Puja Banka

Interstudy variability in cardiac magnetic resonance imaging measurements of ventricular volume, mass, and ejection fraction in repaired tetralogy of fallot: A prospective observational study

To assess the interstudy variability of cardiac magnetic resonance imaging (CMR) parameters of ventricular size and function in repaired tetralogy of Fallot (TOF).

Practice variability and outcomes of coil embolization of aortopulmonary collaterals before fontan completion: A report from the Pediatric Heart Network Fontan Cross-Sectional Study

BACKGROUND The practice of coiling aortopulmonary collaterals (APCs) before Fontan completion is controversial, and published data are limited. We sought to compare outcomes in subjects with and without pre-Fontan coil embolization of APCs using the Pediatric Heart Network Fontan Cross-Sectional Study database which enrolled survivors of prior Fontan palliation. METHODS We compared hospital length of stay after Fontan in 80 subjects who underwent APC coiling with 459 subjects who did not. Secondary outcomes included post-Fontan complications and assessment of health status and ventricular performance at cross-sectional evaluation (mean 8.6 ± 3.4 years after Fontan). RESULTS Centers varied markedly in frequency of pre-Fontan APC coiling (range 0%-30% of subjects, P < .001). The coil group was older at Fontan (P = .004) and more likely to have single right ventricular morphology (P = .054) and pre-Fontan atrioventricular valve regurgitation (P = .03). The coil group underwent Fontan surgery more recently (P < .001), was more likely to have a prior superior cavopulmonary anastomosis (P < .001), and more likely to undergo extracardiac Fontan connection (P < .001) and surgical fenestration (P < .001). In multivariable analyses, APC coiling was not associated with length of stay (hazard ratio for remaining in-hospital 0.91, 95% CI 0.70-1.18, P = .48) or postoperative complications, except more post-Fontan catheter interventions (hazard ratio 1.74, 95% CI 1.04-2.91, P = .03), primarily additional APC coils. The groups had similar outcomes at cross-sectional evaluation. CONCLUSION Management of APCs before Fontan shows marked practice variation. We did not find an association between pre-Fontan coiling of APCs and shorter postoperative hospital stay or with better late outcomes. Prospective studies of this practice are needed.

Relation of Systemic-to-Pulmonary Artery Collateral Flow in Single Ventricle Physiology to Palliative Stage and Clinical Status

Systemic-to-pulmonary collateral arteries (SPCs) are common in patients with single-ventricle physiology, but their impact on clinical outcomes is unclear. The aim of this study was to use retrospective cardiac magnetic resonance data to determine the relation between SPC flow and palliative stage and clinical status in single-ventricle physiology. Of 116 patients, 78 were after Fontan operation (median age 19 years) and 38 were at an earlier palliative stage (median age 2 years). SPC flow was quantified as aortic flow minus total caval flow or total pulmonary vein flow minus total branch pulmonary artery flow. Median SPC flow/body surface area (BSA) was higher in the pre-Fontan group (1.06 vs 0.43 L/min/m(2), p <0.0001) and decreased nonlinearly with increasing age after the Fontan operation (r(2) = 0.17, p <0.0001). In the Fontan group, patients in the highest quartile of SPC flow had larger ventricular end-diastolic volume/BSA (p <0.0001) and were older at the time of Fontan surgery (p = 0.04), but SPC flow/BSA was not associated with heart failure symptoms, atrial or ventricular arrhythmias, atrioventricular valve regurgitation, the ventricular ejection fraction, or peak oxygen consumption. In multivariate analysis of all patients (n = 116), higher SPC flow was independently associated with pre-Fontan status, unilateral branch pulmonary artery stenosis, a diagnosis of hypoplastic left-heart syndrome, and previous catheter occlusion of SPCs (model r(2) = 0.37, p <0.0001). In conclusion, in this cross-sectional study of single-ventricle patients, BSA-adjusted SPC flow was highest in pre-Fontan patients and decreased after the Fontan operation with minimal clinical correlates aside from ventricular dilation.

Biventricular conversion after single ventricle palliation in patients with small left heart structures: short-term outcomes.

