Kürşad Tokel

Malnutrition and growth failure in cyanotic and acyanotic congenital heart disease with and without pulmonary hypertension

AIM To investigate the effect of several types of congenital heart disease (CHD) on nutrition and growth. PATIENTS AND METHODS The prevalence of malnutrition and growth failure was investigated in 89 patients with CHD aged 1−45 months. They were grouped according to cardiac diagnosis: group aP (n = 26), acyanotic patients with pulmonary hypertension; group ap (n = 5), acyanotic patients without pulmonary hypertension; group cp (n = 42), cyanotic patients without pulmonary hypertension; and group cP (n = 16), cyanotic patients with pulmonary hypertension. Information on socioeconomic level, parental education status, birth weight and nutrition history, number of siblings, and the timing, quality, and quantity of nutrients ingested during weaning period and at the time of the examination were obtained through interviews with parents. RESULTS There was no significant difference between groups in terms of parental education status, socioeconomic level, duration of breast feeding, and number of siblings (p > 0.05). Group cP patients ingested fewer nutrients for their age compared to other groups. 37 of the 89 patients were below the 5th centile for both weight and length, and 58 of 89 patients were below the 5th centile for weight. Mild or borderline malnutrition was more common in group aP patients. Most group cp patients were in normal nutritional state, and stunting was more common than wasting. Both moderate to severe malnutrition and failure to thrive were more common in group cP patients. CONCLUSION Patients with CHD are prone to malnutrition and growth failure. Pulmonary hypertension appears to be the most important factor, and cyanotic patients with pulmonary hypertension are the ones most severely affected. This study shows the additive effects of hypoxia and pulmonary hypertension on nutrition and growth of children with CHD.

Acute Renal Failure and Mortality After Open-Heart Surgery in Infants

Acute renal failure (ARF) is a major complication in infants who undergo cardiac surgery. The aim of this investigation was to identify possible risk factors for ARF and mortality in this patients group. Out of 64 patients, 21 (32.8%) cases developed acute renal failure and overall mortality rate was 25%. The mortality rate was higher in the infants who developed ARF than those who did not (66.7% and 4.7%, respectively, p < 0.05). Also, ARF was positively correlated with mortality (r:0.70, p < 0.0001). The nonsurvivors had lower mean serum albumin than did the survivors (p < 0.05), and serum albumin level was negatively correlated with mortality (r = − 0.34, p < 0.05). For the patients with serum albumin level < 3.5 g/dL, the unadjusted odds ratio for mortality was 4.3 (CI 95%:1.05 − 17.86). Total bypass time and aorta clamping time were significantly longer in the nonsurvivor group than in the survivor group (p < 0.05 for both). In conclusion, the significant risk factors for mortality in these patients were development of ARF, low serum albumin level, and long total bypass and aorta clamping times, which may be predictive of poor prognosis.

Diaphragmatic paralysis after cardiac surgery in children: incidence, prognosis and surgical management.

Diaphragmatic paralysis (DP) after cardiac surgery is an important complication especially in infants. We analyzed the incidence, clinical course, surgical management and follow up of the patients with DP, retrospectively. Between 1996 and 2005, 3,071 patients underwent cardiac surgery. Total number of patients with DP was 152 (4.9%). Out of 152 patients, 42 were surgically treated with transthoracic diaphragm plication (1.3%). The overall incidence of diaphragm paralysis was higher in correction of tetralogy of Fallot (31.5%), Blaloc–Taussig (B–T) shunt (11.1%) and VSD closure with pulmonary artery patch plasty (11.1). The incidence of DP which require plication was higher in B–T shunt (23.8%) arterial switch (19%) and correction of tetralogy of Fallot (11.9%). Mean and median age at the time of surgery were 17.8±3.6 and 6 months, respectively. Median time from cardiac surgery to surgical plication was 12 days. Indications for plication were repeated reintubations (n=22), failure to wean from ventilator (n=12), recurrent lung infections (n=5) and persistent respiratory distress (n=3). Mortality rate was 19.1%. Being under 1 year of age, pneumonia and plication 10 days after mechanical ventilation were associated with higher incidence mortality (P<0.05). Phrenic nerve injury is a serious complication of cardiac surgery. It is more common after some special procedures. Spontaneous recovery is very rare. Being under 1 year of age, plication after 10 days from the surgery and pneumonia are major risk factors for mortality even in plicated patients. Transthoracic plication is helpful if performed early.

Elevated CK-MB mass and plasma brain-type natriuretic peptide concentrations following convulsive seizures in children and adolescents: Possible evidence of subtle cardiac dysfunction

Purpose:  To evaluate the presence of myocardial injury during convulsive seizures in children and adolescents by determining serum concentrations of cardiac troponin I (cTnI), creatine kinase‐MB mass (CK‐MB mass), and plasma brain‐type natriuretic peptide (BNP).

