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1.
Circulation ; 102(7): 813-6, 2000 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-10942752

RESUMEN

BACKGROUND: Pulmonary regurgitation can lead to severe right ventricular dysfunction, which is a delicate postoperative problem in the long-term follow-up of patients who had surgery for congenital heart diseases. Clinical conditions of patients suffering from pulmonary valve incompetence are improved by valve replacement with a prosthetic valve. To date, the surgical approach is the only option to replace a pulmonary valve. We report the first experience of percutaneous pulmonary valve implantation. METHODS AND RESULTS: A fresh bovine jugular vein containing a native valve was sutured into a vascular stent and then cross-linked with a 0.6% glutaraldehyde solution for 36 hours. After being hand-crimped onto a balloon catheter, the device was inserted percutaneously according to standard stent-placing techniques. The valved stent was finally deployed in the position of the native pulmonary valve of the lamb. Hemodynamic evaluation was carried out before and 2 months after implantation. Anatomic evaluation was finally performed. Percutaneous pulmonary valve replacement was successful in 5 lambs. No complications were noted. Early and late angiographic and hemodynamic studies confirmed a good position of the stents with a competent valve at the end of the protocol. One stent was slightly stenotic, with macroscopically visible calcifications. CONCLUSIONS: Nonsurgical implantation of pulmonary valves is possible in the lamb. This new technique is similar to standard stent implantation. Thus, it should be feasible in humans, in whom it will lead to a significant reduction of reoperations in patients in need of pulmonary valve replacement.


Asunto(s)
Válvula Pulmonar/trasplante , Trasplante Heterólogo , Angiografía , Animales , Cateterismo , Bovinos , Reactivos de Enlaces Cruzados/farmacología , Glutaral/farmacología , Hemodinámica , Venas Yugulares/cirugía , Periodo Posoperatorio , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/fisiología , Ovinos , Stents , Suturas , Trasplante/métodos
2.
J Am Coll Cardiol ; 26(7): 1725-31, 1995 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-7594110

RESUMEN

OBJECTIVES: Percutaneous balloon valvotomy was studied retrospectively in newborns with critical pulmonary valve stenosis or atresia to assess its potential role as an alternative therapy to operation. BACKGROUND: Severe right ventricular outflow tract obstructions are life-threatening conditions requiring prostaglandin infusion immediately after birth and then relief of the valvular obstruction. To avoid surgical hazards at this age, it would be useful to extend to newborns the balloon valvotomy so effective in older patients. METHODS: Ninety-seven newborns (82 with critical pulmonary valve stenosis, 15 with atresia) underwent balloon valvotomy, provided that they had a well developed right ventricle, including an infundibulum close to the pulmonary artery. In patients with atresia, the outflow tract membrane had to be perforated with a wire needle or a radiofrequency probe. RESULTS: Balloon valvotomy could be performed in 81 patients and was effective in 77. It caused 3 fatal and 16 nonfatal complications. Ten patients with persistent poor right ventricular compliance despite an effective valvotomy required a surgical shunt. Among the 81 patients in whom the procedure could be performed, right ventricular surgery was avoided in 5 (55%) of the 9 patients with atresia (95% confidence interval [CI] 28% to 80%) and 55 (76%) of the 72 patients with stenosis (95% CI 66% to 86%) at the end of the follow-up period (9.7 years). CONCLUSIONS: Balloon pulmonary valvotomy is not always feasible in newborns, but it is relatively safe and effective and should be considered a valid alternative to operation.


Asunto(s)
Cateterismo , Atresia Pulmonar/terapia , Estenosis de la Válvula Pulmonar/terapia , Estudios de Seguimiento , Humanos , Recién Nacido , Estenosis de la Válvula Pulmonar/congénito , Estudios Retrospectivos , Resultado del Tratamiento
3.
J Am Coll Cardiol ; 34(3): 866-70, 1999 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-10483971

RESUMEN

OBJECTIVES: We propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations. BACKGROUND: The alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery. METHODS: Eleven infants (mean weight 2,560 +/- 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 +/- 0.7 mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure. RESULTS: There were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients. CONCLUSIONS: Provided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.


