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Ann Thorac Surg 1997;63:741-744
© 1997 The Society of Thoracic Surgeons

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Original Article: Cardiovascular

Recognition and Management of Obstructed Pulmonary Veins Draining to the Coronary Sinus

Departments of Thoracic and Cardiovascular Surgery and Pediatrics, Loyola University Medical School, Maywood, Illinois

Accepted for publication October 17, 1996.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. Obstruction of the pulmonary veins in total anomalous pulmonary venous drainage to the coronary sinus is generally considered rare. However, if it is present, the usual treatment of unroofing the coronary sinus will lead to a poor result.

Methods. Four patients with total anomalous pulmonary venous drainage to the coronary sinus with obstruction were identified over a 14-month period. Three patients in whom the diagnosis of obstruction was not made underwent coronary sinus unroofing. Retrospective review of the preoperative echocardiograms and Doppler studies showed the presence of obstruction in the vertical vein in 2 patients and in the branches in the other. In the fourth patient, obstruction in the vertical vein was recognized preoperatively with echocardiography and Doppler study. This patient underwent direct common pulmonary vein–left atrial anastomosis.

Results. All 3 patients who had coronary unroofing were seen with obstructed pulmonary veins 2 to 7 months postoperatively. After reoperation, 1 died, and the other 2 have done relatively well 3 and 15 months postoperatively. The patient who had an anastomosis between the common pulmonary vein and the left atrium is doing well 18 months postoperatively.

Conclusions. Obstruction in total anomalous pulmonary venous drainage to the coronary sinus is not as rare as previously reported. To improve outcome, its presence should be sought using complete echocardiography including Doppler studies. When obstruction is present, transection of the vertical vein and common pulmonary vein–left atrial anastomosis through the superior approach is an attractive technique that also eliminates the right-to-left shunting associated with coronary sinus unroofing and simplifies closure of the atrial septal defect.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
See also page 744.

Obstruction of pulmonary veins that drain to the coronary sinus is not well recognized and is generally considered extremely rare [1–4]. However, a 1987 study [1] suggests that it may be more common than previously thought. The usual treatment of unroofing the coronary sinus leads to a poor result if obstruction is present. Early detection and surgical correction of obstruction are necessary to prevent severe right-sided heart failure and death.

We have seen several patients in whom severe pulmonary venous obstruction developed after coronary sinus unroofing. To further increase awareness of and minimize the development of this life-threatening complication, we retrospectively analyzed the data of our patients. Such analysis forms the basis of this report.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
From October 1993 to December 1994, 4 patients with obstruction of the vertical vein draining to the coronary sinus were identified. Their preoperative findings, operations, and outcomes were analyzed. The median age at operation was 2.8 months (range, 3 days to 3.5 months). Median weight at operation was 3.51 kg (range, 3.14 to 3.56 kg). Preoperative oxygen saturation ranged between 75% and 98%. Preoperative respiratory rate ranged between 40 and 72 breaths/min. In all instances, chest radiographs revealed mild cardiomegaly, increased pulmonary vascularity, minimal to no pleural effusions, and no pulmonary edema.

Preoperative Echocardiograms and Doppler Study
All patients had preoperative echocardiograms and Doppler studies. In 3 patients, obstruction was not recognized, although retrospective review of the echocardiograms showed evidence of obstruction. In 2 of the 3 patients, the right and left pulmonary veins formed a confluence or common pulmonary vein that drained to the coronary sinus through a narrowed or obstructed short vertical vein where turbulent flow was noted (Fig 1). In 1 of these 2 patients, the left upper lobe vein drained through another vertical vein to the innominate vein.

View larger version (68K): [in this window] [in a new window]   Fig 1. . (A) (Patient 1.) Echocardiogram showing the common pulmonary vein draining to the coronary sinus ( CS) through an obstructed or narrowed vertical vein (arrows). (B) (Patient 2.) Similar echocardiogram showing a narrowed vertical vein (arrows). The left upper lobe vein drained to the innominate vein. (RA = right atrium.)

View larger version (98K): [in this window] [in a new window]   Fig 2. . (Patient 3.) Echocardiogram showing narrowing at the branches ( arrows). The vertical vein is not obstructed. (CS = coronary sinus.)

Operative Techniques at Initial Operation
Cardiopulmonary bypass was established with an ascending aortic cannula and a single venous cannula through the right atrial appendage. Moderate hypothermia (25°C bladder temperature) was used. Once the first dose of antegrade blood cardioplegia was given, a transverse right atriotomy was made through which the superior and inferior venae cavae were separately cannulated. In the 3 patients in whom the presence of obstruction was not recognized preoperatively, the coronary sinus was unroofed, and the atrial septal defect and the coronary sinus were closed together with a single pericardial patch. In 1 patient, the left upper lobe vein was anastomosed to the base of the left atrial appendage. Antegrade blood cardioplegia and ice slush were used in 10-minute intervals. Warm blood cardioplegia was given before the release of the aortic clamp.

In the fourth patient, whose preoperative diagnosis included vertical vein obstruction, cannulation was accomplished in the same fashion. The common and vertical veins were approached superiorly [5, 6] between the superior vena cava and the ascending aorta (Fig 3). The vertical vein was transected and the distal end, closed. The proximal end was enlarged with an incision going toward both pulmonary vein branches, and the common pulmonary vein was anastomed to the back of the left atrium. The atrial septal defect was closed primarily.

