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1.
Acta Chir Belg ; 114(2): 92-8, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25073205

RESUMEN

BACKGROUND: Arterial switch operation became the golden treatment for simple transposition of the great arteries (sTGA). We describe our experience with the arterial switch operation regarding long-term outcome and the need for re-intervention. Nevertheless, supravalvular pulmonary stenosis (SPS) remains a concern in the long run. We assess the evolution of SPS over time and evaluate the effect of technical modifications on SPS during our experience. METHODS: We performed a retrospective study on 133 patients operated with ASO for TGA between October 1991 and November 2009. Last report method was used. We reviewed our pediatric cardiology and cardiac surgery database to examine the echocardiographic data and electrocardiograms. A mean follow-up of 9.2 years (+/- 5.83 SD) was reached. RESULTS: One (0.8%) patient deceased postoperatively due to cardiogenic shock. The overall actuarial freedom from reoperation (open and percutaneous) was 88.1%, 78.5% and 76.9% at 1, 5 and 10 years. SPS needed to be treated in 17 patients. Valve regurgitation at final investigation was maximal moderate in 5 patients for the aortic valve, 10 for pulmonary valve and 3 in tricuspid valve. CONCLUSIONS: ASO shows excellent long-term results in sTGA with a very low morbidity and mortality and is therefore the procedure of choice. Re-intervention rate is determined by SPS. Since the extensive mobilization of the pulmonary arteries and the creation of a longer neo-pulmonary root, reduction in SPS was seen with no re-interventions in the second half of the group. To obtain a final comparison with the atrial switch operation, a longer Follow-up is necessary.


Asunto(s)
Complicaciones Posoperatorias , Estenosis Subvalvular Pulmonar/epidemiología , Transposición de los Grandes Vasos/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Estenosis Subvalvular Pulmonar/diagnóstico , Reoperación , Estudios Retrospectivos , Tasa de Supervivencia , Factores de Tiempo , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/mortalidad , Resultado del Tratamiento
2.
Int J Cardiol ; 323: 40-46, 2021 01 15.
Artículo en Inglés | MEDLINE | ID: mdl-32860844

RESUMEN

AIMS: Percutaneous pulmonary valve implantation (PPVI) has proven good hemodynamic results. As infective endocarditis (IE) remains a potential complication with limited available clinical data, we reviewed our patient records to improve future strategies of IE prevention, diagnosis and treatment. METHODS: Medical records of all patients diagnosed with Melody® valve IE according to the modified Duke criteria were retrospectively analyzed in three Belgian tertiary centers. RESULTS: 23 IE episodes in 22 out of 240 patients were identified (incidence 2.4% / patient year) with a clear male predominance (86%). Median age at IE was 17.9 years (range 8.2-45.9 years) and median time from PPVI to IE was 2.4 years (range 0.7-8 years). Streptococcal species caused 10 infections (43%), followed by Staphylococcus aureus (n = 5, 22%). In 13/23 IE episodes a possible entry-point was identified (57%). IE was classified as definite in 15 (65%) and as possible in 8 (35%) cases due to limitations of imaging. Echocardiography visualized vegetations in only 10 patients. PET-CT showed positive FDG signals in 5/7 patients (71%) and intracardiac echocardiography a vegetation in 1/1 patient (100%). Eleven cases (48%) had a hemodynamically relevant pulmonary stenosis at IE presentation. Nine early and 6 late percutaneous or surgical re-interventions were performed. No IE related deaths occurred. CONCLUSIONS: IE after Melody® valve PPVI is associated with a relevant need of re-interventions. Communication to patients and physicians about risk factors is essential in prevention. The modified Duke criteria underperformed in diagnosing definite IE, but inclusion of new imaging modalities might improve diagnostic performance.


Asunto(s)
Endocarditis Bacteriana , Endocarditis , Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Válvula Pulmonar , Adolescente , Adulto , Niño , Endocarditis/diagnóstico por imagen , Endocarditis/epidemiología , Endocarditis Bacteriana/diagnóstico por imagen , Endocarditis Bacteriana/epidemiología , Prótesis Valvulares Cardíacas/efectos adversos , Humanos , Venas Yugulares , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Estudios Retrospectivos , Stents , Resultado del Tratamiento , Adulto Joven
3.
Catheter Cardiovasc Interv ; 74(1): 137-42, 2009 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-19405156

