Experience with bidirectional cavopulmonary anastomosis and ...

ARTICLE IN PRESS doi:10.1510/icvts.2010.253567 Editorial

www.icvts.org

Institutional report - Congenital

Received 29 August 2010; received in revised form 16 December 2010; accepted 18 December 2010

Institutional Report

Department of Congenital Heart Defects, Bakoulev Center for Cardiovascular Surgery, Moscow, Russia

Protocol

Ivan A. Yurlov, Vladimir P. Podzolkov, Mikhail M. Zelenikin, Dmitry V. Kovalev, Guvandg K. Babaev, Neele A. Putiato, Sergey B. Zaets*

Work in Progress Report

Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy夞

New Ideas

Interactive CardioVascular and Thoracic Surgery 12 (2011) 563–568

Abstract

Nomenclature Historical Pages Brief Case Report Communication

During 1983–2010, a total of 681 consecutive patients with a functionally single ventricle underwent BCPA or MFO. Thirty-nine of them (5.7%) had a visceral heterotaxy. Thirty-one patients with heterotaxy were subjected to BCPA only, seven to BCPA followed by MFO, and one to MFO only. The age of patients at the time of the aforementioned procedures ranged from one to 29 (median, eight) years. Prior to these interventions, two patients underwent Blalock–Taussig shunt, five a Gore-Tex shunt and two a Vishnevsky–Donetsky shunt (subclavian artery-to-pulmonary

Best Evidence Topic

䊚 2011 Published by European Association for Cardio-Thoracic Surgery

2.1. Patient population

State-of-the-art

夞 Presented at the 24th Annual Meeting of the European Association for Cardio-thoracic Surgery, Geneva, Switzerland, September 11–15, 2010. *Corresponding author. 1 Wall Street 5B, Fort Lee, NJ 07024, USA. Tel.: q1 201 873 8901; fax: q1 609 419 3006. E-mail address: [email protected] (S.B. Zaets).

2. Materials and methods

Follow-up Paper

Surgical treatment of patients with a functionally single ventricle and associated visceral heterotaxy is challenging. Hospital mortality after the initial palliation is high (25%– 60%) and may reach 95% if the lesion is associated with obstructed total anomalous pulmonary venous return w1, 2x. According to ‘classic’ principles of selection for Fontan operation w3x, patients with visceral heterotaxy are not ‘ideal’ candidates for this procedure, because of multiple risk factors related to the presence of concomitant anomalies. This group of patients frequently has anomalies of the systemic and pulmonary veins, the dominant right ventricle with compromised ejection fraction, and the regurgitant common atrioventricular valve as well as a variable anatomy of the sinus node predisposing to arrhythmias. In the 1980s, hospital mortality after modified Fontan operation (MFO) performed in patients with heterotaxy reached 43%–80% w4, 5x. However, the progress achieved since that time in surgical technique and postoperative

care resulted in a significant decrease in mortality w6, 7x. A staged surgical approach, when bidirectional cavopulmonary anastomosis (BCPA) precedes MFO, became a thoroughly used strategy w6, 8x. However, not all investigators share the opinion that visceral heterotaxy is no longer a risk factor for hemodynamic correction of complex congenital heart defects w8x. The goal of this study is to analyze our experience with BCPA and MFO in patients with a functionally single ventricle and concomitant heterotaxy as well as to reveal corresponding risk factors.

Negative Results

1. Introduction

Proposal for Bailout Procedure

Keywords: Heterotaxy syndrome; Right isomerism; Left isomerism; Single ventricle; Modified Fontan operation; Bidirectional cavopulmonary anastomosis

ESCVS Article

The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983–2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at followup. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (Ps0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results. 䊚 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

ARTICLE IN PRESS 564

I.A. Yurlov et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 563–568

shunt with a lyophilized graft). The interval between BCPA and MFO ranged from one to 15 (mean, eight) years. Thirtyseven patients were followed-up after BCPA or MFO.

Table 2. Concomitant cardiac anomalies Concomitant anomaly

Number of cases

2.2. Study protocol The study protocol was approved by the Ethical Committee of Bakoulev Center for Cardiovascular Surgery. Medical records of 39 patients with heterotaxy were reviewed for demographics and preoperative and postoperative parameters. Medical records of patients without heterotaxy subjected to BCPA or MFO were reviewed for outcomes only.

