Brachiocephalic Vein Duplication: Case Report of a Double Left Brachiocephalic Vein in an Infant.

A patient was delivered at 26 weeks (about 6 months) gestation via an emergency caesarian section. A patent ductus arteriosus (PDA) and atrial septal defect (ASD) were discovered during an echocardiogram 13 days after birth. The patient had catheter-based closure of the PDA and ASD. During a routine echocardiogram to check device placements, it was discovered that there was dilation of the superior vena cava (SVC), and it was suspected that a thrombus was present. Computed tomography angiography (CTA) was completed to better define SVC anatomy and flow acceleration. The CTA demonstrated that there was a double innominate vein.

Global Longitudinal Strain Reduction in the Absence of Clinical Cardiac Symptoms in Multisystem Inflammatory Syndrome in Children Associated with COVID-19: A Case Series.

Multisystem inflammatory syndrome in children is a term that encompasses the systemic inflammation seen in children 4-6 weeks following COVID-19 infection. Cardiac involvement is common in this condition and can range from mild myocarditis to severe hypotension and cardiogenic shock, but not all patients display overt cardiac symptoms. We present three such patients who presented with a variety of systemic inflammatory symptoms but lacked apparent cardiac symptoms, and all had normal left ventricular ejection fraction but reduced global longitudinal strain (GLS). GLS is a cardiac tissue deformation index measured by Echocardiography to detect early changes in global function even before changes in ejection fraction are seen. We suggest this finding may indicate subclinical myocardial injury and stress the need for closer evaluation and follow-up for these patients as well as further research on both the short- and long-term effects of COVID-19 on cardiac function in the pediatric population.

Fetal left and right ventricle myocardial performance index: defining normal values for the second and third trimesters--single tertiary center experience.

Myocardial performance index (MPI), or Tei index, has become a commonly used parameter for the noninvasive, Doppler-derived assessment of global systolic and diastolic performance of the heart in both adults and children. Normal values have been established in adults and children; however, limited data exist in fetal hearts. The aim of this study was to further elucidate normal values of fetal left (LV) and right ventricle (RV) MPI values in second- and third-trimester fetuses and compare these values with other previously published data. This was a retrospective study to measure MPI in healthy fetuses. After Institutional Review Board approval, 2000 fetal echocardiography studies (FES) were acquired during a period of 4 years. Demographic parameters examined included gestational age (GA), maternal age (MA), and indication for fetal echocardiography. Fetuses with congenital heart disease, arrhythmias, or significant noncardiac fetal anomalies were excluded. The following echocardiography parameters were collected: LV ejection time (LVET), mitral valve close-to-open time (MVCO), RVET, tricuspid valve CO (TVCO), and fetal heart rate. For simplicity, LV and RV MPI values were calculated as follows: LV MPI = MVCO - LVET/LVET and RV MPI = TVCO - RVET/RVET. Four hundred twenty FES met the study criteria. LV MPI was evaluated in 230 and 190 FES in the second and third trimester, respectively. Of the 420 FES, 250 (150 in the second trimester and 100 in the third trimester) had all of the measurements required for RV MPI calculation. MA ranged between 16 and 49 years. Indications for FES included diabetes mellitus (N = 140; 33 %), suspected fetal anomalies on routine obstetrical ultrasound (N = 80; 20 %), autoimmune disorder (N = 60; 14 %), family history of CHD (N = 76; 18 %), medication exposure (N = 22; 5 %), increase nuchal thickness (N = 13; 3 %), and other indications (N = 29; 6 %). Averaged LV and RV MPI values were 0.464 ± 0.08 and 0.466 ± 0.09, respectively. Further analysis based on gestational period showed slightly greater LV and RV MPI values during the third compared with the second trimester, i.e., 0.48 and 0.49, respectively, with no statistically significant difference. There was no significant association of LV and RV MPI with heart rate. To our knowledge, this is the first study to establish normal values of fetal MPI based on a large fetal population from a single tertiary center. LV and RV MPI values were independent of GA and fetal heart rate. MPI is a useful parameter for the assessment of global cardiac function in the fetus and demonstrates good reproducibility with narrow interobserver and intraobserver variability. Its usefulness should be studied in fetal hearts with complex congenital anomalies.

The pediatric cardiology pharmacopeia: 2013 update.

The use of medications plays a pivotal role in the management of children with heart diseases. Most children with increased pulmonary blood flow require chronic use of anticongestive heart failure medications until more definitive interventional or surgical procedures are performed. The use of such medications, particularly inotropic agents and diuretics, is even more amplified during the postoperative period. Currently, children are undergoing surgical intervention at an ever younger age with excellent results aided by advanced anesthetic and postoperative care. The most significant of these advanced measures includes invasive and noninvasive monitoring as well as a wide array of pharmacologic agents. This review update provides a medication guide for medical practitioners involved in care of children with heart diseases.

