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 (PAp) 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 (Rp:Rs) 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.

Risk factors for interstage death after the Norwood procedure.

Operative survival after the Norwood procedure has significantly improved during the past 10 years. However, there remains attrition among Norwood survivors before reaching planned second-stage palliation. The purpose of this study was to evaluate potential risk factors for interstage mortality among Norwood survivors. All patients undergoing the Norwood procedure at the Medical University of South Carolina from January 1996 through January 2001 were retrospectively reviewed. Patient and procedural variables were examined as potential risk factors for interstage mortality. Among 50 Norwood survivors, 8 (16%) died prior to second-stage palliation. The mean age at death was 102 +/- 72 days (median, 61; range, 35-208). By multivariate analysis, the presence of an arrhythmia in the postoperative period (p = 0.02) and decreased ventricular function at hospital discharge (p = 0.05) were identified as risk factors for interstage mortality. There remains a significant risk for interstage mortality among Norwood survivors. Patients with postoperative arrhythmias and/or decreased ventricular function at discharge are at increased risk for interstage death after Norwood procedure. More frequent follow-up and aggressive medical management of arrhythmia or decreased function may be warranted for these high-risk patients.

Mechanical circulatory support for the treatment of children with acute fulminant myocarditis.

BACKGROUND: Viral myocarditis may follow a rapidly progressive and fatal course in children. Mechanical circulatory support may be a life-saving measure by allowing an interval for return of native ventricular function in the majority of these patients or by providing a bridge to transplantation in the remainder. METHODS: A retrospective chart review of 15 children with viral myocarditis supported with extracorporeal membrane oxygenation (12 patients) or ventricular assist devices (3 patients) was performed. RESULTS: All patients had histories and clinical findings consistent with acute myocarditis. The median age was 4.6 years (range 1 day-13.6 years) with a median duration of mechanical circulatory support of 140 hours (range 48-400 hours). Myocardial biopsy tissue demonstrated inflammatory infiltrates or necrosis, or both, in 8 (67%) of the 12 patients who had biopsies. Overall survival was 12 (80%) of 15 patients, with 10 (83%) survivors of extracorporeal membrane oxygenation and 2 (67%) survivors of ventricular assist device support. Nine (60%) of the 15 patients were weaned from support, with 7 (78%) survivors; the remaining 6 patients were successfully bridged to transplantation, with 5 (83%) survivors. All survivors not undergoing transplantation are currently alive with normal ventricular function after a median follow-up of 1.1 years (range 0.9-5.3 years). CONCLUSION: Eighty-percent of the children who required mechanical circulatory support for acute myocarditis survived in this series. Recovery of native ventricular function to allow weaning from support can be anticipated in many of these patients with excellent prospects for eventual recovery of full myocardial function.

Hemodynamic effects of inspired carbon dioxide after the Norwood procedure.

BACKGROUND: Mortality in the early postoperative period after the Norwood procedure remains substantial. Inspired carbon dioxide (CO2) has been suggested to improve hemodynamic status in this setting. Inspired CO2 can be delivered by one of two strategies, ie, with or without an accompanying increase in minute ventilation. The hemodynamic effects of these two strategies have not previously been studied in a controlled fashion. METHODS: Seventeen infants (median age, 9 days; range, 4 to 49 days) undergoing Norwood procedures were prospectively enrolled in this crossover study. Patients were studied while sedated, paralyzed, and mechanically ventilated 1 day to 6 days after operation. The inspired oxygen fraction was kept constant (mean value, 0.24 +/- 0.01). Measurements were made at five time points: 1 = baseline; 2 = inspired CO2 with increased ventilation; 3 = baseline; 4 = inspired CO2 alone; and 5 = baseline. Mixed venous oxygen saturation was monitored using indwelling lines in the superior vena cava. RESULTS: Inspired CO2 with increased ventilation produced a rise in mean airway pressure with no change in arterial CO2 tension or pH. This strategy had no effect on hemodynamic status or oxygen delivery. Inspired CO2 alone produced a rise in arterial CO2 tension and a fall in arterial pH (respiratory acidosis). This strategy resulted in significant improvement in both variables of systemic oxygen delivery: mixed venous oxygen saturation increased from 48% +/- 2% to 56% +/- 2% (p < 0.05), and arteriovenous oxygen saturation difference decreased from 3% +/- 2% to 26% +/- 2% (p < 0.05). CONCLUSIONS: Inspired CO2 after the Norwood procedure can improve oxygen delivery. This improvement occurs only if minute ventilation is kept constant. There is no improvement if minute ventilation is increased. Clinical use of inspired CO2 may be limited by the accompanying fall in pH. Differentiation of cerebral from total-body effects of inspired CO2 will require further study.

Functional pulmonary atresia in neonatal Marfan's syndrome: successful treatment with inhaled nitric oxide.

