Bovine mesenteric venous graft as an alternative conduit in patients with cyanotic heart disease with e-polytetrafluoroethylene graft failure caused by thrombophilic factor positivity after modified Blalock-Taussig shunt.

Modified Blalock-Taussig shunt (MBTS) is a palliative operation for cyanotic congenital heart disease (CCHD) in patients for whom total correction is not appropriate. Many synthetic or biologic grafts have been proposed as alternative shunt materials. The use of a bovine mesenteric venous graft (BMVG) as a systemic-to-pulmonary artery shunt conduit without the administration of antiaggregant and anticoagulant has been proposed as a treatment for neonates with CCHD, but few reports address the importance of thrombophilic risk factors in MBTS and bovine venous graft as a shunt material. We used BMVG as a shunt material without any antiaggregant or antiplatelet regimen in 13 patients with CCHD, all of whom were candidates for MBTS and had thrombophilic risk factors assessed in our initial study. Early shunt failure occurred in the first 3 patients and was attributed to less surgical experience with this graft. No complications were attributable to graft material or surgery itself. In all cases functioning MBTSs were observed on follow-up. Our study results show that thrombophilic factors should be evaluated before the MBTS procedure. BMVG could be the choice of graft for use without the administration of antiaggregant and anticoagulants in patients with thrombophilic risk factors.

A case of superoinferior ventricular heart with situs ambiguus, dextroventricular loop, and levo transposition of the arteries: prenatal and postnatal echocardiographic diagnosis.

A unique case of superoinferior ventricles, left atrial isomerism, concordant atrioventricular connection, and discordant ventriculoarterial connection is described. The associated anomalies were azygous vein continuation, large subpulmonary and apical ventricular septal defects, and left ventricular outflow tract obstruction (pulmonary stenosis). The diagnosis was mostly made by fetal echocardiography and the superoinferior location of the ventricles was confirmed by postnatal echocardiography. Fetal and postnatal echocardiography facilitated the precise morphologic diagnosis and segmental analysis of this defect.

Thrombosis in children with cardiac pathology: frequency of factor V Leiden and prothrombin G20210A mutations.

This study analyzed 28 thrombotic children with various cardiac disorders. They comprise 17% of a total of 168 patients with thrombosis from a single center. Among the 28 patients, 21 had congenital heart disease and 7 were diagnosed with cardiomyopathy. The patients with thrombosis were evaluated for congenital and acquired thrombotic risk factors. In addition to cardiac disorders, two, three, or more risk factors were present in 61% of the children with thrombosis. Two common mutations, namely factor V Leiden and prothrombin G20210A mutations, were found in 6 patients (22%). Nine patients (32%) died of infection, congenital heart disease, cardiomyopathy, thrombosis, operation, or a combination of these; two patients required surgical intervention. Following cardiac angiography, due to necrosis, amputation of the right index finger and right lower extremity was performed on 1 patient. The second patient's index fingers had to be amputated and resection of the bowel was performed following the operation on coarctation of the aorta. This study indicates that congenital heart disease and cardiomyopathy are two common cardiac disorders that may lead to the development of thrombosis. The majority of thrombosis develops within the heart and/or its great vessels. The second predominant site for thromboembolic symptoms is in the brain, including sinovenous thrombosis.

Bidirectional Glenn shunt in an infant with prune-belly syndrome.

The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.

A late complication with the CardioSEAL ASD occluder device and need for surgical revision.

A late complication of the CardioSEAL atrial septal defect (ASD) occluder is reported. Although left atrial umbrella was completely epithelialized and occluded ASD without residual defect, the right atrial umbrella protruded toward the center of right atrium after 18 months. We believe this may be associated with the structural abnormality of the device.

Preliminary pediatric transesophageal echocardiography experiences.

With transesophageal echocardiography (TEE), a new echocardiographic window is obtained which enables cardiologists to explore the heart from the esophagus and stomach. However, the procedure, when first undertaken, may present certain difficulties for the cardiologist in interpreting the anatomical findings and approaching a diagnosis. We thus convey our first experiences and results of TEE in 107 pediatric patients. Transesophageal echocardiography (TEE) was performed in 107 pediatric patients at our institution between December 1998-February 2001, using the standard techniques and following the standard criteria suggested by the American heart Association. The mean age of 54 male (50.5%) and 53 female (49.5%) patients was 7.8 years. Intubation difficulty was experienced in four cases. In one case, while drawing the transducer back from the esophagus, it kinked at the hypopharynx. None of the cases had major hemorrhage or esophageal rupture, and only a few cases had minor pharyngeal injuries or hemorrhages. We used TEE in detecting vegetations in patients with possible endocarditis, and evaluating the prosthetic valves and abnormal pulmonary venous return. We also used TEE to clarify preooperative anatomical details, postoperative complications and residual defects of complex congenital cardiac anomalies. Transcattheter closure of 47 secundum atrial septal defects (ASD)'s and a muscular ventricular septal defect (VSD) (both during patient selection and during the procedure) were accomplished under TEE guidance. As the pediatric cardiologists gain more experience in performing TEE, this technique will have a wider and more effective use in the pediatric population.