Asunto(s)
Implantes Absorbibles , Coartación Aórtica/cirugía , Defectos del Tabique Interventricular/cirugía , Polidioxanona , Arteria Pulmonar/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/mortalidad , Estudios de Seguimiento , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/mortalidad , Mortalidad Hospitalaria , Humanos , Lactante , Recién Nacido , Selección de Paciente , Complicaciones Posoperatorias/epidemiología , Factores de Tiempo
4.
J Am Coll Cardiol ; 13(5): 1101-5, 1989 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-2926060

RESUMEN

Percutaneous balloon valvuloplasty was attempted in 10 newborn infants with critical aortic valve stenosis and severe congestive heart failure. Three had a very small left ventricle and aortic anulus. In one infant, the aortic valve could not be passed, and in another infant, a technical error resulted in severe valvular damage, aortic insufficiency and death. Among the eight patients who had effective dilation, the stenosis was relieved in seven as assessed by a significant decrease in transvalvular pressure gradient, improvement of left ventricular contraction and eventual inversion of the ductal shunting. The procedure failed in the only patient whose dilation was performed with an undersized balloon. Aortic insufficiency occurred in three infants and was severe (perforated cusp) in one, moderate in one whose valve was dilated with an excessively large balloon and mild and transient in one. None of the three infants with a very small left ventricle recovered (two died and one underwent cardiac transplantation). Among the seven infants with a left ventricle of acceptable size, three underwent subsequent aortic valvotomy; one of these died and two bad good results. The remaining four are doing well 16 +/- 5 months later (mean +/- SD) with mild to moderate residual aortic stenosis and normal left ventricular function. In conclusion, percutaneous balloon valvuloplasty is an acceptable alternative to surgery in neonates with critical aortic valve stenosis. Incidence of complications and good relief of the obstruction depend on a careful technique. Immediate results are similar to those of surgery. Late prognosis depends on the quality of the left heart structures.


Asunto(s)
Estenosis de la Válvula Aórtica/terapia , Cateterismo , Insuficiencia de la Válvula Aórtica/etiología , Estenosis de la Válvula Aórtica/mortalidad , Bradicardia/etiología , Bradicardia/fisiopatología , Cateterismo/efectos adversos , Estudios de Evaluación como Asunto , Humanos , Lactante , Recién Nacido , Pulso Arterial
5.
J Am Coll Cardiol ; 29(1): 202-6, 1997 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-8996315

RESUMEN

OBJECTIVES: We sought to describe a large series of coronary artery obstructions after the arterial switch operation for transposition of the great arteries and to discuss their clinical implications. BACKGROUND: Aortic root angiography and myocardial perfusion imaging yield ambiguous results regarding the fate of the coronary artery anastomoses after the arterial switch operation. Late death related to coronary artery obstruction and growth of the translocated coronary arteries are of major concern in these patients. METHODS: Selective coronary artery angiography was performed prospectively in a total of 165 children. RESULTS: A total of 12 coronary occlusions, 8 major stenoses, 6 minor stenoses of the left ostium and 4 stretchings of one coronary artery were identified. Obstructions were more frequent in types D and E (p < 0.001) of the Yacoub and Radley-Smith classification. Coronary obstruction was documented in all patients with electrocardiographic and ultrasound evidence of myocardial ischemia at time of study. Early postoperative ischemia did not predict coronary artery lesion if the patient had fully recovered. Persistent or delayed myocardial ischemia was highly predictive of coronary artery lesions. The incidence of coronary artery obstruction was very high (11 of 35) in patients operated on by a rapidly abandoned technique of single-orifice reimplantation of both coronary artery ostia. CONCLUSIONS: Selective coronary angiography is the most accurate means to assess coronary artery obstruction after the arterial switch operation. Precise diagnosis of coronary artery lesions after this operation will help to elucidate the pathogenesis, develop adequate therapeutic strategies and might indicate how to prevent coronary complications after operation.


Asunto(s)
Enfermedad Coronaria/epidemiología , Complicaciones Posoperatorias/epidemiología , Transposición de los Grandes Vasos/cirugía , Preescolar , Angiografía Coronaria , Enfermedad Coronaria/diagnóstico por imagen , Enfermedad Coronaria/etiología , Estudios de Seguimiento , Humanos , Incidencia , Recién Nacido , Complicaciones Posoperatorias/diagnóstico por imagen , Estudios Prospectivos , Factores de Tiempo
6.
J Nucl Med ; 41(3): 400-4, 2000 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-10716309

