Clinical and Sociodemographic Factors Associated With Health-Related Quality of Life in Patients With Adult Congenital Heart Disease　- A Nationwide Cross-Sectional Multicenter Study.

BACKGROUND: Little is known about clinical or sociodemographic factors that influence health-related quality of life (HRQoL) in patients with adult congenital heart disease (ACHD).Methods and Results: We conducted a nationwide prospective cross-sectional multicenter study at 4 large ACHD centers in Japan. From November 2016 to June 2018, we enrolled 1,223 ACHD patients; 1,025 patients had an HRQoL score. Patients completed a questionnaire survey, including sociodemographic characteristics, and the 36-Item Short-Form Health Survey (SF-36). To determine factors associated with HRQoL, correlations between 2 SF-36 summary scores (i.e., physical component score [PCS] and mental component score [MCS]) and other clinical or sociodemographic variables were examined using linear regression analysis. In multivariable analysis, poorer PCS was significantly associated with 11 variables, including older age, higher New York Heart Association class, previous cerebral infarction, being unemployed, and limited participation in physical education classes and sports clubs. Poorer MCS was associated with congenital heart disease of great complexity, being part of a non-sports club, current smoking, and social drinking. Student status and a higher number of family members were positively correlated with MCS. CONCLUSIONS: This study demonstrates that HRQoL in ACHD patients is associated with various clinical and sociodemographic factors. Further studies are needed to clarify whether some of these factors could be targets for future intervention programs to improve HRQoL outcomes.

Changes in the Quality of Life of Patients with Left Ventricular Assist Device and their Caregivers in Japan: Retrospective Observational Study.

Left ventricular assist devices (LVAD) improve quality of life (QOL) in many patients with end-stage severe heart failure, but not in some patients. In addition, the burden on caregivers is expected to increase after LVAD patients are discharged. Our study aimed to investigate the impact of LVAD on the QOL of patients and caregivers. Thirty-two LVAD patients were assessed for changes in QOL, mental status, and activity level using the Euro QOL (EQ-5D-5L), Short Form 12 (SF-12), Minnesota Living with Heart Failure Questionnaire, Hospital Anxiety and Depression Scale (HADS), and Frenchay Activities Index. Twenty-four caregivers were assessed for changes in QOL, mental status, and burden of care using the EQ-5D-5L, SF-12, HADS, and Burden Index of Caregiver (BIC-11). The LVAD patients and caregivers responded contemporaneously regarding two points: pre-and post-LVAD. Patients' physical and mental QOL was significantly improved, but not social QOL and activity level. Caregivers' QOL and burden of care did not change, and anxiety was reduced (p = 0.028). The patients were divided into two groups based on whether EQ-5D-5L was improved: twelve patients in the unimproved group (UG) and twenty patients in the improved group (IG). In the UG, 50% had LVAD-related strokes (p = 0.001, IG: 0%), and their social QOL decreased (p = 0.023). The activity levels improved in the IG. Multi-dimensional analyses on the QOL in LVAD patients yielded mixed results. Anticipated benefits derived from LVAD therapy may be limited by LVAD-related complications such as stroke that negatively impacts on the QOL.

The incidence, risk factors, and outcomes of gastrointestinal bleeding in patients with a left ventricular assist device: a Japanese single-center cohort study.

Continuous flow-left ventricular assist devices (CF-LVADs) have become a therapeutic option in the management of advanced heart failure. Several studies show that patients with CF-LVAD are at an increased risk of gastrointestinal bleeding (GIB). However, few reports have presented the characteristics of GIB in Japanese populations. We investigated the incidence, etiology, and outcome of GIB in patients with CF-LVAD. Records of adult patients who received CF-LVADs between October 2008 and January 2017 were reviewed. GIB was defined as detection of bleeding sites by any type of diagnostic imaging. 54 patients received CF-LVAD, of which eight (14%) presented with overt GIB (12 events). GIB patients are significantly older (p = 0.04) and their pre-operative inferior vena cava diameter was larger (p = 0.02). Multivariate analysis revealed that the use of Jarvik 2000 (p = 0.003) was a risk factor for GIB. In total, 85.8% of patients were free from GIB at 1 year. The most common site was the small intestine (67%). The most common cause was angiodysplasia (50%). Six patients required blood transfusion (nine events) and four underwent endoscopic clippings (five events); however, no patients needed surgeries. The incidence of GIB in our cohort was similar to the global registry data. Double balloon endoscopy is useful for diagnosis and treatment of small intestinal lesions. Future efforts to further understand the incidence of GIB in Japanese populations by multicenter data are needed.

