Hypersensitivity to intravenous succinate corticosteroids in patients with nonsteroidal anti-inflammatory drug-exacerbated respiratory disease.

Although there are many case reports of asthma exacerbations with intravenous corticosteroids, especially hydrocortisone succinate, in nonsteroidal anti-inflammatory drug-exacerbated respiratory disease (N-ERD), the frequency and mechanism remain unclear. We hypothesized that N-ERD patients are potentially hypersensitive to succinates, especially succinate corticosteroids, based on the results of previous provocation studies and considered specific mechanisms. The objective of this study was to determine the frequency and mechanism of succinate corticosteroids hypersensitivity in patients with N-ERD. Eleven patients with stable, moderate to severe N-ERD were tested with hydrocortisone sodium succinate (HCs), hydrocortisone sodium phosphate (HCp), methylprednisolone sodium succinate (MPSLs), prednisolone sodium succinate (PSLs), and chloramphenicol sodium succinate (CPs, without a steroidal chemical structure) at doses below the normal dose through intravenous administration using a single-blind test. As a comparison, seven patients with aspirin-tolerant asthma (ATA) also underwent an intravenous provocation test of HCs. The positive intravenous provocation test rates of HCs 100-500 mg, HCp 500 mg, MPSLs 80 mg, PSLs 20 mg, and CPs 500 mg in N-ERD patients were 82% (9/11), 9% (1/11), 50% (5/10), 33% (1/3), and 86% (6/7), respectively. Most positive reactions began with a severe cough within 5 min of intravenous injection. The course of these hypersensitivity symptoms differed from those seen with the usual aspirin challenge test. The HCs 100-500 mg intravenous test was negative in all seven patients with ATA. In conclusion, patients with N-ERD have high rates of potential hypersensitivity to the succinate ester structure, which is not linked to the corticosteroid structure, but to the succinate ester structure. We hypothesized that the mechanism of hypersensitivity observed during rapid intravenous administration of succinate corticosteroids is mast cell activation via succinate receptor stimulation, rather than due to the corticosteroid itself.

The "bud" of an aortic aneurysm.

An overlooked "bud" of an aortic aneurysm on a chest radiography resulted in a saccular aortic aneurysm 9 years later. This eye-catching image showed us the importance of not only documenting abnormalities in regular radiography but also further imaging evaluation for definitive diagnosis and continuous image follow-up.

High-Flow Nasal Cannula Therapy With Early Extubation for Subjects Undergoing Off-Pump Coronary Artery Bypass Graft Surgery.

BACKGROUND: The effects of high-flow nasal cannula (HFNC) therapy on postoperative atelectasis and duration of oxygen therapy after off-pump coronary artery bypass graft are unknown. The purpose of this study was to compare the effects of HFNC therapy for subjects who underwent off-pump coronary artery bypass graft with the effects of standard oxygen therapy in terms of oxygen requirement and atelectasis. METHODS: This prospective single-blinded randomized, controlled trial included 148 subjects who underwent off-pump coronary artery bypass graft between 2010 and 2015 with HFNC (n = 72) or without HFNC (standard O2, n = 76). The primary end point was the percentage difference in loss of lung volume between subjects with or without HFNC therapy. Secondary end points included the total amount of oxygen administered and duration of oxygen therapy with and without HFNC therapy. RESULTS: There were significant between-group differences in the percentage loss of lung volume (P < .001), total amount of oxygen administered (P < .001), duration of oxygen therapy (P < .001), and the need for postoperative diuretic therapy (P = .037). The amount (ρ = 0.569, P < .001) and duration (ρ = 0.678, P < .001) of oxygen administered were correlated with atelectasis volume. CONCLUSIONS: Using HFNC therapy after off-pump coronary artery bypass graft shortened the duration of oxygen therapy and reduced the percentage loss of lung volume and total amount of oxygen administered when compared with standard oxygen therapy.