RESUMEN

UNLABELLED: Abnormalities of norepinephrine uptake have been found to reflect impairment of cardiac adrenergic neuronal function in adults with heart failure. To our knowledge, no data on childhood dilated cardiomyopathy (DCM) are available. The aim of this study was to assess the cardiac neuronal function using 123I-metaiodobenzylguanidine (MIBG) scintigraphy in children with idiopathic DCM. METHODS: We studied 26 patients (mean age, 44+/-50 mo) with DCM and left ventricular dysfunction and 12 control subjects (mean age, 49+/-65 mo) with normal left ventricular function. All subjects underwent planar cardiac imaging after intravenous injection of 20-75 MBq 123I-MIBG. A static anterior view was acquired 4 h after injection. The heart-to-mediastinum count ratio was measured as described previously. RESULTS: On the basis of a reduction of the heart-to-mediastinum count ratio, cardiac neuronal uptake of 123I-MIBG was significantly decreased in patients with DCM compared with cardiac uptake in control subjects (172%+/-34% versus 277%+/-14%; P<0.0001). A significant correlation was found between left ventricular ejection fraction and 123I-MIBG cardiac uptake in patients with DCM (y = 2.5x + 113.3; r = 0.80; P<0.0001). CONCLUSION: Cardiac adrenergic neuronal function is impaired in children with idiopathic DCM. 1231-MIBG cardiac scintigraphy is a useful tool to assess cardiac neuronal function in childhood DCM.


Asunto(s)
Cardiomiopatía Dilatada/diagnóstico por imagen , Corazón/inervación , Radioisótopos de Yodo , Cardiomiopatía Dilatada/fisiopatología , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Corazón/diagnóstico por imagen , Humanos , Lactante , Masculino , Estudios Prospectivos , Cintigrafía , Radiofármacos , Disfunción Ventricular Izquierda/diagnóstico por imagen
7.
Am J Cardiol ; 74(8): 807-9, 1994 Oct 15.
Artículo en Inglés | MEDLINE | ID: mdl-7942555

RESUMEN

The authors report on a cooperative study of 43 cases of bacterial pericarditis observed in children. This disorder was suspected in patients with septicemia who developed symptoms and signs of pericarditis (precordial pain, muffled heart sounds, pericardial friction rub, cardiomegaly). Early diagnosis of this condition is now facilitated by echocardiography. A combination of medical and surgical treatments (appropriate antibiotic therapy after culture and sensitivity tests and early pericardial drainage) led to complete recovery in almost all of the cases (42 of 43). After long-term follow-up, no cases of constrictive pericarditis were observed.


Asunto(s)
Infecciones Bacterianas , Pericarditis , Adolescente , Infecciones Bacterianas/microbiología , Infecciones Bacterianas/terapia , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Infecciones Meningocócicas/terapia , Pericarditis/microbiología , Pericarditis/terapia , Pronóstico , Infecciones Estafilocócicas/terapia
8.
Am J Med Genet ; 46(6): 700-5, 1993 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-8362913

RESUMEN

A linkage analysis has been performed on 6 two-generation families with classical Noonan syndrome to determine whether the syndrome is linked to neurofibromatosis type 1 on chromosome 17q or to neurofibromatosis type 2 on chromosome 22q. A significantly negative location score was obtained between 10 cM centromeric to and 15 cM telomeric from the neurofibromatosis type 1 locus. A significantly negative lod score was obtained with a marker mapping within the region where neurofibromatosis type 2 is thought to be located. These data indicate that Noonan syndrome is not tightly linked to either neurofibromatosis type 1 or type 2.


Asunto(s)
Genes de Neurofibromatosis 1 , Genes de la Neurofibromatosis 2 , Ligamiento Genético , Síndrome de Noonan/genética , Mapeo Cromosómico , Cromosomas Humanos Par 17 , Cromosomas Humanos Par 22 , Femenino , Humanos , Masculino , Síndrome de Noonan/diagnóstico , Linaje , Polimorfismo Genético
9.
Am J Med Genet ; 68(2): 182-4, 1997 Jan 20.
Artículo en Inglés | MEDLINE | ID: mdl-9028455

RESUMEN

Cono-truncal cardiac malformations account for some 50% of congenital heart defects in newborn infants. Recently, hemizygosity for chromosome 22q11.2 was reported in patients with the DiGeorge/Velo-cardio-facial syndromes (DGS/VCFS) and causally related disorders. We have explored the potential use of microsatellite DNA markers for rapid detection of 22q11 deletions in 19 newborn infants referred for cono-truncal heart malformations with associated DGS/VCFS anomalies. A failure of parental inheritance was documented in 84.2% of cases (16/19). PCR-based genotyping using microsatellite DNA markers located within the commonly deleted region allowed us either to confirm or reject a 22q11 microdeletion in 94.3% of cases (18/19) within 24 hours. This test is now currently performed in the infants referred to us for a cono-truncal heart malformation as a first intention screening for 22q11 microdeletion.


