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Asian Cardiovascular & Thoracic Annals Mar 2022Absence of arterial duct, a sixth aortic arch derivative, plays an important etiologic role in Tetralogy of Fallot with absent pulmonary valve syndrome. When fetal...
Absence of arterial duct, a sixth aortic arch derivative, plays an important etiologic role in Tetralogy of Fallot with absent pulmonary valve syndrome. When fetal ductus is absent, the large right ventricular stroke volume dilates the pulmonary trunk leading to pulmonary regurgitation. A proximal extension of the embryonic insult to the entire left sixth arch causes absence of the left pulmonary artery, a common association of absent pulmonary valve syndrome. On the contrary, absence of right pulmonary artery is not reported in absent pulmonary valve syndrome. A rare combination of tetralogy, absent pulmonary valve syndrome and isolation of a hypoplastic right pulmonary artery offered challenges to diagnosis and management.
Topics: Humans; Pulmonary Artery; Pulmonary Atresia; Pulmonary Valve; Tetralogy of Fallot; Treatment Outcome
PubMed: 33789442
DOI: 10.1177/02184923211006309 -
World Journal For Pediatric &... Jul 2023The surgical treatment of malformed semilunar valves in congenital heart defects is challenging in terms of providing both longevity and the potential to grow with the...
The surgical treatment of malformed semilunar valves in congenital heart defects is challenging in terms of providing both longevity and the potential to grow with the recipient. We investigated a new surgical technique "Trileaflet Semilunar Valve Reconstruction" in an acute porcine model, a technique with geometrical properties that could remain sufficient and allow for some growth with the child. An acute 60-kg porcine model was used. With echocardiography, baseline pulmonary valvular geometry and hemodynamics were investigated. On cardiopulmonary bypass, the pulmonary leaflets were explanted, and the Trileaflet Semilunar Valve Reconstruction was performed with customized homograft-treated pericardial neo-leaflets. Off bypass, hemodynamics was reassessed. Twelve animals were investigated. The neo-valves were found sufficient in ten animals and with minimal regurgitation in two animals. The neo-valve had a peak gradient of 3 ± 2 mm Hg with a peak velocity of 0.8 ± 0.2 m/s. The coaptation in the neo-valve had a mean increase of 4 ± 3 mm, < .001. The neo-valve had a windmill shape in the echocardiographic short-axis view, and the neo-leaflets billowed at the annular plane in the long-axis view. In this acute porcine model, the neo-valve had no clinically significant regurgitation or stenosis. The neo-valve had an increased coaptation, a windmill shape, and leaflets that billowed at the annular plane. These geometric findings may allow for sustained sufficiency as the annular and pulmonary artery dimension increase with the child's growth. Further long-term studies should be performed to evaluate the efficacy and the growth potential.
Topics: Swine; Humans; Animals; Heart Valve Prosthesis Implantation; Heart Valve Prosthesis; Aortic Valve; Pulmonary Valve; Heart Defects, Congenital
PubMed: 37039366
DOI: 10.1177/21501351231166662 -
Journal of Cardiac Surgery Dec 2022The objective of this study was to evaluate the predictive value of main pulmonary artery (MPA) Z score, pulmonary valve annulus (PVA) prop, great aortic valve...
OBJECTIVES
The objective of this study was to evaluate the predictive value of main pulmonary artery (MPA) Z score, pulmonary valve annulus (PVA) prop, great aortic valve annulus (GA) ratio, PVA index (PAI), and PVA area index (PAAI) indicators in patients with Tetralogy of Fallot (TOF) and whether or not to undergo transannular patch (TAP).
METHODS
A retrospective analysis was performed on the clinical data of 263 patients with TOF who underwent radical operations from 2010 to 2021 at Beijing Children's Hospital. Sixteen cases were excluded, and 247 cases (male/female = 155/92) were included in this study. Based on whether TAP was selected intraoperatively, the patients were divided into the TAP group (82/247) and the non-TAP group (165/247). The diameter of the PVA, the aortic valve annulus, and the MPA were measured by echocardiography, and the PVA Z score, MPA Z score, PVA prop, GA ratio, PAI, and PAAI indexes were calculated, and statistical analysis was carried out.
