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Congenital left ventricular outflow obstruction represents a multilevel obstruction with several morphological forms. It can involve the subvalvular, valvar, or supravalvular portion of the aortic valve complex, and may coexist. Computed tomography (CT) plays an important supplementary role in the evaluation of patients with congenital LVOT obstruction. Unlike transthoracic echocardiography and cardiovascular magnetic resonance (CMR) imaging, it is not bounded by a small acoustic window, needs for anaesthesia or sedation, and metallic devices. Current generations of CT scanners with excellent spatial and temporal resolution, high pitch scanning, wide detector system, dose reduction algorithms, and advanced 3-dimensional postprocessing techniques provide a high-quality alternative to CMR or diagnostic cardiac catheterization. Radiologists performing CT in young children should be familiar with the advantages and disadvantages of CT and with the typical morphological imaging features of congenital left ventricular outflow obstruction., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

A neonate with antenatally diagnosed severe aortic valve stenosis, left ventricular outflow tract obstruction, left ventricular endocardial fibroelastosis, and severe mixed mitral valve disease was admitted to our center for balloon aortic valvuloplasty. On day 49, we performed a Ross-Konno procedure and mitral valve replacement on the baby, whose weight was 3.4 kg. Right ventricle-to-pulmonary artery continuity was restored with a handmade trileaflet Gore-Tex conduit. We used a handmade cylindrical prosthesis made from decellularized equine pericardium in the mitral position. We observed improvement in left ventricular function and good performance of aortic, pulmonary, and mitral prostheses at four months of follow-up.

Management of right ventricular outflow tract obstruction has undergone much change over the last century. Techniques described in the literature include anatomical repairs and the use of various patches, conduits, and innovative grafts. However, many of these approaches require reoperations or catheter-based interventions, leading to increased morbidity, mortality, and cost. The search for the ideal long-lasting conduit continues and there are new techniques on the horizon, using genetic engineering and nanotechnology. This review discusses the evolution of various techniques for repair of right ventricular outflow tract obstruction, past and current conduits, as well as ongoing research.

Objectives: The support of the pulmonary autograft root by the fibromuscular left ventricular outflow tract is emphasized to address the concern related to the dilatation of the pulmonary autograft structures in the paediatric population., Methods: This retrospective study analyses the outcomes of 75 children who were operated between 1998 and 2012 with the subannular interrupted sutures technique at a median age of 10.2 years (range, 5.3 months-18.0 years). Median follow-up time was 5.2 years (range, 3 days-13.2 years)., Results: There were no deaths, but there were 3 reinterventions on the autograft for regurgitation and 2 resections of left ventricular outflow tract obstruction. There was no significant autograft stenosis, and freedom from moderate-to-severe regurgitation was 95% (95% confidence interval: 89-100) and 88% (95% confidence interval: 77-99) at 5 and 10 years, respectively. Median z-scores at the latest follow-up examination were, at the annulus, 0.31 [interquartile range (IQR) = -0.81 to 1.2]; at the sinus of Valsalva, 2.7 (IQR = 1.5-3.5); and at the sinotubular junction, 3.1 (IQR = 1.7-4.2). The correlation between z-scores and time after the operation was negative at the level of the annulus (r = -0.29, P = 0.034) but positive at the level of the sinus (r = +0.37, P = 0.005) and the sinotubular junction (r = +0.26, P = 0.068). The median rate of change in the z-score at the annulus was low, 0.065 z-score/year (IQR = -0.13 to 0.43)., Conclusions: The subannular interrupted sutures implantation technique is associated with acceptable risks and, in the midterm, delivers limited annular dilatation, autograft regurgitation and delayed need for autograft reintervention., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Accessory mitral valve (AMV) tissue is a congenital anomaly that occurs in association with other congenital anomalies, and is an uncommon cause of left ventricular outflow tract obstruction. It is usually detected in early childhood when accompanied by symptoms of obstruction of the left ventricular outflow tract, and is rarely diagnosed in adults. We present a case of a 53-year-old man who was referred to our institution for evaluation of a systolic heart murmur. Echocardiography disclosed a diagnosis of AMV tissue. This case was uncommon because of the lack of severe obstruction of left ventricular outflow, cardiac symptoms, or other cardiac anomalies. We were able to carry out surgical resection of AMV tissue to avert possible progression of aortic insufficiency and the risk of a cerebrovascular embolization. The patient's postoperative course was uneventful, and postoperative echocardiography showed no residual accessory mitral tissue.

Background: Infants and neonates with severe left ventricular outflow tract obstruction may require pulmonary autograft replacement of the aortic root. In this retrospective multicenter cohort study, we present our experience with the Ross procedure in neonates and infants with a focus on midterm survival and pulmonary autograft durability., Methods: A retrospective observational study was performed in 76 infants (aged less than 1 year) operated on in six congenital cardiac centers in The Netherlands and Germany between 1990 and 2013., Results: Patients had a pulmonary autograft replacement of the aortic valve with (68%) or without (32%) septal myectomy. Median patient age was 85 days (range, 6 to 347). Early mortality (n = 13, 17%) was associated with neonatal age, preoperative use of intravenous inotropic drugs, and congenital aortic arch defects. Five patients (9%) died during follow-up. Freedom from autograft reintervention was 98% at 10 years. Echocardiography demonstrated good valve function, with no or trace regurgitation in 73% of patients. Freedom from right ventricular outflow tract reintervention was 51% at 10 years. Univariable analysis demonstrated superior freedom from reintervention of pulmonary homografts compared with aortic homografts or xenografts., Conclusions: Pulmonary autograft replacement of the aortic valve in neonates and infants is a high-risk operation but offers a durable neoaortic valve. Midterm durability reflects successful adaptation of the autograft to the systemic circulation. Late mortality associated with heart failure was an unexpected finding., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Discrete subaortic stenosis (DSS) is a well-described cause of isolated left ventricular outflow tract obstruction( LVOTO) in children. But prevalence, rate of progression and postoperative data in adults are limited. We report a case of a 30-year-old woman, who was referred to our institution because of chest pain and loss of consciousness. Echocardiography revealed DSS with LVOTO (peak gradient 81 mmHg) and mild aortic regurgitation. Increased age at the time of diagnosis, female sex and preoperative left ventricular outflow tract(LVOT) gradient ≥80 mmHg were thought to be predictors for reoperation, therefore the obstructing membrane was circumferentially excised and concomitant localized myectomy of the ventricular septum was performed to achieve complete relief of the LVOT obstruction. Her postoperative course was uneventful, and she was discharged on the 5th postoperative day.

