Repair Of Supravalvular Aortic Stenosis Research Articles

Long-Term Outcomes of Individualized Repair in Patients with Supravalvular Aortic Stenosis.

Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus on the optimal approach. This study aimed to analyze the outcomes of surgical treatment of SVAS and determine the optimal strategy. The Kaplan-Meier curve was used to demonstrate the survival estimates. The Cox proportional hazard model was used to identify risk factors for residual aortic stenosis and residual stenosis-related reintervention. From December 2008 to December 2023, 98 patients with SVAS undergoing surgical repair in our institution were included [McGoon group, n = 62; Doty group, n = 36]. There were 2 in-hospital deaths and 1 late death. The survival rates at 1, 5, and 15years were 98.0%, 96.7%, and 96.7%, respectively in the whole cohort. Residual aortic stenosis occurred in 18 patients. Multivariable analysis showed that preoperative gradient ≥ 90mmHg (P = 0.002) and Williams syndrome (P = 0.002) were incremental risk factors for residual aortic stenosis, but surgical technique (P = 0.579) was not a risk factor for residual aortic stenosis. In the McGoon group, patients with diffuse type had worse freedom from residual aortic stenosis than patients with discrete type (P = 0.007). However, in the Doty group, patients with diffuse type had comparable freedom from residual aortic stenosis to patients with discrete type (P = 0.911). Residual stenosis-related reintervention occurred in 15 patients. Fifteen patients all underwent residual aortic stenosis-related reintervention. Of 15 patients, 6 patients also underwent residual pulmonary stenosis-related reintervention. On multivariate analysis, Williams syndrome (P < 0.001), preoperative sinotubular junction (STJ) z-score < - 3.5 (P = 0.051), and Doty repair (P = 0.033) were found to be independent risk factors associated with residual stenosis-related reintervention. In the whole cohort, freedom from residual stenosis-related reintervention at 1, 5, and 15years were 97.8%, 89.3% and 76.1%, respectively. Surgical repair of SVAS can be safely achieved using different techniques, with similar long-term mortality. Compared with McGoon repair, Doty repair was significantly associated with decreased residual aortic stenosis rates in patients with diffuse-type SVAS. Patients with preoperative gradient ≥ 90mmHg or Williams syndrome are more prone to residual aortic stenosis. Surgical technique was not associated with residual aortic stenosis rates. Williams syndrome, preoperative STJ z-score < - 3.5, and Doty repair are associated with higher residual stenosis-related reintervention rates.

1226 The Role of Extracorporeal Membrane Oxygenation in William’s Syndrome Following Cardiac Surgery: A Case Report

Abstract Williams-Beuren Syndrome (WS) is a genetic syndrome seen in childhood, characterised by intellectual disabilities, outgoing personalities, elfin-like faces and cardiovascular complications. We report the story of a girl with WS who underwent complex cardiac surgery requiring extracorporeal membrane oxygenation (ECMO), to raise awareness of the perioperative challenges with WS. At 2 years old, a cardiac murmur was incidentally discovered. Further investigations revealed WS with cardiac manifestations including peripheral pulmonary artery stenosis (PPAS) and supravalvular aortic stenosis (SVAS). Isolated SVAS repair was feasible, however the severity of pulmonary artery hypoplasia deemed her inoperable. Her care was redirected to palliation. 18 months later, she remained in good clinical condition which prompted the multidisciplinary team (MDT) to change their decision and proceed with complex SVAS repair surgery (Brom technique) at the age of 5. Postoperatively, 3 failed attempts to wean from cardiopulmonary bypass preceded veno-arterial ECMO, which lasted 7 days. She stayed in intensive care for 17 days, and in hospital for 28 days. Frequent MDT decision making was crucial during her complex care. The patient is currently 15 years old, in good clinical condition, albeit exercise restrictions. WS patients have a known increased risk of perioperative sudden cardiac death and increasing use of paediatric ECMO, hence great attention and thorough preparation is required. Clear perioperative care guidelines for WS are essential to secure good outcomes in this patient population. This case demonstrated that MDT involvement and regular communication with the patient were key to deliver the appropriate care throughout her life.

Supravalvular aortic stenosis repair: surgical training of 2 repair techniques using 3D-printed models.

There are a number of techniques used in the surgical treatment of supravalvular aortic stenosis with excellent results; however, junior surgeons' experience may be limited due to its rare nature. Simulation with 3D-printed models provides a safe platform for surgeons to rehearse surgical techniques prior to patient application but must replicate all features of surgical anatomy. Here, we describe how software manipulation can be used to accurately reproduce the surgical anatomy in supravalvular aortic stenosis and be used to train surgeons in 2 different techniques.

