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Ann Thorac Surg 1998;65:1368-1376
© 1998 The Society of Thoracic Surgeons

Modified Konno-Rastan Procedure for Subaortic Stenosis: Indications, Operative Techniques, and Results

Patrick T. Roughneen, FRCSa, Serafin Y. DeLeon, MDa, Frank Cetta, MDb, Dolores A. Vitullo, MDb, Timothy J. Bell, MDb, Elizabeth A. Fisher, MDb, Bradford P. Blakeman, MDa, Mamdouh Bakhos, MDa

a Department of Thoracic-Cardiovascular Surgery, Loyola University Stritch School of Medicine, Maywood, Illinois, USA
b Department of Pediatrics, Loyola University Stritch School of Medicine, Maywood, Illinois, USA

Address reprint requests to Dr Roughneen, Department of Thoracic-Cardiovascular Surgery, Loyola University Medical Center, 2160 South First Ave, Maywood, IL 60153

Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 26–28, 1998.


   Abstract
Top
 Abstract
Introduction
 Materials and methods
 Results
 Comment
 Acknowledgments
 References
 
Background. Diffuse or unresectable subaortic stenosis (SAS) necessitates an aggressive surgical approach for the elimination of left ventricular outflow tract obstruction. In this article we report our experience with the modified Konno-Rastan procedure, with inherent preservation of the native aortic valve and annulus, in the treatment of diffuse or unresectable SAS.

Methods. Sixteen children (age range, 21 months to 18 years) underwent the modified Konno-Rastan procedure through either a transventricular (n = 12) or a transatrial approach (n = 4) to the conal septum. Indications for operation were recurrent SAS (n = 3), hypertrophic obstructive cardiomyopathy (n = 3), tunnel stenosis (n = 2), SAS related to a canal (n = 3), and SAS after ventricular septal defect closure (n = 5). Eleven patients had undergone previous procedures and 5 underwent the modified Konno-Rastan procedure as their primary operation.

Results. The mean preoperative left ventricular outflow tract gradient of 50 ± 17 mm Hg was reduced to 3 ± 7 mm Hg (p < 0.001) after surgical repair. Postoperative complications included sternal infection (n = 1), heart block (n = 2), mediastinal bleeding (n = 1), and renal and cerebral ischemia (n = 1). There was 1 late postoperative death caused by pneumonia 2 years after operation (6.2% mortality rate). The mean follow-up period was 62 ± 39 months and all patients had complete relief of preoperative symptoms and were in New York Heart Association class I. One patient underwent a successful redo modified Konno-Rastan procedure 7 years after the first operation for residual left ventricular outflow tract obstruction immediately below the aortic valve. One patient is awaiting reoperation for aortic incompetence unrelated to conal enlargement 1.5 years after the first procedure.

Conclusions. The modified Konno-Rastan procedure represents an excellent therapy for diffuse or unresectable SAS in patients with a normal aortic valve. In addition, it produces excellent results in a limited number of patients with hypertrophic obstructive cardiomyopathy, in whom the Morrow procedure traditionally has been performed. Although it usually is performed through a transventricular approach, the modified Konno-Rastan procedure also can be performed through a transatrial approach; this is particularly useful in patients who have had previous ventricular septal defect closure associated with SAS occurring proximal to the prosthetic patch.


   Introduction
Top
 Abstract
 Introduction
Materials and methods
 Results
 Comment
 Acknowledgments
 References
 
Diffuse or unresectable subaortic stenosis (SAS) requires an aggressive surgical approach for effective relief of left ventricular outflow tract obstruction. Aortoventriculoplasty (the Konno-Rastan procedure) [1, 2], apicoaortic conduits [3], and, more recently, the Ross-Konno procedure [4] all have been used successfully in the treatment of diffuse left ventricular outflow tract obstruction. However, all these procedures require a prosthetic or allogeneic valve as well as postoperative anticoagulation therapy, reoperation, or both. The modified Konno-Rastan procedure, which was described by Kirklin [5] and Cooley [6] and ensures augmentation of the left ventricular outflow tract with inherent preservation of the native aortic valve, has hitherto received little attention in the literature [7, 8]. We have used the modified Konno-Rastan procedure in patients with various forms of diffuse or unresectable SAS. We present our experience with this technique and discuss our indications, technical modifications, and results.


