Iatrogenic or predetermined aortic dysplasia?

Coarctation of aorta post-transcatheter occlusion of patent ductus arteriosus is rare. We report a special case of infant with patent ductus arteriosus complicated by bicuspid aortic valve, who presented severe coarctation of aorta and aortic valve dysfunction post-patent ductus arteriosus occlusion during follow-up, eventually receiving surgical operations. A genetic rather than iatrogenic predisposition towards post-procedural complications has been discussed.

Meta-Analyses of Multiple Gene Expression Profiles to Screen Hub Genes Related to Osteoarthritis.

BACKGROUND: This study aimed to screen and validate the crucial genes involved in osteoarthritis (OA) and explore its potential molecular mechanisms. METHODS: Four expression profile datasets related to OA were downloaded from the Gene Expression Omnibus (GEO). The differentially expressed genes (DEGs) from 4 microarray patterns were identified by the meta-analysis method. The weighted gene co-expression network analysis (WGCNA) method was used to investigate stable modules most related to OA. In addition, a protein-protein interaction (PPI) network was built to explore hub genes in OA. Moreover, OA-related genes and pathways were retrieved from Comparative Toxicogenomics Database (CTD). RESULTS: A total of 1,136 DEGs were identified from 4 datasets. Based on these DEGs, WGCNA further explored 370 genes included in the 3 OA-related stable modules. A total of 10 hub genes were identified in the PPI network, including AKT1, CDC42, HLA-DQA2, TUBB, TWISTNB, GSK3B, FZD2, KLC1, GUSB, and RHOG. Besides, 5 pathways including "Lysosome," "Pathways in cancer," "Wnt signaling pathway," "ECM-receptor interaction" and "Focal adhesion" in CTD and enrichment analysis and 5 OA-related hub genes (including GSK3B, CDC42, AKT1, FZD2, and GUSB) were identified. CONCLUSION: In this study, the meta-analysis was used to screen the central genes associated with OA in a variety of gene expression profiles. Three OA-related modules (green, turquoise, and yellow) containing 370 genes were identified through WGCNA. It was discovered through the gene-pathway network that GSK3B, CDC42, AKT1, FZD2, and GUSB may be key genes related to the progress of OA and may become promising therapeutic targets.

Clinical outcomes of percutaneous or surgical closure of ruptured sinus of Valsalva aneurysm.

OBJECTIVE: To evaluate the clinical efficacy, safety, and long-term outcomes of percutaneous closure (PC) and surgical repair of ruptured sinus of Valsalva aneurysm (RSVA). METHODS: Eighty-five consecutive patients with RSVA were included in this study. Patients were considered candidates for PC if they met the criterion, surgical repair was performed on patients who were unsuitable or failed PC. Of them, 30 patients underwent PC, while the other 55 patients had surgical repair. RESULTS: RSVA was successfully occluded in 29 of 30 patients who were treated by PC. The mean narrowest diameter at the ruptured site was 6.45 ± 1.60 mm measured by aortography. One patient developed serious occluder-related aortic regurgitation and underwent surgery. The success rate of the interventional approach was 96.7%. In the surgical group, 23 patients underwent repair of combined RSVA and ventricular septal defect. The hospital mortality rate of the surgical approach was 3.57%. During a median follow-up of 83 months (8-152 months), the improvement in NYHA functional class in the PC group was significantly greater than those in the surgical group (P < .01). One patient died of infective endocarditis in the surgical group. There were no further serious complications. CONCLUSIONS: PC is a safe alternative to surgical repair for patients with isolated RSVA. Surgical repair is more suitable for those who have multiple cardiac lesions requiring surgical treatment or failed PC.

Simultaneous transcatheter therapy for perimembranous ventricular septal defect combined with patent ductus arteriosus.

OBJECTIVE: This study aims to assess the clinical efficiency and safety of simultaneous transcatheter interventional treatment for perimembranous ventricular septal defect (pmVSD) combined with patent ductus arteriosus (PDA). METHODS: Twenty-five patients with pmVSD and PDA treated with simultaneous transcatheter interventions from April 2004 to December 2015 were included in this study. The mean age was 9.80 ± 8.14 years and the mean weight was 29.76 ± 14.82 Kg. Transthoracic echocardiography (TTE) and angiography were performed immediately after the procedure. Patients were re-examined by electrocardiogram, X-ray, and TTE at 2 days, 1 month, 3 months, and 6 months postoperatively. RESULTS: The interventional procedure was successfully performed in all 25 patients. No intraoperative complication was noted. TTE examination of the VSD and PDA immediately after the procedure showed no residual shunt and the occluder was well positioned. Among these patients, four patients showed electrocardiogram changes after the procedure that resolved after drug therapy. The cardiothoracic ratio, left atrial diameter, left ventricular end-systolic diameter, and left ventricular end-diastolic diameter recovered to normal in most patients at 6 months postoperatively. CONCLUSIONS: Simultaneously transcatheter interventional therapy is a safe and effective method for pmVSD combined with PDA.