Double outlet right ventricle Double outlet left ventricle Transposition of great arteries Patent ductus arteriosus Insufficiency of the common atrioventricular valve Mild Moderate Severe Insufficiency of the right atrioventricular valve (moderate) Insufficiency of the left atrioventricular valve (moderate) Wolf–Parkinson–White syndrome

10 2 2

2.3. Anatomy and preoperative hemodynamics

*Successfully treated by radiofrequent ablation prior to MFO. MFO, modified Fontan operation.

Cardiac anatomy and concomitant anomalies are presented in Tables 1 and 2. Patients with the left isomerism had polysplenia, whereas patients with the right isomerism had asplenia. Thirty-one patients (74%) had a regurgitation of different severity at the atrioventricular valves. Preoperative parameters are presented in Table 3. Thirtyeight patients had preoperative sinus rhythm and one had a nodal rhythm. Six patients prior to BCPA and one patient prior to MFO had mean pulmonary arterial pressure exceeding 15 mmHg. Pulmonary vascular resistance (PVR) exceeded 4 units=m2 in two patients prior to BCPA only. Four patients prior to BCPA and two patients before MFO had mild-to-moderate hypoplasiaystenosis of pulmonary arteries, and their pulmonary arterial Nakata index was below 200 mmym2. The average number of exceeded ‘operability’ using Choussat et al. criteria was significantly higher in patients subjected to BCPA than in patients that underwent MFO (2.2"0.7 vs. 1.6"0.6, Ps0.043).

29 6 11 12 1 1 1*

2.4. Surgical techniques One-sided and bilateral BCPA was performed in 18 and 20 patients, respectively. Fontan modifications included extracardiac conduit (ns7) and fenestrated lateral tunnel (ns1). Additional procedures performed simultaneously with BCPA or MFO are presented in Table 4. 2.5. Statistical analysis The data are presented as mean"S.D. andyor median and range, as appropriate or absolute numbers and percentages. The Student’s t-test or analysis of variance (ANOVA) was used to compare continuous variables, as appropriate. Chi-square (x2) analysis was used to test for differences in proportions. Actuarial survival was determined by the Kap-

Table 1. Anatomy of congenital heart defects Anatomy Visceral situs Situs solitus Situs inversus Situs ambiguous Cardiac position Levocardia Dextrocardia Mesocardia Ventricular morphology Dominant left ventricle Dominant right ventricle Atrioventricular valve morphology Common valve Atresia of the right valve Two valves Location of vena cava superior Bilateral Connection of inferior vena cava Atrium Azygosyhemiazygos to SVC Hepatic vein connection To vena cava inferior Separately to the atrium Pulmonary venous connection Normal Partialytotal anomalous 1

Left isomerism (ns21)

Right isomerism (ns18)

Total (ns39)

– – 21 (100%)

– 1 (6%) 17 (94%)

– 1 (3%) 38 (97%)

6 (28.5%) 13 (62%) 2 (9.5%)

8 (44.5%) 8 (44.5%) 2 (11%)

14 (36%) 21 (54%) 4 (10%)

7 (33%)1 14 (67%)2

14 (78%) 4 (22%)

21 (54%) 18 (46%)

15 (71.5%) 2 (9.5%) 4 (19%)

14 (78%) 1 (5.5%) 3 (16.5%)

29 (74%) 3 (8%) 7 (18%)

9 (50%)

21 (54%)

12 (57%) 6 (28.5%)3 15 (71.5%)4

18 (100%) –

24 (61.5%) 15 (38.5%)

7 (33%)5 14 (67%)6

13 (72%) 5 (28%)

20 (51%) 19 (49%)

20 (95%)7 1 (5%)8

11 (61%) 7 (39%)

31 (79%) 8 (21%)

– 8Ps0.014, 0.014, 0.0001, 0.0001, 0.036, 0.036, 0.026, 0.026, respectively vs. right isomerism. SVC, superior vena cava.

