Coarctation repair without cardiac catheterization in infants

Of 35 infants who underwent an operation for coarctation of the aorta, 15 did not undergo cardiac catheterization before surgery. The diagnoses in all uncatheterized patients were made by clinical examination, chest radiography, ECG, and two-dimensional echocardiography combined with pulsed Doppler echocardiography. Associated anomalies diagnosed by two-dimensional/Doppler echocardiography included a patent ductus arteriosus in all patients, bicuspid aortic valve in six, small ventricular septal defect in four, and complete common atrioventricular canal in one. In no instance was the diagnosis of coarctation in error. In addition, the clinical significance of the ventricular septal defect was judged correctly by two-dimensional echocardiography, and no patient required an early reoperation because of significant left-to-right shunt. Two significant lesions were missed in one patient each: mitral stenosis and aortic stenosis. These diagnoses were missed in patients who were first seen with either profound congestive heart failure or shock. Coarctation of the aorta and associated lesions can be diagnosed accurately by two-dimensional echocardiography. This permits proper patient management without the added risk of cardiac catheterization. Although mitral and aortic lesions may be missed because of low cardiac output, this does not result in management errors.     

Accuracy of subcostal two-dimensional echocardiography in prospective diagnosis of total anomalous pulmonary venous connection

Since reparative surgery without cardiac catheterization has been advocated for certain lesions such as total anomalous pulmonary venous connection (TAPVC), it is important to assess the accuracy of two-dimensional (2-D) echocardiography in the prospective diagnosis of this lesion. Abandoning traditional cardiac catheterization and angiography would be justified only if all surgically relevant anatomic features could be consistently displayed noninvasively. Consequently, we reviewed our experience with TAPVC to determine the suitability of subcostal 2-D echocardiography as the sole prospective diagnostic tool. Over a 5-year period, of 2444 infants under 2 years of age who underwent subcostal 2-D echocardiography, 38 were diagnosed as having TAPVC as the only major cardiac defect. There were no false-negative or false-positive cases during the time of the study. Drainage sites were diagnosed correctly in 36 (95%). Of the five cases of mixed-type TAPVC, the second drainage site was missed by 2-D echocardiography in two cases.     

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.     

Echocardiographic versus cardiac catheterization diagnosis of infants with congenital heart disease requiring cardiac surgery

The success of noninvasive preoperative evaluation of infants with congenital heart disease using cardiac ultrasound depends not only on diagnostic accuracy, but also on risk of morbidity and mortality as compared with infants who undergo cardiac catheterization. Fifty-six infants (age 10 weeks or younger) with coarctation of the aorta (n = 16), coarctation with ventricular septal defect (n = 12), valvar aortic stenosis (n = 10) or total anomalous pulmonary venous connection (n = 18) were examined. Thirty-one underwent noninvasive preoperative assessment and 25 underwent evaluation including cardiac catheterization. Age, level and duration of support, pH, renal function, mortality, complications of cardiac catheterization and errors of diagnosis were compared. Significant differences between the 2 groups were more frequent preoperative use of prostaglandin E1 and shorter hospital stay in the noninvasively evaluated coarctation group. Of the infants with coarctation and ventricular septal defect, 1 who had cardiac catheterization required renal transplantation and 1 evaluated noninvasively required surgery at age 3 months for mitral stenosis not discovered on preoperative evaluation. One noninvasively evaluated infant with total anomalous pulmonary venous connection had a stenotic communication between the pulmonary venous confluence and the left atrium not detected by ultrasound. Surgery was successful in the latter 2 infants. Noninvasive preoperative diagnosis of some infants with congenital heart disease can be performed without increasing the risk of operative morbidity and mortality. Eliminating cardiac catheterization reduces hospital costs, decreases total numbers of catheterizations performed and influences the structure of training programs.     

Cardiac rhabdomyoma in a newborn two-dimensional echocardiographic diagnosis

Primary cardiac tumors are quite rare in the newborn period. Prior to surgery, cardiac catheterization and angiocardiography have been performed to confirm the two-dimensional echocardiographic findings. In this report a 2-day-old baby with the clinical impression of severe cyanotic congenital heart disease diagnosed by two-dimensional echocardiography as multiple rhabdomyoma and confirmed by surgery is presented. It is emphasized that two-dimensional echocardiography is a very useful technique for the diagnosis of primary cardiac tumors. By means of this method severely symptomatic newborns may be taken directly to surgery without prior cardiac catheterization, thus minimizing complications.     

