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Mini-Konno procedure using a 10 mm handmade valve for pulmonary atresia with intact ventricular septum and severe aortic stenosis.

ABSTRACT: We herein report the case of a neonate with pulmonary atresia with intact ventricular septum, right ventricular hypoplasia and severe aortic stenosis. Repetitive aortic valve surgeries in early infancy resulted in early relapse of the aortic stenosis due to the small aortic annulus, bicuspid configuration and duct-dependent pulmonary circulation. The infant underwent a successful mini-Konno procedure using a 10 mm handmade valve and bidirectional Glenn shunt at 6 months of age.

SUBMITTER: Matsuhisa H

PROVIDER: S-EPMC4051372 | biostudies-literature | 2014 Mar

REPOSITORIES: biostudies-literature

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Mini-Konno procedure using a 10 mm handmade valve for pulmonary atresia with intact ventricular septum and severe aortic stenosis.

Matsuhisa Hironori H Oshima Yoshihiro Y Tanaka Akiko A Kamei Naoya N

Interactive cardiovascular and thoracic surgery 20131213 3

We herein report the case of a neonate with pulmonary atresia with intact ventricular septum, right ventricular hypoplasia and severe aortic stenosis. Repetitive aortic valve surgeries in early infancy resulted in early relapse of the aortic stenosis due to the small aortic annulus, bicuspid configuration and duct-dependent pulmonary circulation. The infant underwent a successful mini-Konno procedure using a 10 mm handmade valve and bidirectional Glenn shunt at 6 months of age. ...[more]

PMID: 24336795

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Project description:OBJECTIVE:To assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in-utero RV growth and postnatal outcome. METHODS:Patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1-3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV-VTI)?×?heart rate (HR) and tricuspid regurgitation (TR) velocity. Longitudinal data were collected from only those fetuses followed up in our center. Outcome was assessed using the scoring system as described by Roman et al. for non-biventricular outcome. RESULTS:Thirty-five pulmonary valvuloplasties were performed in our institution on 23 fetuses with PAIVS (n?=?15) or CPS (n?=?8). Median gestational age at intervention was 28?+?4 (range, 23?+?6 to 32?+?1) weeks. No fetal death occurred. Immediately after successful intervention, RV/left ventricular length (RV/LV) ratio (P???0.0001), TV/mitral valve annular diameter (TV/MV) ratio (P???0.001), RV filling time (P???0.00001) and TV-VTI?×?HR (P???0.001) increased significantly and TR velocity (P???0.001) decreased significantly. In fetuses followed longitudinally to delivery (n?=?5), RV/LV and TV/MV ratios improved further or remained constant until birth. Fetuses with unsuccessful intervention (n?=?2) became univentricular, all others had either a biventricular (n?=?15), one-and-a-half ventricular (n?=?3) or still undetermined (n?=?3) outcome. Five of nine fetuses with a predicted non-biventricular outcome, in which the procedure was successful, became biventricular, while two of nine had an undetermined circulation. CONCLUSION:In selected fetuses with PAIVS or CPS, in-utero pulmonary valvuloplasty led immediately to larger RV caused by reduced afterload and increased filling, thus improving the likelihood of biventricular outcome even in fetuses with a predicted non-biventricular circulation. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Project description:BackgroundAortic valve stenosis is the most common type of valvular heart disease in the USA and Europe. Aortic valve stenosis is considered similar to atherosclerotic disease. Some studies have evaluated statins for aortic valve stenosis.ObjectivesTo evaluate the effectiveness and safety of statins in aortic valve stenosis.Search methodsWe searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, Embase, LILACS - IBECS, Web of Science and CINAHL Plus. These databases were searched from their inception to 24 November 2015. We also searched trials in registers for ongoing trials. We used no language restrictions.Selection criteriaRandomised controlled clinical trials (RCTs) comparing statins alone or in association with other systemic drugs to reduce cholesterol levels versus placebo or usual care.Data collection and analysisPrimary outcomes were severity of aortic valve stenosis (evaluated by echocardiographic criteria: mean pressure gradient, valve area and aortic jet velocity), freedom from valve replacement and death from cardiovascular cause. Secondary outcomes were hospitalisation for any reason, overall mortality, adverse events and patient quality of life.Two review authors independently selected trials for inclusion, extracted data and assessed the risk of bias. The GRADE methodology was employed to assess the quality of result findings and the GRADE profiler (GRADEPRO) was used to import data from Review Manager 5.3 to create a 'Summary of findings' table.Main resultsWe included four RCTs with 2360 participants comparing statins (1185 participants) with placebo (1175 participants). We found low-quality evidence for our primary outcome of severity of aortic valve stenosis, evaluated by mean pressure gradient (mean difference (MD) -0.54, 95% confidence interval (CI) -1.88 to 0.80; participants = 1935; studies = 2), valve area (MD -0.07, 95% CI -0.28 to 0.14; participants = 127; studies = 2), and aortic jet velocity (MD -0.06, 95% CI -0.26 to 0.14; participants = 155; study = 1). Moderate-quality evidence showed no effect on freedom from valve replacement with statins (risk ratio (RR) 0.93, 95% CI 0.81 to 1.06; participants = 2360; studies = 4), and no effect on muscle pain as an adverse event (RR 0.91, 95% CI 0.75 to 1.09; participants = 2204; studies = 3; moderate-quality evidence). Low- and very low-quality evidence showed uncertainty around the effect of statins on death from cardiovascular cause (RR 0.80, 95% CI 0.56 to 1.15; participants = 2297; studies = 3; low-quality evidence) and hospitalisation for any reason (RR 0.84, 95% CI 0.39 to 1.84; participants = 155; study = 1; very low-quality evidence). None of the four included studies reported on overall mortality and patient quality of life.Authors' conclusionsResult findings showed uncertainty surrounding the effect of statins for aortic valve stenosis.The quality of evidence from the reported outcomes ranged from moderate to very low. These results give support to European and USA guidelines (2012 and 2014, respectively) that so far there is no clinical treatment option for aortic valve stenosis.

Dataset Information

Mini-Konno procedure using a 10 mm handmade valve for pulmonary atresia with intact ventricular septum and severe aortic stenosis.

Publications

Mini-Konno procedure using a 10 mm handmade valve for pulmonary atresia with intact ventricular septum and severe aortic stenosis.

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