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Pii: s1010-7940(02)00035-0European Journal of Cardio-thoracic Surgery 21 (2002) 711–715 Intermediate results with correction of tetralogy of Fallot with absent Viktor Hrasˇkaa,*, A. Ka´ntorova´a, P. Kunovsky´b, D. Haviarc aDepartment of Cardiovascular Surgery, Children’s University Hospital, Limbova 1, 833 40 Bratislava, Slovakia bDepartment of CICU, Children’s University Hospital, Limbova 1, 833 40 Bratislava, Slovakia cDepartment of Radiology, Children’s University Hospital, Limbova 1, 833 40 Bratislava, Slovakia Received 6 September 2001; received in revised form 5 December 2001; accepted 9 January 2002 Objective: The intermediate results achieved with a new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome are reported. Method: Apart from correction of tetralogy of Fallot, this approach includes translocation of the pulmonary arteryanterior to the aorta and away from the tracheobronchial tree. Since November 1998 this technique has been employed in three symptomaticnewborns and three infants with a diagnosis of tetralogy of Fallot with absent pulmonary valve. The median age at surgery was 37 days (range14–256 days). The median weight at operation was 3.4 kg (range 2.9–4 kg). All patients had severe respiratory problems and congestive heartfailure with cyanosis. Results: There was no early or late death during the follow-up (median 27 months). One patient required redo due tofailure to thrive as a consequence of right ventricle volume overload. Valved conduit was inserted to pulmonary position. Respiratorysymptoms disappeared or were significantly reduced in all patients. Postoperative computed tomographic scan showed no compression oftrachea and main bronchi, pulmonary artery was away from tracheobronchial tree in all patients. Patients are doing well with adequategrowth. Conclusions: The new technique described here has a potential to reduce or eliminate bronchial compression by pulmonary artery.
Translocation of pulmonary artery anterior to the aorta takes the dilated pulmonary artery away from the trachea and bronchial tree. Thisapproach was found to be technically feasible and can be useful especially in symptomatic newborns and infants. q 2002 Elsevier ScienceB.V. All rights reserved.
Keywords: Tetralogy of Fallot; Absent pulmonary valve syndrome; Respiratory distress Apart from correction of TOF, this approach includes trans-location of the PA anterior to the aorta and away from the The absent pulmonary valve syndrome occurs in 3–6% of patients with tetralogy of Fallot (TOF). This syndrome is The aim of this study is to report the intermediate results physiologically distinctive from other form of TOF because achieved with this approach with all consecutive patients of tracheobronchial compression resulting from massive undergoing primary repair in our center.
dilatation of the main pulmonary artery (PA) and its first-and second-order branches, and from the abnormal branch-ing of segmental arteries. Clinical presentation reflects the degree of respiratory distress secondary to airway obstruc-tion and infections and heart failure as a result of left to right Six patients with tetralogy of Fallot with absent pulmon- Since 1998 we have used a new technique that should ary valve (TOF/APV) were operated on between November eliminate or reduce compression of tracheobronchial tree.
1998 and December 2000 at the Children’s Hospital ofBratislava (Table 1). Three patients underwent surgery q Presented at the joint 15th Annual Meeting of the European Association during infancy and three underwent neonatal repair (age , for Cardio-thoracic Surgery and the 9th Annual Meeting of the European 30 days). Median age at surgery was 37 days (range 14–256 Society of Thoracic Surgeons, Lisbon, Portugal, September 16–19, 2001.
days). Median weight at operation was 3.4 kg (range 2.9–4 * Corresponding author. Tel.: 1421-2-54777-974; fax: 1421-2-54775- E-mail address: firstname.lastname@example.org (V. Hrasˇka).
All patients had severe respiratory problems and conges- 1010-7940/02/$ - see front matter q 2002 Elsevier Science B.V. All rights reserved.
PII: S 1 0 1 0 - 7 9 4 0 ( 0 2 ) 0 0 0 3 5 - 0 V. Hrasˇka et al. / European Journal of Cardio-thoracic Surgery 21 (2002) 711–715 Respiratory distress, heart failure, cyanosis Respiratory distress, heart failure, cyanosis Respiratory distress, heart failure, cyanosis a PA, pulmonary artery; FTT, failure to thrive.
tive heart failure with cyanosis. Surgery in neonates was performed on semi-urgent basis due to serious respiratorycompromise. Persistent bronchopulmonary infection requir- Data are presented as median and range.
ing prolonged and repeated hospital admission and failure tothrive were the main indication for surgery in infants. Nopatient required either assisted ventilation or extracorporeal membrane oxygenation before surgery.
