Evaluation of a prophylactic transfusion program on obstetric outcomes in pregnant women with sickle cell disease: A single centre retrospective cohort study.

OBJECTIVE: To evaluate the effects of a prophylactic transfusion program (TP) on obstetric and perinatal outcomes in pregnant women with sickle cell disease (SCD). METHODS: This retrospective cohort study included all singleton pregnancies among women with SCD in a French university tertiary care center between 1 January 2004 and 31 December 2017. The TP group included patients selected according to the French guidelines who received regular red blood cell transfusions during pregnancy until delivery. The factors associated with TP indication [year of birth, SCD genotype, history of acute chest syndrome and delayed hemolysis transfusion reaction (DHTR) risk score] were taken into account in a propensity score. A composite obstetric adverse outcome was defined associating birth before 34 gestational weeks and/or pre-eclampsia and/or small for gestational age and/or abruption and/or stillbirth and/or maternal death and/or neonatal death. RESULTS: In total, 246 pregnancies in 173 patients were analyzed. Twenty-two pregnancies with a history of DHTR were excluded. A higher frequency of TP was found before 2013 [119/148 (80.4%) vs 38/76 (50%); p < 0.001]. Rates of preterm birth before 34 gestational weeks (5.6% vs 19.7%; p = 0.001), vaso-occlusive crisis (36.5% vs. 61.8%; p < 0.001), and acute chest syndrome (6.1% vs. 14.5%; p = 0.04) during pregnancy were decreased significantly in the TP group. Among the groups with and without composite obstetric adverse outcomes, the frequency of TP was 52.6% and 74.7%, respectively [odds ratio (OR) 0.30, 95% confidence interval (CI) 0.09-1.02]. The multivariate analysis shows that the TP was associated with a significant reduction in the risk of composite obstetric adverse outcomes (OR 0.28, 95% CI 0.08-0.97; p = 0.04). CONCLUSION: A red blood cell TP may have an independent protective effect on maternal and perinatal adverse outcomes during pregnancy in women with SCD.

Antenatal screening of small for gestational age: Impact on obstetrical management and neonatal outcomes in case of trial of labor after 37 weeks.

OBJECTIVE: Antenatal screening of small fetuses for gestational age (SGA) is a public health challenge. The aim of this study is to assess the obstetrical management and the immediate neonatal outcomes, according to the antenatal screening of the SGA fetuses. METHODS: We performed a retrospective study in a French tertiary care hospital between January 1, 2016 and December 31, 2018. Women were eligible if they had a monofetal pregnancy with a fetus in head presentation and a trial of labor after 37 weeks. A fetus was considered SGA when the estimated fetal weight was less than the 10th percentile at the third trimester ultrasound. A newborn was considered hypotrophic when the birthweight was less than the 10th percentile. RESULTS: 8 153 newborns were included and 948 of the newborns were hypotrophic (308 were suspected for SGA, 640 were not suspected for SGA) and 7205 were eutrophic. Among the hypotrophic neonates, we observed no significant difference regarding the immediate neonatal outcomes between the two groups of fetuses suspected and not suspected for SGA. Among the fetuses not suspected for SGA, the rate of arterial umbilical cord pH below 7.10 was significantly higher in the hypotrophic newborns compared to the non hypotrophic newborns (4.7% vs 3.1%, p = 0.041). CONCLUSION: In our population, unsuspected fetal hypotrophy may be associated with an increased risk of neonatal acidosis. These results emphasize the benefit of improving prenatal screening to identify the SGA fetuses.

Surgical management of complications caused by transcatheter ASD closure.

BACKGROUND: Transcatheter occlusion of uncomplicated atrial septum defects (ASD) is recognized as an effective and minimally invasive method. Sometimes, serious early and late complications require surgical intervention. We therefore investigated reasons and outcomes of the secondary surgical approach. METHODS: 5 patients (aged 5-73 yrs) were admitted to our institution for device explantation and surgical ASD closure. ASDOS devices (A devices) had to be explanted in 4 patients and a SIDERIS "buttoned" occluder (S device) had to be explanted in 1 patient. The period from transcatheter implantation to surgical explantation ranged from 1 hour to 3 years. RESULTS: 3 patients (60 %) had to be operated in an emergency setting. In our youngest patient (5 yrs), the A device separated and embolized into the aorta and pulmonary artery. A pregnant women who needed emergent cesarean section developed hemopericard and tamponade due to atrial perforation by a fractured leg of an A device. In another A device, a suspect endocarditis caused membrane perforation. Malpositioning of an S device was the reason for operation. All patients recovered well without neurological symptoms. CONCLUSIONS: Transcatheter closure of uncomplicated ASD is a feasible alternative but surgical stand-by is essential. Nevertheless more complicated ASD should be operated, especially since the cosmetically satisfactory techniques of minimal invasive heart surgery are available.

