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1.
Rev. colomb. cir ; 36(2): 334-337, 20210000. fig
Article in Spanish | LILACS | ID: biblio-1247567

ABSTRACT

El onfalocele o exónfalos se definen como un defecto congénito de la pared abdominal, que consiste en la herniación de las vísceras abdominales a través del anillo umbilical. Esta entidad rara vez se asocia a la comunicación del divertículo de Meckel con el saco del onfalocele. Teniendo en cuenta la escasa prevalencia de dicha entidad, compartimos el reporte de caso de un paciente recién nacido, con diagnóstico de onfalocele menor, en quien se sospechaba ruptura del saco, sin embargo, de manera intraoperatoria se encontró que la aparente ruptura del saco, correspondía a la comunicación con un divertículo de Meckel. El caso además se asoció con hallazgos ecocardiográficos de tetralogía de Fallot


Omphalocele or exomphalos are defined as a congenital defect of the abdominal wall, which consists of the herniation of the abdominal viscera through the umbilical ring. This entity is rarely associated with the communication of Meckel's diverticulum with the omphalocele sac. Considering the low prevalence of this entity, we report the case of a newborn with a diagnosis of minor omphalocele, in whom rupture of the sac was suspected; however, intraoperatively it was found that the apparent rupture of the sac corresponded to a communication with a Meckel's diverticulum. The case was also associated with echocardiographic findings of tetralogy of Fallot


Subject(s)
Humans , Meckel Diverticulum , Sutures , Tetralogy of Fallot , Hernia, Umbilical
2.
Rev. bras. cir. cardiovasc ; 36(1): 39-47, Jan.-Feb. 2021. tab, graf
Article in English | LILACS | ID: biblio-1155795

ABSTRACT

Abstract Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.


Subject(s)
Humans , Infant , Pulmonary Valve/surgery , Pulmonary Valve Insufficiency , Tetralogy of Fallot/surgery , Cardiac Surgical Procedures , Polytetrafluoroethylene , Treatment Outcome
3.
Rev. chil. cardiol ; 39(2): 165-167, ago. 2020. graf
Article in Spanish | LILACS | ID: biblio-1138530

ABSTRACT

Abstract: Right ventricular restrictive physiology (RVRP) occurs in diverse clinical scenarios, most frequently after repair of Tetralogy of Fallot (TOF). Cardiac magnetic resonance (CMR) can comprehensively evaluate RVRP using 4D flow along with anatomical and fibrosis characterization. Also, RVRP is associated with less pulmonary regurgitation and fewer right ventricle enlargement; its long term protective role is debated. RVRP is a challenging and relevant diagnosis, which hallmark is the presence of antegrade pulmonary arterial Flow in late diastole throughout the respiratory cycle. Also, other hemodynamic findings could aid such us flow in; caval veins, suprahepatic, coronary sinus and tricuspid valve. Obtaining all these flow curves is virtually impossible by echocardiography. CMR with 4DF is a unique and powerful technique enabling this comprehensive hemodynamic evaluation as depicted in this case.


Subject(s)
Humans , Magnetic Resonance Imaging , Ventricular Dysfunction, Right/diagnostic imaging , Imaging, Three-Dimensional/methods , Pulmonary Artery/pathology , Regional Blood Flow , Tetralogy of Fallot/complications , Ventricular Dysfunction, Right/etiology , Ventricular Dysfunction, Right/physiopathology , Hemodynamics
6.
Rev. Fac. Cienc. Méd. Univ. Cuenca ; 37(2): 59-65, Septiembre 2019. graf
Article in Spanish | LILACS | ID: biblio-1025114