Functional pulmonary atresia is characterized by a structurally normal pulmonary valve not opening during right ventricular ejection. We report this rare condition in a premature newborn of a twin pregnancy, in which fetal echocardiography findings were consistent with critical pulmonary stenosis. After birth, features of neonatal Marfan's syndrome were noted. Echocardiography showed a morphologically normal but immobile pulmonary valve with continuous regurgitation. Right ventricular pressure was subsystemic. In this case, initial treatment with nitric oxide, followed by pharmacological duct closure, was successful. Differentiating between anatomic and functional pulmonary valve atresia may be difficult. The echocardiographic criteria are discussed.

Simulium spp. control program in Rio Grande do Sul, Brazil.

Insects of the Simuliidae family have been the object of control in Rio Grande do Sul since the 70s. Their constant attacks became a social-economical problem as well as a problem of Public Health, with serious consequences to men and to the economy of the areas in which the insects develop. At first, the control was done with a chemical larvicide Themephos ABATE 500 E, but an imperfect measuring of outflow to determine the quantity of the product made Simulium spp. resistant to it. From 1983 on, following a study of a new method for the outflow measuring, we started to use a biological larvicide Bacillus thuringiensis serovar israelensis based. The biological control uses the new method in 36.4% of the state area, assisting about 3,500,000 inhabitants.

Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect.

A severely restrictive atrial septal defect (ASD) in neonates with hypoplastic left heart syndrome (HLHS) results in pulmonary venous hypertension, pulmonary edema, and intractable hypoxia. Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheterization at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography. Three underwent blade septostomy with 2 procedure-related deaths, and 1 had balloon atrial septostomy (BAS); all 4 died before surgical intervention. Fifteen underwent Brockenbrough atrial septoplasty with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a stent placed across the atrial septum after transatrial needle puncture. In the 16 patients treated with septoplasty or stent, oxygen saturation increased from 50 +/- 4% to 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting transplantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation. Eight of 14 (57%) survived to hospital discharge. Six of 7 (86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in profound cyanosis demands urgent intervention. BAS is frequently unsuccessful and blade septostomy has high mortality. Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, allowing stabilization before reconstructive surgery or while awaiting transplant.

Combined effects of nitric oxide and oxygen during acute pulmonary vasodilator testing.

OBJECTIVES: We compared the ability of inhaled nitric oxide (NO), oxygen (O2) and nitric oxide in oxygen (NO+O2) to identify reactive pulmonary vasculature in pulmonary hypertensive patients during acute vasodilator testing at cardiac catheterization. BACKGROUND: In patients with pulmonary hypertension, decisions regarding suitability for corrective surgery, transplantation and assessment of long-term prognosis are based on results obtained during acute pulmonary vasodilator testing. METHODS: In group 1, 46 patients had hemodynamic measurements in room air (RA), 100% O2, return to RA and NO (80 parts per million [ppm] in RA). In group 2, 25 additional patients were studied in RA, 100% O2 and 80 ppm NO in oxygen (NO+O2). RESULTS: In group 1, O2 decreased pulmonary vascular resistance (PVR) (mean+/-SEM) from 17.2+/-2.1 U.m2 to 11.1+/-1.5 U.m2 (p < 0.05). Nitric oxide caused a comparable decrease from 17.8+/-2.2 U.m2 to 11.7+/-1.7 U.m2 (p < 0.05). In group 2, PVR decreased from 20.1+/-2.6 U.m2 to 14.3+/-1.9 U.m2 in O2 (p < 0.05) and further to 10.5+/-1.7 U.m2 in NO+O2 (p < 0.05). A response of 20% or more reduction in PVR was seen in 22/25 patients with NO+O2 compared with 16/25 in O2 alone (p = 0.01). CONCLUSIONS: Inhaled NO and O2 produced a similar degree of selective pulmonary vasodilation. Our data suggest that combination testing with NO + O2 provides additional pulmonary vasodilation in patients with a reactive pulmonary vascular bed in a selective, safe and expeditious fashion during cardiac catheterization. The combination of NO+O2 identifies patients with significant pulmonary vasoreactivity who might not be recognized if O2 or NO were used separately.

Inhaled nitric oxide in the neonate with cardiac disease.

As a selective pulmonary vasodilator, inhaled nitric oxide is an important diagnostic and therapeutic agent for the treatment of pulmonary hypertension in patients with congenital heart disease. Among 400 patients treated in our center with nitric oxide, 37% were newborns. Hemodynamic benefit was shown in newborns with total anomalous pulmonary venous connection, in those with congenital mitral stenosis, and in postoperative patients with preexisting left to right shunts and other lesions. It can be used to help discriminate anatomic obstruction to pulmonary blood flow from pulmonary vasoconstriction, and it may be used in the treatment or prevention of pulmonary hypertensive crises after cardiopulmonary bypass. However, none of the purported benefits of inhaled nitric oxide in children with congenital heart disease have been studied in a randomized, placebo-controlled manner.

