Determinants of Increased Aortic Diameters in Young Normotensive Patients With Turner Syndrome Without Structural Heart Disease.

Factors associated with aortic dilation and dissection in patients with Turner syndrome (TS) remain unclear. We assessed magnetic resonance imaging-based aortic diameters at nine predefined anatomic positions and examined associations of increased aortic diameters with B-type natriuretic peptide (BNP), A-type NP (ANP), growth hormone treatment, insulin-like growth factor 1 (IGF1), and estrogen status. Forty-seven patients with TS aged 7.3-21 years and 34 healthy peers were enrolled in this study. Aortic diameters were higher in patients with TS at three positions than in controls (p < 0.05). History of GH treatment, pubertal status, and serum estradiol levels were not associated with increased aortic diameters. Patients with TS had higher plasma BNP and ANP levels than controls. BNP and IGF1 were independently associated with the increase in aortic diameters in TS at three positions of the ascending aorta (R2 = 0.361-0.458, p < 0.05 for all). At two positions of the descending aorta, only BNP emerged as an independent variable (R2 = 0.130-0.139, p < 0.05). We conclude that young, normotensive patients with TS had greater aortic diameters at several positions than healthy controls. BNP and IGF1 were independently associated with increased aortic diameters in TS.

Increased arterial stiffness in young normotensive patients with Turner syndrome: associations with vascular biomarkers.

OBJECTIVES: Factors contributing to arteriopathy in patients with Turner syndrome (TS) remain unclear. We assessed arterial stiffness in young, normotensive patients with TS and correlated arterial stiffness with vascular biomarkers, GH treatment and oestrogen exposure. Sixty-one patients with TS (mean age, 12·6 years; range 6·6-21·3 years) were matched for age and sex with 61 healthy peers. Associations between arterial stiffness and high-sensitivity C-reactive protein (hsCRP), B-type natriuretic peptide (BNP), atrial NP (ANP), plasma aldosterone/plasma renin activity (PRA), IGF1 and IGFBP3 were examined after adjusting for well-established confounders of vascular disease. RESULTS: Carotid intima media thickness standard deviation score (SDS), arterial stiffness index SDS and incremental modulus of elasticity SDS were higher, and distensibility coefficient SDS was lower in patients with TS. The duration of GH treatment and oestrogen exposure was not associated with indices of arterial stiffness. TS patients had higher hsCRP, BNP and ANP. Plasma aldosterone/PRA, IGF1 and IGFBP3 were similar in patients and controls. Multivariable regression analyses (R(2) = 0·200-0·668, P < 0·01) showed that BNP was associated with all indices of arterial stiffness. We found that hsCRP was associated with distensibility coefficient SDS (ß = -0·16, P < 0·01). TS was independently associated with increased arterial stiffness (ß = 0·420-3·424, P < 0·001 for all, R(2) = 0·06-0·31). CONCLUSIONS: Young, normotensive TS patients had increased arterial stiffness than that of healthy peers. BNP, and possibly hsCRP, was independently associated with arterial stiffness in TS. Further research will determine any causal inference of these relationships.

Investigation of cardiomyopathy in children with cirrhotic and noncirrhotic portal hypertension.

BACKGROUND: Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). METHODS: This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. RESULTS: The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). CONCLUSIONS: Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.

Doppler-derived strain imaging detects left ventricular systolic dysfunction in children with Turner syndrome.

OBJECTIVES: Children with Turner syndrome (TS) are at increased risk of cardiovascular disease (CVD), but associations with subclinical CVD are not well-characterized. The purpose of this study was to assess myocardial function using strain imaging (SI) by echocardiography in children with TS and without known CVD. METHODS: The study included 48 children with TS aged 4-16 years and 20 healthy control children. Children with TS were excluded if they had a cardiac malformation, a decreased left ventricular (LV) systolic function, or any chronic disease. Each child had an echocardiographic examination with conventional echocardiography and one-dimensional longitudinal strain (1DST) echocardiography. RESULTS: Septal and lateral systolic strain (S) and strain rate (SR) values, which are indicative of longitudinal myocardial function, were significantly decreased in TS patients. However, LV ejection fraction (LVEF) and LV fractional shortening (LVFS) was not significantly different between groups. LV mass index (LVMi), interventricular septum (IVS) thickness, LV posterior wall (LVPW) thickness, and left atrial (LA) diameter index were significantly higher in TS children compared to controls. Peak transmitral flow velocity in late diastole (peak A) was significantly higher, whereas peak transmitral flow velocity in early diastole (peak E), deceleration time (DT), and the ratio of early to late diastolic filling were significantly lower, in TS patients. CONCLUSION: Reduced LV systolic S and SR in children with TS may indicate early myocardial dysfunction before any detectable change in LVEF.

