(Circulation. 1995;91:1775-1781.)
© 1995 American Heart Association, Inc.
Articles |
Right Ventricular Diastolic Function 15 to 35 Years After Repair of Tetralogy of Fallot
Restrictive Physiology Predicts Superior Exercise Performance
From the Royal Brompton Hospital and the National Heart and Lung Institute (M.A.G., A.L.C., S.C., A.N.R.), London, and Chelsea and Westminster Hospital (C.G.H.N.), London.
Correspondence to Dr Andrew N. Redington, Department of Paediatric Cardiology, Royal Brompton Hospital and the National Heart and Lung Institute, Sydney St, London SW3 6NP, England.
 |
Abstract |
|---|
 Top Abstract IntroductionMethods Results Discussion References |
|---|
Background We have shown previously that transient right ventricular restriction after tetralogy of Fallot repair prolongs postoperative course. This is a prospective study of right ventricular diastolic performance in late follow-up patients.
Methods and Results We studied biventricular function, using
Doppler echocardiographic examination. Pulmonary arterial, tricuspid,
and mitral valves and superior vena cava Doppler spectrals were
obtained in 41 patients (mean age, 28.8 years), 15 to 35 years (mean,
23.6) after complete repair of tetralogy of Fallot. Patients were
considered to have evidence of right ventricular restriction if
antegrade diastolic flow was detected in the main pulmonary artery,
coinciding with atrial systole (A wave), throughout the respiratory
cycle. Exercise function was measured by graded treadmill testing with
respiratory mass spectrometry. Three patients were excluded because of
pulmonary outflow obstruction (Doppler gradient >40 mm Hg) or
residual intracardiac shunts. Of the 38 patients, 37 were in sinus
rhythm. Twenty (52.6%) had definite evidence of restriction with an A
wave in the pulmonary artery, augmented during inspiration. In all 20
cases, there was superior vena caval flow reversal with atrial systole.
Both inspiratory and expiratory transtricuspid E-wave deceleration time
was significantly shorter in the restrictive group (P<.003
and P<.03, respectively). All patients had Doppler evidence
of pulmonary regurgitation, but its duration was shorter in the
restrictive group (P<.01) during inspiration.
Cardiothoracic ratio was significantly lower in the restrictive group
(P<.01), suggesting less severe pulmonary regurgitation.
Both restrictive and nonrestrictive groups had reduced exercise
M
O2 compared with healthy age- and
sex-matched control subjects, but those with restrictive physiology had
significantly better maximum oxygen uptake than the nonrestrictive
group (P<.001).
Conclusions Isolated right ventricular restriction late after tetralogy of Fallot repair is common. Although it reflects abnormal hemodynamics, the A wave contributes to forward pulmonary arterial flow and shortens the duration of pulmonary regurgitation. Consequently, there is less cardiomegaly and improved exercise performance in those patients.
Key Words: tetralogy of Fallot • echocardiography • exercise • ventricles
|
Introduction |
|---|
|
TopAbstractIntroductionMethods Results Discussion References |
|---|
Biventricular repair of tetralogy of Fallot has been performed for almost four decades, with a favorable long-term outcome in most patients.1 2 In recent years, interest has centered around the effects of operation on right ventricular systolic function and exercise performance. As operative techniques and myocardial protection have improved, left ventricular function has been shown to be well preserved after early repair.3 Right ventricular diastolic function, particularly in the current era of even earlier correction with its invariably higher transannular patch rate, needs further evaluation. Although normal before,4 5 right ventricular volumes may increase and ejection fraction decrease after repair.6 7 8 We have shown previously that pulmonary regurgitation, which is now recognized as one of the most important factors in the late follow-up of these patients, is directly related to right ventricular dilation.9 Its association with reduced exercise capacity is now established,10 11 and there is increasing evidence to support its adverse role in the development of arrhythmias and possibly sudden death.12 There can be no doubt, therefore, about the emerging importance of right ventricular diastolic events for the long-term outcome of tetralogy of Fallot repair.
We have recently demonstrated transient abnormalities of right ventricular diastolic function in patients undergoing corrective surgery for tetralogy of Fallot, which prolong their postoperative recovery.13 Pulsed Doppler echocardiography demonstrated forward diastolic flow in the pulmonary artery coincident with atrial systole. Our report focuses on laminar antegrade pulmonary arterial flow in late diastole present throughout the respiratory cycle, which may be seen occasionally in normal children,14 although without documented pulmonary valve opening, and was seen in only 6 (all with right ventricular disease) of 750 adults undergoing echocardiography in another study.15 We proposed that this forward diastolic pulmonary arterial flow reflects reduced right ventricular diastolic compliance, suggesting that the right ventricle is unfillable and truly "restrictive" at end diastole, so acting as a passive conduit between right atrium and pulmonary artery during atrial systole.16
In this study of a self-selected cohort of adult survivors of early surgery, we have used the presence of antegrade diastolic pulmonary arterial flow as evidence of right ventricular restriction and examined its relation to other important indices of right ventricular diastolic function and overall functional performance measured using a formal exercise protocol.