Impact of 2-Dimensional echocardiography on the management of distressed newborns in whom cardiac disease is suspected

The course and management of 40 consecutive newborns (aged less than 2 weeks) who presented with signs and symptoms of congenital heart disease were reviewed to determine the impact of 2-dimensional (2-D) echocardiography on their subsequent management. Of the 40 patients with congenital heart disease, 60% did not undergo cardiac catheterization. Forty-two percent of the patients who were treated surgically went directly to operation without preoperative cardiac catheterization. Only 40% of the patients with congenital heart disease required cardiac catheterization in the newborn period, and 43% of these procedures were primarily therapeutic (that is, balloon atrial septostomy). In each patient 2-D echocardiography correctly identified the major cardiac malformation and there was good agreement with angiographic, surgical, and autopsy findings. The most commonly overlooked defect was a patent ductus arteriosus. Thus, 2-D echocardiography not only allows diagnosis of congenital heart disease in the newborn but can expedite clinical management. No longer is cardiac catheterization necessarily the primary means for an anatomic diagnosis of congenital cardiac malformations in the newborn.     

Cardiac Catheterization in the Early Post-Operative Period After Congenital Cardiac Surgery

ObjectivesThis study sought to demonstrate that early cardiac catheterization, whether used solely as a diagnostic modality or for the use of transcatheter interventional techniques, can be used effectively and with an acceptable risk in the post-operative period.BackgroundCardiac catheterization offers important treatment for patients with congenital heart disease. Early post-operative cardiac catheterization is often necessary to diagnose and treat residual anatomic defects. Experience with interventional catheterization to address post-operative concerns is limited.MethodsThis was a retrospective cohort study. The medical and catheterization data of pediatric patients who underwent a cardiac catheterization ≤30 days after congenital heart surgery between November 2004 and July 2013 were reviewed. Patients who underwent right heart catheterization and endomyocardial biopsy after heart transplantation were excluded.ResultsA total of 219 catheterizations (91 interventional procedures, 128 noninterventional catheterizations) were performed on 193 patients. Sixty-five interventions (71.43%) were dilations, either balloon angioplasty or stent implantation. There was no difference in survival to hospital discharge between those who underwent an interventional versus noninterventional catheterization (p = 0.93). One-year post-operative survival was comparable between those who underwent an intervention (66%) versus diagnostic (71%) catheterization (p = 0.58). There was no difference in the incidence of major or minor complications between the interventional and diagnostic catheterization cohorts (p = 0.21).ConclusionsCardiac catheterization, including transcatheter interventions, can be performed safely in the immediate post-operative period after congenital heart surgery.     

新生儿先天性心脏病的超声心动图诊断—附1294例分析

目的和方法：采用彩色多普勒超声心动图检查新生儿先心病1294例,对检出的各类畸形进行分类及疾病顺们的排列,同时对部分有手术或尸解的病例进行结果对照。结果：非青紫型先心病中,室间隔缺损居本组首位,动脉导管未闭及房间隔缺损分别居第二,三位,青紫型先心病中大动脉转位居首位,法洛四联征及肺动脉闭锁分别居第二,三位。57例有手术或病理结果对照超声诊断单一畸形符合率为100％,复杂畸形诊断符合率为91．2％,结论：彩色多普勒超声心动图诊断新生儿先天性心脏病快速,安全,准确性高 ,是新生儿期先天性心脏病早期确诊及分型的有效方法。     

Heart surgery without previous heart catheterization, in pediatric cardiology]

