Red blood cell abnormalities occur in dogs with congenital ventricular outflow tract obstruction.

OBJECTIVE: To document RBC abnormalities in dogs with congenital ventricular outflow tract obstruction. ANIMALS: 62 dogs with pulmonic stenosis (PS) or aortic stenosis (AS) and 20 control dogs were recruited. PROCEDURES: The proportions of RBCs that were schistocytes, acanthocytes, and keratocytes were assessed. Complete blood cell counts were performed. Tested variables included hemoglobin concentration, hematocrit, and erythrocyte count. RESULTS: Median (interquartile range [IQR]) peak systolic Doppler-derived trans-stenotic pressure gradient (∆P) values were 161 mm Hg (108 to 215 mm Hg) and 134 mm Hg (125 to 165 mm Hg) for dogs with PS and AS, respectively. Hematologic abnormalities were detected in most dogs with AS or PS (54/62 [87%]) versus 8/20 [40%] in control dogs, with schistocytes found in 40 of 62 (65%; median, 0.1% RBCs; IQR, 0% to 0.3%), acanthocytes in 29 of 62 (47%; median, 0.3% RBCs; IQR, 0% to 0.9%), keratocytes in 39 of 62 (63%; median, 0% RBCs; IQR, 0% to 0.2%), and hemolytic anemia in 4 dogs with PS. No significant association was identified between these abnormalities and ∆P. However, 3 of 4 dogs with anemia had a ∆P > 200 mm Hg (range, 242 to 340 mm Hg). The dog with the highest ∆P value also had the most severe anemia and schistocytosis, and both resolved after balloon valvuloplasty. CLINICAL RELEVANCE: Poikilocytosis is common in dogs with congenital ventricular outflow tract obstruction, with anemia only observed in few dogs with high ∆P values.

Double-root inversion for complex malposition of the great arteries with tricuspid valve straddling.

Dextro-transposition of the great vessels associated with pulmonary stenosis, double-outlet right ventricle, and straddling of the tricuspid valve is an uncommon condition. Several treatment options are available for this malformation, but most of them are not optimal. For patients with transposition of the great vessels, the gold standard procedure, which is an arterial switch procedure, would usually be performed, whereas for patients with pulmonary stenosis, a Rastelli operation or a Nikaidoh procedure would be proposed. Both of these methods have several advantages and disadvantages. Selected patients can qualify for the double-root rotation procedure, which is limited by the function of the pulmonary and aortic valves, the position of the coronary arteries, and the skill of the surgeon[1]. After a thorough analysis of all the preoperative test results, our patient qualified for a surgical correction of the malformation. Due to preexisting pulmonary regurgitation and severe dilation of the pulmonary root, the patient was not considered a good candidate for the arterial switch operation. Therefore, it was decided that the double-root inversion was the best option. Introduction The double-root inversion gives the patient the possibility of avoiding a reoperation. If the patient were to have the Nikaidoh or the Rastelli procedure, we know that the pulmonary graft would eventually have to be replaced. For this reason, we would like to share our experience with the double-root inversion method.

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome.

OBJECTIVES: To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). METHODS: We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 (< 24 weeks), 2 (24-30 weeks) and 3 (> 30 weeks). RESULTS: 61/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6% of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44/53 (83.0%) survived. 24/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2 × 1.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (p < 0.001). Tricuspid insufficiency (TI)-Vmax > 2.5 m/s was present in BV outcome group in75.0% (18/24) vs. 14.3% (2/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax < 2.5 m/s, TV/MV ratio < cutoff) to a score, non-BV outcome was correctly predicted when > 1 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4/9 (44.4%) and 5/14 (35.7%) in our interval 2 + 3 with predicted non-BV outcome would have been candidates for intervention. Two (1 × intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention. CONCLUSION: TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.

A cat's scratch or a wolf's bite?

