Systemic Ventricular Outflow Tract Obstruction Research Articles

Combined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries.

The combination of congenitally corrected transposition of the great arteries (ccTGA), Ebstein anomaly of the systemic atrioventricular valve, and critical systemic ventricular outflow tract obstruction is extremely rare. Management of a neonate with these lesions in cardiogenic shock is quite challenging. Tricuspid valve exclusion has been increasingly and successfully used to manage neonatal Ebstein anomaly patients. We extended this idea of tricuspid valve exclusion (modified Starnes) combined with a Norwood Stage 1 operation with a modified systemic to pulmonary artery shunt to successfully manage a neonate with ccTGA, severe systemic atrioventricular valve regurgitation, and critical ventricular outflow tract obstruction.

Improved Long-term Outcome of Damus-Kaye-Stansel Procedure Without Previous Pulmonary Artery Banding

BackgroundThis study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. MethodsThe study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. ResultsThe DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P < .001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P < .001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P < .001). ConclusionsA primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function.

Early- and midterm outcomes of pulmonary artery band as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children's Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(Q(R))), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model. Results: There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(Q(R))) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death. Conclusions: The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.

Effect of multistage surgery in patients with functional single ventricle and risk factors of postoperative death

Objective: To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death. Methods: The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed. The study included 289 patients. The age was 10.0 (6.0, 35.4) months,and there were 198 male and 91 female patients. The patients were followed up at outpatient clinic. Survival rates were calculated with Kaplan-Meier. Multivariate Cox regression analysis was made to determine the risk factors of postoperative death. Results: Seventy-nine patients required the first stage palliation surgery, 232 patients received the Glenn shunt surgery, and 162 patients completed the Fontan procedure. Overall, postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases, late stage 8 cases) , 10 after the Glenn shunt surgery (early stage 5 cases, late stage 5 cases) , and 8 following the Fontan procedure (early stage 6 cases, late stage 2 cases) . Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%) , 85.9% (95%CI 81.8%-90.0%) ,and 84.6% (95%CI 79.7%-89.5%) at 1 year, 5 years and 10 years post operation. Survival rate was 74.4% (95%CI 64.8%-84.0%) , 73.0% (95%CI 63.2%-82.8%) , and 73.0% (95%CI 63.2%-82.8%) at 1 year, 5 years, and 10 years post the first stage palliation surgery, 97.8% (95%CI 95.8%-99.7%) , 95.2% (95%CI 92.3%-98.1%) , and 95.2% (95%CI 92.3%-98.1%) at 1 year, 5 years, and 10 years post Glenn shunt surgery, 95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47, 95%CI 2.71-11.04, P<0.001), atrioventricular valve regurgitation more than moderate (HR=2.52, 95%CI 1.32-4.79, P=0.005), systemic ventricular outflow tract obstruction (HR=3.47, 95%CI 1.30-9.29, P=0.013), and required the first palliation surgery (HR=3.12, 95%CI 1.59-6.15, P=0.001) were risk factors of postoperative death. Conclusions: The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis. Total anomalous pulmonary venous connection, atrioventricular valve regurgitation more than moderate, systemic ventricular outflow tract obstruction, and required the first palliation surgery are risk factors of postoperative death in these patients.

The clinical outcomes of damus-kaye-stansel procedure according to surgical technique.

