Surgical repair of cor triatriatum sinister: the Mayo Clinic 50-year experience.

BACKGROUND: Cor triatriatum is a rare congenital cardiac defect in which the atrium is divided into 2 chambers by a membrane causing obstruction to the blood flow in either the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dexter) eventually leading to cardiac failure. We sought to review our surgical experience with cor triatriatum sinister. METHODS: Twenty-five patients underwent surgical correction of cor triatriatum between May 1960 and September 2012. There were 11 males and 14 females with a mean age of 27.4 years (age range, 1 day to 73 years). RESULTS: All patients underwent excision of cor triatriatum membrane using cardiopulmonary bypass. Twenty patients (80%) required concomitant cardiac surgical procedures. There was no early mortality. None of the patients had any residual atrial obstruction. Two infants who had concomitant repair of complex congenital anomalies died at 2 and 5 months postoperatively after discharge from hospital. Kaplan-Meier survival at 10 years was 83%. All patients were in New York Heart Association class I or II at a mean follow-up of 12.8 years (maximum 44 years). CONCLUSIONS: Surgical repair of cor triatriatum provides satisfactory early and long-term survival with low risk for additional intervention. Cor triatriatum with complex congenital anomalies may be associated with adverse outcome.

Outcome after repair of cor triatriatum.

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 sinistrum in childhood. A single institution's experience.

INTRODUCTION: Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients. OBJECTIVE: To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome. METHODS: Hospital databases were searched to identify patients with CTS who were diagnosed between 1954 and 2005. Medical records with demographic data, clinical evaluation, diagnostic approach, interventions and autopsy results were reviewed. RESULTS: Between 1954 and 2005, 82 patients (43 female children, 52%) with CTS were diagnosed at the institution. Patients were born between 1951 and 2004, and the median age at presentation was eight months (range one day to 16.1 years). The majority of patients (77%) presented with associated cardiac lesions. Of 82 patients, 57 (70%) underwent resection of the fibromuscular diaphragm, 14 (17%) did not require surgery and 11 (13%) did not survive the time to intervention. Nineteen patients (23%) died a median of two months (range one day to 5.5 years) after presentation. Nine of these patients (11%) died before surgery was attempted. Most patients were completely asymptomatic at the time of the last follow-up at a median of three years (range one day to 18 years). Echocardiography significantly facilitated the diagnosis and subsequent management of patients with CTS. CONCLUSIONS: Despite the diversity in presentation and complexity of associated lesions, the outcome for patients with CTS is favourable.

Cor triatriatum sinistrum: one institution's 28-year experience.

Twelve patients with cor triatriatum sinistrum were treated over a 28-year period. Their ages ranged from 1 month to 7.5 years. Congestive heart failure was the most common presentation. Cardiac catheterization was performed on six of the 12 patients and a correct diagnosis of cor triatriatum was made on angiography in only four of the six. Of the remaining six patients, three were diagnosed as having cor triatriatum by echocardiography and three by autopsy. Echocardiography is now considered to be the diagnostic modality of choice in our institution. Seven patients were operated on and five died prior to diagnosis or treatment Associated cardiac anomalies included persistent left superior vena cava, atrial septal defects, coarctation of the aorta, and total anomalous pulmonary venous drainage. A right atrial, transseptal approach to the common pulmonary chamber and excision of the left atrial membrane was found to be the treatment of choice and was used in six of the seven patients operated on. One patient died in the postoperative period. Thus, cor triatriatum sinistrum, a rare and potentially lethal congenital cardiac anomaly, can be diagnosed by echocardiography and successfully treated surgically with a low operative mortality.

Cor triatriatum: clinical presentation and surgical results in 12 patients.

Twelve patients with cor triatriatum have been seen at our institution since 1979. The clinical presentation, diagnostic evaluation, and surgical results are outlined in this retrospective review. Operation is the treatment of choice for this rare congenital cardiac defect. One patient died 1 day before scheduled operation, and 2 patients died postoperatively, yielding a surgical mortality rate of 17% and an overall mortality rate of 25%. Resection of the obstructing atrial membrane was performed using hypothermic cardiopulmonary bypass in all cases. Left atriotomy was performed in 6 patients, and right atriotomy was performed in 7. The two postoperative deaths occurred in patients who had serious associated cardiac defects. Associated anomalies include atrial septal defect, persistent left superior vena cava, and partial anomalous pulmonary venous return. The postoperative course has been excellent in all 9 surviving patients; all remain asymptomatic. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early and when not complicated by other severe cardiac anomalies.