Asunto(s)
Cromosomas Humanos Par 22/genética , Síndrome de DiGeorge/diagnóstico , Síndrome de DiGeorge/genética , Repeticiones de Microsatélite , Alelos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/genética , Humanos , Hibridación Fluorescente in Situ , Recién Nacido , Linaje , Reacción en Cadena de la Polimerasa , Eliminación de Secuencia
10.
J Thorac Cardiovasc Surg ; 102(1): 140-7, 1991 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-2072712

RESUMEN

One hundred eighteen patients, 100 with transposition of the great arteries plus ventricular septal defect and 18 with double-outlet right ventricle and subpulmonary ventricular septal defect have undergone arterial switch and patch closure of the ventricular septal defect since February 1983. In transposition of the great arteries the ventricular septal defect was perimembranous in 70 cases, trabecular in 28, and infundibular in 10. Eleven patients had multiple ventricular septal defects. In addition to 18 patients with double-outlet right ventricle, malalignment of the conal septum was present in 19 cases. Coronary type A distribution was recognized in 79 cases, type C in one, type D in 24, and type E in 14. Great arteries were side by side in 19% of cases. Aortic coarctation was present in 31 cases, and subaortic obstruction in 9. Age at operation ranged from 4 days to 4 years (mean, 3.5 +/- 8.3 months), and mean weight was 4.0 +/- 1.6 kg. Thirty-seven infants were younger than age 1 month. Thirty-six patients underwent previous operations: pulmonary artery banding alone (n = 13), pulmonary artery banding and coarctation repair (n = 13), and coarctation repair alone (n = 10). Mean time between the first procedure and the switch was 2.2 months. Six patients with aortic coarctation underwent one-stage repair, through median sternotomy, aortic reconstruction, closure of the ventricular septal defect, and arterial switch. Perioperative mortality was 13.5% (70% confidence limit 10% to 17.6%, n = 16). It was directly related to coronary artery kinking in 50% of deaths and to anatomy and size discrepancy of the great arteries in the remaining deaths. Univariate analysis could not find any significant risk factor of in-hospital mortality. Mean follow-up of 30.3 +/- 23.5 months was achieved in all but 2 survivors. There was one late death. Ten patients underwent 11 reoperations for recoarctation (n = 1), pulmonary stenosis (n = 7), residual ventricular septal defect (n = 2), and stenosis of superior vena cava (n = 1). Two patients needed a permanent pacemaker. Actuarial survival and freedom from reoperation at 5 years were 84.5% +/- 3.6% and 85.7% +/- 4.6%, respectively. We conclude that anatomic correction of complex transposition is a safe method that offers good early and midterm results.


Asunto(s)
Defectos del Tabique Interventricular/cirugía , Transposición de los Grandes Vasos/cirugía , Preescolar , Ventrículo Derecho con Doble Salida/complicaciones , Ventrículo Derecho con Doble Salida/cirugía , Femenino , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/mortalidad , Defectos del Tabique Interventricular/patología , Humanos , Masculino , Métodos , Complicaciones Posoperatorias , Reoperación , Tasa de Supervivencia , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/patología
11.
J Thorac Cardiovasc Surg ; 96(3): 354-63, 1988 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-3411980

