eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Partial and Total Anomalous Pulmonary Venous Connection, Surgical Treatment: Treatment

Author: Jayme Scott Bennetts, MD, Fellow, Department of Cardiac and Thoracic Surgery, Flinders Medical Centre
Coauthor(s): Christopher A Caldarone, MD, Associate Professor, Department of Surgery, The Hospital for Sick Children, University of Toronto
Contributor Information and Disclosures

Updated: May 1, 2009

Treatment

Medical Therapy

Patients with total anomalous pulmonary venous drainage (TAPVD) usually present in 1 of 2 ways. First, early in the postnatal period, patients present with profound cyanosis, acidosis and shock secondary to obstruction of pulmonary venous return. Second, late in the postnatal period, patients present with right heart failure, poor feeding, and poor growth secondary to right heart volume overload. On occasion, patients present with late-onset obstruction due to progressive stenosis in the pathway for pulmonary venous return or at the site of the intracardiac shunt.

Presentation with obstruction of TAPVD with severe cyanosis and poor systemic perfusion is a medical emergency. Anatomic impediments limit resuscitation to restore cardiac output resulting from obstruction in the pulmonary venous circuit. Maneuvers to increase effective pulmonary blood flow include correction of acidosis, ventilation with 100% oxygen, and hyperventilation. Administration of prostaglandin E₁ (eg alprostadil, PGE₁) may allow some right-to-left shunting at the level of the ductus arteriosus. This maneuver may increase systemic cardiac output, though it may do so at the expense of pulmonary blood flow. Correction of systemic acidosis is limited and may be impossible in this clinical situation.

Obstruction of pulmonary venous drainage in patients with TAPVD is a surgical emergency, and prompt correction of the venous anomaly is required.

In some patients with TAPVD, the obstruction is at the level of a restrictive foramen ovale. In this situation, balloon atrial septostomy (BAS) can decompress the venous circuit and improve cardiac output. This change allows time to correct systemic acidosis with bicarbonate, fluids, and inotropic support.

Patients with partial anomalous pulmonary venous drainage (PAPVD) most commonly present as an incidental finding of murmur or abnormal chest radiographs during routine medical examination. Those who present with primary arrhythmia or right heart failure may benefit from antiarrhythmic agents or management of right heart failure while the diagnostic workup is being completed and before surgical correction is considered.

Surgical Therapy

The goal of surgical therapy for TAPVD is to create unobstructed egress of blood from the pulmonary veins into the LA.³

The goal of surgical therapy for PAPVD is to correct the cardiac anatomy and divert pulmonary venous flow from the anomalous pulmonary vein into the LA in an unobstructed fashion. Associated defects (eg, ASDs) are corrected.⁴

Preoperative Details

Preoperative echocardiographic definition of the pulmonary venous anatomy and associated defects is important in planning the appropriate cannulation technique and surgical approach. This point is especially pertinent in PAPVD, in which the location of the anomalous pulmonary venous drainage influences the use of a minimally invasive sternotomy incision and placement of venous cannulae. A high junction of a right-sided anomalous pulmonary vein into the SVC makes a minimally invasive approach considerably more difficult.

In patients with obstruction resulting in cyanosis and acidosis, resuscitative correction should be attempted while emergency surgical correction is being organized.

Intraoperative Details

Total anomalous pulmonary venous drainage

Aortobicaval cannulation offers flexibility to repair all forms of TAPVD. In some centers, deep hypothermic circulatory arrest is preferred, but this technique is rapidly becoming less common than before.

After cardiopulmonary bypass is started, ductus arteriosus ligation is done, as is systemic cooling to 18-20%. The aorta is cross-clamped, and cold antegrade cardioplegia is administered. The vertical vein is usually ligated, but some centers may prefer to leave this in continuity.

In supracardiac TAPVD, the pulmonary venous confluence is seen in the posterior pericardium by retracting the heart. A longitudinal incision is created in the pulmonary venous confluence to match a corresponding incision in the posterior LA extended out to the LA appendage. With care to avoid distortion, a LA-to-pulmonary confluence anastomosis is created using fine sutures. Controversy exists over the use of absorbable suture material (polydioxanon [PDS]) versus nonabsorbable suture material (eg, Prolene). Many centers report good results using nonabsorbable sutures. The benefits are thought to be due to minimizing the degree of intimal hyperplasia and potential restenosis. The primary goal is a large, unobstructed anastomosis. A short period of low-flow cardiopulmonary bypass may be desirable for a meticulous anastomosis.

For intracardiac TAPVD, a technique similar to that described above is used. An anastomosis is created between the pulmonary venous confluence, lying in the posterior pericardium, and the back of the LA. Other techniques involve unroofing the coronary sinus through an incision between the coronary sinus and the area of the foramen ovale. Then, a patch is used to reconstruct the atrial septum, leaving the pulmonary venous drainage to flow through the unroofed coronary sinus into the LA. Concern has been expressed that this unroofing technique may increase the risk of late stenosis because of the embryologic origin of the connection between the coronary sinus and the pulmonary venous confluence.

