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#3
24.03.2004, 19:48
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Дорогая Ирина!
Не удивляйтесь молчанию аудитории! Зарубежным коллегам могут быть не интересны телекоммуникации с Бакулевским институтом. Хотя для них у меня есть вопрос, с этим связанный: «насколько доступна для таких детей помощь в Вашем регионе? Насколько эта доступность зависит от кошелька их родителей?» Что касается наших соотечественников: например я долго не решался ответить Вам. Может быть, Вы поймете, почему… На текущий момент в нашем регионе имеется обреченная группа детей. Та, о которой пишете Вы. Считается, что их перегоспитализация в центральные клиники невозможна. Это официальная точка зрения. Вероятно, нескольким энтузиастам где-то удавалось вытащить нескольких таких детей? Я не исключаю, что это могло быть возможным только благодаря личным связям и настойчивости отдельно взятой личности. У меня такого опыта нет. Если у он имеется у Вас, то не могли бы Вы поделиться им? |
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#4
24.03.2004, 20:05
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Уважаемый Леонид!
В моей прошлой практике, когда я работала в области, были случаи общения с Бакулевским институтом и госпитализация детей на кардиохирургическое лечение. Так как Екатеринбург /ОДКБ/ не брал таких детей, заявляя диагноз поставили и лечите дальше. У меня получилось отправить на косультацию в Бакулевский институт, благодаря общению с врачами института им Бакулева по e-mail, ребенка в возрасте 1 мес с опухолями в сердце /рабдомиомы/, и детей с тяжелыми синими пороками: один - с праворасположенным правосформированным сердцем, атриовентрикулярная дискордантность, атрезия легочной артерии тип 1,5, стеноз устья левой легочной артерии, отхождение аотры от анатомически правого артериального желудочка, "верхомсидящий" левый трикуспидальный клапан, подаортальный дефект межжелудочковой перегородки, открытое овальное окно, открытый артериальный проток, артериальная гипоксемия - этот ребенок был оперирован в Бакулевском институте, его я видела в возрасте 3 месяцев с положительной динамикой, ну а потоя я уехала, дальнейшая судьба не известна; второй - с атрезией легочной артерии с интактной межжелудочковой перегородкой, резкая гипоплазия правого желудочка, выраженная недостаточность трехстворчатого клапана, межпредсердное сообщение открытый артериальный проток, выраженная артериальная гипоксемия - этот ребенок так же был прооперирован в Бакулевском институте и была положительная динамика. Сейчас, конечно же я не знаю о судьбе этих детей, так как поменяла место жительства. Таким образом, возможно оказать помощь этим детям, если в них быть заинтересованным, но увы сейчас я этого сделать не могу, так как работаю просто врачом в большом городе, где есть кафедралы и доценты. С уважением Ирина |
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#5
24.03.2004, 20:20
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Цитата:
Что касается тактики в роддоме, то если говорить в общем это: 1)диагностика порока; 2)оценка степени стеноза на выходе из правого желудочка; 3)при высокой степени стеноза начало введения простогландина и организация перевода для проведения операции. Для роддома, думаю, достаточно более или менее. |
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#6
24.03.2004, 20:30
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Не уверен
Кажется, в прошлом году, с большой помпой открывали в клинике г-на Габинского (кардиоцентр) отделение (или центр) телемедицины. Компутеры новые, экраны здоровенные. Говорили, что помогут всем. Не знаю правда ли, но попробуйте провентилировать этот вопрос с врачами кардиоцентра.
Ребенку то должны помочь. |
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#8
25.03.2004, 18:51
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В нашей области в 2003г. на каждого неонатолога приходился в среднем один новорожденный, которого пришлось похоронить. Каждый пятый из них умер от врожденного порока сердца. Это значит, что, по крайней мере, более чем полусотни врачей задумались о том, как они могли бы помочь этим детям. Может быть, вентилировали эти вопросы с теми и с другими. Ответ один – нет никакой возможности помочь.
