Cantrell’s pentalogy is a congenital defect characterized by uncompleted fusion of the anterior chest wall, resulting in an extrathoracic location of the heart. Ultrasound diagnosis during the first trimester of prenatal life is possible, and termination of pregnancy is usually chosen by pregnant women. We analysed 57 fetuses: 56 from literature and one additional recent case from our institute (from 2016) to evaluate what was the survival rate reported after prenatal diagnosis, including the possibility to terminate the pregnancy, intrauterine deaths and neonatal deaths. We found 10 survivors - 18% since 1984.
Despite dismal prognosis of fetal ectopia cordis, there is a chance for postnatal survivorship probably due to evolving anatomical structures, not only in the first trimester of pregnancy but also during the following weeks of prenatal life.
Prenatal diagnosis is an integral part of modern perinatal care. In the article results of questionnaires pertaining to the prenatal process of diagnosis are presented. Parents whose children were afflicted with congenital malformations of all types responded to enquiry
Materials and methods:
Between March 2014 and March 2015 150 of 355 infants were hospitalized in the Department of Pediatric Intensive Care and Congenital Malformations in Łódź, and 150 had congenital malformations.
101 parents of 150 children (67,3%) have given the feedback. Anomalies were such as: of the digestive system (37%), CHD (25%), OUN (14 %), genito-urinary (13%), skeletal system (9%) and respiratory system (2%). In 65 children of 101 the defects were detected prenatally. The obstetric US exam was the most frequently pointed out as performed (more than 1200). The biochemical markers and genetic tests in were performed in 34 pregnancies. The high percentage of ability to detect malformation was reported in the group of fetal echo examinations.
1. Prenatal ultrasound exams were the least effective method of making appropriate prenatal diagnosis of congenital malformation.
2. Fetal echocardiography had a high level of sensitivity and specificity in detecting congenital malformations.
3. Prenatal cardiologists proved to be the most effective in detecting congenital malformations 89,3 % of detected abnormalities.
4. Biochemical exams had a positive result in only one case of Down Syndrome.
Civil liability for medical malpractice may be attributed either to a doctor or a hospital when any of these persons’ acts or omissions cause injuries to a patient; it may be also the hospital’s liability for the damage caused by negligence of its staff (doctors and other personnel). The rules that govern this liability and the way of compensating the damage are different due to the grounds on which the doctor performs medical services and, in case of hospital’s liability, the relation between a doctor and a health care institution. A doctor who runs his private medical practice bears civil liability individually and is obliged to pay damages if any of his patient suffers injury in connection with the treatment. However, a doctor who acts as employee of a health care institution is protected by the provisions of the Labour Code and exempted from civil liability to a patient. On the other hand, a so-called independent contractor’s liability is joint and several with a hospital that has engaged him. However, case law seems to protect such doctors and treat them as hospital’s employees if certain premises are fulfilled (like de facto subordination of the doctor to the head of the ward).
Polish National Registry for Fetal Cardiac Malformations ( initiated in 2004) was opened for primary practicioners as well as for the referral centers performing or basic fetal heart evaluation or targeted fetal echocardiography. None of the physicians until current era had regular education of fetal cardiology . It was necessary to create an audit - veryfication system, which was provided as a checking each record by the 3 most experienced fetal cardiologists in Poland, using randomised computer system. The aim of this analysis was a retrospective evaluation of „Negatively Verified”
Material and methods: The total number of fetuses in Registry during 2004 and 2013 was 5682 and there were 170 negative verified cases. Every „negative case” was analyzed and qualified to one of five categories: An error in classification of the severity of CHD ; computer mistakes , reported other prenatal problems but not CHD, different interpretation of the images (freezed frames or cine loops) and bad order of the labels of cardiac anomalies.
Results: The percentage of negative verifications was similar every year and total number of negative verification was 2,9% . The main reason for negative was first of all unproper fetal heart classification in 71 cases (42%). In majority the differences in interpretations were minor: but there were 5 huge differences between primary and secondary interpretation.
It was retrospective analysis of prenatal echocardiography findings in fetuses with congenital heart defects, who died in our institution and had an autopsy exams in years 2010 - 2015.
Material and methods:
Among total 115 deaths the pulmonary hypertension based on histopathology criteria was present in 83 cases (72%) as a leading cause of their deaths. Out of 83 neonates 40 underwent prenatal echo, 43 did not, however in both groups there were similar types of heart defects.
The prenatal echo findings from study group (n=40), from the last echo before the delivery were compared with control group and group of HLHS who did survive neonatal surgery and were discharged from hospital. There were statistical differences between pulmonary artery/aorta ratio in fetuses in control group and fetuses in study group („pulmonary hypertension” after birth) (p=0,044). There were statistical differences between pre-delivery pulmonary artery/aorta ratio in fetuses in study group (with „pulmonary hypertension” after birth) and in group of fetuses with HLHS, alive & well after first surgery (p=0,027). There were no differences between pulmonary artery/ aorta ratio fetuses in control group and fetuses with HLHS, alive & well after first surgery (p=0,38)
1) Pulmonary hypertension was a frequent cause of neonatal deaths among our series of congenital heart defects
2) Dilatation of pulmonary artery (and increased pulmonary/artery ratio ) in fetal echo just before delivery may be an important risk factor for poor neonatal outcome in congenital heart defects.)
We present an extraordinary case of congenital enlargement of the right atrium diagnosed at 31 weeks of gestation. This case emphasizes the fact that timing of the detection of this particular cardiac malformation is of capital importance to tract the optimal treatment strategy in order to monitor further progression (in this case accompanying thrombosis) and prevent complications.
In this report a case of aortopulmonary window (APW) diagnosed at 26 hbd is presented. APW supported the pulmonary circulation in neonate afflicted with pulmonary stenosis. To our knowledge, this is the first report in the literature referring to observing their coincidence in fetal life.