Corrigendum: Use of the index of pulmonary vascular disease for predicting longterm outcome of pulmonary arterial hypertension associated with congenital heart disease.

[This corrects the article DOI: 10.3389/fcvm.2023.1212882.].

Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease.

Aims: Limited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH. Methods: This retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death. Results: The 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45-13.73; P = .009). Conclusions: The IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered.

Delayed migration due to shortening of the lower part of AFX endograft's main body in angled fusiform abdominal aortic aneurysm.

We encountered two fusiform abdominal aortic aneurysm cases with delayed AFX endograft (Endologix Inc) migration >4 years after placement. These cases showed shortening and slight angulation of the main body in the anteroposterior direction. We speculate that the potential mechanism relates to the AFX portion that is easily shortened at the bifurcation of its stent structure. This portion might contribute to delayed migration after slight angulation of the main body. Preoperative three-dimensional computed tomography should be performed from the anteroposterior and lateral views. Although the AFX is useful for narrow bifurcations, one should consider the patient's anatomy before deciding to use an AFX endograft.

[Unroofing Technique on Anomalous Aortic Origin of a Left Coronary Artery;Report of a Case].

An anomalous aortic origin of coronary artery is a cause of sudden death in young people. We report a surgical case of an anomalous aortic origin of the left coronary artery. A 16-year-old man with chest pain, which occurred while playing football, was taken in an ambulance. Electrocardiogram (ECG) revealed an ST-segment elevation in V1-V5. We performed coronary angiogram (CAG) and detected the anomalous aortic origin of left anterior descending artery. The computer tomography( CT) angiography revealed the intramural pathway of the left coronary artery. Surgery performed as an unroofing procedure for releasing the intramural portion of the left coronary artery. We confirmed a dilation of the left coronary artery ostia in postoperative coronary CT angiography. He did not have chest pain and other symptoms while cardiopulmonary exercise testing. He was discharged on the 18th postoperative day. He is seen as an outpatient without any symptoms.

Modified cuffed anastomosis technique to treat pseudoaneurysms following thoracic endovascular aortic repair.

Pseudoaneurysm after thoracic endovascular aortic repair (TEVAR) is very rare. We report a case of thoracic aortic pseudoaneurysms due to flares at the proximal end of a stent graft after TEVAR for ductal aneurysm. We describe a total aortic arch replacement in this case using a modified cuffed anastomosis technique with an elephant trunk procedure leaving the partial stent graft in situ.

[Ebstein's anomaly in an adult].

A 56-year-old female who had been diagnosed with Ebstein's anomaly was admitted with cyanosis and congestive heart failure. The echocardiogram showed severe tricuspid valve incompetence, displacement of the tricuspid valve and dilatation of the atrialized portion of the right ventricle. Atrial fibrillation was detected in the electrocardiogram. She underwent tricuspid valve replacement and right atrial maze procedure. She is released from congestive heart failure and remains in sinus rhythm 48 months after the operation.

[Distal aortic repair after aortic arch replacement].

BACKGROUND: It is crucial to expose the proximal aorta in distal aortic repair, i.e., replacement of the descending thoracic aorta (DTA) or the thoracoabdominal aorta (TAA), after aortic arch replacement. With the elephant trunk (ET), it is usually easy to expose and clamp it. On the other hand, without the ET, it may be difficult or impossible to expose the proximal aorta and deep hypothermic circulatory arrest (DHCA) will be required. METHODS: Between April 1989 and March 2007, 17 patients underwent distal aortic repair after aortic arch replacement. Five patients underwent replacement of DTA and 12 of TAA. Five patients without the ET needed DHCA and open proximal anastomosis [OP (+) group], while in 12 patients, the ET or proximal aorta was successfully clamped [OP (-) group]. RESULTS: The mean extracorporeal circulation time in OP (+) group was significantly longer than that in OP (-) group (415 +/- 131 min v.s. 267 +/- 109 min, p < 0.05). There was no hospital death, cerebral infarction, fatal arrhythmia or low output syndrome in either group, and paraplegia in 2 patients and renal failure requiring hemodialysis in one were found only in OP (+) group. CONCLUSION: The ET procedure enables to avoid DHCA and may contribute to improving operative results in distal aortic repair after aortic arch replacement.

