Quadricuspid aortic valve and atrial septal defect.
Explore the source record for details and available documents.
Abnormalities, Multiple↗
Biomedical subjects
G Nogueira
Publications and source records attributed to G Nogueira.
Idiopathic dilated cardiomyopathy in children: clinical profile and prognostic determinants.
Idiopathic dilated cardiomyopathy is a severe disease with a high mortality rate in childhood. Its clinical evolution and prognosis are important for the selection of cardiac transplantation candidates. In order to characterize its evolution and identify prognostic factors, the clinical records of 41 children with the diagnosis of idiopathic dilated cardiomyopathy, admitted from January 1985 to December 1997, were reviewed. Survivors (Group I) and deceased (Group II) were separately analyzed, according to the following parameters: age, sex, race, clinical severity, electrocardiographic, echocardiographic and haemodynamic findings. Seven children were excluded from the study: six of them were lost to follow-up and one died from a surgical complication. Of the remaining 34 children, 20 were male (M) and 14 were female (F) (M/F: 1.4). Age range at diagnosis was 7 days to 14 years (median: 1.5 years), and follow-up time was from 18 days to 10.5 years (median: 2.5 years). Eleven (32.3%) children fully recovered, 13 (38.2%) survived with left ventricular dysfunction, and ten (29.4%) died, half of them within the first three months of follow-up. Mortality was 23.5% (8 out of 34 children) during the first year of follow-up and 29.4% (ten out of 34 children) at five years. Unfavorable prognosis was more frequently associated to: 1) clinical severity at the time of presentation; 2) lower mean left ventricular shortening fraction (10 +/- 7% in group II and 13 +/- 5% in group I); 3) occurrence of severe arrhythmia (40% in group II and 3.5% in group I). In this series a group of higher mortality risk was identified, based on some of the analyzed parameters, which should be considered as selection criteria for early heart transplantation.
Adolescent↗
Intramyocardial tumors.
Explore the source record for details and available documents.
Child, Preschool↗
The deletions of 22q11--the Portuguese experience.
The patients with a chromosome 22q11 deletion have a variable phenotype which includes DiGeorge (DG) and Velocardiofacial (VCF) syndromes. The aim of the present study is to characterize the phenotype of DG and VCF using facial biometry in 12 portuguese patients. We found 4/12 patients with the DG phenotype: 3/4 had telecanthus, small mouth and retrognathia; 1/4 had telecanthus, short nose with bulbous tip and a normal mouth. These patients had major cardiac defects associated with hypoplastic or absent thymus and monosomy 22q11. We did not find velopharyngeal insufficiency in patients with the so called DG phenotype 8/12 patients had the VCF phenotype: typical facies with variable features. Four of these had velopharyngeal insufficiency and learning disabilities. Four patients had cardiac defects and 5/8 had monosomy 22q11. Probably this clinical variability is due to mutations in critical genes involved in embryonic development.
Adolescent↗
[Cardiovascular morbidity in children with human immunodeficiency virus infection].
UNLABELLED: Abnormalities of cardiovascular structure and function have been described among children with human immunodeficiency virus (HIV). In order to evaluate their occurrence and clinical predictors, 32 children infected with HIV, aged three months to 13 years (mean age = 3.11 +/- 3.51 years) were studied. Perinatal transmission was identified in 90% of the children. Twenty-two patients (pts) (69%) had symptoms, nine being moderately symptomatic and eight severely symptomatic. Fourteen pts had immunological disturbances and eight of them were severely immunosuppressed. Twenty-eight pts (88%) had HIV-1 infection and 6 recent Ebstein-Barr virus coinfection. Nineteen were on zidovudine and 14 on intravenous immunoglobulin treatment. Nineteen cardiovascular abnormalities were found in 15 pts (47%): 11 cases of pulmonary hypertension by echocardiographic criteria (eight of them had interstitial lung infiltrates seen on chest X-ray) and four cases of left ventricular dysfunction requiring anticongestive therapy. Other abnormalities were: patent ductus arteriosus, septal hypertrophy, mitral valve prolapse and pericardial effusion (one case each). Surface ECG displayed right ventricular hypertrophy in four pts, left ventricular hypertrophy in one patient and unspecific ST-T wave changes in two pts. Fourteen pts (44%) had sinus tachycardia with mean heart rate above the 95th percentile on 24-hour Holter monitoring. CONCLUSIONS: Cardiovascular abnormalities are frequent among children with HIV-1 infection in late stages; pulmonary hypertension is the most frequently found cardiovascular anomaly and seems to be related to either chronic or recurrent respiratory disease; cardiological follow-up is recommended for HIV-infected children.