Областная стратегия снижения смертности в этой группе детей сводилась к элиминации пороков: - диагностика задолго до рождения - прерывание беременностей с некурабельными пороками (т.е. в идеале, дети с тетрадой Фалло не должны рождаться) Можно по-разному относиться к такому подходу, но почему-то эта стратегия не работает как задумано. На мой взгляд, этот подход нарушает некие общие этические принципы антенатальной охраны плода. Уважаемый Владлен, как у Вас решаются вопросы прерывания беременности в случае пренатальной диагностики таких пороков? |
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#9
26.03.2004, 08:50
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Уважаемый Владлен!
В нашем роддоме диагноз ВПС мы можем поставить, если ребенок на самостоятельном дыхании и в стабильном состоянии, что бы он смог выдержать транспортировку на руках в другое лечебное учреждение, где это обследование проводится платно, т.е. если мама согласна оплатить это обследование. А потом, если порок сложный, то консультация кардиолога из другой больницы, который на переносном аппарате снимет ЭКГ, а затем порекомендует его отправить в неврологическое отделение для новорожденных, так как кардиологическое отделение не лицензировано для лечения новорожденных, они берут детей только после 1 месяца. В неврологическом отделении, если ребенок случайно закашляет - его быстренько в другую больницу - в инфекционную и т.д. И ребенок мотается из одной больницы в другую. Такая ситуация в наших условиях. Да, в настоящее время по губернаторской программе в ОКБ открывается -открыто кардиохирургическое отделение, где возможно будут оперировать и новорожденных, но когда это будет? Может быть в другом регионе и по другому. Уважаемые коллеги поделитесь опытом. |
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#10
26.03.2004, 09:07
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По поводу пренатальной диагностики ВПС
Obstetrics and Gynecology Clinics
Volume 31 • Number 1 • March 2004 Screening for congenital heart disease Lynn L. Simpson, MD Department of Obstetrics and Gynecology Columbia University Medical Center PH-16 622 West 168th Street New York, NY 10032, USA E-mail address: [Ссылки доступны только зарегистрированным пользователям ] PII S0889-8545(03)00127-X Most pregnant women in the United States undergo at least one ultrasound examination before delivery. Prenatal ultrasound performed after the first trimester requires an assessment of fetal anatomy. Both the American Institute of Ultrasound in Medicine and the American College of Obstetricians and Gynecologists recommend that the four-chamber view be part of this assessment during routine prenatal ultrasonography in the second and third trimesters [1] [2] . Although assessing multiple views of the heart increases the likelihood of prenatal detection of congenital heart disease, screening for cardiac defects in the fetus begins with the four-chamber view. Basics of the four-chamber view Although visualization of the fetal heart is possible in the first trimester, the optimal time to perform cardiac screening is between 18 and 22 weeks gestation using high-resolution real-time ultrasonography. The four-chamber view easily can be incorporated into a 30-minute screening midtrimester ultrasound with adequate visualization in over 95% of fetuses [3] . A simple approach to the four-chamber view includes an evaluation of size, position, anatomy, and function of the fetal heart [4] . The four-chamber view is obtained on a transverse image of the fetal thorax just above the diaphragm (Fig. 1) . In this transverse plane, the fetal heart occupies about one third of the area of the fetal chest with an axis about 45 degrees to the left [5] . The atrial and ventricular chambers, interventricular septum, foramen ovale, and atrioventricular valves can all be assessed on the four-chamber view. The two atria and two ventricles should be similar in size, with the left atrium closest to the spine and the right ventricle closest to the sternum. The flap of the foramen ovale should project into the left atrium through a patent foramen ovale. The internal surface of the left ventricle is smooth-looking compared with the trabeculated right ventricle containing the moderator band. The two atrioventricular valves meet at the junction of the interatrial and interventricular septa to form the crux of the heart. The mitral and tricuspid valves should move freely, with the tricuspid valve attached slightly more toward the apex than the mitral valve on the interventricular septum. Ventricular systolic function can be assessed subjectively by observing the ventricular wall movement during systole. The presence of a pericardial effusion also can be identified on the four-chamber view. Assessing the four-chamber view is more than just counting the chambers of the fetal heart. A significant amount of information easily can be obtained by incorporating