STUDY OBJECTIVE: To determine the influence of echocardiography in the preoperative diagnosis of heart diseases in children. DESIGN: Retrospective study. SETTING: Hospitalized children admitted in Departments of Pediatric Cardiology and Cardiothoracic Surgery. PATIENTS: Children of both sexes, aged from neonate to 14 years old, with heart disease who underwent cardiac surgery. MATERIAL AND METHODS: From January 1989 to July 1990, 220 consecutive cardiac surgeries were performed in children with heart disease. The initial diagnosis was based on data from clinical examination, electrocardiogram, thorax X-Ray and echocardiogram (M-mode, 2D, conventional and colour Doppler). Patients were separated in three groups according to their ages: newborn infants less than 28 days old; infants less than 12 months old; children more than 1 year old and less than 14 years old. Patients were separated according to the investigations used further for preoperative diagnosis: whether they had or not cardiac catheterization performed prior to surgery. RESULTS: From 220 surgical interventions performed, 124 were "open heart" surgeries (9 neonates, 28 infants and 87 children) and the remaining had "closed heart" operations (14 neonates, 37 infants and 45 children). Preoperative diagnosis was mainly dependent on echocardiography, dispensing catheterization in 90 cardiac interventions. The diagnosis was confirmed at surgery or at autopsy. There were 9 deaths, 3 of which occurred in patients not submitted to cardiac catheterization. CONCLUSIONS: Echocardiography is a reliable method for investigation and establishment of the preoperative diagnosis of heart diseases in children. Its use may further reduce the need for diagnostic catheterization in children, particularly in risk groups.     

The use of echocardiography in pediatric interventional cardiac catheterization procedures

The last decade saw a dramatic change in the management of congenital heart defects. The introduction and development in echocardiography and Doppler modalities enable definitive diagnosis of congenital lesions without cardiac catheterization. At the same time, new therapeutic procedures for congenital defects using the catheter as a channel for different procedures were developed. These therapeutic procedures have replaced cardiac surgery in many types of defects. The new developments in echocardiography and Doppler modalities provide accurate imaging and visualization of the transcatheter devices and brought these two imaging modalities into a close marriage. The use of echocardiography and Doppler in the catheterization laboratory became a must, especially when used in atrial (ASD) and ventricular septal defect (VSD) closure. In this article we will detail the role of echocardiography in the different transcatheter interventional procedures in the catheterization laboratory. Those include atrial septostomy, blade atrial septectomy, balloon dilation, ASD closure, VSD closure, patent ductus anterior closure, and pericardiocentesis.     

Comparison of Doppler echocardiography and cardiac catheterization in patients requiring valve surgery: search for a 'gold standard'.

OBJECTIVE: To compare the sensitivities of Doppler echocardiography and cardiac catheterization in the diagnosis of severe valvular heart disease in patients requiring valve surgery. DESIGN: Retrospective analysis of Doppler echocardiograms and cardiac catheterizations. SETTING: Tertiary referral cardiovascular centre in a university setting. PATIENTS: Sixty-nine patients undergoing valve surgery between July 1988 and July 1990. RESULTS: The sensitivities of echocardiography and cardiac catheterization were 84 and 87%, respectively (P = 1.0) in 32 patients who underwent aortic valve surgery primarily for severe aortic stenosis; 83 and 67%, respectively (P = 1.0) in six patients with severe aortic regurgitation, and 100 and 85%, respectively (P = 1.0) in seven patients with combined severe aortic stenosis and regurgitation. The sensitivities of echocardiography and cardiac catheterization in 11 patients who underwent mitral valve surgery for severe mitral stenosis were 73 and 91%, respectively (P = 0.6) and 69 and 92%, respectively (P = 0.3) in 13 patients with severe mitral regurgitation. Sensitivities of echocardiography and cardiac catheterization in the diagnosis of severe tricuspid regurgitation in five patients who had tricuspid valve repair were 100 and 80%, respectively (P = 1.0). Two patients with severe aortic stenosis by echocardiography, but not by catheterization, did not undergo aortic valve replacement during valvular surgery; both required aortic valve replacement within two years of initial surgery because of heart failure. Four patients with severe tricuspid regurgitation identified by echocardiography did not have tricuspid repair; three had pulmonary hypertension and these patients had resolution of tricuspid regurgitation on follow-up. One patient with severe tricuspid regurgitation and absence of pulmonary hypertension required reoperation for tricuspid valve repair 10 months after initial operation. CONCLUSIONS: The sensitivity of echocardiography and cardiac catheterization in the detection of severe valvular lesions requiring surgery is similar. Discordant results should be reviewed carefully with knowledge of the inherent pitfalls of both techniques in order to ensure optimal patient outcome.     