AIDS-Related Opportunistic Infections↗
[Bladder malfunction, urinary tract infection and vesicoureteral reflux in children].
A correlation between urinary tract infection, vesicoureteral reflux and voiding disorders has increasingly been reported. Voiding dysfunction increases the incidence of recurrent urinary tract infection, induces and perpetuates vesicoureteral reflux, even after surgical antireflux treatment, and may result in permanent renal damage. The resolution of the primary cause with voiding normalization is essential to achieve good results in the treatment of secondary problems such as urinary tract infection and vesicoureteral reflux. Thirty seven children with vesicoureteral reflux secondary to voiding disorders were diagnosed and treated between 1990 and 1995 (five years). Forty-nine ureters were studied. The subjects became symptomatic between 1 month and 13 years of age, with the occurrence of urinary tract infection. All children were neurologically and morphologically normal. Symptoms suggesting bladder instability were detected in 34 (91.9%) and dysfunctional sphincter obstruction in three (8.1%). These patients were all evaluated with a renal/bladder sonogram and voiding cystogram, complemented in 17 (45.9%) with urodynamic testing that confirmed clinical diagnosis. 99mTc-dimercaptosuccinic acid renal scans performed on 29 (78.4%) children revealed renal damage in 26 (89.6%). A treatment program of bladder retraining and bowel habit normalization was encouraged in every child, anti-cholinergic drugs were associated in 23 (62.2%), muscle-relaxant drugs in three (8.1%), phenoxybenzamine and intermittent catheterization were used in one child (2.7%). Urinary tract infection prophylaxis was instituted in 34 (91.9%) children. Urinary tract infection was completely resolved in 35 (94.6%) patients, and its frequency decreased in two (5.4%). Thirty-two children (86.5%) with vesicoureteral reflux were cured and four (10.8%) were improved. Evidence of voiding disfunction ceased in 22 (59.5%) cases and improved in 14 (37.8%) with a reduction in the frequency and intensity of complaints. Urgency syndrome and vesicoureteral reflux remained unchanged in one child (2.7%). These findings imply that detection and treatment of bladder/sphincter disfunction are essential in every child with the complex of recurrent urinary tract infection and vesicoureteral reflux.
Adolescent↗
Creutzfeldt-Jakob disease in Argentina.
Explore the source record for details and available documents.
Aged↗
[Surgical management of atrio-ventricular septal defects: a single-institutional experience].
