Branch Pulmonary Artery Research Articles

МНОГОЭТАПНОЕ ЛЕЧЕНИЕ ТЕТРАДЫ ФАЛЛО: ОТ ПАЛЛИАТИВНЫХ ВМЕШАТЕЛЬСТВ ДО ГИБРИДНОГО БИФУРКАЦИОННОГО Y-СТЕНТИРОВАНИЯ ЛЁГОЧНОЙ АРТЕРИИ И РАДИКАЛЬНОЙ КОРРЕКЦИИ (КЛИНИЧЕСКИЙ СЛУЧАЙ)

Primary radical correction of tetralogy of Fallot (ToF) is an optimal surgical option; however, in some conditions, in particular with concomitant extracardiac pathology and hypoplasia of the pulmonary artery (PA) branches, this intervention is not possible. Obstruction and/or hypoplasia of the PA is one of the risk factors for primary radical correction of this congenital heart defect (CHD). In world practice, there are several methods of palliative approach for PA growth with the possibility of further radical correction. However, in some cases, palliative approaches do not give the desired result. In this regard, we present a clinical case of a successful hybrid bifurcation Y-stenting of the PA and radical correction of ToF. Keywords: Tetralogy of Fallot, radical correction, branches of the pulmonary artery, hypoplasia, stenting of the branches of the pulmonary artery, bifurcation Y-stenting, clinical case.

Kiosk 3R-TB-01 - Improved Quantitative Flow Analysis of Main and Branch Pulmonary Arteries Distal to the Vortex in Repaired Tetralogy of Fallot- a Single Center 4D Flow Cardiovascular Magnetic Resonance Study

Kiosk 3R-TB-01 - Improved Quantitative Flow Analysis of Main and Branch Pulmonary Arteries Distal to the Vortex in Repaired Tetralogy of Fallot- a Single Center 4D Flow Cardiovascular Magnetic Resonance Study

Percutaneous dual intervention in a child with Alagille syndrome –A case report.

Alagille syndrome(ALGS) is a rare autosomal dominant (AD) genetic disorder .ALGS involves multisystem which includes liver ,heart, skeleton, Eyes, kidneys with facial abnormalities. ALGS may involve a particular system like facial dysmorphism, cardiac defect or vertebral defect. Some individual may not have liver anomalies or typical features. We report a case of 9 year old male child with large ostium secundum ASD(16 mm) and branch pulmonary artery stenosis(LPA)diagnosed incidentally at 8 year of age. The child had facial dysmorphism (broad forehead, frontal bossing , triangular facies, hypertelorism, prominent ear and pointed chin, straight nose with bulbous tip. The mental development of the child was borderline. He also had butterfly vertebrae in thoracic and lumber region. He was treated successfully with ASD device closure for ASD and balloon pulmonary valvuloplasty for LPA stensis. Both the procedure was performed simultaneously without any complications. There is no curative management for the disorder so symptomatic management is the only treatment option.

Optimisation of virtual monoenergetic reconstructions for the diagnosis of pulmonary embolism using photon-counting detector computed tomography angiography

PurposeComputed tomography (CT) pulmonary angiography is considered the gold standard for pulmonary embolism (PE) diagnosis, relying on the discrimination between contrast and embolus. Photon-counting detector CT (PCD-CT) generates monoenergetic reconstructions through energy-resolved detection. Virtual monoenergetic images (VMI) at low keV can be used to improve pulmonary artery opacification. While studies have assessed VMI for PE diagnosis on dual-energy CT (DECT), there is a lack of literature on optimal settings for PCD-CT-PE reconstructions, warranting further investigation.Material and methodsTwenty-five sequential patients who underwent PCD-CT pulmonary angiography for suspicion of acute PE were retrospectively included in this study. Quantitative metrics including signal-to-noise ratio (SNR) and contrast-to-noise (CNR) ratio were calculated for 4 VMI values (40, 60, 80, and 100 keV). Qualitative measures of diagnostic quality were obtained for proximal to distal pulmonary artery branches by 2 cardiothoracic radiologists using a 5-point modified Likert scale.ResultsSNR and CNR were highest for the 40 keV VMI (49.3 ± 22.2 and 48.2 ± 22.1, respectively) and were inversely related to monoenergetic keV. Qualitatively, 40 and 60 keV both exhibited excellent diagnostic quality (mean main pulmonary artery: 5.0 ± 0 and 5.0 ± 0; subsegmental pulmonary arteries 4.9 ± 0.1 and 4.9 ± 0.1, respectively) while distal segments at high (80-100) keVs had worse quality.Conclusions40 keV was the best individual VMI for the detection of pulmonary embolism by quantitative metrics. Qualitatively, 40-60 keV reconstructions may be used without a significant decrease in subjective quality. VMIs at higher keV lead to reduced opacification of the distal pulmonary arteries, resulting in decreased image quality.

