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Midterm Outcomes of the Supported Ross Procedure in Children, Teenagers and Young Adults Kyle W. Riggs MD , Durham B. Colohan , Daniel R. Beacher MD , Tarek Alsaied MD , Shannon Powell MD , Ryan A. Moore MD , Salil Ginde MD , James S. Tweddell MD PII: DOI: Reference:
S1043-0679(19)30391-0 https://doi.org/10.1053/j.semtcvs.2019.10.020 YSTCS 1418
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Seminars in Thoracic and Cardiovascular Surgery
Please cite this article as: Kyle W. Riggs MD , Durham B. Colohan , Daniel R. Beacher MD , Tarek Alsaied MD , Shannon Powell MD , Ryan A. Moore MD , Salil Ginde MD , James S. Tweddell MD , Midterm Outcomes of the Supported Ross Procedure in Children, Teenagers and Young Adults, Seminars in Thoracic and Cardiovascular Surgery (2019), doi: https://doi.org/10.1053/j.semtcvs.2019.10.020
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Midterm Outcomes of the Supported Ross Procedure in Children, Teenagers and Young Adults Kyle W Riggs1 MD, Durham B Colohan,1 Daniel R. Beacher2 MD, Tarek Alsaied3,4 MD, Shannon Powell3 MD, Ryan A Moore2 MD, Salil Ginde2 MD, James S Tweddell1 MD 1
Cincinnati Children’s Hospital, Division of Cardiothoracic Surgery. 3333 Burnet Ave. Cincinnati,
OH 45229 2
Children’s Hospital of Wisconsin, Division of Pediatric Cardiology. 8915 W Connell Ct.
Milwaukee, WI 53226 3
Cincinnati Children’s Hospital, Division of Pediatric Cardiology. 3333 Burnet Ave. Cincinnati, OH
45229 4
University of Cincinnati College of Medicine, 3333 Burnet Ave. Cincinnati, OH 45229.
Conflict of Interest No authors have a conflict of interest. Corresponding Author: James S. Tweddell, MD, 3333 Burnet Ave., Cincinnati, OH 45229
[email protected] Business: 513-803-8824
Fax: 513-636-0100
This study was determined to be a non-human subjects research on 7/2/2018. Word Count: 2,319/3,500 (Intro-Conclusion) Read at the American Association for Thoracic Surgery 99th Annual Meeting, Toronto, Canada, May 4-7, 2019
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Central picture legend: Excellent freedom from root dilation beyond 10 years following supported Ross operation
Central Message: (<200) The supported Ross operation has demonstrated excellent mid-term support of the aortic root with minimal dilation and regurgitation and no mortality
Perspective: (<50 words) When faced with aortic valve pathology in older children, adolescents and young adults, the supported Ross operation offers excellent mid-term results without the need for life-long anticoagulation
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Abstract (250/250)
Objective: The Ross procedure in children, adolescents and young adults, especially among those with a bicuspid aortic valve with aortic regurgitation, has been associated neoaortic root dilatation and recurrence of aortic regurgitation. We have shown that at intermediate followup, patients who underwent a supported Ross technique were less likely to have neoaortic root dilatation. This study summarizes our most recent outcomes.
Methods: This was a retrospective review of 40 consecutive adolescent and young adult patients undergoing a supported Ross procedure from 2005-2018. Clinical outcomes were reviewed in addition to echocardiographic measures of neoaortic dimension and neoaortic valve function.
Results: The median age at surgery was 16.0 years [13.7-20.2]. Pre-operative diagnosis was aortic regurgitation in 15 (37.5%) and mixed regurgitation/stenosis in 20 (50%). Median followup was 3.5 years [1.4-5.6] with 3 patients followed for more than 10 years. There were no deaths. Five patients had a reintervention, but only one on the aortic valve. One patient returned to the operating room post-operative day one for revision of right coronary button. Two patient required biventricular pacemakers for reduced ejection fraction. One patient developed aortic regurgitation and underwent mechanical valve replacement and another required a re-intervention on the homograft with a percutaneously placed pulmonary valve replacement. At last follow-up, 39 patients had mild or less aortic regurgitation with median sinus z-score of 1.40[0.48-2.07].