OBJECTIVES: Atrio-ventricular septal (AVSD) defects include a variable spectrum of congenital malformations with different forms of clinical presentation. We report the surgical results, from a single institution, with this type of congenital cardiac malformation. Patients with hypoplasia of one of the ventricles were excluded from this analysis. POPULATION: Between November of 1998 and June of 2005, 49 patients with AVSD were operated on by the same team and in the same department. The average age was 37.3 months (medium 6 months) and 31 patients were female. In 38 patients (78%) an inter-ventricular communication was present (AVSD-complete) and of these, 26 were of the type A of Rastelli, being 13 of type B or C. The age for defect correction of the complete form was of 5.5 months, palliative surgery was not carried out on any of the patients. Associated lesions included: Down's syndrome in 22 patients (45%), patent arterial duct in 17 patients (35%), severe AV regurgitation in 4 patients (8%), tetralogy of Fallot in two (4%) and sub-aortic stenosis in one patient (2%). Pre-operatively 10 patients presented severe congestive heart failure and two were mechanically ventilated. RESULTS: Complete biventricular correction was carried out in all patients. The average time on bypass (ECC) was 74.1+/-17.5 min. and time of aortic clamping was 52.0+/-12.9 min. The complete defects were corrected by the double patch technique, and in all patients the mitral cleft was closed, except in two with single papillary muscle. There was no intra-operative mortality, but hospital mortality was 8%(4 patients), due to pulmonary hypertension crises, in the first 15 post-operative days. The mean ventilation time was of 36.5+/-93 hours (medium 7 h) and the average ICU stay was of 4.3+/-4.8 days (medium 3 days). The minimum follow-up period is 1 month and the maximum is 84 months (medium 29.5 months), during which time 4 re-operations (8%) took place: two for residual VSD's and two for mitral regurgitation. There was no mortality at re-do surgery. At follow up there was residual mitral regurgitation, mild in 17 patients and moderate in two. Four other patients presented with minor residual defects. CONCLUSIONS: The complete correction of AVSD can be carried out with acceptable results, in a varied spectrum of anatomic forms and of clinical severity. Despite the age of correction, for the complete forms, predominantly below 12 months, pulmonary hypertension was the constant cause for post operative mortality. Earlier timing of surgery and stricter peri-operative control might still improve results.
Child, Preschool↗
[Ross procedure: mid term results].
UNLABELLED: The Ross procedure has been used in children and young adults for aortic valve replacement and the correction of complex obstruction syndromes of the left ventricular outflow tract. We report the mid-term results of the Ross procedure in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and December 2005, 18 patients were operated on using the Ross procedure. The mean age at the time of surgery was 12 years, being 12 patients male (67%). The primary indication for surgery was isolated aortic valve disease, being the predominant abnormality in 58% of cases aortic regurgitation and in 42% left ventricular outflow tract obstruction. Associated lesions included sub-aortic membrane in 3 patients (16%), small VSD in 2 patients (11%), bicuspid aortic valve in 4 patients (22%) and severe left ventricular dysfunction and mitral valve regurgitation in 1 patient (6%). Ten of the 18 patients (56%) had been submitted to previous surgical procedures or percutaneous interventions. RESULTS: Early post-operative mortality was not seen, but two patients (11%), had late deaths, one due to endocarditis, a year after the Ross procedure, and the other due to dilated cardiomiopathy and mitral regurgitation. The shortest time of follow-up is 6 months and the longest 72 months (median 38 months). Of the 16 survivors, 14 patients are in class I of the NYHA and 2 in class II, without significant residual lesions or need for re-intervention. The 12 patients with more than a year of follow up revealed normal coronary perfusion in all patients and no segmental wall motion abnormalities. Nevertheless, two of the 12 patients developed residual dynamic obstruction of LVOT and in three patients aortic regurgitation of a mild to moderate degree was evident. Significant gradients were not verified in the RVOT. CONCLUSIONS: The Ross procedure, despite its complexity, can be undertaken with excellent immediate results. Aspects such as the dilation of the neo aortic root and homograft evolution can not be considered in a study of this nature, seeing that the mean follow up time does not exceed 5 years.
Adolescent↗
[Ventricular assist in children].
We describe the use of the Berlin Heart biventricular mechanical assistance device, as a bridge to transplant in a two-years old child suffering from end stage dilated cardiomiopathy. Ventricular support lasted for 3,5 months and led to successful transplantation. We describe the clinical case, the protocols used as well as present indications, techniques and problems related to the use of mechanical heart support in children.
Cardiomyopathy, Dilated↗
[Pulmonary ventricle bypass operations].
UNLABELLED: Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.
Adolescent↗