Correlation between Perfusion Index and Left Ventricular Output in Healthy Late Preterm Infants.

OBJECTIVE: The perfusion index (PI) is a noninvasive marker derived from photoelectric plethysmographic signals in pulse oximetry in the evaluation of peripheral perfusion. This study was aimed to determine the correlation between PI and left ventricular output (LVO) in healthy late preterm infants at 48th hour of life. STUDY DESIGN: With new generation pulse oximeter (MASIMO Rad 7 Oximeter) pre- and post-ductal PI values were recorded from healthy late preterm babies at the 48th hour of life. PI was determined simultaneously with LVO as measured by transthoracic echocardiography. RESULTS: A total of 50 late preterm babies were included in the study. The mean gestational age of the cases was 35.4 ± 0.7 weeks and the birth weight was 2,586 ± 362 g. Mean pre- and post-ductal PI values at the postnatal 48th hour of babies' life were found to be 2.0 ± 0.9 and 1.7 ± 1.1. The mean LVO value was 438 ± 124, LVO/kg 175 ± 50. When the LVO value was normalized according to the babies' body weight, there was no statistically significant correlation between the pre- and post-ductal PI and the LVO/kg value (r <0.2, p >0.05 in both comparisons). CONCLUSION: There was no correlation between pre- and post-ductal PI and LVO values in healthy late preterm infants. This may be due to the failure of the LVO, a systemic hemodynamic parameter, to accurately reflect microvascular blood flow due to incomplete maturation of the sympathetic nervous system involved in the regulation of peripheral tissue perfusion in preterm babies. KEY POINTS: · No correlation found between PI and LOV in preterm babies.. · LVO cannot adequately reflect peripheral blood flow.. · Sympathetic nervous system is immature in preterm infants..

Should we start a nationwide screening program for critical congenital heart disease in Turkey? A pilot study on four centres with different altitudes.

BACKGROUND: To investigate the feasibility of critical congenital heart disease (CCHD) screening test by pulse oximetry in four geographical regions of Turkey with different altitudes, before implementation of a nationwide screening program. METHODS: It was a prospective multi-centre study performed in four centres, between December, 2015 and May, 2017. Pre- and post-ductal oxygen saturations and perfusion indices (PI) were measured using Masimo Radical-7 at early postnatal days. The results were evaluated according to the algorithm recommended by the American Academy of Pediatrics. Additionally, a PI value <0.7 was accepted to be significant. RESULTS: In 4888 newborns, the mean screening time was 31.5 ± 12.1 hours. At first attempt, the mean values of pre- and post-ductal measurements were: saturation 97.3 ± 1.8%, PI 2.8 ± 2.0, versus saturation 97.7 ± 1.8%, PI 2.3±1.3, respectively. Pre-ductal saturations and PI and post-ductal saturations were the lowest in Centre 4 with the highest altitude. Overall test positivity rate was 0.85% (n = 42). CCHD was detected in six babies (0.12%). Of them, right hand (91 ± 6.3) and foot saturations (92.1 ± 4.3%) were lower compared to ones with non-CCHD and normal variants (p <0.05, for all comparisons). Sensitivity, specificity, positive and negative predictive values, and likelihood ratio of the test were: 83.3%, 99.9%, 11.9%, 99.9%, and 99.2%, respectively. CONCLUSION: This study concluded that pulse oximetry screening is an effective screening tool for congenital heart disease in newborns at different altitudes. We support the implementation of a national screening program with consideration of altitude differences for our country.

Intravenous paracetamol with a lower dose is also effective for the treatment of patent ductus arteriosus in pre-term infants.