|
Methods |
|---|
|
TopAbstractIntroductionMethods Results Discussion References |
|---|
Patients
We have performed a prospective study of 41 patients who underwent total repair of tetralogy of Fallot at the Westminster Hospital by a single surgeon between 1958 and 1979. Thus, follow-up ranges from 35 to a minimum of 15 years. Contact addresses were available for 80 patients who replied to a written questionnaire regarding their functional and social status in 1987. We were unable to contact 31 patients who were lost to follow-up. Six patients declined to participate. Two patients died, 1 from sudden death and the second from right heart failure. The 41 responders to our formal invitation constitute our study population. Their age at the time of the study was from 16 to 46 years (mean, 28.8 years), and their age at complete repair was from 9 months to 18 years (mean, 5.2 years). Surgery was performed under profound hypothermia. Right and left heart bypass was used, with the patient's lungs being used as oxygenators until 1963 (8 patients) and traditional cardiopulmonary bypass thereafter. Access was via a combined right atriotomy and ventriculotomy although in seven cases the transatrial/transpulmonary approach was used. Further anthropometric details including previous surgery and operative techniques are shown in Table 1
.
|
This study took place at the Royal Brompton National Heart and Lung Hospital between August 1993 and March 1994.
Techniques
All patients were studied echocardiographically
using a 2.5- or
2.0-MHz transducer interfaced with a Hewlett-Packard Sonus 1500
ultrasound system. Transthoracic imaging was performed with the patient
in the left lateral decubitus or supine position for the suprasternal
views. Initially routine diagnostic imaging, including color flow
mapping, and continuous-wave Doppler recordings were obtained. An
M-mode recording of the left ventricular cavity in the parasternal
long-axis view was recorded. Finally, pulsed Doppler recordings were
made in each patient (see Table 2): (1) pulmonary
arterial systolic and diastolic Doppler characteristics (the pulsed
Doppler sample was placed at the midpoint between the pulmonary valve
leaflets and bifurcation), (2) superior vena caval Doppler profile (1
to 2 cm proximal to the right atrium), (3) transtricuspid valve
characteristics (at the level of the tips of the valve leaflets), E-
and A-wave velocity/integral, and E-wave deceleration time, and (4)
transmitral valve characteristics (at the level of the valve leaflets),
E- and A-wave velocity/integral, and E-wave deceleration time.
|
Measurements were made with simultaneous ECG, phonocardiogram, and respiratory motion recordings. Spectral recordings were made with minimal filtering on paper at a speed of 100 cm/s. Doppler recordings were analyzed according to the phase of the respiratory cycle, as previously described.15 Briefly, three consecutive inspiratory and three consecutive expiratory cardiac cycles (defined respectively as the first cardiac cycle after the onset of the inspiratory and expiratory deflection on the respirometer) were analyzed by planimetry and the results for each of the indices averaged.
Qualitative assessment of superior vena caval Doppler spectrals were made, with particular emphasis on the presence or absence of retrograde diastolic flow.
A detailed assessment of right ventricular inflow was
performed using
trans–tricuspid valve E- and A-wave peak velocities, duration,
integral, and E-wave deceleration times. Systolic and diastolic
pulmonary artery antegrade Doppler spectrals (peak velocity, duration,
integral) were analyzed, as was the duration of pulmonary
regurgitation. Restrictive physiology (group 1) was defined from the
presence of laminar antegrade diastolic pulmonary arterial flow
throughout the respiratory cycle (Fig 1).13
|
Left ventricular shortening fraction was derived from the M-mode recordings in the usual way. Right and left ventricular long-axis length and atrioventricular ring diameter were measured from two-dimensional echocardiograms in the four-chamber view at end diastole. All patients had a 12-lead surface resting ECG and a chest radiograph in the posterior-anterior projection.17
Thirty-one patients agreed to perform treadmill exercise using a
modified Bruce protocol. Thirty-one normal subjects were used as a
control group. Control subjects were recruited from among hospital
staff and were known not to have any previous history of
cardiopulmonary disease. The control group was matched for age, body
surface area, and predicted peak
O2.18 None of
the patients
or the control subjects were habitual exercisers or on any medication.
Each subject undertook treadmill exercise using a Bruce protocol
modified by the addition of a "stage 0," that is, 3 minutes at 1
mph, with a 5% gradient. Patients breathed room air through a one-way
valve connected to a respiratory mass spectrometer (Innovision).
Ventilation (E), carbon dioxide
production
(CO2), and oxygen
consumption
(O2) were calculated on
line every 10
seconds using an inert gas dilution technique.19 20
Once
ventilation had become stable at rest for at least 2 minutes, patients
were encouraged to exercise to exhaustion. Blood pressure was measured
by sphygmomanometry. Peak blood pressure was obtained when the
exercising patient indicated that he or she needed to stop. In some
patients, a marked respiratory swing was noted. In these patients, the
highest recorded blood pressure was taken. Heart rate was measured from
the ECG. Respiratory rate was calculated as the average of the last
five breaths from the capnograph. Patients and control subjects in whom
the respiratory quotient at maximal exercise failed to exceed 1.0 were
excluded because inadequate effort may have limited their
performance.
