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a Department of Cardiovascular Surgery, German Heart Center Munich at the Technical University Munich, Germany
b Department of Paediatric Cardiology and Congenital Heart Disease, German Heart Center Munich at the Technical University Munich, Germany
Received 7 November 2007; received in revised form 28 March 2008; accepted 29 April 2008.
* Corresponding author. Address: Clinic of Cardiovascular Surgery, German Heart Center Munich at the Technical University Munich, Lazarettstrasse 36, 80636 Munich, Germany. Tel.: +49 89 12184111; fax: +49 89 12184113. (Email: schreiber{at}dhm.mhn.de).
| Abstract |
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Key Words: Congenital heart disease Partial cavopulmonary connection Bidirectional cavopulmonary connection Total cavopulmonary connection
| 1. Introduction |
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Our described results reflect a potentially changing overall treatment pattern. Patients staging can be accomplished earlier in life, with a BCPC alone proving to be perfectly sufficient until conversion to a TCPC within the first 2–4 years of life. Therefore, a meaningful comparison of previously published reports must take the patients age at each stage and institutional characteristics into account. This manuscript focuses on the assessment of pulmonary artery diameters from BCPC up to TCPC and immediate clinical results after TCPC.
| 2. Patients and methods |
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The origin of pulmonary artery blood flow prior to BCPC varied and is summarized in Table 1 . This study has been approved by the ethics committee of the Technical University Munich (1740/07). The ethics committee waived the need for individual patient consent for this study.
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| 3. Results |
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Pulmonary artery sizes at time of BCPC and just before completion to TCPC are summarized in Figs. 1–3. Fig. 1 depicts the mean increase of the right pulmonary artery between the stages, Fig. 2 the increase of the left pulmonary artery, and Fig. 3 focuses on diameter differences in unilateral and bilateral BCPC. The z-scores for the right pulmonary artery were –0.25 ± 1.7, and –1.01 ± 1.5 for the left pulmonary artery.
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There was no postoperative mortality after BCPC, one patient died after TCPC (1.2%). Mean oxygen saturation increased after BCPC from 74.4% to 79.6% (p < 0.01). Mean pulmonary artery pressures decreased after BCPC from 15.1 to 13.5 mmHg (n.s.). The mean left atrial pressure decreased from 5.8 to 4.9 mmHg prior to TCPC (p = 0.06). The pulmonary/systemic blood flow ratio was 1.4 prior to BCPC and decreased to 0.67 prior to TCPC (p = 0.04). The pulmonary/systemic resistance ratio decreased also from 0.19 to 0.07 prior to TCPC (p < 0.01).
Median duration of pleural effusions was 4 days. In all patients a Gore-Tex® tube of at least 18 mm was inserted at the time of TCPC. Incision of the lower surface of the pulmonary artery, right up to the bifurcation, allowed enlargement of the pulmonary arterial pathway. In only six patients an additional pulmonary artery patch plasty was necessary.
| 4. Discussion |
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It is of note, however, that our institutional policy foresees not only an early staging towards a BCPC, but likewise a swift completion within the first 2–4 years of life. Pulmonary artery size is not of utmost importance when it comes to completing a TCPC in the now preferred fashion of an extracardiac modification. There are consistent findings from studies investigating pulmonary arteries and proof that pulmonary artery growth does not match the increase in body size after staging to a Fontan-like circulation [5–7]. Our findings suggest that the pulmonary arteries grew between the staging phases, however, not proportionally to body surface area with z-scores for the right pulmonary artery of –0.25 ± 1.7 and –1.01 ± 1.5 for the left pulmonary artery. Interestingly, even with APBF, pulmonary arteries do not show a normal growth. This has been clearly shown in the collective of Berdat and co-workers [1] and Yoshida and co-workers [2]. Berdat, who compared patients with a BCPC alone with those who had an APBF, found that left and right pulmonary artery dimensions were smaller than normal in all groups. APBF did show slightly increased pulmonary artery diameters but failed to reach significance compared to BCPC alone. Yoshida suggested an appropriate APBF together with a BCPC. As a guidance to appropriate APBF they monitored central venous pressures intraoperatively. However, even with this measure, pulmonary artery growth retardation could only be suppressed slightly. But again, they have not demonstrated that APBF made any difference in clinical outcome.
Although timing of staging procedures, pulmonary artery pressures and blood flow contribute to pulmonary artery distension and are likely affecting growth, other surgical aspects must also be taken into consideration. Reddy and co-workers [8] were the first to look at parts of the pulmonary artery tree which are generally not involved in any surgical augmentation procedures. In investigating the lower lobe pulmonary artery branches as well as the central areas, they elaborated that measurements of central pulmonary arteries alone, as done in previous publications, may have been a dubious approach in analyzing pulmonary artery growth in the context of a BCPC. In our study, a patch enlargement of the pulmonary artery was performed in 36 patients (42.8%) at time of BCPC.
It is of note, that 13.1% had a bilateral PCPC. As depicted in Fig. 3 the diameters for the lower lobe left pulmonary artery varied for both unilateral and bilateral PCPC. Even though an increase in diameter for the lower lobe pulmonary artery together with a bilateral BCPC was found it failed statistical significance. Still, the findings suggest a benefit of an even blood flow distribution.
