Mending Hearts and Building Bridges: The Save a Child's Heart Foundation.

CONTEXT: Save a Child's Heart addresses the challenges of heart care for children in underdeveloped countries. OBJECTIVE: Save a Child's Heart has created a center of excellence for pediatric cardiac care at the Wolfson Medical Center in Israel, helped develop partner sites for evaluation and referral, and trained medical teams to return and build their own capacity for local cardiac care. RESULTS: Save a Child's Heart has treated more than 3600 children from 48 countries, with 50% from Iraq, Jordan, the Palestinian Authority, and Syria. In cooperation with the Palestinian Authority, Save a Child's Heart has examined more than 6000 children and treated 1750 children, trained 21 medical personnel, and conducted seminars for Palestinian medical personnel. The "Heart of the Matter Project," funded by the European Union, US Agency for International Development, the Palestinian Ministry of Health, and the Israeli Ministry of Regional Cooperation, is currently training a team at the Wolfson Medical Center from the Palestine Medical Complex in Ramallah and provides funds for Palestinian children's care in Israel. CONCLUSIONS: Save a Child's Heart is a model of a global humanitarian health care initiative with a single focus on heart disease in children. The "Heart of the Matter Project" is a model of health care based on regional cooperation despite local political tensions.

Extracorporeal membrane oxygenation in pediatric patients: our experience in the last ten years.

BACKGROUND: The use of extracorporeal membrane oxygenation (ECMO) in children after cardiac surgery is well established. ECMO support is becoming an integral tool for cardiopulmonary resuscitation in specified centers. OBJECTIVES: To review our use of ECMO over a 10 year period. METHODS: All children supported with ECMO from 2000 to 2010 were reviewed. Most of these children suffered from cardiac anomalies. The patients were analyzed by age, weight, procedure, RACHS-1 when appropriate, length of support, and outcome. RESULTS: Sixty-two children were supported with ECMO; their median age was 3 months (range 0-216 months) and median weight 4.3 kg (range 1.9-51 kg). Thirty-four patients (52.3%) needed additional hemofiltration or dialysis due to renal failure. The children requiring ECMO support represented a wide spectrum of cardiac lesions; the most common procedure was arterial switch operation (27.4%, n=17). ECMO was required mainly for failure to separate from the heart-lung machine (n=55). The median duration of ECMO support was 4 days (range 1-14 days); 29 (46.7%) patients were weaned successfully from ECMO during this time period, and 5 of them died during hospitalization, yielding an overall hospital survival rate of 38.7%. CONCLUSIONS: ECMO support has significant survival benefit for patients with post-cardiotomy heart failure. Its early deployment should be considered in cardiopulmonary resuscitation.

Recent use of formaldehyde-'free' hair straightening product and severe acute kidney injury.

Formaldehyde is present in hair straightening products even when labeled as formaldehyde-free. Inhaled absorption of formaldehyde causes renal tubular cytotoxicity. We report a teenager who developed severe acute kidney injury requiring renal replacement therapy shortly after exposure to a formaldehyde-'free' hair straightening product. Kidney biopsy showed acute tubular necrosis and images compatible with microcalcifications. Kidney function improved while on continuous venous-venous hemodialysis.

Mode of ventilation during cardiopulmonary bypass does not affect immediate postbypass oxygenation in pediatric cardiac patients.

STUDY OBJECTIVE: To investigate the impact of different modes of ventilation during cardiopulmonary bypass (CPB) on immediate postbypass oxygenation in pediatric cardiac surgery. DESIGN: Prospective, randomized clinical trial. SETTING: University hospital. PATIENTS: 50 pediatric patients (18 girls, 32 boys), aged 4 months to 15 years, undergoing elective repair of congenital heart disease. INTERVENTIONS: Patients were randomized to receive one of 5 modes of ventilation during bypass. Groups 1 and 2 received high-frequency/low-volume ventilation with 100% (group 1) or 21% oxygen (group 2). Groups 3 and 4 received continuous positive airway pressure of 5 cm H(2)O with 100% (group 3) or 21% oxygen (group 4); and in group 5, each patient's airway was disconnected from the ventilator (passive deflation). MEASUREMENTS: Blood gas analysis and spirometry data were recorded 5 minutes before chest opening, 5 minutes before inducing bypass, 5 minutes after weaning from bypass, and 5 minutes after chest closure. MAIN RESULTS: There were no differences in Pao(2) values among the 5 groups studied and at the different time points. Lung compliance was higher 5 minutes before bypass in group 1 versus group 5 (34 +/- 13 mL/cm H(2)O vs 20 +/- 9 mL/cm H(2)O; P = 0.048). CONCLUSIONS: Mode of ventilation during CPB did not affect immediate postbypass oxygenation.

