Relief Of Subaortic Stenosis Research Articles

Left heart growth and biventricular repair after hybrid palliation.

We evaluated the outcomes of biventricular repair after initial hybrid palliation performed in small infants with various forms of left ventricle hypoplasia. Between September 2010 and January 2020, a total of 27 patients had biventricular repair after hybrid palliation at a median age of 11 days. Indications for the hybrid approach included growth promotion of the left ventricle outflow tract and/or the aortic valve in 14 patients and that of the left ventricle in 13 patients. Seven reinterventions and 7 reoperations were performed during the interstage period. Significant growth of left ventricle parameters was noted during the median interstage period of 62 days. Sixteen subjects had aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients had the Ross-Konno procedure; 5 patients underwent the Yasui procedure; and 1 patient had unbalanced atrioventricular septal defect and aortic arch repair. Twenty-three patients (85.2%) are alive at a median follow-up of 3.3 years. Two and 3 patients died early and late after achieving biventricular circulation, respectively. There were 22 reinterventions and 15 reoperations after biventricular repair. Hybrid palliation can stimulate left heart growth in some patients with left ventricle hypoplasia. More patients may eventually achieve biventricular circulation than was initially thought. Additional interventions and operations are foreseeable. Despite ventricular rehabilitation, some patients with borderline left ventricles may develop restrictive physiology.

Biventricular repair after the hybrid Norwood procedure.

We analysed the outcomes of patients undergoing biventricular repair (BVR) after an initial hybrid Norwood approach as a salvage procedure in extremely sick infants; or as the initial palliation in patients with uncertain feasibility of single-stage BVR due to severe left ventricular outflow tract obstruction; or as part of a left ventricle (LV) recruitment strategy in patients with borderline LVs. Between September 2010 and July 2018, 26 patients underwent BVR after initial hybrid palliation at a median age of 13 days. The rationale for the hybrid approach was to promote the growth of the LV in 10 patients and that of the left ventricular outflow tract and/or aortic valve in 12 patients and to be a salvage procedure in 4 patients. Significant growth of the LV was noted during the interstage period, which had a median length of 65 days (P = 0.008). Fourteen patients underwent aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients underwent the Yasui procedure; 4 patients had the Ross-Konno procedure; 2 patients had an arterial switch operation; and 1 patient had truncus arteriosus repair. Twenty-two patients (84.6%) are alive at a median follow-up period of 1.8 (range 0.04-6.2) years. There were 2 early and 2 late deaths. Nineteen catheter-based reinterventions and 15 reoperations were performed after BVR. The hybrid Norwood procedure permits stabilization of critical infants. It allows for growth of left ventricular structures in some patients with borderline left hearts and in those with severe left ventricular outflow tract obstruction. More patients may eventually have BVR than was thought during the newborn period.

Surgical repair of interrupted aortic arch with ventricular septal defect

AbstractBackgroundInterrupted aortic arch is often associated with subaortic narrowing and hypoplasia of the aortic orifice. The best surgical strategy for the management of these additional lesions is a matter of current debate.Methods and ResultsBetween 1986 and 1996, 19 patients underwent repair of interrupted aortic arch with closure of ventricular septal defect in a single stage, with no attempt at subaortic resection, irrespective of the dimensions of the left ventricular outflow tract. There was no perioperative hospital mortality, and all patients were alive at 1 year. Follow-up ranges from 0.75 −10 years, with a mean 4.2 ± 3.0 years. Seven patients (37%) have required reintervention for relief of subaortic stenosis, 2 of whom have died.ConclusionsOur results suggest that primary one-stage biventricular repair can be accomplished with low perioperative mortality without addressing the subaortic region. Further long-term follow-up will determine whether this is accomplished at the expense of later morbidity and mortality.

Pulmonary artery banding is not contraindicated in double inlet left ventricle with transposition and aortic arch obstruction.

It has been widely stated that pulmonary artery banding (PAB) is contraindicated in the setting of double inlet left ventricle with transposition of the great vessels (DILV/TGA), especially if aortic arch obstruction is present. We postulated that the poor results for this condition reflect the tendency to leave the band in place long-term without early recognition and relief of subaortic stenosis (SAS). Short-term PAB with early relief of SAS remains an attractive option compared to a neonatal "Norwood" strategy. We reviewed our results applying this approach to 18 consecutive infants presenting since 1980 with DILV/TGA and an obstructive anomaly of the aortic arch (coarctation 16, interruption or atresia 2). Four of the infants (22%) were considered to have important SAS at presentation. One underwent neonatal aortopulmonary connection and died. The remaining 17 patients underwent arch repair with PAB (median age 1.4 weeks; range 2 days-22 weeks) with one early death. The 16 survivors have been followed for 5.6 +/- 3.7 years. All but one ultimately developed SAS. Relief of SAS was performed in 15 patients (median age 8 months) using a proximal aortopulmonary anastomosis. There were two early deaths, and one late death. Thirteen of the 18 patients (72%) are alive and well, and 12 have achieved Fontan repair or bidirectional superior cavopulmonary anastomosis (BCPA) with persistent relief of SAS. Most patients with DILV/TGA and aortic arch obstruction will tolerate temporary PAB with adequate protection of the pulmonary vascular bed. Our current approach (in the absence of severe SAS at presentation) is PAB at the time of arch repair, followed by careful surveillance for, and early relief of, SAS combined with BCPA in infancy.

Operations for subaortic stenosis in univentricular hearts

Optimal prevention and treatment of subaortic stenosis (SAS) in the univentricular heart with subaortic outlet chamber and high pulmonary blood flow remains controversial, especially when complicated by aortic arch obstruction. Herein we analyze our surgical results. Group 1 consisted of 11 infants (mean age, 10 days) with univentricular heart and SAS. Ten required repair of interrupted aortic arch (n = 7) or coarctation with hypoplastic arch (n = 7). Four patients had relief of SAS by either Damus-Kaye-Stansel connection (n = 2) or aortopulmonary window (n = 2), with three operative deaths and one late death. Six had one-stage arterial switch and atrial septectomy with arch repair ( 5 / 6 ) with one operative death and one late death. Two survivors have progressed to bidirectional cavopulmonary shunt, a third has had a Fontan operation, and a fourth awaits Fontan. In group 2, 11 children required operation for acquired SAS after pulmonary artery banding. Nine have progressed to Fontan operation with either staged (n = 3) or concurrent (n = 6) relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Fontan mortality was 11% (70% confidence limits, 2% to 32%). Group 3 consisted of 3 patients without pulmonary artery banding who had SAS diagnosed at Fontan evaluation. All 3 survived Fontan operation and relief of SAS by Damus-Kaye-Stansel connection or subaortic resection. Group 4 consisted of 1 patient with previous pulmonary artery banding (no SAS) who underwent Fontan operation but required Damus-Kaye-Stansel connection 30 months later for SAS. We conclude that arterial switch, arch repair, and atrial septectomy effectively palliate newborns with univentricular heart and SAS, although ultimate Fontan suitability is yet to be established. Both Damus-Kaye-Stansel connection and subaortic resection provide good long-term relief of SAS in select patients, and previous or concurrent treatment of SAS does not preclude successful Fontan operation in otherwise suitable patients.