Resection Of Subaortic Stenosis Research Articles

Long-term outcomes and risk factors for reoperation after subaortic stenosis resection in children and adults

Abstract Background Sub-valvular aortic stenosis (SAS) is an extremely heterogeneous form of left ventricular outflow tract (LVOT) obstruction, which can lead to aortic valve damage. Although corrective surgery is considered the most effective treatment, rates of recurrence and reoperation are still high. Previous studies have focused on predictors of reoperation in several subgroups of patients with SAS, but rarely in the whole population of patients with SAS. Purpose We sought to describe long-term outcomes after SAS resection and to determine the predictors of reoperation after successful primary resection. Methods We retrospectively reviewed the records of patients who underwent SAS resection between 1994 and 2021 at our institution with at least 12 months’ follow-up. Patients with a single-ventricle physiology and idiopathic hypertrophic cardiomyopathy were excluded. Demographic, surgical and echocardiographic data were analyzed. Outcomes were reoperation after successful primary resection, death and morbidities, including postoperative complete heart block and aortic regurgitation (AR) grade at latest follow-up. Results Seventy-six patients (paediatric, n = 72 and adult, n = 4) who underwent SAS resection were included in the analysis. Median length of postoperative follow-up was 11.1 years (IQR range: 7.3 – 15.7 years), with two late deaths. Reoperation due to recurrent obstruction or severe AR occurred in 24 patients (31.6%). Median time to reoperation was 7.5 years (IQR range: 3.6 – 11 years). Overall freedom from reoperation at 5 years and 10 years was 84.6% and 69.1% respectively. Gender and association with other cardiac defects did not differ significantly between those with reoperation and those without. In multivariate analysis, independent predictors of reoperation were age at first surgery younger than 5 years (HR 3.54, 95% CI 1.06 – 11.83; P = 0.040) and tunnel-type lesions (HR 4.42, 95% CI 1.70 – 11.51; P = 0.002). Three patients (3.9%) had complete heart block and required placement of an implantable pacemaker. Following SAS resection, progression of AR was common (39/68, 57.3%) but only 5 patients (7.3%) developed sufficient regurgitation to require surgical repair or replacement. Conclusions Reoperation is not infrequent after surgical relief of SAS and reaches a plateau after 10 years. Younger age at first surgery and tunnel-type SAS independently predict reoperation. Progression of AR after corrective surgery of SAS is common, but fortunately only a minority of patients develop hemodynamically significant AR requiring reintervention.

Surgical repair of subaortic stenosis resection: 10 years of single-center experience in 65 patients.

Subaortic stenosis (SAS) was a rare congenital heart disease of left ventricular outflow tract (LVOT), ranging from "isolated" lesions to "tunnel" or "diffuse" lesions. We conducted a retrospective study to describe the characteristics of patients with different lesions and analyze the risk factors for reoperation. In this study, we examined a single-center retrospective cohort of SAS patients undergoing resection from 2010 to 2019. Patients were classified as simple lesion group (n = 37) or complex lesion group (n = 28). Demographics, perioperative findings, and clinical data were analyzed. The surgical effect of the two groups was significantly lower than that before the operation (p < .05). The median age at operation was 6 (3-11.8) years. There was no operative mortality. In complex lesion group, cardiopulmonary bypass time (CPB time), aortic cross-clamping time (ACC time), mechanical ventilation time, and intensive care unit (ICU) stay time were longer. The median follow-up period was 2.8 years (range: 1-3.8), with two late death. Six patients (9.2%) required reoperation due to restenosis or severe aortic insufficiency. The freedom from reoperation rates at 5 years was 66.7% for simple lesion but only 52.3% for complex lesion (p = .036). Although the lesions include many forms, SAS resection was still satisfactory. However, the reoperation after initial surgical treatment was not infrequent, especially in patients with complex lesion.

