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1.
Adv Exp Med Biol ; 1441: 663-670, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38884740

RESUMEN

d-Transposition of the great arteries (d-TGA) is the most common form of congenital heart disease that presents with cyanosis in a newborn. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It constitutes 3-5% of all congenital heart defects. In a simple d-TGA (about two-thirds of patients), there is no other cardiac abnormality other than a patent foramen ovale (PFO) and a patent ductus arteriosus (PDA). In a complex d-TGA additional cardiac abnormalities such as VSD, pulmonary stenosis or coronary abnormalities are present. About one-third to 40% of patients with d-TGA have an associated ventricular septal defect. Among patients with d-TGA, 6% of those with intact ventricular septum and 31% of those with ventricular septal defect have associated pulmonary stenosis. Coronary abnormalities are of importance with regard to the complexity of surgical repair.


Asunto(s)
Transposición de los Grandes Vasos , Transposición de los Grandes Vasos/cirugía , Transposición de los Grandes Vasos/terapia , Humanos , Recién Nacido , Defectos del Tabique Interventricular/diagnóstico por imagen , Defectos del Tabique Interventricular/terapia , Procedimientos Quirúrgicos Cardíacos/métodos , Estenosis de la Válvula Pulmonar/cirugía , Estenosis de la Válvula Pulmonar/terapia , Estenosis de la Válvula Pulmonar/diagnóstico por imagen
2.
Europace ; 25(4): 1482-1490, 2023 04 15.
Artículo en Inglés | MEDLINE | ID: mdl-36794445

RESUMEN

AIMS: In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB. METHODS AND RESULTS: Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th-75th centiles). Seven CCTGA patients aged 15 (9-17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%). CONCLUSION: Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.


Asunto(s)
Bloqueo Atrioventricular , Transposición de los Grandes Vasos , Humanos , Niño , Transposición Congénitamente Corregida de las Grandes Arterias/complicaciones , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/terapia , Estudios Retrospectivos , Sistema de Conducción Cardíaco , Ventrículos Cardíacos/diagnóstico por imagen , Estimulación Cardíaca Artificial/métodos
3.
Artif Organs ; 45(1): 6-14, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32645759

RESUMEN

Mortality and morbidity of children received veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support after cardiac surgery remain high despite remarkable advances in medical management and devices. The purpose of this study was to describe outcomes and risk factors of applying VA-ECMO in the surgical pediatric population. We retrospectively analyzed 85 consecutive pediatric patients (aged <18 years) who received postcardiotomy VA-ECMO from January 2010 to December 2018. Median (IQR) age at ECMO implantation in this cohort was 12.7 (6.4, 43.2) months, median weight was 8.5 (6.0, 12.8) kg, mean ECMO duration was 143.2 ± 81.6 hours and mean hospital length of stay was 48.4 ± 32.4 days. Seventy-five patients (88.2%) were indicated for postcardiotomy cardiogenic shock. The successful ECMO weaning rate was 70.6% and in-hospital mortality was 52.9%. The most common diagnosis was transposition of great arteries (n = 18, 21.2%), while acute kidney injury occurred most often (n = 64, 75.3%). Multivariate logistic regression analysis showed that thrombocytopenia, hemolysis, and nosocomial infection were positively correlated with in-hospital mortality. Multivariate Cox proportional hazard regression analysis presented that thrombocytopenia significantly increased the 180-day mortality in patients with successful weaning. Therefore, multiple factors had adverse effects on prognosis. Patient selection and procedures from ECMO implantation to weaning need to be closely monitored and performed in a timely manner to improve outcome.