Effectiveness and Safety of Transcatheter Closure of Perimembranous Ventricular Septal Defects in Adults.

This study was designed to determine the long-term safety and efficacy of using modified double-disk occluders for perimembranous ventricular septal defect (pmVSD) closure in adults. From January 2004 to December 2014, 337 adults with pmVSDs were treated through transcatheter intervention using 2 types of double-disk occluders; 302 patients received a symmetrical concentric pmVSD occluder, and 35 patients received an asymmetrical concentric pmVSD occluder. All patients were followed up through electrocardiography and transthoracic echocardiography until June 2015. The success rate was 100% for both procedures. During the median 71-month follow-up period, no cases of infective endocarditis, cerebrovascular accidents, heart failure, or death occurred. Two major adverse events (0.6%) were recorded: complete atrioventricular block requiring surgical treatment in one patient and severe tricuspid valvular regurgitation requiring surgical repair in another patient. Cardiac conduction block was the most common minor adverse event. The mean left ventricular (LV) end-diastolic volume decreased from 96.6 ± 23.2 ml before intervention to 86.0 ± 22.0 ml (p <0.05) at the 6-month follow-up visit. Previously enlarged LV chambers decreased to normal sizes during the follow-up period. In conclusion, transcatheter closure of pmVSDs using modified double-disk occluders was both safe and effective and yielded excellent long-term results in adults. The potential benefits of this intervention included remodeling of the heart, a reduced incidence of infective endocarditis and prevention of LV volume overload.

Early- and intermediate-term results of surgical correction in 122 patients with total anomalous pulmonary venous connection and biventricular physiology.

BACKGROUND: We retrospectively reported our 26-year experience with operative repair of total anomalous pulmonary venous connection (TAPVC) with biventricular physiology. METHODS: Between December 1982 and December 2008, 122 TAPVC patients with biventricular heart underwent surgical repair in our department. Moderate or deep hypothermia was induced at the time of cardiopulmonary bypass (CPB). Follow-up was conducted for 5 postoperative years. Surgical outcomes of early and intermediate deaths after TAPVC repair were retrospectively analyzed. RESULTS: Six deaths occurred operatively; and three deaths, during follow-up. The 5-year survival rates after TAPVC repair was 92.6 %, without gradient across the anastomosis. The survival rate of the patients who were younger was 78.8 %, significantly lower than those older than 1 year. It was also lower in those who were less than 6 kg in weight. Three patients died during follow-up. Three patients died of ventricular arrhythmia, right heart failure, and pneumonia, respectively, during follow-up. If the left atrium pressure was higher than 15 mm Hg, the snare of the vertical vein was loosened after CPB ceased in the patients with supracardiac connection. It decreased from 21 ± 5 to 13 ± 3 mm Hg. The vertical vein was ligated in 57 cases and left open in 20 cases. A patient with an intact vertical vein had a large shunt and was cured by intervention afterward. Supraventricular arrhythmia occurred in 19 patients with the supercardiac type repaired through a biatrial incision. One patient died of ventricular arrhythmia, and none of the remaining patients had arrhythmias. CONCLUSION: Surgical treatment of TAPVC carried a low operative risk and had satisfactory immediate and intermediate results. Age younger than 1 year and weight less than 6 kg were risk factors. It was a good choice to leave the vertical vein open in the patients with a left atrial pressure higher than 15 mm Hg.

Trial occlusion to assess the risk of persistent pulmonary arterial hypertension after closure of a large patent ductus arteriosus in adolescents and adults with elevated pulmonary artery pressure.