Objective: To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants., Methods: Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011., Results: Median age and weight at the time of first stent implantation were 8 weeks and 3.3 kg, with 40% of patients <3 kg. A total of 19 patients had suffered from hypoxemic spells, 8 patients were ventilated, 6 on inotropic support and 5 on prostaglandin infusion. Severe concomitant malformations were present in 11 patients, and acute infections in 2. Stenting of the RVOT was successfully performed in 33 patients, improving oxygen saturation from a median of 77 to 90% 2 days after intervention. Besides the 2 patients in whom RVOT stenting was not successful for technical reasons, there were no procedural complications. In 17 of 33 patients, 1-3 reinterventions were performed during follow-up, less than half of those were reinterventions in the RVOT. A total of 27 patients have undergone successful surgical repair 4-162 (median 19.5) weeks after initial RVOT stent implantation, 2 patients are still waiting. There were no perioperative deaths., Conclusions: Stenting of the RVOT provides a safe and effective management strategy for initial palliation in symptomatic young infants, including those patients not suitable or at higher risk for surgical therapy., (© 2015, Wiley Periodicals, Inc.)

We present two cases of isolated right subclavian artery from the right pulmonary artery (PA) associated with interrupted aortic arch, ventricular septal defect, left ventricular outflow tract obstruction, and 22q11 microdeletion. Both patients were successfully managed with bilateral PA banding initially followed by a modified Yasui operation. Isolation of the subclavian artery is rare but should always be taken into account, especially when bilateral PA banding is considered. The banding must then be placed on the PA distal to the origin of the subclavian artery., (© The Author(s) 2014.)

Left ventricular outflow obstructions (LVOTO) encompass a series of stenotic lesions beginning within the anatomic left ventricular outflow tract and ending in the descending thoracic aorta, occurring alone or in series. The degree of stenosis and hemodynamic consequences may vary from mild and well tolerated to severe and functionally limiting. The majority of LVOTO (with the exception of calcific and rheumatic aortic stenosis) are congenital in etiology. Echocardiography has become an integral tool in the assessment of patients with congenital heart disease. This article is a contemporary review of the clinical features of LVOTO and the role of echocardiography in this patient population., (© 2014, Wiley Periodicals, Inc.)

Operations for left ventricular outflow tract abnormalities are centred on hemodynamic conditions that relate to subvalvar stenosis, valvar stenosis/regurgitation, aortic annular hypoplasia, and supravalvar aortic stenosis. Operative interventions over the years have evolved because the intervening outcomes proved to be unsatisfactory. The resection for subvalvar aortic stenosis has progressed from a fibrous "membrane" resection to a more extensive fibromuscular resection. Operative solutions for valvar aortic stenosis and regurgitation have resulted in operative interventions that depend on simple commissurotomy, leaflet extensions, prosthetic mechanical valve replacement, biologic valve replacement, including the pulmonary autograft, and operations to treat aortic annular stenosis. Although there are enthusiastic proponents for all of these strategies, the fact remains that none have proven to be curative; patients can expect to undergo further procedures during their lifetimes. The short- and mid-term solutions to these left ventricular outflow tract abnormalities have improved based on operations that have been attended by increasing operative complexity. The purpose of this review is to chronicle the operative steps of the Ross operation, the Konno-Rastan operation, the modified Konno operation, the Ross-Konno operation, and the modified Ross-Konno operation.

The aim of our project was to study possible etiological factors in the origin of congenital heart defects (CHDs) because in the majority of patients the underlying causes are unclear. Cases with different CHD entities as homogeneously as possible were planned for evaluation in the population-based large data set of the Hungarian Case Control Surveillance of Congenital Abnormalities. Dead or surgically corrected 302 live-born cases with different types of left-ventricular outflow tract obstructive defects (LVOT, i.e., valvular aortic stenosis 76, hypoplastic left heart syndrome 76, coarctation of the aorta 113, and other congenital anomalies of aorta 32) were compared with 469 matched controls, 38,151 controls without any defects, and 20,750 malformed controls with other isolated defects. Medically recorded pregnancy complications and chronic diseases were evaluated based on prenatal maternity logbooks, whereas acute diseases, drug treatments, and folic acid/multivitamin supplementation were analyzed both on the basis of retrospective maternal information and medical records. The results of the study showed the role of maternal diabetes in the origin of LVOT in general, while panic disorder was associated with a higher risk of hypoplastic left heart syndrome and ampicillin treatment with a higher risk of coarctation of the aorta (COA). High doses of folic acid had a protective effect regarding the manifestation of LVOT, particularly COA. In conclusion, only a minor portion of causes was shown in our study; thus, further studies are needed to understand better the underlying causal factors in the origin of LVOT.

