Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum

TM Giglia, KJ Jenkins, A Matitiau, VS Mandell, SP Sanders, JE Mayer, Jr and JE Lockseptum

Influence of right heart size on outcome in pulmonary atresia with intact ventricular

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Influence of Right Heart Size on Outcomein Pulmonary Atresia WithIntact Ventricular Septum

Therese M. Giglia, MD; Kathy J. Jenkins, MD; Abraham Matitiau, MD; Valerie S. Mandell, MD;Stephen P. Sanders, MD; John E. Mayer Jr, MD; James E. Lock, MD

Background. Neonates with pulmonary atresia and intact ventricular septum (PA-IVS) are frequentlyborn with hypoplastic right heart structures that must grow after right ventricular decompression (RVD)procedures for a complete two-ventricle physiology to be achieved. Previous authors have asserted thatneonatal right heart size or morphology will predict right heart growth potential. Since 1983, our bias hasfavored early RVD regardless of initial right heart size. In 1986, we recognized a subset of patients withcoronary artery abnormalities associated with poor outcome after RVD and have defined these patientsas having a right ventricular-dependent coronary circulation (RVDCC).Methods and Results. To assess the influence of right heart size on outcome independent of the presence

of RVDCC, we measured echocardiographic right ventricular (RV) dimensions in 37 neonates withadequate studies presenting between 1983 and 1990. Coronary artery anatomy was adequately assessed byangiography in 36. RV volume and tricuspid valve (TV) diameter were significantly smaller in patientswith RVDCC than in those without. However, there was no statistically significant association between RVvolume or TV diameter and survival among patients with or without RVDCC. Among 29 patients withoutRVDCC, 23 of 24 (95.8%) who achieved RVD are alive compared with 1 of 5 (20%) who did not achieveRVD (P=.001). Twenty-one of the 23 survivors have a complete two-ventricle physiology with low rightatrial pressure. Among 7 patients with RVDCC, 2 patients who underwent RVD died early of leftventricular failure, whereas 4 of 5 who did not undergo RVD have survived single ventricle palliation.

Conclusions. Small right heart size is associated with RVDCC but is not associated with survival inPA-IVS. Patients without RVDCC have improved survival after RVD regardless of neonatal right heartsize. (Circulation. 1993;88[part 11:2248-2256.)KEY WORDs * pulmonary atresia * right ventricle * congenital heart surgery

In the ideal surgical outcome for patients with pulmo-nary atresia with intact ventricular septum (PA-IVS), the right ventricle provides the total pulmo-

nary blood flow at a low filling pressure with no residualright-to-left shunt. At birth, however, patients with PA-IVS have varying degrees of right ventricular (RV) andtricuspid valve (TV) hypoplasia as well as systolic and/ordiastolic dysfunction. Whether the right ventricular out-flow tract (RVOT) should be opened in the neonatalperiod depends to a significant extent on the potential ofthe right heart structures to grow and develop. Somegroups have asserted that initial right heart size1-9 and/ormorphology10-41 will predict whether the ventricle willgrow. These groups have suggested that neonatal RVanatomy can predict subsequent right heart function.

Since 1983, our institutional bias has been to attemptRV decompression in all neonates with PA-IVS regard-less of RV size, TV size, RV anatomy, coronary fistulae,or coronary stenoses. In 1986, this strategy was

Received September 30, 1992; revision accepted July 15, 1993.From the Departments of Cardiology (T.M.G., K.JJ., A.M.,

S.P.S., J.E.L.), Radiology (V.S.M.), and Cardiovascular Surgery(J.E.M.), Children's Hospital, Boston.Correspondence to Therese M. Giglia, MD, Department of

Cardiology, Children's National Medical Center, 111 MichiganAve, NW, Washington, DC 20010.

amended based on coronary artery anatomy. We re-cently published our results defining patients with RV-dependent coronary circulation (RVDCC) as those withright ventricle to coronary artery fistulae plus stenosesof both the right and left coronary systems.42 It is ourcurrent policy to attempt RV decompression in allpatients with PA-IVS who do not have a RVDCCirrespective of right heart size. The purpose of thisinvestigation is to (1) use echocardiographic data toevaluate the relation between neonatal RV or TV sizeand outcome in patients with PA-IVS and (2) assess thesuccess of our current surgical strategy and the re-sources used in following this strategy.