   Materials and methods
Top
 Abstract
 Introduction
 Materials and methods
Results
 Comment
 Acknowledgments
 References
 
Patients
Over a 10-year period, we performed the modified Konno-Rastan Procedure in 16 patients with diffuse or unresectable SAS. Their diagnoses, previous operations, surgical indications, and preoperative echocardiographic or angiographic examinations were evaluated. Their operative records, postoperative echocardiographic or an giographic examinations, functional class, and follow-up were analyzed in a retrospective fashion.

The diagnoses of the patients who underwent the modified Konno-Rastan procedure are listed in Table 1. Indications for the modified Konno-Rastan procedure included recurrent SAS previously treated through a transaortic approach (n = 3), tunnel stenosis (n = 3), SAS associated with a canal (n = 3), SAS associated with previous ventricular septal defect (VSD) repair (n = 4), and hypertrophic obstructive cardiomyopathy (n = 3). Of the 16 patients treated, 12 underwent a transventricular repair. Four patients underwent a transatrial repair, including 3 patients with SAS located proximal to a previously closed VSD. Among the entire study population, 5 patients underwent the modified Konno-Rastan procedure as their first operation for SAS (Table 1). Eleven patients had undergone previous operations, which included fibromuscular SAS resection (n = 3), canal repair (n = 3), and VSD repair (n = 5). In 4 of the 5 patients who had undergone previous VSD repair, the following procedures also were performed: aortic valvotomy (n = 1), resection of SAS (n = 2), and repair of atrial septal defect and partial anomalous pulmonary venous return (n = 1).


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  		Table 1. Clinical Characteristics of 16 Patients Who Underwent the Modified Konno-Rastan Procedure

 
Statistical analysis
All results are expressed as the mean plus or minus the standard deviation. Results were subject to the Student’s paired t-test for statistical analysis. A p value of less than 0.05 was considered statistically significant.

Surgical technique
All procedures were performed through a median sternotomy using cardiopulmonary bypass and moderate hypothermia. Cannulation of the aorta was performed, followed by cannulation of the superior and inferior venae cavae through the right atrial wall. The heart was arrested with antegrade cold blood cardioplegia. In more recent patients, intermittent retrograde cardioplegia also was administered through the coronary sinus every 10 minutes.

An incision was made in the aorta to evaluate the left ventricular outflow tract obstruction from a perspective above the valve. Then either a right ventricular outflow tract incision (n = 12) (Fig 1, 2) or a right atrial incision (n = 4) (Fig 3, 4) was made and the septum was incised. In cases in which the stenosis was resected through a transventricular incision, a conal incision was made inferior to the pulmonary valve. Resection of the obstructive tissue then was performed, with care taken not to injure the aortic valve or the conduction system and with the excision of obstructive tissue performed mainly on the left side of the conal incision to avoid injury to the right bundle. A polytetrafluoroethylene patch then was fashioned and sewn onto the incised septum (see Figs 1B, 2B). The right ventricular outflow tract also was enlarged with a polytetrafluoroethylene patch.



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  		Fig 1. Modified Konno-Rastan procedure performed through a transaortic transventricular approach. (A) Transaortic and transventricular incisions. (B) Completed repair.

 


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  		Fig 2. Modified Konno-Rastan procedure in a patient with hypertrophic obstructive cardiomyopathy performed through a transaortic transventricular approach. (A) Incision in the conal septum (arrow). (B) Polytetrafluoroethylene graft in place.

 


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  		Fig 3. Modified Konno-Rastan procedure performed through a transaortic transatrial approach. (A) The transatrial repair showing the incision inferior to the ventricular septal defect (VSD) patch. (B) The completed repair.