Aborted sudden cardiac death in a child with left ventricular non-compaction.

Left ventricular non-compaction is a rare form of cardiomyopathy believed to be the result of intrauterine arrest of compaction of the endomyocardial morphogenesis, leading to persistence of the embryonic myocardium. Clinical manifestations are highly variable, ranging from no symptoms to a progressive deterioration in cardiac function that results in congestive heart failure, systemic thromboemboli, arrhythmias, and sudden cardiac death. Presented here is the case of a 4-year-old child with a history of aborted sudden cardiac death. Following resuscitation, he was admitted to the intensive care unit with neurologic sequelae that regressed later on. Transthoracic echocardiography and magnetic resonance imaging showed numerous prominent trabeculations and deep intertrabecular recesses at the apical and anterolateral region of the left ventricle. Electrophysiologic study showed polymorphic ventricular tachycardia. An implantable cardioverter-defibrillator (ICD) was implanted following clinical recovery. Five months after implantation, appropriate ICD shock due to ventricular fibrillation was documented.

Jacobsen syndrome without thrombocytopenia: a case report and review of the literature.

Jacobsen syndrome (JS), a rare disorder with multiple dysmorphic features, is caused by the terminal deletion of chromosome 11q. Typical features include mild to moderate psychomotor retardation, trigonocephaly, facial dysmorphism, cardiac defects, and thrombocytopenia, though none of these features are invariably present. The estimated occurrence of JS is about 1/100,000 births. The female/male ratio is 2:1. The patient admitted to our clinic at 3.5 years of age with a cardiac murmur and facial anomalies. Facial anomalies included trigonocephaly with bulging forehead, hypertelorism, telecanthus, downward slanting palpebral fissures, and a carp-shaped mouth. The patient also had strabismus. An echocardiogram demonstrated perimembranous aneurysmatic ventricular septal defect and a secundum atrial defect. The patient was <3rd percentile for height and weight and showed some developmental delay. Magnetic resonance imaging (MRI) showed hyperintensive gliotic signal changes in periventricular cerebral white matter, and leukodystrophy was suspected. Chromosomal analysis of the patient showed terminal deletion of chromosome 11. The karyotype was designated 46, XX, del(11) (q24.1). A review of published reports shows that the severity of the observed clinical abnormalities in patients with JS is not clearly correlated with the extent of the deletion. Most of the patients with JS had short stature, and some of them had documented growth hormone deficiency, or central or primary hypothyroidism. In patients with the classical phenotype, the diagnosis is suspected on the basis of clinical findings: intellectual disability, facial dysmorphic features and thrombocytopenia. The diagnosis must be confirmed by cytogenetic analysis. For patients who survive the neonatal period and infancy, the life expectancy remains unknown. In this report, we describe a patient with the clinical features of JS without thrombocytopenia. To our knowledge, this is the first case reported from Turkey.

Prognostic Value of Tpeak-Tend Interval in Early Diagnosis of Duchenne Muscular Dystrophy Cardiomyopathy.