MethodsPatient Selection

All patients with the diagnosis of PA-IVS who pre-sented for initial management to the Children's Hospi-tal, Boston, between January 1983 and January 1990were identified by a search of the computerized cardi-ology data base.

Echocardiographic Measurement of Neonatal RVVolume and TV DiameterTwo-dimensional echocardiograms were performed

using either a Hewlett-Packard 77020A ultrasound sys-

Giglia et al Right Ventricular Size in PA-IVS 2249

tem or an Acuson cardiac imager with a 5-MHz trans-ducer. Using a Dextra D-200 cardiac analysis system(Dextra Medical Inc, Long Beach, Calif), the endocar-dial border of each right ventricle was traced at enddiastole in the subxiphoid long- and short-axis planes.The RV diastolic volume was estimated using a Simp-son's rule algorithm and was indexed for body surfacearea. The maximum TV annulus was measured in theapical four-chamber view.

Patients in whom there was inadequate visualizationof the endocardial border of the right ventricle at enddiastole and/or inadequate visualization of the TV on

neonatal echocardiogram were subsequently excluded.Measurements of RV volume and TV annulus diameterwere obtained solely for the purpose of this study by tworeviewers (T.M.G., A.M.).

Assessment ofRVAnatomyWe assessed RV anatomy using preoperative neona-

tal right ventricular angiograms. Patients were classifiedas having a unipartite, bipartite, or tripartite rightventricle based on the presence or absence of RVinflow, apex and outflow portion, as previously de-scribed by Bull et al.14

Assessment of Coronary AnatomyPreoperative coronary angiograms were reviewed solely

for the purpose of this study by two investigators (T.M.G.,V.S.M.). Coronary artery anatomy was classified accord-ing to a predetermined protocol as (1) fistulae alone, (2)fistulae plus stenoses to a single coronary artery, and (3)fistulae plus stenoses to multiple coronary arteries. In onepatient (patient 5) without neonatal angiograms, coronary

anatomy was assessed by postmortem exam. Patients weredefined as having RV-dependent coronary circulation(RVDCC) when coronary artery stenoses of both theright and left coronary systems were present, as describedpreviously.42

Definition of Successful RV DecompressionSuccessful RVOT reconstruction, or RV decompres-

sion, was defined as achievement of RV pressure thatwas less than one half of systemic pressure at or before2 years of age.

Determination of OutcomeThe hospital medical records for all patients were

reviewed for surgical procedures, cardiac catheteriza-tions, and clinical course, including death. Follow-uptimes are defined as age on January 1, 1992, the date ofinitial medical record review.

Resource ImplicationsTo estimate the health care resources used by our

current surgical strategy, we computed the total numberof surgical procedures, cardiac catheterizations, echocar-diograms, and in-hospital weeks used per survivor for (1)all patients without RVDCC in whom RV decompres-sion was attempted and (2) all patients with RVDCCwho were managed with a single ventricle palliationwithout RV decompression.

Statistical AnalysisANOVA was used to compare continuous variables

between survivors and nonsurvivors in the group as a

TABLE 1. Patient Characteristics (n=37)

Indexed RV volume, mL/m2(mean±SD)

TV annulus diameter, mm(mean±SD)

RV anatomy

Tripartite

Bipartite

Coronary artery anatomy

Normal

Abnormal

Fistulae only

Fistulae+stenosis of 1 coronaryartery

Fistulae+stenosis of right and leftcoronaries

Follow-up time, y (mean±SD, range)

10.1±5.8

10.4+3.6

31/33 (93.9%)

2/33 (6.1%)

