Pulmonary hypertension (PH), characterized by diverse etiologies and intricate pathological mechanisms, is a complex cardiopulmonary vascular disorder featuring high morbidity and mortality. Percutaneous pulmonary artery denervation (PADN) represents an emerging interventional treatment method, which shows good prospects in the clinical practice of PH. The PADN has attained preliminary achievements in terms of safety and efficacy. Nevertheless, its long-term prognosis, the characteristics of the appropriate patient populations, and the optimization strategies combined with targeted pharmacotherapy remain to be further explored. This article reviews the current clinical applications of PADN as well as the challenges it confronts.
ObjectiveTo compare early clinical outcomes between systemic-pulmonary shunts (SPS) and right ventricular to pulmonary artery connection (RV-PA connection) for patients with pulmonary atresia and ventricular septal defect (PA/VSD), and investigate early management strategies for these 2 different palliative procedures. MethodsWe retrospectively analyzed clinical data of 89 PA/VSD patients who underwent SPS or RV-PA connection in Fu Wai Hospital from January 2009 to December 2011. According to different surgical procedures, all the 89 patients were divided into 2 groups. In SPS group, there were 59 patients including 35 males and 24 females with their median age of 25 months (4 months to 8 years). In RV-PA connection group, there were 30 patients including 19 males and 11 females with their median age of 24 months (28 days to 7 years and 2 months). Early clinical outcomes including mechanical ventilation time, length of ICU stay, morbidity, reexploration, improvement of oxygen saturation (SO2) and mortality were compared between the 2 groups. ResultsAmong 59 patients in SPS group, 3 patients (5.1%) died postoperatively. There was no in-hospital death among 30 patients in RV-PA connection group. The improvement of percentage of SO2 of RV-PA connection group was significantly higher than that of SPS group (31.7% vs. 22.2%, P < 0.05). There was no statistical difference in length of ICU stay (3.6±2.5 days vs. 4.2±5.1 days, P > 0.05), mechanical ventilation time (34.8±33.5 hours vs. 44.3±39.6 hours, P > 0.05), postoperative morbidity (37.3% vs. 30.0%, P > 0.05) or reexploration rate (15.3% vs. 6.7%, P > 0.05) between SPS group and RV-PA connection group. Incidence of serious postoperative complications of SPS group was signi-ficantly higher than that of RV-PA connection group (25.4% vs. 6.7%, P < 0.05). ConclusionEarly clinical outcomes of RV-PA connection is better than SPS for PA/VSD patients including greater SO2 improvement and lower mortality. Mid-term and long-term clinical results as well as larger study samples are needed for better evaluation.
ObjectiveTo compare the benefits and drawbacks of primary patch expansion versus pericardial tube right ventricular-pulmonary artery connection in patients diagnosed with pulmonary atresia with ventricular septal defect (PA/VSD). MethodsA retrospective study was conducted on patients diagnosed with PA/VSD who underwent primary right ventricular-pulmonary artery connection surgery at our center between 2010 and 2020. Patients were categorized into two groups based on the type of right ventricular-pulmonary artery connection: a pericardial tube group and a patch expansion group. Clinical data and imaging findings were compared between the two groups. ResultsA total of 51 patients were included in the study, comprising 31 males and 20 females, with a median age of 12.57 (4.57, 49.67) months. The pericardial tube group included 19 patients with a median age of 17.17 (7.33, 49.67) months, while the patch expansion group consisted of 32 patients with a median age of 8.58 (3.57, 52.72) months. In both groups, the diameter of pulmonary artery, McGoon index, and Nakata index significantly increased after treatment (P<0.001). However, the pericardial tube group exhibited a longer extracorporeal circulation time (P<0.001). The reoperation rate was notably high, with 74.51% of patients requiring further surgical intervention, including 26 (81.25%) patients in the patch expansion group and 12 (63.16%) patients in the pericardial tube group. No statistical differences were observed in long-term cure rates or mortality between the two groups (P＞0.005). Conclusion In patients with PA/VSD, both patch expansion and pericardial tube right ventricular-pulmonary artery connection serve as effective initial palliative treatment strategies that promote pulmonary vessel development and provide a favorable foundation for subsequent radical operations. However, compared to the pericardial tube approach, the patch expansion technique is simpler to perform and preserves some intrinsic potential for pulmonary artery development, making it the preferred procedure.