Bartonella henselae endocarditis mimicking systemic vasculitis has been reported in patients with valvulopathy. Herein, we describe a patient with B. henselae endocarditis involving a prosthetic pulmonic valve with positive anti-dsDNA antibodies misdiagnosed with systemic lupus erythematosus (SLE) based on the revised classification SLE criteria.

Balloon Valvuloplasty to Treat Adult Symptomatic Pulmonary Valve Stenosis with Sequential Follow-Up Using Cardiac Magnetic Resonance Imaging in Combination with Echocardiography.

Pulmonary valve stenosis (PVS) accounts for approximately 10% of all congenital heart defects. Echocardiography and right heart catheterization are the gold standards for diagnosis of PVS and for assessing disease severity and responsiveness to treatment.Recently, cardiac magnetic resonance imaging (cMRI) has been established as an important tool to comprehensively evaluate cardiac structure and function; however, research into the usefulness of cMRI for PVS management is limited. Here, we describe a case of a 59-year-old female with isolated, severe PVS who was successfully treated with balloon pulmonary valvuloplasty (BPV) followed by sequential cMRI at 1 and 12 months. Exertional dyspnea and elevated plasma BNP concentration were observed 1 month after BPV; however, echocardiographic findings did not indicate recurrent stenosis or increased pulmonary valve regurgitation but an increase in mitral E/e'. cMRI demonstrated improved systolic forward flow and RV function with enlargement of LV volume, and the rapid increase in LV preload might be associated with the transient deterioration in symptoms and BNP level, which both gradually improved within 3 months after BPV. cMRI further depicted that a reduced RV mass index and increased RV cardiac output were achieved gradually during the follow-up period.In conclusion, cMRI in combination with echocardiography was sufficiently informative to follow-up this PVS patient both before and after BPV. cMRI is easily reproducible in adult patients; therefore, cMRI should be recommended for long-term follow-up in adult PVS patients.

Percutaneous pulmonary valve implant: Two Colombian case reports.

Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados.Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients.

Implante percutáneo de válvula pulmonar: a propósito de dos casos colombianos / Percutaneous pulmonary valve implant: Two Colombian case reports

Resumen Los pacientes con cardiopatías congénitas que afectan la continuidad del ventrículo derecho con la arteria pulmonar deben someterse con frecuencia a intervenciones debido a la limitada vida útil de los conductos quirúrgicos, lo que lleva al desarrollo de disfunción ventricular derecha por cambios en la geometría ventricular y predisposición a arritmias letales, con el consiguiente riesgo de reintervenciones. El implante valvular percutáneo pulmonar es una nueva alternativa terapéutica, menos invasiva en comparación con la quirúrgica, para pacientes seleccionados. Se realiza una revisión de las publicaciones médicas actuales disponibles y se describe la experiencia inicial del implante valvular pulmonar percutáneo en un centro colombiano de alta complejidad para el tratamiento de enfermedades cardiovasculares, en dos pacientes con disfunción del homoinjerto aórtico en posición pulmonar con doble lesión valvular, en los cuales el implante valvular pulmonar percutáneo fue una conducta exitosa. Se eligió a pacientes con cardiopatías congénitas, conductos quirúrgicos disfuncionales con estenosis o insuficiencia pulmonar significativa, y disfunción y dilatación ventricular derechas. Se empleó la técnica regular para el implante de la válvula pulmonar Melody, sin documentarse complicaciones durante el procedimiento ni al año de seguimiento. El implante percutáneo de la válvula pulmonar es un gran avance en el tratamiento de pacientes con cardiopatías congénitas, con resultados favorables a corto y mediano plazos, lo cual hace posible la restauración de la función ventricular con riesgo mínimo, frente al reemplazo quirúrgico en pacientes seleccionados.

