Lobectomy with ECMO Support in an Infant Who Developed Pulmonary Interstitial Emphysema Following Repair of Hypoplastic Aortic Arch.

Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Incidence and factors influencing the spontaneous closure of Fontan fenestration.

INTRODUCTION: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long-term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration. METHODS: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at our center. Preoperative, intraoperative and postoperative variables including the status of the Fontan fenestration were extracted and analyzed. RESULTS: Of 67 patients included in the study, 15 (22%) had spontaneous closure of the fenestration. Of the remaining 52 patients, 11 (20%) had procedural closure of this fenestration (10 via cardiac catheterization and 1 via surgery) at a median duration of 3 months after the Fontan operation. Patients with higher preoperative pulmonary vascular resistance and a history of postoperative systemic venous thromboembolism had higher likelihood of having persistence of the fenestration with P value of .045 and .037, respectively. CONCLUSIONS: The rate of spontaneous closure of the Fontan fenestration was 22% in our study. Elevated preoperative pulmonary vascular resistance and history of systemic venous thromboembolism are predictive of persistent Fontan fenestration.

Venous bullet embolus to the left pulmonary artery.

We report a venous bullet embolus into the left pulmonary artery following a gunshot wound to the right chest. The diagnosis and surgical management of migrating venous bullet emboli are reviewed.

Transmyocardial migration of a temporary epicardial pacing wire: a pediatric case report.

Transmyocardial migration of a retained temporary epicardial pacing wire has been rarely reported in adult patients after heart surgery. We present the case of a child in whom a temporary epicardial pacing wire was discovered incidentally in the right ventricular outflow tract one year after surgical repair of congenital heart disease. The pacing wire was subsequently extracted using the snare method during cardiac catheterization. Clinicians caring for patients after congenital heart surgery should be aware of this uncommon though potentially life-threatening complication.

Chest wall reconstruction in a pediatric patient with ectopia cordis.

Ectopia cordis is defined as a congenital malposition of the heart outside of the thoracic cavity. It is a rare condition, and complete ectopia cordis can be a fatal condition. Successful surgical reconstruction of this defect has been reported but is uncommon. The general approach to reconstructing the chest wall involves repositioning the heart and providing adequate coverage of the chest wall defect. We describe our experience with a patient who had complete thoracic ectopia cordis treated with staged chest wall reconstruction. The first stage involved temporary closure with synthetic material, and the second stage involved definitive reconstruction with autologous bone and cartilage grafts supported with plates. The patient has been active and without complaints since the second stage and is awaiting tracheal decannulation. There have been a few descriptions of how to approach chest wall reconstruction in patients with ectopia cordis. The 2 stage method described can be considered to repair the chest wall defect in complete thoracic ectopia cordis.

Supravalvar mitral ring with complete atrioventricular septal defect: a case report and three-dimensional echocardiography evaluation.

Supravalvar mitral ring (SVMR) is a rare cause of congenital mitral stenosis. It can occur in isolation but often coexists with other cardiac anomalies, such as a ventricular septal defect or left-sided obstructive lesions. Conversely, a complete atrioventricular septal defect (AVSD) is a much more common anomaly. An AVSD may be associated with other major cardiac defects, such as tetralogy of Fallot, transposition of the great arteries, or double-outlet right ventricle. The authors describe what they believe is the first case of SVMR and complete AVSD occurring together; the SVMR was diagnosed by two-dimensional echocardiography, and its morphology could be more accurately delineate using three-dimensional echocardiography.

Cerebral and somatic oxygen saturation decrease after delayed sternal closure in children after cardiac surgery.

OBJECTIVES: Delayed sternal closure after pediatric cardiac surgery can temporarily impair cardiac output. Cerebral and somatic regional oxygen saturation measured by using near-infrared spectroscopy (NIRS) have been used as potential surrogates of cerebral and somatic mixed venous oxygen saturation. We hypothesized that cerebral and somatic regional oxygen saturation correlate with indicators of hemodynamic compromise after delayed sternal closure in children undergoing cardiac surgery. METHODS: We studied 36 postoperative children (median age, 10 days; range, 1-510 days) undergoing delayed sternal closure 3.7 +/- 2 days after cardiac surgery. Twenty-five had biventricular physiology, whereas 11 had single-ventricle physiology. Cerebral regional oxygen saturation, somatic regional oxygen saturation, and other physiologic parameters (hemodynamic data, respiratory data, blood gas analysis, lactate levels, and inotrope scores) were analyzed at 16 different time points 24 hours before and after sternal closure. One-way analysis of variance and the paired t test were used for statistical comparisons. RESULTS: Cerebral and somatic regional oxygen saturation decreased after delayed sternal closure compared with preclosure levels (P = .02 and P = .01, respectively). Higher heart rate (P = .03), lactate levels (P = .02), and left atrial pressure (P = .001) were also noted, suggesting mild hemodynamic compromise. Arterial pressure and inotrope score were unchanged. Somatic regional oxygen saturation returned to preclosure levels earlier in the biventricular group than in the single-ventricle group, whereas cerebral regional oxygen saturation remained decreased after sternal closure with no evidence of return to preclosure levels during the observation period. Oxygen saturation, Pao(2), and Paco(2) levels were unaffected by sternal closure, although greater positive-pressure ventilation was required (P < .01), suggesting reduced lung compliance. CONCLUSION: Cerebral and somatic regional oxygen saturation decrease after delayed sternal closure in children recovering from congenital cardiac surgery. These indices are in agreement with other physiologic indicators of cardiac performance, suggesting mild and transient hemodynamic compromise after sternal closure. Cerebral and somatic regional oxygen saturation monitoring might be a useful adjunct during delayed sternal closure.

