Clinical and hemodynamic results after Fontan operation.

Twenty-six patients, ranging in age from 2 to 14 years (mean, 6 years and 8 months), with tricuspid atresia or other complex cyanotic cardiac diseases underwent a modified Fontan operation between 1980 and 1990. In 13 patients, palliative operations had been previously performed. There were 7 mortalities within 1 month after the operation. Seventeen of the 19 survivors were followed up for 5-122 months (mean, 27) with no late deaths. Fifteen patients were in the New York Heart Association functional class 1. Two patients required reoperations at 2 and 57 months postoperatively: the former for atrio-ventricular valvular regurgitation, and the latter for late-developing conduit obstruction and residual atrial right to left shunting. Fourteen patients underwent cardiac catheterization after 1-57 months (mean, 9.8) postoperatively. Two patients had a residual right to left shunt at the atrial level, while the oxygen saturation in the remaining 12 patients was above 90%. Thus, the modified Fontan operation offers an alternate surgical approach for patients with complex congenital heart disease for whom no other palliative procedure can be expected to obtain significant improvement. It can be performed with good clinical and hemodynamic results in selected patients.     

彩色多普勒超声心动图在房间隔缺损和室间隔缺损介入治疗中的应用

目的探讨经胸超声心动图(TTE)及实时三维超声心动图在房间隔缺损(ASD)、室间隔缺损(VSD)封堵术中的临床应用价值。 方法应用二维及三维超声心动图对2004 10—2005 10在天津市儿童医院就诊的24例继发孔ASD，10例VSD进行术前检查筛选，术中超声引导及术后随访检查。 结果24例ASD患儿术前经TTE检测ASD直径(15.6±7.9)mm(5~26mm)，所选Mmplatzer封堵器直径为(19.1±5.1)mm(5~32mm)，23例封堵成功。室间隔膜部缺损直径：左室面缺损为5~9mm，右室侧口的直径为2.4~6.0mm，术中选择Amplatzer封堵器型号为4~6mm，10例VSD无残余分流。 结论二维及三维TTE联合应用于ASD、VSD介入治疗具有很好的应用价值。     

Ross procedure for pediatric aortic valve disease.

BACKGROUND AND PURPOSE: Currently, prosthetic selection for aortic valve replacement is still debatable in pediatric patients with aortic valve disease. Ross procedure is a time-consuming and demanding technique which may produce good results. This study assessed the results obtained with various methods of right ventricle outflow tract (RVOT) reconstruction in patients receiving Ross procedure. METHODS: From September 1996 to December 2003, 13 pediatric patients underwent Ross procedure for aortic valve disease. Their ages ranged from 1 month to 17 years (mean, 6.29 +/- 3.62 years; median, 7 years) and 2 patients were less than 1 year of age. Previous procedures in these patients included balloon dilation of the aortic valve in 10 and surgical aortic valvuloplasty in 2. RVOT was reconstructed with heterografts in 3, homografts in 3 or without extracardiac conduits in 7. RESULTS: There was 1 in-hospital death (7.7%) and 1 late death from a non-cardiac cause. One patient developed infective endocarditis with periaortic abscess 2 weeks after the Ross procedure. Eleven patients were followed for a mean of 3.3 years (range, 8 months to 8 years). There was no significant pressure gradient across the neoaorta. Severe stenosis developed gradually in 3 patients who had RVOT reconstruction with heterograft conduits. All 3 had received RVOT redo operation. None of the patients who received RVOT reconstruction without extracardiac conduits had significant pulmonary stenosis but 6 had regurgitation (nil 1, mild 3, moderate 3). No redo operation was required during follow-up (range, 8 months to 4 years). All of the 11 survivors were in New York Heart Association functional class I. CONCLUSIONS: This study found satisfactory results of Ross procedure in pediatric aortic valve disease. RVOT reconstruction without extracardiac conduit is a feasible alternative despite the availability of homograft.     

Surgical repair of ventricular septal defect without ventriculotomy in the first 12 months of life.