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p < 0.001). The follow-up data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignificant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan-Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant difference). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ significantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post-Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan completion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of functional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.

We present a case of an infant prenatally diagnosed with bilateral outflow-tract obstruction and severe aortic regurgitation who underwent cardiac transplantation at 45 days of life. Aortico-left ventricular tunnel was subsequently diagnosed on pathologic examination of the explant heart. Aortico-left ventricular tunnel is a rare congenital cardiac malformation and can remain undiagnosed if the clinician has a low level of suspicion. Aortico-left ventricular tunnel should be considered in any fetus or newborn with aortic regurgitation.

Pulmonary valve replacement (PVR) for pulmonary valve insufficiency (PVI) currently represents the most frequent reoperation performed for adults with congenital heart disease. A variety of pulmonary valve substitutes have been used, but none has proved to be ideal. This report reviews the authors' experience using a porcine prosthetic valve in the pulmonary position. Between January 2001 and December 2011, 76 patients (mean age, 36 years; range, 18-64 years) underwent PVR for chronic PVI using a porcine bioprosthesis. All the patients had previously undergone surgery: 65 for repair of tetralogy of Fallot and 11 for pulmonary surgical valvotomy. Magnetic resonance imaging (MRI) evaluations before surgery and at the 1-year postoperative follow-up evaluation were compared. Aside from the PVR, 59 patients (59/65, 78 %) received 94 associated cardiac surgical procedures. Two hospital deaths occurred. The mean hospital stay was 13 days (range, 7-48 days). At the 1-year control MRI, pulmonary regurgitation fraction, right ventricular end diastolic volume (RVEDV), and RV/LV EDV had improved significantly. During a mean follow-up period of 52 months (range, 6-132 months), one patient died. All the patients were categorized as New York heart association (NYHA) functional class 1. No episodes of structural valve deterioration, endocarditis, or thromboembolic event were noted. Echocardiography showed trivial or no PVI in all the patients. The porcine bioprosthetic valves demonstrated excellent midterm results in the RV outflow tract reconstruction. The hemodynamic characteristics of this valve are comparable with those of homografts or valved conduits. It is easy to implant and allows for avoiding extensive dissection, especially of the pulmonary arteries.

Background: The degree of involvement of left ventricular inflow tract obstruction is the predominant factor determining outcome in Shone's anomaly. In this series of patients with Shone's anomaly, we evaluated the impact of mitral valve (MV) repair strategies performed to correct the components of this anomaly on growth of the valve and long-term functional outcome in children., Methods: In the last 25 years, 45 children, mean age 5.16 ± 5.0 years (median, 3.9; range, 2 months-16.8 years), underwent surgical correction of Shone's anomaly. Coarctation of the aorta was found in 40%, subaortic stenosis due to fibromuscular hypertrophy was found in 55%, and subvalvar membrane was found in 66% of these patients. Left ventricular inflow tract obstruction was brought about by fused commissures with dysplastic and shortened chordae in 53.3%, valve hypoplasia in 11.1%, supravalvar mitral ring in 100%, and parachute valve in 17.8 of patients%., Results: Various repair strategies were performed according to the presenting morphologic characteristics in patients with either previously corrected or concomitant correction of the left-sided obstructive lesions. Mean duration of follow-up was 17.5 ± 1.5 years. Freedom from reoperation was 52.8% ± 11.8%, wherein 23 patients underwent repeated MV repair and 1 patient underwent MV replacement after failed attempts at repair. The cumulative survival rate was 70.3% ± 8.9% at 15 years. Severity and type of mitral abnormalities, left ventricular outflow tract lesions, and pulmonary hypertension are risk factors for reoperation and mortality (p < 0.05)., Conclusions: Repair allowed growth of the MV. Long-term outcome of MV repair in Shone's anomaly is related to the degree that the obstructive lesions can be relieved., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of study was to analyse the mid-term results of the Ross-Konno procedure in infants., Methods: Between 2000 and 2011, 16 infants, including five newborns, with complex left ventricular outflow tract (LVOT) obstruction underwent the Ross-Konno procedure. Twelve patients (75%) required multiple concomitant procedures such as: mitral valve (MV) surgery (four patients), resection of endocardial fibroelastosis (EFE) and myectomy (six patients), closure of ventricular septal defect (four patients) and aortic arch reconstruction (three patients). The median age at operation was 4.2 months (from 6 to 333 days)., Results: There was one late death with a median follow-up of 6.2 years. Actuarial survival is 93.3% at 5 years follow-up (95% confidence interval: 61.2-99.0). Postoperatively, two patients required extracorporeal membrane oxygenation support and one MV replacement. The median length of stay in hospital was 30 days (from 11 to 77 days). At 5 years of follow-up, seven patients had no aortic regurgitation (AR) and nine patients (56%) had trivial AR with no gradient in LVOT. Freedom from mitral regurgitation (MR) ≥ moderate or MV replacement was 70%. MR was associated with either structural abnormalities of MV or with development of EFE. Freedom from redo was 81 and 53% at 1 and 5 years of follow-up. Sixty percent of patients are without medication. All patients are in sinus rhythm., Conclusions: With the technical aspects of this procedure well accomplished, the risk of surgery is minimal and functional outcome is encouraging. However, early postoperative morbidity is significant. At the mid-term follow-up, there was no residual or recurrent outflow tract obstruction and excellent function of the neoaortic valve. A high incidence of MR associated with the development of EFE and structural abnormalities of the MV is worrisome; however, concomitant MV surgery is not associated with increased mortality. In the case of the development of EFE, an early indication for operation might protect MV function. The reoperation rate is high due to early conduit failure.

