Three-Sinus Repair of Elastin Arteriopathy Associated Supravalvar Pulmonary Stenosis With Bilateral Branch Pulmonary Artery Involvement Ed Peng, FRCS(CTh), David N. Campbell, MD, James Jaggers, MD, and Max B. Mitchell, MD Department of Cardiac Surgery, Children’s Hospital Colorado and University of Colorado Denver School of Medicine, Aurora, Colorado
Elastin arteriopathy–associated supravalvar pulmonary stenosis is characterized by a thick intraluminal ringlike obstruction at the sinotubular junction of the pulmonary valve. Extension of disease into the branch pulmonary arteries is common. A three-sinus pulmonary trunk augmentation combined with bilateral branch pulmonary
augmentation is presented. This approach normalizes the pulmonary trunk and allows optimal augmentation of the central pulmonary arteries.
S
origin of the right PA (Fig 1). The pulmonary trunk is retracted anteriorly, and the entire arterioventricular junction is exposed. Vertical incisions are made at the midpoint of each sinus and extended to within 1–2 mm of the annulus (Fig 2). Typically, the distal 2–3 mm of arterial wall at and above the commissures is very thick, with a transition to a more normal artery proximally. Thick luminal tissue above each commissure is thinned using a fine scalpel (Fig 1). The annulus is measured with a Hegar dilator, and the dimension of patches required for each sinus is estimated, adding 2 mm to allow for reduced width after suturing. Each sinus is augmented with shield-shaped patches of pulmonary homograft using continuous running 7-0 monofilament suture beginning at the nadir of the left-facing sinus (Fig 2). Thereafter, each branch PA is incised longitudinally with extension beyond the diseased artery into the lobar branches if necessary. Mobilization, aortic traction, and division of the ligamentum arteriosum facilitate exposure of the branch PAs. Separate patches of pulmonary artery homograft are used to augment the branch pulmonary arteries (Fig 3). Lastly, the reconstructed pulmonary trunk and bifurcation are reconnected using continuous suture aligning the patches on the pulmonary trunk with the branch patches to preserve growth potential. If additional circumference at the bifurcation is required to accommodate the reconstructed trunk, short longitudinal incisions are made into the midpoint of the branch patches, a maneuver that also helps to avoid buckling after weaning from CPB.
Technique After median sternotomy, the pulmonary trunk is separated from the ascending aorta. The ligamentum arteriosum is divided, and each branch PA is mobilized beyond the upper lobe branch. Cardiopulmonary bypass (CPB) is established with aortic and bicaval cannulation. If subvalvar right ventricular muscular obstruction is present, infundibulotomy with patch augmentation is performed during cardioplegic arrest. Otherwise, the SVPS repair is performed on the beating heart. If concomitant SVAS repair is intended, SVPS repair is performed before cardioplegia delivery to minimize ischemic time in patients with biventricular hypertrophy who are particularly vulnerable to myocardial ischemia. Furthermore, aortic suture line bleeding obscures distal branch PA visualization if SVAS is repaired first. The external appearance of the pulmonary trunk may appear deceptively normal without a discrete waist at the sinotubular junction. Once CPB is established, PA wall thickening can be appreciated by occlusion with forceps. The pulmonary trunk is transected just proximal to the Accepted for publication Feb 18, 2015. Address correspondence to Dr Mitchell, Children’s Hospital Colorado’s Heart Institute, Anschutz Medical Campus, Aurora, CO 80045; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Comment SVPS is the least common form of pulmonary stenosis and is easily mistaken for primary valvar pathology, particularly in patients without Williams syndrome. SVPS should be suspected in patients with severe ostial branch pulmonary artery stenosis, and the diagnosis of 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.02.098
FEATURE ARTICLES
upravalvar pulmonary stenosis (SVPS) is a rare manifestation of elastin arteriopathy (EA). It can occur in all three forms of EA: Williams syndrome and sporadic and familial EA [1]. SVPS occurs in isolation or concomitantly with other EA-associated lesions, including supravalvar aortic stenosis (SVAS) and varying degrees of branch pulmonary artery (PA) stenosis. Repair techniques for SVAS are widely recognized with a strong recent bias favoring three-sinus repairs. In contrast, there are limited published descriptions for the repair of SVPS [2–4].