Transcatheter closure of a posttraumatic ventricular septal defect with an Amplatzer occluder device.

Cardiac traumatization may lead to severe complications. Transcatheter closure of a postraumatic ventricular septal defect (VSD) was successfully done using an Amplatzer septal occluder in a man who had previously undergone surgery for myocardial fissure and mitral valve dysfunction. In selected cases, the percutaneous approach may be a valuable option to close muscular VSDs.

[Augmented reality in echocardiography. A new method of computer-assisted training and image processing using virtual and real three-dimensional data sets]. / Augmented Reality in der Echokardiographie--Eine neue Methode der computergestützten Ausbildung und Bildbearbeitung durch virtuelle und reale dreidimensionale Datensätze.

Augmented reality (AR) applications link real with virtual image data, in order to increase their information content. In medicine they are especially useful for education and for supporting the interpretation of three-dimensional (3D) image data. Simulators are used to train risky or expensive procedures. In the AR application EchoCom2 a 3D surface model of the human heart is linked with echocardiographic volume data sets. The 3D echocardiographic data sets are registered with the heart model to synchronize it's temporal and spatial orientation. The heart model together with an animated ultrasound sector represents a reference scenario, which displays the currently selected cutting plane within the echocardiographic volume data set. Modifications of the cutting plane within the echocardiographic data are transferred simultaneously and in real time to the reference scenario. The AR application is used as a simulator to train two-dimensional echocardiographic examinations and as an orientation and navigation aid for the exploration of 3D echocardiographic data sets. Beginners in echocardiography have only a rudimentary conception of the spatial relationship between the actual ultrasound image and the 3D anatomy of the heart. They are unable to translate multiple two-dimensional slices into a coherent 3D mental image of the heart. In EchoCom2 the trainee can interactively explore the 3D heart model and the registered 3D echocardiographic data sets by the animated ultrasound sector, whose position is controlled by an electromagnetic orientation and position system (EPOS). The data from the EPOS are used to calculate the echocardiographic images that are analogue to the position of the animated ultrasound sector. EchoCom2 is also used to support the interpretation of 3D echocardiographic data sets. The analysis of 3D echocardiographic data has to be done during a post processing. Defining the exact position of a cutting plane within the volume is difficult due to the lack of a standardized representation, the independence of the cutting plane of any transducer position and the possibility to calculate an indefinite number of views. The simultaneous representation of the current cutting plane both in the volume data, and in the heart model enables the examiner ad hoc to recognize it's position and the visualized structures.

Augmented reality simulator for training in two-dimensional echocardiography.

In two-dimensional echocardiography the sonographer must synthesize multiple tomographic slices into a mental three-dimensional (3D) model of the heart. Computer graphics and virtual reality environments are ideal to visualize complex 3D spatial relationships. In augmented reality (AR) applications, real and virtual image data are linked, to increase the information content. In the presented AR simulator a 3D surface model of the human heart is linked with echocardiographic volume data sets. The 3D echocardiographic data sets are registered with the heart model to establish spatial and temporal congruence. The heart model, together with an animated ultrasound sector represents a reference scenario, which displays the currently selected two-dimensional echocardiographic cutting plane calculated from the volume data set. Modifications of the cutting plane within the echocardiographic data are transferred and visualized simultaneously and in real time within the reference scenario. The trainee can interactively explore the 3D heart model and the registered 3D echocardiographic data sets by an animated ultrasound probe, whose position is controlled by an electromagnetic tracking system. The tracking system is attached to a dummy transducer and placed on a plastic puppet to give a realistic impression of a two-dimensional echocardiographic examination.