The most common cause of death in patients with Duchenne muscular dystrophy (DMD) is cardiomyopathy. Our aim was to investigate the relationship between the Tpeak-Tend (Tp-e) interval and the premature ventricular contraction (PVC) burden and therefore early arrhythmic risk and cardiac involvement in DMD patients. Twenty-five patients with DMD followed by pediatric cardiology were included in the study. Those with a frequency of <1% PVC in the 24 h Holter were assigned to Group 1 (n = 15), and those with >1% were assigned to Group 2 (n = 10). Comparisons were made with healthy controls (n = 27). Left ventricular ejection fraction (LVEF) was lowest in Group 2 and highest in the control group (p < 0.001). LV end-diastolic diameter was greater in Group 2 than in Group 1 and the control group (p = 0.005). Pro-BNP and troponin levels were higher in Group 1 and Group 2 than in the control group (p = 0.001 and p < 0.001, respectively). Tp-e interval was longer in Group 2 compared to Group 1 and the control group (p < 0.001). The LVEF (OR 0.879, 95% CI 0.812-0.953; p = 0.002) and Tp-e interval (OR 1.181, 95% CI 1.047-1.332; p = 0.007) were independent predictors of PVC/24 h frequency of >1%. A Tp-e interval > 71.65 ms predicts PVC > 1%, with a sensitivity of 80% and a specificity of 90% (AUC = 0.842, 95% CI (0.663-1.000), p = 0.001). Determination of Tp-e prolongation from ECG data may help in the determination of cardiac involvement and early diagnosis of arrhythmic risk in DMD.

Evaluation of the North Star Ambulatory Assessment scale and cardiac abnormalities in ambulant boys with Duchenne muscular dystrophy.

AIM: We evaluated ambulatory patients with Duchenne muscular dystrophy from the cardiovascular standpoint and studied the correlation between the results of electrocardiographic (ECG) findings, left ventricular ejection fraction (LVEF), troponin T and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and patients' North Star Ambulatory Assessment scores. METHODS: Fifty patients of ages 6-12 (8.9 ± 2.8) were enrolled in this cross-sectional study. Cardiac evaluation included electrocardiography, echocardiography and cardiac enzyme tests. RESULTS: North Star scores ranged from 6/34 to 34/34. Twenty-eight patients (56%) had ECG changes. The most frequently seen ECG abnormalities were short PR interval (14%, n= 7), right ventricular hypertrophy (16%, n= 8), prolonged QTc interval (10%, n= 5), prominent Q wave (10%, n= 5) and T wave inversion (44%, n= 22). In 10 patients (20%), LVEF was below 55%, troponin T and NT-proBNP levels were significantly elevated (P= 0.003 and P < 0.001, respectively). When North Star scores were compared to patients' age, enzyme levels, ECG and echocardiographic results, we discovered negative correlation with age (P < 0.001) and troponin T levels (P= 0.02) and positive correlation with LVEF (P= 0.02). CONCLUSION: Patients with North Star scores of ≤16 are more at risk of developing cardiomyopathies. Troponin T is a cardiac index that can be used for evaluating myopathic patients and it seems to be correlated with the proBNP levels and LVEF values.

Cardiovascular abnormalities in Williams syndrome: 20 years'experience in Istanbul.

AIMS: Williams syndrome (WS) is a microdeletion syndrome affecting cardiovascular and connective tissue as well as the endocrine and central nervous systems in 1 in 10,000 live births. This study aims to identify and evaluate cardiovascular abnormalities (CVAs) in 45 WS patients. PATIENTS AND METHODS: We retrospectively reviewed a cohort of WS patients who were followed at our institution from January 1, 1990 through December 31, 2010. WS was clinically diagnosed by an experienced medical geneticist and confimed by fluorescence in situ hybridization. CVAs were assessed using electrocardiography, echocardiography or cardiac catheterization. RESULTS: Twenty-seven patients (60%) were male; 18 were female (40%).The mean age at presentation was 4.6 +/- 3.1 years (3 months-13 years); the follow-up period was 6.9 +/- 4.4 years (6 months-18 years). CVAs were found in 86% of patients, the most common one being supravalvar aortic stenosis (SVAS) in 73% (isolated in 48%), peripheral pulmonary artery stenosis (PAS) in 42%, and mitral valve prolapse (MVP) in 22%. Less common were aortic insufficiency (15%), ventricular septal defect (11%), valvular pulmonary stenosis (11%), and aortic arch hypoplasia (8%) and coarctation (2%). Hypertension was present in 22% of patients. Surgical or catheter-based interventions were performed in 22% of cases. Two patients were lost in the postoperative period. CONCLUSION: CVAs were found in more than four out of five patients, the most common ones being SVAS and PAS. Although surgery was performed in more patients with SVAS than with PAS, SVAS was minimal or mild in most patients and improved in few cases.

Transcatheter Closure of Atrial Septal Defect in Children: Single-Center Experience, Mid-Term Follow-up Results.

