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

Transcription

1 Recognition and Management of Obstructed Pulmonary Veins Draining to the Coronary Sinus Maryann M. DeLeon, MD, Serafin Y. DeLeon, MD, Patrick T. Roughneen, MD, Timothy J. Bell, MD, Dolores A. Vitullo, MD, Frank Cetta, MD, Lynn Lagamayo, MD, and Elizabeth A. Fisher, MD Departments of Thoracic and Cardiovascular Surgery and Pediatrics, Loyola University Medical School, Maywood, Illinois 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 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. (Ann Thorac Surg 1997;63:741 5) 1997 by The Society of Thoracic Surgeons 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 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 Accepted for publication Oct 17, Address reprint requests to Dr DeLeon, Department of Thoracic and Cardiovascular Surgery, Loyola University Medical Center, 2160 S First Ave, Maywood, IL 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. The third patient whose obstruction was not recognized preoperatively showed obstruction of the right and left pulmonary veins at their junction with a dilated vertical vein or coronary sinus (Fig 2). Delineation between the vertical vein and coronary sinus could not be made. None of these 3 patients underwent preoperative cardiac catheterization by The Society of Thoracic Surgeons /97/$17.00 PII S (96)

2 742 DeLEON ET AL Ann Thorac Surg OBSTRUCTED PULMONARY VEINS 1997;63:741 5 A 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. B 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.) Results The postoperative course was uneventful in all patients. All 3 patients who underwent coronary sinus unroofing were seen with obstructed pulmonary veins 2, 3, and 7 months postoperatively. The patient with obstruction at the branches returned 7 months after operation. The main presenting symptoms in all patients were decreasing appetite and poor weight gain. 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 In the fourth patient, obstruction at the vertical vein was recognized on the preoperative echocardiogram. Cardiac catheterization showed a major gradient between the right and left mean pulmonary artery wedge pressures (10 and 21 mm Hg, respectively) and the coronary sinus pressure (5 mm Hg). These data indicated that there was an additional obstruction at the junction of the left pulmonary and common pulmonary veins. The systolic right and left ventricular pressures were 50 mm Hg and 60 mm Hg, respectively. 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 Fig 2. (Patient 3.) Echocardiogram showing narrowing at the branches (arrows). The vertical vein is not obstructed. (CS coronary sinus.)

3 Ann Thorac Surg DeLEON ET AL 1997;63:741 5 OBSTRUCTED PULMONARY VEINS 743 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. 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 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 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 foreknowl-

4 744 DeLEON ET AL Ann Thorac Surg OBSTRUCTED PULMONARY VEINS 1997;63:741 5 edge 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-toleft shunting associated with the unroofing of the coronary sinus. In addition, closure of the atrial septal defect is simplified. References 1. Jonas RA, Smolinsky A, Mayer JE, Castañeda AR. Obstructed pulmonary venous drainage with total anomalous pulmonary venous connection to the coronary sinus. Am J Cardiol 1987;59: Mazzuco A, Rizzoli G, Fracasso A, et al. Experience with operation for total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg 1985;85: Newfeld EA, Wilson A, Paul MH, Reisch JS. Pulmonary vascular disease in total anomalous pulmonary venous drainage. Circulation 1980;61: Wang JK, Lue HC, Wu MH, Young ML, Wu FF, Wu JM. Obstructed total anomalous pulmonary venous connection. Pediatr Cardiol 1993;14: Tucker BL, Lindesmith GG, Stiles QR, Meyer BW. The superior approach for correction of the supracardiac type of total anomalous pulmonary venous return. Ann Thorac Surg 1976;22: Lupinetti FM, Kulik TJ, Beekman RH, Crowley DC, Bove EL. Correction of total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg 1993;106: Sano S, Brawn WJ, Mee RBB. Total anomalous pulmonary venous drainage. J Thorac Cardiovasc Surg 1989;97: Casta A, Wolf WJ. Echo Doppler detection of external compression of the vertical vein causing obstruction in total anomalous pulmonary venous connection. Am Heart J 1988; 116: Smallhorn JF, Pauperio H, Benson L, Freedom RM, Rowe RD. Pulsed Doppler assessment of pulmonary vein obstruction. Am Heart J 1985;110: Van Hare GF, Schmidt KG, Cassidy SC, Gooding CA, Silverman NH. Color Doppler flow mapping in the ultrasound diagnosis of total anomalous pulmonary venous connection. J Am Soc Echocardiogr 1988;1: Smallhorn JF, Burrows P, Wilson G, Coles J, Gilday DL, Freedom RM. Two-dimensional and pulsed Doppler echocardiography in the postoperative evaluation of total anomalous pulmonary venous connection. Circulation 1987;76: 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: Schäfers H-J, Luhmer I, Oelert H. Pulmonary venous obstruction following repair of total anomalous pulmonary venous drainage. Ann Thorac Surg 1987;43: Turley K, Tucker WY, Ullyot DJ, Ebert PA. Total anomalous pulmonary venous connection in infancy: influence of age and type of lesion. Am J Cardiol 1980;45:92 7. INVITED COMMENTARY This report by DeLeon and co-workers reemphasizes the point that pulmonary venous obstruction is an important consideration in the management of total anomalous pulmonary venous connection to the coronary sinus. Embryologically, the coronary sinus is derived from the embryonic cardinal vein system, and there are connections between the venous drainage of the embryonic lung buds and the systemic veins prior to the development of the normal connections to the common pulmonary vein, which then merges with the posterior wall of the left atrium. In the usual case, these embryonic pulmonary venous to systemic venous connections disappear, but the presumptive mechanism for total anomalous pulmonary venous connection is persistence of the embryonic connections. It has been my impression that obstruction is more likely to be present in total anomalous pulmonary venous connection to the coronary sinus when the connection between the pulmonary veins and the coronary sinus is more distant from the coronary sinus orifice, ie, there is a longer length of coronary sinus beyond the site where the pulmonary veins connect. In the experience of my colleagues and myself, the obstruction generally occurs at this connection point rather than in the areas where the coronary sinus has been unroofed. The mechanisms responsible for the development of stenosis at this connection site are unknown, and the reasons why early pulmonary venous stenosis after repair is frequently associated with a progressive obliterative process in the more distal pulmonary venous system (as in patient 3 in 1997 by The Society of Thoracic Surgeons /97/$17.00 PII S (96)