Sixty-eight infants with clinical evidence of a large ventricular septal defect (VSD), refractory to conventional medical treatment, underwent surgical closure within the first 12 months of life from August 1987 to June 1991. There were 43 males and 25 females. The ages of the patients ranged from two to 12 months, with a mean age of 6.6 months. The mean body weight of the patients was 5.4 kg (range, 2.3-10 kg). Surgery was performed because of intractable heart failure in 27 infants (39.7%), failure to thrive in 40 (58.8%), repeated pneumonia in 43 (63.2%) and prolonged endotracheal intubation in nine (13.2%). There were 21 patients with a supracristal VSD (30.9%) and 47 patients with perimembranous VSD (69.1%). Nine patients (13.2%) had preoperative cardiac catheterization. Transatrial repair of perimembranous VSDs and transpulmonary repair of supracristal VSDs was used exclusively without ventriculotomy. Surgically induced heart blocks did not occur in any of the patients. Only two patients (2.9%) died during the early postoperative period. Diagnosis in most cases was confirmed by the present advanced integrated color Doppler echocardiographic technology which is widely used by pediatric cardiologists. There was no need to perform cardiac catheterization in most patients with VSDs. The morbidity and mortality were very low. We strongly suggest that for infants with a large VSD, primary repair should be the procedure of choice.     

Pulmonary hypertension in children following extracorporeal membrane oxygenation therapy and repair of congenital diaphragmatic hernia.

OBJECTIVE: Pulmonary hypertension (PHT) is present in all children at birth, but its degree and rate of resolution in infants diagnosed with congenital diaphragmatic hernia (CDH) requiring extracorporeal membrane oxygenation (ECMO) need to be established. STUDY DESIGN: Twenty-one ECMO/CDH survivors (aged 3.2 +/- 1.4 years) were prospectively evaluated by Doppler echocardiography (ECHO) to determine the presence of PHT. Twenty children without structural heart disease were used as controls. Study patients received a physical examination and an electrocardiograph examination, and their charts were reviewed for neonatal course data. Patients found to have PHT by ECHO received a complete history and exercise treadmill/oxygen desaturation study. RESULTS: Eight of the 21 patients (38%) met echocardiographic criteria for PHT. No neonatal course data were found to be predictive of eventual PHT status. There was no correlation between physical examination or electrocardiographic findings and PHT. Complete histories showed five of the eight patients with PHT had some degree of exercise intolerance and seven had wheezing. Two of the seven patients studied on the treadmill desaturated 5% or greater from baseline. CONCLUSION: There is evidence that PHT either persists or recurs in a significant portion of the ECMO/CDH population and may remain symptomatic well beyond the neonatal period.     

Malignant ovarian germ cell tumors: the KK Hospital experience

Ovarian germ cell malignancies pose a therapeutic challenge especially amongst young patients. This is a retrospective review of 49 patients treated for such malignancies at KK Women's and Children's Hospital over a 13-year period. The relative proportion of such tumors was 6.2%. Age at presentation ranged from 14 to 51 years (mean 25.4 years). Forty-nine percent of tumors were immature teratomas and 81.6% had stage I disease. All patients had surgery initially and 67.3% required postoperative adjuvant chemotherapy. The patients were followed-up for one to 145 months (mean 51.6 months). All the 87.8% of patients on follow-up are alive and disease-free. There was one recurrence. Five patients had eight successful pregnancies, with no congenital anomalies. Mean duration when menstruation was resumed and regular was 2.5 and 3.5 months, respectively. With combination chemotherapy and conservative surgery, the outlook for patients is excellent, with emphasis on preservation of ovarian function and fertility.     

Transatrial-transpulmonary repair of tetralogy of Fallot: extensive infundibular septum resection.

Twenty-seven patients with tetralogy of Fallot underwent total correction during the period from February 1988 through October 1989. Their ages ranged from 11 months to 12 years (mean 3.3 +/- 4.9 years). For 11 patients, the repair was made via the conventional transventricular approach and a partial resection of the infundibular septum. On the other 16 patients, a transatrial-transpulmonary approach was used with total resection of the anterior deviated infundibular septum, with or without a miniventriculotomy incision (1-10 mm). The exposure of the ventricular septal defect (VSD) through the right atrium, and the right ventricular outflow tract (RVOT) through the pulmonary artery in patients with tetralogy of Fallot was excellent. There were no significant differences between transatrial-transpulmonary repair and transventricular repair of the tetralogy of Fallot with respect to pulmonary artery (PA) index (273.2 +/- 36.6 versus 249.9 +/- 63.2 mm2/BSA) or the postoperative ratio of right ventricle/left ventricle (RV/LV) systolic pressure (0.55 +/- 0.16 versus 0.61 +/- 0.17). Postoperative intensive care was simple and uncomplicated, with a significantly lower catecholamine demand, less bleeding, fewer blood transfusions, and shorter stays in the cardiac Intensive Care Unit (ICU). There was no mortality in this series. This method of repair can be successfully accomplished in most patients with tetralogy of Fallot, with resultant preservation of right ventricular function (intact right ventricle).     

