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      A Case of the TOF with APV Complicated with Polyhydramnios and Severe Bronchomalacia

      case-report

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          Abstract

          Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease with severe pulmonary insufficiency, characterized with aneurysmal dilation in the pulmonary artery and one or both of its branches. We presented a rare case with APVS and literature review in this letter. Prenatal USG examination of the fetus at the 26th week of gestation revealed severe polyhydramnios, dilatation at right ventricle, and abnormal appearance of the heart. At the 31st gestational week, the baby was born with cesarean section. The newborn had right heart failure but had no hydrops fetalis. Therefore, severe respiratory distress observed in the infant has been associated with pulmonary complications. The infant, who had respiratory acidosis according to blood gas analysis, was intubated and attached to mechanical ventilator. Despite progressively increased respiratory support and other interventions, the infant died on the 3rd day of admission. Compression against bronchial tree and esophagus due to dilated pulmonary artery and its branches may inevitably lead to bronchomalacia and polyhydramnios. In conclusion, presence of polyhydramnios and the possibility of severe bronchomalacia should be kept in mind; and due to the risk of early neonatal mortality, delivery should be performed in a center where pediatric heart surgery is available.

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          Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus.

          To assess in a population of 21 fetuses diagnosed with absent pulmonary valve syndrome (APVS) the accuracy of prenatal diagnosis, the incidence of extracardiac and chromosomal anomalies and the perinatal outcome. This was a retrospective observational study of 21 fetuses with a confirmed diagnosis of APVS. All of them underwent fetal echocardiography and a detailed anatomical scan. Karyotyping was performed in 20/21 cases, with fluorescent in-situ hybridization analysis to detect the 22q11 microdeletion performed in 16/21 cases. The following variables were retrieved from databases and evaluated: indication for referral, gestational age at diagnosis, presence of cardiomegaly, branch pulmonary dilatation, associated anomalies or intrauterine growth restriction, and fetal/neonatal outcome. Autopsy reports and postnatal surgical/medical files were available for confirmation in all cases. Prenatal diagnosis of APVS proved correct in all cases, with only three cases occurring not in association with tetralogy of Fallot. Additional cardiovascular anomalies were present in five cases (24%). Extracardiac anomalies were found in nine cases (42.8%), and were associated with chromosomal anomalies in five cases (24%). The 22q11 microdeletion was present in 4/16 cases (25%). Fetal/neonatal outcome was as follows: nine terminations of pregnancy, three intrauterine deaths, six postnatal deaths. The remaining three (14.3%) neonates were alive after surgery. Cardiomegaly and marked branch pulmonary dilatation were present in 16 and 15 cases, respectively, and were associated with bronchomalacia in virtually all cases. APVS can be reliably diagnosed and characterized prenatally. The association with major chromosomal anomalies or 22q11 microdeletion is consistent with previous findings. The relatively poor survival rate is due to the high rate of terminations, associated genetic anomalies and bronchomalacia. Bronchomalacia is present in the overwhelming majority of cases featuring cardiomegaly and marked branch pulmonary dilatation.
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            Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve.

            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. 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. 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%). APVS can be accurately diagnosed by fetal echocardiography but screening ultrasound in the mid-second trimester is likely to have a low detection rate, probably due to the incomplete expression of the disease at this point. Many fetuses with APVS have an increased NT in the first trimester and this may help an earlier recognition of the defect. The most common associated karyotype anomaly is 22q11 microdeletion. Enlargement of the central pulmonary arteries is mainly related to the gestational age at diagnosis. Our results confirm that the outlook for these patients is extremely poor. 2006 ISUOG. Published by John Wiley & Sons, Ltd.
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              Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience.

              Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation.
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                Author and article information

                Journal
                Case Rep Obstet Gynecol
                Case Rep Obstet Gynecol
                CRIOG
                Case Reports in Obstetrics and Gynecology
                Hindawi Publishing Corporation
                2090-6684
                2090-6692
                2016
                7 August 2016
                : 2016
                : 3641453
                Affiliations
                1Department of Obstetrics and Gynecology, Dumlupinar University School of Medicine, 43040 Kutahya, Turkey
                2Department of Pediatrics, Dumlupınar University School of Medicine, 43040 Kutahya, Turkey
                3Department of Pediatric Cardiology, Dumlupınar University Kutahya Evliya Çelebi Training and Research Hospital, Kutahya, Turkey
                Author notes

                Academic Editor: Svein Rasmussen

                Author information
                http://orcid.org/0000-0002-4131-7632
                http://orcid.org/0000-0003-3762-4418
                Article
                10.1155/2016/3641453
                4992531
                16aece41-37ba-456c-b4b1-9cc1b7899015
                Copyright © 2016 Ali Seven et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 March 2016
                : 26 June 2016
                : 30 June 2016
                Categories
                Case Report

                Obstetrics & Gynecology
                Obstetrics & Gynecology

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