 


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  		Fig 4. Modified Konno-Rastan procedure in a patient with subaortic stenosis associated with previous ventricular septal defect repair performed through a transaortic transatrial approach. (A) Incision made in the ventricular septal defect patch and septum (arrow). (B) Polytetrafluoroethylene graft in place.

 
In the 3 patients in whom the SAS was proximal to a previously closed VSD, a modified Konno-Rastan procedure through a transatrial approach was used (see Figs 3, 4). An incision was made on the VSD patch or conal septum and directed inferiorly to the site of the obstruction. After the obstruction was relieved, the septum was enlarged with a polytetrafluoroethylene patch and the atriotomy was closed. A transatrial approach also was used in 1 patient (patient 16) who had tunnel stenosis after an attempted transaortic resection. The aorta then was closed primarily in a standard fashion and the cross-clamp was released. The patient was rewarmed, decannulated, and administered protamine.


   Results
Top
 Abstract
 Introduction
 Materials and methods
 Results
Comment
 Acknowledgments
 References
 
After the modified Konno-Rastan procedure, the mean preoperative gradient across the ventricular outflow tract of 50 ± 17 mm Hg (range, 20 to 80 mm Hg) was reduced to 3 ± 7 mm Hg (range, 0 to 20 mm Hg) (p < 0.001). Postoperative complications included sternal infection (n = 1), heart block (n = 2), mediastinal bleeding (n = 1), and renal and cerebral ischemia (n = 1). Representative postoperative echocardiographic studies demonstrated unobstructed left ventricular outflow tract anatomy (Fig 5). The mean follow-up period was 62 ± 39 months (range, 2 to 115 months). No patient had evidence of a significant VSD leak by echocardiography after repair.



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  		Fig 5. Echocardiographic studies of patients who underwent the modified Konno-Rastan procedure for hypertrophic obstructive cardiomyopathy (A, preoperative; B, postoperative) and for subaortic stenosis after ventricular septal defect repair (C, preoperative; D, postoperative).

 
There has been 1 late death (6.2% mortality rate) in a patient (patient 8) who previously had undergone repair of a VSD, and atrial septal defect, and partial anomalous pulmonary venous return. This patient required a modified Konno-Rastan procedure for SAS distal to a previously closed VSD. Her gradient was reduced from 64 mm Hg before operation to 0 mm Hg after operation. The patient did well thereafter, but then died of pneumonia 2 years later.

Of the 15 surviving patients, 2 have postoperative gradients of 20 mm Hg across the left ventricular outflow tract. One of these 2 patients (patient 10) underwent a redo modified Konno-Rastan procedure for relief of the residual obstruction 7 years after the original operation. The obstruction was just inferior to the aortic annulus, with a gradient of 60 mm Hg. This patient was one of our earliest cases and we believe that the postoperative obstruction occurred because aggressive division of the conal septum to the level of the aortic annulus was not accomplished at the initial procedure. The patient tolerated the redo procedure well and has complete elimination of the left ventricular outflow tract gradient. The other patient (patient 16) underwent attempted transaortic resection followed by a transatrial modified Konno-Rastan procedure for tunnel stenosis. After the operation, the patient had a gradient of 20 mm Hg across the left ventricular outflow tract on echocardiographic examination; this has not increased over 98 months of follow-up.

Another patient (patient 1) had a bicuspid aortic valve and moderately severe moderate aortic regurgitation after the operation. This patient initially had coarctation repair and pulmonary artery banding for a large VSD, followed by closure of the VSD, debanding, and aortic valvotomy. Two more aortic valvotomies also had been performed, including an extended valvotomy [9], after which moderately severe aortic regurgitation developed. In retrospect, the SAS resulted from a malaligned VSD. The patient is now 18 months after operation and being followed up closely with consideration of the Ross procedure. In 15 of 16 patients, follow-up echocardiography has demonstrated normal function of the aortic valve and a normal-sized annulus.