Usefulness of 3D reconstructed computed tomography imaging for double outlet right ventricle.

BACKGROUND/PURPOSE: To evaluate the value of 3D reconstructed computed tomography (CT) imaging for patients with double outlet right ventricle (DORV). METHODS: CT images were obtained preoperatively from 17 patients who ranged in age from 5 days to 5 years. Reconstructed 3D images were created using gradient-shading surface rendering, which allowed partial subtraction of the anterior sections of the virtual heart to view the interior. Interpretations of CT, echocardiography and cine-cardioangiography were compared and verified from surgical findings, autopsy, and consensus upon review of all imaging and diagnostic tests. RESULTS: Three subaortic, seven subpulmonary, six non-committed, and one double-committed subtypes of ventricular septal defect (VSD) were observed. The 3D electron beam CT images provided good delineation of the spatial relationship inside the heart. The range of diagnostic accuracy for all VSD types in DORV was 88-100% for 3D CT, 71-94% for echocardiography, and 60-100% for cine-cardioangiography. In comparison, 3D CT offered better diagnostic accuracy for all variants of DORV. CONCLUSION: 3D constructed CT imaging is a good modality for differentiating VSD type in DORV. It allowed us to directly evaluate the inside of cardiac chambers for the right ventricular outlet, great arterial root, and determine the VSD relationships.     

The use of TDMAC-heparin-impregnated shunt for managing aneurysm of the descending thoracic aorta

From April 1981 to December 1988, a total of 23 patients were operated upon for the repair of an aneurysm of the descending thoracic aorta with a tridodecylmethylammonium chloride (TDMAC)-heparin-impregnated shunt. There were 20 men and 3 women. The ages ranged from 29 to 72 years with a mean of 52.3 years. The major pathological change of the aortic wall was medial cystic necrosis in 19 patients and atherosclerosis in 4. Among them, only 6 patients underwent elective surgery, while 17 patients underwent emergency operations with the surgical indication being shock in 4, oliguria in 3, persistence of chest pain in 2, massive hemoptysis in 1, rapid size progression of the aneurysm in 3, and impending rupture of the aneurysm in 4. During surgery, the aortic cross-clamping time ranged from 40 to 76 minutes with a mean of 54 minutes. Hospital death was limited to 2 patients with a mortality rate of 8.7%. Postoperative complications were noted in 5 patients, cerebrovascular accidents in 4 and transient paraparesis in 1. However, the clinical conditions recovered before those patients were discharged. The follow-up period ranged from 4 to 84 months with a mean of 32.3 months. Except for 1 patient who died of anaphylactic shock due to drug allergy, the other 20 survivors had an apparent improvement of their clinical status and life quality.     

Atrial and ventricular septal defects - epidemiology and spontaneous closure.

OBJECTIVE: To present data on the epidemiology and spontaneous closure for septal defects (secundum atrial septal defect (ASD) and/or ventricular septal defect (VSD)). METHODS: Data from the EUROCAT Registry of Congenital Malformations for Funen County, Denmark were analyzed. All infants born from 1986 to 1998, diagnosed with a secundum ASD and/or a VSD as the only cardiac malformation, were included. RESULTS: There were 78 infants with an ASD, 195 with a VSD, and 19 had both an ASD and a VSD. The overall prevalence of septal defects was 4.1 per 1000 livebirths. Among the VSDs 45% were perimembranous and 55% were muscular defects. Associated non-cardiac malformations, syndromes, or karyotype anomalies were present in 54 cases (19% of total) but with a significantly lower proportion among cases with muscular VSDs (7%, p < 0.05). Eighty-one percent of the cases had an isolated cardiac malformation. Five of 61 cases (8%) with isolated ASD were closed surgically before five years of age and 43 (70%) closed spontaneously within the first five years of life. Of the 65 cases with isolated perimembranous VSDs, 13 were closed surgically (20%) and 13 closed spontaneously (20%). Of the 99 cases with isolated muscular VSDs no defects were closed by surgery and 64 (65%) closed spontaneously. The rate of spontaneous closure for perimembranous and muscular VSDs were significantly different (p < 0.0001). CONCLUSIONS: Secundum ASD has a high rate of spontaneous closure. Perimembranous and muscular VSDs seem to be two different diseases with different epidemiology and natural history.     

