Melody transcatheter pulmonary valve replacement: a single-center case series in Southeast Asia.

BACKGROUND: Studies of transcatheter pulmonary valve replacement (TPVR) with the Melody valve have demonstrated good clinical and hemodynamic outcomes. Our study analyzes the midterm clinical and hemodynamic outcomes for patients who underwent Melody valve implantation in Southeast Asia. METHODS: Patients with circumferential conduits or bioprosthetic valves and experiencing post-operative right ventricular outflow tract (RVOT) dysfunction were recruited for Melody TPVR. RESULTS: Our cohort (n = 14) was evenly divided between pediatric and adult patients. The median age was 19 years (8-38 years), a male-to-female ratio of 6:1 with a median follow-up period of 48 months (16-79 months), and the smallest patient was an 8-year-old boy weighing 18 kg. All TPVR procedures were uneventful and successful with no immediate mortality or conduit rupture. The primary implant indication was combined stenosis and regurgitation. The average conduit diameter was 21 ± 2.3 mm. Concomitant pre-stenting was done in 71.4% of the patients without Melody valve stent fractures (MSFs). Implanted valve size included 22-mm (64.3%), 20-mm (14.3%), and 18-mm (21.4%). After TPVR, the mean gradient across the RVOT was significantly reduced from 41 mmHg (10-48 mmHg) to 16 mmHg (6-35 mmHg) at discharge, p < 0.01. Late follow-up infective endocarditis (IE) was diagnosed in 2 patients (14.3%). Overall freedom from IE was 86% at 79 months follow-up. Three patients (21.4%) developed progressive RVOT gradients. CONCLUSION: For patients in Southeast Asia with RVOT dysfunction, Melody TPVR outcomes are similar to those reported for patients in the US in terms of hemodynamic and clinical improvements. A pre-stenting strategy was adopted and no MSFs were observed. Post-implantation residual stenosis and progressive stenosis of the RVOT require long term monitoring and reintervention. Lastly, IE remained a concern despite vigorous prevention and peri-procedural bacterial endocarditis prophylaxis.

Impact of pulmonary stenosis on right ventricular global longitudinal strain in repaired tetralogy of Fallot patients post transcatheter pulmonary valve replacement.

BACKGROUND: Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RV GLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). METHODS: A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0%-49%, 50%-74%, and >75%. RV GLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). RESULTS: Forty-eight patients were included. Every 14.3% increase in preimplantation RVSP above 28% was associated with an absolute magnitude 1% lower RV GLS (p = .001). Preimplantation RVSP when 75% or higher had 3.36% worse RV GLS than the lowest bin (p = .014). Overall, average RV strain magnitude was higher when preimplantation RVSP was less than 50% and had greater improvement over the three time points. Higher post implantation RVSP correlated with lower strain magnitude. CONCLUSION: Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Clinical Presentation and Therapy of d-Transposition of the Great Arteries.

d-Transposition of the great arteries (d-TGA) is the most common form of congenital heart disease that presents with cyanosis in a newborn. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. It constitutes 3-5% of all congenital heart defects. In a simple d-TGA (about two-thirds of patients), there is no other cardiac abnormality other than a patent foramen ovale (PFO) and a patent ductus arteriosus (PDA). In a complex d-TGA additional cardiac abnormalities such as VSD, pulmonary stenosis or coronary abnormalities are present. About one-third to 40% of patients with d-TGA have an associated ventricular septal defect. Among patients with d-TGA, 6% of those with intact ventricular septum and 31% of those with ventricular septal defect have associated pulmonary stenosis. Coronary abnormalities are of importance with regard to the complexity of surgical repair.

Right Ventricular and Outflow Tract Functional Characteristics After Repair of Tetralogy of Fallot with Major Aortopulmonary Collaterals.

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.

Prenatal Predictors for Pulmonary Balloon Valvuloplasty in the Newborn.

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1-58) days and 11 (22%) underwent outpatient PBV at 51.8 (11-174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02-94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS.

Intracardiac Three-Dimensional Image as Surgical Decision-Making Tool of Congenital Heart Disease.

