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      Sudden Intrapartum Fetal Death in Fetuses with Absent Pulmonary Valve Syndrome: Report of Two Cases

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          Abstract

          Objective To describe potential intrapartum complications for fetuses affected by absent pulmonary valve syndrome.

          Study Design Two cases of intrapartum fetal death at full term were collected from our institution's labor and delivery unit records.

          Results In both cases of intrapartum fetal death, the fetuses had been diagnosed with absent pulmonary valve syndrome and had likely experienced acute cardiac events during labor. Both were delivered as stillbirths despite emergency cesarean delivery.

          Conclusion Patients should be counseled prior to labor about potential intrapartum complications for a fetus with absent pulmonary valve syndrome. Plans for fetal monitoring and the extent of aggressive intervention should be in place before labor in case sudden complications occur.

          Most cited references10

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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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            "Agenesis" of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve.

            Four infants with the severe form of the syndrome of tetralogy of Fallot and absent pulmonary valve are described. All had severe respiratory distress due to bronchial compression from massively dilated pulmonary arteries. The clinical diagnosis was confirmed by cardiac catheterization and angiocardiography. The infants died at age 3, 5, 30 and 90 days, respectively. Autopsy revealed, in addition to the usual components of the syndrome, absence of the ductus arteriosus or its remnants. The possible relation of "agenesis" of the ductus arteriosus to the pathogenesis of the severe form of this syndrome is discussed and pertinent studies are reviewed.
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              Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex.

              We describe clinical and echocardiographic features of tetralogy of Fallot with absent pulmonary valve complex (TOF/APVC) and hypothesized that outcome might be related to pulmonary artery enlargement or severity of illness. We examined the clinical records of all 23 patients evaluated at our institution before death or surgical correction of TOF/APVC between 1990 and 2000. Echocardiograms for 16 patients (including 5 fetuses) were also reviewed, and measurements of the semilunar valves and pulmonary arteries were obtained and compared with patient's aortic annulus size and with established normal subjects. Actuarial survival was 15 of 23 patients (68%) at 4 years. Four fetuses were hydropic and none survived; 7 patients were ventilator dependent at operation and only 3 survived. No difference was noted in pulmonary artery diameters in survivors versus nonsurvivors. Pulmonary valve annulus size was larger in nonsurvivors (103 +/- 25% vs 71 +/- 24% of normal, p = 0.03); however, when fetal examinations were excluded, this difference did not persist. Thus, only hydrops and ventilator dependence at diagnosis predicted mortality. There was no correlation between postnatal measurements of pulmonary arteries and outcome. Larger pulmonary annulus size in hydropic fetuses and poor survival among patients diagnosed in utero suggests that the pathophysiology in TOF/APVC is not due entirely to the aneurysmal dilation of the pulmonary arteries but may be related to right-sided cardiac dysfunction.
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                Author and article information

                Journal
                AJP Rep
                AJP Rep
                AJP Reports
                Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                2157-6998
                2157-7005
                26 April 2012
                November 2012
                : 2
                : 1
                : 39-42
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, Tufts Medical Center, Boston, Massachusetts
                [2 ]Department of Maternal Fetal Medicine, Tufts Medical Center, Boston, Massachusetts
                [3 ]Department of Pediatrics, Tufts Medical Center, Boston, Massachusetts
                Author notes
                Address for correspondence and reprint requests Susan F. Wilson, M.D. Department of Obstetrics and Gynecology, Tufts Medical Center 800 Washington Street, Box 022Boston, MA 02111 swilson1@ 123456tuftsmedicalcenter.org
                Article
                02039
                10.1055/s-0032-1311989
                3653510
                23946904
                7d87f228-7a8e-46d4-8957-d8f352e89d60
                © Thieme Medical Publishers
                History
                : 20 April 2011
                : 24 June 2011
                Categories
                Article

                absent pulmonary valve syndrome,intrapartum fetal demise,high-risk pregnancy

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