Subaortic Resection Research Articles

Modified Konno Procedure for left ventricular outflow tract obstruction: report of two cases

Left ventricular outflow tract obstruction with a systolic anterior motion of the mitral valve is a challenging pathology. An intervention, either surgically or with a percutaneous technique is taken into consideration when the pressure gradient reaches critical levels. Subaortic myectomy is still the gold standard treatment modality in these particular patients. Modified Konno procedure may be added to the procedure when a significant gradient persists following subaortic resection, because of its association with increased outflow tract obstruction relief. We herein present our experiences with two cases that underwent modified Konno procedures for the treatment of hypertrophic obstructive cardiomyopathy.

Incidence and management of the left ventricular outflow obstruction in patients with atrioventricular septal defects.

OBJECTIVESLeft ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution.METHODSFrom 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed.RESULTSThe incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7–96.7] and 95% (95% CI; 91.1–97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4–6.7] and 2.6 years (IQR: 2.2–4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2–98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2–65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4–8.9) and 8.6 years (IQR: 5.7–9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4–72.9).CONCLUSIONSIncidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent.

First Harvest of Pediatric and Congenital Heart Surgery Multicenter Database in Turkey: Novel Application of Real-Time Online Reporting.

A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

Left main coronary artery atresia in an infant with Shone’s complex

A 6-month-old infant with Shone's complex was found to have left main coronary artery atresia during evaluation for recurrent subaortic stenosis with depressed left ventricular function. The ventricular function improved after surgical subaortic resection without coronary re-vascularisation. This case demonstrates first the rare finding of left main coronary artery atresia and second that coronary re-vascularisation is not necessarily required in all cases of left main coronary artery atresia.

Management of Interrupted Aortic Arch

The introduction of prostaglandin E1 in the late 1970s revolutionized the management of interrupted aortic arch. Complete resuscitation should proceed over several days if necessary before surgery is undertaken. One-stage primary neonatal repair with direct arch anastomosis and ventricular septal defect closure is the preferred surgical approach. Selective cerebral perfusion with near-infrared monitoring is being used with increasing frequency. Although repair of interrupted arch is physiologically corrective, it should not be viewed as fully corrective because of the high incidence of important late left ventricular outflow tract obstruction. This may respond to a simple surgical reintervention such as subaortic resection, but in some cases, an extensive procedure to enlarge the left ventricular outflow tract is necessary. However, procedures directed against subaortic stenosis should rarely be used as part of the initial surgical management during the neonatal period. Careful developmental follow-up is needed for all patients because of the high incidence of DiGeorge syndrome, which frequently manifests as moderately severe developmental delay.

RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION

Aim . To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fi brosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension. Description . Patient F., 15 years old, was diagnosed with Congenital Heart Disease – double-chambered right ventricle, ventricular septal defect. Fibromuscular subaortic stenosis. Aortic valve insuffi ciency II. Circulatory failure II A., functional class III. Extrahepatic portal hypertension. Splenomegaly. Thrombocytopenia. The state after the imposition of splenorenal anastomosis in September 1998 and the imposition of mesocaval H-shaped anastomosis in 1998. Non-functioning anastomoses. Patient indicated for surgical correction of CHD, however, given the low level of platelets, expressed splenomegaly, leucopenia, the patient was referred for a preliminary treatment to Rogachev’s Pediatric Hematology, Oncology and Immunology Center. The treatment (stimulating thrombopoiesis using romiplostim) gave no signifi cant effect. Platelet count reached 70–90 A~ 10 9 . Seeing of frequent bleeding from esophageal varices, the patient underwent varix sclerosis. At the time of hospitalization – no esophageal varices. The patient appealed to V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs where he was recommended for surgical treatment. Result . The patient performed surgery: radical correction of CHD: resection of the stenosis of the outfl ow tract of the right ventricle, subaortic stenosis resection, closure of ventricular septal defect, AV plasty under cardiopulmonary bypass. The early postoperative period was uneventful. Leukopenia was observed to 1,2 A~ 10 9 , thrombocytopenia 70–90 A~ 10 9 . Despite the low level of platelets bleeding in pre-and postoperative period was not registered. Antibiotic therapy with tienam. Good postoperative results were obtained. Liver function was not affected. Conclusion . Presented clinical case demonstrates the possibility of surgical intervention of combined heart disease with cardiopulmonary bypass in patient with extrahepatic portal hypertension.