(Ann Thorac Surg 2015;100:741–3) Ó 2015 by The Society of Thoracic Surgeons
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HOW TO DO IT PENG ET AL THREE-SINUS REPAIR FOR SUPRAVALVAR PULMONARY STENOSIS
Ann Thorac Surg 2015;100:741–3
FEATURE ARTICLES
Fig 1. (Left) Angiogram demonstrates supravalvar pulmonary stenosis with severe bilateral branch pulmonary artery (PA) disease in a 4-monthold child with sporadic elastin arteriopathy and suprasystemic right ventricular pressure. A blind ending ductus ampulla is a common feature in patients with bilateral branch PA involvement (arrow). (Middle) Divided pulmonary trunk demonstrates extreme thickening of the arterial wall (external diameter 10 mm, internal diameter 4 mm). (Right) Thickened arterial wall is thinned at the pulmonary artery bifurcation.
EA should be considered. SVPS is anatomically and pathologically analogous to SVAS, but occurs with much lower frequency. Operations devised for SVAS are conceptually simple because of the direct distal trajectory and length of the ascending aorta. In contrast, the bifurcation of pulmonary trunk is in close proximity to the sinotubular junction (particularly on the right), and branching occurs at right angles to the trunk. The PA ostia are frequently involved with SVPS, as is distal extension of disease. Consequently, reconstruction of SVPS with ostial and distal branch involvement is more difficult. There is an extremely limited literature on SVPS. Milo described nine patients without recognizing the association with EA and recommended simple valvectomy [5]. The Boston group reported two patients who underwent repair using an inverted Y-shaped patch similar to the Doty repair for SVAS [2]. This repair is suitable only for patients in whom disease does not extend into the branch PA ostia. Bacha described an all-autologous repair of
Fig 2. (Left) The thickened wall of each sinus is incised, and a shieldshape patch of pulmonary homograft is used to augment each sinus. (Right) Completion of three-sinus repair. The main PA is reconstructed with three separate patches to provide symmetrical augmentation.
SVPS, similar to the Myer’s repair of SVAS [4]. This technique is also only suitable for disease confined to the pulmonary trunk. In our experience, such patients are uncommon. Of 11 patients operated at our center since 2005, only one patient did not have ostial branch PA involvement. More recently, Sojak published a case report of simultaneous three-sinus repairs of SVAS and SVPS in a patient with bilateral ostial PA involvement [3]. These authors extended the patches used to augment the rightfacing and nonfacing sinuses directly into the respective branch arteries. We have used similar patch extensions in patients with two-sinus augmentations. In our view, sizing of these patches is more difficult, and buckling can lead to residual obstruction. In our experience, extensive bilateral branch PA disease occurs commonly with SVPS (55% cases). Our technique simplifies the distal reconstructions. We have used this technique in the most recent four consecutive patients (2012–2015), all of whom required bilateral hilar level reconstructions. Pulmonary homograft patches were used preferentially because of its superior handling flexibility and better tissue match. All four patients were well on follow-up with reduction of peak Doppler-estimated outflow tract gradients from a
Fig 3. In the most severe form of elastin arteriopathy, extensive bilateral branch PA arterioplasty is performed. Branch PA plasty is extended to or beyond the diseased artery into the lobar branches as needed. The upper lobar branches were augmented with separate patches to obtain better patch configuration.
Ann Thorac Surg 2015;100:741–3
HOW TO DO IT PENG ET AL THREE-SINUS REPAIR FOR SUPRAVALVAR PULMONARY STENOSIS
mean of 4.8 m/s preoperatively to 1.7 m/s postoperatively with mild (n ¼ 1) or no (n ¼ 3) pulmonary valve regurgitation. Finally, our technique normalizes the anatomy of pulmonary trunk, potentially affording the same advantages commonly ascribed to three-sinus repairs for SVAS.
References 1. Mitchell MB, Goldberg SP. Supravalvar aortic stenosis in infancy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011;14:85–91.
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2. Stamm C, Friehs I, Moran AM, et al. Surgery for bilateral outflow tract obstruction in elastin arteriopathy. J Thorac Cardiovasc Surg 2000;120:755–63. 3. Sojak V, Koolbergen D, Filippini L, Hazekamp M. Simultaneous repair of supravalvar aortic and supravalvar pulmonary stenosis using the 3-patch technique. World J Pediatr Congenit Heart Surg 2010;1:404–6. 4. Bacha EA, Kalimi R, Starr JP, Quinones J, Koenig P. Autologous repair of supravalvar pulmonic stenosis. Ann Thorac Surg 2004;77:734–6. 5. Milo S, Fiegel A, Shem-Tov A, Neufeld HN, Goor DA. Hourglass deformity of the pulmonary valve: a third type of pulmonary valve stenosis. Br Heart J 1988;60:128–33.
