Zsofia Long

All Publications

• Association Between Location of Pulmonary Outflow Obstruction and Right Ventricular Size and Function After Repair of Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals. Pediatric cardiology Brennan, A., Punn, R., Lopez, L., Long, Z., McElhinney, D. B., Mainwaring, R. D., Ma, M., Friedberg, M. K., Hanley, F. L., Arunamata, A. A. 2024

  Abstract

  Quantification of right ventricular (RV) size and function after tetralogy of Fallot repair is critical for determining timing of reintervention and outcomes. Tetralogy of Fallot patients with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCAs) are a unique group in which the RV is subjected to various loading conditions, allowing for direct comparison. Retrospective evaluation of RV echocardiographic indices in repaired pediatric TOF/PA/MAPCAs patients (2/2002 - 4/2018). Patients were categorized as having conduit stenosis (peak gradient ≥ 2.5 m/s) and/or distal pulmonary artery (PA) stenoses (requiring catheter or surgical intervention and > 1/2 systemic RV pressures). A comparison group (N = 9) included distinct age-matched repaired patients without residual lesions. Indexed right heart dimensions were larger in patients with distal PA stenoses (N = 49) compared to conduit stenosis (N = 26), including RV end-diastolic and end-systolic dimensions (p < 0.01), despite earlier time to reintervention (p < 0.0001). RV fractional area change (FAC) was diminished in patients with distal PA stenoses compared to patients without residual lesions (p = 0.006). Patients with conduit stenosis demonstrated a marked decrement in tricuspid annular plane systolic excursion (TAPSE) z-scores. Repaired TOF/PA/MAPCAs patients with residual distal PA stenoses have larger right heart dimensions compared to patients with conduit stenosis, and decreased RV FAC compared to patients without residual lesions at 1-2 years after repair, suggesting early surveillance and immediate intervention on stenotic PA segments appears warranted to decrease the risk of further deterioration of RV systolic function.

  View details for DOI 10.1007/s00246-024-03730-3

  View details for PubMedID 39680069

  View details for PubMedCentralID 3898939

• Right Ventricular and Outflow Tract Functional Characteristics After Repair of Tetralogy of Fallot with Major Aortopulmonary Collaterals. Pediatric cardiology Long, Z. B., Punn, R., Zhang, Y., Arunamata, A. A., Asija, R., Ma, M., Hanley, F. L., McElhinney, D. B. 2024

  Abstract

  This study describes right ventricle (RV) characteristics and right ventricle to pulmonary artery (RV-PA) conduit function pre- and post-repair in patients with tetraology of Fallot with major aortopulmonary collaterals (TOF/MAPCAs). We reviewed patients who underwent single-stage, complete unifocalization, and repair of TOF/MAPCAs between 2006 and 2019 with available pre- and early postoperative echocardiograms. For a subset of patients, 6-12 month follow-up echocardiogram was available. RV and left ventricle (LV) characteristics and RV-PA conduit function were reviewed. Wilcoxon signed rank test and McNemar's test were used. 170 patients were reviewed, 46 had follow-up echocardiograms. Tricuspid valve annular plane systolic excursion (TAPSE) Z-scores were reduced from pre- (Z-score 0.01) to post-repair (Z-score -4.5, p < 0.001), improved but remained abnormal at follow-up (Z-score -4.0, p < 0.001). RV fractional area change (FAC) and LV ejection fraction were not significantly different before and after surgery. Conduit regurgitation was moderate or greater in 11% at discharge, increased to 65% at follow-up. RV-PA conduit failure (severe pulmonary stenosis or severe pulmonary regurgitation) was noted in 61, and 63% had dilated RV (diastolic RV area Z-score > 2) at follow-up. RV dilation correlated with the severe conduit regurgitation (p = 0.018). Longitudinal RV function was reduced after complete repair of TOF/MAPCAs, with decreased TAPSE and preserved FAC and LV ejection fraction. TAPSE improved but did not normalize at follow-up. Severe RV-PA conduit dysfunction was observed prior to discharge in 11% of patients and in 61% at follow-up. RV dilation was common at follow-up, especially in the presence of severe conduit regurgitation.

  View details for DOI 10.1007/s00246-024-03412-0

  View details for PubMedID 38360921

  View details for PubMedCentralID 6035101

• Balloon Angioplasty for Pulmonary Artery Stenosis After Complete Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals JOURNAL OF INVASIVE CARDIOLOGY Long, Z. B., Adamson, G. T., Peng, L. F., Perry, S. B., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2021; 33 (5): E378-E386

  View details for Web of Science ID 000648791300008

• Balloon Angioplasty for Pulmonary Artery Stenosis After Complete Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. The Journal of invasive cardiology Long, Z. B., Adamson, G. T., Peng, L. F., Perry, S. B., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  OBJECTIVES: The objective of this study was to assess procedural outcomes of balloon pulmonary artery (PA) angioplasty procedures after complete repair of tetralogy of Fallot with major aortopulmonary collateral arteries (TOF/MAPCAs).BACKGROUND: Our approach to patients with TOF/MAPCAs emphasizes early complete unifocalization and repair. Major PA reinterventions are relatively uncommon. Balloon PA angioplasty is often used, but the effectiveness of balloon PA angioplasty in this population is unknown.METHODS: The study cohort comprised patients who underwent complete unifocalization and repair of TOF/ MAPCAs at our center between 2002-2018 and underwent balloon PA angioplasty after repair. To assess immediate procedural outcomes, pre- and postintervention PA measurements were compared.RESULTS: We reviewed 134 vessels that were dilated a median of 1.1 years after repair in 60 patients (median 2 PA branches per patient). Treated vessels included 15 central, 64 lobar, and 55 segmental branches. The median PA diameter at the level of stenosis increased from 1.9 mm to 3.3 mm (P<.001), and the median diameter increase was 50%. All but 6 treated vessels were enlarged. The stenosis-distal diameter ratio increased from a median of 64% to 89% (P<.001). The median central PA to aortic systolic pressure ratio was 47% before and 39% after intervention (P<.001).CONCLUSIONS: Balloon PA angioplasty was acutely effective at treating most stenoses of reconstructed PA branches after repair of TOF/MAPCAs. Simple angioplasty can be a useful tool in treating isolated or modest stenoses after unifocalization/PA reconstruction surgery using our approach.

  View details for PubMedID 33908895

• Recurrent fibrovascular granulation on PhotoFix® bovine pericardium causing systemic and pulmonary venous obstruction after repair of scimitar syndrome. Cardiology in the young Taylor, N. P., Long, Z. n., Arunamata, A. n., Ma, M. n. 2021: 1–2

  Abstract

  A young adult with late diagnosis of scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel using PhotoFix® bovine pericardium with recurrent extensive fibrovascular granulation of the patch causing pulmonary and systemic venous obstruction leading to eventual explantation of the bovine pericardium.

  View details for DOI 10.1017/S1047951121000822

  View details for PubMedID 33682649