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PubMedDate not supplied

[Infectious Brucella endocarditis in a case of corrected transposition of the great arteries].

We describe a case of a 14 year old male who developed infections endocarditis due to Brucella sp. He had a corrected transposition of great vessels with valvular pulmonary stenosis and mitral insufficiency. Vegetations were demonstrated by echocardiogram on the pulmonary valve as well as on left atrioventricular valve. He had also proliferative nephritis. Prolonged antimicrobial treatment results in complete healing. Because the rarity of this association we inform this observation.

Adolescent↗
PubMed1983

Verapamil for refractory ventricular fibrillation during cardiac operations in patients with cardiac hypertrophy.

Calcium-entry blockers prevent ventricular fibrillation during acute myocardial ischemia in laboratory animals. They may be useful as an adjunct to cold cardioplegia by preserving the myocardium during cardiopulmonary bypass. Their use may limit myocardial infarct size. However, the clinical application of calcium-entry blockers for ventricular dysrhythmias associated with myocardial ischemia has been little explored, as yet. We describe four patients, all of whom had significant cardiac hypertrophy (two had idiopathic hypertrophic subaortic stenosis; one had transposition of great vessels; and one had aortic stenosis) and in whom ventricular fibrillation developed after rewarming or shortly after the termination of bypass. The dysrhythmias were refractory to multiple attempts at defibrillation and conventional pharmacologic interventions. However, in each case, defibrillation was successful after administration of verapamil.

Adolescent↗
PubMed1986

Infective endocarditis following cardiac catheterization in infancy. A case report and review.

A 4.5-month-old infant with transposition of great vessels and large ventricular septal defect developed acute infective endocarditis following cardiac catheterization. Beta-hemolytic streptococcus was recovered from three blood cultures. The infant survived after 6 weeks intravenous antibiotic therapy. The occurrence of infective endocarditis following cardiac catheterization during infancy is briefly reviewed and discussed. The importance of distinguishing febrile episodes of infancy from infective endocarditis and the use of two-dimensional echocardiography for diagnosis is re-emphasized.

Cardiac Catheterization↗
PubMedDate not supplied

Profile of creatine phosphokinase (CK) and its isoenzyme MB (CK-MB) during corrective procedures of congenital cyanotic heart disease.

This study investigates the influence of inadequate oxygen supply on CK and CK-MB release rate in congenital cyanotic heart disease in fourteen patients. Eleven patients had Tetralogy of Fallot and 3 Transposition of great vessels. Their age ranged between 10 days and 10 years (mean 50.48 +/- 31.82 months). The corrective repair was carried out under CPB with systemic hypothermia (20 degrees-25 degrees C) and intermittent St. Thomas Cardioplegia perfusion in the aortic root until the septal temperature was below 16 degrees C. Three blood samples were taken before, during and 10 minutes after CPB to quantitate the CK and CK-MB. In 6 cases of Fallot, two simultaneous biopsies, one from the right and another from the left ventricular walls were taken at the end of the 10 first minutes of reperfusion to evaluate the ATP, CP and glycogen contents. CK and CK-MB levels showed an increasing evolution; the CK-MB per cent increased sharply after aortic clamp release and then fell abruptly to low values at the 10th minute after CPB arrest. Comparative evaluation between the 3 values for C K showed significant differences (P less than 0.001) in all, except when the first values were compared to the second (P greater than 0.05) and for CK-MB an overall significant differences were found at P less than 0.025 and P less than 0.001. On the other hand, quantification of ATP, CP and glycogen contents from simultaneous biopsies from the left and the right ventricular walls did not demonstrate significant differences between the two ventricles after the ischemic period.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiopulmonary Bypass↗
PubMedDate not supplied

Transcatheter implantation of intravascular stents for postoperative residual stenosis of peripheral pulmonary artery stenosis.