Hypertonic saline/epinephrine treatment in hospitalized infants with viral bronchiolitis reduces hospitalization stay: 2 years experience.

BACKGROUND: We recently published preliminary evidence on the effectiveness of hypertonic saline in infants with viral bronchiolitis. OBJECTIVE: To further establish the efficacy of nebulized hypertonic saline in these infants. METHODS: In a continuing, second-year randomized, doubleblind controlled trial, an additional 41 infants (age 2.6 +/- 1 months) hospitalized with viral bronchiolitis were recruited during the winter of 2001-2002. The infants received inhalation of 1.5 mg epinephrine dissolved either in 4 ml normal (0.9%) saline (Group I, n=20) or 4 ml hypertonic (3%) saline (Group II, n=22). The therapy was repeated three times daily until discharge. Pooling our 2 years of experience (2000-2002), a total of 93 hospitalized infants with viral bronchiolitis were recruited; 45 were assigned to Group I and 48 to Group II. RESULTS: The clinical scores at baseline were 7.6 +/- 0.7 for Group I vs. 7.4 +/- 1.3 for Group II (P = NS). However, the clinical scores at days 1 and 2 after inhalation differed significantly between the two groups, invariably favoring Group II: 7 +/- 1 vs. 6.25 +/- 1.1 (P< 0.05), 6.45 +/- 1 vs. 5.35 +/- 1.35 (P< 0.05), respectively. Adding aerosolized 3% saline to 1.5 mg epinephrine reduced the hospitalization stay from 3.5 +/- 1.7 days in Group I to 2.6 +/- 1.4 in Group II (P< 0.05). The pooled data of both years revealed that adding 3% saline to the inhalation mixture decreased hospitalization stay from 3.6 +/- 1.6 to 2.8 +/- 1.3 days (P< 0.05). CONCLUSIONS: This second-year experience and our 2 year pooled data analysis strengthen the evidence that the combination of 3% saline/1.5 mg epinephrine benefits hospitalized infants with viral bronchiolitis.

Nebulized 3% hypertonic saline solution treatment in hospitalized infants with viral bronchiolitis.

OBJECTIVE: To determine the utility of inhaled hypertonic saline solution to treat infants hospitalized with viral bronchiolitis. DESIGN: Randomized, double-blind, controlled trial. Fifty-two hospitalized infants (mean +/- SD age, 2.9 +/- 2.1 months) with viral bronchiolitis received either inhalation of epinephrine, 1.5 mg, in 4 mL of 0.9% saline solution (group 1; n = 25) or inhalation of epinephrine, 1.5 mg, in 4 mL of 3% saline solution (group 2; n = 27). This therapy was repeated three times every hospitalization day until discharge. RESULTS: The percentage improvement in the clinical severity scores after inhalation therapy was not significant in group 1 on the first, second, and third days after hospital admission (3.5%, 2%, and 4%, respectively). In group 2, significant improvement was observed on these days (7.3%, 8.9%, and 10%, respectively; p < 0.001). Also, the improvement in clinical severity scores differed significantly on each of these days between the two groups. Using 3% saline solution decreased the hospitalization stay by 25%: from 4 +/- 1.9 days in group 1 to 3 +/- 1.2 days in group 2 (p < 0.05). CONCLUSIONS: We conclude that in nonasthmatic, nonseverely ill infants hospitalized with viral bronchiolitis, aerosolized 3% saline solution/1.5 mg epinephrine decreases symptoms and length of hospitalization as compared to 0.9% saline solution/1.5 mg epinephrine.

Nebulized 3% hypertonic saline solution treatment in ambulatory children with viral bronchiolitis decreases symptoms.

OBJECTIVE: To determine the utility of inhaled hypertonic saline solution to treat ambulatory infants with viral bronchiolitis. DESIGN: Randomized, double-blind, controlled trial. Sixty-five ambulatory infants (mean +/- SD age, 12.5 +/- 6 months) with viral bronchiolitis received either of the following: inhalation of 0.5 mL (5 mg) terbutaline added to 2 mL of 0.9% saline solution as a wet nebulized aerosol (control; group 1; n = 32) or 0.5 mL (5 mg) terbutaline added to 2 mL of 3% saline solution administered in the same manner as above (treatment; group 2; n = 33). This therapy was repeated three times every day for 5 days. RESULTS: The clinical severity (CS) scores at baseline on the first day of treatment were 6.4 +/- 1.8 in group 1 and 6.6 +/- 1.5 in group 2 (not significant). After the first day, the CS score was significantly lower (better) in group 2 as compared to group 1 on each of the treatment days (p < 0.005; Fig 1 ). On the first day, the percentage decrease in the CS score after inhalation therapy was significantly better for group 2 (33%) than for group 1 (13%) [p < 0.005; Fig 1 ]. On the second day, the percentage improvement was better in the hypertonic saline solution-treated patients (group 2) as compared to the 0.9% saline solution-treated patients (group 1) [p = 0.01; Fig 1 ]. CONCLUSIONS: We conclude that in nonasthmatic, nonseverely ill ambulatory infants with viral bronchiolitis, aerosolized 3% saline solution plus 5 mg terbutaline is effective in decreasing symptoms as compared to 0.9% saline solution plus 5 mg terbutaline.