ARTICLE IN PRESS I.A. Yurlov et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 563–568

565 Editorial

Table 3. Preoperative clinical and hemodynamic parameters Parameters

Prior to MFO Median

Range

Mean

Median

Range

3.9"0.3 191.2"29.4 72.3"8.2 12.9"4.5 3.0"0.9 302.4"104.6 9.8"4.1 64.0"5.2

4.0 186.0 73.0 12.0 3.0 275.0 10.5 63.0

3–4 158–244 54–82 7–22 1.7–4.7 150–502 4–18 57–72

3.8"0.4 197.0"30.7 75.3"5.8 14.4"3.6 3.1"0.7 323.0"149.1 5.9"2.7* 61.2"4.4

4.0 199.5 77.5 13.0 3.2 305 6.0 60

3–4 160–236 66–82 9–20 2.0–3.9 130–514 3–9 56–68

Surgical intervention

Number of cases

1 5* 1 1 1 7 –

8 – – – 2 1 1

*In three cases, shunts were preserved because of technical difficulties caused by a graft calcinosis (ns1) or low oxygen saturation after the completion of BCPA (ns2). BCPA, bidirectional cavopulmonary anastomosis; MFO, modified Fontan operation.

Table 5. Non-lethal hospital complications after BCPA and MFO

MFO (ns8)

7y38 3* 4 – 2y38 4y38 1y38 1y38 2y29 17**

2y8 (25%) 1* – 1 3y8 (37.5%)1 4y8 (50%)2 – – – 9***

(18%)

(5%) (10.5%) (2.5%) (2.5%) (7%)

1 Ps0.042 vs. BCPA; 2Ps0.031 vs. BCPA. *One patient in each group required permanent pacemaker. **Seventeen complications occurred in 13 patients. ***Nine complications occurred in six patients. BCPA, bidirectional cavopulmonary anastomosis; MFO, modified Fontan operation.

Brief Case Report Communication

BCPA (ns38) Arrhythmias Complete atrioventricular blockade Supraventricular tachycardia Junctional rhythm Prolonged pleural effusion (G5 days) Heart failure Renal and hepatic failure Pneumonia, sepsis Wound infection Total

Historical Pages

Number of cases

Nomenclature

Complication

Best Evidence Topic

Hospital mortality after BCPA reached 7.9% (3y38). Two patients experienced heart failure and hypoxemia after the completion of BCPA. Creation of aorta-to-pulmonary anastomosis did not lead to the increase in arterial blood oxygen saturation (SaO2) and improvement of hemodynamics. The third patient died on the 10th day after BCPA from heart failure and renal failure that required hemodialysis. This patient also suffered from supraventricular tachycardia. In all three cases, preoperative profile was remarkable for moderate insufficiency of the common atrioventricular valve and the presence of bilateral venae cava. One patient also had a hypoplasia of the left pulmonary artery. The hospital mortality after BCPA in patients with and without heterotaxy did not differ significantly w7.9% (3y8) vs. 6.9% (18y259), Ps0.898x. One patient died on the 23rd day after the Fontan operation (extracardiac conduit and replacement of the common atrioventricular valve) from heart and renal failure. There was no dysfunction of the valve prosthesis. Except for the severe insufficiency of the atrioventricular

State-of-the-art

3.1. Course of the hospital period

Follow-up Paper

3. Results

valve, the patient’s preoperative profile was remarkable for a moderate hypoplasia of the right pulmonary artery. The hospital mortality after MFO in patients with and without heterotaxy did not significantly differ w12.5% (1y8) vs. 11.7% (44y376), Ps0.627x. Non-lethal hospital complications after BCPA and MFO are presented in Table 5. Duration of hospital stay was longer in patients after MFO (41.0"18.0 days vs. 15.5"7.3 days, Ps0.048). Patients who died or developed postoperative complications after BCPA or MFO had a significantly higher number of exceeded ‘operability’ criteria than those with uneventful hospital period (2.2"0.5 vs. 1.6"0.4, Ps0.04). SaO2 at discharge after MFO and BCPA was 90.3"2.6% and 85.6"5.8%, respectively (Ps0.034). SaO2 after both surgi-

Negative Results

lan–Meier method. Multivariate logistic regression analysis was used to identify risk factors for lethal outcomes in patients with heterotaxy. Variables with probability (P) values F0.1 determined by univariate model were entered into a multivariate model. Results are given as odds ratios (OR) and 0.95 confidence interval (CI). P-values -0.05 were considered statistically significant.