ABSTRACT

La Tetralogía de Fallot es la enfermedad cardiaca congénita cianótica más común y requiere una corrección quirúrgica temprana; se presenta el caso de una paciente que a los seis meses es sometida a cirugía paliativa de derivación sistémico pulmonar con fistula Blalock Taussing modificada con in-jerto de Goretex, su cuadro no mejora con el paso de los años debiéndose realizar cirugía de corrección total. Al ingreso presenta disnea de medianos esfuerzos, astenia, cianosis peribucal y distal que ceden con el reposo; en tórax presencia de cicatriz infra axilar compatible con cirugía paliativa previa, corazón: R1 y R2 rítmicos, normo fonéticos, presencia de soplo holosistólico en foco pulmonar y soplo infraclavicular izquierdo de fístula sistémico pulmo-nar. En la radiografía de tórax se observa aumento de índice cardiotorácico e hilios vasculares, el ecocardiograma pre quirúrgico transtorácico reporta estenosis infundibular por hipertrofia severa del miocardio, gradiente máximo de 90 mm/Hg. La cirugía de corrección total de Tetralogía de Fallot luego de procedimientos paliativos previos, con control por ecocardiograma transesofágico transoperatorio mejora la calidad de vida del paciente.


Tetralogy of Fallot is the most common cyanotic congenital heart disease and requires early surgical correction, this is a case of a patient who at six months undergoes palliative pulmonary systemic bypass surgery with modified Bla-lock Taussing fistula with Goretex graft, his condition does not improve with the passing of the years and a total correction surgery must be performed.Upon admission, the patient presents: dyspnea of medium efforts, asthenia, peribucal and distal cyanosis that yield with rest; in thorax presence of infra axillary scar compatible with previous palliative surgery, heart: rhythmic R1 and R2, phonetic norm, presence of holosystolic murmur in pulmonary focus and left infraclavicular murmur of pulmonary systemic fistula.The chest radiograph shows an increase in cardiothoracic index and vascu-lar congenital and prominent hilum, transthoracic pre-surgical echocardio-graphy is performed where infundibular stenosis is observed due to severe myocardial hypertrophy, maximum gradient of 90 mm / Hg.Tetralogy of Total Fall correction surgery after previous palliative procedures, with transoperative transesophageal echocardiogram control, improves the patient's quality of life.


Subject(s)
Humans , Female , Child, Preschool , Surgical Procedures, Operative , Tetralogy of Fallot , Blalock-Taussig Procedure , General Surgery , Radiography , Heart Defects, Congenital
7.
Med. infant ; 26(2): 92-98, Junio 2019. tab, ilus
Article in Spanish | LILACS | ID: biblio-1009182

ABSTRACT

Introducción: El síndrome de deleción 22q11.2, también llamado síndrome Velo-Cardio-Facial (VCFS/del22q11.2) o síndrome de DiGeorge, es una entidad causada por una anomalía cromosómica, deleción en la región q11.2 (brazo largo) del cromosoma 22. Se trata de una enfermedad multisistémica de expresión variable que afecta el aparato cardiovascular, la inmunidad, las funciones endocrinológicas, la cavidad oral, el desarrollo neurocognitivo, con una expresión facial particular. La prevalencia estimada es de 1:2000/4000. Objetivos: Identificar y describir las cardiopatías congénitas más frecuentemente asociadas a pacientes con síndrome de microdeleción 22q11.2. Materiales y métodos: Estudio descriptivo, transversal y retrospectivo que analiza los pacientes con diagnóstico de microdeleción 22q11.2 atendidos en el Hospital Garrahan desde Octubre de 1998 hasta Febrero 2018. El criterio diagnóstico fueron signos clínicos compatibles y la presencia de la microdeleción 22q11.2 por técnica de FISH o MLPA. Resultados: Población: 321 pacientes, 151 Femeninos (47%) 170 Masculinos (53%). Rango etario: 0 a 197 meses (1 día a 16,4 años). Mediana de edad al diagnóstico clínico: 31 meses. El 74,4% (239/321) de los pacientes evaluados con microdeleción 22q11.2 tuvieron cardiopatías congénitas asociadas a facies peculiar. Las cardiopatías congénitas más frecuentemente asociadas fueron conotroncales. De los pacientes con cardiopatías congénitas el 68,6% requirió cirugía cardiovascular. Fallecieron 24 pacientes (10%) con cardiopatías congénitas asociadas y en el 93% la causa de muerte estuvo relacionada a la afección cardiológica. Conclusiones: Los pacientes con microdeleción 22q11.2 se asocian con un alto porcentaje de cardiopatías congénitas, la gran mayoría son complejas (conotroncales) y requieren resolución quirúrgica en los primeros años de vida. Es de vital importancia la evaluación multidisciplinaria de este grupo especial de pacientes con cardiopatía asociada a otras alteraciones extra cardíacas para el diagnóstico precoz y tratamiento oportuno (AU)


