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Ann Thorac Surg 1997;64:1150-1153
© 1997 The Society of Thoracic Surgeons

Original Article: Cardiovascular

Surgical Repair of Patients With Tetralogy of Fallot and Unilateral Absence of Pulmonary Artery

Gen-Cheng Zhang, MD, Zeng-Wei Wang, MD, Ren-Fu Zhang, MD, Hun-Yu Zhu, MD, Ding-Hua Yi, MD

Institute of Cardiovascular Surgery, Department of Cardiovascular Surgery, North General Hospital of China, Shenyang, People's Republic of China

Accepted for publication April 24, 1997.


   Abstract
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 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 References
 
Background. Patients with tetralogy of Fallot and unilateral absence of pulmonary artery are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 29-year experience in the treatment of those patients.

Methods. Between May 1966 and February 1995, 2,511 patients underwent correction of tetralogy of Fallot in our department, 24 of those patients with unilateral absence of pulmonary artery (20 had absence of the left pulmonary artery, 4 had absence of the right pulmonary artery). Valved conduits were used in 9 patients, right ventricular patches were used in 4 patients, and transannular patches with a monocusp that was made of the patient's pericardium were used in 11 patients.

Results. There were two operative deaths; both were in patients with hypoplasia of the left ventricle. All survivors had good early and late results.

Conclusions. A right ventricular patch should be used in patients with tetralogy of Fallot and infundibular stenosis; a transannular patch with a monocusp should be used in patients with tetralogy of Fallot and stenosis of the left or right pulmonary artery's origin as well as the pulmonary trunk. A homograft valved conduit is suitable for patients with anomalous coronary artery or pulmonary atresia.


   Introduction
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Patients and Methods
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See also page 1153.

In clinical practice, patients with tetralogy of Fallot (TF) and unilateral absence of the pulmonary artery (PA) are uncommon. From May 1966 to February 1995, 2,511 patients underwent surgical repair of TF in our department. Twenty-four of them (0.956%, Table 1Go) also had unilateral absence of the PA, 20 of the left PA (LPA) and 4 of the right PA (RPA). None of them had palliative operation. The operative mortality was 8.3%. This article discusses the pathologic anatomy, operative indications, operative methods, and late results in this series of 24 patients.


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  		Table 1. . Clinical Data
 

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Clinical Data
The age of the 24 patients (Table 1Go) ranged from 4 to 21 years; 15 were men and 9 were women. All of them demonstrated cyanosis and clubbing of fingers and toes. Only one presented with a hypoxic attack. Electrocardiograms showed right axis deviation and right ventricular hypertrophy in all patients and 4 patients had complete right bundle-branch block. The chest roentgenogram showed the hearts of all patients were boot-shaped, the cardiothoracic ratio was 0.48 to 0.64, lung markings were decreased and the fields of both left and right lungs were not symmetrical, and the lung markings on the side of the absent pulmonary artery were of reticular structure. Of the patients with cardiac malformations (Table 1Go), 5 had right aortic arch, 1 had patent ductus arteriosus, 1 had the entire left anterior descending artery originating from the right coronary artery, which crossed anterior to the right ventricular infundibulum, and 2 had left ventricular hypoplasia. Laboratory studies showed the following: hemoglobin level was 132 to 229 g/L, hematocrit was 33% to 78%, urinary protein of 3 patients was 3+ to 4+. Diagnosis was confirmed by selective right and left ventriculography and pulmonary vein wedge injection before operation, which showed that 20 patients had complete absence of LPA and 4 patients had complete absence of RPA, 4 with normal pulmonary valve ring and pulmonary trunk, 17 with stenosis of the pulmonary valve and ring. All patients had well-developed or slightly dilated pulmonary artery on the healthy side: 19 had subcristal ventricular septal defect (VSD), 5 had subpulmonary VSD. In 20 patients with absence of LPA, 12 had bronchial collateral arteries, 8 had intercostal collateral arteries. In 4 patients with absence of RPA, the collateral arteries originated from the PDA in 1 and 3 had bronchial collateral arteries. Echocardiography showed left ventricular end-diastolic volume index (LVEDVI) in 3 of 24 patients was less than 30 mL/m2 (specifically, 28.5 mL/m2, 23.5 mL/m2, and 25 mL/m2); all the other patients' LVEDVI were more than 30 mL/m2. The pathologic anatomy of the side without the pulmonary artery was explored in the operation. The hilar portion of the LPA in 1 patient was only a stump of tissue with a diameter of 4 mm and length of 2.5 mm, only a swelling of 5 mm of tissue in another patient, and a cord of 4 mm in a third patient; in all 21 of the other patients the central portion of absence of the PA was absent. The PA trunk of those 24 patients was connected directly with the artery on the healthy side.