This is a prospective study of transcatheter implantation of 11 intravascular stents in 7 patients with status/post (S/P) surgical correction of major cardiovascular lesions. The safety and efficacy of balloon-expandable stents for treatment of peripheral pulmonary artery stenosis (PPAS) is evaluated and analyzed. Although the transcatheter implantation of intravascular stents has been reported as a possible treatment for stenotic peripheral pulmonary arteries, the results of intermediate follow-up studies on patients with S/P surgical correction for residual PPAS need to be evaluated. From June 1998 to December 2001, a total of 15 patients with PPAS having S/P surgery for major cardiovascular lesions were enrolled in this study. Eight of them had redo surgery after complete evaluation and the other 7 patients who might be at higher risk of mortality or morbidity from redo surgery, underwent transcatheter implantation of stents to dilate significant PPAS. Tetralogy of Fallot, S/P total correction, was done in 6 and transposition of great vessels, S/P Jatene operation, was done in 1. There were 10 stents (P 308 Palmaz stent x8 and Intrastent x2) implantation for 10 sites of the stenotic PPAS in these 7 patients, who were aged from 3.6 to 17.3 (10.1 +/- 5.6) years and had body weights ranging from 17 to 72.5 (37.1 +/- 23.0) kg. The narrowest diameter of the stenotic peripheral pulmonary arteries and pressure gradients across the stenosis were measured before and after implantation of stents. A follow-up catheterization and pulmonary angiography was performed 1 year later to evaluate the intermediate efficacy of stents implantation. All the stenotic peripheral pulmonary arteries of these 7 patients had a significant reduction of pressure gradients immediately after the procedure. The narrowest mean diameter of pulmonary arteries increased from 6.7 +/- 3.4 to 11.3 +/- 3.0 mm (p < 0.001), and the mean pressure gradient dropped from 31 +/- 9.9 to 11.4 +/- 4.6 mm Hg (p < 0.001). The follow-up catheterization 1 year later revealed a persistent effect in all but 1 patient. Only a young male presented with a recurrent stenosis with a pressure gradient of > or = 20 mm Hg, which was relieved by redilation with implantation of another stent. There was no immediate or intermediate complication. Transcatheter stent implantation for treatment of a significant residual PPAS after surgical correction of complicated congenital heart disease is a safe and effective procedure. Since children are growing with age, a long-term follow-up study to evaluate the effects and possible problems of stent implantation is mandatory.

Adolescent↗
PubMed1985

[Clinical uses of digital subtraction angiography for imaging anatomy immediately following heart operations in childhood].

Within the last three years, digital subtraction angiography (DSA) was performed 60 times in 58 children with congenital heart disease (coarctation of the aorta, tetralogy of Fallot or transposition of the great vessels) in the immediate post-operative period to delineate residual defects. The DSA was carried out 3.7 +/- 1 days postoperatively with a total of 3.8 +/- 2.5 ml (0.47 +/- 0.2 ml/kg) Conray 70 or Solutrast 300 injected by hand into a catheter which generally had been positioned preoperatively at the transition between superior vena cava and right atrium. The outflow of the contrast medium was recorded on video tape and, additionally, single images of important anatomical details were obtained. In six patients with resected aortic coarctation, the patency of the entire aortic arch was documented with DSA; these patients had undergone surgery at a mean age of 3.3 +/- 4.8 years. Seven patients, with a mean age of 2.5 +/- 4.7 years, had tetralogy of Fallot, three additionally with pulmonary valve atresia; in all, postoperatively, the entire right ventricular outflow tract and the large pulmonary vessels could be demonstrated. In 45 patients with transposition of the great vessels, mean age 4.5 +/- 3.1 months, an atrial inversion with the Senning procedure was carried out; postoperatively, in all patients, the confluence of the superior and inferior vena cavae into the systemic venous portion of the atrium could be delineated. The pulmonary veins were visualized in one-third of the patients. In eight patients, DSA demonstrated obstruction at the opening of the superior vena cava into the atrium together with the collateral circulation via the azygos vein and the inferior vena cava.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1977

Juxtaposition of atrial appendages. Reinterpretation as an accessory appendage or atrial diverticulum.

The pathological features of 13 cases with juxtaposition of the atrial appendages in the autopsy files of The Johns Hopkins Hospital were studied. The anomaly occurs most frequently in association with transposition of the great vessels, ventricular septal defect, patent foramen ovale, and obstructive lesions of the tricuspid and pulmonic valves. The malformation complex of tricuspid atresia, atrial and ventricular septal communication, and the transposition of the great vessels seems particularly common with juxtaposition of the atrial appendage. A recent case showed a small accessory right atrial appendage lying in juxtaposition to the left atrial appendage. In addition, a normally situated right atrial appendages in addition to well-formed accessory appendages lying in juxtaposition to the left atrial appendages. It seems that juxtaposition of the atrial appendages should be reinterpreted as an accessory appendage or atrial diverticulum.

