A Patient with Werner's Syndrome Who Underwent Aortic Valve Replacement through Minimally Invasive Cardiac Surgery.

Werner's syndrome (WS) is a genetic disorder presenting with premature senility. In the present study, we performed minimally invasive cardiac surgery (MICS)-aortic valve replacement (AVR) on a patient with Werner's syndrome who presented with aortic stenosis. The patient, a 49-year-old Japanese man, was brought to the emergency room with dyspnea during exercise. On echocardiography, severe aortic stenosis was found and surgery was planned. He had poorly controlled diabetes mellitus and underwent MICS-AVR to avoid the risk of sternal osteomyelitis, which resulted in a good outcome. The aortic valve had sclerotic changes and a genetic disease was suspected based on the onset of aortic stenosis at a young age, characteristic appearance, and various signs of aging. Genetic testing led to the diagnosis of WS.

The impact of lesion complexity on predicting mortality of coronary artery disease patients after out-of-hospital cardiac arrest.

INTRODUCTION: Coronary artery disease (CAD) is the most frequent cause of out-of-hospital cardiac arrest (OHCA). Nevertheless, there have been limited studies focusing on the impact of lesion complexity on resuscitated CAD patients. The purpose of the present study was to investigate the association between coronary lesion complexity and the mortality of CAD patients after OHCA. METHODS: From pooled database of two centers, which comprised 706 successfully resuscitated OHCA patients, 172 patients undergoing coronary angiography were retrospectively investigated. A total of 148 patients exhibited coronary stenosis on angiogram and were included in the final analysis. Baseline characteristics, pre-and post-hospital care, general status after resuscitation and angiographical findings were compared between the patients who deceased within 30 days and those who survived and the predictors of 30-day mortality were determined. RESULTS: Ninety-four patients (63.5%) survived at 30 days. Bystander cardiopulmonary resuscitation (CPR) (Odds ratio (OR) 0.36; 95% confidence interval (CI) 0.14-0.96; P = 0.041), revascularization of coronary stenosis (OR 0.15; 95% CI 0.19-0.86; P < 0.001), GRACE risk score (OR 1.04; 95% CI 1.02-1.05; P < 0.001) and SYNTAX score (OR 1.07; 95% CI 1.01-1.13; P = 0.025) were independent predictors of 30-day mortality. As multiple predictors such as bystander CPR, GRACE score and SYNTAX score were combined, the 30-day mortality gradually deteriorated. CONCLUSIONS: In addition to bystander CPR, GRACE score and revascularization, SYNTAX score independently predicted 30-day mortality of CAD patients after OHCA.

Effect of large-scale disasters on bystander-initiated cardiopulmonary resuscitation in family-witnessed, friend-witnessed and colleague-witnessed out-of-hospital cardiac arrest: a retrospective analysis of prospectively collected, nationwide, population-based data.

IMPORTANCE: The effect of large-scale disasters on bystander cardiopulmonary resuscitation (BCPR) performance is unknown. OBJECTIVE: To investigate whether and how large-scale earthquake and tsunami as well as subsequent nuclear pollution influenced BCPR performance for out-of-hospital cardiac arrest (OHCA) witnessed by family and friends/colleagues. DESIGN AND SETTING: Retrospective analysis of prospectively collected, nationwide, population-based data for OHCA cases. PARTICIPANTS: From the nationwide OHCA registry recorded between 11 March 2010 and 1 March 2013, we extracted 74 684 family-witnessed and friend/colleague-witnessed OHCA cases without prehospital physician involvement. EXPOSURE: Earthquake and tsunamis that were followed by nuclear pollution and largely affected the social life of citizens for at least 24 weeks. MAIN OUTCOME AND MEASURE: Neurologically favourable outcome after 1 month, 1-month survival and BCPR. METHODS: We analysed the 4-week average trend of BCPR rates in the years affected and before and after the disaster. We used univariate and multivariate logistic regression analyses to investigate whether these disasters affected BCPR and OHCA results. RESULTS: Multivariable logistic regression for tsunami-affected prefectures revealed that the BCPR rate during the impact phase in 2011 was significantly lower than that in 2010/2012 (42.5% vs 48.2%; adjusted OR; 95% CI 0.82; 0.68 to 0.99). A lower level of bystander compliance with dispatcher-assisted CPR instructions (62.1% vs 69.5%, 0.72; 95% CI 0.57 to 0.92) in the presence of a preserved level of voluntary BCPR performance (23.6% vs 23.8%) was also observed. Both 1-month survival and neurologically favourable outcome rates during the impact phase in 2011 were significantly poorer than those in 2010/2012 (8.5% vs 10.7%, 0.72; 95% CI 0.52 to 0.99, 4.0% vs 5.2%, 0.62; 95% CI 0.38 to 0.98, respectively). CONCLUSION AND RELEVANCE: A large-scale disaster with nuclear pollution influences BCPR performance and clinical outcomes of OHCA witnessed by family and friends/colleagues. Basic life-support training leading to voluntary-initiated BCPR might serve as preparedness for disaster and major accidents.