a systematic evaluation of the four-chamber view into routine screening ultrasonography. Performance of the four-chamber view The four-chamber view was introduced as a screening tool for the prenatal detection of heart anomalies over 15 years ago [6] [7] . Initial reports suggested that the four-chamber view could detect 80% to 90% of fetuses with congenital heart disease [7] [8] . The sensitivity of the four-chamber view to detect cardiac anomalies varied widely, however, in subsequent studies [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] . For example, only 16% of fetuses with heart defects were detected using the four-chamber view in the highly publicized routine antenatal diagnostic imaging with ultrasound (RADIUS) trial of prenatal ultrasonographic screening, and no cardiac malformations were detected before 24 weeks gestation in facilities other than tertiary-level referral centers [22] . |
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#11
26.03.2004, 09:09
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продолжение
There are many possible explanations for the inconsistent performance of the four-chamber view in screening for congenital heart disease. Different ultrasonographers with varying levels of skill use the four-chamber view under a variety of conditions in clinical practice. Performance is also significantly influenced by such factors as a community setting versus a tertiary care center, high-risk versus low-risk patients, level of ascertainment, and availability of outcome information. Screening by skilled sonographers, experienced perinatologists, and expert pediatric cardiologists at teaching hospitals and tertiary reference centers is expected to be superior to that performed in the community. This is reflected in the poor detection rate reported in nontertiary care centers in the RADIUS study [22] . The sensitivity of any screening test also depends on the prevalence of disease in the population being studied. Tertiary care hospitals see more affected fetuses because they are the facilities to which women with abnormal serum screening, advanced maternal age, and high-risk factors for congenital heart disease are referred for evaluation. The prevalence of congenital heart disease in many tertiary care centers is twice that expected in the general population [23] [24] .
The ability to image the fetal heart is also influenced by gestational age, fetal position, amniotic fluid volume, and the maternal body habitus. Previous abdominal surgery also can adversely affect the image obtained [25] . Even when the four-chamber view is achieved, it is unreasonable to expect that it identifies all cases of congenital heart disease. Certain defects are easily missed on the four-chamber view, such as ventricular septal defects, atrial septal defects, coarctation, tetralogy of Fallot, transposition of the great arteries, double-outlet right ventricle, truncus arteriosus, and total anomalous pulmonary venous return [26] . Prenatal diagnosis of patent foramen ovale and patent ductus arteriosus is precluded by their normal patency in utero. The superior aspect of the interventricular septum tends to be thin, particularly in the apical view, and can be diagnosed incorrectly as a subaortic ventricular septal defect. Small muscular ventricular septal defects are the defects most commonly missed on prenatal ultrasonography. Certain cardiac malformations, such as transposition of the great vessels, double-outlet right ventricle, and tetralogy of Fallot, can be associated with a normal four-chamber view. Although most congenital heart defects occur during the period of organogenesis, some defects are known to evolve over the course of gestation and may be missed at the time of midtrimester screening. Flow abnormalities, such as valvular pulmonic stenosis, aortic stenosis, and coarctation of the aorta, are not easy to detect on the four-chamber view. Ventricular hypoplasia has been observed to develop as pregnancy advances and may not be evident on cardiac imaging in the second trimester [27] . Abnormalities of the distal pulmonary arteries and pulmonary veins are not commonly appreciated prenatally because of limited flow and filling of these vessels in utero. Even under optimal conditions, some defects are not detected at a midtrimester scan by the four-chamber view. When used as a screening tool in the general population, the four-chamber view can be expected to detect 40% to 50% of cases of congenital heart disease [7] [28] . The four-chamber view should be considered abnormal if there is ventricular or atrial disproportion, myocardial hypertrophy, dilation or hypoplasia of the chambers, septal defects apart from the foramen ovale, or abnormalities of the atrioventricular valves. Congenital heart disease may also be associated with abnormal positioning of the heart in the fetal chest and with axis deviation [29] [30] . Defects expected to be associated with an abnormal four-chamber view include hypoplasia of the right or left ventricle, atrioventricular septal defect, double-inlet ventricle, Ebstein's anomaly, single ventricle, and large ventricle septal defect. Screening with the four-chamber view may also identify dextrocardia, situs inversus, ectopia cordis, cardiomyopathies, pericardial effusion, cardiac tumors, valvular atresia, stenosis, and insufficiency. Hypoplastic ventricles and atrioventricular septal defects are the defects most often detected prenatally by the four-chamber view [26] [28] [31] . |
