James W. Fasules

Blade and balloon atrial septostomy for left heart decompression in patients with severe ventricular dysfunction on extracorporeal membrane oxygenation

Extracorporeal membrane oxygenation (ECMO) is used as circulatory support or bridge to transplantation in patients with severe left ventricular (LV) dysfunction. Left heart decompression is needed to reduce pulmonary edema, prevent pulmonary hemorrhage, and reduce ventricular distention that may aid in recovery of function. We reviewed our experience from November 1993 to December 1997 with 10 patients having severe LV dysfunction (7 myocarditis, 3 dilated cardiomyopathy) who required circulatory support with ECMO and who underwent left heart decompression with blade and balloon atrial septostomy (BBAS). Patients ranged in age from 1 to 24 years (median, 3 years). Indications for BBAS included left atrial/left ventricular distension (10), pulmonary edema/hemorrhage (9), or severe mitral regurgitation (2). BBAS was performed electively in eight patients and urgently in two patients. BBAS was performed while on ECMO in seven patients and pre‐ECMO in three. A femoral venous approach was used in all patients. ECMO patients were fully heparinized. Transseptal puncture was required in nine patients while one patient had a patent foramen ovale. Blade septostomy was performed in all patients. Enlargement of the defect was then performed by stationary balloon dilation in nine and Rashkind balloon atrial septostomy in one. Balloon diameters ranged from 10 to 20 mm. Sequential balloon inflations were performed in some patients. Adequacy of the atrial septal defect (ASD) was confirmed by pressure measurement and echocardiography. Adequate left heart decompression was achieved in all patients. Pulmonary edema improved in nine of nine patients. Left atrial mean pressure fell from a mean of 30.5 mm Hg, (range, 12–50 mm Hg) to 16 mm Hg (range, 9–24 mm Hg). Left atrial to right atrial pressure gradient fell from a mean of 20 mm Hg pre‐BBAS to 3 mm Hg post‐BBAS. ASDs ranged in size from 2.5 to 8 mm (mean, 5.9 mm). Complications included needle perforation of the left atrium without hemodynamic compromise (one), ventricular fibrillation requiring defibrillation (one), and hypotension following BBAS which responded to volume infusion (two). Duration of ECMO ranged from 41 hr to 704 hr (mean, 294 hr). Seven patients survived and four patients had recovery of normal LV function. Of those who recovered, two had no ASD at follow‐up while two ASDs are patent 14 days and 3 months post‐BBAS. Three patients underwent successful cardiac transplantation. Three patients died, all of whom had multisystem organ failure with or without sepsis. A patent ASD was noted at transplant (three) or autopsy (two). No patient required a second BBAS. BBAS alleviates severe left atrial hypertension and pulmonary edema. In addition, BBAS avoids the potential bleeding complications of surgical left heart decompression. Stationary balloon dilation of the atrial septum is an effective alternative to Rashkind balloon septostomy in older patients. BBAS achieves left heart decompression that may permit recovery of LV function or allow extended ECMO support as a bridge to transplant. Cathet. Cardiovasc. Intervent. 46:179–186, 1999.

Extracorporeal Membrane Oxygenation for Cardiac Failure After Congenital Heart Operation

Despite continuing improvement in myocardial protection and surgical technique, the repair of complex congenital heart lesions can result in cardiopulmonary compromise refractory to conventional therapy. In a 29-month period, 24 patients (aged 14 hours to 6 years) were treated with extracorporeal membrane oxygenation (ECMO) 28 times for profound cardiopulmonary failure. Four patients required ECMO after each of two cardiopulmonary bypass procedures. Seventeen patients required ECMO to be initiated in the operating room: 12 (71%) were weaned successfully from ECMO, and 8 (47%) survived. Seven patients had ECMO initiated in the intensive care unit: 6 (86%) were weaned, and 5 (71%) survived. Serial echocardiograms demonstrated substantial recovery of cardiac function in 18 of 21 instances (86%) of ventricular failure from myocardial dysfunction. Overall, 18 of 24 patients (75%) were successfully weaned from ECMO including all 4 who underwent 2 ECMO treatments. We conclude that ECMO can successfully salvage children who have serious cardiopulmonary failure immediately after a congenital heart operation and that long-term survival is possible after two ECMO treatments.

Single-institution experience with interhospital extracorporeal membrane oxygenation transport: A descriptive study.