OBJECTIVE: Atrial septal defect is a congenital heart disease usually diagnosed in childhood. This study aimed to evaluate the mid-term follow-up results of patients who underwent trans- catheter closure of atrial septal defect by comparing the devices and methods used in the procedure and investigating the complications of this procedure in children. MATERIALS AND METHODS: This study evaluated 232 patient files retrospectively. Of the 232 patients, 24 were excluded from the study due to missing files or data. Also, patients with multi-fenestrated atrial septal defect and aneurismatic septal tissue were excluded from the study. The following data were evaluated: follow-up time, patient complaints, symptoms, trans- thoracic echocardiography, and transesophageal echocardiography findings (if performed), the size of the defect as measured by balloon-sizing, the size of the device used in the proce- dure, and major and minor complications. RESULTS: The study included 208 children who were diagnosed with atrial septal defect. The mean age of the patients was 88.0 ± 56.5 months. Of the patients, 170 (81.7%) had no com- plaints. The success rate of the procedure was found to be 95.7%. While device embolization was the most common major complication, arrhythmia was the most common minor complica- tion. The complication rate was statistically different according to the device type used in the procedure. CONCLUSION: Transcatheter closure of atrial septal defect is a safe method for atrial septal defect closure in pediatric patients. The study found that defect diameters measured by differ- ent methods were not correlated with each other. The procedure complication rates differed according to device type.

Electrocardiographic findings at initial diagnosis in children with isolated left ventricular noncompaction.

BACKGROUND: The aim of this study was to comprehensively evaluate electrocardiographic (ECG) findings of isolated left ventricular noncompaction (IVNC) patients at initial diagnosis and to explore the correlation between them and the clinical, echocardiographic, and magnetic resonance imaging (MRI) findings. METHODS: Twenty-three patients diagnosed with IVNC by echocardiography and cardiac MRI between January 2006 and June 2010 were enrolled in this study. The patients were examined with standard ECG and 24-hour Holter ECG. For comparison purpose, ECGs of 50 healthy children of similar ages and demographic characteristics were taken. RESULTS: In 87% of patients, ECG abnormalities were found. The most frequently seen ECG findings were left ventricular hypertrophy, ST-segment depression, and negative T wave related to abnormal repolarization particularly in DII, DIII, and V(4-6) leads, as well as prolonged PR and QTc intervals. No ECG features or patterns were found that were specific to the disease. In contrast to adult patients, while no intraventricular conduction defects (particularly in the left bundle brach) were found in any of our patients, 13% had considerable bradycardia and one required a pacemaker. The Holter ECG recordings showed supraventricular tachycardia attacks in two patients and a short ventricular tachycardia attack in one. Patients whose echocardiograms and MRI showed left ventricular systolic dysfunction and left ventricular dilatation had signs of left ventricular hypertrophy and repolarization abnormality on their ECGs, but there was no significant difference in PR, QRS, and QTc intervals. CONCLUSION: Regardless of how frequently left ventricular hypertrophy and repolarization abnormalities are found on IVNC patients' initial ECGs, we think that they are not unique to the disease but are related to the severity of the cardiomyopathy.

[Acute cyanosis after transcatheter balloon valvuloplasty: toxic methemoglobinemia due to local prilocaine use]. / Transkateter balon valvüloplasti sonrasi gelisen akut siyanoz: Lokal prilokain kullanimina bagli toksik methemoglobinemi.

Methemoglobinemia is characterized by varying degrees of cyanosis due to increased hemoglobin concentrations containing oxidized iron. Prilocaine is a widely used local anesthetic and can cause methemoglobinemia in infants even in therapeutic doses. We present two female infants (younger than 2 months) who developed severe cyanosis after transcatheter pulmonary balloon valvuloplasty and were diagnosed with toxic methemoglobinemia. Both infants were anesthetized with local prilocaine application before balloon valvuloplasty. Methemoglobin levels of the patients were measured as 49.6% and 37.7%, respectively. Both were successfully treated with intravenous methylene blue and ascorbic acid.

[A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome]. / Tekrarlayan hisilti ve solunum sikintisinda nadir bir neden: Scimitar sendromu.

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.

Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the Amplatzer septal occluder.