Mechanical and biological prostheses are valid options when aortic valve replacement is necessary. The Ross procedure is also an alternative solution, especially for young patients. We describe the case of a young patient with congenital aortic stenosis and bicuspid aortic valve who presented with dyspnea on exertion. An open commissurotomy was performed, and within 8 months the patient developed recurrent symptoms of severe aortic stenosis. He underwent redo sternotomy and a Ross-Konno procedure with an uneventful recovery.

Fetal ventricular outflow tract obstruction (OTO) is congenital heart disease with significant potential for progression before birth as a consequence of the unique nature of the fetal circulation. The pattern of evolution depends upon the timing of development, severity of obstruction and the influence of the OTO on the fetal atrioventricular valve and myocardial function. Critical aortic (AS) or pulmonary (PS) valve stenosis, the two most common forms of fetal OTO, may be associated with progressive ventricular and great artery hypoplasia if presenting early in gestation or with normal ventricular and great artery growth if evolving later in gestation. In some affected fetuses, AS or PS may lead to the evolution of fetal heart failure. This article will review our current understanding of the natural history of fetal AS and PS, experience with fetal intervention and future directions of research., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Left ventricular apex to descending aorta conduits may be used as a last resort treatment of severe left ventricular outflow tract obstruction in cases in which alternative therapies are contraindicated. Although this technique is rarely used in current practice for congenital cases, its use in the elderly population is increasing, largely due to the expansion of this patient cohort and associated comorbidities precluding aortic valve replacement, the most common of which are a severely calcified "porcelain" aorta and/or previous coronary artery bypass grafts preventing aortic root manipulation. Diagnostic imaging is essential in the presurgical workup and subsequent follow-up of these patients, as complications of the procedure are potentially life threatening and are not rare. Several imaging modalities may be used, each with advantages and disadvantages. Both anatomic and functional assessments play a role in the comprehensive evaluation of both presurgical and postsurgical patients.

Double-chambered right ventricle (DCRV) is a form of right ventricular outflow tract obstruction. It typically presents in childhood or adolescence. Only a handful of previous cases have been described in which DCRV occurred in adulthood. We report here a case of DCRV with ventricular septal defect (VSD) presenting in adulthood.

Background: The left ventricular outflow tract (LVOT) defects aortic valve stenosis (AVS), coarctation of the aorta (COA), and hypoplastic left heart syndrome (HLHS) represent an embryologically related group of congenital cardiovascular malformations. They are common and cause substantial morbidity and mortality. Prior evidence suggests a strong genetic component in their causation., Methods: We selected NRG1, ERBB3, and ERBB4 of the epidermal growth factor receptor (EGFR) signaling pathway as candidate genes for investigation of association with LVOT defects based on the importance of this pathway in cardiac development and the phenotypes in knockout mouse models. Single nucleotide polymorphism (SNP) genotyping was performed on 343 affected case-parent trios of European ancestry., Results: We identified a specific haplotype in intron 3 of ERBB4 that was positively associated with the combined LVOT defects phenotype (p=0.0005) and in each anatomic defect AVS, COA, and HLHS separately. Mutation screening of individuals with an LVOT defect failed to identify a coding sequence or splice site change in ERBB4. RT-PCR on lymphoblastoid cells from LVOT subjects did not show altered splice variant ratios among those homozygous for the associated haplotype., Conclusion: These results suggest ERBB4 is associated with LVOT defects. Further replication will be required in separate cohorts to confirm the consistency of the observed association., (Copyright © 2011 Wiley-Liss, Inc.)

Purpose of Review: To provide a discussion of the current knowledge of the genetics of left outflow tract of the heart, including the aortic stenosis, in children. It addresses the available means of diagnosis for syndromic and nonsyndromic left outflow tract abnormalities and implications for at-risk family members. Options for prenatal testing and recommendations for cardiac follow-up are presented., Recent Findings: Left outflow tract cardiac anomalies in children present as a varied spectrum among and within families. Even nonsyndromic forms can be inherited in an autosomal dominant pattern. These can lead to significant complications in asymptomatic individuals, making diagnosis a challenge and underscoring the importance of evaluation of at-risk family members., Summary: Improved understanding of the genetics of both syndromic and nonsyndromic left outflow tract disorders is hoped to lead to improved identification of affected children and greater ongoing cardiac follow-up for those potentially at risk.

Left ventricular outflow tract obstruction (LVOTO) comprises a spectrum of stenotic lesions. Previous studies have shown that the vascular endothelial growth factor (VEGF) signaling system plays a critical role in cardiac cushion formation, vasculogenesis, and angiogenesis. We hypothesize that VEGFA may be a potential candidate gene associated with the spectrum of LVOTO lesions. However, it remains unclear whether the VEGFA gene is responsible for the development of LVOTO malformations. In this study, we identified three exon mutations in the VEGFA gene in three of 192 nonsyndromic LVOTO patients, and the overall mutation frequency was 1.6% (3/192). The c.454C>T (p.Arg152X) nonsense mutation and c.19&#95;22dupGACA (p.Thr8ArgfsX78) internal tandem duplication mutation each introduced a premature stop codon and are predicted to produce a truncated VEGFA protein. The c.998G>A missense mutation changes a highly conserved arginine to a glutamine at residue 333 (p.Arg333Gln). These mutations were carried by some family members, and average penetrance was 33.3%. The present study suggests, for the first time to our knowledge, that VEGFA mutations may be associated with congenital LVOTO malformations. We provide evidence that LVOTO is likely oligogenic., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

