扩张型心肌病心脏再同步化治疗的术中配合与并发症护理

对7例扩张型心肌病患者行心脏再同步化治疗,术中与医生密切的配合与病情观察,严密监测起搏器性能,进行心电监护,预防切口囊袋感染,避免膈神经刺激及电极移位,防止气胸,血性心包的并发症发生。结果本组病例均安全度过围手术期,无并发症发生,起搏功能良好,心衰症状明显改善,生活质量提高。     

Effect of biventricular pacing therapy in patients with dilated cardiomyopathy with severe congestive heart failure

Objectives: Biventricular pacing (BVP) therapy has recently emerged as an effective treatment for patients with moderate to severe congestive heart failure (CHF) and ventricular asynchrony all over the world. However, this therapy is not yet available in Japan. We evaluated the effects of BVP in patients with severe CHF due to dilated cardiomyopathy (DCM). Subjects: Four patients with medically refractory severe CHF due to DCM in New York Heart Association functional class III or IV heart failure underwent BVP therapy. We combined the implantation of the left ventricular (LV) epicardial lead via small thoracotomy following right atrial and ventricular intravenous leads under general anesthesia. We evaluated to determine whether improvements of ventricular function, ventricular size, mitral regurgitation, functional status, frequency of hospitalization, and quality of life were associated with BVP therapy. Results: BVP improved LV systolic function, decreased LV size and mitral regurgitation, and shortened prolonged QRS interval. The patients’ symptoms, exercise tolerance, frequency of hospitalization, and quality of life were also dramatically improved by BVP. Furthermore, combination of BVP and oral administration of amiodarone significantly prevented recurrence of ventricular tachycardia and paroxysmal atrial fibrillation, and maintained sinus rhythm for a long period. Conclusions: In view of these findings, BVP therapy may contribute to the development of new therapeutic method for patients with severe CHF due to DCM.     

上胸段硬膜外阻滞对扩张型心肌病患者血浆纤维蛋白原的影响

目的 探讨上胸段硬膜外阻滞对原发性扩张型心肌病（DCM）患者血浆纤维蛋白原浓度（FIB）的影响。方法 30例DCM患者，年龄26～70岁，心功能（NYHA分级）Ⅲ或Ⅳ级，随机分成治疗组和对照组（n=15）。治疗组L3，4或T4，5棘突间隙穿刺置管于硬膜外腔，每间隔2h硬膜外腔注射0．5％利多卡因3～5ml，辅以适当药物治疗；对照组仅给常规予药物治疗，疗程4周。于治疗前后进行心脏彩超检查，用微量毛细管加热沉淀法测定静脉血血浆FIB浓度。结果 与对照组比较，治疗后治疗组心功能改善，左室舒张末内径减小，左室射血分数增加，血浆FIB浓度降低（P〈0．05）。结论 上胸段硬膜外阻滞能改善DCM患者心功能，降低血浆FIB浓度。     

扩张性心肌病患者骨代谢指标状况及其临床意义

目的探究扩张性心肌病(dilated cardiomyopathy,DCM)患者骨代谢指标水平状况及其临床意义。方法选取2016年3月至2018年3月在我院诊治的DCM患者48例作为研究对象并归入DCM组,选择同期在我院接受健康体检的成年人30名归入健康组,测定和比较两组的血清骨钙素(BGP)、钙(ICa)、骨特异性碱性磷酸酶(BALP)、甲状旁腺激素(PTH)、25羟基维生素D_3[25(OH) D_3]等骨代谢指标水平,以及大粗隆、股骨颈、腰椎等部位的骨密度(BMD),分析骨代谢指标BGP、ICa、BALP、PTH和25(OH) D_3分别与患者年龄、DCM病程、BMI、肌酸激酶同工酶、肌钙蛋白I和心功能分级等因素的相关性。结果(1) DCM组的BGP、ICa、25(OH) D_3等水平均低于健康组,BALP、PTH水平均高于健康组,差异均有统计学意义(P均0.05)。(2) DCM组大粗隆部位的BMD与健康组比较,差异无统计学意义(P0.05); DCM组的股骨颈及腰椎部位的BMD均低于健康组,差异均有统计学意义(P均0.05)。(3) BGP、ICa和25(OH) D_3分别与年龄、DCM病程及心功能分级呈负相关,BGP与肌酸激酶同工酶、肌钙蛋白Ⅰ呈负相关; BALP与年龄、DCM病程、肌酸激酶同工酶、肌钙蛋白Ⅰ及心功能分级呈正相关,PTH与心功能分级呈正相关(P均0.05)。结论 DCM患者的血清BGP、ICa、BALP、PTH、25(OH) D_3等骨代谢相关指标水平有明显波动,且骨代谢相关指标水平与DCM患者年龄、病程、肌酸激酶同工酶、肌钙蛋白Ⅰ及心功能分级等相关,DCM可能是造成骨代谢指标水平发生异常的重要原因。     