RESUMEN

OBJECTIVE: Interventional targets may be virtually "excluded" due to vascular access problems or complex previous surgical procedures. This study reviews our experience using transapical ventricular puncture to gain direct access to the systemic ventricle. PATIENTS: Patient 1 (74 years, 2 previous sternotomies), patient 2 (66 years, 5 previous sternotomies), and patient 5 (69 years, 3 previous sternotomies) with prosthetic valves had paravalvular mitral valve leaks. Patient 3 (6.3 years, 2 previous sternotomies) with an extracardiac Fontan conduit, had a significant residual leak after two previous surgical attempts of patch closure of a severely regurgitant right atrioventricular valve. Patient 4 (10 months) had failure of standard ablation of the posteroseptal region of the mitral valve with persistent life-threatening episodes of ventricular tachycardia. METHODS: Procedures were performed under general anesthesia. Entry site was percutaneous in three patients and in two (and one conversion) a mini-thoracotomy was used. Sheaths were placed (6 F) using standard Seldinger technique, followed by the procedure as required. Direct surgical closure of the puncture site was done in 4 patients and in patient 3, a percutaneous vascular occlusion device was used. RESULTS: Easy and immediate access was obtained in all patients. The paravalvular leaks were crossed within seconds and completely closed with Amplatzer occluders. In patient 3 the valve was crossed using a Brokenbrough needle and a 12-mm Amplatzer device was placed in the patch leak. Patient 4 was successfully ablated using a 7-F irrigated catheter endo- and epicardially. Complications were in the percutaneous puncture group: in one patient a coronary artery was punctured and in one a hemothorax developed. CONCLUSION: Direct left ventricular puncture offers a very useful alternative access site in selected patients to reach "inaccessible" targets for certain percutaneous interventions in patients where standard approaches may be impossible or difficult.


Asunto(s)
Cateterismo Cardíaco/métodos , Procedimiento de Fontan , Cardiopatías Congénitas/terapia , Implantación de Prótesis de Válvulas Cardíacas , Válvula Mitral/cirugía , Anciano , Cateterismo Cardíaco/efectos adversos , Cateterismo Cardíaco/instrumentación , Ablación por Catéter , Niño , Femenino , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Implantación de Prótesis de Válvulas Cardíacas/efectos adversos , Ventrículos Cardíacos , Técnicas Hemostáticas/instrumentación , Humanos , Lactante , Masculino , Válvula Mitral/diagnóstico por imagen , Falla de Prótesis , Punciones , Radiografía Intervencional , Esternón/cirugía , Toracotomía , Resultado del Tratamiento
4.
Am J Cardiol ; 85(2): 221-5, 2000 Jan 15.
Artículo en Inglés | MEDLINE | ID: mdl-10955381

RESUMEN

Surgical repair of tetralogy of Fallot (TOF) with reconstruction of the right ventricular (RV) outflow tract invariably results in pulmonary regurgitation. Chronic pulmonary regurgitation has been associated with RV dysfunction and decreased exercise performance. The present study assessed the influence of pulmonary valve replacement (PVR) for severe pulmonary regurgitation after previous TOF repair on cardiorespiratory exercise performance and RV function. Eighteen patients, between the ages of 8 and 18 years, underwent an exercise test and a cardiac magnetic resonance imaging scan at least 1 year after PVR. The exercise data were compared with those obtained from 24 age-matched normal controls and 27 age-matched patients with repaired TOF and a moderate degree of pulmonary regurgitation. A subgroup of 11 patients had an exercise test performed before and after PVR. Cardiopulmonary exercise performance was evaluated by determination of the ventilatory anaerobic threshold (VAT) and by the steepness of the slope of oxygen uptake versus exercise intensity (SVO2). After PVR there was a significant increase in VAT (86+/-11% before to 106.9+/-14% after, p = 0.03) and in SVO2 (1.71+/-0.47 to 2.3+/-0.39, p = 0.004). In patients examined after PVR, the VAT and SVO2 values were not significantly different from the values in the normal controls (104+/-15% [p>0.05] and 2.03+/-0.77 after PVR vs. 2.42+/-0.68 [p>0.25], respectively). In contrast, patients with repaired TOF and a moderate degree of pulmonary regurgitation had a significantly lower VAT (86+/-11%, p<0.05) and SVO2 (1.8+/-0.74 vs. 2.42+/-0.68, p<0.05) than normal controls. Magnetic resonance imaging studies revealed residual RV dilatation and dysfunction. However, there was no correlation between RV dilatation and RV dysfunction and aerobic exercise capacity. It is concluded that aerobic exercise capacity substantially improves after PVR for severe pulmonary regurgitation after previous TOF repair. Although the right ventricle remains significantly dilated and hypocontractile, there is no relation between RV function and exercise performance.


