Use of three-dimensional catheter guidance and trans-esophageal echocardiography to eliminate fluoroscopy in catheter ablation of left-sided accessory pathways.

BACKGROUND: Newer technologies such as three-dimensional mapping and echocardiography can decrease x-ray exposure during catheter ablation. Many right-sided tachycardias can now be ablated without fluoroscopy. Left-sided tachycardias, however, have not yet been ablated using a zero fluoroscopy approach. OBJECTIVE: This study sought to examine the utility of trans-esophageal echocardiography (TEE) in providing adequate imaging as an alternative to fluoroscopy for transseptal puncture. When combined with NavX guidance (St. Jude Medical, St. Paul, MN, USA), fluoroscopy may not be necessary. METHODS: Ten pediatric patients with supraventricular tachycardia (SVT) had accessory pathways mapped to the left side. Right atrial and coronary sinus geometries were created using NavX. Once a left-sided pathway was confirmed, a transseptal puncture was performed. A guide wire was placed in the SVC and confirmed by TEE. A transseptal sheath and dilator were advanced over the wire and positioned with TEE guidance so that the tip of the dilator was tenting the fossa ovalis. A transseptal needle was advanced across the fossa. Left atrial location of the needle tip was confirmed on TEE by saline contrast injection. The sheath and dilator were advanced over the needle with continuous pressure monitoring and TEE. Once the sheath was appropriately positioned, the ablation was completed using NavX guidance. RESULTS: All patients had acutely successful ablations and none required the use of fluoroscopy. Number of cryo lesions ranged from five to 19, with a mean of 9. Mean procedure time was 4.4 hours, with a range of 3.2 hours to 7.2 hours. There were no complications. One patient had recurrence. CONCLUSIONS: Three-dimensional mapping combined with TEE shows potential for eliminating fluoroscopy use during catheter ablation.

Mutational status of overexpressed p16 in head and neck cancer: evidence for germline mutation of p16/p14ARF.

Inactivation of the p16 tumor suppressor gene is a common phenomenon in squamous cell carcinoma of the head and neck (SCCHN). Less commonly described is the observation of p16 overexpression in SCCHN. Since overexpression of p16 is a potent predictor of outcome in other cancers, we were interested in determining the level of expression of p16 in our SCCHN specimens as a prerequisite to later prognostic studies. We were also interested in determining the mutational status of p16 in these tumors, in order to determine whether the combination of overexpression and gene alteration may predict a different clinical outcome from overexpression alone. A total of 84 specimens of SCCHN were selected for study. These specimens were obtained from all major sites within the oral cavity, oropharynx, pharynx and larynx. The level of expression of p16 in SCCHN specimens was measured by semi-quantitative RT-PCR. In 35 cases, RNA was also isolated from matched normal tissue obtained from a negative tumor margin. In the other 49 cases, the expression level was compared with the level of expression measured in pooled normal RNA obtained from 10 specimens of normal epithelial tissue. Overexpression of p16 was documented when the level of expression in the tumor specimen was 2-fold or greater above the level of expression found in normal tissue. A total of 46 specimens demonstrated overexpression of p16 (55%). All specimens demonstrating overexpression were then subject to sequence analysis. Thirty specimens (65%) showed p16-specific gene alterations, ranging from intragenic deletions to single point mutations, and 15 of these cases concomitantly affect p14ARF. A single specimen demonstrated a silent point mutation within the p16 reading frame. This mutation produces a stop codon at residue 85 in the context of the p14ARF reading frame, predicting premature termination of p14ARF within a previously determined nucleolar localization signal. This observation suggests that in some cases at least, p14ARF may be a selective target for alteration, independently of p16. Analysis of a normal tissue specimen obtained from a negative tumor margin, and a blood sample obtained approximately five years after surgery indicate that this p14ARF-specific alteration may represent a germline mutation.

Pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection--prenatal echocardiographic diagnosis.

The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.

In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia.