Cardiac arrest upon induction of anesthesia in children with cardiomyopathy: an analysis of incidence and risk factors

Introduction: It is thought that patients with cardiomyopathy have an increased risk of cardiac arrest on induction of anesthesia, but there is little available data. The purpose of this study was to identify the incidence and potential risk factors for cardiac arrest upon induction of anesthesia in children with cardiomyopathy in our institution. Methods: A retrospective chart review was performed. Eligible patients included patients admitted between 1998 and 2008 with the International Statistical Classification of Disease code for cardiomyopathy (ICD‐9 code 425) who underwent airway intervention for sedation or general anesthesia in the operating room, cardiac diagnostic and interventional unit (CDIU) or intensive care unit. Patients undergoing emergency airway intervention following cardiovascular collapse were excluded. For each patient, we recorded patient demographics, disease severity, anesthesia location, and anesthetic technique. Results: One hundred and twenty‐nine patients with cardiomyopathy underwent a total of 236 anesthetic events, and four cardiac arrests were identified. One was related to bradycardia (HR < 60), two were attributed to bradycardia in association with severe hypotension (systolic blood pressure < 45), and the fourth arrest was related to isolated severe hypotension. Two occurred in the operating suite and two in the CDIU. There was no resulting mortality. One patient progressed to heart transplantation. Multiple combinations of anesthetic drugs were used for induction of anesthesia. Conclusion: We performed a review of the last 10 years of anesthesia events in children with cardiomyopathy. We report four cardiac arrests in two patients and 236 anesthetic events (1.7%). To the best of our knowledge, this is the largest review of these patients to date but is limited by its retrospective nature. The low cardiac arrest incidence prevents the identification of risk factors and the development of a cardiac arrest risk predictive clinical tool.     

Histopathologic findings in explanted heart tissue from patients with end-stage idiopathic dilated cardiomyopathy

Explanted hearts were examined to determine whether specific histopathologic features are present in the myocardium of patients with end-stage idiopathic dilated cardiomyopathy (IDC). Extensive histopathologic examination by light microscopy, electron microscopy and immunohistochemistry revealed marked fibrosis in the hearts of 21 of 37 IDC patients and in 26 of 35 patients with heart diseases of known causes. Reactive (interstitial and perivascular) fibrosis predominated in the IDC hearts, whereas both reparative (replacement) fibrosis and reactive fibrosis were found in the comparison group. Endocardial fibroelastosis was found in nine patients with IDC and in 14 patients from the comparison group. Distinct patterns of fibrosis were the sole significant histopathologic difference between myocardial samples from patients with IDC and from those with heart diseases of known causes. The diffuse presence of reactive fibrosis in IDC patients suggests a more generalised dysfunction that affects the composition of the myocardial extracellular matrix. Received: 8 May 2000 Revised: 1 February 2001 Accepted: 8 June 2001     

Bilateral subthalamic stimulation in patients with Parkinson disease: long-term follow up

OBJECT: Bilateral subthalamic nucleus (STN) stimulation is increasingly used in patients with advanced Parkinson disease (PD). This study was performed to evaluate the long-term efficacy and safety of bilateral STN stimulation in cases of PD. METHODS: The authors performed a prospective, open-label study in patients with PD who underwent bilateral STN stimulation. The authors compared motor scores and activities of daily living (ADL) scores based on the Unified PD Rating Scale (UPDRS) obtained before surgery while patients were in the medication-off state with scores obtained at follow-up evaluations of these patients while in the medication-off/stimulator-on state. Data contained in patient diaries were also compared. Thirty-three patients with PD were evaluated 12 months postoperatively and 19 were evaluated at a mean follow-up time of 28 months. A comparison between UPDRS scores obtained in patients in the medication-off/stimulator-on state and those obtained when patients were in the baseline medication-off state showed a 27% improvement in ADL scores and a 28% improvement in motor scores after surgery. There was a 57% reduction in the use of levodopa-equivalent medication doses. The percentage of the waking day that patients were in the medication-on state increased from 38 to 72%. Surgical complications included seizures (three patients), confusion (five patients), hemiballismus (one patient), and visual disturbance (one patient). Stimulation-related adverse effects were mild. Device-related events included nine lead replacements, seven lead revisions, six extension replacements, and 12 implantable pulse generator (IPG) replacements; one IPG was cleaned and one IPG was placed in a pocket because of the presence of a shunt. CONCLUSIONS: Bilateral STN simulation is associated with a significant improvement in the motor features of PD. Device-related events were common in the first 20 patients who underwent surgery, often requiring repeated surgeries.     

