Congenital cardiovascular defects in children with intestinal malrotation Intestinal malrotation -LRB- IM -RRB- and cardiovascular defects -LRB- CCVD -RRB- are both common congenital defects . We investigated the prevalence and types of CCVD in a 25-year IM population , and its association with post-IM-operative morbidity and mortality . Data on the type of CCVD , other congenital defects , syndromes , associations , post-IM-operative morbidity and mortality were retrospectively reviewed from the records of IM patients born between 1980 and 2005 . Data were analyzed on -LRB- significant -RRB- differences between CCVD subgroups , and risk factors for both morbidity and mortality were calculated . Seventy-seven of 284 IM patients -LRB- 27.1 % -RRB- were diagnosed with a major or minor CCVD -LRB- 37 and 40 patients , respectively -RRB- . Syndromes and associations were more frequently diagnosed in patients with major than with a minor CCVD -LRB- 67.6 vs. 40 % , respectively -RRB- . Post-IM-operative complications , although frequently observed -LRB- 61 % -RRB- , did not differ between patients with major and minor CCVD . Physical CCVD signs before IM surgery increased post-IM-operative morbidity significantly -LRB- OR 4.0 , 95 % CI 1.4 -- 11.0 -RRB- . Fifteen patients died -LRB- 19.5 % -RRB- , seven due to cardiovascular cause . Mortality risk was increased by intestinal ischemia and post-IM-operative complications and by major CCVD after correction for age at weight at the time of IM operation . Congenital cardiovascular defects in children with intestinal malrotation are common , with high morbidity and mortality rates after IM operation . Elective IM surgery in young patients with CCVD should be performed in a centre with adequate paediatric cardiac care . Benefits of laparoscopic intervention need further study . Introduction Intestinal malrotation -LRB- IM -RRB- is refers to all abnormalities of intestinal position and attachment and includes the concept of atypical malrotation or malrotation variant -LSB- 1 -RSB- . It is often accompanied by a narrow base of the small bowel mesentery . The incidence of IM is 1:500 -- 1:3,500 living births -LSB- 2 , 3 -RSB- . Volvulus , a rotation of the gut along its mesenteric stalk occurs in 60 -- 70 % of neonates diagnosed with IM , the ultimate strangulation in about 15 % -LSB- 4 -RSB- . Eighty percent of the patients with symptomatic IM present in their first month of life -LSB- 5 -RSB- and require surgery . Delay in diagnosis may lead to intestinal ischemia , intestinal necrosis , septicaemia and short bowel syndrome . The surgical outcome is negatively influenced by the presence of intestinal necrosis -LSB- 6 -RSB- , and the need for operation during the first 3 months of life -LSB- 6 -- 9 -RSB- . To keep mortality and morbidity low , all asymptomatic children with intestinal malrotation are advised to undergo semi-elective surgery -LRB- Ladd 's procedure -RRB- in childhood and adolescence -LSB- 10 , 11 -RSB- . Intestinal malrotation has been described in almost 100 syndromes and combinations . Associated serious abnormalities increase the mortality risk in patients with IM of about 22-fold according to Messineo -LSB- 6 -RSB- . Commonly associated congenital malformations are duodenal atresia , abdominal wall defects or diaphragmatic hernia -LSB- 3 , 12 -RSB- . It is more frequently observed in patients with situs inversus -LSB- 13 -RSB- or heterotaxia , a syndrome associated by congenital cardiovascular defects -LSB- 14 -RSB- . Congenital cardiovascular defects -LRB- CCVD -RRB- are among the most common congenital birth defects occurring in about 1 in 100 living births -LSB- 15 -RSB- . Congenital malformations of the gastrointestinal , musculoskeletal and urinary systems are the most frequently