Asunto(s)
Ejercicio Físico/fisiología , Complicaciones Posoperatorias/cirugía , Insuficiencia de la Válvula Pulmonar/cirugía , Válvula Pulmonar/trasplante , Tetralogía de Fallot/cirugía , Adolescente , Umbral Anaerobio , Fenómenos Fisiológicos Cardiovasculares , Niño , Prueba de Esfuerzo , Humanos , Imagen por Resonancia Magnética , Oxígeno/metabolismo , Respiración , Índice de Severidad de la Enfermedad , Función Ventricular , Función Ventricular Derecha
5.
Am J Med Genet ; 74(5): 515-20, 1997 Sep 19.
Artículo en Inglés | MEDLINE | ID: mdl-9342203

RESUMEN

We report the clinical findings in 5 patients with a terminal deletion of the short arm of chromosome 8. Mild developmental delay was constantly present, in association with microcephaly in 4 of 5 patients. Facial anomalies were mild or absent. A congenital heart defect was present in 3 patients: an atrioventricular septal defect (AVSD) in 2 and an atrial septal defect type II (ASDII) with pulmonary stenosis in one. A highly similar pattern of behavioural difficulties was present in the 3 older children (8-11 years), with outbursts of aggressiveness and destructive behaviour. Follow-up in one patient showed that at the age of 16 years, these behavioural problems had largely disappeared. This observation suggests that in addition to mental retardation, microcephaly, congenital heart defect (typically AVSD), a terminal deletion of chromosome 8p may be associated with a characteristic behavioural phenotype during childhood.


Asunto(s)
Conducta Infantil , Aberraciones Cromosómicas/genética , Deleción Cromosómica , Cromosomas Humanos Par 8 , Fenotipo , Síntomas Conductuales/genética , Niño , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/genética , Cardiopatías Congénitas/psicología , Humanos , Lactante , Recién Nacido , Discapacidad Intelectual/genética , Discapacidad Intelectual/psicología , Masculino , Microcefalia/genética , Microcefalia/psicología
6.
Am J Med Genet ; 35(4): 532-5, 1990 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-2333885

RESUMEN

We report on a boy with Alagille syndrome. Chromosome analysis on a peripheral blood lymphocyte culture showed a de novo deletion of the short arm of chromosome 20 with a 46,XY,del(20)(p11.2) chromosome constitution. This is the second report of a del(20p) in a patient with Alagille syndrome. The possible localisation of this autosomal dominant syndrome on 20p is discussed.


Asunto(s)
Anomalías Múltiples/diagnóstico , Deleción Cromosómica , Cromosomas Humanos Par 20 , Colestasis , Cara/anomalías , Insuficiencia de Crecimiento , Humanos , Recién Nacido , Linfocitos/citología , Masculino , Estenosis de la Válvula Pulmonar , Columna Vertebral/anomalías , Síndrome
7.
Am J Med Genet ; 103(1): 44-7, 2001 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-11562933

RESUMEN

We present a patient with heterotaxy and a de novo, apparently balanced reciprocal translocation with breakpoints at 6q21 and 20p13. Another patient with heterotaxy was previously reported with a de novo balanced translocation involving chromosome band 6q21. The breakpoints in both patients on 6q21 were found to be located in the same chromosomal region spanning maximally 2 Mb. We speculate that the two breakpoints lead to the disruption of the function of a single gene, either directly or through long distance effects. Alternatively, the present observation suggests additional heterogeneity in heterotaxy in humans.


Asunto(s)
Anomalías Múltiples/genética , Cromosomas Humanos Par 6/genética , Anomalías Múltiples/patología , Cromosomas Humanos Par 20/genética , Femenino , Feto , Defectos de los Tabiques Cardíacos/patología , Humanos , Hibridación Fluorescente in Situ , Lactante , Recién Nacido , Cariotipificación , Situs Inversus/patología , Translocación Genética
8.
Heart ; 79(1): 24-8, 1998 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-9505914