Abstract： Objective To summarize our experience of surgical treatment for anomalous origin of one pulmonary artery in infants and children. Methods From March 2005 to May 2010，11 patients with anomalous origin of one pulmonary artery and other concomitant congenital cardiovascular malformations underwent surgical repair in Xijing Hospital of Fourth Military Medical University．The mean age of the patients was 11.5 months with a range from 2 months to 36 months．Their mean body weight was 7.1 kg with a range from 4 to 13 kg. Seven patients had anomalous origin of the right pulmonary artery from the ascending aorta, and four patients had anomalous origin of the left pulmonary artery from the ascending aorta. All the eleven patients had other concomitant intracardiac anomalies or vascular malformations as well as pulmonary hypertension, and underwent one stage surgical repair via median sternotomy under hypothermia and cardiopulmonary bypass. Results Their operation time was 169 - 293 （231±55） min, cardiopulmonary bypass time was 87-210 （138±47） min, and aortic-clamping time was 45-133 （86±28） min. There was one postoperative death who had low cardiac output syndrome after repair for tetralogy of Fallot and anomalous origin of the right pulmonary artery. The overall postoperative mortality was 9.1％. Postoperative echocardiography of all the surviving patients showed their left and right pulmonary artery origined from the right ventricle and pulmonary artery with satisfactory malformation correction but no residual shunt and pulmonary stenosis . All the surviving ten patients were followed up with a follow-up rate of 100% and mean follow-up time of 13.5 months with a range from 3 to 32 months. Their echocardiography during follow-up showed that there was no pulmonary stenosis in all the patients, and pulmonary blood pressure significantly decreased in 9 patients. Conclusion Patients with anomalous origin of one pulmonary artery should undergo surgical repair as early as possible with satisfactory short-term outcomes in infants and children. For elder patients with irreversible pulmonary hypertension, the choice of surgical treatment should be more cautious. During the surgery, the anomalous pulmonary artery and ascending aorta should be dissociated fully, and transection of the ascending aorta is helpful to get a satisfactory operating field view for the surgeon. Repairing aortic defect with autologous pulmonary arterial patch can effectively avoid the occurrence of postoperative aortic aneurysm.
Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation resulting from the failed development or premature involution of the sixth aortic arch during embryogenesis, leading to a failure to establish a connection with the main pulmonary artery. Currently, there is a notable lack of consensus regarding the surgical management of UAPA in China. Drawing upon the latest clinical research, this consensus aims to summarize surgical approaches and techniques to improve the clinical management of UAPA patients and serve as a scientific reference for physicians specializing in pediatric cardiology and structural heart disease. This consensus aims to promote the standardization of UAPA diagnosis and treatment, thereby facilitating improved patient outcomes and long-term management, and stimulating the continuous development and innovation of surgical treatment for this condition in China.
ObjectiveTo analyze the early outcomes of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) patients with severe left ventricular dysfunction after surgical repair, and to explore the predictors for extracorporeal membrane oxygenation (ECMO) support for these patients.MethodsThe clinical data of ALCAPA patients with severe left ventricular dysfunction (left ventricular ejection fraction<40%) who underwent coronary artery reimplantation in the pediatric center of our hospital from 2013 to 2020 were retrospectively analyzed. The patients were divided into an ECMO group and a non-ECMO group. Clinical data of the two groups were compared and analyzed.ResultsA total of 64 ALCAPA patients were included. There were 7 patients in the ECMO group, including 4 males and 3 females aged 6.58±1.84 months. There were 57 pateints in the non-ECMO group, including 30 males and 27 females aged 4.34±2.56 months. The mortality of the patients was 6.25% (4/64), including 2 patients in the ECMO group, and 2 in the non-ECMO group. The postoperative complications rate was significantly higher in the ECMO group than that in the non-ECMO group (P=0.041). There were statistical differences in the cardiopulmonary bypass time [254 (153, 417) min vs. 106 (51, 192) min, P=0.013], aortic cross-clamping (ACC) time (89.57±13.66 min vs. 61.58±19.57 min, P=0.039), and preoperative left ventricular end-diastolic diameter/body surface area (132.32±14.71 mm/m2 vs. 108.00±29.64 mm/m2, P=0.040) between the two groups. Multivariate logistic regression analysis showed that ACC time was an independent risk factor for postoperative ECMO support (P=0.005). Receiver operating characteristic (ROC) curve analysis showed that the area under the ROC curve was 0.757, the sensitivity was 85.70%, specificity was 66.70%, with the cut-off value of 66 min.ConclusionACC time is an independent risk factor for postoperative ECMO support. Patients with an ACC time>66 min have a significantly higher risk for ECMO support after the surgery.
