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J Thorac Cardiovasc Surg 1998;115:1055-1059
© 1998 Mosby, Inc.


SURGERY FOR CONGENITAL HEART DISEASE

Growth of the right ventricle after successful transcatheterpulmonary valvotomy in neonates and infants with pulmonary atresia and intactventricular septum

Caroline Ovaert, MD, Shakeel A. Qureshi, FRCP, Eric Rosenthal, MD, MRCP, Edward J. Baker, MD, FRCP, Michael Tynan, MD, FRCP

From the Department of Paediatric Cardiology, Guy's Hospital,London, United Kingdom.

Received for publication Feb. 3, 1997. Revisions requested March 11, 1997. Revisions received Nov. 11, 1997. Accepted for publication Nov. 11, 1997. Address for reprints: Shakeel Qureshi, MD, Department of PaediatricCardiology, 11th Floor, Guy's Tower, Guy's Hospital, St. ThomasStreet, London SE1 9RT, United Kingdom.


    Abstract
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 
Objectives: Since 1990, transcatheterpulmonary valvotomy has become an alternative to surgical valvotomy in themanagement of neonates and infants with pulmonary atresia and intact ventricularseptum. We sought to determine whether right ventricular growth aftertranscatheter pulmonary valvotomy is commensurate with body growth.
Methods: Laser or radiofrequency-assisted balloonvalvotomy was attempted in 12 neonates and infants with pulmonary atresia andintact ventricular septum. Tricuspid and mitral valve dimensions were measuredretrospectively on the cross-sectional echocardiograms performed before theprocedure and during follow-up. Z-values were used to standardize tricuspidvalve dimensions with body size.
Results:The atretic pulmonary valve was successfully perforated and dilated in nine of12 patients. Five of these nine patients required additional transcatheter orsurgical procedures to augment the pulmonary blood flow. Of six survivors, fiveare regularly followed up with a median follow-up of 60 months (range 37 to 68months). All five have two-ventricle circulation, two of the five patientsrequiring surgical enlargement of the right ventricular outflow tract with orwithout closure of the atrial septal defect. Echocardiographic tricuspid valvedimensions and Z-values before transcatheter valvotomy tended to be smaller inthe patients who died than in the survivors. In the survivors, the absolutetricuspid valve dimensions increased after valvotomy but the Z-values tended todecrease or stayed constant.
Conclusions:Transcatheter valvotomy is a good alternative to surgical valvotomy in patientswith pulmonary atresia and intact ventricular septum. Two-ventricle circulationcan be achieved despite subnormal right ventricular growth. (J ThoracCardiavasc Surg 1998;115:1055-62)


    Introduction
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 
Despite important improvements over the past 30 years in the treatmentstrategies for neonates with pulmonary atresia and intact ventricular septum,mortality and morbidity remain significant.Go Go 1-5 Abnormalities of the coronaryartery circulation together with right ventricular hypoplasia are consideredadverse prognostic factors and may make biventricular repair difficult if notimpossible.Go Go Go 1,5-10The early establishment of right ventricle–pulmonary artery continuity andantegrade flow, either alone or combined with a systemic–pulmonary arteryshunt, may promote the growth of the right ventricle, with the future aim that anormal or near normal right ventricle would support a two-ventricle circulation.Go Go Go 6,11-17 Since 1990, transcatheterpulmonary valvotomy, using laser or radiofrequency heated guide wires followedby balloon dilation, has been introduced in our unit as an alternative tosurgical valvotomy for pulmonary valve atresia and intact ventricular septum.Go Go 18,19In this report, we describe the growth of the right ventricle as assessed byechocardiography after successful pulmonary valvotomy.