Abstract Patients with congenital heart disease that involves reconstruction of the right ventricular outflow tract must frequently undergo interventions derived from the limited useful life of the surgical canals, which leads to the development of right ventricular dysfunction due to changes in the ventricular geometry and predisposition to lethal arrhythmias, with the consequent risk of reinterventions. The percutaneous pulmonary valvular implant is a new therapeutic alternative, less invasive, compared to surgery, for selected patients. A review of the available literature is made and the initial experience of percutaneous pulmonary valve implantation in a Colombian center of high complexity for the treatment of cardiovascular diseases is described, in two patients with aortic homograft dysfunction in a pulmonary position with double valvular lesion, in which the percutaneous pulmonary valve implant was a successful strategy. Patients with congenital heart disease were chosen, with dysfunctional surgical conduits with stenosis or significant pulmonary insufficiency, with dysfunction and right ventricular dilatation. The standard technique for the implantation of the Melody pulmonary valve was used, without complications during the procedure or one year of follow-up. Percutaneous implantation of the pulmonary valve is a great advance in the management of patients with congenital heart diseases, with favorable results in the short and medium term, allowing the restoration of ventricular function with minimal risk, compared to surgical replacement in selected patients.

Weighted gene co­expression network analysis identifies key genes from extracellular vesicles as potential prognostic biomarkers for congenital pulmonary stenosis.

Pulmonary stenosis (PS) is a congenital heart disease characterized by a dynamic or fixed anatomic obstruction of blood flow from the right ventricle to the pulmonary arterial vasculature. In the present study, extracellular vesicle long RNAs (EVLRs) from pregnant females who had healthy infants or PS infants were analyzed by RNA sequencing, and their diagnostic potential for PS during pregnancy was evaluated. A method for the selection of genes that could be considered as informative for the prediction PS based on extracellular vesicles (EVs) from pregnant females using long­read RNA sequencing was developed. Blood samples were collected from females carrying fetuses with PS and females carrying unaffected fetuses (n=6 in each group). Physical characterization of EVs was performed using nanoparticle tracking analysis, transmission electron microscopy and western blotting. EVLRs from plasma were profiled by RNA sequencing and mRNA co­expression modules were constructed by weighted gene co­expression network analysis (WGCNA). Gene Ontology (GO) enrichment analysis was used to predict the function of the genes in each module. Hub genes were filtered out based on WGCNA and visualized using Cytoscape. EVLRs consisted of mRNAs, microRNAs and long non­coding RNA. Overall, 26 modules were identified containing 16,394 genes. All modules were independent of each other. One particular module, referred to as the blue module, was markedly different between the two groups. A total of 735 hub genes in the blue module were identified, of which 33 were visualized, demonstrating the connection between these hub genes. GO enrichment analysis demonstrated that the analyzed hub genes were enriched in 'glucose transport', 'ATP­dependent chromatin remodeling', 'histone deacetylation', 'histone H3­K4 methylation', 'DNA methylation', 'apoptotic signaling pathway' and 'glucocorticoid receptor signaling pathway'. The hub genes identified in this module may provide a genetic framework for prenatal PS diagnosis. Furthermore, functional analysis of these associated genes may provide a theoretical basis for further research on PS pathogenesis.

Congenital right coronary artery aneurysm with fistula to right ventricle associated with isolated pulmonary valvular stenosis.

Congenital aneurysmal dilatation of coronary artery with coronary cameral fistula is rare in childhood. We report an even rarer association of congenital right coronary artery aneurysm and right coronary artery to right ventricle fistula with bicuspid pulmonary valve stenosis and an intact ventricular septum in a 3-year-old child.

Evaluation of the NuCLEUS-X™ balloon valvuloplasty catheter for severe pulmonic stenosis in dogs.

