Long-term outcome of 51 liveborn neonates with non-immune hydrops fetalis

To search for an efficient method of management of non-immune hydrops fetalis (NIHF), the clinical outcome of 51 newborns with NIHF was retrospectively assessed in a single centre. As the short-term outcome, the mortality rate was mainly dependent on the causes of NIHF and the presence of pleural effusion. The survival rate of the patients with pleural effusion (7/28; 25%) was significantly lower than that of those without it (14/23; 61%) (p < 0.02). All 7 survivors with pleural effusion were diagnosed antenatally after 29 weeks of gestation and were delivered after 31 weeks of gestation. With respect to the long-term outcome, 13 (68.4%) of 19 patients who survived beyond 1 y of age showed normal development, 2 mild developmental delay at 1 y of age and 1 mental retardation at 8 y of age, while 3 (15.8%) had severe psychomotor retardation with marked growth failure. Two of these three patients were born as very-low-birthweight infants. The follow-up results indicate that pleural drainage in utero and prevention of premature delivery should be proposed to improve the outcome of NIHF patients.     

Prenatal diagnosis of chylothorax]

Chylothorax is defined as the presence of lymph in the pleural space. Congenital chylothorax is one of the most frequent causes of fetal pleural effusion. It may be primary or secondary. Careful assessment of the etiology and of possible associated anomalies is required. Main complications are pulmonary hypoplasia, hydrops fetalis and the risk of premature delivery. Management is still a mater of controversy, the diagnosis of fetal pulmonary hypoplasia being difficult in utero. Factors such as gestational age, evolution of pleural effusion on two weeks, signs of seriousness (hydrops fetalis), and pulmonary expansion after pleural puncture may help the physician to choose between abstention, pleural tapping or long-term in utero drainage. Post natal treatment consists of pleural drainage and assisted ventilation in cases of respiratory distress, correction of metabolic and immune disorders and exclusive parenteral nutrition. Once chylothorax is resolved, formula feeding without long-chain triglycerides is allowed. If pleural effusion persists despite a well conducted treatment, albumin infusion and diuretics may be used before considering surgical treatment.     

Value of Autopsy in Nonimmune Hydrops Fetalis: Series of 51 Stillborn Fetuses

Nonimmune hydrops fetalis (NIHF) is used to describe fetuses and newborns with generalized edema and cavity effusions. It is helpful to alert physicians about the presence of anemia, heart failure, and/or hypoproteinemia, but this diagnosis is frequently overlooked. We reviewed the autopsy files from 1990 to 2000, selected all cases with NIHF including clinical information (with maternal laboratory tests and ultrasound), and classified patients by etiology. Among 840 stillborn autopsies during the 11-year period, we found 51 with NIHF (6.07%). The clinical summary had mentioned hydrops in 14 patients and the etiology in another 7 by fetal ultrasonography, but without addressing the possibility of hydrops. In the remaining 30 cases neither hydrops nor an etiology was mentioned. Other pertinent diagnoses were maternal diabetes mellitus (4), congenital heart disease (3), and cystic hygroma (2). The following diagnoses were made in one instance each: cardiac tumor, twin transfusion syndrome, congenital adenomatoid malformation, syphilis, Turner syndrome, and cerebral arteriovenous malformation. Postmortem and placental examination confirmed the following etiologies: congenital infections (17); placental pathology significant enough to explain NIHF (10); cardiovascular diseases (8) (further classified as congenital heart disease [3], rhabdomyoma [1], and vascular malformations [4]); chromosomal abnormalities (6); uncontrolled maternal diabetes (4); intrathoracic lesions (2); prune-belly syndrome (2); and idiopathic NIHF (2). Only 3.9% of the cases studied had no identifiable etiology. The cause of hydrops was confirmed by autopsy in 47 fetuses (92%), which further supports the importance of performing an autopsy. Thirty-two cases (62.74%) had placental abnormalities helpful to the etiology (parvovirus, syphilis, Turner's syndrome, etc.). In 20 instances, the clinical summary had no mention of either hydrops or any of the diseases leading to it. The autopsy in conjunction with placental examination and fetal ultrasound represent the best combination to determine the etiology of NIHF among stillborn fetuses.     