Predictors of Reintervention After Repair of Interrupted Aortic Arch With Ventricular Septal Defect

Left ventricular outflow tract obstruction after neonatal repair of interrupted aortic arch with ventricular septal defect may warrant reintervention. We sought to identify clinical and preoperative echocardiographic predictors of reintervention for postoperative left ventricular outflow tract obstruction. Retrospective data were collected on neonates with interrupted aortic arch with ventricular septal defect who underwent single-stage repair from 1995 to 2009. Univariate and multivariate analyses were performed to identify predictors of reintervention. Seventy patients underwent repair, with 16 patients requiring reintervention: 8 underwent surgical reintervention, 5 underwent percutaneous reintervention, and 3 underwent both. The median time to reintervention was 1.2 years (range, 0.2 to 7.7). All surgical reoperations involved subaortic resection, and all percutaneous reinterventions included balloon aortic valve dilation. Several preoperative echocardiographic measurements were significant by univariate analysis; however, smaller preoperative aortic root size was an independent predictor (p = 0.02) by multivariate analysis. Patients with an aortic root size less than 6.5 mm were at greater risk for reintervention compared with patients with a root size greater than 6.5 mm (reintervention rate 44% and 12%, respectively; p < 0.001). Postoperative left ventricular outflow tract gradient by echocardiogram before discharge was significantly higher in the reintervention group. Preoperative aortic root size predicts reintervention for postoperative left ventricular outflow tract obstruction after single-stage repair of interrupted aortic arch with ventricular septal defect. Patients with elevated left ventricular outflow tract gradients at discharge are at higher risk of having progressive obstruction and require closer follow-up to ensure early identification and management.

Fontan completions over 10 years after Glenn procedures

Despite the broadened indications for Fontan procedure, there are patients who could not proceed to Fontan procedure because of the strict Fontan criteria during the early period. Some patients suffer from post-Glenn complications such as hypoxia, arrhythmia, or fatigue with exertion long after the Glenn procedure. We explored the possibility of Fontan completion for those patients. Between 2004 and 2010, five consecutive patients aged between 13 and 31 years (median 21) underwent Fontan completion. These patients had been followed up for more than 10 years (10 to 13, median 11) after Glenn procedure as non-Fontan candidates. We summarise these patients retrospectively in terms of their pre-operative physiological condition, surgical strategy, and problems that these patients hold. Pre-operative catheterisation showed pulmonary vascular resistance ranging from 0.9 to 3.7 (median 2.2), pulmonary to systemic flow ratio of 0.3 to 1.6 (median 0.9), and two patients had significant aortopulmonary collaterals. Extracardiac total cavopulmonary connections were performed in three patients, lateral tunnel total cavopulmonary connection in one patient, and intracardiac total cavopulmonary connection in one patient, without a surgical fenestration. Concomitant surgeries were required including valve surgeries--atrioventricular valve plasty in three patients and tricuspid valve replacement in one patient; systemic outflow tract obstruction release--Damus-Kaye-Stansel procedure in two patients and subaortic stenosis resection in one patient; and anti-arrhythmic therapies--maze procedure in two patients, cryoablation in two patients, and pacemaker implantation in two patients. All patients are now in New York Heart Association category I. Patients often suffer from post-Glenn complications. Of those, if they are re-examined carefully, some may have a chance to undergo Fontan completion and benefit from it. Multiple lesions such as atrioventricular valve regurgitation, systemic outflow obstruction, or arrhythmia should be surgically repaired concomitantly.

Single-centre experience of 101 paediatric and adult Ross procedures: mid-term results

We aimed to determine outcomes for the Ross procedure in paediatric and adult patients, with particular emphasis on survival, complication and reintervention rates. A retrospective review of 101 patients who had the Ross procedure in a congenital cardiac surgical centre serving a population of approximately 2.5 million was performed. There were 69 adults and 32 children with a mean age of 24.8 ± 13.9 years. Indications for surgery were aortic stenosis (48), regurgitation (10), mixed disease (35) and complex left outflow tract obstruction (8). The mean follow-up duration was 4.7 ± 3.7 years. The mini-inclusion technique was used to incorporate the autograft, and in all cases, pulmonary homografts were placed in the right ventricular outflow tract. Sub-aortic resection was also performed in six and Ross-Konno operations in eight patients. There were no early deaths and there was one late death secondary to endocarditis. Freedom from reintervention was 92% at 5 years and 77% at 10 years. Children were significantly more likely to require reintervention (16%, 5 of 32 versus 4%, 3 of 69, P = 0.05). The Ross procedure carries low early and mid-term mortality, and reintervention rates appear acceptable. The Ross procedure should be considered a feasible alternative to prosthetic valves in patients who require aortic valve replacement.

