Determination of anomalous pulmonary venous return with high-pitch low-dose computed tomography in paediatric patients.

BACKGROUND: In this study, we aimed to image pulmonary venous return anomalies and associated cardiovascular and pulmonary abnormalities by high-pitch low-dose computed tomography (CT) in children. MATERIALS AND METHODS: Forty-one patients with total or partial anomalous pulmonary venous return anomalous between May 2012 and June 2019 were retrospectively reviewed. The anomalies were determined using high-pitch low-dose CT. The patients' mean age was 3 years (6 months to 15 years), and 24 of them were female. RESULTS: There were 10 patients with total pulmonary venous return anomalies (TPVRA) and 31 patients with partial pulmonary venous return anomalies (PPVRA). Six (60%) patients with TPVRA had the supracardiac type, 2 (20%) had the cardiac type, and 2 (20%) had the mixed type. All patients with TPVRA had a large atrial septal defect (ASD), 1 patient also had patent ductus arteriosus, and 1 patient had right cardiac hypertrophy. Forty cases of PPVRA were found in 31 patients. Twenty-seven (67%) of them were right-sided, and 13 were left-sided (33%). Twenty (65%) patients also had an additional cardiovascular anomaly (ASD in 12 patients, persistent superior vena cava in 4 patients, patent ductus arteriosus in 3 patients, and aortic coarctation in 2 patients). Of the 27 patients with right-sided PPVRA, it drained into the superior vena cava in 19 patients, the right atrium in 5 patients, and the inferior vena cava in 3 patients. In left-sided cases, the anomalous pulmonary vein drained into the left innominate vein in 9 patients, and in 4 patients, there were accessory pulmonary veins that drained into the left innominate vein. Many of the patients had additional lung anomalies, including pneumonic infiltration (n = 12), atelectasis (n = 8), and lobar emphysema (n = 5), and some of these findings coexisted. CONCLUSIONS: Anomalous pulmonary venous drains and associated cardiac and extra-cardiac anomalies can be detected reliably and quickly with high-pitch low-dose CT without sedation in paediatric patients.

Paroxysmal Complete Atrioventricular Block: A Rare Cause of Syncope in Children.

Paroxysmal atrioventricular block (PAVB) is a rare cause of syncope, mostly reported in adults. In the present study, a child with recurrent syncope due to PAVB is reported. A 12-year old boy was admitted due to syncope. Some syncopal episodes were consistent with vasovagal syncope, while others had atypical features. Electrocardiography, echocardiography and 24-hour electrocardiography monitoring were normal. Head-up tilt table test revealed mixed type vasovagal syncope. With beta-blocker treatment, the frequency of syncopal episodes decreased; however, the episodes with atypical features continued. Intracardiac electrophysiological study was normal, but repeated external loop recorder monitoring revealed transient AVB during a syncopal episode. In children with syncope with atypical features, PAVB should be kept in mind. Repeated external loop recorder monitoring may be useful in the diagnosis of such seldom seen arrhythmic causes.

The utility of multidetector computed tomography for evaluation of congenital heart disease.

BACKGROUND: Congenital heart diseases (CHD) are the leading cause of birth defect-related deaths. Multi detector computed tomography (MDCT) plays an important role for imaging CHD in addition to echocardiography and provides a comprehensive evaluation of complex heart malformations for the referring cardiologist. The aim of the study was to evaluate the utility of MDCT in the assessment of CHD. MATERIALS AND METHODS: A 102 patients with CHD were investigated after initial assessment by echocardiography. The information obtained by MDCT and findings of echocardiography were reviewed together by paediatric cardiologists and cardiac radiologists. Perioperative anatomic descriptions, wherever available(n = 34) formed the gold standard for the comparison. RESULTS: The clinical consensus diagnosis defined 154 cardiovascular lesions in the patients. The results were classified in groups. We present the appearance of various congenital cardiac lesions seen in clinical practice. CONCLUSIONS: MDCT provides important information about anatomic details of CHD for the referring cardiologist. The evaluation of different anatomic structures such as heart, great vessels, lungs and abdomen is possible in one acquisition with this technique.

Mycotic pseudoaneurysm of the ascending aorta following purulent pericardial effusion diagnosed by multi-slice computed tomography.

Mycotic pseudoaneurysm of the aorta is an uncommon disease, especially in childhood but has a high mortality due to spontaneous rupture. It is caused by endarteritis following bacteraemia or fungaemia. Due to spontaneous rupture, early diagnosis is very important.

Cardiac multidetector computed tomography (MDCT) of spontaneously closed ventricular septal defect.