Nonimmune Hydrops Fetalis in the Liveborn: Series of 32 Autopsies

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. We report 32 cases of NIHF from among 429 neonates who underwent autopsies (incidence 7.45%). Sixteen cases (50%) had cardiovascular disease; all were due to low output cardiac failure; 7 had structural congenital heart disease. Three of the children with congenital heart disease also had chromosomal abnormalities: 2 had trisomy 18 and 1 had Noonan syndrome. Among myocardial conditions were five subjects with cardiomyopathies (1 of each of the following types): oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency; 3 had myocarditis, and 1 had cardiac rhabdomyomas. Congenital infections were due to cytomegalovirus in 3 cases, bacteria in 2, and parvovirus in 1. The mechanism of NIHF in these cases might be a combination of decreased myocardial contractility due to myocarditis and fetal anemia. Genitourinary diseases were present in 5 newborns: Two had congenital nephrotic syndrome, 1 had VACTER association, 1 had prune-belly syndrome, and 1 had urogenital sinus malformation. Intrathoracic lesions were found in 2 babies (pulmonary sequestration and diaphragmatic hernia). One twin died of volume overload due to twin transfusion syndrome. Only 2 newborns were classified as idiopathic. Our study shows that cardiovascular diseases that lead to heart failure or impaired venous return are more common in the liveborn (50%), whereas congenital infections are more common in the stillborn with NIHF.     

儿童多浆膜腔积液38例

目的探讨儿童多浆膜腔积液的临床特点及病因,以提高临床诊治水平。方法回顾性分析2000年1月-2011年6月本院确诊的38例多浆膜腔积液患儿的临床资料,浆膜腔积液标本送检行常规、涂片及细菌培养等检查,采用Light标准判断积液性质。结果本组38例多浆膜腔积液患儿中男24例,女14例;年龄41 d～14岁,其中41 d～3岁8例(21.1%),>3～6岁10例(26.3%),>6～14岁20例(52.6%)。病因以外伤最多见[10例(26.3%)],其次是细菌感染[7例(18.4%)]、肺吸虫病[6例(15.8%)]和心源性疾病[5例(13.2%)]。其中41 d～3岁患儿中外伤最多见,>3～6岁患儿中肺吸虫病多见,>6～14岁患儿以细菌感染为主。多浆膜腔积液以胸腔积液+腹腔积液最多(15例),病因以外伤多见;胸腔积液+心包积液11例,以细菌感染为主;胸腔积液+腹腔积液+心包积液11例,以肺吸虫病多见;心包积液+腹腔积液1例,为高处坠落伤患儿。21例患儿行积液检查,结果显示渗出液16例,其中细菌感染6例,肺吸虫4例,结核病、结缔组织疾病各3例;介于渗出液与漏出液之间5例,渗出液与漏出液为肿瘤及先天性心脏病患儿。肺吸虫病患儿血IgE升高明显,嗜酸性粒细胞均增高。结论儿童多浆膜腔积液好发于6～14岁,以渗出液为主,胸腔积液+腹腔积液最多见;常见病因为外伤、细菌和肺吸虫感染,心源性疾病均为先天性心脏病伴心功能不全;各年龄段的病因构成不同,临床表现无特异性。     

Nonimmune hydrops fetalis part II: does etiology influence mortality?

Hydrops fetalis is a condition in which there is an excess of total body fluid, primarily within the fetal interstitial spaces. Etymologically, hydrops fetalis is a Latin term meaning "edema of the fetus." In addition to generalized edema, the fetus has at least one of the following: ascites, pericardial effusion, pleural effusion(s), and an abnormally thick (>6 cm) placenta. Hydrops is classified as nonimmune hydrops fetalis (NIHF) when it occurs without evidence of isoimmunization.     