Mechanical Mitral Valve Replacement: A Multicenter Study of Outcomes With Use of 15- to 17-mm Prostheses

The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses. A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated. Mitral valve replacement was performed in 61 infants (15 mm, n= 17 [28%]; 16 mm, n= 18 [29%]; 17 mm, n= 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n= 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n= 9 [15%]), subaortic stenosis resection (n= 4 [7%]), aortic valve repair (n= 3 [5%], and aortic valve replacement (n= 6 [10%]). Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk.

Mitral Valve Replacement With the 15-mm Mechanical Valve: A 20-Year Multicenter Experience

The aim of this study was to evaluate early and long-term outcomes (mortality and prosthetic valve replacement) after mitral valve replacement with the 15-mm St Jude Medical prosthesis (St Jude Medical, St Paul, MN). A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15-mm St Jude Medical Masters prosthesis at 4 congenital cardiac centers in The Netherlands. Operative results were evaluated and echocardiographic data studied at 0.5, 1, 2, 3, 5, and 10 years after surgery. Surgery was performed in 17 infants. Ten patients (59%) were treated in the intensive care unit before surgery; 8 (47%) were on ventilator support. Median age at surgery was 3.2 months (interquartile range [IQR], 1.2-5.6), and median weight was 5.2 kg (IQR 3.9-5.7). There was 1 early cardiac death and 1 late noncardiac death. Median follow-up time was 9.6 years (IQR, 2.4-13.2), including 8 patients with a follow-up more than 10 years. The first prosthetic valve explantation (n= 11) occurred at a median of 2.9 years (IQR, 2.0-5.4). Other reinterventions were permanent pacemaker implantation (n= 3), subaortic stenosis resection (n= 2), and paravalvular leak repair (n= 1). Prosthetic valve gradients increased from a mean of 5.0 mm Hg (at discharge) to a mean of 14.3 mm Hg (at 5-year follow-up). Mitral valve replacement with the 15-mm prosthesis can safely be performed in infants and even in neonates. Median freedom from prosthesis replacement for outgrowth is 3.5 years. Thromboembolic complications were rare.

Long-term follow-up and outcomes of discrete subaortic stenosis resection in children.

Background:Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution.Materials and Methods:We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency.Results:The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively (P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67–16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1–9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection.Conclusions:Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

Subaortic Stenosis Resection in Children: Emphasis on Recurrence and the Fate of the Aortic Valve.

Recurrence after surgical resection of discrete subvalvar aortic stenosis in children often requires repeat operation. Risk factors for recurrence are poorly understood. We sought to determine potential risk factors for recurrence and postoperative comorbidities in the long term. Retrospective chart review was performed on all pediatric patients who underwent surgical resection of discrete subaortic stenosis at our institution. Demographics, perioperative findings, and clinical data were analyzed for predisposing factors. From 1991 to 2015, a total of 104 patients underwent primary surgical resection of discrete subaortic stenosis. There were no postoperative deaths. Three (2.9%) patients required pacemaker implantation. Nine (8.4%) patients required repeat resection for recurrence of subaortic membrane over a median follow-up of 8.5 years (interquartile range: 5.9-13.5 years). Actuarial freedom from repeat resection was 100%, 94%, and 82% at one, five, and ten years, respectively. Repeat resection occurred more frequently in patients with genetic disease (37.5% vs 10.7%; P = .033) and preoperative mitral regurgitation (MR; 25% vs 1.2%; P < .001). Postoperative aortic insufficiency (AI) that was moderate or worse was associated with older age at the time of first resection (relative risk [RR]: 1.54, P < .05), moderate or severe preoperative AI (RR: 1.84, P = .002), and repeat resection of subaortic stenosis (RR: 1.90, P < .001). The majority of children who undergo surgical resection of subaortic stenosis will not experience recurrence in childhood and those who do require repeat resection may have a higher incidence of genetic disease and preoperative MR. Postoperative AI is associated with repeat resection, older age at the time of surgery, and degree of preoperative AI.