[Effects of Olmesartan Medoxomil on Patients with Thoracic and Thoracoabdominal Aortic Aneurysm;Evaluation of Anti-hypertensive Effect and Possible Suppression of Aneurysmal Dilation( OLM 40 Study)].

We evaluated the blood pressure( BP) lowering effect and possible suppression of aortic enlargement by olmesartan (OLM) in patients with thoracic and thoracoabdominal aortic aneurysm. In this single center prospective, forced titration study, 50 patients were registered between 2008 and 2011. After all patients received any of OLM 10, 20, and 40 mg/day as an initial dose, the dosage of OLM was titrated up to 40 mg as needed during follow-up period. Home BP (HBPs), aortic aneurysm size assessed by computed tomography (CT) scan, indices of renal function were recorded at 3- and 6-months follow-up. Depending on whether 40 mg/day of prescription was continued for more than 4 months or not, the patients were divided into 2 groups:less than 40 mg (<40 mg) and 40 mg groups. Morning HBPs tended to decrease in both groups, and the percent changes in BPs were essentially the same regardless of dosage. The absolute value of aortic diameter tended to slightly enlarge only in <40 mg group. Also in the <40 mg group, the absolute differences in aortic diameter between those at the time of study registration and each follow-up were 0.5±1.8 mm at 3-month and 1.2±2.3 mm at 6-month (p=0.047),whereas the percent changes were 0.9±3.3% and 2.2±4.5% at 3 and 6 months, respectively( p=0.058). As for 40 mg group, the absolute differences and percent changes did not reach statistically significant increase during the follow-up period. No severe renal dysfunction related to OLM 40 mg prescription was observed. Our results imply that OLM 40 mg may suppress aortic aneurysmal dilation independently of blood pressure lowering effect. Further study with larger number of sample size is warranted to assure this observation.

[Splenic Injury during Replacement of the Thoracoabdominal Aorta].

Extraperitoneal approach is commonly employed for thoracoabdominal aortic repair via Stoney incision. It is supposedly rare to encounter abdominal visceral bleeding during that procedure. However, the spleen may spontaneously adhere to the adjacent peritoneum, which could induce incidental injury to the spleen by its anterior mobilization during extraperitoneal approach. Unless we bare its potential risk in mind, bleeding from the spleen may be overlooked, which results in hemodynamic deterioration. We have experienced 3 cases of splenic injury that necessitated hemostatic maneuvers for bleeding during and just after the thoracoabdominal aortic repair.

Evidence of Pulmonary Vascular Reverse Remodeling After Pulmonary Artery Banding Performed in Early Infancy in Patients With Congenital Heart Defects.

BACKGROUND: Histomorphometric evidence of the effect of pulmonary artery banding (PAB) in infancy on pulmonary vascular reverse remodeling has not been fully described.Methods and Results:We retrospectively reviewed 34 patients who underwent serial lung biopsies before and after PAB.Index of pulmonary vascular disease (IPVD) as a measure of the degree of progression of pulmonary arteriopathy significantly decreased after PAB (1.22±0.25 at 1st and 1.13±0.21 at 2nd biopsy, P=0.04). Additionally,DR=100 µmas an indicator of medial thickness of pulmonary arteries significantly decreased after PAB (15.6±3.7 at 1st and 11.4±2.6 at 2nd biopsy, P<0.0001). Patients were divided into 3 groups by age at PAB: <3 months (Group 1), between 3 and 6 months (Group 2), and >6 months (Group 3). The average secondDR=100 µmof groups 1 and 2 was significantly lower than that of group 3 (11.1±2.2 and 9.8±2.0 vs. 14.9±2.8, respectively; P<0.0001). Additionally, the second IPVD was also significantly lower in groups 1 and 2 than in group 3 (1.1±0.2 and 1.1±0.2 vs. 1.3±0.4, respectively; P=0.02). CONCLUSIONS: Histomorphometric evidence of post-PAB pulmonary vascular reverse remodeling is robust. The magnitude of vascular reversibility is pronounced when PAB is performed before 6 months of age.

Is Trisomy 21 a Risk Factor for Rapid Progression of Pulmonary Arteriopathy?　- Revisiting Histopathological Characteristics Using 282 Lung Biopsy Specimens.