INTRODUCTION/OBJECTIVES: Balloon instability is commonly encountered during balloon pulmonary valvuloplasty (BPV) and may result in an unsuccessful procedure. The NuCLEUS-X™ catheter is a recently developed BPV catheter with a unique barbell shape and an ordered pattern of inflation that stabilizes the balloon to span the valve annulus before expansion of the balloon center. ANIMALS: Ten client-owned dogs with severe valvular pulmonic stenosis (PS). MATERIALS AND METHODS: Prospective observational study. The BPV procedure was performed by standard technique with use of NuCLEUS-X™ catheters targeting a balloon-to-annulus ratio between 1.2 and 1.5. Balloon stability, safety, and procedural success were assessed. Procedural success was defined as either a reduction in the Doppler transpulmonic PG by at least 50% of the pre-procedural PG or <80 mmHg one month post procedure. RESULTS: Balloon stability centered at the pulmonic valve on the first inflation was achieved in 10/10 cases. The mean PG before BPV was 141 mmHg ±41 mmHg, and the PG after BPV at one month was 83 mmHg ±41 mmHg. Procedural success was achieved in 56% of patients. All dogs survived the BPV, and no major procedural complications were encountered using the NuCLEUS-X™ catheter. CONCLUSIONS: The use of the NuCLEUS-X™ catheter is feasible for BPV in dogs with severe PS. The unique balloon shape provided catheter stability on the first inflation in all dogs, which may be beneficial when stabilization of a conventional BPV catheter cannot be achieved.

Left main coronary artery compression by the right pulmonary artery in a patient with congenital pulmonic stenosis / Compresión del tronco coronario izquierdo por la arteria pulmonar derecha en un paciente con estenosis pulmonar congénita

Abstract Congenital pulmonary stenosis (PS) can be associated with pulmonary artery (PA) dilatation. In some cases, this can cause compression of nearby structures including the left main coronary artery (LMCA). This compression causes angina and is considered an indication for surgical treatment. We present the case of a patient with PS and angina secondary to LMCA compression by the right PA and review the main indications and options for surgical treatment.

Resumen La estenosis pulmonar congénita se asocia a dilatación de la arteria pulmonar. En algunos casos esto puede causar compresión de las estructuras adyacentes incluyendo el tronco de la coronaria izquierda. Esta compresión causa angina y es considerada una indicación para tratamiento quirúrgico. Presentamos el caso de un paciente con estenosis pulmonar y angina secundaria a compresión del tronco de la coronaria izquierda por la arteria pulmonar derecha y revisamos las indicaciones y opciones de tratamiento quirúrgico.

Outcomes after combined percutaneous balloon valvuloplasty and external beam radiation therapy for the treatment of congenital pulmonic stenosis in four dogs.

Valve restenosis after percutaneous balloon pulmonary valvuloplasty (BPV) for the treatment of congenital pulmonic stenosis (PS) may occur in up to 17% of canine cases. Outcomes in dogs with PS that are treated with repeat BPV after restenosis have not been described. The present report details the clinical courses of four dogs with congenital PS, previously treated with conventional BPV and atenolol (n = 4) or atenolol alone (n = 1), two with anomalous, circumpulmonary coronary artery anatomy, which underwent BPV followed immediately by external beam radiation therapy (BPV + EBRT) to prevent valve restenosis. External beam radiation therapy involved five daily fractions of 3.6 Gray to the pulmonic valve. Echocardiographic and clinical follow-up information for 2-4 years after BPV + EBRT is presented. Three dogs experienced long-term reduction in transpulmonic pressure gradient. In one dog, which was treated with conservative BPV + EBRT as first-line therapy, return of transpulmonic pressure gradient to pretreatment levels was noted by 7 months after BPV + EBRT. Although clinical benefit remains unproven, the addition of EBRT to conventional BPV may be a treatment option for dogs experiencing restenosis after BPV or those in which restenosis is considered likely. Further study to evaluate the efficacy and safety of this approach is needed.

Left main coronary artery compression by the right pulmonary artery in a patient with congenital pulmonic stenosis.

Congenital pulmonary stenosis (PS) can be associated with pulmonary artery (PA) dilatation. In some cases, this can cause compression of nearby structures including the left main coronary artery (LMCA). This compression causes angina and is considered an indication for surgical treatment. We present the case of a patient with PS and angina secondary to LMCA compression by the right PA and review the main indications and options for surgical treatment.