Successful conservative treatment of pulmonary thromboembolism in unrepaired d-transposition of the great arteries with pulmonary hypertension: a case report

Background: Unrepaired dextro-transposition of great arteries (D-TGA) is well known for long-term complications, including tricuspid valve regurgitation, arrhythmias, and coronary artery abnormalities. Pulmonary hypertension (PH) is a frequent, progressive, and fatal complication in unrepaired CHD. This chronic condition can induce pulmonary thromboembolism (PTE). Aim: To report the management of PTE in unrepaired D-TGA with PH. Case Presentation: We present a rare case of an eleven-year-old male in unrepaired D-TGA with PH. He came with hemoptysis with no sign of acute or chronic infection. This condition was not associated with endocarditis or tuberculosis. The patient underwent a CT-pulmonary angiography examination and found a filling defect in the apical and posterior branches of the right superior pulmonary artery, suggesting a PTE that caused pulmonary parenchymal infarction. He was treated with intravenous low molecular weight heparin (LMWH) for ten days (1 mg/kg) and tranexamic acid. On follow-up, he did not experience cardiovascular collapse or hypotension; by the day, the hemoptysis had decreased in frequency and volume. He received long-term warfarin (2 mg/day) for three months. After three months of follow-up, the PTE was not visualized from CT-pulmonary angiography. Summary: Hemoptysis is rare and life-threatening in unrepaired CHD. Heparin administration continued with warfarin, and tranexamic acid provides a good prognosis in patients with PTE with D-TGA and PH.

Ductus Arteriosus Stenting as a Method of Palliative Treatment of Truncus Arteriosus Type A3: Literature Review and Clinical Case

Introduction. Van Praagh A3 variant of truncus arteriosus (TA A3) is a condition when the one pulmonary artery branch origins from the TA, the other is filled through the patent ductus arteriosus (PDA).
 PDA constriction in the early neonatal period can cause absence of blood supply to the one pulmonary artery branch. To prevent this, prolonged infusion of prostaglandin E1, PDA stenting or systemic-to-pulmonary shunt are used. These methods allow to postpone total repair (TR) and reduce mortality risks associated with neonatal period.
 The aim. To analyze research papers dedicated to endovascular approach of TA A3 staged treatment and to present our own experience.
 Materials and methods. We conducted a systematic literature search and analyzed various options for the TA A3 staged treatment. After the review, we used endovascular approach as the first stage of treatment in our clinical case.
 We studied a newborn weighing 4 kg with TA A3 (left pulmonary artery branch origins from the non-coronary sinus of the truncal valve, right pulmonary artery branch filling through the PDA). Nakata index was 83 mm2/m2. McGoon ratio was 1. We decided to perform PDA stenting with the aim of postponing surgical correction until the patient’s optimal age and weight.
 Discussion. In total, the number of publications about TA A3 staged treatment is limited. Most institutions prefer TA A3 staged treatment in newborns, because it is associated with a lower risk of complications than early TR. In our clinical case, the first step was PDA stenting. The staged approach allowed us to perform TR with a good result at the age of 3 months. Before TR, Nakata index and McGoon ratio were increased to 248 mm2/m2 and 1.9, respectively.
 Conclusions. The current trend of TA A3 repair has few different options of staged treatment. Staged approach helps to get out of the newborn period and achieves the optimal condition for TR. Our patient successfully received PDA stenting as the first stage of treatment, which created conditions for the pulmonary artery branches growth. The second stage was TR with good long-term results. After analyzing the publications, we noted that the use of endovascular PDA stenting is a good and safe alternative to surgical palliative treatment.