Rebound pulmonary hypertension after inhalation of nitric oxide.

BACKGROUND: We describe the hemodynamic response to initiation and withdrawal of inhaled nitric oxide (NO) in infants with pulmonary hypertension after surgical repair of total anomalous pulmonary venous connection. METHODS: Between January 1, 1992, and January 1, 1995, 20 patients underwent repair of total anomalous pulmonary venous connection. Nine patients had postoperative pulmonary hypertension and received a 15-minute trial of inhaled NO at 80 parts per million. Five of these patients received prolonged treatment with NO at 20 parts per million or less. RESULTS: Mean pulmonary artery pressure decreased from 35.6 +/- 2.4 to 23.7 +/- 2.0 mm Hg (mean +/- standard error of the mean) (p = 0.008), and pulmonary vascular resistance decreased from 11.5 +/- 2.0 to 6.4 +/- 1.0 U.m2 (p = 0.03). After prolonged treatment with NO, pulmonary artery pressure increased transiently in all patients when NO was discontinued. CONCLUSIONS: After operative repair of total anomalous pulmonary venous connection, inhaled NO selectively vasodilated all patients with pulmonary hypertension. Withdrawal of NO after prolonged inhalation was associated with transient rebound pulmonary hypertension that dissipated within 60 minutes. Appreciation of rebound pulmonary hypertension may have important implications for patients with pulmonary hypertensive disorders when interruption of NO inhalation is necessary or when withdrawal of NO is planned.

Inhaled nitric oxide in children with pulmonary hypertension and congenital mitral stenosis.

We demonstrated selective pulmonary vasodilation in patients with congenital mitral stenosis with inhaled NO, and used NO as prolonged therapy to stabilize the postintervention course of these children. The pulmonary vasoreactivity was greater than that previously reported in adults and may be due to particular sensitivity of pulmonary veins to inhaled NO when pulmonary venous hypertension has been present since birth.

Milrinone: systemic and pulmonary hemodynamic effects in neonates after cardiac surgery.

OBJECTIVE: To evaluate the hemodynamic effects of intravenous milrinone in neonates with low cardiac output after cardiac surgery. DESIGN: Prospective cohort study. SETTING: Pediatric cardiac intensive care unit. PATIENTS: Ten neonates with low cardiac output (cardiac index of < or = 3.0 L/min/m2) after corrective cardiac surgery were enrolled in the study. The neonates' ages ranged from 3 to 27 days (median 5) and their weights ranged from 2.0 to 4.8 kg (median 3.7). The diagnoses were: transposition of the great arteries (n = 6, including two with ventricular septal defect), tetralogy of Fallot (n = 2), truncus arteriosus (n = 1), and total anomalous pulmonary venous connection (n = 1). INTERVENTIONS: Milrinone was intravenously administered in three stages: a) baseline stage, in which patients had a stable hemodynamic status, ventilation and gas exchange, hemostasis, and body temperature; b) loading stage, in which a 50 microgram/kg intravenous loading dose of milrinone was administered over 15 mins; and c) infusion stage, in which milrinone was continuously infused at 0.50 microgram/kg/min for 30 mins. MEASUREMENTS AND MAIN RESULTS: The mean heart rate increased after the loading stage (149 +/- 13 to 163 +/- 12 beats/min, p < .01) but slowed during the infusion stage (154 +/- 11 beats/min, p < .01 vs. loading stage). Both right and left atrial pressures were lowered in all ten neonates. Compared with baseline, mean arterial pressure decreased after the loading stage (66 +/- 12 to 57 +/- 10 mm Hg, p < .01) but did not decrease further at the infusion stage (59 +/- 12 mm Hg); changes in mean pulmonary arterial pressure were comparable. Cardiac index increased from a baseline mean of 2.1 +/- 0.5 to 3.0 +/- 0.8 L/min/m2 (p < .01) with the loading stage, and was maintained at 3.1 +/- 0.6 L/min/m2 during the infusion stage. Systemic vascular resistance index decreased below baseline values with loading, from 2136 +/- 432 to 1336 +/- 400 dyne.sec/cm5.m2 (p < .01), and pulmonary vascular resistance index also decreased with loading dose of milrinone, from 488 +/- 160 to 360 +/- 120 dyne.sec/cm5.m2 (p < .01). There was no change in the rate pressure index, an indirect measurement of myocardial oxygen consumption, throughout the study. CONCLUSIONS: Administration of milrinone in neonates with low cardiac output after cardiac surgery lowers filling pressures, systemic and pulmonary arterial pressures, and systemic and pulmonary vascular resistances, while improving cardiac index. Milrinone increases heart rate without altering myocardial oxygen consumption. While milrinone appears to be effective and safe during short-term use, the relative distribution of inotropic and vasodilatory properties of milrinone remains to be elucidated.