Echocardiographic Predictors of Outcome in Fetuses with Pulmonary Atresia with Intact Ventricular Septum (original) (raw)
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Ultrasound in Obstetrics and Gynecology, 2008
K E Y W O R D S: congenital heart disease; fetal echocardiography; pulmonary atresia with intact ventricular septum (PAIVS); tricuspid regurgitation; ventriculocoronary connections (VCC) ABSTRACT Objective Pulmonary atresia with intact ventricular septum (PAIVS) is a rare cardiac malformation with a broad spectrum of anatomical manifestations, varying from types suitable for biventricular repair to those with diminutive right ventricle and primitive ventriculocoronary connections (VCC), more suitable for single-ventricle palliation or heart transplantation. We sought to test whether tricuspid regurgitation is an easily detectable prenatal criterion with which to identify PAIVS patients at lower risk of needing postnatal single-ventricle palliation. Method We identified retrospectively patients with both fetal diagnosis and postnatal confirmation of PAIVS who were seen at Bambino Gesù Hospital between January 2000 and December 2006. Tricuspid valve/mitral valve (TV/MV) ratio, presence and severity of tricuspid regurgitation and direct visualization of VCC were evaluated by echocardiography both pre-and postnatally. Results We identified 22 patients with a prenatal diagnosis of PAIVS. Four pregnancies were terminated and one fetus was lost to follow-up, leaving 17 patients for the analysis. Based on postnatal cardiac catheterization and/or echocardiography we divided our population in two groups: Group 1 included 10 patients with VCC; Group 2 included seven patients without VCC. At fetal echocardiography, tricuspid regurgitation was absent in all ten Group 1 patients and present in all seven Group 2 patients. VCC were seen directly in 6/10 Group 1 patients and in no Group 2 patients. A cut-off value of 0.56 for the TV/MV ratio was highly predictive of VCC during fetal life, with a sensitivity of 100% and a specificity of 90%. Conclusions The absence of tricuspid regurgitation in fetuses affected by PAIVS is a strong prenatal echocardiographic predictor of VCC, as is a TV/MV ratio < 0.56. Fetuses presenting with tricuspid regurgitation and relatively large right ventricle are at lower risk of needing single-ventricle palliation postnatally. This information could be helpful for appropriate prenatal counseling and postnatal decision-making.
Fetal Diagnosis and Therapy
Introduction: Invasive fetal cardiac intervention (FCI) for pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (PS) has been performed with small single-institution series reporting technical and physiological success. We present the first multicenter experience. Objectives: Describe fetal and maternal characteristics of those being evaluated for FCI, including pregnancy/neonatal outcome data using the International Fetal Cardiac Intervention Registry (IFCIR). Methods: We queried the IFCIR for PAIVS/PS cases evaluated from January 2001 to April 2018 and reviewed maternal/fetal characteristics, procedural details, pregnancy and neonatal outcomes. Data were analyzed using standard descriptive statistics. Results: Of the 84 maternal/fetal dyads in the registry, 58 underwent pulmonary valvuloplasty at a median gestational age of 26.1 (21.9–31.0) weeks. Characteristics of fetuses undergoing FCI varied in terms of tricuspid valve (TV) size, TV regurgi...
Pulmonary stenosis and atresia with intact ventricular septum during prenatal life
Ultrasound in Obstetrics and Gynecology, 2003
K E Y W O R D S: congenital heart disease; fetus; prenatal diagnosis; pulmonary atresia; pulmonary stenosis ABSTRACT Aim To identify fetal echocardiographic characteristics predictive of perinatal outcome in cases with a prenatal diagnosis of pulmonary stenosis or pulmonary atresia.
Ultrasound in Obstetrics & Gynecology, 2019
Objectives: Quantile score is a new reference system for prenatal diagnosis of fetal cardiovascular diseases. The study was to explore the new diagnostic and screening method in fetuses with tetralogy of Fallot (TOF) based on normal fetal q score data model. Methods: A total of 192 cases with TOF were collected and their echocardiographic examinations were retrospectively analysed. Q-score values of 48 fetal echocardiographic variables were calculated in each case. Forty-eight variables from 6272 normal fetal echocardiographic exams were used to determine the normal range of q score values. The core diagnostic variables of fetal TOF were obtained by statistical analysis in forward selection method and Naive Bayes classification, including the variables and combinations of variables within screening and diagnostic demand. Results: The four core diagnostic variables selected by statistical analysis were aortic diameter, pulmonary artery, inferior vena cava S wave. If the first three variables were used to diagnose fetal TOF, the area under the ROC curve is 0.90. If the diagnostic sensitivity of more than 90% is guaranteed, the above three parameters should be used together in the range of false positive rate ≤3%; in the false positive rate≤ 5% within the scope of the joint use of these three parameters can be achieved more than 95%. Conclusions: The diagnostic echo parameters acquired by medical big data statistical methods have higher diagnostic precision and the acquisition of core variables has the advantages of objectivity, facticity and intelligence.