We describe a rare case of right atrial isomerism, levocardia, right-side stomach, obstructed supracardiac total anomalous pulmonary venous connection, double outlet right ventricle with complete atrioventricular septal defect and absent spleen. From the pulmonary venous confluence behind the atrium an ascending as well as a descending vertical vein communicated with the systemic venous system in the supracardiac as well as the infracardiac position. The pulsed and color Doppler examination of the individual pulmonary veins as well as of the vertical vein helped in making the diagnosis of obstructed total anomalous pulmonary venous connection. The diagnosis was made by fetal echocardiographic examination at 22 weeks of gestation and confirmed on postnatal echocardiography, cardiac catheterization, and at surgery.

Congenitally corrected transposition with pulmonary atresia and intact ventricular septum.

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.

Biotransformation of banana waste into protein by pleurotus sajor-caju.

Pseudostems and leaves from banana waste were used for biotransformation into protein by using P. sajor-caju, an oyster mushroom. Treatment of formalin (500 ppm) + carbendazim (12.5 ppm) of these substrates was found to favour relatively high percentage biological efficiency (BE) of P. sajor-caju.. Steam sterilization also exhibited comparable yield performance by P. sajor-caju. Fruiting bodies harvested from all the treatments had relatively higher protein contents. The spent substrate (steam sterilized) was found to be suitable as an ideal animal feed because of its rich nutritive composition.

Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

Double-outlet right ventricle with intact ventricular septum in a foetus with trisomy-18.

A rare case of double-outlet right ventricle with intact ventricular septum diagnosed by foetal echocardiography at 21 weeks of gestation is described. Amniocentesis revealed trisomy-18. The cardiac diagnosis was confirmed at autopsy.

Costs and cost-effectiveness of routine transesophageal echocardiography in congenital heart surgery.

BACKGROUND: The safety and efficacy of transesophageal echocardiography (TEE) during congenital heart surgery is well established. The economic costs and benefits associated with its routine use in this setting are, however, uncertain. We sought to analyze the impact that routine intraoperative TEE had on echocardiographic costs in the setting of congenital heart surgery. METHODS AND RESULTS: A retrospective, case-controlled analysis of echocardiographic costs during the operative and postoperative periods was performed for 63 children undergoing elective, complex intracardiac repair. Similar analysis was performed for a smaller group of patients undergoing simple repairs. To ascertain whether any additional cost savings was realized through the use of TEE, we documented the impact that operative TEE had on altering surgical strategy as well as whether TEE use was associated with any intraoperative complications. Despite the additional expense, routine TEE, in the setting of complex repair, resulted in no significant increases in echocardiographic costs, suggesting the superior information provided may in fact reduce the need for additional postoperative studies in the intensive care setting. Although no child required a return to cardiopulmonary bypass, surgical therapy was altered by TEE findings in 2 (3%) of 63 patients. Complications were rare and self-limited, usually occurring with positioning of the probe in smaller patients. CONCLUSIONS: The findings of improved surgical outcomes in a percentage of patients, coupled with the lack of any significant increment in echocardiographic costs, confirm that intraoperative TEE is a beneficial and cost-effective intervention in children requiring complex cardiac repair.

Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery.

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.

Primary pediatric cardiac tumors: a 17 year experience.

We reviewed 22 cases of primary pediatric cardiac tumors followed at our institution from January 1981 through November 1997, analyzing them by subtype, age and manner of presentation, location, associated findings, interventions, and clinical course. Rhabdomyomas were the most common (11), followed by intrapericardial teratomas (2), myxomas (1), fibromas (1), hemangiomas (1), mesotheliomas (1), and rhabdomyosarcomas (1), with 4 undetermined tumors. The majority (77%) of tumors were diagnosed before the age of one year, including six prenatally. The most common presentations were murmurs (5), arrhythmias (5), and abnormal screening fetal ultrasound examinations (4). Tumors were located most frequently in the right ventricle (13) and left ventricle (7), with multiple tumors being present in 10 cases. Eight patients (36%) had associated arrhythmias or conduction abnormalities, and of the 11 patients with rhabdomyomas, tuberous sclerosis was diagnosed in 8. Eight patients underwent cardiac catheterization, including two for electrophysiologic study with radiofrequency ablation, and seven patients had complete or partial tumor resection. The follow-up period ranged from 2 months to 15 years, and there were 3 tumor-related deaths. Therefore, despite the benign histology of most primary pediatric cardiac tumors, there may be significant associated morbidity and occasional mortality. As echocardiographic techniques such as fetal ultrasonography have continued to improve, however, these cardiac tumors have increasingly been detected early before significant symptoms develop.