Asunto(s)
Lesión Renal Aguda/mortalidad , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Oxigenación por Membrana Extracorpórea/efectos adversos , Paro Cardíaco/terapia , Complicaciones Posoperatorias/mortalidad , Transposición de los Grandes Vasos/terapia , Lesión Renal Aguda/etiología , Adolescente , Niño , Preescolar , Femenino , Paro Cardíaco/etiología , Paro Cardíaco/mortalidad , Mortalidad Hospitalaria , Humanos , Lactante , Recién Nacido , Tiempo de Internación/estadística & datos numéricos , Masculino , Complicaciones Posoperatorias/etiología , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/mortalidad
4.
Acta Obstet Gynecol Scand ; 99(12): 1728-1735, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32640036

RESUMEN

INTRODUCTION: Newborn infants with transposition of the great arteries (d-TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11-year population-based cohort of d-TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care. MATERIAL AND METHODS: The national cohort consisted of all live-born infants with simple d-TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004-2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated. RESULTS: Preoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but the total mortality rate remained unchanged. All prenatally diagnosed infants (n = 27) survived. Lower gestational age (odds ratio 0.68, P = .012) and higher maternal age at birth (odds ratio 1.16, P = .036) were associated with increased mortality in multivariable analysis. Older infant age at time of operation (P = .002), longer aortic clamp time (P < .001), and higher maternal body mass index (P = .027) were associated with longer initial hospital stay. An extended need for hospital care during the first year of life was multi-factorial. CONCLUSIONS: In our cohort, none of the prenatally diagnosed d-TGA infants died. As a result of the limited prenatal detection rates, however, the sample size was insufficient to reach statistical significance. The d-TGA infants born with lower gestational age and to older mothers had increased mortality.


Asunto(s)
Hospitalización/estadística & datos numéricos , Obesidad Materna , Transposición de los Grandes Vasos , Índice de Masa Corporal , Estudios de Cohortes , Femenino , Finlandia/epidemiología , Edad Gestacional , Humanos , Lactante , Mortalidad Infantil , Recién Nacido , Edad Materna , Tamizaje Neonatal/métodos , Obesidad Materna/diagnóstico , Obesidad Materna/epidemiología , Embarazo , Diagnóstico Prenatal/métodos , Diagnóstico Prenatal/estadística & datos numéricos , Factores de Riesgo , Transposición de los Grandes Vasos/diagnóstico , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/terapia
5.
Artículo en Inglés | MEDLINE | ID: mdl-31027566

RESUMEN

Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.


Asunto(s)
Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/terapia , Adulto , Factores de Edad , Humanos , Transposición de los Grandes Vasos/diagnóstico
6.
Artículo en Inglés | MEDLINE | ID: mdl-31027567

RESUMEN

Despite great advances in caring for patients with congenitally corrected transposition of the great arteries (ccTGA), a high proportion of these patients go on to develop heart failure and death in early adulthood. Adults with congenital heart disease (ACHD) only comprise a small number of patients receiving ventricular assist devices (VAD), but ccTGA accounted for 36% of ACHD patients in the INTERMACS database. Review of the literature describing ccTGA patients receiving VAD therapy shows promising results. With newer devices and the assistance of advanced imaging, mechanical circulatory support is becoming a desirable option for this population of patients and has the potential to provide significant long-term support, relieving them of heart failure symptoms and delaying and perhaps in the future avoiding, the need for cardiac transplantation.


Asunto(s)
Insuficiencia Cardíaca/terapia , Corazón Auxiliar , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/terapia , Disfunción Ventricular Derecha/terapia , Adolescente , Adulto , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Humanos , Masculino , Transposición de los Grandes Vasos/diagnóstico , Disfunción Ventricular Derecha/complicaciones , Disfunción Ventricular Derecha/diagnóstico
7.
Cardiol Young ; 28(9): 1175-1177, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-29991376

RESUMEN

Pulmonary hypertension with transposition of the great arteries is associated with significant morbidity and mortality. At the worst end of the spectrum are patients who undergo extracorporeal support perioperatively. We describe our experience with three patients who received preoperative extracorporeal support and separated from cardiopulmonary bypass successfully on conventional postoperative care, with no significant deficits on follow-up.