Combined liver-kidney transplantation: long-term follow up in 18 patients

Abstract Since August 1992, 18 patients underwent combined liver and kidney transplantation. Eight patients had lymphocytotoxic antibodies pretransplant and 5 of these patients (27.7%) had a positive crossmatch. Fifteen patients received cyclosporine-based immunosuppression and 3 patients were treated with a tacrolimus-based immunosuppressive protocol. One patient died in the postoperative course due to intractable bleeding episodes after 96 days and one kidney graft was lost due to technical complications. The 1-year survival rate of patients with combined transplantation was 95% vs 87% in patients with liver transplantation alone. None of the patients with a positive crossmatch experienced a hyperacute rejection of the kidney. The long-term patient and graft survival was not impaired in patients with a positive crossmatch. These results suggest that combined liver-kidney transplantation is a safe treatment for enD-stage liver and renal disease. A positive crossmatch or positive lymphocytotoxic antibodies are not contraindications for a combined transplantation.     

Reversal of cardiomyopathy in propionic acidemia after liver transplantation: a 10‐year follow‐up

Cardiomyopathy is a frequent complication in propionic acidemia. It is mostly rapidly fatal and independent of the metabolic control or medical intervention. Here, we present the reversal of a severe cardiomyopathy after liver transplantation in a patient with propionic acidemia and the long‐term stability after ten years. Liver transplantation in patients with propionic acidemia may be considered a valid and long‐lasting treatment when cardiomyopathy is progressive and unresponsive to medical therapy.     

Chronotropic incompetence in Chagas disease: effectiveness of blended sensor (volume/minute and accelerometer)

Introduction

Technological progress of pacemakers has allowed the association of two or more sensors in one heart rate system response. The accelerometer sensor measures the intensity of the activity; it has a relatively rapid response to the beginning of it, however, it may present insufficient response to less strenuous or of less impact exercise. The minute ventilation sensor changes the pacing rate in response to changes in respiratory frequency in relation to tidal volume, allowing responses to situations of emotional stress and low impact exercises.

Objective

To evaluate the cardiorespiratory response of the accelerometer with respect to the blended sensor (BS=accelerometer sensor+minute ventilation sensor) to exercise in chagasic patients undergoing cardiopulmonary exercise test.

Methods

This was a prospective, observational, randomized, cross-sectional study. Patients who met the inclusion criteria were selected. The maximum heart rate of the sensor was programmed by age (220-age). The results were analyzed through t test with paired samples (P<0.05).

Results

Sample was comprised of 44 patients, with a mean age of 66±10.4 years, 58% were female, 54% as first implant, in 74% were functional class I and 26% were functional class II, left ventricular ejection fraction was 58±7. As for the cardiopulmonary test, maximum expected heart rate and VO2 were not achieved in both the accelerometer sensor and the blended sensor, however, metabolic equivalent in the blended sensor was higher than the expected, all data with P<0.001.

Conclusion

Even though the maximal heart rate was not reached, the blended sensor provided a physiological electrical sequence when compared to the accelerometer sensor, providing better physical fitness test in cardiopulmonary hemodynamics and greater efficiency.     

Echocardiographic and clinical findings in patients with Fabry disease during long-term enzyme replacement therapy: a nationwide Danish cohort study

