Original Article
Does early tetralogy of Fallot total correction give better final lung volumes?
Asian Cardiovascular & Thoracic Annals 21(3) 270–274 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312451580 aan.sagepub.com
Hasan Allah Sadeghi1, Seyed Reza Miri2, Hooman Bakhshandeh3, Yalda Mirmesdagh4 and Nazita Paziraee5
Abstract Background: Pulmonary blood flow may affect lung development in adulthood. Early total correction of tetralogy of Fallot may affect development of final lung volumes. We evaluated the effect of age at total correction on lung volumes years after the operation. Methods: In a retrospective cohort study on patients with totally corrected tetralogy of Fallot (mean age, 13.40 years at the time of follow-up), forced vital capacity, slow vital capacity, forced expiratory volume in 1 s, and other parameters were measured 154.8 46.25 months after the operation. Comparison were made of 3 groups: 42-, 2–8-, and >8-years old at the time of total correction surgery. Results: Among 322 enrolled patients, the mean values of the follow-up spirometry results in 42-, 2–8-, >8-year-olds and the percentage of predicted values were respectively: vital capacity: 4.46 0.57 L (107% 10.96%), 3.89 0.58 L (91.10% 12.25%), 3.25 0.48 L (82.35% 10.62%), p < 0.001; forced vital capacity: 4.28 0.63 L (95.90% 18.77%), 3.76 0.58 L (90.83% 12.52%), 3.14 0.49 L (83.26% 11.71%), p < 0.001; forced expiratory volume in 1 s: 4.22 0.63 L (104.84% 13.64%), 3.66 0.58 L (90.61% 12.59%), 3.02 0.48 L (84.31% 12%), p < 0.001. Conclusion: Early correction of defects or reestablishments of perfusion of tetralogy of Fallot before completion of lung development might improve final adulthood lung volumes and capacities. It is better to consider total correction for all tetralogy of Fallot patients below 2-years old, or at least below 8-years old, if it is technically possible.
Keywords Congenital, follow-up studies, heart defects, pulmonary function tests, spirometry, tetralogy of Fallot
Introduction Pulmonary blood flow and chest wall movement may affect lung development and final lung volume in adulthood.1–3 Pulmonary arterial and alveolar development was found to be abnormal in postmortem examinations of tetralogy of Fallot (TOF) patients who died perioperatively.2 A range of pathologic lung function parameters in patients with hemodynamically significant congenital heart defects has been reported.3–5 The final lung volume of a patient with a small-sized pulmonary artery might be lower than predicted.2–4 Postmortem lung volume in relation to body surface area was generally below normal for age, the alveoli were small, and the total alveolar number was below normal in 5 of 7 cases studied.3 Pulmonary restriction is predominant in patients with TOF.1,5,6 Based on these studies, perhaps it is logical to think about the effects of
lung perfusion and chest motion on final lung volumes and the development of lungs, and also to conclude that 1 Department of Pulmonary Medicine, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 2 Department of Pediatric Cardiology, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 3 Department of Epidemiology, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 4 Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 5 Iranian Blood Council, Tehran, Iran
Corresponding author: Hasan Allah Sadeghi, MD, Department of Pulmonary Medicine, Rajaie Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Niayesh Highway, Vali Asr Ave, Tehran 1996911156, Iran. Email: [email protected]
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early correction of defects or reestablishment of perfusion before complete development of the lungs might improve adult lung volumes. This issue should be considered in TOF patients in whom lung perfusion is low, and increased pulmonary blood flow by shunting and total correction may affect lung growth and development. A study of TOF patients who have hypoperfused lungs may be helpful to evaluate this hypothesis. We studied lung volumes of patients with TOF who had undergone total correction in childhood. The correlation between time of surgery and final lung volume was also investigated.