Successful management of esophagoparaprosthetic fistula after aortic surgery.

Aortoesophageal fistula is a relatively rare but highly fatal condition, especially in the case of secondary aortoesophageal fistulas after previous thoracic aortic surgery in which the aortic prosthetic graft itself may be involved in the infection, resulting in an esophagoparaprosthetic fistula. In this report, we describe a complicated case of esophagoparaprosthetic fistula arising after descending thoracic aortic replacement and endovascular pseudoaneurysm repair that was successfully treated by surgical resection and in situ aortic graft replacement using a homograft completely covered with an omental flap, combined with subtotal esophagectomy and staged reconstruction of the alimentary tract. The patient has been doing well for 24 months without signs of recurrent infection.

Surgical management of aortic regurgitation associated with takayasu arteritis and other forms of aortitis.

BACKGROUND: Surgical management of aortic regurgitation associated with aortitis can be complicated with occurrence of prosthetic valve detachment or formation of pseudoaneurysm at the suture line. Postoperative morbidity includes progressive dilatation of the aortic root. We sought to assess our midterm and long-term results of surgical management of aortic regurgitation associated with aortitis. METHODS: Between January 1989 and September 2004, 15 patients (17 cases) with aortic regurgitation caused by aortitis were surgically treated. Of 17 cases, 6 cases had aortic valve replacement and 11 cases had aortic root replacement. All the patients were followed up from 3 months to 15 years with a mean follow-up period of 70.8 +/- 54.9 months. RESULTS: There was no hospital death in all the procedures. During the follow-up period, 1 patient died of prosthetic valve detachment, and 2 patients required aortic root replacement for prosthetic valve detachment or aortic root dilatation after aortic valve replacement, whereas there was neither cardiac death nor reoperation after aortic root replacement. CONCLUSIONS: Low operative mortality and favorable long-term outcome may justify lowering the threshold toward aortic root replacement for aortic regurgitation with aortitis in view of the propensity for development of prosthetic valve detachment.

External application of rapamycin-eluting film at anastomotic sites inhibits neointimal hyperplasia in a canine model.

BACKGROUND: Stenosis at a vascular anastomotic site has been a significant clinical issue. We tested the hypothesis that rapamycin-eluting biodegradable poly L-lactic acid and epsilon-caprolactone copolymer (PLA-CL) film applied externally can inhibit neointimal hyperplasia in a canine vascular anastomosis model. METHODS: Femoral artery graft interposition was performed in 25 beagles. Beagles were divided into five groups (five in each): graft interposition without PLA-CL film (control); with PLA-CL film only; and PLA-CL containing rapamycin 8 microg, 80 microg, and 800 microg. Orthotopic arterial graft interposition was performed on the left side and vein graft from the ipsilateral femoral vein was interposed on the right. Morphometric and immunochemical analyses were performed at four-week intervals. RESULTS: In arterial graft models, the ratio of intimal area (intimal area divided by the entire vessel area) was significantly reduced in all the three rapamycin-eluting film groups compared with control (0.19, 0.07, 0.05, and 0.38 in 8 microg, 80 microg, 800 microg groups and control, respectively, p < 0.05). In vein graft models, the ratio of intimal area was significantly decreased only in the 800 microg rapamycin group compared with control (0.33 vs 0.54, p < 0.05). Inhibition of neointimal growth was associated with reduced cell proliferation, as evidenced by proliferating cell nuclear antigen immunostaining and diminished alpha-actin positive vascular smooth muscle cells. CONCLUSIONS: Rapamycin-eluting biodegradable PLA-CL film applied externally can inhibit neointimal hyperplasia of arterial and vein grafts in a canine model. The inhibitory effect of rapamycin-eluting film against neointimal growth is more pronounced in the arterial graft than the vein graft.

