Joseph N. Graziano

Decompression of the left atrium during extracorporeal membrane oxygenation using a transseptal cannula incorporated into the circuit.

Objectives:When extracorporeal membrane oxygenation (ECMO) is used in the setting of severe myocardial dysfunction, left ventricular end-diastolic and left atrial pressure can rise to extremely high levels. Decompression of the left atrium in this setting is essential for resolution of pulmonary edema and recovery of left ventricular function. We sought to evaluate whether adequate left atrial decompression can be achieved via percutaneous placement of a transseptal left atrial drain incorporated in the ECMO venous circuit. Design:Retrospective case series. Setting:Tertiary care center pediatric intensive care unit and cardiac catheterization laboratory. Patients:Seven patients (age 8 months to 28 yrs) with cardiac failure on venoarterial ECMO with left atrial hypertension. Interventions:All patients underwent left atrial decompression with transseptal puncture and placement of a drain (8- to 15-Fr) incorporated into the ECMO venous circuit. Catheterization and ECMO records and echocardiograms were reviewed, as were the clinical course and outcome for each patient. Measurements and Main Results:The median time from ECMO cannulation to left atrial decompression was 11 hrs. Average initial left atrial pressure was 31 mm Hg. Successful drain placement was achieved in seven patients with no major procedural complications. Echocardiographic improvement in left atrial dilation was achieved in five patients (71%). Inability to decompress the left atrium was fatal in two patients. Four patients were decannulated (57%), and three survived to hospital discharge (43%). Larger sheath size and higher flow rate correlated with a greater likelihood of success. Conclusions:Adequate decompression of the left atrium can be achieved by transseptal placement of a left atrial drain incorporated into the ECMO circuit. This technique represents a reasonable alternative to blade or balloon atrial septostomy for patients requiring left atrial decompression.

The Influence of a Restrictive Atrial Septal Defect on Pulmonary Vascular Morphology in Patients with Hypoplastic Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.

Adrenocortical response in infants undergoing cardiac surgery with cardiopulmonary bypass and circulatory arrest.

Objective: To detail changes in adrenocorticotropic hormone (ACTH), cortisol, and aldosterone levels following cardiac surgery and to test the hypothesis that postcardiotomy infants requiring excessively high-dose vasopressor support will demonstrate adrenal insufficiency which will be proportional to cardiopulmonary bypass (CPB)/circulatory arrest times and vasopressor requirements. Design: Prospective observational pilot study. Setting: A tertiary care pediatric cardiac intensive care unit. Patients: Prospectively enrolled infants were divided into three subgroups: CPB, CPB with deep hypothermic circulatory arrest (DHCA), and control subjects. Interventions: None. Measurements and Main Results: A representative patient sample from each surgical group underwent preoperative synthetic ACTH testing. Postoperative serum samples for cortisol, ACTH, and inotrope score (IS) were collected at discrete intervals over 48 hrs along with patient demographics, surgical procedure, and CPB/DHCA times. Fifty-eight patients were classified by subgroup: 31 CPB, 22 DHCA, and 5 controls. Ten patients with DHCA, analyzed separately, received intraoperative steroids. Tested patients demonstrated preoperative adrenal competence. Cortisol peaked within 2 hrs of surgery without differences among groups. ACTH inversely correlated with bypass time in patients with DHCA (p = .03) but not with circulatory arrest time. Peak cortisol level did not correlate with simultaneous IS. Although not noted in any DHCA-steroid patients, nine patients had increased ACTH/cortisol ratios in association with elevated ISs suggesting inadequate adrenal responsiveness to endogenous ACTH. Conclusions: The majority of infants with congenital heart disease and intact hypothalamic-pituitary-adrenal axes demonstrated an appropriate adrenocortical stress response to cardiac surgery. Peak serum cortisol was unrelated to CPB/DHCA time and did not predict the level of inotrope support. However, a subset of patients with elevated ACTH/cortisol ratios seemed to have a clinical status consistent with adrenal insufficiency and may be a target group for early postoperative steroid therapy.