La estenosis pulmonar congénita se asocia a dilatación de la arteria pulmonar. En algunos casos esto puede causar compresión de las estructuras adyacentes incluyendo el tronco de la coronaria izquierda. Esta compresión causa angina y es considerada una indicación para tratamiento quirúrgico. Presentamos el caso de un paciente con estenosis pulmonar y angina secundaria a compresión del tronco de la coronaria izquierda por la arteria pulmonar derecha y revisamos las indicaciones y opciones de tratamiento quirúrgico.

Impact of maternal pulmonary insufficiency on fetal growth in pregnancy.

Rationale: It is known that fetal growth is usually proportional to left-sided cardiac output (CO), which parallels the right-sided CO and that congenital right-sided lesions are usually associated with better perinatal outcomes than left-sided lesions.Objective: Our objective was to document whether newborns from mothers with severe residual pulmonary valve insufficiency (PI) after surgical tetralogy of Fallot (TOF) or pulmonary valve stenosis (PS) correction have lower birth weight (BW) than newborns from mothers with absent, mild, or moderate PI.Methods: This is a retrospective cohort study of women affected with repaired TOF and corrected PS with varied severity of residual PI. Exclusion criteria were: left ventricular dysfunction, left-sided valvular heart disease, other right-sided structural heart disease, chronic hypertension, substance addiction, and incomplete follow-up. Pregnancies were divided into three groups: absent or mild PI, moderate PI, and severe PI. A generalized linear model with normal dependent variable distribution was built and the parameter estimation made with Generalized Estimation Equations (GEE) to take into account repeated mother in data. Variables such as gestational age at birth, maternal age, smoking, and body mass index were tested with bivariate analyses to assess their effect on BW. Only gestational age remained in the adjusted model.Results: A total of 45 patients were included (33 TOF and 12 PS) and 97 pregnancies were reported: 22 miscarriages (22.7%) (15 TOF, 7 PS) and 75 successful pregnancies (57 TOF, 18 PS). The patients were divided into three groups: 1) absent or mild PI, 2) moderate PI, and 3) severe PI groups, which comprised, respectively, 29 (15 TOF, 4 PS), 20 (10 TOF, 1 PS), and 26 successful pregnancies (8 TOF, 7 PS). Using three levels of PI (absent or mild, moderate, and severe), the unadjusted model showed a significant effect of level of PI on BW (p = .0118), as well as the adjusted model (p = .0263) with gestational age as a covariate. The estimated mean newborn's BW was 3055.8 g in the severe PI group, 3151.0 g in the moderate PI group, and 3376.4 g in the absent or mild group when adjusted for gestational age. Hence, we estimated that the mean newborn's BW is 321 g lower in the severe PI group compared with absent or mild PI group ((CI: 572.3; -68.9), p = .0087).Conclusions: Pregnancy is usually well tolerated in repaired TOF and corrected PS. Severe PI either from repaired TOF or PS is at higher risk of lower newborn's BW. Special attention must be paid to the severity of PI. Fetal growth surveillance in the third trimester is warranted.

Bidirectional Glenn for residual outflow obstruction in Tetralogy of Fallot.