Statistical Analysis
Group data are expressed as
mean±SD. Student's t
tests were used to compare normally distributed variables; otherwise, a
Mann-Whitney U test was performed. Superior caval flow
characteristics were compared using a standard 
2
test. Exercise data were analyzed across the three groups (restrictive,
nonrestrictive, and control subjects) with ANOVA and where a
significant difference was found were further investigated with
Student's t test adjusted for multiple comparisons
(2 test). Regression analysis was performed
using the method of least squares. The null hypothesis was rejected
when P<.05.
|
Results |
|---|
|
TopAbstractIntroductionMethods Results Discussion References |
|---|
Of the responders to the original questionnaire in 1987, 83.8% were in New York Heart Association functional class I, with 62.5% in full-time employment. Thirty-nine of the 41 responders to our formal invitation to participate in the current study were in functional class I (95.1%), with 75.6% in full-time employment. These differences were not statistically significant, suggesting that although the patients participating in our study were "self-selected," there was no selection bias from the larger group from 1987. Two patients were excluded because of significant right ventricular outflow obstruction (Doppler gradient >40 mm Hg) and 1 because of an atrial septal defect. Anthropometric details for the 38 patients included in the study with details regarding previous palliation, operative techniques, and need for procedures after repair are shown in Table 1
. All
except
one patient were in sinus rhythm, and all of them had a right bundle
branch block QRS pattern in their surface ECG.
Pulmonary Arterial Flow
There were 20 patients with
restrictive physiology (group 1). In
the remaining 18 patients (group 2), there was either no antegrade
diastolic flow or transient flow restricted to the inspiratory phase of
respiration. The A wave in the pulmonary artery, our marker of right
ventricular restriction, accounted for 3.5% to 25% of the total
forward (systolic and diastolic) pulmonary artery Doppler integral in
group 1. There was a marked augmentation (30% to 58%) of the flow
integral during inspiration. There was no significant difference in
heart rate between the two groups at the time of study (mean,
68±10/min for restrictive patients and 75±12/min for
nonrestrictive
patients, P<.08). The duration of pulmonary regurgitation
was significantly shortened in group 1 (300.2±65.4 versus
442.1±54.1
milliseconds, P<.01) during inspiration.
Transtricuspid Flow
Transtricuspid E- and A-wave velocities,
duration, and integrals
were measured in 37 of 38 of the study group (Table 3).
The E-wave deceleration time was significantly shorter in group 1 in
both inspiration (P<.003) and expiration
(P<.03) (Fig 2). Duration of E and A waves
was shorter in expiration. The E/A velocity and integral ratios were
similar in the two groups, however.
|
|
Superior Vena Cava Flow
Retrograde flow in the superior vena
cava, coincident with atrial
systole (Fig 3), was present in all patients from
the first group and in only 8 of 18 from group 2. Peak velocity of this
flow (mean, 20 cm/s) was significantly higher in group 1
(2 test, P<.02). In addition,
predominant antegrade flow in the superior vena cava occurred during
early diastole in 16 of 20 patients from the restrictive group.
|
Left Ventricular Function
Left ventricular systolic
performance was grossly normal in all
with the left ventricular shortening fraction ranging from 29% to
45%. Transmitral E- and A-wave velocities, duration, E-wave
deceleration, integrals, and E/A-wave ratios were measured, and there
was no evidence of reduced left ventricular compliance.
Heart Size
Cardiothoracic ratio measured from
posterior-anterior chest
radiographs was significantly lower in group 1 (mean, 0.513±0.04
versus 0.555±0.04, P<.01), indicating overall smaller
heart size. Biventricular long-axis length and atrioventricular ring
diameter could be measured reliably in 32 and 27 patients,
respectively. Absolute right ventricular long-axis length and tricuspid
valve diameter were lower in group 1 (P<.003 and
P<.004, respectively). This difference was more marked
(Table 4) when expressed as a ratio of right/left
ventricular long-axis length (P<.001) and tricuspid/mitral
valve diameters (P<.001), suggesting that in this group of
patients, the degree of right ventricular dilation guides the overall
heart size and is clearly less severe in the restrictive group. There
was significant correlation between cardiothoracic ratio from the chest
radiograph and the ratio of right/left ventricular length from the
long-axis recordings (r=.45, P<.001).
|
Exercise Function
There were no adverse effects from any of
the exercise tests. All
except two patients were able to exercise to a point where the
respiratory exchange ratio (RER)
(CO2/O2)
exceeded 1.0, indicating at least near maximal exercise.21
These two patients were excluded from the analysis of the exercise
data. The control subjects were matched for age, sex, and body surface
area. In the Fallot group as a whole, the average peak oxygen
consumption achieved was 35.3±7.5
mL · kg-1 · min-1,
representing 93.6±15.3% of the age-predicted maximum but
significantly lower than control subjects (P<.01). In
addition, exercise time was significantly longer in the control group
(1029±22 versus 888±111 seconds, P<.001). Within the
tetralogy of Fallot group, patients from group 1 achieved a
significantly higher percentage of predicted peak
O2 when compared with group
2
(100.5±14.2 versus 82.6±10.1, P<.001). We chose to
use
percentage of predicted peak
O2 in order
to correct any random variation in the age, body build, and sex
distribution between the two groups of patients. Thus, patients from
group 2 are slightly younger and bigger, so that the importance of
their lower peak O2 is the
greater as a
proportion of what they would be expected to achieve. Pulse and blood
pressure responses to exercise were not different between the two
groups (Table 5). There was no significant difference
also in symptoms at peak exercise between the two groups (6 of 17 short
of breath in group 1 and 3 of 12 in group 2; other patients stopped by
fatigue). Resting pulmonary function (FEV1=94.3±11.9%
in
group 1, 93.5±22.7% in group 2, and FVC=102.3±15.7%,
102.1±27.7%,
respectively) was not different between the two groups.
|
No significant difference was found between cases in which the patient's lungs were used as oxygenators as opposed to conventional cardiopulmonary bypass used after 1963. In addition, it was interesting to note that previous palliations or approach for repair were not significant factors in determining whether restrictive right ventricular physiology developed.