BackgroundThe Damus-Kaye-Stansel (DKS) procedure is a method for mitigating the risk of systemic ventricular outflow tract obstruction (SVOTO). However, there have been few reports on which surgical technique shows a better outcome. The objective of this study was to compare the outcome of the DKS procedure according to the surgical technique used.MethodsWe retrospectively reviewed 12 consecutive patients who underwent the DKS procedure from March 2004 to April 2013. When the relationship of the great arteries was anterior-posterior, the double-barrel technique (group A) was performed. If the relationship was side-by-side, the ascending aortic flap technique (group B) was performed.ResultsThere was no early mortality and 1 late mortality in group B. There was no statistically significant difference in the median peak pressure gradient of preoperative subaortic stenosis in both groups: 14 mmHg (range, 4 to 53 mmHg) in group A and 15 mmHg (range, 0 to 30 mmHg) in group B (p=0.526). Further, a significant postoperative pressure gradient was not observed in either group A or group B. More than moderate postoperative neoaortic regurgitation was observed in 1 patient of group B; this patient underwent neoaortic valve replacement 66 months after the DKS procedure. No one had a recurrent SVOTO during follow-up.ConclusionThe DKS procedure is an effective way to minimize the risk of SVOTO, and there is little difference in the outcomes of the DKS procedure according to the surgical technique used.

Anatomic Repair for Corrected Transposition With Left Ventricular Outflow Tract Obstruction

We investigated the long-term outcomes of anatomic repair for congenitally corrected transposition of great arteries (ccTGA) and its variant associated with left ventricular outflow tract obstruction (LVOTO) and ventricular septal defect (VSD). From 1987 to 2011, 47 patients (27 with pulmonary atresia and 20 with pulmonary stenosis) with ccTGA and its variant associated with LVOTO and VSD underwent anatomic repair. The mean operative age was 5.5 ± 3.7 years old (range, 1.6 to 21.3). The preoperative right ventricular end-diastolic volume was 133% ± 31% (81 to 222) of their normal size. The atrial switch procedure was Mustard in 31 patients and Senning in 16, with the latter used in all from 2002. The mean follow-up period was 11.6 ± 7.3 years (maximum, 22.7). The VSD was concomitantly enlarged in 4 patients and Damus-Kaye-Stansel (DKS) anastomosis was added in 9 patients with pulmonary stenosis and restrictive VSD. The overall survival rate at 20 years was 70.2% and no mortality has been observed in 21 consecutive patients since 1997. No patient required reoperation for the postoperative systemic ventricular outflow tract obstruction. A surgical heart block developed in 1 patient (2.1%) who underwent concomitant VSD enlargement. None of the patients developed a moderate or greater aortic or neo-aortic regurgitation. Recent outcomes after anatomic repair for congenitally corrected transposition of great arteries associated with LVOTO and VSD were excellent. For patients with pulmonary stenosis and restrictive VSD, additional DKS anastomosis seems to be an effective approach to avoid postoperative systemic ventricular outflow tract obstruction and surgical heart block.

Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction

Children with various single ventricle anomalies are at risk of developing systemic ventricular outflow tract obstruction (SVOTO) following volume unloading with cavopulmonary connection (CPC). We aim to evaluate the value of Damus-Kaye-Stansel (DKS) anastomosis at the time of CPC in eliminating late SVOTO risk. Retrospective review of single ventricle patients who underwent DKS concurrent with CPC between 1997 and 2012 was performed. Clinical, echocardiographic and angiographic outcomes were analysed. Thirty-six children with single ventricle underwent DKS at the time of Glenn bidirectional CPC (n = 29) or Fontan total CPC (n = 7). The underlying anatomy was double inlet left ventricle (n = 18), double outlet right ventricle (n = 8), unbalanced atrioventricular septal defect (n = 4) and other (n = 6). Prior palliation included pulmonary artery band (n = 35), coarctation/arch repair (n = 11) and atrial septectomy (n = 8). Median age at the time of DKS was 8.9 months (range 3.6 months-9.1 years) and the median weight was 6.7 kg (range 5-27 kg). At the time of DKS, 17 patients (47%) had no SVOT gradient and 19 (53%) had SVOT gradient (mean 23.4 ± 18.7 mmHg). Overall survival was 89 and 83% at 1 month and 5 years, respectively. None of the deaths were related to SVOTO or DKS complications. When present, SVOT gradient decreased from 23.4 ± 18.7 mmHg preoperatively to 0 after DKS (P < 0.001). At the last follow-up, none of the patients developed any SVOT gradient; 78% of them had zero or trivial aortic/neoaortic valve regurgitation while 22% had mild regurgitation. None of the patients had evidence of compression of the left pulmonary artery or bronchus. Eighty-one percent of patients have reached or are suitable candidates awaiting final palliative surgery. DKS can be safely performed in conjunction with CPC without added mortality risk. It is very effective in mitigating SVOTO risk, with sustainable good semilunar valves function. Our data support an aggressive approach to performing DKS concurrent with CPC in children with single ventricle pathologies at risk of developing SVOTO.