Introduction: 22q11.2 deletion syndrome, also called velocardiofacial syndrome (VCFS/del22q11.2) or DiGeorge syndrome, is a condition caused by chromosomal abnormality, a deletion in the q11.2 region (long arm) of chromosome 22. VCFS is a multisystem disease of variable expression that affects the cardiovascular, immune, and endocrine systems, the oral cavity, neurocognitive development, and is associated with specific facial features. The estimated prevalence is 1:2000/4000. Objectives: To identify and describe the most common congenital heart defects associated with 22q11.2 micro-deletion syndrome. Materials and methods: Descriptive, cross-sectional, and retrospective study analyzing patients diagnosed with a 22q11.2 microdeletion seen at Garrahan Hospital from October 1998 to February 2018. Diagnostic criteria were compatible clinical signs and the presence of a 22q11.2 microdeletion identified by FISH or MLPA. Results: Population: 321 patients, 151 female (47%) and 170 Male (53%). Age range: 0 to 197 months (1 day to 16.4 years). Median age at clinical diagnosis: 31 months. Overall, 74.4% (239/321) of patients with a 22q11.2 microdeletion had congenital heart defects associated with a peculiar facies. The most commonly associated congenital heart defects were conotruncal. Of the patients with congenital heart defects, 68.6% required cardiovascular surgery. Of the patients with congenital heart defects 24 patients died (10%) and in 93% the cause of death was related to the heart disease (p 0.002). Conclusions: A high percentage of patients with a 22q11.2 microdeletion have congenital heart defects, which are complex (conotruncal) in the majority, requiring surgical treatment in the first years of life. Multidisciplinary evaluation of this special group of patients with heart defects associated with other extracardiac disorders is essential for an early diagnosis and timely treatment (AU)


Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Chromosomes, Human, Pair 22/genetics , Chromosome Deletion , DiGeorge Syndrome/diagnosis , DiGeorge Syndrome/genetics , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/genetics , Tetralogy of Fallot/etiology , Tetralogy of Fallot/genetics , Cross-Sectional Studies , Retrospective Studies , Heart Defects, Congenital/surgery , Heart Septal Defects, Ventricular/etiology , Heart Septal Defects, Ventricular/genetics
8.
Rev. Assoc. Med. Bras. (1992) ; 65(6): 786-790, June 2019. tab, graf
Article in English | LILACS | ID: biblio-1012975

ABSTRACT

SUMMARY OBJECTIVE: This study was to assess the genetic association of copy number variations in two genes (PRKAB2 and PPM1K) located in two regions (tetralogy of Fallot and ventricular septal defect) in a Chinese Han population. METHODS: A total of 200 congenital heart disease patients (100 tetralogy of Fallot patients and 100 ventricular septal defect patients) and 100 congenital heart defect-free controls were recruited, and quantitative real-time PCR analysis was used to replicate the association of two copy number variations with congenital heart defects in a Chinese Han population. RESULTS: One deletion at PRKAB2 and one duplication at PPM1K were found in two of the tetralogy of Fallot patients, respectively; while all these regions were duplicated in both ventricular septal defect patients and in the 100 congenital heart defects-free controls. CONCLUSIONS: We replicated the copy number variations at the disease-candidate genes of PRKAB2 and PPM1K with tetralogy of Fallot in a Chinese Han population, and in patients with ventricular septal defect mutations in these two genes were not found. These results indicate the same molecular population genetics exist in these two genes with different ethnicity. This shows that these two genes are possibly specific pf tetralogy of Fallot candidates.


