Impact of Neonatal Versus Nonneonatal Total Repair of Tetralogy of Fallot on Growth in the First Year of Life Kristal L. Woldu, MD, Bhawna Arya, MD, Emile A. Bacha, MD, and Ism ee A. Williams, MD, MS
Background. Optimal timing for total repair in tetralogy of Fallot (TOF) is controversial. We aimed to determine if weight at 1 year differs between patients who undergo neonatal total repair versus those who undergo nonneonatal total repair later in the first year of life. Methods. A retrospective review of infants admitted with TOF between January 2004 and June 2011 was conducted. Patient data, including weight, were collected throughout the first year of life, and neonatal total repair versus nonneonatal total repair groups were compared. Results. Of 163 infants, neonatal total repair was undertaken in 36 (22%) of them, whereas 127 (78%) infants had nonneonatal total repair at greater than 28 days of life. The median neonatal intensive care unit length of stay (LOS) was longer for the neonatal total repair group than for the nonneonatal total repair group (17.5 [11–24] versus 7 [0–15] days; p < 0.001). Patients in the neonatal total repair
group were more likely to have a transannular patch (TAP) (p < 0.001) than were those in the nonneonatal total repair group, whereas patients in the nonneonatal total repair group were more likely to have undergone a valve-sparing operation (p [ 0.002). The mean weight-for-age z score was 0.7 higher in the neonatal total repair group compared with the nonneonatal total repair group (p [ 0.03) controlling for birth weight (BW), diagnostic subgroup, and gestational age (GA). Conclusions. Patients with TOF who underwent neonatal total repair were more likely to receive a TAP but had higher weight-for-age scores at 1 year compared with patients who underwent full repair later in the first year of life.
T
Morgan Stanley Children’s Hospital between January 2004 and June 2011 with the diagnosis of TOF or double-outlet right ventricle with pulmonary stenosis with tetralogy-like physiology. The latter congenital cardiac defect was included because of physiologic features and surgical repair similar to those of TOF. Cardiac diagnosis was determined by postnatal echocardiography at our institution within 2 weeks of birth. Patients could have been initially diagnosed in any location, including in the well-baby nursery, at outpatient visits, or in the neonatal or pediatric intensive care units. A pediatric cardiologist (IAW) reviewed patient information, including type of lesion, neonatal oxygen saturation, and ductal dependence. Patients with ductal dependence were included in the statistical analysis because this factor did not stipulate patients would receive a total repair in the neonatal period, only that they would have a neonatal intervention. Exclusion criteria included initial evaluation at our institution outside the neonatal period, lack of neonatal echocardiographic data, and repair beyond the first year of life. Patients were assigned to the neonatal total repair group if they underwent a repair of TOF within the first 28 days of life. We collected weight measurements in the first year of life, as well as birth weight (BW) and gestational age (GA). For patients admitted to our intensive care units who underwent surgical repair at our hospital and were followed postoperatively elsewhere, we contacted the
etralogy of Fallot (TOF) represents approximately 10% of congenital cardiac malformations and is 1 of the most common causes of cyanotic heart disease beyond the newborn period [1]. Surgical intervention is necessary for long-term survival. Without repair, a quarter of patients with severe obstruction will die within the first year of life. There is no current consensus regarding the optimal timing of surgical repair nor is there information on the effect of surgical timing on growth in children with TOF. In this study, we investigated differences in growth between patients with TOF who undergo surgical repair in the neonatal period versus those who undergo repair later in the first year of life. We hypothesized that growth would be improved in patients who underwent neonatal total repair.
Patients and Methods We conducted an institutional review board–approved retrospective review of patients admitted to the level IV* cardiac intensive care units at the New York Presbyterian Accepted for publication May 12, 2014. Address correspondence to Dr Williams, 3959 Broadway, New York, NY 10032; e-mail: [email protected]. *Intensive care units in centers with extracorporeal membrane oxygenation or complex surgical intervention with bypass available.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
(Ann Thorac Surg 2014;98:1399–404) Ó 2014 by The Society of Thoracic Surgeons
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.05.034
CONGENITAL HEART
Departments of Pediatrics and Surgery, Columbia University, NewYork-Presbyterian Morgan Stanley Children’s Hospital, New York, New York
1400
WOLDU ET AL NEONATAL REPAIR AND GROWTH OUTCOMES IN TOF
CONGENITAL HEART
primary cardiologist to obtain growth data at 1 year (range, 10–14 months) of age. Other perioperative data obtained included use of respiratory support, extracorporeal membrane oxygenation, prostaglandin, pressor agents, and other medications such as antibiotics, as well as length of stay (LOS). Operative data, including type of operation, day of life (DOL) on which the operation took place, and surgical complications, were also recorded. Given the possible effect on growth, information regarding genetic syndromes, need for other procedures or surgical repairs, number of hospitalizations during the first year of life, and other complications during the hospital stay were also collected.
