Complete heart block in thalassemia major: A case report

PubMedID: 12405443Cardiac complications of iron overload are the most common cause of death in patients with thalassemia major. These complications include recurrent pericarditis, refractory congestive heart failure and rhythm disorders. The usual rhythm disturbances are supraventricular or ventricular premature contractions and first-or second-degree heart block. Complete heart block is a very rare complication of thalassemia major. Herein, we report a case of complete heart block with thalassemia major. The patient also had serious congestive heart failure. Management of the heart block with pacemaker brought no clinical improvement, and she died in the second month of hospitalization

A rare cause of thrombocytopenia in infants: Vitamin B12 deficiency

Thrombocytopenia is the reduction of platelet number to below normal level. It may be due to the decrease in production, increase in destruction, abnormal spreading in the body or the laboratory errors. B12 deficiency is a rarely seen vitamin deficiency in childhood. It presents with megaloblastic anemia and is difficult to diagnose because of various clinical findings. Here we would like to present two cases which were hospitalized to investigate the etiology of anemia and thrombocytopenia. Cases were detected to have vitamin B12 deficiency which is rarely seen in infantile period. A 12-month old male and a 16-month old female admitted with complaints of weakness, bruise and pallor. On physical examination, there was tremor of upper extremities. Anemia, thrombocytopenia and in peripheral blood macrocytes were detected as laboratory findings. B12 vitamins were found to be low. Patients recovered clinically after parenteral vitamin B12 administration. Laboratory findings also changed into normal

Temporary atrioventricular complete block that develops following the transcatheter closure of ventricular septal defect

PubMedID: 22397051Atrioventricular (AV) block is a potential risk after transcatheter closure of perimembranous ventricular septal defect (VSD) with the Amplatzer perimembranous device. We present herein a case of a 6.5-year-old female patient who developed complete AV block six days after closure of VSD and recovered with steroid and salicylate treatment. It is important to be alert to the development of AV block after transcatheter perimembranous VSD closure. Very careful monitoring of rhythm is mandatory during the short- and long-term follow-up

Long-term follow-up of 799 children with isolated ventricular septal defects [İzole ventriküler septal defektli 799 çocugun uzun süreli takibi]

PubMedID: 22395370Objectives: We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). Study design: The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). Results: The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. Conclusion: Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients'age, defect type, and complications encountered in the follow-up period. © 2012 Türk Kardiyoloji Dernegi

A newborn infant with sternal malformation/vascular dysplasia association

PubMedID: 12017236Sternal malformation/vascular dysplasia complex was described by Hersch et al. in 1985. The principle findings include cleft of the sternum covered by an atrophic skin, a midline abdominal raphe and hemangiomatosis. The inheritance pattern seems to be sporadic. We report a newborn baby with sternal defect, cleft lip and palate, supraumbilical raphe and hemangiomas

Mid-term results of patients following total surgical correction of tetralogy of Fallot

PubMedID: 23692721The aim of this study was to evaluate the mid-term clinical status of patients following tetralogy of Fallot (TOF) repair. We performed a cross-sectional observational analysis of 57 postoperative TOF patients and 58 age-matched controls. Patients were examined with myocardial performance index (MPI), which was obtained by tissue Doppler imaging (TDI) in addition to the conventional methods. Compared with controls, patients had significantly higher right ventricular (RV) dimensions and volumes, RV MPI and left ventricular (LV) MPI, and significantly lower RV ejection fraction (EF), LV E F, exercise duration, and maximum heart rate with exercise (p<0.001 for all parameters). A positive correlation was found between RV MPI and LV MPI (r=0.541, p<0.001). There was a negative correlation between LV MPI and exercise duration (r=0.260, p=0.034). After total surgical correction of TOF, the patients had a good clinical status at the mid-term follow-up; decreased exercise capacity and impaired functions in both ventricles related to the degree of pulmonary regurgitation were found. By using MPI, impaired cardiac functions can identify such conditions before they become clinically symptomatic

Pneumomediastinum and pneumopericardium: Unusual and rare complications of asthma in a 4 years old girl

PubMedID: 11449532We describe a 4-year-old girl with asthma who presented with pneumomediastinum, pneumopericardium and subcutaneous emphysema. She was admitted to our hospital with dyspnea, chest pain, palpitation and cough of two days duration. She had attacks of cough, dyspnea and wheezing from two years of age, but she did not have a diagnosis of asthma previously. She was dyspneic and had subcutaneous emphysema in the neck, axilla and thorax. In the skin prick test (Center Lab. USA) she had positive reaction to Dermatophagoides pteronyssinus, Dermatophagoides farinae, mold mix, tree mix and grass mix. Pulmonary function tests could not be performed. In the chest X-ray air was seen in mediastinum and subcutaneous area and the epicardium was surrounded completely with air. She was treated successfully with inhaled salbutamol and budesonide. Radiological signs of pneumopericardium and pneumomediastinum disappeared completely in ten days period. In the light of this case we want to mention that early diagnosis and treatment of asthma should be done to prevent serious complication of asthma

Scimitar syndrome: Four cases with different clinical presentations [Farkli klinik prezentasyonlarla scimitar sendromu: Dört vaka takdimi]

The scimitar syndrome is a rare congenital cardiopulmonary anomaly that consists in part of total or partial anomalous venous drainage of the right lung to the inferior vena cava (IVC), lung hypoplasia and anomalous systemic arterial supply to the lung. We present four pediatric patients with different clinical findings

Right pulmonary artery - Left atrial communication presenting with brain abscess: A case report

PubMedID: 17290577Direct communication between the right pulmonary artery and left atrium is a very rare vascular malformation. We report a patient with this anomaly. She presented with unexplained cyanosis and brain abscesses. The diagnosis was made with contrast echocardiography and angiography. We treated this anomaly successfully with surgery. Complete cure for this anomaly can be achieved by ligation

Is oral indomethacin effective in treatment of preterm infants with patent ductus arteriosus?

PubMedID: 15214742Twenty-one preterm infants (with a mean gestational age and birth weight of 29.3 weeks and 1288.6 g) and nine pretem infants (with a mean gestational age and birth weight of 29.6 weeks and 1153.1 g) were treated with an enteral preparation of indomethacin and with intravenous indomethacin, respectively, for the closure of hemodynamically significant ductus arteriosus. The patients received three doses of either oral indomethacin capsule (Endol, Deva, Turkey) or intravenous indomethacin (Confortid, Dumex GmBH, Germany) in a dose of 0.2 mg/kg at 12-hour intervals. The ductus was closed in 17 (81%) and 7 (77%) of the babies in the orally and intravenously treated groups, respectively (p>0.05). There was no significant difference in blood urea nitrogen, creatinine levels or thrombocyte counts in either group before and after treatment with indomethacin (p>0.05). No side effect was reported in the oral indomethacin group. Oral indomethacin may be an alternative to the intravenous preparation in developing countries if the intravenous form is not available or not affordable