The defective nature of hepatitis delta virus

Forty-nine out of 66 patients with Turner's syndrome and different karyotypes underwent full cardiological evaluations (physical examination, electrocardiography, chest X-ray and echocardiography). Congenital cardiovascular anomalies were found in 11 patients (22.4%). Among the cardiac anomalies in patients with Turner's syndrome, aortic malformations (aortic coarctation [27%], aortic stenosis [18%] and bicuspid aortic valve [18%]) were the most frequent. We observed that the most severe malformations were found in those with karyotype 45,X or 45,X/46,XX. No anomalies were detected in patients with the X isochromosome or those mosaic with the Y component.     

Treatment of local recurrence of rectal cancer

We report on 2 infants with Ullrich-Turner syndrome in whom partial anomalous pulmonary venous drainage was diagnosed noninvasively by color-coded Doppler sonography. Several patients with the combination of anomalous drainage of one or more pulmonary veins and Ullrich-Turner syndrome have been described in the literature. However, in the majority of those previously reported cases the diagnosis of partial anomalous pulmonary venous drainage was established by angiography during cardiac catheterization performed for confirmation of other cardiovascular malformations. Our patients show that partial anomalous pulmonary venous drainage can be diagnosed easily in neonates and young infants, as long as this anomaly is taken into consideration.     

Noonan syndrome: structural abnormalities of the mitral valve causing subaortic obstruction

Among 41 consecutive children with classic Noonan syndrome, 27 patients (66%) presented cardiac anomalies. Eight patients (19.5%) had a congenital anomaly of the mitral valve consisting of 5 with partial atrioventricular canal defect and 3 with anomalous insertion of the mitral valve on the ventricular septum. Five patients (12%) presented with a significant left ventricular outflow tract obstruction caused by the anterior leaflet of the mitral valve: two cases with atrioventricular canal and three cases with isolated anomalous insertion of the mitral valve. Echocardiography is the best tool for the diagnosis. Cardiac defects of patients with Noonan syndrome may be explained on the basis of anomalies of the extracellular matrix involving cardiac valves including the mitral valve. CONCLUSION: In children with Noonan syndrome and left ventricular hypertrophy a careful echocardiographic assessment of the mitral valve should reveal those in whom the left ventricular outflow tract obstruction is anatomical in nature.     

Prevalence of the microdeletion 22q11 in newborn infants with congenital conotruncal cardiac anomalies

Conotruncal malformations account for about 50% of congenital heart defects diagnosed in newborns. We studied prospectively 104 patients admitted in our neonatal intensive care unit for conotruncal defects by fluorescence in situ hybridization to estimate the prevalence of the interstitial deletion in this category of congenital heart disease. Cardiac phenotypes were: truncus arteriosus (17), interrupted aortic arch (18), tetralogy of Fallot with or without pulmonary valve atresia (55), tetralogy of Fallot with absent pulmonary valves (5), ventricular septal defect with malalignment of the conal septum (9). We discovered a microdeletion 22q11 at loci D22S39 or D22S398 in 50 newborns (48%). The prevalence of this microdeletion in different groups of conotruncal defects was: truncus arteriosus 7/17, interrupted aortic arch 16/18, tetralogy of Fallot 19/55, absent pulmonary valves 2/5, and ventricular septal defect 6/9 respectively. Only two patients without any clinical or biological feature of the so called CATCH22 syndrome exhibited the deletion. Parental studies confirmed that the deletion occurred de novo in 47/50 cases (three parental microdeletions). On the other hand, recurrence of conotruncal heart defects in families of "undeleted probands" was higher than expected (13%). CONCLUSION: In 50/104 newborns with conotruncal defects, an interstitial deletion 22q11 was found. Fluorescence in Situ Hybridization should be performed in newborn infants with conotruncal defect and at least one additional manifestation of the CATCH22 phenotype.     

