Three-dimensional ultrasonography in obstetrics and gynecology: preliminary experience.

OBJECTIVE: Technologic advances in ultrasonographic imaging have revolutionized the management of women's health care. We recently began to evaluate the clinical applications of three-dimensional ultrasonography. STUDY DESIGN: This study prospectively evaluated 161 obstetric and gynecologic patients. Both two- and three-dimensional imaging data were acquired from real-time ultrasonography. Three orthogonal planes were displayed on a monitor and were used to create the rendered three-dimensional images. RESULTS: To date, 201 three-dimensional ultrasonographic studies have been performed, 165 transabdominally and 36 transvaginally. Transabdominally, an average of eight acquisitions per patient were obtained. Of the clinically suspected abnormalities, 29 of 32 (91%) were confirmed by three-dimensional imaging. Three of 32 (9%) improved the diagnostic capabilities or changed the diagnosis. Of the 36 transvaginal studies, an average of four acquisitions per patient were done. Thirty (83%) of these patients had suspected abnormalities and all were confirmed. CONCLUSIONS: Three-dimensional ultrasonographic imaging appears to be highly promising in the clinical setting.

Conjugated hyperbilirubinemia in a newborn infant after maternal (transplacental) treatment with flecainide acetate for fetal tachycardia and fetal hydrops.

An infant with intrauterine supraventricular tachycardia and fetal hydrops, successfully treated with administration of flecainide acetate to the mother had conjugated hyperbilirubinemia shortly after birth. An extensive evaluation failed to disclose a known cause. We believe that in utero exposure to flecainide acetate resulted in conjugated hyperbilirubinemia in this infant.

Central venous line thrombosis in premature infants: a case management and literature review.

Increased risk of central venous line thrombosis in tiny premature infants occurs because the size of the catheter relative to the cross-sectional area of the vessel is large, decreased plasma levels of plasminogen and antithrombin III, and relative low flow of the infusate through the catheter, in comparison with larger infants. A potentially fatal complication of central venous catheters is an intracardiac thrombus. The yield of detecting right atrial thrombi by routine echocardiographic monitoring is very low. Persistent positive blood cultures in infants with central venous lines, in spite of appropriate antibiotic therapy, or signs of catheter occlusion, may increase the yield of echocardiographic detection of intracardiac thrombi. Surgical removal of intracardiac thrombi in infants weighing less than 1500 gm carries a high mortality rate because of the need to use cardiopulmonary bypass with total circulatory arrest and profound hypothermia during surgery. It is in these infants that thrombolysis with urokinase should be considered. A successful therapy with urokinase of a complete occlusion of the right pulmonary artery by an embolus originating from the right atrium is described in a premature infant. For thrombolysis, a loading dose of urokinase of 4400 U/kg followed by 4400 to 8800 U/kg/hr for a few days was used. The thrombolytic effect was manifested by decreased thrombus echogenicity followed by its disappearance, by increased fibrinogen split products, and by decreased plasma fibrinogen. Urokinase therapy may cause massive bleeding, dislodge an intracardiac thrombus causing obstruction of cardiac valves or main vessels or causing embolization to the pulmonary or systemic circulation.(ABSTRACT TRUNCATED AT 250 WORDS)

Impact of intraoperative echocardiography on surgical management of congenital heart disease.

Intraoperative echocardiography was performed by epicardial, 2-dimensional, low- and high-pulsed repetition frequency, continuous-wave Doppler and color flow mapping in 50 patients. Forty studies were performed before and 44 studies after cardiopulmonary bypass. Studies before cardiopulmonary bypass agreed with preoperative evaluation. After cardiopulmonary bypass, studies revealed that 11 of 25 patients who underwent repair of ventricular septal defects had residual ventricular septal defects, and 1 of 25 patients who underwent atrial septal repair had 1 residual atrial communication. One patient with a "Swiss cheese" ventricular septum underwent repeat cardiopulmonary bypass to close residual ventricular septal defects. The patient with a residual atrial communication required immediate reoperation because of a right to left shunt after a modified Fontan procedure. Eight of 10 remaining residual ventricular septal defects spontaneously closed 1 to 41 days after operation. Assessment of postcardiopulmonary bypass and postoperative valvular regurgitation in 21 valves revealed good correlation (p less than 0.01). However, 1 patient required reoperation for mitral valve replacement on the sixth postoperative day. The correlation was fair between postcardiopulmonary bypass and postoperative residual stenotic pressure gradients in 12 surgically repaired stenotic lesions. This study shows that little additional information is added to a comprehensive preoperative evaluation by precardiopulmonary bypass intraoperative echocardiography. Postcardiopulmonary bypass intraoperative echocardiography is useful in identifying residual shunts. Assessment of stenotic gradients and valvular regurgitation must be interpreted in light of a changing hemodynamic state.

Fetal echocardiography: assessment of cardiovascular anatomy and function.