|
Discussion |
|---|
|
TopAbstractIntroductionMethods Results Discussion References |
|---|
Most of the important problems encountered during the late follow-up of patients after repair of tetralogy of Fallot can be related to abnormal right ventricular physiology. Elevated right ventricular end diastolic/end systolic volume indices,9 exercise intolerance,11 and ventricular arrhythmia12 are all influenced by residual pulmonary incompetence. Relatively few data are available concerning other right ventricular diastolic events, however. Our study shows that restrictive right ventricular diastolic dysfunction is present in a significant number of patients late after repair of tetralogy of Fallot. Twenty of 38 patients (52.6%) had Doppler detectable antegrade diastolic flow in the pulmonary artery during atrial systole. This abnormal flow, present throughout the respiratory cycle, reflects decreased right ventricular compliance. The resistance to right ventricular filling exceeds the pulmonary vascular resistance, and as a result, some or all of the transtricuspid atrial systolic flow, demonstrable by Doppler, results in antegrade pulmonary arterial blood flow, not right ventricular filling. Thus, the stiff right ventricle has a limited end diastolic volume and acts as a conduit between right atrium and pulmonary artery. The simultaneous retrograde flow in the superior vena cava in all and rapid deceleration of the early rapid filling velocity, seen in the transtricuspid flow spectral, is consistent with a restrictive right ventricle. The normal E/A-wave ratios in the restrictive group highlight the poor utility of such measurements. As the transtricuspid A-wave flow does not reflect right ventricular filling, this traditionally used index of ventricular filling is inappropriate in these circumstances. Similar Doppler phenomena have been described previously in adults with restrictive myocardial processes involving the right ventricle.22 However, the finding of antegrade diastolic flow has not been consistently documented, presumably because of coincident biventricular restriction with resultant elevation of left atrial and pulmonary arterial diastolic pressures and obviation of the pressure gradient between the right atrium and pulmonary artery. Because of possible confounding influences, we excluded patients with residual intracardiac shunts, significant residual right ventricular outflow obstruction (>40 mm Hg), or any lesion likely to raise the pulmonary arterial diastolic pressure. We also were rigorous in our definition of restrictive physiology. Only patients with laminar antegrade diastolic pulmonary arterial flow throughout the respiratory cycle were included in group 1. There was a clear-cut respiratory variation in these patients, however, with more pronounced pulmonary arterial A-wave flow, shorter duration of pulmonary incompetence, and more rapid E-wave deceleration on inspiration, illustrating the need to consider the effects of respiration whenever right heart hemodynamics are examined.23 Although abnormal, the antegrade diastolic pulmonary arterial flow seen in these patients is important because it contributes to forward flow and shortens the duration of pulmonary regurgitation, in both ways making an important contribution to antegrade pulmonary arterial flow and hence to cardiac output.
There was significantly less cardiomegaly in the restrictive group, presumably reflecting the limited right ventricular end-diastolic volume in these patients.17 The degree of right ventricular dilation after repair of tetralogy of Fallot has been correlated with the amount of residual pulmonary incompetence by Carvalho et al11 and others.7 Whether this lower cardiothoracic ratio is a primary (as a result of intrinsic myocardial disease) or secondary phenomenon (as a result of limitation of pulmonary regurgitation caused by the restrictive physiology itself) or a combination of both is not clear. It is easy to conceptualize the situation whereby an intrinsic myocardial restrictive process (for example, fibrosis) leads to these abnormal filling characteristics, which in turn limits the amount of pulmonary incompetence. This would certainly explain the not-infrequent clinical finding of a normal cardiothoracic ratio and lack of progressive right ventricular dilation seen in some of these patients, even when a transannular patch has been placed.
Endomyocardial fibrosis has been demonstrated in patients after repair of tetralogy of Fallot24 and in animal models of tetralogy of Fallot.25 Our patients underwent correction at a mean age of 5.2 years. The additional adverse effects of less sophisticated methods of cardiopulmonary bypass, ventriculotomy, and inadequate myocardial protection all might be expected to influence the diastolic performance of the right ventricle. The exact relation of these factors to each other remains unknown, but it might be that some degree of right ventricular fibrosis is not disadvantageous for the patient with tetralogy of Fallot. Wessel and coworkers10 showed a significant negative correlation between cardiothoracic ratio and exercise performance in their patients studied after repair of tetralogy of Fallot. They speculated that this relation reflected the adverse effects of residual pulmonary incompetence. This was subsequently confirmed by direct measurement11 and Doppler estimates,26 but no previous study has examined the mechanism for differences in the degree of pulmonary incompetence in these patients. Most of our patients had preservation of the pulmonary valve and annulus, unlike patients operated on in the current era. We cannot speculate how this will affect future cohorts of patients because there are so many factors to be taken into account. Nonetheless, and irrespective of the mechanism, it is clear that the theoretical benefits of increased antegrade flow with reduced retrograde flow (and hence increased cardiac output) are manifest in terms of overall functional performance in our patients. Although most were in functional class I, those with restrictive right ventricular physiology significantly outperformed the group with nonrestrictive ventricles when formally measured by graded exercise testing.