Combined atrial and arterial switch operations for congenitally corrected transposition.

Conventional repair of congenitally corrected transposition of the great arteries (CCTGA) is directed at eliminating the associated defects and leaves the right ventricle in a systemic position. The long-term outcome of this procedure may involve deterioration of right ventricular function with tricuspid regurgitation and failure of the conduction system. We describe two consecutive patients with CCTGA, one of whom had apicocaval juxtaposition. The patients were aged 19 and 16 months, respectively, and both underwent a combination of atrial and arterial switch. These are the first two reported cases of successful completion of this type of operation in Taiwan. Our review of previously reported cases suggested that no significant difference exists in the outcome of patients with this condition who undergo either arterial switch or Rastelli-type repair plus atrial redirection. However, reported patients who underwent anatomic repair had lower early mortality, late mortality, and incidence of complete heart block than those who underwent conventional repair. The present two cases and our review of the literature suggest that, among patients with apicocaval juxtaposition, 1) Mustard operation is optimal for patients with small atrial volume; 2) one-and-one-half ventricular repair may be helpful to the outcome, especially when treatment is combined with Rastelli-type repair; and 3) excellent access to the ventricular septal defect through the tricuspid valve is afforded via a left atriotomy. From the present two cases and our review of the literature, we conclude that anatomic repair is superior to conventional repair of CCTGA in terms of protection against dysfunction and failure of the anatomic right ventricle, tricuspid valve, and conduction system. Long-term follow-up is mandatory.     

Repeat revascularization versus nephrectomy in the treatment of recurrent renovascular hypertension

Between 1960 and 1983, 38 patients underwent multiple operations for treatment of recurrent renovascular hypertension. There were 23 women and 15 men who ranged in age from eight to 69 years old (a mean of 48.5 years). The cause of hypertension requiring repeat operation was determined roentgenographically, three patients had new disease of the contralateral nonoperated renal artery, 21 patients had a new lesion of the ipsilateral (previously operated) renal artery and 14 patients had new lesions of both the previously operated and nonoperated renal arteries. Thirty patients underwent a secondary unilateral operation and eight had a bilateral operation. Sixteen patients had unilateral renal artery revascularization, 14 had unilateral nephrectomy, three had bilateral revascularization and five had unilateral revascularization with contralateral nephrectomy. There were three operative deaths (an operative mortality of 7.9 per cent). At hospital dismissal, 30 of 35 patients were improved. Follow-up study ranged from seven months to 23 years (a mean of 7.2 years). There were eight (22.9 per cent) late deaths. Secondary revascularization alone produced improvement in 77 per cent. Nephrectomy alone produced improvement in 80 per cent. We conclude that secondary revascularization is the treatment of choice in patients with recurrent renal artery stenosis. Nephrectomy should be reserved for patients who cannot undergo a revascularization procedure for technical or medical reasons.     

Implications of prolonged pause in patients with chronic atrial fibrillation with mitral valve disease undergoing atrial compartment operation.

BACKGROUND AND PURPOSE: Prolonged pause is commonly seen in patients with atrial fibrillation (AF), but the electrophysiologic mechanism and clinical importance of this phenomenon are not clear. This study examined the incidence and clinical importance of prolonged pause in patients with chronic AF and mitral valve disease before and after AF surgery. METHODS: Holter recordings were made in 53 mitral valve patients undergoing concomitant valve surgery and atrial compartment operation for chronic AF. There were 38 patients (72%) with successful AF conversion and 15 patients (28%) with failed AF conversion. Cardiac rhythms before and after operation were compared. An R-R interval > or = 2.0 sec was defined as prolonged pause. Serum digoxin and potassium concentration were determined within 24 hours of Holter monitoring. RESULTS: Before operation, prolonged ventricular pause was common during AF in both groups (76% for the successful AF conversion group and 73% for the failed AF conversion group, p > 0.05). There were 62 +/- 77 episodes of prolonged pause in the successful AF conversion group and 59 +/- 44 in the failed group (p > 0.05). The longest pause lasted 2.47 +/- 0.26 sec in the successful AF conversion group and 2.43 +/- 0.41 sec in the failed AF conversion group (p > 0.05), with most prolonged pauses occurring at night (72% in the successful and 73% in the failed AF conversion group, p > 0.05). After conversion to sinus rhythm, only 1 patient (3%) showed an episode of prolonged pause (p < 0.001). No patient exhibited atrioventricular (AV) block. In patients with successful AF conversion, the maximal heart rate decreased from 150 +/- 28 to 126 +/- 17 beats/min (p < 0.001), the minimal heart rate increased from 43 +/- 6 to 56 +/- 5 beats/min (p < 0.001), and ventricular premature beats (VPB) counts decreased from 599 +/- 935 to 223 +/- 453/24 hours (p < 0.05). In contrast, patients with failed AF conversion showed no significant changes in the incidence of prolonged pause, maximal and minimal heart rates, and VPB counts after operation. None of the patients received pacemaker implantation during a mean follow-up period of 42 +/- 11 months in the successful group and 45 +/- 13 months in the failed AF conversion group. CONCLUSION: We conclude that prolonged pause is common in AF with mitral valve disease and does not indicate the presence of sinus or AV nodal dysfunction requiring artificial pacing.     

