Scimitar syndrome with left pulmonary hypertension and right pulmonary artery stenosis in childhood--report of a case of successful surgical repair

An one-year old child with Scimitar syndrome (anomalous drainage of the right pulmonary vein to inferior vena cava with right lung hypoplasia) associated with left side pulmonary hypertension and right pulmonary artery stenosis underwent surgical repair. The anomalous pulmonary vein was anastomosed to right atrium with 10 mm PTFE graft interposition and drained to left atrium through equine pericardial intra-atrial baffle. The stenotic portion of right pulmonary artery was enlarged with porcine pericardial patch. To our knowledge, this is the first successful surgical case of Scimitar syndrome with pulmonary hypertension in childhood reported in literature. A repair of anomalous pulmonary venous drainage to inferior vena cava in childhood is difficult, and a meticulous selection of surgical procedure is necessary.     

Is there a place for pediatric valvotomy in the autograft era?

OBJECTIVE: Valvotomy and the autograft procedure are the most common surgical treatment options for children with valvular aortic stenosis. We evaluated the results of these surgical procedures in our institution. METHODS: Retrospective analysis was done of all patients presenting with aortic stenosis and operated upon before the age of 18. In 11 patients a valvotomy was performed and in 36 an autograft procedure. RESULTS: There was no hospital mortality. Mean follow-up in the valvotomy group was 4.8 years (SD 3.3), in the autograft group 4.5 years (SD 3.3). During follow-up one patient died suddenly 2 months after valvotomy. Two patients in the autograft group died (not valve-related). After valvotomy three patients underwent a balloon valvotomy, in one followed by an autograft procedure and one patient had a repeat valvotomy. In the autograft group one patient was reoperated for severe aortic regurgitation and moderate pulmonary stenosis. At last echocardiography after valvotomy (eight remaining patients) in only two patients (25%) no aortic stenosis or regurgitation was present. In the remaining six patients aortic stenosis is mild in two and moderate in three, including one with moderate aortic regurgitation. In one patient without stenosis, moderate aortic regurgitation was seen. No pulmonary stenosis or regurgitation is present. Echocardiography after autografting (33 remaining patients) showed no aortic stenosis. Aortic regurgitation was mild in seven patients, moderate in two, severe in one. Pulmonary stenosis was present in two patients (16%). Pulmonary regurgitation was mild in three patients and moderate in one. CONCLUSIONS: In selected patients with valvular aortic stenosis who are beyond infancy, valvotomy may be adequate and may postpone further surgery for a significant length of time. After valvotomy the main problem is residual aortic stenosis while after autografting a shift occurs to aortic regurgitation and problems related to the pulmonary valve. Careful clinical and echocardiographic follow-up is therefore warranted in young patients after the autograft procedure.     

Long-term follow-up of supra-annular pulmonary autograft aortic root replacement in patients with bicuspid aortic valve

Objective: The performance of the Ross procedure in the case of geometric mismatch between pulmonary autograft and a bicuspid aortic root has not yet been fully evaluated. To prevent geometrically caused autograft dysfunction, a modification of the surgical technique is necessary. Methods: Between January 1996 and January 2007, 50 patients (33 male, 17 female; mean age 50+/-14 years; range 13-63 years) underwent replacement of a diseased bicuspid aortic valve (stenosis in 14 cases; insufficiency in 21; combined disease in 15) with a Ross procedure. The pulmonary autograft was inserted partially in supra-annular position to correct the geometric mismatch between the deeper base of the non-coronary sinus and the right/left coronary sinus. In 24 of these patients, additional tailoring of the non-coronary sinus was necessary. In eight patients the non-coronary sinus was covered with a glutaraldehyde treated autologous pericardial patch to prevent pseudoaneurysm formation. Patients were followed up 1, 2, 5 and 10 years postoperatively. Results: There were no early or late deaths. There were six reoperations. One patient was reoperated because of persistent severe aortic valve insufficiency 9 months postoperatively. Three patients were reoperated for formation of subannular pseudoaneurysm, 6, 9 and 30 months postoperatively. One patient was reoperated for closure of a paravalvular dehiscence. Another patient was reoperated 1 year postoperatively because of a severe pulmonary stenosis due to excessive calcification of the bioprosthesis. Echocardiographic follow-up of the remaining patients showed no evidence of residual or recurrent pulmonary autograft regurgitation or progression of aortic root dilatation. Conclusion: Autograft replacement of the bicuspid aortic valve is challenging, as the geometric mismatch has to be adjusted. Valve dysfunction is avoided by a supra-annular implantation technique, but pseudoaneurysm formation at the base of the non-coronary sinus is a worrying aspect. Patch reinforcement may solve this issue.     

