Konno aortoventriculoplasty in children and adolescents: from prosthetic valves to the Ross operation

BACKGROUND: In children with complex multilevel left ventricular outflow tract obstruction (LVOTO), the Konno aortoventriculoplasty is used to enlarge the aortic root and increase the size of the aortic valve implanted. We present our experience with the evolution of this surgical approach from prosthetic valves to the Ross operation. METHODS: Between March 1982 and July 2000, 60 patients (36 males and 24 females) had 72 Konno aortoventriculoplasties (prosthetic valve and Konno group [57 patients; redo, 12] and Ross-Konno group [15 patients]). The patients' ages ranged from 5 days to 18 years (mean, 8.2 years). The underlying anatomic diagnoses were congenital aortic stenosis and LVOTO in 51 patients, coarctation of the aorta with bicuspid aortic valve in 13, severe aortic insufficiency associated with a ventricular septal defect in 8, interrupted aortic arch in 6, mitral stenosis in 6, atrioventricular septal defect in 5, and endocarditis in 3. There was no statistical difference in age or diagnosis between the two groups. The prosthetic valve group included 42 mechanical valves, 9 homografts, and 6 xenografts. RESULTS: Follow-up ranged from 10 months to 18.5 years (mean, 9.7 years) in the prosthetic valve and Konno group compared with 5 months to 3.7 years (mean, 2.1 years) in the Ross-Konno group (p < 0.05). There were 10 early deaths and four late deaths in the prosthetic valve and Konno group as compared with one early death and two late deaths in the Ross-Konno group (p = not significant). Reoperations for LVOTO and aortic valve replacement were significantly higher in the prosthetic valve and Konno group as opposed to the Ross-Konno group (16 vs 0, p < 0.05) mainly because of the biological valve and Konno subgroup. There were no statistical differences in reexploration for bleeding, pacemaker insertion, and reoperations for indications other than LVOTO and aortic valve replacement between the two groups. CONCLUSIONS: The Konno aortoventriculoplasty is a good surgical option for complex LVOTO. Patients with a prosthetic valve and Konno carry a high rate of reoperation. Early operative results with the Ross-Konno operation seem promising.     

Extended septoplasty for subaortic stenosis developed 19 years after double-outlet right ventricle repair.

A 27-year-old woman developed subaortic stenosis 19 years after double-outlet right ventricle repair. Subaortic stenosis was caused by a narrow ring of fibromuscular ridge associated with a bulge of the underlying septal muscle. The aortic valve was bicuspid and stenotic. We conducted extended septoplasty, replacing the aortic valve. Postoperative cineangiogram showed an adequate left ventricular outflow pathway. Double-outlet right ventricle repair may thus be followed by subaortic stenosis as long as 19 years after initial surgery. This lesion was assumed due to acquired disease secondary to flow disturbances in the left ventricular outflow, so reconstructing an adequate outflow pathway is effective and appears to help avoid recurring stenosis.     

Surgical treatment of subaortic stenosis: a seventeen-year experience

OBJECTIVE: The aim of the study was to analyze the long-term results of subaortic stenosis relief and the risk factors associated with recurrence and reoperation. METHODS: One hundred sixty patients with subaortic stenosis underwent biventricular repair. Before the operation the mean left ventricle-aorta gradient was 80 +/- 35 mm Hg, 57 patients had aortic regurgitation, and 34 were in New York Heart Association functional class III or IV. Median age at repair was 10 years. For discrete subaortic stenosis (n = 120), 39 patients underwent isolated membranectomy, 67 underwent membranectomy with associated septal myotomy, and 14 underwent septal myectomy. Tunnel subaortic stenosis (n = 34) was treated by myotomy in 10 cases, myectomy in 12, septoplasty in 7, Konno procedure in 3, and apical conduit in 2. Aortic valve replacement was performed in 6 cases, mitral valve replacement in 2 cases, and mitral valvuloplasty in 4 cases. RESULTS: There were 5 early (3.1%) and 4 late (4.4%) deaths. Within 3.6 +/- 3.3 years a recurrent gradient greater than 30 mm Hg was found in 42 patients (27%), 20 of whom had 26 reoperations. According to multivariable Cox regression analysis survival was influenced by hypoplastic aortic anulus (P =.01) and mitral stenosis (P =.048); recurrence and reoperation were influenced by coarctation and immediate postoperative left ventricular outflow tract gradients. At a median follow-up of 13.3 years, mean left ventricle-aorta gradient was 20 +/- 13 mm Hg. Relief of the subaortic stenosis improved the degree of aortic regurgitation in 86% of patients with preoperative aortic regurgitation. Actuarial survival and freedom from reoperation rates at 15 years were 94% +/- 1.3% and 85% +/- 6%, respectively. CONCLUSION: Although surgical treatment provides good results, recurrence and reoperation are significantly influenced by previous coarctation repair and by the quality of initial relief of subaortic stenosis.     

