Pregnant women with complete atrioventricular block: perinatal risks and review of management.

We have summarized the care management of pregnant women with complete atrioventricular block (CAVB) by reviewing data from the published literature as well as our own experience in 32 pregnancies. Obstetrical management of women with a permanent pacemaker implanted prior to conception has been sufficiently reported thus far, and the management of such patients is considered to be of low risk. Since CAVB usually does not cause any specific obstetrical problems during pregnancy, prepregnancy prophylactic placement of a permanent pacemaker is not indicated in all asymptomatic patients. However, when asymptomatic women without pacemakers become pregnant, there is a subset that ultimately develops heart failure during pregnancy. Therefore, close surveillance of pregnant patients with CAVB is warranted. The current increase in the use of permanent pacemakers in young women with symptomatic CAVB will certainly limit the need for intrapartum temporary pacing in patients who do not require a pacemaker before pregnancy. In fact, most women with CAVB, who do not require a permanent pacemaker before delivery, can be safely managed during labor without temporary pacing. However, the clinical symptoms and cardiac function of patients should be carefully followed after delivery, even when pregnancy and delivery are uneventful.

Transient urinary ascites after vesicocentesis observed in a fetus with megacystis caused by posterior urethral valve.

BACKGROUND: Fetal urinary ascites is a condition that is rarely encountered. A review of previous reports suggests that the clinical course of the affected fetuses is highly variable. METHODS: In this report, we describe a case in which urinary ascites was observed after vesicocentesis in a fetus with megacystis caused by posterior urethral valve. RESULTS: The urinary ascites was transient, and the fetal outcome was good after a successful vesicoamniotic shunting operation. CONCLUSIONS: In utero bladder rupture or injury and the subsequent appearance of urinary ascites may be well tolerated by the fetus. Further, spontaneous resolution can be expected during fetal life; therefore, it appears to be preferable to opt for conservative management in fetuses with urinary ascites. Urinary ascites, particularly after vesicocentesis, should be observed with extreme caution.

Fetal hydrothorax resolving completely after a single thoracentesis: a report of 2 cases.

BACKGROUND: Although fetal hydrothorax is uncommon, perinatal mortality from it is high. The clinical course of fetal hydrothorax is highly variable. Despite some cases of spontaneous resolution, fetal hydrothorax progresses to nonimmune hydrops because of impaired venous return and congestive cardiac failure due to compression in many cases. Although the effect of thoracoamniotic shunting is established, the procedure is invasive and involves some risks. CASES: Two cases of fetal hydrothorax completely resolved after a single thoracentesis. In the first case, thoracentesis was performed in a fetus with bilateral isolated pleural effusion at the gestational age of 20 weeks. Fetal hydrothorax achieved complete resolution after a single thoracentesis was performed, and the perinatal outcome was good. In the second case, we performed thoracentesis in a fetus with a unilateral pleural effusion and skin edema at the gestational age of 17 weeks. Complete resolution was achieved after a single thoracentesis, and the outcome was uneventful. CONCLUSION: Thoracentesis is a comparatively simple procedure for mothers and fetuses and should be the first choice for treating fetal hydrothorax.

Local holder exponent analysis of heart rate variability in preterm infants.

Heart rate variability (HRV) displays scale-invariant fractal properties. Recent studies have revealed multifractal properties in the healthy human HRV, which could be characterized by singularities with various strength of local Hölder exponents embedded in HRV. In this paper, HRV time series from preterm infants, whose autonomic nervous system undergoes dramatic development, were collected longitudinally. Changes in fractality/multifractality of those HRV time series as the postmenstrual age were examined in order to see if they could quantify development of the autonomic nervous system. Temporal structure of the singularities at several representative time scales was also analyzed to show that intersingular event intervals could be well described by "power law distribution," and the singular events appeared with age-dependent long-range correlation in its strength. Detailed analyses suggested that fractality and multifractality of HRV, respectively, could quantify the development of the respiratory center and the parasympathetic nervous system in the preterm infants. The results obtained in this study might be beneficial for detecting occurrences of life threatening singular events such as big apnea in preterm infants.

