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Explantation of the Venus P-valve: a first in-human experience

Saram SJ, Leong MC, Sivalingam S

Interact Cardiovasc Thorac Surg, 2021 10 04;33(4):671.
PMID: 34606583 DOI: 10.1093/icvts/ivab129
Matched MeSH terms: Pulmonary Valve Insufficiency*
Fulltext A Novel Restorative Pulmonary Valve Conduit: Early Outcomes of Two Clinical Trials

Morales DL, Herrington C, Bacha EA, Morell VO, Prodán Z, Mroczek T, et al.

Front Cardiovasc Med, 2020;7:583360.
PMID: 33748192 DOI: 10.3389/fcvm.2020.583360

Objectives: We report the first use of a biorestorative valved conduit (Xeltis pulmonary valve-XPV) in children. Based on early follow-up data the valve design was modified; we report on the comparative performance of the two designs at 12 months post-implantation. Methods: Twelve children (six male) median age 5 (2 to 12) years and weight 17 (10 to 43) kg, had implantation of the first XPV valve design (XPV-1, group 1; 16 mm (n = 5), and 18 mm (n = 7). All had had previous surgery. Based on XPV performance at 12 months, the leaflet design was modified and an additional six children (five male) with complex malformations, median age 5 (3 to 9) years, and weight 21 (14 to 29) kg underwent implantation of the new XPV (XPV-2, group 2; 18 mm in all). For both subgroups, the 12 month clinical and echocardiographic outcomes were compared. Results: All patients in both groups have completed 12 months of follow-up. All are in NYHA functional class I. Seventeen of the 18 conduits have shown no evidence of progressive stenosis, dilation or aneurysm formation. Residual gradients of >40 mm Hg were observed in three patients in group 1 due to kinking of the conduit (n = 1), and peripheral stenosis of the branch pulmonary arteries (n = 2). In group 2, one patient developed rapidly progressive stenosis of the proximal conduit anastomosis, requiring conduit replacement. Five patients in group 1 developed severe pulmonary valve regurgitation (PI) due to prolapse of valve leaflet. In contrast, only one patient in group 2 developed more than mild PI at 12 months, which was not related to leaflet prolapse. Conclusions: The XPV, a biorestorative valved conduit, demonstrated promising early clinical outcomes in humans with 17 of 18 patients being free of reintervention at 1 year. Early onset PI seen in the XPV-1 version seems to have been corrected in the XPV-2, which has led to the approval of an FDA clinical trial. Clinical Trial Registration: www.ClinicalTrials.gov, identifier: NCT02700100 and NCT03022708.

Matched MeSH terms: Pulmonary Valve; Pulmonary Valve Insufficiency
Personalized external aortic root support in aneurysm disease

Treasure T, Austin C, Kenny LA, Pepper J

Curr Opin Cardiol, 2022 Nov 01;37(6):454-458.
PMID: 36094493 DOI: 10.1097/HCO.0000000000000990

PURPOSE OF REVIEW: To bring together and annotate publications about personalised external aortic root support reported in the 18 months preceding submission.
RECENT FINDINGS: The total number of personalised external aortic root support (PEARS) operations is now approaching 700 in 30 centres in Australia, Belgium, Brazil, Czech Republic, Great Britain, Greece, Ireland, Malaysia, Netherlands, New Zealand, Poland and Slovakia. There are continued reports of stability of aortic dimensions and aortic valve function with the only exceptions known being where the surgeon has deviated from the instructions for use of the device. The median root diameter of Marfan patients having PEARS was 47 mm suggesting that the existing criterion of 50 mm is due for reconsideration. The peri-operative mortality currently estimated to be less than 0.3%. The first recipient remains alive and well after 18 years. The use of PEARS as an adjunct to the Ross operation to support the pulmonary autograft is being explored in several centres.
SUMMARY: The operation requires proctoring and adherence to a strict operative protocol and with those precautions excellent results are attained. The evidence and opinions provided in the cited publications indicate that PEARS is a proven and successful prophylactic operation for aortic root aneurysm.

Matched MeSH terms: Pulmonary Valve*
Stenting the patent ductus arteriosus in duct-dependent pulmonary circulation: techniques, complications and follow-up issues

Alwi M

Future Cardiol, 2012 Mar;8(2):237-50.
PMID: 22413983 DOI: 10.2217/fca.12.4

Maintaining ductal patency in duct-dependent congenital heart lesions by implantation of coronary stents is an alternative to systemic pulmonary shunt in selected cases and lesions with suitable anatomy. This article focuses on the procedure as the initial palliation in duct-dependent pulmonary circulation, its associated pitfalls and complications. A good understanding of the diverse duct morphology is paramount prior to stenting of the ductus. Long tortuous duct, insufficiently constricted ductus at the pulmonary end and ductus with associated branch pulmonary artery stenosis at the site of insertion are not suitable for stenting. Durability of palliation is generally inferior to a surgical shunt and this may dictate earlier definitive surgical repair. Acceleration of branch pulmonary artery stenosis in certain ductal morphology limits its general applicability. Bioabsorbable and biodegradable stents may offer some solution to this problem.