FEATURE ARTICLES
Elastin arteriopathy–associated supravalvar pulmonary stenosis is characterized by a thick intraluminal ringlike obstruction at the sinotubular junction of the pulmonary valve. Extension of disease into the branch pulmonary arteries is common. A three-sinus pulmonary trunk augmentation combined with bilateral branch pulmonary
augmentation is presented. This approach normalizes the pulmonary trunk and allows optimal augmentation of the central pulmonary arteries.
S
origin of the right PA (Fig 1). The pulmonary trunk is retracted anteriorly, and the entire arterioventricular junction is exposed. Vertical incisions are made at the midpoint of each sinus and extended to within 1–2 mm of the annulus (Fig 2). Typically, the distal 2–3 mm of arterial wall at and above the commissures is very thick, with a transition to a more normal artery proximally. Thick luminal tissue above each commissure is thinned using a fine scalpel (Fig 1). The annulus is measured with a Hegar dilator, and the dimension of patches required for each sinus is estimated, adding 2 mm to allow for reduced width after suturing. Each sinus is augmented with shield-shaped patches of pulmonary homograft using continuous running 7-0 monofilament suture beginning at the nadir of the left-facing sinus (Fig 2). Thereafter, each branch PA is incised longitudinally with extension beyond the diseased artery into the lobar branches if necessary. Mobilization, aortic traction, and division of the ligamentum arteriosum facilitate exposure of the branch PAs. Separate patches of pulmonary artery homograft are used to augment the branch pulmonary arteries (Fig 3). Lastly, the reconstructed pulmonary trunk and bifurcation are reconnected using continuous suture aligning the patches on the pulmonary trunk with the branch patches to preserve growth potential. If additional circumference at the bifurcation is required to accommodate the reconstructed trunk, short longitudinal incisions are made into the midpoint of the branch patches, a maneuver that also helps to avoid buckling after weaning from CPB.
Technique After median sternotomy, the pulmonary trunk is separated from the ascending aorta. The ligamentum arteriosum is divided, and each branch PA is mobilized beyond the upper lobe branch. Cardiopulmonary bypass (CPB) is established with aortic and bicaval cannulation. If subvalvar right ventricular muscular obstruction is present, infundibulotomy with patch augmentation is performed during cardioplegic arrest. Otherwise, the SVPS repair is performed on the beating heart. If concomitant SVAS repair is intended, SVPS repair is performed before cardioplegia delivery to minimize ischemic time in patients with biventricular hypertrophy who are particularly vulnerable to myocardial ischemia. Furthermore, aortic suture line bleeding obscures distal branch PA visualization if SVAS is repaired first. The external appearance of the pulmonary trunk may appear deceptively normal without a discrete waist at the sinotubular junction. Once CPB is established, PA wall thickening can be appreciated by occlusion with forceps. The pulmonary trunk is transected just proximal to the Accepted for publication Feb 18, 2015. Address correspondence to Dr Mitchell, Children’s Hospital Colorado’s Heart Institute, Anschutz Medical Campus, Aurora, CO 80045; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
Comment SVPS is the least common form of pulmonary stenosis and is easily mistaken for primary valvar pathology, particularly in patients without Williams syndrome. SVPS should be suspected in patients with severe ostial branch pulmonary artery stenosis, and the diagnosis of 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2015.02.098
FEATURE ARTICLES
upravalvar pulmonary stenosis (SVPS) is a rare manifestation of elastin arteriopathy (EA). It can occur in all three forms of EA: Williams syndrome and sporadic and familial EA [1]. SVPS occurs in isolation or concomitantly with other EA-associated lesions, including supravalvar aortic stenosis (SVAS) and varying degrees of branch pulmonary artery (PA) stenosis. Repair techniques for SVAS are widely recognized with a strong recent bias favoring three-sinus repairs. In contrast, there are limited published descriptions for the repair of SVPS [2–4].
