Transcatheter closure of a large aortopulmonary window with the novel device Multifunctional occluder (Konar) under TEE guidance (A case report).

Aortopulmonary window is a rare congenital heart disease. It results from incomplete separation of the aorta and pulmonary artery at the conotruncal septum. It accounts for 0.2%-0.6% of all congenital cardiac defects. Closure should be done in all cases. We present the case of a 12-month-old female child with a large aortopulmonary window, complicated by severe pulmonary hypertension. Cardiac catheterization was performed and showed pulmonary vascular resistance 3 Woods units. Ascending aortography showed a large aortopulmonary window measuring 5 mm. Balloon sizing of the defect showed stretched diameter of 8 mm. A multifunctional occluder device 12 × 10 mm (Konar, Lifetech, Shenzhen, China) was properly positioned across the defect under transesophageal echocardiography guidance. Pulmonary angiograms showed a well seated pulmonary disc with mild encroachment on pulmonary bifurcation. Follow up transthoracic echocardiography 24 hours after the procedure showed a well seated device with no residual flow, mean pulmonary artery pressure 25 mmHg, laminar flow into main pulmonary artery and pulmonary bifurcation. To the best of our knowledge this is the first successful closure to be reported for an aortopulmonary window with Konar multifunctional occluder. Having a double disc with a low profile and small sheath size may allow closure of larger defects in smaller children. .

Can Tei Index Predict High Syntax Score in Patients with Chronic Coronary Syndrome and Normal Left Ventricular Systolic Function?

OBJECTIVE: Some patients who had chronic coronary syndrome (CCS) and were recognized as low risk, however, developed cardiovascular events, whereas others who were categorized as high risk did not develop any cardiovascular events. Invasive coronary angiography is the gold standard tool for the assessment of coronary artery disease (CAD) severity. The SYNTAX score (SS) was recently recognized as an invasive angiographic-guided scoring system used in risk stratification of patients who have more than one-vessel CAD and undergoing revascularization with percutaneous cardiovascular intervention. It has a good predictive value of adverse cardiovascular events. Exploration for unique noninvasive modalities that may help in a better way for risk stratification of CCS patients by predicting the severity of CAD (as reflected by SS) would be of a paramount value. Tei index is a promising modality for that objective, which is a Doppler-derived time interval index that combines both systolic and diastolic cardiac performance. METHODS: We examined the relationship between the severity of CAD as assessed by the SS and Tei index in 100 patients with CCS and normal left ventricular systolic function. RESULTS: All the studied 100 patients had a normal ejection fraction with mean = 58.92 ± 7.88; the mean value of Tei index was 0.84 ± 0.26. There was a statistically significant positive association between Tei index and SS (P = 0.0001); moreover, there was a correlation between left anterior descending (LAD) affection and Tei index (P = 0.0001).The cutoff point of Tei index to detect SS above 22 was >0.93 (with specificity of 86.5% and sensitivity of 42.4%). CONCLUSION: Tei index significantly correlates with SS and LAD affection. Moreover, it is a cheap, radiation-free, noninvasive technique and may be used as a further risk stratification modality beyond others.

Feasibility and accuracy of real-time three-dimensional echocardiography in evaluating the aortic valve in children.

BACKGROUND: Aortic valve assessment by 2D transthoracic echocardiography is a relatively complex task owing to the unique anatomical features of the left ventricular outflow tract and its dynamic nature. We aimed to evaluate the accuracy of 3D transthoracic echocardiography [3D TTE] in assessing the aortic valve in children. RESULTS: The first group included 11 males and six females, with a mean age of 5.76 ± 6.39 years. All of these patients had aortic valve disease with a bicuspid variant. The second group included seven males and seven females, with a mean age of 4.4 ± 4.05 years. All of these patients had normal aortic valve morphology and had another congenital cardiac anomaly. The aortic valve annulus was assessed using the three modalities; 2D, 3D echocardiography in the vertical and horizontal diameters, and angiography. The aortic valve area was measured by 2D and 3D echocardiography using multiplane reformatted mode. The results of the analysis were then compared. They revealed that 3D echocardiographic measurement of the aortic annulus (horizontal diameter) correlated better with angiography than 2D and 3D (vertical diameter) echocardiographic measurements. There was a significant difference between the aortic valve area measured by 2D echocardiography and that measured by 3D echocardiography among the two groups, 2D echocardiography seems to underestimate the true aortic valve area. CONCLUSION: The study concluded that 3D TTE with multiplane reformatted mode allows a more accurate assessment of the aortic valve when compared to 2D echocardiography and this correlates better with the angiographic findings.

