A Prospective Evaluation of Nesiritide in the Treatment of Pediatric Heart Failure

This study sought to determine the potential of recombinant B-type natriuretic peptide (nesiritide) for the treatment of pediatric decompensated heart failure. Nesiritide is a widely used and effective treatment for decompensated heart failure (HF) in adults, but its safety and efficacy in pediatric patients is unclear. Outcomes of 55 separate nesiritide infusions of varying durations in 32 patients (13 males and 19 females; mean age, 8.01 years; range, 0.01–20.4) were evaluated prospectively. All patients received nesiritide in the intensive care unit. The starting dose (0.01 μg/kg/min) was titrated to a maximum of 0.03 μg/kg/min. All patients were monitored for clinical signs and symptoms, hemodynamics, urine output, electrolytes, oxygen requirements, and oral intake. Functional status was assessed by patients and/or their parents. All patients successfully underwent initiation and titration of nesiritide infusion. No hypotension or arrhythmias were noted during 478 cumulative days of therapy. Nesiritide was given safely with vasoactive medications. Mean urine output improved from 2.35 ± 1.71 cc/kg/hr on the day before nesiritide initiation (baseline) to 3.10 ± 1.94 cc/kg/hr on day 4 of treatment (p < 0.01). Serum creatinine decreased from 1.04 to 0.92 mg/dl (p = 0.096), mean central venous pressure from 13 to 7 mmHg (p = 0.018), and mean weight from 30.4 to 29.7 kg (p < 0.001) with therapy. Thirst, as subjectively assessed by patients old enough to respond, decreased with infusion in 31 of 42 cases (74%). Mean New York Heart Association functional class improved significantly (p < 0.001). Nesiritide infusion, alone or in combination, is a safe treatment for decompensated HF in pediatric patients. It is associated with decreased thirst and improved urine output and functional status, and it may be efficacious in the treatment of pediatric HF.     

Nesiritide in infants and children with congestive heart failure.

OBJECTIVES: Nesiritide (synthetic B-type natriuretic peptide) has been shown to be effective in the management of acute decompensated heart failure in adults. The role of nesiritide in pediatric heart failure has not been examined. In the present study, we reviewed our initial experience with nesiritide in children with primary heart failure or low cardiac output after heart surgery. METHODS: Nesiritide was administered in an open-label fashion to patients with heart failure who were already receiving inotropic and diuretic therapy. Between July 2003 and August 2004, 30 patients aged 5 days to 16.7 yrs (median age, 4.6 months) received nesiritide therapy. Diagnoses included single-ventricle congenital defect (n = 5), two-ventricle congenital defect (n = 13), heart transplant (n = 5), and dilated cardiomyopathy (n = 7). Sixteen patients were started on nesiritide within 2 wks of corrective or palliative heart surgery. The majority of subjects (n = 24) received an initial bolus dose. Continuous infusion dosage ranged between 0.005 and 0.02 microg.kg.min. Nesiritide was discontinued for possible side effects in two patients (arrhythmia and hypotension). Duration of therapy ranged from 1 to 24 days (median, 4 days). RESULTS: Administration of nesiritide was associated with improvement in fluid balance from positive 0.8 +/- 1.9 mL.kg.hr at baseline to negative 0.3 +/- 1.8 mL.kg.hr after 24 hrs of therapy (p = .02). There was a nonsignificant trend toward a reduction in right atrial pressure (9.2 +/- 3.9 vs. 11.2 +/- 4.1, p = .08). CONCLUSIONS: Nesiritide is well tolerated in children with heart failure and is associated with improved diuresis. Further prospective studies will be needed to compare nesiritide with other vasoactive agents and examine the cost-efficacy of this therapy.     