Asunto(s)
Oxigenación por Membrana Extracorpórea/métodos , Hipertensión Pulmonar/terapia , Cuidados Preoperatorios/métodos , Transposición de los Grandes Vasos/terapia , Humanos , Hipertensión Pulmonar/congénito , Recién Nacido , Masculino
8.
Fetal Diagn Ther ; 42(1): 48-56, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-27654925

RESUMEN

INTRODUCTION: Neonates with D-transposition of the great arteries (dTGA) may die at birth because of the inadequate intracardiac mixing due to a misdiagnosed restrictive foramen ovale. We reviewed our experience in echocardiographic assessment and perinatal management of fetuses with dTGA searching for new features that may predict the need for urgent balloon atrial septostomy (BAS) immediately after birth. PATIENTS AND METHODS: We included fetuses diagnosed with dTGA between January 2000 and December 2014. We assessed pre- and postnatal appearance of the foramen ovale, ductus arteriosus and pulmonary veins. Both the diagnostic findings at the time of last prenatal echocardiogram and those findings deriving from a retrospective reevaluation of stored videos were considered. BAS was defined as urgent if performed in neonates with restrictive foramen ovale and severe hypoxemia. RESULTS: We reviewed 40 fetuses with dTGA. 20/40 fetuses received urgent BAS at birth. Not only the restrictive but also the hypermobile and the redundant appearance of the foramen ovale was significantly associated with urgent BAS (p < 0.0001, p = 0.002 and p = 0.0001, respectively). CONCLUSIONS: Prenatal evaluation of the foramen ovale appearance in fetuses with dTGA is still challenging. Based on our experience, also the redundant foramen ovale appearance may need urgent BAS at birth.


Asunto(s)
Angioplastia Coronaria con Balón/efectos adversos , Operación de Switch Arterial/efectos adversos , Foramen Oval/diagnóstico por imagen , Complicaciones Posoperatorias/prevención & control , Transposición de los Grandes Vasos/diagnóstico por imagen , Ultrasonografía Prenatal , Terapia Combinada/efectos adversos , Conducto Arterial/diagnóstico por imagen , Conducto Arterial/embriología , Ecocardiografía Doppler en Color , Femenino , Foramen Oval/embriología , Hospitales Pediátricos , Humanos , Hipoxia/etiología , Hipoxia/fisiopatología , Hipoxia/prevención & control , Italia/epidemiología , Masculino , Embarazo , Pronóstico , Venas Pulmonares/diagnóstico por imagen , Venas Pulmonares/embriología , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Transposición de los Grandes Vasos/embriología , Transposición de los Grandes Vasos/epidemiología , Transposición de los Grandes Vasos/terapia , Grabación en Video
9.
Pacing Clin Electrophysiol ; 39(10): 1070-1076, 2016 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-27503213

RESUMEN

BACKGROUND: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure. METHODS: Demographic and clinical data as well as data on device implantation, programming, ICD discharges, and complications after atrial baffle procedure for d-TGA from four German centers were analyzed retrospectively. RESULTS: ICD implantation was undertaken in 33 subjects. ICD implantation was undertaken as primary prevention in 29 (88%) and secondary prevention in four (12%) individuals. There were 21 (64%) subjects with atrial reentrant tachycardia (IART). During a median follow-up of 4.8 years, seven appropriate ICD therapies were delivered in three (10%) individuals with primary prevention indication. No appropriate shocks were documented in subjects with secondary prevention indication. A total of 12 inappropriate ICD discharges occurred in eight (24%) individuals due to IART (n = 6) or lead failure (n = 2). ICD-related complications were noted in seven individuals (21%): lead dislodgement/failure in five (15%) and ICD infection in two subjects (6%). CONCLUSIONS: The majority of individuals received an ICD for primary prevention of SCD, thus representing a liberal attitude of physicians for ICD implantation. During a median follow-up of 4.8 years, the rate of appropriate ICD therapies was low and clearly exceeded by inappropriate ICD discharges. Lead failure and IART were present in >20% of the individuals and were frequent reasons for inappropriate ICD discharges. Facing these results, rigorous treatment of IART and careful ICD programming seems mandatory.