INTRODUCTION: Haemodynamically significant patent ductus arteriosus is a significant cause of morbidity and mortality in pre-term infants. This retrospective study was conducted to investigate the usefulness of lower-dose paracetamol for the treatment of patent ductus arteriosus in pre-term infants. MATERIALS AND METHODS: A total of 13 pre-term infants who received intravenous paracetamol because of contrindications or side effects to oral ibuprofen were retrospectively enrolled. In the first patient, the dose regimen was 15 mg/kg/dose, every 6 hours. As the patient developed significant elevation in transaminase levels, the dose was decreased to 10 mg/kg/dose, every 8 hours in the following 12 patients. Echocardiographic examination was conducted daily. In case of closure, it was repeated after 2 days and when needed thereafter in terms of reopening. RESULTS: A total of 13 patients received intravenous paracetamol. Median gestational age was 29 weeks ranging from 24 to 31 weeks and birth weight was 950 g ranging from 470 to 1390 g. The median postnatal age at the first intravenous paracetamol dose was 3 days ranging from 2 to 9 days. In 10 of the 13 patients (76.9%), patent ductus arteriosus was closed at the median 2nd day of intravenous paracetamol ranging from 1 to 4 days. When the patient who developed hepatotoxicity was eliminated, the closure rate was found to be 83.3% (10/12). CONCLUSION: Intravenous paracetamol may be a useful treatment option for the treatment of patent ductus arteriosus in pre-term infants with contrindication to ibuprofen. In our experience, lower-dose paracetamol is effective in closing the patent ductus arteriosus in 83.3% of the cases.

Dilated cardiomyopathy secondary to rickets-related hypocalcaemia: eight case reports and a review of the literature.

INTRODUCTION: Dilated cardiomyopathy is usually idiopathic and may arise secondary to infections or metabolic or genetic causes. Another rare cause is hypocalcaemia. Owing to the fact that calcium plays an essential role in excitation and contraction of myocardial muscle, myocardial contractility may decline in patients with hypocalcaemia. MATERIALS AND METHODS: Patients with symptoms of congestive heart failure and rickets-related hypocalcaemia were assessed clinically and by echocardiography in a paediatric cardiology clinic. Echocardiography was performed for all patients. Rickets was diagnosed according to the clinical, laboratory, and radiologic findings. Maternal lifestyle and living conditions were investigated, and the maternal 25-OH vitamin D3 blood level was measured. RESULTS: We evaluated eight patients who developed heart failure as a result of severe hypocalcaemia associated with rickets between August, 1999 and June, 2012. The age distribution of the patients was 3-12 months. Laboratory results were consistent with advanced-stage rickets. Severe hypocalcaemia was detected in all patients. The maternal 25-OH vitamin D3 levels were low. Echocardiography revealed increased pre-treatment left ventricle end-systolic and end-diastolic diameters for age and reduced ejection fraction and fractional shortening. After clinical improvement, the patients were discharged. CONCLUSIONS: Severe hypocalcaemia associated with rickets must always be kept in mind among the causes of dilated cardiomyopathy and impaired cardiac function in infants. If diagnosed and treated in time, dilated cardiomyopathy and severe heart failure related to rickets respond well.

Rare cause of absence of femoral arterial pulse: bilateral common iliac artery hypoplasia.

Congenital anomaly of the iliac arteries is rare, and, given that patients are generally asymptomatic, diagnosis in the newborn period is difficult. Herein is presented the case of a newborn with bilateral hypoplasia of the common iliac arteries, seen on multi-slice computed tomography while investigating the absence of femoral pulse. This anomaly is a rare cause of absence of femoral arterial pulse.

Accelerated junctional rhythm in children with acute rheumatic fever: is it specific to the disease?

OBJECTIVE: During the course of acute rheumatic fever, some electrocardiographic changes are seen. First-degree atrioventricular block is the most common electrocardiographic abnormality. Second- and third-degree atrioventricular block, ventricular tachycardia, and junctional acceleration are also seen. In the present study, the specificity of accelerated junctional rhythm to acute rheumatic fever was INVESTIGATED. METHODS: The study included patients with acute rheumatic fever (Group 1), healthy children who had suffered from recent group A ß-haemolytic streptococcal upper respiratory tract infection but did not develop acute rheumatic fever (Group 2), and patients who had other diseases that may affect the joints and/or heart (Group 3). RESULTS: Accelerated junctional rhythm was detected in 10 patients in Group 1, but in none of the patients from Group 2 or 3. Specificity of accelerated junctional rhythm for acute rheumatic fever was 100% and the positive predictive value was 100%. CONCLUSION: Accelerated junctional rhythm is specific to acute rheumatic fever. Although its frequency is low, it seems that it can be used in the differential diagnosis of acute rheumatic fever, especially in patients with isolated polyarthritis.