Associated intracardiac anomalies other than TOF and Regular clinical check-up was performed at 3-month ostium secundum defect were present in three patients, intervals during the first postoperative years and at 6–12- including right aortic arch (n ¼ 2) and multiple aortopul- month intervals later on. All patients underwent two-dimen- monary collaterals (n ¼ 1) which required preoperative coil sional echocardiography (ECHO) and Doppler assessment of the intracardiac repair as well as computed tomography(CT) of the mediastinum three months postoperatively.
Follow-up data were obtained in all patients. The median follow-up for the whole group is 27 months (range 17–35 All patients underwent complete repair incorporating the standard intracardiac portion of TOF repair (resection ofright ventricular tract (RVOT) obstruction, patch closureof the ventricular septal defect (VSD)) and translocationof the pulmonary artery anterior to the aorta. The idea ofoperation is to bring the PA anterior to the aorta and awayfrom tracheobronchial tree.
This approach anticipates wide deliberation of the ascending aorta, aortic arch and brachiocephalic vessels.
The superior vena cava (SVC) is dissected free and theazygos vein is transected to improve mobility of SVC.
The left and right pulmonary arteries, including the firstpulmonary artery branches in the hilum of each lung, aredissected free and mobilized. After TOF repair a transverseaortotomy is done above the commissure and tubular ortriangular segment of the aorta is resected (Fig. 1). Thismaneuver brings the future ascending aorta down and tothe left. The pulmonary artery is transected above the annu-lus and brought anterior to the aorta. At this point end-to-end anastomosis of ascending aorta is performed. Finally, adirect connection between pulmonary artery and RVOT isaccomplished (Fig. 2). At this moment, anterior/posteriorPA plication or homograft insertion can be done, if neces-sary. Details of this approach were published elsewhere .
The RVOT was reconstructed with valved homograft in two newborns; a monocusp valve was utilized in one infant.
Combined anterior and posterior plication of pulmonary Fig. 1. Triangular segment of the aorta is resected. The pulmonary artery is artery was performed in three patients.
V. Hrasˇka et al. / European Journal of Cardio-thoracic Surgery 21 (2002) 711–715 Fig. 3. Postoperative CT scans 3 months after operation. There is nocompression of main bronchi; pulmonary artery is translocated anterior tothe aorta.
operative CT scan showed no compression of trachea andmain bronchi, pulmonary artery was away from tracheo-bronchial tree in all patients (Fig. 3). In the hilar portionof the lungs of patients without plication of pulmonary Fig. 2. The pulmonary artery is brought anterior to the aorta. The directconnection between pulmonary artery and right ventricle outflow tract, or arteries, there was an immediate contact between dilated homograft insertion is accomplished.
pulmonary artery and its branches and mainstem andsegmental bronchi (Fig. 4). These patients had slight dyste- lectasis, especially in the right upper lobe of the lung.
Neurological examination revealed only mild tone altera- tion (hypotonia) in one patient at 1 year of age; the rest ofpatients showed normal neurological development.
There was no early death. Hemodynamically, the post- The patients are doing well and are growing adequately.
operative course was generally uneventful; one newborn All patients are on medication (Digoxin and/or diuretics) but had delayed sternal closure and inotropic requirements no one is in need of bronchodilator therapy.
varied from 1 to 4 days. Transient right ventricular failurewith pleural effusion was noted in three patients.
The duration of mechanical ventilation varied from 2 to 13 days (median 7 days) and the intensive care unit (ICU) stayvaried from 9 to 25 days (median 12 days). Recovery from Despite improvements in surgical techniques and critical respiratory distress was delay in one newborn due to inhala-tion pneumonia requiring mechanical ventilation for 13 days.
There was no late death. Three months after neonatal repair one patient required reoperation for failure to thrive.
Valved conduit was inserted into the pulmonary position.
