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2.
Arch. cardiol. Méx ; 90(3): 313-320, Jul.-Sep. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1131049

ABSTRACT

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.


Subject(s)
Humans , Male , Adolescent , Pulmonary Valve Insufficiency/surgery , Pulmonary Valve Stenosis/surgery , Heart Valve Prosthesis Implantation/methods , Prosthesis Design , Pulmonary Valve/surgery , Pulmonary Valve/pathology , Pulmonary Valve Insufficiency/congenital , Pulmonary Valve Stenosis/congenital , Colombia
3.
Arch. cardiol. Méx ; 90(1): 56-58, Jan.-Mar. 2020. graf
Article in English | LILACS | ID: biblio-1131006

ABSTRACT

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.


Subject(s)
Humans , Male , Middle Aged , Pulmonary Artery/pathology , Pulmonary Valve Stenosis/complications , Coronary Stenosis/etiology , Angina Pectoris/etiology , Pulmonary Valve Stenosis/congenital , Coronary Stenosis/complications , Angina Pectoris/surgery
4.
Arch. argent. pediatr ; 117(5): 330-337, oct. 2019. tab
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1054960

ABSTRACT

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.


Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Argentina , Pulmonary Valve Stenosis , Cardiomyopathy, Hypertrophic, Familial , Costello Syndrome , Noonan Syndrome
5.
Bol. méd. postgrado ; 35(1): 35-40, Ene-Jun. 2019. tab
Article in Spanish | LILACS, LIVECS | ID: biblio-1120643

ABSTRACT

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)


Subject(s)
Humans , Male , Female , Aortic Valve Insufficiency , Heart Valve Diseases , Mitral Valve Stenosis , Pulmonary Valve Stenosis , Rheumatic Fever , Cardiovascular Diseases
6.
Rev. colomb. cardiol ; 26(1): 51-51, ene.-feb. 2019. graf
Article in Spanish | LILACS, COLNAL | ID: biblio-1058381

ABSTRACT

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.


Subject(s)
Humans , Female , Infant, Newborn , Pulmonary Valve Stenosis , Stents , Infant, Newborn , Cardiac Surgical Procedures
8.
Article in English | WPRIM | ID: wpr-764512

ABSTRACT

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.


Subject(s)
Congenital Abnormalities , Costello Syndrome , Diagnosis , Ectoderm , Electrocardiography , Genitalia , Genotype , Heart Diseases , Humans , Hypertelorism , Intellectual Disability , Lentigo , Noonan Syndrome , Panthera , Protein Kinases , Puberty, Delayed , Pulmonary Valve Stenosis , Thorax
9.
Article in English | WPRIM | ID: wpr-760643

ABSTRACT

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.


Subject(s)
Adult , Diagnosis , Humans , Laparotomy , Leiomyoma , Magnetic Resonance Imaging , Pathology , Pelvic Pain , Pulmonary Valve Stenosis , Ultrasonography , Uterus
11.
Article in English | WPRIM | ID: wpr-715810

ABSTRACT

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.


Subject(s)
Follow-Up Studies , Heart Septal Defects, Ventricular , Heart Ventricles , Humans , Infant , Infant, Newborn , Methods , Palliative Care , Prognosis , Pulmonary Artery , Pulmonary Atresia , Pulmonary Valve Stenosis , Retrospective Studies , Stents , Tertiary Care Centers , Tetralogy of Fallot
12.
Article in Korean | WPRIM | ID: wpr-787290

ABSTRACT

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.


Subject(s)
Child , Growth and Development , Heart Defects, Congenital , Hemostasis , Humans , Incidence , Incisor , Male , Noonan Syndrome , Overbite , Prevalence , Pulmonary Valve Stenosis , Root Resorption , Tooth , Tooth, Impacted , Traction
13.
Article in Korean | WPRIM | ID: wpr-740583

ABSTRACT

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.


Subject(s)
Anesthesia, General , Anxiety Disorders , Blood Pressure , Cardiovascular Abnormalities , Cardiovascular Diseases , Child, Preschool , Chromosomes, Human, Pair 7 , Congenital, Hereditary, and Neonatal Diseases and Abnormalities , Dental Caries , Eating , Elastin , Emergencies , Humans , Hyperalgesia , Pulmonary Valve Stenosis , Risk Factors , Vital Signs , Williams Syndrome
16.
Rev. am. med. respir ; 17(2): 171-173, jun. 2017. ilus
Article in Spanish | LILACS | ID: biblio-897284

ABSTRACT

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.


Subject(s)
Pulmonary Valve Stenosis , Bronchoscopy
17.
Chinese Medical Journal ; (24): 409-413, 2017.
Article in English | WPRIM | ID: wpr-303138

ABSTRACT

<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>


Subject(s)
Adolescent , Adult , Cardiac Surgical Procedures , Methods , Child , Child, Preschool , Double Outlet Right Ventricle , General Surgery , Female , Heart Septal Defects, Ventricular , General Surgery , Heart Ventricles , General Surgery , Humans , Hypertension, Pulmonary , Infant , Male , Pulmonary Valve Stenosis , General Surgery , Risk Factors , Transposition of Great Vessels , General Surgery , Treatment Outcome , Young Adult
18.
Article in English | WPRIM | ID: wpr-122302

ABSTRACT

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.