seen extra cardiac malformations in patients with CCVD -LSB- 16 -RSB- . Intestinal malrotation has been observed in 2.8 -- 4.1 % of all the patients with CCVD , mainly in patients with single ventricle -LSB- 17 -RSB- . However , the incidence of CCVD in IM patients has not been precisely quantified in the recent literature and the full array of the associated cardiac anomalies has not been well described . The purpose of our study was -LRB- 1 -RRB- to delineate and accurately describe the types and incidence of associated cardiac lesions in our most recent 25-year experience of patients with IM , and -LRB- 2 -RRB- to study the influence of a co-existing CCVDs on IM surgery morbidity and mortality . Materials and methods Population A retrospective review at a single university centre was conducted on all the paediatric patients diagnosed with IM born and between September 1980 and September 2005 . Patients with CCVD were identified for further analysis . Patients demographics recorded included gestational age , weight and gender . Data on the type of CCVD , other congenital defects , known syndromes or associations , post-operative complications , morbidity and mortality were collected . The diagnosis of CCVD was based on clinical , ECG , echocardiography , heart catheterization and peri-operative findings . The IM diagnosis was based on radiological upper gastro-intestinal examination , or during acute surgery . Data analysis Collected data were analyzed by calculating prevalence percentages . Analysis of nominal variables was performed using cross tables for calculating relative risk -LRB- RR -RRB- with the corresponding 95 % confidence interval -LRB- 95 % CI -RRB- . Fisher extact test was used for comparison of percentages of both CCVD groups , expressed as P values . For the comparison of continuous variables , Mann -- Whitney U test was used to calculate P value . The P values are two-sided , and a value of P = 0.05 was considered statistical significant . For the calculation of risk factors for both post-operative morbidity and mortality , -LRB- adjusted -RRB- odds ratios -LRB- OR -RRB- with consequent 95 % CI were calculated by uni - and multi-variate regression analysis . Statistical analysis was performed using SPSS software -LRB- version 12.0.1 ; SPSS Inc. , Chicago , IL , USA -RRB- . Results Demographics During a period of 25 years , 284 patients were diagnosed in our centre with IM . Ninety-three of the 284 patients were identified with CCVD as well . Fifteen patients had persistent pulmonary hypertension -LRB- PPHT -RRB- , all but one with congenital diaphragmatic hernia . One patient had a solitary pre-duodenal portal vein . The records of these 16 patients without structural CCVD were excluded , leaving 77 patients with both IM and CCVD -LRB- 27.1 % of the total IM population in the last 25 years -RRB- for further analysis . The gestational age range was 26 -- 42 weeks with a median of 39 weeks . Median birth weight was 2.86 kg -LRB- range 0.72 -- 4.53 -RRB- . A female predominance was seen with 47 girls -LRB- 61 % -RRB- and 30 boys -LRB- 39 % -RRB- . Median follow-up period after IM diagnosis was 2.8 years -LRB- range 0 -- 24 years -RRB- . The overall survival at the end of follow-up was 80.5 % -LRB- 15 out of 77 patients died -RRB- . Congenital cardiovascular defects The CCVDs were divided into the subgroups , major and minor CCVDs -LRB- see Table 1 -RRB- , varying from small ventricular septal defect -LRB- VSD -RRB- to common arterial trunk -LRB- CAT -RRB- . Thirty-seven out of 77 patients -LRB- 48.1 % -RRB- were diagnosed with major CCVD and 40 patients -LRB- 51.9 % -RRB- with minor CCVD . Table 1Types of major and minor congenital cardiovascular defectsMajor CCVDn = 37 -LRB- 100 % -RRB- Minor CCVDn = 40 -LRB- 