    Methods
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 
Patients.
Between April 1990 and April 1995, 15 consecutive neonates and infantswere admitted to our unit with pulmonary valve atresia and intact ventricularseptum. Of these, 12 patients had cardiac catheterization to attempttranscatheter pulmonary valvotomy as a primary intervention, after fullyinformed consent had been obtained. The criteria for attempting cathetervalvotomy included valvular atresia, a coronary circulation not dependent on theright ventricle, and confluent pulmonary arteries. In three patients,transcatheter intervention was not performed because the right ventricle wasconsidered too small in one, the parents opted for a systemic–pulmonaryartery shunt in one, and the coronary artery was right ventricle–dependentin one. Furthermore, those patients with any evidence of flow through thepulmonary valve, according to either color Doppler echocardiography orangiography, were excluded from the study and treated by conventional balloondilation. In all 12 patients, the arterial duct was maintained open before theprocedure with an infusion of prostaglandin E2. The median age atthe time of the procedure was 9 days (range 1 to 74 days), the median weight3.06 kg (range 0.8 kg to 4.15 kg), and the median body surface area (BSA) 0.21 m2(range 0.11 m2 to 0.25 m2). In three of the patients thediagnosis of pulmonary valve atresia had been made prenatally.

Procedure.
Cardiac catheterization was performed with general anesthesia in all thepatients. The femoral artery and vein were cannulated percutaneously in allexcept one patient who required a cutdown onto the left femoral vein andcannulation of the umbilical artery (preterm baby weighing 800 gm). Heparin (30U/kg) was administered once the vascular access was obtained. Usually a 4Fsheath was inserted in the femoral artery and a 5F sheath in the femoral vein.The technique of pulmonary valvotomy has been described in detail previously.Go Go 18,19In the first six patients, laser-assisted valvotomy was performed with an0.018-inch laser guide wire.Go 18In the last five patients, radiofrequency-assisted valvotomy was performed withthe use of an 0.020-inch or an 0.018-inch radiofrequency guide wire.Go 19 In one patient, both laser andradiofrequency wires were used. Once the wire had perforated the atretic valve,subsequent balloon dilation of the valve was performed with balloons ofprogressively increasing size up to the valve anulus diameter. In four patientsthe arterial duct was also dilated with a balloon during the same procedure toimprove the oxygen saturations, and in one patient the dysplastic, poorly mobiletricuspid valve on echocardiography was also dilated during the same procedure.

Postcatheter management.
After completion of the procedure, the patients were kept in theintensive care unit and progressively weaned from ventilation and prostaglandininfusion when the oxygen saturations were satisfactory. In the patients whoremained severely hypoxic immediately after the procedure despite prostaglandininfusion (oxygen saturations below 65% or desaturations with acidosis) orin the patients who remained prostaglandin-dependent up to 1 month after theprocedure, further interventions were considered, such as balloon dilation ofthe arterial duct, stenting of the arterial duct, repeat balloon dilation of theright ventricular outflow tract, or insertion of a modified Blalock-Taussigshunt. These patients are referred to in the text as "remainingprostaglandin-dependent." In patients in whom the catheter valvotomyprocedure had failed, surgical pulmonary valvotomy or modified Blalock-Taussigshunt insertion was considered.

Follow-up.
After discharge from the hospital, the patients were reviewed regularly,usually with an assessment of oxygen saturation and cross-sectionalechocardiography. Further cardiac catheterizations with or without interventionand surgical interventions were performed when clinically indicated.

Assessment of right ventricular size and growth.
All the cross-sectional echocardiograms were retrospectively reviewed byone person (C.O.). The largest diameter of the tricuspid valve and the mitralvalve anulus were measured in diastole from the apical four-chamber view. Thetricuspid valve dimensions were expressed as Z-values using the nomograms basedon the published echocardiographic data.Go Go 20,21 The transformation of adimension into a Z-value is based on the following equationGo 21:


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.
 
The ratio of the tricuspid valve to BSA was used as an alternative forrelating the tricuspid valve dimensions to growth. The ratio of the tricuspidvalve to the mitral valve was also calculated.