Removal of deadspace volume from arterial catheter: How muchis enough?

OBJECTIVE: To evaluate the amount of volume needed to be removed from arterial catheter systems to compensate for "deadspace" and to allow an accurate measurement of pH and hemoglobin (Hb). DESIGN: Twenty patients undergoing heart surgery were evaluated in a steady state after the induction of anesthesia before surgery. Six blood samples were removed from the arterial catheter, the total volume of which was 1.5 mL at 30-sec intervals and measured for pH and Hb. The first sample was then taken after removing 1.5 mL from the tubing. In subsequent samples, the volume removed before sampling increased by 0.5-mL intervals. All other samples were compared with sample number 6, in which 4 mL of volume were removed before measurements. RESULTS: The first three samples with volumes of 1.5, 2.0, and 2.5 mL before measurement were inaccurate compared with sample number 6 (p <.000), giving artificially low values for both pH and Hb. There was no significant difference between the values measured in sample numbers 4, 5, and 6 (3.0, 3.5, and 4.0 mL, respectively). CONCLUSION: The amount of volume needed to be removed before measurement from an arterial catheter system, the volume of which is 1.5 mL, is 3 mL to achieve accurate measurements of pH and Hb. Removal of less volume results in an artificially low measurement.

Massive pulmonary emboli in children: does fiber-optic-guided embolectomy have a role? Review of the literature and report of two cases.

Massive pulmonary emboli is a rare disease in children, with only 39 reported cases in the last 50 years. Almost 50% of the patients died suddenly without receiving medical treatment. Most of the patients who were managed medically (70% of the treated patients) underwent surgical pulmonary embolectomy with 80% survival. Surgical pulmonary embolectomy is a blind procedure that can be improved by using intraoperative angioscopy. This technique was reported in adults with good results. In this article, we describe two pediatric patients who underwent fiber-optic-guided surgical pulmonary embolectomy. To our knowledge, this technique has never been reported in the pediatric population.

Right ventricular outflow tract strategies for repair of tetralogy of Fallot: effect of monocusp valve reconstruction.

OBJECTIVES: The absence of a pulmonary valve (PV) after tetralogy of Fallot (TOF) repair has been shown to impact postoperative right ventricular (RV) function. The purposes of this study were to (i) compare early outcomes after PV-sparing vs transannular patching (TAP) with monocusp valve reconstruction or TAP alone and (b) assess the mid-term results after polytetrafluoroethylene (PTFE) membrane monocusp reconstruction. METHODS: From 2003 to 2009, 163 patients underwent TOF repair. Sixty-nine patients (42.3%) underwent a PV-sparing procedure (Group A), 74 (45.4%) underwent PTFE membrane monocusp valve reconstruction (Group B) and 20 (12.3%) underwent TAP only (Group C). Early outcomes were evaluated by the right-to-left ventricular pressure ratio, RV outflow tract gradient, tricuspid and PV function, intensive care unit (ICU) parameters and need for reintervention. Group B patients were also evaluated at intermediate term for clinical and echocardiographic parameters, including tricuspid and monocusp valve function and mobility. RESULTS: The median age, weight and PV Z-value of Group B patients were significantly lower; 20.5 months, 9.3 kg and -4, respectively. Postoperatively, the right-to-left ventricular pressure ratio was <0.5 in all groups. Mechanical ventilation time, fluid drainage duration and total ICU stay showed no significant difference between Groups A and B, while Group C was significantly longer (P < 0.01). There were five (3%) early deaths: three from Group A and two from Group B. The incidences of moderate or severe pulmonary insufficiency (PI) on discharge were 8.2% in Group A, 9% in Group B and 50% in Group C (P < 0.001). Among Group B patients, 85% of the evaluated patients had less than moderate PI in the intermediate-term follow-up, QRS duration <140 ms in 83.3% and right-to-left ventricular diameter ratio of 0.6 ± 0.2. Two (2.6%) patients underwent reoperation for monocusp replacement. There were two (2.7%) mid-term deaths. CONCLUSIONS: The use of a PTFE membrane monocusp valve and a valve-sparing strategy prevents immediate PI and improves short-term clinical outcomes. PTFE membrane monocusp appears advantageous in preventing severe intermediate-term PI and facilitates the preservation of RV function.

