Concomitant use of VA-ECMO and Impella with inhaled nitric oxide to treat cardiogenic shock after cardiac surgery: A case report.

Venoarterial extracorporeal membrane oxygenation (VA-ECMO) is used to treat cardiogenic shock. However, a lack of left ventricle (LV) unloading and increased systemic afterload can cause pulmonary congestion. Impella (Abiomed, Danvers, MA, USA), a catheter-mounted micro-axial rotary pump, unloads the LV and provides hemodynamic support. However, Impella cannot support the right ventricle (RV), and RV dysfunction impedes weaning from VA-ECMO. A 50-year-old man with heart failure with reduced ejection fraction due to myocardial infarction developed moderate aortic stenosis and regurgitation, moderate mitral regurgitation, and tricuspid regurgitation. Aortic valve replacement, mitral valve replacement, and tricuspid valve replacement were performed. VA-ECMO with intra-aortic balloon pump (IABP) was initiated as he could not be weaned from cardiopulmonary bypass. The patient could not be weaned from IABP and VA-ECMO; therefore, Impella 5.0 was inserted instead of IABP on day 7. However, Impella 5.0 was ineffective due to RV dysfunction. Increased inhaled nitric oxide (iNO) dose lowered pulmonary vascular resistance, decreased RV afterload, and improved RV distension. He was weaned from VA-ECMO after increasing the flow from the Impella 5.0. Combining VA-ECMO with Impella and iNO improved hemodynamics in a patient with RV dysfunction, and Impella with iNO aided weaning from VA-ECMO. Learning objective: Combining venoarterial extracorporeal membrane oxygenation (VA-ECMO) with Impella (Abiomed, Danvers, MA, USA) and inhaled nitric oxide (iNO) can improve hemodynamics in a patient with right ventricular dysfunction, and iNO may aid weaning from VA-ECMO.

A Giant Left Coronary Button Aneurysm After Aortic Root Remodeling Procedure in a Patient With Marfan Syndrome: A Case Report.

Coronary button aneurysm is a well-known complication of aortic root surgery, especially in patients with Marfan syndrome. We present a case of a giant left coronary button aneurysm that occurred 20 years after an aortic root remodeling procedure was performed. A 32-year-old man with Marfan syndrome underwent the aortic root remodeling procedure for annuloaortic ectasia. Thirteen years later, an aortic aneurysm with chronic aortic dissection was diagnosed, and partial aortic arch replacement was performed. Twenty years after the first procedure, a 73-mm left coronary button aneurysm was observed. Due to dense adhesions from repeated surgeries, we approached the aneurysm through the artificial graft lumen, and the coronary artery was successfully reconstructed using Piehler's technique. When performing aortic root surgery for Marfan syndrome, the risk of coronary artery button aneurysm formation should be considered. Once an aneurysm is formed, a surgical strategy that assumes dense adhesions is essential.

Impact of Complete Revascularization on Long-Term Outcomes After Coronary Artery Bypass Grafting in Patients With Left Ventricular Dysfunction.

BACKGROUND: The long-term outcomes of complete revascularization (CR) in patients with left ventricular (LV) dysfunction undergoing coronary artery bypass grafting (CABG) remain unclear. Methods and Results: We evaluated a consecutive series of 111 patients with LV ejection fraction ≤35% who underwent isolated first-time CABG: 63 underwent CR and 48 underwent incomplete revascularization (IR). At a median follow-up of 10.1 years, the rates of death from any cause, cardiac death, and major adverse cardiac and cerebrovascular events (MACCE) were significantly greater in the IR group. After adjusting for propensity score, no significant difference was found between the CR and IR groups regarding death from any cause (hazard ratio [HR], 1.45; 95% CI: 0.75-2.81; P=0.271) and cardiac death (HR, 1.45; 95% CI: 0.68-3.10; P=0.337). In contrast, IR increased the risk of MACCE (HR, 1.92; 95% CI: 1.08-3.41; P=0.027), which was principally attributed to an increased risk of repeat revascularization (HR, 3.92; 95% CI: 1.34-11.44; P=0.013). CONCLUSIONS: Although IR was not significantly associated with an increased risk of long-term mortality in patients with LV dysfunction who underwent CABG, CR might reduce the risks of repeat revascularization and subsequent MACCE.

Long-term outcome after the original and simple modified technique of valve-sparing aortic root reimplantation in Marfan-based population, David V University of Tokyo modification.

