Electrolytically detachable coils for closure of a modified baffle fenestration in patients with hypoplastic left heart syndrome (HLHS).

Today total cavopulmonary connection (TCPC) is the treatment of choice for long term palliation of patients with hypoplastic left heart syndrome (HLHS). We report a new, relatively simple and effective method to occlude a modified baffle (TCPC) fenestration via the left hepatic vein in two patients with HLHS with the use of electrolytically detachable platinum microcoils after a failed tourniquet closure.

Aortic valvuloplasty in pediatric patients substantially postpones the need for aortic valve surgery: a single-center experience of 188 patients after up to 17.5 years of follow-up.

BACKGROUND: Aortic valvuloplasty (AoVP) is an established procedure regarded as a valid alternative for surgical management of congenital aortic valve stenosis. However, its long-term efficacy in preventing or postponing aortic valve surgery remains uncertain for the individual patient. Therefore, the aim of this study was to study the long-term results of AoVP in pediatric patients and its efficacy in preventing or postponing aortic valve surgery. METHODS AND RESULTS: We reviewed up to 17.5 years of follow-up data of all 188 patients who received AoVP at the Deutsches Herzzentrum München. The patients were divided into those < 1 month of age (group < 1 month; n=68) and those > or = 1 month of age (group > or = 1 month; n=120) at the time of AoVP. After the first and second AoVP, moderate and severe aortic regurgitation developed in 29% and 14%, respectively, of the patients in group < 1 month and in 19% and 29%, respectively, of the patients in group > or = 1 month. Survival after 10 years free from aortic valve surgery was 59% (95% confidence interval, 45 to 73) in group < 1 month and 70% (95% confidence interval, 59 to 81) in group > or = 1 month. CONCLUSIONS: This study shows that the long-term results of AoVP of congenital aortic valve stenosis in pediatric patients and its efficacy in preventing or postponing aortic valve surgery are very good. About two thirds of the patients are free from aortic valve surgery 10 years after AoVP.

Stenting of stenosed shunts in patients after a Norwood-Sano operation.

Early shunt stenosis may occur after a Norwood-Sano operation. We report our experience with three patients who were treated successfully by stent implantation into the Sano shunt. Hence, a surgical shunt revision could be avoided.

The fate of systemic blood pressure in patients after effectively stented coarctation.

AIMS: The current study was designed to assess midterm results of stent implantation into the aorta for native and recurrent coarctation (CoA) in children and young adults. METHODS AND RESULTS: Forty-three patients (native CoA, 8; female, 12) were treated with stent implantation at a median age of 16.8 years (range 7.9-44.8 years). Only stents dilatable to an adult size aorta were implanted. All but two patients with functionally univentricular hearts had arterial hypertension. Exercise tests, 24-h blood pressure, clinical examination, echocardiography, and elective catheterization were used to assess follow-up. The narrowed segment was widened significantly from a median of 8 to 12.4 mm (P < 0.0005). The peak-to-peak gradient between the ascending and the descending aorta was lowered significantly from a median of 22 mmHg to 1 mmHg (P < 0.0005). No major complications occurred. The systolic blood pressure at the right arm was lowered significantly (P < 0.0005) from 144 mmHg before stent implantation to 128 mmHg at the last visit. At a median follow-up of 30 months (3-72 months), 68% of all patients were classified to be normotensive. CONCLUSION: Stent implantation for selected patients with recurrent and native CoA is safe and may effectively reduce the blood pressure gradient across the CoA site. We suggest using only stents dilatable to an adult size aorta. However, arterial hypertension persists in a significant number of the patients. Impaired elastic properties of the aorta may be the cause for this finding.

Assumed oxygen consumption frequently results in large errors in the determination of cardiac output.

OBJECTIVE: We sought to investigate the differences in assumed and measured oxygen consumption values for the determination of cardiac output by using the Fick principle in a pediatric population with congenital heart disease. METHODS: The patient population consisted of 143 patients with a mean age of 11.3 years (age range, 2 days to 23.8 years) undergoing cardiac catheterization during general anesthesia and with mechanical ventilation. Oxygen consumption was measured with a standard commercial analyzing system (Deltatrac II; Datex, Engström, Helsinki, Finland). Assumed oxygen consumption values were calculated according to the formulas of Krovetz and Goldbloom and LaFarge and Miettinen. Comparisons between measurements and assumptions were performed by Bland-Altman plots. Two-sided paired t tests were used to assess a difference of the assumed and measured values. RESULTS: The range of measured oxygen consumption values was between 55.2 and 249 mL . min -1 . m -2 . The Krovetz-Goldbloom formula led to systematically larger values compared with the measured values (P = .0001; mean difference of -53.3 mL . min -1 . m -2 ; 95% confidence interval, -56.7 to -49.8 mL . min -1 . m -2 ). The use of the LaFarge-Miettinen formula tends to overestimate oxygen consumption (P = .0037; mean difference of -15.9 mL . min -1 . m -2 ; 95% confidence interval, -26.5 to -5.4 mL . min -1 . m -2 ). A similarly poor agreement was found when analyzing a subgroup of 25 patients with Fontan-type circulation. CONCLUSION: The use of assumed instead of measured oxygen consumption values introduces large errors in the determination of cardiac output.

Pulmonary blood pressure, not flow, is associated with net endothelin-1 production in the lungs of patients with congenital heart disease and normal pulmonary vascular resistance.

OBJECTIVE: Endothelin-1 concentrations are increased in patients with increased mean pulmonary arterial pressure, pulmonary blood flow, and pulmonary vascular resistance. However, endothelin-1 concentrations have not been well characterized in patients with congenital heart disease and normal pulmonary vascular resistance. In particular, it is unclear whether pressure or flow is the key regulator of endothelin- 1 in this setting. We tested the hypothesis that pulmonary blood pressure and not flow is associated with net endothelin-1 production in patients with congenital heart disease and normal pulmonary vascular resistance. METHODS: With a commercially available immunoassay, we measured endothelin-1 concentrations in pulmonary arterial and pulmonary venous plasma of 56 consecutive patients with congenital heart disease and pulmonary vascular resistance less than 2 U. m(2) undergoing cardiac catheterization. We used multiple linear regression to analyze the effect of demographic and hemodynamic variables on pulmonary arterial and venous endothelin-1 concentrations and on the change of endothelin-1 concentration over the pulmonary vascular bed. RESULTS: Multiple linear regression revealed that of all the hemodynamic variables tested, mean pulmonary arterial pressure had the greatest effect on increasing the change of endothelin-1 concentration over the pulmonary vascular bed (P <.0001). Pulmonary blood flow did not have any effect on endothelin-1 concentrations or on the change of endothelin-1 concentration over the pulmonary vascular bed. CONCLUSIONS: This study shows that pulmonary blood pressure and not flow is associated with net endothelin-1 production in patients with congenital heart disease and normal pulmonary vascular resistance.