Data analysis.
The 12 patients were divided into three groups according to theiroutcome. Group 1 consisted of patients who survived after successfultranscatheter valvotomy (n = 6); group 2comprised patients who died after successful transcatheter valvotomy (n = 3); and group 3 was composed of patients who diedafter unsuccessful transcatheter valvotomy (n =3). The results are expressed as medians with ranges. To compare initial datawith follow-up data, we used a paired Student's ttest.


    Results
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 
Short-term results.
Successful perforation of the pulmonary valve and subsequent balloondilation was achieved in nine (75%) of the 12 patients (Fig. 1). In three (25%) of the 12patients, catheter valvotomy was unsuccessful. In the first patient, the laserwire passed from the infundibulum into the pericardium causing cardiactamponade, which responded to needle aspiration. The child underwent an urgentoperation but died in the operating room of coronary artery air embolism.Coexistent infundibular atresia was noted. The second patient was a preterm babyweighing 800 gm, in whom venous and arterial access required the use of theumbilical artery and cutdown of the femoral vein. The pulmonary artery wasperforated during the procedure and the child died of cardiac tamponade in thecatheterization laboratory. Autopsy also showed coexisting infundibular atresia.In the last patient, both laser and radiofrequency wires were positioned in theoutflow tract sequentially, but a stable position could not be obtained. Theprocedure was therefore abandoned and the patient referred for surgicalvalvotomy 2 days later. After the valvotomy, the child remained deeply cyanotic.Surgical closure of the atrial septal defect was performed 24 hours later butthe child died soon after returning to the intensive care unit.



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Fig. 1. Early results in the 12neonates and infants with pulmonary atresia and intact ventricular septum inwhom transcatheter pulmonary valvotomy was attempted as the initial treatment.RF, Radiofrequency; PGE2,prostaglandin E2, PDA, patentarterial duct; BT shunt, modifiedBlalock-Taussig shunt.

 
Complications.
In four of the nine patients in whom the pulmonary valve was successfullydilated, the main pulmonary artery was perforated by the laser or radiofrequencyguide wire, resulting in cardiac tamponade in three and no hemodynamic effectsin one. Surgical repair of the pulmonary artery perforation was required in onlyone of these patients; in the other two, cardiac tamponade was relieved bypercutaneous pericardiocentesis before completion of the procedure. The patientin whom the pulmonary artery was perforated without cardiac tamponade also had adissection of the femoral artery caused by the catheter, resulting in femoralartery occlusion. Heparin was not used after the procedure because of thepulmonary artery perforation, and the foot pulses returned to normal over thenext 3 days.

Early outcome.
Fig. 1Go summarizes the outcome ofthe 12 patients after cardiac catheterization. Of the nine patients in whom thepulmonary valve had been successfully opened, two were successfully weaned fromprostaglandin infusion 7 and 19 days after the valvotomy. These patients had thehighest initial tricuspid Z-values (Z-value = –0.4 for both). Theother seven patients remained prostaglandin-dependent up to 1 month after theprocedure. In three of these, additional transcatheter procedures were performedon the arterial duct. These included dilation of the arterial duct with aballoon 4 and 7 days after the catheter valvotomy in one patient and, in thesecond, 2 days after the valvotomy. In both of these patients, the arterialoxygen saturation improved and the prostaglandin infusion could be stopped.However, in the second patient, because of recurrent hypoxia, the duct wasredilated 35 days after the catheter valvotomy when it was combined with balloondilation of the right ventricular outflow tract and the tricuspid valve. In thethird patient, a stent was implanted in the arterial duct in addition to repeatballoon dilation of the pulmonary valve 36 days after the initial cathetervalvotomy, after which the prostaglandin infusion was stopped. A further twopatients had modified Blalock-Taussig shunts inserted 5 and 9 days,respectively, after the valvotomy. One of these two continued to have persistentlow arterial oxygen saturation despite the shunt and died of multiorgan failureand sepsis 18 days after the catheter valvotomy. In the other patient, the shuntoperation was performed before the patient returned abroad. The two remainingpatients, who were prostaglandin-dependent, had severe sepsis and multiorganfailure and died 6 and 33 days after the catheter valvotomy. The cross-sectionalechocardiograms performed before death showed unobstructed antegrade flow acrossthe pulmonary valve, significant pulmonary and tricuspid regurgitation, and aright-to-left shunt at the atrial level.