100 % -RRB- Complex cardiovascular defectsn = 18 -LRB- 48.6 % -RRB- Ventricular septal defectn = 16 -LRB- 40.0 % -RRB- Tetralogy of Fallotn = 7 -LRB- 18.9 % -RRB- Atrium septal defectn = 7 -LRB- 17.5 % -RRB- Hypoplastic left heart syndromen = 3 -LRB- 8.1 % -RRB- Peripheral pulmonary stenosisn = 4 -LRB- 10.0 % -RRB- Atrioventricular septal defectn = 3 -LRB- 8.1 % -RRB- Persistent ductus arteriosusn = 3 -LRB- 7.5 % -RRB- Pulmonary valve atresian = 1 -LRB- 2.7 % -RRB- Pulmonary valve stenosisn = 3 -LRB- 7.5 % -RRB- Transposition of the great arteriesn = 1 -LRB- 2.7 % -RRB- Aortic valve stenosisn = 2 -LRB- 5.0 % -RRB- Tricuspid valve atresian = 1 -LRB- 2.7 % -RRB- Aortic coarctationn = 1 -LRB- 2.5 % -RRB- Common arterial trunkn = 1 -LRB- 2.7 % -RRB- Hypertrophic cardiomyopathyn = 1 -LRB- 2.5 % -RRB- Aortic arch hypoplasian = 1 -LRB- 2.7 % -RRB- Subvalvular aortic valve stenosisn = 1 -LRB- 2.5 % -RRB- Dextrocardian = 1 -LRB- 2.7 % -RRB- Left superior caval veinn = 1 -LRB- 2.5 % -RRB- Arteria lusorian = 1 -LRB- 2.5 % -RRB- CCVD Congenital cardiovascular defects The characteristics of the whole group -LRB- n = 77 -RRB- as well as that of the subgroups are described in Table 2 . Forty-two patients underwent cardiac surgery , of which 20 patients had two or more cardiac surgical interventions . Patients with major CCVDs needed cardiac surgery more often . Minor CCVDs that needed surgical intervention most frequent were VSD , atrial septal defects -LRB- ASD -RRB- and aortic valve stenosis -LRB- AS -RRB- . Six patients with severe CCVD did not undergo correction of their heart disease before IM operation : three patients died -LRB- n = 2 due to post IM surgery complications , n = 1 because of other congenital malformations -RRB- . One patient with dextrocardia did not need cardiac correction . The two patients with Tetralogy of Fallot -LRB- TOF -RRB- underwent cardiac correction 6 months after IM surgery . Table 2Characteristics of studied population and its subgroups of congenital cardiovascular defectsCharacteristicAll CCVDMajor CCVDMinor CCVDRelative risk -LRB- 95 % CI -RRB- / P value100 % -LRB- n = 77 -RRB- 100 % -LRB- n = 37 -RRB- 100 % -LRB- n = 40 -RRB- Boys39 % -LRB- n = 30 -RRB- 35.1 % -LRB- n = 13 -RRB- 42.5 % -LRB- n = 17 -RRB- 0.85 -LRB- 0.5 -- 1.4 -RRB- Syndromes or associations53 .2 % -LRB- n = 41 -RRB- 67.6 % -LRB- n = 25 -RRB- 40 % -LRB- n = 16 -RRB- 1.8 -LRB- 1.1 -- 3.1 -RRB- Other congenital defects84 .4 % -LRB- n = 65 -RRB- 83.8 % -LRB- n = 31 -RRB- 85 % -LRB- n = 34 -RRB- 0.95 -LRB- 0.5 -- 1.8 -RRB- Cardiac surgery54 .4 % -LRB- n = 42 -RRB- 83.8 % -LRB- n = 31 -RRB- 27.5 % -LRB- n = 11 -RRB- 4.3 -LRB- 2.0 -- 9.1 -RRB- CCVD diagnosed before IM 57.1 % -LRB- n = 44 -RRB- 78.4 % -LRB- n = 29 -RRB- 37.5 % -LRB- n = 15 -RRB- 2.7 -LRB- 1.4 -- 5.2 -RRB- Age at CCVD diagnosis in days -LSB- median -LRB- range -RRB- -RSB- 4.5 -LRB- 0 -- 210 -RRB- 4 -LRB- 0 -- 98 -RRB- 7 -LRB- 0 -- 210 -RRB- P = 0.12 Birth weight in kilograms -LSB- median -LRB- range -RRB- -RSB- 2.86 -LRB- 0.72 -- 4.53 -RRB- 2.96 -LRB- 0.96 -- 4.53 -RRB- 2.84 -LRB- 0.72 -- 4.30 -RRB- P = 0.4 Age at first CCVD surgery in days -LSB- median -LRB- range -RRB- -RSB- 102 -LRB- 2 -- 3,759 -RRB- 43 -LRB- 2 -- 3,759 -RRB- 117 -LRB- 24 -- 1,705 -RRB- P = 0.15 Values in bold represent statistical significant resultsCCVD Congenital cardiovascular defects , IM Intestinal malrotation Congenital malformations , syndromes and associations Congenital malformations other than IM and CCVD were observed in 65 of the 77 -LRB- 84.4 % -RRB- patients , see also Table 2 . Most frequently observed congenital malformations included omphalocele -LRB- n = 7 -RRB- , congenital diaphragmatic hernia -LRB- n = 7 -RRB- , ano-rectal malformations -LRB- n = 6 -RRB- and heterotaxia -LRB- n = 6 -RRB- . Syndromes