RESUMO OBJETIVO: Este estudo teve como objetivo avaliar a associação genética do número de cópias em dois genes (PRKAB2 e PPM1K) localizados em duas regiões (tetralogia de Fallot e comunicação interventricular) em uma população chinesa da etnia Han. METODOLOGIA: Um total de 200 pacientes com doença cardíaca congênita (100 pacientes com tetralogia de Fallot e 100 com comunicação interventricular) e 100 indivíduos livres de defeitos cardíacos congênitos foram recrutados, e uma análise quantitativa de PCR em tempo real foi utilizada para replicar a associação de duas variações de número de cópia de defeitos cardíacos congênitos, em uma população chinesa da etnia Han. RESULTADOS: Uma supressão em PRKAB2 e duplicação em PPM1K foram encontradas em dois pacientes com tetralogia de Fallot, respectivamente; todas essas regiões estavam duplicadas nos pacientes com comunicação interventricular e nos 100 indivíduos livres de defeitos cardíacos congênitos. CONCLUSÃO: Nós replicado a variações no número de cópias de genes candidatos de doença PRKAB2 e PPM1K com tetralogia de Fallot em uma população chinesa da etnia Han; em pacientes com comunicação interventricular, não foram encontradas mutações nesses dois genes. Estes resultados indicam que a mesma genética de população molecular existe nestes dois genes em diferentes etnias. Isso mostra que esses dois genes são possivelmente candidatos a genes específicos de tetralogia de Fallot.


Subject(s)
Humans , Tetralogy of Fallot/genetics , Protein Tyrosine Phosphatase, Non-Receptor Type 11/genetics , AMP-Activated Protein Kinases/genetics , DNA Copy Number Variations , Heart Septal Defects, Ventricular/genetics , Reference Values , Case-Control Studies , Genetic Association Studies , Real-Time Polymerase Chain Reaction
9.
Article in English | WPRIM | ID: wpr-719593

ABSTRACT

OBJECTIVE: To assess the accuracy and potential bias of computed tomography (CT) ventricular volumetry using semiautomatic three-dimensional (3D) threshold-based segmentation in repaired tetralogy of Fallot, and to compare them to those of two-dimensional (2D) magnetic resonance imaging (MRI). MATERIALS AND METHODS: This retrospective study evaluated 32 patients with repaired tetralogy of Fallot who had undergone both cardiac CT and MRI within 3 years. For ventricular volumetry, semiautomatic 3D threshold-based segmentation was used in CT, while a manual simplified contouring 2D method was used in MRI. The indexed ventricular volumes were compared between CT and MRI. The indexed ventricular stroke volumes were compared with the indexed arterial stroke volumes measured using phase-contrast MRI. The mean differences and degrees of agreement in the indexed ventricular and stroke volumes were evaluated using Bland-Altman analysis. RESULTS: The indexed end-systolic (ES) volumes showed no significant difference between CT and MRI (p > 0.05), while the indexed end-diastolic (ED) volumes were significantly larger on CT than on MRI (93.6 ± 17.5 mL/m² vs. 87.3 ± 15.5 mL/m² for the left ventricle [p < 0.001] and 177.2 ± 39.5 mL/m² vs. 161.7 ± 33.1 mL/m² for the right ventricle [p < 0.001], respectively). The mean differences between CT and MRI were smaller for the indexed ES volumes (2.0–2.5 mL/m²) than for the indexed ED volumes (6.3–15.5 mL/m²). CT overestimated the stroke volumes by 14–16%. With phase-contrast MRI as a reference, CT (7.2–14.3 mL/m²) showed greater mean differences in the indexed stroke volumes than did MRI (0.8–3.3 mL/m²; p < 0.005). CONCLUSION: Compared to 2D MRI, CT ventricular volumetry using semiautomatic 3D threshold-based segmentation provides comparable ES volumes, but overestimates the ED and stroke volumes in patients with repaired tetralogy of Fallot.