RESUMEN

BACKGROUND: The natural history of major aortopulmonary collateral arteries (MAPCAs) in patients with pulmonary atresia and ventricular septal defect (PA-VSD) is frequently complicated by progressive stenosis, leading to pulmonary hypoperfusion and debilitating hypoxaemia. OBJECTIVE: To evaluate balloon dilatation and stenting for relief of stenoses and improvement of pulmonary flow in patients with PA-VSD. DESIGN: Retrospective analysis of all patients where dilatation of MAPCA stenoses was attempted. PATIENTS: Twelve patients with stenotic MAPCAs. INTERVENTIONS: Dilatation was attempted in 25 stenoses. Vessels were stented if elastic recoil was noticed (n = 3), in the presence of long segment stenosis (n = 4) or marked tortuosity (n = 1). MAIN OUTCOME MEASURES: Diameter of stenoses before and after dilatation as well as arterial oxygen saturation data. Patients proceeding to surgical therapy. RESULTS: Two stenosed MAPCAs could not be crossed by a catheter. Four lesions were non-dilatable despite the use of high inflation pressures (18 atm). Six stenoses could be completely dilatated using angioplasty only; in five, only partial dilatation was obtained; eight stenoses needed stenting. In the group with partial expansion the mean (SD) diameter increased from 1.7 (0.8) to 3.5 (1.7) mm (p < 0.05); where full dilatation was achieved it increased from 2.1 (0.8) to 4.8 (1.9) mm (p < 0.05); and in the stented group in increased from 2.3 (0.9) to 5.0 (2.5) mm (p < 0.01). Percutaneous arterial oxygen saturation increased from 75(8%) to 82(8)% (p < 0.001). No complications were experienced during the procedures. Repeat dilatation was attempted in six stenoses, but only two procedures were successful. There were two episodes of vasospasm and in one an aneurysm had developed after redilatation. Two patients proceeded to outflow plasty and two subsequently had a unifocalisation procedure. CONCLUSIONS: Pulmonary blood flow can be improved using balloon angioplasty or stents in patients with stenotic MAPCA; however, 17% of the lesions were not dilatable. Procedures are generally safe, but carry a small risk of vasospasm, dissection, occlusion or aneurysm formation.


Asunto(s)
Angioplastia de Balón/métodos , Aorta , Válvula Aórtica/anomalías , Arteriopatías Oclusivas/terapia , Circulación Colateral , Defectos del Tabique Interventricular/complicaciones , Arteria Pulmonar , Stents , Adolescente , Adulto , Aortografía , Arteriopatías Oclusivas/cirugía , Niño , Preescolar , Terapia Combinada , Estudios de Seguimiento , Humanos , Lactante , Estudios Retrospectivos , Resultado del Tratamiento
9.
Heart ; 77(3): 288-9, 1997 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-9093054

RESUMEN

A six year old boy with an epicardial pacing lead since infancy presented with thoracic pain triggered by movement. The pain was thought to be musculoskeletal, but two weeks later he collapsed and died in ventricular fibrillation. Necropsy showed strangulation of the ventricular apex by the epicardial lead.


Asunto(s)
Muerte Súbita Cardíaca/etiología , Lesiones Cardíacas/etiología , Isquemia Miocárdica/etiología , Marcapaso Artificial/efectos adversos , Niño , Corazón/crecimiento & desarrollo , Bloqueo Cardíaco/terapia , Lesiones Cardíacas/patología , Defectos del Tabique Interventricular/cirugía , Defectos del Tabique Interventricular/terapia , Humanos , Masculino , Isquemia Miocárdica/patología , Factores de Tiempo
10.
Heart ; 79(3): 230-3, 1998 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-9602654

RESUMEN

OBJECTIVE: Obstruction of the venous pathways after Mustard repair for transposition of the great arteries is associated with an increased risk of arrhythmia and sudden death. The purpose of this study was to assess the effectiveness of the largest (tracheal 22 x 40 mm) Wallstents in treating baffle obstructions. DESIGN: Retrospective analysis of patients with stented venous pathways. SUBJECTS: Eleven patients with baffle obstruction after Mustard repair for transposition of the great arteries. INTERVENTIONS: Stenoses were dilated with an 18 or 20 mm balloon. However, recoil was noticed in 11 patients: immediately (n = 7) or on repeat angiography (n = 4). Eighteen stents were implanted (mean (SD)) 18 (3.3) years postoperatively. After dilatation a tracheal Wallstent (11.5 F) was deployed. MAIN OUTCOME MEASURES: Relief of obstruction, haemodynamic improvement. RESULTS: In the inferior vena cava, 10 stents were deployed in seven baffle obstructions with an increase in diameter from 9.8 (2.4) mm to 16.5 (1.4) mm (p < 0.01) and a mean (SD) pressure gradient decrease from 5.1 (3.6) mm Hg to 1.4 (2.0) mm Hg; in the superior vena cava, eight stents were implanted increasing the diameter from 9.1 (3.7) mm to 15.6 (3.8) mm (p < 0.001) with a decrease in mean pressure gradient from 5.1 (2.7) mm Hg to 1.9 (1.5) mm Hg. No complications were experienced during implantation. No anticoagulation was prescribed. During follow up (1.7 (0.6) years; range, 0.9-2.6) no problems were noted; five patients were re-catheterised without change in measurements. There was no evidence of peal formation in any of the stents. CONCLUSION: It is concluded that Wallstents are safe, easy to use, and effective in relieving baffle obstruction. Anticoagulation does not seem necessary.