Echocardiography is used to assist the prenatal diagnosis group in understanding and identifying normal and abnormal structure, function, and rhythm in the fetal heart, normal variations, and change during development. Detailed segmental analysis of the heart's complex structure can be performed by experienced observers and the information used to aid in management decisions and to better understand the complex relationships of fetal cardiovascular anatomy and function, and fetal outcome. When used prudently, the technology appears to be safe to fetus and mother, but all who undergo examination will want to know of potential benefits and risks.

Definitive repair in patients with pulmonary atresia and intact ventricular septum.

Between 1977 and 1988, 22 patients underwent definitive repair for pulmonary atresia with intact ventricular septum. Fifteen underwent biventricular repair (mean age 24 months). All had mild to moderate right ventricular hypoplasia at the time of definitive repair. Repair consisted of closure of the atrial septal defect with enlargement of the right ventricular cavity and outflow tract with a patch in eight, insertion of a valved homograft in three, and superior vena cava-pulmonary artery connection in four. There was one operative death (7%). Seven patients had severe right ventricular hypoplasia at the time of definitive repair and underwent a Fontan procedure (mean age 46 months). In two a valved connection was made to the right ventricle and in five a right atrial-pulmonary artery nonvalved connection. There were two operative deaths (29%). Three patients had right ventricular-coronary sinusoids: Two of them underwent a Fontan procedure and one a biventricular repair. We conclude that with adequate early palliation a biventricular repair may be successfully performed for patients with mild or moderate right ventricular hypoplasia, and the Fontan procedure may be used for those with severe right ventricular hypoplasia.

Pediatric chest pain: a prospective study.

Previous studies of childhood chest pain have been retrospective or considered only limited age groups or referred patients. In this study, all children who were admitted to the emergency department with chest pain were evaluated prospectively. Patients with ill-defined chest pain had ECGs and echocardiograms performed. A total of 407 children were evaluated. The most common causes of the pain were idiopathic (21%) and musculoskeletal (15%). Cardiac problems were found in 4%. Chest pain was acute (of less than 48 hours' duration) in 43% and chronic (of greater than 6 months' duration) in 7%. Pain caused 30% of children to stay out of school and 31% to awaken from sleep. Chest wall tenderness was the most common abnormality. ECGs were obtained in 47%; results of 31/191 were abnormal but only 4/191 ECG abnormalities were related to the diagnosis. Echocardiograms were obtained in 34%; results of 17/139 were abnormal (12/139 showed mitral valve prolapse). Young children are more likely to have cardiorespiratory problems; children older than 12 years of age are more likely to have psychogenic pain. The description and location of the pain and the patient's sex are not related to the diagnosis. Nonorganic disease is related to a family history of heart disease or chest pain or having chronic pain. Organic disease is related to pain of acute onset, abnormal physical examination results, pain that awakens the child from sleep, and the presence of fever. Laboratory tests are rarely helpful in evaluating children with chest pain. Chest pain in children is usually benign. Psychogenic pain and idiopathic pain are less common than previously believed.

Leprechaunism: in vitro insulin action despite genetic insulin resistance.

We recently identified a female leprechaun infant with marked hyperinsulinemia [as high as 10,975 microU/ml (78,746 pmol/liter)], presumably secondary to insulin resistance. She had two physical findings suggestive of possible insulin action: cystic ovarian enlargement with gonadotropin-independent steroid secretion and persistent, severe myocardial hypertrophy. To examine the pathophysiology of this disorder we measured the in vitro sensitivity to insulin and other growth factors of erythroid progenitors and a T-lymphoblast cell line derived from her peripheral blood. Resistance to insulin was demonstrated by failure of her circulating erythroid progenitor cells to augment proliferation in response to physiologic concentrations of insulin (1-10 ng/ml). An immortalized T lymphoblast cell line was established by transforming the cells with the human retrovirus human T cell leukemia virus II. This cell line showed little or no response to physiologic concentrations of insulin contrary to consistently observed stimulation of colony formation by cell lines similarly derived from normals. The patient's T lymphoblasts, however, showed normal sensitivity to insulin-like growth factor I. In response to supraphysiologic insulin concentrations (25-1000 ng/ml), leprechaun T lymphoblasts showed significant augmentation of colony formation (peak 189% above baseline at 50 ng/ml); normal T lymphoblasts also showed responsiveness at these high insulin concentrations. Preincubation with a monoclonal antibody against the insulin-like growth factor I receptor (alpha IR-3 at 5000 ng/ml) blocked the in vitro effect of physiologic concentrations of insulin-like growth factor and supraphysiologic concentrations of insulin on leprechaun and control T lymphoblast colony formation, but had no clear effect upon the response to physiologic insulin concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment of fetal supraventricular tachyarrhythmias.

Supraventricular tachyarrhythmia has been encountered in 18 fetuses at the Yale-New Haven Medical Center during the past 4 years. Fourteen of these fetuses had supraventricular tachycardia and underwent in utero antiarrhythmic therapy with maternally administered digoxin either alone, or on combination with verapamil, propranolol, or procainamide. Thirteen of the 14 fetuses had successful in utero conversion of cardiac rhythm to normal sinus rhythm. The 14th patient underwent successful therapy after birth. All 14 fetuses survived despite severe fetal hydrops at the time of diagnosis in 13 of 14. The four remaining fetuses had either atrial flutter (3) or fibrillation. Two of the fetuses with atrial flutter died at birth, the 3rd survived after electrical cardioversion at birth. The fetus with atrial fibrillation converted to normal sinus rhythm and survived after maternal administration of digoxin. Using M-mode and pulsed Doppler echocardiography, the nature and electrophysiologic mechanism of the arrhythmia may be deduced. The latter information is reviewed along with the fetomaternal pharmacology of various antiarrhythmic agents to devise a rational antiarrhythmic treatment program.