19/36 (52.8%)

17/36 (47.2%)

4/17 (23.5%)

6/17 (35.3%)

7/17 (41.2%)

5.1±2.1 (1.8-8.9)

RV indicates right ventricle and TV, tricuspid valve.

whole and in those patients without RVDCC. Thenonparametric rank-sum test was used to comparecontinuous variables between survivors and nonsurvi-vors in patients with RVDCC. Comparisons of dichot-omous variables were made using a Fisher's exact test.The relation between indexed RV volume and TVdiameter was assessed using a Pearson linear correla-tion coefficient. Ordinal logistic regression was used toadjust for the influence of RV volume on the relationbetween RV decompression and survival in patientswithout RVDCC. A P value of .05 was consideredsufficient evidence to reject the null hypothesis of noassociation between variables.

ResultsPatient CharacteristicsOf 46 patients with PA-IVS identified by computer

search, 9 (19.6%) were subsequently excluded fromanalysis because of inadequate echocardiographic stud-ies for RV volume and TV diameter measurement. Thecharacteristics of the remaining 37 patients are summa-rized in Tables 1 and 2. The characteristics of the 9patients excluded from the data analysis are summa-rized in Table 3.

Lack of Relation Between RV Volume or TVDiameter and Survival

There were 9 deaths in the total group of 37, for anoverall mortality of 24.3%. For the group as a whole, thedifferences in indexed RV volume and TV annulusdiameter between the survivors and nonsurvivors werenot statistically significant (10.9±5.7 mL/m2, mean+ SD,vs 7.7+5.8 mL/m2 and 11.0±3.8 mm vs 8.5±2.0 mm;P=NS).

Indexed RV volumes for the 37 patients ranged from1.0 to 22.1 mL/m2, and TV diameters ranged from 4.3 to20.0 mm. In our laboratory, estimated normal values forRV volumes range from 30 to 50 mL/m2; similarly,normal newborn tricuspid valve dimensions range from9 to 17 mm. Linear regression analysis revealed a

2250 Circulation Vol 88, No 5, Part 1 November 1993

TABLE 2. Neonates With Echocardiograms Adequate for Analysis

Initial SurgicalProcedure

PV*/LMBTS

RVOTP/RMBTS

PV*/LMBTS

RVOTP/RBTS

PV*/RMBTS

RVOTP/RMBTS

PV*/RMBTS

RVOTP/RMBTS

RVOTP*/RMBTS

RVOTP/RMBTS

RMBTS/PA

Plasty/NSD

creation/atrial

septectomy/fistula

ligation

RVOTP*/RBTS

StatusAl

Age at Follow-up(or Death), y

(12 d)

A1 indicates alive with atrial septal defect and shunt closed; A2, alive with atrial septal defect; A3, alive with Glenn; AF, alive withmodified Fontan; AS, alive with shunt; DD, dead after right ventricular decompression; DF, dead with modified Fontan; DS, dead withshunt; PA, pulmonary artery; RMBTS, right (modified) Blalock-Taussig shunt; RVD, right ventricular decompression; RVDCC, rightventricular-dependent coronary circulation; RVOTP, transannular right ventricular outflow tract patch; RW, right ventricular volume;RWI, right ventricular volume indexed; PV, pulmonary valvotomy; and TVD, TV annulus diameter.

*Incomplete RVD.tinadequate angiogram.

