Christoph Czernik

Mirror Syndrome: A Systematic Review of Fetal Associated Conditions, Maternal Presentation and Perinatal Outcome

Introduction: Mirror syndrome, also referred to as Ballantyne’s syndrome, is normally defined as the development of maternal edema in association with fetal hydrops. The incidence of mirror syndrome is low and few cases have been published. We describe a case report in association with fetal Ebstein anomaly and provide a systematic review on the fetal associated conditions, maternal presentation and perinatal outcome reported for mirror syndrome. Data Sources: A PubMed database search was done until December 2008 (English, French or German) without any restriction of publication date or journal, using the following key words: Ballantyne syndrome, Mirror syndrome, Triple edema, Pseudotoxemia, Maternal hydrops syndrome, Pregnancy toxemia, Acute second trimester gestosis, and Early onset preeclampsia. Reported cases were considered eligible when fetal associated conditions, maternal symptoms and fetal outcome were clearly described. Results: Among 151 publications a total of 56 reported cases satisfying all inclusion criteria were identified. Mirror syndrome was associated with rhesus isoimmunization (29%), twin-twin transfusion syndrome (18%), viral infection (16%) and fetal malformations, fetal or placental tumors (37.5%). Gestational age at diagnosis ranged from 22.5 to 27.8 weeks of gestation. Maternal key signs were edema (80–100%), hypertension (57–78%) and proteinuria (20–56%). The overall rate of intrauterine death was 56%. Severe maternal complications including pulmonary edema occurred in 21.4%. Maternal symptoms disappeared 4.8–13.5 days after delivery. Discussion: Mirror syndrome is associated with a substantial increase in fetal mortality and maternal morbidity.

B-Type Natriuretic Peptide to Predict Ductus Intervention in Infants <28 Weeks

Patent ductus arteriosus (PDA) is frequent in neonates with gestational age of less than 28 wk. Clinical and echocardiographic signs define hemodynamic significance of PDA, but do not reveal the need for PDA intervention in the first days of life. B-type natriuretic peptide (BNP) has been proposed as a screening tool for PDA in preterm infants. To determine whether BNP can predict the need for PDA intervention, plasma BNP was measured by chemiluminescence immunoassay in 67 preterm infants <28 wk (median 26) on the second day of life in a prospective blinded study. PDA intervention was based on specified clinical and echocardiographic findings. Twenty-four patients (intervention group) received treatment for PDA and 43 patients (controls) remained without intervention. BNP concentrations were higher in the intervention (median 1069 pg/mL) than in the control group (247 pg/mL, p < 0.001). BNP correlated positively with ductal size (R = 0.46, p < 0.001) and atrial/aortic root ratio (R = 0.54, p < 0.001). In conclusion, plasma BNP proved to be a good predictor for ductus intervention (area under the curve: 0.86) with the best cutoff at 550 pg/mL on the second day of life in ventilated infants less than 28 wk gestation (sensitivity: 83%; specificity: 86%).

Persistently Elevated Right Ventricular Index of Myocardial Performance in Preterm Infants with Incipient Bronchopulmonary Dysplasia

Objectives Elevated pulmonary vascular resistance occurs during the first days after birth in all newborn infants and persists in infants at risk for bronchopulmonary dysplasia (BPD). It is difficult to measure in a non-invasive fashion. We assessed the usefulness of the right ventricular index of myocardial performance (RIMP) to estimate pulmonary vascular resistance in very low birth weight infants. Study Design Prospective echocardiography on day of life (DOL) 2, 7, 14, and 28 in 121 preterm infants (median [quartiles] gestational age 28 [26]–[29] weeks, birth weight 998 [743–1225] g) of whom 36 developed BPD (oxygen supplementation at 36 postmenstrual weeks). Results RIMP derived by conventional pulsed Doppler technique was unrelated to heart rate or mean blood pressure. RIMP on DOL 2 was similar in infants who subsequently did (0.39 [0.33–0.55]) and did not develop BPD (0.39 [0.28–0.51], p = 0.467). RIMP declined steadily in non-BPD infants but not in BPD infants (DOL 7: 0.31[0.22–0.39] vs. 0.35[0.29–0.48], p = 0.014; DOL 14: 0.23[0.17–0.30] vs. 0.35[0.25–0.43], p<0.001; DOL 28: 0.21[0.15–0.28] vs. 0.31 [0.21–0.35], p = 0.015). Conclusions In preterm infants, a decline in RIMP after birth was not observed in those with incipient BPD. The pattern of RIMP measured in preterm infants is commensurate with that of pulmonary vascular resistance.

