Mahmut Gökdemir

Successful Use of Recombinant Factor VIIa (NovoSeven®) During Cardiac Surgery in a Pediatric Patient with Congenital Factor XI Deficiency

We report our experience with the use of recombinant activated factor VII (rFVIIa) during cardiac surgery in a 4.5-year-old boy with severe congenital FXI deficiency and a congenital heart disease. After weaning the patient from cardiopulmonary bypass, the first intravenous dose of rFVIIa (90 μg/kg) was administered. This same dosage was repeated eight more times, at 2- to 4-hour intervals postoperatively. There was no bleeding during and after surgery. rFVIIa treatment may be used successfully in children with severe FXI deficiency in major operations such as open heart surgery.

Evaluation of right ventricular functions and B-type natriuretic peptide levels by cardiopulmonary exercise test in patients with pulmonary regurgitation after repair of tetralogy of Fallot.

Abstract  Impairment of right ventricular functions, especially due to chronic pulmonary regurgitation (PR), is a well‐known entity in patients with tetralogy of Fallot (ToF) after repair. The aim of this study was to examine the relation between B‐type natriuretic peptide (BNP) levels and right ventricular dysfunction by cardiopulmonary exercise test (ET) in patients after repair of ToF. Twenty‐five patients with a mean age of 14.1 ± 4.4 years at follow‐up who underwent repair of ToF at a mean age of 4.9 ± 5.1 years and 29 age‐ and sex‐matched healthy children at a mean age of 13.1 ± 2.8 years were enrolled in this study. Plasma BNP levels were measured at baseline and at maximal exercise. The volume of right ventricle (RV) and the degree of PR were assessed by two‐dimensional echocardiography and color Doppler. Plasma BNP levels were significantly higher in patients with ToF than in controls (28.3 ± 24.1 vs 7.4 ± 2.3 pg/mL, p = 0.0001). Exercise was associated with increased plasma BNP levels in both groups. A greater increase in BNP was noted in patients with ToF than in controls (37.6 ± 27.5 vs 11.3 ± 4.5 pg/mL, p = 0.0001). Forced vital capacity (FVC%) (84.9 ± 16.9 vs 98.4 ± 18.2, p = 0.01) and forced expiratory volume during the 1st second (FEV1%) (91.5 ± 19.3 vs 103.8 ± 16.1, p = 0.02) were decreased, exercise duration (ED) (10.1 ± 1.9 vs 11.4 ± 1.7 min, p = 0.02), maximum heart rate (HRmax) (171.2 ± 18.9 vs 186.4 ± 13.9 /min, p = 0.004), and maximum oxygen uptake (VO2max) (1.56 ± 0.53 vs 2.1 ± 0.6 L/min, p = 0.007) were lower in patients with ToF. There were significant correlations between the degree of PR and ED (r =−0.3, p = 0.009), HRmax (r =−0.4, p = 0.001), and VO2max (r =−0.4, p = 0.001). The correlations were significant both before and after exercise, being more pronounced after exercise between BNP level and the degree of PR (r = 0.6, p = 0.0001). As a result, the severity of PR has a negative influence on right ventricular functions and there is significant relation between right ventricular functions and exercise capacity after repair of tetralogy of Fallot.

Gaucher Disease with Communicating Hydrocephalus and Cardiac Involvement

A 14‐year‐old female with Gaucher disease presented with hydrocephalus, corneal opacities, cirrhosis, and cardiac valvular involvement. A homozygous D409H mutation was identified. She underwent surgery for aortic and mitral valve replacement. Because of severe calcification of the aortic root, no successful valve replacement was performed. She died on the third day after the explorative cardiac surgery. Cardiac abnormalities represent a life‐threatening presentation of the homozygous D409H mutation. Identification of this type is essential prior to initiating appropriate therapy with enzyme replacement and cardiac corrective surgery. Copyright

Anomalous pulmonary venous connection to the azygous vein: surgical approach of a case with 3D computed tomography findings.

Abstract  Echocardiographic evaluation of anomalous pulmonary venous connection (APVC) may be difficult especially in patients with complex congenital heart diseases. Angiography may also be hazardous in sick patients. For this reason, sometimes we need noninvasive and highly demonstrative procedures for the diagnosis and management of APVC. We have presented a patient, operated with the diagnosis of partial APVC to the azygous vein. In this patient the diagnosis was suspected by echocardiography and confirmed by using three‐dimensional computed tomography with angiography.

Greig syndrome based on a de novo translocation.

In affected patients, chromosomal rearrangements such as translocations and deletions resulted in the mapping of the GCPS gene locus to the short arm of chromosome 7 at p13. 1 – 3 The GLI3 gene was identifi ed as the candidate gene in this syndrome. GLI3 is amplifi ed in certain glioblastomas and encodes a transcription factor and is the member 3 of the GLI-Kruppel gene family (OMIM165240). This zinc fi nger gene has been reported to be responsible for limb development. 4,5 The association of the gene with de novo chromosome translocations involving 7p13 has been reported previously. 2,6

Severe hypernatremia associated catheter malposition in an intensive care patient

We present a catheter related severe hypernatremia in a 2-month-old baby who was admitted to the pediatric intensive care. Imbalance of plasma sodium is commonly seen in pediatric intensive care patients. The water and sodium balance is a complex process. Especially, brain and kidneys are the most important organs that affect the water and sodium balance. Other mechanisms of the cellular structure include osmoreceptors, Na-K ATPase systems, and vasopressin. Hypernatremia is usually an iatrogenic condition in hospitalized patients due to mismanagement of water electrolyte imbalance. Central venous catheterization is frequently used in pediatric intensive care patients. Complications of central venous catheter placement still continue despite the usage of ultrasound guidance. Malposition of central venous catheter in the brain veins should be kept in mind as a rare cause of iatrogenic hypernatremia.

Shunt index can be used to predict clinically significant patent ductus arteriosus in premature neonates in early post-natal life.

BACKGROUND This study aimed to examine the differences between arterial and inferior caval vein oxygen saturation, fractional oxygen extraction, and the shunt index, which were calculated in the diagnosis of patent ductus arteriosus. METHODS Twenty-seven preterm infants were included in this study and were divided into two groups according to patent ductus arteriosus. Among them, 11 (41%) infants had haemodynamically significant patent ductus arteriosus and 16 (59%) did not have significant patent ductus arteriosus. Synchronous arterial and venous blood gases were measured during the first post-natal hours after the insertion of umbilical catheters. The differences between arterial and inferior caval vein oxygen saturation, inferior body fractional oxygen extraction, and the shunt index were calculated. Echocardiography was performed before the 72nd hour of life in a selected group of patients who had haemodynamically significant patent ductus arteriosus. Ibuprofen treatment was administered to patients with patent ductus arteriosus. Echocardiography was performed on the 72nd hour of life in preterm infants without any clinical suspicion of patent ductus arteriosus. RESULTS The early measured differences between arterial and inferior caval vein oxygen saturation and inferior body fractional oxygen extraction were found to be lower and the shunt index was found to be higher in the haemodynamically significant patent ductus arteriosus group than in the group without haemodynamically significant patent ductus arteriosus. CONCLUSION We found that the shunt index, calculated in the first hours of life as ≥63%, predicted haemodynamically significant patent ductus arteriosus with a sensitivity of 78% and specificity of 82% in preterm newborns.