We present a rare case of ventriculocoronary fistulae in a patient with d-transposition of the great arteries, hypoplastic left heart, and pulmonary atresia. To our knowledge, this is the first report of this anatomic variant, and raises an important discussion about the development and implications of such fistulous connections.
Studies of the arterial switch operation for Taussig-Bing anomaly demonstrate significant rates of reintervention and mortality, particularly after initial palliation to delay complete repair. We aimed to describe the long-term outcomes of our 21-year practice of single-stage arterial switch operation for all patients with Taussig-Bing anomaly.A retrospective study was performed, and 43 patients with Taussig-Bing anomaly were identified between 1990 and 2011. Median age at arterial switch operation was 7 (range, 2-192) days, and median operative weight was 3.2 (1.4-6.2) kg. Aortic arch obstruction was present in 30 patients (70%). Hospital mortality was 7% (n=3). Follow-up was available for 37 hospital survivors at a mean of 8.1 (± 6.3) years. Late mortality was 2% (n=1). At follow-up, all patients were in New York Heart Association functional class I. Freedom from transcatheter or surgical reintervention was 73% at 1 year, 64% at 5 years, and 60% at 10 years. Eleven patients underwent 13 catheter reinterventions on the pulmonary arteries (n=8) or aortic arch (n=5). Seven patients underwent 11 reoperations, including relief of right ventricular outflow tract obstruction (n=5), pulmonary arterioplasty (n=3), recoarctation repair (n=2), and tricuspid valve repair (n=1). By multivariate analysis, a preoperative aortic valve annulus z score of ≤-2.5 was associated with reintervention (hazard ratio, 7.66 [95% confidence interval, 1.29-45.6], P=0.03).Although reintervention is common, primary correction of Taussig-Bing anomaly with arterial switch operation can be achieved in all patients with low mortality and good long-term outcomes.
Abstract Continuous intravenous (IV) sildenafil may avoid the acute systemic vasodilatory effects of bolus dosing in infants with pulmonary hypertension (PH). We aimed to examine the tolerability of different methods of IV sildenafil administration. Methods: We retrospectively evaluated subjects less than 12 months old with PH, who had been started on IV sildenafil. Vital signs, oxygen requirement, and vasoactive-inotropic score (VIS) before and after sildenafil initiation were noted, as was the need for discontinuation due to side effects. Results: Forty-three subjects were identified (23 continuous, 20 intermittent dosing). There were no statistically significant differences between groups in gender or gestational age, but higher baseline inspired oxygen (FiO2) and VIS in the continuous group suggested a higher baseline severity of illness (p=0.012). After sildenafil initiation, there were no significant differences in the change in blood pressure, oxygen saturation, FiO2, or VIS between groups, and no difference in the number of subjects requiring discontinuation due to side effects (4 in the continuous group, 1 intermittent, p=0.35). Eight subjects (34.8%) in the continuous group and 3 (15%) in the intermittent group died (p=0.024). Conclusions: In this small cohort of infants with PH treated with continuous or intermittent IV sildenafil, there were no statistically significant differences between groups in the change in vital signs, VIS, and oxygen requirement, or the need for discontinuation of therapy due to side effects. A higher mortality rate in the continuous infusion group may be explained by higher baseline illness severity.
Objectives: Prior studies suggest that an elevated troponin I level predicts poor outcomes after the arterial switch operation (ASO) for transposition of the great arteries (TGA). However, no absolute threshold leading to poor outcomes has been convincingly established. This study sought to evaluate the utility of routine troponin measurements in predicting left ventricular (LV) dysfunction after ASO. Methods: Data on 225 consecutive patients undergoing an ASO were analysed. Patients were grouped according to anatomical variations: simple TGA (STGA) (88) and TGA with ventricular septal defect and/or anomalous coronary arteries (137). LV function as determined at discharge by echocardiogram was compared against troponin levels and patterns of troponin rise. Receiver-operating curves were also used to delineate this relationship. Results: Preoperative patient characteristics were similar between anatomical subgroups (Table 1). Baseline troponin levels were higher in patients who underwent a VSD closure. A rise in troponin from baseline at ICU admission was associated with at least mild left ventricular dysfunction at the time of hospital discharge in STGA patients (P < 0.01) and patients with an intact ventricular septum (P < 0.01). LV dysfunction in STGA was predicted with 100% accuracy by a rising troponin level (Fig. 1). The area under the receiver-operating curve was 0.91 when predicting LV dysfunction in patients with an intact ventricular septum. Demogaphic and anatomical characteristics Demogaphic and anatomical characteristics
Abstract Intravenous (IV) sildenafil may be administered as a continuous infusion or intermittent bolus dosing in infants with pulmonary hypertension (PH). We aimed to compare these delivery methods. Methods We retrospectively evaluated subjects less than 12 months old treated with IV sildenafil for PH. Vital signs, oxygen requirement, vasoactive‐inotropic score (VIS), and echocardiogram results before and after sildenafil initiation, and the need for discontinuation due to side effects, were noted. Results Forty‐three subjects were identified (23 continuous, 20 intermittent). There were clinically significant differences in PH classifications between groups. The continuous group was significantly younger ( p = 0.010) with higher baseline severity of illness suggested by higher inspired oxygen (FiO 2 ) and VIS ( p = 0.012). After sildenafil initiation, there were no significant differences in changes in blood pressure, oxygen saturation, FiO 2 , or VIS between groups, and no difference in the number of subjects requiring discontinuation due to side effects (4 continuous, 1 intermittent, p = 0.351). Eight continuous group subjects (34.8%) and 3 intermittent group subjects (15.0%) died ( p = 0.024), but echocardiographic improvement in PH degree was more common in the continuous group (77.8% vs. 33.3%, p = 0.007). Conclusion In this small cohort of infants treated with continuous or intermittent IV sildenafil, in the setting of different baseline characteristics between groups, there were no significant differences in changes in vital signs, VIS, FiO 2 , or need for discontinuation of therapy due to side effects. Higher continuous group mortality may be explained by greater baseline illness severity, but larger prospective, randomized studies are required to investigate these different delivery methods.
