Continuing Medical Education onlineThis activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education through the joint sponsorship of Medscape, LLC and the American Society of Hematology. Medscape, LLC is accredited by the ACCME to provide continuing medical education for physicians. Medscape, LLC designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit(s) ™ . Physicians should claim only the credit commensurate with the extent of their participation in the activity. All other clinicians completing this activity will be issued a certificate of participation. To participate in this journal CME activity: (1) review the learning objectives and author disclosures; (2) study the education content; (3) take the post-test with a 70% minimum passing score and complete the evaluation at http://www.medscape.org/journal/blood; and (4) view/print certificate. For CME questions, see page 2969. Disclosures The authors, the Associate Editor Martin S. Tallman, and CME questions author Laurie Barclay, freelance writer and reviewer, Medscape, LLC, declare no competing financial interests. Learning objectives Upon completion of this activity, participants will be able to:1. Describe the prevalence and clinical characteristics of parainfluenza virus (PIV) infections in patients with leukemia or HSCT, based on a medical record review.2. Describe outcomes of PIV infections in patients with leukemia or HSCT and the factors predicting progression to pneumonia, based on a medical record review.3. Describe risk factors for mortality from PIV infections in patients with leukemia or HSCT, based on a medical record review.
Coarctation of aorta (CoA) is a discrete narrowing in aorta causing obstruction to the flow of blood. It accounts for 6–8% of all congenital heart diseases. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally. Transthoracic echocardiography is mainstay of making initial diagnosis and routine follow-up. Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) are great advanced imaging tools for two-dimensional and three-dimensional imaging of aortic arch in complex cases. Based on type of coarctation, size of patient, severity of lesion, and associated abnormalities various management options like surgical treatment, transcatheter balloon angioplasty and transcatheter stent implantation are available. There is significant improvement in long-term survival from pre-surgical era to post-surgical era. But, among the postsurgical era patients, the long-term survival has not significantly changed between older and contemporary cohort. Patients with coarctation of aorta need lifelong follow-up event after successful initial intervention.
Coarctation of the Aorta (CoA) is a congenital cardiac anomaly consisting of a constricted aortic segment with a prevalence of 5 to 8% of all Congenital Heart Defects (CHD). The classic CoA is located in the thoracic aorta distal to the origin of the left subclavian artery, at about the level of the ductal structure. Significant hypertension and/or congestive heart failure are indications of intervention. If hypertension (rather that heart failure) is the clinical problem, it is better to relieve the aortic obstruction promptly rather that attempting to "treat" hypertension with antihypertensive medications. Surgical relief of the aortic obstruction and catheter interventional techniques (balloon angioplasty and stents) are available alternatives. Since the introduction of surgical correction by Crafoord and Nylin and Gross and Hufnagel in early 1940s, surgical therapy has been the treatment of choice for aortic coarctation. Gruntzig's technique of balloon angioplasty was adopted by Singer and Sperling and their associates to enlarge coarcted aortic segments in post-surgical recoarctation and native coarctation, respectively in early 1980s. This is followed by the application of the technique by other cardiologists to treat native CoA. The procedure consists of inserting a balloon angioplasty catheter across the site of coarctation and inflating the balloon with diluted contrast material. Both immediate and followup results are reasonably good. Residual and recurrent obstructions following surgery and prior balloon angioplasty are also amenable for balloon angioplasty. Despite reasonably good short-term and long-term results of balloon angioplasty, some problems remain and include restenosis, probability of aortic rupture, formation of aneurysms and inability to effectively treat long-segment tubular narrowing. Because of these and other reasons, endovascular stenting of aortic coarctation has gained acceptance over the last decade. The balloon catheter, with the stent mounted on it, is advanced over a stiff guide wire and positioned across the coarctation segment and the balloon inflated, thus implanting the stent. Most cardiologists use stents in adolescents and adults and restrict their use in younger children because of issues related to growth. Stent therapy appears to be an attractive method for treatment of native or recurrent coarctation, aneurysm formation following prior surgical or balloon intervention and for long segment hypoplasia. Role of covered stents to manage aortic coarctation is limited and are used when the assessed risk for development of aneurysm or dissection is high. A comparison of all available treatment modalities was made and while it is difficult to make a definitive statement, the overall data seem to indicate that transcatheter methods may be better than surgery. Selection of the method of therapy is largely based on the age at presentation and anatomy of the coarcted segment and surrounding structures; surgery for infants and long segment coarctations, balloon angioplast...