   Comment
Top
 Abstract
 Introduction
 Materials and methods
 Results
 Comment
Acknowledgments
 References
 
The first description of SAS is credited to Chevers in 1842 [10]. Brock and Flemming [11] first reported on transventricular dilatation in 1956. Spencer and colleagues [12] thereafter reported on SAS repair using cardiopulmonary bypass in 1958, and then described the surgical management of diffuse tunnel stenosis in 1960 [13]. The independent description of aortoventriculoplasty by Konno and associates [1] in 1975 and Rastan and Kaney [2] in 1976 led to its effective treatment. In 1975, Cooley and coworkers [3] described the apicoaortic conduit as an alternative form of therapy for severe left ventricular outflow tract obstruction. In 1984, Vouhé and colleagues [14] described the resection of SAS by division of the aortic annulus and resection of the infundibular obstruction, with primary reconstruction of the annulus and the conal septum. This technique advanced the concept of conal incision for SAS with preservation of the aortic valve.

This concept was advanced further by Kirklin and Barratt-Boyes [5] (modified Konno procedure) to include conal enlargement with a patch and preservation of the aortic valve. Cooley and Garrett [6] reported a similar technique and called it "septoplasty." Both these techniques have a marked advantage in that they preserve the aortic valve, avoid the anticoagulation therapy required with a prosthetic valve, and minimize the need for reoperation in young patients. In essence, the modified Konno-Rastan procedure represents a ventriculoplasty rather than an aortoventriculoplasty, as in the classic Konno-Rastan procedure, because the integrity of the aortic annulus and valve are maintained. In our patient population, there were five indications for the modified Konno-Rastan procedure: recurrent SAS, tunnel SAS, SAS associated with endocardial cushion defects, SAS after VSD repair, and hypertrophic obstructive cardiomyopathy.

Discrete subvalvar stenosis of a fibrous or a fibromuscular type is treated best by excision through the aortic valve [15]. However, with the use of this technique, obstructive anatomy recurs in 10% to 40% of patients [16]. Such recurrence probably is caused by hypoplasia of the outflow tract in some patients, resulting in progressive deposition of obstructive tissue. In patients with recurrent stenosis in whom the aortic valve and annulus are satisfactory, we consider the modified Konno-Rastan procedure to be the operation of choice.

The experience of Kirklin and Barratt-Boyes [5] at Green Lane Hospital has demonstrated that most early and late deaths in patients with discrete subvalvar stenosis occur in those with postoperative left ventricle-to-aorta gradients of greater than 20 mm Hg. Hence, complete or nearly complete elimination of the preoperative gradient is of paramount importance, and in the event that simple excision of localized SAS does not adequately relieve the preoperative gradient, a modified Konno-Rastan procedure should be performed. Preoperative and intraoperative echocardiography, as well as careful intraoperative evaluation of the left ventricular outflow tract, will help identify those patients who are not amenable to simple resection.

Tunnel stenosis is less common than discrete fibrous subvalvar stenosis and accounts for approximately 20% of all cases of subvalvar stenosis. Tunnel stenosis has varying degrees of severity and its spectrum blends into localized subvalvar stenosis, which explains the variation in its incidence in reported series. Simple excision is inadequate in the presence of tunnel stenosis. This opinion is supported by a report by Wright and associates [16], who demonstrated that simple resection of the obstruction through the aortic valve in 6 patients with tunnel stenosis lowered the mean left ventricular outflow tract gradient by 30 ± 17 mm Hg, compared with 52 ± 40 mm Hg in patients with discrete subaortic obstruction. The modified Konno-Rastan procedure is the best surgical option for the tunnel type of SAS in patients who have a normal aortic valve and annulus.

