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J Thorac Cardiovasc Surg 1998;115:1063-1073
© 1998 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Sponsored in part by The Belgian Foundation for Research in PediatricCardiology.
From the Department of Pediatric Cardiology, UZ Leuven, Leuven, Belgiuma;the Department of Pediatric Cardiology, Mayo Clinic, Rochester, Minn.b;Deutsches Herzzentrum, München, Germanyc; and Grown UpCongenital Heart Unit, Royal Brompton and National Heart Hospital, London,United Kingdom.d
Received for publication July 16, 1997. Revisions requested August 25, 1997. Revisions received Nov. 17, 1997. Accepted for publication Nov. 20, 1997. Address for reprints: Marc Gewillig, MD, Pediatric Cardiology,University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium.
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Abstract |
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 Top Abstract IntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Introduction |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Only limited information is presently available on the incidence, thepredisposing factors and the hemodynamic profile of patients who have had theFontan operation and in whom PLE develops.
1,2 Different treatment strategieshave been proposed for PLE after the Fontan operation, but their effect on theoutcome remains largely unknown. Reports on successful management are limited.For the management of these difficult conditions it is, however, crucial to knowwhich approach is doomed to fail so that alternative treatment options can beexplored. The present study is a retrospective multicenter study reviewing theincidence, hemodynamics, treatment strategies, and outcome of patients with PLEafter Fontan-type surgery.
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Materials and methods |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Patient selection.
A study questionnaire was filled out for each patient who had hadFontan-type surgery and in whom clinical symptoms of documented hypoproteinemia(edema, effusions) due to PLE developed. Other causes of hypoproteinemia such asliver failure and renal protein loss needed to be excluded.
In addition, the centers were asked to submit data as to the number ofFontan operations that had been performed, perioperative mortality rates, andfollow-up procedures. This allowed an assessment of the incidence of PLE afterFontan-type surgery. Because of incomplete data sets, detailed information couldnot be gathered on the other patients without PLE who have had Fontanoperations.
Fontan surgery.
Descriptive statistics were performed on the data. The primary outcomevariable was the effect of treatment on the PLE (death, no change, improved,cured). In every patient with PLE, all the requested information was obtained.Student's t test, 
2 test, correlation analysis withFisher's r to z transformation were applied where appropriate. Survival analysiswas performed by the Kaplan-Meier estimation. For the comparison of subgroupsurvival Mantel-Cox log rank analysis was performed. Statistical analysis wasperformed with the software programs Statview 4.5 (Abacus Concepts, Berkeley,Calif.) and GB-stat 6.0 (Dynamic Microsystems, Silver Spring, Md.).
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Results |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Data on all 114 patients with PLE were obtained (72 male and 42 female).The cardiac diagnosis was tricuspid atresia in 36 patients (31.6%),double-inlet left ventricle in 33 patients (29%), and other forms of"complex univentricular heart" in 45 patients (39.4%). Thelast group contained diverse complex congenital heart defects such asatrioventricular canal–type of malformation (8), double-outlet rightventricle (8), mitral atresia (4), single-inlet ventricle (4),L-transposition of the great arteries plussevere pulmonary stenosis (4), double-inlet right ventricle (2), hypoplasticleft heart syndrome (2), pulmonary atresia with intact ventricular septum (2),and other forms of "functional univentricular heart" (10).
Heterotaxy syndrome was present in three patients: two patients withasplenia and one patient with polysplenia. Four patients had situs inversus, andone patient had situs ambiguus.
In 97 of 114 patients (85%) palliative surgery was performedbefore the Fontan operation. The different types of pre-Fontan palliation andthe age at which pre-Fontan surgery was performed are summarized in Table I.In total, 62 of 114 (53%) patients, when initially seen, had lowpulmonary blood flow that caused cyanosis; 33 of 114 (28%) patients hadhigh pulmonary blood flow that required a banding type of operation to protectthe pulmonary circulation. In this series, initial banding was performed at amedian age of 0.6 years (range: 3 days to 3.4 years); five patients were bandedtwice. In 19 of 114 (19%) patients, the initial lung flow was "balanced"and surgical palliation before the Fontan operation was not performed.
