Interact CardioVasc Thorac Surg 2008;7:1180-1182. doi:10.1510/icvts.2007.174367
© 2008 European Association of Cardio-Thoracic Surgery
Total cavopulmonary connection for functionally single ventricle with pulmonary atresia and abnormal arborization of pulmonary arteries – exclusion of overwhelmed area by collateral arteries from Fontan circulation
Mitsugi Nagashimaa,*,
Narutoshi Hibinoa,
Eiichi Yamamotob and
Takashi Higakic
a Department of Cardiovascular Surgery, Ehime Prefectural Central Hospital, 83 Kasuga-cho, Matsuyama-city, Ehime, 790-0024 Japan
b Department of Pediatrics, Ehime Prefectural Central Hospital, Ehime, Japan
c Department of Pediatrics, Ehime University of Medicine, Ehime, Japan
Received 15 January 2008;
received in revised form 9 June 2008;
accepted 28 July 2008
Corresponding author. Tel.: +81-89-947-1111 ext. 2250; fax: +81-89-943-4136.
E-mail address: mitsugi{at}aqua.plala.or.jp (M. Nagashima).
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Abstract
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Functionally single ventricle with major aortopulmonary collateral arteries and pulmonary atresia is very rare. Surgical indication for this combination of abnormalities is still unclear and Fontan procedure is still a crucial challenge for the patient who has this anomaly. We report a case with asplenia, functionally single ventricle, pulmonary artesian and pulmonary abnormal arborization who successfully underwent staged Fontan operation. In this case, the left upper lobe area, where the flow from a Glenn shunt was overwhelmed by the collateral arterial blood flow, was excluded from the Fontan circulation by the ligation of the left upper pulmonary arterial branch at its most proximal side at the time of fenestrated extracardiac total cavopulmonary connection.
Key Words: Pulmonary abnormal arborization; Single ventricle; Staged Fontan operation
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1. Introduction
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Major aortopulmonary collateral arteries (MAPCA) and pulmonary atresia associated with functionally single ventricle is remarkably uncommon. In addition, surgical treatment for these patients is still extremely challenging and surgical indication remains unknown.
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2. Case report
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A boy was born at 35 weeks' gestation with a weight of 2.5 kg. He was diagnosed by echocardiogram as asplenia, common atrioventricular valve, functionally single ventricle and pulmonary atresia with MAPCA. A catheterization at four months of age demonstrated that pulmonary circulation was supplied by two MAPCAs (Fig. 1). One distributed to the right upper lobe (MAPCA 1) and the other (MAPCA 2) connected to the central pulmonary artery. The MAPCA 1 communicated with the right upper branch of the pulmonary artery (PA) in the right upper lobe. The right upper PA was also connected without any stenosis with the central PA. Pulmonary to systemic blood flow ratio was 1:5. Mean pulmonary arterial pressure was 10, 11 and 12 mmHg at the right upper, the right lower and the left lobe, respectively. At five months of age, he underwent bidirectional Glenn shunt with ligation at the base of both the MAPCAs. The postoperative pulmonary arterial pressure was 10 mmHg and arterial oxygen saturation was 85% under 1 l/min oxygen supply through a nasal cannula. Five months after bidirectional Glenn procedure, the lung perfusion scan revealed complete perfusion defect in the left lung. Cardiac catheterization at one year of age demonstrated that several collateral arteries arising from the left internal thoracic artery, the left thyrocervical trunk and the subscapular artery, developed into the left lung. The blood flow from their collateral arteries in the left lung overcame the flow from the Glenn shunt (Fig. 2a). After these collateral arteries were successfully occluded using coil embolization, the pulmonary flow from the Glenn shunt in the left lung increased (Fig. 2b). The subsequent cardiac catheterization at one year and six months of age showed that numbers of meticulous collateral arteries newly developed in the left upper lung. Consequently, the left upper PA was not imaged by the contrast injection at the superior vena cava. It was determined that coil occlusion of these numbers of small collaterals was infeasible. A mean right pulmonary arterial pressure was 11 mmHg although a mean pulmonary vein wedge pressure in the left upper lung was 15 mmHg. At one year and nine months of age, fenestrated total cavopulmonary connection (TCPC) was performed with extracardiac conduit (18 mm PTFE tube graft). Simultaneously, the left upper pulmonary arterial branch was ligated at its most proximal side to exclude the left upper lung lobe from the Fontan circulation. Postoperative hemodynamics were stable with mean superior vena cava pressure of 14 mmHg and the arterial oxygen saturation was 94%. Six months after TCPC, cardiac catheterization disclosed that a mean pulmonary arterial pressure was 12 mmHg and arterial oxygen saturation was 97% under room air inspiration (Fig. 2, left lower (c): superior vena cava angiogram, right lower (d): inferior vena cava angiogram). A newly evolving small venous–venous collateral vessel arising from the innominate vein and draining into the left atrium was successfully occluded using another coil embolization. Five years after TCPC operation, the patient entered an elementary school and was doing well.
