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Hypoplastic left heart syndrome is a complex congenital heart lesion that requires a three-stage procedure for surgical palliation. This clinical trial examines two approaches to the first stage of the procedure, and the results provide important guidance for the most appropriate surgical management of this serious lesion. This clinical trial examines two approaches to the first stage of the Norwood procedure, and the results provide important guidance for the most appropriate surgical management of this serious lesion. Hypoplastic left heart syndrome and related anomalies involving a single right ventricle are characterized by hypoplasia of the left heart and the aorta, with compromised systemic cardiac output (Figure 1). Infants with the syndrome generally undergo a three-stage reconstruction culminating in the Fontan procedure. The first operation (stage I) is the Norwood procedure, in which the right ventricle is connected to a reconstructed aorta with the use of the proximal main pulmonary artery for systemic outflow. Pulmonary blood flow is reestablished by means of a shunt from the pulmonary artery to the systemic circulation. In the second operation (stage II), . . .

To assess the feasibility and outcome of stent strut dilation after arterial duct stenting with associated branch pulmonary artery (BPA) stenosis. Stenting of arterial duct in infants with duct-dependent pulmonary circulation is technically challenging. The presence of BPA stenosis is a relative contraindication for stent implantation. Infants with duct-dependent pulmonary circulation and associated BPA stenosis were assessed either by transthoracic echocardiogram alone or additional computerized tomography angiogram when required. Following ductal stenting, the stent struts of the stenosed BPA were crossed with an additional 0.014″ coronary guide wire and dilated using coronary balloons (2.0 or 2.5 mm in diameter). Seventeen (12 male) patients were considered for the procedure. The median age and weight were 27 days (range 2–94) and 2.6 kg (range 2.2–5), respectively. Fourteen patients (82.4%) underwent stent strut dilation after arterial duct stenting. Struts to left pulmonary artery was opened in 9 (64.3%) and right pulmonary artery in 5 (35.7%). The mean systemic oxygen saturation increased from 66.23 ± 8.9% at baseline to 86 ± 2.2% immediately after the stent deployment and final saturations after stent strut dilation were 89.29 ± 4.3%. Angiographic pulmonary flow improved in all cases. Stent strut dilation could not be done in 3 patients due to unfavorable anatomy. One patient had acute stent thrombosis and died in the hospital. Two others died during follow-up, during an acute febrile illness and gastroenteritis. All survivors underwent cardiac surgery and were on regular follow-up. Strut dilation of BPA stenosis is feasible to augment pulmonary blood flow, following arterial duct stenting. This procedure may be useful in selected patients with BPA stenosis to have uniform growth of pulmonary arteries.

For some surgical procedures to be done, a patient's blood circulation needs to be stopped. In such situations, the maintenance of blood flow to the brain is perceived beneficial even in the presence of deep hypothermia. We aimed to assess the benefits of the maintenance of antegrade cerebral perfusion (ACP) compared with deep hypothermic circulatory arrest (DHCA). Patients aged 18–80 years undergoing pulmonary endarterectomy surgery in a UK centre (Papworth Hospital, Cambridge) were randomly assigned with a computer generated sequence to receive either DHCA for periods of up to 20 min at 20°C or ACP (1:1 ratio). The primary endpoint was change in cognitive function at 12 weeks after surgery, as assessed by the trail-making A and B tests, the Rey auditory verbal learning test, and the grooved pegboard test. Patients and assessors were masked to treatment allocation. Primary analysis was by intention to treat. The trial is registered with Current Controlled Trials, number ISRCTN84972261. We enrolled 74 of 196 screened patients (35 to receive DHCA and 39 to receive ACP). Nine patients crossed over from ACP to DHCA to allow complete endarterectomy. At 12 weeks, the mean difference between the two groups in Z scores (the change in cognitive function score from baseline divided by the baseline SD) for the three main cognitive tests was 0·14 (95% CI −0·14 to 0·42; p=0·33) for the trail-making A and B tests, −0·06 (−0·38 to 0·25; p=0·69) for the Rey auditory verbal learning test, and 0·01 (−0·26 to 0·29; p=0·92) for the grooved pegboard test. All patients showed improvement in cognitive function at 12 weeks. We recorded no significant difference in adverse events between the two groups. Three patients died (two in the DHCA group and one in the ACP group). Cognitive function is not impaired by either ACP or DHCA. We recommend circulatory arrest as the optimum modality for patients undergoing pulmonary endarterectomy surgery. J P Moulton Charitable Foundation.