Towards Zero Phrenic Nerve Injury in Reoperative Pediatric Cardiac Surgery: The Value of Intraoperative Phrenic Nerve Stimulation.

Phrenic nerve injury is a devastating complication that results in significant morbidity and mortality. We developed a novel technique to localize the phrenic nerve and evaluate its success. Two groups of children underwent repeat sternotomy for a variety of indications. Group I (69 patients, nerve stimulator) and Group II (78 patients, no nerve stimulator). There was no significant difference in the mean age and weight between the two groups: (6.4 ± 6.5 years vs. 5.6 ± 6.4 years; p = 0.65) and (25.2 ± 24.1 vs. 22.6 ± 22.1; p = 0.69), respectively. The two groups were comparable in the following procedures: pulmonary conduit replacement, bidirectional cavopulmonary anastomosis, aortic arch repair, and Fontan, while Group I had more pulmonary arterial branch reconstruction (p = 0.009) and Group II had more heart transplant patients (p = 0.001). There was no phrenic nerve injury in Group I, while there were 13 patients who suffered phrenic nerve injury in Group II (p < 0.001). No early mortality in Group I, while five patients died prior to discharge in Group II. Eleven patients underwent diaphragm plication in Group II (p = 0.001). The mean number of hours on the ventilator was significantly higher in Group II (137.3 ± 324.9) compared to Group I (17 ± 66.9), p < 0.001. Group II had a significantly longer length of ICU and hospital stays compared to Group I (p = 0.007 and p = 0.006 respectively). Phrenic nerve injury in children continues to be associated with significant morbidities and increased length of stay. The use of intraoperative phrenic nerve stimulator can be an effective way to localize the phrenic nerve and avoid its injury.

Cross or Not to Cross-Dilemma of the Pulmonary Arteries.

We aimed to assess the frequency of criss-cross pulmonary arteries and associated intracardiac and vascular anomalies in patients who underwent CT angiography due to suspected congenital heart disease or vascular anomaly at our hospital. We retrospectively evaluated the CT angiography images of 355 patients aged 0-18years between April 2018 and December 2022. The presence of the criss-cross pulmonary artery anomaly was assessed. Additionally, in patients with a criss-cross pulmonary artery anomaly, accompanying branch pulmonary artery anomalies, aortic arch anomalies, and other vascular-cardiac anomalies were also evaluated. A total of 331 patients' images were evaluated. Criss-cross pulmonary artery anomaly was present in 57 patients (17.2%). Pulmonary artery branch anomaly was present in 16, aortic arch anomaly in 40 patients (70%) with criss-cross pulmonary artery anomaly, while associated intracardiac pathology (by echocardiography) was detected in 43 patients (75.4%). The frequency of criss-cross pulmonary artery was found to be significantly higher in patients with any aortic arch anomaly (p = 0.01). This study represents one of the largest series of patients with criss-cross pulmonary artery anomalies. Our results suggests that it may be more common than previously recognized and potentially overlooked. It is crucial to consider the presence of this anomaly in patients with complex aortic arch anomalies or cardiac pathologies, as it may have implications for surgical approaches and potential complications. Increased awareness of this anomaly among cardiologists and radiologists is necessary for accurate diagnosis and appropriate management.