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Fig. 1. (a) Descending aortogram before surgery. (b) Ascending aortogram before surgery. Major aortopulmonary collateral artery (MAPCA 1) originated from the descending aorta and perfused in the right upper lobe. MAPCA 2 also originated from the descending aorta and connected with the central pulmonary artery.
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Fig. 2. (a) Angiogram of superior vena cava after bidirectional Glenn. Contrast in the left pulmonary artery was washed out (black arrow) from the collateral flow through the left upper pulmonary branch. (b) The left pulmonary artery re-appeared after coil embolization for the collateral arteries. (c) Angiogram of the superior vena cava and (d) the inferior vena cava after total cavopulmonary connection. The left upper lobe was excluded from Fontan circulation (black arrow).
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3. Discussion
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Abnormal arborization of PA is very rare in patients with functionally single ventricle. Uemura and his colleagues reported that it occurs in only 2% of patients with right isomerism and in no patients with left isomerism [1]. Only a few cases have been previously reported with successful Fontan repair for the patients with functionally single ventricle and abnormal arborization of PA [2, 3]. Reinhartz and colleagues reported 14 patients of functional single ventricles and unifocalization procedures of MAPCAs [3]. In our case, at the time of bidirectional Glenn procedure, it was determined that unifocalization was not required for the reason of the anatomical condition of pulmonary arteries. Bidirectional Glenn concomitant with unifocalization of MAPCAs is the ideal procedure for this anomaly. Nevertheless, this is a very complex procedure and has been associated with high operative mortality. Three patients died early after bidirectional Glenn with unifocalization and four patients died later in the former report from Reinhartz et al. [3]. Fontan operation was still a crucial challenge for these patients and surgical indications for this rare combination of anomalies were uncertain. In patients with ventricular septal defect (VSD) and pulmonary atresia with abnormal arborization, morphology of the PA and the number of the functioning pulmonary vascular segments were influenced by surgical results [4, 5]. Griselli reported that 15 pulmonary segments (i.e. equivalent to 1 lungs) were safe for complete repair for VSD and pulmonary atresia with abnormal pulmonary arborization. In Fontan repair, a similar or stricter condition should be required to obtain good surgical results [5]. In our case, the central PA was present although it was of a slightly small size (Nakata index was 117 mm2/m2). Fifteen or sixteen pulmonary segments connected with the central PA and pulmonary tree developed well. Mean pulmonary arterial pressure was 11 mmHg although left upper pulmonary venous capillary wedge pressure was 15 mmHg. Also, calculated pulmonary vascular resistance was 1.6 Wood units after bidirectional Glenn shunt. As a whole, our case had a good morphology of the peripheral pulmonary artery and low pulmonary vascular resistance. The only negative issue was that the remaining three or four pulmonary segments, which were equivalent in area with the left upper lobe, did not function as the Fontan circulation due to the overwhelmed flow by meticulous small collateral arteries despite catheter coil occlusion for the large collateral arteries. From these preoperative data, it was determined that completion of fenestrated Fontan repair was possible. In addition, we considered that it brought better hemodynamics to exclude the left upper lobe area from Fontan circulation rather than to include it. If the left upper branched PA was included in the Fontan circulation, the high pressure flow from collateral arteries would grow in predominance against the flow from superior and/or inferior vena cava and probably impair the Fontan circulation. The ligation of the PA, which leads to reducing the capacity of the pulmonary vascular bed should be still controversal. A long-term follow-up is required for this patient.
In conclusion, staged Fontan operation is a considerable and useful strategy for patients with functionally single ventricle and pulmonary abnormal arborization, if the patients have an appropriate morphology of the pulmonary arteries and good condition of pulmonary segments, although it is still a challenge. It may be a surgical option to exclude a small area from Fontan circulation where high pulmonary vascular resistance is anticipated due to the unconquerable collateral arteries.
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References
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Abstract
1. Introduction