A patient with Marfan's syndrome who developed an acute aortic dissection at 28 weeks of pregnancy treated with aortic root replacement.

The patient was a 31-year-old pregnant woman who gave birth to her first child by vaginal delivery 7 years ago. She was diagnosed with Marfan's syndrome based on physical findings; however, the condition was not diagnosed before the onset. The patient developed acute aortic dissection at 28 weeks of pregnancy. A cesarean section was first performed to save the patient's life; then, a total hysterectomy was performed to prevent the risk of postpartum hemorrhage. Furthermore, aortic root replacement was performed using a temporary mechanical valve. The patient and her child have survived without any complications.

The Sequential Organ Failure Assessment (SOFA) score predicts mortality and neurological outcome in patients with post-cardiac arrest syndrome.

BACKGROUND: Patients experiencing out-of-hospital cardiac arrest (OHCA) and subsequent post-cardiac arrest syndrome are often compromised by multi-organ failure. The Sequential Organ Failure Assessment (SOFA) score has been used to predict clinical outcome of patients requiring intensive care for multi-organ failure. Thus, the assessment of SOFA score is recommended as a criterion for sepsis. Although post-cardiac arrest patients frequently develop sepsis-like status in ICU, there are limited reports evaluating the SOFA score in post-cardiac arrest patients. We investigated the predictive value of the SOFA score in survival and neurological outcomes in patients with post-cardiac arrest syndrome. METHODS: A total of 231 cardiovascular arrest patients achieving return of spontaneous circulation (ROSC) were finally extracted from the institutional consecutive database comprised of 1218 OHCA patients transferred to the institution between January 2015 and July 2018. The SOFA score was calculated on admission and after 48h. Predictors of survival and neurological outcome defined as having cerebral-performance-category (CPC) 1 or 2 at 30 days were determined. RESULTS: SOFA score was lower in survived patients (5.0 vs 10.0, p<0.001) and those with favorable neurological outcome (5.0 vs 8.0, p<0.001) as compared with the counterparts. The SOFA score on admission was an independent predictor of survival (OR 0.68, 95% confidence interval [CI] 0.59-0.78; p<0.001) and favorable neurological performance (OR 0.79; 95% CI 0.69-0.90; p<0.001) at 30 days. Furthermore, a change in SOFA score (48-0h) was predictive of favorable 30-day neurological outcome (OR 0.71, 95% CI 0.60-0.85; p<0.001). CONCLUSIONS: Evaluation of the SOFA score in the ICU is useful to predict survival and neurological outcome in post-cardiac arrest patients.

Surgical outcomes of acute type A aortic dissection in dialysis patients.

