Currently, various materials are used for the right ventricular outflow tract reconstruction (RVOTR) in the treatment of congenital heart disease; however, from the view point of longevity, none of them yield satisfactory results. We have originally developed a tri-valved conduit made of expanded polytetrafluoroethylene (ePTFE) for RVOTR. This valved conduit consists of an ePTFE graft with bulging sinuses; to the inside of the graft are attached fan-shaped valves made of 0.1 mm thick ePTFE membrane. This valved conduit has been supplied for 52 institutes in Japan, where 325 patients (aged 15 days to 56.8 years old) received the implantation of this conduit between February 2002 and January 2011. Notably, an improved reoperation-free survival (95.4% at 10 years) and preventative effects on pulmonary insufficiency (moderate 4.7%, severe 0.3%) was observed in the mean follow-up of 3.6 years. This improved outcome may partly be attributable to the excellent biocompatibility and low antigenicity of ePTFE, which is expected to prevent calcification and immunological rejection, both of which are the main causes of graft degeneration in homografts and xenografts. Moreover, our experimental study using pediatric right heart circulation model showed that bulging sinuses and fan-shaped leaflets produced two important fluiddynamical properties; that is, vortex flow in the sinus and laminar flow in the conduit, which might also have contributed to the better function of the valves. In conclusion, excellent biocompatibility of ePTFE and fluiddynamical properties provided by bulging sinuses and fan-shaped leaflets are the rationale for the excellent clinical results of our valved conduit.
Background: Hypertrophy of the tonsils and adenoids is the most common cause of airflow obstruction in the upper airway of pediatric patients. In severe cases, chronic upper airway obstruction presents pulmonary hypertension and cor pulmonale. Objective: Effect of tonsillectomy and/or adenoidectomy in pediatric patients on the Fontan track was assessed. Subjects: From 1993 to 2009, tonsillectomies were performed for 9 patients on the so-called Fontan track with upper airway obstruction due to tonsillar hypertrophy. Methods: We assessed the clinical effects of a tonsillectomy by measuring SpO2 in all cases and blood gases and catheterization data both before and after the procedure in available 4 cases. Results: All patients eventually underwent a Fontan operation except 1 who is waiting Fontan completion with good Fontan candidacy after Glenn operation. SpO2 was increased in all patients from 81±7 % (mean±SD) preoperatively to 85 ± 6 % after the procedure. Mean pulmonary arterial pressure was measured using cardiac catheterization in 4 patients before and after the tonsillectomy, and decreased from 22 ± 12 mmHg to 13 ± 4 mmHg. Conclusions: A tonsillectomy for patients on various stages of Fontan track with upper airway obstruction due to tonsillar hypertrophy may relieve pulmonary vascular resistance and improve Fontan hemodynamics.
We report a three-month-old male case of Down syndrome and complete atrioventricular septal defect (c-AVSD) with a single papillary muscle. After fetal diagnosis of c-AVSD, the patient was delivered normally in the 38th week. Cardiac catheterization and echocardiography showed Rastelli type A c-AVSD with well-balanced ventricles and trivial atrioventricular valve regurgitation. On the other hand, disproportional papillary muscles were seen in the left ventricle (LV) suggesting a functionally single papillary muscle. At the age of three months, intra-cardiac repair using the two-patch method was performed. There was only one papillary muscle in the anterolateral position of the LV, and all cords of both bridging leaflets attached to that muscle. To prevent postoperative stenosis of the left atrioventricular valve (AVV), only the base of the cleft was repaired by making exact apposition zones of both leaflets. Although treatment for pulmonary hypertension and pleural effusion was needed, the patient recovered to a stable condition after the surgery. Finally, the patient was discharged from the hospital with good LV contraction and left AVV function under normal pulmonary arterial pressure. So far, several reports mention a single papillary muscle in c-AVSD morphologically but surgical strategies concerning the left AVV cleft and papillary muscle have not been established yet. Therefore, it is important to develop an adequate procedure to prevent postoperative stenosis and regurgitation in repairing the left AVV portion.
Persistent fifth aortic arch is a rare anomaly of aortic arch development. Two children with persistent fifth aortic arch coarctation and an interrupted fourth aortic arch were surgically repaired. A nine-day-old girl was suffered from ductal shock. The arch repair using the extended aortic arch anastomosis to the fourth aortic arch was performed urgently. Another girl patient was referred to us with cardiac murmur when 2 years 1 month old. The arch repair was performed using side-to-side anastomosis between the fourth aortic arch and the fifth aortic arch, and those were anastomosed to the descending aorta with end-to-end fashion. These two patients had uneventful postoperative course with no stenosis at the anastomosis sites when 1 year 11 months old, and 2 years 6 months old.
Aprindine is one of the useful antiarrhythmic agents because it rarely decreases cardiac function or prolongs the QT interval. However, there are few reports regarding the use of aprindine in childhood, especially in early infancy. We report the efficacy of aprindine in three infants with postoperative supraventricular tachycardia and one infant with ectopic atrial tachycardia (EAT) without congenital heart anomalies. Aprindine was effective for one infant with EAT among four cases. Although a relatively large amount of aprindine (a dose of 2 to 3 mg/kg) was administered, serum aprindine concentrations were less than the therapeutic range in most cases. One patient developed liver dysfunction, which may be due to the adverse effect of aprindine, but no other serious adverse effects were noted in three patients. We concluded that aprindine could be a medication to treat early infants with supraventricular tachycardia including EAT.