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Ann Thorac Surg 1998;66:1514-1520
© 1998 The Society of Thoracic Surgeons
a Division of Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto and University of Toronto Faculty of Medicine, Toronto, Canada
Address reprint requests to Dr Caldarone, Division of Cardiovascular Surgery, The Childrens Hospital of Iowa at the University of Iowa Hospitals and Clinics, 200 Hawkins Dr 1616A JCP, Iowa City, IA 52242-1083
e-mail: (Chris-Caldarone{at}UIowa.edu)
Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 2628, 1998.
| Abstract |
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Methods. Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques.
Results. Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes (p < 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died (p < 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years follow-up. Stenting was uniformly unsuccessful.
Conclusions. In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression.
| Introduction |
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In this report, the clinical course of 13 patients with PR-PVS after repair of TAPVD is described. Surgical management of this group is analyzed to provide a background against which to compare a novel surgical technique designed to minimize local injury in stenotic pulmonary veins. Specifically, a sutureless technique is described that augments the size of the left atrium while avoiding any suturing of the divided edges of stenotic pulmonary veins. The sutureless neoatrium technique evolved in response to speculation that intraoperative factors (eg, local tissue injury at the site of anastomosis) may stimulate the perpetuation of a progressive fibrotic response that rapidly spreads through the pulmonary veins. The procedure was applied to 2 patients in the high-risk subset of patients returning to the operating room with pulmonary vein stenosis after apparently successful initial repair.
| Methods |
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Surgical technique
To be included in the study, all 13 patients had at least one intervention for PR-PVS. Three patients had a second reoperation, and 1 patient had a third reoperation. Sixteen of the 17 procedures performed were operative repairs. One patient was treated in the catheterization lab with stenting of the left lower pulmonary vein. Four other stenting procedures were performed in the operating room as a component of other procedures. A breakdown of the operative techniques is summarized in Table 1.
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In 2 patients with bilateral stenosis, a sutureless neoatrium was constructed. The application and description of the procedure can be illustrated by summarizing the hospital course of the 2 patients in which it was used.
Case 1
Patient 1 presented at 14 days of age with TAPVD to the coronary sinus and clinical evidence of stenosis. On echocardiogram, stenosis was noted at the junction of the pulmonary venous confluence and the coronary sinus with a peak gradient of 14 mm Hg. There was an unrestrictive atrial septal defect and right-sided hypertension (right ventricular systolic pressure, 53 mm Hg; cuff systolic pressure, 60 mm Hg). The patient was brought to the operating room, and the coronary sinus unroofed back to the pulmonary venous confluence; a pericardial patch was used to close the remaining atrial septal defect. After the repair, the patient had severe pulmonary hypertension and ultimately required extracorporeal membrane oxygenation support for 48 hours, after which the cannulas were successfully removed from the patient. Residual pulmonary vein stenosis was noted on postoperative day 9. Although the patient had a prolonged stay in the intensive care unit, she was discharged after 6 weeks.
The patient was noted to have progression of right pulmonary vein stenosis by echocardiogram within 8 months, associated with failure to thrive. Perfusion scan demonstrated 87% flow to the left lung and 13% to the right lung. Echocardiogram 2 months later demonstrated bilateral pulmonary vein stenosis. Subsequent catheterization demonstrated right ventricular pressures that were one-half systemic and pulmonary wedge pressures that were 26 mm Hg on the right and 16 mm Hg on the left. Severe stenosis of the right pulmonary veins as well as moderate stenosis of the left pulmonary veins was noted.
The patient was brought to the operating room and was noted to have severe bilateral PR-PVS occurring at the junction of the pulmonary vein and the left atrium with extension of the thickened intima into the lung parenchyma.
Repair was accomplished with the sutureless neoatrium technique while under total circulatory arrest. Working from inside the left atrium, the common orifice to the left upper and lower pulmonary veins was opened with extension of the venotomies into the upper and lower branches. The pericardium (in situ) was then sutured to the left atrium at a distance from the pulmonary vein incisions, thereby creating a "controlled bleed" into a "neo" left atrium. There were no sutures placed in the incised pulmonary veins themselves. A similar procedure was used on the nearly atretic right-side pulmonary veins (Fig 1).