A congenital fistula between the right pulmonary artery (RPA) and left atrium (LA) is a rare condition that results in central cyanosis. An 11-year-old boy was admitted with exertional dyspnea and easy fatigability. He had severe cyanosis of the lips and limbs with clubbing of the fingers. Systemic oxygen saturation was 70%. There was no abnormal finding on electrocardiography, chest radiography, and echocardiography. Agitated saline injection showed early appearance of contrast bubbles in the LA. A pulmonary arteriovenous fistula was suspected and diagnostic cardiac catheterization was performed. Angiography demonstrated a large fistula between the proximal RPA and LA. The narrowest part of the fistula was 13.8 mm in balloon sizing. A 14-mm Amplatzer septal occluder was deployed at the narrowest site; however, the device migrated to the LA and then to the aortic arch. The device was removed and was successfully reimplanted to the fistula. After the procedure, arterial oxygen saturation increased from 70% to 96% and control angiography demonstrated complete occlusion of the fistula. The patient was symptom-free on follow-up evaluations at 6, 12, and 18 months, with a mean oxygen saturation of 96%. This case represents the first pediatric patient in whom a septal occluder was used.

[Dilated cardiomyopathy associated with dystrophic epidermolysis bullosa: role of micronutrient deficiency?]. / Distrofik epidermolizis bülloza ile iliskili dilate kardiyomiyopati: Mikrobesin eksikligi mi?

Autosomal recessive dystrophic epidermolysis bullosa (DEB) is a chronic skin disorder characterized by widespread bullous formation, erosions, and scar formation. There have been reports of dilated cardiomyopathy and death in patients with DEB. The pathogenesis of cardiomyopathy in DEB remains uncertain, but some drugs, viral infections, iron loading, micronutrient deficiencies such as selenium and carnitine have been implicated. A 16-year-old boy who was followed-up from birth with the diagnosis of DEB presented with respiratory distress and heart failure symptoms of two-week history and early fatigue within the past year. Etiological evaluation showed a low plasma selenium level. Echocardiographic examination yielded the diagnosis of dilated cardiomyopathy. Findings of viral serology tests and metabolic screening were normal. Selenium replacement and anticongestive treatment were initiated, which led to partial improvement in cardiac functions. The authors draw attention to the possible role of micronutrient deficiency in the development of cardiomyopathy in patients with DEB.

Kearns-Sayre syndrome presenting as somatomedin C deficiency and complete heart block.

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease in which neuromuscular structures, endocrine glands, and cardiac conduction systems are most commonly involved. An 11-year-old boy was admitted with blurred consciousness, respiratory instability, and bradycardia of two-hour onset. He was immediately intubated. His medical history included growth retardation and myopic refractive defect for six years, therapy for somatomedin C deficiency for 15 months, and bilateral ptosis for three months. On physical examination, he was unconscious, had a peak heart rate of 40/min, blood pressure of 60/20 mmHg, and a weak pulse. Laboratory findings showed elevated blood lactate and blood pyruvate levels and an increased lactate/pyruvate ratio. The electrocardiogram showed complete atrioventricular block and echocardiography showed mitral valve prolapse. Following implantation of a temporary transvenous cardiac pacemaker, his heart rate and clinical condition improved. Further analysis with cranial magnetic resonance (MR) imaging demonstrated hyperintense signal changes in the subcortical white matter of the two cerebral hemispheres, bilateral thalamus, putamen, cerebral peduncles, dorsal medulla, and midbrain. The typical clinical and MR findings confirmed the initial diagnosis of KSS. A permanent cardiac pacemaker was implanted into the right ventricle.

Acute pericarditis and transient erythroblastopenia associated with human parvovirus B19 infection.

We report on an eight-year-old girl with acute pericarditis and transient erythroblastopenia associated with human parvovirus B19 (PVB19) infection. The patient presented with complaints of fever, chest pain, fatigue, and shortness of breath. On physical examination, she had tachycardia, hepatomegaly, and muffled heart sounds. Teleradiography exhibited cardiomegaly and echocardiography showed a pericardial effusion of 25 mm. Serum anti-PVB19 IgM and PVB19 DNA were positive. The patient developed anemia and reticulocytopenia in the second week, both of which persisted for two weeks then resolved spontaneously. At the end of three months, pericardial effusion resolved, hemoglobin and hematocrit levels were normal, and serum anti-PVB19 IgM was negative. This case represents the first report of acute pericarditis associated with PVB19 infection in a pediatric patient.