Objectives: In patients with Fabry disease (FD), left ventricular hypertrophy and arrhythmias are frequently observed and cardiac involvement is the leading cause of death. Long-term efficacy of enzyme replacement therapy (ERT) on cardiac involvement is unclear. We assessed and compared long-term progression of cardiac involvement according to ERT and non-ERT. Methods: We retrospectively assessed and compared long-term progression of cardiac involvement in adult patients with FD in the nationwide Danish cohort. We followed clinical signs, symptoms and findings by echocardiography, electrocardiography and Holter-monitoring. Results: We included 66 patients; 47 patients (27 women) received ERT (ERT group) and 19 patients (15 women) did not (non-ERT group). The groups were followed for a median of 8 [0–12] years and 6 [0–13] years, respectively. Comparison between ERT and non-ERT receiving patients by left ventricular mass (echocardiographic assessment) and Sokolow-Lyon voltage- and Cornell product criteria (electrocardiographic assessment) revealed no significant differences. In the ERT group, we observed no change in left ventricular mass but a decrease in Sokolow-Lyon voltage- and Cornell product criteria from baseline to follow-up; 30?mm [15–53] vs. 25?mm [3–44], p?p?Discussion: We raise concerns regarding the efficacy and benefit of ERT on cardiac involvement in Fabry disease and stress the need for further research.     

Cardiac troponin I (2nd generation assay) in chronic haemodialysis patients: prevalence and prognostic value.

BACKGROUND: Elevated serum cardiac troponin T (cTnT) levels are frequently observed in chronic dialysis patients and have been shown to be associated with increased morbidity and mortality. The aim of this study was to determine whether cardiac troponin I (cTnI), which is less frequently elevated, has similar clinical significance. METHODS: We studied 101 asymptomatic patients with no clinical evidence of coronary artery disease who were undergoing chronic dialytic treatment. We measured their serum cTnI levels immediately before the start of their dialysis sessions by a second-generation assay (OPUS-DADE). Our study included a year-long follow-up with trimestrial cTnI assays as well as clinical, X-ray and echocardiographic surveillance. We considered patients with serum cTnI > or =0.15 ng/ml as positive and those with levels <0.15 ng/ml as negative. RESULTS: Among the 14 patients with high serum cTnI levels, nine (64%) suffered acute cardiac events during the 12-month follow-up. In contrast, among the 72 patients with low cTnI levels only seven (9.7%) had acute events. In another group of 15 patients with variable cTnI levels, three patients (20%) had cardiac events. CONCLUSION: Based on these results, serum cTnI appears to be a valuable predictive marker of cardiovascular events in asymptomatic dialysis patients. For those patients who might benefit from thorough cardiac investigation and treatment, information on cTnI could be useful in preventing cardiac events.     

Cardiovascular complications caused by advanced secondary hyperparathyroidism in chronic dialysis patients; special focus on dilated cardiomyopathy

Background The frequency and prognosis of dilated cardiomyopathy (DCM) caused by secondary hyperparathyroidism (2°HPT) is not known. The purpose of this study was to determine the morbidity of DCM caused by 2°HPT and the efficacy of parathyroidectomy (PTx) in chronic dialysis patients with advanced 2°HPT was analyzed prospectively.Methods Between November 2000 and January 2003, 237 dialysis patients who underwent total PTx with forearm autograft at our department were enrolled in this study. Cardiac complications that existed before PTx were examined. Ten patients (4%) had DCM without valvular disease (VD) or ischemic heart disease (IHD). In these 10 patients with DCM before operation, we estimated left ventricular (LV) function at 6 months after PTx, according to echocardiography findings and clinical symptoms.Results Six months after PTx, left ventricular ejection fraction (LVEF) in these 10 patients was significantly improved, from 31.0 ± 9.8% before PTx, to 56.8 ± 13.5% (P = 0.0003), and left ventricular end-diastolic dimension (LVDd) was reduced, from 59.8 ± 9.7 mm to 46.3 ± 7.0 mm (P = 0.0014). The symptoms due to DCM and the fall of blood pressure that had occurred during dialysis were clearly improved after PTx.Conclusions Advanced 2°HPT can influence LV function, and in patients who suffered from DCM, LV function was dramatically improved by PTx. PTx should be performed immediately in patients with DCM caused by 2°HPT.This study was presented at the American Society of Nephrology Annual Meeting, held at San Diego, CA, USA, on November 12–17, 2003.     

Mitral valve repair with papillary muscle sling for functional mitral regurgitation: application of double mitral ring concept in a case of ischemic cardiomyopathy and a case of idiopathic dilated cardiomyopathy

Functional mitral regurgitation (FMR) is a common complication in patients with ischemic heart disease or idiopathic dilated cardiomyopathy (DCM). We report successful application of a papillary muscle sling (PMS) to maintain the efficacy of undersized mitral annuloplasty (MAP) in two patients with FMR. A 5-mm expanded polytetrafluoroethylene tube was placed around the trabecular base of the papillary muscles and then tightened to make a sling that banded the two papillary muscles together. In case 1, a 57-year-old woman who had experienced anterolateral myocardial infarction underwent mitral valve reconstruction with undersized MAP and PMS in addititon to coronary revascularization for chronic ischemic mitral regurgitation. In case 2, we applied the same mitral valve procedure in a 65-year-old woman with idiopathic DCM. Neither had mitral insufficiency at an intermediate follow-up.     