Nonimmunologic hydrops fetalis--a review of 31 cases

Non-immunologic hydrops fetalis-a review of 31 cases: 31 Patients with non-immunologic hydrops fetalis (NIHF) seen between 1984 and 1987 are described. 13 infants survived. The infants with major congenital malformations and connatal infections died. In 8 of the patients who died a cause for NIHF could not be identified. 10 of the survivors presented chylous ascites and/or chylothorax without major congenital anomalies. 2 infants had fetal tachyarrhythmia and 1 patient showed severe anemia due to fetomaternal hemorrhage.     

Mid-term follow-up of neonatal pleural effusion

Objectives

To investigate the clinical course and mid-term prognosis of neonates admitted with pleural effusion.

Methods

Case records of 38 neonates admitted with pleural effusion were retrieved and analyzed.

Results

16 (42%) patients had congenital and 22 (58%) patients had acquired causes of pleural effusion. The most common causes of congenital pleural effusion and acquired pleural effusion were chylothorax (18%) and congestive heart failure (13%), respectively. Poorer outcome was observed with fetal hydrops, preterm birth (<34 weeks) and associated defects.

Conclusions

Most of the neonates with pleural effusion have good outcome in the mid-term follow-up.     

Fetal case of congenital cystic adenomatoid malformation of the lung: fetal therapy and a review of the published reports in Japan

We herein report a case of type I congenital cystic adenomatoid malformation of the lung (CCAML) with non-immune hydrops fetalis (NIHF), a mediastinal shift and polyhydramnios diagnosed at 24 weeks' gestation by ultrasonography. The fetus was treated with a cyst-amniotic shunt at 29 weeks' gestation. Following a postnatal whole resection of the right lung, postpneumonectomy syndrome appeared and, as a result, the infant died 13 months after delivery due to respiratory failure. Only 19 cases demonstrating CCAML associated with NIHF have been reported previously in Japan. Four cases showed a spontaneous resolution of NIHF, while 5 cases with type I CCAML, which all underwent fetal intervention, demonstrated an excellent outcome.     

非体外循环下有搏动性双向Glenn术治疗小儿复杂先天性心脏病

目的总结非体外循环下有搏动性双向格林手术的经验。方法2001年1月～2006年8月,在非体外循环下行双向格林手术30例,术中不关闭肺动脉,不结扎奇静脉。结果本组治愈28例,手术早期因低心排综合症死亡2例。术后并发乳糜胸1例,胸腔积液3例,均经保守治疗痊愈。出院时末梢血氧饱和度明显提高。结论非体外循环下有搏动性双向Glenn手术创伤小,疗效满意,是治疗肺血减少型复杂先天性心脏病的一种良好选择。     

胎儿先天性心脏病和心律失常的诊断及干预

目的 研究胎儿超声心动图产前诊断先天性心脏病（简称先心病）和经胎盘治疗胎儿心律失常的价值。方法 应用国产FECG－D型胎儿心电图仪筛查,结合胎儿超声心动图检测第1胎患先心病的460例胎龄为18－40周的高危胎儿,超声仪采用ALT、Maker LX和TOSHBA SSA 40A多普勒彩色超声仪,探头频率为3．5MHz。结果 发现先心病8例,同胞一级亲属患病率为1．7％。先心病的类型：房间隔缺损（简称房缺）3例（其中1例合并胎儿水肿）,室间隔缺损（简称室缺）13例（其中1例合并神经管缺损）,完全性的房室管缺损1例,法洛四联症1例。检出23例胎儿心律失常,检出率为5．0％,以室上性心动过速（简称室上速）最多见（13例）,首选地高辛经胎盘治疗,7例有效,其中3例合并先心病或心力衰竭者加用异博定2例有效,1例因有严重的非免疫性胎儿水肿而死亡;3例窦性心运过速（简称窦速）患儿用心得安治疗有效,1例频发室性早博用普鲁卡因酰胺治疗后转复。3例妊娠晚期的室上速和2例室上性早博未用药,经阴道分娩后转复。结论 胎儿超声心动图是产前诊断胎儿心脏异常的重要手段,地高辛是经胎盘治疗胎儿快速心律失常的首选药物。     

Echocardiographic diagnosis and intravenous digoxin management of fetal tachyarrhythmias and congestive heart failure