Commentary: Sometimes the best solution is to simplify

Luo and colleagues1 describe their modified 2-patch technique for the repair of supravalvular aortic stenosis. In their modification, the authors perform a transverse aortic incision above the sinotubular junction and then make 2 vertical incisions into the non- and right-coronary sinuses. These 2 incisions are extended superiorly into the ascending aorta, forming a final H-like incision (hence the name H-repair). Following that, 2 separate rectangle-shaped pericardial patches are utilized to reconstruct the non- and right-coronary sinuses, with the sizes of the distal patches adjusted to achieve normal sinotubular junction diameter.

H-repair in supravalvular aortic stenosis.

H-repair in supravalvular aortic stenosis.

Midterm Results and Predictors for the Postoperative Vascular Stenosis of Supravalvular Aortic Stenosis

This study reviewed the midterm outcomes of supravalvular aortic stenosis (SVAS) repair and determined the risk factors associated with postoperative aortic or pulmonary stenosis. We retrospectively reviewed 225 patients who underwent surgical correction of SVAS from 2010 to 2019. A total of 178 (79.1%), 44 (19.6%) and 3 (1.3%) patients underwent McGoon, Doty, and Brom repair, respectively. The median age at surgery was 2.2 years (interquartile range, 1.2-4.4). The median follow-up time was 3.7 years (interquartile range, 1.9-5.7). Early and late mortality rates were 3.1% and 1.4%, respectively. The overall 5-year survival rate was 97.9%. Eleven patients received reintervention, including 6 (2.8%) reoperations and 5 (2.3%) balloon dilatations. Higher preoperative pressure gradient at the distal ascending aorta or aortic arch was a risk factor for reintervention (P = 0.04). Rates of mortality and complications were not related to the surgical technique. Eleven patients (5.2%) developed sinotubular junction (STJ) stenosis. Freedom from postoperative distal artery stenosis (DAS) of type II SVAS was significantly lower than that of type I (P < 0.01). Higher preoperative pressure gradient at the STJ (P < 0.01) and concomitant bilateral or peripheral pulmonary artery (PA) stenosis (P < 0.01) were risk factors for postoperative DAS. Postoperative PA stenosis occurred more frequently in patients who received bilateral pulmonary arterioplasty (P < 0.01). Postoperative prognosis of the aortic root after SVAS repair was satisfactory. DAS and PA stenosis were common. The results of bilateral pulmonary arterioplasty were unsatisfactory. The surgical timing and technique for PA stenosis should be carefully considered.

Surgical Techniques in Management of Supravalvular Aortic Stenosis in Children

Multiple techniques exist for the repair of supravalvular aortic stenosis (SVAS), but given the lesion's rarity, analyses comparing the efficacy of each repair have been limited. A retrospective review of all children at a single institution who underwent repair of SVAS from June 1995 to May 2019 was performed. Anatomic and physiologic measurements across time points were compared between 2 predominant surgical techniques. Time-to-event outcomes were compared using the log-rank test. SVAS was repaired in 89 patients, by using a single-patch in 31 (35%) and the Doty repair in 58 (65%). Median age at operation was 2.5 years (interquartile range [IQR], 1.0 to 6.8 years), with median follow-up of 5.8 years (IQR, 1.8 to 10.7 years). Reoperation was required in 8 (9%) patients at a median of 1.5 years postoperatively (IQR, 0.3 to 4.8 years). There was 1 death after multiple reinterventions. The change from the preoperative to the postoperative sinotubular junction z-score was greater for patients after Doty repair (median change+2.5; IQR, 1.5, 4.1) than for patients after single-patch repair (median change+0.8; IQR,-0.1, 2.1; P= .001). Freedom from reoperation was longer for patients after Doty repair than after the single-patch technique (P= .008). The Doty repair provides longer freedom from reoperation after supravalvular aortic stenosis repair compared with a single-patch technique, likely through a greater increase in the sinotubular junction at the time of initial operation.