BACKGROUND Patients with borderline small left heart (LH) structures who initially undergo single ventricle palliation (SVP) may eventually become candidates for biventricular conversion (BC). The purpose of this study was to describe our surgical experience with BC in patients with small LH. METHODS We reviewed our institutions records for patients who underwent BC after an initial SVP between 1995 and 2012. Patients underwent an aortopulmonary amalgamation procedure as a part of their initial palliation. Data on imaging, BC operative details, and re-interventions after BC were collected. RESULTS Twenty-eight patients underwent BC. Twenty patients had hypoplastic left heart syndrome (HLHS), 7 patients had unbalanced common atrioventricular canal (uCAVC), and 1 had interrupted aortic arch with VSD. Stage of palliation at BC was stage 1 in 6 patients (21.4%), bidirectional Glenn in 19 (67.9%), and Fontan in 3 (10.7%). Prior to BC, the median left ventricular end-diastolic volume (LVEDV) by echocardiography was 58.1 mL/m(2) in the HLHS group and 28.1 mL/m(2) in the uCAVC group. After BC, the LVEDV increased to 91.3 mL/m(2) in the HLHS group and 58.5 mL/m(2) in the uCAVC group (p < 0.05 compared with pre-BC in both groups). Right ventricular pressure was less than half systemic in 8 patients (53.3% of those measured). Seventeen patients (61%) have required either catheter-based or surgical re-intervention. Twenty-five patients (89.3%) were alive at a median follow-up of 2.6 years. CONCLUSIONS Biventricular conversion can be applied to patients with HLHS and uCAVC and borderline LH with acceptable short-term results. Left heart size increases after BC, but follow-up for potential left atrial hypertension is warranted.

Natural history of exercise function in patients with Ebstein anomaly: A serial study

BACKGROUND The clinical manifestations of Ebstein anomaly (EA) vary greatly; criteria for surgical intervention remain undefined. Decisions regarding surgical intervention in asymptomatic/mildly symptomatic patients would be helpfully informed by a detailed, quantitative understanding of the natural history of exercise intolerance in these patients. However, past studies of exercise function in EA have been of a cross-sectional, rather than a serial, nature. We, therefore, analyzed serial cardiopulmonary exercise (CPX) tests from patients with unrepaired EA to better appreciate the natural history of their exercise function. METHODS All patients with EA who had had at least 2 CPX tests, separated by at least 6 months, between November 2002 and October 2010 were identified. Patients with prior tricuspid valve surgery were excluded from the study. RESULTS Cardiopulmonary exercise data from 23 patients (64 CPX tests; 2.8 ± 1.0 tests/patient) were analyzed. The median time interval between the first and last CPX tests was 3.3 (range, 0.6-7.3) years. The percentage of predicted peak oxygen consumption declined slowly (1.87 ± 8.04 percentage points/y) during the follow-up period. The decline was more pronounced (3.04 ± 6.78 percentage points/y) in patients <18 years old. On multivariate modeling, only the change in oxygen pulse at peak exercise (a surrogate for forward stroke volume) and the change in peak heart rate over time emerged as statistically significant correlates of the change in percentage of predicted peak oxygen consumption. CONCLUSION The exercise function of patients with EA tends to deteriorate over time. This deterioration appears to be related to a progressive decline in their ability to augment their forward stroke volume and heart rate during exercise.