Subject(s)
Bias , Heart Defects, Congenital , Heart Ventricles , Humans , Magnetic Resonance Imaging , Methods , Retrospective Studies , Stroke Volume , Tetralogy of Fallot
10.
Article in English | WPRIM | ID: wpr-765005

ABSTRACT

BACKGROUND: This study aimed to determine the effect of a multidisciplinary approach on the birth rate of fetuses with prenatally diagnosed congenital heart diseases (CHDs). METHODS: Among the fetuses of 724 gravidas who underwent fetal echocardiography in Samsung Medical Center from January 2013 to June 2017, 463 fetuses with normal cardiac structure, arrhythmia or simple left-to-right shunt were excluded, and the remaining 261 were included in the study. The subjects were subdivided into groups based on whether they were consulted multidisciplinarily, that is, consulted simultaneously by pediatric cardiologists, obstetricians and pediatric cardiac surgeons or not. They were also categorized based on the initial fetal echocardiogram results. RESULTS: Among the fetuses in the multidisciplinary group, 64.5% of the fetuses were given birth to, and the proportion was not different from that in the non-multidisciplinary group (68.6%, P = 0.48). The delivery rate in the multidisciplinary consultation group were 69.2% in the transposition of the great arteries group, 63.6% in the tetralogy of Fallot group, 68.8% in the pulmonary atresia or interrupted aortic arch group, 62.5% in the coarctation of aorta group, 60.0% in the atrioventricular septal defect group, 70.0% in the functional single ventricle group, and 55.6% in the hypoplastic left heart syndrome group; there were no significant differences between the 10 echocardiogram groups. However, when the subjects were categorized into Fontan repair group and biventricular repair group, the Fontan repair group showed a significant increase in the likelihood of delivery when a multidisciplinary approach was taken (P = 0.035). CONCLUSION: When a fetus was diagnosed with a CHD where Fontan repair should be considered, a multidisciplinary approach resulted in increased possibility of delivery.


Subject(s)
Aorta, Thoracic , Aortic Coarctation , Arrhythmias, Cardiac , Arteries , Birth Rate , Echocardiography , Fetus , Heart Defects, Congenital , Heart Diseases , Hypoplastic Left Heart Syndrome , Parturition , Prenatal Diagnosis , Pulmonary Atresia , Surgeons , Tetralogy of Fallot
11.
Korean Journal of Radiology ; : 1334-1341, 2019.
Article in English | WPRIM | ID: wpr-760305

ABSTRACT

OBJECTIVE: To evaluate flow pattern characteristics in the ascending aorta (AA) with four-dimensional (4D)-flow MRI and to determine predictors of aortic dilatation late after tetralogy of Fallot (TOF) repair. MATERIALS AND METHODS: This study included 44 patients with repaired TOF (25 males and 19 females; mean age, 28.9 ± 8.4 years) and 11 volunteers (10 males and 1 female, mean age, 33.7 ± 8.8 years) who had undergone 4D-flow MRI. The aortic diameters, velocity, wall shear stress (WSS), flow jet angle (FJA), and flow displacement (FD) at the level of the sinotubular junction (STJ) and mid-AA were compared between the repaired TOF and volunteer groups. The hemodynamic and clinical parameters were also compared between the aortic dilatation and non-dilatation subgroups in the repaired TOF group. RESULTS: The diameters of the sinus of Valsalva, STJ, and AA were significantly higher in the repaired TOF group than in the volunteer group (p = 0.002, p < 0.001, and p = 0.013, respectively). The FJAs at the STJ and AA were significantly greater in the repaired TOF group (p < 0.001 and p = 0.003, respectively), while velocities and WSS parameters were significantly lower. FD showed no statistically significant difference (p = 0.817). In subgroup analysis, age at TOF repair was significantly higher (p = 0.039) and FJA at the level of the AA significantly greater (p = 0.003) and mean WSS were significantly lower (p = 0.039) in the aortic dilatation group. FD were higher in the aortic dilatation group without statistical significance (p = 0.217). CONCLUSION: Patients with repaired TOF have an increased FJA, dilated AA, and secondarily decreased WSS. In addition to known risk factors, flow eccentricity may affect aortic dilatation in patients with repaired TOF.