For infracardiac TAPVD, the technique is similar to that described for supracardiac TAPVD. Pulmonary venous confluence tends to be oriented vertically, creating a Y -shaped confluence, which drains through a vertical vein to the ductus venosus. As a consequence, the incision into the LA is relatively vertical or Y -shaped to maximize the size of the newly created LA. Some surgeons do not ligate the vertical vein to provide the effect of a pressure-relief valve to oxygenated blood to enter the systemic circulation if LA pressure is high in the early postoperative period.

Surgical correction of mixed TAPVD depends on the exact anatomy and site of pulmonary venous connections. A combination of the aforementioned procedures is usually required to completely redirect pulmonary venous blood to the LA.

Sutureless pulmonary venous connection is a novel technique performed to connect the pulmonary vein to the atrium by suturing the atrial wall to the incised pericardial edge overlying the incised pulmonary vein. Hemostasis is provided by continuity of the fibrous tissue between the pericardium and venous confluence. The resulting anastomosis requires no suture placement into the pulmonary vein, minimizing direct surgical trauma and intimal fibrosis and hyperplasia secondary to suturing. Although this technique was initially described for the management of recurrent stenosis in the pulmonary venous tree, indications have been extended to repair of primary pulmonary-vein stenosis and primary repair of TAPVD. For TAPVD the same approach, as outlined above, for each type is used with the anastomotic technique being a sutureless type.

Partial anomalous pulmonary venous drainage

The position of the anomalous pulmonary vein determines the site of cannulation in the SVC. In most patients, the SVC or the innominate vein is cannulated to provide venous drainage for the upper extremities. After cardioplegic arrest, systemic normothermia is maintained. The RA is opened, and the pulmonary veins and any ASDs are identified.

A glutaraldehyde-treated pericardial double-patch technique is typically used to create a baffle, redirecting pulmonary venous blood from an anomalous right upper pulmonary vein beneath the baffle and through an ASD into the LA. The second patch augmenting the remaining SVC above the baffle to prevent SVC obstruction.

A single-patch technique was recently proposed. This involves a transverse atriotomy extending along the lateral RA and across the cavoatrial junction along the SVC to just proximal to the point of inflow of an anomalous right superior pulmonary vein. A single pericardial patch is then fashioned to close the sinus venosus type of ASD and to create a baffle through the posterior SVC and over the origin of the right superior pulmonary vein. The atriotomy is then closed by incorporating the lateral limit into the suture line.

As an alternative, a new SVC-RA junction (Warden operation) is established by dividing the SVC just distal to the anomalous pulmonary venous entry and translocating the cephalic end of the SVC to a site at the RA appendage, where a SVC-to-RA anastomosis is performed. Take care to resect the trabeculations in the appendage to prevent obstruction of systemic venous drainage. The divided cardiac end of the SVC (bearing the anomalous pulmonary venous connection) is closed, and in the RA, a baffle is created from the site of the anomalous pulmonary vein to the ASD and LA.

Postoperative Details

Regarding TAPVD, poor hemodynamics should raise concern of potential obstruction at the site of repair. Chest radiographs demonstrate pulmonary vascular congestion, though this finding is nonspecific. In patients with preoperative pulmonary venous obstruction, chest radiographic findings of pulmonary vascular obstruction often persist for several days despite normal hemodynamics. Any suspicion of residual postoperative pulmonary venous obstruction should prompt echocardiography to examine and interrogate the pulmonary venous anastomosis.

Patients with obstructed TAPVD often have a difficult postoperative course secondary to high pulmonary vascular resistance and poor lung compliance. Some centers advocate the routine use of extracorporeal membrane oxygenation (ECMO) after surgical correction in these patients.

Follow-up

Pulmonary venous obstruction may arise in as many as 15% of patients after repair of TAPVD.³ The principle mechanism may be obstruction at the site of anastomosis, presumably as a result of scarring at the repair site or an inadequate anastomosis at the time of original repair. Repeat surgery is necessary to relieve the obstruction, and results are favorable. However, a sequela more serious than this is a diffuse stenotic process that can progress throughout the pulmonary venous tree. This process may progress to almost complete unilateral occlusion in relatively asymptomatic patients. However, the process is most commonly bilateral, and the prognosis is poor. Recently developed sutureless techniques and/or lung transplantation may improve the outlook for patients in this situation.

Patients with PAPVD should also be followed up for evidence of stenosis at the site of pulmonary venous repair, though the diffuse stenosis described after repair of TAPVD is rare.

Older patients with raised pulmonary artery pressures should be followed up for resolution of these changes after surgery. As an alternative, patients presenting with arrhythmia should be followed up for control of arrhythmia and for ongoing indications for therapy.

Complications

Residual obstruction at the site of pulmonary venous repair is manifested by poor cardiac output and chest radiographic findings of pulmonary congestion. The principle diagnostic feature is turbulence at the pulmonary venous anastomotic site, as noted on echocardiographs. Residual turbulence may create a cycle of local injury, hyperplasia, and increasing turbulence, perpetuating a process of diffuse pulmonary vein stenosis. Pulmonary vein stenosis may remain localized to the site of anastomosis, may progress unilaterally with diffuse pulmonary vein stenosis, or it may progress with bilateral diffuse pulmonary vein stenosis.

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