Background: Hospital mortality for surgical reconstruction of the outflow of the right ventricle with pulmonary homograft is variable., Objectives: To identify risk factors associated with hospital mortality and clinical profile of patients., Methods: Children underwent reconstruction of the outflow tract of right ventricle with pulmonary homograft. Analyzed as risk factors for the clinical, surgical and morphological aspects of the prosthesis., Results: Ninety-two patients operated on between 1998 and 2005 presented mainly pulmonary atresia with ventricular septal defect and tetralogy of Fallot. Forty patients were treated in the first month of life. He needed 38 surgeries to Blalock Taussig due to clinical severity. The median age at surgery for total correction was 22 months, ranging from 1 to 157 months. Size pulmonary homograft ranging from 12 to 26 mm and length of bypass was 132 +/- 37 minutes. After surgery there were seventeen deaths (18% cases) on average 10.5 +/- 7.5 days. The predominant cause was multiple organ failure. In the univariate analysis between the types of heart disease, they found in favor of age at surgery, size of homograft, pulmonary valve Z value, CPB time, maintaining the integrity of the homograft and pulmonary tree change. There was no statistical difference in hospital mortality between the variables and the type of heart disease., Conclusion: The congenital obstructive right requires surgical care in the first days of life. The total correction surgery has a risk rate of 18% but there was no association with any variable studied.

Objectives: Neonatal surgery for tetralogy of Fallot and truncus arteriosus requires reconstruction of the right ventricular outflow tract. Although the method of reconstruction is often dictated by the individual anatomy, choices exist. This review examines the durability and outcomes of right ventricular outflow tract reconstruction in the neonate., Methods: This is a retrospective review of all 278 neonates with the diagnosis of tetralogy of Fallot and truncus arteriosus undergoing right ventricular outflow tract reconstruction at a single center between 1990 and 2007. Diagnostic variants included tetralogy of Fallot/pulmonary stenosis (n = 83), tetralogy of Fallot/pulmonary atresia (n = 81), and tetralogy of Fallot with absent pulmonary valve (n = 17). Truncus arteriosus was present in 97 patients. Patients were analyzed on the basis of diagnosis and the method of right ventricular outflow tract reconstruction: aortic homograft, pulmonary homograft, transannular patch, transannular patch with monocusp pulmonary valve, and nontransannular patch. Freedom from reoperation/reintervention was determined by using the log-rank test., Results: The mean age at right ventricular outflow tract reconstruction was 11.8 +/- 8 days, and hospital survival was 95.0% for the tetralogy of Fallot group and 90.7% for the truncus arteriosus group. Overall freedom from reoperation and reintervention was 76.2% +/- 14.8% in the nontransannular patch group and 59.5% +/- 6.8% in the transannular patch group; both were significantly greater than seen in patients receiving either aortic (0%) or pulmonary (6.7% +/- 4.2%) homografts (P < .05). There was no difference between aortic and pulmonary homografts. Among patients with tetralogy of Fallot/pulmonary stenosis, there was no difference in 10-year freedom from reoperation/reintervention between the transannular (70.8% +/- 7.4%) and nontransannular patch methods (76.2% +/- 14.8%, P = .53). At 10 years, the diagnosis of tetralogy of Fallot/pulmonary stenosis was associated with a greater freedom from reoperation/reintervention (68% +/- 6.8%) when compared with tetralogy of Fallot/pulmonary atresia (5.3% +/- 4.3%, P = .0001), tetralogy of Fallot/absent pulmonary valve (0%, P = .00315), or truncus arteriosus (4.2% +/- 2.8%, P = .0001). Eight patients (4 with tetralogy of Fallot/pulmonary stenosis, 3 with tetralogy of Fallot/pulmonary atresia, and 1 with tetralogy of Fallot/absent valve) underwent placement of a transannular patch with monocusp valve. Among this group, freedom from reoperation/reintervention is 41.7% +/- 20.5% at 2.5 years. Monocusp function, as determined by means of echocardiographic analysis obtained at 11.4 +/- 11.7 months (range, 0.3-31 months) showed an average monocusp gradient of 23.5 +/- 26.1 mm Hg, and 3 (37.5%) patients had more than moderate pulmonary regurgitation., Conclusions: The durability of neonatal right ventricular outflow tract reconstruction is diagnosis and method dependent. Anatomy allowing right ventricular outflow tract patching (either transannular or nontransannular) provides a durability advantage compared with that seen with a homograft. There was no difference in performance between aortic and pulmonary homografts, and the monocusp valve has limited durability and effectiveness in neonatal right ventricular outflow tract surgery. The long-term outcomes of transannular and nontransannular patching techniques for neonatal repair of tetralogy of Fallot/pulmonary stenosis are similar.

We describe the treatment of a 16-year-old girl with calcific aortic stenosis, porcelain aorta, and calcific mitral stenosis with insufficiency using a valved apicoaortic conduit and mitral valve prosthesis. Both valve replacements were porcine bioprostheses, and the apicoaortic conduit was implanted without the use of cardiopulmonary bypass. In cases in which the degree of aortic calcification pre-empts manipulation of the coronary ostia, an apicoaortic conduit may offer a viable solution to improve left ventricular outflow obstruction.

