Pediatric cardiology
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Pediatric cardiology · Jan 2006
Comparative StudyRisk-stratified approach to hybrid transcatheter-surgical palliation of hypoplastic left heart syndrome.
We prospectively employed a risk-stratified approach to first-stage palliation of hypoplastic left heart syndrome. High-risk features included severe tricuspid insufficiency, severe right ventricular dysfunction, a severely restrictive or intact atrial septum, an ascending aortic diameter < or = 2 mm, late presentation, weight < 2 kg, or significant extracardiac issues, Infants without high-risk features underwent a Norwood procedure (with Sano modification), whereas infants with high-risk features underwent a hybrid procedure consisting of bilateral pulmonary artery banding, ductal stenting, and atrial septostomy or a Norwood/Sano. ⋯ Operative survival for 5 infants with high-risk features undergoing hybrid palliation was 100%, compared to 29% in 7 infants with high-risk features undergoing the Norwood/Sano procedure. Although only short-term data are available, this hybrid palliative procedure may have a role for infants with hypoplastic left heart syndrome and high-risk features.
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Pediatric cardiology · Jan 2006
Site of coronary sinus drainage does not significantly affect coronary flow reserve in patients long term after Fontan operation.
This study was designed to investigate the impact of postoperative coronary sinus drainage pressure on coronary flow reserve (CFR) assessed by Doppler guidewire in patients long term after Fontan operation. Twenty-nine patients (median age, 17.4 years female, 11) at a median of 10.6 years after Fontan operation were examined with intracoronary Doppler guidewire during cardiac catheterization. Fourteen patients had coronary sinus (CS) drainage to the systemic venous atrium and 15 patients had CS drainage to the pulmonary venous atrium after Fontan operation. ⋯ The site of coronary drainage into the systemic atrium or the pulmonary venous atrium did not significantly affect CFR. Our data do not support a surgical strategy of elective redirection of coronary sinus blood to a low-pressure compartment but support an early staged approach. The positive correlation between CFR and pulmonary resistance demands further evaluation.
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Pediatric cardiology · Jan 2006
Plasmatic [corrected] factor XIII reduces severe pleural effusion in children after open-heart surgery.
Chylous effusions frequently occur after cardiac surgery due to severe damage to the lymphatic system, thus indicating that the insertion of a chest tube may be necessary. Factor XIII (FXIII) is discussed as being essential for wound healing. The aim of this retrospective study was to evaluate whether the application of a single dose of FXIII results in a reduced amount of pleural effusion, leading to an earlier release of patients from the hospital. ⋯ A single application of FXIII rapidly reduces the amount of chylous effusions in the early period after open-heart surgery. This effect is detectable only for 24 hours after the treatment and does not alter the further clinical outcome. Prospective clinical trials are warranted to determine if repeated application or a higher dose of FXIII may improve the clinical outcome of chylous leakages in children after open-heart surgery.
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Pediatric cardiology · Jan 2006
Case ReportsCongenital stridor: unusual manifestation of coarctation of the aorta.
Coarctation of the aorta is a relatively common congenital condition. Most infantile presentations of aortic coarctation are related to the associated congenital cardiac abnormalities leading to congestive heart failure or systemic hypoperfusion. ⋯ After surgical correction, respiratory signs and symptoms completely resolved. This case report describes a unique cause of stridor in newborn infants and discusses the potential for vascular anomalies to result in tracheal narrowing.
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Pediatric cardiology · Jan 2006
Case ReportsCoincidence of long QT syndrome and propionic acidemia.
Propionic acidemia and long QT syndrome (LQTS) are rare disorders. In addition, both conditions are potentially lethal. ⋯ Although the patient was asymptomatic and arrhythmia free, we started her on atenolol. This is the first report of the association between LQTS and propionic acidemia.