Ann Thorac Surg 1998;66:1394-1397
© 1998 The Society of Thoracic Surgeons
Original articles: cardiovascular
Mixed type of total anomalous pulmonary venous connection
Yutaka Imoto, MDa,
Hideaki Kado, MDa,
Toshihide Asou, MDa,
Yuichi Shiokawa, MDa,
Ryuji Tominaga, MDa,
Hisataka Yasui, MDa
a Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, and Division of Cardiovascular Surgery, Kyushu University, Fukuoka, Japan
Accepted for publication May 1, 1998.
Address reprints requests to Dr Imoto, Division of Cardiovascular Surgery, Research Institute of Angiocardiology, Kyushu University, Faculty of Medicine, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan
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Abstract
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Background. The mixed type of total anomalous pulmonary venous connection is a rare condition in which some diagnostic and surgical problems still remain to be solved.
Methods. In 9 patients a single pulmonary vein was connected to the systemic vein at a site different from the drainage site of the confluence of three other pulmonary veins. In 2 other patients, four pulmonary veins made a confluence which had two drainage sites. Correct diagnosis was made in all 7 patients who received cardiac catheterization but only in 5 of the 9 patients by color Doppler echocardiography. Total correction was performed in 3 patients and the single anomalous pulmonary vein was left uncorrected in 8 other patients.
Results. There were two in-hospital deaths. Seven patients with a single residual anomalous pulmonary vein have been in good condition without clinical symptoms of congestive heart failure or pulmonary hypertension.
Conclusions. Diagnosis of mixed type of total anomalous pulmonary venous correction by echocardiography is sometimes difficult. When a mixed type is suspected, cardiac catheterization is recommended if the condition of the patient permits it. A single anomalous pulmonary vein may be left uncorrected without serious complications, but close observation is needed to prevent congestive heart failure and pulmonary vascular obstructive disease.
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Introduction
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The occurrence of multiple drainage sites in total anomalous pulmonary venous connection (TAPVC) has important implications in preoperative diagnosis and surgical treatment. This report describes our clinical experiences in patients with this type of anomaly.
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Patients and methods
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Among the 121 cases of TAPVC operated on in Fukuoka Children’s Hospital from 1981 to 1996, 11 (9.1%) had a mixed-type form (7 boys and 4 girls). Operative age ranged from 11 days to 5 months (2.1 ± 1.6 months) and the body weight from 2.6 to 6.2 kg (4.0 ± 1.0 kg).
Although the pattern of the pulmonary venous drainage has a wide variety, 9 of 11 patients had confluence of three pulmonary veins (major drainage) and a single pulmonary vein connected to the systemic vein independently (minor drainage). In this report, we describe the anatomy of the pulmonary veins with major drainage in the first term and minor drainage in the second term; a description according to Darling and colleagues [1] is applied to each term: IIa + Ia, for example (Fig 1). In one patient, two pulmonary veins from the total right lung and the one from the left upper pulmonary lobe were confluent, although the latter was atretic, and then connected to the portal vein. The left lower pulmonary vein penetrated the diaphragm and connected to the portal vein as an independent descending vein (type III + III). Two other patients had double drainage sites to the innominate vein and to the coronary sinus from the confluence of four pulmonary veins (described as Ia & IIa in this report), which was reported by Arciprete and associates as a "double connection" [2].
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Fig 1. Anatomy of the pulmonary veins in 11 patients. In patients with major drainage and minor drainage, the major drainage site is described in the first term and the minor drainage in the second term, and these are combined with a plus sign. In patients with a double connection, two terms are combined with an ampersand (see text for details). Numbers of patients are shown in parentheses.
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The operation was performed under cardiopulmonary bypass with moderate hypothermia and cardiac arrest using aortic cross-clamping, topical cooling, and intermittent cold crystalloid cardioplegia. Total correction was performed in 3 patients: 2 with a double connection and 1 with type IIb + Ib. In one of the double connection patients the common pulmonary vein was anastomosed to the posterior aspect of the left atrium, and the ascending vein and the communication to the coronary sinus, which was rather small, were ligated. In the other patient with a double connection, cut back of the coronary sinus and intraatrial patching with autologous pericardium were performed in addition to ligation of the ascending vein. In the patient with type IIb + Ib, all pulmonary veins were diverted into the left atrium through the enlarged atrial septal defect with the autologous pericardial patch. The other 8 patients underwent partial correction with the major drainage diverted to the left atrium and the minor drainage left uncorrected. Locations of the residual anomalous pulmonary veins were left upper pulmonary vein to the left innominate vein in 4, left upper pulmonary vein to the superior vena cava in 1, right upper pulmonary vein to the superior vena cava in 2, and the left lower pulmonary vein to the portal vein in 1. In 1 patient with the right upper pulmonary vein left uncorrected, the superior and the middle lobes of the right lung were involved. In the patient with type III + III, preoperative cardiac catheterization was not performed, as his condition had been critical, and the minor drainage vein could not be detected either by preoperative echocardiography or intraoperative examination. A complete anatomic diagnosis was obtained at autopsy. Therefore, only the major descending vein was anastomosed to the left atrium.