PURPOSE: The authors present the findings of contrast-enhanced electrocardiogram (ECG)-gated cardiac computed tomography (CT) in 18 patients with probably spontaneous closure of muscular ventricular septal defect (VSD). MATERIALS AND METHODS: The study included 2,725 consecutive patients referred to our hospitals for multidetector computed tomography (MDCT) coronary angiography. The patients were between the ages of 5 and 78 [mean+/-standard deviation (SD) 59+/-13.86] years, and 1,816 (66.6%) were male. Evaluating the images for coronary artery pathologies revealed pouches or sacs in the central muscular location of the septa of some patients. RESULTS: All patient records were retrospectively reviewed, and 18 patients were found to have pouches or sacs in the interventricular septum location likely to be spontaneous closure of muscular VSD (0.66% prevalence). MDCT findings of these 18 patients correlated with their echocardiographic findings. Of the patients with muscular VSD, 13 were male and five were female. These 18 patients were initially referred due to indications such as chest pain (n=11), risk-factor assessment (n=3), coronary artery anomaly (n=1), suspected aberrant right subclavian artery due to dysphagia (n=1) and coronary artery bypass graft (n=2). CONCLUSIONS: Our study shows that the incidence of spontaneous closure of muscular VSD with central septum location is probably higher than expected. Thus, some patients initially diagnosed with ventricular diverticula with an apical and marginal septum location may actually have spontaneously closed muscular VSD. This finding may affect previously reported rates of both ventricular diverticula and spontaneous closure of muscular VSD.

Carotid intima-media thickness: is it correlated with stroke side?

OBJECTIVE: The objective of the present study was to investigate the possible correlation between the common carotid artery (CCA) intima-media thickness (IMT) and the infarct side. METHOD: The CCA IMTs in patients with atherosclerotic non-lacunar stroke were measured. RESULTS: The mean age of the patients was 64.3 +/- 10.7 years (range 40-83 years) and 42 of 100 patients were male. The infarcts were at the left side in 53 patients and at the right side in 47 patients. The mean CCA IMT was 1.02 +/- 0.18 mm at the infarct side and 0.87 +/- 0.17 mm at the contralateral side. The difference between them was statistically significant (P < 0.01). Although the mean age of the patients with a left-sided infarct was greater than that of the patients with a right-sided infarct, the difference was not statistically significant. CONCLUSION: Our results suggest that CCA IMT may be used in prediction of possible infarct side, and in the prediction of potential risk of stroke by evaluating the IMT of both CCAs separately.

Serum carnitine levels during the doxorubicin therapy. Its role in cardiotoxicity.

Doxorubicin is an anthracycline antibiotic with a broad spectrum of antineoplastic activity. Cardiotoxicity is a serious long-term complication of the drug. Simultaneous administration of carnitine has been proposed to prevent cardiotoxicity. We aimed to monitor the serum carnitine levels during the treatment of doxorubicin and to determine a relationship between serum carnitine levels and cardiac dysfunction. Fifteen patients were evaluated prospectively. Measurement of carnitine levels and evaluation of cardiac function were performed prior to treatment, and after cumulative doses of 180 and 300 mg/m2 of doxorubicin. A group of 20 healthy children served as control group to obtain reference values. We found subclinical abnormalities in cardiac function, while the cumulative doses of the doxorubicin was increasing. The mean end diastolic and end systolic left ventricular dimensions of the patient group after completion of the treatment were significantly increased compared with initial values. The ejection and shortening fraction of the patient group after cumulative doses of 300 mg/m2 of doxorubicin were significantly lower than those of the control group. A statistically significant augmentation was observed in mitral A, with a decrease in mitral E/A ratio. There was a trend towards lower serum carnitine levels with higher cumulative doses of doxorubicin, although it was not statistically significant. Our results invite new detailed investigations depending on the measurement of serum and urinary free and acyl carnitine and myocardial carnitine levels to evaluate possible roles of carnitine in the prevention of doxorubicin-induced cardiotoxicity.

Microdeletion of 22q11 (CATCH 22) in children with conotruncal heart defect and extracardiac malformations.

CATCH 22 is a medical acronym for cardiac defects, abnormal facies, thymic hypoplasia, cleft palate, and hypocalcemia, and a variable deletion on chromosome 22q11. The deletion within the chromosome region of 22q11 may occur in patients with dysmorphologic and cardiological syndromes: DiGeorge syndrome (DGS), velocardiofacial syndrome (VCFS), and conotruncal anomaly face syndrome (CAFS). In this study, using N25 (D22S75) DiGeorge chromosome region probe. fluorescence in situ hybridization (FISH) analyses were performed on 32 patients with congenital heart diseases. Twenty-nine of 32 patients had conotruncal heart disease. A 22q11 deletion was detected in two patients (6.9%) of the 29 patients with conotruncal heart disease. One of our 22qdel (+) patients had unilateral facial nerve palsy. Although it is not a frequent finding, unilateral facial nerve palsy will be included among the symptoms of CATCH 22 syndrome. After careful clinical evaluation of patients with conotruncal cardiac anomalies, only syndromic cases should be screened for this deletion.