Fetal echocardiography now affords an accurate clinical diagnosis of nonimmune fetal hydrops secondary to fetal tachyarrhythmias and/or certain types of congenital heart disease. Individual case reports of the treatment of tachyarrhythmias have been reported that use various drugs, including digoxin, propranolol hydrochloride, procainamide hydrochloride, and digoxin plus verapamil hydrochloride. We found no report of intrauterine treatment of congestive heart failure due to congenital heart disease with sinus rhythm. The seven cases presented herein include five cases of isolated supraventricular tachycardia, one with supraventricular tachycardia and myocardial tumors, and one of congenital heart disease with congestive heart failure. Maternal treatment with digoxin converted tachyarrhythmia to sinus rhythm in all six fetuses, and resolved the hydrops in the fetus with congenital heart disease. Premature delivery was avoided in five of the seven cases with favorable outcome in six of the cases. In five mothers who were given oral digitalization, the fetuses had delayed (three to 35 days) response to therapy. Two mothers were treated intravenously and the fetus responded to therapy in less than 24 hours. Because of problems possibly related to poor absorption and/or rapid clearance of digoxin, the intravenous route of administration should be used as the best way to achieve adequate therapeutic levels in the fetus.     

Successful pleurodesis with OK-432 in preterm infants with persistent pleural effusion

OK-432 (picibanil) is an inactivated preparation of Streptococcus pyogenes that causes pleurodesis by inducing a strong inflammatory response. Intrapleural instillation of OK-432 has recently been used to successfully treat neonatal and fetal chylothorax. Here we report a trial of intrapleural instillation of OK-432 in two preterm infants who were born with hydrops fetalis and massive bilateral pleural effusion. Both cases showed persistent pleural effusion, refractory to conservative treatment, up to postnatal days 26 and 46, respectively. An average of 80 to 140 mL of pleural fluid was drained daily. In case 1, the infant was treated with OK-432 during the fetal period at gestation 28 weeks and 4 days of gestation, but showed recurrence of pleural effusion and progressed into hydrops. Within two to three days after OK-432 injection, the amount of pleural fluid drainage was dramatically decreased and there was no reaccumulation. We did not observe any side effects related to OK-432 injection. We suggest that OK-432 should be considered as a therapeutic option in infants who have persistent pleural effusion for more than four weeks, with the expectation of the early removal of the chest tube and a good outcome.     

The Role of Pleurodesis in the Management of Chylous Pleural Effusion After Surgery for Congenital Heart Disease

This study aimed to determine the incidence and outcome of postoperative chylous pleural effusion as well as the efficacy of pleurodesis for its management after surgery for congenital heart disease. Medical and surgical databases were used to identify all patients who had surgery for congenital heart disease and subsequently experienced postoperative chylous pleural effusion. Medical records were reviewed and daily chest drainage and management strategies were recorded. From January 2000 to June 2006, 1,166 cardiac operations were followed by 19 cases of chylous pleural effusion, for an incidence of 1.6%. The diagnosis was made a mean of 9 days after the operation. The patients were divided into two groups according to treatment strategy. Group 1 included 9 patients who had received only conventional medical treatment consisting of parenteral nutrition and/or medium-chain triglyceride formula and/or a low-fat diet and/or somatostatin. Group 2 included 10 patients who initially received conventional medical treatment, then subsequently received chemical or mechanical pleurodesis. The amount of the chylous drainage was significantly less in group 1 (14 ml/kg/day) than in group 2 (24 ml/kg/day) (P < 0.05), suggesting a more severe problem in group 2. For group 2, the amount of drainage was significantly less after chemical or mechanical pleurodesis (8 vs 24 ml/kg/day; P < 0.05) than before. Seven patients (70%) responded favorably to the first pleurodesis, and two patients (20%) required more than one pleurodesis. One patient (10%) did not respond to pleurodesis but was treated successfully with thoracic duct ligation. There were no deaths. Pleurodesis is a safe and effective method for treating chylous effusion after surgical treatment of congenital heart disease, especially after failed conservative treatment. However, some patients may need more than one pleurodesis.     