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#12
26.03.2004, 09:12
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продолжение
Ventricular outflow tract views
A limitation of cardiac screening with the four-chamber view alone is that conotruncal anomalies easily can be missed. Defects of the great vessels are associated with an abnormal four-chamber view in only 30% of cases [32] . Normal four-chamber screening can occur with transposition of the great arteries, tetralogy of Fallot, double-outlet right ventricle, pulmonary and aortic stenosis, and coarctation of the aorta [31] . Consequently, many experts recommend that views of the aortic and pulmonary outflow tracts be included with an evaluation of the four-chamber view when screening for congenital heart disease [33] . With adequate training and experience, it is possible to visualize the four-chamber view and outflow tracts in 90% of pregnant women [25] . The long-axis view of the left ventricular outflow tract and the short-axis view of the right ventricular outflow tract are the standard images used to evaluate the ventriculoarterial connections [Figs. 2 and 3]. The aortic and pulmonary outflow tracts are approximately equal in size in the midtrimester and should be seen to cross as they arise from their respective ventricles during real-time imaging. The aorta arises from the posterior ventricle and has branches originating from its arch that supply the head and upper extremities. The pulmonary artery arises from the anterior ventricle and branches into the ductus arteriosus and pulmonary arteries. Performance of outflow tract views Overall, the prenatal detection of cardiac anomalies can be increased from 40% to 50% with the four-chamber view alone to 60% to 80% when the ventricular outflow tracts are also assessed [10] [34] [35] . Although the highest detection rates of congenital heart disease have been reported in high-risk populations screened at referral centers, Kirk et al [28] reported a sensitivity of 66% for screening with both the four-chamber view and outflow tracts in a study of primarily low-risk patients. It is clear that multiple cardiac views are crucial for midtrimester screening for many serious congenital heart defects and that the 20% to 30% increase in detection rate that results from including the outflow tracts along with the four-chamber view can be of clinical importance [36] [37] [38] . Universal screening with the four-chamber and outflow-tract views may be optimal for midtrimester cardiac assessment. Importance of screening Congenital heart disease is a common condition with a prevalence of 8 per 1000 live births [39] . Heart anomalies are estimated to be responsible for most infant and childhood deaths related to congenital malformations. The prenatal diagnosis of congenital heart disease also carries a poor prognosis. Intrauterine fetal death occurs in 20% to 30% of cases; neonatal death in 40% to 60% of cases; and long-term survival rates are low, ranging from 15% to 40% [40] [41] [42] [43] [44] . The presence of extracardiac anomalies and chromosomal abnormalities contributes to the poor outlook. Overall, 25% to 45% of fetuses with congenital heart disease have other malformations and 15% to 50% have abnormal karyotypes [35] [45] [46] . Other poor prognostic signs include hemodynamic abnormalities and the presence of hydrops. When congenital heart disease is detected prenatally, these associated findings can be important in counseling families about the likelihood of postnatal survival. |
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#13
26.03.2004, 09:12
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продолжение
Congenital heart disease is eight times more common than trisomy 21 and four times more common than neural tube defects, two conditions for which universal screening programs are in place. Estimates indicate that 10.3 of 1000 fetuses are affected by congenital heart disease at the time of midtrimester ultrasonographic screening [47] . About 50% of cardiac defects are considered major and likely to have a significant impact on long-term morbidity and mortality. Early prenatal diagnosis of major defects is important for counseling patients about pregnancy options, therapeutic interventions, changes in obstetric care, and alternative plans for delivery.