Analysis of regional congenital cardiac surgical outcomes in Florida using the Society of Thoracic Surgeons Congenital Heart Surgery Database.

BACKGROUND: Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report. METHODS: Eight centres in Florida provide services for congenital cardiac surgery. The Children's Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery. RESULTS: The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity. CONCLUSIONS: A regional consortium of congenital heart surgery centres in Florida under the framework of the Children's Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.

Initial experience with the TandemHeart circulatory support system in children.

Options for mechanical ventricular assistance in pediatric patients are limited. Extracorporeal membrane oxygenation is used in most cases for short-term support. The TandemHeart circulatory support system is an established device that is used in adult patients to provide short-term ventricular support. In this article, we report three children in whom a TandemHeart ventricular assist device was used for right ventricular support, two after heart transplantation and another for failed Fontan physiology. Herein, we report the novel application of this technology to pediatric patients, and we discuss the lessons learned from its utilization.

K(ATP) channel opener protects neonatal rabbit heart better than St. Thomas' solution.

OBJECTIVES: Myocardial protection with ATP-sensitive potassium channel (K(ATP) channel) openers is as effective as St. Thomas' cardioplegia (StTCP) in adult rabbit hearts. This study compares the effectiveness of the K(ATP) channel opener pinacidil to StTCP in protecting neonatal rabbit hearts exposed to global ischemia. METHODS: Seventeen neonatal rabbit hearts (7-9 days old) perfused with Krebs-Henseleit buffer (KHB) on a Langendorff apparatus underwent 90 min of normothermic ischemia. Six (ischemia control) received no pretreatment before or during ischemia. Six others (pinacidil) received a 3-min infusion of 50 microM pinacidil in KHB without StTCP at the onset of ischemia. Five others (StTCP) received a 3-min infusion of StTCP at the onset of ischemia. After 60 min of KHB reperfusion, recovery of left ventricular (LV) performance and coronary flow (CF) were measured and compared to preischemia. A paired t test was used for comparison between drug-treated and untreated groups. RESULTS: Pinacidil-treated hearts had significantly better recovery of left ventricular developed pressure (47 +/- 3.8 mmHg vs 32 +/- 2.5 mmHg, P < 0.05), contractility (+dP/dt(max); 885.4 +/- 74 mmHg vs 643.7 +/- 65 mmHg, P < 0.05), left ventricular end diastolic pressure (10.5 +/- 0.9 mmHg vs 17.4 +/- 1.2 mmHg P < 0.05), compliance (-dP/dt(max); 994.2 +/- 86 mmHg vs 673.6 +/- 69 mmHg, P < 0.05), and CF (5.9 +/- 0.4 ml/min vs 4.2 +/- 0.2 ml/min, P < 0.05) compared to ischemic control. StTCP only improved the recovery of -dP/dt(max) (877.4 +/- 73 mmHg/s vs 673.6 +/- 69 mmHg/s, P < 0.05) and CF (5.7 +/- 0.3 ml/min vs 4.2 +/- 0.2 ml/min, P < 0.05) compared to control. CONCLUSIONS: Pinacidil pretreatment provided superior recovery of systolic performance compared to St. Thomas' cardioplegia solution in neonatal hearts. Myocardial protection by pretreatment with the K(ATP) channel opener pinacidil may be a new strategy for myocardial protection during pediatric cardiac surgery.

Diazoxide protects the rabbit heart following cardioplegic ischemia.

K(ATP) channels are present in sarcolemmal and mitochondrial membranes. This study tests the hypothesis that opening mitochondrial K(ATP) channels with Diazoxide (DZ) improves tolerance to cardioplegic ischemia during surgery. Twenty-two rabbit hearts were perfused with Krebs-Henseleit buffer (KHB) on a Langendorff apparatus and underwent 50 min of 37 degrees C global ischemia with St Thomas' cardioplegia (STCP). Hearts were divided into three groups. Ten (control) received no pretreatment. Seven (DZ) received 10 min of 30 microM DZ, a selective mitochondrial K(ATP) opener, in KHB before arrest with STCP containing 30 microM DZ. Five (5-HD + DZ) received 10 min of 100 microM sodium 5-hydroxydecanoate (5-HD), a selective mitochondrial K(ATP) channel blocker, followed by 10 min of 30 microM DZ and 100 microM 5-HD in KHB before arrest with STCP + 30 microM DZ + 100 microM 5-HD. LV developed pressure (LVDP), dP/dt and coronary flow (CF) were measured after 60 min of reperfusion. Diazoxide pretreatment significantly improved the recovery of LV function and coronary flow compared to control (LVDP: 49 +/- 5* vs. 31 +/- 4; +dP/dtmax 927 +/- 93 vs. 507 +/- 85 mmHg/sec*; CF 33 +/- 4 vs. 22 +/- 2 ml/min, *p < 0.05). Mitochondria K(ATP) channel blockade with 5-HD prevented DZ's salutary effect on the recovery of LV and vascular function. Diazoxide pretreatment protects the rabbit heart during cardioplegic ischemia by opening mitochondrial K(ATP) channels. Opening mitochondrial K(ATP) channels may be a new strategy for improving myocardial protection during cardiac surgery.