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Case 2
Patient 2 presented at birth with obstructed infradiaphragmatic TAPVD and cyanosis. On exploration in the operating room, the pulmonary veins were noted to drain to a vertically oriented confluence that drained below the diaphragm. Under total circulatory arrest, the confluence was anastomosed to the posterior left atrium using continuous Prolene (Ethicon, Somerville, NJ) sutures, the left atrium was enlarged with an autologous pericardial patch, and the descending vertical vein was ligated. Postoperative transesophageal echocardiogram demonstrated no evidence of obstruction. Pulmonary artery pressures, however, remained suprasystemic despite vasodilator and nitric oxide therapy.
By 4 months of age, the patient had clinical evidence of right ventricular hypertension and failure to thrive. Catheterization demonstrated bilateral pulmonary venous obstruction at the junction with the atrium, worse on the left than on the right. The left upper pulmonary vein was completely obstructed. Pulmonary artery pressures were approximately 66% of systemic pressure. At operation, circumferential fibrous stenosis was noted at the orifice of all four pulmonary veins. The repair was made by unroofing all four pulmonary veins. A pedicled flap of right atrium was used to augment the caliber of the right and left upper veins. The left atrial appendage was used as a patch to augment the size of the left lower pulmonary vein.
Echocardiogram at 1 month postoperatively demonstrated no evidence of pulmonary vein stenosis. At 4 months postoperatively, however, the patient was noted to have turbulent flow in all pulmonary veins with a mean gradient of 11 mm Hg on the right and 7 mm Hg on the left. Subsequent catheterization demonstrated nearly complete obstruction of all pulmonary veins and reactive pulmonary hypertension. Pulmonary artery pressures were again approximately 66% of systemic pressure.
At 1 year of age, the patient was again returned to the operating room. Severe stenosis was noted at the origin of all pulmonary veins. Under total circulatory arrest, the strictured pulmonary vein segments were unroofed and the incisions carried into the left atrium. The surrounding in situ pericardium was then sutured to the left atrium, creating a controlled bleed through the incised pulmonary veins into the neoatrial chamber. Again, there were no sutures placed in the incised edges of the pulmonary veins. Pulmonary artery pressures were approximately 50% of systemic pressure at the end of the operation.
Lung perfusion scan at 1 year demonstrated 33% flow to the left lung and 67% flow to the right lung. The patient is clinically asymptomatic. Echocardiogram 1.6 years after repair demonstrated mild 1+ bilateral obstruction with low right ventricular pressures. Clinically, at 1.8 years after repair, the patient is asymptomatic.
| Results |
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A similar pattern was found when comparing the age at operation for initial repair grouped by the presence of eventual unilateral versus bilateral pulmonary vein stenosis. There was no statistical difference in the age at original presentation for the patients who would eventually progress to unilateral or bilateral stenosis. When comparing the interval to intervention for PR-PVS, however, the patients with bilateral stenosis presented after a shorter interval (141 ± 112 days) compared with the patients with unilateral stenosis (828 ± 615 days, p = 0.0059 versus bilateral). Thus, the clinical progression of disease in patients with ultimately bilateral pulmonary vein stenosis was more rapid.
Actuarial survival was 51% at 10 years for patients with PR-PVS (Fig 3). When perioperative (<30 days) mortality is excluded, there was a statistically significant difference in survival between the total group of patients with TAPVD (n = 170) and the 13 patients with PR-PVS (p < 0.001) (Fig 4).
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In contrast to patients with unilateral stenosis, 6 of 9 patients with bilateral pulmonary vein stenosis died during the follow-up period (p < 0.05 versus unilateral stenosis). Of the 3 survivors, 2 underwent the sutureless neoatrium repair. The other survivor presented with mixed drainage, with infracardiac drainage to the left lung through a vertical confluence and right lung draining through the superior vena cavaright atrium junction. The right lung drainage had been decompressed with a baffle through an enlarged atrial septal defect. When stenosis developed, the left pulmonary anastomosis developed a diffuse fibrotic response. The right-sided portion of this bilateral stenosis, however, was limited to obstruction within the baffle. Although this patient is classified as having bilateral stenosis, scarring and fibrosis within the right-sided pulmonary veins was not observed. This patients most recent lung perfusion scan at 1,172 days postoperatively demonstrated 45% flow to the left lung and 55% to the right lung. The patient is entirely asymptomatic.