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The time distribution of the series reflects changes in surgicaltechniques. In 114 patients, an atrioventricular connection was performed in 22(19.3%); an atriopulmonary connection was performed in 75 (65.8%);and a total cavopulmonary connection (with total or subtotal exclusion of theright atrium) was performed in 17 (14.9%). Thus no type of connection isfree of PLE after Fontan surgery.
The early postoperative course was characterized by a median intensivecare unit stay of 8 days (range: 2 to 50 days), a median postoperativehospitalization duration of 26 days (range: 8 to 150 days), and a requirementfor chest tube drainage during 12 days (median range: 0 to 135 days).Postoperative complications were ascites (n = 17), renal failure (n = 13), arrhythmia(n = 26), respiratory problems (n = 8), wound infection (n =5), unilateral diaphragmatic paralysis (n =3), and neurologic complications (n = 2).
In 12 patients additional surgery was performed between the initialFontan operation and the time PLE was diagnosed. This included closure of a leakin the atrioventricular-valve patch (4), atrioventricular-valve replacement (3),pacemaker implantation (3), plication of the diaphragm (1), and aortic valvereplacement (1). In one patient angioplasty of the pulmonary artery wasperformed.
Clinical diagnosis of PLE and hemodynamic evaluation.
As mentioned in the Materials and methods section, PLE was defined inthis study as the occurrence of clinical symptoms related to documentedhypoproteinemia caused by intestinal loss. Other causes of hypoproteinemia hadto be excluded. Moreover, subclinical gastrointestinal protein loss was notconsidered an inclusion criterion for the present study.
At the time of diagnosis of PLE, the patients had edema (79%),ascites (53%), pleural effusion(s) (22%), and/or chronic diarrhea(11%). Other symptoms or clinical findings present at the time ofdiagnosis were dyspnea (5%), fatigue (3.5%), abdominal fullness (5%),carpopedal spasms (1.7%), and a pericardial effusion (1.7%). Theother complications are summarized in Table III.
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In all patients either total protein and/or serum albumin measurementswere obtained. In 89 of 114 patients the serum albumin level at the time ofdiagnosis was 2.4 ± 0.6 gm/dl (normal value: 3.5 to 5.5 gm/dl). In55 of 114 patients the total protein level was 4.3 ± 0.8 gm/dl(normal value: 5.5 to 8.0 gm/dl). The gastrointestinal protein loss wasquantified by calculating the 
1-antitrypsin clearance in 27patients. The mean value was 432 ± 370 ml/24 hr (normal value <27 ml/24 hr). In seven patients gastrointestinal protein loss was quantified byintravenously administered 51chromium-labeled albumin. An increasedenteric protein loss was demonstrated by an increased excretion of the labeledalbumin in the stools: 22.7% ± 7.2% of the injectedradioactive label (normal: <2.0%).
In 83 (73%) of 114 patients hemodynamic evaluation by cardiaccatheterization was performed after PLE was diagnosed. Table IV summarizes thebasic hemodynamic measurements obtained during catheterization.These data are, however, difficult to interpret because nearly all of thepatients were receiving significant doses of diuretics at the time ofcatheterization. Of special note are the mean cardiac index of 2.4 ± 0.8 L/kg/min and the mean right atrial pressure of 17.0 ±5.3 mm Hg. In 29 patients a gradient was reported to be present in some part ofthe Fontan connection. In 22 patients numeric data for gradients were available(mean: 4.3 ± 3.0 mm Hg; range: 2 to 15 mm Hg). Other findings ofrelevance included intrapulmonary shunting (5), residual connection singleventricle–pulmonary artery (3), atrioventricular valve patch leak (1),obstruction of pulmonary venous return (2), obstruction of the atrial bafflefrom the hepatic veins to the pulmonary artery (1), venovenous collaterals (3),residual Blalock-Taussig shunt (1), diaphragmatic paralysis (3), and coronaryartery ligation (1). Atrioventricular valve regurgitation was present in 29patients (severe [5], moderate [12], and mild [12]). Ventricular dysfunction waspresent in 24 patients (severe [9], moderate [9]). In two patients a markedbradycardia associated with sinus node dysfunction and nodal rhythm was present.