Ann Thorac Surg 2014;98:1399–404
Results During the 7-year study period, 165 infants were admitted to the cardiac intensive care units with TOF. Two patients received comfort care from birth (1 with trisomy 13 and 1 with trisomy 18) and were removed from the analysis. Baseline characteristics of the remaining 163 patients are shown in Table 1. Of the entire cohort, 43 (26%) had a hypercyanotic “Tet” spell during the first year of life. Of the 163 patients, 10 of 163 (6%) died before undergoing a total repair: 4 died because of complications from their genetic abnormalities and 6 died because of other complications before surgical repair.
Neonatal Total Repair Group Statistical Methods Data are represented as mean standard deviation (SD), medians with the interquartile range (IQR), or frequencies as appropriate. Weight is expressed as weightfor-age z score based on the World Health Organization (WHO) growth standards, whereby a z score of þ1 is equivalent to a weight 1 SD greater than the mean, and a z score of –1 is equivalent to a weight 1 SD less than the mean [2]. Associations between timing of surgical intervention (neonatal versus nonneonatal total repair) and continuous outcomes were investigated using a Student’s t test or a Mann-Whitney U test when appropriate. Associations between categorical variables were assessed using tests of proportion and the c2 distribution. Multivariable stepwise linear regression modeling was used to test if group assignment (neonatal versus nonneonatal total repair) remained an important predictor of weight-for-age z score at 1 year independent of other known predictors of weight.
Of the 163 patients, 36 (22%) had a neonatal total repair; 24 of 36 (64%) patients had ductal dependency at the time of operation. Of the 36 patients, 30 (83%) underwent a transannular patch (TAP) repair, and 6 (17%) had a conduit or homograft placed (Table 2). None had a pulmonary valvotomy or valve-sparing total repair.
Nonneonatal Total Repair Group Of the 163 patients, 127 (78%) had a nonneonatal total repair; 31 of 127 (24%) had ductal dependency and underwent an initial shunt procedure. Of the 127 patients in the nonneonatal total repair group, 104 of 127 (82%) underwent a total repair at our institution, 13 of 127 (10%) were lost to follow-up after the initial shunt procedure but before total repair, and 10 of 127 (8%) died before total repair. All 10 deaths in our cohort occurred in the nonneonatal total repair group, and none would be considered surgical mortalities by the Society of Thoracic Surgeons database criteria. Of the 104 patients who
Table 1. Baseline Characteristics Total Patients With TOF (N ¼ 163) Characteristic Male sex Prenatally diagnosed Ductal dependency Tet spells TOF/PA TOF/PA/MAPCAS TOF/AVC Genetic syndromes DiGeorge Trisomy 21 VACTERL Trisomy 13 GA (wk) BW (kg) BW z scores
n (%)
Neonatal Repair ¼ 36 n (%) or (mean SD)
Nonneonatal Repair ¼ 127 n (%) or (mean SD)
p Value
99 (61) 120 (74) 55 (32) 43 (26) 28 (17) 19 (12) 9 (6) 49 (30) 18 (11) 14 (9) 5 (3) 4 (3) 162 (99) 163 163
24 (67) 21 (58) 24 (67) 8 (22) 10 (28) 3 (8) 0 (0) 6 (17) 3 (8) 2 (6) 0 (0) 0 (0) 38.4 2.5 3.1 0.7 –0.7 1.6
75 (59) 99 (78) 31 (24) 35 (28) 18 (14) 16 (13) 9 (7) 43 (34) 15 (12) 12 (4) 5 (4) 4 (3) 37.4 1.6 2.8 0.7 –1.2 1.6
0.4 0.02 2 cardiac repairs in first year Required other hospitalizationsa Death before repair No cardiac intervention offered because of genetic syndrome No cardiac intervention offered because of other complications
n (%)
Neonatal Repair ¼ 36 n (%) (mean SD) or median [IQR]
Nonneonatal Repair ¼ 127 n (%) (mean SD) or median [IQR]
p Value
... ... ... ... ... 91 of 140 (65) 24 of 140 (17) 23 of 140 (16) 93 (57) 25 (18) 81 (52)
17.5 [11–24] 19 [12–27] 8.5 [5–12] 7.1 4.6 125 53 30 (83) 4 (11) 0 (0) 32 (89) 9 (25) 19 (53)
7 [0–15] 15.5 [3–28] 136 [62–210] 5.1 6.6 124 77 61 (59) 20 (19) 23 (22) 61 (48) 16 (15) 62 (52)
Background. Optimal timing for total repair in tetralogy of Fallot (TOF) is controversial. We aimed to determine if weight at 1 year differs between patients who undergo neonatal total repair versus those who undergo nonneonatal total repair later in the first year of life. Methods. A retrospective review of infants admitted with TOF between January 2004 and June 2011 was conducted. Patient data, including weight, were collected throughout the first year of life, and neonatal total repair versus nonneonatal total repair groups were compared. Results. Of 163 infants, neonatal total repair was undertaken in 36 (22%) of them, whereas 127 (78%) infants had nonneonatal total repair at greater than 28 days of life. The median neonatal intensive care unit length of stay (LOS) was longer for the neonatal total repair group than for the nonneonatal total repair group (17.5 [11–24] versus 7 [0–15] days; p < 0.001). Patients in the neonatal total repair