Asunto(s)
Enfermedades Vasculares Periféricas/cirugía , Complicaciones Posoperatorias/cirugía , Stents , Transposición de los Grandes Vasos/cirugía , Adolescente , Adulto , Cateterismo , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Enfermedades Vasculares Periféricas/diagnóstico por imagen , Radiografía , Estudios Retrospectivos , Transposición de los Grandes Vasos/diagnóstico por imagen , Resultado del Tratamiento , Vena Cava Inferior/diagnóstico por imagen
11.
Eur J Cardiothorac Surg ; 13(6): 673-7, 1998 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-9686799

RESUMEN

METHODS: Fifty patients underwent a Ross operation between June 1991 and October 1996. Preoperative diagnosis was: congenital aortic valve disease (31 patients), complex left ventricular outflow tract (LVOT) obstruction (11 patients), outgrowth of a small aortic valve prosthesis (five patients) and valve endocarditis (three patients). Half of the interventions were reoperations. All operations were root replacements. A pulmonary homograft was used in 45 patients. An aorticoventriculoplasty was combined with the root replacement in the 16 patients with LVOT obstruction and a too small aortic valve prosthesis. An enlargement or reduction plasty of the ascending aorta was necessary in seven patients. RESULTS: The mean age was 20.9 years (range: 2.5-54 years). The mean follow up was 34.2 +/- 21 months and was 94% complete. Two patients died after 8 days (low cardiac output due to myocardial fibrosis) and 17.4 months (sudden death), respectively, resulting in a survival of 95 +/- 4% at 4 years. Those two deaths occurred in the group of patients undergoing Ross procedure and aorticoventriculoplasty. Two autografts were replaced after respectively 2 days (technical failure) and 44 months (progressive root dilatation) resulting in a reoperation-free incidence of 93 +/- 6% at 4 years. Other postoperative major complications occurred in six patients. All survivors had regular echo-Doppler examination. All autografts except one had a systolic gradient below 10 mmHg at the last examination. Thirty-four autografts had no leak, ten showed grade 1-2 regurgitation. Two patients showed a higher than grade 3 regurgitation: one leak remains stable with normal left ventricular dimensions and function, one autograft was replaced by a mechanical valve. CONCLUSION: This experience demonstrates that the medium-term results of the Ross procedure are excellent even in complex LVOT obstructions.


Asunto(s)
Válvula Aórtica/cirugía , Válvula Pulmonar/trasplante , Obstrucción del Flujo Ventricular Externo/cirugía , Adolescente , Adulto , Niño , Preescolar , Endocarditis Bacteriana/cirugía , Enfermedades de las Válvulas Cardíacas/cirugía , Prótesis Valvulares Cardíacas , Humanos , Lactante , Persona de Mediana Edad , Complicaciones Posoperatorias , Estudios Retrospectivos , Trasplante Autólogo , Resultado del Tratamiento
12.
Eur J Cardiothorac Surg ; 17(6): 655-7, 2000 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-10856855

RESUMEN

BACKGROUND: The surgical approach of neonates with a functionally univentricular heart, transposition of the great arteries and excessive pulmonary bloodflow remains a challenge. Pulmonary artery banding remains a valuable option, but may induce ventricular hypertrophy, restriction of the bulboventricular foramen and dysplastic changes of the pulmonary valve. These secondary changes might compromise a later Damus-Kaye-Stansel connection because of pulmonary regurgitation but also a subsequent Fontan repair because of ventricular hypertrophy. The aim of this study is to investigate whether a previous pulmonary artery banding might compromise the function of a Damus-Kaye-Stansel connection. METHODS: Thirteen neonates underwent pulmonary artery banding for functionally univentricular heart, transposition of the great arteries and pulmonary hypertension. Coarctation repair was associated in seven patients. All but one survived the operation. The twelve survivors underwent at a second stage a Damus-Kaye-Stansel connection after a mean interval of 1.1 years. The length of this interval was dictated by the degree of ventricular hypertrophy, the restriction of the bulboventricular foramen and by the degree of cyanosis. The Damus-Kaye-Stansel connection was constructed without any foreign material and with resorbable sutures. Associated procedures were: Glenn/hemi-Fontan (8 pts), Blalock-shunt (2 pts), biventricular correction with a homograft (1 pt), Fontan repair (1 pt). Four patients underwent successfully a Fontan repair at a third stage; seven patients are waiting for such repair. RESULTS: All patients survived the second and third stage of this surgical approach. The mean follow-up after the Damus-Kaye-Stansel connection was 2.5 years. Echocardiography at the last follow-up revealed: PR grade 0 (1 pt), grade 1 (8 pts), and grade 2 (3 pts). All patients - except one patient with a systolic gradient of 24 mmHg - had laminar flow without any gradient in the ascending aorta. All patients, including those who underwent a definitive repair, are doing extremely well. CONCLUSION: This experience demonstrates that a neonatal pulmonary artery banding does not compromise the function neither of a Damus-Kaye-Stansel connection nor a Fontan repair.