A Genotyped Case of Townes-Brocks Syndrome with Absent Pulmonary Valve Syndrome from Turkey.

Townes-Brocks syndrome (TBS) is a rare syndrome characterized by triad of anal, ear, and thumb anomalies. Further malformations/anomalies include congenital heart diseases, foot malformations, sensorineural and/or conductive hearing impairment, genitourinary malformations, and anomalies of eye and nervous system. Definitive diagnosis for TBS is confirmed by molecular analysis for mutations in the SALL1 gene. Only one known case of TBS with absent pulmonary valve syndrome (APVS) has been previously described to our knowledge. Here, we report a newborn diagnosed with TBS with APVS and tetralogy of Fallot (TOF) who was found to carry the most common pathogenic SALL1 gene mutation c.826C > T (p.R276X), with its surgical repair and postoperative follow-up. To our knowledge, this is the first genotyped case of TBS from Turkey to date. TBS should be suspected in the presence of ear, anal, and thumb malformations in a neonate. If a patient with TBS and TOF-APVS needs preoperative ventilation within the first months of life, this implies prolonged postoperative intubation and increased risk of mortality.

Clinical characteristics of neonates with VACTERL association.

BACKGROUND: The VACTERL association (VA) is the non-random co-occurrence of vertebral anomalies, anal atresia, cardiovascular malformations, tracheoesophageal fistula and/or esophageal atresia, renal anomalies, and/or limb anomalies, and is referred to by the first letters of its components. Studies investigating the clinical characteristics of VA patients and probing of the observed current six component types are limited, and none of them is focused on neonates. We investigated the clinical characteristics of our patients diagnosed as having VA in the newborn period. METHODS: We retrospectively reviewed the neonates whose final diagnosis was VACTERL association. Presence of at least three components of previously reported six anomalies was accepted as VACTERL association. Sex, birthweight, gestational age, postnatal age, anomalies of the systems that are included in VA, and the other features were recorded. RESULTS: There was a male predominance (14/11) of 28 patients; and there were three patients with ambiguous genitalia. The most common observed VACTERL component was vertebral anomalies (n= 26), followed by anal atresia (n= 19), tracheoesophageal fistula/esophageal atresia (n= 17), renal anomalies (n= 15), limb anomalies (n= 15) and cardiac anomalies (n= 14). The most frequent combination was VCTL (n= 4). Fifteen (57%) patients had non-VACTERL anomalies and the most frequent of these was ambiguous genitalia (n= 3). CONCLUSION: VA patients may have different clinical characteristics in different populations, and clinicians may miss some component features if the patients are evaluated after the neonatal period.

Heart rate variability in children with acute rheumatic fever.

OBJECTIVE: Acute rheumatic fever is a systemic inflammatory disease occurring after acute streptococcal tonsillopharyngitis. The PR prolongation in these patients is thought to be due to increased vagal activity. There has been no previous study investigating the autonomic balance in patients with acute rheumatic fever. In this study, we aimed to investigate the balance of the autonomic nervous system in children with acute rheumatic fever by analysis of heart rate variability. METHODS: We evaluated the heart rate variability parameters in 50 patients with acute rheumatic fever and 37 comparable control subjects. Both groups underwent 24-hour electrocardiography monitoring, and time- and frequency-domain heart rate variability parameters were calculated. A total of 39 patients (78%), with (n = 28) or without (n = 11) other major findings, had carditis, and the remaining 11 (22%) did not. The PR interval was found to be prolonged in 10 (20%) of the patients at the beginning. RESULTS: In the study group, the time- and frequency-domain heart rate variability parameters showed a sympathetic dominance compared with the control group, with a p-value less than 0.05. When compared with the control group, the time- and frequency-domain heart rate variability parameters showed a significant sympathetic dominance in patients with both prolonged PR and normal PR intervals in the acute period, with a p-value less than 0.05. When compared with patients with normal PR interval, mean normalised low frequency and normalised high frequency parameters suggested a relatively lower sympathetic dominance in patients with prolonged PR interval, with a p-value less than 0.05. CONCLUSION: Our results indicated that in the acute period of rheumatic fever, sympathetic dominance is apparent; in patients with prolonged PR interval, sympathetic dominance is relatively lower when compared with the patients with normal PR interval.