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Results
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Difficulty in preoperative diagnosis is one of the clinical problems in the mixed type of TAPVC. Preoperative catheterization and angiography were performed in 7 patients and correct diagnoses had been obtained in all of these patients. In contrast, complete diagnosis was made in only 5 of 11 patients by preoperative echocardiography. In the rest of the patients minor drainage veins were overlooked: drainage to the innominate vein in 2 (Ib + Ia and III + Ia; these patients were examined without color Doppler echocardiography), to the superior vena cava in 3 (IIa + Ib, IIb + Ib, and IIb + Ib), and to the portal vein in 1 (III + III). Even if the 2 patients examined without color Doppler are excluded, sensitivity of echocardiography was 6 of 9 (66.7%).
There were two in-hospital deaths. The patient with type III + III died of a pulmonary venous obstruction which had been left uncorrected, and another patient with a double connection who underwent total correction died of intractable supraventricular tachycardia which had existed before operation. Other patients survived the operation and were followed up for 2 to 11 years; no patient has shown postoperative pulmonary venous obstruction. Postoperative hemodynamics in patients who underwent partial correction of the anomalous pulmonary vein drainage is a matter of great concern. In 7 such patients except for the one with type III + III, pulmonary to systemic flow ratio (Qp/Qs) was 1.02 to 1.82 (1.28 ± 0.24) in the early postoperative catheterization. The largest Qp/Qs was obtained in the patient with residual anomalous drainage from the two lobes of the right lung. This patient showed no clinical symptoms of congestive heart failure in the long-term follow-up, but mild cardiomegaly (cardiothoracic ratio = 57.9%) and significant pulmonary high flow (Qp/Qs = 2.0) at the catheterization 37 months after the operation led us to the conclusion that correction of the residual anomalous drainage is preferable. The anomalous pulmonary vein to the superior vena cava was diverted to the left atrium through a newly created atrial septal defect using a right atrial flap, and the superior vena cava was divided and reimplantation on the right atrial appendage was performed to restore normal systemic venous drainage [3]. The operation was performed without complication. In this patient and 2 others who received long-term catheterization, Qp/Qs showed no significant change (Fig 2). The pulmonary to systemic pressure ratio in the 7 patients with a residual anomalous pulmonary vein ranged from 0.34 to 0.67 in the early postoperative catheterization. The long-term catheterization in 3 patients revealed no increase in the pulmonary to systemic pressure ratio (Fig 3). The cardiothoracic ratio by chest roentgenogram in the long-term period ranged from 47% to 59% (55% ± 5%) in patients with a single anomalous pulmonary vein uncorrected, which was not widely different from the cardiothoracic ratios in the patients who underwent complete repair (47% and 53%). No patient showed clinical symptoms of congestive heart failure, and body weight gain was also satisfactory. No patient had a body weight smaller than -1 standard deviation from the normal value in the long term.
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Fig 2. Pulmonary-to-systemic flow ratios (Qp/Qs) in 7 patients who survived the operation with a single anomalous pulmonary vein left uncorrected. (*Patient with a pulmonary vein from the upper and middle lobes of the right lung left uncorrected; postop = postoperative.)
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Fig 3. Pulmonary to systemic pressure ratios (Pp/Ps) in 7 patients who survived the operation with a single anomalous pulmonary vein left uncorrected. (*Patient with a pulmonary vein from the upper and middle lobes of the right lung left uncorrected; postop = postoperative.)
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Comment
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Mixed type of TAPVC is a rare cardiac anomaly, the frequency of which is 5% to 10% of the patients with TAPVC in the literature [4, 5]. There was a wide variety of patterns of pulmonary venous drainage [4, 6].