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In our patient population, 52 (45.6%) patients were treatednoninvasively, 52 (45.6%) patients received a surgical intervention, and10 (8.8%) patients received a percutaneous intervention.
Medical treatment only was given to 52 patients. An overview oftreatments used in this group is shown in Table V.Most patients were treated with a combination of diuretics, digoxin, afterloadreduction, and diet. Albumin infusions were given intermittently in 28 of 52patients. Additional medication was given to 32 of 52 patients. This medicalapproach resulted in a subjective improvement with resolution of the PLEsymptoms in 13 of 52 (25%) patients. In 15 of 52 (28.8%) patientssome improvement was observed, but the patients remained symptomatic (alive withPLE). Twenty-four (46.2%) patients from this treatment group died.
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Nine balloon dilations with four stent placements were performed, threebecause of a stenosis in the Fontan circuit and six for a stenosis in apulmonary artery. The mean gradient before balloon dilation was 4.8 ±4.4 mm Hg that was reduced to 2.0 ± 1.9 mm Hg after the procedures.In eight cases the procedure was considered successful with a significantdecrease in gradient or in increase in blood flow. In one patient a balloondilation of the inferior cavopulmonary anastomosis was unsuccessful with noreduction in gradient. Of the eight successful procedures, three had no effecton PLE and three resulted in a long-lasting symptomatic improvement (in onepatient this occurred after further surgery); in two patients only a temporaryimprovement was obtained with a recurrence of PLE symptoms within a few monthsafter the procedure.
Five fenestrations in the intraatrial septum were created percutaneouslywith different techniques. In three patients the fenestration was created byblade septectomy combined with progressive balloon dilation of the fenestration.In one patient the intraatrial septum was punctured, and progressive balloondilations of the puncture hole were performed. In one patient a stent was placedin the fenestration. The time interval between the diagnosis of PLE and thefenestration was 1.6 years (median range: 0.2 to 5.1 years). The median age atfenestration was 17.9 years (range: 2.8 to 41.7 years). The median time intervalbetween the creation of the fenestration and the time of Fontan surgery was 8.5years (range: 1.6 to 10.7 years). In three of the five patients a marked effectwas observed on the PLE symptoms with nearly complete resolution of allsymptoms. In the remaining two patients only a mild improvement was observed. Intwo patients a stroke occurred after the fenestration was created. One of thesepatients was not given anticoagulants after the fenestration.
In one patient systemic–pulmonary artery collaterals were closed bycoil placement, but this did not improve intestinal protein loss.
Fig. 2 represents the Kaplan-Meier survival analysis for the threedifferent treatment groups. Log rank analysis could not demonstratea significant effect of treatment option on PLE survival (Table VIII).
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Discussion |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Clinical characteristics of patients in whom PLE develops after Fontansurgery.
In a recent study Feldt and associates 1 overviewed the clinicalcharacteristics of the Mayo Clinic patients in whom PLE developed after a Fontanoperation. They studied a total of 427 patients who survived for 30 days after aFontan operation performed between July 1973 and January 1987. In 47 of 427 (11%)patients PLE developed during follow up. Univariate and multivariate analysisindicated that the factors associated with the development of PLE wereventricular anatomy (other anatomy than dominant left ventricle), increasedpreoperative ventricular end-diastolic pressure, longer operative bypass time,increased length of hospital stay, and postoperative renal failure. In thepresent study a multivariate analysis could not be performed because the data onthe patients in whom PLE did not develop after Fontan surgery could not beobtained. However, in the patients described in the present study (which alsoincludes the Mayo Clinic data), several of these risk factors seem to bepresent.
The preoperative hemodynamic evaluation shows that some of the patientsin whom PLE develops after Fontan surgery would nowadays no longer be scheduledfor Fontan surgery (for instance, a patient with a pulmonary vascular resistanceof 5.6 units/m2 or a patient with a mean pulmonary artery pressureof 41 mm Hg). Because some of these patients had been initially treated in thepre-Fontan era, the pre-Fontan palliations used reflect a historical evolutionin the treatment of univentricular hearts. This is demonstrated by the widevariation of different initial palliative techniques, with some patients havinghad suboptimal pre-Fontan procedures with poor timing (banding at 0.9 ±0.9 year). This resulted in prolonged and excessive volume loading of theventricle and elevated pulmonary vascular resistance in some of the laterpatients with PLE. This certainly contributed to a difficult early postoperativecourse in some of these patients (such as, with pleural effusions, renalfailure). Although we have no reference values, a mean hospitalization stay of26 days seems long after a Fontan operation.