Association between parachute mitral valve and Down's syndrome. Report of a case

The trisomy 21 form of Down's syndrome is the most common human chromosomal aberration. Congenital heart disease is found in as many as 50 per cent of patients with this disorder. The two most common cardiac lesions in Down's syndrome are septal ventricular defect and endocardial cushion defect. Secundum atrial septal defect, tetralogy of Fallot and isolated patent ductus arteriosus are also observed in these Down's patients. Transposition of great arteries and coarctation of the aorta are rarely seen. Most patients having Down's syndrome with congenital heart disease have a single lesion. However, as many as 30 per cent may have multiple cardiac defects. Parachute mitral valve is a rare congenital mitral defect: a single papillary muscle in the left ventricle is the hallmark of this lesion. A parachute mitral valve is frequently associated with other left heart disorders such as supravalvular mitral ring, abnormal and stenosed mitral valve, subaortic stenosis and coarctation of the aorta, thus constituting either a complete form of Shone's complex (when all 4 components are present) or an incomplete form when there are fewer. The aim of the present report is to describe the connection between Down's syndrome and isolated, non-stenosed parachute mitral valve, which has never been reported before.     

Echocardiographic diagnosis alone for the complete repair of major congenital heart defects

OBJECTIVES: The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair. BACKGROUND: Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects. METHODS: To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery. RESULTS: Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality. CONCLUSIONS: This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.     

Cardiac surgery for grown-up congenital heart patients: survey of 307 consecutive operations from 1991 to 1994

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 +/- 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 +/- 17 vs 11 +/- 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.     

Congenital malformations of the mitral valve in children. Pathology and surgical treatment

A systematic study of congenital mitral valve malformations was undertaken on a surgical basis in an attempt to develop techniques of valvular reconstruction adapted to the various lesions. Forty-seven children between the ages of 4 months and 12 years (average 6 years, 4 months) have been operated upon between January, 1970, and March, 1976. Valvular lesions were classified into four group: Group I, mitral insufficiency owing to valvular lesions: Group II mitral insufficiency with subvalvular lesions; Group III, mitral insufficiency and stenosis; Group IV, stenosis. Associated lesions (ventricular or atrial septal defects, coarctation, or aortic valve stenosis) were present in 31 patients (65 per cent) and were corrected during the same operation. Valve reconsruction was possible in 38 patients whereas valve replacement was necessary in 9 patients. In the valve repair group there were three operative deaths (8 per cent), no late deaths, one reoperation for residual ventricular septal defect, and one myocardial infarction. In the valve replacement group of 9 patients, there were three operative deaths, three late deaths, and one case of repeated embolization. Thirty-one of 38 patients in the valve repair group were classified into Functional Class I after the operation (86 per cent), 2 were in Class II, and one in Class III. Minimal or moderate regurgitation and cardiomegaly persisted in the majority of the patients. Pulmonary artery pressure significantly decreased, however, as demonstrated by postoperative catheterization in 17 patients.     

Congenital heart defects in adolescence and adulthood: fatalities and morbidity in primary and reoperation

Adolescents and adults with congenital heart disease have become a new, continuously growing group of patients, because currently improved diagnostics and therapy allow the majority of newborns with congenital heart disease to survive to adulthood. The objective of this retrospective study was to investigate lethality and morbidity after surgery for congenital heart disease in adolescents and adults. Between 1989 and 1994, we operated 137 patients (age between 15 and 75 years; mean 33.8 +/- 15.1) because of congenital heart disease. This was equivalent to 2.7% of all patients, who were operated during this period of time. 101 cases were primary operations, in 36 cases (26.3%) a reoperation was performed. The most frequent diagnoses were ostium-secundum-defect (37.9%), anomalies of the aortic valve including sub- and supravalvular stenoses (9.5%), anomalies of the mitral valve (8%), ventricular septal defect (7.3%) and aortic coarctation (7.3%). Overall lethality was 5.8%, including emergency cases and all late deaths, which have been reported so far. Evaluation of morbidity showed an intraoperative cardiac low-output-syndrome in 3.7%, pulmonary failure in 5.8%, postoperative renal failure in 4.4% and postoperative bleeding complications in 7.3% of cases. Mean duration of postoperative ventilation and intensive care treatment were 2.3 +/- 5.3 and 3.6 +/- 7.3 d, respectively. In comparison to their preoperative status, 71% of patients had improved by one or two NYHA classes. We found that the higher perioperative risk related to reoperation had no impact on the operation's functional result, as evaluation of postoperative functional class showed no difference between primary and re-do cases. The general term congenital heart disease describes a very inhomogeneous group of patients with a broad spectrum of different diagnoses. The variable morphology and pathophysiology of the different congenital heart defects require an individual surgical strategy for each patient, in rare cases even the decision for a heart- or heart-lung-transplantation. Furthermore, adequate follow-up and competent ambulatory treatment of these patients require the cooperation of pediatric and adult cardiologists and cardiac surgeons in interdisciplinary outpatient clinics.     