Asunto(s)
Desfibriladores Implantables , Transposición de los Grandes Vasos/terapia , Adulto , Estudios de Cohortes , Desfibriladores Implantables/efectos adversos , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estudios Retrospectivos , Taquicardia por Reentrada en el Nodo Atrioventricular/prevención & control
10.
Cardiol Young ; 26(2): 315-20, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-25732933

RESUMEN

Patients with congenitally corrected transposition frequently benefit from re-synchronisation therapy or ablation procedures. This is likely to require catheterisation of the coronary sinus. Its anatomy, however, is not always appreciated, despite being well-described. With this caveat in mind, we have evaluated its location and structure in hearts with congenitally corrected transposition in order to reinforce the guidance needed by the cardiac interventionist. We dissected and inspected the coronary sinus, the oblique vein of the left atrium, and the left-sided-circumflex venous channel in eight heart specimens with corrected transposition and eight controls, measuring the orifice and length of the sinus and the atrioventricular valves. In two-thirds of the malformed hearts, the sinus deviated from its anticipated course in the atrioventricular groove, ascending obliquely on the left atrial inferior wall to meet the left oblique vein. The maximal deviation coincided in all hearts with the point where the left oblique vein joined the left-sided-circumflex vein to form the coronary sinus. We describe a circumflex vein, rather than the great cardiac vein, as the latter venous channel is right-sided in the setting of corrected transposition. The length of the sinus correlated positively with the diameter of the tricuspid valve (p=0.02). Compared with controls, the left oblique vein in the malformed hearts joined the circumflex venous channel significantly closer to the mouth of the sinus. The unexpected course of the coronary sinus in corrected transposition and the naming of the cardiac veins have important implications for venous cannulation and interpretation of images.


Asunto(s)
Cateterismo Cardíaco , Terapia de Resincronización Cardíaca/métodos , Procedimientos Quirúrgicos Cardíacos/métodos , Seno Coronario/patología , Transposición de los Grandes Vasos/patología , Adolescente , Adulto , Cadáver , Niño , Preescolar , Seno Coronario/diagnóstico por imagen , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Rayos X , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/terapia , Adulto Joven
11.
Circ J ; 79(11): 2367-71, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-26310782

RESUMEN

BACKGROUND: The aim of this study was to investigate the feasibility of static balloon atrial septostomy (BAS) with the double balloon technique for infants. TMP PED balloon catheter, newly designed for static BAS in small children, was used in 3 infants. The balloon catheter has a low profile, short and round shoulder, and smooth deflation without slippage. METHODS AND RESULTS: Three infants (transposition of the great arteries, n=2; pulmonary atresia with intact ventricular septum, n=1) underwent static BAS with double balloon for restrictive interatrial communication between December 2014 and March 2015. Hemodynamic and echocardiographic assessment was done before and after the procedure. Pressure gradient between left and right atrium decreased from 6, 7 and 9 mmHg to 2, 2 and 1 mmHg, respectively. Oxygen saturation in systemic artery increased from 72, 68 and 73% to 78, 70 and 79%, respectively. Maximum defect diameter increased from 3.5, 3.0 and 3.3 mm to 6.6×5.2, 9.0×6.2 and 8.1×5.1 mm, respectively. No complication was recorded. CONCLUSIONS: Static BAS with double balloon technique using the novel TMP PED balloon catheter was safe and effective in producing sufficient interatrial communication for 8-20 weeks in infants. Static BAS is a promising procedure to create interatrial communication in infants.


Asunto(s)
Cateterismo Cardíaco/métodos , Cardiopatías Congénitas/terapia , Atresia Pulmonar/terapia , Transposición de los Grandes Vasos/terapia , Presión Atrial , Biomarcadores/sangre , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Catéteres Cardíacos , Diseño de Equipo , Estudios de Factibilidad , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/fisiopatología , Hemodinámica , Humanos , Lactante , Oxígeno/sangre , Atresia Pulmonar/sangre , Atresia Pulmonar/diagnóstico por imagen , Atresia Pulmonar/fisiopatología , Recuperación de la Función , Transposición de los Grandes Vasos/sangre , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/fisiopatología , Resultado del Tratamiento , Ultrasonografía
12.
Ultrasound Obstet Gynecol ; 45(6): 678-82, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25484180