BACKGROUND: Residual right ventricular outflow obstruction during Tetralogy of Fallot repair necessitates peri-operative revision often requiring trans-annular patch with its negative sequels. Bidirectional Glenn shunt in this setting reduces trans-pulmonary gradient to avoid revision. METHODS: Bidirectional Glenn shunt was added during Tetralogy repair in patients with significant residual obstruction. A total of 53 patients between January, 2011 and June, 2018 were included. Final follow-up was conducted in July, 2018. RESULTS: Mean age at operation was 5.63±3.1 years. Right to left ventricular pressure ratio reduced significantly (0.91±0.09 versus 0.68±0.05; p<0.001) after bidirectional Glenn, avoiding revision in all cases. Glenn pressures at ICU admission decreased significantly by the time of ICU discharge (16.7±3.02 versus 13.5±2.19; p<0.001). Pleural drainage ≥ 7 days was seen in 14 (26.4%) patients. No side effects related to bidirectional Glenn-like facial swelling or veno-venous collaterals were noted. Mortality was 3.7%. Discharge echocardiography showed a mean trans-pulmonary gradient of 32.11±5.62 mmHg that decreased significantly to 25.64±5 (p<0.001) at the time of follow-up. Pulmonary insufficiency was none to mild in 45 (88.2%) and moderate in 6 (11.8%). Mean follow-up was 36.12±25.15 months (range 0.5-90). There was no interim intervention or death. At follow-up, all the patients were in NYHA functional class 1 with no increase in severity of pulmonary insufficiency. CONCLUSION: Supplementary bidirectional Glenn shunt significantly reduced residual right ventricular outflow obstruction during Tetralogy of Fallot repair avoiding revision with satisfactory early and mid-term results.

Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis.

OBJECTIVES: Our preferred approach for the surgical treatment of patients with D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis has been the Rastelli operation. We herein evaluate our 30-year experience with this procedure. METHODS: Clinical records for patients who underwent the Rastelli operation between 1988 and 2017 at our institution were retrospectively reviewed. Primary outcomes included freedom from death or cardiac transplantation and freedom from conduit reintervention. RESULTS: Forty-seven patients met inclusion criteria. Mean follow-up was 11.7 ± 6.8 years. Forty-three (91.5%) patients received a palliative systemic-to-pulmonary artery shunt and/or atrial septostomy prior to the Rastelli procedure. Five (10.6%) patients required ventricular septal defect enlargement at the time of the Rastelli procedure. The overall mean right ventricle-to-pulmonary artery conduit size was 17.0 mm. Mortalities included one early and three late deaths. Freedom from death or cardiac transplantation was 93% and 84% at 5 and 25 years, respectively. Seven patients required pacemaker placement, two immediately postoperatively and five late. Freedom from conduit replacement was 85% and 25% at 5 and 15 years, respectively. Seven (14.9%) patients required a second conduit intervention. Forty-one (87.2%) patients were New York Heart Association class I or II at the most recent follow-up. CONCLUSIONS: The Rastelli operation for D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis offers excellent mid- to long-term survival. The need for conduit replacement remains the most common indication for reintervention, and further study of the optimal choice of conduit will be useful.

Use of magnetic resonance imaging combined with gene analysis for the diagnosis of fetal congenital heart disease.

BACKGROUND: Fetal deformity is a disease caused by abnormal chromosome structure, which may be influenced by genetic factors as well as the maternal and external environment. Magnetic resonance imaging (MRI) may be used to effectively diagnose fetus deformities. However it has been reported that gene analysis is a more accurate diagnostic method. The aim of the present study was to investigate the effectiveness of MRI in combination with gene analysis for the diagnosis of fetal congenital heart disease, a form of fetus deformity. METHODS: MRI, array comparative genome hybridization analysis and fluorescence in situ hybridization were used to analyze the effectiveness of the two methods in a total of 78 pregnant women with suspected fetal congenital heart disease. RESULTS: Our findings demonstrated that the combination of MRI and gene analysis resulted in significantly improved diagnostic accuracy, sensitivity and specificity for fetal congenital heart disease compared with either method alone. MRI combined with gene analysis confirmed 42 fetuses with pulmonary stenosis, 24 with aortic stenosis and 12 healthy fetuses, which was significantly improved compared with MRI or gene analysis alone. It was also observed that gene analysis was a more efficient method of diagnosis compared with MRI; however, the combination of the two methods was the most effective. CONCLUSION: In conclusion, the results of the present study suggest that MRI combined with gene analysis may be a more effective diagnostic method for fetal congenital heart disease compared with the current protocol.