Surgical treatment of subaortic obstruction in adolescent and adults: long-term follow-up

Subaortic stenosis (SAS) is a wide spectrum of anatomical derangements ranging from a discrete fibrous membrane to tortuous fibrous tunnel with or without aortic annulus hypoplasia. We have reviewed 88 patients undergoing surgery for SAS over a 15-year period. There were 47 male and 41 female patients with a mean age of 19.8 +/- 10.6 years (range 11 to 39). Fifty-eight patients had discrete subaortic membrane, and 30 patients had diffuse tunnel subvalvular stenosis. The mean systolic pressure gradients were found to be 86.5 +/- 31.4 mmHg (range 48 to 145 mmHg). Ten patients had mild and 13 patients had moderate-to-severe aortic insufficiency (AI) preoperatively. Nine patients had bicuspid aortic valve. Forty patients (45.4%) had associated cardiac lesions. Isolated membranectomy was performed in six patients. Membranectomy associated with septal myectomy was done in 52 patients. Fifteen patients of them associated hypoplasia of the aortic orifice necessitated aortic valve replacement (AVR) using the Konno-Rastan procedure. Fifteen patients with tunnel SAS and normal aortic valves underwent a combined approach for valve sparing, a modified Konno procedure with patch septoplasty. Also eight patients required AVR because of the severity of AI and five patients aortic reconstruction procedures. Aortic commissurotomy was performed to relief of stenosis in four patients. There were three early deaths (3.4%) and one late death (1.1%) all after the Konno-Rastan procedure. Eight patients (9.1%) had permanent conduction abnormalities. Postoperative left ventricle-aorta gradient was significantly decreased at early postoperative period (p < 0.001) and ranged from 10 to 25 mmHg (mean 14.1 +/- 4.3). Fourteen patients (16.5%) were reoperated for recurrent obstruction or progression of AI. The mean reoperation interval was 4.4 +/- 1.7 years (range 2 to 8 years). Five-year reoperation-free survival was 88.0 +/- 3.6% and 12.5-year reoperation-free survival was 75.5 +/- 7.0%. Our results of aggressive surgical approach of subvalvular aortic stenosis produces relief of obstruction and frees the valve leaflets, significantly reducing associated AI with long-term survival and long-term adequate relief of left ventricular outflow tract obstruction.     

The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve

Patients (n = 109) operated on for coarctation of the aorta were analyzed for occurrence of associated cardiac and noncardiac anomalies. Attention was also paid to the prevalence of cardiac anomalies in the relatives of these patients. Of the patients with coarctation of the aorta, 57 (52%) had a bicuspid aortic valve. Forty-three (39%) of the 109 patients had one or more noncardiac anomalies. In 29 (27%) patients the noncardiac anomaly involved the head/neck structures. Noncardiac anomalies were much more prevalent in patients with coarctation and bicuspid aortic valve, especially anomalies involving the head/neck structures: 44% compared to 8% of patients with a normal aortic valve. Congenital cardiac malformations were present in relatives in the first or second degree of 18% of the patients. Bicuspid aortic valve was more prevalent in patients with an affected relative (75%) than in patients with unaffected relatives (47%). Recent studies showed that the neural crest plays an important role in the development of cardiac and a variety of noncardiac structures. The cardiac structures derived from the neural crest involve the outflow tract of the heart and the aortic arch system. Maldevelopment of neural crest cells could therefore be responsible for the combined occurrence of outflow tract (e.g., bicuspid aortic valve), aortic arch (e.g., coarctation), and noncardiac anomalies. This study supports the concept that some anomalies of the aortic arch system, including aortic coarctation, are cardiovascular manifestations of a spectrum of anomalies involving the head and neck region that may be due to a genetic-environmental disorder of the neural crest.     