RESUMEN

From March 1984 to January 1987, anatomic surgical correction was performed on 110 newborn infants (2 to 23 days old, mean 7.8 +/- 3.5, standard deviation) with simple transposition of the great arteries and 10 additional neonates (7 to 30 days old, mean 17.9 +/- 8.3, standard deviation) with transposition and a large ventricular septal defect. All had preoperative catheterization. Ninety-six percent of the patients underwent balloon atrial septostomy and 90% received prostaglandin E1 infusion until the time of the operation. The anatomy of the coronary arteries according to the Yacoub classification was as follows: type A, 82 patients; type B, 5 patients; type C, 4 patients; type D, 23 patients; and type E, 6 patients. Continuous hypothermic bypass with no circulatory arrest was used for all patients except two. Myocardial protection was ensured by crystalloid cardioplegia. Coronary artery relocation was performed according to the Yacoub technique with some modifications, and pulmonary artery reconstruction was done according to the Lecompte maneuver in all patients, even when the great vessels had a side-by-side relationship. The proximal pulmonary artery was reconstructed with two circular patches for the first 10 patients and with a single large posterior patch for the last 110 patients. Tanned heterologous pericardium was used for the first 25 patients and autologous native pericardium for the last 95 patients. The perioperative mortality rates were 8.3% for the entire series and 5.4% for the last 110 patients, with no deaths in the group having transposition plus ventricular septal defect. Late death from acute myocardial infarction occurred in two patients in the second month after operation. No patient was lost to follow-up, which ranged from 2 to 46 months (mean 16 +/- 11.2, standard deviation). The follow-up included sequential noninvasive evaluations and 32 catheterizations performed 10 to 18 months postoperatively. Two patients were reoperated on for pulmonary stenosis caused by retraction of the two heterologous pericardial patches, but neither died. Six others have mild to moderate pulmonary stenosis. Two patients have trivial aortic regurgitation. None have aortic dilatation or supravalvular aortic stenosis. The 108 survivors have no cardiovascular symptoms. They all are in sinus rhythm, have normal left ventricular function, have no ischemic problems, and receive no medication.


Asunto(s)
Transposición de los Grandes Vasos/cirugía , Vasos Coronarios/patología , Vasos Coronarios/cirugía , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/cirugía , Humanos , Recién Nacido , Métodos , Cuidados Posoperatorios , Complicaciones Posoperatorias , Cuidados Preoperatorios , Reoperación , Transposición de los Grandes Vasos/complicaciones , Transposición de los Grandes Vasos/mortalidad , Transposición de los Grandes Vasos/patología
12.
J Thorac Cardiovasc Surg ; 117(2): 352-7, 1999 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-9918977

RESUMEN

OBJECTIVE: To determine the safety and efficacy of surgical angioplasty of the coronary arteries in children. METHODS: We performed 9 surgical reconstructions of the left main coronary artery and 1 of the right coronary artery ostium in 10 children (mean age 5.7 years; range 2 months-15 years). The basic diseases included the following: congenital atresia of the left coronary artery (n = 2) and atresia of the right coronary artery in a patient with an aortoventricular tunnel (n = 1); stenosis of the left main coronary artery (1) in a patient with Williams syndrome (n = 1), (2) in a patient with familial hypercholesterolemia (n = 1), (3) after the arterial switch operation for transposition of the great arteries (n = 3), (4) after reimplantation of an anomalous left main coronary artery from the pulmonary artery (n = 1), and (5) by compression after a réparation à l'étage ventriculaire procedure (n = 1). Myocardial viability was assessed by single photon emission computed tomography (thallium 201; 7/10). The coronary artery stem was enlarged with a saphenous (n = 5), a pericardial (n = 4), or a polytetrafluoroethylene patch (n = 1). RESULTS: There was 1 hospital death and 9 patients are alive (mean follow-up 46 +/- 30 months; range 12 months to 10.5 years). Eight of 9 survivors had a selective coronary artery angiogram and had normal coronary artery ostia. Two patients had stenosis of the left anterior descending coronary artery, 1 of whom underwent successful internal thoracic artery grafting. CONCLUSIONS: Surgical angioplasty of the coronary stems restores physiologic coronary perfusion and conserves bypass material. It can be performed safely in children and provides encouraging midterm results.


Asunto(s)
Angioplastia/métodos , Vasos Coronarios/cirugía , Adolescente , Niño , Preescolar , Enfermedad Coronaria/congénito , Enfermedad Coronaria/mortalidad , Enfermedad Coronaria/cirugía , Anomalías de los Vasos Coronarios/mortalidad , Anomalías de los Vasos Coronarios/cirugía , Estudios de Seguimiento , Mortalidad Hospitalaria , Humanos , Lactante , Pericardio/trasplante , Vena Safena/trasplante , Mallas Quirúrgicas
13.
J Thorac Cardiovasc Surg ; 100(6): 808-16, 1990 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-2246903