Patients and methods In a retrospective cohort study, we included all known cases of TOF treated surgically within an appropriate time, consecutively as the patients came to the outpatient follow-up clinic at Rajaie Heart Center. After approval of the study protocol had been granted by the institutional review board and ethics committee (Tehran University of Medical Sciences, no. 1836), and written informed consent had been obtained from each patient, all individuals were subjected to a detailed physical examination, electrocardiography, and routine laboratory tests. Evaluations of right and left ventricular function by echocardiography were performed using a GE VIVID 3 device, by a pediatric cardiologist expert in this field, using two-dimensional, pulsed-wave Doppler, and continuous Doppler echocardiography techniques. A posteroanterior chest radiograph was obtained from all patients. Operative data were extracted from the medical files. The patients who had no residual shunting, no signs or episodes of congestive heart failure, no significant postoperative complication that would interfere with lung volume (diaphragm paralysis or pachypleuritis), and no prominent cardiomegaly, were considered for the lung volume study. Spirometry was carried out at our respiratory unit by experienced technicians under supervision. Spirometry was repeated a maximum of 8 times for each individual, using a Fukuda spirometer, and the best measurements were recorded and compared with normal values based on European Respiratory Society standards. All dynamic volumes and flows including forced vital capacity (FVC), forced expiratory volume in the 1st second (FEV1), peak expiratory flow rate (PEFR), forced expiratory flow rate at 25%, 50%, and 75% of vital capacity (FEF25, FEF50, FEF75), maximum voluntary ventilation (MVV), maximal mid-expiratory flow (MMEF), and FEV1/FVC, were measured. All patients who were below 8-years old and/or unable to cooperate to do standard spirometry after 8 trials were excluded. Results were also described
as the percentage of predicted values calculated from equations that have been reported as normal. All spirometry reports were reevaluated by a pulmonologist expert in this field, and tests with poor quality or an obstructive pattern were excluded from the study. The 322 patients who met the criteria were enrolled in this study and divided in 3 groups based on their age at total correction (42-, 2–8- and >8-years old). The data were collected and transferred to SPSS version 15 software (SPSS, Inc., Chicago, IL, USA) for statistical evaluation. Each group was analyzed for demographic data and spirometry values. Values are given as the mean standard deviation. Comparisons of groups were performed by the analysis of variance test. The Pearson correlation test was applied to analyze correlations among the parameters. A p value 8 years. The types and frequencies of procedures and repair approaches are shown in Tables 2 and 3. All patients aged 42 years suffered from right bundle branch block at the time of follow-up versus 153 (96.2%) in the group aged >2- to 8-years and 151 (98.7%) in the group aged >8 years. The follow-up echocardiography findings in the 322 cases included in this study at the time of spirometry are given in Table 4. The mean values of pulmonary function tests were evaluated and compared among the 3 groups (Table 5). The results demonstrated that the mean values of VC, predicted VC, FVC, predicted FVC, FEV1, predicted FEV1, FEV1/FVC, MVV, FEF25, and MMEF were
Downloaded from aan.sagepub.com at UNIV OF MASSACHUSETTS on March 14, 2015
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significantly higher in the age group 42 years compared to the other groups. The mean values of FEF50, FEF75, PEFR, and predicted PEFR tended to be higher in the group of patients aged 42 years, but these differences were not statistically significant.