group were more likely to have a transannular patch (TAP) (p < 0.001) than were those in the nonneonatal total repair group, whereas patients in the nonneonatal total repair group were more likely to have undergone a valve-sparing operation (p [ 0.002). The mean weight-for-age z score was 0.7 higher in the neonatal total repair group compared with the nonneonatal total repair group (p [ 0.03) controlling for birth weight (BW), diagnostic subgroup, and gestational age (GA). Conclusions. Patients with TOF who underwent neonatal total repair were more likely to receive a TAP but had higher weight-for-age scores at 1 year compared with patients who underwent full repair later in the first year of life.
T
Morgan Stanley Children’s Hospital between January 2004 and June 2011 with the diagnosis of TOF or double-outlet right ventricle with pulmonary stenosis with tetralogy-like physiology. The latter congenital cardiac defect was included because of physiologic features and surgical repair similar to those of TOF. Cardiac diagnosis was determined by postnatal echocardiography at our institution within 2 weeks of birth. Patients could have been initially diagnosed in any location, including in the well-baby nursery, at outpatient visits, or in the neonatal or pediatric intensive care units. A pediatric cardiologist (IAW) reviewed patient information, including type of lesion, neonatal oxygen saturation, and ductal dependence. Patients with ductal dependence were included in the statistical analysis because this factor did not stipulate patients would receive a total repair in the neonatal period, only that they would have a neonatal intervention. Exclusion criteria included initial evaluation at our institution outside the neonatal period, lack of neonatal echocardiographic data, and repair beyond the first year of life. Patients were assigned to the neonatal total repair group if they underwent a repair of TOF within the first 28 days of life. We collected weight measurements in the first year of life, as well as birth weight (BW) and gestational age (GA). For patients admitted to our intensive care units who underwent surgical repair at our hospital and were followed postoperatively elsewhere, we contacted the
etralogy of Fallot (TOF) represents approximately 10% of congenital cardiac malformations and is 1 of the most common causes of cyanotic heart disease beyond the newborn period [1]. Surgical intervention is necessary for long-term survival. Without repair, a quarter of patients with severe obstruction will die within the first year of life. There is no current consensus regarding the optimal timing of surgical repair nor is there information on the effect of surgical timing on growth in children with TOF. In this study, we investigated differences in growth between patients with TOF who undergo surgical repair in the neonatal period versus those who undergo repair later in the first year of life. We hypothesized that growth would be improved in patients who underwent neonatal total repair.
Patients and Methods We conducted an institutional review board–approved retrospective review of patients admitted to the level IV* cardiac intensive care units at the New York Presbyterian Accepted for publication May 12, 2014. Address correspondence to Dr Williams, 3959 Broadway, New York, NY 10032; e-mail: [email protected]. *Intensive care units in centers with extracorporeal membrane oxygenation or complex surgical intervention with bypass available.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
(Ann Thorac Surg 2014;98:1399–404) Ó 2014 by The Society of Thoracic Surgeons
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.05.034
CONGENITAL HEART
Departments of Pediatrics and Surgery, Columbia University, NewYork-Presbyterian Morgan Stanley Children’s Hospital, New York, New York
1400
WOLDU ET AL NEONATAL REPAIR AND GROWTH OUTCOMES IN TOF
CONGENITAL HEART
primary cardiologist to obtain growth data at 1 year (range, 10–14 months) of age. Other perioperative data obtained included use of respiratory support, extracorporeal membrane oxygenation, prostaglandin, pressor agents, and other medications such as antibiotics, as well as length of stay (LOS). Operative data, including type of operation, day of life (DOL) on which the operation took place, and surgical complications, were also recorded. Given the possible effect on growth, information regarding genetic syndromes, need for other procedures or surgical repairs, number of hospitalizations during the first year of life, and other complications during the hospital stay were also collected.