Long-term Surgical Prognosis of Primary Supravalvular Aortic Stenosis Repair

Supravalvular aortic stenosis (SVAS) represents a heterogeneous group, including Williams syndrome, familial elastin arteriopathy, sporadic cases, and others. This study sought to evaluate long-term outcomes of SVAS repair. A total of 87 patients underwent surgical repair of congenital SVAS at Boston Children's Hospital in Boston, Massachusetts, between 1997 and 2017. A total of 41 patients had Williams syndrome, and 46 did not. Of the 46 patients who did not have Williams syndrome, 23 had sporadic SVAS, and 13 had familial elastin arteriopathy. Demographic data and outcomes were reviewed and analyzed from medical records. The median age at operation was 2.9 years. Mean z score of the sinotubular junction was -3.29 ± 1.42 and of the aortic root was-0.09 ± 1.19. A total of 26% (n= 22) patients had coronary ostium stenosis, and 41% (n= 9) of them required patch plasty. Survival rates at 5, 10, and 20 years were all 94.3%. Freedom from left ventricular outflow tract reoperation at 5, 10, and 20 years was 78.5%, 70.3%, and 70.3%, respectively. Freedom from aortic arch reintervention at 5, 10, and 20 years was 98.6%, 94.3%, 89.3%, respectively. In risk factors analysis, age younger than 1 year, z scores of the aortic valve and aortic root, and concomitant right ventricular outflow tract surgical repair were predictive of the need for reoperation and reintervention for left or right ventricular outflow tract obstruction. Excellent long-term survival rates can be achieved with surgical repair of SVAS. Age younger than 1 year, small aortic valve and aortic root z scores, and concomitant right ventricular outflow tract surgical repair were predictors of reoperation and reintervention.

Three-Patch Aortic Root Reconstruction With Extended Left Main Coronary Artery Patch Augmentation in Neonates and Infants

Left main coronary artery (LMCA) stenosis is present in approximately 5% of patients with congenital supravalvular aortic stenosis (SVAS) (Fig.1)1 and is associated with an increased risk of sudden cardiac death.2 However, patients undergoing coronary artery intervention at the time of SVAS repair are at the highest risk of experiencing major adverse cardiac events.3 Literature reports of surgical techniques and outcomes of concomitant coronary artery repair in these high-risk patients are diverse and inconsistently described. We have recently adopted a standardized surgical technique for management of this complex pathology by combining extended LMCA patch augmentation with a 3-patch aortic root reconstruction (Brom's technique). In this report, we describe our contemporary surgical technique of 3-patch aortic root reconstruction with extended LMCA patch augmentation for patients with congenital SVAS with ostial LMCA stenosis and bilateral outflow tract obstruction. Institutional review board approval was obtained for retrospective review of patient charts.

Surgical strategies and outcomes of congenital supravalvular aortic stenosis

Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus upon the optimal approach. This study reviewed our institutional experience in the management of SVAS. Ninety patients undergoing surgery for SVAS were identified between 2009 and 2016. Based on surgical techniques, patients were divided into three groups: McGoon repair (n = 63), Doty repair (n = 24), and Brom repair (n = 3). Median follow-up was 38.5 months (range, 4 months-7.5 years). Patient status, cumulative event-free survival rate, and risk factors for adverse clinical outcomes were assessed. The early mortality rate was 3.3%. There was one late death and two reinterventions. No differences were observed among three surgical groups. Event-free survival was 98.4% at 3 years and 96.5% at 5 years. Diffuse-type SVAS and a preoperative gradient greater than 60 mmHg were risk factors for adverse cardiac remodeling within 6 months post-surgery. Residual aortic stenosis was associated with male gender, preoperative aortic valve stenosis, and a preoperative peak gradient greater than 90 mmHg. Eleven patients (out of 30) who underwent concomitant pulmonary artery patching had a residual pulmonary gradient greater than 40 mmHg. Surgical repair of SVAS can be safely achieved using different techniques, with similar midterm mortality and reintervention rates. Higher preoperative gradient is associated with worse clinical results. Issues regarding surgical timing and concomitant pulmonary artery stenosis need to be further addressed.

New four Patch Repair [Modified Brom’s ] Technique for Supravalvular Aortic Stenosis

The objective of this paper is to describe the novel four-patch technique for repair of supravalvular aortic stenosis (SVAS). Supravalvular aortic stenosis is a rare malformation as a result of an abnormal thickening of the aortic wall. SVAS may present in two forms: a localized form (affecting only the aortic sinotubular junction) and a diffuse form, where the aortic arch and its side branches are also affected. We present the novel four-patch technique as modification of Brom three patch technique. After transection of the aorta at the sinotubular junction, three longitudinal incisions are made into the three sinuses. The aortic root geometry is then restored by placement of three separate patches of autologous pericardium in the opened sinuses. One additional diamond shaped pericardial patch placed anteriorly between proximal and distal aorta. This modified four patch technique provides a complete and symmetric restoration of the aortic anatomy with insignificant gradient and good short-term and potentially good long-term results. Key words: Supravalvular aortic stenosis, Brom three patch technique, Pericardial patch.