Discussion In a review of the literature, we found one report with a similarly large number of cases of TOF.7 Our data show that TOF is more common in boys, with a male/female ratio of 2/1. There were 8 (2.4%) patients with an obstructive pattern on spirometry, based on the American Thoracic Society criteria (FEV1/FVC 80%. The FEV1/FVC ratio was mainly normal, indicating a restrictive ventilatory pattern on pulmonary function tests. These findings are similar to the data of Pianosi and colleagues.7 Our patients had increased cardiothoracic ratios on radiography, but we do not think it was the main cause of decreased lung volumes or the restrictive pattern observed, because we excluded those with obvious cardiomegaly, and only asymptomatic patients with a cardiothoracic ratio >50% were included in this study. The reduction in final lung development and alveolar volume and size probably
Table 3. Repair approaches in age groups of patients undergoing correction of tetralogy of Fallot. Approach
42 years 2–8 years
Transatrial 2 (20%) Trans-right 8 (80%) ventricle Total 10
>8 years
Total
15 (9.4%) 22 (14.6%) 39 (12.1%) 144 (90.6%) 131 (85.6%) 283 (87.6%) 159
153
322 (100%)
% of predicted value
VC (L) 3.60 0.64 FVC (L) 3.48 0.64 FEV1 (L) 3.38 0.64 FEV1/FVC 96.94% 3.66% MVV (Lmin1) 74.31 11.03 FEF at 25% VC (Lmin1) 3.83 0.70 FEF at 55% VC (Lmin1) 4.85 0.83 FEF at 75% VC (Lmin1) 5.85 0.96 MMEF (Lmin1) 4.34 0.81 PEF (Lmin1) 5.53 1.13
87.54% 12.70% 87.39% 12.97% 88.06% 13.04%
Table 4. Follow-up echocardiography findings at the time of spirometry.
74.59% 11.98%
97.40% 45.04%
FEF: forced expiratory flow; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; MMEF: maximal mid-expiratory flow; MVV: maximal voluntary ventilation; PEFR: peak expiratory flow rate; SD: standard deviation; VC: vital capacity.
Variable
42 years 2–8 years
No or mild PS/PI/ RVOT obstruction Moderate PS/PI/RVOT obstruction Severe PS/PI/RVOT obstruction Total
7 (70%)
>8 years
Total
138 (86.8%) 131 (85.6%) 276
1 (10%)
10 (6.3%)
14 (9.1%)
25
2 (20%)
11 (6.9%)
8 (5.2%)
21
10
159
153
322
PI: pulmonary insufficiency; PS: pulmonary stenosis; RVOT: right ventricular outflow tract.
Table 2. Procedures in age groups of patients undergoing correction of tetralogy of Fallot. Variable
42 years
2–8 years
>8 years
Total
Subvalvular myomectomy Pulmonary commissurotomy/valvotomy/myomectomy Transannular patch Extensive patching of branch(es) Subvalvular myomectomy þ RVOT patch Rastelli operation Total
3 1 2 1 3 0 10
26 20 73 17 22 1 159
25 16 58 25 27 2 153
54 37 133 43 52 3 322
RVOT: right ventricular outflow tract.
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(16.8%) (11.5%) (41.3%) (13.4%) (16.1%) (0.9%) (100%)
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273
Table 5. Pulmonary function tests after correction of tetralogy of Fallot. Variable
42 years
2–8 years
>8 years
p value
VC (L) % of predicted FVC (L) % of predicted FEV1 (L) % of predicted FEV1/FVC MVV (Lmin1) % of predicted FEF at 25% VC (Lmin1) FEF at 50% VC (Lmin1) FEF at 75% VC (Lmin1) MMEF (Lmin1) PEFR (Lmin1) % of predicted
4.46 0.57 107% 10.96% 4.28 0.63 95.90% 18.77% 4.22 0.63 104.84% 13.64% 98.67% 1.14% 76.28 9.25 73.40% 16.62% 4.20 0.75 5.11 1.01 6.10 1.21 4.75 1.07 5.95 1.31 99.20% 18.69%
3.89 0.58 91.10% 12.25% 3.76 0.58 90.83% 12.52% 3.66 0.58 90.61% 12.59% 97.34% 3.34% 75.93 11.38 75.99% 11.60% 3.95 0.63 4.93 0.81 5.94 0.90 4.44 0.78 5.58 1.13 95.83% 17.26%
3.25 0.48 82.35% 10.62% 3.14 0.49 83.26% 11.71% 3.02 0.48 84.31% 12% 96.40% 3.99% 72.49 10.54 73.21% 11.95% 3.68 0.73 4.75 0.84 5.7 0.99 4.20 0.81 5.45 1.11 98.91% 62.83%
Does early tetralogy of Fallot total correction give better final lung volumes?