RESUMEN

From Jan. 1, 1983, to Jan. 1, 1988, 66 consecutive neonates with coarctation and severe hypoplasia of the transverse arch underwent coarctation repair by resection of the coarctation and reconstruction of the aortic arch. Mean age at operation was 14 +/- 8 days, ranging from 2 to 30 days; 63% of the newborn infants were less than 2 weeks of age. The coarctation was isolated in 23%, associated with a ventricular septal defect in 39%, and associated with complex anomalies in 38%, including 16 cases of transposition of the great arteries or doublet-outlet right ventricle plus ventricular septal defect, two cases of simple transposition, two of corrected transposition plus ventricular septal defect, and five cases of "hypoplastic" left ventricle. The surgical technique comprises a wide resection of the coarctation extended to the contiguous ductal tissue followed by the reconstruction of the aortic arch in bringing the descending aorta into the concavity of the aortic arch. This technique is able to relieve the obstruction of the aortic arch provided that (1) the descending aorta is widely dissected to allow mobilization and (2) the incision of the transverse arch is extended proximal to the ostium of the left carotid artery. The operation was performed through a left thoracotomy in 62 patients and through a sternotomy in four additional neonates with transposition and ventricular septal defect who underwent a one-stage repair with aortic reconstruction, closure of the defect, and arterial switch. The overall early mortality rate (less than 30 days) was 14% (9/66; 95% confidence limits = 5% to 22%), including four deaths occurring within the first month, at a concomitant or subsequent repair of the associated anomaly. There were six late deaths, all related to the associated lesions. The overall mortality rate was 23% (15/66; 95% confidence limits = 13% to 33%). The mean follow-up was 21 +/- 10 months, ranging from 6 to 66 months. Actuarial survival rates at 5 years are 72% +/- 10% for the overall group; 87% +/- 17% for simple coarctation; 88% +/- 12% for coarctation and ventricular septal defect; and 52% +/- 18% for complex coarctation. The rate of recurrent coarctation was 12.5% (95% confidence limits = 2% to 23%), leading to five reoperations with no deaths. Freedom from reoperation was 89.5% +/- 9% at 5 years. This technique of coarctation repair offers several advantages: low operative mortality, complete relief of the left ventricular obstruction, wide resection of the ductus tissue, absence of prosthetic material, and preservation of the left subclavian artery.


Asunto(s)
Aorta Torácica/anomalías , Coartación Aórtica/cirugía , Aorta Torácica/cirugía , Coartación Aórtica/complicaciones , Coartación Aórtica/mortalidad , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/mortalidad , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Métodos , Complicaciones Posoperatorias , Recurrencia , Reoperación , Tasa de Supervivencia
14.
Intensive Care Med ; 15 Suppl 1: S76-9, 1989.
Artículo en Inglés | MEDLINE | ID: mdl-2656818

RESUMEN

Organ transplantations have opened a new field in medicine and particularly in pediatrics. The kidney was the first organ to be regularly transplanted and there are now more than 2000 children who have received a kidney graft. Cadaver kidneys or living-related donor (LRD) kidneys can be used since an adult kidney may be grafted in a young child. Cadaver graft survival exceeds 85% at 1 year in recent single centre reports and patient survival is above 95%, the results being even better with LRD. Some complications may be observed in the long term, such as bone osteonecrosis, hypertension or infections. Rehabilitation is generally remarkable and growth which remained abnormal in 1/3 of cases under conventional treatment seems to improve markedly with cyclosporin. In the future, the development of kidney transplantation could lead hopefully to a drastic decrease in the number of children on dialysis. Liver transplantation is now performed in children with biliary atresia and metabolic diseases. There is no age limit for grafting a liver, the only problem being to find a pediatric cadaver donor of similar size. It is also possible to use an adult liver after hepatectomy. There were 170 liver grafts recorded in children in Europe in December 1986, and the long-term survival thanks to cyclosporin is exceeding 80% in some units. Thanks to cyclosporin, programs of cardiac transplantation for children are rapidly developing. The main indications are gross-congenital cardiac malformations or severe functional abnormality not compatible with life. Newborns have been grafted with success. The only problem is also to find a suitable pediatric donor.(ABSTRACT TRUNCATED AT 250 WORDS)


Asunto(s)
Trasplante , Factores de Edad , Niño , Francia , Trasplante de Corazón , Humanos , Inmunosupresores/uso terapéutico , Intestino Delgado/trasplante , Trasplante de Riñón , Trasplante de Hígado , Trasplante de Pulmón
15.
Ann Thorac Surg ; 51(6): 1017-9, 1991 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-2039302

RESUMEN

A case of single-stage repair of aortic atresia with normal-sized left ventricle and ventricular septal defect in a neonate is reported. The surgical procedure included rerouting of the left ventricular bloodstream to the pulmonary artery through the ventricular septal defect and connection of the pulmonary trunk to the aortic arch. The right ventricular outflow tract was then reconstructed with an extracardiac valved conduit. Three years after the initial operation, replacement of the valved conduit was performed uneventfully. The clinical status of the child is very satisfactory.