A case report of congenital absence of the pericardium that was diagnosed by cardiac computed tomography angiogram (CCTA).

This case report describes a 40-year-old male who presented to the emergency department (ED) with chest pain. Initial diagnostic workup was concerning for a congenital cardiac anomaly, further imaging revealed complete congenital absence of the pericardium (CAP) which is a rare condition. Multimodality cardiac imaging including cardiac computed tomography angiogram (CCTA) was used to confirm the diagnosis of CAP. We briefly discuss various clinical presentations of CAP along with potential complications and other anomalies that could be associated with pericardial agenesis.

Predictors of Cardiovascular Affection in Patients with Active Rheumatoid Arthritis: Secondary Analysis of a Randomized Controlled Trial.

OBJECTIVE: This is a secondary analysis of a randomized controlled trial that aimed to assess subclinical atherosclerosis in patients with rheumatoid arthritis (RA) by measuring carotid artery intima-media thickness (CIMT) and correlating it with disease activity and inflammatory markers (including levels of matrix metalloproteinase-3(MMP-3) and matrix metalloproteinase-9 (MMP-9)) and to detect the effectiveness of agents that inhibit matrix metalloproteinases (MMPs) as doxycycline in RA therapy. METHODS: One hundred and sixty RA patients were assigned in a randomized clinical trial (clinicaltrial. gov NCT03194204). Disease activity score 28(DAS28), laboratory markers, including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), MMP-3, and MMP-9 were evaluated and mean CIMT was measured. Subjects were allocated randomly into one of two treatment arms, either methotrexate (MTX) alone or MTX with doxycycline 200mg per day orally. Follow up ESR, CRP, DAS28, MMP-3, and MMP-9 levels were re-evaluated after 3 months. RESULTS: There were positive significant correlations between CIMT and disease duration (r = 0.461, p = 0.001), age (r=0.459, p= 0.001), DAS28 score (r= 0.547, p = 0.001), ESR (r =0.413, p = 0.001), CRP (r = 0.281, p = 0.001), MMP-3 (r = 0.476, p = 0.001), and MMP-9 (r = 0.593, p =0.001). Patients treated with MTX and doxycycline showed lower levels of DAS28, ESR, CRP, MMP-3, and MMP-9 and this was statistically significant. CONCLUSION: CIMT seems to be the ultimate method to screen for subclinical atherosclerosis in RA patients. MMP-3 and 9 play a key role in both RA synovitis and cardiovascular changes, making them important therapeutic targets, especially with safe and cost-effective agents like doxycycline. This clinical trial was carried out in Assiut University Hospital (AUH), Assiut, Egypt (Clinical Trial Registration No. clinicaltrial.gov NCT03194204).

Evaluation of transforming growth factor alpha and vascular endothelial growth factor in diagnosis of hepatocellular carcinoma.

Hepatocellular carcinoma (HCC) is one of the most frequent malignant tumors. It is important to detect disease and recurrence at its earlier period. We aimed to evaluate the usefulness of TGF-alpha and VEGF in diagnosis of HCC patients. Thirty patients with liver cirrhosis, 30 patients with confirmed HCC and 20 healthy volunteers were subjected to abdominal ultrasonography, and alpha-fetoprotein, TGF-alpha and VEGF were assessed. Serum level of AFP was significantly higher in HCC than cirrhotic patients and controls and in cirrhosis patients than controls. The level of TGF-alpha was significantly increased in HCC and cirrhosis groups than in control group with no difference between cirrhosis and HCC groups. Serum VEGF was higher in HCC than in cirrhosis group and in both groups than in control group. Sensitivity and specificity of makers in diagnosis of HCC were 63%, 90% respectively for AFP using a cutoff value of 19.96 ng/ml; 60% and 92% for VEGF at cut off 268 and 73% and 84 % for TGF-alpha using a cutoff value of 13.95 pg/ml. VEGF may be useful serum marker for detection of HCC in addition to traditional markers.

Value of serum and synovial fluid activin A and inhibin A in some rheumatic diseases.

