RESUMO
OBJECTIVE: Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature. METHODS: Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions. RESULTS: Heart failure occurred in 14 patients, and cyanosis in 3:2 from GB (tetralogy of Fallot--TF, and double outlet right ventricle--DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients. CONCLUSION: APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.
Assuntos
Defeito do Septo Aortopulmonar , Adolescente , Adulto , Defeito do Septo Aortopulmonar/diagnóstico , Defeito do Septo Aortopulmonar/fisiopatologia , Defeito do Septo Aortopulmonar/cirurgia , Criança , Pré-Escolar , Ecocardiografia Doppler em Cores , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
The authors describe the cross-sectional echocardiographic and angiographic features besides the postmortem findings of a newborn with aortic atresia associated with the aortopulmonary window and an interrupted aortic arch, as the third known similar case reported in the literature. Anatomical and clinical similarities with common arterial trunk are emphasized and a more correct management is established to avoid risks, in which Norwood-type techniques would be preferable.
Assuntos
Anormalidades Múltiplas , Aorta/anormalidades , Comunicação Interventricular/complicações , Artéria Pulmonar/anormalidades , Aorta/diagnóstico por imagem , Evolução Fatal , Comunicação Interventricular/diagnóstico por imagem , Humanos , Recém-Nascido , Masculino , Artéria Pulmonar/diagnóstico por imagem , UltrassonografiaRESUMO
A case of pulmonary atresia with intact ventricular septum and a hypoplastic right ventricle, with three wall formed portions, that became naturally well developed until 2 years of age, is related. A Blalock-Taussig shunt had been performed early in life. Z value of tricuspid valve of 0.3 and left ventricular in ECG has been changed to a well developed tricuspid annulus of 22 mm diameter and to a right ventricle overload after 2 years of follow-up, being possible surgical correction at that time. This observation, until today unknown, the natural increase of a congenitally hypoplastic cardiac chamber, favors the possibility to postpone the corrective operation to a better stage in this anomaly, when the risk becomes lower.
Assuntos
Ventrículos do Coração , Atresia Pulmonar/cirurgia , Ecocardiografia , Eletrocardiografia , Feminino , Humanos , Recém-Nascido , Atresia Pulmonar/diagnósticoRESUMO
We analyzed the findings in 23 patients with atresia of the left atrioventricular valve and a patent aorta seen in the period from January 1980 to July 1989. Having divided the cases according to the anatomical findings, we made a subsequent analysis of the clinical and surgical results with the aim of establishing the management most likely to diminish risks, still high, in treatment of this complex anomaly. From the anatomical viewpoint, three variants were observed. In the first, made up of 15 cases, there was absence of the left atrioventricular connexion. The characteristic finding in the second group, with five cases, was an imperforate left atrioventricular valve in the setting of concordant atrioventricular connexions. The third group, of these cases, was dominated by the presence of isomerism of the atrial appendages, both appendages being of left morphology in one case, and of right morphology in the other two. Further anatomical variation was then found in each group. Nine of the 15 with absence of the left atrioventricular connexion had the right atrium connected to a dominant left ventricle in presence of a rudimentary and incomplete right ventricle associated with discordant ventriculo-arterial connexions, all of them being in usual atrial arrangement and three with pulmonary stenosis. The remaining six in this first variant had the right atrium connected to a dominant right ventricle. In the five patients with imperforate left atrioventricular valves, two had discordant and three had concordant ventriculo-arterial connexions. In the three cases with isomerism, two had absence of the left atrioventricular connexion, with a dominant right ventricle. The last patient had an imperforate left atrioventricular valve and a discordant ventriculo-arterial connexion. From the functional viewpoint, there were 14 patients (10 with absence of an atrioventricular connexion, four with imperforate atrioventricular valve) with congestive heart failure and nine patients (five from the first, one from second, and three from the third variant) with hypoxia. Long-term follow-up (median 16.4 months--varying from 1 to 41 months--in the group with congestion and 27.7 months--varying from 12 to 57 months--in those with hypoxia) showed favorable clinical evolution in 11 (91%). We conclude that an anatomico-functional division can point towards the most appropriate management in this complex anomaly.