Aortic root replacement with the pulmonary autograft: an invariably competent aortic valve?

BACKGROUND: Pulmonary autograft aortic root replacement was used in adults. Risk factors for aortic regurgitation (AR), and for pulmonary allograft valve stenosis are identified. METHODS: From February 1991 through April 1998, 80 adults (mean age 34.4 years) underwent pulmonary autograft aortic root replacement. Primary diagnosis was AR in 43 (53.7%) patients, aortic stenosis in 13 (16.3%) and mixed disease in 24 (30%) patients. A root reinforcement ring was used in 32 (40%) patients. RESULTS: There was no hospital mortality. Estimated patient survival is 100% at 7 years. A total of 3 patients underwent reoperation: 2 on the autograft for severe AR, 1 for pulmonary allograft stenosis. Freedom from reoperation on the autograft is 96.7 +/- 2.4% at 7 years. Multivariate analysis indicated bicuspid aortic valve disease as an incremental risk factor for AR at discharge (p = 0.036, odds 3.5). Univariate analysis identified operation for pure AR as risk factor for AR during follow-up (p = 0.041). Mild AR or more increased from 2.5% at discharge to 11.3% during follow-up (p = 0.008). Progression of AR was limited by the use of a reinforcement root ring (p = 0.031). Freedom from mild AR or more in patients with and without a reinforcement root ring was 100% and 72.9 +/- 9.3% respectively, at 5 years (p = 0.119). Pulmonary allograft stenosis occurred in 15 (22.5%) patients. Multivariate analysis revealed that large sized pulmonary allografts were less prone to stenosis (p = 0.048, odds 0.13). CONCLUSIONS: Pulmonary autograft root replacement can be performed with few complications. During follow-up, a significant increase in mild AR or more is observed. The use of a reinforcement root ring is effective in preventing progression of AR.     

A new operation for ventricular septal defect with aortic incompetence

Two boys, 10 years of age, with ventricular septal defect and severe aortic insufficiency were treated using a new method. An autograft of anterior pulmonary artery wall and the adjacent leaflet and subpulmonary muscle was excised. The excised pulmonary artery segment was used to replace the prolapsed right coronary cusp, and the autograft muscle was used to close the ventricular septal defect. The right ventricular outflow tract and pulmonary valve were repaired using an autologous pericardial patch with an autologous single cusp. The results in both cases were satisfactory.     

Severe mitral regurgitation--relations between magnetic resonance imaging,echocardiography and natriuretic peptides

Objective - The validity of the Ross operation as freestanding root replacement in adult patients with bicuspid aortic valve disease has lately been questioned. We have analyzed retrospectively our results in 23 adult patients (19 males) operated for bicuspid aortic valve disease ad modum "Ross" employing a freestanding root replacement technique. Design - In 9 patients the dominant aortic valve lesion was stenotic (aortic stenosis group) and in the remaining 14 patients it was aortic insufficiency (aortic insufficiency group). The fate of the pulmonary autograft in the two groups was studied. The intraoperatively measured aortic and pulmonary annuli diameters from the two groups were compared with those from a population of normal looking aortic and pulmonary valves matched for body surface area. Results - The aortic insufficiency group needed significant reduction of the aortic annulus diameter to conform to the size of the pulmonary autograft. The pulmonary autograft annuli in this group were significantly larger in diameter than the ones in the aortic stenosis group. The mean pulmonary annulus diameter in the aortic stenosis group was, on the other hand, significantly smaller when compared with that in the normal matched population. After a mean follow-up period of about 19 months, the aortic insufficiency group showed significant dilatation of the neo-aortic sinuses. Between the two groups, the remaining echocardiographic variables remained either stable or improved at follow-up. Conclusion - Pre-existing larger diameters of the aortic and pulmonary annuli in the aortic insufficiency group combined with the significantly increased left ventricular end-diastolic diameters, may predispose these patients to significant dilatation of the unsupported aortic sinuses after a Ross operation. This dilatation does not, however, lead to increase in the autograft valve insufficiency at short-term follow-up if the aortic annulus and the distal ascending aorta are tailored to the size of the pulmonary autograft. Ross operation, employing freestanding aortic root replacement technique, may therefore be recommended in adult patients with bicuspid aortic valve disease with excellent short-term results.     