Patient1

RWI, mL/m24.3

TVD, mm

Age atSuccessful

TABLE 3. Neonates With Echocardiograms Inadequate for Analysis

Age at InitialSuccessful Surgical Age at Follow-up

Patient RWI, mL/m2 TVD, mm RVDCC RVD Procedure Status (or Death), y

1 ... ... ... 2 d RVOTP/F-PDA A1 9.0

2 ... ... ... ... PV*/LMBTS DS (2.3)

3 ... .. ... 2d PV A1 6.8

4 ... ... ... 12mo PV* A1 8.0

5 ... ... ... ... RBTS DS 1.0

6 ... ... + ... t DC (0.5)

7 ... ... ... 2 d RVOTP/F-PDA DD (14 d)

8 ... ... ... ... RVOTP/F-PDA AF 9.7

9 ... ... ... 4d PV/RBTS A1 9.9

A1 indicates alive with atrial septal defect and shunt closed; AF, alive with modified Fontan; AS, alive with shunt; d, dead; DD, deadafter right ventricular decompression; DC, dead with collaterals; DF, dead with modified Fontan; DS, dead with shunt; F-PDA, formalininfiltration of patent ductus arteriosus; PA, pulmonary artery; RMBTS, right (modified) Blalock-Taussig shunt; RVD, RV decompression;RVDCC, right ventricular-dependent coronary circulation; RVOTP, transannular RV outflow tract patch; RW, right ventricular volume;RWI, right ventricular volume indexed; PV, pulmonary valvotomy; and TVD, TV annulus diameter.

*Incomplete RVD.tPulmonary blood supply via aortopulmonary collaterals (no surgery).

significant correlation between indexed RV volume andTV annulus diameter (r=.73).

RVDCC and RV SizeThirty-six of the 37 patients (97.3%) had RV angio-

grams of sufficient quality to determine coronary arteryanatomy. Seven of these 36 patients (19.4%) were

classified as having RVDCC, whereas 29 patients(80.6%) did not have RVDCC. Both the indexed RVvolume (3.8±1.7 mL/m2 vs 11.9±5.0 mL/m2, P=.001)and the TV annulus diameter (6.9± 1.6 mm vs 11.5 ±3.2mm, P=.013) were significantly smaller in the patientswith RVDCC in comparison to those without RVDCC.These data are shown in Fig 1.The single patient (patient 13) whose coronary artery

anatomy could not be determined had an indexed RVvolume of 1.5 mL/m2 and a TV diameter of 7.0 mm. Thispatient underwent initial placement of a right modified

COE 15m E

0 5 10 15

RV Volume (mi/m2)20

FIG 1. Plot of tricuspid valve (TV) annulus diameter vs

indexed right ventricular (RV) volume. No association wasfound between RV volume or TV annulus diameter andsurvival. A significant association was found betweenboth indexed RV volume and TV diameter and the pres-ence of RV-dependent coronary circulation (RVDCC).

Blalock-Taussig shunt (BTS) without RV decompres-sion and died at 25 days of life.

Outcome of Patients With RVDCCOf the 7 patients with RVDCC, 2 underwent neonatal

RV decompression before 1986 (see Fig 2). Both of thesepatients died within 18 hours of surgery with clinical andechocardiographic evidence for left ventricular dysfunc-tion (patients 5 and 6). Of the 5 remaining patients, 4(80%) are alive with a single ventricle palliation (1 Fontan,3 BTS) at a follow-up of 5.5±3.1 years (patients 8, 9, 10,11). One patient (20%) died late, 1.3 years after place-ment of a right BTS (patient 14). There was no statisticallysignificant difference in indexed RV volume (3.1± 1.0mLim2 vs 4.8±2.0 mL/m2) or TV annulus diameter(5.5±0.5 mm vs 7.1±0.7 mm) between survivors andnonsurvivors among patients with RVDCC (P=NS).

Outcome of Patients Without RVDCCOf the 29 patients without RVDCC, RV decompres-

sion was attempted in 26 (89.7%) and was successful in24 (82.8%). Twenty-three of the 26 (89%) who hadattempted RV decompression are alive in comparisonto 1 of 3 (33.3%) who did not undergo decompression(P=.07). Twenty-three of 24 (95.8%) who achievedsuccessful RV decompression are alive in comparison to1 of 5 (20.0%) who were not successfuly decompressed(P=.001). Thus, achievement of a successful RV decom-pression was highly related to survival among patientswithout RVDCC. See Fig 2.Of the 5 patients who did not achieve a successful RV

decompression, 3 did not undergo RV decompressionbecause of the choice of their individual physician, and2 had an unsuccessful operative decompression. Oneinfant in the "physician's choice" group had severe

tricuspid incompetence producing RV pressures lessthan 50% of systemic pressures despite pulmonaryatresia. This patient died early from right heart failure(patient 33). A second patient who did not undergo RVdecompression based on physician's choice subse-