[Ventricular septal defect following cardiac trauma: closure with the Amplatzer Septal Occluder]

Ventricular septal defect following cardiac trauma: percutaneous closure with the Amplatzer septal occluder. HISTORY AND CLINICAL FINDINGS: A 36-year old roofer fell 8 m and suffered a severe polytrauma. A complicating pericardial tamponade was relieved as an emergency and myocardial fissure of the left ventricle about 1 cm in length sutured. 2 weeks later, a severe mitral insufficiency due to rupture of the papillary muscle occurred, that was cared by the implantation of a bioprosthesis. 6 weeks later dyspnoea and restricted physical capability were clinically impressive. INVESTIGATIONS AND DIAGNOSIS: Echocardiography demonstrated a posttraumatic muscular ventricular septal defect. Doppler echocardiography and heart catheterization showed a ventricular septal defect still restrictive with a left-to-right shunt (pulmonary-to-systemic flow ratio Qp/Qs 1.8:1). Under exercise, there was a significant increase in mean pulmonary arterial pressure from 27 to 60 mmHg. TREATMENT AND COURSE: The patient who had already been operated twice before was treated by the percutaneous occlusion of the ventricular septal defect from arterio- to venofemoral, a guide catheter was inserted transseptally into the left ventricle. An Amplatzer Septal Occluder, a self-expandable, self-centering wire-mesh double disc with a connecting central stent part, was loaded and then implanted in the ventricular septal defect. The intervention was controlled by fluoroscopy and echocardiography. Post intervention, only a trivial residual shunt was seen. The pumping efficacy of the left ventricle increased, in particular of the septal and apical segments. Clinically, the patient was markedly more load-bearing, the exercise-induced dyspnoea reduced. CONCLUSIONS: Following a cardiac trauma, various complications may occur that can manifest themselves clinically at two or more times. A posttraumatic ventricular septal defect of a patient already operated was successfully occluded by an Amplatzer Septal Occluder. Alongside established surgical methods, the non-operative implantation of new occlusion systems could mean an effective treatment option for muscular ventricular septal defects.

Acute mitral regurgitation due to chordal rupture in a patient with neonatal Marfan syndrome caused by a deletion in exon 29 of the FBN1 gene.

The neonatal Marfan syndrome is an autosomal dominantly inherited disease with an extremely poor prognosis. This report gives a clinical and echocardiographic description of an infant with a mutation in exon 29 of the fibrillin-1 gene (FBN1), a region in which this severe form of Marfan syndrome seems to cluster. The infant died at the age of 3 months due to severe acute mitral regurgitation leading to intractable heart failure.

Color Doppler echocardiographic evaluation of tricuspid regurgitation and systolic pulmonary artery pressure in the full-term and preterm newborn.

Color Doppler echocardiography of tricuspid valve regurgitation (TR) is a valid, noninvasive method of determining systolic pulmonary artery pressure (SPAP). In a prospective study the authors examined 56 healthy full-term newborns (group I), 36 healthy preterm newborns (group II), and 10 preterm newborns with severe respiratory distress syndrome requiring surfactant replacement therapy (group III). Doppler studies were repeated until the transtricuspid gradient was < 20 mm Hg. In 83.3% of children a reproducible spectral curve was recorded at least once. The authors estimated the transtricuspid gradient delta p (RV-RA) by using the modified Bernoulli equation. Within the first twenty-four hours delta p (RV-RA) was < 20 mm Hg in 72.7%, 50%, and 25% of children with measurable TR in groups I, II, and III, respectively, increasing to 91.1%, 78.6%, and 55.6% within forty-eight hours. There was no significant correlation between SPAP and gestational age, birth weight, mode of delivery, and ductal closing time. Continuous holosystolic envelope tracing of TR was recorded in 16.6%. In these patients delta p (RV-RA) was measured markedly higher (mean of 30.1 mm Hg) than in the others (mean 17.3 mm Hg). The authors conclude that there is a high prevalence of TR in neonates, which allows estimation of SPAP in > 80% of newborns without considerable impairment. Normalization of SPAP takes place within four days in most patients, but there is a delay in preterm infants with severe respiratory distress syndrome.

Total UK multi-centre experience with a novel arterial occlusion device (Duct Occlud pfm).