Hexagonal Six-point Traction Technique for Optimum Surgical Exposure of the Subaortic Region

Surgery of the subaortic region is challenging because of spatial limitations. We present our technique of enhanced exposure of this region that allows for a smooth, expeditious, and safe repair of such lesions as subaortic stenosis, hypertrophic cardiomyopathy, subaortic ventricular septal defects, and related pathologies. Using a hexagonal 6-point traction technique, approximately 84% of the cross-sectional area of the aortic root is made available to the surgeon for a transaortic subaortic resection keeping the use of retractors to a minimum. The technique ensures protection of the aortic valve leaflets and the enhanced exposure may contribute to improved surgical outcomes.

Left Ventricular Pseudoaneurysm in Children Following Subaortic Muscular Resection

Left ventricular pseudoaneurysm is an extremely rare cardiac complication, especially in early childhood. This potentially catastrophic complication may develop following cardiac operations for left ventricular outflow tract obstruction. Surgical repair is indicated when the complication is detected. This article presents 2 cases of left ventricular pseudoaneurysm development following subaortic muscular resection.

Impact of Aortic Annular Size on Rate of Reoperation for Left Ventricular Outflow Tract Obstruction After Repair of Interrupted Aortic Arch and Ventricular Septal Defect

The neonatal repair of interrupted aortic arch and ventricular septal defect (IAA/VSD) presents a surgical challenge. Although one-stage repair has become well established, left ventricular outflow tract obstruction (LVOTO) continues to be an important factor affecting survival and reintervention rates after IAA/VSD repair. We investigated the relationship between the preoperative aortic annulus and the rates of reoperation for LVOTO. Between July 1994 and July 2006, 38 patients with IAA/VSD have undergone complete single-stage repair. Patients with single ventricle physiology, transposition of the great arteries, or truncus arteriosus were excluded. Surgical technique involved the aortic arch reconstruction and VSD closure. Three patients underwent subaortic resection at the time of first operation and one patient underwent Yasui operation. The patients were divided into two groups according to whether the aortic annulus is greater than the patient's weight (kg) + 1.5 mm or less. The average follow-up was 7.9 +/- 4.2 years. Among the patients with small aortic annulus (n = 12), there was one hospital death and 6 reoperations for LVOTO, and one late death. There was only one reoperation for LVOTO among the patients with larger aortic annulus (n = 26, p < 0.001). The patients whose aortic annulus is less than patient's weight (kg) + 1.0 mm had poor outcomes if the LVOTO is not addressed at the time of the first operation. Neonatal single-stage repair for IAA/VSD achieves excellent survival. For the patients whose aortic annulus is greater than patient's weight (kg) + 1.5 mm, low reoperation rate for LVOTO is expected. For the patient whose aortic annulus is less than patient's weight + 1.5 mm, almost half of them needed reoperation. An LVOT bypass procedure (Yasui or Norwood) is recommended if the aortic annulus is less than the patient's weight + 1.0 mm.

Long-term Outcome and Risk of Heart Block After Surgical Treatment of Subaortic Stenosis