Right Aortic Arch With Aberrant Left Innominate Artery A Rare Vascular Ring

Two children referred for cardiac imaging were found to have an unusual form of a vascular ring. The first patient was an asymptomatic 10-year-old girl with a membranous ventricular septal defect, prolapse of the right coronary aortic cusp, and mild aortic regurgitation. Her past history was notable for gastroesophageal reflux disease as an infant, which had prompted a barium swallow at a few months of age demonstrating a posterior compression defect on the esophagus. Detailed imaging of the thoracic vasculature was not pursued at the time given the lack of respiratory symptoms and resolution of her reflux. She was now referred for cardiac magnetic resonance imaging to quantify the degree of left-to-right shunt, aortic regurgitation, and left ventricular dilation. Cardiac magnetic resonance demonstrated a small membranous ventricular septal defect with pulmonary-to-systemic flow ratio of 1.2, mild aortic regurgitation, and a mildly dilated left ventricle with normal systolic function. A right aortic arch was incidentally detected with branches arising in the following order: proximal to distal: right common carotid artery, right subclavian artery, and left innominate artery. The left innominate artery originated from a …Two children referred for cardiac imaging were found to have an unusual form of a vascular ring. The first patient was an asymptomatic 10-year-old girl with a membranous ventricular septal defect, prolapse of the right coronary aortic cusp, and mild aortic regurgitation. Her past history was notable for gastroesophageal reflux disease as an infant, which had prompted a barium swallow at a few months of age demonstrating a posterior compression defect on the esophagus. Detailed imaging of the thoracic vasculature was not pursued at the time given the lack of respiratory symptoms and resolution of her reflux. She was now referred for cardiac magnetic resonance imaging to quantify the degree of left-to-right shunt, aortic regurgitation, and left ventricular dilation. Cardiac magnetic resonance demonstrated a small membranous ventricular septal defect with pulmonary-to-systemic flow ratio of 1.2, mild aortic regurgitation, and a mildly dilated left ventricle with normal systolic function. A right aortic arch was incidentally detected with branches arising in the following order: proximal to distal: right common carotid artery, right subclavian artery, and left innominate artery. The left innominate artery originated from a …

Cardiovascular magnetic resonance parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation

BackgroundWe sought to identify cardiovascular magnetic resonance (CMR) parameters associated with successful univentricular to biventricular conversion in patients with small left hearts.MethodsPatients with small left heart structures and a univentricular circulation who underwent CMR prior to biventricular conversion were retrospectively identified and divided into 2 anatomic groups: 1) borderline hypoplastic left heart structures (BHLHS), and 2) right-dominant atrioventricular canal (RDAVC). The primary outcome variable was transplant-free survival with a biventricular circulation.ResultsIn the BHLHS group (n = 22), 16 patients (73%) survived with a biventricular circulation over a median follow-up of 40 months (4–84). Survival was associated with a larger CMR left ventricular (LV) end-diastolic volume (EDV) (p = 0.001), higher LV-to-right ventricle (RV) stroke volume ratio (p < 0.001), and higher mitral-to-tricuspid inflow ratio (p = 0.04). For predicting biventricular survival, the addition of CMR threshold values to echocardiographic LV EDV improved sensitivity from 75% to 93% while maintaining specificity at 100%. In the RDAVC group (n = 10), 9 patients (90%) survived with a biventricular circulation over a median follow-up of 29 months (3–51). The minimum CMR values were a LV EDV of 22 ml/m2 and a LV-to-RV stroke volume ratio of 0.19.ConclusionsIn BHLHS patients, a larger LV EDV, LV-to-RV stroke volume ratio, and mitral-to-tricuspid inflow ratio were associated with successful biventricular conversion. The addition of CMR parameters to echocardiographic measurements improved the sensitivity for predicting successful conversion. In RDAVC patients, the high success rate precluded discriminant analysis, but a range of CMR parameters permitting biventricular conversion were identified.