This study aimed to evaluate the anatomical reproducibility of a preoperative intracardiac 3D image (IC image) created using computed tomography, and to investigate its usefulness as a surgical decision-making tool. Between 2012 and 2022, ventricular septal defect (VSD) patients, and double outlet right ventricle (DORV) or transposition of the great arteries (TGA) with pulmonary stenosis (PS) patients who underwent cardiac surgery and had preoperative computed tomography were enrolled. SYNAPSE VINCENT® (Fujifilm) was used to create an IC image which was analyzed retrospectively. In 14 VSD patients, the diagnostic consistency rate in the Soto classification with intraoperative findings was 100% (14/14) for IC image versus 64% (9/14) for transthoracic echocardiography (P = 0.04). The defect size showed a higher correlation coefficient with IC image (0.837, P = 0.001) than with transthoracic echocardiography (0.567, P = 0.034). In 11 DORV/TGA with PS patients, the diagnostic consistency rate in the Lev classification was 100% (9/9) for IC image versus 77% (7/9) for transthoracic echocardiography (P = 0.47). The secondary interventricular foramen (SVF)/left ventricular outflow tract (LVOT) ratio by IC image was significantly smaller in the biventricular-repair group (median 0.71, IQR 0.67-1.06) than in the univentricular-repair group (median 1.79, IQR 1.53-2.42) (P = 0.006). An IC image is useful as a surgical decision-making tool for simple VSDs and complex congenital heart diseases such as DORV or TGA with pulmonary stenosis. The SVF/LVOT ratio determined from the IC image may be a useful indicator for avoiding LVOT obstruction.

Percutaneous approach to residual pulmonary bifurcation stenosis in conotruncal diseases.

Residual stenosis after right ventricle outflow tract surgery represents a major issue to manage in the children and adult patient with conotruncal defects. Despite a detailed multimodality imaging, the anatomy of distal pulmonary trunk and pulmonary artery bifurcation may be challenging in these patients.The aim of this study was to analyse retrospectively the outcome of the percutaneous transcatheter treatment in children with post-surgical stenosis of pulmonary artery bifurcation.We enrolled 39 patients with a median age of 6.0 years. Standard high-pressure balloon dilation was attempted in 33 patients, effective in 5 of them. Pulmonary branch stenting was performed in 10 patients, effective in 6. A kissing balloon approach was chosen in 17 patients (6 after angioplasty or stenting failure), and this technique was effective in 16 cases. Finally, a bifurcation stenting was performed in 10 patients (second step in 9 cases), effective in all the cases. None of the patients approached by kissing balloon needed a bifurcation stenting.In conclusion, standard balloon angioplasty and standard stenting might be ineffective in post-surgical stenosis involving pulmonary artery bifurcation. In this population, kissing balloon or bifurcation stenting, followed by side branch de-jailing, may be more effective in relieving the gradient.

Transcatheter atrial septal defect closure late after completion of biventricular circulation in patients with pulmonary atresia intact ventricular septum or critical pulmonary stenosis.

OBJECTIVE: This study aimed to explore anatomical and hemodynamic features of atrial septal defect, which was treated by transcatheter device closure late after completion of biventricular circulation in patients with pulmonary atresia and intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). METHODS: We studied echocardiographic and cardiac catheterization data, including defect size, retroaortic rim length, single or multiple defects, the presence of malalignment atrial septum, tricuspid and pulmonary valve diameters, and cardiac chamber sizes, in patients with PAIVS/CPS who underwent transcatheter closure of atrial septal defect (TCASD), and compared to control subjects. RESULTS: A total of 173 patients with atrial septal defect, including 8 patients with PAIVS/CPS, underwent TCASD. Age and weight at TCASD were 17.3 ± 18.3 years and 36.6 ± 13.9 kg, respectively. There was no significant difference in defect size (13.7 ± 4.0 vs. 15.6 ± 5.2 mm, p = 0.317) and the retro-aortic rim length (3.7 ± 4.3 vs. 3.6 ± 0.3.1 mm, p = 0.948) between the groups; however, multiple defects (50% vs. 5%, p < 0.001) and malalignment atrial septum (62% vs. 14%. p < 0.001) were significantly frequent in patients with PAIVS/CPS compared to control subjects. The ratio of pulmonary to systemic blood flow in patients with PAIVS/CPS was significantly lower than that in the control patients (1.2 ± 0.4 vs. 2.0 ± 0.7, p < 0.001); however, four out of eight patients with atrial septal defect associated with PAIVS/CPS had right-to-left shunt through a defect, who were evaluated by the balloon occlusion test before TCASD. The indexed right atrial and ventricular areas, the right ventricular systolic pressure, and mean pulmonary arterial pressure did not differ between the groups. After TCASD, the right ventricular end-diastolic area remained unchanged in patients with PAIVS/CPS, whereas it significantly decreased in control subjects. CONCLUSIONS: Atrial septal defect associated with PAIVS/CPS had more complex anatomy, which would be a risk for device closure. Hemodynamics should be individually evaluated to determine the indication for TCASD because PAIVS/CPS encompassed anatomical heterogeneity of the entire right heart.