Remarkable in the present study is the predominance of patients with aleft ventricular morphologic condition (68%), with only 18% of allpatients with PLE having a right ventricular morphologic condition. In 14%of all patients ventricular morphologic condition could not be determined. Thisdistribution is to be compared to the whole Fontan population, but it shows thatfurther study is required to better identify the risk factors associated withPLE.
PLE hemodynamics and pathophysiology.
PLE in cardiac disease is mostly associated with disorders in whichchronically elevated systemic venous pressures and especially elevated superiorcaval venous pressures are present (constrictive pericarditis, 4 long-standing congestive heartfailure, 5 baffle obstructionafter the Mustard operation for transposition of the great arteries 6; after the Glenn operation 7 and Fontan operation 1,2,8-11).The exact pathophysiology of PLE is unknown. It is hypothesized that chronicvenous congestion with elevated superior vena caval pressures leads to disturbedlymph drainage through the thoracic duct. 10Moreover, the increased inferior vena caval and portal vein pressures lead toincreased intestinal congestion and lymph production. Proteins and lymphocytesleak from the dilated lymphatics. This functional disorder may progress tointestinal lymphangiectasia. Maybe an inflammatory response caused by chroniccongestion or leakage of proteins from the enteric lymphatic system is alsoinvolved in the pathogenetic process. The beneficial therapeutic effect ofsteroids observed in some patients could be explained by suppression of thisinflammatory reaction. 12,14 No consistent effect ofsteroids can, however, be found, which indicates that additional mechanisms areprobably involved. The actual molecular basis for PLE remains unknown. It hasbeen shown that sulphated glycosaminoglycans may be important in regulatingvascular and renal albumin loss. In a recent paper three children with PLE wereshown to have an enteric heparin sulfate deficiency. 15 In those three infants a completeabsence of enterocyte heparin sulphate was found by histochemistry. The recentfinding that high molecular-weight heparin improves PLE after the Fontanoperation 19 could beconsistent with this. Chronic congestion could interfere with the production anddistribution of heparin sulfate leading to intestinal albumin loss. Anotherpossibility is a disturbance in endothelial function in the lymphatic system.The exact pathophysiologic mechanism presently remains obscure and requiresfurther study. Especially the impact of chronically elevated systemic venouspressures on intestinal lymphatic functioning should further be explored inexperimental studies.
Because systemic venous pressure is the driving force for the pulmonarycirculation in a Fontan circulation, chronic venous congestion is alwayspresent. It is not known why intestinal protein loss therefore occurs in a smallpercentage of patients. Our data suggest that not only hemodynamic factors areinvolved in the process of PLE but that the complication may result from acombination of unfavorable factors. Increased systemic venous pressures arecertainly not the only underlying factor because, in 23% of the patientswith PLE in whom right atrial pressures were obtained, mean right atrialpressure was below 15 mm Hg (a "good" value for a patient after aFontan operation). Even in those patients where an unfavorable hemodynamicfactor was detected on hemodynamic evaluation (e.g., stenosis on the conduit),improvement of the hemodynamics (e.g., removal of the stenosis) did not alwaysresult in improvement of PLE. These are patients in whom the Fontan circulationis not tolerated without any good explanation. It may be that the intestinallymphatic system operates at the limit of its capacities in all patients afterthe Fontan operation but only fails in a small percentage of patients.Structural and physiologic differences in lymphatic function could explain theoccurrence of PLE but these are extremely difficult to study. Anotherpossibility is that the standard hemodynamic evaluation methods in thesepatients fail to uncover hemodynamic disturbances causing PLE. Especiallythe evaluation of right atrial pressures and pulmonary vascular resistance inpatients who have been aggressively treated with diuretics before the cardiaccatheterization is questionable. Other factors such as pulmonary vascularreactivity may not be adequately evaluated during catheterization.
PLE treatment and outcome.
