Valvuloplasty in 103 fetuses with critical aortic stenosis: outcome and new predictors for postnatal circulation.

OBJECTIVES: To review our experience with fetal aortic valvuloplasty (FAV) in fetuses with critical aortic stenosis (CAS) and evolving hypoplastic left heart syndrome (eHLHS), including short- and medium-term postnatal outcome, and to refine selection criteria for FAV by identifying preprocedural predictors of biventricular (BV) outcome. METHODS: This was a retrospective review of all fetuses with CAS and eHLHS undergoing FAV at our center between December 2001 and September 2020. Echocardiograms and patient charts were analyzed for pre-FAV ventricular and valvular dimensions and hemodynamics and for postnatal procedures and outcomes. The primary endpoints were type of circulation 28 days after birth and at 1 year of age. Classification and regression-tree analysis was performed to investigate the predictive capacity of pre-FAV parameters for BV circulation at 1 year of age. RESULTS: During the study period, 103 fetuses underwent 125 FAVs at our center, of which 87.4% had a technically successful procedure. Technical success per fetus was higher in the more recent period (from 2014) than in the earlier period (96.2% (51/53) vs 78.0% (39/50); P = 0.0068). Eighty fetuses were liveborn after successful intervention and received further treatment. BV outcome at 1 year of age was achieved in 55% of liveborn patients in our cohort after successful FAV, which is significantly higher than the BV-outcome rate (23.7%) in a previously published natural history cohort fulfilling the same criteria for eHLHS (P = 0.0015). Decision-tree analysis based on the ratio of right to left ventricular (RV/LV) length combined with LV pressure (mitral valve regurgitation maximum velocity (MR-Vmax)) had a sensitivity of 96.97% and a specificity of 94.44% for predicting BV outcome without signs of pulmonary arterial hypertension at 1 year of age. The highest probability for a BV outcome was reached for fetuses with a pre-FAV RV/LV length ratio of < 1.094 (96.4%) and for those fetuses with a RV/LV length ratio ≥ 1.094 to < 1.135 combined with a MR-Vmax of ≥ 3.14 m/s (100%). CONCLUSIONS: FAV could be performed with high success rates and an acceptable risk with improving results after a learning curve. Pre-FAV RV/LV length ratio combined with LV pressure estimates were able to predict a successful BV outcome at 1 year of age with high sensitivity and specificity. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Fetal aortic valvuloplasty may rescue fetuses with critical aortic stenosis and hydrops.

OBJECTIVE: Critical aortic stenosis (CAS) with a restrictive interatrial septum may lead to fetal congestive heart failure and hydrops, usually culminating in fetal demise if left untreated. The aim of this study was to assess the effects of fetal aortic valvuloplasty (FAV) on hemodynamics and outcome in these patients. METHODS: This was a retrospective review of fetuses with CAS and signs of hydrops that underwent FAV in our center between 2000 and 2020. Echocardiograms and patients' charts were analyzed for ventricular and valvular dimensions and for outcome. RESULTS: Hydrops was present at the time of intervention in 15 fetuses with CAS that underwent FAV at our center during the study period. All but one patient had at least one technically successful procedure. There were no procedure-related deaths, but three intrauterine deaths occurred. Twelve subjects were liveborn, of whom two died within 24 h after birth owing to persistent hydrops. Ventricular function improved and hydrops resolved within 3-4 weeks after FAV in 71.4% (10/14) of fetuses with a technically successful intervention. A biventricular outcome was achieved in 50% of the successfully treated patients. CONCLUSIONS: Fetuses with CAS and hydrops can be successfully treated with FAV. The procedure has the potential to restore sufficient fetal cardiac output, which may lead to resolution of hydrops. Surviving patients seem to be good candidates for a biventricular outcome. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Immediate effects and outcome of in-utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis.