A AMiv. ADa Ao MvDCC

A AAA A A

A A aA AA A

* A AA ~~A A

. i@*h

FIG 2. Flow chart of outcomes of36 patients with (+) and without (-)right ventricular-dependent coro-nary circulations (RVDCC). BTS in-dicates Blalock-Taussig shunt; RVD,right ventricular decompression;and Vent, ventricle.

quently died after a modified Fontan operation (patient12). Of the 2 patients with unsuccessful operativedecompression, both had a single surgical procedurewith no further attempts at RV decompression. Onechild was found to have intracavity RV muscle bundlescausing persistent severe RV hypertension 1 year afterplacement of an RV outflow tract patch; the patientdied suddenly awaiting surgical relief (patient 30). Asecond patient underwent an initial unsuccessful de-compression procedure and died of fungal sepsis 12days after surgery (patient 32). Of this group who neverachieved successful RV decompression, only one pa-tient is alive at a follow-up of 7.0 years. This patient didnot undergo an attempt at RV decompression based onphysician's choice and has subsequently undergone amodified Fontan procedure (patient 7).Of the 24 patients who achieved a successful RV

decompression, 17 (70.8%) achieved decompressionafter a single surgical procedure, 3 with RV transannu-lar pericardial outflow tract patch reconstruction alone,11 with RV outflow tract patch with BTS, and 3 withpulmonary valvotomy with or without BTS. Seven ofthese 24 (29.2%) required two operations to decom-

press their right ventricles. In 6 of the 7 requiring twooperations to decompress the right ventricle, the initialattempt was a pulmonary valvotomy. Subsequent suc-cessful RV decompression was achieved via RV trans-annular pericardial outflow tract patch in 6 and trans-catheter dilation of the RVOT in 1. Pulmonaryvalvotomy was successful as an initial surgical procedurein only 3 of 9 patients (33%) compared with RVtransannular pericardial outflow tract patch as an initialsuccessful surgical procedure in 14 of 15 (93.3%,P=.004). Overall, successful RV decompression in these24 patients was achieved at a median age of 4 days(range, 2 days to 23 months).Of the 24 patients who achieved a successful RV

decompression, 23 (95.8%) are alive at a mean fol-low-up of 5.0 years (range, 2.2 to 8.6). Twenty-one ofthese 23 (91.3%) have achieved a complete two-ventri-cle repair with closure of both residual atrial septaldefects and systemic to pulmonary artery shunts. An-giograms showing RV growth for one patient in thisgroup are shown in Fig 3. One patient has a residualsmall right-to-left atrial shunt (patient 23), and one hasa right Glenn anastomosis (patient 4). Eventual com-

FIG 3. Right ventriculogram obtained on first day of life shows tiny right ventricle in frontal (top left) and lateral (bottomleft) projections. There is severe right ventricular hypoplasia and a single fistula to the mid left anterior descendingcoronary artery (LAD). The LAD is irregular with proximal and mid stenoses. The right coronary, which is unobstructed,fills from collaterals to the LAD. Right ventriculogram in frontal (top right) and lateral (bottom right) projections taken afterright ventricular decompression, shunt closure, and closure of atrial septal defect with clamshell device showsremarkable right ventricular growth sufficient to carry a full cardiac output.

plete two-ventricle repair is anticipated in both of thesepatients. Postoperative catheterization data were avail-able in 20 of these 23 patients (87.0%). The medianright atrial pressure was 5 mm Hg, with a range of 1 to14 mm Hg. Five of the 23 patients required additionalinterventional procedures including right pulmonaryartery dilation (n=6) and TV dilation with or withouttricuspid valvotomy (n=2).Only 1 of the 24 patients without RVDCC who

underwent a successful RV decompression has died.This patient had a coronary artery fistula with a singleright coronary artery stenosis and underwent RV de-compression with placement of a RV outflow tract patchand a BTS at 4 days of age. His course was complicatedby prematurity (32 weeks, 2000 g), a preoperativeintraventricular hemorrhage, and BTS revision withsubsequent thrombosis. He died 4 days after surgerywith signs of left ventricular dysfunction (patient 31).