Matched MeSH terms: Pulmonary Valve Stenosis/radiography; Pulmonary Valve Stenosis/surgery; Pulmonary Valve Stenosis/therapy*
Fulltext Absent pulmonary valve syndrome--a case report

Ram SP, Lim MK, Mazeni A

Med J Malaysia, 1994 Mar;49(1):93-5.
PMID: 8057999

A 7-year old female child was admitted for recurrent bronchopulmonary since one week of life. She was diagnosed to have ventricular septal defect and was treated conservatively. At seven years of life, repeat echocardiogram revealed a large perimembranous ventricular septal defect, absent pulmonary valve with overriding of aorta, narrow pulmonary artery annulus, and dilated main pulmonary artery and its branches. She was treated conservatively, discharged and follow-up at the National Heart Institute Kuala Lumpur, for corrective surgery.

Matched MeSH terms: Pulmonary Valve/abnormalities*; Pulmonary Valve/surgery; Pulmonary Valve/ultrasonography
Initial Clinical Trial of A Novel Pulmonary Valved Conduit

Prodan Z, Mroczek T, Sivalingam S, Bennink G, Asch FM, Cox M, et al.

Semin Thorac Cardiovasc Surg, 2021 May 10.
PMID: 33984478 DOI: 10.1053/j.semtcvs.2021.03.036

Valved allografts and xenografts for reconstruction of the right ventricular outflow tract (RVOT) lack durability and do not grow. We report the first clinical use of a completely bioabsorbable valved conduit (Xeltis pulmonary valve - XPV) in children. Twelve children (six male), median age five (two to twelve) years and median weight 17 (10 to 43) kg, underwent RVOT reconstruction with the XPV. Diagnoses were: pulmonary atresia with ventricular septal defect (VSD) (n=4), tetralogy of Fallot (n=4), common arterial trunk (n=3), and transposition of the great arteries with VSD and pulmonary stenosis (n=1). All had had previous surgery, including prior RVOT conduit implantation in six. Two diameters of conduit 16mm (n=5) and 18mm (n=7) were used. At 24 months none of the patients has required surgical re-intervention, 9 of the 12 are in NYHA functional class I and three patients in NYHA class II. None of the conduits has shown evidence of progressive stenosis, dilation or aneurysm formation. Residual peak gradient of >40 mm Hg was observed in three patients, caused by kinking of the conduit at implantation in 1 and distal stenosis in the peripheral pulmonary arteries in 2 patients. Five patients developed severe pulmonary valve insufficiency (PI); the most common mechanism was prolapse of at least one of the valve leaflets. The XPV conduit is a promising innovation for RVOT reconstruction. Progressive PI requires however an improved design (geometry, thickness) of the valve leaflets.

Matched MeSH terms: Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis
Infolding of the Venus P-Valve After Transcatheter Pulmonary Valve Implantation

Riahi M, Ang HL, Jones M, Prachasilchai P, Baruteau AE, Promphan W, et al.

Circ Cardiovasc Interv, 2018 04;11(4):e005923.
PMID: 29618579 DOI: 10.1161/CIRCINTERVENTIONS.117.005923
Matched MeSH terms: Pulmonary Valve/physiopathology; Pulmonary Valve/surgery*; Pulmonary Valve Insufficiency/physiopathology; Pulmonary Valve Insufficiency/surgery*
Fulltext Pulmonary valve echocardiogram in the evaluation of pulmonary arterial hypertension in the presence of intracardiac shunts

Fong CY, Singham KT

Med J Malaysia, 1986 Sep;41(3):198-204.
PMID: 3670135

The purpose of this study was to assess M-mode echocardiographic criteria for pulmonary artery hypertension in patients with intracardiac shunts, and to observe any signs not previously described.
Forty-seven consecutive cardiac cathetherised patients with routine M-mode echocardiogram done were reviewed. Twenty-six patients had atrial septal defect, nine had ventricular septal defect (VSD), four had patent ductus arteriosus (PDA), five had Eisenmenger syndrome and three had combined VSD and PDA.
Fifteen of 20 patients with pulmonary artery mean pressure (PAMP) of more than 50 mmHg demonstrated diminished a-dip (p < 0.001), reduced e-f slope (p < 0.01) and increased b-e slope to > 380 mm/sec (p < 0.01). The presence of systolic flutter or mid-systolic notch, or both was not observed in patients with normal PAMP, but was noted in all of the 20 patients with raised pressure(> 50 mmHg).
This study confirms the limitation of M-mode echocardiographic features in the evaluation of pulmonary artery hypertension in patients with intracardiac shunt, though clinically useful.