Ross operation for bicuspid aortic valve disease in adults: is it a valid surgical option?

OBJECTIVE: The validity of the Ross operation as freestanding root replacement in adult patients with bicuspid aortic valve disease has lately been questioned. We have analyzed retrospectively our results in 23 adult patients (19 males) operated for bicuspid aortic valve disease ad modum "Ross" employing a freestanding root replacement technique. DESIGN: In 9 patients the dominant aortic valve lesion was stenotic (aortic stenosis group) and in the remaining 14 patients it was aortic insufficiency (aortic insufficiency group). The fate of the pulmonary autograft in the two groups was studied. The intraoperatively measured aortic and pulmonary annuli diameters from the two groups were compared with those from a population of normal looking aortic and pulmonary valves matched for body surface area. RESULTS: The aortic insufficiency group needed significant reduction of the aortic annulus diameter to conform to the size of the pulmonary autograft. The pulmonary autograft annuli in this group were significantly larger in diameter than the ones in the aortic stenosis group. The mean pulmonary annulus diameter in the aortic stenosis group was, on the other hand, significantly smaller when compared with that in the normal matched population. After a mean follow-up period of about 19 months, the aortic insufficiency group showed significant dilatation of the neo-aortic sinuses. Between the two groups, the remaining echocardiographic variables remained either stable or improved at follow-up. CONCLUSION: Pre-existing larger diameters of the aortic and pulmonary annuli in the aortic insufficiency group combined with the significantly increased left ventricular end-diastolic diameters, may predispose these patients to significant dilatation of the unsupported aortic sinuses after a Ross operation. This dilatation does not, however, lead to increase in the autograft valve insufficiency at short-term follow-up if the aortic annulus and the distal ascending aorta are tailored to the size of the pulmonary autograft. Ross operation, employing freestanding aortic root replacement technique, may therefore be recommended in adult patients with bicuspid aortic valve disease with excellent short-term results.     

Tracheal surgery in children: an 18-year review of four techniques

Objective: Review the short- and long-term outcomes of a single institution experience in infants with congenital tracheal stenosis, comparing four different operative techniques used from 1982 through 2000. Methods: Hospital and clinic records of 50 infants and children who had surgical repair of congenital tracheal stenosis secondary to complete tracheal rings were reviewed. Age at surgery ranged from 7 days to 72 months (median, 5 months, mean 7.8±12 months). Techniques included pericardial patch tracheoplasty (n=28), tracheal autograft (n=12), tracheal resection (n=8), and slide tracheoplasty (n=2). All procedures were done through a median sternotomy with cardiopulmonary bypass. Seventeen patients had a pulmonary artery sling (35%), and 11 had an intracardiac anomaly (22%). Results: There were three early deaths (6% early mortality), two after pericardial tracheoplasty and one after autograft. There were six late deaths (12% late mortality), five after pericardial tracheoplasty and one after slide tracheoplasty. Length of stay (median) was 60 days (pericardial tracheoplasty), 28 days (autograft), 14 days (resection), and 18 days (slide). Reoperation and/or stent placement was required in seven patients (25%) after pericardial tracheoplasty, in two patients (17%) after autograft, in no patients after resection, and in one patient (50%) after slide tracheoplasty. Conclusions: Our current procedures of choice for infants with congenital tracheal stenosis are resection with end-to-end anastomosis for short-segment stenoses (up to eight rings) and the autograft technique for long-segment stenoses. Associated pulmonary artery sling and intracardiac anomalies should be repaired simultaneously.     

The Ross procedure in adults: intermediate-term results

BACKGROUND: The durability of the Ross procedure may be optimized by appropriate geometric matching of the aortic and pulmonary artery roots. We employed a surgical strategy to standardize the operation in order to avoid more readily a geometric mismatch. METHODS: The Ross procedure was performed as a root replacement. Without regard for patient body surface area, the aortic annulus was plicated to 23 mm and externally buttressed with felt. Geometric mismatch of the distal autograft anastomosis was avoided by liberal use of a synthetic interposition graft, and the anastomosis was also externally buttressed with felt. An over-sized pulmonary homograft (27 to 28 mm) was routinely used to reconstruct the right ventricular outflow tract. RESULTS: Forty-four consecutive patients (27 men and 17 women; mean age, 49 +/- 9 years) were operated on between January 1997 and March 2002. Mean follow-up was 38 +/- 5 months. Twenty-nine patients had aortic stenosis and 15 had aortic regurgitation. Aortic annular plication was done in 41 (93%) and an aortic interposition was used in 14 (32%). There were three hospital deaths, with no subsequent deaths. Only 1 patient required reoperation 2.5 years postoperatively from recurrent endocarditis. No patient has more that "trivial" autograft insufficiency, and the mean autograft gradient was 7 +/- 3 mm Hg. No patient has significant pulmonary homograft stenosis. CONCLUSIONS: Geometric matching of the aortic and pulmonary roots may be readily accomplished using a standardized approach to the Ross procedure. In turn, this may optimize the durability of the operation.     