Lack of Relation ofRV Volume or TV Diameter toOutcome Among Patients Without RVDCC

Patients without RVDCC who did not undergo RVdecompression tended to have smaller indexed RVvolumes than patients who underwent RV decompres-sion (9.4+6.4 mL/m2 vs 12.4±4.8 mL/m2, P=.23). How-

ever, there was no statistically significant differencebetween indexed RV volume (12.2±5.0 mL/m2 vs10.7±6.0 mL/m2) or TV annulus diameter (11.7±3.5mm vs 10.0±1.8 mm) among survivors and nonsurvivorsby univariate analysis. In a regression model adjustingfor indexed RV volume, achievement of successful RVdecompression remained highly associated with survival(P=.006). Kaplan-Meier survivor curves for patientswithout RVDCC who did and did not achieve successfulRV decompression are shown in Fig 4.

Resource ImplicationsThese data indicate that optimum strategy for patients

without RVDCC is decompression, whereas optimumstrategy for patients with RVDCC is probably singleventricle palliation. Thus, 31 of 36 patients (86.1%) weretreated according to an optimum surgical strategy. Twen-ty-six patients without RVDCC underwent RV decom-pression procedures with 23 survivors. These 26 patientsused a total of 45 surgical procedures, 67 catheterizations,151 echocardiograms, and 111 in-hospital weeks. There-fore, the strategy of RV decompression for patients with-out RVDCC consumed, on average, 2.0 operations, 2.9catheterizations, 6.6 echocardiograms, and 4.8 in-hospitalweeks per survivor. Similarly, 5 patients with RVDCC did

sM 0.8s

a<,z 0.6 A

°0.2-Without RV Decompression

0.0 . . . .0 1 2 3 4 5 6 7

Follow-up Time (yrs)Log Rank p = 0.0001

FIG 4. Kaplan-Meier survival curves for patients withoutright ventricular-dependent coronary circulation who did(n=24) and did not (n=5) achieve right ventricular de-compression. RVDCC indicates right ventricular-depen-dent coronary circulation and RV, right ventricle.

not undergo RV decompression. Four patients had initialplacement of systemic to pulmonary artery shunts, and 1patient had an unsuccessful pulmonary valvotomy with nosubsequent decompression attempts. There were 4 survi-vors. These 5 patients used 13 surgeries, 20 catheteriza-tions, 27 echocardiograms, and 25 in-hospital weeks.Therefore, following a strategy of not pursuing RV de-compression in patients with RVDCC consumed, on av-

erage, 3.3 operations, 5.0 catheterizations, 6.8 echocardio-grams, and 6.4 in-hospital weeks per survivor. There was

no difference in follow-up time between the two groups. Incontrast, only a single patient (patient 7) survived subop-timal therapy (ie, RV decompression with RVDCC or

initial isolated shunt procedure without RVDCC). Thus,these suboptimal strategies used 17 surgeries, 20 catheter-izations, 34 echocardiograms, and 32 in-hospital weeks per

survivor (Fig 5).

0 20..0

Surgical Catheteri- Echo- Weeks inProcedures zations cardiograms Hospital

FIG 5. Bar graph of resource utilization: total number ofprocedures and weeks in hospital per survivor, usingoptimal (solid and hatched bars) vs suboptimal (openbars) strategy. Optimal strategy is right ventricular (RV)decompression in patients without RV-dependent coro-

nary circulation (RVDCC) and single ventricle palliation inpatients with RVDCC. Suboptimal strategy is RV decom-pression in patients with RVDCC or initial isolated shuntin patients without RVDCC.