BACKGROUND: Pulmonary hypertension (PH) is more progressive in trisomy 21 patients. However, pulmonary arteriopathic lesions in these patients have not been fully characterized histopathologically.Methods and Results:A retrospective review of a lung biopsy registry identified 282 patients: 188 patients with trisomy 21 (Group D) and 94 without (Group N). The mean age at lung biopsy was 3 and 7 months (P<0.0001). Pulmonary arterial pressure (PAP) and pulmonary vascular resistance were similar between the 2 groups. There were no significant differences in the proportion of patients with irreversible intimal lesions or the index of pulmonary vascular disease (IPVD; a measure of the degree of pulmonary arteriopathy progression) between the 2 groups. In addition, after propensity score matching for patient background (n=43 in each group), there were no significant differences in IPVD (P=0.29) or the ratio of irreversible intimal changes between the D and N groups (P=0.39). Multivariate analysis identified age (P<0.0001) and PAP (P=0.03) as the only risk factors for progression of pulmonary arteriopathy. CONCLUSIONS: Histopathologically, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Although we cannot exclude the possibility of bias in the Group D and N patients who were slated for lung biopsy, factors other than pulmonary arteriopathy may affect the marked progression of clinical PH in trisomy 21 patients.

Application of actuator-driven pulsed water jet for coronary artery bypass grafting: assessment in a swine model.

Actuator-driven pulsed water-jet (ADPJ) dissection is an emerging surgical method for dissecting tissue without heat and mechanical injury to vessels. We elucidated the mechanical properties of the piezo ADPJ and evaluated its usefulness and safety in coronary artery bypass grafting procedures. The relationship between the input voltage (10-100 V) and peak pressure of the pulsed water jet was evaluated. The tissue strengths of swine internal thoracic and coronary arteries and the surrounding tissues were measured to assure tissue-selective dissection. Internal thoracic arteries were harvested by conventional electric cautery and the water jet in four swine, and eight coronary arteries surrounded by myocardium were attempted to be exposed with the water jet. The dissected specimens were histologically evaluated. The peak pressure of the pulsed water jet was positively correlated with the input voltage (R 2 = 0.9984, P < 0.001). The breaking strengths of the target vessels (internal thoracic and coronary arteries) and the surrounding tissues were significantly different (P = 0.002 and P < 0.001, respectively). Histologic examination revealed that internal thoracic arteries were isolated with less heat damage using the pulsed water jet (P = 0.002) compared with electric cautery, and coronary arteries also were dissected without apparent histologic damage. ADPJ has the possibility of assuring tissue selectivity among the internal thoracic and coronary arteries. The results also indicated that the use of ADPJ may enhance safe procedures to harvest grafts during coronary artery bypass grafting.

Predictors of the need for pacemaker implantation after the Cox maze IV procedure for atrial fibrillation.

PURPOSE: The Cox maze IV (CMIV) procedure is being used increasingly frequently for surgical ablation of atrial fibrillation (AF). This study aimed to identify the risk factors of the need for postoperative pacemaker implantation (PMI) after CMIV. METHODS: Preoperative, intraoperative, and postoperative data were retrospectively collected from 67 consecutive patients who underwent CMIV at our institution; 7 (10.4%) required PMI (as a treatment of brady AF or sick sinus syndrome). RESULTS: Patients who needed PMI tended to have lower preoperative heart rates than those who did not on a 12-lead electrocardiogram (ECG; 68.7 ± 11.6 vs. 79.1 ± 18.5 bpm, p = 0.07) and a 24-h ECG (94,772 ± 9800 vs. 109,854 ± 19,078 beats/day, p = 0.03). A multivariate analysis identified a low amplitude of the fibrillatory wave on preoperative ECG as a risk factor of PMI necessity after CMIV [odds ratio = 14.7; 95% confidence interval (CI) 1.9-324.7; p = 0.007] and internal use of angiotensin-converting enzyme inhibitors or angiotensin receptor blockers (ACEIs/ARBs) as a negative risk factor (odds ratio = 0.16; 95% CI 0.02-0.99; p = 0.049). CONCLUSIONS: A low amplitude of the fibrillatory wave was identified as a risk factor of PMI necessity, whereas the internal use of ACEIs/ARBs diminished the need for PMI. These factors should be considered before CMIV is performed.

The impact of liver disorders on perioperative management of reoperative cardiac surgery: a retrospective study in adult congenital heart disease patients.

