Cor Triatriatum Surgery in the Pediatric Patient Treatment & Management

  • Author: David L Morales, MD; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
 
Updated: Feb 06, 2015
 

Medical Therapy

In the patient with pulmonary edema or right heart failure, the usual medical management should be instituted. However, at this stage, the disease is usually quite progressive, and surgical therapy should be instituted as soon as possible.

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Preoperative Details

Preoperatively, the surgeon must try to determine with near certainty the connections and drainage of all of the pulmonary and systemic veins. Particular attention is paid to the drainage of the individual pulmonary veins and the presence and drainage of a left superior vena cava; at times, this may require imaging with echocardiography (CT or MRI). However, if the patient is nearly obstructed, one should not spend too much time trying to delineate exactly where all the venous structures are. This can be delineated in the operating room with a systematic and careful inspection of the heart.

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Intraoperative Details

The approach to these patients varies with the echocardiographic presentation. In general, the largest atrial chamber appearing on the right side of the heart should be initially opened at operation. The enlargement of the proximal left atrial chamber in typical cor triatriatum makes approach through an incision in the right side of this chamber the easiest. A right atrial approach can also be useful, particularly if a septal defect is open between the proximal left atrial chamber and the right atrium.

After the usual preparations, moderately hypothermic cardiopulmonary bypass using bicaval cannulation is instituted. After clamping the aorta and cardioplegic arrest, the right atrium is opened and then the septum primum is incised. At this point, careful inspection of all structures should be undertaken. Only when clear of where the proximal chamber and the mitral valve are should the common pulmonary venous chamber be opened. This can be done through the atrial septum or with an incision anterior to the right pulmonary veins, exactly as for mitral valve surgery. The diaphragm is exposed by appropriate retraction. The opening in the diaphragm is identified. One or two radial incisions from the opening of the diaphragm outward to the atrial wall or septum enhance exposure considerably. Most of the diaphragm is excised only after the pulmonary veins and mitral valve are clearly identified.[27]

If exposure is obtained through the right atrium, the atrial septal defect is enlarged to provide good exposure with the common pulmonary venous chamber and the right atrium. As before, the diaphragm is identified, and radial incisions from the center of the orifice outward are used to aid in exposure distal to the diaphragm.

Once the diaphragm is removed, the atrial septum has to be closed. If anomalous pulmonary veins are found, then the atrial septum should be recreated using a fresh pericardial patch, ensuring that the pulmonary veins are on the left side of the atrium and the superior and inferior vena cava drain into the right atrium. These seem like obvious tasks; however, in some of these patients with combinations of cor triatriatum and anomalous pulmonary venous return, this requires careful reconstruction or realignment of the atrial septum.

The cardiotomy is closed, the patient rewarmed, and the left heart deaired per routine. The remainder of the operation is carried out in the usual fashion, which includes a postoperative transesophageal echocardiogram (TEE) to ensure all structures are unobstructed and draining to the appropriate chamber. Cyanosis after separating for cardiopulmonary bypass should make one very closely examine the drainage of the superior vena cava (SVC) and inferior vena cava (IVC) to ensure entrance into the right atrium. A bubble study can be helpful to delineate this.

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Postoperative Details

Postoperative care for these infants is similar to other infants undergoing open heart surgery. However, the exception is that those infants presenting with severe obstruction, pulmonary edema, and pulmonary hypertension should be considered to be at risk for pulmonary hypertensive crises postoperatively and treated much like patients after repair of obstructive anomalous pulmonary venous return.

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Follow-up

Rarely, these children can develop pulmonary vein stenosis or re-stenosis of the orifice between the proximal and distal left atrial chambers (although this generally implies incomplete resection). Most re-stenoses occur within the first 1-2 years following resection, as observed following repair for total anomalous venous return. For this reason, follow-up care should be fairly frequent until the child no longer appears to be at risk for the development of re-stenosis.

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Complications

Hospital fatalities are uncommon after repair of CTS, except in the case of infants with other severe cardiac anomalies or presenting with severe obstruction.[28] Early complications are not dissimilar to any other operation performed on the left side of the heart in infants. Late complications generally consist of pulmonary vein stenosis or restenosis of the left atrial orifice as described above. In the follow-up period of the largest published series of CTS patients, most were in New York Heart Association functional class I or II, while 4% of the patients were functional class-III owing to pulmonary vein stenosis.[5]

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Outcome and Prognosis

Following complete resection, the life expectancy after repair of classic cor triatriatum is not significantly different from the general population. This, however, has not been clearly demonstrated.

Alphonso and colleagues have reported a survival of 96% and 88% at 5 and 15 years, respectively.[29] A 2014 retrospective review by Saxena et al involving 25 patients over a 52-year period reported an 83% at 10 years, with all patients in New York Heart Association class I or II at a mean follow-up of 12.8 years (maximum, 44 y).[27]

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Contributor Information and Disclosures
Author

David L Morales, MD Associate Professor of Surgery, Michael E DeBakey Department of Surgery, Division of Congenital Heart Surgery, Baylor College of Medicine; Surgical Director, Hospital Wide ECMO Program, Director, Cardiac Mechanical Circulatory Support Team, Director, Clinical and Industrial Research, Division of Congenital Heart Surgery, Director, North American Berlin Heart VAD Training and Reference Center, Texas Children’s Hospital; Cardiovascular Surgeon, Texas Heart Institute, St Luke’s Episcopal Hospital

David L Morales, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Association for Academic Surgery, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons, Texas Pediatric Society, Michael E DeBakey International Surgical Society

Disclosure: Nothing to disclose.

Coauthor(s)

M Shoaib Khan, MD Resident Physician, Department of Family Medicine, University of Oklahoma at Tulsa College of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John Myers, MD Director, Pediatric and Congenital Cardiovascular Surgery, Departments of Surgery and Pediatrics, Professor, Penn State Children's Hospital, Milton S Hershey Medical Center

John Myers, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, American Medical Association, Congenital Heart Surgeons Society, Pennsylvania Medical Society, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Daniel S Schwartz, MD, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, FACS is a member of the following medical societies: Society of Thoracic Surgeons, Western Thoracic Surgical Association, American College of Chest Physicians, American College of Surgeons

Disclosure: Nothing to disclose.

References
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Relevant intracardiac anatomy. The pulmonary veins empty into a chamber, which then empties into the right atrium. The proximal and distal left atrium is divided by a membranous wall. The right atrium then communicates to the right ventricle via the tricuspid valve and to the left atrium via a secundum atrial septal defect.
Color flow imaging confirms flow from the pulmonary veins into the proximal left atrial chamber, exiting directly into the right atrium. Flow is shown crossing the atrial septum via an atrial septal defect and entering the left atrium. No flow is seen coursing between the proximal and distal chambers in the left atrium.
Color flow imaging confirms flow from the pulmonary veins into the proximal left atrial chamber, exiting directly into the right atrium. Flow is shown crossing the atrial septum via an atrial septal defect and entering the left atrium. No flow is seen coursing between the proximal and distal chambers in the left atrium.
 
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