OBJECTIVE: To report the total UK multicentre experience of a novel arterial occlusion device (Duct Occlud pfm). DESIGN: Descriptive study of selected non-randomised paediatric patients with a variety of aortopulmonary connections. SETTING: Five UK tertiary referral centres for congenital heart disease. PATIENTS AND METHODS: Between March 1994 and February 1995, 57 children aged 2 weeks to 14 years (median 50 months) underwent attempted closure of their aortopulmonary connection. Fifty one had persistent arterial ducts and 9 of them had had a Rashkind umbrella device implanted. Five patients had superfluous modified Blalock-Taussig shunts (mBTS). In one there was also a native major aortopulmonary collateral artery (MAPCA). Another patient had a native major aortopulmonary connection (APC). Transcatheter occlusion was attempted in all cases through a 4 F delivery catheter. RESULTS: Devices were successfully deployed in 49/57 (86%) patients. Seven of 51 cases with persistent arterial ducts were judged too large for the device and a Rashkind umbrella was used. 40 (91%) of the 44 in whom the detachable coil device was used had complete occlusion at 24 hours on colour flow Doppler echocardiography. Devices were successfully deployed in all 6 remaining patients (4 mBTS, 1 mBTS + MAPCA, and 1 APC). Embolisation of a device occurred on 4 occasions. Two devices were not retrieved but caused no apparent clinical problems. CONCLUSION: This novel detachable coil type occlusion system compares favourably with other methods of transcatheter occlusion of native, residual, or surgically created aortopulmonary shunts. The delivery system allows its use in small children.

A small interventional device to occlude persistently patent ductus arteriosus in neonates: evaluation in piglets.

OBJECTIVES: We attempted to evaluate the efficacy and tissue reaction of a new miniature interventional ductal occlusion device in neonatal pigs. BACKGROUND: A variety of devices are used to close persistent ductus arteriosus (PDA) by interventional measures. Because of the size of these devices, they have not been applied to term or preterm neonates. Newborn piglets are comparable in size and fragility to human term and preterm neonates. METHODS: Memory-shaped double-cone stainless steel coils were mounted on a titanium-nickel core wire. A snap-in mechanism attaches the coil to the delivery wire, allowing intravascular coil retrieval and repositioning. The system was placed through a 3F Teflon catheter. Two piglet models of PDA were used: 1) ductal patency maintained by stents (n = 6), and 2) ductal patency produced by angioplasty (n = 7) to avoid stent-coil interaction. RESULTS: Placement of the coils within the PDA was possible in all piglets. Before final detachment, the coils were retrieved or repositioned, or both, up to eight times. In all but two piglets the ductus was closed within 1 h of the procedure. The coils were never dislocated and caused no infections or relevant aortic and pulmonary artery obstruction (95% confidence interval for missing complications [0 of 13] extends to 23%). Histologic and electron microscopic studies revealed endothelial coverage of the implants and histiocytic reaction but no local or systemic inflammation or erosion of the implant. CONCLUSIONS: The device was effective in experimental models of PDA. The information obtained warrants initial trials of the device in neonates.

Occlusion of the neonatal patent ductus arteriosus with a simple retrievable device: a feasibility study.

PURPOSE: To examine the feasibility of transvenous placement of a new memory-shaped, small, retrievable coil that has a smaller-caliber delivery system than currently available devices, for closure of the patent ductus arteriosus (PDA). METHODS: Through 4 or 5 Fr vascular sheaths and 4 or 5 Fr end-hold catheters, the coils were delivered and placed in piglets (n = 10) with PDA. The coils were made from 0.018" (0.46 mm) or 0.028" (0.71 mm) stainless steel guidewire. Mounted for delivery, the new device has the appearance of a conventional guidewire. This neonatal PDA model was created without major surgery or drugs by stenting the ductus arteriosus. RESULTS: The memory-shaped coils were easily delivered. Coils not optimally placed were retrieved and repositioned. Occlusion of the ductus arteriosus as early as a half-hour after delivery was shown angiographically and confirmed by histopathology. CONCLUSION: This new, small-caliber, simple device was found to be effective for closure of the PDA in this animal model. Longer-term observations are needed.

Closure of Blalock-Taussig shunts using a new detachable coil device.

Two children underwent interventional closure of a modified Blalock-Taussig shunt using a new detachable coil system (Duct-Occlud). This procedure has advantages over currently available materials to occlude these and other communications between the systemic and pulmonary circulations.