The Impact of Prenatal Diagnosis of Complex Congenital Heart Disease on Neonatal Outcomes

Prenatal diagnosis of congenital heart disease (CHD) is increasingly common. However, the current impact of prenatal diagnosis on neonatal outcomes is unclear. Between January 2004 and January 2008, a retrospective chart review of infants who underwent surgical repair of CHD before discharge at our institution was conducted. Obstetric and perioperative variables were recorded. Of 439 neonates, 294 (67%) were diagnosed prenatally (PREdx). Infants with PREdx had a lower mean birth weight (3.0 ± 0.6 vs. 3.1 ± 0.6 kg, p = 0.002) and gestational age (37.9 ± 2.1 vs. 38.6 ± 2.4 wk, p < 0.001) than those with postnatal diagnosis (POSTdx). Severe lesions were more likely to be PREdx: Neonates with single-ventricle (SV) physiology (n = 130 patients [31.2%]) had increased odds of PREdx (n = 113/130, odds ratio [OR] 4.7; 95% confidence interval [CI] 2.7–8.2, p < 0.001). PREdx was associated with decreased preoperative intubation (OR 0.62; 95% CI 0.42–0.95, p = 0.033), administration of antibiotics (OR 0.23; 95% CI 0.15–0.36, p < 0.001), cardiac catheterization (OR 0.54; 95% CI 0.34–0.85, p = 0.01), and emergency surgery (OR 0.18; 95% CI 0.06–0.5, p < 0.001) compared with POSTdx infants. There was no difference in APGAR scores, preoperative pH, day of life of surgery, operative complications, hospital length of stay, or overall mortality in the PREdx versus POSTdx groups, even when controlling for lesion severity. PREdx was not independently associated with neonatal mortality, despite having included more severe cardiac lesions. PREdx was significantly associated with decreased neonatal morbidity in terms of decreased use of preoperative ventilator, administration of antibiotics, cardiac catheterization, and emergency surgery.     

Simplified Pulmonary Vasodilatory Testing in the Cardiac Catheterization Laboratory with Nasal Cannula Nitric Oxide

In patients with pulmonary hypertension, pulmonary vasodilator testing with inhaled nitric oxide (NO) during cardiac catheterization provides valuable data for defining future care plans. Previously, the use of delivery systems for spontaneously breathing individuals required a tight-fitting seal by face mask and an approved delivery and dilution device. We hypothesized that a simplified delivery system using nasal cannula could be utilized to effectively deliver NO during cardiac catheterization. We developed a simple delivery system to deliver through a nasal cannula a concentration of NO at 50 ppm at the nares along with supplemental oxygen (O₂) via face tent. We prospectively employed this system for 10-minute intervals on 11 patients (age range, 7 months to 41 years) with pulmonary hypertension undergoing scheduled cardiac catheterization. Mean pulmonary artery pressure (PA_p) decreased from 62 mmHg (range, 38–99) at room air testing to 45 mmHg (range, 36–91) with the addition of NO plus O₂ (p = 0.014). Pulmonary vascular resistance (PVR) decreased from 11.6 U · m² (range, 4.5–43.4) to 6.3 U · m² (range, 2.0–34.2) (p = 0.001). A response of 20% or more reduction in PVR was seen in all 11 patients. The initial ratio of pulmonary to systemic vascular resistance (R_p:R_s) was 0.49 (range, 0.25–3.5) and decreased to 0.35 (range 0.1–2.6) (p = 0.002). No adverse side effects were noted. We found this NO delivery system to be a simple and effective method of pulmonary vasodilatory testing that may have wide applicability in the cardiac catheterization laboratory.     

Nesiritide Use for Critically Ill Children Awaiting Cardiac Transplantation

Background This study aimed to explore the effects of nesiritide in children awaiting cardiac transplantation. Methods At the discretion of the attending physician, nesiritide was added to standard therapy for seven children (median age, 11 months) and continued until clinical improvement or transplantation. Blood pressure and urine output data were compared with data from the day before nesiritide institution. Safety parameters were assessed throughout the study. Results Urine output increased from 2.6 to 3.6 ml/kg/h (p = 0.04). No significant blood pressure changes or adverse effects related to nesiritide were noted. Conclusion Nesiritide may be safe and effective for children with severe heart failure. Supported by an unrestricted educational grant from Scios Inc.     