Asian Cardiovascular & Thoracic Annals 21(3) 270–274 ß The Author(s) 2012 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312451580 aan.sagepub.com
Hasan Allah Sadeghi1, Seyed Reza Miri2, Hooman Bakhshandeh3, Yalda Mirmesdagh4 and Nazita Paziraee5
Abstract Background: Pulmonary blood flow may affect lung development in adulthood. Early total correction of tetralogy of Fallot may affect development of final lung volumes. We evaluated the effect of age at total correction on lung volumes years after the operation. Methods: In a retrospective cohort study on patients with totally corrected tetralogy of Fallot (mean age, 13.40 years at the time of follow-up), forced vital capacity, slow vital capacity, forced expiratory volume in 1 s, and other parameters were measured 154.8 46.25 months after the operation. Comparison were made of 3 groups: 42-, 2–8-, and >8-years old at the time of total correction surgery. Results: Among 322 enrolled patients, the mean values of the follow-up spirometry results in 42-, 2–8-, >8-year-olds and the percentage of predicted values were respectively: vital capacity: 4.46 0.57 L (107% 10.96%), 3.89 0.58 L (91.10% 12.25%), 3.25 0.48 L (82.35% 10.62%), p < 0.001; forced vital capacity: 4.28 0.63 L (95.90% 18.77%), 3.76 0.58 L (90.83% 12.52%), 3.14 0.49 L (83.26% 11.71%), p < 0.001; forced expiratory volume in 1 s: 4.22 0.63 L (104.84% 13.64%), 3.66 0.58 L (90.61% 12.59%), 3.02 0.48 L (84.31% 12%), p < 0.001. Conclusion: Early correction of defects or reestablishments of perfusion of tetralogy of Fallot before completion of lung development might improve final adulthood lung volumes and capacities. It is better to consider total correction for all tetralogy of Fallot patients below 2-years old, or at least below 8-years old, if it is technically possible.
Keywords Congenital, follow-up studies, heart defects, pulmonary function tests, spirometry, tetralogy of Fallot
Introduction Pulmonary blood flow and chest wall movement may affect lung development and final lung volume in adulthood.1–3 Pulmonary arterial and alveolar development was found to be abnormal in postmortem examinations of tetralogy of Fallot (TOF) patients who died perioperatively.2 A range of pathologic lung function parameters in patients with hemodynamically significant congenital heart defects has been reported.3–5 The final lung volume of a patient with a small-sized pulmonary artery might be lower than predicted.2–4 Postmortem lung volume in relation to body surface area was generally below normal for age, the alveoli were small, and the total alveolar number was below normal in 5 of 7 cases studied.3 Pulmonary restriction is predominant in patients with TOF.1,5,6 Based on these studies, perhaps it is logical to think about the effects of
lung perfusion and chest motion on final lung volumes and the development of lungs, and also to conclude that 1 Department of Pulmonary Medicine, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 2 Department of Pediatric Cardiology, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 3 Department of Epidemiology, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 4 Heart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Tehran, Iran 5 Iranian Blood Council, Tehran, Iran
Corresponding author: Hasan Allah Sadeghi, MD, Department of Pulmonary Medicine, Rajaie Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Niayesh Highway, Vali Asr Ave, Tehran 1996911156, Iran. Email: [email protected]
Downloaded from aan.sagepub.com at UNIV OF MASSACHUSETTS on March 14, 2015
Sadeghi et al.
271
early correction of defects or reestablishment of perfusion before complete development of the lungs might improve adult lung volumes. This issue should be considered in TOF patients in whom lung perfusion is low, and increased pulmonary blood flow by shunting and total correction may affect lung growth and development. A study of TOF patients who have hypoperfused lungs may be helpful to evaluate this hypothesis. We studied lung volumes of patients with TOF who had undergone total correction in childhood. The correlation between time of surgery and final lung volume was also investigated.
