Influence of umbilical cord clamping time on cerebral oxygenation and early cardiac function in term infants.

BACKGROUND: Delayed cord clamping is the standard of care for both term and preterm infants worldwide. The aim of this study was to evaluate the effect of 60-second or 180-second delayed cord clamping during labor on cerebral oxygenation and cardiac function. METHODS: Healthy newborns were divided into two groups: a 60-second delay in cord clamping (60-s DCC) and a 180-second delay in cord clamping (180-s DCC) at birth. Pulse oximetry and cerebral near-infrared spectroscopy (cNIRS) probes were placed during postnatal care. A total of 84 healthy newborns were included in this study. Preductal oxygen saturation (SpO2), heart rate, and cNIRS values were recorded at 5 and 10 minutes after delivery. The cardiac function of the infants was assessed by echocardiography at 3-7 days postnatally. RESULTS: There was no significant difference between the groups in SpO2 and cNIRS values at 5 and 10 min. While there was no significant difference in the number of neonates with targeted SpO2 at the 5th and 10th min and targeted cNIRS values at the 10th min, there was a significant difference in the number of neonates with target cNIRS values at the 5th min between groups (p <  0.001). Echocardiographic findings showed that pulmonary flow velocity was increased in the 180-s DCC group; the difference was statistically significant (p = 0.04). CONCLUSION: Our results showed that the number of infants with normal cNIRS values regarding cerebral oxygenation was higher in the 180-s DCC group. The pulmonary flow velocity was significantly increased in the 180-s DCC group in terms of echocardiographic findings.

Combined primary repair of tetralogy of Fallot and aortic root replacement.

Tetralogy of Fallot associated with ascending aortic aneurysm and aortic valve regurgitation is unusual. This combination necessitates a change in operative strategy during complete tetralogy repair. We present a 43-year-old woman who successfully underwent composite graft replacement of the ascending aorta and aortic valve during primary complete tetralogy repair. Histologic examination of the aorta revealed medial degeneration.

Evaluation of renal functions in children with congenital heart disease before and after cardiac angiography.

Radiocontrast nephrotoxicity, which has increased in incidence with widespread use of radiological methods in medicine, is a serious complication of radiocontrast materials. In this study, we have prospectively investigated whether children with cyanotic congenital heart disease are at risk for radiocontrast nephrotoxicity with the use of a nonionic low osmolar contrast agent. Thirty-five children (17 cyanotic and 18 acyanotic patients) who underwent diagnostic cardiac catheterization were subjects of the study. The age range was from five days to 13 years. The volume of contrast material was 3.11 +/- 1.37 ml/kg in cyanotic patients and 2.67 +/- 0.86 ml/kg in acyanotic patients. Blood samples and timed urine samples were taken from all patients 24 hours before and 48 hours after cardiac catheterization. Blood urea nitrogen, creatinine, sodium, and phosphorus in serum, and creatinine and N-acetyl-beta-D-glucosamine in urine were analyzed. There was not a statistically significant difference between the values before and after angiography. As a result, we could find no evidence of radiocontrast nephrotoxicity with the use of a nonionic contrast agent in cyanotic and acyanotic patients who underwent cardiac angiography.

Kearns-Sayre syndrome. A case report.

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder. There is a large-scale mitochondrial DNA (mtDNA) deletion in most of the case. In this article, a case of KSS who has progressive external ophthalmoplegia (PEO), retinitis pigmentosa (RP), complete heart block, encephalopathy attacks, type-1 diabetes mellitus, ragged-red fiber (RRF) and lactic acidosis is presented and discussed in light of the literature available on this subjects. Diagnosis is confirmed by determination of mtDNA deletion.

Noninvasive cardiac evaluation of young patient with cystic fibrosis.

Cardiac involvement was evaluated by echocardiography in 26 young cystic fibrosis patients. The mean age was 48.4 months (range 3 months to 15 years). The findings were compared with 26 age- and sex-matched children without a history of cardiopulmonary complaints. All patients had normal values of left ventricular ejection fraction and fractional shortening. Interventricular septal and posterior left ventricular wall thicknesses were similar to control group but right ventricular free wall thickness was found greater than in the control group. Abnormal septal motion was documented in six patients. Right ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in two patients and there was a negative correlation with clinical Shwachman scores (r: -0.55). Left ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in five patients. For both mitral and tricuspid valves, the mean ratios of peak velocity during passive filling (E) phase of diastole to peak velocity during atrial contraction (A) phase were found significantly lower than in the control group (p < 0.05). Early diastolic peak velocity was similar to that in the control group but late atrial peak velocity was higher in the patient group (p < 0.05). Isovolumic relaxation time was found the same as in the control group. We conclude that cardiac changes in diastolic and systolic functions begin at very young ages in cystic fibrosis patients.

Late cardiac effects after treatment for childhood Hodgkin's disease with chemotherapy and low-dose radiotherapy.

Twenty-four patients under 18 years when treated for Hodgkin's disease (20 male, four female) were examined no less than five years after the completion of the treatment. The mean age was 17 years (range 9.5-25.0 years) at the time of study. All patients received six courses of cyclophosphamide-oncovin-procarbazine-prednisolone chemotherapy; in addition, nine patients received low-dose radiotherapy excluding the mediastinum and eight of 24 patients received mediastinal radiotherapy; the dose was between 20-30 Gy. All patients had normal cardiovascular findings on clinical examination. ECG and chest radiography were within normal limits in all patients. Resting left ventricular ejection fraction and fractional shortening were decreased in only one patient (4%), but there was no significant difference between the patient group and a control group for left ventricular systolic function (p > 0.05). In the patient group, early diastolic peak velocity, peak velocity at atrial contraction, left ventricular isovolumic relaxation time, and the rate of decrease of flow velocity in early diastole were significantly different from that of the control group (p < 0.05). In conclusion, the late effects of our treatment protocol for Hodgkin's disease appear to be minimal. These observations support combined modality, low-dose irradiation regimens in children and adolescents and suggest the need for careful cardiac screening of treated patients.

