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@#Objective To investigate the management of hepatic vein (HV) in patients with functional single ventricle (FSV) and separate hepatic venous drainage (SHVD) undergoing total cavopulmonary connection (TCPC) and evaluate this kind of surgery. Methods The clinical data of 32 consecutive patients with SHVD who underwent modified TCPC operation from August 2005 to January 2017 in our center were retrospectively analyzed. There were 25 males and 7 females with an average age of 2-19 (8.0±5.0) years and body weight of 11-66 (25.4±15.8) kg. Results There were 20 patients with heterotaxy syndrome and 12 patients with other types of FSV. SHVD was diagnosed preoperatively in 27 patients, among whom 20 patients were connected by intra-extracardiac Gore-Tex conduit, and the other 7 patients were connected by extracardiac Gore-Tex conduit. Because of the missed diagnosis of SHVD, the other 5 patients showed severe decrease of blood oxygen saturation in the early postoperative period and underwent re-operation soon. The postoperative blood oxygen saturation was 92.0% (90.0%, 96.0%), central venous pressure was 10-23 (15.5±3.5) mm Hg, mechanical ventilation assisted time was 16.0 (7.5, 24.0) h, and ICU stay time was 3.0 (2.0, 5.5) d. There were 3 early and 1 late deaths. Conclusion Intra-extracardiac conduit is an effective and feasible modified TCPC operation for patients with FSV and SHVD, while the surgical details need to be formulated in combination with individual anatomical structure. Preoperative missed diagnosis of SHVD must be avoided. Otherwise, after TCPC, a large amount of stealing blood from HV with low circulation pressure into atrium would lead to unacceptable hypoxemia.
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@#A 12-year-old girl with double outlet of right ventricle, complete atrioventricular septal defect, pulmonary valve stenosis, single atrium, inferior vena cava-hemiazygos vein drainage and left isomerism, who had undergone surgical left superior vena cava-pulmonary artery connection was referred to our hospital. Echocardiography revealed thickening, crimping and malalignment of common atrioventricular valve, moderate-to-severe regurgitation mainly from the left side and the main pulmonary artery was poorly developed. We preserved the autologous valve and used the 31# mechanical mitral valve which was sutured to mitral annulus. After the main pulmonary artery was transected, proximal end was sutured and a 20# Gore-tex artificial vessel was anastomosed to the distal end of the main pulmonary artery. We excised hepatic vein from the right atrium which was then anastomosed to another artificial vessel. Postoperative oxygen saturation was 95%-100%. Echocardiography and CT showed that the function of mechanical valve and heart was good before discharge. Common atrioventricular valvuloplasty is the preferred choice of the management of impaired valve. However, when dysfunction of valve is too severe and valvuloplasty is more likely to fail, the risk of barely performing a valvuloplasty will increase dramatically. On this condition, the mechanical valve replacement should be performed.
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@#Objective To analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations. Methods We retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation. Results The top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation. Conclusion Series operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.
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Objective To explore the value of MSCT in follow-up of functional single ventricle (FSV) after the Fontan palliation. Methods Data of MSCT and cardioangiography (CAG), as well as clinical information of 32 FSV patients after the Fontan procedures were analyzed retrospectively. All patients underwent MSCT examinations with individually manual triggered dual-phase scanning. Qualitative assessment of Fontan conduit structures and post-operative complications were performed. The diameters of pulmonary arteries and descending aorta at the level of diaphragm were measured to calculate McGoon index, in order to evaluate pulmonary artery development. The correlation between diameters of pulmonary artery and pressure of pulmonary artery in diastole phase, as well as between McGoon index and resistent index of pulmonary artery were analyzed. Results MSCT detected 3 cases of right atrio-pulmonary connection, 10 cases of total cavopulmonary connection, 11 cases of extracardiac Fontan and 8 cases of Y-graft Fontan conduits. Moreover, 8 cases of post-operative complications were detected with MSCT, including 2 cases of pulmonary arteriovenous malformations, 3 cases of aortopulmonary collaterals, 2 cases of mural thrombus in the Fontan pathway and 1 case of ventricular aneurysm. The diameters of left and right pulmonary artery measured on MSCT were (1.36±0.22) cm and (1.40±0.15) cm, both negatively correlated with the pressure of pulmonary artery in diastole phase (r=-0.755, -0.678, both P<0.001). McGoon index showed negative correlation with the resistent index of pulmonary artery (r=-0.676, P<0.001). Conclusion With advanced imaging post-processing technology and individual scanning scheme, MSCT can supply several important indicators in the follow-up for patients with FSV after Fontan palliation, showing important clinical value.
