Repolarization disparity as a predictor of response to Head up Tilt-table Test in pediatric syncope.

BACKGROUND: Head up Tilt-table Test (HUTT) is a practical examination of the most common type of pediatrics syncope. The electrocardiographic (ECG) changes during this test, show the autonomic defects that cause neuraly-mediated syncope in response to tilting process. METHODS: All pediatric syncope patients referred to our center in a 1-year period, were included in the study. HUTT was performed and patients were classified into two groups of Negative and Positive HUTT results, and the latter group was subclassified as three subgroups of "vasodepressor", "cardioinhibitory" and "mixed type" responses to HUTT. QT and corrected QT (QTc) dispersion was measured by the baseline standard 12-lead ECG obtained before HUTT. RESULTS: Eighty-six patients with a mean age of 12.19 ± 5.34 were included. Patients with positive HUTT were significantly younger and male gender was more prevalent in this group. Mean QT dispersion was significantly higher in patients with positive HUTT result and also in patients with mixed response to HUTT compared to isolated vasodepressor response. Duration of QTc interval did not change between different study groups. Reciever-Operating-Characteristic (ROC) analysis showed that QT dispersion higher than 32 ms is a significant predictor of positive HUTT result (with 92% sensitivity and 98% specificity) and values higher than 40 ms can predict the mixed type of response to HUTT (with 84% sensitivity and 63% specificity). CONCLUSIONS: Baseline myocardial repolarization disparity significantly correlates with susceptibility to symptomatic vasovagal syncope. This pathology seems to play its role mainly via excessive vagotonic response to sympathetic activation during HUTT process (known as cardioinhibitory response).

Non diagnosed PAPVC induce large reverse venovenous shunt after modified Fontan surgery: A case report of a rare anomaly and embolization therapy.

Fontan operation is a reliable palliative surgery for patients with single ventricle physiology. Still, the development of complication is common; one of these complications that need to interventional approach is veno-venous collaterals between systemic and pulmonary veins. A 16-yearoldgirl with a history of modified Fontan operation at 9 years ago was referred with progressive cyanosis and dyspnea on exertion. In contrast trans-thoracic echocardiography (TTE), no fenestration was seen in Fontan circulation. Cardiac magnetic resonance revealed partial anomalous pulmonary vein connection (PAPVC) from left upper pulmonary vein to vertical vein and then into the in nominate vein and SVC with the reverse flow from superior vena cava (SVC) to left upper pulmonary vein(LUPV). This anomalous vein became severe engorged and tortuous. Possibly, LUPV and the verticalvein was dilated gradually as a result of increased pressure in the Fontan circuit. Finally, she underwent successful coil embolization in the midpart of the vertical vein. The oxygen saturation increased from80% to 93%.

Complete isolation of right subclavian artery supplied by the thoracic aorta and bilateral patent ductus arteriosus.

Disconnection of the subclavian artery from the aortic arch, defined as isolation, is a rare aortic arch anomaly. In this anomaly, loss of continuity between one subclavian artery and the aorta with persistent connection to the homocollateral pulmonary artery through a patent or remnant ductus arteriosus is present. This isolation is almost always seen on the side contralateral to the aortic arch position. Complex cardiac and genetic anomalies are frequently associated with the isolation of a subclavian artery. We report a rare case of right subclavian artery originating from right pulmonary artery with bilateral patent ductus arteriosus in an otherwise normal infant.

Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: The purpose of this study was to compare the safety and efficacy of surgical, stent, and balloon angioplasty (BA) treatment of native coarctation acutely and at follow-up. BACKGROUND: Controversy surrounds the optimal treatment for native coarctation of the aorta. This is the first multicenter study evaluating acute and follow-up outcomes of these 3 treatment options in children weighing >10 kg. METHODS: This is a multicenter observational study. Baseline, acute, short-term (3 to 18 months), and intermediate (>18 months) follow-up hemodynamic, imaging data, and complications were recorded. RESULTS: Between June 2002 and July 2009, 350 patients from 36 institutions were enrolled: 217 underwent stent, 61 underwent BA, and 72 underwent surgery. All 3 arms showed significant improvement acutely and at follow-up in resting systolic blood pressure and upper to lower extremity systolic blood pressure gradient (ULG). Stent was superior to BA in achieving lower ULG acutely. Surgery and stent were superior to BA at short-term follow-up in achieving lower ULG. Stent patients had shorter hospitalization than surgical patients (2.4 vs. 6.4 days; p < 0.001) and fewer complications than surgical and BA patients (2.3%, 8.1%, and 9.8%; p < 0.001). The BA patients were more likely to encounter aortic wall injury, both acutely and at follow-up (p < 0.001). CONCLUSIONS: Stent patients had significantly lower acute complications compared with surgery patients or BA patients, although they were more likely to require a planned reintervention. At short-term and intermediate follow-up, stent and surgical patients achieved superior hemodynamic and integrated aortic arch imaging outcomes compared with BA patients. Because of the nonrandomized nature of this study, these results should be interpreted with caution.