Echocardiography is currently the only preoperative diagnostic study performed in most cases of TAPVC; however, surgeons must always keep in mind the possibility of a mixed type of TAPVC. In our series, the sensitivity of diagnosis with echocardiography alone was 67%, whereas the sensitivity by catheterization was 100%. Intraoperative confirmation of the four pulmonary veins is very important. When a mixed type is suspected by echocardiography, cardiac catheterization and angiography are preferable if the condition of the patient is stable.
The operative procedure for this type of TAPVC is also a matter of concern. Delius and colleagues divided patients into two groups in terms of surgical management: "3 + 1," in which three pulmonary veins made confluence with the remaining vein draining at another site, and "2 + 2," in which the pulmonary veins of each side connected with systemic vein at separate sites [4]. The patients in the latter group lacked a venous confluence behind the heart, which made anastomosis of pulmonary veins and the left atrium difficult. Moreover, primary correction of all anomalous pulmonary veins seemed essential. There was no such patient in our series. In the former group, anastomosis of the confluence of the three pulmonary veins was easily performed, but correction of a single separate pulmonary vein might be controversial. No doubt total correction of all anomalous pulmonary veins is the ideal operation with regard to postoperative hemodynamics, but postoperative pulmonary vein stenosis is anticipated because of the small-sized anastomosis for the single separate pulmonary vein. One solution to this problem is to leave the single anomalous drainage uncorrected; a good prognosis was reported using this strategy [7]. In our experience, 7 such patients excluding the pulmonary venous obstruction case with type III + III tolerated the operation without operative or late death. These patients showed no clinical signs of congestive heart failure or pulmonary hypertension in the long-term follow-up, and growth of the patients was within normal limits. As postoperative pulmonary vein stenosis is less frequent with a larger pulmonary vein size [8], repair of the residual anomalous drainage in the second operation may be performed safely if needed. One question regarding leaving a single anomalous pulmonary vein uncorrected is how much the postoperative Qp/Qs value would be. In the literature, preoperative Qp/Qs values in patients with a partial anomalous pulmonary venous connection are around 1.5 [9, 10]. Therefore, patients with a single anomalous pulmonary vein uncorrected are expected to tolerate the operation. However, there have been some reports that pulmonary vascular obstructive disease developed in patients with a single anomalous pulmonary vein [9, 10]. Mechanisms responsible for the development of pulmonary vascular obstructive disease are unknown, but some explanations have been discussed: (1) possible pressure-sensitive receptors at the anomalous vein–vena cava junction; (2) an axon reflex triggered by right atrial distention; and (3) the results of increased flow as seen in patients with an atrial septal defect [10]. Therefore, close observation is needed to follow up these patients.
This strategy, leaving a single pulmonary vein uncorrected, is feasible only when it is unobstructed. If it is obstructed, anastomosis to the left atrium should be made. When the single pulmonary vein is obstructed and anastomosis is difficult, lobectomy may be another solution [11]. In 1 patient (type III + III) in our series, an obstructed minor drainage from the left lower pulmonary lobe to the portal vein was unintentionally left uncorrected, which resulted in in-hospital death. Long-term survival in this patient might have been attained if concomitant left lower lobectomy had been performed.
In summary, mixed type of TAPVC is a rare cardiac anomaly and has a wide variety in pulmonary venous anatomy. There seems to be a limitation in diagnosis by echocardiography only; cardiac catheterization, if the condition of the patient permits, is recommended when mixed-type TAPVC is suspected. Careful intraoperative examination is very important in all patients. A single anomalous pulmonary vein may be left uncorrected without serious complications, but close observation is needed to guard against congestive heart failure and pulmonary vascular obstructive disease.
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References
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- Darling R.C., Rothney W.B., Craig J.M. Total pulmonary drainage into the right side of the heart. Lab Invest 1957;6:44-64.[Medline]
- Arciprete P., McKay R., Watson G.H., Hamilton D.I., Wilkinson J.L., Arnold R.M. Double connections in total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1986;92:146-152.[Abstract]
- Gustafson R.A., Warden H.E., Murray G.F. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg 1995;60:S614-S617.
- Delius R.E., de Leval M.R., Elliott M.J., Stark J. Mixed total anomalous pulmonary venous drainage: still a surgical challenge. J Thorac Cardiovasc Surg 1996;112:1581-1588.[Abstract/Free Full Text]
- Delisle G., Ando M., Calder A.L., et al. Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J 1976;91:99-122.[Medline]
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- Jenkins K.J., Sanders S.P., Coleman L., Mayer J.E., Colan S.D. Pulmonary vein size and outcome in infants with totally anomalous pulmonary venous connection [Abstract]. Circulation 1991;84(Suppl 2):351.
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Abstract
Introduction