AIM: The purpose of the study is to measure serum and synovial fluid levels of activin A and inhibin A in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and osteoarthritis (OA) and correlate them with disease activity parameters. SUBJECTS AND METHODS: This study included 60 patients with various rheumatic diseases (20 with RA, 20 with SLE and 20 with OA), as well as 10 healthy controls. All of them were subjected to complete history-taking, examination and estimation of disease activity index. The following investigations were done for all subjects: serum and synovial activin A, inhibin A, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), anti-dsDNA and complements 3 and 4. RESULTS: Serum levels of activin A were significantly higher in RA, SLE and OA than controls and in RA and SLE versus OA The mean values of serum inhibin A were significantly higher in all studied groups than controls. Synovial activin A and inhibin A were significantly higher in RA than OA. Positive correlations were found between serum activin A and disease activity parameters of RA. In SLE, positive correlations were found between serum activin A and inhibin A with ESR and SLE Disease Activity Index. CONCLUSIONS: Serum activin A and inhibin A were significantly higher in RA and SLE. Serum levels correlated positively with disease activity parameters of RA and SLE. However, synovial levels were significantly higher in RA than OA but showed no correlation or negative correlation with disease activity. We recommend further studies to detect the exact role of activin A and inhibin A in these conditions.

Helex septal occluder: feasibility study of closure of atrial septal defect.

BACKGROUND: A variety of transcatheter atrial septal defect (ASD) occluders are currently in use, the most commonly used device is the Amplatzer Septal Occluder (ASO) yet there is no perfect device. The Helex Septal Occluder is a new device (by W.L. Gore & Associates, Inc.) designed to improve the results of transcatheter ASD closure. We report our first experience in closing secundum ASDs with this new device after its recent modifications. METHODS: Thirteen patients were selected for Helex device closure with median age of 8 years (2.5-44 years) and median weight 30 Kg (12-96 Kg). Inclusion criteria were: small to moderate Secundum ASDs with sufficient rims by transthoracic echo (TTE) and confirmed by transoesophageal echo (TEE). Two cardiologists carried out the decision of device size. One of the five available Helex ASD device sizes was used (15-35 mm). Follow up TTE was performed the next day, one month, six months and one year later. RESULTS: Twelve patients had successful Helex septal occluder implantation. One patient was switched to Cribriform ASD device during the procedure because of failure of the locking mechanism. Two patients had trivial residual left to right shunt which disappeared at one month follow up in one patient. No embolic event, AV block or mortality was observed. CONCLUSION: This feasibility study of the novel Helex septal occluder after its recent modification showed that it can successfully and safely close well selected secundum ASDs. There are several advantages over the currently available devices.

Cribriform amplatzer device closure of fenestrated atrial septal defects: feasibility and technical aspects.

Fenestrated atrial septal defects (F-ASDs) may pose a challenge to device closure; recently, a cribriform device with a minimal connecting intrawaist diameter and large, equal left- and right-sided discs has been designed to cover more than one adjacent defect. This study demonstrates the feasibility and technical aspects of closing F-ASDs using this new device. Sixteen patients between August 2003 and January 2006 were included in this study. The inclusion criterion was the presence of a F-ASD diagnosed by transesophageal echocardiography. One of the three available cribriform ASD device sizes (18, 25, or 35 mm) was implanted. Patients were followed for at least 1 year after the procedure. Thirteen patients had successful cribriform ASD device implantation (median age and weight, 12.5 years and 36 kg, respectively). Ten patients (62%) had an associated atrial septal aneurysm. The mean procedure time was 75.6 +/- 28.5 min and the mean fluoroscopy time 14.8 +/- 6.3 min. The RVEDD was significantly reduced, from a mean of 24.2 mm to 21.0 (p < 0.05). One patient developed atrial tachycardia requiring cardioversion during the procedure. There were no embolic events, heart block, or mortality. Complete closure was 10 of 13 (77 %) the next day and 12 of 13 (92%) at 6 and 12 months. We conclude that the cribriform Amplatzer device can be successfully and safely used in patients with F-ASDs. Complete closure may take up to 6 months.

Percutaneous closure of ventricular septal defect associated with tunnel-shaped aneurysm using the Amplatzer duct occluder.

Different devices have been used for percutaneous closure of ventricular septal defects (VSDs). Over the past 6 months, we have used the Amplatzer duct occluder for VSDs associated with tunnel-shaped aneurysm in three patients here at our institution. Due to the anatomic resemblance between such VSDs and the duct occluder, we selected this device for percutaneous closure. There was trivial residual shunt in one patient. All three patients tolerated the procedure well and there were no untoward complications.

The value of lipoprotein (a), homocysteine, and Doppler of carotid and femoral arteries in assessment of atherosclerosis in asymptomatic cardiovascular risk patients.