伴有心脏受累的静脉内平滑肌瘤病临床分析

目的:探讨侵入心脏的静脉内平滑肌瘤的临床特点和治疗方法。方法:回顾分析北京协和医院1983年1月至2004年10月收治的心脏受累的静脉内平滑肌瘤患者3例的临床资料。结果:3例患者有心慌、胸闷、晕厥或静脉栓塞等症状。影像学检查在右心房或右心室发现瘤栓。其中,1例2年前因子宫肌瘤行子宫切除术,2例合并有盆腔包块。3例均无肺转移,均接受了静脉瘤栓取出术,其中1例残留心房内瘤栓,1例手术前后接受大剂量孕激素治疗。结论:静脉内平滑肌瘤病的发生率低,侵入心脏的病例更为罕见。治疗以手术为主,抗雌激素治疗可能改善预后。     

Reoperation for palliation of recurrent malignant bowel obstruction in ovarian carcinoma

OBJECTIVE: While initial surgical treatment for palliation of malignant bowel obstruction is well described, data on reoperation for palliation of recurrent obstruction in ovarian carcinoma are limited. The purpose of this study was to analyze the outcome of patients undergoing reoperation for repeat bowel obstruction. METHODS: We reviewed the records of all patients with ovarian cancer who underwent repeat surgery for recurrent, malignant bowel obstruction at our institution between 1994 and 2002. RESULTS: Ten patients were identified. All patients had bowel obstruction caused by recurrent ovarian carcinoma and had a previous corrective surgical procedure for malignant bowel obstruction. The mean age at diagnosis of repeat obstruction was 54.1 years (range, 34-74 years). All patients had initial stage III or IV disease with moderately to poorly differentiated cancers. No patient received prior radiation therapy. The sites of obstruction in patients were as follows: small bowel, 3; large bowel, 3; both small and large bowel, 4. The mean number of prior laparotomies was 2.7 (range, 2-5). The mean interval from previous surgery for bowel obstruction to recurrent bowel obstruction was 8.3 months (range, 1-22 months). Surgical correction was possible in 5 (50%) of 10 patients, with 3 (60%) of these 5 patients obtaining successful palliation. Successful palliation is defined as the ability to tolerate a regular or low-residue diet at least 60 days postoperatively. Complications included enterocutaneous fistulas in three patients (two had enterotomies at time of surgery) and wound infection in one patient. There were no postoperative mortalities. The mean postoperative stay was 15.8 days (range, 8-29 days). Two of the three patients successfully palliated presented with a subsequent obstruction at 3 and 5 months postoperatively and were treated with gastrostomy tubes. The median length of survival for the entire cohort from the date of surgery for repeat obstruction was 4.5 months (range, 3-17 months). CONCLUSIONS: Patients undergoing repeat surgery for recurrent bowel obstruction have a low likelihood of successful palliation (30%). The surgery is associated with significant complications after surgery, rapid development of subsequent bowel obstructions, and limited survival rates. Alternative management approaches such as percutaneous endoscopic gastrostomy (PEG) tube placement should be considered in this group of patients.     