Pulmonary valve stenosis in a recipient twin in twin-to-twin transfusion syndrome with successful balloon valvuloplasty after birth: a case report.

BACKGROUND: Pulmonary stenosis (PS) is a congenital heart diseases (CHDs) with a spectrum of stenosis. Monochorionic (MC) twins are at increased risk of CHDs, especially acquired CHDs in twin-twin transfusion syndrome (TTTS). PS/Pulmonary atresia (PA) is a rare coincidence with TTTS. MC twin pregnancies have increased in last decades due to increasing in maternal age and extensive use of assisted reproductive technologies. Therefore, attention to this group is important for heart abnormalities, especially in twins with TTTS. Multiple cardiac abnormalities in MC twins with TTTS are to be expected due to cardiac hemodynamic changes and may be eliminated by Fetoscopic laser photocoagulation treatment. Prenatal diagnosis of PS is necessary given the importance of treatment after birth. CASE PRESENTATION: We here present a case of coexistence of TTTS with PS in a growth restricted recipient twin who successfully treated with balloon pulmonary valvuloplasty in neonatal period. Also, we detected infundibular PS after valvuloplasty that treated with medical therapy (propranolol). CONCLUSIONS: It is important to detect acquired cardiac abnormalities in MC twins with TTTS, and follow them up after birth to determine the need of intervention in neonatal period.

An infant with suspected missed diagnosis of Williams syndrome failed weaning off CPB after surgical correction of pulmonary stenosis: a case report and literature review.

Williams syndrome (WS) is a rare congenital developmental disorder caused by the deletion of between 26 and 28 genes on chromosome 7q11.23. For patients with WS, in view of the particularity of the supravalvular aortic stenosis, choosing appropriate arterial cannula, maintaining higher perfusion pressure as well as strengthening myocardial protection during cardiopulmonary bypass (CPB) is essential to the clinical outcome. Here, we report a child with pulmonary artery valvular stenosis who failed to wean off CPB because of malignant arrhythmias and cardiac insufficiency after surgical correction of pulmonary valvular stenosis. With the assistance of extracorporeal membrane oxygenation (ECMO), emergency cardiac catheterization revealed supravalvular aortic stenosis (SVAS), which suggests a suspected missed diagnosis of WS. Finally, under the support of ECMO, the cardiac function gradually returned to normal, and the child was discharged 23 days after surgery.

Prophylactic use of a lidocaine constant rate infusion versus saline in dogs undergoing balloon valvuloplasty for management of pulmonic stenosis: A randomized control trial.

OBJECTIVE: To evaluate the effect of a prophylactic lidocaine constant rate infusion (CRI) on the incidence and malignancy of catheter-induced ventricular ectopic complexes (VECs) during balloon valvuloplasty for management of pulmonic stenosis in dogs. STUDY DESIGN: Single-centre, prospective, randomized study. ANIMALS: Client-owned dogs (n = 70) with pulmonic stenosis. METHODS: Dogs were randomly assigned to one of two anaesthetic protocols: administration of lidocaine 2 mg kg-1 bolus followed by a CRI (50 µg kg-1 minute-1; group LD) or a saline placebo (group SL) during balloon valvuloplasty. All dogs were premedicated with methadone (0.3 mg kg-1) intramuscularly and a digital three-lead Holter monitor was applied. Anaesthetic co-induction was performed with administration of alfaxalone (2 mg kg-1) and diazepam (0.4 mg kg-1), and anaesthesia was maintained with isoflurane vaporised in 100% oxygen. CRIs were started on positioning of the dog in theatre and discontinued as the last vascular catheter was removed from the heart. All dogs recovered well and were discharged 24 hours postoperatively. Blinded Holter analysis was performed by an external veterinary cardiologist using commercially available dedicated analysis software; p < 0.05. RESULTS: Of the 70 dogs enrolled in the study, 61 were included in the final analysis: 31 in group LD and 30 in group SL. There was no significant difference between sinus beats (p = 0.227) or VECs (p = 0.519) between groups. In group LD, 19/31 (61.3%) dogs had a maximum ventricular rate ≥250 units and 20/30 (66.7%) dogs in group SL (p = 0.791). CONCLUSION AND CLINICAL RELEVANCE: In this study, the use of a prophylactic lidocaine bolus followed by CRI in dogs undergoing balloon valvuloplasty for management of pulmonic stenosis did not significantly decrease the incidence nor the malignancy of VECs during right heart catheterization compared with a saline CRI.