Objective Comparing postoperative change of blood gas and hemodynamic status in patients underwent a right ventricletopulmonary artery (RVPA) conduit or a modified BlalockTaussig (mBT) shunt for pulmonary atresia with ventricular septal defect and without major arterial pulmonary collaterals (MAPCAs), to affirm the effect on oxygen supply /demand with different procedure. Methods From July 2006 to October 2007, 38 patients with pulmonary atresia and ventricular septal defect without MAPCAs were divided into two groups according to different procedures: RVPA group (n=25) and mBT group (n=13).Perioperative mortality, blood gas and hemodynamic data during postoperative 48 hours, including heart rate, blood pressure, systemic oxygen saturation, mixed venous oxygen saturation, oxygen excess factor, inotropic score were compared in both groups. Results The difference in the mortality between RVPA group (4.0％，1/25) and mBT group （7.7％，1/13) showed no statistical significance（Pgt;0.05）. The total of 33 patients were followed up, the followup time was from 6 to 18 months.11 patients (4 patients in mBT group, 7 patients in RVPA group) underwent corrected procedures during 9 to 18 months after palliative procedures, one case died of elevated pulmonary vascular resistance and right ventricle failure. The mixed venous oxygen saturation at 24h and 48h after surgery were higher than that at 6h after surgery (Plt;0.01) both in RVPA group and mBT group. The systolic blood pressures at 6h, 24h, 48h after surgery in RVPA group were lower than those in mBT group (P=0.048，0.043， 0.045)，the mean systemic blood pressures in RVPA group were higher than those in mBT group (P=0.048, 0.046, 0.049),the diastolic blood pressures in RVPA group were higher than those in mBT group (P=0.038, 0.034, 0.040)， the inotropic scores in RVPA group were lower than those in mBT group (P=0.035， 0.032，0.047). Conclusion The blood pressures and inotropic scores are found significantly different in RVPA conduit and mBT procedures, while postoperative systemic oxygen delivery areequivalent. Both RVPA and mBT patients decline to nadir in hemodynamic status at 6 h after surgery.
Objective To review the experience of the surgical treatment of adult patients with anomalous left coronary artery from the pulmonary artery (ALCAPA). Methods A retrospective, single institution review was conducted on thirty-six adult patients with ALCAPA surgical treatment from November 1991 to November 2017 in Fuwai Hospital. Of these patients, nine were males and twenty-seven were females. The mean age was 36.6±13.3 years. The mean weight was 60.0±9.4 kg. The preoperative echocardiography showed the mean left ventricular ejection fraction (LVEF) was 57%±6% and the mean left ventricular end-diastolic dimension (LVEDD) was 52.3±6.3 mm. Severe mitral regurgitation (MR) was seen in one patient, moderate in five patients. The operative procedures included coronary artery re-implantation in seventeen patients, Takeuchi operation in sixteen patients, ligation of left coronary artery plus coronary artery bypass graft in three patients. In addition, six patients underwent mitral valve repair. Results There was no in-hospital mortality. The mean cardiopulmonary bypass time was 152.5±72.9 min and aortic cross clamp time was 101.9±43.6 min, respectively. The mean mechanical ventilation time and ICU time was 17.3±16.3 h and 43.1±30.7 h, respectively. The mean postoperative LVEF was 59%±6%, which did not significantly improve compared with preoperative LVEF. However, the mean postoperative LVEDD of 46.9±5.9 mm had significant reduction compared with the preoperative LVEDD. Of the six patients with mitral valve repair, one was mild and the other five were trivial. Thirty-five patients (97.2%) completed the follow-up with a mean time of 5.5 years. All the patients survived with New York Heart Association class Ⅰor Ⅱ. Two patients needed interventional occlusion or re-operation due to the fistula of internal tunnel within the pulmonary artery. At the latest echocardiography, the mean LVEF of 69%±7% improved significantly compared with the preoperative LVEF. Mild MR was detected in ten patients, moderate in two patients during the follow-up period. Conclusion The surgical treatment of adult patients with ALCAPA has satisfactory short- and long-term results. The patients who underwent Takeuchi procedure may need re-operation due to fistula of internal tunnel within the pulmonary artery during the long-term follow-up.