Immediate and short term effects of percutaneous atrial septal defect device closure on cardiac electrical remodeling in children.

BACKGROUND: The beneficial effects of atrial septal defect (ASD) device closure on electrical cardiac remodeling are well established. The timing at which these effects starts to take place has yet to be determined. OBJECTIVES: To determine the immediate and short term effects of ASD device closure on cardiac electric remodeling in children. METHODS: 30 pediatric patients were subjected to 12 lead Electrocardiogram immediately before ASD device closure, 24 h post procedure, 1 and 6 months after. The maximum and minimum P wave and QT durations in any of the 12 leads were recorded and P wave and QT dispersions were calculated and compared using paired T test. RESULTS: The immediate 24 h follow up electrocardiogram showed significant decrease in P maximum (140.2 ±â€¯6 versus 130.67 ±â€¯5.4 ms), P dispersion (49.73 ±â€¯9.01 versus 41.43 ±â€¯7.65 ms), PR interval (188.7 ±â€¯6.06 ms versus 182.73 ±â€¯5.8 ms), QRS duration (134.4 ±â€¯4.97 ms versus 127.87 ±â€¯4.44), QT maximum (619.07 ±â€¯15.73 ms versus 613.43 ±â€¯11.87), and QT dispersion (67.6 ±â€¯5.31 versus 62.6 ±â€¯4.68 ms) (P = 0.001). After 1 month all the parameters measured showed further significant decrease with P dispersion reaching 32.13 ±â€¯6 (P = 0.001) and QT dispersion reaching 55.0 ±â€¯4.76 (P = 0.001). These effects were maintained 6 months post device closure. CONCLUSION: Percutaneous ASD device closure can reverse electrical changes in atrial and ventricular myocardium as early as the first 24 h post device closure.

Radiation exposure in children during the current era of pediatric cardiac intervention.

Cardiac catheterizations are among the X-ray procedures with the highest patient radiation dose and therefore are of great concern in pediatric settings. This study aimed to evaluate factors that influence variability of X-ray exposure in children with congenital heart diseases during cardiac catheterization. The study included 107 children who underwent either diagnostic (n = 46) or interventional (n = 61) procedures. A custom-made sheet for patient and procedural characteristics was designed. Data were collected, and different correlations were applied to determine factors that influence variability of X-ray exposure. The fluoroscopy time (FT) differed significantly between the diagnostic (8.9 ± 6.3 min) and intervention (12.8 ± 9.98 min) groups (P = 0.032). The mean dose-area product (DAP) differed significantly between the two groups (3.775 ± 2.5 Gy/cm(2) vs. 13.239 ± 15.4 Gy/cm(2); P = 0.003). The highest DAP was during left anterior oblique (LAO) cranial 30° angulation (2.8 Gy/cm(2)/4 s cine). The mean cumulative dose (CD) was 0.053 Gy in diagnostic cases and 0.48 Gy in intervention cases. The effective dose was 5.97 ± 7.05 mSv for therapeutic procedures compared with 3.42 ± 3.64 mSv for diagnostic procedures. The FT correlated significantly with both the DAP (r = 0.718; P < 0.001) and the CD (r = 0.701; P < 0.001). Other correlations were reported. An increasing number of therapeutic catheterization procedures are being performed for children. The justification for these procedures is evident because they avoid complicated surgery. However, the complexity of these procedures results in higher radiation exposures.

Noninvasive assessment of elevated pulmonary vascular resistance in children with pulmonary hypertension secondary to congenital heart disease: A comparative study between five different Doppler indices.

