Objective: This study aimed to identify the best graft-to-pulmonary artery (PA) anastomosis angle measuring pulmonary blood flow, wall shear stress (WSS), and shunt flow. Methods: A tetralogy of Fallot with pulmonary atresia computer model was used to study three different modified Blalock-Taussig shunt (mBTS) anastomosis angle configurations with three different PA diameter configurations. Velocity and WSS were analyzed, and the flow rates at the right PA (RPA) and left PA (LPA) were calculated. Results: A 4-mm and 8-mm diameter of RPA and LPA, respectively with vertical shunt angle produces the highest total flow. In the RPA larger diameter than the LPA configutations, the left-leaning shunt produces the lowest total PA flow whereas in the LPA larger diameter than the RPA configuratios, the right-leaning shunt produces the lowest total PA flow. Therefore, the shunt anastomosis should not be leaned through the narrow side of PA to reach best flow. As the flow inside the shunt increased, WSS also increased due to enhanced velocity gradients. Conclusion: The anastomosis angle between the conduit and PA affects the flow to PA. Vertical anastomosis configurations increase the total PA flow; thus, these configurations are preferable than the leaned configurations.

In patients undergoing congenital cardiac surgery, it is crucial to maintain oxygen demand-consumption balance. Central venous oxygen saturation (ScvO2) is a useful indicator of oxygen demand and consumption balance which is an invasive method. Near-infrared spectroscopy (NIRS) is a noninvasive, continuous monitoring technique that measures regional tissue oxygenation. NIRS that is placed over the internal jugular vein cutaneous area (NIRSijv) has the potential to show ScvO2 indirectly. In this study, we aimed to determine the correlation between ScvO2 with NIRSijv in pediatric patients undergoing congenital cardiac surgery. Fifty children participated in the study. Four patients were excluded for the inability of internal jugular vein (IJV) catheterization due to technical difficulties. After anesthesia induction, NIRS probes were placed on the IJV site with ultrasound guidance for the measurement of continuous transcutaneous oxygen saturation. The catheter insertion was also done through the IJV from the other side using ultrasound guidance. Cerebral oxygenation monitoring was done using NIRS with a single pediatric probe placed on the right forehead. Values of NIRSijv, cerebral NIRS (NIRSc) and ScvO2, were recorded at certain times until postoperative 24th hour. Data were collected at 8 different time points. There was a significant correlation between ScvO2 and NIRSijv in all measurement time points (r = 0.91), (p = 0.001). The mean bias between ScvO2 and NIRSijv was 2.92% and the limits of agreement were 11 to -5.2%. There was a moderate correlation between ScvO2 and NIRSc (r = 0.45), (p = 0.001). The mean bias between ScvO2 and NIRSc was 2.7% and the limits of agreement were +26 to -20%. In this study, we found a strong correlation between ScvO2 and NIRS measurements taken from the internal jugular vein site. Accordingly, continuous noninvasive monitoring with transcutaneous NIRSijv can be an alternative method as a trend monitor for the central venous oxygen saturation in pediatric cardiac patients undergoing congenital cardiac surgery.

Background. Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); non-inflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported. Hereby we report this unique association with CACP syndrome. Case. An eleven-year-old girl presented with non-productive cough, dyspnea, and orthopnea. She was diagnosed CACP syndrome at the age of seven and a biallelic frameshift mutation in the PRG4 gene was determined. The physical examination revealed pectus excavatum, camptodactyly, genu valgum, tachypnea and orthopnea. The functional capacity was NYHA III-IV. She had 2/6 soft pansystolic murmur at 4th left intercostal space and a rumbling diastolic murmur at apex. Echocardiography revealed an enlarged left atrium, severe stenotic mitral valve with a mean diastolic transmitral gradient of 22.5 mmHg, mild mitral regurgitation and mild apical pericardial effusion. The patient had mitral comissurotomy and partial pericardiectomy operation. Her post-operative transmitral gradient decreased to 6.9 mmHg and the pulmonary pressure was 30 mmHg. Her functional capacity increased to NYHA I-II. Conclusions. The main defect is the proteoglycan 4 protein which acts like a lubricant in articular and visceral surfaces. Therefore, the leading clinical feature is arthropathy. Cardiac involvement other than clinically mild pericarditis is not usually expected. Three types of proteoglycans (decorin, biglycan, and versican) are present in the mitral valve. This could be the reason of mitral valve involvement in rare cases as like ours. It is important that these patients undergo echocardiographic examination regularly.