Mitral valve repair in patients with end stage cardiomyopathy: who benefits?

Objective: Patients with end stage cardiomyopathy frequently present with additional severe mitral regurgitation. We analyzed the outcome of mitral valve reconstruction in this high risk patient group. Methods: Sixty-six patients with significant mitral regurgitation and an ejection fraction (EF) below 30% (dilated CARDIOMYOPATHY=53, ischemic cardiomyopathy (ICM)=13) were retrospectively evaluated from 07/96 and 02/02. All received annuloplasty ring implantation and additional repair (n=4) if required. Mean follow-up was 28±18 months. Results: Mitral valve repair (MVR) was technically feasible in all patients. Intraoperative transesophageal echocardiography (TEE) revealed none (n=60) or only trivial (n=6) residual mitral regurgitation. Thirty day mortality was 6.1%. Actuarial survival after 1 and 5 years was 86±4 and 66±8%, respectively. During follow-up seven patients were transplanted due to lack of clinical improvement after 10±7 months (range 1–23). Echocardiography revealed a significant improvement in EF (25±10.5% pre-op, 34±15% post-op) and a slight decrease in left ventricular end-diastolic diameter (69±10 mm pre-op, 67±13 mm follow up). Patients were in NYHA functional -class 3 (median) preoperatively and in class 2 at long term-follow-up. Gender, left ventricular enddiastolic diameter, preoperative ejection fraction or type of surgical approach (sternotomy, right lateral minithoracotomy) had no significant influence on patient outcome. Patients with ICM or patients older than 60 years showed an increased risk for clinical events both early post-operatively and at long-term follow-up. Conclusion: MVR can be performed with low perioperative morbidity and mortality even in patients with advanced heart failure, modifying selection criteria for potential candidates may further improve long term outcome.     

Primary preventive cardioverter‐defibrillator implantation (Pro‐ICD) in patients awaiting heart transplantation. A prospective,randomized, controlled 12‐year follow‐up study

The aim of this study was to evaluate whether short‐term primary preventive cardioverter‐defibrillator (ICD) implantation as bridge to heart transplantation (HTX) provides any survival benefit. Thirty‐three patients awaiting HTX were randomized to either conventional therapy (control group) or primary preventive ICD implantation (ICD group). Fourteen patients had ischemic cardiomyopathy (ICM) and 19 patients had dilated cardiomyopathy (DCM). Sixteen patients were randomized to the ICD group and 17 patients were randomized to the control group. Twenty patients (61%) were transplanted after a waiting time of 10 ± 9 months. The remaining 13 patients (39%) were not transplanted because of clinical improvement (n = 5), cerebral hemorrhage (n = 3), or death (n = 5). On the waiting list, 3 ICD patients with DCM developed slow VTs without ICD intervention, two patients with ICM (6%) had fast VT terminated by the ICD, and no arrhythmic death was observed. After 11.9 years (median), 13 of 20 HTX patients (65%) and 5 of 13 non‐HTX patients (38%) were alive. Survivors had a higher LVEF (22 ± 6 vs. 17 ± 4%, P = 0.0092) and a better exercise capacity (75 ± 29 vs. 57 ± 24 Watt, P = 0.0566) at baseline as compared to nonsurvivors. This study may not support the general use of primary preventive ICDs as a short‐term bridge to heart transplantation.     

Prevalence of gallstones in 1,229 patients submitted to surgical laparoscopic treatment of GERD and esophageal achalasia: associated cholecystectomy was a safe procedure

Background

Association between esophageal achalasia/ gastroesophageal reflux disease (GERD) and cholelithiasis is not clear. Epidemiological data are controversial due to different methodologies applied, the regional differences and the number of patients involved. Results of concomitant cholecistectomy associated to surgical treatment of both diseases regarding safety is poorly understood.

Aim

To analyze the prevalence of cholelithiasis in patients with esophageal achalasia and gastroesophageal reflux submitted to cardiomyotomy or fundoplication. Also, to evaluate the safety of concomitant cholecistectomy.