The Myocardium of Fetuses with Endocardial Fibroelastosis Contains Fewer B and T Lymphocytes than Normal Control Myocardium

The observation that endocardial fibroelastosis (EFE) can result from an immune response to maternal autoantibody deposition in the fetal myocardium raises the possibility that the fetal immune system may contribute to the pathogenesis of idiopathic EFE and dilated cardiomyopathy (DCM). This study sought to characterize myocardial immune cell presence in fetuses and neonates with idiopathic EFE + DCM, in those with EFE + structural heart disease, and in normal control subjects. Paraffin tissue sections from fetuses identified from the pathology database were stained for B cell, T cell, macrophage, and general hematopoietic cell surface markers. Of the 14 fetuses included in the study, 5 had EFE + DCM, 4 had EFE + structural heart disease, and 5 were normal control fetuses. The EFE + DCM group had fewer B cells than the control group (0.15 vs. 0.44 cells/mm²; p = 0.005). The EFE + heart disease group had both fewer B cells (0.18 vs. 0.44 cells/mm²; p = 0.08) and T cells (0.29 vs. 0.80 cells/mm²; p = 0.04) than the control group. The CD4/CD8 ratio was similar in the EFE + DCM and EFE + heart disease groups (1.0 vs. 0.9; p = 0.17) but higher in the EFE + DCM group than in the control group (0.9 vs. 0.3; p = 0.03). The myocardium of fetuses with EFE contains fewer B and T lymphocytes than normal control fetuses.     

Effectiveness of Teaching Cardiac Auscultation to Residents During an Elective Pediatric Cardiology Rotation

This study aimed to assess the effectiveness of randomized tracks of prerecorded cardiac sounds as a teaching tool for cardiac auscultation. The study focused on recognizing murmurs when present, distinguishing functional from organic murmurs, and detecting heart disease by auscultation. At both pre- and posttesting, 26 residents listened to 15 randomized tracks of live-recorded cardiac sounds and identified key features. The results indicate that the residents improved at detecting any murmur (66% vs 76%, p = 0.007) and functional murmur (37% vs 54%, p = 0.048), and marginally improved at detecting organic murmur (75% vs 84%, p = 0.129). Detection of absence of murmur declined slightly (69% vs 62%, p = 0.723). The posttest difference in identifying organic versus functional murmurs was striking (84% vs 54%, p < 0.001). Detection of heart disease (sensitivity) improved significantly (76% to 86%, p = 0.016), but there was scant improvement in detecting no disease (specificity) (55% vs 59%, p = 0.601). The residents increased in their ability to detect heart disease when present. However, the false-positive rate for a diagnosis of heart disease remained quite high. To ensure that appropriate referrals will be made, teaching should specifically target the confident recognition of functional murmurs.     

Use of near-infrared spectroscopy for estimation of renal oxygenation in children with heart disease

We evaluated whether near-infrared spectroscopy (NIRS) measurement from the flank correlates with renal vein saturation in children undergoing cardiac catheterization. Thirty-seven patients <18 years of age were studied. A NIRS sensor was placed on the flank, and venous oxygen saturations were measured from the renal vein and the inferior vena cava (IVC). There was a strong correlation between flank NIRS values (rSO₂) and renal vein saturation (r = 0.821, p = 0.002) and IVC saturation (r = 0.638, p = 0.004) in children weighing ≤ 10 kg. In children weighing > 10 kg, there was no correlation between rSO₂ and renal vein saturation (r = 0.158, p = 0.57) or IVC saturation (r = –0.107, p = 0.67). Regional tissue oxygenation as measured by flank NIRS correlates well with both renal vein and IVC oxygen saturations in children weighing <10 kg undergoing cardiac catheterization, but not in larger children.     