Preclinical cardiac dysfunction in transfusion-dependent children and young adults detected with low-dose dobutamine stress echocardiography.

Transfusion-dependent (TD) patients develop cardiac iron overload that will eventually lead to cardiac pump failure. Low-dose dobutamine stress echocardiography may complement resting echocardiography and identify preclinical myocardial dysfunction caused by early cardiac hemosiderosis. Twenty-six iron-overloaded TD patients had stress echocardiography with 5 microg/kg per minute of dobutamine. Indexed left ventricular (LV) mass, LV dimensions, meridional wall stress, and cardiac index were significantly increased. TD patients had similar LV shortening fraction by M-mode (40.5% +/- 5.6% vs 39.4% +/- 4.5%) but had a lower mean LV ejection fraction (53.3% +/- 3.9% vs 46.8% +/- 6.9%, P < .002) and a subnormal increase in cardiac index during dobutamine stress (35% +/- 20% vs 11% +/- 16%, P < .0001). Impairment in LV relaxation was demonstrated by a prolonged isovolumetric relaxation time (0.060 +/- 0.005 vs 0.088 +/- 0.019 seconds, P < .0001), increased peak mitral E wave, and abnormal E/A ratio. Asymptomatic TD patients demonstrate decreased systolic functional reserve and abnormal left ventricular relaxation that may be caused by cardiac hemosiderosis. Low-dose dobutamine stress echocardiography may be useful for detecting and following cardiac dysfunction in patients at risk for cardiac hemosiderosis.

Electrophysiologic study and radiofrequency ablation in patients with intracardiac tumors and accessory pathways: is the tumor the pathway?

INTRODUCTION: There is a strong association of cardiac rhabdomyomas with the Wolff-Parkinson-White syndrome. This report describes the results of investigations in two patients with accessory pathway-mediated AV reciprocating tachycardia coexisting with intracardiac tumors. METHODS AND RESULTS: Two patients with intracardiac tumors had mapping of the accessory pathway. Echocardiograms obtained in the electrophysiology laboratory while the ablation catheter was at the site of successful radiofrequency ablation demonstrated a close correspondence between the site of intracardiac tumor and the location of the accessory pathway. CONCLUSIONS: These results lend support to the hypothesis that accessory pathways in patients with intracardiac tumors, such as rhabdomyomas, are not typical Kent bundles, but instead are either part of the intracardiac tumor or are closely related to the tumor. Ablation is possible in at least some patients with accessory pathways associated with intracardiac tumors.

Prenatal myocardial infarction suspected by fetal echocardiography.

Myocardial infarction is rare in the newborn period. We describe a case in which myocardial infarction was suspected at 25 weeks of gestation by fetal echocardiography. There was an aneurysm of the left ventricular apex with paradoxical motion and bulging of the left ventricular free wall during systole. The diagnosis was confirmed by postnatal electrocardiogram, vectorcardiogram, and thallium myocardial perfusion imaging.

Association of systemic to pulmonary collateral arteries with tetralogy of Fallot and absent pulmonary valve syndrome.

Two patients with tetralogy of Fallot and absent pulmonary valve syndrome with significant systemic to pulmonary collateral arteries are described. Search for these rarely reported vessels should be included in the workup of these complex patients.

Technetium-99m DISIDA hepatobiliary tract imaging in pseudotumors of the liver.

Tc-99m sulfur colloid images of the liver are frequently falsely reported as tumor or metastases in certain space occupying lesions of the liver. Whether a primary malignancy is known or not, every suspicious space-occupying lesion of the liver should be given the benefit of alternate diagnostic studies before a firm diagnosis of neoplasia is made. This is critical when the decision regarding chemotherapy has to be made. Timely intervention by other diagnostic modalities may obviate the consideration of chemotherapy in cases where there are no liver metastases. The case presented below illustrates the value of hepatobiliary tract imaging in pseudotumors of the liver.