PURPOSE: We evaluated the preoperative prevalence of risk factors for liver disorders and the relationship between the liver disorders and perioperative outcomes in adult congenital heart disease (ACHD) patients. METHODS: This retrospective study included 32 ACHD patients who underwent reoperative cardiac surgery. RESULTS: Preoperatively, 38% of the study patients had risk factors, including congestive liver (CL) due to right heart failure (31%), chronic hepatitis C (HC) (22%), and both CL and HC (16%). The numbers of patients with Child-Pugh scores 5, 6, 7 and 8 were 22, 7, 2 and 1. Median (range) preoperative platelet count and fibrinogen values were 155 (61-330) × 103/µl and 250 (145-367) mg/dl, respectively. The patients with higher Child-Pugh scores tended to have longer duration of anesthesia and surgery (p = 0.078, 0.078, respectively), and had significantly higher platelet transfusion (p = 0.031). Lower platelet count was associated with longer duration of anesthesia, surgery and cardio pulmonary bypass (CPB), and larger amount of blood loss and platelet transfusion (p = 0.01, 0.011, 0.024, 0.033, 0.021). Lower fibrinogen value was associated with longer duration of anesthesia, surgery and CPB, and larger amount of platelet transfusion (p = 0.015, 0.009, 0.009, 0.023). CONCLUSION: ACHD patients who underwent reoperative cardiac surgery had a high prevalence of risk factors for liver disorders preoperatively, and liver disorders aggravated some intraoperative outcomes. These findings suggest that the prevention of liver disorders is important for reducing the occurrence of poor outcomes, and that ACHD patients with liver disorders need attentive perioperative management.

[Six Cases of Anesthetic Management of Adult Patients Undergoing Coarctation of Aorta Repair].

BACKGROUND: In Japan, every year more than 9,000 patients with congenital heart disease reach adulthood due to improved medical treatments. Adult coarctation of aorta (CoA) patient is likely to need surgery for re- CoA, progressive CoA and/or aortic aneurysm. There- fore, it is necessary to consider anesthetic management of complicated aortic surgery of CoA patient Methods: This is a retrospective study of six pa- tients with CoA who underwent the CoA repair sur- gery in adulthood from 2005 to 2013. RESULTS: Pre-operative CoA diameter was 7.8?2.5 mm and pressure gradient was 37±13 mmHg. Four patients had aortic aneurysm. Three patients received lateral thoracotomy and one-lung ventilation; five patients underwent selective cerebral perfusion. As perioperative complications, all two patients with re-CoA surgery had pneumothorax; two of five patients with arch repair surgery had recurrent nerve paralysis; a patient with left subclavian artery ischemia/reperfu- sion, and elevation and flexion of left upper extremity position had left upper extremity paralysis. CONCLUSIONS: In anesthetic management of adult CoA repair, it is important to understand various surgical approach and corresponding ventilation and extracorporeal circulation methods and possible complications.

Poly-2-methoxyethylacrylate-coated cardiopulmonary bypass circuit can reduce transfusion of platelet products compared to heparin-coated circuit during aortic arch surgery.

Several coating techniques for extracorporeal circulation have been developed to reduce the systemic inflammatory response during cardiopulmonary bypass (CPB). We compared the clinical effectiveness and biocompatibility of poly-2-methoxyethylacrylate (PMEA)- and heparin-coated CPB circuits in total aortic arch replacement (TAR) with the prolonged use of the bypass technique. Twenty patients who underwent elective TAR were divided randomly into two equal groups: group P (n = 10) to use PMEA-coated circuits and group H (n = 10) to use heparin-coated circuits. Clinical outcomes, hematological variables, and acute phase inflammatory response were analyzed perioperatively. Demographic, CPB, and clinical outcome data were similar for both groups. Hemoglobin and platelet count showed similar time-course curves. However, the amount of platelet products transfused intraoperatively was significantly larger in group H (group P 26.0 ± 7.0 units; group H 33.0 ± 6.7 units, p = 0.04). Total protein, and albumin levels were significantly higher in group P during and after the operation (total protein, p = 0.04; albumin, p = 0.02). The use of PMEA-coated circuit is associated with retainment of perioperative plasma proteins levels and may help to reduce transfusion of platelet products in TAR in comparison with the heparin-coated circuit.

Surgical Ligation for Patent Ductus Arteriosus in Extremely Premature Infants: Strategy to Reduce their Risk of Neurodevelopmental Impairment.

