Right atrial and tricuspid hypoplasia.

Cor triatriatum dexter is a rare cardiac anomaly with division between the sinus and primitive atrial portions of the right atrium. It is supposed that the failure of the venous valves to regress appropriately may create abnormalities in fetal circulation predisposing the fetus to maldevelopment of the right heart structures. The underdevelopment of the right ventricle with hypoplasia or atresia of the tricuspid valve is a well known abnormality, but underdevelopment of the right atrium with tricuspid hypoplasia due to multiple venous valves is extremely rare. This is a case of complex type of cor triatriatum dexter in which the right atrium is hypoplastic and like a tube formed by the superior and inferior vena cava and is divided into 3 portions by 2 membranes.

Right iliac vein agenesis, varicosities, and widespread hemangiomas: report of a rare case.

We present a probable variant of the Klippel-Trénaunay syndrome with the clinical features of capillary hemangiomas, varicosities, and agenesis of the right iliac venous system, but without limb hypertrophy. To our knowledge, this is the 1st such case reported in the medical literature.

The effect of etomidate induction on plasma cortisol levels in children undergoing cardiac surgery.

OBJECTIVE: To investigate the effect of a single induction dose of etomidate on plasma cortisol and adrenocorticotrophic hormone (ACTH) levels in pediatric patients with congenital heart disease. DESIGN: A prospective, randomized study. SETTING: A university hospital. PARTICIPANTS: Thirty children undergoing cardiopulmonary bypass. INTERVENTIONS: Patients were randomly allocated into two groups to receive etomidate, 0.3 mg/kg, and fentanyl, 1 microg/kg, or ketamine, 1 mg/kg, and fentanyl, 1 microg/kg intravenously, for anesthesia induction. Anesthesia was maintained with 50% nitrous oxide and 0.5% isoflurane in oxygen. Plasma cortisol and ACTH levels were measured on five occasions: preoperatively, after induction of anesthesia, after cross-clamping, at the end of surgery, and 24 hours postoperatively. MEASUREMENTS AND MAIN RESULTS: Plasma cortisol levels of the etomidate group decreased with anesthesia induction and remained significantly low during cardiopulmonary bypass, at the end of operation, and 24 hours postoperatively. Plasma cortisol levels of the etomidate group after the anesthesia induction, during cardiopulmonary bypass, and at the end of the operation were significantly lower than the ketamine group. CONCLUSION: These results show that etomidate is a suitable agent for suppressing the increase in cortisol levels associated with the stress response caused by cardiopulmonary bypass in children with congenital heart defects, and can be used safely.

Spontaneous hemothorax in a hemodialysis patient.

An 8-year-old girl who had undergone chronic hemodialysis for 1 year presented with respiratory distress 24 h after a hemodialysis session. She had a massive pleural effusion of the left chest that was shown to be hemothorax by thoracentesis. After chest tube insertion, drainage was maintained for 2 days. Pleural effusion recurred after withdrawal of the chest tube. The antecubital arteriovenous fistula on the left arm was found to have an excessive flow with a thrill which was felt all over her left shoulder and left hemithorax. The pleural effusion resolved spontaneously a week after ligation of the fistula. Excessive flow in the arteriovenous fistula was thought to be the cause of the hemothorax and should be included in the differential diagnosis of hemothorax in hemodialysis patients.

Incidence and severity of arrhythmias and conduction disturbance after repair of tetralogy of Fallot.

Ventricular and supraventricular arrhythmias and conduction disturbances were evaluated by routine electrocardiography and 24-hour ambulatory monitoring in 31 patients who underwent correction of tetralogy of Fallot. The interval from operation to the study was 1 month to 14 years (mean 4.8 +/- 2.8). Complete right bundle branch block occurred in 22 (71%) patients and incomplete right bundle branch block in 9 (29%) patients. Bifascicular block with right bundle branch block and left axis deviation (LAD) occurred in one patient. Two patients had second degree type II atrioventricular block. Twenty-four-hour ambulatory electrocardiographic monitoring was performed in all patients and they were divided in two groups according to the frequency of ventricular arrhythmias (Lown classification). Group 1 included the 23 patients who had no arrhythmia or rare ventricular arrhythmias (Lown grade 0-1). Group 2 was comprised of eight patients (26%) with significant ventricular arrhythmias (Lown grade 2-5). Twelve patients (39%) had supraventricular arrhythmias, three patients rare supraventricular tachycardia attacks, and seven patients occasional supraventricular ectopies. One patient had bradycardia-tachycardia attacks and one patient had junctional tachycardia. There was no correlation between age at the time of surgery and ventricular arrhythmias. Of the patients who had ventricular and supraventricular arrhythmias of various degrees on ambulatory monitoring, two had significant arrhythmias on routine electrocardiogram. Symptoms were rare in these patients. In conclusion, both supraventricular and ventricular arrhythmias were found in considerable frequency in our patients. As ventricular arrhythmias may be the cause of sudden death and supraventricular arrhythmias are a main cause of morbidity, it is important to evaluate ventricular and supraventricular arrhythmias by ambulatory monitoring in patients who have undergone correction of tetralogy of Fallot.