Patients and methods In a retrospective cohort study, we included all known cases of TOF treated surgically within an appropriate time, consecutively as the patients came to the outpatient follow-up clinic at Rajaie Heart Center. After approval of the study protocol had been granted by the institutional review board and ethics committee (Tehran University of Medical Sciences, no. 1836), and written informed consent had been obtained from each patient, all individuals were subjected to a detailed physical examination, electrocardiography, and routine laboratory tests. Evaluations of right and left ventricular function by echocardiography were performed using a GE VIVID 3 device, by a pediatric cardiologist expert in this field, using two-dimensional, pulsed-wave Doppler, and continuous Doppler echocardiography techniques. A posteroanterior chest radiograph was obtained from all patients. Operative data were extracted from the medical files. The patients who had no residual shunting, no signs or episodes of congestive heart failure, no significant postoperative complication that would interfere with lung volume (diaphragm paralysis or pachypleuritis), and no prominent cardiomegaly, were considered for the lung volume study. Spirometry was carried out at our respiratory unit by experienced technicians under supervision. Spirometry was repeated a maximum of 8 times for each individual, using a Fukuda spirometer, and the best measurements were recorded and compared with normal values based on European Respiratory Society standards. All dynamic volumes and flows including forced vital capacity (FVC), forced expiratory volume in the 1st second (FEV1), peak expiratory flow rate (PEFR), forced expiratory flow rate at 25%, 50%, and 75% of vital capacity (FEF25, FEF50, FEF75), maximum voluntary ventilation (MVV), maximal mid-expiratory flow (MMEF), and FEV1/FVC, were measured. All patients who were below 8-years old and/or unable to cooperate to do standard spirometry after 8 trials were excluded. Results were also described
as the percentage of predicted values calculated from equations that have been reported as normal. All spirometry reports were reevaluated by a pulmonologist expert in this field, and tests with poor quality or an obstructive pattern were excluded from the study. The 322 patients who met the criteria were enrolled in this study and divided in 3 groups based on their age at total correction (42-, 2–8- and >8-years old). The data were collected and transferred to SPSS version 15 software (SPSS, Inc., Chicago, IL, USA) for statistical evaluation. Each group was analyzed for demographic data and spirometry values. Values are given as the mean standard deviation. Comparisons of groups were performed by the analysis of variance test. The Pearson correlation test was applied to analyze correlations among the parameters. A p value 8 years. The types and frequencies of procedures and repair approaches are shown in Tables 2 and 3. All patients aged 42 years suffered from right bundle branch block at the time of follow-up versus 153 (96.2%) in the group aged >2- to 8-years and 151 (98.7%) in the group aged >8 years. The follow-up echocardiography findings in the 322 cases included in this study at the time of spirometry are given in Table 4. The mean values of pulmonary function tests were evaluated and compared among the 3 groups (Table 5). The results demonstrated that the mean values of VC, predicted VC, FVC, predicted FVC, FEV1, predicted FEV1, FEV1/FVC, MVV, FEF25, and MMEF were
Downloaded from aan.sagepub.com at UNIV OF MASSACHUSETTS on March 14, 2015
272
Asian Cardiovascular & Thoracic Annals 21(3)
significantly higher in the age group 42 years compared to the other groups. The mean values of FEF50, FEF75, PEFR, and predicted PEFR tended to be higher in the group of patients aged 42 years, but these differences were not statistically significant.