脱细胞生物组织补片在盆底重建手术中的应用

目的初步探讨脱细胞生物组织补片在盆腔器官膨出患者盆底重建手术中的应用情况。方法选择北京大学人民医院妇科2006年5月至12月期间接受盆底修补和重建手术并应用脱细胞生物补片的盆腔器官膨出患者20例,其中子宫脱垂19例,子宫切除术后阴道穹隆脱垂Ⅱ度1例;合并存在膀胱膨出20例、直肠膨出17例。20例患者中17例同时行阴道前后壁修补术,3例行阴道前壁修补术;阴道前壁置入补片15例,阴道后壁置入补片2例,阴道前壁和后壁同时置入补片3例。结果20例患者总手术时间平均为113．1min（70～180min）,其中放置补片的时间平均为10min。术中出血平均为175ml（50～300ml）。术后恢复良好,平均随访9．3个月（6～12个月）,未发现补片侵蚀阴道黏膜情况,无感染发生。随访期间4例（20％）患者出现盆腔器官膨出复发,3例为膀胱膨出Ⅰ度,复发时间均为6个月复查时,其中2例随访12个月时仍为膀胱膨出Ⅰ度,另1例随访8个月时也为膀胱膨出Ⅰ度,未见加重;1例为膀胱膨出Ⅱ度,复发时间为6个月复查时;所有复发患者均无临床症状。结论脱细胞生物组织补片用于盆底重建手术,方法简单,操作容易,未见补片侵蚀发生,其长期效果有待进一步观察。     

Peripartum cardiomyopathy: echocardiographic features in five cases

Five cases of peripartum cardiomyopathy are presented. All patients were aged less than 35 years, and four were multiparous. Two cases followed twin deliveries. Pulmonary embolism was diagnosed in four patients. Electrocardiograms showed left ventricular hypertrophy or left bundle branch block. On echocardiography, left ventricular minor axis dimensions were increased (diastolic, 67 +/- 7 mm; systolic, 59 +/- 7 mm) and mean fractional shortening was reduced (13% +/- 5%). All patients had hypokinesis or akinesis of the left ventricular segments and two had right ventricular dilatation. Gallium scanning performed in three patients was negative. Viral serologic testing was negative in all cases. All patients died within 5 years, three within 5 months. Survival duration was closely correlated with left ventricular fractional shortening. Autopsy in three patients confirmed the diagnosis of a dilated cardiomyopathy.     

Hemolysis following correction of ventricular septal defect

Hemolysis following repair of a ventricular septal defect (VSD) is an unusual complication. Three cases of patients who had simple VSD are described here. After repair of the VSD, the postoperative course was complicated by the intravascular hemolysis induced by the interaction between the red blood cells and the double velour Dacron patch. Thus the patients' hemodynamic status deteriorated. The first 2 patients had a course of hemoglobinuria, hyperamylasemia, hyperglycemia, malignant hypertension, hepatospenomegaly, acute renal failure and hypertensive encephalopathy. One patient, who did not undergo reoperation, died due to multiple organ failure; the other one, who underwent operation revision in order to arrest the hemolysis, died of sepsis. Early reoperation was performed in the remaining patient to replace the double velour Dacron patch with a pericardium-covered knitted Dacron patch, and he survived. Hemolysis ceased in both patients who had the double velour Dacron patch replaced with a pericardium-covered patch. A review of the literature failed to show previous reports concerned with hemolysis associated with repair of a simple VSD. We now report what we believe to be the first cases in the literature with this unique clinical course following hemolysis.     

FREE COMMUNICATION

Several conservative and surgical methods have been proposed for patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. The technique described by Vecchietti is a combination of both dilatation and surgery. We describe a minimally invasive modification of this approach including dissection of the vesico-rectal septum and present long-term patient outcome. Eight patients who underwent surgery at our department between 1996 and 2005 for MRKH syndrome were included in the present analysis. Post-operative follow-up and a semi-structured telephone interview were performed to obtain information on neovaginal functionality, immediate and long-term post-operative outcome, sexual function and physiological and psychological well-being evaluated on a 10-point scale and by the Sintonen 15D questionnaire. After a median post-operative (median operation time: 88 min, range: 63–116 min) follow-up period of 40.3 months (range: 18–84 months) without major complications and a median post-operative stay of 13.8 days (range: 6–15 days), a functional neovagina was created in seven of eight patients. Median vaginal length at the time of discharge from the hospital ranged between 7 and 10 cm (median: 9.6 cm) and reached 11.5 cm (range: 5–15 cm) at the time of evaluation. Except for one patient who neither had regular sexual intercourse nor used the vaginal dilator, sexual intercourse was quoted as satisfactory in six cases (one patient still used the dilator only). Patients reported an improvement of quality of life (mean: 8.1, range: 5–10) and self-confidence (8.1, range: 5–10) and general well-being evaluated by the Sintonen 15D questionnaire (average score of 1.8, SD = 0.06). The laparoscopic-assisted Vecchietti procedure with dissection of the vesico-rectal septum is a safe and effective method for creation of a neovagina in MRKH patients.