Five pulmonary vein stenting procedures were performed in 4 patients. Three of these 4 patients had bilateral stenosis and had bilateral stents placed. A fourth patient had a unilateral stent placed. Turbulent flow was present in all stented veins by postoperative Doppler echocardiography. All stented veins progressed to lethal bilateral stenosis (n = 3) or near complete unilateral pulmonary vein occlusion (n = 1).
Catheterization data before repair for PR-PVS were complete for 9 of the 13 patients. Of note, the ratio of pulmonary artery to systemic pressure tended to be higher in the nonsurvivors (0.71 ± 0.23 versus 0.46 ± 0.11). This difference, however, did not reach statistical significance (p = 0.08).
Median follow-up in the 13 cases of PR-PVS (100% complete) was 6.7 years. For the entire series (n = 170), the median follow-up was 4.7 years, and the duration of follow-up was greater than 1 year in 83% of the survivors.
| Comment |
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Wilson and colleagues [6] have attempted to define variables associated with improved outcome after repair of TAPVD. In their report, the actuarial freedom from restenosis was 82%. By multivariate analysis, the only risk factor for restenosis identified was the use of a type I repair (defined as suturing the common venous chamber directly to the left atrium with continuous suture through an apical or right-sided exposure). A type II repair, which used interrupted suture and a pericardial patch to augment a portion of the left atrialcommon venous chamber anastomosis was not identified as a risk factor for restenosis. Interestingly, intraoperative technical details were described as important determinants of recurrent stenosis. The authors speculate that "low grade venous obstruction present at the end of the procedure ... results in reactive fibrosis and self-perpetuating stenosis."
In the present study, we have attempted to define the clinical characteristics of the subset of patients presenting with PR-PVS. These patients presented within a year after their initial repair. More rapid development of PR-PVS is associated with a more rapid progression of diffuse stenotic lesions and early mortality. The presence of bilateral obstructive processes is also associated with the same rapid progression and dismal prognosis. Although the patients with unilateral PR-PVS had 100% survival, 2 of the 4 patients had progression of their unilateral disease to near complete or complete obstruction of the involved pulmonary veins, further emphasizing the relentless nature of the obstructive process.
Previous studies by Smallhorn and associates [7] have noted that the presence of echocardiographically defined turbulence within the pulmonary veins after repair is associated with restenosis. We hypothesize that imperfections in the surgically created venoatrial anastomosis can create local turbulence in some cases. Subsequently, focal injury from the suture line, suture material, or handling of the vein tissue can also serve as a focal point of acute injury. The local tissue injury and anastomotic turbulence is hypothesized to act in combination to generate a progressive upstream cycle of stenosis, increase turbulent flow, and further local injury.
The operative strategy in the present study was designed to minimize trauma at the suture line and avoid postoperative turbulence. By using a sutureless technique, we minimize handling of tissues and avoid any suture-related injury at the anastomosis. Furthermore, the lack of suture in the anastomosis should minimize any deformation of the common pulmonary chamber caused by tension or inflexibility from the suture itself. The pulmonary vein tissue geometry will be imposed nearly completely by the pulmonary vein flow through the anastomosis, rather than by a combination of pulmonary venous flow and the constraints imposed by restrictive characteristics of the suture line. The procedure also augments the size of the left atrium by creating a neoatrium.
Although the sutureless neoatrium technique could be used in routine cases of TAPVD at the original presentation; the relatively low incidence of PR-PVS makes it unlikely to be of easily demonstrable benefit when compared with more commonly used repairs. The subset of patients returning with bilateral postrepair obstruction, however, is a subset with a high rate of recurrent stenosis and poor overall prognosis. Within this subset, we have used the technique in 2 of the 9 patients with bilateral PR-PVS. Both patients, representing 2 of the 3 survivors in this series, are alive at 1.8 years follow-up.
In conclusion, PR-PVS can be manifest as a relentlessly progressive diffuse stenotic lesion within the pulmonary veins. Stenting has been uniformly unsuccessful for palliation. The presence of bilateral disease or rapid development of the lesion after initial repair is associated with poor prognosis. Even unilateral disease may progress to total obstruction in the involved lung. Because intraoperative factors may predispose to generation of PR-PVS, intraoperative strategies to minimize postoperative turbulence may help to reduce the incidence of this lesion.
| Footnotes |
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| References |
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