RESUMEN

OBJECTIVE: To evaluate temporal trends in the prenatal diagnosis of transposition of the great arteries with intact ventricular septum (TGA/IVS) and its impact on neonatal morbidity and mortality. METHODS: We included in this study cohort newborns with TGA/IVS who were referred for surgical management to our center over a 20-year period (1992-2011). The study period was divided into five 4-year periods and the primary outcome was rate of prenatal diagnosis. Secondary outcomes included neonatal preoperative status and perioperative survival. RESULTS: Of the 340 patients with TGA/IVS, 81 (23.8%) had a prenatal diagnosis. The rate of prenatal diagnosis increased over the study period, from 6% in 1992-1995 to 41% in 2008-2011 (P < 0.001). Compared to patients with a postnatal diagnosis, balloon atrial septostomy (BAS) was performed earlier in patients with a prenatal diagnosis (0 days after delivery vs 1 day after delivery, respectively; P < 0.001) and fewer prenatally diagnosed neonates required mechanical ventilation (55.6% vs 68.0%; P = 0.03). Between patients with a prenatal or postnatal diagnosis of TGA/IVS, there were no statistically significant differences in the incidence of preoperative acidosis (16.0% vs 25.5%; P = 0.1), need for preoperative extracorporeal membrane oxygenation (2.5% vs 2.7%; P = 1.0) or mortality (one preoperative and no postoperative deaths among prenatally diagnosed patients compared with four preoperative and six postoperative deaths among postnatally diagnosed patients). CONCLUSIONS: The prenatal detection rate of TGA/IVS has improved but still remains below 50%, suggesting the need for strategies to increase detection rates. The mortality rate was not statistically significantly different between prenatally and postnatally diagnosed patients, however, there were significant preoperative differences with regard to earlier BAS and fewer neonates that required mechanical ventilation. Ongoing work is required to ascertain whether prenatal diagnosis confers long-term benefits.


Asunto(s)
Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/mortalidad , Ultrasonografía Prenatal/tendencias , Adolescente , Adulto , Cateterismo Cardíaco/métodos , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Embarazo , Respiración Artificial , Estudios Retrospectivos , Factores de Tiempo , Transposición de los Grandes Vasos/terapia , Adulto Joven
13.
BMC Pediatr ; 15: 58, 2015 May 16.
Artículo en Inglés | MEDLINE | ID: mdl-25982522

RESUMEN

BACKGROUND: For major congenital heart disease, the benefits of antenatal diagnosis on some post-natal measures have been suggested. However, findings have been inconclusive and focus on short term outcome measures alone with little data from a UK population. Our aim is to describe differences in reported outcomes for patients born with isolated Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries in a UK population, following either antenatal or postnatal diagnosis. METHODS: Retrospective population-based study with case note review covering a 15 year period (1st January 1998 to 31st December 2012) in the British county of Leicestershire. Cases were identified from two local registers: the East Midlands and South Yorkshire Congenital Anomaly Register and a list of surgical patient held by the East Midlands Congenital Heart Centre. RESULTS: In total 52 cases of Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries were identified with 24 (46.2%) diagnosed antenatally. Maximum and minimum follow up was 181 and 16 months respectively. Median follow up was 83 months (IQR: 44-111). The risk of intubation in the postnatal period (OR: 4.64, 95% CI: 1.40 - 15.32) was greater in cases of Hypoplastic Left Heart Syndrome or Transposition of the Great Arteries diagnosed after birth when compared to those diagnosed antenatally. There was a non-significant increase in the risk of metabolic acidosis in the postnatal period (OR: 12.5, 95% CI: 0.64 - 245.46). No differences in mortality or long-term outcomes were demonstrated between antenatally and postnatally diagnosed cohorts. CONCLUSIONS: These results confirm data from American and European populations that, for a British population, an antenatal diagnosis of a major congenital heart disease can have a favourable impact on some postnatal outcome measures. There appears to be no evidence that time of diagnosis impacts on long-term outcome measures.