Late outcome.
Of the six surviving patients, one is being followed up abroad and isclinically well 8 months after catheter valvotomy and modified Blalock-Taussigshunt insertion, but no detailed echocardiographic data are available. Themedian follow-up for the other five is 60 months (range 37 to 68 months). Sincedischarge from the hospital, two of the five patients have required no furthertranscatheter or surgical intervention. In one of these two patients, a stenthad been inserted in the arterial duct 36 days after the catheter valvotomy andbefore discharge. The stent was found to be occluded 2 years after insertionwithout any clinical deterioration or fall in oxygen saturation.

Three other patients have required further balloon dilation of the rightventricular outflow tract 3 to 45 months after the valvotomy; in one of them noother treatment was needed. In one, this was followed by surgical rightventricular outflow tract reconstruction with atrial septal defect closure 67months after the initial catheter valvotomy, and one patient had surgicalclosure of an atrial septal defect 41 months after the catheter valvotomy. Thustwo of the patients have required surgical closure of the atrial septal defect.At the latest follow-up, the systemic arterial oxygen saturation for the fivepatients ranged between 94% and 98%, suggesting no significantright-to-left shunting. The Doppler gradients across the pulmonary valve rangedbetween 4 and 25 mm Hg. Mild pulmonary regurgitation was present in four and nopulmonary regurgitation in one. All of the five patients thus have two-ventriclecirculation with the help of surgery in two and without any surgery in three.

Growth of the right ventricle
Initial tricuspid valve anulus measurements.
Table I shows the clinical and echocardiographic data for the threegroups of patients at the time of the initial cardiac catheterization. The tricuspidvalve of one of the patients in group 3 was 3.5 mm and could not be expressed asa Z-value because no normal echocardiographic data exist for his BSA (0.11 m2).


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Table I. Comparison of the clinical andechocardiographic data at the time of catheter pulmonary valvotomy among thethree groups
 
Growth of the tricuspid valve anulus.
Five of the six survivors have been followed up by serial cross-sectionalechocardiography. In Fig. 2, the serial dimensions of the tricuspid valve,expressed in millimeters (Fig. 2, a), thetricuspid valve Z-values (Fig. 2, b), and thetricuspid/mitral valve ratios (Fig. 2, c) arerelated to BSA. In all patients, the absolute dimensionsof the tricuspid valve increased with growth. However, in three of the patients,the absolute dimensions of the tricuspid valve diverged below 2 standarddeviations from the mean derived from the normal data published by King andassociatesGo 20 (Fig. 2,aGo).This was further confirmed by the Z-values of the tricuspid valve growth, andtherefore the right ventricular growth lagged behind the normal body growth. Theratio of the tricuspid valve to mitral valve dimensions showed a linear increasein four of the five patients, but in only one patient has the ratio reached 1.0(Fig. 2, cGo). Table II compares the initial data,expressed as medians with ranges, in these five patients with those at thelatest follow-up. There is asignificant change in the tricuspid valve anulus, its Z-values, and the ratio oftricuspid valve to mitral valve.



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Fig. 2. Growth of the tricuspidvalve as measured on serial cross-sectional echocardiograms in five of sixsurvivors. a, Tricuspid valve (TV) dimensions, expressed in millimeters. Normaldimensions with mean and standard deviations, adapted from King's normalechocardiographic data,Go 20are shown. b, Tricuspid valve Z-values.c, Tricuspid valve/mitral valve ratio (TV/MV).

 

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Table II. Comparison of initial echocardiographic data with data atlatest follow-up examination in five of the six survivors
 
All five patients have two-ventricle circulation, with or withoutadditional surgery, despite smaller than normal tricuspid valve dimensions orZ-values.