Proposal for Bailout Procedure

MFO

ESCVS Article

Ligationysuturing of pulmonary trunk Liquidation of previous systemic-to-pulmonary shunt Enlargement of atrial septal defect Patch closure of tricuspid component of common atrioventricular valve Suturing of mitral component of common atrioventricular valveqannuloplasty of tricuspid component by Gore-Tex semi-ring Suture annuloplasty of common atrioventricular valve Replacement of common atrioventricular valve

BCPA


Table 4. Additional surgical interventions performed during BCPA or Fontan operation

Protocol

*Ps0.017 vs. BCPA. MPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; PAI, pulmonary arterial (Nakata) index; SVEDP, systemic ventricle end-diastolic pressure; SVEF, systemic ventricle ejection fraction; BCPA, bidirectional cavopulmonary anastomosis; SaO2, oxygen saturation; MFO, modified Fontan operation.


Mean

New Ideas

NYHA class Hemoglobin (gyl) SaO2 at room air (%) MPAP (mmHg) PVR (units=m2 ) PAI (mmym2) SVEDP (mmHg) SVEF (%)

Prior to BCPA



cal interventions significantly improved, compared with preoperative levels (Ps0.000). 3.2. Course of the follow-up period Follow-up of patients after BCPA and MFO is presented in Table 6. The duration of follow-up after BCPA was significantly longer than after MFO (Ps0.000). One patient died 10 years after BCPA (Table 7). Lethal outcomes after MFO occurred at three months, six months, and three years after surgery, respectively. Actuarial fiveand 10-year survival after BCPA and MFO was 83% and 76%, respectively. Late mortality after BCPA and MFO in patients with and without heterotaxy did not significantly differ (8.7% and 9.5%, respectively; Ps0.926). Patients after both surgical interventions that underwent follow-up examination at our institution significantly improved their functional class and SaO2 compared to preoperative values (Table 6). SaO2 after MFO was significantly higher than after BCPA (Ps0.000). Transluminal balloon dilatationystenting of pulmonary arteries was successful after BCPA and led to only temporary partial relief of symptoms in a patient after MFO. Residual regurgitation at atrioventricular valves (Table 6) worsened patients’ condition. 3.3. Risk factors for BCPA and MFO The only independent risk factor for a hospital or late lethal outcome in patient with heterotaxy was moderateto-severe preoperative insufficiency of atrioventricular valves (Ps0.012) (Table 8).

Table 7. Lethal outcomes and complications at follow-up

Lethal outcomes Hemorrhage at sternotomy (attempt of MFO) Pulmonary embolism Progressive congestive heart failure Complications Stenosis of pulmonary artery branches andyor BCPA Recanalization of hemiazygos vein Sinus bradycardia Supraventricular tachycardia Atrial flutter Complete atrioventricular blockade

BCPA

MFO

1 – –

– 1 2

3* 1** – 1 1 1***

1*,1 – 1 – – 1***,1

*Required transluminal balloon dilatationystenting. **Required embolization. ***Required permanent pacemacker. 1 Patient died. BCPA, bidirectional cavopulmonary anastomosis; MFO, modified Fontan operation.

4. Discussion In the majority of published series, the number of MFO performed in patients with visceral heterotaxy ranges from 20 to 60 w4, 8–12x. The largest series include 135–142 cases w6, 7x. Most commonly, hospital mortality ranges from 3% to 20% w6, 8–11x. Over the years, the hospital mortality dramatically dropped from 40%–80% in the 1980s w4, 5x to 4%–5% in surgical series published in 2006–2010 w8, 10x. There is a dependency of mortality rate on the year of operation w6, 7x. In our small series of MFO collected during year period, the hospital mortality reaches 12.5%. Despite the reduction in mortality, it remains disputable whether a visceral heterotaxy is a risk factor for lethal outcomes after MFO w8, 9, 13x. It has become clear that not heterotaxy per se but the complexity of cardiac malformations asso-

Table 6. Characteristics of follow-up period

Number of followed-up patients Duration of follow-up (years) Number of patients lost for follow-up Number of patients examined in our institution NYHA class I II III IV Mean NYHA class At follow-up Before surgery Dynamics of regurgitation at atrioventricular valves No correction of mildymoderate regurgitation at surgery: BCPA (ns13) or MFO (ns2) Remained mild or absent Worsened from mild-to-moderate Remained moderate Worsened from moderate-to-severe After surgical correction of severe regurgitation: BCPA (ns6) or MFO (ns3) Absentymild Moderate Severe SaO2 At follow-up At discharge Before surgery

BCPA

MFO

31 1–18 (median – 9) 4 25

6 0.25–3 (median – 1.5) 1 5

– 7 (28%) 11 (44%) 7 (28%)

2 (40%) 1 (20%) 2 (40%) –

3.0"0.81 3.8"0.4

2.0"0.92 3.8"0.4

2 2 6 3

1 – 1 –

2 3 1

1 2 –

83.9"6.73 88.1"4.7 73.4"7.9

92.0"4.44 90.3"2.6 76.5"6.6

1 Ps0.000 vs. before surgery. 2 Ps0.003 vs. before surgery. 3 Ps0.004 vs. before surgery and Ps0.134 vs. at discharge. 4 Ps0.008 vs. before surgery and Ps0.53 vs. at discharge. BCPA, bidirectional cavopulmonary anastomosis; MFO, modified Fontan operation; SaO2, oxygen saturation.