Ann Thorac Surg 2014;98:1399–404
Results During the 7-year study period, 165 infants were admitted to the cardiac intensive care units with TOF. Two patients received comfort care from birth (1 with trisomy 13 and 1 with trisomy 18) and were removed from the analysis. Baseline characteristics of the remaining 163 patients are shown in Table 1. Of the entire cohort, 43 (26%) had a hypercyanotic “Tet” spell during the first year of life. Of the 163 patients, 10 of 163 (6%) died before undergoing a total repair: 4 died because of complications from their genetic abnormalities and 6 died because of other complications before surgical repair.
Neonatal Total Repair Group Statistical Methods Data are represented as mean standard deviation (SD), medians with the interquartile range (IQR), or frequencies as appropriate. Weight is expressed as weightfor-age z score based on the World Health Organization (WHO) growth standards, whereby a z score of þ1 is equivalent to a weight 1 SD greater than the mean, and a z score of –1 is equivalent to a weight 1 SD less than the mean [2]. Associations between timing of surgical intervention (neonatal versus nonneonatal total repair) and continuous outcomes were investigated using a Student’s t test or a Mann-Whitney U test when appropriate. Associations between categorical variables were assessed using tests of proportion and the c2 distribution. Multivariable stepwise linear regression modeling was used to test if group assignment (neonatal versus nonneonatal total repair) remained an important predictor of weight-for-age z score at 1 year independent of other known predictors of weight.
Of the 163 patients, 36 (22%) had a neonatal total repair; 24 of 36 (64%) patients had ductal dependency at the time of operation. Of the 36 patients, 30 (83%) underwent a transannular patch (TAP) repair, and 6 (17%) had a conduit or homograft placed (Table 2). None had a pulmonary valvotomy or valve-sparing total repair.
Nonneonatal Total Repair Group Of the 163 patients, 127 (78%) had a nonneonatal total repair; 31 of 127 (24%) had ductal dependency and underwent an initial shunt procedure. Of the 127 patients in the nonneonatal total repair group, 104 of 127 (82%) underwent a total repair at our institution, 13 of 127 (10%) were lost to follow-up after the initial shunt procedure but before total repair, and 10 of 127 (8%) died before total repair. All 10 deaths in our cohort occurred in the nonneonatal total repair group, and none would be considered surgical mortalities by the Society of Thoracic Surgeons database criteria. Of the 104 patients who
Table 1. Baseline Characteristics Total Patients With TOF (N ¼ 163) Characteristic Male sex Prenatally diagnosed Ductal dependency Tet spells TOF/PA TOF/PA/MAPCAS TOF/AVC Genetic syndromes DiGeorge Trisomy 21 VACTERL Trisomy 13 GA (wk) BW (kg) BW z scores
n (%)
Neonatal Repair ¼ 36 n (%) or (mean SD)
Nonneonatal Repair ¼ 127 n (%) or (mean SD)
p Value
99 (61) 120 (74) 55 (32) 43 (26) 28 (17) 19 (12) 9 (6) 49 (30) 18 (11) 14 (9) 5 (3) 4 (3) 162 (99) 163 163
24 (67) 21 (58) 24 (67) 8 (22) 10 (28) 3 (8) 0 (0) 6 (17) 3 (8) 2 (6) 0 (0) 0 (0) 38.4 2.5 3.1 0.7 –0.7 1.6
75 (59) 99 (78) 31 (24) 35 (28) 18 (14) 16 (13) 9 (7) 43 (34) 15 (12) 12 (4) 5 (4) 4 (3) 37.4 1.6 2.8 0.7 –1.2 1.6
0.4 0.02 2 cardiac repairs in first year Required other hospitalizationsa Death before repair No cardiac intervention offered because of genetic syndrome No cardiac intervention offered because of other complications
n (%)
Neonatal Repair ¼ 36 n (%) (mean SD) or median [IQR]
Nonneonatal Repair ¼ 127 n (%) (mean SD) or median [IQR]
p Value
... ... ... ... ... 91 of 140 (65) 24 of 140 (17) 23 of 140 (16) 93 (57) 25 (18) 81 (52)
17.5 [11–24] 19 [12–27] 8.5 [5–12] 7.1 4.6 125 53 30 (83) 4 (11) 0 (0) 32 (89) 9 (25) 19 (53)
7 [0–15] 15.5 [3–28] 136 [62–210] 5.1 6.6 124 77 61 (59) 20 (19) 23 (22) 61 (48) 16 (15) 62 (52)