Asunto(s)
Anomalías Múltiples/cirugía , Cardiopatías Congénitas/cirugía , Ventrículos Cardíacos/anomalías , Ventrículos Cardíacos/cirugía , Hipertensión Pulmonar/cirugía , Arteria Pulmonar/cirugía , Anomalías Múltiples/diagnóstico por imagen , Procedimientos Quirúrgicos Cardíacos/métodos , Ecocardiografía Doppler , Femenino , Estudios de Seguimiento , Procedimiento de Fontan , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Hipertensión Pulmonar/diagnóstico por imagen , Recién Nacido , Masculino , Transposición de los Grandes Vasos/diagnóstico por imagen , Transposición de los Grandes Vasos/cirugía , Resultado del Tratamiento , Grado de Desobstrucción Vascular , Resistencia Vascular
13.
Eur J Cardiothorac Surg ; 15(2): 143-9, 1999 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-10219546

RESUMEN

OBJECTIVE: Pulmonary regurgitation after valveless repair of right ventricular outflow tract obstruction (RVOTO) results in progressive right ventricular (RV) dilatation and dysfunction in an increasing number of patients. Since 1989, we have exclusively used cryopreserved homografts to restore pulmonary valve competence in these patients. Our 9-year-experience with pulmonary valve insertion (PVI) in such cases has been reviewed to evaluate the indications for this procedure and its benefits. METHODS: From 1989 to 1998, 49 patients (original diagnosis: tetralogy of Fallot in 42 patients and pulmonary stenosis in seven) aged from 3 to 42 years (mean 18 +/- 9 years) underwent PVI with homografts late (mean 13 +/- 7 years) after valveless repair of RVOTO (transannular patch, n = 38; pulmonary valvulotomy therefore tau chi infundibular patch, n = 11). Preoperatively, all patients had severe pulmonary regurgitation, cardiomegaly, significant to severe RV dilatation and dysfunction, fatigue, reduced exercise tolerance, and were in NYHA class II (n = 43) or III (n = 6). Ten patients had ventricular arrhythmia. RESULTS: There was one early death, due to air embolism, and one late death, due to ventricular arrhythmia. All survivors but one, who subsequently underwent heart transplant, had symptomatic improvement after homograft insertion. The mean RV end-diastolic diameter decreased from 38 +/- 9 to 26 +/- 8 mm (P < 0.01), and cardiothoracic ratio decreased from 0.62 +/- 0.07 to 0.54 +/- 0.04 (P < 0.01). Good late homograft function was the rule, with all the survivors being free of reoperation for valve failure. At a mean follow-up of 42 +/- 28 months, 41 patients (87% of the survivors) were in New York Heart Association (NYHA) class I and six in class II. Within this group three patients are still in treatment for RV failure and five for ventricular arrhythmias. In these patients, the average interval between RVOTO repair and PVI was significantly longer than in the others (18 +/- 7 vs. 12 +/- 6 years, P < 0.01). CONCLUSION: Homograft PVI is safe and provides clinical improvement with a significant reduction in RV volume overload and excellent mid-term results in most patients with severe PR late after RVOTO repair. This procedure should be undertaken early in symptomatic patients, before severe RV failure and ventricular arrhythmias ensue.


Asunto(s)
Implantación de Prótesis de Válvulas Cardíacas , Insuficiencia de la Válvula Pulmonar/cirugía , Obstrucción del Flujo Ventricular Externo/cirugía , Adolescente , Adulto , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Masculino , Insuficiencia de la Válvula Pulmonar/etiología , Reoperación , Estudios Retrospectivos , Trasplante Homólogo , Resultado del Tratamiento , Obstrucción del Flujo Ventricular Externo/complicaciones
14.
Genet Couns ; 10(1): 3-9, 1999.
Artículo en Inglés | MEDLINE | ID: mdl-10191424

RESUMEN

During the last 5 years, we diagnosed in Leuven 130 patients with a 22q11 deletion. The deletion was familial in 14 out of 110 index patients (12%), which is significantly less compared to previous studies. In 10 patients, the deletion was maternal, in 4 patients paternal. A cardiac defect was the main presenting symptom in 49% of patients. The other patients were ascertained through developmental delay (16%), behavioural disturbances (7%), otorhinolaryngological manifestations (6%), psychiatric manifestations (3%) and mental retardation (2%). In one patient hypocalcemia was the presenting symptom. In another patient the severe immune deficiency led to diagnosis. Most patients presented a wide variety of the classical features of the Velo-Cardio-Facial syndrome. Velopharyngeal incompetence, learning difficulties or mostly mild mental retardation were almost always present, whereas clinical significant hypocalcemia or immune disturbances were rare. Previously un(der)recognised features include polyhydramnios, renal malformations and laryngotracheamalacia or laryngeal stenosis.