DiscussionPrevious authors have suggested that various pre-

operative measures of RV or TV hypoplasia mightpredict which patients with PA-IVS had right ventriclesthat were capable of growth to normal size and thuswhich patients would benefit from early RV decompres-sion.2-10,12-21,23,26-34,36-38,40,41,45-49 Most studies have deter-mined that patients with significant RV and TV hypopla-sia did poorly and have concluded that tiny right heartstructures were incapable of normal growth with pre-

served long-term RV function,2-7'9-1'13-17'19-22'24-36'38-41'43although a few studies have documented normal growth ofmarkedly hypoplastic right ventricles after decompressionin some patients.8,18,233744-48

In contrast, this study shows no statistically significantassociation between indexed RV volume or TV diameterand survival among patients with PA-IVS. This studydoes, however, show a significant association betweenboth indexed RV volume and TV diameter and thepresence of coronary artery fistulae with stenoses tomultiple coronary arteries (RVDCC), which we havepreviously shown contributes to mortality in thesepatients.42

In addition, patients who did not have RV-dependentcoronary circulation but achieved successful RV decom-pression had a markedly improved survival (95.8%)compared with similar patients who did not achievesuccessful decompression (20.0%). Moreover, despitethe presence of patients with very hypoplastic rightheart structures (minimum indexed RV volume, 4.3mL/m2; minimum TV diameter, 4.3 mm) in this group,

most patients (21 of 23, 91.3%) currently have evidencefor excellent RV function at a mean follow-up of 5.0years, as evidenced by their having achieved a completetwo-ventricle repair, at low right atrial pressure. Al-though 2 patients had evidence of TV stenosis afterRVD, this did not preclude eventual attainment of a

complete two-ventricle repair.Also, among the small group of patients with RV-

dependent coronary circulation in this series, patientswho underwent RV decompression died soon aftersurgery with evidence for severe left ventricular dys-function, whereas 4 of 5 patients who did not undergoRV decompression are alive with a single ventriclepalliation.

Study Limitations

Certain limitations must be recognized in this study.First, it is a retrospective design. Second, pulmonaryatresia with intact ventricular septum is a rare disease,and the number of patients with markedly hypoplasticright ventricles is limited. In addition, since the strategyof our institution is to decompress all right ventricles inthis disease except in the presence of a RVDCC, thenumber of patients in the nondecompressed group isobviously small, thereby limiting the statistical power ofour analyses. Finally, 9 patients of our total patientpopulation were excluded from analysis secondary toinadequate neonatal echocardiograms for assessment ofright ventricle size. Since the clinical course of these 9patients was similar to the clinical course in the 37patients analyzed, the exclusion of these patients fromthe analysis does not seem to alter the inferences.

* No RVDCC* RVDCC[OSuboptimal Strategy

Despite these limitations in sample size and selection,several conclusions appear warranted.

ConclusionsThese results support our current hypothesis that

coronary artery anatomy and not RV or TV hypoplasiapredicts which patients with PA-IVS will do well afterearly RV decompression. Earlier authors may haveconcluded that outcome after RV decompression inpatients with PA-IVS could be predicted by RV and TVsize because of the frequent presence of coronary arteryfistulae with stenoses of multiple coronary arteries inpatients with markedly hypoplastic right hearts.These results also support our current strategy for

aggressive angiographic determination of coronary ar-

tery anatomy in neonates with PA-IVS and early RVdecompression using RV outflow tract patch for allpatients without RV-dependent coronary circulation,independent of RV or TV size. Assessment of manage-ment strategy for patients with RV-dependent coronary

circulation is limited by the small number of patientswith RVDCC in this series. However, the available datasuggest that single ventricle palliation without RV de-compression may be an appropriate management strat-egy for these patients.

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Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum

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