Surgical treatment of the left circumflex coronary artery from the pulmonary artery in an adult patient

We report an exceptional case of ischemic heart disease due to the origin of the left coronary circumflex artery from the pulmonary artery in a 50-year-old woman. She had undergone surgery for aortic coarctation when she was 16 years old. This abnormality was associated with other congenital defects such as tunnel subaortic stenosis, small aortic valve annulus, numerous left ventricular false tendons, and aortic bicuspid valve. Cardiac surgery verified the origin of the left circumflex from the pulmonary artery. The left internal mammary artery was positioned on the obtuse marginal coronary branch. Her clinical state was moderately improved 3 months after surgery.     

Reduction aortoplasty for dilatation of the ascending aorta in patients with bicuspid aortic valve

BACKGROUND: Individuals with bicuspid aortic valve tend to develop a dilatation of the ascending aorta. It is controversial whether the dilated ascending aorta should be replaced with a tube graft or whether the diameter of the aorta should be reduced by reduction aortoplasty. Furthermore, it is unclear whether an external prosthetic support of the reduction aortoplasty is necessary. The aim of this study is to analyze the results of reduction aortoplasty with and without external prosthetic support. METHODS: Between 1985 and 1999, a total of 115 patients with bicuspid aortic valve and dilatation of the ascending aorta underwent reduction aortoplasty in combination with other types of open-heart procedure at our institution. The diameter of the ascending aorta was measured before and early after surgery and then later between 12 and 144 months (mean 40 months) postoperatively using echocardiography and computed tomography. RESULTS: The reduction aortoplasty decreased the internal diameter of the aorta from 48.7+/-5.1 mm preoperatively to 36.9+/-3.6 mm early after surgery (p = 0.0001). During follow-up, there was no increase of the aortic diameter either in patients with external prosthetic support or in 97 of 106 patients without external prosthetic support. The diameter increased only in 9 (8.5%) of 106 patients without external aortic support by 4 to 8 mm. In patients with postoperative diameter increase, the aortic diameter after operation had been higher than in patients without a postoperative increase of the aortic diameter (41.4+/-3.1 mm vs 36.6+/-3.4 mm; p < 0.0001). CONCLUSIONS: Reduction aortoplasty showed good long-term results in patients with bicuspid aortic valve and dilatation of the ascending aorta. Redilation of the aorta occurred only in patients with a suboptimal diameter reduction.     

The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study.

OBJECTIVE: To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). METHODS: Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). RESULTS: LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). CONCLUSIONS: LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.     

Surgical management of diffuse subaortic stenosis: an integrated approach

An integrated approach to the surgical management of diffuse subaortic stenosis has been designed to provide adequate relief of left ventricular outflow tract obstruction whatever the anatomical features encountered at operation. This approach was used in 22 patients with tunnel subaortic stenosis (19 patients) or diffuse hypertrophic obstructive cardiomyopathy (3 patients). The obstructive tissue was resected through an aortoseptal approach. In 18 patients, associated hypoplasia of the aortic orifice necessitated aortic valve replacement using the Konno procedure; in 4 patients with a normal-sized aortic orifice, the native aortic valve was preserved. There were two early deaths and one late death (all after a Konno operation). Long-term adequate relief of left ventricular outflow tract obstruction was achieved in all survivors. Operation for diffuse subaortic stenosis should be performed with two main goals: (1) to obtain complete relief of the left ventricular outflow tract obstruction by the appropriate procedure and (2) to preserve the native aortic valve whenever possible, particularly in young patients.     