and associations were recognized in 41 -LRB- 53.2 % -RRB- patients , of which Down syndrome -LRB- n = 7 -RRB- , heterotaxia -LRB- n = 6 -RRB- , VACTERL -LRB- n = 5 -RRB- , Ivemark -LRB- n = 5 -RRB- and lateralization abnormalities -LRB- n = 4 -RRB- were most frequently recognized . Kartagener syndrome occurred in two patients . Several other syndromes all occurred once . Syndromes and associations were more frequent in patients with major CCVD . Intestinal malrotation surgery Characteristics of IM-operated patients and their major and minor CCVD subgroups are described in Table 3 . Five patients did not undergo IM surgery yet ; in three patients the surgery was about to take place in the near future , in one patient no surgical intervention was planned and one patient died of an unknown cause before IM surgery could take place . A Ladd procedure was performed for IM correction . Twenty-eight out of the 72 operated patients needed acute IM surgery , at a median age of 4.0 days -LRB- range 0 -- 455 -RRB- . Only 4 out of these 28 patients had previously undergone surgical correction of the CCVD . Five patients had ischemic bowel disease at time of acute IM intervention ; three of these patients died , one due to massive intestinal necrosis -LRB- one patient died due to cardiovascular cause , one patient died because of severe infection -RRB- . At the time of IM surgery , signs and symptoms of CCVD , e.g. , hepatomegaly , central cyanosis , cardiac murmurs and respiratory insufficiency , were more often observed twice in patients with a major CCVD -LRB- 62.2 vs. 30.0 % , respectively , P = 0.004 -RRB- . Most common complications were intestinal -LRB- Platzbauch , leakage of the bowel anastomosis resulting in sepsis , feeding difficulties with long-term necessity of nasogastrical tube feeding -RRB- , infections and cardiovascular complications -LRB- e.g. bradycardia , thrombosis , arrhythmia -RRB- and they did not differ between both subgroups . Thirteen patients needed relaparotomy because of leakage of the bowel anastomosis -LRB- n = 4 -RRB- , Platzbauch -LRB- n = 2 -RRB- , suspected bowel obstruction with feeding difficulties -LRB- n = 1 -RRB- and necrotising enterocolitis -LRB- n = 1 -RRB- . Five patients were reoperated for an adhesion ileus , all but one more than 1 month after surgery . Table 3Characteristics of general population , major and minor congenital cardiovascular defects groups operated on intestinal malrotationCharacteristicAll CCVDMajor CCVDMinor CCVDP valueIM surgery100 % -LRB- n = 72 -RRB- 100 % -LRB- n = 34 -RRB- 100 % -LRB- n = 38 -RRB- − Physical signs CCVD prior to IM surgery48 .6 % -LRB- n = 35 -RRB- 67.6 % -LRB- n = 23 -RRB- 30.0 % -LRB- n = 12 -RRB- 0.004Post-IM-operative complications61 .1 % -LRB- n = 44 -RRB- 67.6 % -LRB- n = 23 -RRB- 55.3 % -LRB- n = 21 -RRB- 0.3Post-IM-operative cardiovascular complications18 .1 % -LRB- n = 13 -RRB- 20.6 % -LRB- n = 7 -RRB- 15.8 % -LRB- n = 6 -RRB- 0.8 Relaparotomy18 .1 % -LRB- n = 13 -RRB- 17.6 % -LRB- n = 6 -RRB- 18.4 % -LRB- n = 7 -RRB- 1.0 Age at IM surgery in days -LSB- median -LRB- range -RRB- -RSB- 17 -LRB- 0 -- 1,293 -RRB- 104 -LRB- 0 -- 1,196 -RRB- 6 -LRB- 0 -- 1,293 -RRB- 0.006 Weight at IM surgery in kilograms -LSB- median -LRB- range -RRB- -RSB- 3.21 -LRB- 1.20 -- 11.4 -RRB- 4.84 -LRB- 1.20 -- 11.4 -RRB- 2.88 -LRB- 1.24 -- 10.6 -RRB- 0.02 Death in days after IM surgery -LSB- median -LRB- range -RRB- -RSB- 28 -LRB- 0 -- 1,814 -RRB- 33 -LRB- 0 -- 1,814 -RRB- 3 -LRB- 3 -- 59 -RRB- 0.2 Age at death in days -LSB- median -LRB- range -RRB- -RSB- 40.5 -LRB- 3 -- 3,010 -RRB- 46 -LRB- 22 -- 3,010 -RRB- 8 -LRB- 3 -- 60 -RRB- 0.1 Values in bold represent statistical significant resultsCCVD Congenital cardiovascular defects , IM Intestinal malrotation Patients suffering