Background: Percutaneous pulmonary valve implantation (PPVI) has been introduced as therapy for right ventricular (RV) to pulmonary artery conduit dysfunction in patients with congenital heart disease. It has been shown that RV systolic function improved early after PPVI. The effects of PPVI on RV diastolic function and RV hypertrophy have not yet been studied., Purpose: The objective of this study is to assess early and late changes in systolic and diastolic RV function and RV mass after PPVI., Materials and Methods: Fourteen patients underwent PPVI (7 male, median age 15 years). Cardiac magnetic resonance imaging was performed before and at 2 time points after PPVI (at 1 and 16 months). Right ventricular volume and systolic and diastolic function as well as RV mass were assessed., Results: At 1 and 16 months after PPVI, the RV mass decreased from 28.6 +/- 2.1 to 25.6 +/- 2.2 g/m(2) (P = .03) and to 22.3 +/- 2.1 g/m(2) (P = .002). E/A volume ratio increased from 1.91 +/- 0.4 to 2.6 +/- 0.4 (not significant [NS]) and to 3.3 +/- 0.4 (P = .01). E/A peak flow ratio increased from 1.34 +/- 0.14 to 1.48 +/- 0.16 (NS) and to 1.73 +/- 0.14 (P = .04). E-wave deceleration time increased from 142 +/- 25 to 160 +/- 27 milliseconds (NS) and to 211 +/- 26 milliseconds (P = .007). At 1 month, RV end-diastolic volume decreased from 124 +/- 8 to 113 +/- 8 mL (P = .01) and RV ejection fraction increased from 36% +/- 2% to 46% +/- 2% (P = .001) without further improvement at 16 months., Conclusion: After PPVI, in contrast to rapid improvement of RV systolic function, the improvement of RV diastolic function is delayed. The reduction of RV mass appears to be the underlying mechanism for improvement of RV diastolic function. Long follow-up for patients with PPVI is recommended.

Background and Aim of the Study: Due to hemodynamic performance and potential for growth of the pulmonary autograft, the Ross operation is considered to be the surgery of choice for irreparable aortic valve disease in pediatric patients. The study aim was to analyze the long-term clinical and echocardiographic results of the Ross operation., Methods: Between February 1993 and July 2006, 55 consecutive patients (mean age 10.0 +/- 6.2 years; range: 3 months to 18 years) underwent eithera Ross operation (n=46) or a Ross-Konno procedure (n=9). The underlying left ventricular outflow tract pathology was mainly congenital (n=47). Among patients, 23 (42%) had undergone a previous aortic valve procedure. Concomitant procedures were performed in 16 patients (29%). The Ross operation was performed as a root replacement in all cases; the mean cross-clamp time was 132 min (range: 100-188 min). The autograft diameter was indexed to the body surface area and compared to normal values. The mean follow up was 5.5 +/- 3.8 years, and was 100% complete., Results: There was one early death (2%) and two late deaths (4%). The actuarial patient survival was 93% at 10 years. None of the patients developed moderate or severe autograft regurgitation. All measured maximal root diameters were above the 90th percentile of normal aortic diameter, without correlation to autograft regurgitation. Five patients (9%) had a mean homograft gradient > or = 40 mmHg, and two (4%) were reoperated on. The freedom from reoperation for homograft degeneration was 91% at 10 years., Conclusion: Autograft regurgitation after the Ross and Ross-Konno procedures is uncommon, and the risk of homograft degeneration appears low. Autograft dilatation is common but does not correlate with autograft regurgitation. When considering long-term freedom from autograft and homograft degeneration, the results of the present study confirm the Ross operation as the surgery of choice for irreparable aortic valve disease in infants, children and young adults.

We describe an infant with severe obstruction of infra-cardiac totally anomalous pulmonary venous connection associated with right isomerism, atrioventricular septal defect, pulmonary atresia, and multiple aortopulmonary collateral arteries. Implantation of a stent into the obstructed descending vertical vein provided effective palliation, with a dramatic increase in saturations of oxygen obviating the need for urgent high-risk surgery.

Congenital aortic valve stenosis (AVS), coarctation of the aorta (COA) and hypoplastic left heart syndrome (HLHS) are congenital cardiovascular malformations that all involve the left ventricular outflow tract (LVOT). They are presumably caused by a similar developmental mechanism involving the developing endothelium. The exact etiology for most LVOT malformations is unknown, but a strong genetic component has been established. We demonstrate here that mutations in the gene NOTCH1, coding for a receptor in a developmentally important signaling pathway, are found across the spectrum of LVOT defects. We identify two specific mutations that reduce ligand (JAGGED1) induced NOTCH1 signaling. One of these mutations perturbs the S1 cleavage of the receptor in the Golgi. These findings suggest that the levels of NOTCH1 signaling are tightly regulated during cardiovascular development, and that relatively minor alterations may promote LVOT defects. These results also establish for the first time that AVS, COA and HLHS can share a common pathogenetic mechanism at the molecular level, explaining observations of these defects co-occurring within families.

Microvillus inclusion disease is a life-threatening diarrheal disorder of infancy characterized by the presence of microvillus inclusions within the intestinal epithelium. We report a case of a neonate with microvillus inclusion disease that was associated with coarctation of the aorta and bicuspid aortic valve, cardiac malformations within the spectrum of left ventricular outlet tract obstruction. Possible links between the intestinal and cardiac phenotypes are discussed.

