The first successful DeBakey VAD child implantation as a bridge to transplant.

A 14-year-old boy with repaired transposition of the great arteries and ventricular septal defect presented with atrial flutter and severe congestive heart failure. Despite successful cardioversion and optimal medical therapy, the patient deteriorated and was supported with extracorporeal membrane oxygenation (ECMO). Two days after initiating ECMO support, we implanted the DeBakey VAD Child ventricular assist device (MicroMed Technology, Inc., Houston, TX) under the Humanitarian Device Exemption program. Later, he was able to pursue normal daily activities including physical rehabilitation and ambulation in the hospital. After 56 days, he underwent a successful cardiac transplantation. After 3 months, he had good cardiac function and no evidence of rejection. The DeBakey VAD Child device is a valuable option for cardiac support as a bridge to transplantation.

Expanding the donor pool: use of a donor heart having undergone recent cardiac surgery.

The current shortage of donor organs is a well-recognized global phenomenon. The goal of contemporary transplant practice is to optimize and expand the organ donor pool. Despite biologic and technologic advancements, the single most important limitation remains underutilization and non-recovery of potential organs. We report the use of a donor cardiac organ from a 5-year-old child who had undergone recent (<72 hours) closure of an atrial septal defect. The heart was harvested successfully and implanted with no apparent hemodynamic compromise.

Pediatric arteriovenous extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation.

BACKGROUND: Since 1990, extracorporeal membrane oxygenation (ECMO) has been used as a bridge to cardiac transplantation in 47 patients. METHODS: A review of the ECMO database, approved by the Arkansas Children's Hospital institutional review board, forms the basis of this report. We made statistical comparison using Fisher's exact probability testing. The ECMO circuitry was a roller occlusion pump with computer-assisted perfusion system technology. RESULTS: Thirty-two (68%) patients underwent transcatheter septostomy for cardiac decompression. Diagnosis at presentation was either congenital heart disease (CHD, n = 15) or cardiomyopathy (n = 32). Ages ranged from 1 day to 22 years old (median, 18 months old), and weight ranged from 2.9 to 100 kg (median, 10 kg). The average duration of support was 242 hours (range, 22-1078 hours). Overall long-term survival was 47%, with 16 (34%) patients successfully bridged to cardiac transplantation (of which 9 [56%] survived) and 13 (28%) successfully weaned from ECMO. Patients undergoing ECMO after cardiotomy had 31% survival. Survival was improved significantly (p < 0.02) in patients with cardiomyopathy (59%) vs those with CHD (20%). Patients with cardiomyopathy underwent 8 transplantations with 7 survivors (88%), whereas in the CHD group, there were 8 transplantations with only 2 survivors (25%), p < 0.05. Sub-analysis of the cardiomyopathy group revealed that patients with acute cardiomyopathy in association with documented viral illness had a 75% chance of being weaned from ECMO without undergoing transplantation. Complications during ECMO occurred in 45% of survivors and were more frequent in non-survivors. Infectious complications were most frequent, followed by neurologic complications, technical ECMO problems, and renal insufficiency. CONCLUSIONS: Patients with cardiomyopathy has a better prognosis than did those with CHD when using ECMO as a bridge to transplantation or survival. Complications are significant and increase with the duration of support. Extracorporeal membrane oxygenation for salvage and subsequent transplantation in this high-risk group of patients requires critical review. Alternative support options must be developed in the pediatric population that will allow improved outcomes, comparable with outcomes achieved in the adult population.

Mitral valve replacement in children: predictors of long-term outcome.

BACKGROUND: Mitral valve replacement (MVR) in children has been associated with a high complication rate. We sought to assess predictors of outcomes in children undergoing MVR. METHODS: A retrospective review of clinical, surgical, and echocardiographic records of patients undergoing MVR was performed. Between 1982 and 2000, 53 children underwent 76 MVR procedures at a median age of 5 years (range, 1 day to 18 years) and weight of 17 kg (range, 3 to 121 kg). Eighteen patients (34%) had more than one MVR. Previous cardiac surgery had been performed in 39 (74%), with 27 (51%) undergoing previous mitral repair. Patients were followed for 9.2 +/- 4.8 (range, 2 to 20) years. RESULTS: There were 14 patient deaths, with 6 patients dying within 30 days, and five transplants (36%). Ten-year freedom from reoperation was 66%. Long-term survivors were older at initial repair (7.0 vs 2.5 years, p = 0.02), with a lower incidence of residual cardiac lesions (3% vs 37%, p < 0.001) and a lower incidence of surgical procedures at the time of MVR (31% vs 63%, p = 0.04). Survivors had better left ventricular function preoperatively (ejection fraction, 68% vs 54%; p = 0.001) and placement of a prosthetic valve within 1 z-score of the echocardiographically measured mitral valve annulus (p = 0.02). CONCLUSIONS: Adverse outcome after MVR is common, particularly in the young child undergoing palliative surgery or requiring additional surgical procedures. Preoperative assessment of mitral valve size and ventricular function is essential for risk stratification of these patients.

Selective timing for the arterial switch operation.