Objective: Patients with refractory cardiopulmonary failure may benefit from extracorporeal membrane oxygenation, but extracorporeal membrane oxygenation is not available in all medical centers. We report our institutions nearly 20-yr experience with interhospital extracorporeal membrane oxygenation transport. Design: Retrospective review. Setting: Quaternary care childrens hospital. Patients: All patients undergoing interhospital extracorporeal membrane oxygenation transport by the Arkansas Childrens Hospital extracorporeal membrane oxygenation team. Interventions: Data (age, weight, diagnosis, extracorporeal membrane oxygenation course, hospital course, mode of transport, and outcome) were obtained and compared with the most recent Extracorporeal Life Support Organization Registry report. Results: Interhospital extracorporeal membrane oxygenation transport was provided to 112 patients from 1990 to 2008. Eight were transferred between outside facilities (TAXI group); 104 were transported to our hospital (RETURN group). Transport was by helicopter (75%), ground (12.5%), and fixed wing (12.5%). No patient died during transport. Indications for extracorporeal membrane oxygenation in RETURN patients were cardiac failure in 46% (48 of 104), neonatal respiratory failure in 34% (35 of 104), and other respiratory failure in 20% (21 of 104). Overall survival from extracorporeal membrane oxygenation for the RETURN group was 71% (74 of 104); overall survival to discharge was 58% (61 of 104). Patients with cardiac failure had a 46% (22 of 48) rate of survival to discharge. Neonates with respiratory failure had an 80% (28 of 35) rate of survival to discharge. Other patients with respiratory failure had a 62% (13 of 21) rate of survival to discharge. None of these survival rates were statistically different from survival rates for in-house extracorporeal membrane oxygenation patients or for survival rates reported in the international Extracorporeal Life Support Organization Registry (p > .1 for all comparisons). Conclusions: Outcomes of patients transported by an experienced extracorporeal membrane oxygenation team to a busy extracorporeal membrane oxygenation center are very comparable to outcomes of nontransported extracorporeal membrane oxygenation patients as reported in the Extracorporeal Life Support Organization registry. As has been previously reported, interhospital extracorporeal membrane oxygenation transport is feasible and can be accomplished safely. Other experienced extracorporeal membrane oxygenation centers may want to consider developing interhospital extracorporeal membrane oxygenation transport capabilities to better serve patients in different geographic regions.

Implementing recommended screening for critical congenital heart disease.

BACKGROUND AND OBJECTIVE: Critical congenital heart disease (CCHD) is endorsed by the US Secretary of Health and Human Services as part of the recommended uniform screening panel for newborns. Although initial recommendations for implementation exist, as states and hospitals have moved forward with implementation of screening, new challenges and areas for additional focus have been identified. The objective of this study was to develop recommendations to address current challenges and areas of focus surrounding CCHD newborn screening. METHODS: A workgroup of experts and stakeholders was convened in Washington, District of Columbia, for a 1-day meeting in February 2012. At the beginning of the meeting, the stakeholders held a brainstorming session to identify areas of main priority based on their experience. After this, stakeholders broke into small groups to refine recommendations, which were then finalized by consensus. RESULTS: Recommendations to address selection of screening equipment, standards for reporting of screening outcomes to stakeholders, training of health care providers and educating families, future research priorities, payment for screening, follow-up diagnostic testing, and public health oversight, and advocacy to facilitate effective and comprehensive screening were proposed. Suggestions for future work were developed. CONCLUSIONS: Screening for CCHD presents novel challenges and opportunities; however, addressing these will strengthen newborn screening and newborn care networks, and ultimately improve health outcomes.

Prolonged extracorporeal life support for bridging to transplant: technical and mechanical considerations.

Through July 1995, the Extracorporeal Life Support Organization (ELSO) registry listed 87 patients who received extracorporeal life support (ECLS) as a bridge to cardiac transplantation with a survival rate of 41%. At Arkansas Children’s Hospital, 17 patients (aged between two days and 24 years) with diagnoses of dilated cardiomyopathy (seven), postcardiotomy (seven) and acute viral myocarditis (three) were bridged with ECLS. Mechanical complications only occurred in two patients, neither of which necessitated withdrawal of ECLS. Decompression of the left heart was performed in 11 patients, six via a surgically placed vent and five with a blade/balloon atrial septostomy. Documented infection occurred in 11/17 patients, but only one patient died from infection. Fifteen of 17 patients (88%) recovered or were transplanted, of which 13 (76%) were discharged home. With left-heart decompression and appropriate treatment of infection, ECLS may be used as a bridge to cardiac transplantation or until the return of cardiac function.