    Discussion
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 
Initial treatment strategy.
The management of neonates with pulmonary valve atresia and intactventricular septum remains difficult because of the wide variations of theanatomy, such as the size and the morphology of the right ventricle and possibleassociated abnormalities of the coronary artery circulation. Treatmentstrategies have been influenced by these anatomic variations.Go Go Go Go Go Go Go Go 3-6,8,9,11-13,15,17In those patients with a severely hypoplastic bipartite right ventricle or aright ventricle–dependent coronary artery circulation, a systemic–pulmonaryartery shunt is usually the initial treatment. In such cases, a two-ventriclecirculation in the future is unlikely and a palliative approach such as theFontan operation is preferred. When the coronary artery circulation is notdependent on the high-pressure right ventricle or when the right ventricle is ofa "reasonable" size and has an infundibulum, early opening of theright ventricular outflow tract has been advocated to try to stimulate growth ofthe right ventricle. In such cases, the usual approach is a surgical valvotomyor right ventricular outflow tract patch with or without an additional systemic–pulmonaryartery shunt. The importance of obtaining adequate relief of the rightventricular outflow tract obstruction with low right ventricular pressures hasbeen stressed to promote as much as possible regression of right ventricularhypertrophy and growth of the right ventricular cavity.Go Go 4,17A prospective, multicenter study between 1987 and 1991 reported by Hanley andassociatesGo 5 showed survivalafter surgical valvotomy, with or without a shunt, to be influenced by theinitial tricuspid valve Z-value. For patients with an initial tricuspid valveZ-value of –2 or higher (similar to our population), survival at 6 monthswas at least 86% with shunts and 77% without shunts.

In 1991, transcatheter pulmonary valvotomy was first described by Qureshiand colleaguesGo 18 as analternative to surgical valvotomy to establish antegrade flow in pulmonary valveatresia. Since then, the technical skills for this demanding procedure haveimproved, the indications have become more precise, and more centers have begunto use this approach.Go Go Go 19,22-26The current report includes the entire learning curve with regard to theselection of patients and the technique. This explains the high overallmortality and morbidity when compared with the current surgical results.Go 5 Two of the patients in our seriesin whom the procedure failed were considered in retrospect to be unsuitablecandidates for catheter valvotomy because of the presence of infundibularatresia. In addition, most procedural complications were encountered in ourearly experience. From the experience of other centers performing cathetervalvotomy by these methods, it seems clear that the procedure can be consideredas an alternative to surgical valvotomy, when the patients are carefullyselected.Go Go 24-28 Only those patients withmembranous valvular atresia and a patent infundibulum, in the setting of anon-right ventricle–dependent coronary circulation, should be consideredfor this procedure.

In addition to opening the right ventricular outflow tract, the need fora systemic–pulmonary shunt is an important question. The length of timethat the pulmonary blood flow needs to be augmented by a shunt is not clear,because it is difficult, if not impossible, to judge when the right ventriclehas become capable of supporting the pulmonary circulation on its own withoutthe help of such a shunt. Kirklin and Barratt-BoyesGo 1 estimated the freedom from systemic–pulmonaryartery shunt 1 month after surgical valvotomy or pulmonary atresia to be 48%when the Z-value of the tricuspid valve was –1 and 34% when theZ-value was –2. In our study, 1 month after catheter valvotomy, 33%(2/6) of the surviving patients were not duct-dependent. In our institution, wehave attempted to avoid surgical shunts, when possible, to avoid distortion ofthe pulmonary arteries. Thus, of the four duct-dependent patients, only one hada surgical Blalock-Taussig shunt, whereas the others had catheter approaches tomaintain the arterial duct open. The advantage of such an approach is that theduct can close spontaneously over weeks or months after balloon dilation or evenstent implantation, but by then, it appears as though the right ventricle hasbecome capable of being the sole supply of pulmonary blood flow. Other centersperforming this procedure may have lower thresholds and different indicationsfor proceeding to a surgical or transcatheter shunt.Go 26