In utero diagnosis and management of fetal tachypnea. A case report.

Fetal tachypnea, defined as more than 60 fetal breaths per minute, has been reported to occur almost always in diabetic pregnancies. We treated a patient for fetal tachypnea and tachycardia, the initial presentation of which led to the diagnosis of diabetes mellitus.

Neuroblastoma with intraspinal (dumbbell) extension.

Twenty-three patients with neurogenic tumors having extradural extension (dumbbell tumors) were treated over a 30-year period. Three had ganglioneuroma, 6 had ganglioneuroblastoma, and 14 had neuroblastoma. Nineteen patients with malignant tumors had symptoms of spinal-cord compression; three patients with ganglioneuroma and one with a malignant tumor had no neurologic symptoms. Delays in diagnosis were frequent ranging from 6 weeks to 3 years in 10 patients. Sixteen patients had abnormal x-rays of the spine. All patients with malignant tumors had positive myelograms. Three patients with ganglioneuroma and 13 with malignant tumors are alive and free of disease. Seven patients with malignant tumors died: five with stage IV and 1 with stage III tumors from disease, and 1 with stage II tumor during laminectomy. Age and stage were important prognostic factors; location was not. Treatment consisted of excision and radiation or radiation only. Patients receiving lower-dose (less than 2000 rad) radiation did as well as those receiving much larger doses. Morbidity in patients with malignant tumors was high; 4 have spinal deformity (3 severe) and 8 of 13 surviving patients have residual neurologic deficits. Earlier diagnosis, a surgical approach which does not introduce morbidity, and low-dose radiation in younger patients are important factors in reducing morbidity.

Ultrasonic evaluation of the fetal human heart.

The fetal echocardiogram has demonstrated its utility for visualizing the fetal human heart during the second and third trimesters of pregnancy. The potential utility of the technique for establishing accurate anatomic diagnoses of major congenital cardiac malformations has been established. These data have been utilized to counsel prospective parents and formulate management plans for the remainder of pregnancy and the intrapartum and immediate postpartum periods. Diagnoses have been established as early as the 16th to 18th week of gestation, raising in some cases the moral and ethical question of termination of pregnancy.

Major hepatic resections for neoplasia in children.

In a consecutive series from 1968 to 1978, 11 hepatic resections encompassing at least two hepatic segments were carried out for neoplasia in pediatric patients varying in age from 7 days to 14 years. There were no operative deaths. These resections consisted of four right lobectomies, three extended right lobectomies, one right lobectomy with right nephrectomy and inferior vena caval resection, two left lobectomies, and one left extended lobectomy. Diagnoses were hepatoblastoma in six patients, Wilms' tumor invading the liver in two, hemangioendothelioma in two, and malignant mesenchymoma in one. Selective angiography and technetium Tc 99m sulfur colloid scintigraphy were important preoperative aids. Complications were infrequent and there were no major infections or biliary fistulas. There was one death eight months postoperatively due to recurrent hepatoblastoma. Vigorous hepatic regeneration occurred in all instances. Major hepatic resections are well tolerated in children and allow good subsequent development.

Operative management of stress ulcers in children.

The operative management of stress ulcer in children is controversial. Between the years 1969 and 1981, ten children were operated on at the Babies Hospital for stress ulcer. Their illnesses included connective tissue disorders (3), sepsis (2), Reye's syndrome (1), hemolytic uremic syndrome (1), leukemia (1), closed head injury (1), and renal failure (1). In those with bleeding (8), aggressive conventional medical management was attempted prior to operation. Four children also received intravenous cimetidine. Four patients underwent embolization of a feeding artery and/or selective vasopressin infusion. In those patients who perforated (2), operation was performed after a brief period of resuscitation. Ten patients underwent 11 operations. In those who bled, multiple ulcerations were the most common finding. Operative procedures consisted of partial gastrectomy and vagotomy (4), partial gastrectomy alone (2), and vagotomy and pyloroplasty (2). One child who underwent vagotomy and pyloroplasty required partial gastrectomy for recurrent bleeding. Of the two children who perforated, one was managed by plication and the other by partial gastrectomy. There were two deaths (20%), both occurring in patients who had undergone gastrectomy. One survivor has mild dumping. This experience suggests that in children (1) stress ulcers are commonly multiple when associated with major medical illnesses; (2) partial gastrectomy with or without vagotomy affords maximum protection against recurrent bleeding; (3) lesser procedures are effective for solitary bleeding duodenal ulcers or perforation; and (4) selective arterial embolization or vasopressin infusion are unreliable methods for controlling bleeding.