Anything that diminishes the effect of atrial systole may be detrimental. Thus, maintenance of sinus rhythm is particularly important for patients with right ventricular restriction. Although our patients were studied at a mean of 23.6 years after repair, their sinus rhythm was preserved in all except one, who had intermittent junctional rhythm. Despite the limitation of this study, in which patients were assessed at a single point in time, one could reasonably speculate that their hemodynamics had existed for some time at no obvious expense of their effective atrial activity. The very nature of isolated right ventricular restriction may explain this. Unlike left ventricular restriction, the right atrium is able to decompress itself by emptying into the pulmonary artery via the right ventricle. Thus, there is little or no elevation on the mean atrial pressure and no obvious atrial dilation, making atrial dysrhythmias less likely.
Limitations of the Study
Our patient population is not
representative of current
surgical practice. Repair was performed at a mean age of 5.2 years, and
only 1 out of 41 required a transannular patch, the rest having their
pulmonary valve annulus preserved. They are therefore a highly selected
group of survivors of early surgery. There are, however, increasing
numbers of such patients entering adult life, and our data reflect this
population of historical survivors and act as a cohort against which
contemporary results can be compared. While indeed the current trend
toward early primary repair may reduce the risk of endomyocardial
fibrosis, our data suggest that it might in turn predispose to more
significant impact of pulmonary regurgitation, with its deleterious
long-term effects. Careful, repeated evaluation of long-term right
ventricular diastolic performance will be needed in these younger
patients.
Summary
Right ventricular restriction occurs in a significant
proportion
of patients late after complete repair of tetralogy of Fallot,
protecting them from the detrimental effects of severe pulmonary
regurgitation.
|
Acknowledgments |
|---|
We would like to acknowledge the late Charles Drew for his pioneering work in the field of open heart surgery in the United Kingdom. We also would like to thank Dr D. Gibson for his help with the analysis of the data and M. Josen for providing echo lab support.
Received September 26, 1994; accepted October 18, 1994.
|
References |
|---|
|
TopAbstractIntroductionMethods Results Discussion References |
|---|
1. Fuster V, McGoon DC, Kennedy MA, Ritter DG, Kirklin JW. Long-term evaluation (12 to 22 years) of open heart surgery for tetralogy of Fallot. Am J Cardiol. 1980;46:635-642. [Medline] [Order article via Infotrieve]
2.
Murphy JG, Gersh BJ, Mair DD, et al. Long-term outcome in
patients undergoing surgical repair of tetralogy of Fallot. N
Engl J Med. 1993;329:593-599.
3.
Borow KM, Green LH, Castaneda AR, Keane JF. Left ventricular
function after repair of tetralogy of Fallot and its relationship to
age at surgery. Circulation. 1980;61:1150-1158.
4. Lange PE, Onnasch DGW, Bernhard A, Heintzen PH. Left and right ventricular adaption to right ventricular overload before and after surgical repair of tetralogy of Fallot. Am J Cardiol. 1982;50:786-794. [Medline] [Order article via Infotrieve]
5.
Jarmakani JMM, Nakazawa K, Isabel-Jones J, Marks RA. Right
ventricular function in children with tetralogy of Fallot before and
after aortic-to-pulmonary shunt. Circulation. 1976;53:555-561.
6. Oku H, Shirotani H, Sunakawa A, Yokoyama T. Postoperative long-term results in total correction of tetralogy of Fallot: hemodynamics and cardiac function. Ann Thorac Surg. 1986;41:413-418. [Abstract]
7.
Graham TP, Cordell D, Atwood GF, et al. Right ventricular
volume characteristics before and after palliative and reparative
operation in tetralogy of Fallot. Circulation. 1976;54:417-423.
8. Bove EL, Byrum CJ, Thomas FD, et al. The influence of pulmonary insufficiency on ventricular function following repair of tetralogy of Fallot. J Thorac Cardiovasc Surg. 1983;85:691-696. [Abstract]
9.
Redington AN, Oldershaw PJ, Shinebourne EA, Rigby ML. A new
technique for the assessment of pulmonary regurgitation and its
application to the assessment of right ventricular function before and
after repair of tetralogy of Fallot. Br Heart J. 1988;60:57-65.
10. Wessel HU, Cunningham WJ, Paul MH, Bastanier CK, Muster AJ, Idriss FS. Exercise performance in tetralogy of Fallot after intracardiac repair. J Thorac Cardiovasc Surg. 1980;80:582-593. [Abstract]
11.
Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN.
Exercise capacity after complete repair of tetralogy of Fallot:
deleterious effects of residual pulmonary regurgitation. Br Heart
J. 1992;67:470-473.
12. Zakha KG, Horneffer PJ, Rose SA, Nell CA, Manolio TA, Kidd L. Long-term valvular function after total repair of tetralogy of Fallot: relation to ventricular arrhythmias. Circulation. 1988;78:14-19.
13. Cullen S, Shore DF, Redington AN. Characterization of right ventricular diastolic performance after complete repair of tetralogy of Fallot: restrictive physiology predicts slow postoperative recovery. Circulation. 1992;86(suppl I):I-774. Abstract.
14. Gibbs JL, Wilson N, Witsenburg M, Williams GJ, Goldberg SJ. Diastolic forward blood flow in the pulmonary artery detected by Doppler echocardiography. J Am Coll Cardiol. 1985;6:1332-1338.
15.
Wann LS, Weyman AE, Dillon JC, Feigenbaum H. Premature
pulmonary valve opening. Circulation. 1977;55:128-133.
16. Redington AN, Penny D, Rigby ML, Hayes A. Antegrade diastolic pulmonary arterial flow as a marker of right ventricular restriction after complete repair of pulmonary atresia with intact septum and critical pulmonary valve stenosis. Cardiol Young. 1992;2:382-386.