OBJECTIVES: To evaluate the role of non-traditional biochemical markers, and carotid and femoral Doppler in early detection of atherosclerosis in asymptomatic cardiovascular risk patients. SUBJECTS AND METHODS: This study was conducted on 72 patients with various cardiovascular risk factors (22 diabetic, 15 hypertensive, 20 smokers, and 15 obese), in addition to 18 healthy controls. All of them were subjected to complete examination, anthropometric measurements, calculation of Framingham risk score (FS) and measurement of lipoprotein (a) (Lp(a)), homocysteine, insulin, C-reactive protein (CRP) and microalbumin in urine (UAER) and calculation of insulin sensitivity. Intima-media thickness (IMT) and plaque formation were assessed in both carotid and femoral arteries. RESULTS: Mean values of serum insulin, homocysteine, CRP, UAER, and the IMT of right and left common carotid, and right femoral arteries revealed significant elevation in all studied groups compared to controls and insulin sensitivity showed significant reduction. In group C (FS≥20%) the mean values of triglycerides, Lp(a), and IMT of right femoral, right carotid, and left carotid arteries showed significant elevation compared to group A (FS<10%) (p<0.01). Age, waist circumference, waist/hip ratio, homocysteine, triglycerides, and CRP were predictors of IMT of carotid artery, while age, CRP, and triglycerides were predictors of atheroma. CONCLUSION: Non-traditional biochemical markers were elevated in all studied cardiovascular risk groups. IMT of carotid artery correlated with traditional risk factors and FS, as well as non-traditional risk factors. Carotid IMT measurement is recommended in both primary and secondary coronary heart disease prevention.

Noncompaction cardiomyopathy in the State of Qatar.

OBJECTIVE: To study the development of noncompaction of the ventricular myocardium (NCCM) in the state of Qatar and to highlight the prognostic parameters in those patients. METHODS: We conducted this study from 2000 to 2004 on patients who were referred to Hamad General Hospital with questionable echocardiographic features of cardiomyopathy with or without clinical manifestations of heart failure and were found to have NCCM. The diagnosis of NCCM was made according to echocardiographic criteria in 12 cases and those patients are followed up for 2-5 years. RESULTS: The mean age at diagnosis of NCCM was 6.5 years. Among them, 4 were males and 8 were females. Family history of NCCM was reported in 5 cases. Normal ejection fraction was detected in 5 patients; in this group pulsed-Tissue Doppler Imaging revealed evidence of subclinical systolic dysfunction in 4 cases. All patients showed variable degrees of diastolic dysfunction. Severely impaired ejection fraction was found in 3 cases. Progression to dilated cardiomyopathy occurred in 4 cases. Site of noncompaction included left ventricle apex in all cases, inferoposterior in 11 cases, and lateral wall in 11 cases while biventricular noncompaction was noted in 4 cases. Electrocardiogram findings included right bundle branch blocker (3) patients, left bundle branch blocker (2), left ventricular hypertrophy (6) and right ventricular hypertrophy in 3 cases. Atrial tachyarrhythmias developed in 4 cases. Wolff-Parkinson-White syndrome was detected in one patient. Associated congenital anomalies included ventricular septal defect, pulmonary stenosis, aortic coarctation, and Ebstein anomaly. The overall mortality rate was 25%. CONCLUSION: Noncompaction cardiomyopathy is so rare to be easily missed. The prognosis is poor in symptomatic cases; however, detection of subclinical systolic dysfunction is needed.

Thyroid disorders and autoantibodies in systemic lupus erythematosus and rheumatoid arthritis patients.

To determine the patterns of thyroid dysfunction and autoantibodies associated with SLE and RA patients, twenty patients with SLE and another group of twenty with RA were studied. The results were compared with those of twenty apparently healthy age- and sex- matched controls. All patients were subjected to complete history taking, thorough clinical examination and joint examination. All patients and controls were subjected to the following investigations: T3, T4, TSH, antithyroglobulin antibodies (ATGAb) and thyroid peroxidase antibodies (TPOAb). Also, complete blood picture, ESR, RF, ANA, CRP and LE cells were done. This study revealed that thyroid disorders were significantly increased in SLE patients (50%) when compared to RA (15%) (P<0.05). In SLE group, 20% had euthyroid sick syndrome, 20% had hypothyroidism (10% subclinical and 10% biochemical), and 10% had hyperthyroidism (5% subclinical and 5% biochemical). However, in RA, 10% had hypothyroidism (subclinical) and 5% had subclinical hyperthyroidism. TPOAb was found in 15% of SLE and 5% of RA patients and 10% of controls, but the titres were higher in SLE and RA patients. Also, ATGAb was found in 5% of SLE, 30% of RA patients and 10% of controls, but the titres were higher in SLE and RA patients. It is concluded that thyroid abnormalities are more implicated with euthyroid sick syndrome and hypothyroidism (subclinical and overt) than hyperthyroidism in SLE patients. SLE and RA were associated with antithyroid antibodies (TPOAb in SLE and ATGAb in RA). Performance of thyroid function tests in patients with SLE, in particular and RA as a part of the biochemical and immunological profiles, may help in early detection of associated thyroid disorders.