Adolescent↗
PubMed1976

Mitral and tricuspid valve closure in congenital heart disease.

Echocardiography was used to evaluate mitral and tricuspid valve closure in patients 1 day to 20 years of age. When possible, simultaneous phonocardiograms were obtained. The difference in time between the Q wave of the electrocardiogram and mitral closure and between Q and tricuspid closure was designated the delta value. Four groups of patients were assessed: 1) normals (40), secundum atrial septal defect (ASD) (10), mitral valve prolapse syndrome (Barlow's syndrome) (13), pulmonary hypertension (12), and pulmonic stenosis (6); 2) Ebstein's anomaly (10); 3) transposition of the great vessels (15); 4) right bundle branch block (RBBB) (25). Ten patients with surgically induced right bundle branch block were studied by phonocardiography alone. Group I had values of 50 msec or less (-5 to 50 milliseconds) and served as controls. Ebstein's anomaly showed prolongation of the delta value to 65 msec or greater in eight out of ten patients. Patients with transposition of the great vessels showed a striking difference from the preceding groups in that an average negative delta value was obtained. Twenty-two patients of group 4 (RBBB) had delta values within the normal range. This study has shown that a delta value greater than 65 msec is suggestive of Ebstein's anomaly. In addition, if the delta value is negative, transposition of the great vessels can be suspected.

Adolescent↗
PubMed1999

A requirement for neuropilin-1 in embryonic vessel formation.

Neuropilin-1 is a membrane protein that is expressed in developing neurons and functions as a receptor or a component of the receptor complex for the class 3 semaphorins, which are inhibitory axon guidance signals. Targeted inactivation of the neuropilin-1 gene in mice induced disorganization of the pathway and projection of nerve fibers, suggesting that neuropilin-1 mediates semaphorin-elicited signals and regulates nerve fiber guidance in embryogenesis. Neuropilin-1 is also expressed in endothelial cells and shown to bind vascular endothelial growth factor (VEGF), a potent regulator for vasculogenesis and angiogenesis. However, the roles of neuropilin-1 in vascular formation have been unclear. This paper reported that the neuropilin-1 mutant mouse embryos exhibited various types of vascular defects, including impairment in neural vascularization, agenesis and transposition of great vessels, insufficient aorticopulmonary truncus (persistent truncus arteriosus), and disorganized and insufficient development of vascular networks in the yolk sac. The vascular defects induced by neuropilin-1 deficiency in mouse embryos suggest that neuropilin-1 plays roles in embryonic vessel formation, as well as nerve fiber guidance.

Animals↗
PubMed1988

Kabuki make-up (Niikawa-Kuroki) syndrome: a study of 62 patients.

These 62 patients with the Kabuki make-up syndrome (KMS) were collected in a collaborative study among 33 institutions and analyzed clinically, cytogenetically, and epidemiologically to delineate the phenotypic spectrum of KMS and to learn about its cause. Among various manifestations observed, most patients had the following five cardinal manifestations: 1) a peculiar face (100%) characterized by eversion of the lower lateral eyelid; arched eyebrows, with sparse or dispersed lateral one-third; a depressed nasal tip; and prominent ears; 2) skeletal anomalies (92%), including brachydactyly V and a deformed spinal column, with or without sagittal cleft vertebrae; 3) dermatoglyphic abnormalities (93%), including increased digital ulnar loop and hypothenar loop patterns, absence of the digital triradius c and/or d, and presence of fingertip pads; 4) mild to moderate mental retardation (92%); and 5) postnatal growth deficiency (83%). Thus the core of the phenotypic spectrum of KMS is rather narrow and clearly defined. Many other inconsistent anomalies were observed. Important among them were early breast development in infant girls (23%), and congenital heart defects (31%), such as a single ventricle with a common atrium, ventricular septal defect, atrial septal defect, tetralogy of Fallot, coarctation of aorta, patent ductus arteriosus, aneurysm of aorta, transposition of great vessels, and right bundle branch block. Of the 62 KMS patients, 58 were Japanese, an indication that the syndrome is fairly common in Japan. It was estimated that its prevalence in Japanese newborn infants is 1/32,000. All the KMS cases in this study were sporadic, the sex ratio was even, there was no correlation with birth order, the consanguinity rate among the parents was not high, and no incriminated agent was found that was taken by the mothers during early pregnancy. Three of the 62 patients had a Y chromosome abnormality involving a possible common breakpoint (Yp11.2). This could indicate another possibility, i.e., that the KMS gene is on Yp11.2 and that the disease is pseudoautosomal dominant. These findings are compatible with an autosomal dominant disorder in which every patient represents a fresh mutation. The mutation rate was calculated at 15.6 X 10(6).