A third group in which the modified Konno-Rastan procedure is indicated are those patients who have SAS in association with endocardial cushion defects. In these patients, the subaortic obstruction may be localized and of the tunnel type or secondary to abnormalities of the valve [17–20]. Such abnormalities include anterior displacement of the mitral valve, malattachment of the chordae and papillary muscle, accessory mitral leaflets, and the presence of accessory tricuspid tissue protruding through a ventricular septal defect into the outflow tract. Localized and diffuse SAS have a combined incidence of approximately 3% to 7% in this cohort [17–19]. The deficiency of the muscular septum and the abnormal displacement of the mitral valve result in a long left ventricular outflow tract with a predisposition toward SAS.

Depending on the anatomic features of the obstruction, surgical options in this subgroup include simple resection, the modified Konno-Rastan procedure, mitral valve replacement, and repositioning of the malattached mitral valve from the outflow tract. In patients who require operation for SAS, simple resection alone may not be appropriate; it may result in recurrence and the need for more aggressive resection of the obstruction (eg, the modified Konno-Rastan procedure, anterior leaflet uplifting), with a high mortality rate. In patients who previously have undergone repair of endocardial cushion defects, the possibility that SAS could develop must be borne in mind. Closure of the cleft of the anterior leaflet of the mitral valve may contribute to narrowing of the left ventricular outflow tract [19]. Therefore, long-term surveillance of these patients is necessary.

The fourth subset of patients who require the modified Konno-Rastan procedure are those who have SAS associated with previous VSD closure. In these patients, the stenosis usually is proximal to the VSD patch. It is of interest that Newfield and coworkers [21] reported a 20% incidence of VSD in patients with discrete subvalvar aortic stenosis. This outflow tract obstruction is progressive and often becomes hemodynamically significant after closure of the VSD, as demonstrated by 5 such cases in our series. Two patients with malaligned VSD had obstruction distal to the VSD and 3 patients with normal positioning of the conal septum had obstruction proximal to the VSD patch. Hence, careful follow-up of these patients is important to detect the postoperative development of obstructive pathology.

In patients who have SAS after VSD closure, we believe the modified Konno-Rastan procedure should be performed through a transatrial approach [22]. This avoids a right ventriculotomy and the need for augmentation of the right ventricular outflow tract (Fig 3, 4). Patch enlargement of the left ventricular outflow tract is carried out by opening the VSD patch through the tricuspid valve and extending the incision either proximally or distally through the area of the obstruction and the left ventricular body. With conal enlargement performed through a right atrial approach, all our patients had satisfactory relief of their preoperative gradients and none required a right ventriculotomy. We believe that the transatrial approach is both simple and effective in relieving SAS associated with previous VSD closure. We do not believe that the transatrial approach should be used routinely for patients with SAS that is not associated with previous VSD closure. In these patients, the SAS tends to be more distal than that seen after VSD closure and a right ventriculotomy approach allows for division of the conal septum all the way up to the aortic annulus.

The final subset of patients who underwent the modified Konno-Rastan procedure in our series were those who had hypertrophic obstructive cardiomyopathy as their primary pathology. Three of our patients had this pathology, for which the Morrow procedure traditionally has been performed [23]. Although septal myotomy-myectomy can significantly reduce or eliminate resting outflow gradients, some persist with exercise [24]. We previously have reported on the benefits of the modified Konno-Rastan procedure in patients with hypertrophic obstructive cardiomyopathy [25]. This procedure ensures complete relief of gradients across the outflow tract; our 3 patients had complete relief of symptoms and preoperative gradients after operation. In addition, we have demonstrated a reduction in left ventricular free wall and septal thickness after the modified Konno-Rastan procedure. It has been documented that patients with obstructive cardiomyopathy who receive surgical treatment do better both symptomatically and hemodynamically [26]. We propose that the modified Konno-Rastan procedure represents an alternative to the Morrow procedure [23] in the treatment of this disease because of its more complete relief of outflow gradients. Residual dynamic gradients represent an increased risk in this patient cohort, and we believe that the modified Konno-Rastan procedure can be carried out with morbidity and mortality comparable to that of the Morrow procedure.

