Asian Cardiovasc Thorac Ann 2003;11:18-22
© 2003 Asia Publishing EXchange Ltd
Repair of Total Anomalous Pulmonary Venous Connection in Early Infancy
Anil Kumar Dharmapuram, MCh,
Raghavan Nair Suresh Kumar, DM1,
Narasinga Rao Pantula, MCh,
Mahmoud Hassan Mohamed, MCh,
Sushil Chandran, MCh,
Achal Kumar Dhir, FRCA2,
Dileep Kumar Saxena, MD2,
Sivan Pillay Azhagappan, MD2,
Velayudhan Ramakrishna Pillai, DM1,
Venkitachalam Chokkanathapura Gopalakrishnan, DM1,
Mohamed Amin Fikree, FRCP1,
Yoosuph Abdul Nazer, MCh,
Timothy Boyd Cartmill, FRACS,
Ivatury Mrutyunjaya Rao, MCh
Department of Cardiac Surgery
1 Department of Cardiology
2 Department of Cardiac Anaesthesia, Al Mafraq Hospital, Abu Dhabi, UAE
For reprint information contact: Ivatury Mrutyunjaya Rao, MCh Tel: 971 2 503 1336 Fax: 971 2 582 1549 email: imrao{at}emirates.net.ae Department of Cardiac Surgery, Al Mafraq Hospital, P.O. Box 2951, Abu Dhabi, UAE.
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ABSTRACT
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From May 1995 through October 2001, 19 infants less than 90 days old underwent surgical correction of total anomalous pulmonary venous connection. In 15 babies with isolated total anomalous pulmonary venous connection, there was one operative death. In 4 with complex anomalies, there were 2 operative deaths. The vertical vein was not ligated in 6 cases for various reasons. Two patients died during reoperation for early pulmonary venous obstruction. In the late follow-up, 2 babies required reoperation for late anastomotic stricture; one needed additional balloon dilatation. Of the 14 surviving patients, one had a small residual gradient and infrequent supraventricular tachycardia, the others were asymptomatic and without gradients. Surgical correction of total anomalous pulmonary venous connection can be carried out in early infancy with low mortality and morbidity. However, associated complex cardiac anomalies and small caliber pulmonary arteries and veins carry higher risks. Recurrent pulmonary venous obstruction and diffuse pulmonary vein stenosis are causes of early reoperation and poor surgical outcome.
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INTRODUCTION
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Surgical management of infants with total anomalous pulmonary venous connection (TAPVC) has improved remarkably in recent years. Many centers have reported decreased morbidity and mortality.1–3 This might be attributed to very early detection, improvements in intraoperative anesthetic management, perfusion and myocardial preservation, better surgical technique, and more effective management of pulmonary hypertensive crises. However, associated complex cardiac anomalies such as heterotaxy, single ventricle, pulmonary atresia, small caliber pulmonary arteries and veins, and progressive pulmonary vein fibrosis continue to be determinants of poor surgical outcome.2–4 We present our experience in 19 infants who underwent TAPVC repair, with special attention to the fate of those who did not undergo vertical vein ligation during initial repair.5
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PATIENTS AND METHODS
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From May 1995 through October 2001, 19 infants aged less than 90 days underwent surgical correction of TAPVC in our institution. There were 14 males and 5 females. Their ages ranged from 5 to 90 days (median, 30 days); 12 were neonates, of whom 11 were less than 15 days old. Weights varied from 1.5 to 4.5 kg (median, 3.5 kg). Preoperatively, all babies were in congestive cardiac failure requiring medication. Six neonates were ventilated preoperatively, of whom 4 required inotropic support due to shock. One preterm neonate weighing 1.5 kg was ventilated preoperatively because of severe pulmonary arterial (PA) hypertension and congestive heart failure. Fifteen babies had isolated TAPVC and 4 had complex TAPVC associated with heterotaxy, isomerism, dextrocardia, single ventricle, pulmonary atresia, l-transposition, mitral atresia, and systemic venous anomaly (Table 1
). The anatomic types were supracardiac (10), infracardiac (4), and mixed (5). In the mixed type, most of the drainage from the pulmonary veins was into the coronary sinus, with the remaining venous drainage to the vertical vein. Cardiac catheterization was performed in 4 cases for specific reasons; 3 were in the complex TAPVC group (Table 1) and in one of these, the diagnosis of TAPVC was made after a modified Blalock-Taussig shunt had been performed for single ventricle and pulmonary atresia. In one case of isolated TAPVC, cardiac catheterization was undertaken to document unusually small caliber pulmonary arteries with an arborization defect. Twelve infants had obstruction of the common pulmonary venous channel (6 with supracardiac, 4 with infracardiac, and 2 with the mixed type of TAPVC). In supracardiac TAPVC, the obstruction was at the level of the vertical vein in all cases. In infradiaphragmatic TAPVC, the obstruction was at the level of the descending vertical vein or the constricted ductus venosus. In the mixed variety, the site of obstruction was at the level of the coronary sinus opening.