OBJECTIVE: To assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in-utero RV growth and postnatal outcome. METHODS: Patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1-3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV-VTI) × heart rate (HR) and tricuspid regurgitation (TR) velocity. Longitudinal data were collected from only those fetuses followed up in our center. Outcome was assessed using the scoring system as described by Roman et al. for non-biventricular outcome. RESULTS: Thirty-five pulmonary valvuloplasties were performed in our institution on 23 fetuses with PAIVS (n = 15) or CPS (n = 8). Median gestational age at intervention was 28 + 4 (range, 23 + 6 to 32 + 1) weeks. No fetal death occurred. Immediately after successful intervention, RV/left ventricular length (RV/LV) ratio (P ≤ 0.0001), TV/mitral valve annular diameter (TV/MV) ratio (P ≤ 0.001), RV filling time (P ≤ 0.00001) and TV-VTI × HR (P ≤ 0.001) increased significantly and TR velocity (P ≤ 0.001) decreased significantly. In fetuses followed longitudinally to delivery (n = 5), RV/LV and TV/MV ratios improved further or remained constant until birth. Fetuses with unsuccessful intervention (n = 2) became univentricular, all others had either a biventricular (n = 15), one-and-a-half ventricular (n = 3) or still undetermined (n = 3) outcome. Five of nine fetuses with a predicted non-biventricular outcome, in which the procedure was successful, became biventricular, while two of nine had an undetermined circulation. CONCLUSION: In selected fetuses with PAIVS or CPS, in-utero pulmonary valvuloplasty led immediately to larger RV caused by reduced afterload and increased filling, thus improving the likelihood of biventricular outcome even in fetuses with a predicted non-biventricular circulation. © 2018 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.

Fetal hemodynamic response to aortic valvuloplasty and postnatal outcome: a European multicenter study.

OBJECTIVE: Fetal aortic stenosis may progress to hypoplastic left heart syndrome. Fetal valvuloplasty (FV) has been proposed to improve left heart hemodynamics and maintain biventricular (BV) circulation. The aim of this study was to assess FV efficacy by comparing survival and postnatal circulation between fetuses that underwent FV and those that did not. METHODS: This was a retrospective multicenter study of fetuses with aortic stenosis that underwent FV between 2005 and 2012, compared with contemporaneously enrolled natural history (NH) cases sharing similar characteristics at presentation but not undergoing FV. Main outcome measures were overall survival, BV-circulation survival and survival after birth. Secondary outcomes were hemodynamic change and left heart growth. A propensity score model was created including 54/67 FV and 60/147 NH fetuses. Analyses were performed using logistic, Cox or linear regression models with inverse probability of treatment weighting (IPTW) restricted to fetuses with a propensity score of 0.14-0.9, to create a final cohort for analysis of 42 FV and 29 NH cases. RESULTS: FV was technically successful in 59/67 fetuses at a median age of 26 (21-34) weeks. There were 7/72 (10%) procedure-related losses, and 22/53 (42%) FV babies were delivered at < 37 weeks. IPTW demonstrated improved survival of liveborn infants following FV (hazard ratio, 0.38; 95% CI, 0.23-0.64; P = 0.0001), after adjusting for circulation and postnatal surgical center. Similar proportions had BV circulation (36% for the FV cohort and 38% for the NH cohort) and survival was similar between final circulations. Successful FV cases showed improved hemodynamic response and less deterioration of left heart growth compared with NH cases (P ≤ 0.01). CONCLUSIONS: We report improvements in fetal hemodynamics and preservation of left heart growth following successful FV compared with NH. While the proportion of those achieving a BV circulation outcome was similar in both cohorts, FV survivors showed improved survival independent of final circulation to 10 years' follow-up. However, FV is associated with a 10% procedure-related loss and increased prematurity compared with the NH cohort, and therefore the risk-to-benefit ratio remains uncertain. We recommend a carefully designed trial incorporating appropriate and integrated fetal and postnatal management strategies to account for center-specific practices, so that the benefits achieved by fetal therapy vs surgical strategy can be demonstrated clearly. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Natural history of 107 cases of fetal aortic stenosis from a European multicenter retrospective study.