Clinical role, accuracy, and technical aspects of cardiovascular magnetic resonance imaging in infants

Cardiovascular magnetic resonance imaging (CMRI) has been shown to be an important alternative to echocardiography and catheterization for diagnostic imaging in older patients with congenital heart disease. However, little information is available on its clinical role, accuracy, and technical aspects in infants. We retrospectively identified all patients <1 year of age who underwent a CMRI examination at our institution over a 4-year period (from 1999 to 2002) and reviewed their medical records. Ninety-nine CMRI examinations were performed in 91 patients (median weight 4.9 kg; range 1.2 to 16.3) at a median age of 102 days (range 1 to 358). Technical modifications were used to improve signal-to-noise ratio and temporal resolution, and specialized personnel and equipment were employed. All CMRI studies were performed under general anesthesia without any complications, and all outpatients (n = 44) were discharged the same day. The primary referral questions for CMRI were delineation of the thoracic vasculature (n = 54), assessment of possible airway compression (n = 25), evaluation of cardiac tumor (n = 6), and other (n = 14). For the thoracic vasculature group, findings at surgery (n = 37) and catheterization (n = 16) were concordant with the CMRI diagnoses. In the airway compression group, findings at surgery (n = 16) were also in agreement with CMRI findings. Tumor types by CMRI in the 3 patients with preoperative studies were confirmed by histologic results. Thus, CMRI has a limited but important diagnostic role in infants with cardiovascular disease. It is an accurate and safe method to delineate the thoracic vasculature, evaluate possible airway compression, and characterize cardiac tumors. CMRI may obviate cardiac catheterization and bronchoscopy in selected cases.     

Surgical correction of atrioventricular canal without heart catheterization

In our department, 35 patients with atrioventricular canal underwent elective surgery between June 1983 and May 1989. The diagnosis was based on clinical and echocardiographic assessment without cardiac catheterization. Nineteen patients (age 2-12 years; mean 6.6 years) had a partial atrioventricular canal and 16 (age 5-8 months; mean 7 months) presented a complete atrioventricular canal. The echocardiographic diagnosis was confirmed at surgery in all but one patient (2.8%). The latter was diagnosed as having a complete canal but at surgery a transitional canal with a restrictive ventricular septal defect was found. We based our patient selection on the natural history which excluded cases with early heart failure and pulmonary hypertension. We also relied on an accurate morphological study using two-dimensional echocardiography which excluded cases with associated cardiac malformations. Our recent experience (1986-1989) showed that 50% of the patients with complete canal and 60% of patients with partial canal underwent surgery without cardiac catheterization.     

Underlying cardiac lesions in adults with infective endocarditis. The changing spectrum

The spectrum of recognized cardiac lesions underlying infective endocarditis has been changing as a result of the decline in incidence of rheumatic heart disease, the recognition of the entity of mitral valve prolapse, and the improvement in cardiac diagnostic techniques. Sixty-three cases of native valve endocarditis diagnosed in Memphis hospitals between 1980 and 1984 were reviewed. All diagnoses of underlying cardiac lesions were confirmed by two-dimensional echocardiography, cardiac catheterization, and/or histopathologic examination of valve tissues. Major categories of underlying lesions were as follows: mitral valve prolapse, 29 percent; no underlying disease, 27 percent; degenerative lesions of the aortic or mitral valve, 21 percent; congenital heart disease, 13 percent; rheumatic heart disease, 6 percent. Thus, mitral valve prolapse and, in the elderly, degenerative lesions have displaced rheumatic and congenital heart diseases as the major conditions underlying endocarditis. Redundancy of the mitral valve leaflets was noted in 17 of 18 patients in whom endocarditis was superimposed upon mitral valve prolapse. The risk of infective endocarditis appears to be substantially increased in the subset of patients with mitral valve prolapse who exhibit valvular redundancy.     