[Complex congenital heart disease : about a case of Noonan syndrome]. / Cas clinique. Cardiopathie congénitale complexe : à propos d'un cas de syndrome de Noonan.

We present the case of a young girl in whom pre-natal echocardiography showed double outlet right ventricle associated with severe infundibular- and pulmonary valve stenosis. The genetic testing has shown a mutation on the LZTR1 gene, which confirms the diagnosis of a Noonan Syndrome, also present in the mother and an elder sister. The infant was born premature at 34 weeks and 5 days of gestational age. During the neonatal period, feeding difficulties are noted linked to oral aversion and exacerbated by difficulties in the mother-child bond. At 1 month of age, the child presented hypoxic spells caused by the infundibular stenosis which required emergency aorto-pulmonary anastomosis placement ensuring sufficient pulmonary blood flow. This anastomosis needed to be replaced by a larger one at 9th month of age. The child is now 4 years old and has undergone a complete surgical correction. The multidisciplinary management englobes not only follow up in cardiology, genetics, neurology, ophthalmology and hematology but also feeding support and psychomotor development support. The socio-economic precariousness of the family leads to a constant assistance to allow the best possible development of the child.

Nous présentons le cas d'une fillette chez qui le diagnostic de cardiopathie congénitale de type ventricule droit à double issue avec communication interventriculaire, sténose infundibulaire et valvulaire pulmonaire sévères a été posé en période fœtale. Le bilan génétique a montré la présence d'une mutation du gène LZTR1, confirmant le diagnostic d'un syndrome de Noonan que présentent également la maman et une sœur aînée. L'enfant naît prématurément à 34 semaines et 5 jours d'aménorrhée. La période néonatale est marquée par des difficultés alimentaires liées à des troubles de l'oralité exacerbés par un attachement mère-enfant compromis. à l'âge de 1 mois, l'enfant présente des malaises hypoxiques en raison de la sténose infundibulaire nécessitant la mise en place en urgence d'une anastomose aorto-pulmonaire assurant un débit pulmonaire suffisant, anastomose qui devra être remplacée par une plus large à l'âge de 9 mois. La fillette actuellement âgée de 4 ans vient de bénéficier d'une cure chirurgicale complète. Le suivi multidisciplinaire comprend, outre les suivis cardiologique et génétique, le support à l'alimentation entérale, le suivi neurologique, ophtalmologique et hématologique ainsi que le soutien à la psychomotricité. La précarité psycho-socio-économique familiale nécessite une aide constante pour permettre à l'enfant d'évoluer favorablement dans son milieu familial.

Novel echocardiographic score to predict duct-dependency after percutaneous relief of critical pulmonary valve stenosis/atresia.