His postoperative course was uneventful. He was the onlyprimarily operated newborn in whom the valve homograftwas not utilized for reconstruction of RVOT. Anotherpatient had ventriculo-peritoneal shunt implanted due tohydrocephalus.
On Doppler echocardiography two patients with homo- graft had gradients of 30 and 17 mmHg, respectively. Smallresidual VSD was noted in one patient. There were no signsof SVC obstruction.
Fig. 4. In the hilar portion of the lungs there is an immediate contact At follow-up, respiratory symptoms completely disap- between dilated pulmonary artery and its branches and segmental bronchi peared (three patients) or were significantly reduced. Post- V. Hrasˇka et al. / European Journal of Cardio-thoracic Surgery 21 (2002) 711–715 care, controversy persists regarding the management of homograft with anterior and posterior plication of PA.
patients with TOF/APV. The mortality for symptomatic With the technical aspects of this procedure well accom- newborns and infants remains considerable secondary to plished, functional results are quite encouraging with zero airway obstruction by dilated PA [2,4–6].
mortality events in the youngest age group of patients. The Massively dilated right and left PA up to the hilum are small number of patients and the short period of follow-up amenable to surgical intervention [2–6]. Unfortunately abnormalities of arborization, with tufts of arteries encir-cling and compressing the intrapulmonary bronchi, cannot be addressed during the surgery . This can partiallyexplain the high rate of failure with the treatment of the I am grateful to Michal Sˇaga´t for the illustrations.
youngest, symptomatic group of patients.
A number of surgical techniques for reduction of bron- chial obstruction have been proposed with questionable results. The method of choice especially in symptomaticnewborns and infants is still controversial. All strategies  Rabinovitch M, Grady S, David I, Van Praagh R, Sauer U, Buhlmayer have focused on plication, reduction of the anterior or K, Castaneda AR, Reid L. Compression of intrapulmonary bronchi by posterior wall of the normally positioned PA with or without abnormally branching pulmonary arteries associated with absentpulmonary valves. Am J Cardiol 1982;50:804–813.
pulmonary valve replacement [4–6].
 Godart F, Houyel L, Lacour-Gayet F, Serraf A, Sousa-Uva M, Our approach applies a well-known technique (Lecompte Bruniaux J, Petit J, Piot JD, Binet JP, Conte S, Planche C. Absent maneuver)  that has the potential to reduce or eliminate pulmonary valve syndrome: Surgical treatment and consideration.
bronchial compression by PA. Translocation of PA anterior to the aorta displaces the dilated PA anterior, away from the  Hrasˇka V. A new approach to correction of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg 2000;69:1601–1603.
trachea and bronchial tree. There are several technical  Snir E, deLeval MR, Elliott MJ, Stark J. Current surgical technique to pitfalls to keep in mind during the performance of this repair Fallot’s tetralogy with absent pulmonary valve syndrome. Ann procedure. It is essential to gain adequate room between SVC and ascending aorta for the translocated right PA. In  Conte S, Serraf A, Godart F, Lacour-Gayet F, Petit J, Bruniaux J, addition to SVC mobilization, shortening of the ascending Planche C. Technique to repair tetralogy of Fallot with absent pulmon-ary valve. Ann Thorac Surg 1997;63:1489–1491.
aorta allows the aorta to ultimately reside posteriorly and to  Donnell BE, Raff GW, Gaynor JW, Ryhlik J, Godinez RI, DeCampli the left of its usual location. This maneuver calls for a thor- WM, Spray TL. Outcome after repair of tetralogy of Fallot with absent ough mobilization of the aortic arch and brachiocephalic pulmonary valve. Ann Thorac Surg 1999;67:1391–1396.
vessels. Shortening of the ascending aorta and mobilization  Lecompte Y, Zannini L, Hazan E, Jarreau MM, Bex JP, Tu TV, of PA beyond pericardial reflection avoids the potential Neveux JY. Anatomic correction of transposition of the great arteries.