The effect of obesity on echocardiographic and metabolic parameters in childhood.

BACKGROUND Obese children are predisposed to left ventricular (LV) hypertrophy and cardiac dysfunction. We evaluated ventricular function of obese children with conventional echocardiography and tissue Doppler imaging (TDI) and correlated these with fasting serum glucose, lipid and insulin levels. METHODS Thirty obese children were examined by conventional echocardiography and TDI and compared with an age-matched control group. In the obese children, fasting serum glucose, lipid, and insulin levels were obtained. RESULTS Systolic and diastolic function of the LV was normal in obese children. LVM/ht2.7 (LVM normalized for height), relative wall thickness (RWT), and LV end-diastolic diameter were significantly greater in obese children. The early to late relaxation velocity ratio (E/A) determined by TDI for the right ventricle and interventricular septum (IVS) were significantly lower in the obese group, LVM/ht2.7 and RWT correlated with body mass index (BMI). In the obese group, IVS and LV posterior wall thickness correlated with insulin, very low density lipoprotein, and triglyceride levels. However, we failed to show this correlation when these measurements were indexed to height. CONCLUSION Left ventricular hypertrophy, as evidenced by increased LV mass, was present in obese children. Higher BMI, insulin, very low density lipoprotein, and triglyceride levels were associated with LV hypertrophy. TDI revealed subclinical changes in diastolic function of the right ventricle and IVS.

Oral versus intranasal midazolam premedication for infants during echocardiographic study

Movement and anxiety during echocardiographic study may reduce the reliability and affect the quality of echocardiographic images. Thus, sedation is an essential component when it is performed in infants. This randomized, single-blinded, placebo-controlled study was undertaken to evaluate the acceptability and effectiveness of intranasal midazolam (INM) versus oral midazolam (OM) in infants during transthoracic echocardiography. Eighty patients between the ages of 6 mo and 3 y who presented for elective echocardiographic study were divided into 3 groups: the OM group received 0.4 mg/kg of injectable midazolam mixed with an equal volume of cherry juice, the INM group received 0.2 mg/kg as drops, and the control group was given oral cherry juice or intranasal serum physiologic. A blinded clinician assessed and scored the level of sedation and comfort during the procedure for each child, and a score for ease of administration was recorded by the nurse. The intranasal route was more acceptable to infants than the oral route (P<.001). No significant difference in the effects of sedation was observed between the OM group and the INM group (P=.583), but significant differences were observed between the sedated groups and the control group (P<.001). The procedure was significantly more comfortable in groups given OM and INM than in the control group (P<.001). Although no difference in sedation score was seen between the oral and nasal routes, INM was better accepted by infants than OM. Echocardiography was performed more reliably and comfortably in those given midazolam than in those in the control group.

Brain Abscess Associated with Isolated Left Superior Vena Cava Draining into the Left Atrium in the Absence of Coronary Sinus and Atrial Septal Defect

A previously healthy 12-year-old girl presented with severe headache for 2 weeks. On physical examination, there was finger clubbing without apparent cyanosis. Neurological examination revealed only papiledema without focal neurologic signs. Cerebral magnetic resonance imaging showed the characteristic features of brain abscess in the left frontal lobe. Cardiologic workup to exclude a right-to-left shunt showed an abnormality of the systemic venous drainage: presence of isolated left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect. This anomaly is rare, because only a few other cases have been reported.

Clinical and hematologic findings in Noonan syndrome patients with PTPN11 gene mutations

Reports on Noonan syndrome (NS) have documented multiple types of coagulation defects and bleeding diathesis, and a wide range of clinical presentations. Early studies suggested that a large proportion of NS patients have coagulation defects, whereas more recent reports indicate low rates of coagulopathy. The aim of this study was to evaluate phenotypic characteristics, PTPN11 gene mutations, and hematological and coagulation parameters in 30 clinically diagnosed cases of NS. One of the NS patients had a history of easy bruising; however, his hematological and coagulation tests were normal. None of the other patients had clinical coagulation problems. In the NS group, values for platelet count, activity of factors XI, XII, and protein C were significantly lower than the corresponding means for the control group. However, the results of coagulation tests in the NS group were diagnostically inconclusive and only one patient had clinical signs of coagulopathy. Interestingly, two NS patients had low protein C activity. One of these children had an A1517C mutation and transient myelodysplasia. The other patient had a C1528G mutation in exon 13 that has not been reported previously. Neither of these individuals experienced a thrombotic event or any complication during approximately 3 years of follow‐up. For all patients clinically diagnosed with NS, a thorough history of coagulation issues should be taken and first‐line coagulation testing should be done to evaluate for bleeding diathesis. However, if these assessments reveal nothing abnormal, complications related to coagulation are unlikely and extensive testing is unnecessary.