70例主动脉瓣下狭窄的外科治疗

目的：总结70例主动脉瓣下狭窄的手术治疗经验。方法：全组70例中男46例，女24例；年龄3～46岁。平均11．8岁。均经超声心动图、左心导管和造影检查及术中证实为主动脉瓣下狭窄，局限型64例(纤维隔膜型42例、纤维肌隔型22例)，隧道型6例。局限型行狭窄隔膜切除术58例，加左室肌肉切除术6例；隧道型行左室流出道疏通术6例。合并畸形59例，同期行矫正手术。结果：手术死亡2例。仅l例发生二尖瓣损伤。术后38例左室主动脉收缩压力差0～30mmHg(1mmHg=0．133kPa)，平均7．5mmHg。术后经1、3、5、10、20年各随访58、54、48、32、6例，随访率分别为83％、77％、68％、45％、9％。症状均消失。无再狭窄需手术者。结论：本病一旦确诊，应尽快手术；术前超声心动图检查及术中常规主动脉根部探查，对防止有合并畸形时本病的漏诊尤为重要；手术关键是彻底疏通左室流出道，术中防止二尖瓣、主动脉瓣及传导束损伤。     

A single-stage operation for bicuspid aortic valve, annulo-aortic ectasia, hypoplastic aortic arch, and coarctation of the aorta: A case report.

The patient was an 18-year-old man who had been diagnosed as having a bicuspid aortic valve and dilatation of the ascending aorta six years previously. As he grew up, aneurysmal change of the ascending aorta and hypertension in the upper body gradually progressed. Preoperative evaluation showed annulo-aortic ectasia and the following congenital abnormalities: bicuspid aortic valve, hypoplastic aortic arch, and coarctation of the aorta. Composite graft replacement and extended total aortic arch replacement were carried out.     

Risk factors for reoperation after relief of congenital subaortic stenosis

Background: Congenital subaortic stenosis entails a lesion spectrum, ranging from an isolated obstructive membrane, to complex tunnel narrowing of the left outflow associated with other cardiac defects. We review our experience with this anomaly, and analyze risk factors leading to restenosis requiring reoperation. Methods: From 1994 to 2006, 58 children (median age 4.3 years, range 7 days–13.7 years) underwent primary relief of subaortic stenosis. Patients were divided into simple lesions (n = 43) or complex stenosis (n = 15) associated with other major cardiac defects. Age, pre- and postoperative gradient over the left outflow, associated aortic or mitral valve insufficiency, chromosomal anomalies, arteria lusoria, and operative technique (membrane resection (22) vs associated myectomy (34) vs Konno (2)) were analyzed as risk factors for reoperation (Kaplan–Meier, Cox regression). Results: There was no operative mortality. Median follow-up spanned 2.7 years (range 0.1–10), with one late death at 4 months. Reoperation was required for recurrent stenosis in 11 patients (19%) at 2.6 years (range 0.3–7.5) after initial surgery. Risk factors for reoperation included complex subaortic stenosis (p = 0.003), younger age (p = 0.012), postoperative residual gradient (p = 0.019), and the presence of an arteria lusoria (p = 0.014). For simple lesions, no variable achieved significance for stenosis recurrence. Conclusions: Surgical relief of congenital subaortic stenosis, even with complex defects, yields excellent results. Reoperation is not infrequent, and should be anticipated with younger age at operation, complex defects, residual postoperative gradient, and an arteria lusoria. Myectomy concomitant to membrane resection, even in simple lesions, does not provide enhanced freedom from reoperation, and should be tailored to anatomic findings.     