The Single Ventricle Reconstruction (SVR) Trial was a randomized prospective trial designed to determine survival advantage of the modified Blalock-Taussig-Thomas shunt (BTTS) vs the right ventricle to pulmonary artery conduit (RVPAS) for patients with hypoplastic left heart syndrome. The primary aim of the long-term follow-up (SVRIII) was to determine the impact of shunt type on RV function. In this work, we describe the use of CMR in a large cohort follow up from the SVR Trial as a focused study of single ventricle function. The SVRIII protocol included short axis steady-state free precession imaging to assess single ventricle systolic function and flow quantification. There were 313 eligible SVRIII participants and 237 enrolled, ages ranging from 10 to 12.5 years. 177/237 (75%) participants underwent CMR. The most common reasons for not undergoing CMR exam were requirement for anesthesia ( n  = 14) or ICD/pacemaker ( n  = 11). A total of 168/177 (94%) CMR studies were diagnostic for RVEF. Median exam time was 54 [IQR 40–74] minutes, cine function exam time 20 [IQR 14–27] minutes, and flow quantification time 18 [IQR 12–25] minutes. There were 69/177 (39%) studies noted to have intra-thoracic artifacts, most common being susceptibility artifact from intra-thoracic metal. Not all artifacts resulted in non-diagnostic exams. These data describe the use and limitations of CMR for the assessment of cardiac function in a prospective trial setting in a grade-school-aged pediatric population with congenital heart disease. Many of the limitations are expected to decrease with the continued advancement of CMR technology.

A 9-day-old male neonate was found to have a systolic murmur during a routine follow-up for skin jaundice. Imaging revealed a large mass at the bifurcation of the main pulmonary artery, causing significant bilateral stenosis. The patient underwent emergency surgery due to critically compromised pulmonary blood flow. Intraoperative exploration demonstrated a large ductus arteriosus aneurysm (DAA) with extensive thrombosis. The DAA was completely resected under deep hypothermic circulatory arrest, followed by reconstruction of the descending aorta and repair of the pulmonary artery bifurcation defect. The patient was discharged on the 20th postoperative day and remained asymptomatic at the 3-month follow-up, exhibiting normal growth and cardiac function.

Background Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events. Case presentation A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg. Conclusion ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

Not all stents are suitable for children. For instance, premounted stents can be used in infants and small children but cannot dilate with age to accommodate adult-sized pulmonary arteries. Conversely, the Pul-Stent adapts to somatic growth. Thus, our hospital implemented the Pul-Stent in pediatric patients with branch pulmonary artery stenosis. This study summarizes our initial experience with Pul-Stents in this patient population, including the efficacy and safety. We implanted 37 Pul-Stents in 35 patients between August 2014 and June 2015. The patients’ mean age and weight at stent implantation were 6.7 ± 3.0 years and 20.9 ± 8.7 kg, respectively. Bench testing revealed that axial shortening of the Pul-Stent was minimal with further dilation, and the radial strength did not change. The stents were successfully deployed in all cases, except two with minor malpositioning. Primarily, 8–12 mm mounting balloons were used for the initial implantation, and a long sheath (8–10 F) was used for delivery. After stent implantation, the minimal lumen diameter in the stenosed segment increased by 50% in 97% (34/35) of patients. Furthermore, the pressure gradient across the stenosed segment decreased by 50% in 77% (23/30) of biventricular patients. One stent fracture and one stent restenosis were noted during the follow-up visits (mean follow-up time: 4.6 ± 1.7 years). Eighteen patients (51%) underwent repeat catheterization; ten had successful redilation. No aneurysms or stent fractures were observed. Our initial results indicate that the Pul-Stent is safe and effective in pediatric patients and can be further dilated over time to accommodate somatic growth. Moreover, the Pul-Stent has good compliance and adequate radial strength to treat pulmonary artery stenosis effectively.

