Ann Thorac Surg 1996;61:210-213
© 1996 The Society of Thoracic Surgeons
Case Report
Staged Repair of Tetralogy of Fallot and Pulmonary Atresia Without Central Pulmonary Arteries
Yoshiya Toyoda, MD,
Masahiro Yamaguchi, MD,
Hidetaka Ohashi, MD,
Masanao Imai, MD,
Yoshihiro Oshima, MD,
Naoki Yoshimura, MD,
Teruo Yamashita, MD
Department of Cardiothoracic Surgery, Kobe Children's Hospital, Kobe, Japan
Accepted for publication July 12, 1995.
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Abstract
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A 15-year-old boy with tetralogy of Fallot and pulmonary atresia without central pulmonary arteries who was successfully treated using a staged approach is presented. The first stage consisted of the creation of central pulmonary arteries. In the second stage, the continuity between the right ventricle and the pulmonary arteries was established with closure of the ventricular septal defect. Some patients previously considered to be unsuitable candidates for correction may be successfully repaired using this approach.
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Introduction
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There have been recent improvements in the treatment of tetralogy of Fallot with pulmonary atresia [1]. The outcome after surgical repair is generally determined by the anomalies of the pulmonary arterial circulation. In this report, a patient with tetralogy of Fallot and pulmonary atresia without central pulmonary arteries who underwent successful surgical treatment is presented.
The patient was born at 41 weeks of gestation in 1975, weighing 4,825 g. A cardiac murmur associated with cyanosis was noticed at 1 month of age. Angiography performed at 10 months of age demonstrated tetralogy of Fallot and pulmonary atresia. Neither a pulmonary trunk nor central pulmonary arteries were visualized. The pulmonary circulation was dependent on two major aortopulmonary collateral arteries (MAPCAs), one to the right lung from the aortic arch and the other to the left lung from the descending aorta. The patient was deemed unsuitable for repair, and was maintained on digoxin. He suffered from mild exercise intolerance and cyanosis.
He was admitted for a second cardiac evaluation at the age of 14 years. At that time he weighed 34 kg and was 154 cm tall. Physical examination revealed mild cyanosis, clubbing of the fingers, and a grade II/VI continuous murmur auscultated on the back. Electrocardiography demonstrated left ventricular hypertrophy. Chest roentgenography showed slight hypervascularity in the left lung, slight hypovascularity in the right lung, and moderate cardiomegaly. Cardiac catheterization revealed pulmonary hypertension on the left. Selective MAPCA angiography showed that the right collateral had a narrowing between the aorta and the hilar pulmonary artery, and that the left collateral had no evidence of stenosis and supported a high volume flow. There were no arborization anomalies (Fig 1
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Fig 1. . (A) Aortogram showing a right major aortopulmonary collateral artery running to the right lung from the aortic arch. The artery is stenotic (arrow). (B) Left selective major aortopulmonary collateral artery cineangiogram showing no stenosis in the artery running to the left lung from the descending aorta (arrow).
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The first stage of the operation was performed in two parts. First, a right central pulmonary artery was created using a 15-mm-diameter, 5-cm-long equine pericardial conduit (Xenomedica AG, Switzerland). The operation was performed through a thoracotomy in the right fourth intercostal space. The conduit was anastomosed to the hilar pulmonary artery. Then, a modified Blalock-Taussig shunt consisting of a 6-mm Golaski tube was placed about 5 mm from the proximal end of the conduit. The right MAPCA was ligated. The proximal end of the conduit was directed forward and anchored just behind the superior vena cava. Nine days later, a left central pulmonary artery was created in a similar fashion. Because the hilar pulmonary artery connected to the left MAPCA was in a caudal position that could not be easily accessed, the xenograft was anastomosed to the left upper pulmonary artery, and a modified Blalock-Taussig shunt consisting of a 6-mm Golaski tube was placed in the middle of the conduit. The proximal end was placed into the pericardial cavity.
The patient had an uneventful postoperative recovery. Histologic examination of the left lung showed mild pulmonary hypertension (Heath-Edwards grade I). He was anticoagulated with warfarin (0.05 mg • kg-1 • day-1). Cardiac catheterization performed 7 months after the first stage of the operation revealed a pulmonary venous wedge pressure of 23 mm Hg in the right lung and 28 mm Hg in the left lung. Angiography demonstrated good blood flow to both lungs and no stenosis of the conduits (Fig 2). The pulmonary artery index [2] was 211 mm2/m2, and the right ventricular end-diastolic volume index was 61 mL/m2. We decided that the patient was suited for definitive repair at this point.
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Fig 2. . Selective pulmonary arteriography through the Golaski grafts. (A) Note that the right equine pericardial conduit is connected to the right pulmonary artery at the hilum (arrow). (B) Note that the left conduit (thin arrow) is connected to the left upper pulmonary artery (thick arrow), which was placed in the pericardial cavity.