Can subclinical valvitis detected by echocardiography be accepted as evidence of carditis in the diagnosis of acute rheumatic fever?

AIM: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. METHODS AND RESULTS: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9+/-2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. CONCLUSION: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.

Transcatheter closure of secundum atrial septal defects, a ventricular septal defect, and a patent arterial duct.

We report our clinical experience with the newly developed Amplatzer device in transcatheter closure of nine atrial septal defects (ASDs), one ventricular septal defect (VSD), and one patent arterial duct (PDA). Eleven patients with ASD (age range 2.5-18 years) selected according to the location and size of the defect by transesophageal echocardiography (TEE), a five-year-old patient with muscular VSD and a one-year-old patient with PDA were considered for transcatheter closure with Amplatzer devices. All procedures were performed under general anesthesia with fluoroscopic and TEE guidance, following a routine hemodynamic evaluation in the catheter laboratory. The optimal device size was selected after the balloon sizing of the ASDs. The sizes of the VSD and PDA were measured on TEE and angiography. The patients were discharged at 24 hours, after an evaluation with x-ray, electrocardiogram (ECG), and echocardiography; they were on 3-5 mg/kg/day aspirin and infective endocarditis prophylaxis for six months after the procedure. They were reassessed at six to eight weeks and Holter monitoring was done in addition. Devices were used for nine ASD patients, and for the VSD and the PDA patients. Mean ASD size was 14.3 +/- 5.3 mm at TEE and 18.3 +/- 4.3 mm at balloon sizing (p=0.02). The mean size of the device was 18.7 +/- 4.2 mm. The procedure time and the fluoroscopy time were 46.1 +/- 12.3 and 12.9 +/- 1.6 minutes, respectively. Immediately after the procedure, four patients (44%) had trivial shunts (TS). TS remained in only two during discharge, and no shunt was observed at second evaluation. The devices were similarly applied to VSD (12-7 mm) and PDA (8-6 mm) patients. Both cases had TS immediately, which disappeared at 24 hours. None of the patients had major complications. Junctional rhythm developed in one patient, and another patient had frequent supraventricular extrasystoles. Amplatzer is an effective and safe device for transcatheter closure of ASD, VSD, or PDA, especially in pediatric patients.

Severe hemolytic anemia after repair of primum septal defect and cleft mitral valve.

Two cases are described in which severe mechanical hemolytic anemia developed after surgical repair of primum atrial septal defect (ASD) and cleft mitral valve. In both cases there was residual mitral regurgitation after repair. Moderate mitral regurgitation and collision of the regurgitant jet with the teflon patch used for repair of the primum ASD were detected by color-Doppler echocardiography imaging. Laboratory tests showed normochromic normocytic anemia, increased indirect serum bilirubin, decreased plasma haptoglobin and hemoglobinuria. The peripheral blood smear contained numerous fragmented red cells. Following another surgical correction of the mitral valve (repair or mitral valve replacement), there was no more hemolysis. The two presented cases show that foreign materials in association with localized intracardiac turbulence may cause severe hemolysis.

Unguarded tricuspid orifice diagnosed by echocardiography: a clinical study.

Complete absence of tricuspid valve tissue and apparatus with a normal orifice between the right atrium and the right ventricle is defined as "unguarded tricuspid orifice". Very few case report of this anomaly have appeared in the literature. In this article, we present five cases of unguarded tricuspid orifice, isolated or in combination with other anomalies. All patients were males, aged six days to five years; only one case is alive at present. In our opinion, this anomaly is not so infrequent as it is believed to be, and the diagnosis can be made easily with echocardiography if it is kept in mind.

Ventricular diastolic filling indices in pulmonary stenosis.