Treatment of Permanent Junctional Reciprocating Tachycardia in a Preterm Neonate: Case Report.

Permanent junctional reciprocating tachycardia (PJRT) is most often observed in infants and children and can lead to incessant tachycardia. PJRT is usually refractory to medical treatment. Tachycardia may infrequently occur in the fetus in which case fetal tachycardia transplacental treatment should be started immediately. Term delivery is recommended for fetuses with tachycardia in the absence of significant clinical compromise to avoid complications of preterm birth. Herein, a 36-week preterm neonate presented with PJRT. He had tachycardia in the fetal period and was treated with digoxin, amiodaron, and esmolol therapy after birth without undergoing the catheter ablation procedure.

Midterm experience with implantable cardioverter-defibrillators in children and young adults.

AIMS: This single-centre study was undertaken to review our experience with implantable cardioverter-defibrillator (ICD) implantation in children with relatively different aetiologies. METHODS AND RESULTS: We retrospectively reviewed the records of the paediatric patients who underwent ICD implantation between October 2001 and December 2008. The data of these patients were collected by reviewing the patients' medical records and computerized departmental pacemaker databases. A total of 28 patients who underwent ICD implantation during this period were included in this study. The median age was 12 years and median weight was 32 kg. Most of the patients had ion-channel diseases (n = 13) or cardiomyopathy (n = 11). Devices were implanted for either secondary (n = 22) or primary (n = 6) prevention. The selected ICD generator type was single chamber in 22 patients, dual chamber in 5 patients, and biventricular in 1. Nineteen patients received 122 shocks. Fifteen of 22 patients (68.2%) from the secondary prevention group and 2 of 6 patients (33.3%) from the primary prevention group experienced at least one appropriate shock during a median period of 11.3 months (range: 4 days-6.5 years). Forty-two inappropriate shocks were delivered in seven (31.8%) patients from the secondary prophylaxis group during a median period of 11.3 months. The most important reason for inappropriate shocks was T-wave oversensing. In six patients, lead-related acute or chronic complications occurred. CONCLUSION: The ICD was safe and effective in interrupting malignant arrhythmias in children and adolescents with a high risk of sudden cardiac death. However, the occurrence of lead complications is significant. The incidence of therapies delivered by the device, with appropriate and inappropriate shocks, was high and interfered with the quality of life. The most important reason for inappropriate shocks was T-wave oversensing. Careful programming is mandatory to reduce the inappropriate shocks.

Asymptomatic rhythm and conduction abnormalities in children with acute rheumatic fever: 24-hour electrocardiography study.

Some rhythm and conduction abnormalities can occur in children with acute rheumatic fever. These abnormalities have been defined based on standard electrocardiography; however, the real prevalence of these abnormalities has not been investigated previously by the evaluation of long-term electrocardiographic recordings. In this study, we evaluated the asymptomatic rhythm and conduction abnormalities in children with acute rheumatic fever by evaluating the 24-hour electrocardiography. We evaluated the standard electrocardiography and the 24-hour electrocardiography of 64 children with acute rheumatic fever. On standard electrocardiography, the frequency of the first-degree atrioventricular block was found to be 21.9%. Electrocardiography at 24 hours detected three additional and separate patients with a long PR interval. Mobitz type I block and atypical Wenckebach periodicity were determined in one patient (1.56%) on 24-hour electrocardiography. While accelerated junctional rhythm was detected in three patients on standard electrocardiography, it was present in nine patients according to 24-hour electrocardiography. Premature contractions were present in 1.7% of standard electrocardiography, but in 29.7% of 24-hour electrocardiography. Absence of carditis was found to be related to the presence of accelerated junctional rhythm (p > 0.05), and the presence of carditis was found to be related to the presence of premature contractions (p = 0.000). In conclusion, our results suggest that in children with acute rheumatic fever, the prevalence of rhythm and conduction abnormalities may be much higher than determined on standard electrocardiography. Further studies are needed to clarify whether or not these abnormalities are specific to acute rheumatic fever.

Comparison of safety and effectiveness of two different transfusion rates in children with severe anemia.