ARTICLE IN PRESS I.A. Yurlov et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 563–568

567 Editorial

Table 8. Risk factors for hospital and late lethal outcomes after BCPA or MFO in patients with heterotaxy syndrome

0.012

3.521 (1.133–14.415)

0.162 0.247

1.006 (0.963–1.009) 0.932 (0.904–1.041)

BCPA, bidirectional cavopulmonary anastomosis; MFO, modified Fontan operation; CI, confidence interval.

Proposal for Bailout Procedure Negative Results Follow-up Paper State-of-the-art Best Evidence Topic Nomenclature Historical Pages Brief Case Report Communication

systemic ventricle w10x, and prolonged cardiopulmonary bypass w10x. Our analysis has shown that only preoperative moderate-to-severe regurgitation at atrioventricular valves is a risk factor for lethal outcomes after BCPA and MFO in patients with heterotaxy. Late mortality after MFO in patients with heterotaxy ranges from 3% to 10% w6, 8–11x. There is almost no comparison of functional status in patients with and without heterotaxy after surgery. Surprisingly, despite the high frequency of arrhythmias in patients with heterotaxy subjected to Fontan operation, 80%–100% of them are classified as I–II NYHA class w6, 7, 10x. Atz et al. did not find differences in exercise performance after MFO in patients with and without heterotaxy w13x. Our data indicate that residual regurgitation at atrioventricular valves in patients with heterotaxy contributes to deterioration of their condition both after BCPA and MFO. According to the literature, 40%–76% patients with heterotaxy subjected to MFO have at least mild regurgitation at the atrioventricular valves at follow-up. Our study has several limitations. First of all, this a nonrandomized, retrospective study. BCPA and MFO in patients with visceral heterotaxy syndrome were performed throughout a 27-year period. During three decades, significant improvements in surgical technique, anesthesiological, and postoperative care were achieved. Thus, there was no standard of care applied to the patient population. Second, we have compared only the frequency of lethal outcomes after BCPA and MFO but not the frequency of complications in patients with and without heterotaxy. Besides, the number of patients that were subjected to MFO was not enough to determine risk factors for MFO and BCPA separately. Last, the period of follow-up after MFO was short. In conclusion, our study did not reveal significant differences in mortality after BCPA and MFO in patients with a functionally single ventricle with and without concomitant visceral heterotaxy. Longer periods of follow-up are needed to conclude whether heterotaxy is a risk factor for these interventions. Preoperative insufficiency of atrioventricular valves negatively influences surgical results. That is why not only a severe but also a moderate regurgitation at

ESCVS Article

ciated with this syndrome may negatively influence results of hemodynamic correction. Variable anatomy of the sinus node is typical for heterotaxy syndrome. Cardiac rhythm disturbances were found in 23%–36% patients with visceral heterotaxy prior to BCPA or MFO w6, 11, 14x. Surprisingly, we have revealed preoperative nodal bradycardia in one (3%) patient only. This is probably because of the absence of a routine Holter monitoring. In our series, 74% patients with heterotaxy had common atrioventricular valve, which is consistent with the observations of other authors (73%–83%) w6, 14, 15x. The common valve demonstrated moderate-to-severe regurgitation in 79% patients, and had a multileaflet anatomy (four to six leaflets) in 34% of cases. Thus, patients with visceral heterotaxy may accumulate potential risk factors for MFO. However, there are only a few works that have statistical power to determine if patients with heterotaxy have worse outcomes after this intervention than those without heterotaxy. Culbertson et al. did not report significant differences in mortality after MFO between patients with and without heterotaxy (20% vs. 8.5%) w9x. Kim et al. showed that hospital mortality in the heterotaxy group was significantly higher (4.8% vs. 2.4%) w8x. At the same time, the authors failed to reveal differences in postoperative hemodynamics, duration of mechanical ventilation, chest tubes, intensive care unit and hospital stay between patients with and without heterotaxy. Koudieh et al. demonstrated that both hospital and late mortality after BCPA was significantly higher in patients with heterotaxy (9% vs. 0.8% and 6% vs. 1.6%) w14x. Moreover, the duration of mechanical ventilation, inotropic support, intensive care unit and hospital stay was also significantly longer in patients with heterotaxy. In our series, hospital and late mortality after BCPA and MFO did not significantly differ in patients with and without heterotaxy. There is more data showing which factors influence surgical results in patients with visceral heterotaxy. These factors are regurgitation at atrioventricular valves or valve replacement w7, 9, 14x, elevated pulmonary arterial pressure w12x or PVR w9x, elevated end-diastolic pressure of the