Hybrid cell-gene therapy for pulmonary hypertension based on phagocytosing action of endothelial progenitor cells.

BACKGROUND: Circulating endothelial progenitor cells (EPCs) migrate to injured vascular endothelium and differentiate into mature endothelial cells. We investigated whether transplantation of vasodilator gene-transduced EPCs ameliorates monocrotaline (MCT)-induced pulmonary hypertension in rats. METHODS AND RESULTS: We obtained EPCs from cultured human umbilical cord blood mononuclear cells and constructed plasmid DNA of adrenomedullin (AM), a potent vasodilator peptide. We used cationic gelatin to produce ionically linked DNA-gelatin complexes. Interestingly, EPCs phagocytosed plasmid DNA-gelatin complexes, which allowed nonviral, highly efficient gene transfer into EPCs. Intravenously administered EPCs were incorporated into the pulmonary vasculature of immunodeficient nude rats given MCT. Transplantation of EPCs alone modestly attenuated MCT-induced pulmonary hypertension (16% decrease in pulmonary vascular resistance). Furthermore, transplantation of AM DNA-transduced EPCs markedly ameliorated pulmonary hypertension in MCT rats (39% decrease in pulmonary vascular resistance). MCT rats transplanted with AM-expressing EPCs had a significantly higher survival rate than those given culture medium or EPCs alone. CONCLUSIONS: Umbilical cord blood-derived EPCs had a phagocytosing action that allowed nonviral, highly efficient gene transfer into EPCs. Transplantation of AM gene-transduced EPCs caused significantly greater improvement in pulmonary hypertension in MCT rats than transplantation of EPCs alone. Thus, a novel hybrid cell-gene therapy based on the phagocytosing action of EPCs may be a new therapeutic strategy for the treatment of pulmonary hypertension.

Identification of development and autonomic nerve activity from heart rate variability in preterm infants.

Heartbeat intervals, which are determined basically by regular excitations of the sinoatrial node, show significant fluctuation referred to as the heart rate variability (HRV). The HRV is mostly due to nerve activities through the sympathetic and parasympathetic branches of the autonomic nervous system (ANS). In recent years, it has been recognized that the HRV shows a greater complexity than ever expected, suggesting that it includes much information about ANS activities. In this study, we investigated relationship between HRV and development in preterm infants. To this end, heartbeat intervals were continuously recorded from 11 preterm infants in NICU. The recording periods were ranging from several days to weeks depending on the individuals. The HRV at various ages was then characterized by several indices. They include power spectrum as well as the mean and standard deviation of the series. For the power spectrum, the low-frequency band power (LF), high-frequency band power (HF), LF/HF (the ratio between LF and HF), beta (scaling exponent of the spectrum) were estimated. The detrended fluctuation analysis (DFA) was also employed to obtain short- and long-range scaling exponents. Each of these indices showed a correlation with the age. We showed that the long-range scaling exponent, derived from the DFA, was most significantly correlated with the age, suggesting that it could be a robust index to characterize the development of preterm infants.

Magneto-encephalographic measurement of neural activity during period of vertigo induced by cold caloric stimulation.

The aim of this study was to investigate neural activity during period of vertiginous sensation, induced by caloric stimulation. After caloric vestibular stimulation (CVS) by cold water of five volunteers (n=5, age: 30+/-10), auditory evoked magnetic fields (AEFs) during the subsequent period of vertiginous sensations were measured by magnetoencephalography (MEG). Current-arrow maps (CAMs) were produced to estimate the spatial current distribution of the AEF responses, and a rotation value (dI(rot)) was calculated from the CAM. The worth of the dI(rot) values as indicators of vertigo was evaluated by comparing them with earlier reported values for elderly control (n=11, age: 67+/-5) and chronic dizziness (CD) (n=27, age: 68+/-8) groups (obtained from AEF responses with no the CVS). Although all volunteers felt vertigo during the AEF measurements, the AEF waveforms and CAM pattern only showed slight changes. While the dI(rot) values (1.43+/-0.73) just after CVS were not significantly different from those (1.59+/-0.46) for the elderly controls, they were significantly different from those (3.54+/-1.34) for the CD patients. These findings suggest that (i) the new parameter (dI(rot)) is more sensitively indicates dizziness (non-rotatory sensation) than vertigo (ii) the auditory cortical region may play an important role in body-balance perception of floating sensations.