Randomized,double-blind,placebo-controlled trial of polyethylene glycol (MiraLAX®) for urinary urge symptoms

ObjectivePolyethylene glycol (PEG) is common first-line therapy for urinary symptoms despite minimal evidence-based support. We performed a randomized, double-blind, placebo-controlled study of PEG for initial treatment of overactive bladder (OAB) symptoms in children.Patients and methodsPatients aged >3 years underwent baseline urinary symptom questionnaire (USQ, scored 0–16), bowel symptom questionnaire (scored 0–20) and abdominal X-ray (KUB). Patients were randomized to placebo/PEG regardless of parent's perception of constipation. After 1 month, patients completed follow-up questionnaires and KUB. Improvement was defined as decrease in USQ (ΔUSQ) ≥3 points. Secondary analyses compared urinary and bowel symptoms to KUB.ResultsOf 138 enrolled patients, 71 (51.4%) completed 1 month of therapy. Analyses of those randomized to placebo vs. PEG and non-completers demonstrated similar demographics, baseline symptoms, and KUB. Patients treated with placebo and PEG both had significant improvement in USQ scores (p < 0.0001). Patients treated with placebo and PEG responded similarly to placebo (ΔUSQ 3.7 vs. 3.4, p = 0.773), with improvement in nearly half (48.5% PEG vs. 44.7% placebo). There was no correlation between KUB and urinary or bowel symptoms.ConclusionsNearly 50% of patients with urinary urge symptoms treated with either placebo or PEG for 1 month had improvement in urinary symptoms. KUB did not correlate with baseline or follow-up urinary or bowel symptoms.     

Doppler Echocardiographic Study of the Pulmonary Artery and Its Branches in 114 Normal Neonates

It has been shown that there are pressure gradients between the main pulmonary artery (MPA) and its two branches in infants undergoing catheterization. This study investigated the blood flow velocities and pressure gradients in the right and left pulmonary arteries (RPA and LPA, respectively) in normal neonates. The MPA and its two branches were examined echocardigraphically in 114 term consecutive healthy neonates aged 1–6 days. The pressure gradients between the MPA and RPA or LPA were calculated. Thirty neonates with pressure gradients above 2.5 mmHg were followed by 3–6 months. The peak velocities in the RPA and LPA (1.16 ± 0.19 and 1.01 ± 0.18 m/s) were significantly higher than that in the MPA (0.84 ± 13 m/s) (both p < 0.001), with that in the RPA slightly higher than in the LPA (p < 0.001). There was an estimated pressure gradient of 2.5–8.3 mmHg between the MPA and RPA in 43% and of 2.5–6.6 mmHg between the MPA and LPA in 16.7% of all neonates. The gradients disappeared within 3–6 months in 12 (40%) of the 30 neonates with an initial gradient above 2.5 mmHg. The differences in blood flow velocities or pressure gradients in the RPA or LPA were probably attributable to the variations in pulmonary arterial pressure, cardiac output, age, and birth weight and can be considered physiologically characteristic in neonates.     

Is Doppler echocardiography useful for estimating left ventricular filling pressures in pediatric heart transplant recipients?

LV E/E′ ratio obtained using Doppler echocardiography is considered a surrogate for LV filling pressure in adults but has performed poorly in children. We hypothesized that LV E/E′ ratio Z‐score, adjusted for age, will relate more strongly to LV filling pressures than LV E/E′ ratio in pediatric HT recipients. We analyzed 751 echocardiograms performed within 24 hours of a heart catheterization in 122 pediatric HT recipients (median age at HT 13 years, median 6 studies per patient). The primary end‐point was PCWP, assessed both as a continuous and a binary variable. Associations with LV E/E′ ratios and z‐scores were assessed using generalized estimating equations models. PCWP, LV E/E′ ratios (using E′ from LV free wall, septum, and their average), and LV E/E′ ratio Z‐scores, all declined over time after HT. LV E/E′ ratios and their Z‐scores were significantly associated with PCWP assessed as a continuous variable (P < 0.001 for all); however, the relationship was weak (R² range, 0.083 to 0.121). LV E/E′ ratios and their Z‐scores were also significantly associated with PCWP as a binary variable (P < 0.001 for all) but with only modest ability to discriminate PCWP ≥15 mm Hg (c‐statistic range, 0.660 to 0.695). The association between LV E/E′ ratio and PCWP in pediatric HT recipients is modest. Using a LV E/E′ ratio Z‐score did not result in significantly improved association with PCWP. Current Doppler echocardiographic methods are unreliable for estimating LV filling pressures in pediatric HT recipients.     

