Pediatric cardiology
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Pediatric cardiology · Jan 2013
Case ReportsLeft thoracoscopic sympathectomy used as primary therapy for a young child with intractable long QT syndrome.
A 3-year-old boy with familial long QT syndrome type 2 presented with recurrent syncope despite adequate beta-blocker therapy. Two family members had experienced sudden cardiac arrest, and one other relative had experienced sudden cardiac death. ⋯ The chest tube was removed on day 1 after surgery, and the patient was discharged on postoperative day 4. During 14 months of follow-up evaluation, no intervening episodes of ventricular arrhythmia or syncope and no symptoms of Horner's syndrome were noted.
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Pediatric cardiology · Jan 2013
Case ReportsNoonan syndrome and different morphologic expressions of hypertrophic cardiomyopathy.
Hypertrophic cardiomyopathy is the disease with the largest heterogeneity in clinical cardiology. An underrecognized and incompletely described subgroup within this spectrum comprises patients with ventricular diverticulum, coronary myocardial bridging, and left ventricular crypts and crevices. This report presents the case of a Noonan syndrome patient with these four hypertrophic cardiomyopathy characteristics.
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Pediatric cardiology · Jan 2013
Pain management after comprehensive stage 2 repair for hypoplastic left heart syndrome.
Achieving optimal pain control for children after complex cardiac surgery can be challenging. Recently, the hybrid approach to palliation of hypoplastic left heart syndrome (HLHS) was introduced as an alternative to the classic Norwood procedure. The second stage of the hybrid approach is a complex procedure known as comprehensive stage 2 (CS2). ⋯ This group of patients generally can be managed with fentanyl NCA. Achieving a balance between a proper level of analgesia and sedation in the setting of early tracheal extubation to optimize postoperative physiology can be challenging. The preliminary data suggest that improvements in pain management should be investigated given that more than 30 % of the pain scores in the CS2 group were in the moderate to severe range compared with 18 % after the Glenn procedure.
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Pediatric cardiology · Jan 2013
Decreasing the need for transfusion: infant cardiac surgery using hemodilution and recombinant factor VIIa.
Many strategies, including intraoperative acute normovolemic hemodilution (ANH) and pharmacologic agents, exist to minimize the use of allogeneic blood products in pediatric congenital heart surgery. Recombinant activated factor VIIa (rFVIIa) is a hemostatic agent approved for the treatment of bleeding episodes and prevention of bleeding in surgical interventions in patients with hemophilia A or B with inhibitors, acquired hemophilia, or congenital factor VII deficiency. Off-label use in nonhemophilic patients for uncontrolled hemorrhage is increasing although still under investigation. ⋯ Of the nine patients, the three who underwent the most aggressive hemodilution received rFVIIa most rapidly and required no allogeneic blood products to achieve hemostasis although they had an average lower fibrinogen level on admission to the cardiothoracic intensive care unit. These preliminary data suggest that hemodilution before surgical stimulation and the rapid administration of rFVIIa, along with the reintroduction of autologous blood, may decrease or potentially eliminate the need for allogeneic blood products. Prospective trials are warranted to further explore this technique.
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Pediatric cardiology · Jan 2013
Case ReportsMaternal diclofenac medication in pregnancy causing in utero closure of the fetal ductus arteriosus and hydrops.
This report describes a case of premature closure in utero of the ductus arteriosus (DA) diagnosed postnatally in a baby with hydrops and cardiac failure. An echocardiogram 6 h postnatally showed marked dilation of the right atrium and right ventricle with marked hypertrophy and impaired function, elevated pulmonary pressures, a small pericardial effusion, and no flow through the DA. The mother was unaware of her pregnancy until she presented in labor, and she had taken diclofenac medication in the preceding months. This case and the accompanying literature review illustrate the potential fetal and neonatal complications resulting from antenatal closure of the DA due to maternal diclofenac medication during pregnancy.