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Conclusions: Mid-term follow-up of adolescent and young adult patients undergoing a supported Ross operation for various aortic valve pathologies demonstrate excellent mid-term results with minimal neo-aortic root dilation and re-intervention. Keywords: Ross operation; congenital; Supported Ross operation; teenagers; adults
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Introduction
Aortic valve replacement is commonly required in children, teenagers and young adults due to bicuspid semilunar valve pathology and replacement options remain a challenge. Replacement of the aortic valve with a pulmonary autograft was first described by Ross in 1967 and multiple studies have demonstrated excellent early and late survival.(1-6) However a subgroup of patients, those with a bicuspid aortic valve and aortic regurgitation, require reintervention due to progressive neoaortic dilatation, particularly at the sinotubular junction, with associated neoaortic regurgitation.(7-9) In order to prevent neoaortic root dilatation and preserve autograft function, the use of an externally supported Dacron tube graft similar to the technique used for valve sparing root reconstruction as described by Ungerleider and colleagues was instituted in 2005.(10) We first started using a sinus of Valsalva polyester tube graft designed to mimic the geometry of the normal aortic root (Gelweave sinus of Valsalva Graft, Terumo Cardiovascular Systems, Ann Arbor, MI) to provide external support to the neoaortic root. We have shown that compared to the standard Ross operation the supported Ross operation had less neoaortic root dilatation and aortic valve regurgitation at intermediate follow-up.(11) The supported Ross has continued to be our procedure of choice in older children, teenagers and young adults requiring aortic valve replacement. Herein, we report the mid-term outcomes of 40 sequential adolescents and young adults undergoing the supported Ross procedure.
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Methods
The Institutional Review Boards of the Cincinnati Children’s Hospital Medical Center and the Children’s Hospital of Wisconsin approved this study and waived the need for parental or patient consent. Patients The cardiovascular surgical databases of the participating institutions were used to identify patients who had previously undergone a supported Ross procedure from January 2005 to June 2018. Follow-up period was through March 2019. A single surgeon performed the Ross procedure in all 40 patients. The Ross procedure was performed as a root replacement and was performed as previously described and shown in Figure 1.(11) Data were extracted from the medical record and included preoperative, operative, and postoperative variables. Echocardiograms were reviewed offline from the pre-operative period, time of discharge, and at most recent follow-up. When dilation of the root was noted on most recent echocardiogram, prior echocardiograms were reviewed to identify the first known time of dilation. Echocardiographic Measurements: Absolute dimensions of the aortic and neoaortic annulus, aortic sinuses, sinotubular junction, and ascending aorta were measured for each echocardiogram. The maximum systolic dimensions of the aortic and neoaortic annulus (measured at the level of valve hinge points of the leaflets), aortic sinuses (at the mid-sinus level of the native or neo-aortic tissue) and ascending aorta (at the level of the right pulmonary artery) were measured from the twodimensional parasternal long-axis views. The indices for these measurements were calculated
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from the patient’s body surface area by using the formula by Haycock and colleagues.(12) The z- scores for these diameters were determined using standardized data.(13) The degree of aortic stenosis was determined in the 4-chamber apical and suprasternal views on all available echocardiograms. Mild stenosis correlated with a peak jet velocity less than 3 m/s or a mean gradient of less than 25 mm Hg, moderate stenosis with a peak velocity of 3 to 4 m/s or a mean gradient of 25 to 40 mm Hg, and severe stenosis with a peak velocity exceeding 4 m/s or a mean gradient exceeding 40 mm Hg. The degree of valve regurgitation was assessed by measurement of the vena contracta, measured in the parasternal long-axis views.(14) A color Doppler jet width of less than 4 mm indicated trivial to mild aortic regurgitation, 4 to 6 mm indicated moderate regurgitation, and >6 mm indicated severe regurgitation. Moderate and severe regurgitation was also verified by the presence of left ventricular dilatation and holodiastolic flow reversal in the descending or abdominal aorta, or both. The absolute systolic and diastolic dimensions of the left ventricle were measured to determine the presence and degree of left ventricular dilatation and left ventricular hypertrophy. A left ventricular end-systolic dimension index of 19 to 22 mm/m2 was considered mild dilatation, 22 to 25 mm/m2 or larger was moderate dilatation, and more than 25 mm/m2 was severe dilatation. The Canadian Society of Echocardiography left ventricular mass and left ventricular mass index calculator, which uses the patient’s height, weight, sex, left ventricular end-diastolic diameter, posterior wall diameter, and interventricular septal diameter, was used to determine the presence and degree of left ventricular hypertrophy. The left ventricular measurements were obtained in the parasternal short-axis views at the level of the papillary muscles in all available 7
preoperative and postoperative echocardiograms. Left ventricular function, using both the parasternal short-axis and 4- chamber apical views, was calculated in all available echocardiograms. A single advanced imaging cardiology fellow and attending cardiologist from each institution reviewed all of their institutional echocardiograms.