(Ann Thorac Surg 2015;100:741–3) Ó 2015 by The Society of Thoracic Surgeons
742
HOW TO DO IT PENG ET AL THREE-SINUS REPAIR FOR SUPRAVALVAR PULMONARY STENOSIS
Ann Thorac Surg 2015;100:741–3
FEATURE ARTICLES
Fig 1. (Left) Angiogram demonstrates supravalvar pulmonary stenosis with severe bilateral branch pulmonary artery (PA) disease in a 4-monthold child with sporadic elastin arteriopathy and suprasystemic right ventricular pressure. A blind ending ductus ampulla is a common feature in patients with bilateral branch PA involvement (arrow). (Middle) Divided pulmonary trunk demonstrates extreme thickening of the arterial wall (external diameter 10 mm, internal diameter 4 mm). (Right) Thickened arterial wall is thinned at the pulmonary artery bifurcation.
EA should be considered. SVPS is anatomically and pathologically analogous to SVAS, but occurs with much lower frequency. Operations devised for SVAS are conceptually simple because of the direct distal trajectory and length of the ascending aorta. In contrast, the bifurcation of pulmonary trunk is in close proximity to the sinotubular junction (particularly on the right), and branching occurs at right angles to the trunk. The PA ostia are frequently involved with SVPS, as is distal extension of disease. Consequently, reconstruction of SVPS with ostial and distal branch involvement is more difficult. There is an extremely limited literature on SVPS. Milo described nine patients without recognizing the association with EA and recommended simple valvectomy [5]. The Boston group reported two patients who underwent repair using an inverted Y-shaped patch similar to the Doty repair for SVAS [2]. This repair is suitable only for patients in whom disease does not extend into the branch PA ostia. Bacha described an all-autologous repair of
Fig 2. (Left) The thickened wall of each sinus is incised, and a shieldshape patch of pulmonary homograft is used to augment each sinus. (Right) Completion of three-sinus repair. The main PA is reconstructed with three separate patches to provide symmetrical augmentation.
SVPS, similar to the Myer’s repair of SVAS [4]. This technique is also only suitable for disease confined to the pulmonary trunk. In our experience, such patients are uncommon. Of 11 patients operated at our center since 2005, only one patient did not have ostial branch PA involvement. More recently, Sojak published a case report of simultaneous three-sinus repairs of SVAS and SVPS in a patient with bilateral ostial PA involvement [3]. These authors extended the patches used to augment the rightfacing and nonfacing sinuses directly into the respective branch arteries. We have used similar patch extensions in patients with two-sinus augmentations. In our view, sizing of these patches is more difficult, and buckling can lead to residual obstruction. In our experience, extensive bilateral branch PA disease occurs commonly with SVPS (55% cases). Our technique simplifies the distal reconstructions. We have used this technique in the most recent four consecutive patients (2012–2015), all of whom required bilateral hilar level reconstructions. Pulmonary homograft patches were used preferentially because of its superior handling flexibility and better tissue match. All four patients were well on follow-up with reduction of peak Doppler-estimated outflow tract gradients from a
Fig 3. In the most severe form of elastin arteriopathy, extensive bilateral branch PA arterioplasty is performed. Branch PA plasty is extended to or beyond the diseased artery into the lobar branches as needed. The upper lobar branches were augmented with separate patches to obtain better patch configuration.
Ann Thorac Surg 2015;100:741–3
HOW TO DO IT PENG ET AL THREE-SINUS REPAIR FOR SUPRAVALVAR PULMONARY STENOSIS
mean of 4.8 m/s preoperatively to 1.7 m/s postoperatively with mild (n ¼ 1) or no (n ¼ 3) pulmonary valve regurgitation. Finally, our technique normalizes the anatomy of pulmonary trunk, potentially affording the same advantages commonly ascribed to three-sinus repairs for SVAS.
References 1. Mitchell MB, Goldberg SP. Supravalvar aortic stenosis in infancy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011;14:85–91.
743
2. Stamm C, Friehs I, Moran AM, et al. Surgery for bilateral outflow tract obstruction in elastin arteriopathy. J Thorac Cardiovasc Surg 2000;120:755–63. 3. Sojak V, Koolbergen D, Filippini L, Hazekamp M. Simultaneous repair of supravalvar aortic and supravalvar pulmonary stenosis using the 3-patch technique. World J Pediatr Congenit Heart Surg 2010;1:404–6. 4. Bacha EA, Kalimi R, Starr JP, Quinones J, Koenig P. Autologous repair of supravalvar pulmonic stenosis. Ann Thorac Surg 2004;77:734–6. 5. Milo S, Fiegel A, Shem-Tov A, Neufeld HN, Goor DA. Hourglass deformity of the pulmonary valve: a third type of pulmonary valve stenosis. Br Heart J 1988;60:128–33.
FEATURE ARTICLES