BACKGROUND: No method is available to predict whether patients with patent ductus arteriosus (PDA) and severe pulmonary arterial hypertension (PAH) will show persistent postprocedural PAH (PP-PAH) after PDA closure. This study evaluated the usefulness of trial occlusion for predicting PP-PAH after transcatheter PDA closure in patients with severe PAH. METHODS AND RESULTS: Trial occlusion was performed in 137 patients (age ≥12 years) with PDA and severe PAH. All patients undergoing trial occlusion had a mean pulmonary artery pressure ≥45 mm Hg, pulmonary:systemic flow (Qp/Qs) ratio >1.5, and pulmonary:systemic resistance (Rp/Rs) ratio <0.7. A total of 135 patients (98%) showing stable hemodynamics during occlusion trial underwent successful device closure. Linear correlation analysis revealed weak or moderate relationships between the baseline and post-trial pulmonary artery pressures and pulmonary:systemic pressure (Pp/Ps) ratios. Patients were followed up for 1 to 10 years (median: 5 years). PP-PAH (systolic pulmonary artery pressure >50 mm Hg by Doppler echocardiography) was detected in 17 patients (13%), who displayed no significant differences in sex and age compared with patients without PP-PAH. According to discriminant analysis, the strongest discriminators between patients with and without PP-PAH were the baseline left ventricular end-diastolic volume and the baseline and post-trial systolic Pp/Ps ratios. In particular, a post-trial systolic Pp/Ps ratio >0.5 correctly classified 100% of the PP-PAH and non-PAH patients. CONCLUSIONS: Trial occlusion is a feasible method to predict PP-PAH in patients with PDA and severe PAH. A post-trial systolic Pp/Ps ratio >0.5 indicates a high risk of PP-PAH occurrence after device closure.

Long-term efficacy of transcatheter closure of ventricular septal defect in combination with percutaneous coronary intervention in patients with ventricular septal defect complicating acute myocardial infarction: a multicentre study.

AIMS: To assess the immediate and long-term outcomes of transcatheter closure of ventricular septal defect (VSD) in combination with percutaneous coronary intervention (PCI) in patients with VSD complicating acute myocardial infarction (AMI). METHODS AND RESULTS: Data were prospectively collected from 35 AMI patients who underwent attempted transcatheter VSD closure and PCI therapy in five high-volume heart centres. All the patients who survived the procedures were followed up by chest x-ray, electrocardiogram and echocardiography. Thirteen patients underwent urgent VSD closure in the acute phase (within two weeks from VSD) while the others underwent elective closure at a median of 23 days from VSD occurrence. The percentage of VSD closure device success was 92.3% (36/39) and procedure success was 91.4% (32/35). The incidence of in-hospital mortality was 14.3% (5/35). At a median of 53 months follow-up, only two patients died at 38 and 41 months, respectively, and other patients' cardiac function tested by echocardiography improved significantly compared to that evaluated before discharge. CONCLUSION: The combination of transcatheter VSD closure and PCI for treating VSD complicating AMI is safe and feasible and is a promising alternative to surgery in patients with anatomically suitable VSD and coronary lesion.

Acute hemodynamic responses to adenosine and iloprost in patients with congenital heart defects and severe pulmonary arterial hypertension.

BACKGROUND: Detection of pulmonary vasoreactivity is important for the evaluation of patient with pulmonary arterial hypertension (PAH). The present study aimed to investigate the acute hemodynamic responses to adenosine and iloprost in patients with congenital heart defects (CHDs) and severe PAH. PATIENTS AND METHODS: From Mar 2007 to Nov 2009, 75 patients with severe PAH secondary to left-to-right shunt CHDs underwent acute vasodilator test using aerosolized iloprost (n = 50) or intravenous adenosine (n = 25). The hemodynamics were detected and analyzed. RESULTS: Decreased mean pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) were observed in 39 and 43 patients in the iloprost group, and in 16 and 19 patients in the adenosine group, respectively. However, the mean PAP was higher than 40 mm Hg in both groups. No significant difference was observed in the age and baseline hemodynamics between the patients with the decrease of PVR and mean pulmonary-to-aortic pressure (Pp/Ps) ratio greater than 10% and the remaining patients. Adenosine decreased both PAP and systemic arterial pressure significantly, while iloprost inhalation selectively reduced the PAP and increased the oxygen saturation of femoral arterial blood and the pulmonary-to-systemic flow (Qp/Qs) ratio. Compared with adenosine, iloprost caused a more profound decline in the Pp/Ps ratio, PVR and pulmonary-to-systemic vascular resistance ratio, and increase in the Qp/Qs ratio. CONCLUSIONS: The acute haemodynamic responses to adenosine and iloprost varied among the patients with CHDs and severe PAH. Different to adenosine, inhaled iloprost exerted selective pulmonary vasodilative effects and was beneficial for pulmonary gas exchange.

Transcatheter closure of congenital coronary artery fistulae: immediate and long-term follow-up results.