BACKGROUND: Acute type A aortic dissection (ATAAD) is relatively uncommon in dialysis patients, and characteristics and repair outcomes are not fully understood. PATIENTS AND METHODS: Patients with ATAAD (n = 960) were divided into a dialysis group (n = 19) and non-dialysis group (n = 941), depending on whether they required dialysis for preoperative end-stage renal disease (ESRD). Hospital charts and imaging data were reviewed, and characteristics and outcomes were compared between the groups. Segmental aortic wall or intima/media flap calcification in the thoracic and abdominal aorta was assessed in the dialysis patients. RESULTS: The leading primary causes of ESRD were polycystic kidney disease (n = 5) and chronic glomerulonephritis (n = 5). There were no significant differences (dialysis group vs. non-dialysis group) in age (60.5 vs. 64.5 years), preoperative hemodynamics, or organ ischemia. Dialysis patients were more likely to have an entry tear in the aortic arch (42% vs. 15%, p = 0.003). These patients showed moderate-to-severe calcification (multiple focal or single focal calcification > 10 mm) in the ascending aorta (17%), aortic arch (61%), descending aorta (67%), and abdominal aorta (83%). Arch replacement was common in this group (37% vs. 18%, p = 0.030). Although in-hospital mortality was increased in this group (21% vs. 7%, p = 0.059), morbidities did not differ significantly. Six-year survival was 60.3 ± 13.4% and 78.8 ± 1.6%, respectively (p = 0.01). CONCLUSIONS: Dialysis patients tend to have aortic calcification and a primary tear in the aortic arch. Outcomes are acceptable.

Total Occlusion of Abdominal Aortic Endograft Successfully Treated with Axillobifemoral Bypass.

We report a case of total occlusion of a Zenith bifurcated stent graft 16 months after implantation. A 72-year-old man was admitted to our hospital complaining of bilateral lower extremity numbness, followed by severe rest pain 4 h after sudden onset of symptoms. Computed tomography showed total occlusion of the endograft at the mid-portion of the main body. He underwent left axillobifemoral bypass using a reinforced polytetrafluoroethylene T-shaped graft, leading to resolution of symptoms 7 h after onset. Axillobifemoral bypass successfully relieved acute lower extremity ischemia caused by total occlusion of the abdominal aortic endograft.

Bentall operation for prosthetic valve endocarditis with hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT; Osler-Weber-Rendu syndrome) is an uncommon disease characterized by abnormal telangiectasias and arteriovenous malformations that cause recurrent bleeding. Here, we present the case of a patient with HHT, who had a history of pulmonary and hepatic arteriovenous malformations and endocarditis of a prosthetic aortic valve that was caused by methicillin-resistant Staphylococcus aureus. The patient underwent the Bentall operation after coil embolization for pulmonary arteriovenous malformations. The postoperative course was uneventful.

Successful non-operative management of left atrioesophageal fistula following catheter ablation.

Atrioesophageal fistula (AEF) is a potentially lethal complication of catheter radiofrequency ablation for atrial fibrillation. A 49-year-old man with paroxysmal atrial fibrillation who underwent catheter ablation around the pulmonary vein was admitted 31 days after the procedure, suffering seizures and fever. Magnetic resonance imaging of the brain showed ischemia and multiple lesions of acute infarction in the right occipital lobe of the cerebrum. Computed tomography (CT) of the chest showed a small accumulation of air between the posterior left atrium and the esophagus, suggesting an AEF. Endoscopic snaring of the esophageal mucosa, repeated a few times, supported by nil by mouth and antibiotic therapy, resulted in improvement of his condition with no recurrence of symptoms. Subsequent chest CT scans confirmed disappearance of the leaked air and the patient was discharged home 45 days after admission with no neurological compromise.

Is the threshold for postoperative prosthesis-patient mismatch the same for all prostheses?

PURPOSE: The effective orifice area index (EOAI) is used to define the prosthesis-patient mismatch (PPM) after aortic valve replacement (AVR). However, few studies have so far evaluated whether the cutoff value for PPM varies across prostheses. This study assessed the hemodynamics in patients given a mechanical valve and then re-evaluated the validity of the commonly accepted threshold. METHODS: The subjects included 329 patients that underwent AVR with a St. Jude Medical Regent valve. The transvalvular pressure gradient and EOAI were determined echocardiographically, and the commonly accepted threshold was analyzed in relation to survival. RESULTS: The mechanical valves very often yielded a postoperative transvalvular pressure gradient >10 mmHg, and thus, clinically significant residual pressure, regardless of the EOAI. The slope of the curve describing the relationship between the transvalvular pressure gradient and EOAI was gentler than that reported for bioprosthetic valves, for which the pressure gradient rises sharply at EOAI <0.85 cm(2)/m(2). The commonly defined PPM did not affect the long-term survival or regression of the left ventricular mass index. CONCLUSIONS: The relationship between the transvalvular pressure gradient and the EOAI in patients given a mechanical prosthesis differed from the reference standard. These data suggest the need to reconsider the appropriate cutoff value for PPM in relation to different prostheses.