Assuntos
Aorta/patologia , Valva Mitral/anormalidades , Grau de Desobstrução Vascular , Aorta/fisiopatologia , Cateterismo Cardíaco , Cardiomegalia/fisiopatologia , Criança , Pré-Escolar , Ecocardiografia , Eletrocardiografia , Feminino , Seguimentos , Cardiopatias Congênitas/patologia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/cirurgia , Insuficiência Cardíaca/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Valva Mitral/diagnóstico por imagem , Valva Mitral/patologia , Valva Mitral/fisiopatologia , Valva Mitral/cirurgia , Cuidados Paliativos , Estenose da Valva Pulmonar/patologia , Estenose da Valva Pulmonar/fisiopatologia , Estenose da Valva Pulmonar/cirurgia , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
PURPOSE: To analyse hemodynamic parameters (left ventricles ejection fraction, cardiac index, mean pulmonary wedge pressure and left ventricle diastolic diameter, in a group of children with active myocarditis (diagnosed by endomyocardial biopsy) pre and post treatment with conventional therapy and immunosuppressive drugs (isolated prednisone or prednisone associated with azathioprine or cyclosporine). PATIENTS AND METHODS: Forty-four pediatric patients with active myocarditis were studied. Twenty males and 24 females from 10 months to 15 years old (median = 1.3 years). All patients were submitted to hemodynamic study and endomyocardial biopsy. The hemodynamic parameters mentioned above were analysed before and after the proposed therapy. The patients were distributed in group according to the admission in the protocol, group I (9 pts)--conventional therapy (CT); group II (12 pts)--CT plus prednisone; group III (16 pts)--CT plus prednisone plus azathioprine; group IV (13 pts)--CT plus prednisone plus cyclosporine. RESULTS: Forty-four patients were submitted to four different groups of therapeutic protocol. The hemodynamic parameters were analysed in each of them. Left ventricle ejection fraction were no significantly different in the pre and post therapeutic scheme in group I and II, they were significantly higher (p less than 0.05) in group III and IV. The same happened with cardiac index. The mean pulmonary wedge pressure no presented statistical differences in group I and II before and after treatment but significantly lower lends were observed in group III and IV. The same behavior were noted in the left ventricle end diastolic diameter. CONCLUSION: The association of azathioprine or cyclosporine to prednisone presented better results in the left ventricle function, when compared with conventional drugs or isolated use of prednisone, based in the analysis of same hemodynamic parameters.
Assuntos
Azatioprina/uso terapêutico , Ciclosporinas/uso terapêutico , Hemodinâmica/efeitos dos fármacos , Miocardite/tratamento farmacológico , Prednisona/uso terapêutico , Adolescente , Criança , Pré-Escolar , Quimioterapia Combinada , Feminino , Radioisótopos de Gálio , Humanos , Lactente , Masculino , Miocardite/diagnóstico por imagem , Miocardite/fisiopatologia , CintilografiaRESUMO
PURPOSE: To study the hypoplastic left heart syndrome (HLHS) emphasizing clinical aspects and therapeutic measures. PATIENTS AND METHODS: Forty patients, 24 male, ages ranging between one to 120 days (mean six days). Diagnosis was established by clinical elements, eletrocardiogram, chest X-ray and Doppler echocardiogram. Eight patients underwent cardiac catheterization. In 21 patients anatomical diagnosis was confirmed by necropsy. RESULTS: Twenty-two patients (55%) died, in spite of all possible clinical measures. In 16 (40%) palliative surgery was performed, Norwood technique in 14 and pulmonary artery banding with Dacron graft interposition between pulmonary trunk and brachiocephalic trunk in two. Immediate postoperative results were favorable in three patients (Norwood technique). Both patients underwent the other described technique died at 12th and 30th postoperative day due to infection. CONCLUSION: HLHS is a severe cardiac anomaly with a fast downhill evolution mostly at first week of life. The good results depend basically of early diagnosis as well as of an adequate clinical management for an appropriate time in indication of the palliative surgical technique.