Changes in the serum levels of CK-MB, LDH, LDH1, SGOT and myoglobin due to cardiac surgery.

Elevation of serum levels of enzymes due to myocardial necrosis is considered to be a specific finding for myocardial infarction. This study was designed to determine the changes in the levels of LDH, SGOT enzymes, LDH1, CK-MB isoenzymes and myoglobin in patients who have had coronary bypass grafting and who have had atriotomy for surgical correction without any evidence of myocardial infarction diagnosed by clinical, hemodynamic and electrocardiographic findings. It was found that CK-MB, LDH, LDH1 and myoglobin showed significant elevations which mimicked a perioperative myocardial infarction. SGOT also increased, but remained within the normal range. CK-MB, LDH and LDH1 increased more in the coronary bypass grafted patients whereas myoglobin increased in the patients who had atriotomies. It was concluded that in order to demonstrate a perioperative myocardial infarction it is necessary to determine at least two of these enzymes for a long period and to correlate these results with the clinical, hemodynamic and electrocardiographic findings.

Mediastinitis after open heart surgery. Analysis of risk factors and management.

Mediastinitis developed following 58 of 8803 operative procedures involving median sternotomy at Türkiye Yüksek Ihtisas Hospital. There were 40 men and 18 women. Mediastinitis occurred within, on average, 10 days after the operation. No positive cultures were obtained in 37 patients; Staphylococcus aureus was the responsible pathogen in 18, Escherichia coli in 2, and Enterococcus in 1 patients. Thirty patients had a valve procedure, 24 aortocoronary bypass grafting, 1 a total correction for tetralogy of Fallot, 1 a primary closure of an atrial septal defect, and 2 had Bentall's procedure for an ascending aortic aneurysm. Fifty patients were treated by radical debridement and closed irrigation of the mediastinum with antibiotic solutions and 8 patients were treated with sternal debridement and transposition of the omentum. The mortality rate was 36% for the former, and 12.5% for the latter technique. Omentum transposition promoted rapid healing with a low mortality rate.

Complement activation during cardiopulmonary bypass.

We researched complement activation of fifteen patients who had open heart surgery and on ten patients who had closed heart surgery. Our results showed that the complement system was partially activated by the anaesthesia and partially by tissue damage. This activation was aggravated when plasma contacted the pump-oxygenator system, continued in the intensive-care unit and became normal in the 24th hour after the operation. Complement activation occurred both via the alternative and classical pathways but the alternative pathway was activated more than the classical with increase in bypass time. Pulmonary sequestration of leucocytes which occurred due to the complement activation and the complement derived inflammatory mediators could have contributed to the pathogenesis of the post-pump syndrome.

Radial ray defect with vascular and vertebral anomalies.

A new case with combined intercalary radial ray defect, high origin of the radial and ulnar arteries, and anomalies of the thoracic vertebrae is presented. The diagnosis was based on roentgenography, arteriography, and electroneuromyography. The case was regarded as a thoracic outlet syndrome due to the first rib abnormality and was treated surgically.

A case of ulnar aneurysm. Clinical and surgical considerations.

Although ulnar artery aneurysms have been known since the 17th century, fewer than 100 cases have been reported. Because of the rarity of this condition, a case of traumatic ulnar artery aneurysm which was operated on in our department is presented and the clinical findings, diagnosis and surgical implications are considered.

Hemoglobin S and some other hemoglobinopathies in Eti-Turks.

Frequencies of various hemoglobinopathies were examined in a total of 1,922 individuals of Eti-Turk origin by electrophoretical techniques. Hemoglobin A2 (Hb A2) and hemoglobin F (Hb F) determinations were also performed in 651 and 1,642 cases, respectively. Mean hemoglobin S (Hb S) frequency was found to be 15.3%. Variations among the different age groups were insignificant. Hemoglobin E (Hb E) and beta-thalassemia frequencies wer 0.47 and 1.23%, respectively. Hemoglobin Hacettepe and hemoglobin D were found once. Red cell glucose-6-phosphate dehydrogenase (G6PD) deficiency was found in 6.5% of males.