Shone's anomaly: operative results and late outcome

Shone's anomaly, a congenital cardiac malformation, consists of multiple levels of left heart obstruction including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and coarctation. The prognosis for patients with Shone's anomaly is poor. To assess operative results and late outcome, we reviewed the records of 30 consecutive patients seen with Shone's anomaly at our institution between 1966 and 1989. Anatomical diagnoses in these patients were supravalvar mitral ring (22 patients), mitral valve abnormalities including parachute mitral valve, fused chordae, or single papillary muscle (26 patients), subaortic gradients (26 patients), and coarctation (29 patients). Nineteen patients had all four lesions. Other common defects were bicuspid aortic valve (19 patients) and ventricular septal defect (20). Two patients were treated medically. The other 28 patients required 84 operative procedures with 18 patients undergoing more than one procedure. Operations included coarctation repair (28 patients), mitral valve repair or replacement (11), ventricular septal defect closure (8), subaortic resection (8), and complex left ventricular outflow tract reconstruction or bypass (4). Age at first operation ranged from 7 days to 7 years (median age, 3 months). There were no operative deaths at the first operation. However, mortality rose to 24% (4/17) after the second operation. All operative deaths were secondary to severe mitral valve disease. The survivors have been followed from 1 to 16 years (mean follow-up, 6 +/- 1 years). There were no late or sudden deaths. Morbidity has included stroke (1), gastrointestinal bleeding (2), permanent heart block (1), and persistent congestive heart failure (6).(ABSTRACT TRUNCATED AT 250 WORDS)     

Balloon angioplasty before Wheat's operation in a patient with Turner's syndrome

The case of a 19-year-old woman with Turner's syndrome, dissecting aneurysm of the ascending aorta, coarctation of the aorta and bicuspid aortic valve stenosis is presented. Balloon angioplasty was performed before surgery to relieve the pressure gradient caused by coarctation of the aorta. Successful surgical treatment was carried out using Wheat's method. Four-year follow-up has revealed no signs of complications such as restenosis or aneurysm formation.     

Aneurysm of the ascending aorta in its coarctation

In long-term period after resection of aorta coarctation, 9-14% patients developed aortic aneurysms, one third of them localized in the ascending aorta. From 146 patients operated on for aneurysm of the ascending aorta, 3 had aortic coarctation. In 2 patients aneurysms of the ascending aorta formed late after resection of the coarctation, the third patient was hospitalized with clinical picture of cardiac insufficiency at terminal stage due to coarctation of the aorta and a giant aneurysm of the ascending aorta with significant aortic insufficiency. The patients underwent successful surgeries: 1) ascending aorta grafting; 2) aortic valve and ascending aorta grafting by Bentallo de Bono method; 3) aortic valve and ascending aorta grafting by Bentallo de Bono method with bypass of descending aorta from the conduit. Cystic medianecrosis and two-volume aortic valve were revealed in all the patients. It is concluded that patients after surgery for coarctation of the aorta require long-term follow-up to defect cardiovascular complications early.     

Treatment of coarctation and late complications in the adult

This article discusses coarctation of the aorta in the adult. Effective treatments for coarctation have come from surgery since 1945 and from interventional cardiology since 1982. Long-term outcome data are available only for surgical approaches. Thirty-year survival rate is 72% to 82%. Complications include recoarctation or residual coarctation, hypertension, aneurysms at the repair site, spinal cord injury. Other sequelae include bicuspid aortic valve disease, ascending aortic aneurysm, premature coronary disease, and infective endocarditis or endarteritis. Interventional catheter therapy is now the preferred therapy for recurrent coarctation, when the anatomy permits and necessary skills are available. Its use in native or unoperated coarctation is less well established. Treatment may be with balloon angioplasty alone or with a stent. Outcomes are good in skilled hands, but residual or recurrent coarctation with resultant hypertension and repair site aneurysms can occur. Catheter treatment can cause death from aortic rupture and dissection, but mortality compares favorably with surgery if coarctation is recurrent, and perhaps for initial treatment.     

Single-stage surgical repair of type II acute aortic dissection associated with coarctation of the aorta

A 36-year-old man, who was referred for severe chest pains, was found to have acute type II aortic dissection associated with a dilated ascending aorta, aortic coarctation, and congenitally bicuspid valve. A single-stage surgical repair consisting of valve-sparing aortic root implantation and graft replacement of the ascending aorta, arch, and the coarctation segment was successful.     