OBJECTIVES: Fetal aortic valvuloplasty (FV) aims to prevent fetal aortic valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history of the disease in fetal life remains poorly defined. The primary aim of this study was to describe the natural history of fetal aortic stenosis, and a secondary aim was to test previously published criteria designed to identify cases of emerging HLHS with the potential for a biventricular (BV) outcome after FV. METHODS: From a European multicenter retrospective study of 214 fetuses with aortic stenosis (2005-2012), 107 fetuses in ongoing pregnancies that did not undergo FV were included in this study and their natural history was reported. We examined longitudinal changes in Z-scores of aortic and mitral valve and left ventricular dimensions and documented direction of flow across the foramen ovale and aortic arch, and mitral valve inflow pattern and any gestational changes. Data were used to identify fetuses satisfying the Boston criteria for emerging HLHS and estimate the proportion of these that would have been ideal FV candidates. We applied the threshold score whereby a score of 1 was assigned to fetuses for each Z-score meeting the following criteria: left ventricular length and width > 0; mitral valve diameter > -2; aortic valve diameter > -3.5; and pressure gradient across either the mitral or aortic valve > 20 mmHg. We compared the predicted circulation with known survival and final postnatal circulation (BV, UV or conversion from BV to UV). RESULTS: Among the 107 ongoing pregnancies there were eight spontaneous fetal deaths and 99 livebirths. Five were lost to follow-up, five had comfort care and four had mild aortic stenosis not requiring intervention. There was intention-to-treat in these 85 newborns but five died prior to surgery, before circulation could be determined, and thus 80 underwent postnatal procedures with 44 BV, 29 UV and seven BV-to-UV circulatory outcomes. Of newborns with intention-to-treat, 69/85 (81%) survived ≥ 30 days. Survival at median 6 years was superior in cases with BV circulation (P = 0.041). Those with a postnatal UV circulation showed a trend towards smaller aortic valve diameters at first scan than did the BV cohort (P = 0.076), but aortic valve growth velocities were similar in both cohorts to term. In contrast, the mitral valve diameter was significantly smaller at first scan in those with postnatal UV outcomes (P = 0.004) and its growth velocity (P = 0.008), in common with the left ventricular inlet length (P = 0.004) and width (P = 0.002), were reduced significantly by term in fetuses with UV compared with BV outcome. Fetal data, recorded before 30 completed gestational weeks, from 70 treated neonates were evaluated to identify emerging HLHS. Forty-four had moderate or severe left ventricular depression and 38 of these had retrograde flow in the aortic arch and two had left-to-right flow at atrial level and reversed a-waves in the pulmonary veins. Thus 40 neonates met the criteria for emerging HLHS and BV circulation was documented in 13 (33%). Of these 40 cases, 12 (30%) had a threshold score of 4 or 5, of which five (42%) had BV circulation without fetal intervention. CONCLUSIONS: The natural history in our cohort of fetuses with aortic stenosis and known outcomes shows that a substantial proportion of fetuses meeting the criteria for emerging HLHS, with or without favorable selection criteria for FV, had a sustained BV circulation without fetal intervention. This indicates that further work is needed to refine the selection criteria to offer appropriate therapy to fetuses with aortic stenosis. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.

Tissue Doppler imaging in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome before and after fetal aortic valvuloplasty.

OBJECTIVES: Fetal aortic valvuloplasty can improve filling and reduce afterload of the left ventricle in critical aortic stenosis. Success of an intrauterine intervention is currently measured by technical success, clinical survival and eventual postnatal biventricular physiology. In the present study we investigated the use of tissue Doppler imaging (TDI) to evaluate changes in ventricular function assessed before and after prenatal aortic valvuloplasty. METHODS: Between October 2008 and December 2012, cardiac function was assessed by TDI before and after intervention in 23 fetuses that underwent technically successful valvuloplasty for critical aortic stenosis and in which postnatal outcome was known. The measurements were transformed into gestational age-independent Z-scores where appropriate. RESULTS: Mean ± SD gestational age at intervention was 27.5 ± 3.1 weeks. Of the 23 fetuses, 14 had biventricular outcome. Before intervention all left ventricular (LV) TDI-derived parameters and mitral annular plane systolic excursion (MAPSE) were severely abnormal. It was possible to demonstrate considerably improved cardiac function after technically successful valvuloplasty. Among fetuses with postnatal biventricular outcome, TDI-derived LV myocardial peak velocity during early diastole (E') and myocardial peak velocity during systole in the ejection phase (S') significantly increased, E'/myocardial peak velocity during late diastole with atrial contraction (A') increased towards normal values, and LV transmitral-to-mitral-annular diastolic velocity ratio (E/E') and myocardial performance index (MPI') decreased but remained abnormally elevated. In addition, right ventricular A', S' and MPI' significantly improved after intervention. CONCLUSION: Technically successful fetal aortic valvuloplasty led to significantly improved myocardial performance. It was possible to use TDI to detect distinct changes in ventricular function and TDI-derived parameters correlated with a biventricular outcome after birth. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