Weaning of neonates from mechanical ventilation by use of nasopharyngeal high-frequency oscillatory ventilation: a preliminary study

Abstract Objective. To investigate the feasibility of nasopharyngeal high-frequency oscillatory ventilation (nHFOV) immediately after extubation in difficult-to-wean preterm infants. Study design. This was an observational study of 20 mechanically ventilated neonates [median (range) birth weight 635 (382–1020)g, median gestational age 25.3 (23.7–27.6) weeks] at high risk for extubation failure. Nine infants had failed at least one previous extubation. Fourteen infants were given hydrocortisone. All 20 infants were extubated into nHFOV, with a mean airway pressure of 8 cmH2O, an amplitude of 20 cmH2O, and a frequency of 10 Hz. Results. Infants remained on nHFOV for a median duration of 136.5 (7.0–456.0) h until further weaning to continuous positive airway pressure (n =14) or reintubation (n = 6). Reintubation was performed in 1 of 11 infants who had not experienced any previous extubation, and in five of nine infants who had experienced at least one previous extubation (P < 0.05). PaCO2 was virtually unchanged from preextubation levels 2 h after extubation, but declined significantly at 32 h from 59.8 (45.0–92.3) mmHg to 50.7 (39.8–74.4) mmHg (P < 0.01). PaCO2 returned to preextubation levels upon discontinuation of nHFOV. Conclusion.This small observational study demonstrates that nHFOV can be successfully applied to wean premature infants from ventilator support.

LEFT VENTRICULAR LONGITUDINAL STRAIN AND STRAIN RATE MEASURED BY 2-D SPECKLE TRACKING ECHOCARDIOGRAPHY IN NEONATES DURING WHOLE-BODY HYPOTHERMIA

The purpose of the study was to assess changes in cardiac performance in newborn infants with hypoxic-ischemic encephalopathy during therapeutic hypothermia and rewarming with two-dimensional speckle tracking echocardiography. For eight asphyxiated neonates (median birth weight (range): 3038 (2725-3253) g; umbilical artery pH: 6.9 (6.8-7.18) undergoing whole-body hypothermia (33-34°C), left ventricular longitudinal strain and strain rate, as well as heart rate, cardiac output and left ventricular fractional shortening, were determined at four points in time: the start (T1) and end of hypothermia (T2), immediately after rewarming (T3) and the age of 5 to 7 d (T4). Mean (standard deviation) heart rate increased from 93 (12) beats/min at T1 to 133 (12) beats/min at T4 (p < 0.001). Cardiac output was low during hypothermia (T1: 207 [43] mL/kg/min, T2: 240 [70] mL/kg/min) and increased significantly (p < 0.001) afterward (T3: 329 [70] mL/kg/min, T4: 388 [78] mL/kg/min). Left ventricular fractional shortening remained unchanged. Left ventricular global longitudinal peak systolic strain did not differ significantly between hypothermia and rewarming, whereas the systolic strain rate increased from -1.1 (0.3) s(-1) at T1 to -1.8 (0.26) s(-1) at T4 (p = 0.001). Hypothermia affects peak systolic strain rate, heart rate and cardiac output, with complete recovery after rewarming, whereas peak systolic strain and fractional shortening remain stable.

Hydrops fetalis – has there been a change in diagnostic spectrum and mortality?

Objective. To investigate the impact of medical progress on the diagnostic spectrum and outcome of infants with hydrops fetalis (HF). Study design. We reviewed the charts of all live-born HF infants (n = 70) over a 16-year period (1993–2009). Data were compared to two published case series (Wafelman LS, Pollock BH, Kreutzer J, Richards DS, Hutchison AA. Biol Neonate 1999;75:73–81, Gainesville, Florida 1983–1992, n = 62; Simpson JH, McDevitt H, Young D, Cameron AD. Fetal Diagn Ther 2006;21:380–382, Glasgow, UK 1990–2004, n = 30). Results. Only two cases were immune HF. The proportion of infants with unexplained HF (30%), lymphatic (24%), cardiac (17%), hematologcial (6%) or chromosomal anomalies (6%) did not differ from the published case series. There was also no difference in overall mortality (57% vs. 55% or 67%, respectively). Low gestational age (<34 weeks), low 5-min Apgar scores (<4), and heart failure were independently associated with fatality. Conclusion. The diagnostic spectrum and mortality of HF has changed little over the last 25 years. In the future, new techniques in mutational analysis will be needed to reduce the high rate of unexplained cases of HF.