Long-term outcome in a case of translocated mitral valve replacement for massive mitral annular calcification.

Few reports have described long-term outcomes after translocated mitral valve replacement. We describe tips, potential pitfalls, and long-term outcome associated with the construction of a new mitral annulus and reinforcement of prosthesis attachment using a mitral prosthetic valve with an equine pericardial collar in a woman with extensive mitral valve calcification.

Outcomes of prosthetic valve replacement in women of child-bearing age.

PURPOSE: The outcomes of pregnancy are more favorable for women with bioprostheses than for those with mechanical prostheses. However, bioprostheses are associated with a high reoperation rate in young women and it remains unclear whether these young women can give birth without any complications. We analyzed the outcomes of prosthetic valve replacement and investigated the effectiveness and problems associated with bioprostheses in women of child-bearing age in Japan. METHODS: The subjects of this study were six consecutive young adult women aged under 40 years, who underwent prosthetic valve replacement between January 2007 and April 2016. RESULTS: Bioprostheses were selected for four of these six women in consideration of their child-bearing age. Mechanical valves were selected for the other two women who underwent the Konno procedure and double valve replacement (AVR, MVR) in view of their high risk for reoperation. The cardiac operations, although without mortality or morbidity, were complex and some involved multi-time procedures. Three of the women with bioprostheses had uneventful term pregnancies. CONCLUSIONS: These young women with bioprostheses were able to give birth safely; however, as multiple operations are often required, and bioprostheses may not be ideal for young women. Prosthetic valve selection for young women of child-bearing age requires adequate pregnancy counseling and long-term planning.

Aortic valve replacement in a patient with MPO-ANCA-positive Goodpasture disease.

Goodpasture disease (GD) is a rare autoimmune disorder characterized by the development of pathologic autoantibodies against both glomerular and alveolar basal membranes. Approximately one third of the patients with GD are also positive for anti-neutrophil cytoplasmic antibody (ANCA). In this case report, a 74-year-old woman was diagnosed as having myeloperoxidase (MPO)-ANCA-positive GD with severe aortic valve stenosis (AS). She underwent immunosuppressive therapy and plasmapheresis that led to GD remission. Whether a cardiac surgery affects a MPO-ANCA-positive GD in remission is unknown. We reported the outcomes after aortic valve replacement for severe AS in a patient with MPO-ANCA-positive GD.

A novel biodegradable external mesh stent improved long-term patency of vein grafts by inhibiting intimal-medial hyperplasia in an experimental canine model.

OBJECTIVES: Increased hemodynamic stress on vein grafts used in the arterial system is associated with vein graft disease. We determined whether a novel biodegradable external mesh stent could inhibit medial-intimal hyperplasia by suppressing hemodynamic stress on vein grafts and improve long-term patency. METHODS: Twenty-four beagles underwent bilateral femoral interposition grafting using reversed femoral veins. Vein grafts were externally supported by a novel poly L-lactide-ε-caprolactone copolymer (P(LA/CL)) biodegradable mesh stent or a nonabsorbable mesh stent. Vein grafts with no reinforcement were used as controls. The grafts were harvested 6 and 12 months after implantation for morphometric and immunohistochemical assessment. RESULTS: The endoluminal circumferential vein graft length was smaller in the P(LA/CL) and nonabsorbable groups (17.2 ± 2.9 and 19.0 ± 0.3 mm, respectively), than that in the control group (25.0 ± 2.6 mm, P < 0.01) at 12 months. The mean intimal-medial thickness was thinner in P(LA/CL) and nonabsorbable stent groups (0.18 ± 0.05 and 0.16 ± 0.05 mm, respectively), than that in the control group (0.30 ± 0.08 mm, P < 0.01). Differences in the intimal-medial thickness among the groups were associated with the magnitude of cellular proliferating activity. The graft patency ratio (100 %) was higher in the P(LA/CL) group than that in the nonabsorbable and control groups (72.2 and 63.6%, respectively, P < 0.05). CONCLUSIONS: The biodegradable P(LA/CL) external mesh stent improved vein graft patency for 12 months and prevented vein graft dilatation and intimal hyperplasia associated with suppressed neointimal layer cellular proliferating activity.

