Hipertrofia grave del ventrículo derecho secundaria a estenosis valvular pulmonar en lactante mayor con síndrome de Noonan: reporte de caso / Severe right ventricular hypertrophy secondary to pulmonary valve stenosis in an older infant with Noonan syndrome: a case report

Introducción: El Síndrome de Noonan se caracterizada por alteraciones del crecimiento, retraso psicomotor y mental, dismorfia facial, alteraciones musculo-esqueléticas y alteraciones cardíacas hasta en el 80 % de los pacientes, miocardiopatía hipertrófica 30%, estenosis valvular pulmonar 50 % y defectos septales, estenosis de ramas pulmonares, tetralogía de Fallot o coartaciones aórticas. Caso clínico: lactante de 8 meses con hipertelorismo, ptosis palpe-bral, orejas con implantación baja, cuello corto y escoliosis. Se presenta con cianosis y disnea asociada a hipotonía muscular. Peso: score Z: -3, talla: score Z: -3, a la auscultación cardiaca: soplo meso-sistólico grado 4/6 en segundo espacio intercostal izquierdo, línea para-esternal. En el ecocardiograma se observa estenosis pulmonar valvular de grado moderado (gradiente sistólico de 52 mmHg) y dilatación del tronco arterial pulmonar. Evolución: Se efectúa cateterismo cardíaco con evidencia estenosis valvular pulmonar grave, reacción infundibular, hipertrofia del ventrículo derecho, apertura valvular en domo y conducto arterioso persistente filiforme "tipo E", estos hallazgos justificaban el desarrollo de hipertrofia cardíaca. Se realizó una valvuloplastia pulmonar con balón que mejoró las presiones cardíacas. Conclusiones: Las alteraciones cardíacas presentes en un lactante con síndrome de Noonan fueron: Hipertrofia biventricular, hipertensión pulmonar, estenosis valvular pulmonar, conducto arterioso persistente.

Introduction: Noonan syndrome is characterized by growth disorders, psychomotor and mental retardation, facial dysmorphia, musculoskeletal disorders, and cardiac disorders in up to 80% of patients, hypertrophic cardiomyopathy in 30%, pulmonary valve stenosis in 50%, septal defects, pulmonary branch stenosis, tetralogy of Fallot, and aortic coarctations. Clinical case: 8-month-old infant with hypertelorism, palpebral ptosis, low-set ears, short neck, and scoliosis. It presents with cyanosis and dyspnea associated with muscle hypotonia. Weight: Z score: -3, height: Z score: -3, on cardiac auscultation: mid-systolic murmur grade 4/6 in the second left intercostal space, parasternal line. The echocardiogram shows moderate valvular pulmonary stenosis (52 mmHg systolic gradient) and dilatation of the pulmonary arterial trunk. Evolution: Cardiac catheterization was performed with evidence of severe pulmonary valve stenosis, infundibular reaction, right ventricular hypertrophy, dome valve opening, and "type E" filiform patent ductus arteriosus. These findings justified the development of cardiac hypertrophy. Pulmonary balloon valvuloplasty was performed, which improved cardiac pressure. Conclusions: The cardiac alterations present in an infant with Noonan syndrome were biventricular hypertrophy, pulmonary hypertension, pulmonary valve stenosis, and patent ductus arteriosus.

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.

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.

Caracterización clínica y molecular de niños con síndrome de Noonan y otras RASopatías en Argentina / Clinical and molecular characterization of children with Noonan syndrome and other RASopathies in Argentina