Clinical investigations over 13 years to establish the nature of the cardiac defects in patients having abnormalities of lateralization

INTRODUCTION The first step in diagnosing congenital cardiac malformations is to assess the arrangement of the atrial appendages. In patients with abnormal lateralization of the organs of the body, the arrangement of atrial appendages is neither normal, nor a mirror image of normal. There are 2 categories of abnormal arrangement based on the morphology of the atrial appendices, namely right isomerism and left isomerism, and in almost all instances these are found in the setting of so-called heterotaxy syndromes. OBJECTIVE To evaluate the various congenital cardiac malformations those are associated with abnormalities of lateralization, and to discuss the diagnostic tools, therapeutic options, and outcome for these patients. PATIENTS AND METHODS We studied 134 patients, who had been admitted to our department of paediatric cardiology with known abnormalities of lateralization and congenital cardiac defects between 1990 and 2003. The data relating to each patient was evaluated retrospectively. The arrangement of the atrial appendages was established echocardiographically, and/or angiographically, and/or on the basis of morphologic investigations during the operation. These studies showed that 43 (32.1%) of the patients had right isomerism, and 88 (65.7%) had left isomerism. In 2 (1.5%), there was mirror-imaged arrangement, while in the final patient (0.07%), we were unable to determine sidedness with certainty. The median age at diagnosis was 0.66 years, and the females outnumbered the males in a ratio of 3 to 1. Patients in the 2 isomeric groups were compared with regard to age, cardiac defects, diagnostic tools and outcomes. RESULTS The difference in mean ages of the two groups of patients was statistically significant, those with right isomerism being 1.0 minus or plus 1.5 years, as opposed to those with left isomerism being 3.3 minus or plus 4.7 years (P is less than 0.005). Of the patients with right isomerism, 32 (74.4%) had left-sided, and 11 (25.6%) right-sided hearts, whereas in those with left isomerism, the hearts were left-sided in 65 (73.9%), and right-sided in 21 (23.9%), with 2 (2.2%) positioned in the midline. Extracardiac totally anomalous pulmonary venous connection was more common in those with right isomerism, being found in 13 patients (30.2%) as opposed to 5 patients (5.7%) with left isomerism. In only 8 of those with right isomerism did we find two perforate atrioventricular valves (18.6%), this arrangement being found in 34 (38.6%) of those with left isomerism. Pulmonary atresia and stenosis were present in 40 (93.0%) of those with right isomerism, but also in 41 (46.6%) of the patients with left isomerism. Angiographic and echocardiographic investigations were concordant in about three-quarters of patients with both right and left isomerism. All patients with extracardiac totally anomalous pulmonary venous connection died. Overall, 22 of the patients with right isomerism died (51.2%), as opposed to 20 (22.7%) of those with left isomerism. CONCLUSION Our experience confirms that patients with right isomerism have more complex cardiac defects than those with left isomerism. Overall, the presence of isomerism carries a poor prognosis, the more so for right isomerism, with this related to the complex cardiac abnormalities. In our cohort, extracardiac totally anomalous pulmonary venous connection with pulmonary arterial obstruction was always a fatal combination. The mapping of cardiac and abdominal morphologies is still essential for proper diagnosis of these syndromes, especially in fetal life.

The effect of corrective surgery on serum IGF-1, IGFBP-3 levels and growth in children with congenital heart disease.

Abstract The aim of this study is to evaluate growth and insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 3 (IGFBP-3) levels in infants with congenital heart disease (CHD) pre- and postoperatively over a period of a year. Anthropometric values and serum levels of IGF-1 and IGFBP-3 of 40 infants with CHD (20 cyanotic and 20 acyanotic) were compared with 32 healthy controls. Acyanotic infants and infants with pulmonary hypertension (PH) presented significantly more growth failure. Preoperatively, serum IGF-1 and IGFBP-3 levels were lower in the acyanotic group than the cyanotic and the control groups (p=0.22; p<0.01). The upward trend in IGF-1 and IGFBP-3 levels in this year-long study demonstrated that the values in the third month and the first year were higher than the preoperative values (p<0.05). The parallel increase of weight gain and IGF-1, IGFBP-3 levels were the best evidence that these parameters are good nutritional indicators. Timing the corrective surgery before chronic malnutrition or PH develops is an important issue to maintain a normal growth for children with CHD.