Surgical management of patients with interrupted aortic arch and severe subaortic stenosis

Ten patients underwent palliative surgery for interrupted aortic arch and severe subaortic obstruction due to posterior displacement of the conal septum. Their ages ranged between 4 and 28 days (mean, 11.0 +/- 7.7 days) and their weights, between 2.1 and 4.2 kg (mean, 2.85 +/- 0.6 kg). Preoperative echocardiography and cardiac catheterization were performed on all patients. The ratios of the left ventricular outflow tract diameters and the ascending aortic diameters to the descending aortic diameters were 0.56 +/- 0.03 and 0.56 +/- 0.06, respectively, compared with 0.81 +/- 0.12 and 0.95 +/- 0.17, respectively, in 20 patients with interrupted aortic arch but without obstruction (p less than 0.001). Four of the 10 patients underwent pulmonary artery banding and insertion of a bypass graft between the ascending and the descending aorta. All 4 died of low cardiac output soon after operation (100% operative mortality). The remaining 6 patients underwent banding and insertion of a graft between the main pulmonary artery proximal to the band, and the descending aorta. All of these patients survived, and all except 1 are doing well 3 months to 4 years postoperatively. The use of a pulmonary artery-descending aorta conduit and of distal pulmonary artery banding provides good palliation for patients with interrupted aortic arch and major subaortic stenosis.     

Posterior pericardial approach for ascending aorta-to-descending aorta bypass through a median sternotomy

BACKGROUND: Bypass grafting for repeat operation or complex forms of descending aortic disease is an alternative approach to decrease potential complications of anatomic repair. METHODS: Between December 1985 and February 1998, 17 patients (13 men, 4 women; mean age, 47.6 +/- 18.5 years) underwent ascending aorta-to-descending aorta bypass through a median sternotomy and posterior pericardial approach. Indications for operation were coarctation or recoarctation of aorta in 8 patients, Takayasu's aortitis in 2, prosthetic aortic valve stenosis associated with coarctation of aorta, complex descending aortic arch aneurysm, reoperation for chronic descending aortic dissection, long-segment stenosis of descending aorta, acquired coarctation after repair of traumatic transection of descending aorta, severe aortic atherosclerosis, and false aneurysm of descending aorta after repair of coarctation in 1 patient each. Concomitant procedures were performed in 12 patients. RESULTS: No early or late mortality has occurred. Follow-up was 100% complete and extended to 12 years (mean, 2.7 +/- 3.3 years). No late graft-related complications have occurred; 1 patient had successful repair of perivalvular leak after mitral valve replacement, and 1 patient had replacement of lower descending and abdominal aorta. CONCLUSIONS: Exposure of the descending aorta through the posterior pericardium for ascending aorta-descending aorta bypass is a safe alternative and particularly useful when simultaneous intracardiac repair is necessary.     

Outcome following single-stage repair of coarctation with ventricular septal defect.

OBJECTIVE: A recent multi-institutional study suggested improved survival (97%) after staged repair of coarctation with ventricular septal defect (VSD) compared with single-stage repair. The current study was undertaken to determine outcome and need for reintervention following single-stage repair of coarctation and VSD at our institution. METHODS: Retrospective review of patients undergoing single-stage repair of coarctation with VSD between October 1, 1994 and August 15, 1999. RESULTS: Single-stage repair of coarctation with VSD was performed in 25 infants (12 males, 13 females) at a median age of 12 days (range 1-87 days) and median weight of 3.3 kg (range 1.3-4.4 kg). The VSD was conoventricular in ten patients, malalignment type with posterior deviation of the infundibular septum in ten, muscular in four and conal septal hypoplasia in one. Arch hypoplasia was present in all patients and bicuspid aortic valve in 13. At least moderate subaortic narrowing was present in six patients (secondary to prolapse of tricuspid valve tissue across the VSD in four). Repairs were performed via a median sternotomy with a mean circulatory arrest time of 38+/-12 min. Overall patient survival was 96% with one operative death and no late deaths at a median follow-up of 16 months (range 1-50 months). Reinterventions included balloon dilatation of recurrent coarctation (five), closure of residual VSD (one) and Ross-Konno procedure (two). Actuarial freedom from reintervention for the hospital survivors was 81% (95% confidence limit (CL) 61%, 92%) at 6 months, 71% (95% CL 47%, 87%) at 1 year and 59% (95% CL 31%, 82%) at 2 years following surgery. CONCLUSION: Single-stage repair of coarctation with VSD can be performed with low operative mortality and good midterm survival equivalent to reported results for staged repair.