Use of a Stent Graft for Patent Ductus Arteriosus in an Octogenarian Eliminates Ductus Flow.

Closure of a patent ductus arteriosus (PDA) in the elderly is a high-risk procedure because of tissue fragility and many possible complications. The patient in our case was an 81-year-old woman with a window-type PDA caused by cardiac failure. Based on the anatomy of the PDA and aorta and to minimize invasion, we used a stent graft to close the PDA. This approach was successful; hemodynamics improved and ductus flow was eliminated during the follow-up period without intervention from the pulmonary artery side.

An exploratory trial of intravenous immunoglobulin therapy for idiopathic pulmonary fibrosis: a preliminary multicenter report.

BACKGROUND AND AIMS: Idiopathic pulmonary fibrosis (IPF) is a fatal disorder without specific treatments. Although the efficacy of intravenous immunoglobulin (IVIG) therapy for autoimmune diseases has been reported, that for IPF remains unknown. This study aims to determine the efficacy and safety of IVIG for IPF. METHODS: In an exploratory, multicenter, non-randomized and prospective trial, patients with progressive IPF were enrolled. Patients were treated with IVIG for five consecutive days (5-day IVIG) or once monthly for five consecutive months (5-month IVIG). Changes in the vital capacity (VC), diffusion capacity of the lung for carbon monoxide (DLCO), 6-min walk test (6MWT) and high-resolution computed tomography (HRCT) findings were evaluated. RESULTS: A total of 10 patients with IPF were treated with IVIG: 6 were in 5-day IVIG and 4 were in 5-month IVIG group. In 5-day IVIG group, the treatment effects were temporal, and physiological and HRCT findings deteriorated in three of six patients. In 5-month IVIG group, changes in %VC, %DLCO and walk distance in 6MWT at 6 months were -0.9 ± 5.3%, 6.9 ± 12.6% and 79 ± 58 m (mean ± standard deviation), respectively, and the treatment effects were long lasting. The change in VC 6 months after starting IVIG was smaller than that of 6-12 months after starting IVIG (after cessation of IVIG) (-0.02 ± 0.15 vs -0.33 ± 0.14 L, P = 0.022). Ground glass opacities were diminished in two of four patients. Adverse events were mild and tolerable. CONCLUSION: This preliminary study shows that once-monthly IVIG treatment may be effective and tolerable in patients with IPF.

[Ascending-descending Aortic Bypass and Aortic Valve Replacement for Aortic Coarctation with Bicuspid Aortic Valve and an Aberrant Right Subclavian Artery;Report of a Case].

A 53-year-old woman was developed congestive heart failure. She was diagnosed as having aortic coarctation, incompetent bicuspid aortic valve and an aberrant right subclavian artery by using echocardiography and enhanced computed tomography. Ankle brachial pressure index(ABI)in the right was 0.71 and 0.69 in the left. Blood pressure of the right arm was 60 mmHg lower than that of the left arm. To avoid perioperative adverse cardiac events due to a 2-staged operation, we performed ascending-descending aortic bypass and aortic valve replacement simultaneously through a median sternotomy. The heart was retracted cranially, and a vascular prosthesis was anastomosed to the descending aorta just above the diaphragm in an end-to-side manner. Then the graft was placed curvilinearly around the right atrium and was anastomosed to the ascending aorta. After the operation, the right and left ABI increased to 0.90 and 0.98 respectively. There was no pressure difference between the arms. The postoperative course was uneventful.

Purulent pericardial effusion and mycotic pseudoaneurysm following insertion of a bare metal stent.