J Thorac Cardiovasc Surg 1981;82:629–631.
compression of right coronary artery and SVC. Anotherrelevant detail is shortening of the left PA (which is alwaystoo long) by oblique transection of the PA trunk with connection to the RVOT. Finally, insertion of valve homo-graft with anterior and posterior plication of PA can Mr J. Monro (Southampton, UK): I think you showed you had to go back decrease wall tension and prevent later development of and insert a homograft in one patient. I must say, we routinely use ahomograft in all these neonates and young infants with this condition.
aneurysmal dilatation of PA . This is our current What I have done is to put a Hegar dilator down the pulmonary arteries approach for symptomatic newborns and infants, which and oversew it to narrow it to whatever diameter you want, taking great care was found to be technically feasible and useful. Our not to mobilize the back of the pulmonary artery, and then leave the stitch management strategy is consistent with the report of that you have used and at the end of the procedure, bring it up through the McDonnell and co-workers who recommended not only chest wall and just tie it. So this hitches the pulmonary artery forward, andcertainly that opens up the bronchi, which is the main problem with this anterior and posterior plication of PA but insertion of homo- graft, especially in newborns and infants .
Dr Hrasˇka: Of course, it is possible to perform the operation like this, There is no mutual consensus on the timing for surgery for babies presenting with TOF/APV. Obviously, sympto- Dr J. Amato (Chicago, IL, USA): I just wondered, how much of the aorta matic patients need to proceed directly to surgery. In addi- did you remove and did you have any problem with the LeCompte maneu-ver in coming forward, and if you did, in one of the earlier papers today I tion to this, we believe that early repair in asymptomatic heard that they used a piece of that aorta to serve as a junction or a bridge patients can eliminate the potentially harmful effect of between the pulmonary artery and the right ventricle? dilated PA on the tracheobronchial tree.
Dr Hrasˇka: Well, actually we haven’t had a problem to bring the In conclusion, from 1998 we started to use a new techni- pulmonary artery anterior because the operation was performed in very que that has the potential to eliminate or reduce bronchial small babies. So you usually don’t have this problem in very small babies.
In fact, one has to be very careful about the resection of the aorta because if compression by PA. Apart from correction of TOF, this you resect too much, you can be very close, especially to the origin of the approach includes translocation of the PA anterior to the left bronchus. It depends, of course, on the aortic arch anatomy. So one has aorta and away from the airways, and insertion of valve V. Hrasˇka et al. / European Journal of Cardio-thoracic Surgery 21 (2002) 711–715 Dr Amato: I guess what I mean is, do you do a circumferential resection and I think, for the timing of the operation, if you perform the operation or just an anterior, a piece of the anterior wall to pull it forward? pretty early you can avoid all this trouble like you mentioned.
Dr Hrasˇka: It is like a tubular, circumferential resection.
Dr Asfour: I refer to the same-age very young patients and bronchoscopy Dr B. Asfour (Mu¨nster, Germany): I have considered to use your tech- with these really thin fiberscopes which is possible.
nique because you have published it before; however to my understanding, Dr T. Tlaskal (Prague, Czech Republic): I would like to ask you, do you many of these children have bronchomalacia. So I’d rather not want to recommend to do this rather complex repair in all patients with tetralogy dissect the pulmonary artery away from the bronchus, and then by redu- and a dysplastic pulmonary valve or only in the symptomatic neonates and cing the size of the pulmonary arteries there is some tension on the connection and the adhesion of the pulmonary artery which then keeps The other question is, I think that in some patients in the follow-up some the bronchus open because it is so soft. Therefore we used bronchoscopy progression of pulmonary stenosis could be expected with some postopera- intraoperatively to monitor the bronchi after reducing the size of the tive dilatation of the hilar part of both pulmonary branches. Don’t you think pulmonary arteries open. How many of these children had bronchomalacia that this might be a problem in the follow-up? and did you need to use anything like stents or something to keep these Dr Hrasˇka: Well, based on my experience, we haven’t had any problem with postoperative pulmonary stenosis. As for your first question, I think Dr Hrasˇka: Well, we performed the operation in very small babies, like I this technique can be useful, especially for symptomatic patients. So there is mentioned, and actually we didn’t check the situation as far as the bronchus no need to do it for everybody, I guess. But for symptomatic patients it can by bronchoscopy. So basically I don’t think that even one patient had help because it makes sense to bring the pulmonary artery anterior and bronchomalacia in this group. But definitely they were very symptomatic, away from the tracheobronchial tree.
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