The Damus-Kaye-Stansel 0peration: Management of systemic ventricular outflow tract obstruction.

The Damus-Kaye-Stansel (DKS) anastomosis was originally described for the treatment of transposition of the great arteries but it is now used mainly for the treatment of single ventricle anomalies in patients who are at risk of developing systemic ventricular outflow tract obstruction (SVOTO). In most instances, DKS is performed as a concomitant procedure at time of bidirectional cavopulmonary connection in these patients. This tutorial we demonstrate our modified double-barrel approach, along with anterior patch augmentation of the distal ascending aorta. We have found that this technique is very effective in mitigating SVOTO risk while preserving semilunar valve anatomy and function.

Ross-Konno Procedure With Cylinder Mitral Valve Replacement in 49 Days Old Infant.

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.

Midterm results of the Ross procedure in children: an appraisal of the subannular implantation with interrupted sutures technique.

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.

Asymptomatic and isolated accessory mitral valve tissue in an adult.

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.

Ross Procedure in Neonates and Infants: A European Multicenter Experience.

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.

[Discrete Subaortic Stenosis in an Adult; Report of a Case].

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.

Isolated right subclavian artery with interrupted aortic arch, ventricular septal defect, and left ventricular outflow tract obstruction.

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.

Echocardiographic evaluation of congenital left ventricular outflow obstruction.

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.

Outcomes of the modified norwood procedure: hypoplastic left heart syndrome versus other single-ventricle malformations.

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.

Porcine bioprosthetic valve in the pulmonary position: mid-term results in the right ventricular outflow tract reconstruction.

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.

Repair of left ventricular inflow tract lesions in Shone's anomaly: valve growth and long-term outcome.

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.

Ross-Konno procedure in infants: mid-term results.

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.

The Ross-Konno procedure as reoperative treatment in a young adult with congenital aortic stenosis.

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.

Progression of outflow tract obstruction in the fetus.

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.

Association of common variants in ERBB4 with congenital left ventricular outflow tract obstruction defects.

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.

Hospital mortality in surgery for reconstruction outflow right ventricle with pulmonary homograft.

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.

Mutations in VEGFA are associated with congenital left ventricular outflow tract obstruction.

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_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.

Mortalidade hospitalar na cirurgia de reconstrução da via de saída do ventrículo direito com homeonxerto pulmonar / Hospital mortality in surgery for reconstruction outflow right ventricle with pulmonary homograft

FUNDAMENTO: Mortalidade hospitalar na cirurgia de reconstrução da via de saída do ventrículo direito com homoenxerto pulmonar é variável. OBJETIVOS: Identificar os fatores de risco associados à mortalidade hospitalar e ao perfil clínico dos pacientes. MÉTODOS: Estudo de crianças submetidas à reconstrução da via de saída do ventrículo direito com homoenxerto pulmonar. Analisados como fatores de risco as variáveis clínicas, cirúrgicas e de aspectos morfológicos da prótese. RESULTADOS: Noventa e dois pacientes foram operados entre 1998 e 2005, apresentando principalmente atresia pulmonar com comunicação interventricular e a tetralogia de Fallot. Quarenta pacientes foram atendidos no primeiro mês de vida. Necessitaram de 38 cirurgias de Blalock Taussig devido à gravidade clínica. A idade mediana na cirurgia de correção total foi de 22 meses, variando de 1 mês a 157 meses. O tamanho homoenxerto pulmonar variou de 12 a 26 mm e o tempo de extracorpórea foi 132 ± 37 minutos. Após a cirurgia houve 17 óbitos (18 por cento casos), em média 10,5 ± 7,5 dias após. A causa predominante foi falência de múltiplos órgãos. Na análise univariada entre os tipos de cardiopatia, estas deferiram na idade, momento da cirurgia, tamanho do homoenxerto, valor Z da valva pulmonar, tempo de circulação extracorpórea, manutenção da integridade do homoenxerto e alteração da árvore pulmonar. Não houve diferença estatística com relação à mortalidade hospitalar entre as variáveis e o tipo de cardiopatia. CONCLUSÃO: As cardiopatias obstrutivas do lado direito necessitam de atendimento cirúrgico nos primeiros dias de vida. A cirurgia de correção total apresenta risco de mortalidade de 18 por cento, mas não houve associação com nenhuma variável estudada.

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 percent 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 differencein 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 percent but there was no association with any variable studied.

Long-term results of right ventricular outflow tract reconstruction in neonatal cardiac surgery: options and outcomes.

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.

Simultaneous apicoaortic conduit placement and mitral valve replacement in an adolescent with porcelain aorta, aortic stenosis, and mitral stenosis.

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.