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Biomedical subjects

C Barrea

Publications and source records attributed to C Barrea.

11 recordsLinked to original sources

[Coarctation of the aorta presenting as a pulsatile occipital mass].

A healthy 18 months old boy, is referred to our hospital for a thrilling pulsatile mass behind the left ear. Aortography in the ascending aorta shows a severe coarctation with almost interruption of the aorta between the left carotid and left subclavian artery. Both external carotid arteries provide an important collateral pathway through occipital arteries to two dilated vertebral arteries. Descending aorta is feeding by a reverse blood flow into vertebral and subclavian arteries. The child has been operated. This is an original presentation of severe coarctation of the aorta with development of an important and vital collateral pathway.

Aorta↗

[Risks and benefits of cardiac surgery in Down's syndrome with congenital heart disease].

UNLABELLED: Down's syndrome is the most frequent chromosomic anomaly. Fifty percent are associated with a congenital heart disease. Life expectancy and quality of life are increasing since 15 years with improving surgical techniques. PATIENTS AND METHODS: This study presents 73 patients with Down's syndrome who underwent a surgical repair of congenital heart disease between 1992 and 2002. Among them, 37 (50.6%) had a complete atrio-ventricular septal defect (AVSD) and 36 an other anomaly. RESULTS: Five children died in the post operative period: all had an AVSD. Two late deaths occurred due to extra cardiac causes during the follow up. Two children who underwent a repair of AVSD had to be reoperated. The majority of the children are doing well, without any cardiac symptom. Thirty-eight percent of patients with AVSD repair have a middle mitral or tricuspid regurgitation and three had a massive mitral or tricuspid regurgitation. One child presents neurological sequelae related to surgery. CONCLUSION: Congenital heart diseases in Down's syndrome can be repaired with a limited risk of death. Final results are good for many children with significant improvement of the quality of life and life expectancy.

Cardiac Surgical Procedures↗

[Retro-tracheal left pulmonary artery. Report of 2 cases].

The authors report the cases of two neonates with retro-tracheal left pulmonary arteries (pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardiography showed the vascular anomaly associated with a large ventricular septal defect (VSD). After standard radiological investigation and bronchoscopy to exclude an associated tracheo-bronchial malformation, the VSD was repaired surgically with reimplantation of the left pulmonary artery. Unfortunately, the patient died of major airways obstruction in the postoperative period. Autopsy showed tracheo-bronchial anomalies which had not been diagnosed preoperatively. The second patient presented with hypoventilation of the right lung. After echocardiographic diagnosis of the anomaly, a thorough investigation (thoracic CT, helicoidal scan, bronchoscopy) was carried out and no associated bronchial malformations were observed. Reimplantation of the left pulmonary artery was successful and the postoperative course was uneventful. Retro-tracheal left pulmonary artery is a rare malformation Which is difficult to diagnose. It requires extensive pulmonary investigations and a multi-disciplinary approach. The prognosis is poor when there are associated tracheo-bronchial malformations.

Bronchi↗

Partial and total anomalous pulmonary venous connection in the fetus: two-dimensional and Doppler echocardiographic findings.