Different treatment options have been tried in patients in whom PLEdevelops after the Fontan operation, but only a few successful approaches havebeen published. 12,14,16,20
Our patients experienced a wide variety of different treatmentstrategies. To organize the different treatment options, we categorized ourpatients into three different classes: purely medical treatment, surgicaltreatment, and treatment by interventional catheterization. This division issomewhat arbitrary, because all patients who underwent surgical treatment werealso receiving medication. Moreover the "surgery" group is a veryheterogeneous group going from Fontan redo surgery to Fontan takedown and hearttransplantation. No relation between strategy and outcome of PLE was observed.
Medical treatment was aimed at some symptomatic relief (diuretics,intermittent infusion of albumin) to improve hemodynamics (digitalis,ventricular afterload reduction), to influence an unknown mechanism (steroids),or to prevent further complications (antiarrhythmics, anticoagulation). Resultswere disappointing, and this probably reflects, in most cases, the naturalhistory of PLE after the Fontan operation.
Surgery in patients with PLE was associated with a very high mortalityrate. Especially in patients with ventricular dysfunction,atrioventricular-valve insufficiency, or increased pulmonary vascularresistance, mortality rate is extremely high. Poor hemodynamics and chronichypoproteinemia with resulting catabolism probably compromised the outcome ofmajor cardiac surgery. In this context it should be noted that a long timeinterval between the diagnosis of PLE and the time of surgery was observed inthe present study (mean: 1.8 years; range: 0.01 to 8.0 years). Reluctance of thecardiologist or the surgeon to perform major surgery in a "mildly"symptomatic patient probably caused a major delay in many cases. Of thosepatients with PLE who survived surgery, only 50% were cured of PLE aftersurgery. This could be due to insignificant improvement in hemodynamics aftersurgery. So, for instance, converting one type of Fontan circulation intoanother type of circuit may not result in a hemodynamic amelioration that issufficient to cure PLE. In vitro hemodynamic studies and computer simulationshave shown that some types of Fontan circuits are hemodynamically more favorablethan others. 20,23 It seems that if a patient isnot tolerating one type of circuit, the small differences in energy loss betweenthe different surgical connections may not really be clinically relevant. Alsothe changes in the enteric system may become irreversible and not responsiveeven to normalization of the hemodynamics. In one cardiac transplant patient,PLE resolved completely in the immediate postoperative period but recurred a fewyears after surgery despite good hemodynamics. Thus the effect oftransplantation on PLE after Fontan surgery also requires further study.
Because of the high risk associated with surgery, interventionalprocedures to optimize the Fontan circuit are a potential alternative. Differenttypes of interventions were performed. The balloon dilations and stentplacements resulted in symptomatic improvement in most patients. However, onlyone patient was actually considered as not having symptomatic protein loss afterthe procedure. Previous reports have already mentioned a beneficial effect offenestrating the intraatrial septum on PLE. 16-18,24 This is confirmed in thepresent study where a fenestration was performed with improved Fontanhemodynamics in five patients and reduced protein loss after the procedure inall patients. It is the only treatment where a consistent positive effect wasobserved. However, in two patients a thromboembolic event occurred withneurologic complications that indicated the absolute requirement foranticoagulation before and after the fenestration. Moreover, there was a hightendency for the percutaneous fenestrations to close spontaneously withdeterioration of PLE symptoms. Newer techniques like stent placement in theintraatrial septum could prevent this, but only limited experience is presentlyavailable. 25
Limitations of the study.
It is evident that this study has the limitations of any multicenterclinical study, such as center differences in patient evaluation. Moreover, thisstudy has the limitations of any retrospective study that comprisesincompleteness of data, making conclusions very difficult. An additionallimitation is the absence of a control group of patients who underwent a Fontanoperation and in whom PLE did not develop. Ideally we should have obtained dataon all 2854 patients who are still in follow-up in the 35 participating centers,but this was practically unfeasible. Maybe the creation of an internationalFontan registry could help for studying more rare complications of thisoperation.
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Conclusion: Therapeutic strategies for PLE after Fontan surgery |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Appendix: List of participating physicians and centers |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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Footnotes |
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References |
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TopAbstractIntroductionMaterials and methodsResultsDiscussionConclusion: Therapeutic...Appendix: List of participating...References |
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