Surgical ligation for patent ductus arteriosus (PDA) in extremely low birth weight infants (ELBWIs) has been shown a possible association with neurodevelopmental impairment (NDI) because of its invasiveness. However, we have undergone surgical ligation for ELBWIs immediately after cyclooxygenase inhibitor failed to close a hemodynamically significant PDA (hsPDA) to maintain proper systemic circulation. We aimed to determine the effect of surgical ligation for hsPDA on NDI in ELBWIs. In enrolled 71 ELBWIs, the clinical parameters, including the developmental quotient (DQ), were collected and compared among three groups that were divided by closure mode: spontaneous closure (n = 11), cyclooxygenase inhibitor therapy (n = 37) and surgical ligation (n = 23). No significant differences in DQ at the age of 36 months among the three groups were found: Median (interquartile range): 92.0 (31.0), 89.0 (22.0) and 92.0 (24.5), respectively. In a comparison between groups of DQ < 70 (n = 15) and DQ ≥ 70 (n = 56), a significant difference was found in the parameters related to prematurity (p < 0.05 for each): gestational age [23.9 (1.70) vs. 25.4 (2.50) weeks], birth weight [595 (183) vs. 714 (192) g], Apgar score < 5 (1 min) (67% vs. 36%), and laser photocoagulation for retinopathy of prematurity (73% vs. 43%), but there was no significant association with hsPDA. Therefore, we propose that surgical ligation for hsPDA in ELBWIs should be immediately carried out for preventing future neurodevelopmental deterioration if the cyclooxygenase inhibitor failed to close hsPDA.

[Evaluation of Portable Coagulation Analyzer in Comparison with Conventional Hospital Laboratory Instrument with Stratification of International Normalized Ratios by Therapeutic Upper Limit].

Point of care devices have been widely applied to outpatients receiving anticoagulation therapy with warfarin for monitoring prothrombin time-international normalized ratio (PT-INR) regularly. However, accuracy in measurement with the device remains undetermined when PT-INR exceeds therapeutic range. We evaluated the performance of a portable CoaguChek XS coagulation analyzer in comparison with a conventional laboratory method according to therapeutic and supra-therapeutic PT-INR values in cardiac outpatients on oral vitamin K antagonists. All participants were classified into 2 groups on the basis of PT-INR 3.0 by the laboratory method; therapeutic group less than or equal to 3.0 (n=48) and supra-therapeutic group above 3.0 (n=8). The correlation coefficients in therapeutic and in supra-therapeutic groups were r=0.82 and r=0.78, respectively (p<0.05). The difference in PT-INR between the laboratory method and the CoaguChek XS was significantly larger in supra-therapeutic group than therapeutic group (1.03±0.73 versus 0.34±0.26, p=0.042). Our study indicates that CoaguChek XS can be useful handheld coagulation analyzer to determine PT-INR rapidly; however, the device may underestimate PT-INR in supra-therapeutic range.

[Preoperative Use of Tolvaptan in a Patient with Constrictive Pericarditis].

Tolvaptan is a new selective vasopression V2-receptor antagonist. We report our experience with a use of tolvaptan for preoperative fluid management in a patient with severe constrictive pericarditis. A 66-year-old man presented with heart failure symptoms derived from constrictive pericarditis. Chest X-ray showed right pleural effusion and chest computed tomography demonstrated severe pericardial calcification. Despite that he received optimal conventional medical treatments, his hemodynamic condition further exacerbated during hospitalization. We administered tolvaptan in an attempt to optimize preoperative fluid management. Tolvaptan was found to be remarkably effective in that regard. The body weight decreased and the heart failure symptoms improved. Pericardiectomy was performed successfully, and he recovered uneventfully.

[Issues in Coronary Artery Bypass Grafting for Kawasaki Disease].

Progressive narrowing at the entrance and exit of coronary artery aneurysm can develop at late phase of Kawasaki disease (KD). Evaluation and prediction of progressive coronary lesions remain a challenge in the treatment of post-KD coronary artery disease. We aimed to elucidate long-standing issues imposed on the patients who underwent coronary artry bypass grafting (CABG) for coronary artery lesions associated with KD. Between January, 2000 and December, 2013, CABG for coronary artery lesions associated with KD were performed in 6 patients (male/female: 5/1, mean age 14.5±10.0). There was no operative mortality. Follow-up for the 6 patients has been performed with the average period of 5 years (1~9 years). Cardiac events occurred in 2 patients during follow-up. In 1 patient, left internal thoracic artery (LITA) occluded due to flow competition between the native artery and LITA graft after LITA to LAD bypass grafting. The other patient required a re-do CABG using the free right internal thoracic artery to the circumflex branch because of occlusion at the coronary artery aneurysms after 4 years postoperatively. Meticulous preoperative diagnostic evaluations of coronary artery aneurysm may further improve the long-term outcome after surgical intervention for coronary lesions in conjunction with an aneurysm.