Trends in congenital heart disease in Dallas County births. 1971-1984

To examine the changes in birth cohort prevalence rates and severity of congenital heart disease, we studied children with congenital heart disease born to blacks, whites, and Mexican-Americans in Dallas County from 1971 through 1984. Diagnoses were made by pediatric cardiologists' clinical evaluations, echocardiography, catheterization, surgery, or autopsy. During this study period, 2,509 of 379,561 liveborn infants were diagnosed, a prevalence rate of 6.6/1000. The rates for whites was significantly higher than for blacks or Mexican-Americans--7.2/1,000, 5.6/1,000, and 5.9/1,000, respectively. The rate for severe cases requiring cardiac catheterization or surgery or undergoing autopsy was 3.1/1,000 and did not differ among the three groups. The time trend for rates of congenital heart disease suggested an apparent increase in prevalence rate during the 1970s; however, the prevalence rate of severe forms remained relatively stable. This indicates that the apparent rise in prevalence could be accounted for by an increase in detection of mild cases. These findings were interpreted as reflecting a greater tendency for pediatricians to refer asymptomatic children with significant heart murmurs to a pediatric cardiologist.     

Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?

Between January 1976 and July 1983, 217 patients with atrial septal defect underwent surgical repair at Children's Hospital. Thirty with a primum atrial septal defect and 26 who underwent cardiac catheterization elsewhere before being seen were excluded from analysis. Of the 161 remaining patients, 52 (31%) underwent preoperative cardiac catheterization, 38 because the physical examination was considered atypical for a secundum atrial septal defect and 14 because of a preexisting routine indication. One hundred nine (69%) underwent surgery without catheterization, with the attending cardiologist relying on clinical examination alone in 5, additional technetium radionuclide angiocardiography in 5, M-mode echocardiography in 13 and two-dimensional echocardiography in 43; both M-mode echocardiography and radionuclide angiography were performed in 24 and two-dimensional echocardiography and radionuclide angiography in 19. Since 1976, there has been a trend toward a reduction in the use of catheterization and use of one rather than two noninvasive or semiinvasive techniques for the detection of atrial defects. Of the 52 patients who underwent catheterization, the correct anatomic diagnosis was made before catheterization in 47 (90%). Two patients with a sinus venosus defect and one each with a sinus venosus defect plus partial anomalous pulmonary venous connection, partial anomalous pulmonary venous connection without an atrial septal defect and a sinoseptal defect were missed. Of 109 patients without catheterization, a correct morphologic diagnosis was made before surgery in 92 (84%). Nine patients with a sinus venosus defect, three with sinus venous defect and partial anomolous pulmonary venous connection, four with partial anomalous pulmonary venous return without an atrial septal defect and one with a secundum defect were incorrectly diagnosed.(ABSTRACT TRUNCATED AT 250 WORDS)     

The diagnostic accuracy of combined clinical and noninvasive cardiac evaluation: Comparison with findings at cardiac catheterization

The accuracy of combined clinical and noninvasive cardiac diagnostic evaluation was prospectively examined in 108 consecutive patients referred to the heart station for echocardiographic examination prior to cardiac catheterization. History, physical examination, scalar electrocardiography, chest roentgenography, phonocardiography and pulse recording, and M-mode echocardiography were employed by the heart station cardiologist, who assigned one or more diagnoses to each patient. In addition, one of three management strategies was proposed for each patient: 1) surgery without cardiac catheterization; 2) medical therapy without cardiac catheterization; or 3) cardiac catheterization for clarification of the diagnosis. The results of the combined clinical and noninvasive evaluation were independently reviewed for each patient and compared with the diagnosis determined by cardiac catheterization, results of cardiac surgery, and total hospital course. Diagnostic predictions employing combined clinical and noninvasive cardiac evaluation were completely correct in 86% of patients, and management strategy was correct in 97% of individuals. In approximately one-half of all patients full cardiac catheterization or coronary arteriography was recommended. All management strategy errors and two-thirds of diagnostic errors occurred in patients with mitral regurgitation, aortic regurgitation, or coronary artery disease. Combined clinical and noninvasive evaluation results in accurate diagnostic information adequate for the formulation of appropriate management strategies in the majority of patients, but many individuals with cardiac disease still require invasive evaluation for complete diagnosis.     