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@#Objective To explore the surgical management method and outcome of functional univentricular heart with total anomalous pulmonary vein drainage (UVH-TAPVD). Methods We reviewed the surgical procedures and results for 44 UVH-TAPVD patients in our hospital between the year 2010 and 2016. There were 34 males and 10 females. The age of the patients was 312 (77-4 220) d when they accepted the first surgical treatment. Results There were 8 deaths in stage Ⅰ palliation, 1 death in stage Ⅱ palliation and 5 deaths during the follow-up. The overall survival rate was 68.2% (30/44). Glenn operation was undertaken in 34 patients with 5 deaths. Fontan operation was undertaken in 9 patients with 2 deaths. Conclusion Surgical strategies for UVH-TAPVD should be planned according to different anatomical and pathophysiological conditions in different patients. Staged palliations can reduce mortality and morbidity. But pulmonary venous obstruction and heterotaxy syndrome are still risk factors for these patients.
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@#Objective To explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and Fontan procedure.
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Objective: To summarize the early- and med-term outcomes of modified Fontan operation in patients with functional single ventricle and separate hepatic venous (SHV) drainage. Methods: A total of 39 patients with functional single ventricle and SHV drainage received modified Fontan operation in our hospital from 2009-01 to 2015-12 were retrospectively analyzed. There were 26 (66.7%) male, the average age was (6.98±3.62) years, body weight was (20.79±9.66) kg and pre-operative mean pulmonary artery pressure was (9.77±2.51) mmHg. Based on if SHV combining Apicocaval Juxtaposition (ACJ), the patients were divided into 2 groups: SHV group,n=29 and SHV+CAJ group,n=10. The early (3 months) - and med (5 years)-term post-operative outcomes were analyzed. Results: General clinical data was similar between 2 groups,P>0.05. There were 20/39 patients with one-stage operation, 19 with two-stage operation (18 after Glenn operation and 1 after B-T operation). Modified Fontan operation method was mainly used extracardiac conduit-common open technique (30 patients, 76.9%). 19 patients had CPB with aorta clamping, CPB time was 72-446 (189.74±84.96) min and aorta clamping time was 26-171 (89.05±43.96) min; the other 19 patients had CPB without aorta clamping, CPB time was 60-209 (109.99±98.86) min. 10 patients received re-surgery due to early post-operative complications. 3 patients died at early post-operation with the mortality at 7.7%. The average follow-up time was 3.9 (0.83-7.17) years, 1 patient had cardiac function at NYHA II and the others at NYHA I; the 5-year survival rate was 100%. Conclusion: The early- and med-term outcomes of modified Fontan operation were good in patients with functional single ventricle and SHV drainage. Selection of modified Fontan operation method should be based on the distance form inferior vena cava to SHV and presence of ACJ.
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Objective To review the outcomes of atrioventricular valve replacement in patients with functional single ventricle and evaluate risk factors for mortality.Methods Retrospectively reviewed our experience with atrioventricular valve (AVV replacement) in patients with functional single ventricle from January 2008 to December 2014.Outcome included mortality and valve-related complications.Results Thirteen patients required AVV replacement.Prosthetic valve type was St.Jude bileaflet mechanical mitral valve.Valve size ranged from 27 to 31 mm,including 27 mm in 2,29 mm in 7,and 31 mm in 4 patients.Median age at valve replacement was 4.5 years(range:1.2 years to 18.0 years).Concomitant procedures included bidirectional Glenn shunt in 3,pulmonary artery banding in 1,and repair of pulmonary vein stenosis in 1 patient.There were four early deaths with a perioperative mortality of 30.8％.Complications after AAV replacement included complete atrioventricuiar block in 1,intracerebral hemorrhage in 1 patient due to valve-related anticoagulation.No patient had perivalvular leakage.There was one late death during a mean follow-up of 3 years (range:0.5-5.0 years).Five-year Kaplan-Meier survival was 61.5％.Fisher exact probability test showed that lower weight(＜ 10 kg) at operation and prosthetic size/weight ratio ＞ 2.0 were risk factors for overall mortality.Of the survival patients,functional status is NYHA class Ⅰ in 3,class Ⅱ in 5.No patient developed valve-related complications.Conclusion Atrioventricular valve replacement can be performed in patients with functional single ventricle with acceptable mortality.Lower weight and increased prosthetic size/weight ratio at operation were significantly associated with worse survival.