[Echocardiographic aspects in pediatric patients with sickle cell disease]. / Aspects échocardiographiques au cours de la drépanocytose en milieu pédiatrique.

BACKGROUND: Cardiovascular involvement is not well studied in children with sickle cell disease. The aim of this study was to evaluate the echocardiographic parameters of children with sickle cell disease. PATIENTS AND METHODS: We performed a transversal and case-control study including 80 subjects of six months to 16 years of age divided into four groups of 20 children each: heterozygous sickle cell disease, homozygous sickle cell anemia, anemia of other causes than sickle cell, and healthy children. All children had a complete physical examination, biological screening including hemogram and hemoglobin electrophoresis, chest x-ray, electrocardiogram, and Doppler echocardiogram. Data were compared using the chi 2 method and the Student's t-test. RESULTS: The mean age was 8.5 years and the sex-ratio was 1. Echocardiographic abnormalities were observed in seven patients with anemia unrelated to sickle cell, 15 children with heterozygous anemia and all the homozygous patients. The main abnormalities were: left ventricular enlargement (ten homozygous patients, one heterozygous subject, five of the patients with another cause of anemia), increased contractility of the heart (18 homozygous patients, seven heterozygous patients, five in the anemia group) and mild to moderate mitral or tricuspid regurgitation (12 homozygous, five heterozygous and five patients in the anemia group). One homozygous child had a dilated and hypokinetic cardiomyopathy with pulmonary hypertension. The parameters of left ventricular systolic function and left heart chamber dimensions were lower in the control group (P < 0.04). DISCUSSION: This study shows the frequency of heart chamber dilatation with left ventricular hyperkinesis usually described in the literature. The lack of case of chronic cor pulmonale may be due to the young age of our patients. One case of dilated and hypokinetic cardiomyopathy suggests that other causes than anemia should be considered, particularly myocardial ischemia, which could not be demonstrated because of our limited investigative methods. CONCLUSION: These results emphasize the frequency of the heart involvement in sickle cell disease, particularly in the homozygous type, and point out the importance of the cardiologic screening of these patients.

[Cri-du-chat syndrome. A case report]. / Syndrome du cri du chat. A propos d'un cas.

We report a documented case in Senegal with cri-du-chat syndrome diagnosed in a 3 months old girl. Our patient benefited from clinical examination, ECG (15 derivations), chest X ray and standard laboratory tests. The cry has been recorded on a magnetic band. We performed also a pulsed-Doppler, two dimensional and TM echocardiography. Chromosomal analysis has been realized. These data are discussed and compared to the literature. At admission this patient presents characteristic cat like cry. At examination, there is a facial dysmorphy, important growth retardation and feeding dyspnea. Auscultation shows a 3/6 left sub-clavicular systolic murmur. Laboratory tests show anemia (hemoglobin = 7.8 g/dl). Chest x-ray showed a cardio-thoracic ratio at 0.61 with increased pulmonary vascular markings. ECG showed right ventricular hypertrophy. Echocardiography-Doppler revealed persistent ductus arteriosus (PDA). Chromosomal analysis shows deletion of the short arm of chromosome 5. After treatment with digitalis and diuretics there was an improvement of cardiac failure. Diagnosis of cri-du-chat syndrome is easy when characteristic cat-like-cry is present. Cardiovascular abnormalities are unfrequent in this syndrome (20% of the cases). They are dominated by ventricular septal defect and PDA. Hemodynamic failure and related growth retardation can lead to cardiac surgery.

[Tetralogy of Fallot. Anatomo-clinical, prognostic and therapeutic features]. / Tétralogie de Fallot. Aspects anatomo-cliniques, pronostiques et thérapeutiques.

Between Feb. 1992 and Aug. 1995 during a prospective study Tetralogy of Fallot (TF) has been diagnosed in 34 children among 207 with congenital heart disease (CHD). Our purpose is to assess prevalence of TF among CHD, to analyse clinical and paraclinical aspects in patients with TF and to point out associated abnormalities in that disease. In all patients diagnosis was made by echocardiography-Doppler (ATL MK 600). In our patients with CHD, TF represent the third abnormality encountered accounting for 16.49% after ventricular septal defect (30.9%) and persistent ductus arteriosus (18.8%). Mean age of patients with TF is 8.36 years. History showed cardiopathy in the family of one patient. The brother had persistent ductus arteriosus. In our patients with TF, clubbing is present in 70.58% of the cases, cyanosis in 79.41% and squatting in 76.47%. Anoxic spells are present in six patients. On chest x-ray mean cardio-thoracic ratio is 0.57 +/- 0.076. On ECG mean QRS axis is +121 +/- 22.91 degrees. In one patient with Cornélia Delange syndrome TF is associated with complete endocardial cushion defect. Mean follow-up is 332,42 days. Endocarditis on the pulmonary valves is present in one case. Four patients died after complications. Because of poor clinical tolerance and complications, surgery is indicated for all our patients. Only 7 patients underwent surgery. Two of them were operated in Dakar. There was two postoperative complications, one patient had brain abscess which necessitate reoperation, and the other had hypertension in the right ventricle and atrial right to left shunt. Our study shows that TF is an important nosologic group. In our country this frequent malformation have a poor prognosis in the majority of cases because late detection and frequent complications. Because palliative surgery for TF is now available in Senegal prognosis of the patients is going to improve. Future Development of open heart surgery will offer a corrective approach for patients with TF in Senegal.

[Complete D-transposition of the great vessels diagnosed late in a 10-month infant. Rashkind manoeuvre]. / D-transposition complète des gros vaisseaux diagnostiquée tardivement chez un nourrisson de deux mois. Manoeuvre de Rashkind.

We report a first documented case in Senegal with simple transposition of the great arteries diagnosed in a 2 months old girl treated by Rashkind atrioseptostomy. Our patient benefited from clinical examination, ECG (15 derivations), chest X ray and standard laboratory tests. Pulsed-Doppler, two dimensional and TM echocardiography have been performed with an ATL MK 600 echocardiograph. Cardiac catheterism, angiocardiography and Rashkind procedure have been realized in our Department. These data are discussed and compared to the literature. At admission this patient presents with major cyanosis and polypnea. At examination, there is a 3/6 murmur at the left sternal border and a subclavicular continuous murmur. Laboratory tests showed metabolic acidosis and severe hypoxemia. Chest x-ray showed a cardio-thoracic ratio at 0.64 with increased pulmonary vascular markings. ECG showed right ventricular hypertrophy. Echocardiography-Doppler revealed ventriculo-arterial discordance with restrictive atrial septal defect and persistent ductus arteriosus. Rashkind procedure was followed by an increased aortic saturation. After 6 weeks there was an improvement of cyanosis and cardiac failure. Diagnosis of transposition of the great arteries is actually easier with development of ultrasonography which is useful when performed by experienced cardiologist. Spontaneous prognosis of this malformation is very poor. Rashkind atrioseptostomy is an important step for the initial treatment of transposition of the great arteries in terms of survival before open heart surgery.