Introducción. Las RASopatías son un conjunto de síndromes fenotípicamente superpuestos causados por mutaciones en genes implicados en la vía RAS/MAPK. La herencia es autosómica dominante, presentan características clínicas comunes, como baja talla, dismorfias craneofaciales, cardiopatia congénita, manifestaciones ectodérmicas y mayor riesgo de cáncer. El diagnóstico molecular es clave. Objetivo. Identificar mutaciones en los genes PTPN11, SOS1,RAF1, BRAFy HRAS,y comparar las principales características clínicas en pacientes con confirmación molecular. Población y métodos. Se estudiaron niños con diagnóstico clínico de RASopatía evaluados entre agosto de 2013 y febrero de 2017. Resultados. Se identificaron mutaciones en el 71 % (87/122) de los pacientes. El estudio molecular confirmó el diagnóstico en el 73 % de los pacientes con síndrome de Noonan. La mutación más prevalente fue c.922A>G (p.Asn308Asp) en el gen PTPN11. Se detectó una variante no descrita en RAF1, c.1467G>C (p.Leu489Phe). Se confirmó el sindrome cardiofaciocutáneo en el 67 % de los casos con mutaciones en el gen BRAF. El síndrome de Costello y el síndrome de Noonan con múltiples lentigos se confirmaron en todos los casos. Conclusión. La confirmación del diagnóstico clínico permitió un diagnóstico diferencial más preciso. Se determinó la prevalencia de las mutaciones en PTPN11 (el 58 %), SOS1 (el 10 %) y RAF1 (el 5 %) en niños con síndrome de Noonan, en PTPN11 (el 100 %) en el sindrome de Noonan con múltiples lentigos, en BRAF (el 67 %) en el síndrome cardiofaciocutáneo y en HRAS (el 100 %) en el sindrome de Costello.

Introduction. RASopathies are a set of syndromes with phenotypic overlapping features caused by gene mutations involved in the RAS/MAPK pathway. They are autosomal dominantly inherited and share common clinical characteristics, including short stature, craniofacial dysmorphisms, congenital heart disease, ectodermal manifestations, and a higher risk for cancer. A molecular diagnosis is a key factor. Objective. To identify PTPN11, SOS1, RAF1, BRAF, and HRAS mutations and compare the main clinical characteristics of patients with molecular confirmation. Population and methods. Children with a clinical diagnosis of RASopathy assessed between August 2013 and February 2017. Results. Mutations were identified in 71 % (87/122) of patients. The molecular test confirmed diagnosis in 73 % of patients with Noonan syndrome. The most prevalent mutation was c.922A>G (p.Asn308Asp) in the PTPN11 gene. A previously undescribed variant in RAF1 was detected: c.1467G>C (p.Leu489Phe). Cardiofaciocutaneous syndrome was confirmed in 67 % of cases with BRAF mutations. Costello syndrome and Noonan syndrome with multiple lentigines were confirmed in all cases. Conclusion. The confirmation of clinical diagnosis allowed for a more accurate differential diagnosis. The prevalence of PTPN11 (58 %), SOS1 (10 % ), and RAF1 mutations (5 %) in children with Noonan syndrome, of PTPN11 mutations (100 %) in those with Noonan syndrome with multiple lentigines, of BRAF mutations (67 %) in those with cardiofaciocutaneous syndrome, and of HRAS mutations (100 %) in those with Costello syndrome was determined.

Etiología de la enfermedad valvular cardíaca: Centro Cardiovascular Regional ASCARDIO-Barquisimeto / Etiology of valvular heart disease: Centro Cardiovascular Regional ASCARDIO-Barquisimeto

La enfermedad valvular cardíaca representa un problema de salud pública a nivel mundial. El objetivo del presente estudio de recolección retrospectiva de datos fue determinar la etiología valvular en pacientes que asisten a la consulta de patología valvular del Centro Cardiovascular Regional ASCARDIO-Barquisimeto en el periodo comprendido entre 2002 y 2015. Se revisaron 1760 historias médicas obteniendo como resultado que la insuficiencia mitral fue la lesión valvular más frecuente (66,13%). Con respecto a patología aórtica, la estenosis aórtica severa predominó en un 69,2% de casos seguido de insuficiencia aórtica leve (37,7%). La etiología degenerativa fue la más frecuente para los casos de estenosis e insuficiencia aórtica (76,4% y 59,5% respectivamente). En cuanto a la lesión valvular mitral, predominaron la estenosis (58,3%) e insuficiencia severa (42,4%) mientras que la etiología de la estenosis e insuficiencia mitral fue predominantemente reumática (89% y 33%, respectivamente). La estenosis tricuspídea fue exclusivamente reumática y la insuficiencia tricuspídea y pulmonar se consideró principalmente funcional. La estenosis pulmonar fue exclusivamente congénita. Este estudio aportará información para evaluar la situación actual de la enfermedad valvular cardíaca en el estado Lara(AU)