Objective To confirm the changes of pulmonary artery pressure, neo pulmonary artery stenosis and reoperation in children with unilateral absence of pulmonary artery (UAPA) undergoing pulmonary artery reconstruction. Methods The clinical data of the infants with UAPA undergoing pulmonary artery reconstruction in our hospital from February 19, 2019 to April 15, 2021 were analyzed. Changes in pulmonary artery pressure, neo pulmonary artery stenosis and reoperation were followed up. Results Finally 5 patients were collected, including 4 males and 1 female. The operation age ranged from 13 days to 2.7 years. Cardiac contrast-enhanced CT scans were performed in all children, and 2 patients underwent pulmonary vein wedge angiography to confirm the diagnosis and preoperative evaluation. Preoperative transthoracic echocardiography and intraoperative direct pulmonary arterial pressure measurement indicated that all 5 children had pulmonary hypertension, with a mean pulmonary arterial pressure of 31.3±16.0 mm Hg. Pulmonary arterial pressure decreased immediately after pulmonary artery reconstruction to 16.8±4.2 mm Hg. The mean follow-up time was 18.9±4.7 months. All 5 patients survived during the follow-up period, and 1 patient had neo pulmonary artery stenosis or even occlusion and was re-operated. Conclusion Pulmonary artery reconstruction can effectively alleviate the pulmonary hypertension in children with UAPA. The patency of the neo pulmonary artery should be closely followed up after surgery, and re-pulmonary angioplasty should be performed if necessary.
ObjectiveTo review the experience of the surgical treatment of child patients with anomalous left coronary artery from the pulmonary artery (ALCAPA). MethodsWe retrospectively analyzed the clinical data of 56 children patients with ALCAPA underwent coronary re-implantation in our hospital from April 2004 through February 2015. There were 35 males and 21 females at mean age of 25.5 (7.3-60.0) months. Nineteen patients (33.9%) were less than 1 year of age. The mean weight was 11.8 (7.8-19.8) kg. ResultsThere was one death in-hospital. The mean cardiopulmonary bypass time and cross-clamp time was 131.8± 61.2 min and 83.4± 32.1min, respectively. The mean mechanical ventilation time and intensive care unit time was 12.5 (6.5-43.8) h and 49.0 (21.0-116.0) h, respectively. Three patients underwent extracorporeal membrane oxygenation (ECMO) support and weaned off successfully. The mean postoperative left ventricular ejection fraction (LVEF, 63.4%± 15.8% vs. 50.6%± 18.7%) and left ventricular end diastolic diameter (LVEDD, 36.4± 32.5 mm vs. 42.3± 7.4 mm) significantly improved compared postoperative (P < 0.05). The mitral regurgitation (MR) distribution in the 15 patients underwent mitral valve repair was:moderate in 2 patients, mild in 8 patients, trivial in 2 patients and none in 3 patients. The MR in the other 41 patients improved or did not change. The survivors completed the follow-up for a mean time of 45.4± 23.6 months. During the follow-up period, one patient died due to noncardiac reason. No patient required reoperation or readmission. All the patients survived with New York Heart Association heart function classⅠor classⅡ. At the latest echocardiography, the mean LVEF (62.8%± 5.0%) significantly improved compared with the LVEF of discharge. The MR distribution was moderate in 6 patients, mild in 24 patients, trivial in 4 patients and none in 21 patients. ConclusionThe coronary re-implantation has a satisfactory mid-term result for patients with ALCAPA. Mitral valve repair is recommended for the patients with severe regurgitation and evident ischemic lesions of the papillary muscles.