Abnormal auditory neural networks in patients with right hemispheric infarction, chronic dizziness, and moyamoya disease: a magnetoencephalogram study.

The purpose of this study was to determine whether the auditory cortex is sensitive to cortical insults and to determine the specificity of the insults in three clinical situations with different cortical involvement. Auditory-evoked magnetic fields of ten normal subjects, 8 patients with right hemispheric infarction, 11 with chronic dizziness, and 2 with moyamoya disease were measured. To analyze the abnormality of auditory neural networks, the magnitude ratio and the angle difference (Deltatheta) between response vectors, which were determined from maximum current arrows corresponding to the N100m peak for contralateral and ipsilateral stimuli were used. A normal range of the parameters was defined so that abnormal values could be determined. Of the three parameters, Deltatheta was the most sensitive: 4 patients with right hemispheric infarction, 4 with chronic dizziness, and 1 with moyamoya disease had abnormal Deltatheta. The electrical activity in the patients with such abnormal Deltathetas had a circular current pattern. These findings suggest that right infarction lesions sometime affect the left auditory neural network, dizziness is caused by abnormal neural networks between the vestibular cortical area and the auditory cortex or by an imbalance between left and right auditory-cortex activities, and moyamoya-disease patients have almost normal auditory-electrical activity.

[Anesthetic experience of endoscopic umbilical cord ligation in a case of twin pregnancy with acardia].

We had an opportunity to engage in anesthetic management for umbilical cord ligation under endoscopy in a case of twin pregnancy with acardia. The patient was a 24-year-old woman. At the time of surgery, she was at 18 weeks and 2 days of pregnancy. Anesthesia was induced with diazepam 10 mg, fentanyl 150 micrograms, and vecuronium 8 mg, and it was maintained with oxygen (1 l.min-1), air (3 l.min-1) and isoflurane 0.8 to 1.2%. To prevent uterine contraction, ritodrine administration was started before surgery and continued throughout the anesthesia. Throughout the surgery, the fetuses remained immobile, with no sign of uterine contractions. However, an arterial blood sample obtained after anesthetic induction showed mild lactic acidosis, suggesting its relation to ritodrine administration. Anesthetic management for fetal surgery is unique in that it is a non-obstetrical surgical procedure performed on a pregnant patient. Particular attention must be directed to specific aspects, such as the anesthetic effect on the fetus, uterine relaxation during peri-operative period, and prevention of spontaneous abortion or premature labor after surgery. The use of tocolytic agents and fetal monitoring remain for further investigation.

Prenatal diagnosis and antenatal history of persistent truncus arteriosus: A case report.

We report the prenatal diagnosis and the natural history of a fetus with truncus arteriosus. A 31-year-old woman, gravida 3, was referred for detailed examination for suspected cardiac anomaly during her 33rd week of gestation. Fetal echocardiography demonstrated a large truncal vessel overriding a ventricular septal defect, a right-side aortic arch, and a dilated ascending aortic arch. Pulsed-wave and color-flow Doppler imaging showed truncal valvular stenosis without insufficiency. No sign of congestive heart failure was detected during the prenatal period. A fetus weighing 2860 grams was delivered during the 38th week of gestation. Cyanosis was noted the day after delivery, however, and a Rastelli procedure was carried out 9 days after delivery. The baby died 7 days after the operation.

Intrauterine hemorrhage from an umbilical cord ulcer associated with fetal duodenal atresia: a case report.

In the recent years, the association between congenital intestinal atresia and umbilical cord ulceration has been demonstrated; however, this complication is rarely encountered. To the best of our knowledge, only 14 cases on this association have been published previously. We encountered a case of acute intrauterine hemorrhage from an umbilical cord ulcer in a fetus with duodenal atresia that resulted in early neonatal death. There is little information available on the association between congenital intestinal atresia and umbilical cord ulceration. Greater understanding may alter the obstetric management. Although the prevention of sudden perinatal death is considered to be difficult, frequent fetal monitoring and detailed ultrasonographic examinations may facilitate the early detection of this condition, thereby enabling the rescue of the affected fetuses. Further investigation is required to establish the management protocol.