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The second stage of the repair was performed through a median sternotomy 7 months after the first stage. No pulmonary trunk or central pulmonary arteries were identified. Before the patient was placed on cardiopulmonary bypass, the anastomosis between the newly created left pulmonary artery placed in the pericardial cavity and the bridging conduit (diameter, 15 mm) was created. This was performed after placing a vascular clamp proximal to the modified Blalock-Taussig shunt. There was no thrombus inside the conduit. A right ventricular-to-pulmonary artery conduit (diameter, 24 mm) was anastomosed to the bridging conduit. This was fashioned using a rolled composite ventricular outflow patch and an equine pericardial patch. The proximal portion of the newly created right pulmonary artery was in a position that made its dissection risky. Thus it was isolated after the establishment of deep hypothermic cardiopulmonary bypass and ligation of both modified Blalock-Taussig shunts. The right pulmonary artery was brought anteriorly over the right main bronchus and anastomosed to the bridging conduit behind the aorta. The aorta was then cross-clamped, and the heart was cooled topically and protected with blood cardioplegia. The hypertrophic infundibular muscle bundles were then resected through a transverse ventriculotomy in the outflow tract, and a 30-mm ventricular septal conus defect was closed with a Dacron patch. Anastomosis between the proximal end of the right ventricular-to-pulmonary artery conduit and the right ventricle was then performed. The cardiopulmonary bypass time was 250 minutes. After an easy wean from bypass, the right ventricular pressure was 61/7 mm Hg (mean, 30 mm Hg), and the aortic pressure was 78/45 mm Hg (mean, 59 mm Hg). The peak right ventricular-to-systemic pressure ratio was 0.85.
Postoperatively, the patient did well and was extubated on the fourth postoperative day. On the 14th day, cardiac tamponade developed, which was successfully drained. Thereafter, his recovery was uneventful. Cardiac catheterization 1 month after operation revealed satisfactory parameters with a right-to-left ventricular pressure ratio of 0.47. Angiography demonstrated no interventricular shunt and good flow to both lungs (Fig 3). The patient was well at the most recent follow-up examination (2 years postoperatively), and continues to take warfarin (0.05 mg/kg) and aspirin (2 mg/kg).
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Fig 3. . Postoperative pulmonary arteriography showing the new central pulmonary artery created with equine pericardial conduit, and good flow from the right ventricle to both lungs.
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Comment
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The techniques for the surgical repair of tetralogy of Fallot and pulmonary atresia are continuing to evolve. Patients with coexisting anomalies of the pulmonary arterial circulation, such as hypoplastic or absent central pulmonary arteries or arborization anomalies, present a challenging management problem. Not long ago, these patients were deemed unsuitable for repair [1]. However, the concept of unifocalization, advocated by Haworth and associates [3], has allowed for complete repair in some of these patients [4–8]. The pulmonary arteries supplied by MAPCAs give rise to the regional imbalance of pulmonary arterial growth and pulmonary vascular resistance. For this reason, it is recommended that the first stage of the repair be performed at an early age [8]. In our patient, the first stage was performed when the patient was 15 years 9 months old, which is relatively late. Despite this, the complete repair was successfully performed with sufficiently large conduits. The results were satisfactory, although the left lung was slightly hypertensive (Heath-Edwards grade I). Although the importance of early first-stage repair should be stressed, we believe that stable infants without significant desaturation or congestive heart failure may be safely observed, especially those who will require conduit repair because of the technical difficulties and the need for reoperations. Some older patients who were previously regarded as unsuitable for repair may successfully undergo correction today.
The absence of the central pulmonary arteries is no longer a contraindication to repair of tetralogy of Fallot and pulmonary atresia. We did, however, encounter one technical problem. The proximal portion of the left conduit can be easily placed into the pericardial cavity because there is no pulmonary trunk on that side, but this is not true on the right. We opted to place the right conduit behind the superior vena cava, which required a difficult dissection and anastomosis to the bridging conduit. Alternatives would have been to use a curved conduit to pass over the right main bronchus behind the superior vena cava, or to place the conduit anterior to the superior vena cava. The former is a more physiologic course but requires a difficult dissection. The latter is technically, but not physiologically, easier and could potentially compress the superior vena cava as the patient grows. Further studies will be necessary to elucidate the solution of this problem.
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Footnotes
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Address reprint requests to Dr Toyoda, Department of Cardiothoracic Surgery, Kobe Children's Hospital, 1-1-1 Takakura-dai, Suma-ku, Kobe, Japan 654.
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References
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- Kirklin JW, Barratt-Boyes BG, ed. Cardiac surgery, second ed. New York: Churchill Livingstone, 1993:942--73.
- Nakata S, Imai Y, Takanashi Y, et al. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610–9.[Abstract]
- Haworth SG, Rees PG, Taylor JFN, Macartney FJ, de Leval M, Stark J. Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: effect of systemic pulmonary anastomosis. Br Heart J 1981;45:133–41.[Abstract/Free Full Text]
- Puga FJ, Leoni FE, Julsrud PR, Mair DD. Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries. J Thorac Cardiovasc Surg 1989;98:1018–29.[Abstract]
- Kirklin JW, Blackstone EH, Shimazaki Y, et al. Survival, functional status, and reoperations after repair of tetralogy of Fallot with pulmonary atresia. J Thorac Cardiovasc Surg 1988;96:102–16.[Abstract]
- Iyer KS, Mee RBB. Staged repair of pulmonary atresia with ventricular septal defect and major systemic to pulmonary artery collaterals. Ann Thorac Surg 1991;51:65–72.[Abstract/Free Full Text]
- Barbero-Marcial M, Rizzo A, Lopes AAB, Bittencourt D, Junior JOA, Jatene AD. Correction of pulmonary atresia with ventricular septal defect in the absence of the pulmonary trunk and the central pulmonary arteries (so-called truncus type IV). J Thorac Cardiovasc Surg 1987;94:911–8.[Abstract]
- Sawatari K, Imai Y, Kurosawa H, Isomatsu Y, Momma K. Staged operation for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg 1989;98:738–50.[Abstract]
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Abstract
Introduction