BACKGROUND: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. METHODS: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. RESULTS: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15-46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3-83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). CONCLUSIONS: Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.

Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method.

OBJECTIVE: Results of the repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) have improved. Direct implantation of the anomalous coronary artery into the ascending aorta establishes a dual-coronary system and is the goal of current surgical approaches. We report the development of our surgical technique for ALCAPA. METHODS: Between September 1993 and December 2000, 13 patients underwent surgery for ALCAPA. There were four males and nine females. Ages ranged from 1 month to 25 years (median=3.9) and weight ranged from 2.6 to 102kg (median=16.8). One patient had previously undergone an operative procedure at an outside institution. RESULTS: Direct implantation of the anomalous coronary artery into the ascending aorta was feasible in 12 of 13 patients. In situ transfer was performed in one patient with an intramural coronary artery. The first case in the series required an intrapulmonary baffle reconstruction (Takeuchi procedure) because the coronary artery arose remotely from the ascending aorta from the left-anterior sinus of the PA. For coronary transfer, a trapdoor flap was created on the ascending aorta for the implantation of the coronary button and the sinus defect in the main PA was augmented with a pericardial patch. The left ventricular (LV) shortening fraction was improved from a median value of 27% (range 12-36%) preoperatively to 33% (range 24-45%) in the immediate postoperative period (P=0.004). The LV end-diastolic dimension decreased from a median value of 36 mm (range 22-70 mm) preoperatively to 29 mm (range 19-56 mm) in the immediate postoperative period (P=0.004). There has been no mortality or reoperation during a median follow-up of 36 months. CONCLUSIONS: Using a standard technique, direct implantation of the anomalous coronary artery into the ascending aorta was achieved in all cases but one. At intermediate follow-up, LV function had improved by echocardiography. No postoperative mechanical circulatory support was required in any of these patients. This operative technique is reproducible and is applicable to the majority of patients with ALCAPA.

Failed extubation after cardiac surgery in young children: Prevalence, pathogenesis, and risk factors.

Background: Most children who undergo congenital heart surgery require postoperative mechanical ventilation. Failed extubation (FE) may result in physiologic instability, delay, or set back of the weaning process. FE is statistically associated with prolonged mechanical ventilation. Purpose: We sought to identify frequency, pathogenesis, and risk factors for FE after congenital heart surgery in young children. SETTING: Pediatric intensive care unit. PATIENTS: Children

Noninvasive cerebral oximeter as a surrogate for mixed venous saturation in children.

We evaluated the relationship between regional cerebral oxygen saturation (rSO(2)) measured by near-infrared spectroscopy (NIRS) cerebral oximeter with superior vena cava (SVC), inferior vena cava (IVC), right atrium (RA), and pulmonary artery (PA) saturation measured on room air and 100% inspired oxygen administered via a non-rebreather mask (NRB) in children. Twenty nine pediatric post-orthotopic heart transplant patients undergoing an annual myocardial biopsy were studied. We found a statistically significant correlation between rSO(2) and SVC saturations at room air and 100% inspired oxygen concentration via NRB (r = 0.67, p = 0.0002 on room air; r = 0.44, p = 0.02 on NRB), RA saturation (r = 0.56, p = 0.002; r = 0.56, p = 0.002), and PA saturation (r = 0.67, p < 0.001; r = 0.4, p = 0.03). A significant correlation also existed between rSO(2) and measured cardiac index (r = 0.45, p = 0.01) and hemoglobin levels (r = 0.41, p = 0.02). The concordance correlations were fair to moderate. Bias and precision of rSO(2) compared to PA saturations on room air were -0.8 and 13.9%, and they were 2.1 and 15.6% on NRB. A stepwise linear regression analysis showed that rSO(2) saturations were the best predictor of PA saturations on both room air (p = 0.0001) and NRB (p = 0.012). In children with biventricular anatomy, rSO(2) readings do correlate with mixed venous saturation.

Cardiopulmonary bypass for adults with congenital heart disease: pitfalls for perfusionists.

The fixed incidence of congenital heart defects and improved survival have resulted in increasing numbers of adults with congenital heart disease (CHD) who have undergone complex repairs and/or palliations. Eventually, there will be more adults with CHD than children. They will require cardiac surgical interventions associated with progression of their CHD or for age-related disease, such as coronary revascularization. During bypass, anatomical shunts may exist within or without the heart. Left-to-right shunts can result in dramatically lower systemic blood flow than pump flow due to 'steal', while pulmonary edema ensues due to excessive pulmonary flow. Right-to-left shunts carry risks of massive air embolism and double or triple venous cannulation may be necessary. Cannulation of composite reconstructed aortas may be difficult, risking dissection or aortic obstruction, and double arterial cannulation may be indicated. Aberrant coronary arterial and venous anatomy may .preclude adequate myocardial preservation with common techniques and can be complicated by aortic insufficiency. Valves and conduits may exhibit failure. Conventional monitoring, such as central venous oximetry, may be misleading. Monitoring, such as serial lactate measurement, near-infrared spectroscopy and transcranial Doppler blood velocity, offer advantages for such patients. The perfusionist needs to be aware of such conditions as much congenital aberrancy may present unexpectedly during cardiac surgery.