5 Ann Thorac Surg DeLEON ET AL 1997;63:741 5 OBSTRUCTED PULMONARY VEINS 745 the accompanying report) are equally unclear. However, it seems that when the initial repair results in an unobstructed pulmonary venous pathway, the incidence of this pulmonary venous obliterative process is low. For this reason, I would agree that the preoperative identification of patients who are at risk for the development of pulmonary venous obstruction is critical to their initial management. The technique used primarily by DeLeon and associates in their fourth patient (in whom the diagnosis of obstruction was made preoperatively) and in the other 3 patients at reoperation where a direct connection was made between the common pulmonary vein and the posterior wall of the left atrium is virtually identical to the one I have used when the pulmonary vein coronary sinus connection is more distant from the heart and is either already obstructed or is likely to develop obstruction postoperatively. A reasonable argument can be made for employing this technique in all cases of total anomalous pulmonary venous connection to the coronary sinus, and this is now my approach in most patients. However, the important biologic question that should be addressed is the mechanism for the progressive pulmonary venous obliteration, which is fatal in a high proportion of the patients in whom it develops. An increased understanding of the signaling between the endothelium and the remainder of the vein wall may provide additional insights into the prevention of and therapy for this process. John E. Mayer, Jr, MD Department of Cardiovascular Surgery The Children s Hospital 300 Longwood Ave Boston, MA Notice From the Southern Thoracic Surgical Association The Forty-fourth Annual Meeting of the Southern Thoracic Surgical Association will be held at the Naples Registry Resort, Naples, Florida, November 6 8, The Postgraduate Course will be held the morning of Thursday, November 6, 1997, and will provide in-depth coverage of thoracic surgical topics selected primarily as a means to enhance and broaden the knowledge of practicing thoracic and cardiac surgeons. Members wishing to participate in the Scientific Program should submit an original abstract and one copy by May 9, 1997, to Irving Kron, MD, Program Chairman, Southern Thoracic Surgical Association, 401 North Michigan Avenue, Chicago, IL Abstracts must be submitted on the Southern Thoracic Surgical Association abstract form. These forms may be obtained from the Association s office or in the issue of The Annals of Thoracic Surgery. Manuscripts of accepted papers must be submitted to The Annals of Thoracic Surgery prior to the 1997 meeting or to the Secretary-Treasurer at the opening of the Scientific Session. Applications for membership should be completed by September 1, 1997, and forwarded to Michael S. Sweeney, MD, Membership Committee Chairman, Southern Thoracic Surgical Association, 401 North Michigan Avenue, Chicago, IL D. Glenn Pennington, MD Secretary-Treasurer Southern Thoracic Surgical Association 401 North Michigan Avenue Chicago, IL by The Society of Thoracic Surgeons Ann Thorac Surg 1997;63: /97/$17.00 PII S (97)