The effect of DALI lipid apheresis in the prognosis of homozygous familial hypercholesterolemia: Seven patients' experience at a DALI apheresis center.

INTRODUCTION: Familial hypercholesterolemia (FH) is characterized by severe hypercholesterolemia that can result in coronary artery disease occurring at an early age. If patients are not cured with lipid-lowering drugs and diets, lipid apheresis may be an effective treatment option in these cases. Here, we evaluate the efficacy, selectivity and safety of the DALI apheresis technique. MATERIALS AND METHODS: Seven pediatric patients (2 girls; 5 boys) with ages between 7 and 14 years (mean age: 6.5±2.1 years) with HFH were included in this study. We restrospectively evaluated clinical and laboratory findings. We used the DALI system for lipid apheresis concomitant with medical treatment and diet for hyperlipidemia. RESULTS: The cohort's mean T.cholesterol level prior to apheresis was 700.57±136.36 mg/dl,the mean LDL-C value was 526.86±131.56 mg and the mean HDL-C level was 36.57±4.58 mg/dl.The mean cholesterol levels after apheresis were consecutively 317.57±93.70 /257.29±90.38 / 33.36±4.78 mg/dl.We noted a 51.1% reduction in LDL-C level and an 8.7% reduction in HDL-C level in our apheresis sessions.The reduction in LDL-C was statistically significant (p<0.05). During 1025 apheresis therapy, the most frequent mild and moderate adverse events were deviceaccess problems and hypotension (in all patients);severe adverse events were mainly due to cardiac problems(myocardial infarct and arrhythmia) and hypotension. CONCLUSION: Lipid apheresis is an inevitable alternative treatment for HFH. Despite all of its application problems, DALI system is an effective therapy for decreasing atherogenic lipids from circulation.

Quantification of Hepatic and Splenic Stiffness After Fontan Procedure in Children and Clinical Implications.

We aimed to investigate Fontan associated liver disease in children by shear wave elastography (SWE). This is a single-center, prospective case-control study included 41 patients with Fontan physiology and 30 healthy controls. Hepatic and splenic shear wave elasticity values were exhibited both as kPa and m/s. The mean hepatic SWE values of Fontan patients (n = 41; 15.8 ± 3.2 kPa or 2.5 ± 1.8 m/s) were significantly higher than the control group (n = 30; 5.59 ± 0.6 kPa or 1.37 ± 0.07 m/s) (P < 0.001). The mean splenic SWE values of Fontan patients were (25.6 ± 4.61 kPa or 2.85 ± 0.22 m/s) significantly higher than the control group (15.9 ± 1.44 kPa or 2.29 ± 0.1 m/s) (P < 0.001). There were statistically significant positive correlations among the follow-up duration after the Fontan procedure with NT-proBNP (P = 0.008, r = 1) and prothrombin time (P = 0.009, r = 0.4) as well as the hepatic SWE values with alanine aminotransferase (P = 0.039, r = 0.32), gamma-glutamyl transferase (P = 0.045, r = 0.31), and PT (P = 0.011, r = 0.39). There has been statistically significant moderate positive correlations of splenic stiffness values with PT (P = 0.047, r = 0.34), and INR (P = 0.038, r = 0.35). The sensitivity and specificity of liver stiffness cutoff value as 11.1 kPa for detection of Fontan associated liver disease were 95% and 100%, respectively. The hepatic and splenic stiffness increase independently in Fontan patients due to parenchymal disease. Hepatic SWE is a reliable and noninvasive predictor of early hepatic alterations that could not be detected only by biochemical results or routine ultrasound examinations.

Transient complete atrioventricular block during liver transplantation.

Intraoperative dysrhythmias commonly occur in the surgical management of congenital heart diseases. It may also be seen in other surgical procedures. The initiating factors for an arrhythmia during surgery is usually a transient insult such as hypoxemia, cardiac ischemia, catecholamine excess, electrolyte abnormality and acidosis. CAVB is a life-threatening dysrhythmia in all ages. We herein report a case of transient CAVB in a 30-month-old boy during living-related liver transplantation for bile duct paucity-associated liver cirrhosis. Moreover, we discuss the probable etiology and treatment of CAVB in liver transplantation.