Abnormalities, Multiple↗
PubMed2006

Familial diseases revealed by a fetal anomaly.

OBJECTIVES: The recognition of a fetal anomaly can lead to the same diagnosis being made in one of the asymptomatic parents unaware of the problem. We analyzed cases in which the discovery of a fetal anomaly led to the discovery of a genetic familial disorder. METHODS: Families in which the recognition of a fetal anomaly led to the same diagnosis being made in one of the asymptomatic parents were included. RESULTS: Twenty couples were included in the study. The fetal anomalies were cleft lip and palate (4), cardiac anomalies (2), cerebral anomalies (1), bilateral club feet with polyhydramnios, akinesia or camptodactily (5), nuchal anomalies (2), micromelia (3), polydactyly (2), and limited elbow extension (1). Genetic counselling helped establish nine maternal diseases as follows: Steinert disease (3), spinal muscular atrophy (1), antecubital pterygium (1), DiGeorge (1), Wardenburg type II (1), Charge (1) and Greig syndromes (1). Eleven paternal diseases were discovered, which were Noonan-like syndrome (1), paternal cervical anomalies (1), Goldenhar syndrome (1), dominant autosomal arthrogryposis (1), osteogenesis imperfecta (3), tuberous sclerosis (1), dominant transposition of great vessels (1), Weyers acrofacial dysostosis (1), and autosomal dominant holoprosencephaly (1). Twelve couples continued with pregnancy and eight opted for termination of pregnancy. CONCLUSION: The fetus is central in giving the first insight into a familial disorder. It can reveal familial diseases undiscovered in the parent and help understand the mode of transmission of an anomaly, mainly the autosomal dominant diseases with variable expressions.

Abnormalities, Multiple↗
PubMed2003

The epidemiology of cardiovascular defects, part I: a study based on data from three large registries of congenital malformations.

To analyze complex and noncomplex cardiac malformations regarding prevalence and in relation to demographic variables, we pooled data on infants (age 1 year or younger) with congenital cardiovascular defects from three large birth defect registries in California, Sweden, and France. Altogether, 12,932 infants had one or more congenital heart defects out of 4.4 million live births and stillbirths. The registries in Sweden and France obtained data through reporting from various sources; in California, medical records were reviewed. As expected, definitions and ascertained conditions differed among each of the registries. The total rates for severe defects were similar (1.43 per 1,000), but differed for specific defects. Clear differences in epidemiological characteristics existed for specific defects; for example, severe cardiac defects sex ratios were significantly high for hypoplastic left heart syndrome, d-transposition of great vessels, double outlet right ventricle, total anoralous pulmonary venous return, tetralogy of Fallot, and significantly low for pulmonary atresia without ventricular septal defect and endocardial cushion defect. Few defects were similar for several epidemiological characteristics, but, for example, the combination of ventricular and atrial septal defects appeared equivalent with endocardial cushion defect under some circumstances, yet behaved differently with regard to associated noncardiovascular defects.

Age Factors↗
PubMed1994

Epidemiology of congenital heart disease in the United States.

Data from the National Center for Health Statistics were examined to aid in assessing the burden of congenital heart disease in the United States. In 1979 through 1988 combined, 46,450 deaths were attributed to CHD, 26,319 occurring in the first year of life. Another 14,283 deaths were attributed to other congenital anomalies of the circulatory system. In 1988 through 1990 combined, there were > 300,000 hospital discharges with any diagnosis of CHD, with 2.7 million days of care provided. For infants aged < 1 year, the rate was 13.7 per 1000 live births per year. Another 242,049 discharges had any diagnosis of other congenital circulatory anomalies. In 1983 through 1985, > 700,000 persons reported a history of CHD. In 1989, heart malformations were reported on the birth certificate of 4621 live births in 45 states and the District of Columbia. In 1988 through 1990 there were an estimated 37,445 repairs of atrial and ventricular septa. In addition, 45,635 cardiac catheterizations were performed in patients aged < 5. Average annual percentage declines in infant mortality rates for the period 1979 through 1988 were consistent with an effect of improvements in surgical technique and intensive care. The average annual infant mortality rate for hypoplastic left heart syndrome increased 1.3%, and rates for five causes amenable to treatment such as transposition of great vessels declined. Infant mortality rates in nonmetropolitan areas were higher than in metropolitan areas, suggesting that limited access to care might be associated with mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗
PubMed1995

Isolated atrial inversion in situs inversus: a rare anatomic arrangement.