Redo total aortic arch replacement using an extended homograft for graft infection.

A 65-year-old man developed graft infection 3 years after total aortic arch replacement for a chronic dissecting aneurysm. Redo total aortic arch replacement was performed using an extended homograft following 2 days of open drainage of the mediastinum. The patient has been free from a recurrent mediastinitis over the last 3 years.

The wrapping method using biodegradable felt strips has a preventive effect on the thinning of the aortic wall: experimental study in the canine aorta.

OBJECTIVES: Wrapping methods have been widely used to reinforce the anastomotic site in vascular surgery; however, postoperative changes in the aortic wall wrapped by nonbiodegradable felt have not been well characterized. The purposes of this investigation are to elucidate the sequelae of wrapping with nonbiodegradable felt on the aortic wall and to modify those changes by using biodegradable felt with or without basic fibroblast growth factor (bFGF). METHODS: The descending thoracic aortas of 15 beagles were wrapped with three different materials: nonbiodegradable polytetrafluoroethylene (PTFE) felt, biodegradable polyglycol acid (PGA) material, and PGA with 100 microg bFGF (n = 5 in each group). The descending thoracic aorta was resected after 3 months. The thickness of the aortic wall, vessel density in the media and the adventitia, and the wall strength were assessed. Untreated native aortic wall served as a normal control. RESULTS: The thickness of the media of the PTFE group was lower than that of the PGA + bFGF group (66% +/- 5% vs 85% +/- 6% of control, P < .05). The adventitia-media ratio in the PTFE group decreased compared with controls (59.1% of normal, P < 0.05), whereas those in the PGA and PGA + bFGF groups increased (172.1% and 189.6% of normal, respectively, P < .01). The collagen-smooth muscle ratio in the media was higher in the PTFE group than in the controls (0.14 +/- 0.02 vs 0.07 +/- 0.01, P < .01). The number of vessels in the adventitia was higher in the PGA + bFGF group than those in PTFE or PGA groups (29.6 +/- 2.5/mm2 vs 6.4 +/- 0.8/mm2, 19.0 +/- 1.1/mm2, P < .01). The PGA + bFGF group demonstrated larger failure force than the PTFE group (4.0 +/- 0.3 kgf vs 1.6 +/- 0.3 kgf, P < .01). The failure stress in the PGA and PGA + bFGF groups was larger than that in PTFE group (PTFE:PGA + bFGF = 5.3 +/- 0.9 x10(2) kPa:11.7 +/- 1.7 x 10(2) kPa, P < .01; PTFE:PGA = 5.3 +/- 0.9 x 10(2) kPa:11.2 +/- 1.2 x 10(2) kPa, P < .05). CONCLUSION: The aortic wall wrapped with nonbiodegradable PTFE felt showed a reduced thickness and diminished vessels in the adventitia. Biodegradable felt (PGA), with or without bFGF, modified these histologic changes. The vessel-rich thickened adventitia, after wrapping by PGA with bFGF, was associated with increased aortic wall strength. CLINICAL RELEVANCE: This investigation was conducted in an attempt to elucidate mechanisms underlying the occurrence of late postoperative false aneurysm after aortic surgery. We hypothesized that sustaining compression of the aorta by the felt strip may cause structural derangement and local ischemia on the aortic wall. We used a simple wrapping of the aorta with a felt strip rather than a felt strip at anastomotic sites to simplify the experimental model and to exclude confounding factors brought by technical inconsistency on the surgical anastomosis. We further attempted to find a clue for preventing adverse effects of wrapping with a conventional felt strip. Practically, we pursued a possible application of a biodegradable felt strip to aortic wrapping in our experimental model before we proceed in a clinical application of the new material.