The Efficacy of Mitral Valve Surgery in Children with Dilated Cardiomyopathy and Severe Mitral Regurgitation

Severe mitral regurgitation predicts poor outcomes in adults with left ventricular dysfunction. Frequently, adult patients now undergo initial mitral valve surgery instead of heart transplant. Pediatric data are limited. This study evaluates the efficacy of mitral valve surgery for severe mitral regurgitation in children with dilated cardiomyopathy. This is a single-institution experience in seven children (range, 0.5–10.9 years) with severe mitral regurgitation and dilated cardiomyopathy who underwent mitral valve surgery between January 1988 and February 2005, with follow-up to January 2006. Children with dilated cardiomyopathy had a depressed fractional shortening preoperatively (24.4% ± 6.1%) that remained depressed (22.9% ± 7.6%) 1.3 ± 1.2 years after surgery (p = 0.50). Left ventricular end-diastolic (6.5 ± 1.5 to 4.8 ± 1.8 z-scores, p < 0.01) and end-systolic (6.8 ± 1.5 to 5.5 ± 2.1 z-scores, p < 0.05) dimensions improved. Hospitalization frequency had a median decrease of 6.0 hospitalizations per year (p < 0.02). Three patients were transplanted 0.2, 2.4, and 3.5 years after surgery. There was no perioperative mortality. Mitral valve surgery in children with dilated cardiomyopathy was performed safely and improved symptoms, stabilizing ventricular dysfunction in most patients. Mitral valve surgery should be considered prior to heart transplant in children with dilated cardiomyopathy and severe mitral regurgitation.     

Prostacyclin Treatment for Persistent Pulmonary Hypertension of the Newborn

To study the effect of prostacyclin treatment on pulmonary arterial pressure (PAP), systolic pressure (BP), and systemic oxygenation, eight infants with persistent pulmonary hypertension of the newborn (PPHN) born between 34 and 42 weeks' gestation and having a birth weight of 2540–4130 g were studied using Doppler echocardiography. At a mean age of 19 hours (range 3–32 hours), despite maximal ventilator therapy and an FiO₂ of 1.0, the mean PaO₂/PAO₂ was 0.07 (range 0.04–0.09) and the AaDO₂ was 616 mmHg (range 521–654 mmHg). After volume correction and during inotropic medication with dopamine and dobutamine, the mean PAP by echocardiography was 68.6 ± 6.5 mmHg and the mean BP 59.8 ± 4.8 mmHg. Prostacyclin infusion was then started at a dose of 20 ng/kg/min and increased stepwise to a mean dose of 60 ng/kg/min (range 30–120 ng/kg/min) over 4–12 hours, at which time PAP decreased to 49.2 ± 3.5 mmHg (p= 0.0005) and BP to 53.2 ± 9.1 mmHg (p= 0.17); the PAP thereafter remained below the BP. After 72 hours of prostacyclin infusion, PAP was 49.6 ± 18 mmHg, BP 66.1 ± 5.4 mmHg, PaO₂/PAO₂ 0.14 ± 0.12, and AaDO₂ 428 ± 189 mmHg at FiO₂ 0.65. The median duration of prostacyclin infusion was 3.6 days and of respirator treatment 7.0 days. All patients survived without extracorporeal membrane oxygenation. At 6–12 months, none of the patients had severe central nervous system complications, but two had bronchopulmonary dysplasia. These findings indicate that prostacyclin is able to reverse the right-to-left shunt in PPHN by decreasing PAP, and that systemic hypotension can be prevented with adequate volume correction and inotropic medication.     