Pharmacokinetics and pharmacodynamics of bumetanide in neonates treated with extracorporeal membrane oxygenation

Eleven term neonates treated with extracorporeal membrane oxygenation received bumetanide to treat volume overload. All patients had stable renal function, no history of prior diuretic therapy, and no overt evidence of hepatobiliary disease or hypoalbuminemia. Pretreatment creatinine clearance was 35.2 +/- 4.5 ml/min per 1.73 m2 (range, 20.3 to 57.5). Bumetanide, 0.095 +/- 0.003 mg/kg, was administered for 2 minutes into the postmembrane side of the extracorporeal membrane oxygenation circuit. Serial plasma and urine samples were collected for measurement of bumetanide and electrolyte concentrations. Total plasma and renal clearances for bumetanide were 0.63 +/- 0.11 and 0.16 +/- 0.04 ml/min per kilogram, respectively. The steady-state volume of distribution (0.44 +/- 0.03 L/kg) and the elimination half-life (13.2 +/- 3.8 hours) were greater than similar values reported in previous studies of bumetanide disposition in premature and term neonates who were not treated with extracorporeal membrane oxygenation. At observed rates of bumetanide excretion, the diuretic, natriuretic, and kaliuretic responses were linear. Significant diuresis, natriuresis, and kaliuresis were observed, although the duration of these effects was less than expected given the prolonged renal elimination of bumetanide. Nonrenal elimination of bumetanide was variable (47.2% to 96.9%) but higher than expected; this may explain the relatively brief diuretic and kaliuretic response.

Pharmacokinetics and Pharmacodynamics of Ranitidine in Neonates Treated with Extracorporeal Membrane Oxygenation

The pharmacokinetics and pharmacodynamics of ranitidine were studied in 13 term neonates with stable renal and hepatic function who were treated with extracorporeal membrane oxygenation (ECMO). Ranitidine was initially administered as a single 2 mg/kg dose over 10 minutes and intragastric pH was monitored to determine response. Within 90 minutes after administration of ranitidine, intragastric pH for all of the patients whose initial reading was ≤ 4 had increased to > 5. Intragastric pH remained < 4 for a minimum of 15 hours. Mean ± 1 standard deviation elimination half‐life was 6.61 ± 2.75 hours, and 41.5 ± 22.2% of the single dose was eliminated in urine within 24 hours. Total plasma clearance of ranitidine correlated well with estimated glomerular filtration rate. Twenty‐four hours after the initial dose, a continuous infusion of ranitidine (2 mg/kg/24 hr) was started and continued for 72 hours or until ECMO was discontinued. Eleven patients completed 48 hours of continuous infusion and seven completed all 72 hours. Plasma clearance and elimination half‐life were determined from steady‐state plasma ranitidine concentrations 24, 46, and 72 hours after the start of the infusion. There were no significant differences in clearance between these intervals. These data suggest that for term neonates with stable renal and hepatic function, ranitidine does not need to be administered more frequently than every 12 hours. A continuous infusion of 2 mg/kg/24 hours maintained intragastric pH above 4 in more than 90% of our patients, and in our opinion is the preferred method for delivering ranitidine to term neonates undergoing ECMO who require H2 antagonists. Response to therapy should be monitored by repeated measurement of gastric pH and the dose should be adjusted accordingly.

Mobile extracorporeal membrane oxygenation

Mobile extracorporeal membrane oxygenation (ECMO) is being offered by select ECMO centers in the United States. Mobile ECMO can be performed for the critically ill patient who is unable to be transported by conventional ambulance transport. This article discusses the logistics and safety considerations associated with mobile ECMO.

Hemodynamic troubleshooting for mechanical malfunction of the extracorporeal membrane oxygenation systems using the PPP triad of variables

Prolonged usage of disposable extracorporeal membrane oxygenation (ECMO) circuitry increases the risk of mechanical complications due to breakdown or malposition of the circuit elements. Often, such complications are life threatening for the critically ill patient. Such problems need to be rapidly identified and corrected. Algorithms can be especially helpful in such acute, life-threatening situations. We have outlined an algorithm that uses the relationship between three hemodynamic variables that can be used to rapidly identify mechanical dysfunctions associated with use of the ECMO circuit. These hemodynamic variables are premembrane pressure, pump flow, and patient mean systemic arterial pressure (the PPP triad). These variables are interrelated as a change in one variable results in a change in another. Mechanical malfunction can eliminate this relationship. Changes in one variable only suggest mechanical impairment or failure of the ECMO system. When such a change is detected, a checklist can be rapidly reviewed that directs an immediate logical assessment of potential mechanical causes of hemodynamic compromise.