Operative Treatment
Operative repair was performed using standard hypothermic cardiopulmonary bypass. Myocardium was preserved by putting small pieces of ice on the heart and filling the coronary artery with oxygenated cold crystalloid solution or cold blood cardioplegic solution. The cardiopulmonary bypass was set up by (1) employing a median sternotomy; (2) situating the aortic cannula in the ascending aorta; (3) cannulating the superior and inferior vena cave through the right atrium; and (4) placing the decompression cannula in the left atrium through the base of the right superior pulmonary vein. Four patients who had only an infundibular stenosis and subcristal VSD were treated with resection of the infundibular stenosis and repair of the VSD, and enlargement of the right ventricular outflow with a polyethylene terephthalate (Dacron) patch. Ten patients with pulmonary valve, ring, trunk, and infundibular stenoses were treated with (1) incision of stenoses of the valve, ring, and pulmonary trunks as well as LPA or RPA; (2) resection of the hypertrophic septal band and parietal band; and (3) repair of the VSD with a Dacron patch. We incised the stenoses at the origin of the LPA or RPA and enlarged them with pericardium, and incised the pulmonary trunk stenosis and inserted the transannular monocusp patch. Note that the patch should not be too wide and the monocusp area should be big enough. The upper edge of the monocusp should be more than 2 to 3 mm higher than the patient's pulmonary valve so that the free edge of the monocusp will close with the edge of the patient's pulmonary valve. Before 1983, 8 patients were treated with placement of a homograft valved conduit (diameter, 1.8 to 2.0 cm) between the right ventricle and the PA, the distal end of which was connected in an end-to-side anastomosis with the pulmonary trunk and the proximal end of which was anastomosed to the incision in the right ventricle [1]. Of the 16 patients who were treated after 1984, only 1 received a homograft valved conduit because of the anomalous coronary artery, and the others all received a transannular patch with a monocusp. The aortic cross-clamp times ranged from 34 minutes, 6 seconds to 91 minutes, and cardiopulmonary bypass times ranged from 54 minutes, 27 seconds to 300 minutes.

At the end of the operation, all patients were evaluated in terms of the pressure of the left and right ventricles and the PA, which indicated that the difference between the systolic pressure of the right ventricle and that of the PA was less than 2.0 kPa for all but one (2.67 kPa). Fourteen had a ratio of systolic pressure of the right ventricle to that of the left ventricle less than 0.5, 8 had a ratio between 0.51 and 0.6, and 2 had a ratio more than 0.8.


   Results
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Two patients died within 2 hours after the operation because they both had left ventricular hypoplasia in which the preoperative LVEDVI was 23.5 mL/m2 and 25 mL/m2, respectively, and which resulted in disturbance of the general circulation after stopping cardiopulmonary bypass. The others recovered well after the operation. The routine echocardiography showed that there was no remaining VSD or pulmonary incompetence before they were discharged from the hospital. Electrocardiography indicated that there was no heart block.

The follow-up observation period for the patients ranged from 6 months to 15 years (mean, 7.25 years); that only 1 patient died 8 years after the operation without clear cause (Fig 1Go), and 21 could move freely without the symptom of cyanosis and administration of cardiotonic and diuretic drugs.



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  		Fig 1. . Late survival rate.

 

   Comment
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 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
References
 
Unilateral pulmonary arterial defect in TF is caused by backward displacement of the truncus of the conical artery [2]. Another reason for the pulmonary arterial atresia may be related to long-term hypoxia, erythrocytosis, elevated blood viscosity, decreased pulmonary blood flow, and pulmonary thrombosis [3]. Presbitero and associates [4] found in the operation that 1 infant patient had an obstruction of fresh thrombus in the PA and another patient had atresia at the origin of the LPA. Thus, they concluded that some of the so-called pulmonary arterial defect was caused by atresia of the pulmonary artery on one side. Our finding that there were remnants of atretic PA in 3 patients also supports this hypothesis. Most of the patients with TF combining with the pulmonary arterial defect have an absence of the LPA. In these 24 patients, 20 patients had an LPA defect, and only 4 patients had an RPA defect. Diagnosis of which PA is absent should be confirmed by selective right ventriculography in addition to the chest roentgenogram examination and aortography to prove that the PA on one side does not originate from the aorta abnormally. During the operation, more attention should be paid to investigate those remains of the defective PA on one side to find out whether the original portion of the PA is greatly narrowed, completely atretic, or completely absent [5].

As soon as the diagnosis is confirmed, the operation ought to be undertaken because of the poor condition and high natural mortality of patients with unilateral absence of the PA and TF. The indications for corrective operation were (1) a well-developed left ventricle and PA on the healthy side and peripheral pulmonary; (2) the diameter of the PA on one side that is 1.5 times that of the descending aorta at the diaphragmatic plane; and (3) LVEDVI more than 30 mL/m2. The monocusp patch is used to widen the right ventricular outflow in those patients with an abnormal pulmonary valve and stenosis of the pulmonary trunk and annulus requiring transannular enlargement or valvectomy [6], the early and late results of which are good. The homogaft valved conduits might be used in treating those with abnormal coronary artery transversely crossing the right ventricular outflow tract or pulmonary arterial atresia.