Data Outcomes End-points included survival, cardiac reintervention and aortic dimensions. Echocardiographic measurements of neoaortic root dimensions, neoaortic valve regurgitation, degree of left ventricular dilatation, and residual lesions were analyzed on previously defined available echocardiograms throughout the entire follow-up period. Significant neoaortic dilatation was defined as a neoaortic root z-score exceeding 2.5. We chose 2.5 as the cutoff to exclude patients with borderline dilatation (z-scores of 2 to 2.5). A z-score greater than 4 was considered greater than mild dilation. Significant neoaortic valve regurgitation was defined as more than mild regurgitation. Statistical Analysis Continuous data are presented as median [inter-quartile range] and categorical variables as n(%). Follow-up echocardiographic measurements are compared using paired sample T-test. Time to neo-aortic root dilation is demonstrated by Kaplan-Meier curve. Data analysis was performed using SPSS (IBM, Armonk, NY, version 24). Results There were 40 patients who underwent a supported Ross. Most of the patients were males (28 [70%]) with a median age of 16 years-old [14-20] at time of supported Ross operation as seen in 8
Table 1. Over half of the patients had a prior intervention (23 [57.5%]). Three patients had prior aortic valve replacements, one for endocarditis. The most common pathologic diagnosis was bicuspid aortic valve (37 [92.5%]) and indications for surgery included 15 patients (37.5%) with aortic regurgitations, 5 (12.5%) with aortic stenosis, and 20 (50.0%) with mixed regurgitation and stenosis. Echocardiographic measurements at time of Ross operation are displayed in Table 1. Most patients (87%) had moderate or severe aortic regurgitation with only 2 having left ventricular dysfunction.
Outcomes and follow-up of the patients are presented in Table 2. Median pulmonary homograft size was 29 mm [28-30 mm] while Sinus of Valsalva graft size was 28 mm [28-30 mm]. Enlargement of the aortic annulus using an anterior aortoventriculoplasty (Konno) operation was performed in 16 (40%) of the patients. Median length of follow-up was 3.5 years [1.4-5.6 years] with 3 patients followed over 10 years. Post-operatively, 3 (7.5%) patients had a sinus z-score >2.5. Two patients normalized their z-score during the follow-up period. One of them underwent replacement of the autograft valve with a mechanical aortic valve replacement with aorto-ventriculoplasty for aortic regurgitation without dilation at 10 years post-operatively. The third patient remains dilated >7 years post-operatively. Notably, these patients were all <13 years old at the time of their supported Ross operation. Three more patients developed root dilation over the course of the study period at 94 days, 1.2 years and 3.6 years, Figure 2. At most recent follow-up, 87.5% of patients had normal left ventricular systolic function. Figure 3 shows the change in the z-score for the aortic annulus, sinus,
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sinotubular junction, and ascending aorta with stable z-scores for all categories. Of note, no patient had more than mild aortic root dilation at the most recent follow-up.