Diagnostic and Perinatal Management of Fetal Extrasystole

Fifty fetuses referred to the Polish Mother's Memorial Hospital for fetal echocardiography between January 1, 1991 and June 1, 1995 were evaluated. The mean fetal gestational age at the time of diagnosis of arrhythmia was 34.1 weeks, and the mean gestational age at the time of delivery was 38.7 weeks. Checkup echocardiographic examinations were performed every 10–14 days, for a mean 2.4 studies per fetus. In most cases (48/50, 96%), premature atrial contractions were present during the first echocardiography examination. The fetal heart study was normal in 30 cases; in 7 (14%) there was tricuspid valve regurgitation, in 7 (14%) an atrial septal aneurysm, in 4 congenital heart defects, in 1 myocardial hypertrophy, and in 1 disproportion in the four-chamber view. Of the 50 fetuses, 43 underwent regular echocardiographic monitoring alone; in 7 cases, based on the presence of additional echocardiographic findings, pharmacotherapy was applied (digoxin, verapamil, or both). Three neonates died after delivery owing to malformations in two cases (one critical aortic stenosis, one spina bifida plus hygroma colli) and due to myocarditis in one case. In six of seven newborns treated in utero, myocarditis was diagnosed after birth (including the one with neonatal demise). Most of the newborns were in good condition after birth, their mean Apgar score being 8.6 and the mean birth weight 3259 g. We concluded that most extrasystoles represent an isolated anomaly, not affecting the fetal condition. Their presence should not influence the obstetric care and may require only echocardiographic monitoring. In most of our cases the premature contractions subsided after birth, although sometimes they preceded fetal supraventricular tachycardia or appeared after congenital myocarditis.     

Outcome of Prenatally Diagnosed Isolated Congenital Complete Atrioventricular Block Treated with Transplacental Betamethasone or Ritodrine Therapy

The effectiveness of transplacental drug therapy for prenatally diagnosed isolated congenital complete atrioventricular block (CCAVB) is controversial. Nine cases of prenatal isolated CCAVB were treated from 2002 to 2007. Ritodrine was administered transplacentally to all fetuses and betamethasone to those whose mothers tested positive for maternal anti-SSA/Ro antibodies. Six of the nine patients had an anti-SSA/Ro-positive mother and received transplacental betamethasone 4 mg/day at a median gestational age of 28 weeks (range, 24–31 weeks). No patients exhibited an improvement in the degrees of complete heart block, and one patient died in utero. No serious adverse events occurred. After the mean follow-up period of 1.7 ± 1.3 years, all five patients treated with transplacental betamethasone experienced a good cardiac function, whereas one of the three patients not treated with transplacental betamethasone experienced cardiomyopathy and died at the age of 4 months. Pacemaker implantation was required for seven of the eight live-born infants. Transplacental betamethasone therapy for the patients with isolated CCAVB neither improved the degree of atrioventricular block nor decreased the rate of patients requiring pacemaker implantation, but it probably reduced the risk for the development of myocardial disease.     

Progression of congenital heart disease in the prenatal period

BACKGROUND: Prenatal echocardiography has shown evidence of prenatal development of congenital heart disease. Prenatal cardiac anatomy, chamber size and function change during gestation, so that the appearance of cardiac structure in abnormal hearts may be different from that which is usually seen postnatally. METHODS: Published prenatal echocardiographic studies were reviewed and in utero development of congenital heart disease from midtrimester to the early postnatal period is discussed. RESULTS: The growth of the great vessels and ventricles is reduced in fetuses with ventricular outflow obstruction. Valve regurgitation may progress. The foramen ovale and ductus arteriosus have been reported to become restrictive in utero in several settings. Pulmonary vascular obstructive changes may progress prenatally. Fetal arrhythmia (both bradycardia and tachycardia) may develop in utero. Development of congestive heart failure is a very important issue during follow up of fetuses with significant cardiac or extracardiac problems. Some may progress to fetal hydrops and prognosis of the affected fetuses is usually very poor. CONCLUSIONS: Correct knowledge of possible development is important for accurate prenatal diagnosis. Information on prenatal progression of the cardiac anomaly is also important to make plans for follow up and perinatal management, to predict outcomes and to counsel family. Furthermore, the benefits of prenatal treatment instead of postnatal treatment should be assessed by the accurate prediction of the progression of the cardiac problem in utero. Further extensive studies using a large number of cases is required to predict progression accurately. In addition, further studies for elucidating the mechanisms of progression is important to provide better outcomes for fetuses with various congenital heart diseases.     