Methods

Retrospective analysis of 1410 patients operated from 2000 to 2013. They were divided into two groups: patients with GERD submitted to laparocopic hiatoplasty plus Nissen fundoplication and patients with esophageal achalasia to laparoscopic cardiomyotomy plus partial fundoplication. It was collected epidemiological data, specific diagnosis and subgroups, the presence or absence of gallstones, surgical procedure, operative and clinical complications and mortality. All groups/subgroups were compared.

Results

From 1,229 patients with GERD or esophageal achalasia, submitted to laparoscopic cardiomyotomy or fundoplication, 138 (11.43%) had cholelitiasis, occurring more in females (2.38:1) with mean age of 50,27 years old. In 604 patients with GERD, 79 (13,08%) had cholelitiasis. Lower prevalence occurred in Barrett''s esophagus patients 7/105 (6.67%) (p=0.037). In 625 with esophageal achalasia, 59 (9.44%) had cholelitiasis, with no difference between chagasic and idiopathic forms (p=0.677). Complications of patients with or without cholecystectomy were similar in fundoplication and cardiomyotomy (p=0.78 and p=1.00).There was no mortality or complications related to cholecystectomy in this series.

Conclusions

Prevalence of cholelithiasis was higher in patients submitted to fundoplication (GERD). Patients with chagasic or idiopatic forms of achalasia had the same prevalence of cholelithiasis. Gallstones occurred more in GERD patients without Barrett''s esophagus. Simultaneous laparoscopic cholecystectomy was proved safe.     

Long-term outcomes in patients with vestibular schwannomas treated using gamma knife surgery: 10-year follow up

OBJECT: Gamma knife surgery (GKS) has been a safe and effective treatment for vestibular schwannomas in both the short and long term, although less is known about long-term outcomes in the past 10 years. The aim of this study was to clarify long-term outcomes in patients with vestibular schwannomas treated using GKS based on techniques in place in the early 1990s. METHODS: Eighty patients harboring a vestibular schwannoma (excluding neurofibromatosis Type 2) were treated using GKS between May 1991 and December 1993. Among these, 73 patients were assessed; seven were lost to follow up. The median duration of follow up was 135 months. The mean patient age at the time of GKS was 56 years old. The mean tumor volume was 6.3 cm3, and the mean maximal and marginal radiation doses applied to the tumor were 28.4 and 14.6 Gy, respectively. Follow-up magnetic resonance images were obtained in 71 patients. Forty-eight patients demonstrated partial tumor remission, 14 had tumors that remained stable, and nine demonstrated tumor enlargement or radiation-induced edema requiring resection. Patients with larger tumors did not fare as well as those with smaller lesions. The actuarial 10-year progression-free survival rate was 87% overall, and 93% in patients with tumor volumes less than 10 cm3. No patient experienced malignant transformation. CONCLUSIONS: Gamma knife surgery remained an effective treatment for vestibular schwannomas for longer than 10 years. Although treatment failures usually occurred within 3 years after GKS, it is necessary to continue follow up in patients to reveal delayed tumor recurrence.     

The ileo neo-rectal anastomosis in patients with familial adenomatous polyposis: a prospective case series with long-term follow up

Aim In patients with familial adenomatous polyposis (FAP), removal of the colonic mucosa is essential to reduce the lifetime risk of developing cancer). For this purpose, ileo‐pouch anal anastomosis (IPAA) has been the gold standard, but morbidity related to the dissection of the pelvis remains substantial. In an attempt to reduce the procedure‐related complications of pelvic dissection, ileoneo‐rectal anastomosis (INRA) has been developed. In this case series of FAP patients, the long‐term functional results, morbidity and quality of life (QoL) of the INRA procedure were evaluated and compared with its early outcome. Method Long‐term follow up of a consecutive group of eight FAP patients with an INRA procedure (between 1998 and 2005) was undertaken. Data on functional results, complications, manometry and endoscopy were recorded prospectively. Results Eight patients with FAP underwent the INRA procedure. The median number of defaecations over 24 h was five. No pelvic sepsis or bladder dysfunction occurred. One patient, in whom concomitant Crohn’s disease was diagnosed in retrospect, was converted to IPAA. In the INRA patients, no sexual dysfunction occurred. Endoscopic examination showed normal mucosa without any evidence of polyp formation. Conclusion Restorative surgery by means of the INRA procedure yields good functional results in FAP patients, without any pelvic dissection‐related morbidity or regrowth of polyps in the neo‐rectum.