Accessory mitral valve tissue is an uncommon finding, usually associated with other congenital cardiac malformations and diagnosed during childhood. We present the case of a 70-year-old patient referred to our hospital for aortic valve and ascending aorta replacement. At preoperative examination, no evidence of mitral valve structural abnormalities was detected. Anaesthesia induction was complicated by sudden haemodynamic impairment. Intraoperative transoesophageal echocardiography showed two anomalous mitral chordae. The presence of abnormal mitral tissue also allowed to assess the mechanism of unexpected haemodynamic worsening: anomalous chordae caused anterior displacement of the anterior mitral leaflet, resulting in left ventricular outflow tract obstruction and mitral regurgitation. Medical therapy was instituted and the planned surgical approach was modified according to the new anatomy observed at echocardiographic examination. A valve-sparing technique was successfully applied. This case provides a good example of the diagnostic usefulness of routine intraoperative transoesophageal echocardiography, especially when facing unexpected findings. It also shows how transoesophageal echocardiography may help guide medical and surgical treatment.

The case is presented of a congenital left ventricular outflow tract (LVOT) obstruction caused by a malpositioned hypertrophied papillary muscle. The patient was initially treated with subaortic membrane resection that did not address the papillary muscle. Subsequently, she underwent a Ross procedure after her native aortic valve had deteriorated. Neither of these operations alleviated the symptoms, nor did they relieve the LVOT obstruction caused by the papillary muscle. Shortly after the Ross operation the autograft dilated and caused severe regurgitation, whereupon the patient's symptoms worsened. Herein are presented the authors' considerations in repairing or replacing each of the patient's four valves, including reversing the Ross procedure, in an attempt to repair the congenital defect and minimize the need for future surgery.

Background: Multilevel obstruction of left-sided heart structures was originally characterized by Shone and colleagues. The formulation of an appropriate operative strategy remains problematic. Surgical outcomes have generally been poor. This review examines operative strategies and mid-term outcomes., Methods: A retrospective review was done of 28 patients who presented with the diagnosis of Shone complex at a median age of 14 days, with 46% presenting within the first week of life. At presentation, 5 patients were in cardiovascular collapse, and 10 were at New York Heart Association functional class III or IV. All patients had a structurally abnormal mitral complex. A mean transvalvular pressure gradient of 8 mm or greater existed in 15 patients, 6 had a supraannular mitral ring, and 26 had a coarctation of the aorta. Twelve mitral valve operations were done, with an average time from presentation to operative intervention of 15 months., Results: Two patients were lost to follow-up. Of 26 remaining patients, 2 died after a second operative intervention. The average follow-up of 24 patients was 6.3 years (range, 1 to 16 years). Overall survival was 93%. All surviving patients are in class I or II congestive heart failure., Conclusions: Long-term survival of patients diagnosed with Shone complex is excellent. Operative strategies for this complex group should be individualized. Mitral interventions may generally be deferred.

Background: In neonates, left-sided obstructive heart defects are critical diseases requiring early recognition, initial stabilization, and transfer to Cardiac Center., Objectives: To assess management of these neonates in primary care hospitals., Methods: Retrospective analysis of medical records of neonates admitted to Cardiac Intensive Care Unit in whom diagnosis of left-sided obstructive heart defect was established., Results: During 8-year period records of 117 patients were evaluated. Of those, 83 had hypoplastic left heart syndrome, 13 aortic stenosis, 12 coarctation of aorta, and 9 interruption of aortic arch. In 13 (11%) patients diagnosis of heart defect was established prenatally, other neonates presented postnatally with heart failure (28.2%), shock (65.4%) or severe cyanosis (4.8%). Continuous infusion of PGE1 and dopamine was administered in 84.6% and 30.8% patients, respectively. Thirty-two (30.8%) patients were mechanically ventilated and 36 (34.6%) received sodium bicarbonate. In referring hospitals the heart defect was most often diagnosed using echocardiography and diagnosis was established accurately in 55% patients., Conclusion: The study showed that in primary care hospitals neonates with known or suspected left-sided obstructive heart disease need more aggressive resuscitative measures than those used in this patient series. Prenatal diagnosis rate of these heart defects is very low (Tab. 3, Fig. 1, Ref 8). Full Text (Free, PDF) www.bmj.sk.

Background: Although numerous signaling pathways are known to be activated in experimental cardiac hypertrophy, the molecular basis of the hypertrophic response inherent in human heart diseases remains largely unknown. Integrin-linked kinase (ILK) is a multifunctional protein kinase that physically links beta-integrins with the actin cytoskeleton, suggesting a potential mechanoreceptor role., Methods and Results: Here, we show a marked increase in ILK protein levels in hypertrophic ventricles of patients with congenital and acquired outflow tract obstruction. This increase in ILK was associated with activation of the Rho family guanine triphosphatases, Rac1 and Cdc42, and known hypertrophic signaling kinases, including extracellular signal-related kinases (ERK1/2) and p70 S6 kinase. Transgenic mice with cardiac-specific expression of a constitutively active ILK (ILK(S343D)) or wild-type ILK (ILK(WT)) exhibited a compensated ventricular hypertrophic phenotype and displayed an activation profile of guanine triphosphatases and downstream protein kinases concordant with that seen in human hypertrophy. In contrast, transgenic mice with cardiomyocyte-restricted expression of a kinase-inactive ILK (ILK(R211A)) were unable to mount a compensatory hypertrophic response to angiotensin II in vivo., Conclusions: Taken together, these results identify ILK-regulated signaling as a broadly adaptive hypertrophic response mechanism relevant to a wide range of clinical heart disease.