Only a few researchers have reported operative treatment of TF with unilateral absence of the PA, and the operative mortality in the past was 44% to 48% [3]. Eight cases were reported by Turinetto and colleagues [2], and only 1 survived. Williams and associates [7] believed that it could be treated with systemic–pulmonary arterial shunting. The main reason for high mortality in the early stage after the operation is that the widespread pulmonary thrombosis on one side causes the high resistance and pressure of the pulmonary vessels or pulmonary incompetence causes the overload and failure of the right ventricle. We used the autologus monocusp pulmonary valve, which provides a good solution for various forms of pulmonary regurgitation as others have shown [8–10]. Evidence from clinical and experimental studies confirmed that unilateral pulmonary vessels could endure the total cardiac output with normal or slightly elevated pulmonary pressure [11]. Pulmonary blood flow might return to normal by closing the VSD and properly removing the obstruction of the right ventricular outflow tract during the operation. After the operation, the decrease of blood viscosity and dissolution of the microthrombus may gradually decrease the resistance of the pulmonary blood vessels. Therefore, the crux of promoting the operative results is to completely remove the obstruction of the right ventricular outflow tract in the operation and to avoid heart failure at an early stage after the operation that is caused by pulmonary valvular incompetence. It is essential to enlarge the stenotic LPA or RPA and the pulmonary trunk. Mistrot and associates [3] believed that pulmonary valvular regurgitation could be avoided and the operative results promoted by using the valved extracardial conduit to replace the patch of the right ventricular outflow tract. Although for infants only an extracardial conduit with a small diameter can be selected, this will need to be replaced again several years later because it surely limits the cardiac output as patients grow. Because of immunologic and possibly hemodynamic mechanisms, there will be a significant decrease in the diameter and hence in the cross-sectional area and Z-value of homograft conduits in the pulmonary circulation with time, and the conduit's function will decrease and even cause the obstruction of the conduit at a late stage [12]. Of 24 patients, only 9 received an extracardial conduit because of the abnormal coronary artery and the others received the patch of the right ventricular outflow tract with the monocusp to widen the right ventricular outflow tract. Our practice suggests that the patch of the right ventricular outflow tract with the monocusp can avoid incompetence of the PA valve and promote a good late-operative result, and it also can avoid the complication of obstruction at the late stage that is common in those patients treated with the extracardial conduit.


   Footnotes
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 Footnotes
Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 References
 
Address reprint requests to Dr Zhang, Graduate School Dorm, the 4th Military Medical University, Xi'an, Shaanxi 710032, People's Republic of China.


   References
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  1. Wang Z-w, Fei C-j, Zhang R-f, et al. The application of the aortic valved homograft in treatment of complex heart disease. Med J Chin PLA 1983;8:18–21.
  2. Turinetto B, Coli G, Donati A. Absent right pulmonary artery complicating tetralogy of Fallot. J Cardiovasc Surg 1975;19:322–6.
  3. Mistrot JJ, Bernhard WF, Rosenthal A, Castañeda A. Tetralogy of Fallot with a single pulmonary artery: operative repair. Ann Thorac Surg 1977;23:249–53.[Abstract]
  4. Presbitero P, Bull C, Haworth SG, et al. Absence of occult pulmonary artery. Br Heart J 1984;52:178–85.[Abstract/Free Full Text]
  5. Wang Z-w, Wang W-x. The report of one patient with absent left pulmonary artery complicating tetralogy of Fallot successfully treated by using valved tract operation. Med J Chin PLA 1980;5:73–4.
  6. Mishaly D, Birk E, Elami A, Vidne B. Autologous monocusp pulmonary valve: preliminary results. Ann Thorac Surg 1996;61:1811–5.[Abstract/Free Full Text]
  7. Williams GD, Dungan WT, Campbell GS. Surgical treatment of tetralogy of Fallot with unilateral absence of a pulmonary artery. Ann Thorac Surg 1972;14:483–93.[Medline]
  8. Rowe SA, Zahka KG, Manolio TA, et al. Lung function and pulmonary regurgitation limit exercise capacity in postoperative tetralogy of Fallot. J Am Coll Cardiol 1991;17:461–6.[Abstract]
  9. Marx GR, Hicks RW, Allen HD, et al. Non-invasive assessment of hemodynamic responses to exercise in pulmonary regurgitation after operation to correct pulmonary outflow obstruction. Am J Cardiol 1988;61:595–601.[Medline]
  10. Zahka KG, Horneffer PT, Rowe SA, et al. Long-term valvular function after total repair of tetralogy of Fallot: relation to ventricular arrhythmias. Circulation 1988;78(Suppl 3):14–9.
  11. Werber J, Ramilo J, London R, et al. Unilateral absence of pulmonary artery. Chest 1983;84:729–32.[Abstract/Free Full Text]
  12. Ando M, Imai Y, Hoshino S, Ishihara K. Autologous reconstruction of pulmonary trunk at reoperation after extracardiac conduit repair. Ann Thorac Surg 1995;59:621–5.[Abstract/Free Full Text]

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