Cor triatriatum represents <0.1% of all congenital cardiac malformations. Symptoms in patients with cor triatriatum are related to pulmonary venous obstruction and pressure loading of the right side of the heart. The aim of this study was to describe our institutional experience with repair of cor triatriatum. From June 1963 to June 2010, 65 patients underwent repair at a median age of 7.2 months (range 2 days to 47.6 years). Among these patients, 49 (75%) had associated congenital heart defects. Atrial septal defect (n = 29), ventricular septal defect (n = 15), partially or totally anomalous pulmonary venous return (n = 14), mitral valve abnormalities (n = 11), and supravalvar mitral ring (n = 5) were the most common associated defects. Surgical treatment consisted of excision of the membrane, along with additional procedures in 47 patients (72%). Five patients had new postoperative supraventricular arrhythmias. During a median follow-up period of 5.4 years, no patients underwent reintervention for recurrent left atrial obstruction, 7 patients were noted to have minor residual cor triatriatum without obstruction, and 8 patients (including 4 diagnosed before cor triatriatum repair) had pulmonary vein stenosis, 6 of whom underwent intervention for that reason. In conclusion, in this large surgical series of patients who underwent repair of cor triatriatum, there were no cases of significant residual or recurrent cor triatriatum. Although the association between cor triatriatum and pulmonary vein stenosis has been described previously, the relative frequency of this condition in our cohort (>10%, including patients diagnosed before and after cor triatriatum repair) is noteworthy. Abnormalities of the mitral valve and a supravalvar mitral ring were also seen more often than the existing research would suggest, which may be another important consideration in evaluating and following these patients.
Cor triatriatum is a rare condition, occurring in <1 in 1,000 patients with congenital heart disease. In its most common form, cor triatriatum sinister, the left atrium is divided into proximal (common pulmonary) and distal (left atrial) chambers, with a fibromuscular membrane that can cause restricted pulmonary venous return. The proximal chamber is usually located in a posterior-superior position, and the distal chamber is in an inferior-anterior position containing the atrial appendage and mitral valve. It is thought that failure of incorporation of the common pulmonary vein into the left atrium causes this problem. Symptoms in patients with cor triatriatum are related to pulmonary venous obstruction and pressure loading of the right side of the heart. Its clinical manifestations depend on the size of the orifice in the fibromuscular membrane and the pressure in the proximal chamber. Failure to thrive, recurrent pneumonia, feeding difficulties, tachypnea, resting or exertional dyspnea, tachycardia, heart failure, neonatal cyanosis, and arrhythmias, can also be presenting symptoms. Cor triatriatum can be diagnosed at any age, depending on the severity of the obstruction and concomitant symptoms. In symptomatic patients, treatment consists of resection of the membrane and, if necessary, treatment of the associated congenital heart defects. Lewis et al performed the first repair of cor triatriatum in 1956. Resection of the membrane is easily performed and resolves the related symptoms. The outcome of the disease is often dependent on the associated lesions more than the cor triatriatum itself. Approximately 250 cases of cor triatriatum repair have been described in the published research, most as case reports or small case series ( Table 1 ). Although cor triatriatum can be an isolated lesion, in many cases, it is associated with other congenital cardiovascular anomalies.
| Study | Number of Patients | Median/Mean (Range) Age at Repair (years) | Period of Study | Among Patients With Associated Anomaly | PAPVR/TAPVR | Left SVC | Pulmonary Vein Stenosis | Mitral Valve Anomaly | AV Canal Defect | ASD | VSD | Median/Mean (Range) Follow-Up Duration (years) | Recurrent Cor Triatriatum |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Humpl et al (2010) | 57 | 0.7 (1 d to 16.1 yrs) | 1954–2005 | 46 (81%) | 17 (30%) | 0 | 5 (9%) | 0 | 0 | 31 (58) | 5 (9%) | 3 (1 d to 18 yrs) | 0 |
| Huang et al (2007) | 9 | 1.2 (20 d to 31 yrs) | 1992–2005 | 5 (56%) | 3 (33%) | 0 | 0 | 0 | 1 (11%) | 4 (44%) | 0 | 4.3 ± 3.6 | 0 |
| Krasemann et al (2007) | 10 | 0.8 (30 d to 54 yrs) | 1992–2003 | 9 (90%) | 1 (10%) | 1 (10%) | 0 | 0 | 0 | 7 (70%) | 1 (10%) | — | — |
| Al Qethamy et al (2006) | 20 | 0.5 (30 d to 11 yrs) | 1983–2002 | 9 (45%) | 4 (20%) | 1 (5%) | 0 | 0 | 0 | 5 (25%) | 2 | Maximum 13 yrs | 0 |
| Alphonso et al (2005) , ⁎ | 28/27 | 0.6 (30 d to 20.2 yrs) | 1981–2003 | 22 (81%) | 8 (30%) | 6 (22%) | 0 | 1 (4%) | 0 | 14 (52%) | 4 (15%) | 8.2 (2 d to 23.6 yrs) | 0 |
| Gheissari et al (1992) , ⁎ | 12/7 | 0.9 (30 d to 7.5 yrs) | 1960–1988 | 9 (75%) | 1 (8%) | 3 (25%) | 0 | 0 | 1 (8%) | 3 (25%) | 0 | — | — |
| van Praagh and Corsini (1993) | 13 | 4 (0.6 to 57 yrs) | 1960–1992 | 9 (70%) | 0 | 3 (23%) | 0 | 0 | 2 (15%) | 3 (23%) | 1 (8%) | 15.7 (0.7 to 32) | 0 |
| Salomone et al (1991) | 15 | 0.4 (15 d to 48.1 yrs) | 1973–1988 | 10 (67%) | 2 (13%) | 2 (13%) | 0 | 0 | 0 | 10 (67%) | 1 (7%) | 4.8 ± 5.4 | 0 |
| Rodefeld et al (1990) | 11 | 1.5 (2 d to 15 yrs) | 1979–1989 | 6 (55%) | 2 (18%) | 1 (9%) | 0 | 0 | 0 | 4 (36%) | 1 (9%) | 1.8 | 0 |
| Oglietti et al (1983) | 25 | 2 (120 d to 38 yrs) | 1959–1980 | 20 (80%) | 7 (28%) | 9 (36%) | 2 (8%) | 0 | 2 (8%) | 11 (44%) | 1 (4%) | — | 1 (4%) |
| Total | 200 | — | 1954–2005 | 145 (73%) | 45 (23%) | 26 (13%) | 7 (4%) | 1 (0.5%) | 6 (3%) | 92 (46%) | 16 (8%) | — | 1 |
⁎ Results from all patients, not only those who underwent repair. All patients with cor triatriatum diagnosis/operated patients.