Neurologic involvement in a child with systemic capillary leak syndrome.

Idiopathic systemic capillary leak syndrome (SCLS) is a rare and life-threatening disorder of unknown pathology. It is characterized by recurrent episodes of shock resulting from leakage of plasma, which is reflected by accompanying hemoconcentration, hypoalbuminemia, and edema. Since its first description (Clarkson B, Thompson D, Horwith M, Luckey A. Am J Med. 1960;29:193-216), there have been only 3 descriptions of children with the disorder. Familial cases have not been reported. Brain involvement has only been described for adults and with minimal radiologic findings. We report here an unusual case of an 8-year-old boy with multiple episodes of SCLS since the age of 5 months and an exceptional presentation characterized by substantial neurologic involvement with cerebellar edema and autonomic dysfunction. The patient's family history was remarkable for 8 more relatives with the disorder, including his sister who died during a similar episode in infancy and a first-degree cousin of his father who was diagnosed as suffering from recurrent episodes of SCLS. Our patient is, to our knowledge, the first patient with SCLS with a family history of the disorder. Additional genetic studies in the extended family might shed light on the pathogenesis of this rare disorder.

Aerosol delivery in respiratory syncytial virus bronchiolitis: hood or face mask?

OBJECTIVES: To compare the utility of the hood versus the face mask for delivery of inhaled medications to infants hospitalized with viral bronchiolitis. STUDY DESIGN: Randomized, double-blinded, controlled trial; 49 hospitalized infants with viral bronchiolitis, age 2.75 +/- 2.2 months (mean +/- SD), were grouped to either the hood (n = 25) or the mask (n = 24). Each subject received inhalation treatments with the use of both devices. Half of the Hood Group received the active drug treatment (1.5 mg epinephrine in 4 mL saline [3%]) via hood followed immediately by placebo treatment (normal saline) via mask, whereas the other half received the opposite order. Half of the Mask Group received the active drug treatment via mask followed immediately by placebo treatment via hood, whereas the other half received the opposite order. Therapy was repeated 3 times daily until discharge. Outcome measures included clinical scores and parental preference. RESULTS: Percent improvement in clinical severity scores after inhalation was significant in both groups on days 1, 2, and 3 after admission (Hood Group: 15%, 15.4%, and 16.4%, respectively; Mask Group: 17.5%, 12.1%, and 12.7%, respectively; P < .001). No significant difference in clinical scores improvement between groups was observed. Eighty percent (39/49) of parents favored the hood over the mask; 18% (9/49) preferred the mask and 2% (1/49) were indifferent. CONCLUSIONS: In infants hospitalized with viral bronchiolitis and in whom aerosol treatment is considered, aerosol delivery by hood is as effective as by mask. However, according to parents, the tolerability of the hood is significantly better than that of a mask.

Association between common Toll-like receptor 4 mutations and severe respiratory syncytial virus disease.

BACKGROUND: The clinical spectrum of respiratory syncytial virus (RSV) bronchiolitis in previously healthy infants is extremely variable. Thus, it is likely that factors such as genetic heterogeneity contribute to disease severity. Toll-like receptor 4 (TLR4) and CD14 are part of a receptor complex involved in the innate immune response to RSV. METHODS: The association of the TLR4 mutations (Asp299Gly and Thr399Ile) and the CD14/-159 polymorphism were analyzed in 99 infants hospitalized with severe RSV bronchiolitis (group I). Eighty-two ambulatory infants with mild RSV bronchiolitis (group II) and 90 healthy adults (group III) composed the 2 control groups. The TLR4 mutations and the CD14/-159 polymorphism were genotyped by use of reverse-transcriptase polymerase chain reaction and restriction fragment-length polymorphism analysis, respectively. RESULTS: Each of the TLR4 mutations, either alone or in cosegregation, were associated with severe RSV bronchiolitis: the Asp299Gly and Thr399Ile mutations were significantly overrepresented in group I, compared with groups II and III. No association between the CD14/-159 polymorphism and RSV bronchiolitis was found. CONCLUSIONS: These findings suggest that TLR4 mutations, but not the CD14/-159 polymorphism, are associated with an increased risk of severe RSV bronchiolitis in previously healthy infants.