Echocardiographic features defining right dominant unbalanced atrioventricular septal defect: a multi-institutional Congenital Heart Surgeons' Society study.

BACKGROUND: Definition and management of right dominant unbalanced atrioventricular septal defect (AVSD) remains challenging because unbalance entails a spectrum of left heart hypoplasia. Previous work has highlighted atrioventricular valve (AVV) index as a reasonable defining echocardiographic measure. We sought to assess which additional echocardiographic features might provide further characterization. METHODS AND RESULTS: From a multi-institutional cohort of complete AVSD, 52 preoperative echocardiograms of patients with presumed right dominant unbalanced AVSD (based on AVV index) and 60 randomly selected preoperative echocardiograms from patients with presumed balanced AVSD were reviewed. Cluster analysis of echocardiographic variables was used to group patients with similar features. Discriminant function analysis was used to explore which variables differentiated these groups. Three groups were identified from the cluster analysis. Echocardiographic variables that differentiated these groups were right ventricle:left ventricle inflow angle, LV width/LV length, left AVV color diameter at smallest inflow, left AVV color diameter at annulus, right AVV overriding left atrium, and LV width. Based on procedures and outcomes, 1 group likely represented balanced patients, whereas 2 groups with similar outcomes likely represented unbalanced patients. The dominant differentiating echocardiographic variable between the 3 cluster groups was the right ventricle:LV inflow angle (partial R²=0.86), defined as the angle between the base of the right ventricle and LV free wall, using the crest of the ventricular septum as apex of the angle. CONCLUSIONS: The angle of right ventricle/LV inflow and other surrogates of inflow may be important defining echocardiographic measures of right dominant unbalanced AVSD, although confirmation is needed.

Unbalanced atrioventricular septal defect: defining the limits of biventricular repair.

Unbalanced atrioventricular septal defect (uAVSD) is a challenging lesion with suboptimal outcomes in the current era. Severe forms of uAVSD mandate univentricular repair with well-documented outcomes. Determining the feasibility of biventricular repair (BVR) in patients with moderate forms of uAVSD is difficult. Ventricular hypoplasia has traditionally formed the cornerstone of defining uAVSD. However, malalignment of the atrioventricular junction and related derangements of the anatomy and physiology of the atrioventricular inflow play a central role in establishing and sustaining a biventricular end state. Atrioventricular valve index, left ventricular inflow index, and right ventricle/left ventricle inflow angle are important recently described measures of inflow physiology. Additional patient anatomic and physiologic factors that impact BVR feasibility undoubtedly exist. A recently launched Congenital Heart Surgeons Society prospective inception cohort study will address these and other issues that impair our ability to predict BVR feasibility in uAVSD.

Echocardiographic definition and surgical decision-making in unbalanced atrioventricular septal defect: a Congenital Heart Surgeons' Society multiinstitutional study.

BACKGROUND: Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes. METHODS AND RESULTS: Diagnostic echocardiograms and hospital records of 356 infants with complete AVSD at 4 Congenital Heart Surgeons' Society (CHSS) institutions (2000-2006) were reviewed and AVVI measured (n=315). Patients were classified as unbalanced if AVVI≤0.4 (right dominant) or ≥0.6 (left dominant). Surgical strategy and outcomes were examined across the range of AVVI. Competing risks analysis until the time of commitment to a surgical strategy examined 4 end states: biventricular repair (BVR), univentricular repair (UVR), pulmonary artery banding (PAB), and death before surgery. A prediction nomogram for surgical strategy based on AVVI was developed. The majority of patients had balanced AVSD (0.4

Unbalanced atrioventricular septal defect: definition and decision making.

Unbalanced atrioventricular septal defect is an uncommon lesion with widely varying anatomic manifestations. When unbalance is severe, diagnosis and treatment is straightforward, directed toward single-ventricle palliation. Milder forms, however, pose a challenge to current diagnostic and therapeutic approaches. The transition from anatomies that are capable of sustaining biventricular physiology to those that cannot is obscure, resulting in uneven application of surgical strategy and excess mortality. Imprecise assessments of ventricular competence have dominated clinical decision making in this regard. Malalignment of the atrioventricular junction and its attendant derangement of inflow physiology is a critical factor in determining the feasibility of biventricular repair in the setting of unbalanced atrioventricular septal defect. The atrioventricular valve index accurately identifies unbalanced atrioventricular septal defect and also brings into focus a zone of transition from anatomies that can support a biventricular end state and those that cannot.

Dosage and effectiveness of intrapleural doxycycline for pediatric postcardiotomy pleural effusions.

STUDY OBJECTIVE: To determine the effectiveness of intrapleural doxycycline for the treatment of postcardiotomy pleural effusions in pediatric patients. DESIGN: Retrospective case series. SETTING: Intensive care unit in a pediatric tertiary care center. PATIENTS: Sequential sample of 12 pediatric patients who underwent cardiotomy for congenital heart disease and received doxycycline pleurodesis for persistent pleural effusion that lasted more than 7 days between December 21, 2001, and May 23, 2005. MEASUREMENTS AND MAIN RESULTS: Mean age of the patients was 1 year (range 2 wks-2.5 yrs). Eighteen courses of doxycycline were administered among the 12 patients. An average dose of 19.1 mg/kg/dose of parenteral doxycycline was diluted in normal saline to a final syringe concentration of 2-8 mg/ml and injected through a chest tube. The patient was rotated according to a protocol. The doxycycline dose remained in the pleural space for approximately 6 hours before being drained under suction. Treatment success was defined as achievement of 0-ml/hour chest tube output after a doxycycline dose. The overall treatment success rate was 94% (17 of 18 courses). The mean times from dosing to treatment success and chest tube removal were 76 hours (range < 1 to 140 hrs) and 130 hours (range 8-453 hrs), respectively. Seventy-two percent of the courses (13 of 18) achieved treatment success within 96 hours and chest tube removal within 168 hours after dosing. Doxycycline concentration did not appear to be related to treatment success. Chest pain was the most common adverse effect. CONCLUSION: Intrapleural doxycycline infusion is effective for postcardiotomy pleural effusion in pediatric patients with persistent chest tube drainage lasting more than 7 days.