Subject(s)
Aorta , Dilatation , Female , Hemodynamics , Humans , Magnetic Resonance Imaging , Male , Risk Factors , Sinus of Valsalva , Tetralogy of Fallot , Volunteers
12.
Article in English | WPRIM | ID: wpr-760268

ABSTRACT

OBJECTIVE: To characterize the changes in right ventricular (RV) volume, volume load, and function measured with cardiac computed tomography (CT) over the entire time course of tetralogy of Fallot (TOF). MATERIALS AND METHODS: In 374 patients with TOF, the ventricular volume, ventricular function, and RV volume load were measured with cardiac CT preoperatively (stage 1), after palliative operation (stage 2), after total surgical repair (stage 3), or after pulmonary valve replacement (PVR) (stage 4). The CT-measured variables were compared among the four stages. After total surgical repair, the postoperative duration (POD) and the CT-measured variables were correlated with each other. In addition, the demographic and CT-measured variables in the early postoperative groups were compared with those in the late postoperative and the preoperative group. RESULTS: Significantly different CT-based measures were found between stages 1 and 3 (indexed RV end-diastolic volume [EDV], 63.6 ± 15.2 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed stroke volume (SV) difference, 7.7 ± 10.3 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001), and between stages 2 and 3 (indexed RV EDV, 72.4 ± 19.7 mL/m2 vs. 147.0 ± 38.5 mL/m2 and indexed SV difference, 5.7 ± 13.1 mL/m2 vs. 32.2 ± 16.4 mL/m2; p < 0.001). After PVR, the effect of RV volume load (i.e., indexed SV difference) was reduced from 32.2 mL/m2 to 1.7 mL/m2. Positive (0.2 to 0.8) or negative (−0.2 to −0.4) correlations were found among the CT-based measures except between the RV ejection fraction (EF) and the RV volume load parameters. With increasing POD, an early rapid increase was followed by a slow increase and a plateau in the indexed ventricular volumes and the RV volume load parameters. Compared with the preoperative data, larger ventricular volumes and lower EFs were observed in the early postoperative period. CONCLUSION: Cardiac CT can be used to characterize RV volume, volume load, and function over the entire time course of TOF.


Subject(s)
Humans , Multidetector Computed Tomography , Postoperative Period , Pulmonary Valve , Pulmonary Valve Insufficiency , Stroke Volume , Tetralogy of Fallot , Ventricular Function
13.
Rev. bras. cir. cardiovasc ; 33(5): 505-510, Sept.-Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-977447

ABSTRACT

Abstract Objective: To identify the best time for corrective surgery of tetralogy of Fallot (TF) in children aged 0-12 months and to report the most frequent complications during the first 3 years postoperatively. Methods: Systematic review of studies published between 2000 and 2017 on corrective surgery for TF. Articles were selected through search of electronic databases (PubMed, SciELO, Scopus, Lilacs, Google Scholar, and Cochrane). Length of stay in intensive care unit, duration of mechanical ventilation, and peri/postoperative complications were analyzed for data discussion and research interpretation. Conclusion: Definitive corrective surgery is the best alternative, and the earlier it is performed, the lower the occurrence of harmful effects and the greater the chances of cardiorespiratory recovery. This systematic review suggests that the best time to perform definitive corrective surgery for TF in the first year of life is during 3-6 months of age in children with no or mild symptoms. Children with severe symptoms should undergo surgery immediately.


Subject(s)
Humans , Infant, Newborn , Tetralogy of Fallot/surgery , Postoperative Complications , Time Factors , Risk Factors , Cardiac Surgical Procedures , Length of Stay
14.
West Indian med. j ; 67(3): 274-278, July-Sept. 2018. graf
Article in English | LILACS | ID: biblio-1045846