17. Amplatz K, Moller JH, eds. Radiology of Congenital Heart Disease. St Louis, Mo: Mosby-Year Book; 1993:94.
18. Wasserman K, Hansen JE, Sue DY, Whipp PJ. Principles of Exercise Testing and Interpretation. Philadelphia: Lea & Febiger; 1987.
19. Davies N, Denison DM. Measurement of metabolic gas exchange and minute volume by mass spectrometry alone. Respir Physiol. 1979;36:261-267. [Medline] [Order article via Infotrieve]
20.
Lipkin DP, Perrins J, Poole-Wilson PA. Respiratory gas
exchange in the assessment of patients with impaired ventricular
function. Br Heart J. 1985;54:321-328.
21.
Clark AL, Poole-Wilson PA, Coats AJS. Effects of motivation of
the patient on indices of exercise capacity in chronic heart failure.
Br Heart J. 1994;71:162-165.
22. Appleton CP, Hatle LK, Popp RL. Demonstration of restrictive ventricular physiology by Doppler echocardiography. J Am Coll Cardiol. 1988;4:757-768.
23. Riggs TW, Snider AR. Respiratory influence on right and left ventricular diastolic function in normal children. Am J Cardiol. 1989;63:858-861. [Medline] [Order article via Infotrieve]
24. Jones M, Ferrans VJ. Myocardial degeneration in congenital heart disease: comparison of morphologic findings in young and old patients with congenital heart disease associated with muscular obstruction to right ventricular outflow. Am J Cardiol. 1977;39: 1051-1063.
25. Garson A. Ventricular arrhythmias after congenital heart surgery: a canine model. Pediatr Res. 1984;18:1112-1120.[Medline] [Order article via Infotrieve]
26. Rowe SA, Zahka KG, Manolio TA, Horneffer PJ, Kidd L. Lung function and pulmonary regurgitation limit exercise capacity in post-operative tetralogy of Fallot. J Am Coll Cardiol. 1991;17: 461-466.
This article has been cited by other articles:
 |
|
 |
|
  C. A. Warnes Adult Congenital Heart Disease: Importance of the Right Ventricle J. Am. Coll. Cardiol., November 17, 2009; 54(21): 1903 - 1910. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Bedard, K. P. McCarthy, K. Dimopoulos, G. Giannakoulas, M. A. Gatzoulis, and S. Y. Ho Structural abnormalities of the pulmonary trunk in tetralogy of fallot and potential clinical implications: a morphological study. J. Am. Coll. Cardiol., November 10, 2009; 54(20): 1883 - 1890. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. Kogon, C. Plattner, P. Kirshbom, K. Kanter, T. Leong, T. Lyle, S. Jennings, M. McConnell, and W. Book Risk factors for early pulmonary valve replacement after valve disruption in congenital pulmonary stenosis and tetralogy of Fallot J. Thorac. Cardiovasc. Surg., July 1, 2009; 138(1): 103 - 108. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. M. Wald, A. N. Redington, A. Pereira, Y. L. Provost, N. S. Paul, E. N. Oechslin, and C. K. Silversides Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? Eur. Heart J., February 1, 2009; 30(3): 356 - 361. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. Haddad, P. Couture, C. Tousignant, and A. Y. Denault The Right Ventricle in Cardiac Surgery, a Perioperative Perspective: I. Anatomy, Physiology, and Assessment Anesth. Analg., February 1, 2009; 108(2): 407 - 421. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. Haddad, P. Couture, C. Tousignant, and A. Y. Denault The Right Ventricle in Cardiac Surgery, a Perioperative Perspective: II. Pathophysiology, Clinical Importance, and Management Anesth. Analg., February 1, 2009; 108(2): 422 - 433. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 2006 WRITING COMMITTEE MEMBERS, R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, et al. 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons Circulation, October 7, 2008; 118(15): e523 - e661. [Full Text] [PDF]
|
|
|
|
|
|
R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, P. T. O'Gara, et al. 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., September 23, 2008; 52(13): e1 - e142. [Full Text] [PDF]
|
|
|
|
 |
|
 G. F Greil, P. Beerbaum, R. Razavi, and O. Miller Imaging the right ventricle Heart, June 1, 2008; 94(6): 803 - 808. [Full Text] [PDF]
|
|
|
|
|
|
F. Haddad, R. Doyle, D. J. Murphy, and S. A. Hunt Right Ventricular Function in Cardiovascular Disease, Part II: Pathophysiology, Clinical Importance, and Management of Right Ventricular Failure Circulation, April 1, 2008; 117(13): 1717 - 1731. [Full Text] [PDF]
|
|
|
|
|
|
R. R Chaturvedi and A. N Redington Pulmonary regurgitation in congenital heart disease Heart, July 1, 2007; 93(7): 880 - 889. [Full Text] [PDF]
|
|
|
|
 |
|
 A. Sadiq, K. G. Shyamkrishnan, S. Theodore, S. Gopalakrishnan, J. M. Tharakan, and J. Karunakaran Long-Term Functional Assessment After Correction of Tetralogy of Fallot in Adulthood Ann. Thorac. Surg., May 1, 2007; 83(5): 1790 - 1795. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G.-P. Diller, K. Dimopoulos, D. Okonko, A. Uebing, C. S. Broberg, S. Babu-Narayan, S. Bayne, P. A. Poole-Wilson, R. Sutton, D. P. Francis, et al. Heart Rate Response During Exercise Predicts Survival in Adults With Congenital Heart Disease J. Am. Coll. Cardiol., September 19, 2006; 48(6): 1250 - 1256. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, P. T. O'Gara, et al. ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons J. Am. Coll. Cardiol., August 1, 2006; 48(3): e1 - e148. [Full Text] [PDF]