0.725 0.323 0.401 0.221 0.106 0.169 0.002 0.143 0.074 0.095 0.351 0.128 0.289 0.205 0.421

Odds ratio (95% CI)

Protocol

Age )16 years Right isomerism Left isomerism Systemic right ventricle Ejection fraction of the systemic ventricle -60% End-diastolic ventricular pressure )10 mmHg Moderate-to-severe atrioventricular valve regurgitation Mean pulmonary artery pressure )15 mmHg Pulmonary vascular resistance )3.5 units=m2 Pulmonary arterial (Nakata) index -200 mmym2 Anomalous systemic venous return Interrupted vena cava inferior Anomalous pulmonary venous return Cardiopulmonary bypass time )180 min Aortic cross-clamp time )90 min

Multivariate P-value


Univariate P-value

New Ideas

Potential risk factors (tested variables)



atrioventricular valves should be corrected. However, the long-lasting effectiveness of valvular repair remains challenging. References w1x Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol 2000;36:908–916. w2x Gaynor JW, Collins MH, Rychik J, Gaughan JP, Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg 1999;117:506–513. w3x Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan procedure. In: Anderson RH, Shinebourne EA, editors. Pediatric Cardiology. Edinburgh: Churchill Livingstone, 1977:559– 566. w4x Humes RA, Feldt RH, Porter CJ, Julsrud PR, Puga FJ, Danielson GK. The modified Fontan operation for asplenia and polysplenia syndromes. J Thorac Cardiovasc Surg 1988;96:212–218. w5x Marcelletti C, Di Donato R, Nijveld A, Squitieri C, Bulterijs AH, Naeff M, Schuller J, Becker AE. Right and left isomerism: the cardiac surgeon’s view. Ann Thorac Surg 1983;35:400–405. w6x Stamm C, Friehs I, Duebener LF, Zurakowski D, Mayer JE, Jonas RA, del Nido PJ. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg 2002;74:1967–1977. w7x Bartz PJ, Driscoll DJ, Dearani JA, Puga FJ, Danielson GK, O’Leary PW, Earing MG, Warnes CA, Hodge DO, Cetta F. Early and late results of the modified Fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol 2006;48:2301–2305. w8x Kim SJ, Kim WH, Lim HG, Lee CH, Lee JY. Improving results of the Fontan procedure in patients with heterotaxy syndrome. Ann Thorac Surg 2006;82:1245–1251. w9x Culbertson CB, George BL, Day RW, Laks H, Williams RG. Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure. J Am Coll Cardiol 1992;20:678–684. w10x Naito Y, Aoki M, Matsuo K, Nakajima H, Aotsuka H, Fujiwara T. Intracardiac Fontan procedure for heterotaxy syndrome with complex systemic and pulmonary venous anomalies. Eur J Cardiothorac Surg 2010;37:197–203. w11x Azakie A, Merklinger SL, Williams WG, Van Arsdell GS, Coles JG, Adatia I. Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes. Ann Thorac Surg 2001;72:1636– 1640. w12x Nakano T, Kado H. Long-term surgical results of total cavopulmonary connection in children with visceral heterotaxy syndrome; comparison between lateral tunnel method and extracardiac conduit method. Kyobu Geka 2003;56:299–303. w13x Atz AM, Cohen MS, Sleeper LA, McCrindle BW, Lu M, Prakash A, Breitbart RE, Williams RV, Sang CJ, Wernovsky G, Investigators of the Pediatric Heart Network. Functional state of patients with heterotaxy syndrome following the Fontan operation. Cardiol Young 2007;17(Suppl 2):44–53. w14x Koudieh M, McKenzie ED, Fraser CD. Outcome of Glenn anastomosis for heterotaxy syndrome with single ventricle. Asian Cardiovasc Thorac Ann 2006;14:235–238. w15x Takeuchi K, Murakami A, Hirata Y, Kitahori K, Doi Y, Takamoto S. Surgical outcome of heterotaxy syndrome in a single institution. Asian Cardiovasc Thorac Ann 2006;14:489–494.