Respiratory Variation in Aortic Blood Flow Velocity as a Predictor of Fluid Responsiveness in Children After Repair of Ventricular Septal Defect

This study aimed to compare respiratory variation in transthoracic echo-derived aortic blood flow velocity (∆Vpeak) and inferior vena cava diameter (∆IVCD) with central venous pressure (CVP) as predictors of fluid responsiveness in children after repair of ventricular septal defect (VSD). A prospective study conducted in pediatric intensive care unit investigated 21 mechanically ventilated children who had undergone repair of VSD. Standardized volume replacement (VR) was the intervention used. Hemodynamic measurements including CVP, heart rate, mean arterial pressure, transthoracic echo-derived stroke volume (SV), cardiac output, ∆Vpeak, and ∆IVCD were performed 1 h after patient arrival in the intensive care unit. Hemodynamic measurements were repeated 10 min after VR by an infusion of 6% hydroxyethyl starch 130/0.4 (10 ml/kg) over 20 min. The volume-induced increase in the SV was 15% or more in 11 patients (responders) and less than 15% in 10 patients (nonresponders). Before volume replacement, the ∆Vpeak (23.1 ± 5.7% vs. 14.0 ± 7.7%; p = 0.006) and ∆IVCD (26.5 ± 16.2% vs. 9.2 ± 9.1%; p = 0.008) was higher in the responders than in the nonresponders, whereas CVP did not significantly differ between the two groups. The prediction of fluid responsiveness was higher with the ΔVpeak, as shown by a receiver operating characteristic curve area of 0.83 (95% confidence interval [CI], 0.61–1.00; p = 0.01), a ΔIVCD of 0.85 (95% CI, 0.69–1.00; p = 0.01), and a CVP of 0.48 (95% CI, 0.22–0.73; nonsignificant difference). The ∆Vpeak and ∆IVCD measured by transthoracic echocardiography can predict the response of SV after volume expansion in mechanically ventilated children at completion of VSD repair.     

The effect of treatment with angiotensin-converting enzyme inhibitors on survival of pediatric patients with dilated cardiomyopathy

Summary Outcome in 81 pediatric patients with dilated cardiomyopathy was reviewed to assess whether treatment with angiotensin-converting enzyme (ACE) inhibitors affected survival. Age at onset was 3.6±0.6 years. Twenty-seven children (group 1) were treated with ACE inhibitors. Conventional therapy was used in the remaining 54 patients (group 2). There were no significant differences between the two groups in age at onset, left ventricular shortening fraction, left ventricular end-diastolic pressure, or mean pulmonary artery pressure. Patients treated with ACE inhibitors had a significantly better survival during the first year (p<0.05) with continuation of this trend throughout the second year (p=0.06). Beyond 2 years there was a tendency toward better survival in ACE inhibitor-treated patients, but the differences were no longer significant (p=0.14). These data, along with observations in adult patients with chronic cardiac failure, indicate that converting enzyme inhibitors have a beneficial effect on prolonging survival of infants and children with severe left ventricular dysfunction from dilated cardiomyopathy.     

Percutaneous Transluminal Angioplasty for Stenosis of the Aorta Due to Aortic Arteritis in Children