Methods
We reviewed the data for all patients who underwent surgical repair of cor triatriatum at Children’s Hospital Boston from June 1963 to June 2010, after approval from the Children’s Hospital Boston Institutional Review Board. Medical records were reviewed for demographic, diagnostic, and procedural data. Cross-sectional follow-up was obtained either from our institution or, if followed elsewhere, from the current cardiologist, with a focus on reintervention and recurrent intra-atrial obstruction. The study was primarily descriptive. Median values are presented for continuous variables, along with minimum and maximum values. Dichotomous variables are presented as frequencies and percentages of the relevant denominators. For comparison of pre- and postoperative data within patients, Wilcoxon’s signed-rank test was used.
Results
Sixty-six patients underwent repair of cor triatriatum during the study period, 1 of whom had cor triatriatum dexter. This report includes the 65 patients with left-sided cor triatriatum. There were 36 male patients (55%) and 29 female patients (45%). Eleven patients underwent surgery in the 1960s and 1970s, 7 in the 1980s, 22 in the 1990s, and 25 since 2000. The median age at operation was 6.9 months (range 2 days to 47 years); 6 patients (9%) were aged <30 days, and 37 (56%) were aged <12 months. Among infants aged <30 days, 1 had severe right ventricular hypertension due to isolated cor triatriatum, while the others underwent repair or palliation of complex heart defects during the same surgery. Most patients presented with signs and symptoms of congestive heart failure; additional presenting symptoms included failure to thrive (n = 10) and murmur (n = 8).
Associated congenital cardiovascular anomalies were present in 49 patients (75%), while 16 (25%) had isolated cor triatriatum ( Table 2 ). Among 14 patients with partially (n = 10) or totally (n = 4) anomalous pulmonary venous return, sites of anomalous drainage varied, as listed in Table 2 . Four patients were known to have pulmonary vein stenosis or atresia before cor triatriatum repair, with stenosis of multiple veins bilaterally in 2, right-sided pulmonary vein atresia and left-sided pulmonary vein stenosis in 1, and left lower pulmonary vein stenosis in 1.
| Associated Anomaly | n |
|---|---|
| Atrial septal defect | 29 (45%) |
| Secundum defect | 24 |
| Primum defect | 3 |
| Sinus venosus defect | 1 |
| Secundum and sinus venosus defect | 1 |
| Ventricular septal defect | 15 (23%) |
| Mitral valve abnormality ⁎ | 11 (17%) |
| Mitral valve prolapse | 4 |
| Mitral valve stenosis or hypoplasia | 4 |
| Mitral valve cleft | 2 |
| Parachute mitral valve | 1 |
| Partially anomalous pulmonary venous return ⁎ | 10 (15%) |
| Innominate vein | 3 |
| Right atrium | 2 |
| Left SVC | 1 |
| Right SVC | 1 |
| SVC–right atrium junction | 1 |
| Left SVC | 7 (11%) |
| To the left atrium (upper chamber) | 4 |
| To the coronary sinus | 3 |
| Bicuspid aortic valve | 7 (11%) |
| Supravalvar mitral ring/stenosis | 5 (8%) |
| Totally anomalous pulmonary venous return ⁎ | 4 (6%) |
| Coronary sinus | 1 |
| Left SVC | 1 |
| Infradiaphragmatic | 1 |
| Mixed drainage (coronary sinus and right atrium) | 1 |
| Pulmonary vein stenosis or atresia | 4 (6%) |
| Coarctation of the aorta | 4 (6%) |
| Aberrant right subclavian artery | 3 (5%) |
| Atrioventricular canal defect | 2 (3%) |
| Hypoplastic left heart syndrome | 2 (3%) |
| Transposition of the great arteries or double-outlet right ventricle | 2 (3%) |
| Tricuspid stenosis or atresia | 1 (1%) |
| Subaortic stenosis | 1 (1%) |
| Valvar pulmonary stenosis | 1 (1%) |
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