Fetal aortic valvuloplasty: investigating institutional bias in surgical decision-making.

OBJECTIVES: Fetal aortic valvuloplasty may prevent the progression of aortic stenosis to hypoplastic left heart syndrome and allow biventricular rather than univentricular postnatal treatment. This study aimed to investigate whether blinded simulation of a multidisciplinary team approach aids interpretation of multicenter data to uncover institutional bias in postnatal decision-making following fetal cardiac intervention for aortic stenosis. METHODS: The study included 109 cases of prenatally diagnosed aortic stenosis from 13 European countries, of which 32 had undergone fetal cardiac intervention. The multidisciplinary team, blinded to fetal cardiac intervention, institutional location and postnatal treatment, retrospectively assigned a surgical pathway (biventricular or univentricular) based on a review of recorded postnatal imaging and clinical characteristics. The team's decisions were the numerical consensus of silent voting, with case review when a decision was split. Funnel plots showing concordance between the multidisciplinary team and the local team's surgical choice (first pathway) and with outcome (final pathway) were created. RESULTS: In 105 cases the multidisciplinary team reached a consensus decision regarding the surgical pathway, with no decision in four cases because the available imaging records were inadequate. Blinded multidisciplinary team consensus for the first pathway matched the decision of the surgical center in 93/105 (89%) cases, with no difference in agreement between those that had undergone successful fetal cardiac intervention (n = 32) and no (n = 74) or unsuccessful (n = 3) valvuloplasty (no fetal cardiac intervention) (κ = 0.73 (95% CI, 0.38-1.00) vs 0.74 (95% CI, 0.51-0.96)). However, funnel plots comparing multidisciplinary team individual decisions with those of the local teams displayed more discordance (meaning biventricular-univentricular conversion) for the final surgical pathway following fetal cardiac intervention than they did for cases without such intervention (36/74 vs 34/130; P = 0.002), and identified one outlying center. CONCLUSIONS: The use of a blinded multidisciplinary team to simulate decision-making and presentation of data in funnel plots may assist in the interpretation of data submitted to multicenter studies and permit the identification of outliers for further investigation. In the case of aortic stenosis, a high level of agreement was observed between the multidisciplinary team and the surgical centers, but one outlying center was identified.

Maternal aspects of fetal cardiac intervention.

OBJECTIVES: Fetal cardiac interventions have the potential to alter natural disease progression and reduce morbidity and mortality in children. Although there are already encouraging data on fetal outcome, information on maternal morbidity and mortality after intervention is scarce. The aim of the present study was to assess maternal aspects, pregnancy-associated risks and adverse events in 53 intrauterine cardiac interventions. METHODS: Between October 2000 and December 2012, 53 fetal cardiac interventions were performed in 47 patients (43 aortic valve dilations in 39 patients, seven pulmonary valve dilations in six patients and three balloon atrioseptostomies in two patients). Median gestational age was 26 + 4 (range, 20 + 3 to 33 + 1) weeks. Interventions were performed by an ultrasound-guided percutaneous approach under general anesthesia. All medical records and patient charts were analyzed retrospectively. RESULTS: All women were considered to be healthy in the preoperative assessment; 39 (83%) patients continued pregnancy until term and eight of 47 patients had an intrauterine fetal death (IUFD) and were induced. Postoperative nausea was reported in 29.8% of patients and abdominal pain in 36.2% of patients on the day of surgery. Preterm contractions were observed in two patients; no preterm prelabor rupture of membranes occurred. One severe postpartum hemorrhage was observed in a patient with IUFD and subsequent induction; however, this was unrelated to the balloon valvuloplasty. No intensive care unit admission and no major anesthesia-associated complications (aspiration, anaphylactic reaction, cardiovascular collapse, damage to teeth, laryngeal damage, awareness or hypoxic brain damage) were observed. Maternal mortality was zero. A significant learning curve was observed in terms of duration of intervention. CONCLUSION: In our experience, percutaneous needle-guided fetal cardiac intervention seems to be a safe procedure for the mother. In 53 procedures no major maternal complication directly related to the intervention was observed.