Molluscicidal activity of Nerium indicum bark

The antiphospholipid antibody syndrome (APS) is defined broadly by the presence of antiphospholipid antibodies, venous and arterial thrombosis, thrombocytopenia and fetal wastage. APS can be primary or secondary, in which APS occurs in the context of another defined disease such as autoimmune disease, malignancy, drug-induced disease, etc. APS is primary in one-half of patients and secondary in the rest, mainly to systemic lupus erythematosus. Several cardiac manifestations of APS have been reported. These include valvular heart disease, coronary artery disease, intracardiac thrombosis and cardiomyopathy. The literature has shown a prevalence of approximately 35% of valvular abnormalities detected by echocardiography in patients with APS. A patient with primary APS who developed aortic stenosis with vegetations on a bioprosthetic porcine valve is presented.     

Deep hypothermia

Early correction of congenital cardiac defects has been facilitated by the use of deep hypothermia and cardiocirculatory arrest. The technique currently used by us consists of surface cooling to 24 degrees C, total hemodilution, cardiopulmonary bypass to 20 degrees C and complete cardiocirculatory arrest. Rewarming is achieved with a pump oxygenator. In over 266 small children, 67% of whom were in the first year of life, lesions corrected included transposition of the great arteries (TGA)(43%), ventricular septal defect (16%), tetralogy of Fallot (16%), and total anomalous pulmonary venous drainage (5%). Early primary correction with deep hypothermia has a risk that is comparable to or better than staged repair.     

Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies

BACKGROUND: Recent reports have cited improving results for surgical management of isolated total anomalous pulmonary venous drainage. Complex cases (with other cardiac anomalies) are less frequently reported and are associated with higher mortality. METHODS: Retrospective review identified 170 consecutive patients treated for total anomalous pulmonary venous drainage from 1982 to 1996: 44 cases were "complex" (with significant associated cardiac lesions) and 126 cases were "simple." RESULTS: Operative mortality for simple cases decreased from 26% to 8%, and mortality for complex cases remained constant at 52%. Age, size, and the presence of atrial isomerism were univariate predictors of mortality. Multivariable analysis identified only univentricular hearts and associated cardiac lesions as predictors of operative mortality. Pulmonary artery (n = 16) and arteriopulmonary (n = 7) shunting strategies for complex cases resulted in less than 30% long-term survival. CONCLUSIONS: Despite improvement in survival for simple cases, management of total anomalous pulmonary venous drainage with single-ventricle hearts or other associated cardiac lesions remains problematic.     

On the generalization of the receiver operating characteristic analysis to the population of readers and cases with the jackknife method: an assessment

Interstitial deletions in chromosome 22 and features associated with CATCH-22 syndrome have been reported in patients with conotruncal congenital heart anomalies. Absent pulmonary valve syndrome is characterized by absent or rudimentary pulmonary valve cusps, absent ductus arteriosus, conoventricular septal defect, and massive dilation of the pulmonary arteries. Because absence of the ductus arteriosus is a key element in the pathogenesis of this syndrome and aortic arch malformations are frequently seen in patients with CATCH-22 syndrome, we hypothesized that patients with absent pulmonary valve syndrome would have a high incidence of deletions in the critical region of chromosome 22. Eight patients with absent pulmonary valve syndrome were studied. Metaphase preparations were examined with fluorescent in situ hybridization of the N25 (D22S75) probe to the critical region of chromosome 22q11.2. Deletions were detected in 6 of 8 patients. The presence of deletions in chromosome 22 in most of the patients we have examined with a diagnosis of absent pulmonary valve syndrome supports a specific genetic and embryologic mechanism involving the interaction of the neural crest and the primitive aortic arches as one cause of congenital absence of the pulmonary valve.     