RESULTS
The PVA Z score, MPA Z score, PVA prop, GA ratio, PAI, and PAAI of the TAP group were lower than those of the non-TAP group (p < .0001, p < .0001, p < .0001, p < .0001, p < .0001, and p < .0001). Receiver-operating curve analysis showed that the cut-off value of PVA Z score was -1.96 (area under the curve [AUC]: 0.822; 95% confidence interval [CI]: 0.769-0.874); the cut-off value of MPA Z score was -1.04 (AUC: 0.778; 95% CI: 0.711-0.845); the cut-off value of PVA prop was 0.37 (AUC: 0.812; 95% CI: 0.751-0.874); the cut-off value of GA ratio was 0.64 (AUC: 0.812; 95% CI: 0.750-0.874); the cut-off value of PAI is 0.78 (AUC: 0.812; 95% CI: 0.750-0.874); and the cut-off value of PAAI is 0.4 (AUC: 0.812; 95% CI: 0.750-0.874). Pulmonary valve bicuspid malformation is one reason why predictive models fail to predict the possible avoidance of TAP. Pearson's correlation and linear regression analysis showed that PAI had the strongest correlation with PVA Z score, followed by that between PVA prop and PVA Z score, and the weakest correlation between PAAI and PVA Z score.
CONCLUSIONS
PVA prop, GA ratio, PAI, and PAAI can well predict TAP selection, and the measurement is simple and convenient. Compared with PVA Z score, they are not hindered by other confounding factors, and can well replace the application value of PVA Z score in TAP prediction. The predictive efficacy of PAI and PVA prop is numerically better than the GA ratio, and PAAI, PAI, and PVA prop combined with MPA Z score can improve the predictive value of PAI and PVA prop, respectively. Although various echocardiographic parameters can be used as indicators to predict surgical approach in patients with TOF, PV morphology and tissue characteristics should also be considered.
Topics: Child; Humans; Male; Female; Infant; Tetralogy of Fallot; Retrospective Studies; Pulmonary Valve; Echocardiography; Regression Analysis; Treatment Outcome
PubMed: 36378883
DOI: 10.1111/jocs.17191 -
JACC. Cardiovascular Interventions Jul 2020
Topics: Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Treatment Outcome
PubMed: 32646694
DOI: 10.1016/j.jcin.2020.05.016 -
Interactive Cardiovascular and Thoracic... Feb 2022This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native...
This new and easily reproducible pulmonary valve-sparing technique for the correction of Tetralogy of Fallot is based on a conservative management of the native pulmonary valve to preserve its growth potential. From July 2015 to December 2019, 67 children presenting with a Tetralogy of Fallot were operated consecutively in a single centre using this technique in all cases. A T-shaped infundibulotomy is used to release the anterior pulmonary annulus from any muscular attachment. After myocardial resection and ventricular septal defect closure, an extensive commissurotomy is achieved. Finally, the right ventricular outflow tract remodelling is completed by a shield-shaped bovine patch with an oversized square superior edge, attached directly on the pulmonary valve annulus, with an effect of systolic traction. Sixty patients (89.5%) had a Tetralogy of Fallot repair with preservation of the pulmonary valve. To date, with a median follow-up of 38.2 [14-64] months, no patient has needed a surgical or interventional procedure for pulmonary valve stenosis or regurgitation, with low residual gradients. This procedure could provide a significant increase in native pulmonary valve preservation. Long-term studies are needed to assess pulmonary valve growth and the consequent reduction in surgical or interventional reoperations.
Topics: Animals; Cattle; Child; Humans; Infant; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome
PubMed: 34606581
DOI: 10.1093/icvts/ivab275 -
Journal of the American College of... Jun 2018
Topics: Allografts; Female; Humans; Pregnancy; Pregnancy Outcome; Pulmonary Valve; Pulmonary Valve Insufficiency
PubMed: 29880127
DOI: 10.1016/j.jacc.2018.04.007 -
Echocardiography (Mount Kisco, N.Y.) Feb 2020We present a case of a 70-year-old woman with exertional shortness of breath. A transthoracic echocardiogram showed a large mass on the ventricular side of the pulmonary...
We present a case of a 70-year-old woman with exertional shortness of breath. A transthoracic echocardiogram showed a large mass on the ventricular side of the pulmonary valve. The anatomy of the mass was additionally investigated with a transesophageal echocardiogram, which delineated the anatomical details of the structure. The mass was surgically excised, and histopathology confirmed a cardiac papillary fibroelastoma.
Topics: Aged; Cardiac Papillary Fibroelastoma; Echocardiography, Transesophageal; Female; Fibroma; Heart Neoplasms; Humans; Pulmonary Valve
PubMed: 32045044
DOI: 10.1111/echo.14604 -
Cardiology in the Young Nov 2021Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by...
OBJECTIVES
Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by pre-transcatheter pulmonary valve implantation physiology.