Successful treatment of intraoperative heart failure caused by ampulla cardiomyopathy by intra-aortic balloon pumping and percutaneous cardiopulmonary support: report of a case.

An 82-year-old woman underwent total gastrectomy for advanced gastric cancer with invasion to the lower esophagus. Her blood pressure dropped alarmingly during the operation, which was performed via the transabdominal and left-side transthoracic approach. Using echocardiography, we diagnosed intraoperative-onset reversible heart failure caused by ampulla cardiomyopathy. Because the infusion of catecholamines is associated with secondary heart failure, we gave her calcium antagonists and nicorandil, then started intra-aortic balloon pumping (IABP) and the percutaneous cardiopulmonary support system (PCPS). On postoperative day (POD) 7, the IABP and PCPS were removed and on POD 12, she was extubated successfully. The patient was discharged on POD 54 and has remained well. The factors predisposing her to ampulla cardiomyopathy were left-side thoracotomy, hypoxia caused by one-lung ventilation, and the infusion of high-dose catecholamines. Prompt diagnosis and timely treatment of the heart failure with IABP and PCPS prevented any further complications.

Anterior spinal artery syndrome after minimally invasive direct coronary artery bypass grafting under general combined epidural anesthesia.

A 65-year-old patient with ischemic heart disease and severe diabetes mellitus underwent minimally invasive direct coronary artery bypass grafting under general combined with epidural anesthesia. Paraplegia developed after surgery and the diagnosis of anterior spinal artery syndrome was made based on the patient's neurological condition and magnetic resonance imaging findings. Paraplegia following epidural anesthesia is a rare but recognized complication and this complication should be taken into account, especially in patients at risk, when considering epidural analgesia techniques in the minimally invasive cardiac surgery.

Stent grafting technique using Matsui-Kitamura (MK) stent for patients with aortic arch aneurysm.

OBJECTIVE: Although several methods of stent-grafting for patients with aortic arch aneurysm have been reported, these methods have been associated with several complications such as endoleak and migration. We developed a new method using Matsui-Kitamura (MK) stent-graft following extra-anatomic arch vessels bypass by selective cerebral perfusion (SCP) under left heart bypass (LHB). METHODS: Between December 2001 and December 2003, 12 patients with aortic arch aneurysm were treated according to this new method. All patients were male with an average age of 71.3+/-6.4 years. There were 5 patients with severe pulmonary dysfunction, 4 with renal dysfunction, one with severe cardiac dysfunction and 3 with preoperative cerebral infarction. Under SCP using LHB, the extra-anatomic arch vessel bypass was established. The MK stent-graft was delivered into the aortic arch. Coronary artery bypass grafting (CABG) was concomitantly performed in one patient. RESULTS: There were no cases of endoleak, migration or hospital death. One patient, who had a past history of cerebrovascular disease, suffered a minor stroke, and one patient, who was performed CABG to the mid-left anterior descending branch (LAD) using the left internal thoracic artery (LITA), presented paraparesis. Although two patients of chronic renal failure underwent scheduled CHDF on account of using the contrast medium during the procedure, all of them were weaned from hemodialysis. However, there were no other postoperative complications such as, respiratory failure or cardiac dysfunction. CONCLUSIONS: Endovascular stent grafting EVSG using the MK stent with extra-anatomic arch vessel bypass under SCP using LHB could be a useful and less invasive method for patients with aortic arch aneurysm who are at a high surgical risk.

Results of aortic surgery in patients with Marfan syndrome.