BACKGROUND: In valve-sparing aortic root replacement (VSARR), how to reproduce Valsalva sinus has been an issue. In the original David V procedure, they put plication stitches at sinotubular junction level, although the reefing effect is limited and distal graft remains larger than native. Other modified techniques are two-grafts technique and ready-made Valsalva graft. However, the former needs graft-graft anastomosis and may not be cost-effective, while in the latter, the shape of sinus is fixed and minor adjustment is difficult. David V University of Tokyo modification (David V-UT) is our original solution to that, creating pseudosinus with one straight graft by longitudinal size-reduction running sutures above each pseudosinus. The purpose of the present study is to investigate long-term outcome of David V-UT. METHODS: We analyzed 59 David V-UT patients from February 2004 to February 2013 and long-term outcomes were investigated by Kaplan-Meier methods. Risk factors for adverse events "death or recurrent aortic insufficiency (AI) with or without aortic valve reoperation" were analyzed by using Cox proportional hazard models. RESULTS: Mean age was 33.1±14.5 years, and 38 patients (64%) were male. Marfan syndrome (MFS) accounts for 47 patients (80%). Only one patient was with bicuspid aortic valve. No in-hospital mortality was observed. Mean follow-up was 4.9±2.4 years. Estimated survival was 94.0±3.4% at 5 years. Freedoms from aortic valve reoperation and recurrent AI greater than mild were 95.7±3.0% and 88.9±4.7% at 5 years, respectively. In Cox proportional hazard analysis, preoperative AI greater than mild and Z score of annular diameter were significant risks for adverse events (p=0.027 and 0.045, hazard ratio 6.084 and 1.432, 95% C.I. 1.225-30.21 and 1.008-2.035, respectively). CONCLUSIONS: Even in Marfan-characterized population, David V-UT provided satisfactory long-term outcome, comparable to other VSARR modifications. It is simple but can freely reproduce trilobed sinus with one straight graft.

[Endovascular stent-grafting for rupture of descending aorta with a patient with severe arterial occlusive disease; report of a case].

A patient was a 63-year-old woman with poor cardiac function and systemic arterial occlusive disease, who complained of severe back pain. Computed tomography (CT) revealed a rupture of the descending aorta. Initially, abdominal aortic approach through open laparotomy was attempted, but a guide wire caused local aortic dissection. Then, after median sternotomy, endovascular stent was successfully deployed through the ascending aorta. Postoperative course was uneventful. Follow-up CT showed no endoleak. A less invasive endovascular repair would be an alternative treatment for high risk patients such as this case, but several access routes should be considered, especially in a patient with systemic arterial occlusive disease.

Aortic arch rerouting and OPCAB in a patient with situs inversus totalis.

Endovascular therapy approaches for aortic aneurysm have lowered the mortality and morbidity rates even in high-risk patients; moreover, these approaches are applied in the management of aortic arch pathologies by transposition of the supra-aortic branches. We present the case of a 75-year-old female patient with situs inversus totalis on hemodialysis. The patient underwent off-pump aortic arch rerouting and thoracic endovascular aortic repair concomitant with coronary artery bypass grafting for distal aortic arch aneurysm and ischemic heart disease.

Risk model of cardiovascular surgery in 845 Marfan patients using the Japan adult cardiovascular surgery database.

The aim of this study was to evaluate the short-term operative results of patients with Marfan syndrome who underwent thoracic or abdominal aortic surgery in a 4-year period in Japan. Data were collected from the Japan Cardiovascular Surgery Database (JCVSD). We retrospectively analyzed the data of 845 patients with Marfan syndrome who underwent cardiovascular surgery between January 2008 and January 2011. Logistic regression was used to generate risk models. The early mortality rate was 4.4% (37/845). Odds ratios (OR), 95% confidence intervals (CI), and P values for structures and processes in the mortality prediction model were as follows: renal insufficiency (OR, 11.37; CI, 3.7234.66; P < 0.001); respiratory disorder (OR, 11.12; CI, 3.20-38.67; P < 0.001); aortic dissection (OR, 13.02; CI, 2.8060.60; P = 0.001); pseudoaneurysm (OR, 11.23; CI, 1.38-91.66; P = 0.024); thoracoabdominal aneurysm (OR, 2.67; CI, 1.22-5.84; P = 0.014); and aorticrupure (OR, 4.23; CI, 1.26-14.23; P = 0.002). The mortality prediction model had a Cindex of 0.82 and a Hosmer-Lemeshow P value of 0.56. In conclusion, this study demonstrated that renal insufficiency and respiratory disorder had great impact on the operative mortality of Marfan patients undergoing cardiovascular surgery. Because patients with aortic dissection or aortic rupture showed high operative mortality, close follow-up to avoid emergency operation is mandatory to improve the operative results. Achieving good results from surgery of the thoracoabdominal aorta was quite challenging, also in Marfan patients.

Intraoperative assessment for mitral valve competency in a beating heart under retrograde coronary perfusion.