Long-term results of aortic root replacements with pulmonary autografts (Ross procedure) in five cases]

Aortic root replacement with a pulmonary autograft (the Ross Procedure) has been successfully performed in our hospital since November, 1992. The long-term results of five of the earliest cases are reported in this paper (2-3 year follow-up). The patients' ages were two months to eighteen years old. Four of the patients suffered from aortic valve stenosis, and one suffered from aortic regurgitation. Severe left ventricular failure was recognized in three cases. However, the patients recovered from surgery smoothly and without significant aortic regurgitation and left ventricular outflow tract gradients. A serious concern exist as to whether the implanted autograft in 2 months old infant would grow. In this patient, postoperative cardiac catheterization was performed after sixty days, one year, and two years. The diameter of the anulus of the pulmonary autograft enlarged from 12 mm to 18 mm over the period of two years. Compared with the calculated aortic valvular diameter from a standardized body surface area, these diameters were equivalent to 150%-162% of the standardized size at each age. The implanted pulmonary autograft has subsequently enlarged gradually and proportionally. Its function as an aortic valve was maintained even after significant enlargement of the aortic anulus to 18 mm. We therefore conclude that the Ross procedure can be recommended because of the apparent ability the pulmonary autograft to grow over time.     

Long-term results of aortic root replacements with pulmonary autografts (Ross Procedure) in five cases

Aortic root replacement with a pulmonary autograft (the Ross Procedure) has been successfully performed in our hospital since November, 1992. The long-term results of five of the earliest cases are reported in this paper (2-3 year follow-up). The patients’ ages were two months to eighteen years old. Four of the patients suffered from aortic valve stenosis, and one suffered from aortic regurgitation. Severe left ventricular failure was recognized in three cases. However, the patients recovered from surgery smoothly and without significant aortic regurgitation and left ventricular outflow tract gradients. A serious concern exist as to whether the implanted autograft in 2 months old infant would grow. In this patient, postoperative cardiac catheterization was performed after sixty days, one year, and two years. The diameter of the anulus of the pulmonary autograft enlarged from 12 mm to 18 mm over the period of two years. Compared with the calculated aortic valvular diameter from a standardized body surface area, these diameters were equivalent to 150%–162% of the standardized size at each age. The implanted pulmonary autograft has subsequently enlarged gradually and proportionally. Its function as an aortic valve was maintained even after significant enlargement of the aortic anulus to 18 mm. We therefore conclude that the Ross procedure can be recommended because of the apparent ability the pulmonary autograft to grow over time.     

Aortic arch reconstruction with pulmonary autograft patch aortoplasty

OBJECTIVE: The optimal technique for aortic arch reconstruction through median sternotomy is still under debate. We have introduced the technique of pulmonary autograft patch aortoplasty as a reliable alternative. METHODS: The outcomes of 51 infants who underwent neonatal repair of interrupted aortic arch (n = 28) or coarctation associated with ventricular septal defect (n = 23) since 1992 were analyzed. The patients were reviewed in three groups according to the aortic arch reconstruction technique: group I underwent direct anastomosis (n = 23), group II underwent homograft or pericardial patch aortoplasty (n = 8), and group III underwent pulmonary autograft patch aortoplasty (n = 20). The pulmonary autograft patch consisted in the anterior wall of the main pulmonary artery, between the supracommissural level and the divided ductus arteriosus. The created defect was replaced with fresh autologous pericardium. RESULTS: All patients except 1 were discharged without significant residual gradient at the level of the aortic arch. At a median delay of 7 months (range 2-51 months), 11 patients (22%) had recurrence of arch obstruction and underwent balloon angioplasty (n = 8) or surgical correction (n = 3). One patient who had undergone direct anastomosis required reoperation for bronchial compression. At a median follow-up of 29 months, the actuarial freedoms from recurrent arch obstruction were 81% for direct anastomosis, 28% for homograft or pericardial patch aortoplasty, and 100% for pulmonary autograft aortoplasty (P =.03 for group III vs group I and P <.0001 for group III vs group II). CONCLUSIONS: The aortic arch repair associated with pulmonary autograft patch augmentation resulted in superior midterm outcomes and therefore constitutes a reliable alternative to the direct anastomosis technique. It allowed complete relief of anatomic afterload and diminished the anastomotic tension, thus reducing the risk of restenosis and tracheobronchial compression. We observed a significantly higher rate of recurrence after patch aortoplasty with other materials.     