Fetal Right-Ventricular Hypertrophy With Pericardial Effusion and Maternal Untreated Hyperthyroidism

Uncontrolled/untreated maternal hyperthyroidism has been associated with fetal tachycardia. We report a case of right-ventricular (RV) hypertrophy with pericardial effusion related to untreated maternal Graves’ disease. A 33-year-old G4P1021 woman with uncontrolled Graves’ disease presented at 29 weeks gestation with abdominal pain and vaginal bleeding. Fetal echocardiogram showed severe RV hypertrophy and a pericardial effusion. The infant was born prematurely, and initial transthoracic echocardiogram showed severe RV hypertrophy and a small pericardial effusion. The infant had clinical findings consistent with congenital thyrotoxicosis and was treated for this. Follow-up imaging at 4 weeks showed improvement of the cardiac hypertrophy and pericardial effusion. This article describes the presentation of fetal RV hypertrophy with congenital thyrotoxicosis and underscores the importance of screening for this prenatally in mothers with uncontrolled or untreated hyperthyroidism.     

Fetal echocardiography in the diagnosis of congenital heart disease

Abstract Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper reviews the accuracy and outcomes of fetal cardiac ultrasound studies at The Prince Charles Hospital over the past 4 years.
A total of 43 fetuses, of gestational ages 16–39 weeks, were studied. The indications for these were: previous sibling with cardiac anomaly (16 cases); abnormal heart on obstetric ultrasound scan (USS) (13 cases); abnormalities found on obstetric USS with normal appearing heart (one case); fetal bradycardia (seven cases); fetal tachycardia (four cases); irregular fetal heart beat (two cases).
At birth all fetuses assessed because of a previously affected sibling were normal. Of the 13 referred because of structural heart lesions suspected on obstetric USS, eight were abnormal, four were normal and one was terminated without autopsy. Important rhythm disturbances occurred in nine of the 11 referred because of abnormal heart rates. One false positive diagnosis of a possible coarctation was made, and in four cases an abnormality was noted on USS but the diagnosis was not completely correct. Nine of the 19 infants with abnormalities detected have died.
Fetal echocardiography is an accurate and useful method of diagnosing congenital heart disease in utero . Although the mortality of affected fetuses is high, antenatal diagnosis allows planning of medical care and offers the greatest chance of a successful outcome.     

Fetal echocardiography in the diagnosis of congenital heart disease

Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper reviews the accuracy and outcomes of fetal cardiac ultrasound studies at The Prince Charles Hospital over the past 4 years. A total of 43 fetuses, of gestational ages 16-39 weeks, were studied. The indications for these were: previous sibling with cardiac anomaly (16 cases); abnormal heart on obstetric ultrasound scan (USS) (13 cases); abnormalities found on obstetric USS with normal appearing heart (one case); fetal bradycardia (seven cases); fetal tachycardia (four cases); irregular fetal heart beat (two cases). At birth all fetuses assessed because of a previously affected sibling were normal. Of the 13 referred because of structural heart lesions suspected on obstetric USS, eight were abnormal, four were normal and one was terminated without autopsy. Important rhythm disturbances occurred in nine of the 11 referred because of abnormal heart rates. One false positive diagnosis of a possible coarctation was made, and in four cases an abnormality was noted on USS but the diagnosis was not completely correct. Nine of the 19 infants with abnormalities detected have died. Fetal echocardiography is an accurate and useful method of diagnosing congenital heart disease in utero. Although the mortality of affected fetuses is high, antenatal diagnosis allows planning of medical care and offers the greatest chance of a successful outcome.