Assuntos
Aorta/anormalidades , Ventrículos do Coração/anormalidades , Aorta/diagnóstico por imagem , Ecocardiografia Doppler , Eletrocardiografia , Feminino , Septos Cardíacos/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Masculino , Cuidados Paliativos , Prognóstico , SíndromeRESUMO
From October 84 to September 88 we performed pulmonary valvuloplasty with balloon catheter (PVBC) in 90 patients (pt) with pulmonary valve stenosis (PVS). The patients were divided in 3 groups (Gr) by their ages: group I (Gr. I) 7 pt (8%) aged 4 days to 11 months, Gr. II 66 pt (73%) aged 1 year to 12 years and Gr. III 17 pt aged 13 to 34 years. This study consists of clinical and echocardiographic evaluation adopting the following parameters of indication: a) transvalvar pulmonary gradient (G); b) absence of dysplasia or hypoplasia of the pulmonary annulus. There were associated lesions which were found in each group: Gr. I atrial septal defect (ASD) in 2 pt and tricuspid insufficiency (TI) in 1 pt, in Gr. II supra valvar stenosis (SVS) in 1 pt and patent arterial ductus (PAD) in 1 pt and in the Gr. III (ASD) in 1 pt. The results after pulmonary valvuloplasty with balloon catheter (PVBC) were: (Gr.I) in 5 pt the G were reduced below 50 mmHg and the average of right ventricular systolic pressure (RVSP) changed from 92 to 62 mmHg and the average of G from 76 to 44 mmHg. One of these pt was submitted to surgery 1 month after the procedure and 2 developed restenosis in 2 and 3 years respectively, being submitted to another dilatation with good results. The 2 pt in which the procedure was not effective were submitted to surgery. In the second group the PVBC was effective in 61 pt (92%) showing the RVSP changed from 118 to 64 mmHg and G changed from 86 to 23 mmHg.(ABSTRACT TRUNCATED AT 250 WORDS)
Assuntos
Cateterismo , Estenose da Valva Pulmonar/terapia , Adolescente , Adulto , Fatores Etários , Criança , Pré-Escolar , Eletrocardiografia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , PrognósticoRESUMO
Central vein cannulation is used as routine for long-time treatment in paediatric intensive care units. The Sylastic catheter is now a days the most common used because of its non-thrombogenic effect. However, in the case here reported, fragmentation during its removal occurred in a eleven month baby. Non surgical removal was done by percutaneous technique using the grasping forceps from Cook Co. into a Lehman 7F catheter. The problem of being no radiopaque, was solved by using the echocardiography study as the guideline for the successful removal of the catheter.
Assuntos
Cateterismo Venoso Central/efeitos adversos , Corpos Estranhos , Veia Cava Inferior , Cateteres de Demora/efeitos adversos , Ecocardiografia , Corpos Estranhos/diagnóstico por imagem , Humanos , Lactente , Masculino , RadiografiaRESUMO
The aortic origin of the right pulmonary artery was recognized in two infants by cross-sectional echocardiography. The sub-costal short axis view was sufficient to make the diagnosis. The Doppler and color-Doppler techniques were useful in adding the hemodynamic information to the anatomical diagnosis. Early surgical correction was performed in the infants with success without needing to resort to cardiac catheterization.
Assuntos
Aorta/anormalidades , Permeabilidade do Canal Arterial/diagnóstico , Ecocardiografia Doppler , Artéria Pulmonar/anormalidades , Aorta/cirurgia , Velocidade do Fluxo Sanguíneo , Permeabilidade do Canal Arterial/cirurgia , Feminino , Seguimentos , Humanos , Lactente , Complicações Pós-Operatórias/diagnóstico , Artéria Pulmonar/cirurgiaRESUMO
Prostaglandin E1, used since 1975, has changed favorably the clinical and surgical prognosis of neonates with congenital heart defects, mainly those with a variety of ductus-dependent defects. Due to recent modifications that have been observed with this drug as far as dosage, side effects, duration of venous infusion and the response of the different cardiac anomalies, this study was undertaken on 47 neonates evaluated with drug infusion between December 1985 and April 1988. The ages of the patients varied from 12 hours to 70 days (median age of 10.3 days), body weight ranged from 1990 to 4430 g (median of 3005 g). The average dose corresponded to 0.021 mcg/kg/min, varying between 0.013 to 0.0089 mcg/kg/min. The therapy was considered effective in 36 (76.5%) patients, evaluated by clinical improvement, increase of arterial oxygen saturation greater than 15 vol. O2% and increased ductus diameter measured by echocardiographic study. In the correlation between the therapeutic result and the patient age, the greatest elevation of arterial oxygen saturation occurred until 21 days of age, especially up to 7 days of age where the elevation in this period was of 24.5 vol. O2%. The cardiac defects that best responded to PGE1 were pulmonary atresia with or without ventricular septal defect, Ebstein's anomaly, tricuspid atresia, pulmonic stenosis, double outlet right ventricle, and those that the arterial oxygen saturation increase was less than 10 vol. O2% such as the hypoplastic left heart syndrome, tetralogy of Fallot and transposition of the great arteries. (ABSTRACT TRUNCATED AT 250 WORDS)