Heparin coating of extracorporeal circuits inhibits contact activation during cardiac operations

OBJECTIVE: The choice of a valve substitute remains a challenge in young patients, with numerous reports of early degeneration and calcification of biological valves in this age group. Therefore an assessment of the long-term results after mechanical aortic valve replacement in children was initiated. METHODS: A retrospective study was conducted in 54 consecutive patients aged 1.1 to 17 years (mean 12.8 +/- 4 years) operated on between 1975 and 1993. Aetiology was congenital in 34 patients, rheumatic in 13, infectious in 5, and dystrophic in 2. Concomitant surgery included mitral valve replacement (10), aortic annulus enlargement (9), correction of truncus arteriosus (7), Bentall operation (2), coarctation repair (2), tricuspid valvuloplasty (2), correction of double outlet right ventricle (1), and replacement of a right ventricle to pulmonary artery conduit (1). A Bjork-Shiley valve was implanted in 14 patients, and a St Jude Medical valve in 40. All patients were given Warfarin with a monthly INR control. Follow-up was completed through questionnaires mailed to referring physicians and direct clinical examination. RESULTS: Overall early mortality was 13% (7 cases), and 6% (2 cases) in the 32 patients operated on after 1984. Follow-up was complete in 45 survivors (2 lost to follow-up), with a total follow-up of 261 patient-years. There were 6 late deaths, 4 being cardiac and due to persistent LV dysfunction, and 2 valve-related, due respectively to major gastro-intestinal bleeding and massive thromboembolism. Linearized rates of valve thrombosis and anticoagulant-related hemorrhage were both 0.3% per patient-year. Actuarial survival rate was respectively 84.5% at 5 years and 70.2% at 10 years. Reoperation was necessary in 3 patients for recurrent LV outflow tract obstruction. One patient with severe LV dysfunction is awaiting a heart transplant. CONCLUSION: We conclude that the longterm outcome after mechanical aortic valve replacement in children and adolescents is satisfactory and comparable to currently available reports on biological substitutes. The mandatory anticoagulant therapy is well tolerated in this age group.     

Teams and seams: skill mix in primary care

OBJECTIVES: We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality. BACKGROUND: Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality. METHOD: All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed. RESULTS: A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001). CONCLUSIONS: Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.     

Liver changes in protein malnutrition. An experimental study in rats

Congenital rubella syndrome has a wide variety of severe ophthalmic and systemic complications. A worldwide rubella epidemic from 1963 to 1965 affected thousands of infants. This is a 20 year follow up study of patients with congenital rubella syndrome analysing the prevalence of ophthalmic disorders, associated systemic problems, and correlations among these defects. The authors statistically analysed 125 cases of congenital rubella seen in the Mayo clinic ophthalmology department over a 32 year interval. Most patients were young adults. Ocular disease was the most commonly noted disorder (78%), followed by sensorineural hearing deficits (66%), psychomotor retardation (62%), cardiac abnormalities (58%), and mental retardation (42%). Multiorgan disease was typical (88%). Ocular disease and hearing loss were frequently associated (53% had both) but not significantly correlated. A similar association existed between ocular and cardiac disease. Cataracts and microphthalmia were significantly correlated with poor visual acuity (each p < 0.0001). Glaucoma was significantly correlated with cataracts (p = 0.0002) and microphthalmia (p = 0.0024) but not poor visual acuity. Four patients with microphthalmia developed late onset glaucoma. No significant association was found between gestational age at time of maternal infection and the incidence of individual ocular conditions. However, several cardiac disorders were significantly associated with gestational age. Although new cases of congenital rubella are rare, surviving victims continue to challenge the ophthalmic and medical communities with a wide range of ocular and systemic disorders.     

Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage

BACKGROUND: Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure. METHODS: Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described. RESULTS: There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome. CONCLUSIONS: Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.     