Intraoperative transesophageal echocardiographic predictors of recurrent left ventricular outflow tract obstruction in children undergoing subaortic stenosis resection.

Intraoperative transesophageal echocardiography (iTEE) is used to assess for residual left ventricular outflow tract obstruction (LVOTO) after surgical resection of subaortic membrane causing subaortic stenosis (sub-AS). We aimed to identify the iTEE features associated with recurrence of LVOTO. We conducted a retrospective study of children undergoing sub-AS resection from June 2006 to June 2014. Doppler assessment of the flow velocity and the anatomical features of the left ventricular outflow tract were analyzed from stored echocardiograms. Recurrent LVOTO was defined as an increase in the mean pressure gradient across the left ventricular outflow tract of > 15mmHg on the most recent follow-up echocardiogram from the mean pressure gradient on the predischarge echocardiogram or as doubling of the mean pressure gradient to a value ≥20mmHg. Thirty-five patients were included, with median age at surgery was 8.1 years (range: 0.7-29 years) and median follow-up was 47 months (2-91 months). Ten patients (29%) had recurrent LVOTO, which was associated with a shorter distance between the narrowest diameter of the outflow tract and the aortic valve on iTEE [median 0.59 cm (range 0.39-0.74) vs 0.98 cm (0.75-1.5), P=.03]. No patients with more than mild residual LVOTO on iTEE regressed to mild or no LVOTO on follow-up echocardiograms. LVOTO recurrence after sub-AS resection is common, and residual LVOTO remains the same or increases over time. Proximity of the LVOTO to the aortic valve is a risk factor for recurrent LVOTO. These findings may be useful in counseling patients and to guide the frequency of postoperative follow-up.

Useful surgical instruments for the resection of subaortic stenosis

Furthermore, it is difficult for individual cardiovascularsurgeons to gain sufficient experience outside high-volume centers because of the low incidence of surgicalcases. To perform this difficult surgical technique moreeasily,weusemade-to-ordersurgicalinstrumentsforresec-tionofthemembraneormuscleinpatientswithSAS.Thesesurgical instruments are custom-made for the depth andtargetangleinthetransaorticapproach.Wereporttheiruse-fulness in a sample surgical case of SAS.TECHNIQUE AND RESULTSInstrumentsHere we describe the special features of surgical instru-ments for resection of the subaortic region (Figure 1). Thescalpel has a total length of 22 cm and an angle of 140 at3 cm from the tip. It is used by attaching an edge to thetip. The scissors has a total length of 25 cm and an angleof160 rightinthemiddle.Theretractorshaveatotallengthof 30 cm and an angle of 120 at 5 cm from the tip with agroove.CaseAn 11-year-old girl had a diagnosis of discrete SAS withconstrictivepericarditis.Althoughshecouldnotreportsub-jective symptoms because of severe mental disability, weelectedtoperformpericardiotomywithresectionofthesub-aorticmembraneinlightofthehighpeakinstantaneous leftventricular outflow tract (LVOT) gradient of 80 mm Hg.Theoperationwasperformedthroughamediansternotomy.Afterpericardiotomy,thepatientwascooledto32 Cforce-rebral protection under cardiopulmonary bypass andsubsequentcardiacarrest.Afteraortotomy,thesubaorticre-gion was excellently exposed with angled retractors with agroove (Figure 2, A). The obstructive fibrous membrane,which appeared on the LVOT (Figure 2, B), and a part ofthe septal muscle were resected with a scalpel and scissorsangled to enter the septal muscle at a shallow angle(Figure 2, C and D). We confirmed passage of the diameterdilator(15mm).Afterweconfirmedthattherewasnoaorticregurgitation and no injury of the aortic valve, the incisionof the aorta was closed. The postoperative peak instanta-neous LVOT gradient was decreased, and this patient wasdischarged from the hospital on postoperative day 11.DISCUSSIONAlthoughearlysurgicalrepairofSASisassociatedwithasignificant recurrence risk and aortic regurgitation progres-sion,surgicalinterventionisrequiredinpatientswithahighLVOTgradient.

