Pulmonary capillary haemangiomatosis (PCH) is a rare disorder of the lung, well described in adult literature. PCH is characterized by capillary proliferation, infiltrating the interstitium and alveolar walls. This leads to development of respiratory distress and to end-stage pulmonary hypertension. Mostly young adults are affected. The affection of newborn is described in less than ten cases in literature in the past forty years. PCH is a mostly deadly ending disease. We present a preterm born infant with antepartal diagnosed hydrops fetalis, who died 30 minutes after birth. Autopsy revealed PCH as lethal reason and not cardial disease as presumed before.
OBJECTIVES: To evaluate short- and long-term growth in fetuses with growth restriction (FGR) and elevated umbilical artery Doppler (UAD) systolic/diastolic (S/D) ratios.
METHODS: In this prospective observational study, two UAD waveforms were obtained from each umbilical artery weekly and were classified as normal or abnormal. Fetal growth was assessed every 3 weeks. Short-term growth was calculated from the first visit with elevated ratios until next growth assessment. Results were grouped by number of initial elevated S/D ratios (maximum, 4). Long-term growth was evaluated by change in estimated fetal weight from diagnosis of FGR to birth weight. Fetuses were grouped by average number of elevated S/D ratios and compared to a reference population of growth restricted fetuses with normal testing.
RESULTS: Of 241 fetuses evaluated, 105 demonstrated elevated S/D ratios. Short-term growth was impaired when fetuses had elevated S/D ratios. Long-term growth was affected when the average number of elevated S/D ratios was ≥1 per visit. Progressive 3 or 4 growth delay was noted as the average number of abnormal S/D ratios increased.
CONCLUSIONS: Short- and long-term fetal growth are affected by elevated UAD S/D ratios. Fetuses with more abnormal values initially and those with a higher average of elevated values over pregnancy demonstrate decreased growth.
Introduction: APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 - 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality. Material and methods: In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome. Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05). Conclusions: There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.
Heart defects which includes narrowing of aortic isthmus - aortic coarctation (CoA) are one of the most prevalent birth defects. Making a correct prenatal diagnosis of CoA is very difficult and problematic. We are still observing many false (+) and false (-) diagnoses. In presenting 3 cases with prenatal suspicion of CoA only one patient confirmed this defect in the postnatal life. In the fetal echocardiography inappropriate dimensions of great vessels and PA/Ao ratio are very relevant in the CoA diagnostics. Based on such suspicion before delivery we can select a group in which birth in the tertiary center, prostin infusion, control ECHO examinations and planned cardiac surgery will be needed. But wide differential diagnosis including pulmonary dilatation (due to pulmonary hypertension or fetal blood redistribution due to possible infection) is required.
This is a case report about very rare findings in 2nd half of pregnancy (after normal 1 trimester scan ) at 18th week of gestation fetal macrosomia was detected unrelated to maternal diabetes, and acceleration fetal growth later on with unusual cardiac abnormalities (fetal cardiomegaly, cardiomyopathy, partial abnormal venous connection ). Progressive features of congestive heart failure with polyhydramnios in a fetus with estimated 5500 g predicted a poor outcome and severe neonatal condition, which was presented and discussed with the parents to be. Casearean section was performed at 33rd weeks of gestation due to maternal dyscomfort, severe legs edema and her tachypnoe. Baby boy was delivered with birth weight of 5050g, Apgar 4 with mutiple tumors. Conservative care was introduced and neonated died on the 3rd day. Differential diagnosis was discussed with special attention to Costello syndrome however without proved by genetic make-up from neonatal blood.
Introduction: Echocardiography of the fetal heart is an ultrasound examination that allows the evaluation of the anatomical structure and cardiovascular system usually performed in first half of pregnancy. Material and methods: This work presents the data of 107 fetuses with normal heart anatomy (NHA) and normal heart study (NHS) and their neonatal follow-up. In this group (in an addition to routine prenatal work-up) has been performed also a echocardiography examination in the third trimester of pregnancy, after 37th week of gestation: 61% of pregnant women were referred to the prenatal cardiology center due to the presence of high-risk pregnancies and 39% were low-risk pregnancies. Results: In two cases episodes of fetal arrhythmias were present during obstetrical examinations. After birth in the study group of 107 fetuses, 72% of newborns left the hospital during the 4 days and 28% newborns stayed in the hospital for longer time. In 16 cases their stay was extended due to maternal reasons and in 14 for newborns reasons. Conclusions: 1) In the neonate group, after prenatal cardiac evaluation > the 37th week of gestation such as “ normal fetal heart anatomy & normal heart study”, all newborns in our center were born in good general condition. 2) Late prenatal echocardiography in 3rd trimester of pregnancy maybe considered as additional tool to prove fetal well being, specially in high risk pregnancies.
Introduction: Fetal echocardiography is a method of choice for diagnosing cardiovascular anomalies prenatally. However, in the majority of cases, the complexity of a defect creates a diagnostic challenge. Moreover, postnatal validation of sonographic findings rarely can be obtained. Nevertheless, the feedback is vital for improving diagnostic capabilities. Thus, the aim of this research was to compare results of prenatal echocardiography with postnatal angio-CT in patients with anomalies of great vessels. Material and methods: We retrospectively compared results of prenatal echocardiography and postnatal angio-CT in 10 patients with selected anomalies of the aortic arch. This was a qualitative analysis, thus discrepancies in recognized anomalies were compared between these two modalities. Results: In 8/10 patient diagnoses were fully consistent. Nevertheless, the tiny caliber of vessels created a diagnostic challenge (e.x. to differentiate the hypoplastic aortic arch from the aortic arch interruption). In the remaining case, the discrepancy was due to a problem with complete visualization of all branches of the aortic arch in prenatal ultrasound. Conclusions: Fetal echocardiography in tertiary center was a reliable method for assessment of great vessels anomalies. However, critically narrow vessels remain a diagnostic challenge and neonatal angio-CT seems to be the method of choice in cases of diagnostic doubts.
Vein of Galen Malformation (VGM) it is the most common cerebral arteriovenus malformation in fetuses and children. Usually VGM causes volume overload of the heart and can induce mass effect in the brain, causing progressive neurological impairment. Modern treatment allow on earlier therapy (before the 5th monthof life). This gives the newborn with isolated VGM and heart failure a chance of survival. This work presents case of isolated vein of Galen malformation with diagnosed cardiomegaly, monophasic flow through the tricuspid valve, large right heart, pericardiac effusion, dilatation of SVC and abnormal doppler exam. Postnatal management included aggressive medical treatment of cardiac failure and early transarterial embolization of the selected vessels feeding the aneurysm at 6 days of age. The newborn is 6 month old, growing normally without neurological deficits or developmental retardation and waiting of next stage therapy. New techniques of treatment give fetuses with VGM and prenatal signs of cardiac insufficiency a chance of survival and healthy life, in a group of selected cases. The analysis of the cardiovascular system in fetuses with VGM according to uniform criteria is essential in order to assess the influence of these malformations on the survivability of newborns.
International Prenatal Cardiology Collaboration Group (IPCCG) links specialists from prenatal cardiology all over the world. In this recommendation we would like to focus on the fetal/prenatal echocardiography official report. So far many recommendations focused mainly on technical aspects of the fetal heart examination.