Subject(s)
Angiography , Angioplasty, Balloon, Coronary , Animals , Balloon Valvuloplasty , Congenital Abnormalities , Dogs , Pulmonary Valve Stenosis , Standard of Care , Stents
19.
Chinese Medical Journal ; (24): 1069-1073, 2017.
Article in English | WPRIM | ID: wpr-266860

ABSTRACT

<p><b>BACKGROUND</b>Congenital heart disease (CHD) is the most common congenital malformations with high mortality and morbidity. The prevalence of CHD reported previously ranged from 4 per 1000 live births to 50 per 1000 live births. In this cross-sectional study, we aimed to document the prevalence of CHD in Langfang district of Hebei Province, China by analyzing data collected by hospitals located in 11 the counties of the district, as supported by a public health campaign.</p><p><b>METHODS</b>A total of 67,718 consecutive 3-month-old infants were included from July 19, 2012 to July 18, 2014. Structural abnormalities were diagnosed based on echocardiography findings, including two-dimensional and color Doppler echocardiography results.</p><p><b>RESULTS</b>Of the 67,718 infants, 1554 were found to have cardiac structural abnormalities. The total prevalence of CHD was 22.9 per 1000 live births, a value significantly higher than the previously reported prevalence of 8 cases per 1000 live births. The top five most common cardiac abnormalities were as follows: atrial septal defect (ASD, 605 cases, 8.93‰); ventricular septal defect (550 cases, 8.12‰); patent ductus arteriosus (228 cases, 3.37‰); pulmonary stenosis (66 cases, 0.97‰); and tetralogy of Fallot (32 cases, 0.47‰). The CHD prevalence differed by gender in this study ( χ2 = 23.498,P < 0.001), and the majority of ASD cases were females. Regional differences in prevalence were also found ( χ2 = 24.602,P < 0.001); a higher prevalence was found in urban areas (32.2 cases per 1000 live births) than in rural areas (21.1 cases per 1000 live births). There was a significant difference in the prevalence of CHD in preterm versus full-term infants ( χ2 = 133.443,P < 0.001). Prevalence of CHD in infants of maternal aged 35 years or over was significantly higher ( χ2 = 86.917,P < 0.001).</p><p><b>CONCLUSIONS</b>The prevalence of CHD in Langfang district was within the range reported using echocardiography. Echocardiography can be used to early diagnose the CHD.</p>


Subject(s)
China , Cross-Sectional Studies , Ductus Arteriosus , Pathology , Echocardiography , Female , Heart Defects, Congenital , Pathology , Heart Septal Defects, Atrial , Pathology , Humans , Male , Prevalence , Pulmonary Valve Stenosis , Pathology , Tetralogy of Fallot , Pathology
20.
Korean Circulation Journal ; : 786-793, 2017.
Article in English | WPRIM | ID: wpr-78945

ABSTRACT

BACKGROUND AND OBJECTIVES: We investigated the effectiveness of balloon dilatation of homograft conduits in the pulmonary position in delaying surgical replacement. SUBJECTS AND METHODS: We reviewed the medical records of patients who underwent balloon dilatation of their homograft in the pulmonary position from 2001 to 2015. The pressure gradient and ratio of right ventricular pressure were measured before and after the procedure. The primary goal of this study was to evaluate the parameters associated with the interval to next surgical or catheter intervention. RESULTS: Twenty-eight balloon dilations were performed in 26 patients. The median ages of patients with homograft insertion and balloon dilatation were 20.3 months and 4.5 years, respectively. The origins of the homografts were the aorta (53.6%), pulmonary artery (32.1%), and femoral vein (14.3%). The median interval after conduit implantation was 26.7 months. The mean ratio of balloon to graft size was 0.87. The pressure gradient through the homograft and the ratio of right ventricle to aorta pressure were significantly improved after balloon dilatation (p<0.001). There were no adverse events during the procedure with the exception of one case of balloon rupture. The median interval to next intervention was 12.9 months. The median interval of freedom from re-intervention was 16.6 months. Cox proportional hazards analysis revealed that the interval of freedom from re-intervention differed only according to origin of the homograft (p=0.032), with the pulmonary artery having the longest interval of freedom from re-intervention (p=0.043). CONCLUSION: Balloon dilatation of homografts in the pulmonary position can be safely performed, and homografts of the pulmonary artery are associated with a longer interval to re-intervention.


Subject(s)
Allografts , Angioplasty, Balloon , Aorta , Catheters , Dilatation , Femoral Vein , Freedom , Heart Ventricles , Humans , Medical Records , Pulmonary Artery , Pulmonary Valve Stenosis , Rupture , Transplants , Ventricular Pressure
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