Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum

Mishima, Akira; Asano, Masahisa; Sasaki, Shigeru; Yamamoto, Shigeki; Saito, Takayuki; Ukai, Tomohiko; Suzuki, Yoshimasa; Manabe, Tadao

doi:10.1007/bf03218112

Cited by 26 publications

(8 citation statements)

References 25 publications

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“…These parameters ultimately determine the postnatal circulation and the type of postnatal repair, namely, the BV repair or 1.5 ventricle or UV palliation. If the type of postnatal repair also determines the long-term prognosis that is discussed contradictorily, as overall long-term outcome is as good in the UV as in the BV group with 81 and 86% survival rates after 15 years, respectively [7,11,44], and 95% of survivors with UV palliation live with NYHA functional class I or II [27,45]. Survival rate in our cohort was 69.4% overall and 77.3% intention-to-treat; however, postnatal demise occurred exclusively in neonates and children that were candidates for UV repair.…”

Section: Discussionmentioning

confidence: 99%

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

et al. 2019

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Purpose: To assess the intrauterine course, the outcome, and to establish a new prenatal echocardiographic scoring system to predict biventricular (BV) versus univentricular (UV) outcome of fetuses with severe pulmonary stenosis or atresia with intact ventricular septum (PSAIVS). Methods: All cases of PSAIVS diagnosed prenatally over a period of 14 years were retrospectively collected in 2 tertiary referral centers. Results: Forty-nine fetuses with PSIVS (n = 11) or PAIVS (n = 38) were identified prenatally. Nineteen (38.8%) fetuses had additional ventriculocoronary connections (VCCs) and 21 (42.9%) fetuses had right ventricular hypoplasia. Four (8.2%) pregnancies were terminated, 2 (4.1%) ended in intrauterine fetal death, 4 (8.2%) in neonatal death, and 5 (10.2%) children died in infancy or childhood, including one case with compassionate care. Thirty-four of 44 (77.3%) fetuses with the intention-to-treat were alive at latest follow-up, 25 (73.5%) with BV, and 9 (26.5%) with UV circulation. Most significant predictive markers of UV circulation were Vmax of tricuspid regurgitation (TR) < 2 m/s, right ventricle/left ventricle length ratio ≤0.6, and presence of VCC. A scoring system including these 3 markers had 100% sensitivity and 100% specificity predicting an UV outcome if more than one of these criteria was fulfilled. All 25 liveborn infants that were suitable for BV repair survived, whereas only 9 out of 14 candidates for UV repair survived. None of the 14 fetuses with predicted UV outcome would have met the inclusion criteria for fetal intervention, as 10 of them had VCC and the remaining 4 had absent TR or Vmax < 2 m/s. Conclusion: The prognosis of prenatally diagnosed PSAIVS is good if BV circulation can be achieved, while postnatal mortality in UV circulation is high within the first 4 months of life. Postnatal outcome can be predicted prenatally with high accuracy using a simple scoring system. This information is mandatory for parental counseling and may be useful in selecting fetuses for intrauterine valvuloplasty.

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Section: Discussionmentioning

confidence: 99%

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

et al. 2019

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“…Mishima et al 24 reported actuarial survival of 86% at 14 years after a biventricular repair in selected previously palliated patients. All survivors, however, showed progressive right atrial dilatation reflecting impaired right ventricular function with a propensity to arrhythmias.…”

Section: Follow-upmentioning

confidence: 99%

Pulmonary atresia with intact ventricular septum: from fetus to adult

Shinebourne

Rigby²,

Carvalho³

2007

Heart

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“…In the absence of high-grade tricuspid regurgitation (TR) this lesion leads to a hypoplastic right ventricle (RV), which may preclude a biventricular circulation after birth 1,4,5 . Even if biventricular repair can be achieved postnatally, RV function may remain abnormal and the potential of postnatal RV growth is limited 6,7 . Therefore, the goal of fetal pulmonary valvuloplasty is to stimulate and promote prenatal RV growth in order Prenatal intervention in fetuses with PAIVS or CPS 231 to avoid significant RV hypoplasia at birth.…”

Section: Introductionmentioning

confidence: 99%

Immediate effects and outcome of in‐utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis

Tulzer

Arzt

Gitter

et al. 2018

Ultrasound in Obstet & Gyne

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ObjectiveTo 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.MethodsPatients 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.ResultsThirty‐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.ConclusionIn 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.

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Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum

Abstract: Although biventricular repair for pulmonary atresia and intact ventricular septum proved to attain a satisfactory long-term result, it failed to resolve right heart dysfunction. Postoperative arrhythmia was prone to precipitate progressive dilatation of the right atrium.

Cited by 26 publications

References 25 publications

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

Pulmonary atresia with intact ventricular septum: from fetus to adult

Immediate effects and outcome of in‐utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis

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