A 65-year-old male was diagnosed with purulent pericarditis, caused by Staphylococcus aureus five weeks after bare metal stenting for a 90% stenosis of the right coronary artery ostium. Subsequently, he developed a pseudoaneurysm in the right coronary sinus of Valsalva (CSV) requiring surgical intervention during the treatment of the pericarditis. Bacteremia after percutaneous coronary intervention (PCI) occurs in < 1% of patients and usually has insignificant clinical sequelae. We present an infected coronary bare metal stent of the proximal right coronary artery after PCI that resulted in a purulent pericardial effusion and mycotic pseudoaneurysm of the right coronary sinus of Valsalva (CSV). The patient successfully underwent surgical treatment.

Midterm results of left coronary artery reimplantation through the transverse sinus of the pericardium in adult Bland-White-Garland syndrome.

The anomalous origin of the left coronary artery from the pulmonary artery - known as Bland-White-Garland syndrome - is a rare congenital malformation that affects 1 in 300,000 live births. Most patients die in infancy without any surgical treatment. Some patients who survive past childhood often have varying symptoms such as myocardial ischemia, impaired left ventricular function, mitral regurgitation, and progressive heart failure, depending on the development collateral circulation. In the present report, we describe a procedure wherein the left coronary artery ostium was translocated through the transverse sinus of the pericardium in a 43-year-old mother with Bland-White-Garland syndrome and concomitant mitral regurgitation and report on the associated midterm results.

Exploratory analysis of a phase III trial of pirfenidone identifies a subpopulation of patients with idiopathic pulmonary fibrosis as benefiting from treatment.

BACKGROUND: A phase III trial in Japan showed that pirfenidone is effective for idiopathic pulmonary fibrosis (IPF). To find out which patients specifically benefit from pirfenidone, we analyzed in an exploratory manner the data from the phase III trial. METHODS: The patients in the phase III trial were stratified by baseline percentage predicted vital capacity (%VC), arterial oxygen partial pressure (PaO(2)), and the lowest oxygen saturation by pulse oximetry (SpO(2)) during the 6-minute steady-state exercise test (6MET). In the subpopulations, changes in VC and subjective symptoms (cough and dyspnea on the Fletcher, Hugh-Jones [F, H-J] Classification scale) were evaluated in patients treated with high-dose (1800 mg/day) pirfenidone, low-dose (1200 mg/day) pirfenidone, and placebo at week 52. RESULTS: Significant efficacy of pirfenidone in reducing the decline in VC could be seen in a subpopulation having %VC ≥ 70% and SpO(2) < 90% at baseline. This favorable effect was accompanied by categorical change in VC and progression-free survival time. In the subpopulation, pirfenidone significantly suppressed cough and dyspnea. CONCLUSIONS: IPF patients having %VC ≥ 70% and SpO(2) < 90% at baseline will most likely benefit from pirfenidone when evaluated using changes in VC (and %VC), and cough and dyspnea symptoms. This subpopulation could expect to benefit most from pirfenidone treatment. TRIAL REGISTRATION: This clinical trial was registered with the Japan Pharmaceutical Information Center (JAPIC) on September 13th, 2005 (REGISTRATION NUMBER: JAPICCTI-050121).

The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: extended analysis of the pirfenidone trial.