Trends and Outcomes After Prenatal Diagnosis of Congenital Cardiac Malformations by Fetal Echocardiography in a Well Defined Birth Population, Atlanta, Georgia, 1990–1994

Objectives. In this study we used a population-based approach to assess the impact of fetal echocardiography on a well defined birth population with nearly complete ascertainment of cardiac defects.

Background. Although fetal echocardiography is being used more frequently in the prenatal diagnosis of congenital cardiac malformations, its impact on the diagnosis and surveillance of cardiac defects has not been described in defined populations.

Methods. All stillborn and live-born infants with diagnosed cardiac defects and whose mothers resided in the metropolitan Atlanta area from January 1990 through December 1994 were ascertained through an established birth defects surveillance system. All fetuses with cardiac defects diagnosed prenatally by a pediatric cardiologist were identified from clinical records. The spectrum of cardiac defects, diagnostic trends and adverse fetal outcomes were described.

Results. We identified 1,589 infants with congenital cardiac malformations, for a live-birth prevalence rate of 8.1/1,000 (95% confidence interval [CI] 7.8 to 8.6). Overall, 97 (6.1%) of these cases of cardiac malformations were diagnosed prenatally. The proportion of cardiac defects diagnosed prenatally rose from 2.6% in 1990 to 12.7% in 1994, a nearly fivefold increase. The proportion of cardiac defects diagnosed prenatally during the study varied by the type of defect, from a low of 4.7% for atrial septal defects to a high of 28% for hypoplastic left heart syndrome. Prenatally diagnosed cardiac malformations were associated with a high incidence of infant mortality (30.9%, 95% CI 2.4 to 5.4) and fetal wastage (17.5%, 95% CI 6.2 to 11.3).

Conclusions. These data show that fetal echocardiography is being used increasingly in the prenatal diagnosis of congenital cardiac malformations in metropolitan Atlanta. Few pregnancy terminations were reported as a result of such diagnoses. However, the study had limited power (10%) to detect a meaningful decrease in birth prevalence rates for congenital heart disease. In addition, survival of infants was not improved after prenatal diagnosis with fetal echocardiography.

(J Am Coll Cardiol 1996;28:1805–9)>     

Heart disease presenting in the first week of life: implications of advances in diagnosis and management since 1973

The spectra of congenital heart diseases presenting in the first week of life - diagnosis, treatment, and prognosis - were analyzed in a recent series and compared with a series 9 years earlier. Eighty percent of all infants aged 0 to 7 days referred to the pediatric cardiology service in these series had actual cardiovascular defects; 42% had critical cardiovascular disease. Use of echocardiography in the neonate with suspected congenital heart disease increased from 0 to 82% in the 9-year period, whereas, frequency of cardiac catheterization decreased. Treatment with prostaglandin E(1) and catecholamine-derived pressor agents increased. Surgical mortality in critical neonatal heart disease fell from 58 to 18%, whereas, early cardiac deaths fell from 49 to 24%. These findings illustrate a favorable trend in the care of the neonate with congenital heart disease based on less hazardous diagnostic methods, more effective medical stabilization, and advances in surgical technique.     

Seasonality in live births with congenital heart disease in Malta.

This study was carried out to analyse seasonal variations in live births with congenital heart disease in the overall population of Malta. Included were all patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post mortem, by 1 year of age, and who were born between in Malta between 1990 and 1994. Results were analysed by X2 and by Edwards' cyclic method. There were 231 cases of live born congenital heart disease, which were divided into 114 cases not requiring intervention and 117 cases requiring intervention. Although the overall prevalence of congenital heart disease did not demonstrate any seasonal variation, the lesions requiring intervention showed a significant peak in September by Edwards' method (p = 0.03), which was enhanced by seasonal analysis (p = 0.003). These results were confirmed by conventional X2 and X2 for trend. The lesions not requiring intervention, which were comprised almost entirely (96%) of mild pulmonary stenosis and small ventricular septal defects, failed to show any seasonal trend. The literature regarding such seasonal trends in these malformations and statistical analysis of seasonality of congenital heart disease are reviewed. An environmental factor such as a maternal viral infection or treatment of such infections during the first trimester of pregnancy from November to January may precipitate congenital heart disease in predisposed Maltese foetuses.