Outcomes of inferior sinus venosus defect repair

OBJECTIVE Inferior sinus venosus defect is an unusual form of interatrial communication with few published data on surgical outcomes. We sought to compare outcomes of surgical repair of inferior sinus venosus defect with those of large secundum atrial septal defects. METHODS Patients undergoing surgical closure of an isolated interatrial defect were reviewed, and those with inferior sinus venosus defect were identified on the basis of predetermined anatomic criteria. For each case, 2 controls with secundum atrial septal defect, matched for age and year of surgery, were selected. Technical outcome scores and other perioperative outcomes were compared. RESULTS Compared with the secundum atrial septal defect group (n = 90), the inferior sinus venosus defect group (n = 45) had worse technical outcome scores (P = .02), a higher rate of reintervention (9% vs 1%, P = .04), longer median total cardiopulmonary bypass (48 vs 39 minutes, P < .001) and crossclamp (29 vs 20 minutes, P < .001) times, and were more likely to stay more than 1 day in the intensive care unit (20% vs 8%, P = .04) and more than 3 days in the hospital (29% vs 13%, P = .03). Only 16 (36%) of the patients with inferior sinus venosus defect had a correct diagnosis preoperatively. Patients with an incorrect diagnosis had worse technical outcome scores than the secundum atrial septal defect group (P = .003), whereas those with a correct diagnosis had scores similar to those of the secundum atrial septal defect group (P = .55). CONCLUSIONS Compared with patients with secundum atrial septal defect, patients with inferior sinus venosus defect have more residual defects and longer durations of cardiopulmonary bypass and hospitalization. Rates of misdiagnosis of inferior sinus venosus defect are high and associated with worse technical outcome scores. Accurate preoperative diagnosis of this lesion may lead to improved outcomes.

Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedown

Objective Patients with a borderline left ventricular hypoplasia in the hypoplastic left heart syndrome variant or an unbalanced atrioventricular canal who undergo initial single‐ventricle palliation may be candidates for biventricular (BiV) conversion following left ventricle (LV) recruitment procedures. We investigated associations among preoperative parameters and postoperative outcomes in patients undergoing BiV conversion. Methods We performed a retrospective review of patients who underwent BiV conversion to determine variables associated with clinical outcomes. Predictor variables included cardiac diagnosis, age and weight, LV dimension, LV end diastolic volume, LV mass, preoperative LV end diastolic pressure (LVEDP), and preoperative left atrial pressure. Primary outcome was a composite of death, heart transplant, or BiV takedown. Results Of 51 patients, 11 experienced primary outcome (22%). Patients with hypoplastic left heart syndrome variant were more likely to experience primary outcome than those with an unbalanced atrioventricular canal (30% vs 6%; P = .03). Receiver operating characteristic analysis demonstrated that preoperative LVEDP had good predictive accuracy in classifying patients with and without the primary outcome (area under the curve, 0.757; 95% confidence interval, 0.594‐0.919; P = .012). The Youden J‐index indicated a cutoff of LVEDP ≥ 13 mm Hg as optimal for predicting the primary outcome. Multivariable Cox regression demonstrated that LVEDP ≥ 13 mm Hg (adjusted hazard ratio, 4.00; P = .037) and postoperative right ventricle pressure > 3/4 (adjusted hazard ratio, 21.75; P < .001) were significantly associated with primary outcome, independent of age, weight, and diagnosis. Conclusions Elevated preoperative LVEDP is a risk factor for suboptimal postoperative hemodynamic parameters and adverse outcome following BiV conversion from single‐ventricle palliation.

MRI guided iron assessment and oral chelator use improve iron status in thalassemia major patients

Oral iron chelators and magnetic resonance imaging (MRI) assessment of heart and liver iron burden have become widely available since the mid 2000s, allowing for improved patient compliance with chelation and noninvasive monitoring of iron levels for titration of therapy. We evaluated the impact of these changes in our center for patients with thalassemia major and transfusional iron overload. This single center, retrospective observational study covered the period from 2005 through 2012. Liver iron content (LIC) was estimated both by a T2* method and by R2 (Ferriscan®) technique. Cardiac iron was assessed as cT2*. Forty‐two patients (55% male) with transfused thalassemia and at least two MRIs were included (median age at first MRI, 17.5 y). Over a mean follow‐up period of 5.2 ± 1.9 y, 190 MRIs were performed (median 4.5 per patient). Comparing baseline to last MRI, 63% of patients remained within target ranges for cT2* and LIC, and 13% improved from high values to the target range. Both the median LIC and cT2* (cR2* = 1000/cT2*) status improved over time: LIC 7.3 to 4.5 mg/g dry weight, P = 0.0004; cR2* 33.4 to 28.3 Hz, P = 0.01. Individual responses varied widely. Two patients died of heart failure during the study period. Annual MRI iron assessments and availability of oral chelators both facilitate changes in chelation dose and strategies to optimize care. Am. J. Hematol. 89:684–688, 2014.