There were no mortalities, but 5 (12.5%) patients required a re-intervention on the cardiovascular system. One patient underwent an early post-operative revision of a coronary button. Two patients had biventricular pacemakers placed at 3 and 8 months post-operatively. Another patient underwent implantation of a Melody valve (Medtronic, Dublin, Ireland) in the pulmonary position 10 years after supported Ross, and the final patient had a mechanical aortic valve replacement and aorto-ventriculoplasty along with a right ventricle to pulmonary artery conduit replacement 10 years after supported Ross. The aortic root was not dilated at the time of the reoperation in this patient. The biventricular pacemakers were placed in two patients who developed post-operative left bundle branch blocks and ventricular dysfunction. The third patient returned to the operating room on post-operative day one for revision of the connection of the right coronary artery to the autograft. This patient tolerated the reoperation and is doing well at last follow-up. One patient required a Melody valve for progressive right ventricle to pulmonary artery conduit stenosis with good result. Lastly, a patient developed regurgitation at 10 years post-operatively which was repaired by another surgeon and we have minimal details on this patient. They elected to perform a mechanical aortic valve replacement with anterior aortoventriculoplasty (Konno) operation. The right ventricle to pulmonary conduit was stenotic and replaced at that time too.
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Discussion Although technically more demanding, large population based studies from the United Kingdom, the Netherlands and Germany show the Ross procedure to have better survival and freedom from reintervention in children, adolescents, young adults and even middle-aged adults.(4, 5, 15-17) In addition to the increased complexity of the operation there has been concern about the development of neoaortic root dilatation with development of aortic regurgitation. This is more common among patients with bicuspid aortic valve pathology with aortic regurgitation as the hemodynamic indication for surgery.(7-9) Bicuspid aortic valve pathology with regurgitation is a common substrate encountered in North America. In order to prevent dilatation and preserve autograft function we began performing the supported Ross procedure in 2005. This report summarizes our longitudinal follow-up series of 40 children, teenagers and young adults undergoing supported Ross over 12 years. We found the supported Ross technique to provide excellent mid-term support to the aortic root with minimal dilation and a low recurrence of aortic regurgitation. Importantly, there have been no mortalities in this young cohort and only one repeat intervention on the neo-aortic valve. Patient size is an important consideration when selecting patients for the supported Ross technique. We have previously shown the supported Ross to be superior to the standard Ross operation in preventing dilation of the aortic root.(11) The drawback of the supported Ross is the loss of growth potential. In order to prevent a patient outgrowing their autograft, we have been reluctant to use a sinus of Valsalva graft smaller than 28 mm, but two factors permit extension of the supported Ross to younger patients who have not completed their somatic growth, some as young as 10 years of age. First, The pulmonary autograft is slightly 11
larger than the normal sized aortic root, allowing for application of the supported Ross to patients slightly less than “full grown” size. In addition, there is acute enlargement of the pulmonary autograft when subjected to systemic pressure. In these cases, the supported Ross autograft is initially larger than normal for the child or younger teenager, but with time patients “grow into” their new aortic root as seen by a decrease in their z-score. Figure 4 demonstrates two examples of this in patients whose z-score was initially >2.5 but normalized after 7-10 years of follow-up when they were nearly full grown. These patient were 10 and 12 years old which is why we were comfortable “over-sizing” their root at the time of their operation. Second, the autograft can be placed in a slightly oversized sinus of Valsalva graft. While initially, the autograft may not be in apposition to the sinus of Valsalva graft, over time the autograft enlarges to meet the walls of the sinus of Valsalva graft. This phenomenon accounts for the apparent dilatation of the aortic root to a z-score >2.5 months to years after supported Ross in a few individuals as demonstrated by Figure 5. While the autograft root may dilate, the sinus of Valsalva graft does not. In these patients, there is initially, space between the neoaortic root sinus and the sinuses of Valsalva graft. However, at follow-up echocardiogram, this space is obliterated by the dilation of the autograft until it reaches the internal border of the sinus of Valsalva graft. Comparison between aortic root diameter at late follow-up to diameter of sinus of Valsalva graft post-operatively show that they are identical. In these patients, despite the fact that the neoaortic root is slightly larger than normal, the sinus of Valsalva graft preserves the relationship of the annulus, sinuses and sinotubular junction and preserves autograft function. For these reasons, we generally consider patients with an autograft of at
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least 25 mm eligible for a modified supported Ross which has general made the lower age limit 10-12 years though age is not the most important factor. Our report is unique in that a sinus of Valsalva graft has been used exclusively which we believe is more anatomically correct and allows for normal neoaortic leaflet excursion, reducing the risk of leaflet damage that can be seen in other techniques, particularly those using a straight Dacron tube graft.(6, 10) We have generally used a sinus of Valsalva graft which is sized to be 4 mm larger than the autograft annular dimension as determined by direct measure with Hagar dilators at the time of surgery. Other techniques have used autologous tissue to wrap the neo-aortic root, but this may still be susceptible to long-term dilation and has been reported to have 5% reintervention for aortic regurgitation.(9) Additionally, we believe that this technique obviates the need for strict blood pressure management post-operatively which has been advocated for by some groups to prevent dilation. Limitations This is a single-center retrospective review of a modest number of patients operated on over a 13 year period with all of the limitations of that study design. Conclusions Mid-term follow-up of older children, adolescents and young adults undergoing a supported Ross operation with a sinus of Valsalva graft for various aortic valve pathologies demonstrate excellent mid-term results with minimal neo-aortic root dilation and reintervention. This technique should be the preferred method of repair in adolescents and young adults of adequate size without contraindications.