Hydrops fetalis caused by a large intrapericardial teratoma

flow and interfere with cardiac function, and may cause arrhythmias, stillbirth, or sudden death3. A cardiac tumor may also cause congestive heart failure and hydrops, leading to stillbirth4. Cardiac teratomas are rare and account for less than 2% of cardiac tumors in pediatric patients, but are often detected antenatally. They originate from either the pericardium or within the heart. Most occur in the pericardial cavity and are attached to the great vessels, and a few arise from within the myocardium of the atrium or ventricle5. Typically, intrapericardial teratomas are attached to the root of the pulmonary artery and aorta and compress the adjacent atrium or ventricle depending on their size. The tumors range in size from 2 to 9 cm in diameter6. The main presenting findings in the fetus or neonate with a pericardial teratoma are a tumor on imaging studies and a pericardial effusion7,8. The tumor and/or pericardial effusion can impede venous return and cause cardiac tamponade, resulting in fetal hydrops and stillbirth9–11. The development of fetal hydrops requires treatment by early delivery, in-utero pericardiocentesis, or fetal surgery depending on the gestational age, the anatomy of the teratoma, and the degree of cardiac decompensation9,12,13. A recently published review of the literature14 revealed 31 cases of prenatally diagnosed pericardial teratoma, of which 77% developed concomitant hydrops. In 11 cases pericardiocenteses were performed in utero with subsequent improvement of the cardiac function. Seven of the 31 mothers decided to terminate the pregnancy, 10 of the affected fetuses died perinatally and 14 patients survived the neonatal period. All the surviving newborns underwent surgical resection of the tumor postpartum. The survival rate in this cohort was significantly dependent on the presence or absence of hydrops or other signs of cardiac insufficiency. Our case demonstrates that repeated pericardiocenteses in fetuses with a pericardial teratoma are followed by prompt improvement of the cardiac function that can be assessed by spectral Doppler of the ductus venosus, and result in rapid resolution of hydrops. Furthermore, worsening of the Doppler indices of the ductus venosus can be used to assess the appropriate time points for pericardiocentesis and other interventions.

Development of left ventricular longitudinal speckle tracking echocardiography in very low birth weight infants with and without bronchopulmonary dysplasia during the neonatal period.

Objectives In preterm infants, postnatal myocardial adaptation may be complicated by bronchopulmonary dysplasia (BPD). We aimed to describe the development of left ventricular function by serial 2D, Doppler, and speckle tracking echocardiography (2D-STE) in infants with and without BPD during the neonatal period and compare these to anthropometric and conventional hemodynamic parameters. Study Design Prospective echocardiography on day of life (DOL) 1, 7, 14, and 28 in 119 preterm infants <1500 g birth weight of whom 36 developed BPD (need for oxygen supplementation at 36 weeks gestational age). Non-BPD and BPD infants differed significantly in median (IQR) gestational age (25.5(24–26.5) weeks vs. 29(27–30) weeks, p<0.001) and birth weight (661(552–871) g vs. 1100(890–1290) g, p<0.001). Results The intra- and inter-observer variability of the 2D-STE parameters measured did not depend on time of measurement, although there were significant differences in the reproducibility of the parameters. Low intra- and inter-observer variability was seen for longitudinal systolic strain and strain rate mid septum with a median CV (coefficient of variation) of <4.6%. Much higher CVs (>10%) were seen for the apical segment. While anthropometric parameters show rapid development during the first 4 weeks of life, the speckle tracking parameters did not differ statistically significantly during the neonatal period. Infants with and without BPD differed significantly (p<0.001) in the development of anthropometric parameters, conventional hemodynamic parameters except for heart rate, and 2D-STE parameters: global longitudinal systolic strain rate (GLSSR) and longitudinal systolic strain for the mid left wall (LSSR). The largest differences were seen at DOL 1 and 7 in GLSSR (p<0.001) and in LSSR (p<0.01). Conclusions Reproducible 2D-STE measurements are possible in preterm infants <1500 g. Cardiac deformation reveals early (DOL 1 and 7) ventricular changes (GLSSR and LSSR) in very low birth weight infants who develop BPD.

Interdisciplinary surveillance of intraventricular haemorrhage associated conditions in infants <1000 g

Aim: Intraventricular haemorrhage (IVH) causes some of the most adverse outcomes in infants with birthweight <1000 g. Incomplete antenatal steroids, acidosis, inflammation, postnatal transfer, delayed surfactant administration, hypothermia, hypotension, hypocapnia, persistent ductus arteriosus and pneumothorax are all associated with IVH. We hypothesized that prospective surveillance of these IVH‐associated conditions decreases their frequency and thus the frequency of IVH.

Comparison of left ventricular cardiac dimensions between small and appropriate for gestational age preterm infants below 30 weeks of gestation

Abstract Objective: The development of the left cardiac structures in the early life of small for gestational age (SGA) preterm infants has been inadequately studied. The aim of the study was to compare the left ventricular cardiac dimensions between SGA and appropriate for gestational age (AGA) preterm infants. Methods: Echocardiography was performed on day 2 (T1) and at age 1 month (T2) in 19 SGA preterm infants (birthweight <3rd percentile) and 21 AGA controls (P25-50) matched for gestational age (<30 weeks). Results: The left ventricular cardiac dimensions did not differ significantly between SGA and AGA infants at either T1 or T2 after correction for body length. The left ventricular mass was similar for SGA and AGA infants at T1 and T2 after correction for body weight or body length. The left ventricular output increased significantly from T1 to T2 in both SGA and AGA infants [SGA: 350 (T1)–640 (T2) mL/kg/min, P<0.05; AGA: 300 (T1)–510 (T2) mL/kg/min, P<0.05]. Conclusions: The left cardiac structures related to body length are comparable between SGA and AGA preterm infants in the first month of life and show similar development.