Asunto(s)
Aorta/anomalías , Ventrículos Cardíacos/patología , Aorta/patología , Aorta/cirugía , Aortografía , Angiografía Coronaria , Femenino , Defectos del Tabique Interventricular/complicaciones , Defectos del Tabique Interventricular/patología , Defectos del Tabique Interventricular/cirugía , Humanos , Recién Nacido
16.
Ann Thorac Surg ; 55(4): 990-2, 1993 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-8466357

RESUMEN

Two cases of neonatal aortic arch thrombosis are reported. One patient, who had ascending aortic thrombosis, died preoperatively. The other had reoperation and is alive and well at 6 months' follow-up. No obvious cause was found.


Asunto(s)
Aorta Torácica , Trombosis/congénito , Adulto , Aorta Torácica/cirugía , Femenino , Humanos , Recién Nacido , Masculino , Reoperación , Trombosis/complicaciones , Trombosis/patología , Trombosis/cirugía
17.
Ann Thorac Surg ; 56(6): 1239-47, 1993 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-8267419

RESUMEN

Among 54 children who underwent 55 heart transplantations, 24 (44%) (mean age, 4.9 +/- 4.8 years; range, 9 days to 18 years) had congenital defects with the following diagnoses: single-ventricle variants (6), hypoplastic left heart syndrome variants (5), transposition complex (6), and miscellaneous defects (7). Twenty patients (83%) had undergone 43 prior operations. Additional surgical procedures included repositioning of transposed great arteries (11), reconstruction of the aortic pathway (4), reconstruction of the pulmonary pathway (8), correction of situs inversus (1), and correction of anomalous pulmonary (1) or systemic (1) venous drainage. Reconstructive procedures were performed using donor or recipient tissue or both. There were six early deaths (hyperacute rejection, 1 patient; pulmonary hypertension, 1; graft failure, 2 patients; infection, 2) and six late deaths (sudden death, 2; chronic rejection, 2; nonspecific graft dysfunction, 1; lymphoproliferative disease, 1). The survival rate was 43% +/- 12% at 3 years. No deaths were related to surgical technique. Survival was not significantly different in pediatric recipients with cardiomyopathy (67% +/- 9%; p = 0.22). Accelerated coronary artery disease was noted in 4 operative survivors (22%; 70% confidence limits, 12% to 36%). All late survivors were free from cardiac symptoms after a mean follow-up of 34 +/- 24 months (range, 6 to 71 months). Based on this study, we reached three conclusions. (1) Careful planning of both harvesting and transplantation procedures allows heart transplantation in recipients with congenital heart diseases. (2) The surgical technique may be demanding, but the early risk is not increased.(ABSTRACT TRUNCATED AT 250 WORDS)


Asunto(s)
Cardiopatías Congénitas/cirugía , Trasplante de Corazón , Adolescente , Niño , Preescolar , Enfermedad Coronaria/etiología , Ecocardiografía , Rechazo de Injerto/diagnóstico por imagen , Rechazo de Injerto/epidemiología , Rechazo de Injerto/etiología , Rechazo de Injerto/mortalidad , Trasplante de Corazón/efectos adversos , Trasplante de Corazón/mortalidad , Humanos , Incidencia , Lactante , Recién Nacido , Infecciones/etiología , Trastornos Linfoproliferativos/etiología , Tasa de Supervivencia , Resultado del Tratamiento
18.
Heart ; 76(2): 173-7, 1996 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-8795483

RESUMEN

OBJECTIVE: To develop a simple and versatile catheter system for complex cardiac catheterisation because angiography and pressure measurements during diagnostic and interventional cardiac catheterisation are often unsatisfactory. METHODS: The Multi-Track Angio catheter system is a single lumen side-hole catheter with a short distal extension containing a lumen for a standard guidewire. The catheter is introduced over a previously placed guidewire running through this distal extension. It can then be manipulated within the heart by sliding along the guidewire. The tip of the catheter is always stabilised by the guidewire. This stability enhances angiography and pressure recordings. RESULTS: The Multi-Track Angio catheter system was used in 84 patients (age 1 day-20 years). Thirty one procedures were diagnostic and 53 interventional. The decision to use the Multi-Track Angio catheter was based on three criteria: firstly, unsatisfactory angiography obtained with conventional equipment; secondly, difficult catheter course requiring use of a guidewire; and thirdly, requirement for angiography and pressure recordings during interventional procedures. No complications were encountered. High quality angiography could be performed in all cases without catheter recoil. CONCLUSIONS: The Multi-Track Angio catheter system allows for high quality angiography and pressure recordings during diagnostic and interventional cardiac catheterisation. The advantage of the system is that both angiography and pressure recordings can be performed repeatedly from stable catheter positions using a previously placed guidewire. This reduces the need for guidewire manipulations or catheter exchanges and decreases procedure time and the risk of complications.