Intracardiac thrombosis diagnosed by echocardiography in childhood: predisposing and etiological factors.

Eleven cases of intracardiac thrombi caused by different factors including protein-C deficiency are presented for discussion of the etiology and predisposing factors of intracardiac thrombi during infancy and childhood, and to stress the importance of protein-C deficiency as an etiological factor. Thrombi were localised in the left heart in five patients and right heart in five patients. One patient had both-sided thrombi. Four of our patients had dilated cardiomyopathy, one had mitral valve hypoplasia, and one had pulmonary valvar stenosis as the predisposing factors for thrombus formation. In three patients whose cardiac anatomies were completely normal, we determined protein-C deficiency as an etiological factor of thrombus formation. One of these had congenital protein-C deficiency and the other two had acquired temporary protein-C deficiency due to sepsis. In conclusion we recommend that protein-C deficiency should be investigated as an etiological factor in all cases of intracardiac thrombi irrespective of whether or not another predisposing factor is identified.

Congenital insensitivity to pain with anhidrosis.

Congenital insensitivity to pain with anhidrosis is a rare disorder. Its primary clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mental retardation. A five-year-old boy with consanguineous parents and no family history of the disorder presented with ulcerating lesions on both knees and elbows. His family had discovered the lack of sensation to pain and anhidrosis. Physical examination revealed ulcers on both knees and elbow, self-mutilation of the tongue, fingers, and toes. Sensation to touch was intact and lacrimation was normal. Moderate mental retardation and analgesia were noted in an otherwise normal neurologic examination. The results of electromyographic examination were normal and the application of pilocarpine showed anhidrosis. A skin biopsy specimen was also examined.

Mycotic aneurysm of the descending aorta diagnosed by echocardiography.

We present a 3-yr-old girl with coarctation of aorta and patent ductus arteriosus in whom mycotic aneurysm and bacterial endarteritis developed postoperatively and was diagnosed by two-dimensional and Doppler echocardiography. Five weeks after the operation of ligation of ductus and resection of coarctated segment, the patient was readmitted with complaints of vomiting, fever and coughing. Bacterial endarteritis, empyema and septic arthritis were diagnosed. Suprasternal echocardiographic examination demonstrated an aneurysmatic appearance 60 x 65 mm in size at the location of coarctation. The patient died, most probably due to aortic rupture, before surgical treatment could be undertaken. Autopsy study confirmed our diagnosis.

Perigraft seroma diagnosed by echocardiography: a complication following Blalock-Taussig shunt.

Perigraft seroma is a specific clinicopathologic entity, which is a collection of clear, sterile fluid, confined within a nonsecretory fibrous pseudomembrane surrounding a vascular graft. Serous leaks and/or seroma formation from polytetrafluoroethylene grafts have been reported in peripheral arteriovenous anastomoses, arterial grafts and subclavian pulmonary anastomoses. We present a case of seroma formation which developed after a Blalock-Taussig shunt operation constructed with a polytetrafluoroethylene graft in a child with tricuspid atresia and which was diagnosed by echocardiography.

Successful treatment of a nondeflatable balloon atrial septostomy catheter.

We present a rare complication of balloon atrial septostomy in an infant with complete transposition of the great arteries and review the management of this problem. The balloon could not be deflated after the eighth pass across the atrial septum. We punctured the balloon with a stylet which was passed through the circulation lumen of the double-lumen septostomy catheter. We suggest that this method should once be tried before other methods are employed.

Left ventricular thrombosis due to acquired protein C deficiency diagnosed by two-dimensional echocardiography.

We present a patient with left ventricular thrombus diagnosed by two-dimensional echocardiography. Thrombosis was due to acquired transient protein C deficiency which was caused by impaired liver function due to hepatitis, sepsis and heart failure. With proper treatment the thrombus disappeared on the fourth day. Eighteen weeks later the protein C level returned to normal. We recommend echocardiographic evaluation and follow-up of suspected cases for intracardiac thrombus. The measurement of protein C level in such cases is proposed. This is the first case with left-sided cardiac thrombus associated with protein C deficiency in the medical literature.

Cardiomyopathies in children. Clinical, epidemiological and prognostic evaluation.

In this article, the clinical and epidemiological characteristics of 137 children with cardiomyopathy admitted to the Hacettepe Pediatric Cardiology Unit were studied and the prognosis was evaluated after a follow-up period of 24 +/- 5 months. It was found that the highest proportion of patients were residents of Ankara, followed by the Northern and the Central Anatolian regions, and consanguinity between their parents was more common than the proportion for Turkey as a whole. Most of the patients had dilated cardiomyopathy (78.9%) and the age at which symptoms appeared varied according to the type of cardiomyopathy. A high proportion of patients came to the hospital with complaints of dyspnea and a decrease in effort capacity. The most common findings on the physical examination were hepatomegaly and tachycardia. Electrocardiographic and echocardiographic evaluations were made for all patients and cardiac catheterization and endomyocardial biopsy were performed when necessary. The outcome was as follows: 34.5% improved, 45.7% remained stable, 9.5% deteriorated and 10.3% died.