Pulmonary Artery Measurements as Postnatal Prognostic Tool in Right Congenital Diaphragmatic Hernia

BackgroundRight-sided congenital diaphragmatic hernia (RCDH) is a rare and often fatal congenital anomaly, primarily attributed to lung hypoplasia, which is associated with small branch pulmonary artery (PA). This study investigated whether postnatal PA measurements obtained through echocardiography are associated with mortality or the extracorporeal membrane oxygenation (ECMO) requirement in neonates with RCDH. MethodsA retrospective study was conducted on neonates with RCDH born between 2008 and 2022. Echocardiography was performed on the day of birth. The diameter of the main PA (MPA) was measured at the maximal dimension, and the diameters of the left PA (LPA) and right PA (RPA) were measured at the bifurcation. The primary outcome was mortality or ECMO requirement. Parameters, including the LPA:MPA ratio, RPA:MPA ratio, Nakata index, McGoon ratio, and ejection fraction (EF), were analyzed and compared with the observed-to-expected lung-to-head ratio (o/e LHR), initial blood gas, and defect size as predictive values. ResultsAmong 39 neonates with RCDH, 25 (64.1 %) survived without ECMO. The non-survivor or ECMO group exhibited lower o/e LHR, reduced EF, smaller LPA and RPA diameters, and larger MPA diameter than survivors. Lower LPA:MPA ratio, Nakata index, McGoon ratio, and higher initial PaCO2 were associated with adverse outcomes. Notably, the LPA:MPA ratio showed the highest predictive capability (area under the curve, 0.983; p < 0.001). ConclusionThe LPA:MPA ratio is a promising postnatal predictor of mortality or ECMO requirement in neonates with RCDH. Additionally, Nakata index, McGoon ratio, and initial PaCO2 are significantly correlated with outcomes. Level of evidenceThis is a level III. Type of StudyPrognostic study.

Normative computed tomography angiography values of the main and branch pulmonary arteries in children.

Non-invasive cardiac imaging like echocardiogram, cardiac magnetic resonance imaging (CMR), and computed tomography angiography (CTA) play a key role in the diagnosis, aid in management and follow-up of congenital heart disease patients. Normative data for intracardiac and extracardiac vascular structures in children are currently available for echocardiogram, CMR, and non-gated CTA. We sought to establish systolic and diastolic normative data for main and branch pulmonary arteries in children using electrocardiogram (ECG)-gated CTA. Diameters and cross-sectional areas of the main and branch pulmonary arteries were measured in systole and diastole based on the aortic valve position (open versus closed) in 100 subjects who had ECG-gated cardiac CTA at our center between January 2015 through December 2020 and met our inclusion criteria. The allometric exponent (AE) for each parameter was derived, and the parameter/body surface area (BSAAE) was established using the previously described methods. A total of 100 children aged 0-18years were analyzed; mean age was 5.3years (SD, 6.1years). Z-score curves were plotted in relation to the BSA for the mean, maximum, and minimum diameters and cross-sectional area of the main and branch pulmonary arteries for systole and diastole. Conclusion: We report systolic and diastolic mean, maximum, and minimum diameters and cross-sectional areas along with Z-scores and normative curves for the main and branch pulmonary arteries in children derived using ECG-gated cardiac CTA. We believe our results can help identify abnormally sized main and branch pulmonary arteries. What is Known: • Normative data for intracardiac and extracardiac vascular structures in the pediatric population are available for echocardiography, cardiac MRI and non-ECG gated CTA. • Z-scores with standard deviations are commonly used in children, but SDs are not constant across body sizes due to heteroscedasticity. What is New: • Allometric exponent was derived for each parameter and the parameter/body surface area (BSA) was established. • This is the first ECG-gated CTA study to provide normative en face systolic, diastolic diameters and cross-sectional areas along with Z-scores and normative curves for the main and branch pulmonary arteries in children.

Congenital Bilateral Branch Pulmonary Artery Hypoplasia: Prenatal Diagnosis and Parental Counseling.

Congenital Bilateral Branch Pulmonary Artery Hypoplasia: Prenatal Diagnosis and Parental Counseling.