Percutaneous transluminal balloon angioplasty for stenosis of the aorta due to aortic arteritis was attempted on 45 lesions in 41 children (age range, 4–14 years; mean, 9.9±4.2 years) presenting with symptoms of hypertension, severe congestive heart failure, and lower limb claudication. Balloon dilatation was technically successful in 38 (92.7%) patients for 41 stenotic lesions (91.1%). The mean peak systolic pressure gradient (PSG) decreased from 71.7 ± 23.9 mmHg to 23.2 ± 17.5 mmHg (p < 0.001) and the diameter of the stenosed segment increased from 3.3 ± 1.1 mm to 7.5 ± 2.2 mm (p < 0.001) immediately after angioplasty. Patients with short-segment (<3 cm) stenosis had a lower residual gradient (17.9 ± 11.1 mmHg vs 30.5 ± 22.6 mmHg; p < 0.05) and a wider diameter of the aorta (8.8 ± 1.1 mm vs 7.5 ± 2.2 mm; p < 0.02) compared to patients with long-segment (≥3 cm) stenosis. Four patients required stent implantation; 2 for flow-limiting dissection, 1 for failure to reduce PSG by >50%, and 1 for recurrent restenosis. There was marked hemodynamic and angiographic improvement in these 4 patients. Hemodynamic and angiographic restudy in 21 of the 41 patients at mean follow-up period of 6.2 ± 4.2 months (range, 3–24 months) showed restenosis in 4 (19%) patients. Restenosis was more common in patients with long-segment stenosis than those with short-segment stenosis (30% vs 9.1%). Late restudy in 8 patients, done at 3–7 years after first restudy, showed no recurrence of aortic narrowing. On clinical follow-up of 38 patients for a mean of 58.8 ± 36.0 months (range, 8–146 months) there was marked improvement in symptoms. Hypertension was cured in 11 (29%), improved in 24 (63%), and persisted in 3 (8%). Six patients with associated severe renal artery stenosis showed further improvement in hypertension after successful renal angioplasty. Severe congestive heart failure improved in 21 (95.4%) of 22 patients. Mean left ventricular ejection fraction improved from 0.32 ± 0.08 to 0.48 ± 0.10 (p < 0.001) at a mean follow-up of 28.7 ± 8.4 months in these patients. Hemodynamic restudy in 10 of these patients showed improvements in left ventricular end-diastolic pressure from a mean 37 ± 9 mmHg (range, 25–55 mmHg) to 16.4 ± 6.2 mmHg (range, 6–25 mmHg) (p < 0.001). Lower limb claudication improved in all 4 patients. Our results suggest that percutaneous transluminal balloon angioplasty in children is safe and highly effective in relieving stenosis of the aorta due to aortic arteritis, with marked clinical improvement, and should be the treatment of choice particularly for discrete stenosis.     

Peak Circulatory Power as an Indicator of Clinical Status in Children After Fontan Procedure

Peak circulatory power (CircP), a product of peak exercise oxygen uptake (VO₂) and peak mean or systolic arterial blood pressure, has proved to be a strong predictor of poor outcome in adults with congenital heart disease. This study sought to compare CircP with other cardiopulmonary exercise (CPX) test variables and to assess whether CircP is superior in categorizing patients into well-functioning vs. poorly functioning at-risk groups in the pediatric population after a Fontan procedure. The CPX test reports of 50 patients were retrospectively reviewed after the Fontan procedure. The patients were divided into two groups. The well-functioning group included patients in New York Heart Association (NYHA) classes 1 and 2 (n = 36). The poorly functioning at-risk group included patients in NYHA classes 3 and 4 and those with significant indicators or outcomes of a poor prognosis (n = 14). The patients in the well-functioning group had significantly higher CircP values based on mean blood pressure (MBP) (P < 0.001), higher CircP values based on systolic blood pressure (SBP) (P < 0.001), and higher peak VO₂ (P = 0.004) than those in the poorly functioning at-risk group. At a cutoff value less than 2100.4 mmHg/mlO₂/kg/min, CircP MBP had a sensitivity of 85% in categorizing children to the poorly functioning at-risk group. CircP correlated well with the clinical status of our patients. CircP and peak VO₂ did not differ significantly in ability to identify poorly functioning patients. Further prospective analysis is needed to assess whether CircP can serve as a prognostic marker for the pediatric population after Fontan procedure.     

The effect of montelukast on early‐life wheezing: A randomized,double‐blinded placebo‐controlled study

Background

Cysteinyl‐leukotrienes are increased in the airways of infants with virus‐associated wheezing. We aimed to determine the effects of a cysteinyl‐leukotriene‐1 receptor antagonist on symptoms during an early‐life wheezing illness and to investigate the factors that affect the response to this drug.