OBJECTIVE: Prenatal diagnosis of total (TAPVC) or partial (PAPVC) anomalous pulmonary venous connection in isolation or associated with other cardiac disease is important for appropriate prenatal counseling and perinatal management. We sought to assess the echocardiographic clues to the fetal diagnosis of TAPVC and PAPVC in a cohort of affected fetuses. METHODS: We retrospectively reviewed 29 fetal echocardiograms performed in 16 pregnancies with fetal TAPVC or PAPVC, systematically analyzing heart chamber size, presence of a confluence behind the left atrium or of a vertical vein, and Doppler flow patterns. RESULTS: Prenatal diagnosis was made at a mean gestational age of 27 +/- 7 weeks. TAPVC was found in 11 cases; five cases for each of supracardiac and infracardiac types and one mixed type. PAPVC was diagnosed in five fetuses, four of which had scimitar syndrome. Ten fetuses had an additional major cardiac defect, including hypoplastic left heart syndrome and right atrial isomerism. In three cases the prenatal diagnosis was only made at follow-up assessment. Among TAPVC cases, visualization of a confluence behind the left atrium (10/11) and a vertical vein (11/11) were the most consistent echocardiographic clues. Dextrocardia and a small right pulmonary artery suggested scimitar syndrome. The diagnosis was confirmed postnatally or at autopsy in 12 cases. In six fetuses with TAPVC and obstruction confirmed postnatally, continuous turbulent flow in the vertical vein and monophasic continuous flow in the pulmonary veins were demonstrated by color and spectral Doppler. CONCLUSIONS: Fetal echocardiography permits prenatal diagnosis of TAPVC or PAPVC. Spectral and color Doppler provide clues to the presence of an obstructed pulmonary venous pathway.

Cohort Studies↗

Elevated right ventricular pressures are not a contraindication to liver transplantation in Alagille syndrome.

BACKGROUND: Elevated right ventricle pressure resulting from pulmonary artery stenoses may affect outcome and survival after liver transplantation in patients with Alagille syndrome. METHODS AND RESULTS: Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for Alagille syndrome (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable pruritus, and failure to thrive. All patients had pulmonary artery stenosis. In 10 patients considered to have elevated RV pressure on ECG and/or Doppler-echocardiography, a cardiac catheterization was performed before liver transplantation. Mean RV systolic pressure was 55 mmHg (median 49.5 mmHg, range 35-98 mm Hg), mean RV to left ventricular systolic pressure ratio 0.53 (median 0.53, range 0.29-0.78) with a ratio above 0.5 in 6 patients (median 0.66, range 0.5-0.8). All patients underwent successful liver transplantation. Five patients died 1 to 9 months after transplantation from noncardiac causes. In two of them, cardiac catheterization before transplantation showed a RV to left ventricular pressure ratio of 0.51 in one and 0.37 in the second. In the three others, echocardiography before transplantation estimated RV pressures below 0.5 systemic pressures. At follow-up (median 6 years, range 1.5-15 years), liver tests were normal in all, none complained of pruritus and body weight was normalized in 70%. None of the patients presented cardiac symptoms, arrhythmias, or worsening of their cardiac status. CONCLUSIONS: Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pressure.

Adolescent↗

Diagnosis, natural history, and outcome of fetal heart disease.

Advances in the prenatal diagnosis of heart disease have improved accuracy in the evaluation of structural heart lesions, dysrhythmia mechanisms, and functional pathology and will lead to better perinatal management and counseling. Additional technical advances will lead to earlier diagnosis, perhaps even during embryonic development. Newer developments, including the transumbilical and transuterine approaches, may make antenatal intervention possible and safer for a larger number of affected pregnancies.

Disease Progression↗

Changes in perceived health of children with congenital heart disease after attending a special sports camp.

Sports camps for children with cardiac anomalies have existed for many years. However, no formal evaluation of the benefits of attending such camps has been undertaken heretofore. We assessed potential changes in the self-perceived health of children with congenital heart disease who attended a special sports camp. Thirty-one children with cardiac anomalies attended a 3-day multisports camp. Sixteen children, all of whom were 10 years or older, literate, and Dutch- or French-speaking, completed the Child Health Questionnaire (CHQ-CF87) before and after attending the camp. The scores of the children were compared with those of healthy peers by calculating mean standardized differences. After attendance at the sports camp, the children achieved significant improvements in the self-perception of their physical functioning, role functioning due to emotional problems, role functioning due to behavioral problems, mental health, and general behavior. The children's self-esteem and general behavior after the camp were significantly better than that of their healthy counterparts. We conclude that children with congenital heart disease who participate in activities at special sports camps may reap benefits in terms of their subjective health status. Although further research is needed, we recommend the participation in sport activities by children with heart defects, and more specifically their participation in sports camps.

Adolescent↗