Intensive Care and Perioperative Management of Patients With Complete Atrioventricular Septal Defect

Operative repair of an atrioventricular septal defect is not without risk. The purpose of this review is to highlight various key topics in the perioperative treatment of patients with atrioventricular septal defects, including challenges related to Down syndrome, postoperative arrhythmias, pulmonary hypertension, hypothyroidism, postoperative residual and recurrent lesions, including systemic atrioventricular valvar regurgitation and left ventricular outflow tract obstruction, sedation and analgesia, and vascular access.

Cardiac Magnetic Resonance Imaging and Multidetector Computed Tomography Scan Illustrating Damus–Kaye–Stansel Operation

The Damus–Kaye–Stansel (DKS) operation was first described in 1975 independently by 3 authors, Damus,1 Kaye,2 and Stansel,3 and was soon felt to be an effective method to relieve systemic ventricular outflow–tract obstruction in patients with ventriculoarterial discordance. Since then, the procedure has been applied to patients with several forms of complex congenital heart disease in which systemic outflow is obstructed. The operation consists of an anastomosis of the proximal end of the transected pulmonary artery to the side of the ascending aorta, ensuring unobstructed outflow to the systemic circulation through the pulmonary valve. The pulmonary circulation can be then reestablished by a right ventricle to the distal main pulmonary artery conduit in cases of biventricular repair, or by a Fontan procedure (systemic venous return to the pulmonary artery without the subpulmonary ventricle) in cases in which only a univentricular repair can be offered. We present 2 cases that illustrate the utility of cardiac magnetic resonance imaging and multidetector computed …

Modified Damus–Kaye–Stansel procedure using aortic flap technique for systemic ventricular outflow tract obstruction in functionally univentricular heart

Damus-Kaye-Stansel procedure is a useful method to relieve the systemic ventricular outflow tract obstruction in functionally univentricular heart. Regurgitation of pulmonary valve and recurrence of systemic ventricular outflow obstruction are the major concerns at the late phase of this procedure. Modification of original Damus-Kaye-Stansel procedure that can prevent the use of prosthetic materials is evaluated. The modified Damus-Kaye-Stansel procedure using aortic flap technique was performed in eight patients with functionally univentricular heart. Patients' ages ranged from 3 to 28 months (mean 14 months). Follow-up period was 37 months as a mean (9-71 months), and the follow-up was complete. There was no operative mortality and no late death. In addition, there was no recurrence of systemic ventricular outflow tract obstruction throughout the follow-up period. Regurgitation of the pulmonary valve estimated by echocardiography at the latest follow-up was none to trivial in seven patients and mild in one. The modified Damus-Kaye-Stansel procedure using aortic flap technique is a safe, useful and reproducible technique to solve systemic ventricular outflow tract obstruction in functionally univentricular heart, and it can be an alternative for original technique or the so-called double-barrel modification.