Isolated atrial inversion in situs inversus is a rare congenital cardiac malformation. Its physiology resembles transposition of great vessels, and the best option for its surgical treatment is the atrial switch operation. In this article, we present a case of isolated atrial inversion in concordance with visceral situs inversus diagnosed at birth by echocardiography and cardiac catheterization, which was successfully treated at 8 months of age by a Senning procedure.

Coronary Circulation↗
PubMed2006

Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children.

BACKGROUND: Troponin-I (Tn-I) is a well-recognized early postoperative marker for myocardial damage in adults and children. The present prospective study was undertaken to investigate whether a postoperative Tn-I value higher than 35 microg/l is able to predict long-term outcome as it does in early postoperative course, after surgery for congenital heart defects (CHD). MATERIALS AND METHODS: Five hundred and twenty patients (median age 11 months; male 54.7%: 284 patients) undergoing congenital heart defect repair on cardiopulmonary by-pass were prospectively updated in our database including postoperative Tn-I values. Seventy of them (13.4%) (mean age 2.6+/-5.8 months) (70/520) experienced low output syndrome in the early postoperative period. According to the complexity of their malformations, we have arbitrarily divided these patients into two groups: group A included atrial and ventricular septal defects (13 patients), while group B included hypoplastic left heart syndrome, atrio-ventricular canal, transposition of great vessels, tetralogy of Fallot, double outlet right ventricle, truncus arteriosus, total anomalous venous return, and other combined diseases (57 patients). These patients are the object of our study. We reviewed clinical, laboratory, and echocardiographic data performed in the immediate postoperative course (within 24 h) and in the follow-up. RESULTS: In this study, 13 patients died (13/70 patients; 18.5%), 12 in group B and 1 in group A. In deceased patients, mean Tn-I value was 130+/-175 microg/l (CK-MB 570+/-280 microg/l). Conversely, survivors showed a lower mean Tn-I value (25.5+/-28.9 microg/l; CK-MB 76+/-86 microg/l). Overall, Tn-I peak value was higher than 35 microg/l in 19 patients (19/70; 27.2%); among these, 9 died (median Tn-I was 163+/-186 microg/l), whereas in survivors it was 73.4+/-37 microg/l (p=0.37). The remaining four patients who died had a median Tn-I value of 21 microg/l. When Tn-I exceeded 35 microg/l (>100 microg/l in two cases), at echocardiogram a severely depressed cardiac function was evident. Nevertheless, at long-term follow-up (12+/-6 months), the echocardiogram showed an enhanced cardiac performance with an ejection fraction of 70+/-8.5% in all; none of these patients presented with worsened ventricular function. CONCLUSION: Cardiac Tn-I is a specific and sensitive marker of myocardial injury after cardiac surgery and it may predict early in-hospital outcomes. However, by long-term echocardiographic analysis, cardiac Tn-I value looses its prognostic significance and therefore it is not a predictor of long-term ventricular dysfunction.

Adolescent↗
PubMed2004

Postpericardiotomy syndrome after minimally invasive repair of pectus excavatum.

Minimally invasive repair of pectus excavatum (MIRPE) was first reported in 1998 and has gained wide acceptance since then. A 17-year-old girl who had undergone thoracotomy and cardiac surgery for transposition of great vessels at the age of 18 months presented with a deep, long pectus excavatum with asymmetry. After initial uneventful postoperative clinical course after MIRPE, the patient had bilateral pleural and pericardial effusion on the sixth postoperative day. Suspecting postpericardiotomy syndrome, systemic steroids were administered, and the symptoms resolved without affecting wound healing. Manifestation of a pericardial effusion combined with bilateral pleural effusion after MIRPE, especially in patients after cardiac surgery, may indicate a postpericardiotomy syndrome that can be treated successfully by intravenous steroids.

Adolescent↗