We compared the safety and efficacy of 2 transfusion regimens in children with severe anemia (hemoglobin <5 g/dL) and without overt signs of congestive heart failure requiring transfusion of packed red blood cells (PRBCs). Forty-three patients were randomly divided into 2 groups: group A and group B. The transfusion regimens consisted of continuous infusion of PRBC at a rate of 1 mL/kg/h for group A and 3 mL/kg/h for group B. The patients were closely monitored for any clinical signs of heart failure throughout transfusion. Heart and respiratory rate, and arterial blood pressure were measured hourly for 6 hours during transfusion. None of the patients developed any signs of cardiac failure during or after the transfusion. The mean heart rate, respiratory rate, diastolic and systolic blood pressure were similar in both groups throughout transfusion. Group A needed significantly more PRBC units than group B to attain the same hemoglobin increase. Transfusion of PRBC at a rate of 3 mL/kg/h, and at a rate of 1 mL/kg/h, is a safe regimen for children with severe anemia of gradual onset requiring transfusion therapy.

Congenital cardiac malformations in neonates with apparently isolated gastrointestinal malformations.

BACKGROUND: The association of congenital cardiac malformations (CCM) with malformations of the gastrointestinal tract/abdominal wall is known. Nevertheless, the data presently available are derived from patient populations that include some special conditions known to be associated with a high rate of CCM. The aim of the present study was therefore to determine the incidence of cardiac malformations among neonates with apparently isolated malformations of the gastrointestinal tract/abdominal wall. METHODS: A total of 201 neonates with apparently isolated gastrointestinal malformations were screened on echocardiography. RESULT: Thirty-six (17.9%) of the neonates were diagnosed as having a CCM. When the four most frequent gastrointestinal malformations were evaluated, a CCM was diagnosed in 11/69 (15.9%) with anal atresia, in 9/38 (23.7%) with tracheoesophageal fistula/esophageal atresia, in 2/25 (8%) with diaphragmatic hernia and in 5/17 (29.4%) with intestinal atresia. In 11 of 36 patients (30.6%) with CCM, the cardiac problems were hemodynamically significant, requiring anti-congestive and/or surgical treatment. CONCLUSION: A significant number of neonates with apparently isolated gastrointestinal malformations had CCM. Because almost all patients with malformations of the gastrointestinal tract/abdominal wall require early surgical intervention, they should be evaluated on echocardiography to investigate CCM at the earliest opportunity.

Successful use of intravenous amiodarone in a child with combined postoperative junctional and ectopic tachycardias.

Early postoperative arrhythmias are a known complication of cardiac surgery. It is unusual, however, to encounter postoperative junctional and ectopic atrial tachycardias in the same patient. We describe our experience with a 2-year-old girl who suffered both these tachycardias after repair of a ventricular septal defect, the abnormal rhythms being controlled solely with intravenous administration of amiodarone.

Isolated congenital palatal fistula without submucous cleft palate.

Congenital fistula of the palate is a rare deformity. It has been generally associated with cleft palate. Treatment of cleft palate is surgical intervention. We present a child with congenital fistula of palate that was not associated with submucous cleft and closed spontaneously at 18 months.

Clinical, electrocardiographic, and laboratory findings in children with amitriptyline intoxication.