Asunto(s)
Síndrome del Corazón Izquierdo Hipoplásico/diagnóstico , Diagnóstico Prenatal , Transposición de los Grandes Vasos/diagnóstico , Acidosis/etiología , Puntaje de Apgar , Femenino , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/complicaciones , Síndrome del Corazón Izquierdo Hipoplásico/mortalidad , Síndrome del Corazón Izquierdo Hipoplásico/terapia , Recién Nacido , Intubación Intratraqueal , Masculino , Pronóstico , Respiración Artificial , Estudios Retrospectivos , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/terapia , Reino Unido/epidemiología
14.
Acta Paediatr ; 104(6): 557-65, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25641083

RESUMEN

AIM: This study quantified the contribution of pulse oximetry screening to an early diagnosis of simple transposition. We also estimated the proportion of neonates with this life-threatening cardiac defect who could benefit from prenatal diagnosis, even when pulse oximetry screening had been implemented. METHODS: This population-based, retrospective, cohort study of cases born from 2003 to 2013 used data from surgical files, the Swedish Causes of Death Registry and the National Forensic Medicine database. The main outcome measures were early postnatal circulatory instability, pre-operative death, neurological morbidity and risk of discharge before diagnosis. RESULTS: We identified 89 cases, including three diagnosed prenatally. Hospitals using pulse oximetry screening diagnosed all 31 neonates before discharge, including seven as a result of screening, but 10 of 55 infants in the nonscreening hospitals were discharged undiagnosed (p = 0.017). Prenatal diagnosis could have decreased the risk of early circulatory instability, pre-operative death or neurological morbidity in 12 of the 72 infants born in referring hospitals (17%). There was one pre-operative death and no postoperative deaths at a mean follow-up of 4.7 years. CONCLUSION: Pulse oximetry screening minimised the risk of discharging infants with transposition, but prenatal diagnosis would have been necessary to avoid early circulatory instability in 17% of cases.


Asunto(s)
Tamizaje Neonatal , Oximetría , Transposición de los Grandes Vasos/diagnóstico , Humanos , Recién Nacido , Recien Nacido Prematuro , Diagnóstico Prenatal , Estudios Retrospectivos , Suecia/epidemiología , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/terapia
15.
Pacing Clin Electrophysiol ; 37(5): 546-53, 2014 May.
Artículo en Inglés | MEDLINE | ID: mdl-24313876

RESUMEN

OBJECTIVE: The purpose of this study is to review a series of patients with complex congenital heart disease in whom preprocedural imaging was used to assist placement of cardiac resynchronization therapy (CRT) leads. BACKGROUND: CRT may be beneficial in patients with a failing systemic ventricle and transposition of the great arteries (TGA). However, complex coronary venous anatomy can be challenging for placement of CRT leads. METHODS: Between October 2006 and June 2012, seven patients with either dextro-TGA (d-TGA) or levo-TGA (l-TGA) underwent preprocedural imaging prior to placement of CRT leads (three, d-TGA and four, l-TGA). Three patients underwent cardiac computed tomography (CT) and four underwent coronary angiography, which included levophase imaging of the coronary sinus (CS) or direct contrast injection of the CS. Where CS anatomy was appropriate with drainage into the systemic venous circulation, a transvenous approach was planned. In all other cases, the patient was referred for surgical placement of epicardial leads. RESULTS: Seven patients were identified with either d-TGA or l-TGA who had undergone preprocedural imaging prior to placement of CRT leads (three, d-TGA and four, l-TGA). Three patients underwent cardiac CT and four underwent coronary angiography, which included levophase imaging of the CS or direct contrast injection of the CS. All seven patients had successful CRT lead placement guided by preprocedure imaging. Three patients required surgical placement whereas four were able to undergo transvenous placement. There were no complications. The majority of patients (four of seven) had improvement in New York Heart Association class as well as subjective improvement in exercise tolerance and energy. The majority of patients also had subjective improvement in systemic right ventricular function by echocardiogram and objective improvement in fractional area change of the right ventricle. The follow-up period ranged from 13 months to 55 months with a mean follow-up of 39 months. CONCLUSIONS: Placement of biventricular leads for CRT in patients with l-TGA or d-TGA is feasible. Preprocedural imaging of the CS allows for better assessment of its anatomy and helps determine procedural approach for CRT placement, thereby limiting unnecessary procedures. In the majority of patients, there was subjective improvement in functional status and right ventricular function; in addition, there was objective improvement in echocardiographic parameters of right ventricular function after CRT placement.