Although mortality following repair of subaortic obstruction is low, aggressive resection may increase morbidity. We sought to evaluate outcomes and risk of atrioventricular heart block (AVB) after subaortic resection in the current era. Simple obstruction was defined as a discrete subaortic membrane and complex as multilevel or diffuse narrowing. Limited resection included membranectomy and limited myomectomy. Aggressive resection included Konno, modified Konno, and Ross. Specified variables were obtained from a chart review. The 185 consecutive patients (1991-2008) ranged in age from 1 day to 21.8 years (5.1 ± 5.1 years) with 2 early and 4 late deaths. Actuarial survival was 97%, 95%, and 95% at 1, 5, and 10 years, respectively. Reoperations were required in 29 of 185 patients (15.7%); 2 required a third operation (1%). Freedom from reoperation in all patients was 97%, 83%, and 73% at 1, 5, and 10 years, respectively. Accessory mitral valve tissue (P < .001) and age <3 months (P = .004) predicted the need for reoperation. Transient or permanent high-degree AVB was documented in 33 of 185 patients (17.8%). Complex anatomy (P = .01) and aggressive resection (P < .001) increased the risk of acquiring AVB. The AVB was permanent in 21 of 185 (11.4%) patients, and pacemaker implantation was undertaken in 20 of 185 (10.8%) patients. Complex anatomy (P = .04) and modified Konno procedure (P = .03) increased the risk of acquiring a pacemaker. Aggressive resection lowered the frequency of recurrence but increased the risk of AVB. When aggressive resection is considered for long-term relief of subaortic obstruction, the risk of reobstruction must be balanced with the risk of AVB and the need for pacemaker implantation.

Long-term results of atrioventricular groove patch plasty — original method and its modification☆

Anatomically corrected malposition of great arteries (ACMGA) is associated with the leftward deviation of the proximal right coronary artery (RCA) away from the right atrioventricular (AV) groove. This anatomic feature allows a transannular subpulmonary patch plasty along the right AV groove between the RCA and the tricuspid annulus for relief of subpulmonary stenosis. This technique is also applicable to similar malformations that belong to the same spectrum. The long-term results of this original method and its modification were reviewed. This surgical technique with a monocuspid transannular patch for subpulmonary stenosis was applied in 13 patients (seven patients with ACMGA, four patients with transposition of the great arteries after arterial switch operation (Shaher type 4 or 9) who underwent the original Konno or subaortic resection simultaneously, and two patients with discordant AV connection who have undergone conventional left ventricle to pulmonary artery repair or ventricular septation and pacemaker implantation previously). The mean age at operation was 15 years. Postoperative catheterisation revealed adequate relief of pulmonary stenosis, with a pressure gradient of 5.6 ± 4.5 mmHg and with normalised right ventricular pressure (35 ± 12 mmHg). There was no late death or re-operation during a postoperative follow-up period of 90 ± 58 months. All patients were in normal sinus rhythm, postoperatively, except for 3 patients (one AV block due to subaortic resection, and two pacemaker rhythms implanted previously). The present report describes the excellent long-term results of the AV groove patch plasty for subpulmonary stenosis in ACMGA and similar malformations by using morphological advantages. This technique provides a promising alternative to the Rastelli-type conduit repair.

Management of Systemic Outlet Obstruction in Patients Undergoing Single Ventricle Palliation

Early or late systemic outlet obstruction in patients undergoing staged palliation for single ventricle variants may preclude a successful Fontan circulation. In assessing the patient presenting with real or impending obstruction, the surgeon is justified in pursuing an aggressive approach in protecting ventricular and semilunar valve function. Options include subaortic resection (transventricular, transatrial, or via a semilunar valve), modifications of the Damus-Kaye-Stansel connection, or a palliative arterial switch operation. In this review, the various options will be discussed and presented in the context of application to specific morphologic elements that make one more favorable than another in an individual patient.

Prevalence and Associated Risk Factors for Intervention in 313 Children With Subaortic Stenosis

We sought to determine the prevalence of intervention and associated factors in children presenting with subaortic stenosis. We also investigated whether a protocol adopted in 1994 of early subaortic resection at a preoperative mean systolic gradient across the left ventricular outflow tract (LV gradient) greater than 30 mm Hg was supported by longitudinal outcomes. Record review of all children (n = 313) diagnosed with subaortic stenosis was conducted between 1975 and 1998 at our institution. Cox proportional hazard models determined the prevalence and associated factors for initial subaortic resection. Mixed models of serially obtained echocardiographic data (n = 933) established longitudinal LV gradient trends and identified factors associated with more rapid LV gradient progression. Median age at presentation was 8 months. Freedom from initial subaortic resection was 40% at 16 years from diagnosis. Earlier progression to subaortic resection was associated with patient characteristics at presentation, including a higher initial LV gradient (p < 0.001), larger aortic annulus z-score (p = 0.005), smaller body surface area (p < 0.001), and smaller mitral annulus z-score (p = 0.003). Initial resection was also associated with a faster rate of LV gradient progression (p = 0.003). Factors determining the increased rate of LV gradient progression included an initial LV gradient greater than 30 mm Hg (p < 0.001), initial aortic valve thickening (p = 0.003), and attachment of subaortic stenosis to the mitral valve (p = 0.003). Worse aortic regurgitation grade with time was also associated with an initial LV gradient greater than 30 mm Hg (p < 0.001). Subaortic resection should be delayed until the LV gradient exceeds 30 mm Hg because most children with an initial LV gradient less than 30 mm Hg have quiescent disease.