BACKGROUND: Amitriptyline is one of the major tricyclic antidepressants, and the data on amitriptyline poisoning in children are limited. OBJECTIVES: To present our experiences with amitriptyline poisoning in children, particularly with regard to its effects on electrocardiogram (ECG) and relation with clinical status. METHODS: Clinical, laboratory, and electrocardiographic findings in 52 children admitted with amitriptyline poisoning were reviewed. Patients were divided into 2 groups according to age, as 6 years or younger (group A) and older than 6 years (group B). RESULTS: Mean age was 4.6 +/- 3.0 years. Thirty-one patients were male, and 41 were 6 years or younger. Ingested amitriptyline dose was known in 23 patients (range, 2.3 mg/kg-27 mg/kg). The most frequent findings were lethargy (76.9%), sinus tachycardia (57.7%), and coma (48.1%). Four patients had a history of convulsion. The most common laboratory abnormalities were hyponatremia (26.9%) and leukocytosis (25%). Elevated transaminase levels were observed in 4 patients. In ECG, 11 (22.4%) patients had QTc prolongation and in 4 (8.2%) of them, it was significant. In 4 patients (8.2%), the QRS duration was 100 ms or longer and in 15 patients, the R wave in aVR was 3 mm or longer. The frequencies of clinical, laboratory, and electrocardiographic findings were similar between the 2 age groups (P > 0.05). No clinically apparent arrhythmias were observed. The positive predictive value of a widened QRS was 100% in terms of coma. None of the patients with an R wave in aVR of less than 3 mm developed convulsion; thus, the negative predictive value of an R wave in aVR of 3 mm or longer was 100% in terms of convulsion. CONCLUSIONS: Amitriptyline poisoning may result in severe toxicity. Frequencies of clinical, laboratory, and ECG findings were similar in the 2 age groups. Amitriptyline overdose results in some ECG changes that can possibly help to predict the results of poisoning. Absence of an R wave in aVR of 3 mm or longer predicts seizures with a high negative predictive value, and a QRS duration of 100 or longer ms predicts coma with a high positive predictive value.

An unreported side effect of risperidone in children: sinus arrest with long pauses causing syncope.

Risperidone has been reported as a well-tolerated antipsychotic in children. The most common side effects of risperidone are anxiety, headaches, insomnia, and agitation, which frequently appear with high doses. Although it is known that risperidone affects the cardiac conduction system, symptomatic bradycardia has not been reported previously in children. We report a child who developed syncope because of sinus arrest with long pauses (>3 seconds) with therapeutic doses of risperidone. This case suggests that close monitoring is mandatory even during therapeutic treatment with risperidone.

Spontaneous pneumomediastinum in a child as a rare cause of chest pain.

Spontaneous pneumomediastinum is a rare disorder in children occurring mostly due to some triggering events. In general, no underlying cause is found. A 10-year-old boy was admitted with chest pain of acute onset, suggestive of pericardial effusion. His body temperature, blood pressure, pulse rate and respiratory rate were normal. Femoral pulses were palpable and he did not have cyanosis. Cardiac auscultation was normal except for a crunching sound. Electrocardiography showed no abnormality. There was no subcutaneous emphysema over the chest, neck or face. On the chest radiogram, suspected mediastinal air was noted. A left lateral chest X-ray revealed apparent presence of air in the anterior mediastinum. No etiologic cause could be documented. Pneumomediastinum resolved spontaneously within a week.

Clinical characteristics of pediatric patients with first-attack acute rheumatic fever following the updated guideline.

AIM: To evaluate the clinical features of children diagnosed as having acute rheumatic fever between June 2015 and November 2018, and the changes observed in patient groups in comparison with data obtained in previous years. The diagnosis of acute rheumatic fever was made using the updated Jones criteria. MATERIAL AND METHODS: The medical records of pediatric patients who were diagnosed as having acute rheumatic fever between June 2015 and November 2018 using the updated criteria, were examined retrospectively. The data of a previous study that used the old criteria were reorganized and the two groups were compared. RESULTS: A total of consecutive 50 patients [22 males (44%)] who presented in the study period and were diagnosed as having first-attack acute rheumatic fever, were included in our study. Carditis was found in 42 (84%) patients. Manifest carditis was found in 24 patients and silent carditis was found in 18 patients. Joint involvement was present in 34 (68%) patients. Accompanying carditis was present in all 14 patients (28%) who were found to have chorea. Erythema marginatum and subcutaneous nodules were not found in our patients. When evaluated in terms of the updated criteria, a diagnosis of rheumatic fever was made with silent carditis+polyarthralgia in two patients, with silent carditis+monoarthritis in two patients, with polyarthralgia in four patients, and with monoarhtritis in one patient in our study. A diagnosis could be made by means of the updated criteria in a total of 9 (18%) patients. When compared with the previous study, an increase in the rate of silent carditis (from 21.8% to 36%) and a reduction in the rate of total carditis (from 92% to 84%) were found. CONCLUSION: Our results show that the updated Jones criteria prevent under diagnosis of acute rheumatic fever in an important number of patients.