Asunto(s)
Anomalías Múltiples/genética , Deleción Cromosómica , Cromosomas Humanos Par 22 , Síndrome de DiGeorge/genética , Insuficiencia Velofaríngea/genética , Anomalías Múltiples/diagnóstico , Adolescente , Adulto , Niño , Preescolar , Síndrome de DiGeorge/diagnóstico , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Países Bajos , Embarazo , Diagnóstico Prenatal , Insuficiencia Velofaríngea/diagnóstico
16.
Heart ; 92(11): 1661-6, 2006 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-16644857

RESUMEN

OBJECTIVE: To determine the safety, feasibility and effectiveness of stent expansion of hypoplastic aortic segments with pressure gradients in patients with arterial hypertension. DESIGN: Non-randomised prospective clinical trial. SETTING: Tertiary referral centre, congenital cardiac unit. PATIENT SELECTION: 20 consecutive patients (median age 14.5 years, range 11.6-38.8 years) with arterial hypertension and a hypoplastic segment of the aorta. Seventeen patients had successful previous arch interventions in a coarctation site. INTERVENTIONS: Stent deployment in hypoplastic arch segments. MAIN OUTCOME MEASURES: Gradient across the aortic arch; complications early and during follow up; residual hypertension. RESULTS: 23 stents were deployed: 13 in the cross and 10 in the isthmus. The mean gradient across the aortic arch decreased from 16 (SD 6) (median 17) to 3 (4) (median 1) mm Hg (p < 0.001). In a few patients a mild gradient persisted just distal to the left carotid artery due to residual orificial narrowing or acute angulation. No complications occurred during or after the procedure. During follow up of 2.2 years (range 0.2-4.8 years) arterial hypertension resolved in 10 patients and 10 required residual drug treatment with better control of blood pressures. CONCLUSIONS: Pressure loss due to residual hypoplastic aortic segments can be treated effectively and safely with stent expansion. Some patients remain mildly hypertensive and require additional drug treatment.


Asunto(s)
Angioplastia de Balón/métodos , Coartación Aórtica/terapia , Hipertensión/terapia , Stents , Adolescente , Adulto , Presión Sanguínea , Niño , Estudios de Factibilidad , Estudios de Seguimiento , Humanos , Estudios Prospectivos
17.
Pediatr Cardiol ; 23(3): 292-306, 2002.
Artículo en Inglés | MEDLINE | ID: mdl-11976774

RESUMEN

From a temporally resolved radio-frequency data set, a series of new developing indices of regional myocardial deformation can be measured. These may be important new data sets to quantify regional radial and long axis function of the left or right ventricle in children with acquired or congenital heart disease. Using color Doppler methodology unidimensional regional myocardial strain rates (SR) and natural strain (e) can now be calculated from local myocardial velocity data sets. Normal regional velocity, SR, and e values have already been determined in older children and should form the database for studies into acquired or congenital heart disease in this age group. Neonatal and infant values have yet to be determined. This article will present preliminary data on the potential clinical value of ultrasonic regional SR and e imaging in children and will discuss the close interrelation of these new regional function parameters with an alternative approach to quantification: the measurement of local cyclical changes in integrated backscatter levels.


Asunto(s)
Ecocardiografía Doppler en Color/métodos , Cardiopatías/diagnóstico por imagen , Cardiopatías/fisiopatología , Corazón/fisiopatología , Velocidad del Flujo Sanguíneo , Niño , Cardiopatías/congénito , Humanos , Miocardio/patología , Función Ventricular
18.
Eur J Pediatr ; 154(6): 462-8, 1995 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-7671944

RESUMEN

UNLABELLED: Hypophosphataemic rickets was diagnosed in a 6-year-old boy with a negative family history. After 16 years of medical treatment he developed a malignant sarcoma of the right distal thigh. Removal of the tumour by high amputation of the leg resulted in disappearance of the phosphate leak. In spite of surgery and chemotherapy, the patient died due to extensive lung metastases. Retrospective analysis of the initial X-ray films showed a benign lesion on the lateral side of the right distal femur. This lesion is believed to be at the origin of the rickets. This is the first paediatric case reported with malignant degeneration of a benign tumour causing rickets. CONCLUSION: Patients with the classical hallmarks of X-linked, familial hypophosphataemic rickets but no affected family members should have a careful periodic search for a tumour, even years after onset of the disorder.


