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.

Cardiac transplantation in twins with autosomal dominant Emery-Dreifuss muscular dystrophy.

Emery-Dreifuss muscular dystrophy (EDMD) is a neuromuscular disorder resulting in progressive muscle weakness, contractures, and cardiomyopathy. We report successful cardiac transplantation in identical twin brothers with autosomal dominant EDMD, complicated by ventricular arrhythmias and end-stage cardiomyopathy. Early recognition of progressive cardiac disease and subsequent cardiac transplantation are lifesaving in children with EDMD.

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.

The extracardiac Fontan procedure without cardiopulmonary bypass: technique and intermediate-term results.

BACKGROUND: The extracardiac Fontan procedure (ECF) usually requires cardiopulmonary bypass (CPB). In this report, the results and techniques of this procedure without CPB at a single institution are presented. METHODS: Between August 1992 and December 2001, ECF without CPB was achieved in 24 of 44 patients undergoing an ECF. Mean age at surgery was 5.9 +/- 2.9 years, and mean weight was 20.7 +/- 12.6 kg. Diagnoses were tricuspid atresia in 9 patients, single-ventricle with pulmonary outflow tract obstruction in 7, pulmonary atresia/intact septum in 5, and other complex single-ventricle physiology in 3. Initial palliation was by arterial to pulmonary artery shunt in 21 and pulmonary artery banding in 1. A bidirectional cavopulmonary connection was created in 23 patients. A temporary inferior vena caval-to-atrial shunt was used to complete the procedure without CPB. Median graft size was 16 mm (range 14 to 20 mm). RESULTS: There was no early mortality, and 68% of patients were discharged without complications. Complications included persistent cyanosis in 4 patients, persistent pleural effusions in 2 (one chylous), and phrenic nerve injury in 1. Median postoperative hospital stay was 16 days (range 10 to 50) days. At a mean follow-up of 44 +/- 28 months, there was no conduit obstruction. One patient died 11 months postoperatively, and 1 patient received a heart transplant 26 months post-ECF. CONCLUSIONS: At intermediate term follow-up, the ECF without CPB appears to be safe and technically reproducible in selected cases. Ongoing follow-up of these patients is necessary to document the theoretical advantages of avoiding CPB.

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.

Ductus arteriosus aneurysm with community-acquired methicillin-resistant Staphylococcus aureus infection and spontaneous rupture: a potentially fatal quandary.

We present the case of a 6-month-old previously healthy girl who presented with high fever, labored breathing, and an enlarged cardiac silhouette on her chest radiograph. Comprehensive evaluation discovered a ductus arteriosus aneurysm and pericardial effusion with methicillin-resistant Staphylococcus aureus bacteremia. Despite pericardiocentesis and appropriate intravenous antibiotics, there was rapid enlargement of the aneurysm and accumulation of echogenic material within the ductus arteriosus aneurysm. Infected aneurysm rupture was identified during emergency surgery. This infant also had vocal cord paresis, a likely complication of the surgery. The clinical course, diagnosis, and treatment of this patient are discussed. Infection of a ductus arteriosus or an infected ductal arteriosus aneurysm is a rare and potentially fatal clinical entity. In the era of increasing community-acquired methicillin-resistant S aureus infections, this is a diagnosis that requires a high index of suspicion.

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.

Gadolinium-enhanced magnetic resonance angiography of right ventricle to pulmonary artery shunts following Norwood 1 palliation in infants.

The classic Norwood I palliation for the treatment of hypoplastic left-heart disease includes an atrial septectomy, neoaorta creation, and a systemic-to-pulmonary artery shunt. The Norwood I palliation has been modified by the use of a right ventricle-to-pulmonary artery shunt instead of a traditional systemic-to-pulmonary artery shunt. Right ventricle-to-pulmonary artery shunts were non-invasively imaged in four infants using gadolinium-enhanced magnetic resonance angiography. Diagnostic MR angiograms were performed in all infants throughout the length of the shunt and were useful in the detection of shunt patency, stenosis, and aneurysm. Associated surgical conduits and shunts were also evaluated. All findings were verified by subsequent conventional angiography or 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.

Thoracic outlet syndrome in a child--transaxillary resection of anomalous first rib.

Thoracic outlet syndrome (TOS) is extremely rare in children. Only several pediatric cases have been reported, and all presented with neurologic symptoms. The authors report on a 6-year-old boy with an asymptomatic left subclavian artery aneurysm caused by compression of the subclavian artery by an anomalous first rib with poststenotic aneurysmal dilatation. This was successfully managed by transaxillary resection of the anomalous rib. This represents the youngest reported case of TOS and the first description of a vascular manifestation of TOS in the pediatric population. Access to the costoscalene triangle utilizing a transaxillary approach was straightforward and afforded excellent exposure, even in a child.

Magnetic resonance flow analysis of classic and extracardiac Fontan procedures: the seesaw sign.

BACKGROUND: Energy inefficiencies lead to Fontan procedure failures. Our purpose was to quantify energy inefficiencies of various Fontan procedures using flow analysis. MATERIALS AND METHODS: The study included 12 patients with Fontan operations; 7 with classic trans-atrial flow and five extra-cardiac Fontans. Flow analysis was used to determine the systolic and diastolic flow in the superior vena cava and inferior venous circulations (IVC, right atrium or conduit). Retrograde flow fractions were calculated. Inferior venous flow analysis was obtained in 12 patients and superior vena cava in 9 patients. RESULTS: A seesaw pattern (augmented inferior venous flow during diastole and augmented superior vena cava flow in systole) was present in five of seven patients with trans-atrial Fontan procedures, but no patient with an extra-cardiac Fontan procedure. Significant retrograde flow occurred in the superior vena cava in three children with trans-atrial Fontan procedures (retrograde flow fractions of 15-22%). Inferior venous flow occurred predominantly during diastole in five of seven children with trans-atrial Fontan procedures. Retrograde flow occurred in all seven children with the retrograde flow fractions ranging from 9 to 37% (mean 25%). Extracardiac Fontan flow was characterized by continuous balanced flow during systole and diastole. The association of the seesaw pattern with trans-atrial Fontan procedures was statistically significant (p = 0.028). CONCLUSIONS: Classic trans-atrial Fontan procedures are characterized by energy inefficiency creating a seesaw flow pattern of forward and reverse flow. Extra-cardiac Fontan procedures are more energy efficient. Magnetic resonance imaging is useful in detecting flow inefficiencies in patients palliated by the Fontan procedure.

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.