A case of mitral valve replacement with autologous pulmonic valve in congenital mitral stenosis

A one-year-old infant with congenital mitral stenosis and pulmonary hypertension underwent by mitral valve replacement with his pulmonic valve autograft and pulmonary tract reconstruction with heterogeneous pericardial conduit. His postoperative hemodynamics data showed that left atrial pressure decreased and pulmonary hypertension continued. Echocardiography showed that the implanted autograft functioned properly. On the seventh postoperative day, he died of pulmonary hypertension. In case with congenital mitral stenosis with the small mitral annulus and the small left ventricular cavity, it is difficult to perform mitral valve replacement by commercially available mechanical or tissue valves. Because these valves are not suitable for the small mitral annulus. The mitral valve replacement with pulmonic valve autograft is recommended in such a case with the congenital mitral stenosis.     

Congenital bicuspid pulmonary valve. An uncommon impede for Ross procedure

We report a case of successful heterograft aortic valve replacement following an impede Ross procedure in a 48-year-old man presenting with a congenital bicuspid pulmonary valve. The patient was admitted for aortic valve stenosis that required an aortic valve replacement (AVR). Owing to his young age and reluctance to long-term anticoagulation therapy, it was decided to do an AVR by pulmonary autograft. During surgery, the anatomical unsuitability of the graft was discovered leading to the procedure’s readjustment.     

Midterm results of total aortic root replacement with pulmonary autograft (Ross operation)

Between February 1995 and December 1999, 18 patients underwent Ross operation. Age at the operation ranged from 2 to 31 years. Diagnosis includes congenital aortic stenosis and/or regurgitation in 15, and adult aortic regurgitation in 3. In all cases autograft was implanted by the method of total aortic root replacement, associated with annuloplasty for the dilated aortic annulus in 2 and aortoventriculotomy by the Konno procedure in 3 (Ross-Konno). Right ventricular outflow tract was reconstructed by a pulmonary homograft in 12, a xenopericardial conduit in 3, or the other reconstructive procedures with autologous tissue and outflow patch in 3. There was no operative and late death. Reoperation was needed in 1 patient due to stenosis of pericardial conduit 4 years after the initial operation. Pressure gradient across implanted autograft valve was negligible (4.8 +/- 0.5 mmHg), and echocardiography revealed no aortic regurgitation in 12 cases and trivial to mild in 6, over a mean follow-up period of 23 +/- 18 months (range 2 to 60 months), signifying excellent durability of implanted autograft. Right ventricular outflow tract reconstruction with the homograft resulted in excellent mid-term performance as showing pressure gradient of 9.0 +/- 4.6 mmHg and no regurgitation in 11 of 12 cases, whereas pressure gradient was 17.9 +/- 13.1 mmHg in the patients underwent the other reconstructive procedures. We conclude that Ross procedure associated with the concomitant procedures to adjust the size discrepancy between the native aortic annulus and autograft has provided good midterm results with excellent autograft durability. And this procedure was thought to be a preferable method for children as well as young adults with congenital aortic stenosis.     

Reconstruction of right ventricular outflow tract with stentless xenografts in Ross procedure

To investigate whether the use of a stentless porcine aortic xenograft can be an alternative for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure, 9 patients underwent the Ross procedure and RVOT reconstruction with a stentless xenograft since January 2000. After the aortic valve was replaced with a pulmonary autograft, a stentless xenograft with a xeno- pericardial roll was implanted in the RVOT. One patient required subsequent aortic valve replacement because of severe regurgitation of the pulmonary autograft. All patients recovered well from the operation. The right ventricle-pulmonary arterial pressure gradient was 18 +/- 7 mm Hg at discharge and was not significantly increased during the 2-year follow-up period. Although 1 patient died of ventricular arrhythmia 5 months after, his cardiac function was normal, and transpulmonary valve pressure was 19 mm Hg in the follow-up. The other 7 patients are currently in New York Heart Association functional Class I. Although long-term follow-up is required to explain the durability, the stentless xenograft with a pericardial roll is considered to be an alternative for reconstruction of the RVOT within 2 years after the Ross procedure.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

Surgical treatment of a distal arch pseudoaneurysm and migrated stent-graft after interrupted aortic arch repair.