OBJECTIVES: This study aimed to identify clinical, hemodynamic, or echocardiographic predictive features of persistent duct-dependency of pulmonary circulation (PDDPC) after effective percutaneous relief of pulmonary atresia with the intact ventricular septum (PA-IVS) or critical pulmonary stenosis (CPS). METHODS: From 2010 to 2021, 55 neonates with PA-IVS or CPS underwent percutaneous right ventricle (RV) decompression at our Institution. After successfully relief of critical obstruction, 27 patients (group I) showed PDDPC, whereas RV was able to support the pulmonary circulation in the remaining 28 patients (group II). Clinical, hemodynamic, and echocardiographic features of these two groups were compared. RESULTS: No significant difference in clinical and hemodynamic data was found between the groups, although the group I had a lower oxygen saturation at hospital admission. However, tricuspid valve (TV) diameter <8.8 mm, TV z-score ←2.12, tricuspid/mitral valve annular ratio <.78, pulmonary valve diameter <6.7 mm, pulmonary valve z-score ←1.17, end-diastolic RV area <1.35 cm2 , end-systolic right atrium area >2.45 cm2 , percentage amount of interatrial right-to-left shunt >69.5%, moderate/severe tricuspid regurgitation, RV systolic pressure >42.5 mmHg, tricuspid E/E' ratio >6.6 showed each significant predictive value of PDDPC. These parameters were used to build a composite echocardiographic score (PDDPC-score), assigning one point each above the respective cut-off value. A score ≥4.00 showed high sensitivity (100%) and specificity (86%) in predicting PDDPC. CONCLUSION: Clinical and hemodynamic features fail to predict the short-term fate of the pulmonary circulation after successful treatment of PA-IVS/CPS. However, a simple, composite echocardiographic score is useful to predict PDDPC and could be crucial in the management of this frail subset of patients.

Preserving the pulmonary valve in Tetralogy of Fallot repair: Reconsidering the indication for valve-sparing.

BACKGROUND: Tetralogy of Fallot (TOF) repair is a frequent procedure, and although valve-sparing (VS) repair is preferred, determining which patients can successfully undergo this operation remains controversial. We sought to identify parameters to determine a selective, accurate indication for VS repair. METHODS: We reviewed 71 patients (82%) undergoing VS repair. We analyzed hemodynamic data, intraoperative reports, and follow-up echocardiography results to identify acceptable indications. Patients requiring pulmonary valve (PV) reintervention versus no reintervention were compared. RESULTS: PV annulus size at repair was z-score of -2.0 (-5.3, 1.3). Approximately half (51%) had a z-score less than -2. Cox regression results showed this was not a risk factor for reintervention (p = .59). Overall, 1-, 3-, 5-, and 10-year freedom from PV reintervention rates were 95.8%, 92.8%, 91% and 77.8%, respectively. Residual pulmonary stenosis (PS) at initial repair was relatively higher in the reintervention group compared with no reintervention group (40 [28, 51] mmHg vs. 30 [22, 37] mmHg; p = .08). For patients with residual PS, pressure gradient (PG) was consistent over time across both groups (PV reintervention: -3 [-15, 8] mmHg vs. no reintervention: 0 [-9, 8] mmHg). The risk of PV reintervention is 3.7-fold higher when the PG from intraoperative TEE is greater than 45 mmHg (p = .04). CONCLUSIONS: Our review of the midterm outcomes of expanded indication for VS suggests intraoperative decision to convert to transannular patch is warranted if intraoperative postprocedure TEE PG is greater than 45 mmHg or RV pressure is higher than half of systemic pressure to prevent reintervention.

Levoatriocardinal vein in a patient with transposition of great arteries and pulmonary stenosis.

We report a case of a 3-month-old boy transposition of great arteries, ventricular septal defect, and pulmonary stenosis where computed tomography (CT) angiography detected a "levoatriocardinal vein" connecting the left superior pulmonary vein and the left brachiocephalic vein with a preserved normal connection between the left superior pulmonary vein and left atrium. This case highlights the hemodynamic implications of a "levoatriocardinal vein" as well as the role of CT angiography in detecting such associated cardiovascular anomalies in patients with complex congenital heart defects.

A hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly, and coronary sinus to left atrial communication.