Intrauterine aortic valvuloplasty in fetuses with critical aortic stenosis: experience and results of 24 procedures.

OBJECTIVE: Valvuloplasty of the fetal aortic valve has the potential to prevent progression of critical aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). The aim of the study was to assess 24 aortic valvuloplasties regarding indications, success rate, procedure-related risks and outcome. METHODS: Between January 2001 and December 2009 we performed 24 aortic valvuloplasties in 23 fetuses with critical AS at a median gestational age of 26 + 4 (range, 21 + 3 to 32 + 5) weeks by a transabdominal ultrasound-guided approach. Four fetuses had hydrops as a late sign of heart failure. RESULTS: In 16/24 procedures (66.7%) corresponding to 16/23 fetuses (69.6%) the procedures were technically successful, with one intrauterine death in this group. After an initial learning curve, success rate improved to 78.6% (11 of the last 14 interventions were successful). In 10 out of the 15 (66.7%) successfully-treated and liveborn fetuses a biventricular circulation could be achieved postnatally. All four fetuses with hydrops had successful interventions, hydrops disappearing within 5 weeks. In 8/24 interventions (33.3%) the aortic valve could not be treated successfully, with intrauterine fetal death in two of these cases. In one fetus a repeat procedure was successful. All surviving fetuses with unsuccessful (n = 5) or no (n = 5) procedure performed developed HLHS until delivery. CONCLUSIONS: Fetal aortic valvuloplasty could be performed successfully in selected fetuses with critical AS and evolving HLHS, with a biventricular outcome in two thirds of the patients. Safety and success rate were dependent on patient selection and the level of experience of the whole interventional team. In fetuses with AS and hydrops, aortic valvuloplasty could reverse end-stage heart failure and hydrops and ensure fetal survival.

Early and mid-term outcome of the arterial switch operation in 114 consecutive patients : A single centre experience.

BACKGROUND: The arterial switch operation (ASO) has become the treatment of choice in patients with simple or complex transposition of the great arteries (TGA). The purpose of this study was to assess early and mid-term outcome after ASO in a single centre. PATIENTS AND METHODS: Between 1995 and December 2005, 114 consecutive patients underwent an ASO at our institution, performed by one single surgeon. Patients charts, surgical reports and echocardiograms were retrospectively reviewed. Patients were analyzed in 3 different groups: Group I consisted of 77 neonates with TGA and intact ventricular septum, group II of 13 patients with TGA and ventricular septal defect which had to be closed surgically and group III of 24 patients with various forms of TGA in a complex setting. The patient's median weight was 3.23 kg (1.65-8.30). Twenty-five neonates were born preterm, 18 were diagnosed prenatally. Median follow-up time was 20.7 months (0.3-128.6). RESULTS: The thirty day mortality was 1.75% (2/114), late mortality 0.88% (1/112) accounting for an overall mortality of 2.63%. There was only one early coronary event and so far no late events. Ten of 111 survivors required reoperation, all but 1 from group III. Prevalence of supravalvular pulmonary stenosis was 4.7%. Freedom from reoperation at 5 years of follow-up time was 87.5%. One patient required permanent pacing, no other late arrhythmias occurred. In our series the only risk factor for increased mortality and morbidity was a body weight of less than 2500 g at the time of operation. No better outcome could be demonstrated in the prenatally diagnosed patients. CONCLUSION: The ASO can be performed safely and with low mortality and morbidity even in patients with complex TGA. Follow-up of these patients is required to detect residual problems like supravalvular pulmonary stenosis, coronary problems, arrhythmias and aortic valve dysfunction.