New four patch repair [Modified Brom's] technique for supravalvular aortic stenosis

Abstract The objective of this paper is to describe the novel four-patch technique [modified Brom's] for repair of supravalvular aortic stenosis (SVAS). After transection of the aortaat the sinotubular junction, three longitudinal

Surgical Repair of Supravalvular Aortic Stenosis in Children With Williams Syndrome: A 30-Year Experience

Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution. Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]). A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4(24%) late deaths, occurring at 6 weeks, 3.5 years, 4years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years. Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up.

Outcomes of repair of supravalvular aortic stenosis in children

Outcomes of repair of supravalvular aortic stenosis in children

Outcome of Surgical Correction of Congenital Supravalvular Aortic Stenosis With Two- and Three-Sinus Reconstruction Techniques

Several surgical techniques for the treatment of congenital supravalvular aortic stenosis have been developed, yet there is no consensus about the optimal approach. We reviewed our institutional experience with 2- and 3-sinus reconstruction techniques. Thirty-eight patients operated on for supravalvular aortic stenosis between 1987 and 2012 in our institution were analyzed retrospectively. Eight patients (21%) were infants and in 5 (13.2%) diffuse stenosis was present. Mean peak pressure gradient was 86.1±28.7 mm Hg preoperatively. Surgical procedures included single-patch enlargement (McGoon, n=3), inverted bifurcated-patch aortoplasty (Doty, n=22), 3-sinus patch augmentation (Brom, n=8), and autologous slide aortoplasty (n=5). Major concomitant procedures were performed in 10 patients (26.3%). Early mortality was 2.6%. Follow-up continued for a median of 7.5 years (range 3 weeks to 22 years). Overall survival estimates were 94% and 90% and overall freedom from reoperation was 83% at 5 and 20 years, respectively. No differences were found between surgical techniques in respect to survival, clinical course, hemodynamic outcome, or freedom from reoperation rates. A significantly worse outcome in regard to survival and reoperation rates was observed in infants. Our study demonstrates equally good results for the repair of supravalvular aortic stenosis with both 2- and 3-sinus reconstruction. No evidence of a superior outcome for 3-sinus reconstruction techniques was found. Operation in infancy is an important factor associated with unfavorable outcome.

Mid-term outcome after surgical repair of congenital supravalvular aortic stenosis by extended aortoplasty.

Congenital supravalvular aortic stenosis (SVAS) is a rare arteriopathy associated with the Williams-Beuren syndrome (WBS) and other elastin gene deletions. Our objective was to review the mid-term outcomes of SVAS repair with extended aortoplasty. Congenital SVAS repairs from 2001 to 2010 were retrospectively reviewed. The follow-up records, reintervention and reoperation data and most recent echocardiograms were obtained. From 2001 to 2010, 21 patients (15 males) underwent surgical repair of SVAS by extended aortoplasty with autologous pretreated pericardium, which is a modification of the Doty technique. The mean age was 3.1 ± 4.2 years. WBS was diagnosed in 14 of the patients. There was no early mortality, but one late death was observed. At the latest follow-up (mean follow-up, 4.3 ± 2.9 years; range, 1-108 months), echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 15 ± 8 mmHg. The majority of the patients had minimal to mild aortic insufficiency. No reoperation or reintervention was required. Extended aortoplasty provides excellent mid-term relief of SVAS and, in addition, reshapes the aortic root geometry to a much more favourable anatomical configuration. It can be performed without any increase in operative risks. The mid-term results are excellent.