Stenting of aortic coarctation: acute, intermediate, and long-term results of a prospective multi-institutional registry--Congenital Cardiovascular Interventional Study Consortium (CCISC).

INTRODUCTION: Since the 1980s, stent implantation has evolved as an important therapeutic strategy for coarctation of the aorta. However, available data is frequently flawed by short follow-up, lack of adequate follow-up imaging, and retrospective nature of data collection. METHODS: Data was prospectively collected using a multicenter registry congenital cardiovascular interventional study consortium (CCISC). Between 2000 and 2009, 302 patients from 34 centers with a median weight of 58 kg underwent stent implantation for coarctation. Eligible patients (44%) completed intermediate follow-up (3-18 months) with integrated imaging (cath, CT, MRI), whereas 21% completed long-term follow-up (>18-60 months). Procedural success was defined as UL/LL systolic gradient of less than 20 mm Hg, lack of significant recurrent obstruction, and freedom from unplanned repeat intervention. RESULTS: Acute procedural success was 96%. Cumulative intermediate success was 86%, and cumulative long-term success was 77%. Unplanned repeat interventions were required in 4%, and aortic wall complications were seen in 1% of patients (dissection n = 1 and aneurysm n = 3). Other adverse events (n = 15) occurred mainly acutely and included technical complications such as stent malposition (n = 9). At long-term follow-up, 23% of patients continued to have systolic blood pressure above the 95th centile, 9% had an upper-to-lower limb blood pressure gradient in excess of 20 mm Hg, and 32% were taking antihypertensive medication. CONCLUSIONS: This study documented acute, intermediate, and long-term outcome data comparable or superior with other surgical or interventional series. However, even with successful initial stent therapy, patients continue to require long-term follow-up and have associated long-term morbidity, relating to aortic wall complications, systemic hypertension, recurrent obstruction as well as need for repeat intervention.

Trans-catheter closure of patent ductus arteriosus-What is the best device?

BACKGROUND: Over the past three decades, transcatheter occlusion of patent ductus arteriosus (PDA) has evolved to be the procedure of choice. Gianturco and Flipper coils are the most commonly used coils in the United States for closure of small and moderate size PDAs. For larger PDAs, interventionalists in the United States commonly use the Amplatzer Duct Occluder (ADO) and those in Europe use the ADO or the Nit-Occlud Coils (NOC). A comparison between Gianturco coils, Flipper coils, ADO, and NOC has never been made. OBJECTIVE: To compare the success and complication rate associated with the four different devices used for transcatheter closure of PDA. Success was defined as complete closure of PDA with absence of a residual shunt (R.S.) at six months follow-up. METHODS: Two institutions collaborated in combining their data to evaluate the results of transcatheter closure of PDA. RESULTS: Totally, 546 patients underwent successful PDA occlusion at both institutions. Gianturco and Flipper coils were used in 120 (22%) and 119 (22%) patients respectively. A total of 152 (28%) patients received ADO and 155 (28%) patients received NOC. Immediate R.S. were noted in 226 (41.4%) patients in the entire study group with the NOC group having the highest percentage of R.S. (80/155, 51.6%, P = 0.004). Of the 484 patients with follow-up echocardiograms at 6 months, 35 (7.2%) patients had persistent R.S. The NOC (3/143, 2.1%) and ADO (5/150, 3.3%) groups had the least R.S. at six months follow-up. CONCLUSION: Per our definition of success, the Nit-Occlud coils and the Amplatzer duct-occluder devices had significantly higher success rate for PDA occlusion versus the coils. © 2010 Wiley-Liss, Inc.

Cervical mass following carotid attempt at interventional repair of aortic coarctation: a case report.

A 4-month-old boy was admitted to our hospital following an unsuccessful attempt at interventional repair of aortic coarctation via the right carotid artery, which seemed to have given rise to the formation and growth of a cervical mass overlying the entry site. Despite the initial anticipation of difficulty during intubation due to the pressure effect of the mass, anesthesia progressed uneventfully, the mass, which was a hematoma, was evacuated, and the coarctation was repaired. The patient was discharged after the operation. At three weeks' follow-up, there was no significant lesion in the neck and transthoracic echocardiography demonstrated no residual coarctation.