Adequacy of peritoneal dialysis in children following cardiopulmonary bypass surgery.

Acute renal failure requiring renal replacement therapy can complicate cardiopulmonary bypass in children. Peritoneal dialysis has been shown to stabilize electrolytes and improve fluid status in these patients. To assess dialysis adequacy in this setting, we prospectively measured Kt/V and creatinine clearance in five patients (6-839 days of age) requiring renal replacement therapy at our institution. Median dialysis creatinine clearance was 74.25 L/week/1.73m(2) (range 28.28-96.63 L/week/1.73m(2)). Residual renal function provided additional solute clearance as total creatinine clearance was 215.97 L/week/1.73m(2) (range 108.04-323.25 L/week/1.73m(2)). Dialysis Kt/V of >2.1 (median 4.84 [range 2.12-5.59]) was achieved in all patients. No dialysis-associated complications were observed. We conclude that peritoneal dialysis is a safe, simple method of providing adequate clearance in children who develop acute renal failure following exposure to cardiopulmonary bypass.

Management of potential gas embolus during closure of an atrial septal defect in a three-year-old.

Gas embolism occurring in adult patients supported with cardiopulmonary bypass is usually associated with mechanical complications. Management of gas embolism is less often described for the pediatric or neonatal patient. Measures to counteract gas embolism must be undertaken immediately if a satisfactory outcome is to be achieved. Here, the management of a three-year-old female patient, who was undergoing repair of a secundum atrial septal defect when the aortic cannula became dislodged and air entered the aorta, is described. Immediate implementation of an air embolism protocol, including (among other maneuvers) removal of air from the circuit, retrograde cerebral perfusion via the superior vena cava, and induction of cerebral hypothermia, may have aided in an acceptable outcome.

Management of continuous venous gas emboli during extracorporeal life support utilizing the Kolobow gas trap.

Extracorporeal life support (ECLS) with a roller pump system uses a closed cardiopulmonary bypass (CPB) circuit not equipped with a venous reservoir. Hence, gas emboli cannot escape the ECLS circuit, predisposing to clot formation, membrane failure and potential gas embolism. Rarely, some patients may develop a continuous release of gas into the venous circulation from multiple sources. Two pediatric ECLS cases are presented with continuous venous gas embolism. A 'gas trap' was devised by creating a column of fluid erected vertically on the venous line. This allowed gas to rise within the column, separating it from the ECLS circuit, thus, preventing gas from lodging in the membrane.

Successful surgical management of a neonate with a saddle pulmonary embolus.

A female neonate presented with evidence of a massive ventilation-perfusion mismatch. She was subsequently found to have a saddle pulmonary embolus. The infant successfully underwent surgical pulmonary embolectomy.

Optimizing cardioplegia strategy for donor hearts.

Variability in organ preservation strategy has thus far prevented meaningful analysis of clinical donor heart cardioplegia strategies. This paper describes our donor heart procurement protocol, techniques, and recovery team responsibilities. We present 21 patients receiving cardiac transplantation at our institution with an adopted cardioplegia protocol. The procurement team perfusionist ensures consistent attention to myocardial protection with standards that are similar to those applied to native hearts for all congenital heart surgeries.

Venovenous extracorporeal membrane oxygenation for cyanotic congenital heart disease.

BACKGROUND: Severe, refractory hypoxemia complicating uncorrected cyanotic congenital heart disease is a potentially lethal condition, even when urgent surgical intervention is undertaken. When a viral pneumonia initiates hypoxemia, the likelihood of a satisfactory outcome is further reduced. We examined our policy of venovenous extracorporeal membrane oxygenation support through the hypoxic event and performing delayed surgery, if required, to separate from extracorporeal membrane oxygenation. METHODS: A single institution, retrospective review of an Institutional Review Board approved database was undertaken. Over a 6-year period, 18 instances were identified for 17 patients who became acutely hypoxemic from either inadequate pulmonary blood flow (8 instances) or a viral pneumonia (10 instances) complicating their cyanotic heart disease. Demographics, duration of venovenous extracorporeal membrane oxygenation and outcomes are reported. RESULTS: The length of venovenous extracorporeal membrane oxygenation ranged from 13.5 to 362.5 hours (mean 130 +/- 121 hours). During 10 supports, operations were performed to facilitate weaning from support. In 7 patients, extracorporeal support was weaned during this surgery. Follow-up was obtained in all patients over a period ranging from 4 months to 7 years (mean 39.0 +/- 23.0 months). There were two late deaths due to sepsis 1.4 and 2.5 months after extracorporeal support. CONCLUSIONS: Venovenous extracorporeal membrane oxygenation allows time for the recovery of acute hypoxic insult and resolution of some viral pneumonia processes. Palliative surgical procedures may be safely undertaken during extracorporeal support. Viral pneumonia is a risk for prolonged support. Venovenous extracorporeal membrane oxygenation is useful in these high-risk patients.

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.