Matched MeSH terms: Pulmonary Valve/physiopathology*
Fulltext Cardiopathy and ocular abnormalities in Noonan syndrome

Ram SP, Krishna TN

Singapore Med J, 1994 Aug;35(4):397-9.
PMID: 7899901

We describe three children with Noonan syndrome with cardiopathy. One female child had cardiopathy and ocular abnormalities. The other two male children had congenital heart disease of which one had uncommon association of tricuspid valve dysplasia with regurgitation associated with endocardial cushion defect. Karyotypes of the female and one of the male children were normal. The growth hormone and thyroid hormone studies in the first and second male children were normal. All the three children were managed conservatively and followed-up.

Matched MeSH terms: Pulmonary Valve Stenosis/pathology
Aneurysmal dilatation without distal obstruction: a rare complication of valved bovine jugular vein conduit

Qureshi AU, Abbaker AE, Sivalingam S, Latiff HA

World J Pediatr Congenit Heart Surg, 2014 Apr;5(2):348-51.
PMID: 24668992 DOI: 10.1177/2150135113509819

Valved bovine jugular vein (Contegra) conduit is considered a suitable choice for pediatric population with congenital heart defect requiring right ventricle to main pulmonary artery connection. Intermediate follow-up studies have shown the propensity of developing distal conduit stenosis and valve thrombosis. We present a rare case of aneurysmal dilatation of the conduit leading to valve failure requiring conduit explantation.

Matched MeSH terms: Pulmonary Valve/radiography; Pulmonary Valve/surgery
Pulmonary atresia with intact septum: the use of Conquest Pro coronary guidewire for perforation of atretic valve and subsequent interventions

Alwi M, Budi RR, Mood MC, Leong MC, Samion H

Cardiol Young, 2013 Apr;23(2):197-202.
PMID: 22640635 DOI: 10.1017/S1047951112000595

Objective: To determine the feasibility and safety of the Conquest Pro wire as an alternative to radiofrequency wire for perforation of atretic pulmonary valve and subsequent balloon dilatation and patent ductus arteriosus stenting in patients with pulmonary atresia with intact ventricular septum.
Background: Radiofrequency valvotomy and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum in many institutions today.
Methods: We report eight consecutive patients in whom we used the Conquest Pro coronary guidewire, a stiff wire normally reserved for revascularisation of coronary lesions with chronic total occlusion, for perforation of atretic pulmonary valve and subsequent balloon dilatation, and stenting of the patent ductus arteriosus.
Results: Perforation of atretic pulmonary valve was successful in seven out of eight cases. Radiofrequency valvotomy was employed after failure of perforation by the Conquest Pro wire in one case where the right ventricular outflow tract was broad based and tapered towards the pulmonary valve, and was heavily trabeculated. Failure of the Conquest Pro wire to perforate the pulmonary valve plate was mainly attributed by the failure to engage the wire at the correct position.
Conclusion: The Conquest Pro wire for perforation and subsequent interventions in the more straightforward cases of pulmonary atresia with intact ventricular septum is effective and safe, simplifying the entire procedure. However, the radiofrequency generator and wires remain essential tools in the paediatric interventional catheter laboratory.

Matched MeSH terms: Pulmonary Valve/surgery*
Fulltext Surgical outcomes of absent pulmonary valve syndrome: An institutional experience

Nair AK, Haranal M, Elkhatim IM, Dillon J, Hew CC, Sivalingam S

Ann Pediatr Cardiol, 2020 06 23;13(3):212-219.
PMID: 32863656 DOI: 10.4103/apc.APC_111_19

Background: Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms. We report the mid-term outcomes of surgical correction of patients with APVS.
Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients.
Results: The overall 10-year survival was 82.1%. There was 81.1% overall freedom from re-intervention at 10 years. No statistically significant difference was found in 10-year survival (P = 0.464) and reoperation rates (P = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival (P = 0.039) and freedom from re-intervention (P = 0.016) compared to infants. Patients without respiratory complications had 100% 10-year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications.
Conclusion: There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems.

Matched MeSH terms: Pulmonary Valve