Asian Cardiovasc Thorac Ann 2006;14:7-9
© 2006 Asia Publishing EXchange Ltd
Cor Triatriatum: Operative Results In 20 Patients
Howaida O Al Qethamy, MD,
Saber Aboelnazar, MD,
Yahya Al Faraidi, MD,
Khalid Al Jubair, MD,
Mohamed Gadura, MBBCh,
Mohamed Fagih, MD
Department of Cardiac Surgery, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia
For reprint information contact: Saber Aboelnazar, MD Tel: 966 1 479 1000 Ext 8551 Fax: 966 1 476 0543 Email: saberaboelnazar{at}hotmail.com, Prince Sultan Cardiac Center, PO Box 7897 (Box No. N-641), Riyadh 11159, Saudi Arabia
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ABSTRACT
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Cor triatriatum is an uncommon cardiac anomaly occurring in 0.1% of children with congenital heart disease. We reviewed our results from 20 patients presenting with cor triatriatum in the last 20 years (from March 1983 to August 2002). Mean age at repair was 20 months (1 – 132 months). During a mean follow-up period of 31 months (2 – 156 months) there were no deaths. All patients were in sinus rhythm except one patient who needed pacemaker insertion one week after repair. A total of 14 patients were asymptomatic and 6 were in New York Heart Association Class I. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early, and when not associated with other severe cardiac anomalies.
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INTRODUCTION
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Cor triatriatum is an uncommon congenital cardiac anomaly in which all the pulmonary veins enter a common pulmonary venous chamber located behind the heart and separated from the true left atrial chamber (which contains the atrial appendage and the mitral valve) by a diaphragm that has one or more almost always restrictive ostia. In the current report, we describe our clinical experience with this malformation over the last 20 years.
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METHODS
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From March 1983 to August 2002, 20 patients (11 males and 9 females) presented with cor triatriatum. Diagnosis was made by cardiac catheterization in 12 patients and by cross-sectional two dimensional echocardiography in 4 patients. In another 4 patients both techniques were performed to exclude associated cardiac anomalies. (Table 1
). Chest roentgenogram demonstrated cardiomegaly in 9 patients and increased pulmonary vascularity of pulmonary venous congestion in 8 patients. The mean age at operation was 20 months (1 – 132 months) and the mean body weight was 7.3 kg (2.4 – 18.5 kg). The mean pulmonary artery pressure (PAP) was 35 mm Hg (23 – 88 mm Hg). A total of 12 patients had associated cardiac anomalies as shown in Table 1.
In all 20 patients, corrective surgery was performed with the use of standard cardiopulmonary bypass and moderate hypothermia with topical cooling. A trans-septal approach through the right atrium was used in 19 patients. A left atrial approach was used only in one patient. In all patients, the diaphragm was excised flush with the atrial septum and the left atrial free wall. Associated cardiac anomalies were corrected during the same operation. Echocardiogram was performed routinely before discharge to evaluate adequacy of the repair, in addition to mitral valve and ventricular function.
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RESULTS
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There was no operative death. Mean crossclamp time was 39 minutes and mean cardiopulmonary bypass time was 48 minutes. Follow-up data was obtained from the records of clinical examination and echocardiographic findings documented at the time of the patient’s last visit to the outpatient clinic. All patients were in sinus rhythm except one who needed pacemaker insertion one week after repair. Fourteen patients were asymptomatic, 4 were in New York Heart Association (NYHA) Class I and 2 patients were lost to follow-up (Table 1
).
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DISCUSSION
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Cor triatriatum was first described by Church in 1868.1 It occurs in approximately 0.1% of children who have congenital heart disease, with a male to female ratio of roughly 1:1. Successful operation for this anomaly was described in the literature in 1956 by two different investigators.2,3 The number of surgically treated patients who have since been reported is small.
Cor triatriatum produces symptoms by causing pulmonary venous obstruction and pressure loading of the right side of the heart. The clinical symptoms may be attenuated by a large opening in the diaphragm, the presence of a patent foramen ovale or atrial septal defect between the common pulmonary venous chamber and the right atrium, the presence of a partial anomalous pulmonary venous connection or a decrease in pulmonary blood flow.4,5,6
Patients with a small opening in the diaphragm in isolated cor triatriatum have a large left atrial gradient and usually require medical attention considerably earlier in life than patients with a less restrictive opening in the diaphragm, or with other mechanisms that reduce pulmonary venous chamber hypertension. On the basis of the arforementioned mechanisms, the diagnosis may be difficult to establish in the differential presentation of cor triatriatum in which the cross-sectional area of the opening in the diaphragm is sufficiently large to produce minimal or no gradient.4 The diagnosis may also be incorrect or incomplete in patients with cor triatriatum who have associated cardiac defects in which pulmonary venous obstruction is hemodynamically mild or masked. This situation may prevail when a patent foramen ovale or an atrial septal defect between the pulmonary venous chamber and the right atrium is present, a partial anomalous pulmonary venous connection to the right atrium exists, or pulmonary blood flow is decreased, such as with tetralogy of Fallot, pulmonary stenosis, or banding of the pulmonary artery. If cor triatriatum is not diagnosed, pulmonary venous obstruction may occur after repair of the associated defects and thus, the outcome is adversely affected.7 Cardiac catheterization has occasionally not disclosed the diagnosis of cor triatriatum, especially when this anomaly occurred in combination with other cardiac defects.8,9,10,11
The surgical approach in the current series was chosen primarily on the basis of the presence of associated lesions and the preference of the surgeon. A left atrial approach opens the common pulmonary venous chamber, offers a direct exposure of the diaphragm, and is useful when the left atrium is enlarged. It was used in one patient. A right atrial, trans-septal approach was used in 19 patients. It provides a wider exposure of the anomaly, especially if the left atrium is small. After the atrial septum has been incised, the entire obstructing diaphragm can be clearly seen and completely excised. The right atrial approach is also preferred when associated lesions such as sinus venosus atrial septal defect must be repaired.
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CONCLUSION
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Accurate preoperative or intraoperative diagnosis of cor triatriatum followed by complete excision of the diaphragm between the pulmonary venous chamber and the true left atrium has yielded excellent long-term results up to 13 years after operation. Surgical correction of cor triatriatum restores a normal anatomic and hemodynamic status, which, especially in patients without major associated cardiac anomalies, allows functional Class I activity and probably normal life expectancy.
This paper was presented at the 15th Annual Meeting of the Mediterranean Association of Cardiology and Cardiac Surgery (MACCS), September 10 – 13, 2003, Beirut, Lebanon.
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REFERENCES
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- Church WS. Congenital Malformation of heart: Abnormal Septum in left auricle. Trans Pathol Soc Lond 1868;19:188–90.
- Lewis FJ, Varco RL, Taufic M, Niazi SA. Direct vision repair of triatrial heart and total anomalous pulmonary venous drainage. Surg Gynecol Obstet 1956;102:713–20.[Medline]
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ABSTRACT
INTRODUCTION