Successful surgical repair of pulmonary artery aneurysm and regurgitation.

Pulmonary artery aneurysm (PAA) is generally a rare lesion, and there is no definitive approach for it. We report the case of a 45-year-old man who was admitted for the evaluation of dyspnea. In childhood, he had been diagnosed with PAA with congenital pulmonary valve stenosis and regurgitation, and he had a percutaneous transvenous pulmonary valve commissurotomy. Transthoracic echocardiogram showed dilatation of the right atrium and right ventricle, with right ventricular hypertrophy. There was severe pulmonary valve regurgitation, and the main pulmonary artery was dilated to 68 mm in diameter. From the surgical findings, the left leaflet of pulmonary valve was torn from commissure with failure to coapt with the other leaflet. After direct sutures of edges of the left leaflet, a nearly normal valvular competence was restored. The PAA was repaired with a Y-shaped 24 × 12 mm Dacron graft replacement. The postoperative course was uneventful, and the patient was discharged.

Clinical outcomes and hemodynamics of the 19-mm Perimount Magna bioprosthesis in an aortic position: comparison with the 19-mm Medtronic Mosaic Ultra Valve.

BACKGROUND: When aortic valve replacement is performed in patients with a small aortic annulus, prosthesis-patient mismatch (PPM) is of concern because it can affect postoperative clinical outcomes. Although larger bioprosthetic valves have been well studied, the hemodynamics of 19-mm bioprostheses have been reported in only a small number of patients. The effectiveness as well as the impact of PPM on outcomes are thus still unclear. METHODS AND RESULTS: Postoperative clinical and hemodynamic variables were compared in 67 patients with a 19-mm Carpentier Edwards Perimount Magna bioprosthesis and in 10 patients with a 19-mm Medtronic Mosaic Ultra valve. Mean follow-up time was 13 months. There was no in-hospital mortality. Echocardiography 6.5±4.0 months after surgery showed significant decreases in the mean left ventricular (LV)-aortic pressure gradient, and decreases in the mean LV mass index. Reduction in LV mass index did not differ between the valve groups, despite a higher pressure gradient in the Mosaic group. Although PPM was detected in 21 patients in the Magna group, it did not affect regression of the LV mass index during the follow-up period. CONCLUSIONS: Use of the 19-mm Magna bioprosthesis appears to provide satisfactory clinical results. LV-aortic pressure gradient was lower in the Magna group. The present data suggest that PPM is not related to reduction in the LV mass index.

Mid-term outcomes after aortic valve replacement with the 17-mm St. Jude Medical Regent valve.

BACKGROUND: When aortic valve replacement (AVR) is performed in patients with a small aortic annulus, prosthesis-patient mismatch (PPM) is of concern. We investigated the mid-term outcomes of AVR with a 17-mm mechanical prosthesis. METHODS AND RESULTS: Seventy-eight patients with aortic stenosis underwent AVR with a 17-mm St. Jude Medical Regent prosthesis. Echocardiography was performed preoperatively, at discharge, and at follow-up (mean follow-up, 33 months). Patients were divided into 2 groups: with and without PPM at discharge. Between-group differences in postoperative variables, particularly survival, were analyzed. Overall hospital mortality was 2.6%. Actuarial 1- and 5-year survival rates were 95% and 79%, respectively. Diabetes and renal insufficiency were associated with long-term mortality. Freedom from major adverse valve-related cardiac events at 1 year and 5 years was 97.3% and 93.9%, respectively. Diabetes was shown to be an independent risk factor for major adverse valve-related cardiac events. Echocardiography 13 months after AVR showed a significant increase in mean effective orifice area index, decrease in mean left ventricular-aortic pressure gradient, and decrease in mean left ventricular mass index. PPM at discharge did not influence long-term survival or left ventricular mass regression. CONCLUSIONS: The 17-mm Regent prosthesis provided satisfactory clinical and hemodynamic results. It is a reliable choice for patients with a small aortic annulus.