Spectral Doppler Parameters of Fetal Main Branch Pulmonary Artery at 20 to 40 Weeks of Gestation: Reference Ranges and Percentile Calculators

The published reference ranges for Doppler parameters of the fetal pulmonary artery (PA) are usually derived from small sample sizes with no practical standard score or percentile ranking, which hinders systematic comparisons of Doppler figures across different gestational ages (GAs). This study aimed to establish comprehensive reference ranges and provide a percentile ranking solution for key spectral Doppler parameters. This is a cross-sectional study of 465 uncomplicated singleton pregnancies during 20 to 40weeks of gestation. Spectral waveforms of the fetal main branch PA were obtained with a pulsed-wave Doppler interrogation site within 5mm from the vascular origin. Fifteen spectral Doppler parameters were identified. Associations between these parameters with GA and fetal heart rate were assessed and used to develop percentile calculators via different statistical models. The root mean squared error of each model was calculated to determine the best performance solution. Acceptable spectral waveforms were obtained for 94.1% (438/465) of the fetuses. All Doppler parameters except pulsatility index, manually traced pulsatility index, peak systolic velocity, and time to systolic notch/acceleration time ratio were significantly correlated with GA, while acceleration time, ejection time, time to systolic notch, peak early-diastolic reversal flow, and peak early-diastolic reversal flow/peak systolic velocity ratio were additionally significantly correlated with fetal heart rate. Support vector machine models with radial basis kernel yield the best percentile estimation (root mean squared error of 2.17-4.08 and R2 of >0.98). Furthermore, the top 5% and bottom 5% outliers could be identified with positive predictive values of 0.71 to 0.97. An online user interface of percentile calculators is available at https://github.com/cmb-chula/fetoPAD. This study presents normal reference ranges and percentile calculators for 15 spectral Doppler parameters of the fetal main branch PA, some of which have not been published. The estimated percentiles enhance comparison and outlier detection of the spectral Doppler figures among fetuses at different GAs.

Mid-term results following pulmonary artery patch augmentation in congenital heart disease.

Treatment of pulmonary artery (PA) stenosis in congenital heart disease is associated with adverse outcomes. The aim of this retrospective cohort study was to compare outcomes after surgical patch augmentation of PA stenosis in patients with biventricular congenital heart disease using different patch materials. We identified all patients from our institutional congenital heart disease database who underwent patch augmentation for PA stenosis on the main pulmonary artery (MPA) or PA branches between 2012 and 2018. Patch materials used were glutaraldehyde fixated autologous pericardium (AP), expanded polytetrafluoroethylene (ePTFE), equine pericardium (EP), and bovine pericardium (BP). The primary study endpoint was the composite of catheter-based re-intervention or re-operation to relieve recurrent stenosis at the site of prior implanted patch material. A total of 156 patients (median age, 5 months, range, 0-85 months; median weight, 6.2 kg, range, 2.8-15.0 kg) underwent patch augmentation using 163 patches (ePTFE =99, 61%; EP =34, 21%; AP =25, 15%; BP =5, 3%). Overall, 131 (84%) patients underwent patch augmentation at the MPA, and 25 (16%) patients underwent patch augmentation at one or both PA branches. Over a mean follow-up period of 4±2 years, 30 patients (19%) reached the study endpoint. Freedom from primary endpoint was 92%±3% for the MPA and 25%±9% for PA branches at 5 years, respectively (P<0.001). Comparison of patch materials revealed similar re-intervention rates between ePTFE, AP, and EP. In contrast, outcomes were significantly decreased following the usage of BP when compared to other materials (ePTFE vs. BP, P=0.01; EP vs. BP, P=0.005). In the multivariable analysis, lower weight at index operation, patch augmentation of PA branches, and usage of BP were independently associated with re-intervention. Patch augmentation of the MPA was associated with acceptable outcomes, while patch augmentation of PA branch stenosis remained independently associated with re-intervention. None of the used patch materials demonstrated superiority; however, BP had a higher rate of re-interventions.

Novel mutation in ELN gene causes cardiac abnormalities and inguinal hernia: case report

BackgroundElastin-driven genetic diseases are a group of complex diseases driven by elastin protein insufficiency and dominant-negative production of aberrant protein, including supravalvular aortic stenosis (SVAS) and autosomal dominant cutis laxa. Here, a Chinese boy with a novel nonsense mutation in the ELN gene is reported.Case presentationWe report a 1-year-old boy who presented with exercise intolerance, weight growth restriction with age, a 1-year history of heart murmur, and inguinal hernia. Gene sequencing revealed a novel nonsense mutation in the ELN gene (c.757 C > T (p.Gln253Ter), NM_000501.4). Due to severe branch pulmonary artery stenosis, the reconstruction of the branch pulmonary artery with autologous pericardium was performed. The inguinal hernia repair was performed 3 months postoperatively. After six months of outpatient follow-up, the child recovered well, gained weight with age, and had no special clinical symptoms.ConclusionWe identified a de novo nonsense mutation in the ELN gene leading to mild SVAS and severe branch pulmonary artery stenosis. A new phenotype of inguinal hernia was also needed to be considered for possible association with the ELN gene. Still, further confirmation will be necessary.