BACKGROUND
Transcatheter pulmonary valve implantation is a widely available option to treat residual or recurrent pulmonary stenosis and pulmonary insufficiency. Transcatheter pulmonary valve implantation acutely increases pulmonary artery size and diastolic pressure in patients with pulmonary insufficiency and acute pulmonary edema has been reported after transcatheter pulmonary valve implantation, possibly related to acute left ventricular volume loading. However, the impact of transcatheter pulmonary valve implantation on left ventricular diastolic function has not been established.
METHODS
Patients who underwent transcatheter pulmonary valve implantation from 2010 to 2017 at our centre were grouped by indication for transcatheter pulmonary valve implantation as pulmonary stenosis, pulmonary insufficiency, or mixed disease. Separate analysis was performed on those who underwent transcatheter pulmonary valve implantation for pulmonary stenosis versus pulmonary insufficiency or mixed disease. Intracardiac haemodynamics immediately before and after transcatheter pulmonary valve implantation and echocardiographic assessment of left ventricular systolic and diastolic function at baseline, 1-day post transcatheter pulmonary valve implantation, and 1-year post transcatheter pulmonary valve implantation were compared between groups.
RESULTS
In 102 patients who underwent transcatheter pulmonary valve implantation, the indication was pulmonary stenosis in 29 (28%), pulmonary insufficiency in 28 (29%), and mixed disease in 44 (43%). There were no significant differences in left ventricular systolic or diastolic function between groups at baseline, immediately after transcatheter pulmonary valve implantation, or 1-year post implantation. The mean pulmonary artery wedge pressure increased equally across groups.
CONCLUSIONS
While patients with pulmonary insufficiency likely have acute left ventricular volume loading following transcatheter pulmonary valve implantation, this does not appear to be haemodynamically significant as transcatheter pulmonary valve implantation was not associated with measurable changes in left ventricular systolic or diastolic function acutely or 1-year post implantation.
Topics: Aortic Valve Stenosis; Diastole; Heart Valve Prosthesis Implantation; Hemodynamics; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Treatment Outcome; Ventricular Function, Left
PubMed: 33722314
DOI: 10.1017/S1047951121001013 -
Fetal and Pediatric Pathology Feb 2019The main characteristics of absent pulmonary valve syndrome (APVS) include the absence or hypoplasia of the pulmonary valve, stenosis of the pulmonary valve annulus, and... (Review)
Review
INTRODUCTION
The main characteristics of absent pulmonary valve syndrome (APVS) include the absence or hypoplasia of the pulmonary valve, stenosis of the pulmonary valve annulus, and aneurysmal dilatation of the pulmonary trunk and its branches. In the more common type 1, the tetralogy of Fallot-like type, there is a ventricular septal defect, overriding aorta, pulmonary arterial dilatation, and absence of ductus arteriosus, The second type has an intact ventricular septum, less pulmonary artery dilatation, and a patent ductus arteriosus, with or without tricuspid atresia.
CASE REPORT
This APVS had an intact ventricular septum with an absent ductus arteriosus.
CONCLUSION
The APVS with intact ventricular septum with an absent ductus arteriosus may represent a third type of APVS.
Topics: Fetus; Heart Defects, Congenital; Humans; Male; Pulmonary Valve
PubMed: 30661433
DOI: 10.1080/15513815.2018.1529066 -
Heart and Vessels Jan 2022Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with...
Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. A systematic literature search was performed to collect clinical and anatomical data of APV with TA/TS. Institutional medical records were retrospectively reviewed to identify APV with TA/TS patients. In a total of 62 (59 reported and 3 new) cases, patent ductus arteriosus was present in 98% of APV patients with TA/TS. A large ventricular septal defect, dilatation of the pulmonary arteries, which is typically found in APV with tetralogy of Fallot, and respiratory distress at birth were rarely reported. Most of the recent cases were successfully managed by the Glenn or Fontan procedure. Coronary artery anomaly and ventricular arrhythmia were more frequently reported as the cause of death or severe neurological sequelae (9/16 and 3/8, respectively). Additional surgical intervention was required in the mid/long-term period in three cases due to left-ventricular outflow obstruction and in two due to aortic dilatation. The Fontan and Glenn procedures improved the survival in the last two decades. In addition to coronary artery anomaly and ventricular arrhythmia, left-ventricular outflow tract obstruction and aortic dilatation should be carefully monitored.
Topics: Constriction, Pathologic; Humans; Pulmonary Atresia; Pulmonary Valve; Retrospective Studies; Tricuspid Atresia
PubMed: 34089363
DOI: 10.1007/s00380-021-01887-y