The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: early and midterm outcomes.

OBJECTIVE: Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes. METHODS: From September 1992 to April 2005, 285 patients, median age 4.5 years (1.4-44 years), median weight 16 kg (9.4-94 kg), underwent a primary extracardiac conduit Fontan procedure. Early and late outcomes were analyzed for the entire cohort and for 2 patient groups depending on whether an oxygenator was used in the bypass circuit (166 patients; 58%) or not (119 patients; 42%). RESULTS: Early failure (including death and takedown) occurred in 7 patients (2.5%). Prevalence of new early postoperative sinus node dysfunction necessitating a permanent pacemaker was 0.4%, and that of new tachyarrhythmias necessitating discharge home on a regimen of antiarrhythmia medications was 2.5%. Ten-year actuarial freedom from Fontan failure, new sinus node dysfunction necessitating a permanent pacemaker, and reoperation for conduit thrombosis or stenosis was 90%, 96%, and 98%, respectively. Fenestration rate was lower (P = .001) in the no-oxygenator group (8%) than in the oxygenator group (25%). Patients in the no-oxygenator group had lower intraoperative Fontan pressure (12.0 +/- 2.3 vs 13.5 +/- 2.4 mm Hg, P < .001), common atrial pressure (4.6 +/- 1.8 vs 5.3 +/- 1.8 mm Hg, P = .003), and transpulmonary gradient (7.5 +/- 2.1 vs 8.3 +/- 2.2 mm Hg, P = .013) than did the oxygenator group. CONCLUSIONS: The extracardiac conduit Fontan operation coupled with minimal use of extracorporeal circulation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, and minimal early and late rhythm disturbance.

Accuracy of coronary artery anatomy using two-dimensional echocardiography in d-transposition of great arteries using a two-reviewer method.

We evaluated echocardiographic accuracy for defining coronary artery course in d-transposition of great arteries and the impact of a 2-reviewer method on this accuracy. The echocardiogram reports of 108 patients with d-transposition of great arteries were reviewed for coronary anatomy and compared with the operative report. In method 1, from January 1995 to December 1997, a single reader performed the echocardiogram. In method 2, from January 1998 to December 2000, 2 readers scanned individually and a consensus diagnosis was made. Comparing methods 1 and 2, the sensitivity of the echocardiogram to detect variants in coronary anatomy was 68% versus 86%, and negative predictive value was 72% versus 91%. Using a 2-reviewer method improved the accuracy of echocardiographic diagnosis of coronary anatomy with d-transposition of great arteries, aiding in risk assessment and treatment of the patient preoperatively. This study also illustrates that echocardiographic accuracy may be lower in an institution with a surgical volume more representative of the usual pediatric cardiothoracic surgical center.

Neurodevelopmental outcome of infants supported with extracorporeal membrane oxygenation after cardiac surgery.

OBJECTIVES: To evaluate the long-term neurodevelopmental outcome of infants who underwent cardiac surgery and required extracorporeal membrane oxygenation (ECMO) support, and to examine variables that predict death or disability in these patients. METHODS: We studied all infants who had congenital heart disease and were supported postoperatively with ECMO from 1990 to 2001 at our institution (n = 53). Medical records were reviewed retrospectively to obtain clinical variables. Neurologic and age-appropriate developmental examinations occurred at ages 1, 1.5, 2.5, and 4.5 years. Median age at follow-up was 55 months (9-101). Cognitive outcome was defined as suspect when scores were between 1 and 2 SD below the mean for age and abnormal when scores were >2 SD below mean for age. Neuromotor outcome was defined as suspect when the patient manifested clumsiness, tremor, or mild tone and reflex changes without functional limitations, and abnormal when there were functional limitations. RESULTS: In-hospital survival was 17 (32%) of 53. Of survivors, 14 (88%) of 16 are living and 1 patient was lost to follow-up. Of the 53 patients, 7 survived completely intact (13%). Seven (50%) of 14 patients had a normal cognitive outcome, 3 (21%) had a suspect cognitive outcome, and 4 (29%) were abnormal. Ten (72%) of 14 patients had a normal neuromotor outcome, 1 (7%) patient had a suspect neuromotor outcome, and 3 (21%) were abnormal. No survivor with an aortic cross-clamp time >40 minutes had a normal cognitive outcome. Nonsurvivors were more likely than survivors to have had cardiac arrest as an indication for ECMO (31% vs 6%), to have had a longer aortic cross-clamp time (mean 73 minutes vs 32 minutes), and to have required continuous arteriovenous hemofiltration (78% vs 35%). The age and weight at cannulation, gender, cardiac diagnosis, interval from surgery to ECMO, cardiopulmonary bypass time, diagnosis of sepsis or mediastinitis, and duration of ECMO were not significantly associated with survival. CONCLUSIONS: Although mortality was 68% in infants who had congenital heart disease and were treated with ECMO postoperatively, of those who survive to hospital discharge, 75% have a normal neuromotor outcome and 50% have a normal cognitive outcome. These high rates of mortality and disability suggest that increased attention be paid to neuroprotection in these complex disorders.