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.

Ascending-to-Descending Aortic Bypass: A Simple Solution to a Complex Problem

Surgical correction of complex aortic coarctation can be associated with significant risks. Extraanatomic bypass may represent a safer alternative. Between January 1985 and December 2012, 80 consecutive patients with complex coarctation underwent ascending-to-descending aortic bypass through a median sternotomy. Patients were a median age of 42 years (range, 15 to 75 years), and 51 (64%) were males. Recurrent coarctation was present in 52 patients (65%), with 6 (8%) having undergone balloon dilatation. Uncontrolled hypertension was present in 63 patients (79%). The most common concomitant pathology was aortic valve stenosis in 21 patients (26%), subaortic stenosis in 10 (13%), and Shone complex in 4 (5%). There were no early deaths. The most common concomitant procedures were aortic valve replacement, coronary artery bypass grafting, and resection of subaortic stenosis. The mean aortic cross-clamp and cardiopulmonary bypass times were 33 ± 40 and 106 ± 54 minutes, respectively. Morbidity included atrial fibrillation in 17 patients (21%) and reexploration for bleeding in 6 (8%). There was no paraplegia or stroke. Upper extremity blood pressure significantly improved (p < 0.001). Mean systolic blood pressure decreased from 153 ± 26 mm Hg preoperatively to 123 ± 15 mm Hg postoperatively. Mean follow-up was 7 ± 6 years (maximum, 22 years). Late deaths occurred in 5 patients (6%) and were not graft-related. Three patients (4%) required reoperation for repair of periprosthetic regurgitation in 2 and mitral valve replacement in1. The ascending-to-descending aortic bypass can be performed with low morbidity and mortality. It is an effective solution to complex aortic coarctation and represents a safe single-stage approach for patients with concomitant cardiac pathology.

Subaortic Stenosis Surgical Correction After Previous Substernum Coloesophagoplasty

Alternate approaches for aortic valve procedures are rare but occasionally used in cardiac surgery because of previous mediastinum radiotherapy or other procedures that preclude median sternotomy. We present one case of right lateral thoracotomy for membrane subaortic stenosis resection in a child with a restrosternal gastric tube.

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.

Combined obstructive hypertrophic cardiomyopathy and double outlet right ventricle in an infant with Down syndrome

Patient: Male, 2Final Diagnosis: Obstructive hypertrophic cardiomyopathySymptoms: Congestive heart failureMedication: —Clinical Procedure: Left ventricular septal myectomy • repair of congenital heart diseaseSpecialty: CardiologyObjective:Rare diseaseBackground:Hypertrophic cardiomyopathy (HCM) is uncommon in Down syndrome (DS). When combined with congenital heart disease (CHD) both morbidity and mortality may be greater compared to CHD alone. Whether HCM in DS patients is related to having trisomy 21 versus a second site mutation is unknown.Case Report:We report a case of severe HCM in an infant with DS in combination with double outlet right ventricle (DORV) who required surgery for relive of sub-aortic obstruction and congestive heart failure. We predicted that this infant would have a second site mutation involving either a sarcomeric protein or metabolic disorder as a cause for his HCM. Using current genetic and metabolic testing as well as histologic assessment of excised cardiac tissue we sought to further characterize the nature of the HCM. A successful resection of sub-aortic stenosis and DORV repair was performed. Genetic and metabolic testing was negative for gene defects and/or syndromes commonly associated with familial HCM. Excised cardiac tissue from the ventricular septum exhibited myocyte hypertrophy and sub-endocardial fibrosis but no sarcomeric disarray, myocyte fibrosis or glycogen storage. Metabolic testing for common forms of mitochondrial disease was negative. Post-operative echocardiograms show persistent, non-obstructive septal hypertrophy.Conclusions:Unlike prior reports, this child required a surgical intervention to relieve his sub-aortic obstruction. Thus, HCM in this population can be more serious that previously suspected. Although testing did not reveal the cause of his HCM, we still suggest screening for known causes of HSC until the etiology of the HCM in DS is well understood.

Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries

Recently, the double-switch operation for congenitally corrected transposition of the great arteries has become the procedure of choice in our institute; however, the long-term follow-up is uncertain. From 1983 to 2010, 90 patients with congenitally corrected transposition of the great arteries underwent the double-switch operation, which comprised of an atrial switch plus intraventricular rerouting (with or without extracardiac conduits) in 72 patients (group I), and an atrial switch plus arterial switch in 18 patients (group II). The mean age at operation was 7.4 years old in group I vs. 4.3 years old in group II. The mean follow-up period was 12.9 years in group I vs. 10.9 years in group II. Hospital and late mortality, reoperation, arrhythmia and NYHA status were analysed retrospectively. The Kaplan-Meier survival, including hospital and late mortality at 20 years, was similar (75.7% in group I vs. 83.3% in group II). The freedom from reoperation was 77.6% in group I (redo-Rastelli in five patients, subaortic stenosis resection in three, tricuspid valve replacement in one and mitral valve plasty in one) vs. 94.1% in group II (P < 0.05 vs. group I; aortic valve replacement in one). The freedom from arrhythmia was 57.1% in group I vs. 78.6% in group II (P < 0.05 vs. group I). The ratio of NYHA class I to II at outpatient clinic was similar (86% in group I vs. 86% in group II). The long-term prognosis of the double-switch operation for congenitally corrected transposition of the great arteries was acceptable. In particular, an atrial switch plus arterial switch could be performed with low morbidity, and it should be considered as the optimal procedure.

The Surgical History, Management, and Outcomes of Subaortic Stenosis in Adults

Subaortic stenosis (SAS) is a curtain of tissue involving the subaortic region, the aortic and mitral valves, the septum, and the fibrous trigones. Little is known of its course or the outcomes of its surgical management in adults. We reviewed our experience of the surgical management of SAS in adults from 1999 to 2010. We divided patients into three groups: (1) those presenting for first-time SAS resection (6 patients, 4 male, median age of 46.9 ± 17 years, mean follow-up of 5 ± 2.7 years); (2) those requiring redo resection of SAS without organic aortic valve dysfunction (8 patients, 3 male, median age of 25.3 ± 5 years, mean follow-up of 8 ± 3.08 years); and (3) those with SAS and aortic valve dysfunction (8 patients, 4 males, median age of 34.8 ± 12 years, mean follow-up of 4.5 ± 2.5 years; 5 had previous SAS surgery). Patients underwent extensive excision of the SAS, release of the fibrous trigones, and a septal myectomy if required. There was 1 early death in group 2 and 1 in group 3. In group 3, 1 patient underwent the Ross procedure and 7 patients had mechanical valve implantation. No patient required permanent pacemaker implantation. Overall follow-up was 3.3 ± 3 years (range, 6 months to 10 years). The preoperative left ventricular outflow tract gradient ranged from 40 to 120 mm Hg, and the postoperative left ventricular outflow tract gradient ranged from 0 to 16 mm Hg. At latest follow-up, no patient in groups 1 or 2 had greater than mild native aortic regurgitation. Subaortic stenosis resection in adults can successfully relieve left ventricular outflow tract obstruction, with low mortality. The complexity of SAS increases with time; therefore a longer duration of follow-up is needed to further validate our conclusions.

Reduction aortoplasty for moderately sized ascending aortic aneurysms.