Clarifying the Pulmonary Arterial Morphology and Pulmonary Blood Supply in Patients with Tetralogy of Fallot and Pulmonary Atresia on Computed Tomography Angiography

Aim. The present study sought to characterize the pulmonary arterial morphology and pulmonary blood supply in patients of tetralogy of Fallot and pulmonary atresia (TOF-PA) on CT angiography. Materials and Methods. We retrospectively reviewed our departmental database to identify patients with TOF-PA evaluated using CT angiography. The images were analysed to detect the presence of main and branch pulmonary arteries and pulmonary arterial confluence, presence of major aortopulmonary collateral arteries (MAPCAs), laterality and relation with pulmonary arterial morphology, and presence of patent arterial duct and associated intra- and extracardiac anomalies. Results. TOF-PA was identified in 177 patients (114 (64.4%) males; median age: 9 months). Pulmonary arteries were confluent in 142/177 (80.2%) patients. According to Somerville classification, type II pulmonary atresia was the most frequent pattern seen in 127/177 (71.8%). Based on McGoon’s ratio, pulmonary arteries were adequate for surgery in 123/177 (69.5%) patients. Patent arterial duct was present in 84/177 (47.5%) patients while MAPCAs were present in 124 (70.1%) patients, of which 72/124 (58.1%) patients had at least 1 essential MAPCA supplying either lung. According to Congenital Heart Surgeons’ Society classification, type B pulmonary arterial anatomy was the most prevalent, seen in 103/177 (57.6%) patients. Conclusion. TOF-PA is associated with marked morphologic variability in the pulmonary arterial arborization to supply the lungs. Cardiac CT angiography can accurately delineate the pulmonary arterial morphology, sources of pulmonary blood supply, and associated cardiovascular anomalies in patients with TOF-PA which aids in planning appropriate surgical management including decisions regarding the need for unifocalization of MAPCAs.

Short-term outcomes following total correction of tetralogy of fallot in adult patients

ObjectivesTetralogy of Fallot (TOF) is a common congenital heart disease which should be corrected. The recommended time for the Tetralogy of Fallot Total Correction (TFTC) surgery is during the infancy for the possible difficulties during the surgery and the related issues. However, sometimes TOF is diagnosed and managed during the adulthood.MethodsThis study is a descriptive and retrospective one which included all patients who underwent TFTC at the age of 15-year and older in 10 years (between the years 2010 and 2020) to identify short-term (in-hospital mortality, ICU stay, postoperative bleeding, respiratory complications after the surgery such as pulmonary edema, pneumonia, etc.) and one-year (left ventricle ejection fraction (LVEF), right ventricle (RV) ejection fraction, the severity of tricuspid and aortic regurgitation after surgery) outcomes. All data were taken from medical records at Rajaie Cardiovascular Medical and Research Center. Data were analyzed using SPSS 22.Results94 patients with the mean ± SD age of 26.7 ± 9.6 years were enrolled. Most of them were male (59.6%) (P-value: 0.009). In-hospital mortality in our study were 5.3%. Tricuspid regurgitation (TR) was significantly resolved after the surgery (P-value: 0.006). Of 17 (18.1%) patients with small or hypoplastic pulmonary artery (PA) branches, 14 patients had acceptable PA branch size after surgery.ConclusionTFTC at an older age is safe with acceptable results. Age is not a contraindication for TFTC and surgery should be recommended if the patients are diagnosed with TOF in adulthood. Also, the TOF diagnosis should be considered in adult patients with suspicious signs and symptoms.