BACKGROUND: Transcatheter closure of coronary artery fistulae (CAF) has emerged as an alternative to surgery, but there are no long-term outcome results. OBJECTIVE: We report immediate and long-term results in 24 patients after transcatheter closure of congenital CAF. METHODS: A total of 24 patients aged 5 to 56 years old with congenital CAF underwent attempted percutaneous transcatheter closure using various devices between November 1998 and August 2008. The immediate closure results and clinical follow-up were reviewed. RESULTS: Of the 24 patients, 20 (83.3%) underwent successful transcatheter closure using various devices. An angiogram right after device deployment revealed complete occlusion in 15 patients (75%) and trivial- to mild- residual flow in 5 patients (25%). Four patients (20%) had transient ST-T wave changes after the procedure. The left ventricular end-diastolic volume decreased from 165+/-31.4 mm3 to 128.6+/-24.4 mm3 (P = 0.012) 24 hours after procedure, and the cardiothoracic ratio from 0.57+/-0.02 to 0.53+/-0.01 (P = 0.003). Follow-up was 100% complete and ranged from 3 months to 10 years. There were no early or late deaths. All patients were asymptomatic with complete closure of CAF except 1 patient (5%) who had a recurrence of shunt at 6-month follow-up, which was re-closed by percutaneous technique. CONCLUSION: Transcatheter closure of CAF is feasible and safe in anatomically suitable vessels and is a promising alternative to surgery in most patients.

[Interventional therapy for structural heart diseases in People's Liberation Army hospitals between 2005 and 2006].

OBJECTIVE: To evaluate the efficacy and safety of interventional therapy in structural heart diseases in 38 PLA hospitals. METHODS: Data including patient number underwent interventional therapy for structural heart disease, procedure details, immediate procedural complications were retrospectively collected in all the military hospitals between January 2005 and December 2006. RESULTS: Successful interventional therapy was achieved in 8692 out of 8862 patients (98.08%) with structural heart disease. Transcatheter closure of ventricular septal defect (VSD), atrial septal defect (ASD) and patent ductus arteriosus (PDA), and percutaneous balloon dilatation of mitral valve stenosis (MS) and pulmonary valve stenosis (PS) were the most performed procedures (97.99%). Up to 91.23% patients underwent transcatheter closure with domestic devices. The incidence of procedure-related complications was 4.33% (n = 384) which were most frequently associated with VSD closure. The commonest procedural complications included conduction blockades (n = 260), residue shunt (n = 42), device detachment (n = 30) and tricuspid incompetence (n = 22). Although the procedures performed in 2005 and 2006 increased 57.32% compared with those in 2003 and 2004, the success rate and the incidence of complications remained unchanged. CONCLUSION: Percutaneous treatment of structural heart disease is a safe and feasible alternative to surgery. Simulate complications arise long after the treatment, which suggests the importance of long-term follow-up for those patients who hare undergone interventional therapy.

Transcatheter occlusion of the ruptured sinus of Valsalva aneurysm with an Amplatzer duct occluder.

BACKGROUND: Ruptured sinus of Valsalva aneurysm (RSVA) can be associated with ventricular septal defects or isolated lesions. Percutaneous transcatheter closure of RSVA has been an alternative strategy to surgery. The results of transcatheter closure of the RSVA in 10 patients were presented. METHODS: From January 2000 to May 2006, 10 patients (4 males, 6 females) aged from 7 years to 69 years (mean ages 37+/-18.8 years) were involved in the present report. The diagnosis of RSVA was made based on a combination of several imaging modalities. Of them, 9 patients were identified as congenital cause and one did as acquired RSVA. Two-dimensional and color Doppler echocardiography revealed the rupture of right coronary sinus into right ventricle in 5 cases and into right atrium in 3 cases, while non-coronary sinus ruptured into right atrium in 2 cases. Aortogram showed that the estimated size of the defect was 6.2+/-2.3 mm (2-10 mm). After the establishment of the arterio-venous wire loop, Amplatzer duct occluder (ADO) was deployed by antegrade venous approach in all patients. RESULTS: ADO with 1-3mm larger than the defect was used. All defects were successfully occluded without any complications. On the follow-up, echocardiography showed neither residual shunt nor aortic regurgitation, and there was also no device embolization, infective endocarditis in any of the patients. CONCLUSIONS: Transcatheter closure is a feasible and effective alternative for both congenital and acquired RSVA. However, long-term follow-up is mandatory.

[Radiofrequency perforation and balloon valvuloplasty in infants with pulmonary atresia and intact ventricular septum].