Heart murmur is the most common reason for a referral to a pediatric cardiologist. Virtually all children have a heart murmur during their childhood. Less than 1% of murmurs are pathological in children. Innocent/functional heart murmur is the most common type of heart murmur. There are multiple theories proposed to identify etiology of innocent heart murmur with varying consensus, but everybody agrees that innocent heart murmur does not carry any morbidity or mortality risk. Even today, heart murmur is associated with high physician uncertainty and parental anxiety. Extensive cardiac evaluation for such a benign finding is also associated with high health care utilization and cost. This article attempts to review this long-known finding which continues to remain a diagnostic challenge.
A 16-year-old male presented to the emergency department with chest pain after smoking a synthetic cannabinoid from a vape pen. He had rising troponin I levels, and his exercise stress echocardiogram showed distal apical and septal hypokinesis that resolved at six-month follow-up. This case report raises concern about cardiac ischemia related to synthetic cannabinoid abuse in the pediatric population in the current era of cannabis legalization.
Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs. While transthoracic echocardiography is usually adequate, older children and adults may need more advanced imaging modalities like computed tomography and magnetic resonance imaging prior to intervention. Depending on the type of lesion, management options currently available include surgery and percutaneous balloon angioplasty and stenting. Even after successful interventions, these patients need life-long surveillance for residual aortic obstruction and systemic hypertension with variable long-term clinical outcomes.
For adults with sickle cell disease (SCD), cardiac changes are well described and may account for up to one-fourth of disease-related mortality. Much less is known about heart disease in pediatric SCD patients. This retrospective study analyzed a contemporary cohort of children with SCD followed at a single tertiary care center. The study aimed to evaluate the effect of SCD on left main coronary artery (LMCA) dimensions in the contemporary pediatric population. The echocardiography database was searched for studies of children with SCD from 2000 to 2009. The LMCA diameter was remeasured offline from digital images. Digital echocardiographic studies of 68 children (35 boys) 11.7 ± 4.6 years of age with SCD were analyzed. All the patients had normal systolic function as measured by fractional shortening. The left ventricular end-diastolic dimension (LVEDD) for 34 % of the pediatric SCD patients showed a dilated LV. In 24 % of the children, a LV mass index (LVMI) greater than 50 g/m(2.7) denoted LV hypertrophy. Application of sex-specific normal values showed that 60 % of the boys and 33 % of the girls had an increased LVMI. The LMCA diameter was enlarged in 43 % of the SCD children, including 26 % with an LMCA z-score higher than 3. This study found a higher incidence of LMCA dilation in a pediatric referral population with SCD. The findings also validated an increased LVMI in the contemporary SCD patient population, which was noted previously. This study adds the need to include assessment of coronary arteries in cardiac evaluation of SCD patients, and special attention should be paid to patients with a high-normal to high coronary z-score.
A relationship between epilepsy and impairment of neuropsychological functioning has been observed in children with epilepsy. Interictal epileptiform discharges on electroencephalograms (EEGs) are regarded as a correlate of persistent pathological neuronal discharges. Paroxysmal abnormalities in the frontal area may be associated with neuropsychological impairments. Investigations of paroxysmal EEG abnormalities are also needed. Subclinical discharges contribute to the psychosocial problems of children with epilepsy. A combination of spike rate and extended periods of high-frequency paroxysmal EEG abnormalities may predict the atypical evolution of benign childhood epilepsy with centrotemporal spikes. In addition, children with epilepsy with continuous spike-waves during slow sleep have cognitive and behavioral disturbances that are correlated with frequent epileptiform discharges. On the other hand, several studies have suggested that EEG characteristics, especially the localization of paroxysmal discharges, might be important predictors for subsequent epilepsy. Patients with febrile seizure presenting with frontal paroxysmal EEG abnormalities may be at risk for epilepsy. In addition, the frontal paroxysms may indicate a higher risk of epilepsy in autism spectrum disorders. Moreover, the location of paroxysmal EEG abnormalities correlates with behavioral disturbances. Frontal EEG paroxysms may be associated with a higher risk of developing atypical clinical features such as seizure recurrence and cognitive/behavioral problems. Previous and current studies suggest that a combination of spike frequency, extended periods of high-frequency paroxysmal EEG abnormalities, and frontal EEG focus might predict atypical clinical manifestations, such as neuropsychological impairments, and ultimate neuropsychological outcome in children with epilepsy. To prevent these disturbances and problems in children with epilepsy, treatment to remit seizures and EEG abnormalities as soon as possible may be required to achieve the optimal prognosis for children with cognitive/behavioral problems.
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