Monochorionic twins in which at least one fetus has a congenital heart disease with or without twin-twin transfusion syndrome.

AIMS: To review the course of monochorionic (MC) twin gestations in which one or both fetuses were diagnosed with congenital heart disease (CHD) and to identify clinical characteristics regarding the related intrauterine environmental factors particularly associated with non-twin-twin transfusion syndrome (TTTS). METHODS: This study is a review of a two-center experience of MC twin pregnancies with CHD born between 2000 and 2006. RESULTS: There were 87 MC twin pregnancies of which 11 were associated with fetal heart disease. We found concordance of CHD in 2 of the 11 twin sets. Among all of the discordant cases, the size of the affected twin was smaller. TTTS was observed in one case associated with CHD; in this case the donor twin had a coarctation of the aorta. Abnormal cord insertion was observed in 9 (69%) out of the 13 affected infants, and a significant relation was found between the presence of CHD and abnormal cord insertion. CONCLUSIONS: Of the MC twins, the smaller seemed more likely to be affected by primary structural cardiac anomalies. CHD in MC twins is possibly related to an abnormal cord insertion. Further studies are warranted to determine whether a causal relationship exists.

Megacystis-microcolon-intestinal hypoperistalsis syndrome: in utero sonographic appearance and the contribution of vesicocentesis in antenatal diagnosis.

OBJECTIVE: The purpose of this report is to describe the in utero sonographic appearance of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) and examine the role of vesicocentesis. METHODS: Two cases of the sonographic appearance of MMIHS were reviewed. We performed vesicocentesis in 2 fetuses with MMIHS and performed vesicoamniotic shunting in 1 of them. The sonographic findings after these procedures were compared with those before puncture. RESULTS: In both cases, ultrasound examination showed a massively enlarged fetal bladder and bilateral hydroureteronephrosis with a normal amount of amniotic fluid. Fetal urinary sodium and chloride concentrations were within normal limits in both cases; this suggested normal fetal renal function. Generally, it is difficult to detect the dilated fetal bowel in MMIHS, probably because of the disturbance caused by a large bladder. In our cases, however, the ultrasound examination clearly showed fetal bowel dilatation after these procedures; this made the antenatal diagnosis of MMIHS more definite. CONCLUSIONS: The sonographic features after vesicocentesis may be helpful in confirming the antenatal diagnosis of MMIHS.

Successful treatment of extremely severe fetal anemia due to anti-Jra alloimmunization.

BACKGROUND: Although Jr(a) hemolytic disease of the newborn (HDN) is generally considered to be mild, we encountered a case of anti-Jr(a) alloimmunization hydrops fetalis due to extremely severe fetal anemia that required aggressive intrauterine treatment. CASE REPORT: A Japanese woman developed hydrops fetalis at the 29th week of gestation. Blood examination was highly positive for anti-Jr(a) antibodies. Cordocentesis demonstrated that the fetus had anti-Jr(a) antibodies and the lowest fetal hemoglobin concentration was 2.3 g/dl. After a total of four intravascular transfusions, fetal hydrops improved and a healthy girl of 2,120 g was delivered at the 35th week of gestation. The initial neonatal hemoglobin concentration at birth was 7.2 g/dl. After two straight transfusions, the infant's anemia did not worsen. Anti-Jr(a) antibodies became negative without developing hyperbilirubinemia. CONCLUSION: The incidence of Jr(a) negativity can lead to underestimation of the risk of Jr(a) alloimmunization. This case shows that anti-Jr(a) alloimmunization may cause HDN and hydrops fetalis.

Nationwide survey of pregnancy and delivery in patients with coronary arterial lesions caused by Kawasaki disease in Japan.