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Webcast: You can watch a Webcast of this AATS meeting presentation by going to: https://aats.blob.core.windows.net/media/19%20AM/Saturday_May4/202BD/202BD/S16%20%20Surgical%20Management%20of%20Aortic/S16_5_webcast_105644083.mp4
Acknowledgements We would like to thank Christine Sulok for the illustration of Figure 1.
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Tables Table 1:
Table 1. Patient Characteristics at Time of Ross Externally Supported Ross Variables (n = 40) Gender (female) 12 (30%) Age at Ross (years) 16.0 [13.7-20.2] Height, cm 168 [157-176] Weight, kg 62.8 [52.3-72.0] Body Surface Area, kg/m2 1.72 [1.55-1.88] Diagnosis of Bicuspid Aortic Valve 37 (92.5%) Hemodynamic Indications Aortic Regurgitation 15 (37.5%) Aortic Stenosis and Regurgitation 20 (50.0%) Previous Intervention 23 (57.5%) Prior Aortic Valve Replacement 3 (7.5%) Aortic Annulus at Ross Diameter, mm 23.0 [21.0-25.7] Index, mm/m2 14.6 [12.4-16.2] z-score 2.37 [0.49-3.91] Aortic Sinus at Ross Diameter, mm 29.1 [26.1-32.7] Index, mm/m2 17.7 [14.3-20.9] z-score 1.35 [-0.77-2.01] ST Junction at Ross Diameter, mm 25.0 [22.9-30.8] Index, mm/m2 15.8 [12.9-18.0] z-score 2.23 [-0.07-3.57] Ascending Aorta at Ross Diameter, mm 33.6 [28.5-40.0] Index, mm/m2 20.7 [17.3-22.6] z-score 3.24 [1.92-5.63] >Mild Degree Aortic Regurgitation 33 (87.5%) LV Ejection Fraction 65% [62%-70%] LV Systolic Function Normal (>55%) 38 (95%) Mildly Diminished (45-55%) 2 (5%) >Mild LV Dilation 15 (37.5%) 15
Continuous data are presented as median [interquartile range]. EF, ejection fraction; LV, left ventricle; ST sinotubular.
Table 2.
Table 2. Supported Ross Outcomes Externally Supported Ross (n=40) 28 [28-30] 29 [28-30]
Variable Sinus of Valsalva Graft, mm Pulmonary Homograft, mm Discharge Echocardiogram Aortic Sinus z-score 1.59 [0.69-1.95] Dilated Root 3 (7.5%) Most Recent Echocardiogram Aortic Sinus z-score 1.40 [0.48-2.07] Aortic Sinus z-score > 2.5 4 (10.0%) >Mild Degree Aortic Regurgitation 1 (2.5%) LV Systolic Function Normal (>55%) 35 (87.5%) >Mild LV Dilation 3 (7.5%) Length of Follow-up, years 3.5 [1.4-5.6] Re-interventions 5 (12.5%) Mortality 0 (0%) Continuous data are presented as median [interquartile range]; LV, left ventricle.