Right ventricular outflow tract obstruction after arterial switch operation for the Taussig–Bing heart☆

Incidence of right ventricular outflow tract obstruction (RVOTO) may be suspected to be higher after arterial switch operation (ASO) for Taussig-Bing heart than after ASO for transposition of the great arteries (TGA), as Taussig-Bing anomaly is frequently associated with aortic arch obstruction and subvalvular aortic stenosis. We evaluated the risk to develop RVOTO after ASO for Taussig-Bing heart. The 34 Taussig-Bing cases who underwent ASO from 1984 to 2005 were reviewed. RVOTO was defined as peak echo-gradient >or=30 mmHg across right ventricular outflow tract. Kaplan-Meier method was used to estimate time-related events. Subaortic stenosis was resected in 25 patients, 20 of whom (80%: 20/25) were discharged from hospital free from RVOTO. There was one early death: 2.9% mortality. Three patients died late. Actuarial survival was 85.1%+/-7.0% from 54 month onwards. Eleven survivors (36.7%: 11/30) experienced postoperative RVOTO. Obstruction was seen in 82% (9/11) of cases at subvalvular and/or valvular level. Surgery (n=4) or percutaneous intervention (n=2) was required in six patients. Patients discharged from hospital with RVOTO (n=8) were more likely to undergo reintervention for RVOTO (p=0.026). Freedom from reintervention for RVOTO decreased rapidly in the first two years to 86.5+/-6.3%, slowly thereafter (80.4+/-8.4% at year 7) and stabilized at 70.3+/-11.9% from year 11 on. Risk for RVOTO occurrence was 23.5+/-7.3% early after repair and progressively increased to level out at 53.6+/-11% at year 11. Patients who underwent subaortic resection were more likely (p=0.023) to be free from RVOTO occurrence or development. In the period under review, for patients who underwent ASO for simple (n=355) and complex (n=92) TGA, reoperation rate for neopulmonary stenosis was 0.3% (1/355) and 5.4% (5/92), respectively, to be compared to 11.8% (4/34) RVOTO rate of reoperation for Taussig-Bing heart in this study. Postoperative right-sided obstruction occurs more frequently after ASO repair of Taussig-Bing heart than after TGA arterial switching, leading to higher reintervention rate. Resection of the commonly associated subaortic stenosis often prevents RVOTO development.

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification☆

The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO). We reviewed our experience with DORV patients and two ventricles that underwent repair, applying this nomenclature. Between January 2000 and January 2005, 50 patients with DORV and two viable ventricles underwent surgical intervention: 44 patients had biventricular repair, 3 had 1.5 ventricular repair, 2 underwent a Fontan, and 1 died prior to corrective surgery. Median age at repair was 9.1 months (range: 4D-4Y). Eighteen patients (36%) were DORV-Fallot (including 5 with AVSD and heterotaxy), 9 (18%) were DORV-TGA (Taussig-Bing), 12 (24%) were DORV-VSD, and 11 (22%) were DORV non-committed VSD. Corrective surgery included 35 repairs with a VSD-aorta baffle+/-RVOTO procedure and 12 arterial switches with a VSD-PA baffle (9 Taussig-Bing and 3 DORV-ncVSD). Associated procedures included 13 VSD enlargements, 8 subaortic resections, 9 arch repairs, 5 AVSD repairs, and 7 others. There were three deaths in the 50 patients studied (overall mortality of 6%). Excluding one patient that died prior to corrective surgery and the two patients palliated with a Fontan procedure, the actual surgical mortality for a corrective repair was 4.3% (2/47 patients). Two surgical deaths occurred following, respectively, one repair of a Taussig-Bing with an interrupted arch and a Swiss cheese VSD and one repair of ncVSD-type with pulmonary atresia that had undergone a previous cavo-pulmonary anastamosis. No late deaths occurred. Two late reoperations included a heart transplant in a DORV-Fallot patient with Swiss cheese VSD and subaortic resection in a DORV-ncVSD patient. Angioplasties were needed for PA stenosis (n=2) and aortic arch obstruction (n=2). Four patients had LV to aorta baffle gradients between 10 and 20 mmHg. All patients were of NYHA class I/II. The STS-EACTS International Nomenclature provides more uniform analysis of outcomes with respect to acceptable surgical risk and mortality. Biventricular repair can be safely achieved on selected DORV, including DORV-ncVSD and DORV with AVSD and heterotaxy lesions traditionally indicated for a single ventricle palliative approach.