View larger version (19K): [in this window] [in a new window]   Fig 3. . (A) Total anomalous pulmonary venous drainage to coronary sinus with narrowed vertical vein (VV). (B) Transection of the VV through the superior approach. Incision is made on the back of the left atrium (LA) and in the common pulmonary vein (CPV). (C) A direct CPV–LA anastomosis is performed. Incision on the CPV is extended to the branches as needed.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

Echocardiograms and Doppler studies in all 3 patients showed turbulent flow either at the branches or at the vertical vein. The right ventricular wall was quite hypertrophied as a result of systemic right ventricular pressure. Cardiac catheterization confirmed the obstruction of the vertical vein in 2 patients and the branches in 1 patient. The right ventricle had systemic pressure. The left upper lobe vein that was anastomosed to the left atrial appendage in 1 patient was widely patent.

Reoperation was performed in all 3 patients through the superior approach between the superior vena cava and the ascending aorta. The vertical vein was transected, and the common pulmonary vein was anastomosed directly to the back of the left atrium in 2 patients. In the patient who had obstruction at the branches, the narrowing between the right pulmonary veins and the coronary sinus was patched with pericardium. The left upper lobe vein, which was completely occluded, was followed toward the hilum of the lung and anastomosed to the base of the left atrial appendage. The left lower lobe vein could not be found.

One patient died of a pulmonary hypertensive crisis on the fifth postoperative day. The patient with a widely patent left upper pulmonary vein–left atrial appendage anastomosis has done well for 15 months postoperatively. The patient who had branch stenosis required two more reoperations. In the last operation, intravascular stents were implanted in the orifices of the right and left pulmonary veins. This patient has done well for 3 months postoperatively.

The fourth patient, who had primary repair using direct anastomosis between the common pulmonary vein and the left atrium after transection of the vertical vein, has done well for 18 months postoperatively.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Early studies of predominantly autopsy material in total anomalous pulmonary venous drainage to the coronary sinus showed an extremely low incidence of pulmonary venous obstruction [1]. In carefully analyzed clinical series, however, the incidence can be as high as 36% [1].

The most common site of pulmonary venous obstruction in total anomalous pulmonary venous drainage to the coronary sinus appears to be at the vertical vein that joins the confluence of the right and left pulmonary veins (common pulmonary vein) to the coronary sinus [1]. Obstruction can also occur at the junction of the branches with the vertical vein or coronary sinus. Obstruction of the coronary orifice itself is quite rare [6, 7].

Although the presence of pulmonary venous obstruction should theoretically result in lower skin oxygen saturation preoperatively, it was not helpful in our patients because of the wide range (75% to 98%). The chest radiographs also failed to show pulmonary edema in any of our patients. The reflex pulmonary vasoconstriction probably masks the presence of obstruction and pulmonary edema in these patients.

Complete echocardiography that includes cross-sectional images and color Doppler studies appears to be a very reliable tool (100% sensitivity, 85% specificity) in detecting the presence of obstruction [4]. It did provide evidence of obstruction in all 4 of our patients, albeit retrospectively in 3 of them. It showed obstruction by demonstrating a narrowed segment in the pulmonary venous pathway and the presence of a nonphasic Doppler spectrum or turbulent flow in the pulmonary vein confluence, vertical vein, or coronary sinus [7–10]. It has been suggested to be superior to angiography in the detection of obstruction [4].

Although cardiac catheterization was used extensively in early series to diagnose total anomalous pulmonary venous drainage and the presence of obstruction, its current role is quite diminished because of the sophistication of complete two-dimensional and Doppler echocardiography [1, 4]. Although cardiac catheterization was helpful in 1 of our patients who had an obstructed vertical vein and an obstruction of the left pulmonary veins, these would have been easily detected and appropriately managed at operation even without foreknowledge of their existence. Cardiac catheterization probably should be reserved for instances when pulmonary hypertension is detected by echocardiography and the presence or absence of obstruction cannot be clearly established. It has been suggested that when the pulmonary artery pressure is 85% of the systemic pressure or higher, the presence of obstruction is likely [1, 3, 11]. In questionable cases, retrograde visualization of the vertical vein or pulmonary vein orifices or both through the coronary sinus at the initial procedure would help. The noninvasive form of preoperative assessment saves the patient from the risks of cardiac catheterization and allows that patient to be in better clinical condition for operation [4, 7].

Although the presence of obstruction has some adverse impact on the long-term outcome in total anomalous pulmonary venous drainage, the prognosis is still quite favorable when the obstruction is relieved at the initial operation. However, the prognosis becomes grim when obstruction appears or develops postoperatively [12–14]. Pulmonary venous obstruction leads to rapid development of pulmonary arterial and venous medial hypertrophy [3, 7]. In 1 of our patients, such problems developed in the obstructed right and left lower pulmonary veins but not in the unobstructed left upper lobe vein, findings indicating that the hypertrophic changes in the vessels are secondary to the presence of obstruction.

In the presence of obstruction in the vertical vein, direct anastomosis between the common pulmonary vein and the left atrium should bypass the obstruction and improve outcome. The superior approach appears to be simple and ideal for this repair. The incision on the common pulmonary vein can be extended to the branches if there is an additional obstruction there. Transection of the vertical vein eliminates the right-to-left shunting associated with the unroofing of the coronary sinus. In addition, closure of the atrial septal defect is simplified.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Address reprint requests to Dr DeLeon, Department of Thoracic and Cardiovascular Surgery, Loyola University Medical Center, 2160 S First Ave, Maywood, IL 60153.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

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12. Hammon JW, Bender HW, Graham TP, Boucek RJ, Smith CW, Erath HG. Total anomalous pulmonary venous obstruction in infancy. J Thorac Cardiovasc Surg 1980;80:544–51.[Abstract]
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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Serafin Y. DeLeon Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by DeLeon, M. M. Articles by Fisher, E. A. Search for Related Content PubMed PubMed Citation Articles by DeLeon, M. M. Articles by Fisher, E. A. Related Collections Related Articles