Studies have shown that when major congenital heart disease was diagnosed in the second trimester of pregnancy, from 8% to as many as 80% of patients chose to terminate pregnancy [31] [39] . In one survey of 65 women who previously had borne a child with congenital heart disease, 58% said they would elect to terminate a subsequent affected pregnancy [48] . Without universal ultrasonographic screening, the prenatal diagnosis of congenital heart disease is often made late in pregnancy when abortion is no longer available [39] . With early screening and detection of congenital heart disease, patients and their families can consider the options and make the choices that are best for them. Therapeutic interventions and improved neonatal survival are possible when congenital heart disease is detected prenatally and the timing, mode, and location of delivery can be planned. Referral to a tertiary care center where immediate therapeutic and palliative interventions are available can be lifesaving. Prompt infusion of prostaglandin E1 or balloon atrial septostomy can significantly improve prognosis for newborns with certain cardiac defects that require postnatal maintenance of fetal flow pathways. Experienced neonatologists, pediatric cardiologists, and cardiac surgeons may have a significant impact on an infant's condition and ultimate outcome. Population for screening Considering its prevalence, clinical significance, and the availability of therapeutic interventions, congenital heart disease has the characteristics of a disease for which prenatal screening is appropriate. One strategy for prenatal detection is to screen patients identified to be at risk. Unfortunately, most cardiac malformations are missed if screening is done only on patients with recognized risk factors, such as a positive family history, maternal diabetes, and exposure to teratogens [49] . The traditionally accepted indications for cardiac screening are not highly predictive of subsequent congenital heart disease. More than half of prenatally detected cardiac defects are found in patients with no predisposing risk factors [34] . Overall, 90% of such defects occur in patients who are at low risk for these problems; therefore, screening on the basis of risk factors alone is not particularly effective. Interestingly, the factor most predictive of congenital heart disease is an abnormal cardiac examination at the time of prenatal ultrasonography. In the study by Cooper et al [49] of fetal echocardiography, only 4% of patients were referred because of an abnormal cardiac screen, yet defects were detected at a rate of 68% in this group, far in excess of the rate for all other risk factors combined. In many centers, an abnormal cardiac screen during routine ultrasonography has become the most common reason for referral for fetal echocardiography [43] [50] . Universal screening makes sense because the detection of an abnormal fetal heart on prenatal ultrasonography identifies more cardiac defects than does any of the other traditional risk factors for congenital heart disease. Summary Congenital heart disease has the characteristics of a disease that is suited to screening, and the four-chamber view is an effective screening tool with a sensitivity of 40% to 50%. The use of multiple cardiac views can increase the prenatal detection to 60% to 80%. Given that most infants with congenital heart disease are born to low-risk women, routine screening is warranted. Early prenatal diagnosis provides an opportunity to exclude associated extracardiac and chromosomal abnormalities, discuss pregnancy options, adjust obstetric management, prepare parents for delivery of an affected baby, and plan delivery in a tertiary care center. Despite the widespread use of ultrasonography, only 15% to 30% of infants with congenital heart disease are identified prenatally [39] [49] . There is a need to do better. |
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#14
26.03.2004, 19:56
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Уважаемый Владлен!
Огромное спасибо за интересную статью по пренатальной диагностике ВПС. В условиях родильного дома, когда такой ребенок уже родился, где и как он оперируется в Вашем регионе? Как он транспортируется, если внутриутробно не диагностирован? Часто ли в Вашем регионе бывают дети с тяжелыми пороками сердца? Владлен на медико.ру есть тоже врачебные конференции по неонатологии, интенсивной терапии - сайт Спб, так там долго обсуждался вопрос нужен ли нам индометацин внутривенный. Так как он не зарегистрирован и его нет на нашем рынке. В родильном доме, мы не пользуемся и простогландинами. Так как диагностики на месте у нас нет, мы не можем подтвердить-оценить дуктус-зависмость, поэтому и применение данного препарата пока не возможно. Пока у нас еще очень много проблем, требующих своевременного решения. С уважением Ирина |
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#15
26.03.2004, 21:06
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Кстати говоря, "роддом" - это очень не удобно. Родильное отделение многопрофильной больницы - это значительно более функционально. И больных новорожденных, включая недоношенных, далеко перевозить не надо и специалисты под боком и т.д., и т.п. У нас это именно так и устроено. |
Линейный вид