Asunto(s)
Cateterismo Cardíaco/instrumentación , Cardiopatías Congénitas/diagnóstico , Adolescente , Adulto , Niño , Preescolar , Diseño de Equipo , Cardiopatías Congénitas/terapia , Humanos , Lactante , Recién Nacido
19.
Heart ; 76(4): 363-6, 1996 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-8983686

RESUMEN

OBJECTIVE: To assess the value of pulmonary balloon valvuloplasty in the interim palliation of complex congenital heart disease and pulmonary stenosis in children, who often require numerous palliative operations before definitive surgical repair. METHODS: Evaluation of pulmonary balloon valvuloplasty procedures performed over a five year period in 18 patients (age 8 days--29 years; mean 5.5 years) with complex cyanotic congenital heart disease. RESULTS: After pulmonary balloon valvuloplasty oxygen saturation increased from a mean (SD) of 69 (7.5)% to 83 (7.0)% (P < 0.001). Mean pulmonary artery pressure increased from a mean (SD) of 11.3 (3.8) mm Hg to 15.7 (3.9) mm Hg (P < 0.001). Transient complete atrioventricular block occurred in one patient. No other complications were encountered. In 5 patients (28%) there was an inadequate improvement in cyanosis compared with pre-procedure values (72 (4.7)% v 66 (8.1)%). Reasons for failure were increasing infundibular stenosis in three and inadequate mixing in one child. In 13 patients (72%) pulmonary balloon valvuloplasty gave adequate interim palliation over a mean follow up of 1.1 (1.3) years. Oxygen saturation was 81 (5.6)% at last follow up compared with 70 (7.3)% before pulmonary balloon valvuloplasty (P < 0.001). CONCLUSION: Pulmonary balloon valvuloplasty is a safe and effective technique in the palliation of patients with complex cyanotic congenital heart disease associated with pulmonary valve stenosis.


Asunto(s)
Cateterismo , Cardiopatías Congénitas/terapia , Cuidados Paliativos/métodos , Estenosis de la Válvula Pulmonar/terapia , Adolescente , Adulto , Niño , Preescolar , Cianosis/terapia , Estudios de Seguimiento , Cardiopatías Congénitas/sangre , Humanos , Lactante , Recién Nacido , Oxígeno/sangre
20.
Heart ; 76(3): 274-9, 1996 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-8868989

RESUMEN

OBJECTIVE: Concern continues to be expressed about the long-term impact of coronary artery translocation after the arterial switch operation for transposition of the great arteries. This study was conducted to determine the prevalence of obstructions of the translocated coronary arteries by the use of selective coronary artery angiography. METHODS AND RESULTS: 64 children (mean age 7.6 (SD) 1.5 years) who had survived an arterial switch operation underwent evaluation. They had been operated on by one surgeon and they were followed up by a single hospital. Selective coronary artery angiography was possible in 58 patients. Five patients showed occlusion or stenosis of a coronary artery: one occlusion and two stenoses of the left coronary trunk, two occlusions of the circumflex artery. The prevalence of late coronary artery complications was 7.8 (SD) 6.6% (95% CI 1.2 to -14.4%). The three patients with occlusion of one coronary artery had perioperative ischaemic complications, with associated electrocardiogram evidence of ischaemia and left ventricular dysfunction with mitral valve insufficiency. Both patients with stenosis of the left main coronary artery trunk did not have any evidence of an anomaly before catheterisation. CONCLUSIONS: The prevalence of the late coronary artery complications after an arterial switch operation was low in this series. This accords with the view that the arterial switch operation remains the preferred treatment for such patients. Screening for late coronary artery patency should be done by using selective coronary artery angiography, because even patients who remain symptom free can have coronary artery anomalies.


Asunto(s)
Angiografía Coronaria , Enfermedad Coronaria/etiología , Transposición de los Grandes Vasos/cirugía , Enfermedad Coronaria/diagnóstico por imagen , Estudios de Seguimiento , Humanos , Recién Nacido , Transposición de los Grandes Vasos/diagnóstico por imagen
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