Infection of pacemaker lead by penicillin-resistant Streptococcus Pneumoniae.

Penicillin-resistant Streptococcus pneumoniae (PRSP) infections have steadily increased worldwide; however, there are only a few reports of permanent pacemaker-related infections caused by PRSP. Here, we describe a patient who developed 7 episodes of endocarditis and sepsis from PRSP infection of the pacemaker lead in the right atrium. By periodic administration of vancomycin and extraction of both leads, we resolved the infection.

Rudimentary right ventricle to pulmonary artery shunt in the Norwood procedure.

We describe here successful palliative repair of tricuspid atresia, hypoplastic right ventricle, transposition of the great arteries, and hypoplastic aortic arch in a neonate. The repair consisted of the Norwood procedure with a rudimentary right ventricle to pulmonary artery shunt, which was located on the right side of a neo-aorta. This procedure could be a useful adjunct to avoid left ventriculotomy and its subsequent dysfunction.

[The trial of the domiciliary nutrition support team in which a home medical care office offers].

The function of home care support by a medical office is to offer the best medical care for the patient. It is essential that the medical office is capable of taking a call 24-hour a day and 365-day a year from a patient who needs help at home. Our medical office was specialized in home care treatment. Furthermore, we offer a home rehabilitation or a home nutrition education to the patient. On the other hand, a nutritional support is important as well as medical supports. To offer a high quality medical care at home, we created a nutrient support system in our hospital, and formed an at-home nutrition support team(at-home NST). The team is consisted of a medical staff and dietitian, a physical therapist and a speech therapist. As a result of the at-home NST, We improved the followings: (1) we were able to collect a nutritional data basis including a patient 's height and weight, (2) we made a good use of patient's eating habit at home during the medical treatment, and (3) we could make a good use of medical service to a home care patient by managing the information accumulated by nutritional surveillance. In multidisciplinary collaboration, at-home NST can grasp a versatility status of the patient positively. We continue to offer a medical care that is demanded from a home care patient because the activity of the at-home NST raises a quality of medical service we provide.

Aortic relocation for transposition complex with aortic arch obstruction.

We describe successful primary repair of 2 cases of transposition complex with aortic arch obstruction. A new aortic arch was reconstructed by direct anastomosis between the well-mobilized ascending aorta and the descending aorta. The neoaortic root with transferred coronary arteries was subsequently anastomosed to the undersurface of this new aortic arch. This technique deals with the significant size discrepancy between the 2 great arteries, and anomalous coronary artery patterns.

Clinical experience with the Bicarbon heart valve prosthesis.

BACKGROUND: We have previously reported mid-term results of a study, which ended in January 2000, on the Bicarbon valve. The study concluded that the valve showed excellent clinical results, associated with a low incidence of valve-related complications. In the present study, the same patients were prospectively followed for an additional 5 years. METHODS: Forty-four patients had aortic valve replacement (AVR), 48 had mitral valve replacement (MVR), and 13 had both aortic and mitral valve replacement (DVR). The mean age of the 105 patients was 61.2 +/- 11.3 years. The mean follow-up was 6.1 +/- 1.9 years with a cumulative follow-up of 616 patient-years. RESULTS: There were 5 early deaths (4.7%: 4 in the AVR group and 1 in the MVR group) and 21 late deaths (3.4%/patient-year: 5 valve related deaths and 16 valve unrelated deaths). Survival at 8 years was 75.2 +/- 7.0% in the AVR group, 76.6 +/- 6.2% in the MVR group, and 55.4 +/- 16.1% in the DVR group. The linearized incidence of thrombo-embolic complications, hemorrhagic complications, and paravalvular leaks in all patients was 0.65 +/- 1.48%, 0.81 +/- 1.69%, and 0.16 +/- 0.54%/patient-year respectively. No other complications were observed. CONCLUSION: The Bicarbon prosthetic heart valve has shown excellent long-term clinical results, associated with a low incidence of valve-related complications.