Valvular heart disease represents a public health problem worldwide. The goal of this study was to determine the etiology of valvular disease in patients that attend the cardiac valvular pathology consult of the Centro Cardiovascular Regional ASCARDIO-Barquisimeto during the period 2002 to 2015. We reviewed 1760 medical charts and the results show that mitral regurgitation was the most frequent lesion (66.13%). In regards to aortic valvular pathology, severe aortic stenosis predominated in 69.2% of cases followed by mild aortic regurgitation (37.7%). The main cause was degenerative for both aortic stenosis and regurgitation (76.4% and 59.5%, respectively). With respect to mitral valve lesion, stenosis and severe regurgitation was more common (58.3%, and 42.4%, respectively), while the etiology of mitral stenosis and insufficiency was in most cases rheumatic. Tricuspid as well as pulmonary regurgitation was considered mainly functional. Pulmonary stenosis was exclusively congenital. This study provided information to evaluate the current situation of cardiac valvular disease in Lara state(AU)

Implantación de stent en drenaje venoso anómalo supracardiaco obstructivo / Stent implantation in obstructive supracardiac anomalous drainage

Resumen El drenaje venoso anómalo pulmonar total representa un grupo de defectos cardíacos congénitos con baja incidencia pero alto potencial de complicaciones. Se expone el caso de una lactante menor de bajo peso al nacer, con drenaje venoso anómalo pulmonar total obstructivo, programada para implante de stent en vena vertical y posterior cirugía correctiva, evidenciando obstrucción residual mínima en la llegada de la vena pulmonar derecha y reducido tamaño auricular izquierdo, con prolongada estancia en cuidado intensivo secundaria a dificultad para el destete de la ventilación mecánica debido a edema venocapilar. Este caso demostró que la intervención endovascular es una opción en neonatos de alto riesgo con drenaje venoso anómalo pulmonar total obstructivo como parte de la estrategia de estabilización cardiovascular preoperatoria; la cirugía correctiva debe considerarse una vez las condiciones clínicas lo permitan, con el objetivo de minimizar la morbilidad asociada al compromiso de la distensibilidad de las cavidades izquierdas.

Abstract Total anomalous pulmonary venous return is a group of congenital cardiac defects. They have a low incidence but a high potential for complication. The case is presented of a young infant of low birthweight, with total obstructive anomalous pulmonary venous return, scheduled for a stent implant in a vertical vein, and subsequent corrective surgey. This showed evidence of a minimal residual obstruction on reaching the right pulmonary vein, and a reduced left atrial size. She had a long stay in intensive care secondary to difficulty in weaning off mechanical ventilation due to the presence of venous-capillary edema. This case demonstrates that endovascualr intervention is an option in high risk infants with a total obstructive anomalous pulmonary venous return as part of the strategy of pre-operative cardiovascular stabilisation. Corrective surgery should be considered when the clinical conditions permit it, with the aim of minimising the morbidity associated with the compromise of the distensibility of the left cavities.