Enlargement of the ascending aorta may coexist with concomitant valvular, coronary, or other cardiac diseases. If dilation is moderate (i.e., <6 cm diameter) and another cardiac procedure is being performed, we have reduced the diameter of the ascending aorta with an S-shaped incision and excision of the curves of the "S" as a modified Z-plasty. We have performed the procedure in 23 patients with concomitant procedures including aortic valve replacement in 21 (1 as a pulmonary autograft), coronary bypass in 1, and resection of subaortic stenosis in 1. There were 15 males and 8 females with a mean age of 53 years (range 8-67 years). The mean maximal preoperative diameter on transesophageal echocardiography was 5.0+/-0.7 cm (range 3.2-6.6 cm). The mean intraoperative postreduction diameter was 3.1+/-0.6 cm (range 2.1-4.1) (p<0.01). All patients tolerated their procedures well. Sixteen patients were studied by transthoracic echocardiography postoperatively. These patients had a mean intraoperative postreduction diameter of 2.9+/-0.65 cm that increased to 3.1+/-0.45 cm (p = NS) after a mean follow-up of 9.9+/-12.6 months. Of these, seven patients were studied >1 year postoperatively. Their mean intraoperative postreduction diameter of 2.9+/-0.5 cm increased to 3.1+/-0.35 cm (p = NS) after a mean follow-up of 22.1+/-9.2 months. No known recurrences of the aneurysms have occurred. We feel this technique is valid in patients with moderate aortic dilation undergoing concomitant cardiac procedures and in whom more aggressive aortic interventions are not warranted.

Mechanical Aortic Valve Replacement in Children and Adolescents After Previous Repair of Congenital Heart Disease

Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown-ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart defects. Between July 2002 and November 2008, 15 (10 male and 5 female) consecutive patients (mean age 14.5 +/- 10.5 years) underwent mechanical AVR. Hemodynamic indications for AVR were aortic stenosis in four (27%), aortic insufficiency in eight (53%), and mixed disease in three (20%) after previous repair of congenital heart defects. All patients had undergone one or more previous cardiovascular operations due to any congenital heart disease. Concomitant cardiac procedures were performed in all of them. In addition to AVR, in two patients, a mitral valve exchange was performed. One patient received a right ventricle-pulmonary artery conduit replacement as concomitant procedure. The mean size of implanted valves was 23 mm (range 17-29 mm). There were neither early deaths nor late mortality until December 2008. Reoperations were necessary in five (33%) and included implantation of a permanent pacemaker due to complete atrioventricular block in two (15%), mitral valve replacement with a mechanical prosthesis due to moderate to severe mitral regurgitation in one (7%), aortocoronary bypass grafting due to stenosis of a coronary artery in one (7%), and in one (7%), a redo subaortic stenosis resection was performed because of a secondary subaortic stenosis. At the latest clinical evaluation, all patients were in good clinical condition without a pathological increased gradient across the aortic valve prosthesis or paravalvular leakage in echocardiography. Mechanical AVR has excellent results in patients after previous repair of congenital heart defects in childhood, even in combination with complex concomitant procedures. Previous operations do not significantly affect postoperative outcome.

Bicuspidization of the Unicuspid Aortic Valve: A New Reconstructive Approach

Unicuspid anatomy of the aortic valve is infrequent but may require intervention by age 40 for severe regurgitation. We propose a new repair technique for the regurgitant unicuspid valve by converting it into a bicuspid aortic valve. Between November 2003 and September 2007, 20 patients underwent regurgitant unicuspid aortic valve repair: 13 had aortic regurgitation (AR) and 7 had combined regurgitation and stenosis. Four patients had previously undergone balloon valvuloplasty for critical aortic stenosis. The aim of the repair was to construct a bicuspid valve with two normal commissures and unrestricted cusp motion. The fused cusp tissue was divided anteriorly and a new commissure of normal height was created. Noncoronary and right coronary cusps were extended with autologous pericardium. Concomitant operations included ascending aortic replacement in 7 and resection of subaortic stenosis in 1. No early or late deaths occurred. Intraoperative echocardiography revealed minimal or no AR in 19 patients. Follow-up was 4 to 47 months. One patient underwent valve re-repair for recurrent and progressive aortic regurgitation 3 years postoperatively. All other valves remained stable throughout the follow-up period. Freedom from relevant aortic insufficiency (> or = II) at 4 years was 77%; freedom from reoperation was 67%; and freedom from valve replacement was 100%. The regurgitant unicuspid aortic valve can be repaired successfully and reproducibly by converting it into bicuspid anatomy. The functional results are comparable with those obtained in reconstructed bicuspid aortic valves. With this approach, replacement can be avoided in most patients with regurgitant unicuspid aortic valves.