Complications of the modified Konno-Rastan procedure are similar to those encountered during aortoventriculopasty [1, 2], the Ross procedure [27], and the Morrow procedure [23], and include VSD, injury to the conduction system, infection of the prosthetic material used in the repair of the left ventricular outflow tract, and impaired right heart function after ventriculotomy. Two of our patients (12.5%) had complete heart block after operation and required a permanent pacemaker. One of these patients had left bundle-branch block from previous transaortic resection of SAS before the modified Konno-Rastan procedure was performed. The other patient underwent attempted transaortic resection of SAS. It became obvious that the left ventricular outflow tract was hypoplastic and a transatrial modified Konno-Rastan procedure was performed. Although the conal incision is anterior to the bundle of His, the right bundle is susceptible to injury and complete heart block will develop if the left bundle is injured through transaortic resection. We believe that this complication can be minimized by staying on the left side of the conal septum during the resection of obstructive tissues. The patient in whom cerebral infarction and transient renal insufficiency developed had undergone concomitant aortic arch enlargement and repair of coarctation. These complications were related more closely to these procedures than to the modified Konno-Rastan procedure. Although injury to a dominant first septal artery is possible, we have not encountered this complication. Preoperative angiography may play a role in avoiding this problem.

An alternative surgical approach to the relief of SAS with aortic valve preservation has been described by Vouhé and colleagues [7, 14]. This group reported their experience with the use of an aortoseptal approach to the subaortic area accomplished through division of the commissure between the left and right coronary sinuses. By simultaneously traversing the infundibulum of the right ventricle, they approached the subaortic region, excised the stenosis, and repaired the divided tissues. The aortic annulus was reconstructed without injury to the left and right cusp [14]. These investigators did comment that reconstruction of the interleaflet triangle may make the annulus a little narrower, which may be a drawback in patients with a borderline annulus [7]. Vouhé and associates [7] achieved satisfactory relief of outflow gradients (mean, 6 ± 10 mm Hg; range, 0 to 25 mm Hg) in their series with the use of the aortoseptal approach. Aortic regurgitation occurred in 1 patient among a total of 11 patients who underwent aortoseptal repair (n = 5) or conal enlargement without aortic annulus division (n = 6) [7]. The overall mortality rate in their series was 9% and the reoperation rate was 20%. This approach, combined with the patch enlargement of the conal septum that is included in the modified Konno-Rastan procedure, may have a role in ensuring complete incision and relief of subaortic obstruction in some patients.

In 1987, Clarke [28] described aortoventriculoplasty performed by a "miniroot" technique as an alternative to the modified Konno-Rastan procedure in patients with combined valvar and subvalvar aortic stenosis. In this technique, an aortic homograft with coronary reimplantation is used to repair the valvar stenosis along with the associated subvalvar stenosis. Other groups have described a similar procedure with the use of a pulmonary autograft in the aortic position (Ross-Konno procedure) to lessen the need for reoperation on the left ventricular outflow tract [4]. This procedure has clear advantages in pediatric patients in whom the aortic valve is diseased.

In conclusion, we believe that the modified Konno-Rastan procedure is the procedure of choice in the treatment of patients with diffuse or unresectable left ventricular outflow tract obstruction at the subaortic level in whom the annulus is of satisfactory size and the aortic valve is either normal or reparable. This procedure can be performed through a transventricular or a transatrial approach. We believe that the procedure produces maximum surgical benefit, with complete relief of left ventricular outflow tract gradients and preservation of the native aortic valve. In addition, we believe that the modified Konno-Rastan procedure can be considered as an alternative to the Morrow procedure for surgical candidates with hypertrophic obstructive cardiomyopathy.


   Acknowledgments
Top
 Abstract
 Introduction
 Materials and methods
 Results
 Comment
 Acknowledgments
References
 
We thank Anne Mikesh for her excellent help in the preparation of the manuscript.


   References
Top
 Abstract
 Introduction
 Materials and methods
 Results
 Comment
 Acknowledgments
 References
 

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