Background: The Ross aortic valve replacement with a modified Konno-type enlargement Ross-Konno procedure of the aortic annulus and subannular region allows an autograft aortic valve replacement for children with significant annular and subannular hypoplasia. The potential for growth and the proven durability of the autograft make the Ross-Konno procedure an ideal aortic valve replacement for this subgroup with multilevel left ventricular outflow tract obstruction. We reviewed our institutional midterm experience to assess autograft and homograft hemodynamics, and management after a Ross-Konno procedure., Methods: Between 1995 and 2005, 14 consecutive children (mean age, 6.4 +/- 5.9 years; range, 1 month to 17 years) underwent the Ross-Konno procedure. All children had severe to critical aortic stenosis or multilevel left ventricular outflow tract obstruction., Results: There was 1 early and 1 late death with a mean follow-up of 5.7 +/- 3.6 years. Actuarial survival at 10 years was 86%. Three patients underwent right ventricular outflow tract reoperation for conduit replacement for homograft dysfunction and one patient required redo aortic root replacement with a mechanical valves for progressive aortic insufficiency. Freedom from right ventricular outflow tract and autograft reoperation at 10 years is 77% and 92%, respectively. Aortic annular dilation was not observed in all patients. Univariate and multivariate analysis identified no risk factors for autograft or homograft valve-related reoperation., Conclusions: The Ross-Konno procedure is an excellent technique to treat complex multilevel left ventricular outflow tract obstruction in children with significant annular and subannular hypoplasia. The autograft demonstrated durability without development of aortic stenosis or progressive dilation and a low incidence of developing progressive aortic insufficiency. Enlargement of the aortic annulus appear to parallel somatic growth in most instances.

We present a case of fetal tricuspid valve dysplasia, which was diagnosed at 22 weeks of gestation during a routine obstetrical examination. Serial fetal echocardiographic evaluation revealed progressive right ventricular outflow tract obstruction and persistent cardiomegaly. A female infant weighing 1,916 g was delivered by elective cesarean section at 38 weeks of gestation. Longstanding compression of the fetal lungs secondary to the persistent cardiomegaly resulted in severe respiratory distress and cyanosis immediately after birth. Although, mechanical ventilation and continuous infusion of prostaglandin were instituted, the infant died of respiratory failure at 21 days of age. During the pregnancy, the fetus exhibited intrauterine growth restriction, but hydrops did not occur. In regard to the fetal hemodynamics in this cardiac anomaly, transatrial communication is essential for fetal survival. The diameter of the fossa ovalis, which is a marker of transtrial blood flow, was adequate in this case. However, marked enlargement of the right heart associated with regurgitation interfered with left ventricular filling and output, which resulted in restriction of the combined ventricular output and intrauterine fetal growth restriction.

Background and Aim of the Study: A variety of valve substitutes are used for right ventricular outflow tract (RVOT) reconstruction in children and young adults after previous RVOT surgery that has led to significant pulmonary insufficiency and/or stenosis. Herein, the authors' experience with pulmonary valve replacement (PVR) using a porcine valved conduit late after previous RVOT surgery was reviewed., Methods: Between August 2002 and April 2005, 31 patients (mean age 14.5 +/- 9.5 years; range: 1.2-33.1 years) underwent PVR using the Medtronic Freestyle porcine aortic root. These patients averaged two prior operations (range: 1-5) for the following diagnoses: tetralogy of Fallot +/- pulmonary atresia (n = 21); persistent truncus arteriosus (n = 5); aortic stenosis (Ross-Konno procedure) (n = 2); pulmonary atresia with intact ventricular septum; (n = 1); congenital pulmonary stenosis (n = 1); and transposition of the great arteries (n = 1). Of these patients, 29 (93.5%) underwent additional procedures at the time of pulmonary valve insertion including: branch pulmonary artery reconstruction (n = 21), atrial septal defect closure (n = 5), ascending aorta replacement (n = 4), pacemaker or defibrillator placement (n = 3), tricuspid valve repair (n = 2), ventricular septal defect closure (n = 2), and other procedures (n = 2)., Results: There were no early or late deaths. The mean hospital stay was 8.3 +/- 5.9 days (range: 4-25 days). One patient had a subarachnoid hemorrhage with transient left hemiparesis, and two patients had acute tubular necrosis with temporary dialysis treatment. All patients were well at a mean follow up of 13 +/- 9.3 months (range: 0.5-31 months). Echocardiography showed trivial or no pulmonary insufficiency in 30 patients (96.7%). The calculated mean peak systolic RVOT gradient by echocardiography was 23.4 +/- 7.6 mmHg., Conclusion: The Medtronic Freestyle bioprostheses demonstrated excellent short-term results for repeat RVOT reconstruction. This valve's hemodynamic characteristics are comparable to those of homografts, and it is an attractive alternative given the limited availability of homograft valves. A lack of availability in sizes smaller than 19 mm limits use of this valve in pediatric patients, and long-term results remain to be determined.

We report the rare combination of a severely hypoplastic left ventricle and discordant ventriculoarterial connections, with associated subpulmonary obstruction of the left ventricular outflow tract. The long tortuous arterial duct originated at an acute angle from the aorta, suggesting that the subpulmonary obstruction developed early in fetal life. Residual flow via the hypoplastic left ventricle to the pulmonary circulation after insertion of an aortopulmonary shunt resulted in haemodynamic deterioration, which was instantly reversed by closing the pulmonary trunk.

Accessory mitral valve tissue is an extremely rare congenital cardiac anomaly. Most of the cases reported in the literature were associated with left ventricular outflow tract obstruction. In this report, a second cerebrovascular event is presented in the same patient two years after an operation in which septal myectomy was performed but accessory mitral valve tissue was not excised.