Long-term follow-up of surgical patients with single-ventricle physiology: prognostic anatomical determinants

Although univentricular or biventricular repair is not always possible for hearts with single-ventricle physiology, it has been proven to produce a satisfactory outcome. It is hypothesized that patient prognosis can be predicted by anatomical diagnosis at presentation. Between 1961 and 1995, 158 patients with single-ventricle physiology, including tricuspid atresia and hypoplastic left heart syndrome, were referred to the authors' institute, and underwent 260 surgical interventions. Follow-up was 99% complete. Patient survival and anatomical risk factors were examined by the Kaplan–Meier method, and multivariate analysis including the Cox proportional hazard model. Mean (s.e.m.) actuarial survival rates at 1, 5, 10 and 20 years following birth were 70.3(3.6)%, 56.3(4.0)%, 48.8(4.2)%, and 40.9(4.7)%, respectively. Definitive palliation was attempted in 38 patients (univentricular in 35 and biventricular in three). Multivariate analysis identified systemic ventricular outflow tract obstruction, mitral atresia, situs ambiguus, and pulmonary vein drainage tract obstruction as independent prognostic factors for overall survival. Visceral heterotaxy was the only independent risk factor for lack of application or failure (death or take-down within 30 days of operation) of univentricular or biventricular repair. In conclusions, these anatomical factors in hearts with single-ventricle physiology affect long-term mortality, despite multiple and heterogeneous surgical efforts.

Long-Term Follow-Up of Surgical Patients with Single-Ventricle Physiology: Prognostic Anatomical Determinants

Although univentricular or biventricular repair is not always possible for hearts with single-ventricle physiology, it has been proven to produce a satisfactory outcome. It is hypothesized that patient prognosis can be predicted by anatomical diagnosis at presentation. Between 1961 and 1995, 158 patients with single-ventricle physiology, including tricuspid atresia and hypoplastic left heart syndrome, were referred to the authors' institute, and underwent 260 surgical interventions. Follow-up was 99% complete. Patient survival and anatomical risk factors were examined by the Kaplan-Meier method, and multivariate analysis including the Cox proportional hazard model. Mean (s.e.m.) actuarial survival rates at 1, 5, 10 and 20 years following birth were 70.3(3.6)%, 56.3(4.0)%, 48.8(4.2)%, and 40.9(4.7)%, respectively. Definitive palliation was attempted in 38 patients (univentricular in 35 and biventricular in three). Multivariate analysis identified systemic ventricular outflow tract obstruction, mitral atresia, situs ambiguus, and pulmonary vein drainage tract obstruction as independent prognostic factors for overall survival. Visceral heterotaxy was the only independent risk factor for lack of application or failure (death or take-down within 30 days of operation) of univentricular or biventricular repair. In conclusions, these anatomical factors in hearts with single-ventricle physiology affect long-term mortality, despite multiple and heterogeneous surgical efforts.

Repair of double-outlet right ventricle. Experience of 13 cases.

Surgery for double-outlet right ventricle (DORV) was performed in 13 patients between November 1974 and January 1979. Subaortic ventricular septal defect (VSD) was present in 11 patients, complicated forms of DORV in 2 patients and 5 patients had important concomitant cardiac defects. Six infants (mean age 0.6 years) without pulmonary stenosis (PS) required operation because of pulmonary hypertension, whereas the 7 patients with PS underwent surgery at a considerably later stage (mean age 6 years). Interventricular tunnel-repair established continuity between the systemic ventricle and great artery in 12 patients. One case with subpulmonic VSD was managed by transposition of both venous return and arterial outflow, while the use of valved external conduits was generally avoided. Atrial incision was sufficient to permit complete intraventricular repair in 8 patients, including one pulmonary valvulotomy. Important co-existing PS was otherwise treated as in cases of tetralogy of Fallot and required transannular patch grafting in 2 instances. Hospital mortality was 3/13 patients (23%) and mainly confined to serious associated cardiac malformations which were not amenable to correction. All 10 survivors are functionally improved 1.5-5 years after surgery. Clinical and invasive re-evaluation (3 patients) could not identify the development of systemic ventricular outflow tract obstruction. One patient, who underwent enlargement of a restrictive VSD, presented angiographic evidence of a moderate aortic incompetence. No other important complications were associated with the tunnel-repair and none of the 10 survivors had complete heart block.