication between the left and right atria has seldom been reported, but it was found in one of the patients in this study.11,12 Associated anomalies observed previously include patent ductus arteriosus, ventricular septal defect, total and partial anomalous pulmonary venous connection, coarctation of the aorta, pulmonary stenosis, atrioventricu-lar septal defect, and double-outlet right ventricle.6,14–16 Rare associations of cor triatriatum with tetralogy of Fallot, Ebstein's anomaly, and transposition of the great arteries have also been reported.17–19
Up to 50% of affected patients may die within the first 2 years of life, but successful surgical correction can be carried out.2,20 Excision of the cor triatriatum membrane is usually accomplished via a right atrial approach, but an additional left atrial incision is needed sometimes to clarify the anatomy.11,12 This happened in one of the cases in this study. All associated anomalies should be corrected simultaneously. Death occurs mainly from low cardiac output in patients with associated complex anomalies.4 Late postoperative echocardiography in survivors usually reveals no flow obstruction within the left atrium and good development of the left cardiac chambers.
It was concluded from this study that echocardiography is the diagnostic modality of choice for cor triatriatum, and this rare defect can be corrected surgically with low mortality and good long-term results. Cor triatriatum seldom appears as a solitary heart defect. Of the various associated lesions, the most common is an atrial septal defect.
Presented at the 8th Annual Meeting of The Asian Society for Cardiovascular Surgery, Fukuoka, Japan, September 6–8, 2000.
|
References
|
|---|
-
Arey JB. Cor triatriatum. In: Cardiovascular pathology in infants and children. Philadelphia: Saunders, 1984:298–9.
-
Behrendt DM. Cor triatriatum sinistrum, pulmonary vein stenosis and atresia of the common pulmonary vein. In: Arciniegas E, editor. Pediatric cardiac surgery. Chicago: Year Book, 1985:367–71.
-
Brigui M, Jenayeh N, Remadi F, Belkhiria N, Hassine R, Ata J. Study of cor triatriatum. Apropos of 6 cases [French]. Ann Cardiol Angeiol (Paris) 1993;42:546–9.[Medline]
-
Rodefeld MD, Brown JW, Heimansohn DA, King H, Girod DA, Hurwitz RA, et al. Cor triatriatum: clinical presentation and surgical results in 12 patients. Ann Thorac Surg 1990; 50:562–8.[Abstract]
-
Gupta PR, Sinha PR, Avasthey P. Cor triatriatum with atrial septal defect and bicuspid aortic valve diagnosed by cross-sectional echocardiography. Indian Heart J 1990; 42:457–8.[Medline]
-
Robida A, Eltohami EA. Double-outlet right ventricle associated with cor triatriatum sinistrum. Chest 1994; 105:290–1.[Abstract/Free Full Text]
-
Miki O, Kitagawa T, Yoshizumi M, Masuda Y, Fukumura Y, Katomi I. A case report of Lucas-Schmidt IIA type cor triatriatum in neonate [Japanese]. Nippon Kyobu Geka Gakkai Zasshi 1992;40:2212–6.[Medline]
-
Castela E, Antunes M, Ribeiro L, Marinho A, Manuela E, Sa e Melo A, et al. Cor triatriatum. Echo/Doppler diagnosis followed by immediate surgical correction. Report of a clinical case [Portuguese]. Rev Port Cardiol 1990;9: 981–3.[Medline]
-
Ronderos Dumit MA, Flores JC, Aza Ortiz J, Rico Gomez F, Maitre Azcarate MJ, Quero Jimenez M. Cor triatriatum. Diagnosis by 2-dimensional echography: apropos of 3 cases [Spanish]. Rev Esp Cardiol 1986;39:308–10.[Medline]
-
Sethia B, Sullivan ID, Elliot MJ, de Leval M, Stark J. Congenital left ventricular inflow obstruction: is the outcome related to the site of the obstruction? Eur J Cardiothoracic Surg 1988;2:312–7.[Abstract]
-
van Son JA, Danielson GK, Schaff HV, Puga FJ, Sewara JB, Hagler DJ, et al. Cor triatriatum: diagnosis, operative approach, and late results. Mayo Clin Proc 1993;68: 854–9.[Medline]
-
Oda K, Ando F, Okamoto F, Ikeda T, Yamanaka K, Otani S, et al. A surgical case of cor triatriatum [Japanese]. Kyobu Geka 1993;46:1137–9.[Medline]
-
Mori K, Dohi T. Mitral and pulmonary vein blood flow patterns in cor triatriatum. Am Heart J 1989;117:1167–9.[Medline]
-
Nagata M, Ishii K, Onitsuka T, Yonezawa T, Kuwahara M, Nakamura H, et al. Surgical treatment of cor triatriatum: report of four cases [Japanese]. Kyobu Geka 1990;43: 793–8.[Medline]
-
Nishikawa Y, Yamaguchi M, Oshima Y, Ohashi H, Hosokawa Y, Tei T, et al. Cor triatriatum associated with coarctation of the aorta and VSD [Japanese]. Kyobu Geka 1990;43:929–32.[Medline]
-
Starc TJ, Bierman FZ, Bowman FO Jr, Steeg CN, Wang NK, Krongrad E. Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double-outlet right atrium. J Am Coll Cardiol 1987;9:830–3.[Abstract]
-
Binotto MA, Aiello VD, Ebaid M. Coexistence of divided left atrium (cor triatriatum) and tetralogy of Fallot. Int J Cardiol 1991;31:97–9.[Medline]
-
Babliak DE, Ivaniv IA. Successful surgical treatment of the left tri-atrial heart associated with Ebstein's anomaly [Russian]. Kardiologiia 1987;27:113–5.
-
Thies WR, Matthies W, Minami K, Pott U, Meyer H, Körfer R. Surgical repair and postoperative course of an infant with infracardiac total anomalous pulmonary venous connection, cor triatriatum sinistrum and transposition of the great arteries. Eur J Cardiothorac Surg 1990;4:45–7.[Abstract]
-
Nagatsu M. Clinical classification and surgical treatment of cor triatriatum [Japanese]. Nippon Kyobu Geka Gakkai Zasshi 1992;40:473–84.[Medline]
TOP
Abstract
Introduction