Surgical treatment of congenital heart disease (CHD) involves complex vascular reconstructions utilizing artificial and native surgical materials. A successful surgical reconstruction achieves an optimal hemodynamic profile through the graft in spite of the complex post-operative vessel growth pattern and the altered pressure loading. This paper proposes a new in silico patient-specific pre-surgical planning framework for patch reconstruction and investigates its computational feasibility. The proposed protocol is applied to the patch repair of main pulmonary artery (MPA) stenosis in the Tetralogy of Fallot CHD template. The effects of stenosis grade, the three-dimensional (3D) shape of the surgical incision and material properties of the artificial patch are investigated. The release of residual stresses due to the surgical incision and the extra opening of the incision gap for patch implantation are simulated through a quasi-static finite-element vascular model with shell elements. Implantation of different unloaded patch shapes is simulated. The patched PA configuration is pressurized to the physiological post-operative blood pressure levels of 25 and 45 mmHg and the consequent post-operative stress distributions and patched artery shapes are computed. Stress–strain data obtained in-house, through the biaxial tensile tests for the mechanical properties of common surgical patch materials, Dacron, Polytetrafluoroethylene, human pericardium and porcine xenopericardium, are employed to represent the mechanical behavior of the patch material. Finite-element model is experimentally validated through the actual patch surgery reconstructions performed on the 3D printed anatomical stenosis replicas. The post-operative recovery of the initially narrowed lumen area and post-op tortuosity are quantified for all modeled cases. A computational fluid dynamics solver is used to evaluate post-operative pressure drop through the patch-reconstructed outflow tract. According to our findings, the shorter incisions made at the throat result in relatively low local peak stress values compared to other patch design alternatives. Longer cut and double patch cases are the most effective in repairing the initial stenosis level. After the patch insertion, the pressure drop in the artery due to blood flow decreases from 9.8 to 1.35 mmHg in the conventional surgical configuration. These results are in line with the clinical experience where a pressure gradient at or above 50 mmHg through the MPA can be an indication to intervene. The main strength of the proposed pre-surgical planning framework is its capability to predict the intra-operative and post-operative 3D vascular shape changes due to intramural pressure, cut length and configuration, for both artificial and native patch materials.

Branch pulmonary artery stenosis is common after surgical repair in patients with biventricular CHD and often requires reinterventions. However, (long-term) effects of percutaneous branch pulmonary artery interventions on exercise capacity, right ventricular function, and lung perfusion remain unclear. This review describes the (long-term) effects of percutaneous branch pulmonary artery interventions on exercise capacity, right ventricular function, and lung perfusion following PRISMA guidelines. We performed a systematic search in PubMed, Embase, and Cochrane including studies about right ventricular function, exercise capacity, and lung perfusion after percutaneous branch pulmonary artery interventions. Study selection, data extraction, and quality assessment were performed by two researchers independently. In total, 7 eligible studies with low (n = 2) and moderate (n = 5) risk of bias with in total 330 patients reported on right ventricular function (n = 1), exercise capacity (n = 2), and lung perfusion (n = 7). Exercise capacity and lung perfusion seem to improve after a percutaneous intervention for branch pulmonary artery stenosis. No conclusions about right ventricular function or remodelling, differences between balloon and stent angioplasty or specific CHD populations could be made. Although pulmonary artery interventions are frequently performed in biventricular CHD, data on relevant outcome parameters such as exercise capacity, lung perfusion, and right ventricular function are largely lacking. An increase in exercise capacity and improvement of lung perfusion to the affected lung has been described in case of mild to more severe pulmonary artery stenosis during relatively short follow-up. However, there is need for future studies to evaluate the effect of pulmonary artery interventions in various CHD populations.

To evaluate pulmonary vascular development and outcomes of complete correction following palliative treatment in infants with critical tetralogy of Fallot. This prospective, randomized, two-center study included infants with tetralogy of Fallot who underwent surgery between June 2018 and 2022. The patients were divided into two groups - those who underwent stenting of the right ventricular outflow tract (stent group, n=21) and those who underwent modified Blalock-Taussig shunt placement (shunt group, n=21). In the stent group, a significantly greater increase in Nakata index was observed, with mean values rising from 104.2 to 208.6 mm2/m2, compared to an increase from 107.3 to 169.4 mm2/m2 in the shunt group (P<0.01). According to the mixed model analysis, the rate of growth of the right pulmonary artery in the stent group was 2.05*10-2 z score/day, which was 3.01 times greater than that in the shunt group (P<0.01). The rate of growth of the left pulmonary artery in the stent group was 2.3*10-2 z score/day, which was 1.47 times greater than that in the shunt group (P<0.01). In one patient (4.8%), after 76 days following the stenting of the RVOT, a severe infectious process with sepsis occurred, leading to a fatal outcome. Complete correction in the stent group involved transannular patch repair of the right ventricular outflow tract to the pulmonary artery in 12 patients (60%), while the same procedure was performed in 15 patients (71.4%) in the shunt group (P=0.52). Stenting of the right ventricular outflow tract provides hemodynamic stabilization and symmetric growth of the pulmonary vascular bed compared to the formation of a modified Blalock-Taussig shunt.