BACKGROUND: Our phase III clinical trial of pirfenidone for patients with idiopathic pulmonary fibrosis (IPF) revealed the efficacy in reducing the decline of vital capacity (VC) and increasing the progression-free survival (PFS) time by pirfenidone. Recently, marginal decline in forced VC (FVC) has been reported to be associated with poor outcome in IPF. We sought to evaluate the efficacy of pirfenidone from the aspects of 5% change in VC. METHODS: Improvement ratings based on 5% change in absolute VC, i.e., "improved (VC ≥ 5% increase)", "stable (VC < 5% change)", and "worsened (VC ≥ 5% decrease)" at month 3, 6, 9 and 12 were compared between high-dose pirfenidone (1800 mg/day; n = 108) and placebo (n = 104) groups, and (high-dose and low-dose (1200 mg/day; n = 55)) pirfenidone (n = 163) and placebo groups. PFS times with defining the disease progression as death or a ≥ 5% decline in VC were also compared between high-dose pirfenidone and placebo groups, and low-dose pirfenidone and placebo groups. Furthermore, considering "worsened" and "non-worsened (improved and stable)" of the ratings at months 3 and 12 as "positive" and "negative", respectively, and the positive and negative predictive values of the ratings were calculated in each group. RESULTS: In the comparison of the improvement ratings, the statistically significant differences were clearly revealed at months 3, 6, 9, and 12 between pirfenidone and placebo groups. Risk reductions by pirfenidone to placebo were approximately 35% over the study period. In the comparison of the PFS times, statistically significant difference was also observed between pirfenidone and placebo groups. The positive/negative predictive values in placebo and pirfenidone groups were 86.1%/50.8% and 87.1%/71.7%, respectively. Further, the baseline characteristics of patients worsened at month 3 had generally severe impairment, and their clinical outcomes including mortality were also significantly worsened after 1 year. CONCLUSIONS: The efficacy of pirfenidone in Japanese phase III trial was supported by the rating of 5% decline in VC, and the VC changes at month 3 may be used as a prognostic factor of IPF. TRIAL REGISTRATION: This clinical trial was registered with the Japan Pharmaceutical Information Center (JAPIC) on September 13th, 2005 (REGISTRATION NUMBER: JAPICCTI-050121).

Hancock valve deterioration in tricuspid position for Ebstein's anomaly.

A 65-year-old woman with a Hancock valve implanted 25 years earlier for Ebstein's anomaly underwent a successful second tricuspid valve replacement with a Mosaic valve because of significant tricuspid regurgitation. At surgery, it was found that the Hancock valve had a cylinder-shaped hole and had lost its entire structure. Tricuspid valve dysfunction may be tolerated for a long time before surgery is contemplated.

Aortic valve stenosis associated with Bazin's disease.

Bazin's disease was first reported in 1861, and is described as erythema induratum or nodular vasculitis. The condition is seen occasionally in middle-aged women on the skin of the calf, and a relationship to a tuberculosis infection has been proposed. In the present patient, valvular lesions occurred simultaneously with Bazin's disease, with granulomatous changes being demonstrated by the aortic valve pathology.

Infective endocarditis associated with Sjogren's syndrome.

Sjögren's syndrome is one of the major autoimmune diseases, however infective endocarditis associated with Sjögren's syndrome has not previously been reported. A patient with Sjögren's syndrome associated with aortic valve regurgitation due to infective endocarditis, underwent successful aortic valve replacement. Patients with Sjögren's syndrome tend to be at a higher risk of intraoral infections due to diminished secretion of saliva. Particular care must be taken of Sjögren's syndrome patients as they can also be at an increased risk of infective endocarditis.

Pulmonary artery aneurysm with ascending aortic aneurysm concomitant with bilateral bicuspid semilunar valves.

Aneurysm of both the pulmonary trunk and the ascending aorta concomitant with bilateral bicuspid valves is very rare. The reason for the formation of aneurysm with bicuspid semilunar valve is still inconclusive. Surgical repair was performed successfully (ie, aortic valve replacement, graft replacement for the ascending aorta, and plication of the pulmonary artery). Pathology of the pulmonary artery wall did not demonstrate cystic medial necrosis. The hemodynamic turbulence by the bilateral bicuspid valve may cause the formation of aneurysms even at low pressure. This case demonstrates an explanation for aneurysm with the bicuspid valve.

Creation of coronary sinus using left atrial diaphragm in the patient with cor triatriatum and unroofed coronary sinus.

Surgical correction of cor triatriatum with unroofed coronary sinus consisted of creation of the coronary sinus using the left atrial diaphragm and closure of the atrial septal defect in an adult patient. No materials other than intracardiac components were used to repair all anomalies.