Experience with a multidisciplinary antenatal diagnosis and management model in fetal medicine.

BACKGROUND: Fetal medicine is a new and evolving specialty. Complex fetal conditions may require the multidisciplinary input of clinicians from many different specialties. METHODS: Referral of fetal patients was made to a multidisciplinary antenatal diagnosis and management (MADAM) board if more than one specialty (in addition to maternal-fetal medicine) needed to be intimately involved in the evaluation or care of the fetus; consultation would probably lead to alterations in fetal or perinatal management; or development or revision of management guidelines was anticipated. The case log of the MADAM conferences was reviewed retrospectively for number and type of fetal anomalies, and outcome of the presentation to the MADAM board. RESULTS: During a 5-year period, 1% of 25654 pregnant women who were evaluated required consultations with individual pediatric and pediatric surgical specialists. Of these, 114 patients were referred to one of 77 MADAM conferences for consensus recommendation. Of these 77 discussions, 32 (42%) led to an alteration in prenatal management, 14 (18%) led to co-ordination of postnatal management and 12 (16%) led to the establishment of a new treatment guideline, or the modification of an existing one. In all, perinatal management was altered in 75% of cases. CONCLUSION: The MADAM model functions as a forum for exchange of up-to-date scientific information, development of evidence-based treatment protocols and continuity of care through the pre-, peri- and postnatal periods.

Fetal cardiac arrhythmias: diagnosis and management.

The diagnosis and management of fetal cardiac arrhythmias requires complex skills and knowledge, and has had a great impact on the care of infants with congenital heart disease and their families. Optimal benefits will be derived from a thoughtful team approach, with skillful internal communication, and especially when parental involvement is encouraged in the decision making process.

In utero development of hypertensive necrotizing pulmonary arterial lesions: report of a case associated with premature closure of the ductus arteriosus and pulmonary hypoplasia.

Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.

Cardiac causes of syncope in the adolescent.

The optimal approach to the adolescent with syncope relies on a good working knowledge of its varied etiology; specifically, knowledge of the most common and generally benign type, vasovagal syncope, as well as the important distinguishing characteristics of the more serious and life threatening forms of cardiac syncope. A thorough history and physical examination are the cornerstones of investigation and will suggest the diagnosis in the vast majority of cases. Screening electrocardiography complements the history and physical, and is important in ruling out symptomatic arrhythmias impossible to diagnose by history and physical alone. Extensive invasive testing is usually unnecessary. Vasovagal syncope generally responds to reassurance and avoidance of precipitating triggers and positional changes. Beta blocker therapy is usually the first line of pharmacologic therapy. Treatment for other forms of syncope should be directed at the suspected underlying cause.

Genetics of congenital heart disease: strategies.

Congenital malformations of the heart are the most common of all birth defects. Traditionally, a multifactorial model combining genetic predisposition with environmental influence has been cited as the cause of greater than 90% of heart disease. This may be too broad inasmuch as linkage analysis combined with the explosion of information derived from the Human Genome Project has allowed the identification of genetic defects for many types of acquired and congenital diseases of the heart. This review provides a summary of cardiac conditions for which genetic etiologies are apparent, as well as an introduction to some basic clinical genetic concepts. Despite significant advances, it is important to remember that we are still very early in our understanding of the relationship of genotype to phenotype and that the clinical implications of the genetic defects identified are incompletely understood and have only begun to be studied.

Child with idiopathic ventricular tachycardia of prolonged duration.

Ventricular tachycardia is a dangerous dysrhythmia most commonly encountered in adult patients with heart disease. It is uncommon for a previously healthy child to present to the emergency department with hemodynamically stable ventricular tachycardia. The diagnosis and management of this dysrhythmia may pose a significant challenge to the emergency physician. We present the case of a previously healthy child with a structurally normal heart who had ventricular tachycardia for a prolonged period. Common causes of childhood tachycardia and options for treatment of stable and unstable patients are discussed.

Unusual combination of congenital heart defects in an infant with Noonan syndrome.

Congenital heart disease occurs in 35-50% of patients diagnosed with Noonan syndrome. We present an infant with an unusual combination of congenital heart defects not previously reported, including partial atrioventricular septal defect, polyvalvular dysplasia, and progressive hypertrophic cardiomyopathy. We discuss the possible interaction between these lesions that may have led to the patient's rapid demise.

Foramen ovale size in the normal and abnormal human fetal heart: an indicator of transatrial flow physiology.

We used fetal echocardiography to measure the sizes of the foramen ovale and atrial septum in 80 human fetuses from 17.5 to 38 weeks gestation. Forty-six fetuses had normal cardiac anatomy, 19 had left heart obstructive lesions, and 15 had right heart obstructive lesions. Pulsed and color flow Doppler studies were incorporated when available. We found that normal fetuses had a foramen ovale/atrial septum size ratio of 0.33 +/- 0.04 (mean +/- SD). In all (52%) fetuses in which it was available, pulsed Doppler study revealed bidirectional, but predominantly right-to-left, flow through the foramen ovale. Fetuses with left heart obstructive lesions had a foramen ovale/atrial septum size ratio smaller than normal (0.28 +/- 0.05; p < 0.001). Seven of nine fetuses with left heart obstructive lesions who had color Doppler studies demonstrated reversal of the normal flow pattern, exhibiting unidirectional left-to-right transforamenal flow. Those with right heart obstructive lesions had a larger than normal foramen ovale/atrial septum size ratio (0.47 +/- 0.04; p < 0.001). Nine fetuses with right heart obstructive lesions had color Doppler studies which demonstrated almost exclusive right-to-left transforamenal flow. We conclude that foramen ovale/atrial septum size ratio and Doppler interrogation of transatrial flow are helpful adjuncts in determining the presence of congenital heart disease in utero. In addition, early detection of abnormal foramen ovale/atrial septum size ratio may predict whether left or right ventricular development will be impaired throughout gestation, even before gross disparity of ventricular size is apparent.

The impact of an AIDS symposium on attitudes of providers of pediatric health care.

Following reports of concern among health-care workers regarding the occupational risk of infection with the human immunodeficiency virus (HIV), a symposium was designed in 1987 to demonstrate to health-care providers at three hospitals in The Bronx, New York, the low risk of occupational HIV infection and techniques for avoiding infection. After the symposium, 103 of the health-care providers who had attended it completed a questionnaire assessing the impact of the symposium on their attitudes; the responses from 100 of these providers were used in this study. Twenty-nine of the responding providers reported that the symposium had increased their concerns regarding their risk of HIV infection; this group was composed of seven of the 15 medical students who responded, 12 of the 36 housestaff, seven of the 28 faculty, and three of the 21 other medical staff. The findings of the present study suggest that a symposium designed to decrease concerns of occupational HIV infection among health-care workers may have the opposite effect on some of those who attend it, especially medical students. Education alone may be inadequate to reassure some providers. The authors recommend small-group sessions addressing the emotional aspects of health-care providers' concerns.