from a post-operative IM surgery complication were younger -LRB- 5.5 vs. 94.5 days , P = 0.031 -RRB- and had a lower body weight -LRB- 2.86 vs. 4.84 kg , P = 0.003 -RRB- at time of IM surgery . Major CCVD , co-occurrence of a syndrome , or other congenital malformations did not significantly increase the risk on post-IM-operative morbidity . Correction for age and weight at the time of IM surgery in both CCVD groups showed , although not significant , a clear trend in post-IM-operative morbidity risk in patients diagnosed major CCVD -LRB- OR 2.9 , 95 % CI 0.9 -- 9.6 -RRB- . Patients with pre-operative signs of CCVD had a four times increased risk on post-operative complications -LRB- 95 % CI 1.4 -- 11.0 -RRB- . This risk factor was more prominent after correction for age and weight at the time of IM surgery -LRB- OR 8.2 , 95 % CI 2.1 -- 30.7 -RRB- . Mortality Fifteen -LRB- 19.5 % -RRB- out of 77 patients died , 9 with major CCVDs . One patient with a minor CCVD died prior to IM surgery at the age of 1.4 years ; cause of death remained unclear . Known causes of death after IM surgery were : cardiovascular complications -LRB- n = 7 -RRB- , massive intestinal necrosis -LRB- n = 2 -RRB- , sepsis -LRB- n = 2 -RRB- , respiratory failure -LRB- n = 2 -RRB- and abstention of medical care because of unsuccessful surgical intervention on malformed trachea -LRB- n = 1 -RRB- . In both CCVD groups , death due to a cardiovascular cause remained the most frequent one . Median age at time of death was 40.5 days -LRB- range 3 -- 3,010 -RRB- , with a median of 28.5 days after IM surgery -LRB- range 0 -- 1,814 -RRB- . Of the 14 deceased patients , 9 -LRB- 64.3 % -RRB- had a major CCVD . Median age at time of death in the major CCVD group was 46 days -LRB- range 22 -- 3,010 -RRB- and in the minor CCVD group 8 days -LRB- range 3 -- 60 days -RRB- , although this difference was statistically not significant -LRB- P = 0.4 -RRB- . Twenty percent of the patients -LRB- 7/35 -RRB- with pre-operative physical signs of CCVD died , only one due to a cardiovascular cause . Three out of five patients with ischemic bowel at the time of IM surgery died post-operatively . The risk factors for mortality are shown in Table 4 . Table 4Risk factors -LRB- expressed as odds ratios and 95 % confidence interval -RRB- for mortality in the whole study population -LRB- n = 77 -RRB- CharacteristicDeceasedAliveOdds ratio -LRB- 95 % CI -RRB- Adjusting for weight and age at time of IM surgery OR -LRB- 95 % CI -RRB- Major CCVD9281 .8 -LRB- 0.6 -- 5.7 -RRB- 5.0 -LRB- 1.2 -- 20.7 -RRB- Minor CCVD634Syndrome/association7340 .7 -LRB- 0.2 -- 2.2 -RRB- 1.0 -LRB- 0.3 -- 3.4 -RRB- No syndrome or association828Other congenital defects13521 .3 -LRB- 0.2 -- 6.4 -RRB- 1.1 -LRB- 0.2 -- 5.9 -RRB- No other congenital defects210Physical CCVD signs prior to IM-surgery7281.1 -LRB- 0.3 -- 3.4 -RRB- 1.3 -LRB- 0.4 -- 4.4 -RRB- No physical CCVD signs prior to IM-surgery730Intestinal ischemia327 .6 -LRB- 1.1 -- 51.2 -RRB- 5.9 -LRB- 0.9 -- 42.4 -RRB- No intestinal ischemia1156Post-IM-operative complications12711 .3 -LRB- 1.4 -- 92.3 -RRB- 8.7 -LRB- 1.0 -- 75.7 -RRB- No post-IM-operative complications1331Post-IM operative cardiovascular complications765 .5 -LRB- 1.5 -- 20.5 -RRB- 4.5 -LRB- 1.1 -- 18.4 -RRB- No post-IM-operative cardiovascular complications851Values in bold represent statistical significant resultsCCVD Congenital cardiovascular defects , IM Intestinal malrotation Discussion This study addresses the co-existence of malrotation with different types of congenital cardiovascular defects . We found 27.1 % of all the patients registered in the last 25 years for IM to have a major or minor CCVD . Major CCVDs were seen in almost half of our population . Stewart et al. -LSB- 2 -RSB- found only 12 % in a