In all patients, surgery was undertaken on an urgent basis after establishing the diagnosis. Operations were performed using standard cardiopulmonary bypass (CPB). Aprotinin was used both intravenously and in the pump. The drug was given in a dose of 40,000 kallikrein inhibiting units (KIU) as a bolus intravenously on induction of anesthesia; the same dose was also added to the pump prime. The drug was continued as an intravenous infusion of 10,000 KIU•kg-1•h-1 throughout surgery and thereafter until satisfactory cessation of bleeding. The infusion was temporarily interrupted during total circulatory arrest. To prevent pre-bypass ventricular fibrillation, CPB was established with an aortic cannula and a venous cannula in the right atrial appendage, followed by relocation of angled venous cannulae into the superior and inferior venae cavae. The ductus or ligamentum arteriosum was dissected routinely in all patients, and looped and ligated immediately after initiating CPB. The vertical vein in infants with a supracardiac or infracardiac connection was handled only after initiating CPB. In infants with a supracardiac connection, the vertical vein was controlled extrapericardially between the pericardium and the left pleura.6 Myocardial protection was achieved with cold hyperkalemic antegrade blood cardioplegia and topical hypothermia.
In infants with a supracardiac connection, the anastomosis was constructed by the superior approach, using 7/0 polypropylene suture, between a widely opened common pulmonary vein and the roof and posterior wall of the left atrium, including the appendage. Exposure was facilitated by retraction of the ascending aorta and pulmonary artery. A foramen ovale or atrial septal defect was closed through a separate incision in the right atrium. In infants with an infradiaphragmatic connection, the right atrium was retracted towards the left, and a wide anastomosis was made between the pulmonary venous chamber and the left atrium. In infants with the mixed variety, the coronary sinus was unroofed and the openings of the coronary sinus and the atrial septal defect were closed separately. The decision to ligate the vertical vein was taken when coming off bypass. The vertical vein was not ligated in 6 cases, of whom 5 had supracardiac TAPVC; the reason for non-ligation was hemodynamic instability. In one case of infradiaphragmatic TAPVC, the descending vertical vein was electively left non-ligated.
The mean CPB time was 104.3 min (range, 70–190 min) and the mean aortic crossclamp time was 40.4 min (range, 23–70 min). Total circulatory arrest was used in 7 infants (mean duration, 26.2 min). In one infant with a supracardiac connection associated with single ventricle and pulmonary atresia, TAPVC repair was carried out under total circulatory arrest, followed by a right modified Blalock-Taussig shunt. In all infants, hemofiltration at a mean rate of 100 mL•kg-1 was performed during CPB. Filtration was started when the temperature reached 34°C, and continued until the patient was fully rewarmed. In the last 7 neonates, modified ultrafiltration was continued for 10 min after weaning from CPB, to achieve a satisfactory hematocrit. Inotropic support with dopamine was used during weaning from CPB. Routinely, PA lines were placed through the right ventricle to monitor PA pressure postoperatively.
The duration of postoperative ventilation ranged from 36 to 168 hours (mean, 72 hours). Two infants required prolonged postoperative ventilation: one had supracardiac TAPVC that was diagnosed only after failure to extubate after a Blalock-Taussig shunt for single ventricle and pulmonary atresia; the other had supra-systemic PA pressures. In all neonates, a Tenckhoff peritoneal dialysis catheter (Quinton Instrument Co., Bothell, WA, USA) was placed and removed before extubation. Dialysis was performed if there was evidence of significant capillary leak.