The short term effects of cardiopulmonary bypass on neurologic function in children and young adults

Cognitive deficits are common in adults following surgery utilizing cardiopulmonary bypass (CPB). A previous retrospective study suggested that surgical closure of an ASD in children was associated with neurologic injury, while transcather therapy was not. In a prospective study, we sought to determine whether neurologic deficits occur following repair of non-complex congenital heart lesions in school-age children and young adults. Inclusion criteria were: age between 5 and 20 years, cardiac surgery utilizing CPB without deep hypothermic circulatory arrest, and no prior cardiac surgery. Patients underwent psychometric testing 1-3 days prior to surgery and re-evaluation 7-18 days after surgery. In order to determine the test/re-test effect an age-matched cohort of children undergoing transcather closure of ASD under general anesthesia was also evaluated. The primary outcome measures were verbal and picture memory. Additional psychometric tests included: computerized performance test (CPT) and Digit Span (DS). Forty-one patients were enrolled, 29 undergoing surgery with CPB and 12 controls. Surgical procedures included ASD closure (n=13), VSD closure (n=10), resection of sub-aortic stenosis (n=3), mitral valvuloplasty (n=3). Mild hypothermia was used in all cases. The mean duration of CPB was 54+/-22 min. There was no significant difference in any of the psychometric test scores between subjects undergoing surgery with CPB or controls. There are no marked adverse neurologic effects of CPB in school-age children and young adults undergoing non-complex open-heart surgery. These data are important in counseling patients and families and should be considered in the debate as to the relative merits of transcather versus open-heart repair of various heart lesions.

Posterior Pericardial Ascending-to-Descending Aortic Bypass

Background Coarctation of the aorta is commonly associated with recoarctation or additional cardiovascular disorders that require intervention. The best surgical approach in such patients is uncertain. Ascending-to-descending aortic bypass graft via the posterior pericardium (CoA bypass) allows simultaneous intracardiac repair or an alternative approach for the patient with complex coarctation. Methods and Results Between 1985 and 2000, 18 patients (13 males and 5 females, mean age 43±13 years) with coarctation of the aorta underwent CoA bypass through median sternotomy. Before operation, average New York Heart Association class was II (range I to IV), and 15 patients (83%) had systemic hypertension. One or more previous cardiovascular operations had been performed in 12 patients (67%); 10 patients had ≥1 prior coarctation repair. Two patients had prior noncoarctation cardiovascular surgery. Concomitant procedures performed in 14 patients (78%) included the following: aortic valve replacement in 9; coronary artery bypass surgery in 3; mitral valve repair in 2; and septal myectomy, mitral valve replacement, aortoplasty, subaortic stenosis resection, ventricular septal defect closure, and ascending aorta replacement in 1 patient each. All patients survived the operation and were alive with patent CoA bypass at a mean follow-up of 45 months. No graft-related complications occurred, and there were no instances of stroke or paraplegia. Systolic blood pressure fell from 159 mm Hg before surgery to 125 mm Hg after surgery. Conclusions CoA bypass via median sternotomy can be performed with low morbidity and mortality. Although management must be individualized, extra-anatomic CoA bypass via the posterior pericardium is an excellent single-stage approach for patients with complex coarctation or recoarctation and concomitant cardiovascular disorders.