Results of Transcatheter Therapy with Balloon Versus Stent for Treating Isolated Branch Pulmonary Artery Stenosis in Children and Adolescents: A Midterm Study

Background: Branch pulmonary artery stenosis presents as either an isolated disease or in association with congenital heart diseases (in 2 - 3% of cases). Balloon angioplasty (BA) and stent implantation (SI) are two percutaneous methods that are commonly used to treat this condition. We compared the outcomes of these 2 methods in children and adolescents with isolated branch pulmonary artery stenosis. Methods: Pediatric patients with severe branch pulmonary artery stenosis who were referred to our center and underwent transcatheter insertion via either BA or SI between 2010 and 2019 were studied. Patients’ demographic data, including age, gender, and body weight, were recorded. Standard procedures for BA and SI were applied. Post-procedure angiography and echocardiographic parameters were determined and compared between the 2 groups. Patients who were followed up for at least 2 years were selected for this study, and comparative evaluations were performed during the follow-up. Results: Forty patients with a median age of 2.1 years and a median weight of 10 kg were enrolled in the study, of whom 25 and 15 underwent BA and SI, respectively. The trans-stenotic pulmonary artery pressure gradient significantly reduced immediately after both procedures, but there was no significant difference between the BA and SI groups in terms of pulmonary artery pressure gradient early after the procedures (P-value = 0.014). There was a significant decline in the peak right ventricular pressure after both procedures. Restenosis occurred more frequently in the BA group. In the BA group, patients under one year old and weight lower than 10 kg had a lower restenosis recurrence rate, evidenced by echocardiographic measurements, compared to those with a weight over 10 kg. Three patients (12%) in the BA group needed balloon reinsertion. Conclusions: Immediate therapeutic success was similar between BA and SI in patients with branch pulmonary artery stenosis. Re-stenosis occurred more often in the BA group; however, the chance of re-stenosis was lower among patients weighing less than 10 kg over a 2-year follow-up.

Decreased clinical performance in TGA-ASO patients after RVOT re-interventions; multicenter European collaboration

Abstract Background In patients with transposition of the great arteries and an arterial switch operation (TGA-ASO) right ventricular outflow tract (RVOT) obstruction is a common complication requiring one or more RVOT reinterventions. However, data on the impact of reinterventions on clinical performance is scarce. Therefore, we aimed to assess in TGA-ASO patients the cardiopulmonary exercise capacity (CPET) and right ventricular function stratified for the number of RVOT reinterventions. Method This multicenter observational study was performed in adult (≥16 years) TGA-ASO patients from the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). All patients underwent a comprehensive clinical workup including CPET and echocardiography, including right ventriculo-arterial (VA) coupling (defined as tricuspid annular plane systolic excursion (TAPSE) / right ventricular systolic pressure (RVSP) ratio). Patients were stratified for the number of reinterventions at the RVOT, including re-interventions at the pulmonary valve, main pulmonary artery and pulmonary branches. Association between RVOT re-intervention(s) and functional parameters, including predicted (%) peak oxygen uptake (peak VO2), TAPSE, right ventricle (RV) S’ and VA coupling of the RV were analyzed with linear regression. Results A total of 449 TGA patients were included in this study, with a mean age of 25.4 ± 5.6 years and median time until ASO was 8 days [IQR 6-15]. Eleven (2.5%) patients with previous re-interventions at the left ventricular outflow tract (LVOT) or aortic arch were excluded from the analysis. Twenty-six percent of the included patients had a ventricular septal defect (VSD) and 1.6% were diagnosed with double outlet right ventricle (DORV). In the total cohort, 13% of the patients had 1 previous RVOT re-intervention, 8% had 2 RVOT re-interventions and 5% had 3 RVOT re-interventions. Patients had echocardiographic examination and 55% underwent CPET. Predicted VO2peak was significantly reduced in patients with 1 RVOT re-intervention (72%), 2 RVOT re-interventions (69%) and 3 RVOT re-interventions (71%) compared to patients without any-reintervention (77%) (p=0.0268). Right VA coupling was a significant predictor (p=0.017) for reduced hemodynamic performance in patients with 1 RVOT re-intervention (0,50), 2 RVOT re-interventions (0,51) and 3 RVOT re-interventions (0,26). Conclusion In TGA-ASO patients, RVOT reinterventions were associated with a reduced clinical performance. VA coupling of the right ventricle was the most sensitive marker for hemodynamic performance in TGA-ASO patients.