The PA pressures were monitored by the in-situ PA lines. Every effort was made to prevent pulmonary hypertensive crises through a multipronged strategy. All patients were given phentolamine (0.1 mg•kg-1) intravenously on initiating CPB, and the same dose was given later during rewarming. The last 7 babies underwent modified ultrafiltration routinely while coming off CPB. Postoperative strategy included complete sedation with paralysis until weaning from the ventilator, keeping endotracheal suction to a minimum, and the use of pharmacologic agents. All patients were kept on intravenous sodium nitroprusside (0.05 to 3 µg•kg-1•min-1). Captopril was given by nasogastric tube after extubation (0.1 to 0.2 mg•kg-1 3 times daily) while simultaneously coming off sodium nitroprusside. The PA lines were removed on the 7th postoperative day. Two-dimensional echocardiography was carried out in the immediate postoperative period and then daily to assess the anastomosis and left ventricular function. Inotropic support was tapered on the basis of left ventricular function; this was generally on the day after extubation, but inotropics had to be continued for a further 24 to 48 hours in 3 babies. Hospital stay ranged from 10 to 25 days (mean, 17 days). After discharge from the hospital, echocardiography was repeated at 1, 3, and 6 months during follow-up. Cardiac catheterization was performed when indicated in 6 infants at 2 months to 2 years.
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RESULTS
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There was one operative death among the 15 babies with isolated TAPVC, due to PA hypertensive crisis. This baby was preterm (1.5 kg) and required ventilation preoperatively due to severe PA hypertension and congestive heart failure. In the complex TAPVC category, there were 2 operative deaths (Table 1
). In one neonate, the pulmonary arteries were of small caliber with an arborization defect seen in the preoperative pulmonary angiogram. Inhaled nitric oxide (10 to 100 ppm) was given for 150 hours and he was successfully extubated. However, he needed reoperation for pulmonary venous obstruction 4 weeks after the initial surgery and died in the immediate postoperative phase due to intractable pulmonary hypertensive crisis. Reoperation was also required in a baby with mixed TAPVC and 3 pulmonary veins draining into the coronary sinus. After repair, the postoperative echocardiogram showed good pulmonary venous flow into the left atrium, with no anastomotic gradient. Six weeks postoperatively, the baby was readmitted with congestive heart failure due to a significant gradient at the pulmonary venous confluence. During reoperation, it was noted that the right-sided pulmonary veins showed diffuse fibrosis extending to the left atrium. The baby died intraoperatively.
Late anastomotic obstruction occurred in 2 infants who required reoperation for revision of the anastomosis (Table 2). One did well, but the other developed a significant residual gradient at the level of the anastomosis, involving the left pulmonary vein and associated with supraventricular tachycardia, 6 months after reoperation. This was treated by balloon dilatation resulting in only a small residual gradient, and supraventricular tachycardia became infrequent. Vertical vein ligation had to be performed in 2 babies in the follow-up period, due to significant left-to-right shunting through the patent vertical vein (Table 2). However, these babies were asymptomatic in functional class I with no anastomotic gradients.
All patients were followed up for 1 to 42 months (mean, 22.3 months). Six babies underwent cardiac catheterization; 2 had normal PA pressures and adequate anastomosis demonstrated angiographically, 4 were investigated for non-ligated vertical vein. Their hemodynamic and angiographic data are summarized in Table 2. The 13 surviving babies were all doing well and in functional class I without significant anastomotic gradients on Doppler color-flow imaging. The baby who had a reoperation followed by balloon dilatation for recurrent pulmonary venous obstruction had infrequent supraventricular tachycardia and a small residual gradient. She was doing well clinically at the last follow-up.
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DISCUSSION
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Recent improvements in surgical technique and postoperative management of TAPVC have reduced morbidity and mortality even in the most critically ill neonates. The early mortality ranges from to 0% to 11%.1–3 Primary obstruction at presentation in an infant with TAPVC is no longer considered to correlate with early mortality after surgery.2 In this series, the patients presented very early in infancy. Although the neonates were sick with congestive heart failure due to obstructed TAPVC (some requiring preoperative ventilation and inotropics), this was not a risk factor for operative mortality.
The surgical technique for correction of TAPVC has undergone several modifications since the initial repair by Muller in 1951.7 The inferior approach with retraction of the apex anteriorly might distort the cardiac and pulmonary venous anatomy and result in kinking of the anastomosis.1,8,9 In our experience, the superior approach between the superior vena cava and the ascending aorta as proposed by Tucker and colleagues10 provided good exposure of the roof of the left atrium as well as the common pulmonary vein.