[MR angiography of the heart and thoracic blood vessels. Use of rapid ECG-triggered techniques with multiplanar reconstruction capability]. / MR-Angiographie des Herzens und der thorakalen Gefässe. Anwendung schneller EKG-getriggerter Techniken mit multiplanaren Rekonstruktionsmöglichkeiten.

Segmented 2D times-of-flight (TOF) magnetic resonance angiography (MRA) with ECG gating is a suitable adjunct to conventional magnetic resonance tomography. Based on our experience with 106 patients, cardiovascular MRA primarily aids in the diagnosis of congenital heart disease, but is of minor importance for the evaluation of acquired cardiovascular disease, such as aortic aneurysms. Changes in flow dynamics as a result of spin dephasing may render partial signal loss. Due to flow turbulence and resulting signal loss distal to stenoses, difficulties may arise in correctly determining the degree of stenosis and differentiating between partial thrombosis and slowly flowing blood. The most promising approach for optimizing 2D TOF MRA is enhancement of the intravascular signal and suppression of background signal. For the equipment we use with a 0.5 Tesla scanner, the most powerful combination to enhance intravascular signal and suppress back-ground noise was applying a flip angle of 65 degrees and a short inversion prepulse. The influence of the flip angle and prepulse on the intravascular signal varies, dependent on the flow velocities and heart rates. Secondary reconstructions, such as the MIP technique, contribute to better spatial orientation by using rotating projection views. To avoid overprojection artifacts and loss of diagnostic information, 2D single slices should be the primary source of image analysis.

MR angiography of congenital heart disease: value of segmented two-dimensional inflow technique and maximum-intensity-projection display.

Magnetic resonance (MR) angiography of the cardiovascular system was evaluated in 41 patients with congenital heart disease by using a two-dimensional (2D) inflow technique based on a magnetization-prepared gradient-echo pulse sequence with segmented k-space data acquisition and electrocardiographic gating at 0.5 T. Inversion and saturation prepulses were used to suppress stationary tissue and enhance intravascular signal. Presaturation slabs were applied where certain vascular structures had to be suppressed. Sequence parameters were optimized by evaluating signal intensity and contrast characteristics for various flip angles and inversion and saturation delay times. The heart and intrathoracic vasculature were encompassed with 40-50 overlapping sections. Both 2D angiograms and maximum-intensity-projection images were evaluated. Combining data sets acquired in the sagittal and transverse orientations provided the most satisfactory information about the pulmonary arteries. The highest signal-to-noise ratios were obtained with a flip angle of 65 degrees and short prepulse delay times. Two-dimensional MR angiography can provide useful diagnostic information but requires a thorough understanding of in-plane and hemodynamically induced signal intensity changes.

Longitudinal study in 18 cases of fetal supraventricular tachycardia: Doppler echocardiographic findings and pathophysiologic implications.

A longitudinal study was performed in nine nonhydropic and nine hydropic fetuses with supraventricular tachycardia (SVT). First, because of a lack of reference methods in utero, the validity of spatial (length and area) and temporal parameters for semiquantitative grading of atrioventricular (AV) valve regurgitation by color Doppler flow mapping and M-Q mode imaging was evaluated by a longitudinal intraindividual study before and after drug-induced cardioversion to sinus rhythm and by correlation with the severity of hydrops and the time required for complete remission of hydrops. Second, with the demonstration of AV valve incompetence and changes in venous blood flow velocity wave forms, new data were collected concerning chronic SVT in the fetus. AV valve incompetence occurred in eight fetuses: during SVT only in three severely hydropic fetuses, during sinus rhythm in one nonhydropic fetus, and in seven hydropic fetuses including those previously mentioned, where AV incompetence outlasted tachycardia. The length and area of the regurgitant jet as imaged by color Doppler flow mapping and the temporal duration of regurgitation in relation to the systolic phase as measured by M-Q mode continuously diminished intraindividually but with great interindividual differences in the time span required for complete remission, which ranged from 5 to 42 days. Pulsed-wave Doppler studies of blood flow in the inferior vena cava and left hepatic vein demonstrated normal biphasic forward flow with a systolic and a diastolic surge during sinus rhythm in all fetuses and in two instances also during SVT of 190 and 195 beats/min, respectively. In all cases a pulsatile reversal of blood flow with systolic forward flow and diastolic reverse flow was observed during tachycardia greater than 220 beats/min. Thus the presence of functional AV valve incompetence as a result of annular enlargement seems to be a sign of SVT-induced "cardiomyopathy" during fetal life. AV valve incompetence during SVT was always associated with extremely severe hydrops and seemed to indicate the most severe degree of ventricular dysfunction, which could influence the selection of antiarrhythmic drugs. After termination of SVT, severe AV valve insufficiencies also occurred in other cases of hydrops, probably because of the markedly increased diastolic filling distending the already enlarged annular ring. The observed great interindividual differences in time required for disappearance of AV valve incompetence and hydrops could be explained by a different state of progression of tachycardia-induced "cardiomyopathy" at the time of drug-induced cardioversion.(ABSTRACT TRUNCATED AT 400 WORDS)