[Effectiveness of a Simulation of the Donor Surgery with Other Extraction Teams to Improve Various Problems in Pulmonary Extraction from a Brain-dead Donor].

The pulmonary extraction from a brain-dead donor is one of the important elements for the success of lung transplantation, but the current scarcity of practical training opportunities is a major problem. We performed a simulation of the donor surgery of multiple organs using a pig with other extraction teams to provide more training opportunities. The effectiveness of this simulation lies in its potential to improve the surgical procedure;furthermore, it may solve problems associated with communicating with other extraction teams. However, it is difficult to judge whether the donor lung is suitable for transplantation, as it would be inappropriate to use such a lung for simulation in training. Since this simulation system is considered to be effective to solve various problems in the current donor surgery, it should be available more frequently to improve a technical level of the donor surgery and to aid surgeons in the rapid implementation of next-generation techniques.

[Early Postoperative Outcomes and Evaluation of Hemodynamics after Mitral Valve Replacement with Epic Mitral Bioprosthesis].

BACKGROUND: In Guideline for Surgical and Interventional Treatment of Valvular Heart Disease, revised by Japanese Circulation Society in 2012, mitral valve replacement (MVR) with bioprosthesis is class II b recommendation for patients aged 70 years or older who have no risk factors for thromboembolism. The aim of this study was to evaluate the early postoperative surgical outcomes and the hemodynamic performance with the Epic mitral bioprosthesis. METHODS: Twenty-six consecutive patients underwent MVR with Epic mitral bioprostesis at Tohoku University Hospital between April 2011 and July 2014. Twenty-five cases of 26 were evaluated their hemodynamics at discharge, and of which 19 cases of 26 were evaluated at the outpatient clinic during follow-up period. RESULTS: There was 1 hospital death. Long-term mortality or reoperation for any valve abnormality was not observed in the median follow-up of 23.9 ± 11.3 months. Hemodynamic date at discharge obtained by transthoracic echocardiography included mean hemodynamics of mitral valve bioprosthesis as below. Effective orifice area (EOA):2.44 ± 0.62 cm², peak mitral pressure gradient (pMPG):15.8 ± 5.3 mmHg, mean mitral pressure gradient(mMPG):7.2 ± 2.4 mmHg. Hemodynamic parameters at follow-up were found to be stable as EOA:2.25 ± 0.64 cm², pMPG:17.3 ± 5.7 mmHg, mMPG:6.2 ± 2.3 mmHg, respectively. CONCLUSION: We have attempted to elucidate our preliminary postoperative outcomes and hemodynamics after MVR with Epic mitral bioprosthesis. These in vivo hemodynamic data can serves a clinical reference.

[Two cases of fulminant myocarditis with mesenteric ischemia].

Fulminant myocarditis (FM) can lead to a refractory cardiogenic shock and multiple organ failure. Mechanical circulatory support (MCS) is not infrequency required to salvage patients in a profound shock. Advanced technology in MCS such as a temporary ventricular assist device can yield improved early outcome. However, incessant mesenteric ischemia remains a challenge to be resolved even with MCS. We here-in report 2 cases of FM associated with mesenteric ischemia that was extremely difficult to diagnose and treat. Therapeutic strategy for FM with mesenteric ischemia is discussed.

Anesthetic management of pulmonary valve replacement for pulmonary regurgitation in six patients with surgically repaired tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) encountered in CHD patients surviving into adulthood. A number of patients with surgically repaired TOF have significant pulmonary regurgitation (PR) that can lead to right ventricular (RV) dilatation, RV failure, and arrhythmia. We describe the anesthetic management for pulmonary valve replacement (PVR) in six PR patients with surgically repaired TOF. Although all patients had dilated RVs and depression of RV ejection fraction preoperatively, and arrhythmia and unexpected bleeding perioperatively, they could tolerate a well-managed PVR operation. Anesthesiologists should be aware of the multiplicity of comorbidities, sequelae, and residua in patients with surgically repaired TOF. RV function should be monitored using transesophageal echocardiography, and inotropic vasodilators and alpha-adrenergic agents should be administered, as appropriate. Arrhythmias, vascular injury during removal of adhesions during re-sternotomy, and bleeding from collateral vessels are also frequent complications.