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Figure Legends
Figure 1: Surgical technique for the supported Ross procedure. A. After establishing cardiopulmonary bypass the aorta is cross clamped and the heart is arrested with cardioplegia. B. A portion of the sinus aorta surrounding the coronary ostia (coronary buttons) are excised and the proximal coronary arteries are mobilized. The pulmonary autograft is harvested and implanted in a sinus of Valsalva graft. In general, the sinus of Valsalva graft is equal to 4 mm plus the diameter of the pulmonary autograft annulus. C. The pulmonary autograft is implanted within the sinus of Valsalva graft. The sinus of Valsalva graft is trimmed to within 2-3 rings of the sinus portion of the graft. The distal end of the pulmonary autograft is trimmed so that the distance from the tops of the commissure to the autograft edge is equal at around 10mm for all three commissures. Running 5-0 polypropylene suture is used to secure the 17
proximal muscular collar of the autograft to the sinus of Valsalva graft and the distal ends are tacked at each commissure and halfway between each commissure with interrupted 5-0 polypropylene suture. D. The supported pulmonary autograft is positioned so that the midportion of one of the sinuses faces the left coronary artery button. The proximal suture line securing the supported pulmonary autograft to the left ventricular outflow tract is performed using running 4-0 polypropylene suture. E. The coronary buttons are implanted using running 5-0 polypropylene suture incorporating all three layers; the pulmonary autograft, sinus of Valsalva graft and the coronary button. Typically, the distal end of the right ventricle to pulmonary artery conduit is performed before completion of the anastomosis between the supported pulmonary autograft and the ascending aorta. F. The anastomosis between the supported pulmonary autograft and the ascending aorta is completed. Then the proximal right ventricle to pulmonary artery conduit anastomosis is completed.
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Figure 2: Kaplan-Meier curve demonstrating the excellent freedom from dilatation of the aortic root, based on a z-score < 2.5, in patients undergoing a supported Ross operation. Number of patients with follow-up data is shown with 2 followed greater than 12 years.
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Figure 3: Comparison of z-score measurements of the aortic annulus, aortic sinus, sinotubular junction, and ascending aorta from time of discharge to most recent follow-up demonstrating a stable aortic size over time. AAo, ascending aorta; STJ, sinutubular junction.
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Figure 4: Chart demonstrating the change in aortic root z-score in relation to BSA in two patients who “grew” into their supported Ross operation. The red dots represents discharge data with z-score > 2.5 and the green dots represent follow-up data where z-score has normalized. Echocardiographic measurements are separated by 7 years (orange) and 10 years (blue) with patients being nearly fully grown at time of green dot. BSA, body surface area.
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Figure 5: Demonstration of gap between native tissue sinus and the sinuses of the Valsalva graft indicated by red arrows at discharge (left) and at follow-up (right) demonstrating obliteration of the space with dilation of the native tissue which is halted at the diameter of the Gelweave graft.
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Jacobsen RM, Earing MG, Hill GD, Barnes M, Mitchell ME, Woods RK, et al. The Externally Supported Ross Operation: Early Outcomes and Intermediate Follow-Up. Ann Thorac Surg. 2015;100:631–8. 12. Haycock GB, Schwartz GJ, Wisotsky DH. Geometric method for measuring body surface area: a height-weight formula validated in infants, children, and adults. J Pediatr 1978;93: 62–6.
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13. SluysmansT, ColanSD. Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol. 2005;99:445–57. 14. Jenkins KJ, Hanley FL, Colan SD, Mayer JE Jr, Castaneda AR, Wernovsky G. Function of the anatomic pulmonary valve in the systemic circulation. Circ. 1991;84(5 Suppl):III173–9. 15. Ouzounian M, Mazine A, David TE. The Ross procedure is the best operation to treat aortic stenosis in young and middle-aged adults. J Thorac Cardiovasc Surg. 2017;154:778–82. 16. David TE. Aortic Valve Replacement with Pulmonary Autograft: Subcoronary and Aortic Root Inclusion Techniques. Oper Tech Thorac Cardiovasc Surg. 2012;17:27–40. 17. Juthier F, Banfi C, Vincentelli A, Ennezat P-V, Le Tourneau T, Pinçon C, et al. Modified Ross operation with reinforcement of the pulmonary autograft: Six-year results. J Thorac Cardiovasc Surg. 2010;139:1420–3.
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