Reoperation After Supravalvular Aortic Stenosis Repair

Supravalvular aortic stenosis (SVAS) is the rarest type of left ventricular outflow tract obstruction. We reviewed our experience with this anomaly and analyzed risk factors for death or reoperation. Between 1984 and 2009, 49 patients had surgery for SVAS. A single-patch technique was used in 3, two-sinus enlargement in 39, and three-sinus enlargement in 7. Variables evaluated included age at surgery (<2 versus >2 years old), presence of pulmonary artery stenosis, type of SVAS (focal versus diffuse), presence of valvular aortic stenosis, and era of surgery. The only early death occurred in a patient who experienced cardiac arrest during anesthesia induction and could not be separated from bypass after surgery. There were 2 late deaths at 3 and 11 years after SVAS repair, both related to treatment for pulmonary artery stenosis. Actuarial survival at 5, 10, and 20 years was 95%, 95%, and 90%, respectively. Sixteen patients required 23 reoperations: for pulmonary artery stenosis (n = 10), distal aortic stenosis (n = 9), aortic valve stenosis (n = 4), and coronary artery stenosis (n = 1). Actuarial reoperation-free survivals at 5, 10, and 20 years were 73%, 58%, and 52%, respectively. Coexistent pulmonary artery stenosis, young age at surgery, and diffuse type SVAS were predictors of lower freedom from death or reoperation by both univariate and multivariate analyses. Survival after surgical repair of SVAS is excellent. However, reoperation is frequent, especially when the patients also have pulmonary artery stenosis, diffuse type SVAS, and initial surgery at a young age.

Midterm Outcomes in Supravalvular Aortic Stenosis Demonstrate the Superiority of Multisinus Aortoplasty

Surgical techniques for repair of supravalvular aortic stenosis (SVAS) include McGoon's one-patch, Doty's two-patch, and Brom's three-patch method. In this review we evaluated mid-term clinical outcomes of these techniques at our institution. Our cardiac surgery database identified patients with SVAS repair from 1990 to 2008. Follow-up records, reintervention and reoperation data, and most recent echocardiograms were obtained. From 1990 to 2008, 20 patients (70% male) underwent surgery for SVAS. Mean age was 3.6 +/- 5.6 years. In chronological sequence, 8 patients had single-patch aortoplasty, 4 had the Doty procedure, and 8 received Brom's symmetric three-patch aortoplasty. Of the Brom patients, 6 had Williams syndrome. Aortic cross-clamp times were 40.1 +/- 13.6 minutes (one-patch), 60.3 +/- 38.8 minutes (Doty), and 104 +/- 20.5 minutes (Brom). Perioperative mortality was 5.0% (1 patient in one-patch group). Mean postoperative length of stay was 10 +/- 10.6 days. Follow-up data were available for all survivors (mean follow-up, 6.3 +/- 6.0 years; range, 6 months to 16 years). There were no late deaths. Follow-up echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 33 +/- 18.0 mm Hg (one-patch), 10 +/- 1 mm Hg (Doty), and 18 +/- 12 mm Hg (Brom). All patients in the Doty and Brom groups had less than moderate aortic insufficiency. Reoperations were required in 5 of 8 one-patch patients (62%) for residual aortic stenosis (n = 3), aortic insufficiency (n = 1), and subvalvar stenosis (n = 1). No Doty or Brom patient has required aortic reoperations, which was nearly statistically associated with freedom from reoperation (p = 0.06). Subvalvar stenosis was the only risk factor associated with reoperation (p = 0.0028). Despite a longer cross-clamp time, SVAS repair by Doty or Brom aortoplasty restores normal hemodynamics and reduces the need for reoperation when compared with the classic one-patch technique. Our current preference for SVAS repair is the Brom three-patch symmetric aortoplasty.

Supravalvular aortic stenosis of the diffuse type: 29 years follow-up after aortic endarterectomy and symmetric enlargement of the ascending aorta and of the three coronary sinuses

Supravalvular aortic stenosis is a rare congenital anomaly characterized by variable amounts of left ventricular outflow tract obstruction distal to the aortic valve. Macroscopically, it is categorized into three morphologic subtypes: membranous, hourglass, and diffuse. The diffuse type is the most rare, and its surgical repair is the most challenging due to variable length of ascending aorta hypoplasia. Surgical treatment options of supravalvular aortic stenosis are well established for the membranous and hourglass type, whereas they are challenging and less well defined for the diffuse type. We present a case of long-term follow-up (29 years) after a very complex surgical repair of supravalvular aortic stenosis of the diffuse type, with focus on technical aspects. To our knowledge, the present case represents one of the longest follow-up routines in the English language literature of surgical treatment of supravalvular aortic stenosis.

Mild Supravalvular Aortic Stenosis With Left Coronary Obstruction in a Neonate

A premature infant with a large patent ductus arteriosus and mild supravalvular aortic stenosis had evidence of severe myocardial ischemia after ductus ligation. Urgent reoperation was required to relieve severe obstruction of the left coronary orifice and the supravalvular stenosis was repaired. This unusual presentation early in infancy emphasizes that left coronary artery obstruction should be suspected even in the presence of mild supravalvular stenosis.