Catheter‐based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective

Infants with hypoplastic left heart syndrome (HLHS) and restrictive or intact atrial septum (rAS) present with cyanosis, pulmonary edema, and are critically ill. A previous report from our institution on emergent Norwood for HLHS with rAS showed 10% survival. We hypothesized that transcatheter left atrial (LA) decompression in HLHS with rAS would safely and effectively relieve LA hypertension, improve oxygenation, and improve Norwood survival. Between 1996 and 2004, 30 patients with HLHS and rAS underwent cardiac catheterization for pre‐Norwood intervention. Twenty‐eight atrial septostomies were performed: 23 static balloon dilations, 4 Rashkind septostomies, and 1 intra‐atrial stent. Two procedures were aborted due to perforation (n = 1) or inability to enter the LA (n = 1). Eight total patients required surgical septectomy, for a failure rate of 27%. There were no catheter‐related mortalities, although two patients died within 36 hr of the procedure after surgical septectomy. Major complications occurred in three patients (10%)—atrial perforations requiring intervention. Mean atrial septal defect gradient fell from 16.7 ± 4.9 to 6.3 ± 3.4 mm Hg (P < 0.001; n = 18). Mean LA pressure dropped from 21.8 ± 5.5 to 13.1 ± 6.5 mm Hg (P < 0.001; n = 16). Mean PaO2 rose from 29.5 ± 9.1 to 36.5 ± 5.1 torr (P < 0.001; n = 23). Seventeen of 30 patients (57%) survived to discharge from Norwood. Thirteen have undergone hemi‐Fontan and nine Fontan. Sixteen of 22 successful decompressions (73%) survived to discharge. Transcatheter decompression of the LA for patients with HLHS and rAS can be performed safely, reduces the transatrial gradient, and improves oxygenation. Catheter intervention improves survival compared to historical controls undergoing emergent Norwood.

Transcatheter occlusion of aortopulmonary shunts during single-ventricle surgical palliation.

Development of aortopulmonary collaterals during the course of surgical palliation for single‐ventricular anatomy has been linked to adverse outcomes following Fontan palliation. We investigated the hemodynamic significance of aortopulmonary collaterals during presurgical cardiac catheterization of patients with single‐ventricle surgically palliated anatomy. Thermal indicator dilution studies were performed to determine degree of shunt. A total of 52 patients were studied and the data were analyzed. Measurements by thermal indicator dilution correlated significantly with qualitative angiographic grading of aortopulmonary collaterals. However, the hemodynamic significance of these aortopulmonary collaterals, as measured by thermal indicator dilution, did not correlate with postoperative outcome variables. This study demonstrated that thermal indicator dilution measurement holds promise for hemodynamically quantifying the significance of aortopulmonary shunts in surgically palliated single‐ventricular patients. However, the degree of recirculation from aortopulmonary collaterals does not correlate with outcome after Fontan surgery in this patient cohort.

Thrombosis in the Intensive Care Unit: Etiology, Diagnosis, Management, and Prevention in Adults and Children

Venous thromboembolism, a well-recognized complication in postoperative patients, is emerging as a frequent complication in critically ill patients in intensive care units. Diagnosis can be particularly difficult in such patients because underlying systemic illnesses may mask common presenting signs and symptoms. Although numerous independent risk factors have been identified, the critical role of both central venous catheters and prothrombotic disorders as significant risk factors is a common theme in the pediatric and adult literature. Various diagnostic tests exist, with venography remaining the gold standard and newer, less invasive methods such as ultrasonography and impedance plethysmography becoming increasingly popular. Standard unfractionated heparin remains the mainstay of therapy and prophylaxis, although the use of low molecular weight heparins is becoming more commonplace. Thrombolytic therapy continues to be reserved for severe, life-threatening, acute thrombosis. In this article, we review the common risk factors, diagnostic modalities, and treatment options for venous thromboembolism in critically ill adult and pediatric patients.

Late Migration of a Sideris Buttoned Device for Occlusion of Atrial Septal Defect

We describe a 17‐year‐old patient with an atrial septal defect who underwent device closure with a second generation Sideris buttoned device at 4 years of age. She presented 13 years after the procedure with the acute onset of chest discomfort, at which time a chest radiograph showed migration of the wire of the right atrial counter‐occluder to the lateral aspect of the right atrial wall, with an associated right atrial perforation. The patient underwent uneventful surgical device retrieval and atrial septal defect closure. Late migration of a portion of the Sideris buttoned device can occur, suggesting the need for continued follow up, and a high index of suspicion for device failure should a patient become symptomatic.

Amplatzer Closure of Atrial Septal Defect and da Vinci Robot-Assisted Repair of Vascular Ring

Technology for minimally invasive approaches to congenital heart disease is a rapidly evolving field. This case report reviews a novel approach to combining two of the newer technologies available to treat a pediatric patient with an atrial septal defect (ASD) and a vascular ring. This report is the first to describe the use of the da Vinci surgical system to assist in a thoracoscopic procedure for a pediatric patient. The da Vinci assisted division of the vascular ring, joined with an Amplatzer closure of the ASD, demonstrates how maximum benefit can be obtained for patients by combining emerging technologies.

Acute Myocardial Ischemia in a Healthy Male Child: An Atypical Presentation of Acute Epstein-Barr Virus Infection:

to pediatric emergency departments.1 Despite the fact that chest pain often evokes anxiety in patients and parents, it rarely represents significant pathosis. Furthermore, amidst the vast differential diagnosis of childhood chest pain, cardiac causes are rare and represent only 1% to 4% of cases. Nonetheless, it is important to consider the possible cardiac causes of chest pain when evaluating a child with this complaint because of their potential severity. We present a 14-year-old male who presented with chest pain who was found to have cardiac enzyme elevations and electrocardiographic evidence of ischemia associated with acute Epstein-Barr virus infection. Patient Report