ABSTRACT

ABSTRACT Potts anastomosis is a central systemic-pulmonary surgical shunt between the descending aorta and the left pulmonary artery, developed and subsequently disbanded in the 1950s to provide pulmonary blood flow in patients with tetralogy of Fallot. Blalock-Taussig shunt is a peripheral systemic pulmonary communication which was varied to make the modified Blalock-Taussig shunt, which is now the standard of surgical care for temporary or permanent blood flow to the right or left pulmonary artery from the subclavian artery. The central shunts were disbanded in the 1950s as early development of pulmonary hypertension and its sequelae were the major prohibitive complications. This is a case report of a patient with tetralogy of Fallot with a rare combination of doubly committed sub-arterial ventricular septal defect, anomalous left anterior descending coronary artery crossing the right ventricular outflow tract and patent ductus arteriosus, who developed pulmonary hypertension within four years of Potts anastomosis and then required cardiac and lung transplantation. The transthoracic echocardiographic images are the focussed feature in this paper, confirming clearly defined structural anatomy in complex structural congenital heart disease.


RESUMEN La anastomosis de Potts - desarrollada y disuelta posteriormente en los años 50 - es una derivación quirúrgica sistémico-pulmonar central entre la aorta descendente y la arteria pulmonar izquierda, cuyo fin es proporcionar flujo de sangre pulmonar en pacientes con la tetralogía de Fallot. La derivación de Blalock-Taussig es una comunicación pulmonar sistémica periférica. Este procedimiento sufrió cambios que condujeron a la derivación modificada de Blalock-Taussig, que es ahora el procedimiento quirúrgico estándar para tratar el flujo de sangre temporal o permanente hacia la arteria pulmonar derecha o izquierda desde la arteria subclavia. Las derivaciones centrales fueron disueltas en los años 50, cuando el desarrollo temprano de la hipertensión pulmonar y sus secuelas eran las complicaciones prohibitivas principales. Éste es un reporte de caso de un paciente con la tetralogía de Fallot con una rara combinación de defecto septal con compromiso doble subarterial ventricular, arteria coronaria descendente anterior izquierda anómala a través del tracto de salida ventricular derecho, y conducto arterioso persistente. El paciente desarrollo hipertensión pulmonar a los cuatro años de una anastomosis de Potts, y requirió entonces trasplante cardíaco y pulmonar. Las imágenes ecocardiografias transtorácicas constituyen el aspecto central de este trabajo, que confirma la anatomía estructural claramente definida de la enfermedad cardíaca congénita, estructuralmente compleja.


Subject(s)
Humans , Male , Adolescent , Tetralogy of Fallot/surgery , Anastomosis, Surgical/methods , Ductus Arteriosus, Patent/diagnostic imaging , Tetralogy of Fallot/diagnostic imaging , Heart-Lung Transplantation , Hypertension, Pulmonary
15.
Rev. bras. cir. cardiovasc ; 33(4): 418-423, July-Aug. 2018. tab, graf
Article in English | LILACS, SES-SP, SESSP-IDPCPROD, SES-SP | ID: biblio-958433

ABSTRACT

Abstract Tetralogy of Fallot (ToF) is one of the most prevalent congenital heart disease. Its surgical corrections may haemodinamically correct a disease, but the incisions may create scars that will originate ventricular arrhythmias. Even though life threatening arrhythmias are not common, some patients present unstable ventricular tachycardia (VT) of ectopic ventricular beats triggering heart failure and symptoms. We describe the treatment of a 16-years-old woman with late ToF repair and drug refractory Implantable cardioverter defibrillator (ICD) shocks. The patient underwent successful ablation of VT using X-ray and anatomic landmarks without the use of electroanatomical mapping. We were able to reduce drugs after one month of ablation and improve quality of life and symptoms. In this paper we describe the indications and perform a brief review of the key points for successful radiofrequency catheter ablation of VT in ToF patients.