Since we never leave any APBF there is also generally no need for extensive dissection and search for the sometimes-difficult access to the pulmonary trunk or any shunt at the time of TCPC.
Other institutions even claim underdeveloped pulmonary arteries as a reason not to complete staging treatment towards a TCPC [2]. Likewise, Tanoue and co-workers [9] reported on 333 patients with BCPC and an APBF in 163. Median age was 15 months. A TCPC was performed after a mean of 23 months, however, 66 patients are still awaiting completion. In our experience on now more than 150 patients after a TCPC since 1999, and the introduction of the extracardiac approach [10], we usually go ahead with completion once the patient weighs 10 kg, regardless of any other cardiac features. To us, the patients age at each staging procedure seems to be the key to potential procedure related mortality and morbidity. As we have shown in a cohort of 132 consecutive patients after unfenestrated TCPC [11] in a multivariate analysis early BCPC a short interval from BCPC to TCPC influenced the overall results positively. Of these patients, the majority was relatively young at the time of TCPC, with a median age of 31 months. A BCPC without APBF was accomplished in 91%. The median duration of hospitalization among the 30 days survivors was 20 days (range 5–128 days). Among 6 variables with values of p below 0.10 in single covariate models, the multiple covariate model revealed only older age at the time of extracardiac TCPC (p = 0.040) as a risk factor for prolonged hospitalization.
To add to this discussion of pulmonary artery size and its possible impact on outcome, Hosein and co-workers [12] published a recent study on whether conventional selection criteria were still applicable in the current era. Their analysis identified that outcome was influenced by preoperatively impaired ventricular function, elevated preoperative pulmonary artery pressures and an earlier year of operation. Risk factors for reintervention were right atrial isomerism and preoperative small pulmonary artery size. It is of note, that not only is their follow-up longer than in our study, but also that pulmonary artery size alone is not necessarily predictive in both early- and mid-term outcome. We assessed not only pulmonary artery sizes but also pulmonary/systemic blood flow ratio and pulmonary/systemic resistance ratio. Mean pulmonary artery pressures were low. We believe that this is probably the more important issue. Likewise, Hosein and co-workers comment was ... that only two criteria have emerged as carrying significant impact on both early and late outcome: preoperative ventricular function and preoperative pulmonary artery pressure. Most authors would suggest that the mean pulmonary artery pressure before TCPC should be less than 18 mmHg, or ideally less than 15 mmHg, with a calculated pulmonary vascular resistance less than 2.0 units/m2 [13,14]. Regarding the diameter of the pulmonary arteries the measurements do not take into consideration the compliance of the vascular bed.
Data of Forbes and co-workers [15] indicated that a BCPC facilitated ventricular volume unloading and regression of ventricular mass in younger children (<3 years of age), and that the beneficial effect of this operation on ventricular end-diastolic volume and mass was clearly age-dependent. Their data also showed that older patients benefited less in terms of enhancing the systemic oxygen saturation from the BCPC. Also Mainwaring and co-workers [3] findings support our preferred strategy. They investigated 149 patients. Ninety-three of these had elimination of all sources of APBF. The results after completion to TCPC demonstrated a significant survival advantage when APBF is eliminated at time of BCPC. They postulated that this survival advantage may be based on improvement in pulmonary resistance and ventricular function. Other long-term follow-up studies demonstrate significant late morbidity and mortality following Fontan-type of surgery related (among others) to ventricular dysfunction [16,17]. Indeed, reduced systolic ventricular function has been assessed in this group of patients [18]. A BCPC has been shown to significantly reduce ventricular end-diastolic volume [19,20]. Applying different echocardiographic techniques (Doppler myocardial echocardiography and M-mode echocardiography), systolic ventricular function can be assessed as demonstrated in a group of patients with tricuspid atresia after staged univentricular palliation [21]. Performing a Fontan operation at a later state is known to result in increased ventricular diameters at short and mid-term follow-up [22].
Diffuse intrapulmonary shunts are reported shortly after a BCPC. This may be due to a lack of liver factors as blood from the liver bypasses the lung in this condition, if no accessory pulmonary blood flow is present [23,24]. However, we could show that the only effective treatment for this condition is a cavopulmonary completion [25]. Early completion to a total cavopulmonary connection may prevent recurrent severe cyanosis, a well known late complication after a classical Glenn shunt operation [26]. Hence, we believe that there is absolutely no reason to volume load a functionally univentricular heart at the time of a BCPC with an additional shunt. Early completion to a total cavopulmonary connection leads to early volume unloading of the systemic ventricle and minimizes the risk to severe intrapulmonary right-to-left shunting. To date we do not know how pulmonary artery sizes develop after an extracardiac TCPC at long-term as most patients do well and do not require repeat catheterization. A prospective trial with, for example, repeated magnetic resonance imaging may allow for further insight into the issue of pulmonary artery growth together with our preferred timely completion to an extracardiac TCPC.
| Appendix A |
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Mr V. Tsang (London, United Kingdom): The hemodynamics in your patient population, the PA pressure was 15, the LA pressure was 5 or 4, the Qp/Qs was 1.4, and you mentioned you dont fenestrate and there are no effusions. Why dont you do it in one stage and do it early?