A case of isotretinoin embryopathy with bilateral anotia and Taussig-Bing malformation.

We report a newborn infant with multiple congenital anomalies (anotia and Taussig-Bing malformation) due to exposure to isotretinoin within the first trimester. In this paper we aim to draw to the fact that caution is needed when prescribing vitamin A-containing drugs to women of childbearing years.

Comparison of mid-term clinical experience with steroid-eluting active and passive fixation ventricular electrodes in children.

Although active fixation ventricular leads seem to have advantages over passive fixation leads, this study compares the follow-up results of active and passive fixation leads in children. We evaluated the implantation and follow-up data of 41 children with active (Accufix II DEC, group 1) (n = 20) or passive (Membrane E, group 2) (n = 21) fixation, steroid-eluting ventricular leads. All but one of the patients in group 1 completed the 12-month follow-up. The mean follow-up period in group 2 was 10.4 +/- 2.9 months (range 3-12 months, median 12 months). In both groups the mean pacing threshold was measured as 0.51 +/- 0.09 V versus 0.48 +/- 0.15 V (P > 0.05) at 0.5-ms pulse width, mean R wave amplitude as 9.9 +/- 2.5 mV versus 9.4 +/- 3.2 mV (P > 0.05), and mean impedance as 557 +/- 92 omega versus 664 +/- 160 omega (P < 0.05), respectively, at implantation. After the first week of pacing, mean threshold values in group 1 were significantly lower than those of group 2 (P < 0.01 and P < 0.05, respectively). During the follow-up period, lead impedance measurements did not show a significant difference between the two groups. In one patient from group 1, the lead (by unscrewing) was removed easily because of pacemaker pocket infection. No lead dislodgement or helix deformation occurred in group 1. Nevertheless, in one patient from group 2, the lead was extracted at 4-month postimplantation because of lead displacement. We conclude that the steroid-eluting active fixation lead (Accufix II DEC) have advantages of easier implantation and lower acute and chronic stimulation thresholds compared to the passive fixation lead (Membrane E). Therefore, Accufix II DEC is superior to Membrane E, and it is a better first choice in children with an implanted single chamber ventricular pacemaker.

Clinical and genetic correlate in childhood onset Friedreich ataxia.

We analyzed the clinical and genetic aspects of 28 FRDA patients from 20 families. 19 families were consanguineous. The onset was between 4 and 13 1/2 years of age (mean 15.4 +/- 6.2). Three patients presented with cardiomyopathy, one with weakness, and the rest with ataxia. There were two patients with preserved lower-limb deep tendon reflexes. Sensory nerve action potentials were reduced in 14/14 patients. Cardiac echograms were abnormal in 17/19 cases, and this was between 6 and 16 years of age (mean 10.1 +/- 3.5). Four families were multiplex. Clinical intra-familial variability was observed. Increased GAA repeats of the X25 gene were found in 27/28 patients studied, all in a homozygous state. 88.9% of patients had a smaller allele larger than 500 repeats, and 66.7% had more than 700 repeats. The patient who did not have increased GAA repeats in both alleles had peculiar findings. Significant correlation of expansion was obtained for the early onset, and cardiomyopathy as the onset.

Torsade de pointes associated with encephalitis.

Torsade de pointes is a polymorphic ventricular tachycardia. Causes of torsade de pointes are well described. Although intracranial disease can produce dramatic electrocardiographic (ECG) changes, we are not aware of previous cases with torsade de pointes and encephalitis. We report a case with encephalitis who developed torsade de pointes, and was treated with temporary ventricular pacing and magnesium infusion.

Vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum: a rare cause of respiratory distress in newborn infants.

Vascular ring, in which the trachea and esophagus are completely surrounded by vascular structures, is one of the causes of respiratory distress in children. Right aortic arch with aberrant left subclavian artery is a common aortic arch anomaly; however, respiratory distress due to vascular ring is seldom associated with this anomaly. We report herein a newborn infant treated surgically because of severe respiratory distress caused by vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. As laryngomalacia was first thought to be the reason for respiratory distress, we suggest that patients with respiratory distress diagnosed with laryngomalacia be evaluated for possible vascular ring.