Noonan syndrome and RASopathies: Clinical features, diagnosis and management

Noonan syndrome (NS) and NS-related disorders (cardio-facio-cutaneous syndrome, Costello syndrome, NS with multiple lentigines, or LEOPARD [lentigines, ECG conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth and sensory neural deafness] syndrome) are collectively named as RASopathies. Clinical presentations are similar, featured with typical facial features, short stature, intellectual disability, ectodermal abnormalities, congenital heart diseases, chest & skeletal deformity and delayed puberty. During past decades, molecular etiologies of RASopathies have been growingly discovered. The functional perturbations of the RAS-mitogen-activated protein kinase pathway are resulted from the mutation of more than 20 genes (PTPN11, SOS1, RAF1, SHOC2, BRAF, KRAS, NRAS, HRAS, MEK1, MEK2, CBL, SOS2, RIT, RRAS, RASA2, SPRY1, LZTR1, MAP3K8, MYST4, A2ML1, RRAS2). The PTPN11 (40–50%), SOS1 (10–20%), RAF1 (3–17%), and RIT1 (5–9%) mutations are common in NS patients. In this review, the constellation of overlapping clinical features of RASopathies will be described based on genotype as well as their differential diagnostic points and management.

Leiomyoma development in Mayer-Rokitansky-Küster-Hauser syndrome: a case report and a narrative review of the literature

The development of leiomyomas on the grounds of an aplastic/hypoplastic uterus in patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) has been rarely described. We report the first case of development of multiple leiomyomas in a patient with MRKHS complicated with pulmonary valve stenosis, and we present a narrative review of the existing literature. A 44-year-old patient with MRKHS attended our clinic because of pelvic pain, which was attributed to a pelvic mass found on ultrasound. Magnetic resonance imaging revealed a multinodular mass, indicating either ovarian pathology or the presence of leiomyomas. Exploratory laparotomy was performed, and multiple solid masses on the grounds of two rudimentary uterine buds were observed. Histological analysis revealed multiple leiomyomas arising from parametrial or paratubal tissue. We searched medical databases for articles relevant to leiomyomas and MRKHS. We present a review of the current literature and summarize the clinical manifestation, diagnosis, management, and histopathological findings of all the cases described. We underline that it is important for gynecologists to be aware of this rare clinical entity, and symptomatic leiomyomas cannot be excluded in patients with MRKHS.

Oral Features in a Child with Noonan Syndrome : A Case Report / 대한소아치과학회지

Noonan syndrome is characterized by distinctive facial features, short stature, and congenital heart disease. It is a congenital genetic disorder with a prevalence of between 1/1,000 and 1/2,500 in both genders.An 11-year-old boy with Noonan syndrome visited the hospital with an ectopically positioned tooth. A pulmonary stenosis was diagnosed and his growth and development were delayed. In many cases of this diseases there is obvious hemostasis, which he was not experiencing. His facial appearance showed characteristic features of Noonan syndrome. The patient showed a dental class II relationship, labioversion of the upper anterior teeth, and a shallow overbite. Radiographic examination revealed that the upper right canine was ectopically positioned, which led to root resorption of the upper right lateral incisor. A lateral cephalometric radiograph revealed a craniofacial pattern that was within normal limits. Surgical opening and button attachment on the impacted upper right canine were performed and traction was applied on the impacted tooth using a removable appliance.This patient was mildly affected by Noonan syndrome and showed some dental problems. However, few studies have reported the oral characteristics of Noonan syndrome despite its high incidence. Thus, this case report describes the oral features and management of Noonan syndrome.

Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center / 소아과

PURPOSE: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. METHODS: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. RESULTS: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1–98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2–31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. CONCLUSION: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.