Discussion In a review of the literature, we found one report with a similarly large number of cases of TOF.7 Our data show that TOF is more common in boys, with a male/female ratio of 2/1. There were 8 (2.4%) patients with an obstructive pattern on spirometry, based on the American Thoracic Society criteria (FEV1/FVC 80%. The FEV1/FVC ratio was mainly normal, indicating a restrictive ventilatory pattern on pulmonary function tests. These findings are similar to the data of Pianosi and colleagues.7 Our patients had increased cardiothoracic ratios on radiography, but we do not think it was the main cause of decreased lung volumes or the restrictive pattern observed, because we excluded those with obvious cardiomegaly, and only asymptomatic patients with a cardiothoracic ratio >50% were included in this study. The reduction in final lung development and alveolar volume and size probably
Table 3. Repair approaches in age groups of patients undergoing correction of tetralogy of Fallot. Approach
42 years 2–8 years
Transatrial 2 (20%) Trans-right 8 (80%) ventricle Total 10
>8 years
Total
15 (9.4%) 22 (14.6%) 39 (12.1%) 144 (90.6%) 131 (85.6%) 283 (87.6%) 159
153
322 (100%)
% of predicted value
VC (L) 3.60 0.64 FVC (L) 3.48 0.64 FEV1 (L) 3.38 0.64 FEV1/FVC 96.94% 3.66% MVV (Lmin1) 74.31 11.03 FEF at 25% VC (Lmin1) 3.83 0.70 FEF at 55% VC (Lmin1) 4.85 0.83 FEF at 75% VC (Lmin1) 5.85 0.96 MMEF (Lmin1) 4.34 0.81 PEF (Lmin1) 5.53 1.13
87.54% 12.70% 87.39% 12.97% 88.06% 13.04%
Table 4. Follow-up echocardiography findings at the time of spirometry.
74.59% 11.98%
97.40% 45.04%
FEF: forced expiratory flow; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; MMEF: maximal mid-expiratory flow; MVV: maximal voluntary ventilation; PEFR: peak expiratory flow rate; SD: standard deviation; VC: vital capacity.
Variable
42 years 2–8 years
No or mild PS/PI/ RVOT obstruction Moderate PS/PI/RVOT obstruction Severe PS/PI/RVOT obstruction Total
7 (70%)
>8 years
Total
138 (86.8%) 131 (85.6%) 276
1 (10%)
10 (6.3%)
14 (9.1%)
25
2 (20%)
11 (6.9%)
8 (5.2%)
21
10
159
153
322
PI: pulmonary insufficiency; PS: pulmonary stenosis; RVOT: right ventricular outflow tract.
Table 2. Procedures in age groups of patients undergoing correction of tetralogy of Fallot. Variable
42 years
2–8 years
>8 years
Total
Subvalvular myomectomy Pulmonary commissurotomy/valvotomy/myomectomy Transannular patch Extensive patching of branch(es) Subvalvular myomectomy þ RVOT patch Rastelli operation Total
3 1 2 1 3 0 10
26 20 73 17 22 1 159
25 16 58 25 27 2 153
54 37 133 43 52 3 322
RVOT: right ventricular outflow tract.
Downloaded from aan.sagepub.com at UNIV OF MASSACHUSETTS on March 14, 2015
(16.8%) (11.5%) (41.3%) (13.4%) (16.1%) (0.9%) (100%)
Sadeghi et al.
273
Table 5. Pulmonary function tests after correction of tetralogy of Fallot. Variable
42 years
2–8 years
>8 years
p value
VC (L) % of predicted FVC (L) % of predicted FEV1 (L) % of predicted FEV1/FVC MVV (Lmin1) % of predicted FEF at 25% VC (Lmin1) FEF at 50% VC (Lmin1) FEF at 75% VC (Lmin1) MMEF (Lmin1) PEFR (Lmin1) % of predicted
4.46 0.57 107% 10.96% 4.28 0.63 95.90% 18.77% 4.22 0.63 104.84% 13.64% 98.67% 1.14% 76.28 9.25 73.40% 16.62% 4.20 0.75 5.11 1.01 6.10 1.21 4.75 1.07 5.95 1.31 99.20% 18.69%
3.89 0.58 91.10% 12.25% 3.76 0.58 90.83% 12.52% 3.66 0.58 90.61% 12.59% 97.34% 3.34% 75.93 11.38 75.99% 11.60% 3.95 0.63 4.93 0.81 5.94 0.90 4.44 0.78 5.58 1.13 95.83% 17.26%
3.25 0.48 82.35% 10.62% 3.14 0.49 83.26% 11.71% 3.02 0.48 84.31% 12% 96.40% 3.99% 72.49 10.54 73.21% 11.95% 3.68 0.73 4.75 0.84 5.7 0.99 4.20 0.81 5.45 1.11 98.91% 62.83%