Asunto(s)
Dispositivos de Terapia de Resincronización Cardíaca , Angiografía Coronaria/métodos , Electrodos Implantados , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/terapia , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/prevención & control , Adulto , Anciano , Estudios de Factibilidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Cuidados Preoperatorios/métodos , Implantación de Prótesis/métodos , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y Especificidad , Cirugía Asistida por Computador/métodos , Resultado del Tratamiento
16.
Rev Med Liege ; 69(1): 16-25, 2014 Jan.
Artículo en Francés | MEDLINE | ID: mdl-24640304

RESUMEN

The number of patients with Grown-Up Congenital Heart disease (GUCH) consulting adult cardiologists is steadily increasing. These patients have either a non-diagnosed congenital heart disease revealed at adulthood, or a diagnosed congenital heart disease for which one or multiple interventions have possibly been performed during childhood. In this article, we summarize the recommendations of the European Society of Cardiology of 2010 for complex congenital heart disease.


Asunto(s)
Cardiopatías Congénitas/terapia , Adulto , Factores de Edad , Procedimientos Quirúrgicos Cardiovasculares/normas , Procedimientos Quirúrgicos Cardiovasculares/estadística & datos numéricos , Transposición Congénitamente Corregida de las Grandes Arterias , Anomalía de Ebstein/diagnóstico , Anomalía de Ebstein/terapia , Humanos , Tetralogía de Fallot/diagnóstico , Tetralogía de Fallot/terapia , Transposición de los Grandes Vasos/diagnóstico , Transposición de los Grandes Vasos/terapia
17.
J Paediatr Child Health ; 49(1): E68-73, 2013 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23282145

RESUMEN

AIMS: The aim of the study was to examine the prevalence and management of outborn babies with a post-natally confirmed diagnosis of transposition of the great arteries (TGA) requiring transport by the Newborn and Paediatric Emergency Transport Service (NETS), New South Wales during the epoch 1991-2010. METHOD: A retrospective audit of NETS database and case notes. The physiological status, interventions and any complications encountered from the point of referral to NETS (pre-transport), stabilisation (transport) and subsequent admission to the receiving hospital (post-transport) were evaluated. RESULTS: One hundred fifty-seven infants with TGA were transported, with an average of eight per year (1:11,598 births). Seven (4%) had an antenatal diagnosis, and 72 (46%) had a post-natal diagnosis prior to referral. Physiological and clinical parameters demonstrated overall clinical stability; however, 47% of the babies had a PaO(2) <30 mmHg, and approximately one-fifth had oxygen saturations <70%. Rates of mechanical ventilation and prostaglandin E(1) administration were approximately 50%. A quarter of transported babies encountered a transport-related event, including one death and two babies for which a decision was reached to forego life-sustaining treatment at the referring hospital. CONCLUSIONS: Most newborns with TGA remain stable or improve during transport. There is a rate of adverse events; however, this reinforces the need to facilitate delivery where there is ready access to interventional paediatric cardiology services.


Asunto(s)
Transferencia de Pacientes/estadística & datos numéricos , Transporte de Pacientes/estadística & datos numéricos , Transposición de los Grandes Vasos/terapia , Femenino , Humanos , Recién Nacido , Modelos Lineales , Masculino , Auditoría Médica , Nueva Gales del Sur/epidemiología , Embarazo , Diagnóstico Prenatal/estadística & datos numéricos , Prevalencia , Estudios Retrospectivos , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/diagnóstico , Transposición de los Grandes Vasos/epidemiología
18.
Expert Rev Cardiovasc Ther ; 21(6): 389-396, 2023 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-37143366