Considerations for dental treatment of Williams syndrome patients / 대한구강보건학회지

Williams syndrome is a rare congenital disorder with various physical abnormalities and characterized by facial, oral, and dental features. Individuals with Williams syndrome typically have eating disorders in the early childhood, which lead to prolonged night feeding. Prolonged night feeding is a risk factor for rampant dental caries. Williams syndrome is caused by the microdeletion of chromosome 7, resulting in elastin deficiency. Elastin is integral to cardiovascular health. Many patients with Williams syndrome have complex cardiovascular abnormalities that must be considered a part of dental management. Complications related to cardiovascular diseases may induce adverse effects such as dangerously elevated blood pressure. This may occur in patients during stressful dental treatment. In addition, characteristics of auditory hyperalgesia and anxiety disorders among patients with William syndrome, complicate receiving routine dental management. Therefore, dental treatment under sedation or general anesthesia may be preferable for patients with Williams syndrome; in particular, patients who are very uncooperative and/or needs extensive dental treatment. A thorough evaluation of each patient's physical condition is required before making decisions regarding dental treatment. Careful monitoring and preparation for emergencies are very important during and shortly after dental treatment under general anesthesia or sedation. Monitoring is critical until vital signs have stabilized and return to normal. A 28-month-old man diagnosed as having Williams syndrome, visited the Dental Hospital of OO University for the management of rampant dental caries. We reported on the management of this patient who had peripheral pulmonic stenosis, and received dental treatment under general anesthesia. We also reviewed the characteristics of Williams syndrome and discussed considerations for dental treatment under general anesthesia.

Nueva prótesis traqueobronquial metálica autoexpandible. Indicaciones y técnica: A propósito de nuestra experiencia en tres casos / New self-expanding metal tracheobronchial prothesis. Indications and technique. Our experience in tree cases

Si bien hasta el momento los stents más utilizados en vía aérea son los de silicona, desde hace un tiempo se utilizan los stents metálicos. Su indicación principal es la estenosis neoplásica maligna traqueal o bronquial. Hemos tenido la oportunidad de colocar un nuevo tipo de SMA en dos indicaciones distintas a la mencionada, por lo que nos parece interesante la descripción de los casos.

Although the most commonly used stents in the air are silicone, stents have been used for some time. Its main indication is malignant tracheal or bronchial neoplastic stenosis. We have had the opportunity to place a new type of SMA in two different indications to the mentioned one, reason why we consider interesting the description of the cases.

Balloon dilation of congenital supravalvular pulmonic stenosis in a dog

Percutaneous balloon valvuloplasty is considered the standard of care for treatment of valvular pulmonic stenosis, a common congenital defect in dogs. Supravalvular pulmonic stenosis is a rare form of pulmonic stenosis in dogs and standard treatment has not been established. Although, there have been reports of successful treatment of supravalvular pulmonic stenosis with surgical and stenting techniques, there have been no reports of balloon dilation to treat dogs with this condition. Here, a case of supravalvular pulmonic stenosis diagnosed echocardiographically and angiographically in which a significant reduction in pressure gradient was achieved with balloon dilation alone is presented.

Surgical Treatment of Double Outlet Right Ventricle Complicated by Pulmonary Hypertension / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Double outlet right ventricle (DORV) is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension (PH) is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH.</p><p><b>METHODS</b>From June 2004 to November 2016, 61 patients (36 males and 25 females) aged 2 weeks to 26 years (median: 0.67 years and interquartile range: 0.42-1.67 years) with DORV (two great arteries overriding at least 50%) complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure (PASP), pulmonary artery diastolic pressure (PADP), and mean pulmonary artery pressure (mPAP) were measured directly before cardiopulmonary bypass (CPB) was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student's t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice.</p><p><b>RESULTS</b>Fifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7 ± 11.6 mmHg (t = 14.05, P < 0.001), PADP fell from 29.7 ± 12.5 mmHg to 18.6 ± 7.9 mmHg (t = 7.39, P < 0.001), and mPAP fell from 40.3 ± 10.6 mmHg to 25.7 ± 8.3 mmHg (t = 11.85, P < 0.001). Six (9.8%) patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure (PAP) dropped significantly in infant and child patients. Mortality of both infants (13.9%) and adults (33.3%) was high.</p><p><b>CONCLUSIONS</b>PAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect (VSD) type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.</p>