Method

This placebo‐controlled double‐blinded randomized controlled trial recruited children aged 3‐36 months with wheezing illness and randomized to active drug or placebo for 56 days. A symptom score diary (SSD) was kept by the children's caregivers.

Results

One‐hundred patients completed the study, and 62 (30 montelukast and 32 placebo) were analyzed. There were no significant differences in the percent of symptom‐free days, symptom scores, and the need for rescue salbutamol between the two groups. However, the percent of symptom‐free days within the first week was significantly higher for the montelukast than for the placebo group (13.8 ± 4.1% vs. 5.4 ± 3.4%; P = 0.028); wheezing score at 7th day was significantly lower for the montelukast than for the placebo group (0.5 ± 0.1 vs. 1.4 ± 0.2; P = 0.002). In addition, the number of inhaled ß₂‐agonist rescue episodes per day during the first week was significantly lower for the montelukast compared with the placebo group (12.7 ± 1.8 vs. 19.2 ± 1.6; P = 0.013).

Conclusions

Our results indicate that montelukast may be effective for reducing caregiver‐observed wheezing and the need for salbutamol during the first week of treatment for early‐life wheezing. The impact for caregivers and the optimal duration of treatment will need to be explored in studies of larger size.     

Exercise testing after surgical repair of coarctation of the aorta

Summary After repair of coarctation, exercise testing was performed in 20 patients with an isolated coarctation (group I) and in 26 with additional congenital cardiac malformations (group II). Ages at time of operation were significantly different in both groups (7.9±6.0 years in group I; 4.6±3.8 years in group II;p≤0.01). Simultaneous blood pressures were obtained from upper and lower limbs at rest and after exercise. There was no significant difference regarding the systolic blood pressures at rest (122.5±15.6 mmHg in group I versus 119±15.8 mmHg in group II). Seven (14%) of the patients were hypertensive; five of them had blood pressure gradients between arms and legs of 15–45 mmHg. But the gradients at rest were found to be significantly different in both groups (9.0±10.5 mmHg in group I; 18.5±16.1 mmHg in group II;p≤0.05). Six patients, all in group II, had gradients ≥30 mmHg at rest. After exercise there were no significant differences in systolic blood pressure and gradients in both groups. Values for blood pressures and gradients at rest and after exercise showed a positive correlation (blood pressurer=0.76,p≤0.001; gradientr=0.44,p≤0.01). Thus exercise testing can provide valuable information about blood pressure and gradient changes during physical activity, but angiography is required to reveal restenosis or residual stenosis.     

Perfil ingestivo salino y presión arterial en la fibrosis quística

BackgroundHigh blood pressure (BP) is not considered a problem in patients with cystic fibrosis (CF). The loss of sodium in these patients may affect their sensitivity to the taste of salt.ObjectivesTo study the BP in a group of patients with CF and to analyse their salt intake profile and the relationship with their BP levels.Patients and methodsCross-sectional analytical study with control group. Index group: 20 subjects, 4–30 years old with diagnosis of CF. Control group: 73 healthy subjects. Physical examination, BP measurement and specific tests to determine the salt ingestion profile.ResultsSystolic BP (SBP) and diastolic BP (DBP) values were lower in the CF group. SBP: 99.63±9.11 mmHg vs. 111.94±10.71 mmHg, P: 0.001. DBP: 57.84±7.40 mmHg vs. 70.05±8.11 mmHg, P: 0.001. When these values were adjusted for age, sex, weight and height of the participants, differences did not remain statistically significant. Values of the salt intake profile did not differ significantly between the two groups. While the control group showed a significant negative correlation between SBP and salt taste sensitivity (r: ?0.341, P=0.003), this correlation was not confirmed in CF patients (r: ?0.115 P=0.6).ConclusionsBP values and the salt intake profile values in CF patients are equivalent to the normal population values when their differences are adjusted to the potential confounding factors. There is no correlation between BP levels and salt taste sensitivity in patients with CF.