Doppler assessment of pulmonary artery flow patterns and ventricular function after the Fontan operation

To assess the relation between ventricular systolic and diastolic function and pulmonary artery (PA) flow patterns after the Fontan operation, 15 postoperative patients were prospectively evaluated with echocardiography. Blood flow velocities in the PA were recorded with pulsed Doppler echocardiography. Ejection fraction was measured by 2-dimensional echocardiography using Simpson's rule. Indexes of diastolic function were measured from the systemic atrioventricular valve inflow Doppler and included peak E and A velocities, peak filling rate normalized for stroke volume, the fractions of filling in early and late diastole (E and A area fractions), and the E/A velocity and area ratios. Compared with 15 age-matched control subjects, the 15 patients who had undergone the Fontan procedure had decreased peak E velocity (0.65 +/- 0.20 vs 0.87 +/- 0.10 m/s), decreased E/A velocity ratio (1.29 +/- 0.23 vs 1.98 +/- 0.46), decreased normalized peak filling rate (6.09 +/- 0.90 vs 6.81 +/- 0.83 s-1), decreased E area fraction (0.63 +/- 0.09 vs 0.72 +/- 0.07), increased A area fraction (0.37 +/- 0.07 vs 0.24 +/- 0.06), and decreased E/A area ratio (1.77 +/- 0.45 vs 3.33 +/- 1.15) (p < 0.05). These diastolic filling abnormalities are consistent with impaired ventricular relaxation and decreased early diastolic transvalvular pressure gradient. PA Doppler recordings showed 2 distinct patterns of flow. Pattern I, observed in 9 patients, showed biphasic forward flow with peak velocities in mid to late systole and middiastole. Pattern II, observed in the remaining 6 patients, showed decreased systolic forward flow, a late systolic to early diastolic flow reversal, and delayed onset of diastolic forward flow. Compared with pattern I patients, pattern II patients had no significant differences in any of the Doppler indexes of diastolic function; however, pattern II patients had a significantly tower ejection fraction (43 +/- 9 vs 57 +/- 5%). Thus, many patients undergoing the Fontan procedure have impaired ventricular relaxation, but, in the presence of a normal ejection fraction, biphasic forward PA flow is maintained. With the development of decreased ejection fraction, atrial systolic filling pressures are likely increased, the ventricular suction effect is decreased, and PA flow is diminished or absent in systole and early diastole.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/29071/1/0000106.pd

Design and Rationale of the Fontan Udenafil Exercise Longitudinal (FUEL) Trial

The Fontan operation creates a circulation characterized by elevated central venous pressure and low cardiac output. Over time, these characteristics result in a predictable and persistent decline in exercise performance that is associated with an increase in morbidity and mortality. A medical therapy that targets the abnormalities of the Fontan circulation might, therefore, be associated with improved outcomes. Udenafil, a phosphodiesterase type 5 inhibitor, has undergone phase I/II testing in adolescents who have had the Fontan operation and has been shown to be safe and well tolerated in the short-term. However, there are no data regarding the long-term efficacy of udenafil in this population. The Fontan Udenafil Exercise Longitudinal (FUEL) Trial is a randomized, double blind, placebo controlled phase III clinical trial being conducted by the Pediatric Heart Network in collaboration with Mezzion Pharma Co., Ltd. This trial is designed to test the hypothesis that treatment with udenafil will lead to an improvement in exercise capacity in adolescents who have undergone the Fontan operation. A safety extension trial, the FUEL Open-Label Extension Trial (FUEL OLE), offers the opportunity for all FUEL subjects to obtain open-label udenafil for an additional 12 months following completion of FUEL, and evaluates the long-term safety and tolerability of this medication. This manuscript describes the rationale and study design for FUEL and FUEL OLE. Together, these trials provide an opportunity to better understand the role of medical management in the care of those who have undergone the Fontan operation

Anomal\uedas cong\ue9nitas de las arterias coronarias

Las anomal\uedas cong\ue9nitas de las arterias coronarias no son frecuentes pero implican un riesgo significativo de isquemia mioc\ue1rdica, de disfunci\uf3n del miocardio. de insuficiencia cardiaca congestiva y muerte s\ufabita. Este riesgo parece ser m\ue1s alto durante la infancia y la adolescencia; por tanto, la comprensi\uf3n de estas anomal\uedas es importante para el pediatra y el cardi\uf3logo pediatrico. En este art\uedculo se hace hincapi\ue9 en los aspectos anat\uf3micos, el modo de presentaci\uf3n, los datos diagn\uf3sticos y los tratan\ufacntos quir\ufargicos disponibles para los tipos m\ue1s frecuente, de anomal\uedas coronarias congenitas aisladas, que se vinculan con isquemia tnioc\ue1rdica durante la infancia: 1) origen an\uf3malo de la arteria coronaria a partir del seno opuesto de Valsalva. con un trayecto interaterial entre las grandes arterias y 2) origen :an\uf3malo de arteria coronaria a partir de la arteria pulmonar. Completa este art\uedculo una descripci\uf3n breve de las conexiones fistulosa, de arterias coronarias

Genetic disturbances in patients with bodily isomerism from a single center: clinical implications of affected genes and potential impact of ciliary dyskinesia

So-called heterotaxy or isomerism is characterized by abnormal lateralization and malformations of the bodily organs. The mechanism is unclear, although there is growing evidence that ciliary dyskinesia is involved. We reviewed genetic findings from patients with isomerism to determine if affected genes were known to be associated with isomerism and ciliary dyskinesia and determine associations between genotype and clinical findings. We identified patients with isomerism cared for over a 16- year period. Characteristics were compared between those with and without identified mutations. A total of 83 patients with isomerism were identified. Of those who had genotyping, 14/27 had mutations identified, most frequently involving the CFC1 and NODAL genes. Specific mutations were associated with clinical findings, with NODAL mutations often portending need for increased clinical support. Genes associated with isomerism and/or ciliary dyskinesia were identified in the cohort. Specific gene mutations may help predict clinical course