Arquivos Brasileiros de Cardiologia, 2000
A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.
Ultrasound in Obstetrics & Gynecology, 2002
BackgroundRight ventricular outflow obstruction with an intact ventricular septum leads to severe hemodynamic problems in the newborn, with hypoxemia and congestive heart failure due to tricusp regurgitation. Atresia of the pulmonary valve occurs in less than 3% of all congenital heart lesions, with a frequency of 1:25 000 of live births. Use of fetal echocardiography permits prenatal diagnosis of this and other congenital defects, for a better knowledge and therapy.Case report35-year-old pacient, with a previous newborn with an interventricular comunication. On her 30 week of pregnancy, a fetal echocardiography showed atresia of the pulmonary valve with an intact interventricular septum, severe tricuspid regurgitation, a right atrial dilatation, restriction of flow in the oval foramen of 8 m/seg and ductus arterioso with retrograde or bidirectional flow, from the aorta to the pulmonary circulation. Vaginal delivery occurred at 38 weeks, obtaining a female newborn, 2630 g, in good conditions. However she developed severe cardiac failure and died at day 4, in spite of pharmacologic with prostaglandins and surgical therapy.ConclusionsEchocardiography is the basis of prenatal diagnosis of congenital heart disease, like pulmonary valve atresia, and is of great help to understand and decide the best perinatal therapy.
Anatomic Variability and Outcome in Prenatally Diagnosed Absent Pulmonary Valve Syndrome
The Annals of Thoracic Surgery, 2014
Background. We sought to describe current outcomes and risk factors for mortality for fetuses diagnosed with absent pulmonary valve syndrome (APV). Fetuses with APV were divided into two cohorts, those with underlying tetralogy of Fallot (TOF/APV) and those without underlying TOF and either an intact ventricular septum or small ventricular septal defect (APV/IVS). Methods. The fetal echocardiographic database was reviewed from January 1, 2001, until June 1, 2010, and all subjects with a diagnosis of APV were included. Multiple clinical and fetal echocardiographic measurements were recorded. Statistical analysis was performed by c 2 analysis and t tests. Survival analysis was performed by Kaplan-Meier analysis. Significant relationships between variables were explored by regression analysis. Significance was set at p [ 0.05. Results. The cohort consisted of 15 fetuses with TOF/APV and 6 fetuses with APV/IVS. There were no
Pediatric Cardiology, 2019
Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74-86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein's anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short-and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein's anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.
Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve
K E Y W O R D S: absent pulmonary valve syndrome; nuchal translucency; outcome; prenatal diagnosis ABSTRACT Objectives To analyze fetal echocardiographic findings of absent pulmonary valve syndrome (APVS), its association with chromosomal and extracardiac anomalies including nuchal translucency (NT) and the outcome after diagnosis. Methods Data of 14 fetuses with confirmed APVS retrospectively collected in two tertiary referral centers between 1998 and 2004 were analyzed. The variables examined were: reason for referral, gestational age at diagnosis and associated abnormalities, including first trimester NT thickness. Cardiac evaluation included measurement of cardiothoracic ratio, diameter of pulmonary arteries and Doppler flow in the pulmonary trunk. Information was retrieved from clinical files, recorded videotapes and stored images. Karyotyping including examination for the 22q11 deletion was performed in all cases. Results Mean gestational age at diagnosis was 28 weeks, with 5/14 (36%) diagnosed before 22 weeks. In 13/14 (93%) there was an associated ventricular septal defect (subaortic in 12 fetuses and inlet-type in one) and all 13 had tetralogy of Fallot. Enlargement of the central pulmonary arteries and cardiomegaly were present in all cases diagnosed after 22 weeks. Of the five fetuses in which APVS was detected before 22 weeks, four (80%) had a normal pulmonary trunk diameter, two (40%) had normal pulmonary branches and three (60%) had normal cardiac size. The arterial duct was absent in 11/14 (79%). A correlation between presence of the arterial duct and the size of the central pulmonary arteries or cardiomegaly could not be established. Increased NT was observed in 4/10 cases (40%) for which this information was available. 22q11 microdeletion was diagnosed in three fetuses (21%). There were five terminations of pregnancy, one intrauterine death, five neonatal deaths and one infant death. Of the six neonates with respiratory distress, only one (17%) survived and of the eight babies in whom there was an intention to treat, two survived (25%).