Subject(s)
Humans , Female , Adolescent , Tetralogy of Fallot/surgery , Tachycardia, Ventricular/surgery , Catheter Ablation/methods , Tetralogy of Fallot/diagnostic imaging , Radiography , Treatment Outcome , Tachycardia, Ventricular/diagnostic imaging , Defibrillators, Implantable , Electrocardiography , Anatomic Landmarks , Medical Illustration
17.
Article in English | WPRIM | ID: wpr-718235

ABSTRACT

PURPOSE: This study aimed to analyze changes in QRS duration and cardiothoracic ratio (CTR) following pulmonary valve replacement (PVR) in patients with tetralogy of Fallot (TOF). METHODS: Children and adolescents who had previously undergone total repair for TOF (n=67; median age, 16 years) who required elective PVR for pulmonary regurgitation and/or right ventricular out tract obstruction were included in this study. The QRS duration and CTR were measured pre- and postoperatively and postoperative changes were evaluated. RESULTS: Following PVR, the CTR significantly decreased (pre-PVR 57.2%±6.2%, post-PVR 53.8%±5.5%, P=0.002). The postoperative QRS duration showed a tendency to decrease (pre-PVR 162.7±26.4 msec, post-PVR 156.4±24.4 msec, P=0.124). QRS duration was greater than 180 msec in 6 patients prior to PVR. Of these, 5 patients showed a decrease in QRS duration following PVR; QRS duration was less than 180 msec in 2 patients, and QRS duration remained greater than 180 msec in 3 patients, including 2 patients with diffuse postoperative right ventricular outflow tract hypokinesis. Six patients had coexisting arrhythmias before PVR; 2 patients, atrial tachycardia; 3 patients, premature ventricular contraction; and 1 patient, premature atrial contraction. None of the patients presented with arrhythmia following PVR. CONCLUSION: The CTR and QRS duration reduced following PVR. However, QRS duration may not decrease below 180 msec after PVR, particularly in patients with right ventricular outflow tract hypokinesis. The CTR and ECG may provide additional clinical information on changes in right ventricular volume and/or pressure in these patients.


Subject(s)
Adolescent , Arrhythmias, Cardiac , Atrial Premature Complexes , Child , Electrocardiography , Heart Valve Prosthesis Implantation , Humans , Pulmonary Valve Insufficiency , Pulmonary Valve , Tachycardia , Tetralogy of Fallot , Ventricular Premature Complexes
18.
Article in English | WPRIM | ID: wpr-717303

ABSTRACT

Pulmonary artery sling is a rare congenital cardiac anomaly, in which the left pulmonary artery originates from the right pulmonary artery and courses leftward between the trachea and the esophagus. Tetralogy of Fallot associated with pulmonary artery sling is even rarer, and only a few cases have been reported in the literature. We present a case of tetralogy of Fallot associated with pulmonary artery sling that was repaired successfully.


Subject(s)
Esophagus , Pulmonary Artery , Tetralogy of Fallot , Trachea
19.
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
20.
Insuf. card ; 12(2): 91-95, jun. 2017. ilus
Article in Spanish | LILACS | ID: biblio-892767

ABSTRACT

La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente del adulto. El síndrome de válvula pulmonar ausente constituye una variante poco frecuente, que representa del 3% al 6% de los pacientes con tetralogía de Fallot. Presentamos el caso de un paciente masculino de 29 años de edad, con tetralogía de Fallot y síndrome de válvula pulmonar ausente, los hallazgos del examen físico y los principales exámenes complementarios; como así también su evolución y una revisión de la literatura.


The tetralogy of Fallot is the most frequent cyanotic congenital heart disease in adult. Absent pulmonary valve syndrome is a rare variant, representing 3% to 6% of patients with tetralogy of Fallot. We present the case of a 29-year-old male patient with tetralogy of Fallot and absent pulmonary valve syndrome, physical examination findings and major complementary examinations; as well as its evolution and a review of the literature.


A tetralogia de Fallot é a doença congênita cianótica mais comum cardíacas em adultos. A síndrome valva pulmonar ausente é uma variante rara, que representa de 3% a 6% dos pacientes com tetralogia de Fallot. Apresentamos o caso de um paciente masculino de 29 anos de idade, com tetralogia de Fallot e síndrome da valva pulmonar ausente, exame físico e os principais estudos complementares; bem como a sua evolução e uma revisão da literatura.


Subject(s)
Humans , Pulmonary Valve , Tetralogy of Fallot , Heart Defects, Congenital
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