Dr Schreiber: That's a good question. Maybe well embark on that in the near future. Our recent paper on completion to TCPC is out just now. We report on 132 consecutive patients in whom we did not perform a fenestration. There are some patients who were in intensive care for 4 weeks, but eventually they all went home without a fenestration. Even with our extensive experience we still cannot predict precisely who will worsen after completion, or who will go home after one week. I think work is in progress. Professor de Leval was giving a very nice lecture on this topic yesterday, highlighting the controversies. Maybe there are a few surgeons here who will, with a good working BCPC and a nicely oxygenated child with good ventricular function, just wait 2, 3, 4, 5, 6 years and only then go for a TCPC. Our presented results represent retrospective findings, but we should just continue to exchange ideas in the future.
Dr J. Fragata (Lisbon, Portugal): We have not been doing exactly this. We have always been leaving an additional source of blood to the pulmonary arteries, aiming for the pulsatile flow and the growth for the arteries. But doing them earlier, completing the second stage earlier, by 2 years of age, and doing them extracardiac, as we are doing, what kind of artificial conduits do you use, or do you try to mobilize and do direct anastomosis? The size of the conduit, we dont know exactly how big, but I am always reluctant to use less than a 16 or even an 18.
Dr Schreiber: We only use 18 mm tubes which fit nicely into a child weighing 10 kg.
Dr Fragata: Even at this age group?
Dr Schreiber: Yes.
Dr Fragata: So at 2 years of age?
Dr Schreiber: Yes. And maybe because we prefer simplicity we only dissect the right side of the heart out. Without any sources of additional blood flow not even the pulmonary trunk or a shunt have to be freed from adhesions. After dissection of the right side of the heart you can go on CPB for 30 to 40 min and simply complete the extracardiac pathway.
Dr B. Maruszewski (Warsaw, Poland): I want to confirm from our experience in the past that we were also leaving additional pulsatile blood flow, and we found in our series of patients that the PAs do not grow, and that if you look at the normalized PA size over time, it's actually decreasing. So we stopped doing this. What is your rationale for the final stage, for the TCPC; what is your decision-making if you do it in a 1-1/2-year-old patient or a 4-year-old patient? Is it the size of conduit? And do you believe that an 18 mm conduit in an extracardiac Fontan will last forever, will be enough for the adult life?
Dr Schreiber: For the last part of your question, there are quite a few publications out there that were investigating adults. It looks like an 18 mm diameter tube system will allow for older age. Because of that, you probably do not have to go back. If there will be some kind of distortion once the infant has grown-up, we do not know yet. And to the first part of your question, that is just our institutional policy. We try to avoid fistulae which might develop. We try to allow for hepatic blood to go into both lungs. Our data represent our institutional policy for the last 5 or 6 years. And finally, referring to the pulmonary sizes, the available publications give proof that even with an additional blood flow pulmonary arteries do not grow as they grow in healthy children.
Dr A. Amodeo (Rome, Italy): We can probably answer the question if the conduit, an 18 mm can last. We inserted the first extracardiac conduit in 1988, and in the first year we used a Dacron conduit. So the first group of patients, 35 patients, have a mean follow-up of almost 15 years, and in these patients we have never changed any conduit. So probably there is really no worry about the late fate of an extracardiac conduit. And, as I told you, the first conduit we had been using was a Dacron conduit. When you say that you use 18 mm conduits in any patient, well, sometimes I find it very difficult to put an 18 mm conduit in any kilogram patient, believe me, and I have to put a 16 Gore-Tex conduit, and even 14 sometimes in a smaller patient.
Mr V. Tsang (London, United Kingdom): Can I just push one point. You guys in Munich are crazy about early unloading and no antegrade flow.
Pascal, can you comment on mixed circulation?
Dr P. Vouhe (Paris, France): Well, I can comment, but I always say the same thing. I think it's a matter of philosophy. Either you think that the Fontan circulation is the best thing for the patient, and you should do it as early as possible, as you do, with excellent early and midterm results. On the contrary, you think that the Fontan circulation is the worst thing, as we do, because of all the potential complications which may occur. In this case the best for the patient is to delay Fontan completion as long as possible, and if you want to delay as long as possible, it is usually useful to leave some source of additional blood flow. We have shown in previous papers that it is not deleterious to leave some additional pulmonary blood flow.
Mr Tsang: It's nice to have a balanced view about things.
Dr C. Sebening (Heidelberg, Germany): I have a question relating to the complex that Pascal evoked. In the sole source of pulmonary blood supply by an exclusive bidirectional Glenn shunt, if you palliate them early, did you observe an increased incidence of pulmonary arteriovenous fistula, if this is the sole source of pulmonary blood supply?
Dr Schreiber: No.
| Footnotes |
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Presented at the 21st Annual Meeting of the European Association of Cardio-thoracic Surgery, Geneva, Switzerland, September 16–19, 2007.
1 Both these authors contributed equally towards this paper. ![]()
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