Children with valvar pulmonary stenosis have right ventricular diastolic filling abnormalities that may be due to either right ventricular hypertrophy or right ventricular outflow obstruction. In order to investigate the reason for this abnormality, 23 consecutive cases with pulmonary stenosis (mean age 7.94 +/- 3.33 years) undergoing transluminal pulmonary balloon valvuloplasty without significant tricuspid or pulmonary valvar regurgitation were studied prospectively. Right ventricular diastolic filling indices and pulmonary valvar systolic gradients were measured in these children one day before and after pulmonary balloon valvuloplasty and were re-examined six months later. Right ventricular diastolic indices based on rapid early diastolic filling peak velocity (peak E), peak velocity during atrial contraction (peak A), and ratio of E/A were determined by pulsed Doppler echocardiography. In conclusion, right ventricular diastolic filling indices in patients with pulmonary stenosis did not improve after pulmonary balloon valvuloplasty in the first day but when re-examined by the sixth month there was a significant improvement. These data suggest that diastolic filling abnormalities are more likely a result of right ventricular hypertrophy than of right ventricular outflow obstruction.

Congenital subclavian artery to subclavian vein fistula presenting with congestive heart failure in an infant.

Contrary to acquired arteriovenous fistulas, the congenital malformations are very rare. Here we report a case of congenital arteriovenous fistula forming a communication between the right subclavian artery to subclavian vein. To our knowledge, there is no similar case in which an infant has been successfully operated on for cardiac failure.

Isolated cardiac metastasis of osteosarcoma.

Osteosarcoma metastasis to the heart caused by tumor thrombosis is very rare. A 7-year-old girl with an osteosarcoma of the right humerus, refused the treatment, at first, then, 1 year later, referred to the hospital with metastasis to the heart. The mass invading the pulmonary arteries was successfully removed with open-heart surgery.

Double outlet left ventricle with subpulmonary ventricular septal defect and pulmonary hypertension.

A two-month old male infant with the rare occurrence of double outlet left ventricle, subpulmonary ventricular septal defect and pulmonary hypertension is presented. The infant was managed temporarily with banding of the pulmonary trunk, with a favorable result, and is scheduled for definitive intraventricular repair.

The accuracy of antenatal fetal echocardiography.

The purpose of this study was to evaluate our experience with a group of patients who were either selected by us or referred by an obstetrician or geneticist with the indication of fetal echocardiography. This prospective study was done on 128 cases between 1996-1998. Maternal age range was between 16 and 41 years (mean: 28.79). Gestational age range was between 15 and 37 weeks (mean: 26). In the postnatal period the newborn babies were reevaluated for cardiovascular system abnormalities by physical examination, ECG, telecardiogram and, if it was necessary, by echocardiography, cardiac catheterization and angiography. By comparing prenatal and postnatal findings, sensitivity and specificity of fetal echocardiographic diagnosis were determined. Among the total cases studied, nine had major congenital heart disease in postnatal evaluation. Two cases had false negative; there were no false positive prenatal diagnoses. Sensitivity of echocardiographic diagnosis was 100 percent and specificity 78 percent. Three patients had paroxysmal atrial tachycardia and two atrioventricular block We concluded that the fetal echocardiography is a very useful technique in the evaluation of the fetal cardiovascular system. However, awkward fetal position, severe maternal obesity, and technologic insufficiency of the echo machine may result in unfavorable scanning conditions. Minuteness of anomaly may also result in a false negative prenatal evaluation.

Echocardiographic assessment of left and right ventricular diastolic functions in children with dilated cardiomyopathy.

We evaluated left and right ventricular diastolic functions by pulsed Doppler echocardiography in 16 children with dilated cardiomyopathy and in 20 healthy age-matched control subjects. The cardiomyopathy group demonstrated an abnormal relaxation pattern of the left ventricle. In the cardiomyopathy group compared to normal subjects, peak early filling velocities (43.3 +/- 11 cm/s versus 60.4 +/- 11 cms/s, p < 0.01), the corresponding velocity-time integrals (3.3 +/- 1.4 cm versus 4.6 +/- 1.2 cm, p < 0.01) and the ratio of peak early filling velocity to late filling velocity (1.22 +/- 0.47 versus 1.49 +/- 0.23, p < 0.05) were significantly lower whereas isovolumic relaxation time was significantly longer (58.9 +/- 19.8 ms versus 49.7 +/- 8.9 ms, p < 0.05). In addition, right ventricular diastolic filling was also impaired in children with dilated cardiomyopathy. Peak early filling velocities (41 +/- 7.9 cm/s versus 47.5 +/- 8.8 cm/s, p < 0.05) and the corresponding velocity time integrals (3.0 +/- 1.0 cm versus 3.87 +/- 1.1 cm, p < 0.05) were significantly decreased, while isovolumic relaxation time was significantly increased (60.6 +/- 16.3 ms versus 52.2 +/- 12.8 ms, p < 0.05) in the cardiomyopathy group. The study suggests that abnormalities of both right and left ventricular diastolic function may occur, and should be searched for, in pediatric patients with dilated cardiomyopathy.