PURPOSE: We reviewed the outcome of aortic operations in patients with Marfan syndrome to evaluate the immediate and long-term results of surgical treatment. METHODS: Between January 1985 and June 2002, 22 patients with Marfan syndrome underwent surgical treatment for aortic diseases at our hospital. Eight patients had Stanford type A aortic dissection and ten patients had aortic root aneurysm with aortic regurgitation. We performed aortic root replacement in 21 patients. Subsequent aortic operations were done in 11 patients, 6 of whom required a third operation. Five patients underwent repeat surgery of the previously operated aortic segment. A collective a total of 42 aortic operations were performed in the 22 patients. Seven patients underwent total aortic replacement. RESULTS: The in-hospital mortality rate was 4.5%, and there were three late deaths: two caused by rupture of the dissected aorta, 17 months and 24 months after the last surgical intervention, respectively, and one caused by heart failure 27 months after a total aortic replacement. The overall actuarial survival was 90.2% at 5 years and 74.4% at 10 years. CONCLUSIONS: Surgical treatment of patients with Marfan syndrome can be accomplished with low mortality; however, new aortic lesions should be promptly explored, and appropriately timed surgical treatment can substantially improve the prognosis of patients with Marfan syndrome.

Quality of life in elderly patients following thoracic aortic surgery.

OBJECTIVE: While early outcomes following thoracic aortic surgery are improving, the long-term quality of life in elderly patients following this procedure remains uncharacterized. Thus, the goal of this retrospective study was to investigate quality of life in elderly patients following thoracic aortic surgery in regard to age, urgency of operation, operative procedures, skin incision, selective cerebral perfusion (SCP) time, and cardiopulmonary bypass time. METHODS: One hundred-and-eleven surviving patients that underwent thoracic aortic surgery between 1987 and 1999 were enrolled in this study. The Short Form-36 (SF-36) health questionnaire was administered to all participants. RESULTS: Some measures of quality of life were lower in those patients that underwent the procedure as compared to age- and gender-matched normal population. Quality of life (QOL) of the elderly patients with prolonged SCP time (>120 minutes) was significantly lower in the dimension of role-physical of SF-36. On the other hand, there was no significant difference between the subgroups in terms of urgency of operation, operative procedure, skin incision, and cardiopulmonary bypass time. CONCLUSIONS: Physical and mental quality of life was significantly lower in elderly patients undergoing thoracic aortic surgery. Prolonged SCP time (>120 minutes) was a negative factor for long-term QOL. We advocate discussing the data with patients when obtaining informed consent for this procedure.

Expression of the von Willebrand factor in atrial endocardium is increased in atrial fibrillation depending on the extent of structural remodeling.

BACKGROUND: The incidence of stroke in patients suffering atrial fibrillation (AF) is increased when left atrial enlargement occurs. Recently, the platelet adhesive molecule, von Willebrand factor (vWF), located in the atrial endocardium, has been shown to be increased in patients with a variety of heart diseases compared with patients who have no cardiac problems. METHODS AND RESULTS: We investigated the expression of vWF mRNA and protein in the endocardium as a possible prothrombotic alteration of AF in association with atrial structural remodeling. Atrial appendage specimens were obtained during either heart surgery or at an autopsy from AF patients with and without underlying heart disease. The immunohistochemical and in situ hybridization signals for vWF in the endocardium were well correlated and varied widely among the individual atrial appendages examined. The increased expression of vWF in the endocardium was associated with enlarged left atrial dimensions in mitral valvular disease or increased myocyte diameters in the underlying myocardium. Platelet adhesion/aggregation on the endocardium was always found under the fresh thrombi and was colocalized with strong vWF staining, but not necessarily with fibrinogen and/or fibrin staining. CONCLUSIONS: Endocardial overexpression of vWF may occur during the process of atrial structural remodeling contributing to the thrombotic predilection of AF in association with underlying heart disease.

Use of cuffed anastomosis in total aortic arch replacement.

The distal aortic anastomosis portion of the total arch surgery remains technically complex especially in cases in which an aortic arch aneurysm extends below level of carina. We present the cuffed anastomosis that overcomes this difficulty. We applied this technique in 49 patients of elective total aortic arch aneurysm repair using selective cerebral perfusion from 1996 to 2001. Hospital mortality was 2%.