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J Thorac Cardiovasc Surg 1998;115:468-470
© 1998 Mosby, Inc.
BRIEF COMMUNICATIONS |
Toronto, Ontario, Canada
From the Division of Cardiovascular Surgery, Department ofSurgery, Hospital of Sick Children, and University of Toronto Faculty ofMedicine, Toronto, Ontario, Canada.
Received for publication May 9, 1997 Accepted for publication Sept. 18, 1997. Address for reprints: John G. Coles, MD, Division of CardiovascularSurgery, The Hospital of Sick Children, 555 University Ave., Toronto, OntarioM5G 1X8 Canada.
Pulmonary vein (PV) stenosis develops as a progressive and usually lethalcomplication after surgical repair of total anomalous PV connection.Conventional surgical repair for the management of recurrent PV stenosis hasbeen generally unsuccessful because of proliferative neointimal hyperplasiaresulting in recurrent PV obstruction. The factors that result in recurrentstenosis after the usual types of patch venoplasty are unknown. We speculatedthat direct suturing of PVs and patch material may be the substrate forturbulent blood flow triggering intimal hyperplasia and eventual narrowing ofthe vein. On the basis of these considerations, we developed a suturelesstechnique for repairing PV stenosis with in situ pericardium. We present hereits early but promising results.
Patients
PATIENT 1. Patient 1 was born in January 1995 with infradiaphragmatictotal anomalous PV connection and severe obstruction of the descending verticalvein. On day 1, the infradiaphragmatic anomalous vein was ligated and theconfluence was anastomosed to the left atrium under conditions of hypothermiccirculatory arrest. At 6 months, the child had conspicuous tachypnea.Echocardiography revealed obstructed left PVs with a mean gradient of 7 mm Hg(peak 16 mm Hg) and suprasystemic right ventricular pressure. Angiocardiographydemonstrated variable obstruction of all four PVs, with a mean pulmonary arterypressure of 38 mm Hg (systolic 92 mm Hg). At reoperation, obstruction of all PVswas confirmed. Under conditions of circulatory arrest, a pedicled flap of freeright atrial wallsuperior vena cava junction based at the inferior venacava was used to patch the right-sided veins and carried behind the aorta topatch the left upper PV. The left lower vein was repaired with a flap createdfrom the left atrial appendage.
1
After that operation, the child had repeated admissions with respiratorytract infections, complicated at 1 year by hemoptysis. Echocardiography revealedrecurrence of obstruction. At the second reoperation, atretic left-sided veinsand severe stenosis of right-sided veins were noted. The PVs were opened, and anin situ pericardial sutureless patch was used for reconstruction.Echocardiography a year later showed patent veins, with mean gradients of 4 mmHg on the right side and 5 mm Hg on the left. The estimated mean pulmonaryartery pressure was 10 mm Hg. Perfusion lung scan showed 67% perfusion onthe right side and 33% on the left. The child currently has no symptoms.
PATIENT 2. Patient 2, a female infant, was born on January 1995 withtotal anomalous PV connection to coronary sinus with echocardiographic evidenceof partial obstruction. At 2 weeks, she underwent repair consisting of unroofingof the coronary sinus, as described by Van Praagh and Harken.
2 On completion of the procedure, thepatient could not be weaned from cardiopulmonary bypass. At this time the rightventricular pressure was suprasystemic. The child was placed on extracorporealmembrane oxygenator and subsequently weaned after 3 days, with delayed sternalclosure in 10 days as a result of persistent hemodynamic instability.Echocardiographic findings before discharge revealed unobstructed PV confluenceleftatrium connection. However, echocardiography 4 months later revealed anobstructed right upper PV with a mean right ventricular pressure of 25 mm Hg.Perfusion scan showed 87% perfusion to the left lung and 13% tothe right. Eleven months after the operation, echocardiography and cardiaccatheterization revealed pulmonary hypertension, with a mean pulmonary arterypressure of 34 mm Hg and anatomically right PV stenosis.
At reoperation, the presence of severe bilateral PV stenosis extendingfrom left atrium a variable distance into the intraparenchymal PVs wasconfirmed. An in situ pericardial baffle was used for repair, as described here.Cardiac catheterization 6 months later revealed normal venous drainage on theleft side and mild obstruction on the right side. The mean pulmonary arterypressure was 16 mm Hg. Perfusion lung scan showed 42% to left lung and 58%to right. The child has no symptoms at 15 months of postoperative follow-up.
Technique. Standard cardiopulmonarybypass technique is used. The incision is made into the left atrium and extendedinto both upper and lower PV ostia separately (Fig. 1, A). This incision can be carried into thesecondary, and if necessary, the tertiary branches of both the upper and lowerlobe veins to a level at which the intima appears grossly normal. Thepericardium overlying the entrance of the PVs is used to create an enlargedcommunication between the opened PVs and the left atrium. The neoatrium iscreated by suturing the pericardium to the epicardium of the left atrium,completely circumscribing the opening in the left atrium and PVs so that the PVeffluent is contained by pericardium. The reconstruction is initiated with twoparallel vertical incisions in the pericardium extending from anterior toposterior; the superior incision is slightly above the junction of the superiorPV with the left atrium, and the inferior incision is slightly below the levelof the inferior PV. Suturing is begun in the pericardium just above the junctionof the superior PV with the left atrium
(Fig. 1, B)taking care to avoid distortion of the PV. A few tacking sutures may be placedto secure the adventitia of the opened PV to the adjacent pericardium tomaintain the PVs in the fully opened position. The suture line is continuedtoward the left atrium opening, joining the medial aspect of the left atrialincision. A second inferior suture is started below the inferior PV andcontinued in the same manner to the left atrial incision to join the superiorsuture line
(Fig. 1, C). Care should be taken whilesuturing the inferior limb of the pericardial baffle to obliterate thecommunication with the oblique sinus, especially on the left side, if notalready obliterated by adhesions. On both the left and right sides, it isnecessary to carefully mobilize the phrenic nerve with its accompanying vesselsand fat pad from the adjacent pericardium to avoid entrapment in the pericardialsuture line.
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We, as well as others,
6have speculated that turbulent flow accompanying congenital PV stenosis or thatoccurring after imperfect surgical repair of total anomalous PVs acts as astimulus for PV intimal injury and triggers a neointimal fibrous proliferativeresponse. The injury response may be further amplified by the trauma associatedwith direct suturing of the PV intima. The sutureless in situ pericardialreconstruction described in this report was designed to minimize factors thatcould contribute to the pathogenesis of PV obstruction. This technique issimilar to that described in a case report in which the authors describedexcision of the PVs, left atrial connection, and reconstruction with asutureless pericardial pouch.
7Phrenic nerve injury, a potential complication of this procedure, was avoided inour two cases by careful mobilization of the neurovascular pedicle.
In our previous experience,
4recurrent obstruction has invariably occurred within a few months after surgicalprocedures, with or without adjunctive stent implantation, to relieve bilateralPV obstruction that has developed after repair of total anomalous PV drainage.This provides some optimism for the durability of the sutureless technique,which has successfully eliminated PV obstruction without evidence of progressiveflow alteration during 18 months of follow-up.
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