Abstract 15837: Anomalous Bronchial Artery Origin- The Canary in the Coal Mine- For the Diagnosis of Chronic Thromboembolic Pulmonary Hypertension

Introduction- Anomalous bronchial artery origin is seen in 8.3% to 35% of cases with most originating from the aortic arch, internal mammary artery, and subclavian artery. Origin of this vessel from a coronary artery is extremely rare. Case presentation- A 49-year-old male with history of unprovoked pulmonary embolisms (PE) in the setting of triple antibody positive Antiphospholipid antibody syndrome on lifelong anticoagulation presented with left sided chest pain and dyspnea. Initial lab work showed a troponin of 5.46 ng/mL, BNP of 602 pg/mL and D-dimer of 0.62 ug/mL. EKG showed normal sinus rhythm with non-specific ST segment changes in anterior leads. Computed Tomography Angiography (CTA) was positive for a PE. There was concern for a false positive result due to previous history of multiple PEs while on therapeutic levels of warfarin and near normal D-dimer. Echocardiogram showed ejection fraction of 38% with akinesis of anteroapical and inferoapical segments and no evidence of shunt on bubble study. Cardiac catheterization showed non-obstructive coronaries. From the ostium of the right coronary artery there appeared a fistula like structure extending into the pulmonary vasculature. Pulmonary angiogram was performed and showed chronic occlusion in branches of right pulmonary artery (RPA), pulmonary hypertension (PH) with mean pulmonary artery pressure of 36 mmHg and no evidence of acute thrombus. There was aberrant origin of the right bronchial artery from the right coronary artery that was hypertrophied as it supplied collateral flow to the area of the occluded RPA. The patient was started on guideline directed medical therapy for heart failure and follow up was arranged for further outpatient management of PH. Discussion- A rare congenital anomaly was used to guide the diagnosis of a potentially devastating and treatable medical condition. It shows the limitations of a CTA in the setting of chronic PE.

Abstract 14631: A Perfect Storm: Alcoholic Cardiomyopathy, Influenza B and the Battle Against Biventricular Thrombi and Pulmonary Embolism

Introduction: Biventricular thrombi (BT) are a rare occurrence in clinical practice and when they occur in alcoholic dilated cardiomyopathy (ADCM), they are a complication of this condition. Secondary predisposing conditions that can promote thrombus formation include infections like Influenza B. Herein, we report a rare case of ADCM and influenza B propagating the development of biventricular thrombi and pulmonary embolism. Case Presentation: A 47-year-old healthy gentleman with a history of tobacco, marujuana and alcohol abuse presented with shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, non-productive cough, fevers and bilateral lower extremity swelling. Initial vitals were significant for tachycardia and slightly elevated blood pressure. On examination, the patient had decreased breath sounds in the right lower lobe with rales and trace lower extremity edema. Initial labs revealed elevated BNP, High-sensitivity troponin and d-dimer. Rapid influenza B was positive. Chest x-ray showed pleural effusion and CT angiogram chest revealed filling defect in the segmental and subsegmental branches of the right pulmonary artery. Echocardiogram revealed biventricular thrombi and LVEF of 10-15% with global LV hypokinesis. He was treated with oseltamivir, enoxaparin and was started on guideline directed medical therapy. Discussion: BT formation can occur in heart failure with reduced ejection fraction. ADCM induces LV dilation which creates a stagnant environment conducive to thrombus development. Influenza B can cause a prothrombotic state due to endovascular injury by proinflammatory mediators. Due to advanced diagnostic techniques, the incidence of BT has increased however, they remain a significant challenge due to embolization risks. This is the first case that highlights that patients with ADCM in combination with influenza B are predisposed to hypercoagulable states inducing fatal BT formation thus, prompt recognition is vital.