smaller IM population to have any CCVD . Complex CCVDs -LRB- 23 % of our population , 6.3 % of all patients with IM in the last 25 years -RRB- and VSDs -LRB- 21 % of our population , 5.6 % of all patients with IM in the last 25 years -RRB- were most frequently diagnosed . Pradat et al. -LSB- 17 -RSB- reported the co-occurrence of IM in patients with single ventricle to be as high as 8.5 % . Further data on co-occurrence of CCVD in IM patients are missing . The high prevalence of the CCVD in our IM cohort needs some thoughts on common aetiology . The gut is developed at a later stage in embryogenesis than the heart does . Abnormal abdominal development , e.g. by early development of an intra-abdominal cyst in embryogenesis , is known to enhance the chance of developing IM -LSB- 18 -RSB- . Changes in intra-abdominal space caused by a CCVD might also increase the risk on IM development during embryogenesis -LSB- 4 -RSB- . Another explanation might be a common genetic cause . The gut and the heart are both organs that show a characteristic left -- right asymmetry , caused by a specific biochemical cascade during embryogenesis -LSB- 19 -RSB- . Two signalling molecules inevitable for this cardiac and gastro-intestinal asymmetry are nodal and pitx2c -LSB- 20 -RSB- . Reduced , diffuse or absent expression of pitx2c in the embryo cause abnormal looping from both heart and gut in animal studies -LSB- 21 -- 24 -RSB- , resulting mainly in abnormal outflow tract orientation -LRB- e.g. unseptated and misaligned great artery trunks , symmetrical outflow tract cushions , double outlet right ventricle , common atrioventricular trunk -RRB- -LSB- 23 , 24 -RSB- . This disturbed expression of pitx2c could cause a spectrum of isomerism -LSB- 25 -RSB- . More research is needed to reveal a possible common genetic cause for both CCVD and IM . Our study group explores a high percentage of syndromes or associations . Although IM has been recognized in many syndromes and associations , this high prevalence should make us aware of co-occurrence of CCVD in these patients , as well as the possibility of a syndrome or association in patients diagnosed with both IM and CCVD . The post-operative morbidity after IM surgery in the general paediatric population is between 0 and 16.7 % -LSB- 9 , 26 -RSB- . A higher morbidity rate -LRB- 47 % -RRB- has been reported by Murphy et al. -LSB- 27 -RSB- , yet the surgical intervention was acute in 65 % of their study population . Consistent with earlier reports -LSB- 6 -- 8 -RSB- , we found post-operative morbidity and mortality to be associated with younger age at the time of surgery . However , ischemic bowel -LRB- at the time of IM surgery -RRB- did not increase post-IM-operative morbidity as reported by others -LSB- 2 , 6 , 8 , 28 -RSB- . Re-laparotomy in our population was consistent with earlier reports in the general population -LSB- 26 , 27 -RSB- . The current study showed that patients with a CCVD had a relatively high morbidity rate after IM surgery -LRB- 61.1 % -RRB- . Syndromes , associations or the presence of other congenital malformations were not found to be risk factors for post IM morbidity . Cardiovascular complications were seen in 18.1 % of our population . There are neither data on prevalence of cardiovascular complications after IM surgery in the general paediatric population , nor on influence of physical condition prior to IM surgery on the morbidity after the operation . Although the type of CCVD was not significantly associated with an increased risk on morbidity , there was a clear trend -LRB- towards increased risk on post-operative morbidity -RRB- in patients with major CCVD . Furthermore , patients with pre-operative physical signs of CCVD had a high post-operative morbidity rate -LRB- 77 % -RRB- . The