BACKGROUND: Our purpose was to determine the outcome of pregnancy and delivery in patients with coronary arterial lesions caused by Kawasaki disease. METHODS AND RESULTS: We surveyed by mail the Japanese national experience of pregnancy and delivery in patients known to have Kawasaki disease. The first questionnaire was returned by 154 of 207 (74%) institutions, and 16 of the 154 had knowledge of deliveries in their patients. Based on a second questionnaire, and previous Japanese case reports, we identified 46 deliveries in 30 patients from 16 institutions. The age at delivery ranged from 18 to 35 years, with a median of 27 years. Of the patients, 4 had undergone coronary arterial bypass grafting. Low-dose aspirin was given in 16 patients. The deliveries, 27 in all, had been vaginal in 20 patients, albeit that 7 required assistance by forceps or vacuum extraction under epidural anesthesia. Caesarean section had been performed in 11 patients, 3 for obstetric indications, and 1 for chest discomfort in the third trimester. Although there were no cardiac events, obstetric complications occurred in 2. CONCLUSION: The results of pregnancy and delivery were favourable. The mode of delivery should be primarily determined by obstetrical considerations, rather than the coronary arterial lesions caused by Kawasaki disease. Excessive anticoagulant therapy may not be needed for this population.

Prenatal diagnosis of congenitally corrected transposition of the great arteries. A case report.

Congenitally corrected transposition of the great arteries (CTGA) is an uncommon congenital cardiac anomaly. Prenatal sonographic diagnosis of CTGA is very difficult because the ventricular outflow tract may appear to arise correctly from the right and left ventricles. Few cases of CTGA diagnosed in utero have been reported. We report a case of CTGA with complete heart block, ventricular septal defect and pulmonary valve stenosis diagnosed at 30 weeks' gestation after the mother was referred to our hospital because of persistent fetal bradycardia.

Experience of temporary inferior vena cava filters inserted in the perinatal period to prevent pulmonary embolism in pregnant women with deep vein thrombosis.

OBJECTIVE: We placed temporary inferior vena cava filters to prevent pulmonary thromboembolism in patients with deep vein thrombosis (DVT) who were presumed to have an increased risk of pulmonary embolism in the perinatal period. These experiences of using temporary inferior vena cava filters in pregnant women are reported. METHODS: We reviewed 11 patients with DVT who underwent placement of a temporary inferior vena cava filter and delivered in our hospital between 1998 and 2004. All of the filters were placed at the suprarenal inferior vena cava before delivery. During filter placement, anticoagulant therapy was routinely performed, and we stopped the administration of anticoagulant agents intrapartum. RESULTS: No complications occurred at filter insertion or during placement. No symptomatic pulmonary thromboembolism occurred during or after delivery. All of the filters were successfully removed, one of which was exchanged for a permanent filter because the temporary filter captured a large thrombus. CONCLUSION: Intrapartum temporary inferior vena cava filters may reduce the incidence of pulmonary thromboembolism in pregnancy with DVT. Temporary inferior vena cava filters appear to be safe for pregnant women.

Successful intrauterine treatment of cystic hygroma colli using OK-432. A case report.

A 36-year-old multiparous woman was referred at 25 weeks of gestation with suspected cystic hygroma. Ultrasonographic examination demonstrated large multiple cysts around the fetal neck. We treated them by injection of OK-432 at 29 and 32 weeks of gestation. The cysts decreased in size after the intrauterine treatment. A female baby was delivered at 38 weeks of gestation. She was healthy, and there was only a small swelling of the skin in the nuchal area at birth. One month later, the slight swelling of the skin had completely disappeared. Intrauterine injection of OK-432 was a safe and effective therapy for fetal cystic hygroma.

Prenatal demonstration of major aortopulmonary collateral arteries with tetralogy of Fallot and pulmonary atresia.

Major aortopulmonary collateral arteries were prenatally demonstrated in a case of tetralogy of Fallot and pulmonary atresia (TOF-PA). Color Doppler and pulsed Doppler ultrasonography revealed abnormal arteries arising directly from the descending aorta. An aortogram after birth confirmed two collateral arteries arising from the descending aorta and one from the left subclavian artery. Fetal echocardiography was essential for the diagnosis of TOF-PA, but further examination was needed to find collateral arteries, which provided useful information for management after birth.