Surgical repair of aorto-right ventricular tunnel in an infant.

A rare case of aorta to right ventricle tunnel with associated pulmonary stenosis was corrected on cardiopulmonary bypass in a 3-month-old infant. Both the aortic and ventricular openings were closed with a patch. Although the repair was successful and the child was discharged from the hospital, septicemia developed and she died later. Early repair of this defect is recommended.

Prenatal diagnosis of atrioventricular canal malformations with up-to-date echocardiographic technology: report of 14 cases.

Fourteen fetuses with atrioventricular canal malformations were examined by two-dimensional echocardiography, pulsed-wave Doppler echocardiography, and color Doppler flow mapping. Eleven fetuses had complete and three fetuses had partial atrioventricular canal malformations. Nonimmune hydrops fetalis was associated with six cases, and fetal arrhythmia was seen in three cases. With two-dimensional echocardiography, the atrioventricular canal malformations could be diagnosed accurately. The inclusion of color Doppler flow mapping, however, provided additional hemodynamic information that was important from the prognostic point of view. Incompetence of atrioventricular valves could be demonstrated in 10 of 14 cases by Doppler echocardiography. In nine cases, detailed Doppler echocardiographic evaluation of the regurgitation jet was possible. The proportion of systolic time during which atrioventricular valve insufficiency was demonstrated was related to the occurrence of nonimmune hydrops fetalis. When insufficiency of atrioventricular valves was associated with hydrops (four cases), a pansystolic insufficiency was always present. In cases without hydrops (five), regurgitation was confined to early systole. Thus a reliable method for semiquantitative evaluation of the degree of insufficiency seems to have been found. Moreover, an association appeared to exist between the occurrence of hydrops fetalis and the proportion of atrial area that was taken up by regurgitant jet area, as determined by planimetry in the four-chamber view. Prenatal diagnosis was confirmed by autopsy or neonatal cardiac evaluation. Only one neonate survived in our series. Two were stillborn, four died during the neonatal period, two died during infancy, and pregnancy was electively terminated prematurely in five cases. Eight fetuses were found to have a karyotypic abnormality.

Fetal tachyarrhythmias: transplacental and direct treatment of the fetus-a report of 60 cases.

From 1981 to 1990, 60 fetuses with tachyarrhythmia (21-39 weeks of gestation) were treated in utero. Of these, 54 were cases of supraventricular tachycardia, and six of atrial flutter. Non-immune fetal hydrops was present in 21 cases with supraventricular tachycardia and in five cases with atrial flutter, a total of 26 cases.Transplacental treatment by maternally administered antiarrhythmic drugs (digoxin only or in combination with verapamil) produced good results in non-hydropic fetuses. In this group, all 34 fetuses survived. In fetuses with hydrops, 20 out of 26 survived. In 13 fetuses of the 26 with hydrops, direct fetal therapy was performed in addition to the transplacental therapy when the tachyarrhythmia was refractory to transplacental treatment. During the 9 years of this study, a variety of direct treatment regimes have been used consisting of intraperitoneal and/or umbilical intravenous administrations of different drugs. Since 1988, umbilical vein punctures have shown that the transplacental passage of digoxin (and amiodarone) is hampered in the presence of hydrops, and direct treatment may he necessary in these cases. Amiodarone seems to he the drug of choice for direct therapy. It is highly effective in supraventricular tachycardia and atrial flutter. The long elimination half-time of amiodarone reduces the number of umbilical cord punctures needed to maintain the therapeutic drug level in the fetus.