RESUMEN

INTRODUCTION: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect characterized by atrioventricular and ventriculo-arterial discordance. CcTGA can be diagnosed at any stage of life. The natural history of the disease depends on concomitant anomalies present in most of the cases, progression of systemic ventricular dysfunction and conduction disturbances. AREAS COVERED: This review describes diagnosis of the anomaly and summarizes the current knowledge on etiology and prognosis in ccTGA patients. Furthermore, interventional and pharmacological approaches to ccTGA management are discussed. The areas requiring further research are highlighted. EXPERT OPINION: Although advances in diagnosis and treatment continue to improve outcomes for ccTGA, patients are burdened with significant morbidity and mortality. Optimal approaches to surgical management of the anomaly, as well as prevention and management of heart failure, are still not established. Future research should focus on the long-term effect of anatomic repair, potential benefits of novel pharmacological strategies for heart failure therapy, and the optimal mode of pacing in ccTGA patients. However, the issues might be difficult to address due to rarity of the disease and its heterogenous clinical presentation. As the life-expectancy of ccTGA patients improves, acquired cardiovascular disorders will become another serious concern.


Asunto(s)
Insuficiencia Cardíaca , Transposición de los Grandes Vasos , Femenino , Embarazo , Humanos , Transposición Congénitamente Corregida de las Grandes Arterias/complicaciones , Transposición de los Grandes Vasos/diagnóstico , Transposición de los Grandes Vasos/terapia , Transposición de los Grandes Vasos/complicaciones , Pronóstico , Diagnóstico Prenatal , Insuficiencia Cardíaca/complicaciones
19.
Pacing Clin Electrophysiol ; 35(10): e296-8, 2012 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-21507021

RESUMEN

Congenitally corrected transposition of the great vessels (CCTGV) is a rare congenital heart defect associated with multiple cardiac morphological abnormalities and conduction defects. Complete atrioventricular (AV) block occurs in 30% of patients and it may be present at birth or develop later with a rate of 2% per year; moreover, a systemic right ventricle is frequently characterized by heart failure in adult life. We used a bifocal cardiac stimulation for a young woman affected by CCTGV and atrioventricular Mobitz 2 and 2:1 block, considering structural, anatomic condition, and the high rate of pacing she underwent.


Asunto(s)
Arritmias Cardíacas/terapia , Bloqueo Atrioventricular/terapia , Marcapaso Artificial , Transposición de los Grandes Vasos/terapia , Adulto , Bloqueo Atrioventricular/diagnóstico , Transposición Congénitamente Corregida de las Grandes Arterias , Electrocardiografía , Femenino , Humanos , Resultado del Tratamiento
20.
Turk Kardiyol Dern Ars ; 39(5): 422-6, 2011 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-21743269

RESUMEN

Aiming to increase mixing at the atrial level, atrial septal stenting was performed in two pediatric cases with cyanotic congenital cardiac diseases. The first case was a 3-month-old male infant with transposition of the great arteries. The second case was an 18-month-old male infant with increased central venous pressure due to postoperative right ventricular outflow tract obstruction. Premounted bare stents of 8 mm in diameter were used in both cases. The length of the stent was 20 mm in the first case and 30 mm in the latter. The procedure was accomplished without any complications. In the first case, oxygen saturation increased approximately 20-25% with no significant interatrial gradient. In the latter, central venous pressure decreased from 16 to 8 mmHg immediately after the procedure. The patient was weaned from the ventilator on the second day and discharged from intensive care unit on the fifth day. Follow-up echocardiograms of both patients showed patent stents with good position relative to the atrial septum. Stenting of the atrial septum seems to be a safe and effective method to create a reliable, nonrestrictive interatrial communication.


Asunto(s)
Stents , Transposición de los Grandes Vasos/diagnóstico , Obstrucción del Flujo Ventricular Externo/diagnóstico , Cianosis , Diagnóstico Diferencial , Ecocardiografía Transesofágica , Atrios Cardíacos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/terapia , Humanos , Lactante , Masculino , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/terapia , Obstrucción del Flujo Ventricular Externo/diagnóstico por imagen , Obstrucción del Flujo Ventricular Externo/terapia
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