overall mortality rate in literature after IM surgery varies between 3 and 9 % -LSB- 6 , 27 -- 29 -RSB- . Ilçe et al. -LSB- 8 -RSB- found a mortality rate varying between 0 and 36 % in patients mainly younger than 1 year , having almost all emergency intervention . A recent study of Murphy et al. -LSB- 27 -RSB- reported only 1 post-operative death in 46 patients due to cardiovascular consequences in a patient with congenital cyanotic heart disease . In the current study , the mortality rate was higher : almost one-fifth of the patients died . Patients who died were significantly younger at the time of IM surgery . Other risk factors for death were major CCVD -LRB- after correction for age and weight at the time of IM surgery -RRB- , ischemic bowel at the time of IM surgery , post-IM-operative complications in general and post-IM-operative cardiovascular complications . Diagnosis and treatment of cardiac diseases have incredibly improved in the last two decades . Associated congenital abnormalities , including IM , have increased in significance . However , since mortality rates after surgical intervention for IM in patients with an associated CCVD are high , intervention should be well thought over . Recent studies discuss the advantages of laparoscopic inspection for IM , also in acute paediatric abdomen -LSB- 30 -RSB- . No data on laparoscopy in IM patients with CCVD are available . Possible advantages of laparoscopy for IM diagnosis , followed by operation , even in acute cases , might improve the outcome -LSB- 30 -RSB- , even in patients with major CCVD . Referral to a centre equipped with paediatric cardioanesthesiological care is imperative . Study limitations This is a retrospective study of all consecutive live-born IM patients referred to a single university centre over a 25-year period . There are asymptomatic IM patients who never consult a doctor . Patients with IM and a very mild CCVD might have been missed . Screening for CCVD in IM patients could give us more information on the exact prevalence of CCVD in IM patients . Furthermore , since CCVD are very common congenital defects , a prospective study on the prevalence of IM in all patients with minor and major CCVD would also be of great interest . Each retrospective study has its limitations , e.g. some variables were not -LRB- yet -RRB- available ; e.g. syndromes and associations were common in our study population . Yet , the actual prevalence of syndromes might have been higher than reported . Certain syndromes and associations are quite new and nowadays there might be more attention on searching for a fitting syndrome or association diagnosis than 20 years ago . As regards treatment , we perform 3 years laparoscopic inspection , followed by correction of the anomaly by laparoscopy or laparotomy in very young children -LSB- 31 -RSB- . Conclusion The prevalence of CCVD in patients with IM is high -LRB- 27.1 % -RRB- . Major and minor CCVDs are both frequently seen . Major CCVD is a risk factor for death after IM operation . Since morbidity and mortality rates in IM patients with CCVD are much higher than previously reported , a pre-operative screening for co-existing CCVD is indicated . Elective IM surgery in young patients with major CCVD should be conducted in a centre with adequate paediatric cardiac care . The benefit of laparoscopic intervention needs further follow-up .

O O O O O O B I B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O B I O O O O O O 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O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O B I O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O O 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congenital cardiovascular defect
post-operative morbidity
post-operative mortality