Acute Rheumatic Fever: Case Report and Literature Review.

A previously healthy 8-year-old Native American female presented with right-sided weakness and joint pain for two weeks. Following an initially unremarkable workup including negative brain and spine MRI she was noticed to have chorea and subsequently diagnosed with acute rheumatic fever (ARF). ARF is a group A streptococcus-related disease that most commonly is a sequelae of pharyngitis. The diagnosis of ARF utilizes the Jones criteria which includes heart disease, arthritis, chorea, the characteristic rash of erythema marginatum, and subcutaneous nodules. The most serious consequences of ARF include rheumatic heart disease and chorea. ARF can be treated with a combination of antibiotics and anti-inflammatories like aspirin.

Pediatric Viral Myocarditis - A Review.

Pediatric myocarditis is a common pediatric illness most commonly secondary to a preceding viral infection. It is a leading cause of acquired heart failure, cardiomyopathy, and cardiac transplantation in pediatrics. Due to the variability in presentation, the diagnosis is often unrecognized until later in the disease course. It should be considered in the differential diagnosis of all children presenting with respiratory distress, since this is the most common presentation. Imagining modalities, such as cardiac magnetic resonance imaging have become a useful diagnostic tool in recent years; however, endomyocardial biopsy remains the gold standard diagnostic test. Treatment of viral myocarditis is mainly supportive, with debatable role of anti-inflammatory, immunosuppressing immunomodulators and anti-viral therapy. Although, the outcome is generally favorable, delay in supportive care may be associated with a worse prognosis. We focus on the clinical presentation, review diagnostic and treatment options of viral myocarditis.

Endovascular stent provides more effective early relief of SVC obstruction compared to balloon angioplasty.

OBJECTIVE: To determine whether superior vena cava (SVC) stent implantation is superior to balloon angioplasty for relieving SVC stenosis. BACKGROUND: SVC stent and balloon dilation have been used as treatment for SVC stenosis. Although safe and effective, outcome data comparing the two methods are limited. METHODS: A Pediatric Cardiac Care Consortium review identified SVC stenosis. Patients who required SVC intervention were divided into two subgroups-balloon dilation (Group A) and stent implantation (Group B). Logistic regression and the log-rank test were used to test the need for re-intervention within 6 months after the initial procedure. RESULTS: SVC intervention was performed on 210/637 patients with SVC stenosis (33%). There were 108/210 (51%) patients with balloon dilation (Group A) and 102/210 (49%) with stent implantation (Group B). Re-intervention within 6 months of the initial intervention was more common in Group A compared to Group B [Group A = 31/40 (77.5%); Group B = 5/22 (22.7%)]. The odds-ratio for re-intervention within 6 months of the initial procedure for balloon vs. stent, is 7.3 [95% CI: (2.91, 22.3), P < 0.0001]. In addition, during the first 6 months after an intervention for SVC stenosis the proportion of patients with stent implantation that remained free of re-intervention was significantly higher than after balloon angioplasty (log-rank test, P < 0.0001). Neither age nor weight was significantly associated with the need for re-intervention. CONCLUSIONS: SVC stent implantation is more effective than angioplasty in relief of SVC obstruction. Weight and age are not risk factors for early re-intervention.

Frequency of superior vena cava obstruction in pediatric heart transplant recipients and its relation to previous superior cavopulmonary anastomosis.

The risk factors for superior vena cava (SVC) obstruction after pediatric orthotopic heart transplantation (OHT) have not been identified. This study tested the hypothesis that pretransplant superior cavopulmonary anastomosis (CPA) predisposes patients to SVC obstruction. A retrospective review of the Pediatric Cardiac Care Consortium registry from 1982 through 2007 was performed. Previous CPA, other cardiac surgeries, gender, age at transplantation, and weight at transplantation were assessed for the risk of developing SVC obstruction. Death, subsequent OHT, or reoperation involving the SVC were treated as competing risks. Of the 894 pediatric OHT patients identified, 3.1% (n = 28) developed SVC obstruction during median follow-up of 1.0 year (range: 0 to 19.5 years). Among patients who developed SVC obstruction, 32% (n = 9) had pretransplant CPA. SVC surgery before OHT was associated with posttransplant development of SVC obstruction (p <0.001) after adjustment for gender, age, and weight at OHT and year of OHT. Patients with previous CPA had increased risk for SVC obstruction compared with patients with no history of previous cardiac surgery (hazard ratio 10.6, 95% confidence interval: 3.5 to 31.7) and to patients with history of non-CPA cardiac surgery (hazard ratio 4.7, 95% confidence interval: 1.8 to 12.5). In conclusion, previous CPA is a significant risk factor for the development of post-heart transplant SVC obstruction.

"Vanishing" pulmonary valve stenosis.

OBJECTIVE: Both spontaneous resolution and progression of mild pulmonary valve stenosis (PS) have been reported. We reviewed characteristics of the pulmonary valve (PV) to determine factors that could influence resolution of mild PS. METHODS: Fifteen asymptomatic pediatric patients with spontaneous resolution of isolated mild PS were retrospectively reviewed. RESULTS: There was no correlation between the PV gradient, clinical presentation, age at diagnosis, or PV morphology. The PV annulus was small at initial presentation, which normalized at follow up. When corrected for the body surface area (z-score), the PV annulus was normal in all patients, including at initial evaluation. CONCLUSIONS: Based on our observation, neither age at diagnosis, nor PV-morphology-influenced resolution of mild PS. The variable clinical presentation makes it difficult to categorize and observe mild PS by auscultation alone. The PV annulus z-score could be a useful adjunct to determine the course and serial observation of mild PS.

Delayed arterial switch operation for d-transposition of the great arteries and glucocorticoid remediable aldosteronism.

The Jatene arterial switch operation (ASO) for dextro-transposition of the great arteries is ideally performed within the first 2 weeks of life. Clinical circumstances, however, may dictate a delayed ASO and left ventricle "retraining" prior to the procedure. Glucocorticoid remediable aldosteronism (GRA) accounts for 0.5% to 1% of primary aldosteronism. It presents as severe hypertension in infants and children, with poor response to standard antihypertensive medications. To the authors' knowledge, this is the first reported case of GRA in the context of transposition physiology. The management of GRA and a rationale for delayed ASO are discussed.

Successful Coil Embolization of a Large Ascending Aortic Pseudoaneurysm Following Explantation of the EXCOR Pediatric Ventricular Assist Device in a Patient With Acute Fulminant Myocarditis.

Mechanical ventricular assistance has become a reliable tool for the support of children and infants with heart failure. The devices have shown efficacy both as a bridge to transplantation and as a bridge to recovery. The potential complications that may occur with long-term support have not been fully described. This article reports the occurrence of a large pseudoaneurysm associated with the ascending aorta following explantation of the EXCOR Pediatric ventricular assist device. A management strategy for this potentially lethal complication is described.