Invasive intrauterine treatment of pulmonary atresia/intact ventricular septum with heart failure.

The mortality and morbidity of children with pulmonary atresia/intact ventricular septum (PA/IVS) are linked to the degree of right ventricular (RV) hypoplasia. Opening up the pulmonary valve (PV) in fetal life could result in improved growth of the RV making it amenable to biventricular repair postnatally. Successful valvulotomy of the PV was performed in a fetus with heart failure at 28 weeks. Following the procedure there was significant growth of the tricuspid valve and RV. The neonate was delivered at 38 weeks with a RV suitable for biventricular repair. In utero pulmonary valvulotomy is feasible and may change the natural history of the condition in affected fetuses with PA/IVS.

[Real time 3D sonography of the normal fetal heart--clinical evaluation]. / Klinische Evaluierung der Realtime-3D-Sonographie beim normalen fetalen Herzen.

AIM: Aim of the study was to determine the clinical feasibility of real time 3D ultrasound in the examination of the normal fetal heart compared to conventional 2D fetal echocardiography. METHOD: Twenty fetuses with normal hearts at 20 to 38 weeks of gestation underwent real time 3D ultrasound. Examination data were stored on an external notebook. Later analysis and interpretation was done by a different sonographer using a special software. RESULTS: In the assessment of the four chamber view and the out flow tracts real time 3D ultrasound was equivalent to conventional ultrasound. Advantages of realtime 3D ultrasound were the possibility of later time-independent off-line analysis and post-processing of volume data and generation of new views not available in 2D imaging. Disadvantages included low frame rate (16 frames/s), low lateral resolution and the lack of Doppler and colour information. CONCLUSION: Simultaneous display of 3 different views of the fetal heart as well as the construction of spatial perspectives ("new views") provide additional useful information to conventional fetal echocardiography. Later off-line analysis of 3D volume data can be used for sequential analysis of the normal fetal heart with good diagnostic results. Currently it remains unclear whether this new method may provide additional important information in the assessment of fetal congenital heart defects.

[Emergencies in pediatric cardiology]. / Kinderkardiologische Notfälle.

Congenital heart defects, not recognized antenatally, may lead to emergency situation immediately after birth. Fast recognition of clinical symptoms and the use of correct diagnostic tools are life-saving under these circumstances. Principles of cardio-pulmonary resuscitation will be presented in this article as well as diagnosis and treatment of hypoxemic spells, pulmonary hypertensive crises and (usually postoperative) pericardial effusion.

Fetal cardiology.

The prevalence of congenital heart disease increases with increasing NT, and many defects can be detected by echocardiography performed by a specialist at 13 to 17 weeks. There is increasing evidence that a prenatal diagnosis improves fetal outcome. Physiology and pathophysiology of the human fetal pulmonary circulation play an important role in the fetal circulation, and recent studies about the reactivity of these vessels in the third trimester have contributed to increase our understanding. The main issue in fetal arrhythmias (brady- and tachycardia) is the correct indication, efficacy, and safety of treatment.

[Spontaneous constriction of the fetal ductus arteriosus]. / Spontane Konstriktion des fetalen Ductus arteriosus.

The case report describes the pre- and postnatal clinical and sonographic findings of a fetus with spontaneous constriction of Ductus arteriosus at 39 weeks gestation. An abnormal four-chamber view due to a hypertrophied and enlarged right ventricle, increased peak systolic (2.39 m/sec), increased peak diastolic (0.88 m/sec) velocities and the decreased pulsatility index of 0.92 were the diagnostic criteria. The flow of the right pulmonary artery was retrograde. After birth estimated pulmonary pressure was 50 mmHg. It is concluded that constriction of the fetal ductus arteriosus may also occur spontaneously in the absence of maternal drugs.