Assessment of right ventricular size.
Inasmuch as the right ventricular volume is difficult to assess,tricuspid valve dimensions are generally used in the determination of treatmentstrategies.Go Go 1,5 Different studies have reportedthat a good correlation exists between the dimension of the tricuspid valve andthe size of the right ventricle in pulmonary atresia and intact ventricularseptum.Go Go Go 1,5,11 Z-values have been widely used tostandardize dimensions and require reliable and representative normal values.Go 21 Autopsy,Go 29 echocardiographic,Go 20 and angiographicGo 30 data on tricuspid valves innormal children are available but differ substantially from each other. Severalauthors have used Z-values of tricuspid valves derived by echocardiography orangiography, but based on Rowlatt's autopsy data for their studies.Go Go Go Go 4,5,21,29It is difficult to compare measurements taken by different methods, and there isno clear correlation between autopsy and echocardiographic measurements withgrowth. Serial echocardiographic measurements are hard to interpret if they areexpressed as Z-values based on autopsy data. Because of our serialechocardiographic measurements, we have opted to use echocardiographicmeasurements published by King and associates in calculating the Z-values.Go 20

Growth of the right ventricle.
The potential for growth of the right ventricle after valvotomy remainslargely unknown and seems difficult to predict. Even severely hypoplasticventricles may grow.Go Go 13,15 In addition, it is impossibleto judge the size of the right ventricle that will contribute to a successfultwo-ventricle circulation. Questions arise as to whether the initial size of theright ventricle is really important in the initial treatment strategy andwhether the right ventricle needs to be of normal size to support the pulmonarycirculation on its own. The minimal tricuspid valve Z-value before cathetervalvotomy in our study was –2.1 except in the preterm baby (800 gm), forwhom echocardiographic normal Z-values are not available. Thus the rightventricle was of a "reasonable" size in all the patients. Thetricuspid valve measurements, Z-values, and the ratio of the tricuspid valve toBSA tended to be smaller in groups 2 and 3 than in group 1, suggesting thatthese children had more severe hypoplasia of the right ventricle. This supportsthe suggestion that the initial Z-value of the tricuspid valve, and thus thesize of the right ventricle, is likely to be a determinant of survival aftervalvotomy.Go 5 In five of thesix survivors, although the absolute tricuspid valve dimensions increased duringfollow-up, there was a trend toward deviation away from the normal range inthree of the patients. The Z-values of the tricuspid valve also decreased inmost of the patients. Despite the subnormal size of the right ventricle, five ofsix children have two-ventricle circulation. Therefore it appears as thoughnormal tricuspid valve growth is not mandatory to have a right ventricle capableof sustaining the pulmonary circulation. Hanley and associatesGo 5 analyzed combined data from severalstudies on right ventricular growth after surgical treatment.Go Go Go 6,12,13 This multivariate analysisindicated that the Z-value of the tricuspid valve did not change during thefollow-up period in patients in whom right ventricular–pulmonary arterycontinuity was established as the initial procedure. However, it is difficult tocompare our data with the data of Hanley and coworkers, because their Z-valueswere derived from autopsy measurements.

Limitations of our study.
The small number of patients and the retrospective nature of the studyare major limitations. No surgical control group is available with which tocompare the results of the transcatheter approach. In addition, the patients donot cover the whole spectrum of pulmonary atresia and intact ventricular septum,and only the favorable end of the spectrum is seen with patients with initiallyreasonably good-sized right ventricles. No data are thus available on thepotential for growth of "very small" right ventricles.


    Conclusion
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 
Transcatheter pulmonary valvotomy is an alternative treatment forneonates and infants with pulmonary valve atresia and intact ventricular septum,although proper selection of patients is required. The technique allows growthof the right ventricle and makes it possible for the patients to havetwo-ventricle circulation, even when right ventricular growth is suboptimal.


    References
 Top
 Abstract
 Introduction
 Methods
 Results
 Discussion
 Conclusion
 References
 

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