A 13-year-old girl, who had undergone a modified Blalock-Park operation using an equine pericardial patch for a type A aortic arch interruption at 10 days of age, and patch repair of a ventricular septal defect at 1 year, presented with recurrent stenosis of the aorta. She underwent balloon angioplasty which resulted in a pseudoaneurysm at the distal suture line of the patch. Endovascular stent-grafting was employed. Soon after placement, the stent-graft had migrated into the distal portion of the descending aorta. The distal aortic arch and proximal descending thoracic aorta were replaced with a prosthetic graft. The migrated stent-graft was removed. The operation was performed through an anterior approach under hypothermic circulatory arrest and selective cerebral perfusion. The postoperative course was uneventful. The migration was likely caused by the limited ability to bend the stent-graft to match the curvature of the arch, the stiffness of the scarred equine pericardial patch, and the shape of the aorta.     

The Ross/Konno procedure in neonates and infants: intermediate-term survival and autograft function

BACKGROUND: The Ross procedure has been increasingly applied to neonates and infants. Addition of a modified Konno-type enlargement of the aortic annulus allows the application of this procedure to neonates and infants with significant annular hypoplasia. The potential for growth and the proven durability make the autograft an ideal aortic valve replacement. METHODS: Between March 1993 and December 2000, 10 patients under 1 year of age underwent a Ross/Konno procedure at our institution (range, 2 to 349 days; median 16). All patients had severe to critical aortic stenosis. All patients required aortic annulus enlargement for size mismatch between the aortic and pulmonary valves. RESULTS: There were no deaths at a median follow-up of 48 months (range, 1 to 74 months). All patients had none to mild aortic stenosis on Doppler echocardiography. Eight patients had a 0 to 1+ aortic insufficiency, 1 patient had a 2+ aortic insufficiency, and 1 patient had a 3+ aortic insufficiency. Aortic annular dilatation was not observed. Aortic sinus dilatation occurred initially (mean change in z-value: 0 to 12 months, +2.1) and then stabilized (mean change in z-value: 12 to > 36 months, +0.6). No patient required additional procedures for aortic valve disease. Two patients required three pulmonary allograft replacements. CONCLUSIONS: The Ross procedure with a modified Konno-type enlargement of the aortic annulus is an excellent approach to aortic valve disease in the neonate and infant. The procedure can be accomplished with low morbidity and mortality, and low rates of reoperation. The pulmonary autograft demonstrates durability without developing aortic stenosis, aortic insufficiency, or progressive dilatation. Enlargement of the aortic annulus parallels somatic growth.     

Ross procedure in rheumatic aortic valve disease.

OBJECTIVE: To assess the results of aortic valve replacement with the pulmonary autograft in patients with rheumatic heart disease. METHODS: From October 1993 through September 2003, 81 rheumatic patients with aortic valve disease, mean age 29.5+/-11.9 years (11-56 years) underwent, the Ross procedure with root replacement technique. Forty patients were 30 years of age or below (young rheumatics). Associated procedures included mitral valve repair (n=19), open mitral commissurotomy (n=15), tricuspid valve repair (n=2), and homograft mitral valve replacement (n=2). RESULTS: Early mortality was 7.4% (six patients). Mean follow-up was 92.3+/-40.9 months (7-132 months, median 109 months). Sixty of the 73 patients whose follow-up was available (82%) had no significant aortic regurgitation. Re-operation was required in seven (8.4%) patients for autograft dysfunction with failed mitral valve repair (n=3), autograft dysfunction alone (n=2) and failed mitral valve repair alone (n=2). No re-operations were required for the pulmonary homograft. There were six (7.5%) late deaths. Actuarial survival and re-operation-free survival at 109 months were 84.5+/-4.1% and 90.5+/-3.7%, respectively. Freedom from significant aortic stenosis or regurgitation was 78.4+/-5.2% and event-free survival was 64.6+/-5.8%. When compared to rheumatics above 30 years of age, the relative risk of autograft dysfunction was high in the young rheumatics. CONCLUSION: The Ross procedure is not suitable for young patients with rheumatic heart disease. However, it provides acceptable mid-term results in carefully selected older (>30 years) patients with isolated rheumatic aortic valve disease.