European Study on Maternal and Fetal Management of Fetal Supraventricular Tachyarrhythmia:Proposed Protocol for an International Project

> Objectives: This European protocol is designed to estimate the efficacy of transplacental digoxin administered intravenously to the mother in sustained fetal supraventricular tachyarrhythmias (FSVT) with or without cardiac failure and in intermittent runs of FSVT with cardiac failure. The natural history of FSVT is evaluated in self-limited runs of FSVT without cardiac failure. Study structure: The fetal criteria for inclusion in the study are: gestational age <35 weeks, a normal cardiac anatomy, no severe extracardiac anomalies and a heart rate of the FSVT >200 beats/min. The maternal criteria include no prior treatment with digoxin or drugs effecting fetal heart rate and hemodynamics, and no contraindication to digoxin. Doppler fetal echocardiography is utilized for the diagnosis of the type of FSVT, sequential evaluation, and assessment of fetal hemodynamics. Fetuses are assigned two groups: Group I includes all fetuses with sustained FSVT and those with intermittent runs and cardiac failure; Group II comprises fetuses with intermittent runs and no cardiac failure. Fetuses in Group I will be treated. Group II will receive no treatment initially and will be observed. Treatment is based on a acute loading phase with digoxin administered intravenously to the mother for a period of 7 days. Doses are adjusted to through levels of maternal digoxin, maternal clinical condition, and electrocardiogram. If sinus rhythm is obtained, a maintenance phase follows with oral digoxin. Another drug, according to local experience and preference, is added to oral digoxin if digoxin intravenously fails to restore sinus rhythm and gestational age is <36 weeks. Post natal outcome will be followed by sequential clinical and Holter examinations. The data collected will be reviewed in a blinded fashion. The collection of the data for the protocol requires extensive information on the maternal, fetal, and long-term follow-up studies. They are detailed in the Appendix section. Expected results: We propose to obstetricians and pediatric cardiologists involved in fetal cardiology a rationale for the management and treatment of FSVT. Our expectation is to obtain a controlled study on a large number of cases at an international scale and thus provide a homogeneous maternal and fetal therapeutic strategy for FSVT.

[Doppler ultrasound of the umbilical vein in fetal 3rd degree atrioventricular block]. / Doppler der Nabelvene beim fetalen AV-Block 3. Grades.

A 21 week fetus was diagnosed with complete heart block. From the first diagnosis until 38 weeks gestation, umbilical venous pulsations occurred during ventricular systole. The ventricular frequency was 49 bpm. The venous pulsations corresponded with the ventricular frequency. At 38 weeks venous pulsations also occurred during ventricular diastole. Umbilical pulsations during diastole were associated with a decrease of ventricular frequency to 28 bpm and fetal hydrops. The venous pulsations during ventricular systole seem to be characteristic of complete heart block. The pulsations during ventricular diastole reflect the fetal heart failure. Doppler ultrasound aids in the diagnosis and surveillance of the fetus with complete heart block.

[Symptomatic congenital complete atrioventricular block--a medical challenge]. / Symptomatischer kongenitaler kompletter atrioventrikulärer Block--eine medizinische Herausforderung.

Congenital complete atrioventricular block is a rare entity. The association between this disease, maternal connective tissue disease and maternal antibodies [anti-Ro (SS-A) resp. anti-La (SS B)] is well known. Diagnosis can be made by means of fetal Doppler-echocardiography by the 16th week of gestation. In our case diagnosis was established in the 21st week of gestation. Ventricular rate was 55/min, atrial rate 70/min. There were no signs of fetal hydrops. There were no signs of maternal connective tissue disease, but anti-Ro and anti-La antibodies could be detected. The mother was treated with steroids from the time of diagnosis until the end of pregnancy. Altogether 9 Doppler-echocardiographic studies were performed. A recurrence of normal rhythm did not occur. A slow but continuous decrease of atrial and ventricular rate was observed. Interestingly, there was no development of fetal hydrops until the very end of pregnancy when the fetal heart rate reached a low of 28 beats per minute. We speculate, that the therapy with steroids might have played an important role in the prevention of early hydrops. At the onset of fetal hydrops delivery should be considered. In symptomatic complete atrioventricular block we prefer the implantation of a permanent pacemaker system immediately after birth. Efficient care for the fetus resp. the newborn can only be achieved through well planned cooperation.