Long-term results of surgical repair in patients with congenital subaortic stenosis

The aim of the study was to analyze the long-term results of congenital subvalvar aortic stenosis (SAS) relief and the risk factors associated with recurrence and reoperations. Between January 1960 and March 2005, 190 patients underwent surgical correction for discrete (n=140) and tunnel (n=50) congenital subaortic stenosis. There were 115 male and 75 female patients ranging in age from 1 week to 36 years (mean age, 8.2+/-4.4 years). Preoperatively, 133 patients were in NYHA functional class I or II, 57 in class III or IV. There were several initial surgical procedures performed in patients with congenital subaortic stenosis: fibrous or fibromuscular subaortic resection, apical aortic conduit insertion, aortic valve replacement with mechanical valve, Ross and/or Konno procedure. There were 7 early (4%) and 10 late (5%) deaths. Actuarial survival including operative mortality of patients with discrete and tunnel SAS was 94% and 84% at 40 years (P=0.14), respectively. Within 7.1+/-6.2 years a recurrent peak aortic gradient >50 mmHg and moderate to severe aortic insufficiency were found in 50 patients (28%), all of whom had 104 reoperations. At late follow-up, ranging from 6 months to 42 years (mean 9.6+/-7.5 years), the left ventricle-aorta gradient was higher in patients with tunnel versus discrete obstruction (28+/-11 mmHg vs. 13+/-9 mmHg; P=0.01) with a 40-year poor freedom from reoperation (14% vs. 89%; P<0.001). Patients with tunnel SAS and complex multilevel left ventricular outflow tract obstruction required higher reoperation rates. Aortic valve replacement with pulmonary autograft (Ross procedure) performed at our institution resulted in low mortality and morbidity.

Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow

The infant with a functional single ventricle (SV) and unobstructed pulmonary blood flow (UPBF) requires early protection of the pulmonary vascular bed to ensure suitability for a subsequent Fontan procedure. Systemic obstruction by aortic arch obstruction, subaortic stenosis, or combination of both, has been widely recognized as an important risk factor for poor outcome in children with SV-UPBF who are palliated with pulmonary artery banding (PAB). We reviewed our experience with primary PAB in the subset of patients with SV-UPBF to identify risk factors for subsequent palliative procedures and Fontan completion. Between January 1990 and May 2004, 80 patients (median age, 14 days) with functional SV and UPBF underwent PAB as their primary palliative procedure. Thirty-five neonates had concomitant aortic coarctation or interrupted aortic arch repair (44%). A Damus-Kaye-Stansel procedure was subsequently performed in 19 patients, and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in five. There were 4 operative deaths, and 15 late deaths. The actuarial overall survival is 84% at 1 year, 76% at 5 and 15 years. Follow-up is complete in all but six children at a mean interval of 4.9+/-3.7 years (range, 2 months-15 years). Thirty-seven patients (49%; 37 of 76) have undergone the hemi-Fontan procedure (with three hospital deaths) and 40 patients (53%; 40 of 76; 12 children without previous hemi-Fontan) have undergone the completion Fontan procedure without mortality or Fontan takedown. In infants with single ventricle physiology with or without systemic outflow obstruction and unobstructed pulmonary blood flow, a strategy of pulmonary artery banding carries acceptable operative and mid-term mortality in a high-risk group of patients. Pulmonary artery banding does not compromise performance of subsequent Damus-Kaye-Stansel procedure or completion Fontan palliation.