Some controversy exists regarding routine ligation of the vertical vein at the time of TAPVC repair. In cases of infradiaphragmatic TAPVC, many surgeons elect to leave the vertical vein open because of the suggestion that the high resistance of the liver parenchyma eventually leads to complete cessation of flow through this low-pressure venous channel.11 Cope and colleagues12 extended this concept to management of the ascending vertical vein in supracardiac TAPVC. They suggested that ligation of the vertical vein is not necessary routinely; this could be lifesaving if the hemodynamics are unstable. During follow-up of these patients, they noted that the non-ligated vertical vein closed spontaneously if the anastomosis between the common pulmonary vein and left atrium was adequate.12 Our data show that such spontaneous closure does not necessarily occur (Table 2 ). In patient no. 1 who had late anastomotic stricture and high pressure in the common pulmonary vein, the vertical vein remained closed. In patient no. 2, in spite of an adequate anastomosis, the vertical vein remained large and required surgical ligation because of a significant left-to-right shunt. In patient no. 3, the descending vertical vein remained patent in spite of an adequate anastomosis after repair of infradiaphragmatic TAPVC. Patient no. 4 had patency of the vertical vein associated with anastomotic stricture. These findings do not support elective non-ligation of the vertical vein after repair of TAPVC; spontaneous closure cannot be taken for granted. Such patients should receive close echocardiographic monitoring until complete cessation of flow is demonstrated.5 If the vertical vein remains patent, the adequacy of the anastomosis should be verified. If the anastomosis is adequate, the vertical vein should be interrupted surgically or interventionally. On the other hand, if the vertical vein is not ligated, left-to-right shunting could be a significant consequence.13
Pulmonary hypertensive crisis is a major cause of morbidity and mortality in the surgical management of TAPVC in early infancy. Our strategy for prevention of such episodes failed in only 2 babies (10%), contributing to death. Inhaled nitric oxide was useful, but it did not alter the final outcome. Recurrent venous obstruction and pulmonary venous stenosis might compromise the long-term outcome after TAPVC correction but it can be managed by an early aggressive approach of reoperation and repeated balloon dilatation.14 In our experience, recurrent pulmonary venous obstruction tended to occur in the first few months following surgery. The infants who survived this period continued to do well in the long term. One patient who underwent reoperation for late anastomotic stricture developed pulmonary venous stenosis requiring balloon dilatation; she improved clinically. However, in another patient, pulmonary venous stenosis recurred very early after repair of a coronary sinus type of TAPVC. De Leon and colleagues15 recently drew attention to this problem which was originally reported by Jonas and colleagues16 in 1987. They found that in the coronary sinus type of TAPVC, preoperative obstruction was more common than previously suspected and could cause early restenosis and affect the long-term outcome. This might have been the pathology in our case. It might have been avoided if the operative technique had been direct anastomosis of the pulmonary venous confluence rather than conventional unroofing of the coronary sinus, as suggested by De Leon and colleagues.15
It was concluded that surgical correction of TAPVC can be carried out in early infancy with very low mortality and morbidity in the present era. Small caliber pulmonary arteries and veins can adversely affect the surgical outcome. Association of TAPVC with complex cardiac anomalies constitutes a high risk. Our experience does not support elective non-ligation of the vertical vein in surgical correction of TAPVC. If the vertical vein is not ligated, close echocardiographic follow-up is necessary as spontaneous closure of the vertical vein cannot be taken for granted.
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REFERENCES
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- Cope JT, Kron IL. Anomalies of pulmonary venous return and cor triatriatum. In: Kaiser LR, Kron IL, Spray TL, editors. Mastery of cardiothoracic surgery. Philadelphia: Lippincott-Raven, 1998:867–79.
- Bando K, Turrentine MW, Ensing GJ, Sun K, Sharp TG, Sekine Y, et al. Surgical management of total anomalous pulmonary venous connection. Thirty-year trends. Circulation
1996;94(Suppl I):1112–6.
- Sinzobahamvya N, Arenz C, Brecher AM, Blaschczok HC, Urban AK. Early and long-term results for correction of TAPVD in neonates and infants. Eur J Cardio-thoracic Surg
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- Serraf A, Belli E, Roux D, Sousa-Uva M, Lacour-Gayet F, Planche C. Modified superior approach for repair of supracardiac and mixed total anomalous pulmonary venous drainage. Ann Thorac Surg
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ABSTRACT
INTRODUCTION