BACKGROUND: We report a hitherto unreported combination of pulmonary stenosis, single coronary artery anomaly and coronary sinus to left atrial communication. Our case highlights the important value of coronary computed tomographic angiography and transthoracic echocardiography for the diagnosis of such anomalies and guidance for proper management. METHODS AND RESULTS: A 64-year-old male presented chest tightness and shortness of breath for 2 days. Transthoracic echocardiography revealed a thickened pulmonary valve leaflet and subvalvular outflow tract stenosis, colour flow Doppler showed a significant accelerated blood flow in the pulmonary artery cavity originating from the subvalvular outflow tract, continuous wave Doppler revealed the transpulmonary valvular pressure gradient of 63mmHg. Computed tomographic angiography image reveals thickened pulmonary valve leaflets and subvalvular outflow tract stenosis, single coronary artery anomaly and levoatriocardinal vein. The patient underwent percutaneous pulmonary valve balloon dilatation, the post-procedural course was uneventful. DISCUSSION: Pulmonary stenosis can occur as part of more congenital cardiac malformations or as rare primary isolated pulmonary stenosis, which includes the valvular, sub-valvular, or supra-valvular pulmonary stenosis. Single coronary artery anomalies are very rare, anomalous right coronary artery originates from proximal to mid-left anterior descending coronary artery is one such single coronary artery anomaly, in most cases, it is asymptomatic, diagnosed incidentally, and a benign entity has a better prognosis except if the right coronary artery is passing between the aorta and pulmonary artery. This course of the right coronary artery anomaly is malignant. Coronary sinus to left atrial communication includes a direct or indirect communication. The direct communication is described as a partial or complete absence of the roof between the coronary sinus and left atrium, as it is well known as the unroofed coronary sinus syndrome. The indirect communication is an anomalous bridging vein communicating the coronary sinus to the left atrium, which can be distinguished from classical unroofed coronary sinus syndrome. The venous collateral channel communication between the coronary sinus to the left atrium by a bridging vein is also categorized as a variant type of unroofed coronary sinus syndrome. Understanding coronary venous variations has significant clinical implications particularly in the realm of electrophysiology. The anatomical variations can have important consequences for procedures such as biventricular pacing and trans-coronary vein ablations. CONCLUSION: Pulmonary stenosis combined with single coronary artery anomaly and bridging vein communication between the coronary sinus and the left atrium is an extremely rare. Coronary computed tomographic angiography and transthoracic echocardiographyplay an important role the diagnosis of such anomalies and guidance for clinical Treatment.

Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.

BACKGROUND: Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway. METHODS: PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair. RESULTS: Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5-62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs). CONCLUSIONS: Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.

Blood cyst of the pulmonary valve causing right ventricular outflow tract obstruction: A case report.

Blood cysts are rare cardiac tumors, often involve the atrioventricular valves. Blood cysts are usually small and tend to be clinically silent. Here, we report a case of blood cyst adhered to the junction of the pulmonary valve and the right ventricular outflow, causing severe outflow obstruction. This case was initially misdiagnosed as pulmonary valve stenosis by transthoracic echocardiography (TTE) and even underwent percutaneous balloon pulmonary valvuloplasty (PBPV). As the right ventricle-to-pulmonary artery peak gradient did not decrease after PBPV procedure, the boy underwent TTE again and the diagnosis was corrected as blood cyst. The cystic mass was successfully resected subsequently, and the boy recovered well.

Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease.

OBJECTIVE: To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease. STUDY DESIGN: Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs. RESULTS: Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation. CONCLUSIONS: Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS.

COVID-19 related callback in blood donors; Outcomes in blood donors and patients.

Call back as a procedure to report post donation symptoms or illness by donors has been established since 2009 in Iranian Blood Transfusion Organization (IBTO). During the first phase of COVID-19 outbreak, all blood donors were requested to report any respiratory infection symptoms after donation. The study investigated the callback data of COVID-19 in Tehran Blood Center during the first 3 months of the outbreak in Iran. The purpose of this study was to estimate the frequency of post donation COVID-19 related call back reports and determine its implications for blood donors and patients. A telephone interview was conducted with donors who had reported COVID-19 symptoms. Some questions were asked to evaluate donor's health at the time of blood donation. The donors categorized into three groups: laboratory-confirmed, suspected, and COVID-19 irrelevant based on their answers. In cases that the blood component obtained from a laboratory-confirmed donor had been released, the hospital was notified and asked to follow up the recipient for COVID-19. The results showed 30 donors (0.08 %) had callback related to COVID-19 and 76.63 % of the obtained component was disposed. The results also showed that only one donor had a laboratory-confirmed result with the RBC unit processed from her whole blood released for transfusion. The RBC unit recipient did not show any signs or symptoms of infection during a 46-day follow-up. Concluded that callback system was effective to remove most of the components obtained from the donors who reported to be COVID-19 suspected or confirmed. Moreover, the result did not support virus transmission through blood transfusion.