Echocardiographic Assessment of Pulmonary Arteries Pulsatility Index in Fontan Circulation

BACKGROUND: Late complications after Fontan procedure may be due to the absence of pump and pulsatile pulmonary blood flow in this type of palliation. Our aim was to quantify the degree of pulsation by echocardiographic method in patients with extracardiac total cavopulmonary connection (ECTCPC) in comparison with biventricular circulation and few cases of pulsatile Fontan. METHODS: In a case series study, pulsatility index (PI) derived by echocardiographic method were compared between 20 patients with ECTCPC, 6 patients with pulsatile Fontan and 18 normal individual aged 4 to 20 years old. All patients were in New York Heart Association class of I and there was no report of complication. RESULTS: In patients with ECTCPC pulmonary artery branches Doppler flow study showed lower peak and mean velocities compared to the pulsatile Fontan and normal groups. ECTCPC patients had PI of 0.59 +/- 0.14 and 0.59 +/- 0.09 for right and left pulmonary arteries (RPA and LPA) respectively. PI was higher in patients with preserved antegrade flow (RPA PI = 0.94 +/- 0.26, LPA PI = 0.98 +/- 0.27) and in normal individuals (RPA PI = 1.59 +/- 0.12, LPA PI = 1.64 +/- 0.17) for both branches (p = 0.000). CONCLUSION: Using a Doppler derived index for pulsatility, patients with ECTCPC had the least pulsation. The pulmonary artery flow pattern in patients with preserved antegrade flow showed higher pulsatility indices in both branches. Normal individuals had the greatest pulsatility index.

Incidence and etiology of chylothorax after congenital heart surgery in children

Chylothorax is a rare but serious postoperative condition with a high rate of morbidity and may lead to the mortality of children undergoing congenital heart disease [CHD] surgery. This study evaluated the specific surgical procedures associated with the higher risk of postoperative chylothorax. We assessed 435 cases undergoing CHD surgery between April 2003 and May 2006. We detected postoperative chylothorax in 6 patients. The diagnosis of chylothorax was established based on the presence of an odorless fluid with the characteristic milky appearance of the fluid [except when the patients were fasting in the immediate postoperative period], a triglyceride level greater than 110 mg/dL or between 50 and 110 mg/dL with a pleural fluid white cell count greater than 1000, and more than 80% lymphocytes on differential when the pleural fluid was not chylous. Over a 37-month period, 435 [mean age = 51.6 months; 232 males] patients underwent various types of surgical procedures for CHD; 6 patients developed chylothorax after the Fontan operation; one patient died due to severe chylothorax; 3 patients were managed by nutritional modifications, diuretics, and thoracocentesis; and 2 patients required thoracic duct ligation. The Fisher exact test analysis showed a significant association between the Fontan operation and postoperative chylothorax [p value < 0.0001]. Our study showed a significant association between the Fontan surgery and chylothorax

[Long QT in children with congenital deafness: a brief report]

Long QT syndromes [LQT] are genetic abnormalities of ventricular repolarization, with an estimated incidence of about one per 10000 births. It is characterized by prolongation of the QT interval in electrocardiogram [EKG] and associated with a high risk for syncope and sudden death in patients. Type of this syndrome is association with congenital deafness. Our objective was to evaluate QT interval in children with congenital deafness. For 219 patients referred to Imam Khomeini Hospital audiometric clinic in 2011, questionnaire were completed. A total of 23 congenitally deaf children were included. All patients' examinations were done by a pediatric cardiologist. Electrocardiogram is conducted in all children [23 patients] with sever and deep congenital deafness. Then the QT interval was measured based on Bazett's formula. Echocardiography was also performed in these children to assess left ventricular function and the presence of mitral valve prolapse. The overall patients were two hundred and nineteen children. A total of twenty three congenitally deaf children were included and electrocardiogram was obtained. Three children had obviously prolonged QTc [0.48 +/- 0.02] second. The median age of them was 6.1 +/- 5 year, the median weight was 18 +/- 11.3 kilogram and the median of QT interval was 0.48 +/- 0.02 second. The QT interval obtained 0.48 +/- 0.02 second. In the present study we found prolonged QT in congenital deafness, thus we recommend to evaluate the electrocardiogram of children with congenital deafness.

Relationship between Helicobacter pylori infection and serum ferritin level in primary school children in Tehran-Iran

Iron deficiency can cause cognitive and functional learning disorders in children. Some studies have reported a relationship between low serum ferritin levels in patients with anemia and Helicobacter pylori [H. pylori] infection. Therefore, we aimed to determine the relationship between these two common diseases. This descriptive analytical cross-sectional study was performed to assess serum ferritin levels and H. pylori antibody titers [IgG] among 6-12 year old healthy primary school children in Tehran during the academic year 2005-2006. Specimen collection was done by cluster and randomization methods [multistage sampling]. Personal information and laboratory results were compiled in questionnaires and data were analyzed by descriptive and analytical statistics via SPSS software. 165 primary school children [43% boys, 57% girls] with mean age 9.2 +/- 1.5 years were enrolled in the study. H. pylori IgG antibody titer was positive in 26% of cases with mean values of 0.79 +/- 0.42 units in boys and 0.75 +/- 0.39 units in girls, which showed a significant statistical difference [P=0.004]. H. pylori infection was more common among children of large families or those with low economic status [P=0.002]. 29% of children had low serum ferritin levels. Out of the children with low serum ferritin levels, 71% and 28% had negative and positive anti H. pylori antibody titers [IgG levels], respectively. Also, 296 children [25%] with normal ferritin levels had H. pylori infection. We did not find a significant relationship between H. pylori infection and low serum ferritin levels or iron deficiency anemia

Microdeletion study in children with selective congenital heart disease; an Iranian multicenter study

Determining the frequency of chromosome 22q11.2 microdeletion in children with congenital cardiac conotruncal abnormalities using Fluorescence in-situ Hybridization [FISH] technique and estimating relation between DiGeorge Syndrome and cardiac conotruncal abnormalities. One-hundred and eighty cases [106 Males, 74 Females] with selective congenital heart disease [conotruncal abnormalities] referred to the hospitals affiliated to Tehran University during 2004-2007 were evaluated by pediatric cardiologists. All patients were assessed for chromosome 22q11.2 microdeletion using FISH technique. Consequently, patients with 22q microdeletion were studied for T cell abnormalities. Median age of the patients at the time of study was 18 months [3d-16y]. The microdeletion of chromosome 22q11.2 was detected in 17 [9.5%] patients with conotruncal abnormalities, including 5 [29.4%] Tetralogy of Fallot plus Supravalvular Pulmonary Stenosis, 4 [23%] Truncus Arteriosus, 5 [29.4%] Pulmonary Artesia with Ventricular Septal Defect, 2 [11.8%] CO AO+Intrrupted Aortic Arch and one case of Valvular Pulmonary Stenosis. Five of uncorrelated cases had crananiofacial dysmorphism. Chromosome 22q11.2 microdeletion FISH study should be considered in patients with cardiac lesions particularly conotruncal abnormality with or without syndromic problems [craniofacial dysmorphism and developmental delay] to provide an appropriate genetic counseling with more accurate estimation of recurrence risk and ultimately prenatal diagnosis in affected families