OBJECTIVE: To investigate the efficacy and safety of percutaneous radiofrequency perforation and valvuloplasty in infants with pulmonary atresia with intact ventricular septum (PA/IVS). METHODS: Four infants (body weight 4 - 10 kg) aged 11 months, 9 months, 12 days and 9 months old, respectively, were hospitalized for dyspnea and cyanosis. All patients had a continuous murmur in the left second intercostal space. Doppler echocardiogram showed membranous pulmonary atresia with intact ventricular septum. Right ventriculogram showed a tripartite right ventricle, vasiform infundibulum, and membranous pulmonary valve atresia without ventriculocoronary connections. Descending thoracic aortogram showed good-sized confluent pulmonary arteries being filled from a ductus arteriosus. All the patients were taken up for radiofrequency perforation followed by a balloon dilatation. A 6F Judkins right coronary guiding catheter was positioned in the right ventricular outflow tract and under the atretic pulmonary valve membrane. The radiofrequency perforation catheter along with coaxial injectable catheter was then passed through the right coronary guiding catheter, using it as the guide to the imperforate membrane. The proximal end of the radiofrequency perforation catheter was then connected to radiofrequency generator. After the cusps of pulmonary valve were perforated, the coaxial injectable catheter was moved into the main pulmonary artery. A tiny floppy-tipped coronary guidewire was then passed through the coaxial injectable catheter into the main pulmonary artery and directed through the patent ductus arteriosus into the descending thoracic aorta or directed into pulmonary arteriola. Thereafter, serial balloon dilation catheters were introduced across the pulmonary valve, and dilations were sequentially performed with increasing balloon diameters. The balloon was dilated until the concave of the balloons disappeared. The radiofrequency energy (5 to 8 W) was delivered for 2 to 5 seconds once, but commonly twice, to perforate the valves. After a predilation with a 3 mm x 20 mm to 5 mm x 20 mm balloon at 6 - 14 atm pressure, the valve was subsequently dilated with 10 mm x 30 mm to 14 mm x 30 mm balloon once or twice. The duration of procedures was 120 to 150 min and exposure time was 25.4 to 43.9 min. RESULTS: The primary procedure was successful in all the infants except one who died early of cardiac perforation with tamponade. After a follow-up period ranging from 2 to 8 months (mean 4.3 m), the remaining 3 survivors achieved complete biventricular circulation. Two of them were awaiting occlusion of the patent ductus arteriosus and 1 needed right ventricular outflow tract reconstruction because of infundibular obstruction. CONCLUSION: PA/IVS consists of 0.7% to 3.1% of congenital heart defects. 85% of the untreated patients die within half a year. Surgical repair for the infants with PA/IVS is associated with a high mortality. In carefully selected patients with PA/IVS, radiofrequency perforation and balloon dilatation of the pulmonary valve is feasible and may represent a new alternative to surgery due to its low mortality and avoidance of cardiopulmonary bypass.

Transplantation of autologous adipose-derived stem cells ameliorates cardiac function in rabbits with myocardial infarction.

BACKGROUND: Adipose-derived stem cells (ADSCs) are capable of differentiating into cardiomyogenic and endothelial cells in vitro. We tested the hypothesis that transplantation of ADSCs into myocardial scar may regenerate infracted myocardium and restore cardiac function. METHODS: ADSCs were isolated from the fatty tissue of New Zealand white rabbits and cultured in Iscoves modified dulbeccos medium. Three weeks after ligation of left anterior descending coronary artery of rabbits, either a graft of untreated ADSCs (UASCs, n = 14), 5-azacytidine-pretreated ADSCs (AASCs, n = 13), or phosphate buffer saline (n = 13) were injected into the infarct region. Transmural scar size, cardiac function, and immunohistochemistry were performed 5 weeks after cell transplantation. RESULTS: ADSCs in culture demonstrated a fibroblast-like appearance and expressed CD29, CD44 and CD105. Five weeks after cell transplantation, transmural scar size in AASC-implanted hearts was smaller than that of the other hearts. Many ADSCs were differentiated into cardiomyocytes. The AASCs in the prescar appeared more myotube-like. AASCs in the middle of the scar and UASCs, in contrast, were poorly differentiated. Some ADSCs were differentiated into endothelial cells and participate in vessel-like structures formation. All the ADSC-implanted hearts had a greater capillary density in the infarct region than did the control hearts. Statistical analyses revealed significant improvement in left ventricular ejection fraction, myocardial performance index, end-diastolic pressure, and peak +dP/dt, in two groups of ADSC-implanted hearts relative to the control hearts. AASC-implanted hearts had higher peak -dP/dt values than did control, higher ejection fraction and peak +dP/dt values than did UASC-implanted hearts. CONCLUSIONS: ADSCs transplanted into the myocardial scar tissue formed cardiac islands and vessel-like structures, induced angiogenesis and improved cardiac function. 5-Azacytidine pretreatment before implantation is desirable for augmenting myogenesis. Transplantation of 5-azacytidine-treated ADSCs into the myocardial scar was more efficient than that of untreated ADSCs in preservation of cardiac function.