BACKGROUND: Pulmonary vascular resistance (PVR) is an important hemodynamic parameter in patients with congenital heart disease (CHD). Noninvasive estimation of PVR represents an attractive alternative to invasive measurements. METHODS: The study included 175 patients with pulmonary hypertension (PH) secondary to CHD. All patients underwent full echocardiographic study and invasive hemodynamic measurements. The study population was then subdivided into four subgroups. Each of the following Doppler indices was measured in one of these four subgroups: peak tricuspid regurgitant velocity (TRV), the ratio of the TRV to the velocity time integral of the right ventricular outflow tract (TRV/TVIRVOT), peak velocity of tricuspid annular systolic motion (TSm), heart rate corrected acceleration time and infliction time of the proximal left pulmonary artery (ATc, InTc). The data obtained was correlated with invasive PVR measurement. An ROC curve analysis was done to generate cutoff points with the highest balanced sensitivity and specificity to predict PVR > 6WU/m(2). The receiver operating characteristics (ROC) curves were compared with each other to determine the most reliable cutoff point in predicting elevated PVR > 6WU/m(2). RESULTS: There was a significant correlation between both the TRV and TSm and invasive measurement of PVR (r = -0.511, 0.387 and P value = 0.0002, 0.006 respectively). The TSm and TRV cutoff values were the most reliable to predict elevated PVR > 6 WU/m(2). A TSm cutoff value of ⩽16.16 cm/s provided the best balanced sensitivity (85.7%) and specificity (66.7%) to determine PVRCATH > 6 WU/m(2). A cutoff value less than 7.62 cm/s had 100% specificity to predict PVRCATH > 6 WU/m(2). A TRV cutoff value of >3.96 m/s provided the best balanced sensitivity (66.7%) and specificity (100%) to determine PVRCATH > 6 WU/m(2). Both TRV and TSm had the highest area under the ROC curve among the 5 DOPPLER indices studied. CONCLUSION: Prediction of elevated PVR in children with PH secondary to CHD could be achieved noninvasively using a number of Doppler indices. Among the five Doppler indices examined in the current study, the peak TRV and the TSm of the lateral tricuspid annulus had the highest balanced sensitivity and specificity to predict PVRI > 6 WU/m(2).

Immediate- and medium-term effects of balloon pulmonary valvuloplasty in infants with critical pulmonary stenoses during the first year of life: A prospective single center study.

BACKGROUND: Balloon pulmonary valvuloplasty (BPV) represents the standard of management for all patients with severe pulmonary stenosis (PS) irrespective of their age. Nevertheless neonates and infants with critical PS represent a high-risk group that needs to be studied. METHODS: The study population included 72 infants with severe congenital valvular PS and four infants with imperforate pulmonary valve (PV) who were subjected to detailed history taking, full clinical examination, resting 12-lead ECG, Chest roentgenogram and transthoracic echocardiography. BPV was attempted in all infants with a peak-to-peak gradient across the PV of 50 mmHg or greater at catheterization-laboratory. Full echocardiographic evaluation was done 24 hours after the procedure as well as 3 and 6 months later. RESULTS: Seventy-six infants with severe PS or imperforate PV with a mean age of 5.63 ± 2.99 months were subjected to BPV with or without wire perforation. Immediately after the procedure patients had a significant reduction of the right ventricular systolic pressure (RVSP) (104.69 ± 24.98 mm Hg Vs 43.6 ± 13 mm Hg, p < 0.001) and RV-PA systolic pressure gradient (PG) (82.5 ± 23.76 mm Hg Vs 17.35 ± 8.96 mm Hg, p < 0.001). The immediate success rate defined as the drop in the RVSP to less than or equal to 50% of the baseline measurement was achieved in 85% of the cases. There was a progressive drop in the PG across the PV by Doppler echocardiogram throughout a follow-up period of six months from a mean of 93.3 ± 28.2 mm Hg to a mean of 17.4 ± 10.42 mm Hg (p < 0.001). There was a significant increase of the mean PV annulus diameter after balloon dilatation (p < 0.001). There was also a highly significant inverse correlation between the growth of the pulmonary annulus and the annular size at the baseline before dilatation (r = -0.74, p value <0.001). The incidence of PR significantly increased immediately after BPV to 64% followed by a progressive decline over a 6 months period of follow-up to 20%. There was a significant decrease in the incidence of tricuspid regurgitation (TR) over the same period of follow-up (from 55.6% at baseline to less than 20% at follow-up). CONCLUSION: BPV is safe and effective to relieve critical PS in infants during the first year of life. The balloon promotes advantageous changes in both, pulmonary annulus and PG across the RVOT. In addition, the Doppler gradient observations during the follow-up support the expectation that BPV is a "curative" therapy.