Evaluation of HBsAg carrier rate in acute viral hepatitis and high risk individuals using RPHA and ELISA

INTRODUCTION: Balloon valvuloplasty in neonates and small infants with critical aortic stenosis is a palliative procedure. The present report describes the results of the technique in our center. METHODS: From April 1993 to March 1995, six consecutive patients with critical aortic valve stenosis underwent catheter-balloon valvuloplasty. Their ages ranged from 2 to 120 days old (45.5 +/- 47.5 days). Four patients had associated lesions: 2 had coarctation of the aorta, 1 had ischemic dilated cardiomyopathy and 1 had endocardial fibroelastosis. Percutaneous femoral artery access was used in four cases and axillary artery dissection in two. RESULTS: The balloon-annulus diameter ratio was 0.92 +/- 0.12. The peak systolic ejection gradient decreased from 66.1 +/- 26.4 to 38 +/- 15.7 mmHg (p < 0.05) and the left ventricle systolic pressure decreased from 136.3 +/- 26.8 to 115 +/- 22.5 mmHg (p < 0.05). There were no mortalities related to the procedure. Both patients who had aortic coarctation developed aortic regurgitation and died after repairing of the coarctation. The patient with endocardial fibroelastosis died during an attempt to perform the Norwood operation (Stage I) and the other patient with ischemic dilated cardiomyopathy survived after cardiac transplantation. The remaining two patients with isolated aortic valve stenosis are currently asymptomatic. CONCLUSIONS: Catheter-balloon valvuloplasty is an effective procedure in the initial treatment of critical aortic stenosis and may be life saving.     

Cardiac teratogenesis of halogenated hydrocarbon-contaminated drinking water

OBJECTIVES: The purpose of this study was to test the hypothesis that administration of trichloroethylene and dichloroethylene to pregnant rats during organogenesis would produce a significant fetal cardiac teratogenic effect. It was also hypothesized that administration of these compounds only before pregnancy would not be associated with fetal cardiac teratogenesis. BACKGROUND: Epidemiologic observations demonstrated an increased number of congenital cardiac defects in children whose mother resided in an area with drinking water contaminated by trichloroethylene and dichloroethylene. A prior provocative intrauterine exposure study in rats established a positive link between these contaminants and an increased number of fetal hearts with congenital cardiac defects. METHODS: Sprague-Dawley rats were given pure tap drinking water (control subjects) or water contaminated with high or low dose of trichloroethylene or dichloroethylene (experimental groups) during prepregnancy only, prepregnancy and pregnancy or during pregnancy alone. RESULTS: A total of 2,045 fetuses were examined. Trichloroethylene or dichloroethylene delivered exclusively in the period before pregnancy caused no increase in congenital cardiac malformations over the control level. Compared with the control group, rats exposed to these agents both before and during pregnancy, had a significantly greater number of fetuses with cogenital cardiac malformations. Trichloroethylene (high dose only) administered only during pregnancy produced a significant increase in cardiac defects. Other fetal variables, including noncardiac congenital abnormalities, showed no significant difference between control and treated groups. CONCLUSIONS: Trichloroethylene and dichloroethylene administered during organogenesis are cardiac, but not general, teratogens. The data indicate that these agents administered in drinking water to pregnant rats caused an increased number of congenital cardiac defects in rat fetuses.     

Contribution of pulsed Doppler echocardiography to ultrasound diagnosis of congenital heart diseases

The authors document the importance of echocardiograhy (ECHO) with special respect to the pulsed Doppler technique (PDE) in the diagnosis of congenital heart diseases. Typical ECHO and PDE findings in atrial and ventricular septal defects, patent ductus arteriosus, valvular stenosis and insufficiency, absence of pulmonary valves, tricuspid and pulmonary atresia, Fallot's tetralogy, arterial trunk, double-outlet right ventricle, transposition of the great arteries, total anomalous pulmonary venous drainage and hypoplastic left-heart syndrome are described. The authors conclude that a combination of M-mode, cross-sectional and pulsed Doppler echocardiography permits a comparatively detailed non-invasive diagnosis of the entire spectrum of congenital heart diseases.