Asunto(s)
Neoplasias Femorales/complicaciones , Fibrosarcoma/complicaciones , Hipofosfatemia/etiología , Raquitismo/etiología , Niño , Resultado Fatal , Neoplasias Femorales/diagnóstico , Neoplasias Femorales/terapia , Fibrosarcoma/diagnóstico , Fibrosarcoma/terapia , Humanos , Neoplasias Pulmonares/secundario , Masculino
19.
Heart ; 84(1): 46-52, 2000 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-10862587

RESUMEN

OBJECTIVE: To assess the relation between exercise intensity and oxygen uptake during graded exercise in paediatric patients who underwent surgical repair of congenital heart disease, and to compare it with conventional measures of aerobic exercise function. DESIGN: Cross sectional study. Exercise testing was performed on a treadmill and gas exchange was measured on a breath by breath basis. PATIENTS: 29 patients who underwent an atrial switch operation for transposition of the great arteries (TGA) (mean (SD) age at testing 10.3 (2.5) years) and 30 patients who underwent total repair of tetralogy of Fallot (TF) (age 12.1 (3.3) years) performed graded exercise testing. Exercise responses were compared with data obtained in 24 normal controls (age 11.4 (2.6) years). RESULTS: The slope of oxygen uptake versus exercise intensity averaged 1.50 (0. 64) ml O(2)/min(2)/kg in the patients with TGA and 1.68 (0.75) ml O(2)/min(2)/kg after TF repair, both lower (p < 0.005) than in normal controls (2.42 (0.68) ml O(2)/min(2)/kg). The lower slope of oxygen uptake was correlated with a subnormal value for ventilatory anaerobic threshold, which averaged 78.0 (13.3)% of normal in TGA and 85.1 (10.6)% in TF. This was associated with a steeper slope (p = 0.001) of carbon dioxide output versus oxygen uptake above the ventilatory anaerobic threshold in TGA (1.26 (0.20)) and TF (1.20 (0. 18)) compared with the normal controls (1.05 (0.13)), and also a steeper slope of ventilation versus carbon dioxide in TGA (47.0 (15. 4)) and TF (41.5 (13.7)) than in the controls (30.3 (8.5)). CONCLUSIONS: Calculation of the steepness of the slope of oxygen uptake versus exercise intensity is a valid measurement of oxygen flow to the exercising tissues, which may be limited in congenital heart disease.


Asunto(s)
Prueba de Esfuerzo , Consumo de Oxígeno , Transposición de los Grandes Vasos/metabolismo , Transposición de los Grandes Vasos/cirugía , Adolescente , Adulto , Estudios de Casos y Controles , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Periodo Posoperatorio , Intercambio Gaseoso Pulmonar , Tetralogía de Fallot/cirugía
20.
Pediatr Res ; 44(4): 607-11, 1998 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-9773854

RESUMEN

Patients with a microdeletion on chromosome 22q11 demonstrate the clinical picture of the velocardiofacial syndrome. We report on three members of the same family with this microdeletion and velocardiofacial syndrome, all having an increase in platelet size and a mild decrease in platelet number. Their platelet function, however, tested by aggregation and by adherence to collagen in a whole blood perfusion system, was normal. We retrospectively studied the files of 35 other patients with 22q11 deletion and also found that their platelets had an increased size compared with cardiac controls. Moreover, their platelet size correlated negatively with platelet number. Knowing that patients with 22q11 deletion are obligate carriers for a heterozygous glycoprotein Ib beta deletion, these patients can be considered to be heterozygous Bernard-Soulier patients. In addition, a significant increase in platelet size may be a positive predictor for the clinical diagnosis of the velocardiofacial syndrome.


Asunto(s)
Plaquetas/patología , Deleción Cromosómica , Cromosomas Humanos Par 22 , Anomalías Craneofaciales/genética , Recuento de Plaquetas , Tetralogía de Fallot/genética , Niño , Mapeo Cromosómico , Anomalías Craneofaciales/sangre , Femenino , Marcadores Genéticos , Heterocigoto , Humanos , Hibridación Fluorescente in Situ , Discapacidad Intelectual/sangre , Discapacidad Intelectual/genética , Masculino , Núcleo Familiar , Adhesividad Plaquetaria , Síndrome , Tetralogía de Fallot/sangre
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