Intraoperative assessment of a repaired mitral valve is of paramount importance for reparative mitral surgery. From September 2010 through November 2012, 20 consecutive patients underwent mitral valve plasty for mitral regurgitation. The patients who underwent surgery after June 2012 received assessment of the repair with the heart beating (HB group, n = 10), and the patients who underwent the operation before May 2012 were assessed for the repair only under cardioplegic heart arrest (non-HB group, n = 10). Intermittent cold retrograde blood cardioplegia was used in all patients. In the HB-group, after completion of the procedures, pump blood without a crystalloid additive was delivered into the coronary sinus. The function of the mitral valve was assessed under beating conditions. There were no differences between the two groups in aortic cross clamp time and operation time, although operative and concomitant procedures were slightly more complicated in the HB group than in the non-HB group. Postoperative echocardiography revealed none or mild mitral regurgitation in all the patients in both groups. Reopening of the closed left atrium for additional repair was necessary only in one patient in the HB group and 3 patients in the non-HB group. In conclusion, the method of perfusing the myocardium retrogradely via the coronary sinus with warm blood is safe and effective for assessing the competency of the mitral valve in a beating heart.

Circulating transforming growth factor ß-1 level in Japanese patients with Marfan syndrome.

Marfan syndrome (MFS) is an inherited connective tissue disorder mainly caused by the fibrillin-1 mutation. Deficient fibrillin-1 is thought to result in the failed sequestration of transforming growth factor ß (TGFß) and subsequent activation of the TGFß signaling pathway, suggesting that the circulating TGFß level may be elevated in MFS, although its accurate measurement is complex due to ex vivo release from platelet stores upon platelet activation. We measured the plasma TGFß1 levels of 32 Japanese MFS patients (22 medically untreated, 10 treated, 20 males, 30.1 ± 9.6 years old) and 30 healthy volunteers (19 males, 29.5 ± 5.8 years old) by ruthenium-based electrochemiluminescence platform (ECL). PF4 was also measured by enzyme immunoassay (EIA) as a platelet degranulation marker. There was no significant difference in the mean plasma TGFß1 level between the MFS group (1.31 ± 0.40 ng/mL) and controls (1.17 ± 0.33 ng/mL) (P = 0.16, NS). Also, there was no significant difference between the untreated (1.24 ± 0.37 ng/mL) and treated (1.46 ± 0.45 ng/mL) MFS patients (P = 0.15, NS). We also measured PF4, which showed wide deviations but no significant difference between the two groups (P = 0.50). A difference in circulating TGFß1 levels between MFS patients and controls was not detected in this Japanese population. Circulating TGFß1 is not a diagnostic and therapeutic marker for Japanese MFS patients, although our findings do not eliminate the possible association of TGFß with the pathogenesis of MFS.

[UT modification of the "David-V" procedure].

Valve-sparing aortic root reimplantation with creation of pseudosinuses, so-called "David-V" procedure,is a promising surgical choice to treat annuloaortic ectasia (AAE). We have developed a simple modification of this procedure, which facilitates exposure and also enables good adjustment of the native aortic root anatomy and the graft. In this article we describe our original technique and its mid-term results.

Evaluating Japanese patients with the Marfan syndrome using high-throughput microarray-based mutational analysis of fibrillin-1 gene.

Marfan syndrome (MS) is an inherited connective tissue disorder, and detailed evaluations of multiple organ systems are required for its diagnosis. Genetic testing of the disease-causing fibrillin-1 gene (FBN1) is also important in this diagnostic scheme. The aim of this study was to define the clinical characteristics of Japanese patients with MS and enable the efficient and accurate diagnosis of MS with mutational analysis using a high-throughput microarray-based resequencing system. Fifty-three Japanese probands were recruited, and their clinical characteristics were evaluated using the Ghent criteria. For mutational analysis, an oligonucleotide microarray was designed to interrogate FBN1, and the entire exon and exon-intron boundaries of FBN1 were sequenced. Clinical evaluation revealed more pulmonary phenotypes and fewer skeletal phenotypes in Japanese patients with MS compared to Caucasians. The microarray-based resequencing system detected 35 kinds of mutations, including 23 new mutations. The mutation detection rate for patients who fulfilled the Ghent criteria reached 71%. Of note, splicing mutations accounted for 19% of all mutations, which is more than previously reported. In conclusion, this comprehensive approach successfully detected clinical phenotypes of Japanese patients with MS and demonstrated the usefulness and feasibility of this microarray-based high-throughput resequencing system for mutational analysis of MS.

Use of DuraHeart® support for more than 1 year as the first successful bridge to heart transplantation in Japan.

We report the first successful case in Japan of use of a DuraHeart(®) for 437 days as a bridge to heart transplantation. A 55-year-old woman developed dilated cardiomyopathy at the age of 47. Her heart condition gradually deteriorated, and she became dependent on catecholamine support, therefore a DuraHeart(®) was implanted. She had an uncomplicated postoperative course, and was discharged on postoperative day (POD) 54. Only once, the alarm of the controller of the DuraHeart(®) gave a problem, and had to be replaced. She had no adverse events while she awaited heart transplantation as an outpatient. She underwent heart transplantation on POD 437. Strong adhesion of the drive line to the liver and peritoneal membrane made resection difficult. No thrombus formation was found inside the DuraHeart(®), but a wedge thrombus was observed around the inflow cannula in the left ventricle. She was discharged on POD 38 after the transplantation.