|
|
|
|
|
|
U. K. Chowdhury, S. Sathia, R. Ray, R. Singh, K. K. Pradeep, and P. Venugopal Histopathology of the right ventricular outflow tract and its relationship to clinical outcomes and arrhythmias in patients with tetralogy of Fallot. J. Thorac. Cardiovasc. Surg., August 1, 2006; 132(2): 270 - 277.e4. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. S Rathore, S. K Agrawal, and A. Kapoor Restrictive physiology in tetralogy of fallot: exercise and arrhythmogenesis. Asian Cardiovasc Thorac Ann, August 1, 2006; 14(4): 279 - 283. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Reddy, J. C. Osorio, A. M. Duque, B. D. Kaufman, A. B. Phillips, J. M. Chen, J. Quaegebeur, R. S. Mosca, and S. Mital Failure of Right Ventricular Adaptation in Children With Tetralogy of Fallot Circulation, July 4, 2006; 114(1_suppl): I-37 - I-42. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. Odim, H. Laks, and T. Tung Risk factors for early death and reoperation following biventricular repair of pulmonary atresia with intact ventricular septum. Eur. J. Cardiothorac. Surg., May 1, 2006; 29(5): 659 - 665. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P A Davlouros, K Niwa, G Webb, and M A Gatzoulis The right ventricle in congenital heart disease Heart, April 1, 2006; 92(suppl_1): i27 - i38. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. V. Babu-Narayan, P. J. Kilner, W. Li, J. C. Moon, O. Goktekin, P. A. Davlouros, M. Khan, S. Y. Ho, D. J. Pennell, and M. A. Gatzoulis Ventricular Fibrosis Suggested by Cardiovascular Magnetic Resonance in Adults With Repaired Tetralogy of Fallot and Its Relationship to Adverse Markers of Clinical Outcome Circulation, January 24, 2006; 113(3): 405 - 413. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. van Straten, H. W. Vliegen, H. J. Lamb, S. D. Roes, E. E. van der Wall, M. G. Hazekamp, and A. de Roos Time Course of Diastolic and Systolic Function Improvement After Pulmonary Valve Replacement in Adult Patients With Tetralogy of Fallot J. Am. Coll. Cardiol., October 18, 2005; 46(8): 1559 - 1564. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G.-P. Diller, K. Dimopoulos, D. Okonko, W. Li, S. V. Babu-Narayan, C. S. Broberg, B. Johansson, B. Bouzas, M. J. Mullen, P. A. Poole-Wilson, et al. Exercise Intolerance in Adult Congenital Heart Disease: Comparative Severity, Correlates, and Prognostic Implication Circulation, August 9, 2005; 112(6): 828 - 835. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Bouzas, P. J. Kilner, and M. A. Gatzoulis Pulmonary regurgitation: not a benign lesion Eur. Heart J., March 1, 2005; 26(5): 433 - 439. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. T. Lovell Anaesthetic implications of grown-up congenital heart disease Br. J. Anaesth., July 1, 2004; 93(1): 129 - 139. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. A Helbing Right ventricular function: the comeback of echocardiography? Eur J Echocardiogr, March 1, 2004; 5(2): 99 - 101. [Full Text] [PDF]
|
|
|
|
|
|
A D'Andrea, P Caso, B Sarubbi, M D'Alto, M Giovanna Russo, M Scherillo, M Cotrufo, and R Calabro Right ventricular myocardial activation delay in adult patients with right bundle branch block late after repair of Tetralogy of Fallot Eur J Echocardiogr, March 1, 2004; 5(2): 123 - 131. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M M H Cheung, A M Davis, J L Wilkinson, and R G Weintraub Long term somatic growth after repair of tetralogy of Fallot: evidence for restoration of genetic growth potential Heart, November 1, 2003; 89(11): 1340 - 1343. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Kuehne, M. Saeed, K. Gleason, D. Turner, D. Teitel, C. B. Higgins, and P. Moore Effects of Pulmonary Insufficiency on Biventricular Function in the Developing Heart of Growing Swine Circulation, October 21, 2003; 108(16): 2007 - 2013. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. d'Udekem d'Acoz, A. Pasquet, L. Lebreux, C. Ovaert, F. Mascart, A. Robert, and J. E. Rubay Does right ventricular outflow tract damage play a role in the genesis of late right ventricular dilatation after tetralogy of Fallot repair? Ann. Thorac. Surg., August 1, 2003; 76(2): 555 - 561. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I-S. Kang, A. N. Redington, L. N. Benson, C. Macgowan, E. R. Valsangiacomo, K. Roman, C. J. Kellenberger, and S.-J. Yoo Differential Regurgitation in Branch Pulmonary Arteries After Repair of Tetralogy of Fallot: A Phase-Contrast Cine Magnetic Resonance Study Circulation, June 17, 2003; 107(23): 2938 - 2943. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. M. Dubin, J. A. Feinstein, V. M. Reddy, F. L. Hanley, G. F. Van Hare, and D. N. Rosenthal Electrical Resynchronization: A Novel Therapy for the Failing Right Ventricle Circulation, May 13, 2003; 107(18): 2287 - 2289. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. A. C. Pedra, H. Justino, D. G. Nykanen, G. V. MD, J. G. Coles, W. G. Williams, R. M. Freedom, and L. N. Benson Percutaneous stent implantation to stenotic bioprosthetic valves in the pulmonary position J. Thorac. Cardiovasc. Surg., July 1, 2002; 124(1): 82 - 87. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Faidutti, J. T. Christenson, M. Beghetti, B. Friedli, and A. Kalangos How to diminish reoperation rates after initial repair of tetralogy of Fallot? Ann. Thorac. Surg., January 1, 2002; 73(1): 96 - 101. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S Brili, C Aggeli, K Gatzoulis, A Tzonou, C Hatzos, C Pitsavos, C Stefanadis, and P Toutouzas Echocardiographic and signal averaged ECG indices associated with non-sustained ventricular tachycardia after repair of tetralogy of Fallot Heart, January 1, 2001; 85(1): 57 - 60. [Abstract] [Full Text]
|
|
|
|
|
|
M. A. Maluf, D. M. Braile, C. Silva, R. Catani, A. C. Carvalho, and E. Buffolo Reconstruction of the pulmonary valve and outflow tract with bicuspid prosthesis in tetralogy of Fallot Ann. Thorac. Surg., December 1, 2000; 70(6): 1911 - 1917. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. R. Chaturvedi, D. F. Shore, C. Lincoln, S. Mumby, M. Kemp, J. Brierly, A. Petros, J. M.G. Gutteridge, J. Hooper, and A. N. Redington Acute Right Ventricular Restrictive Physiology After Repair of Tetralogy of Fallot : Association With Myocardial Injury and Oxidative Stress Circulation, October 5, 1999; 100(14): 1540 - 1547. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. N. Oechslin, D. A. Harrison, L. Harris, E. Downar, G. D. Webb, S. S. Siu, and W. G. Williams REOPERATION IN ADULTS WITH REPAIR OF TETRALOGY OF FALLOT: INDICATIONS AND OUTCOMES J. Thorac. Cardiovasc. Surg., August 1, 1999; 118(2): 245 - 251. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Conte, R. Jashari, B. Eyskens, M. Gewillig, M. Dumoulin, and W. Daenen Homograft valve insertion for pulmonary regurgitation late after valveless repair of right ventricular outflow tract obstruction Eur. J. Cardiothorac. Surg., February 1, 1999; 15(2): 143 - 149. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. Munkhammar, S. Cullen, P. Jogi, M. de Leval, M. Elliott, and G. Norgard Early age at repair prevents restrictive right ventricular (RV) physiology after surgery for tetralogy of Fallot (TOF): Diastolic RV function after TOF repair in infancy J. Am. Coll. Cardiol., October 1, 1998; 32(4): 1083 - 1087. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. J. Knott-Craig, R. C. Elkins, M. M. Lane, J. Holz, C. McCue, and K. E. Ward A 26-year experience with surgical management of tetralogy of fallot: risk analysis for mortality or late reintervention Ann. Thorac. Surg., August 1, 1998; 66(2): 506 - 511. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G Norgård, M A Gatzoulis, M Josen, S Cullen, and A N Redington Does restrictive right ventricular physiology in the early postoperative period predict subsequent right ventricular restriction after repair of tetralogy of Fallot? Heart, May 1, 1998; 79(5): 481 - 484. [Abstract] [Full Text]
|
|
|
|
|
|
I. M. Yemets, W. G. Williams, G. D. Webb, D. A. Harrison, P. R. McLaughlin, G. A. Trusler, J. G. Coles, I. M. Rebeyka, and R. M. Freedom Pulmonary Valve Replacement Late After Repair of Tetralogy of Fallot Ann. Thorac. Surg., August 1, 1997; 64(2): 526 - 530. [Abstract] [Full Text]
|
|
|
|
|
|
M. A. Gatzoulis, J. A. Till, and A. N. Redington Depolarization-Repolarization Inhomogeneity After Repair of Tetralogy of Fallot: The Substrate for Malignant Ventricular Tachycardia? Circulation, January 21, 1997; 95(2): 401 - 404. [Abstract] [Full Text]
|
|
|
|
|
|
G. Norgard, M. A. Gatzoulis, F. Moraes, C. Lincoln, D. F. Shore, E. A. Shinebourne, and A. N. Redington Relationship Between Type of Outflow Tract Repair and Postoperative Right Ventricular Diastolic Physiology in Tetralogy of Fallot: Implications for Long-term Outcome Circulation, December 15, 1996; 94(12): 3276 - 3280. [Abstract] [Full Text]
|
|
|
|
|
|
M. A. Gatzoulis, J. A. Till, J. Somerville, and A. N. Redington Mechanoelectrical Interaction in Tetralogy of Fallot : QRS Prolongation Relates to Right Ventricular Size and Predicts Malignant Ventricular Arrhythmias and Sudden Death Circulation, July 15, 1995; 92(2): 231 - 237. [Abstract] [Full Text]
|
|
|
|
 |
|
 A. A. W. Roest, W. A. Helbing, P. Kunz, J. G. van den Aardweg, H. J. Lamb, H. W. Vliegen, E. E. van der Wall, and A. de Roos Exercise MR Imaging in the Assessment of Pulmonary Regurgitation and Biventricular Function in Patients after Tetralogy of Fallot Repair Radiology, April 1, 2002; 223(1): 204 - 211. [Abstract] [Full Text] [PDF]
|
|
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||