Conference discussion Dr. L. Galletti (Bergamo, Italy): Dr. Yurlov and his colleagues today presented their experience with cavopulmonary shunts in the treatment of patients with single-ventricle and visceral heterotaxia. Their experience spans a very long period of time, 27 years.

Heterotaxy syndrome still certainly constitutes a very, very difficult group of patients mainly due to the problems related to AV valve dysfunction, systemic and pulmonary venous anomalies, and high incidence of arrhythmias, all factors that could impair suitability for Fontan operation. In their experience, 39 patients underwent a cavopulmonary shunt with a mortality around 8%. Among the 35 survivors, only eight patients underwent Fontan completion with an early mortality of 12% and a one-year survival around 60%. I suspect that this number is significantly lower than other single-ventricle groups, for example, tricuspid atresia. So your findings confirm what is already seen in the literature that these patients tolerate much worse Fontan circulation than Glenn physiology. My first question is related to the indication for Fontan procedure. Does the fact that only 22% of patients who survived the Glenn underwent a Fontan procedure reflect the general policy of your institution or a particularly strict selection process for this kind of patient? In other words, what is, in your experience, the argument to complete the Fontan, or more important, not to proceed with the completion of the Fontan? The second point is partly related to the first one. As you know, heterotaxic patients, especially the polysplenic or left isomeric patient, show a particular propensity to develop pulmonary arteriovenous malformation possibly related to the lack of hepatic flow to the pulmonary circulation. In your manuscript, you state that almost every time you ligate the pulmonary trunk at the time of the Glenn. So what has been the reason for suppression of this accessory flow to these patients when they have little chance to have their Fontan completed in the future? And did you observe recurrent cyanosis during the follow-up related to this problem, and what has been the treatment for this condition? Dr. Yurlov: I believe there is only one question. Yes, it is a very difficult group of patients, and heterotaxy syndrome for a long time was just the end of discussion. For a long time, we made only BCPAs and did not think about Fontan operation at all. But still there was a group of Kawashima operation with interrupted inferior vena cava and continuation to the left and to the right superior vena cava. And these patients became very cyanotic in several years. And that is the first reason for this group, just to try to complete a Fontan. One patient of this group was in very poor condition because of intrapulmonary malformations late after surgery. And in the year or year and a half after completion of Fontan, he improved. His X-rays didn’t show any signs of intrapulmonary malformations. We did not do any angio at this time, but on X-rays it improved. Dr. R. Hosseinpour (Seville, Spain): A question of terminology. I am probably just exposing my own ignorance more than anything else. But I have never heard these procedures referred to as hemodynamic correction because there is nothing corrective about them. The only time they were referred to as corrective was in the very early papers by Fontan and Baudet because in those days they used to think it was corrective. So why this choice of terminology? Most people call them Fontan or Glenn or bidirectional Glenn. Hemodynamic correction is new to me, but perhaps it is just me. Maybe it is my ignorance. Dr. Yurlov: I can advise you on other terminology. Our Glenn anastomosis is called Russian anastomosis because at the same time as Glenn, such kinds of operation had been done by Dr. Galankin in Moscow. So that explains the terminology. I think the main reason for doing it is to help patients in any way because it is very difficult to see them with heterotaxy or without it, with function of the single ventricle, the hemodynamics, and so on. So I believe that we try to do more and more for them, with any type of operation. Dr. G. Ziemer (Tuebingen, Germany): I will just support Dr. Yurlov. I mean, first of all, he is right. It was not Dr. Glenn who did the first cavo-pulmonary anastomosis. It is just that in Western countries, we think it is. Dr. Yurlov: Thank you very much. Dr. Ziemer: The first research and actual patients were done in the Soviet Union at that time. And secondly, I think it is a matter of translation. I think Dr. Yurlov wants to say that after his surgery the defect is ‘less wrong’. They just make it a little bit ‘more correct’. We know now that it is not a correction. We agree on that.