Introduction 1 2 3 4 5 6 6 9 10 11 6 3 12 13 14 15 16 17 The purpose of our study was (1) 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 (2) 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 P U P P P  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 (PPHT), 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 (27.1% of the total IM population in the last 25 years) for further analysis. The gestational age range was 26–42 weeks with a median of 39 weeks. Median birth weight was 2.86 kg (range 0.72–4.53). A female predominance was seen with 47 girls (61%) and 30 boys (39%). Median follow-up period after IM diagnosis was 2.8 years (range 0–24 years). The overall survival at the end of follow-up was 80.5% (15 out of 77 patients died). Congenital cardiovascular defects 1 Table 1 Types of major and minor congenital cardiovascular defects Major CCVD n Minor CCVD n Complex cardiovascular defects n Ventricular septal defect n Tetralogy of Fallot n Atrium septal defect n Hypoplastic left heart syndrome n Peripheral pulmonary stenosis n Atrioventricular septal defect n Persistent ductus arteriosus n Pulmonary valve atresia n Pulmonary valve stenosis n Transposition of the great arteries n Aortic valve stenosis n Tricuspid valve atresia n Aortic coarctation n Common arterial trunk n Hypertrophic cardiomyopathy n Aortic arch hypoplasia n Subvalvular aortic valve stenosis n Dextrocardia n Left superior caval vein n Arteria lusoria n CCVD  n 2 n n Table 2 Characteristics of studied population and its subgroups of congenital cardiovascular defects Characteristic All CCVD Major CCVD Minor CCVD P  (n n n Boys (n (n (n 0.85 (0.5–1.4) Syndromes or associations n (n (n 1.8 (1.1–3.1) Other congenital defects (n (n (n 0.95 (0.5–1.8) Cardiac surgery (n (n n 4.3 (2.0–9.1) CCVD diagnosed before IM  (n (n (n 2.7 (1.4–5.2) Age at CCVD diagnosis in days [median (range)] 4.5 (0–210) 4 (0–98) 7 (0–210) P Birth weight in kilograms [median (range)] 2.86 (0.72–4.53) 2.96 (0.96–4.53) 2.84 (0.72–4.30) P Age at first CCVD surgery in days [median (range)] 102 (2–3,759) 43 (2–3,759) 117 (24–1,705) P Values in bold represent statistical significant results CCVD  IM  Congenital malformations, syndromes and associations 2 n n n n n n n n n Intestinal malrotation surgery 3 P n n n n Table 3 Characteristics of general population, major and minor congenital cardiovascular defects groups operated on intestinal malrotation Characteristic All CCVD Major CCVD Minor CCVD P  IM surgery (n  (n  (n − Physical signs CCVD prior to IM surgery (n (n  (n 0.004 Post-IM-operative complications  (n (n  (n 0.3 Post-IM-operative cardiovascular complications  (n (n  (n 0.8 Relaparotomy  (n  (n  (n 1.0 Age at IM surgery in days [median (range)] 17 (0–1,293) 104 (0–1,196) 6 (0–1,293) 0.006 Weight at IM surgery in kilograms [median (range)] 3.21 (1.20–11.4) 4.84 (1.20–11.4) 2.88 (1.24–10.6) 0.02 Death in days after IM surgery [median (range)] 28 (0–1,814) 33 (0–1,814) 3 (3–59) 0.2 Age at death in days [median (range)] 40.5 (3–3,010) 46 (22–3,010) 8 (3–60) 0.1 Values in bold represent statistical significant results CCVD  IM  P P Mortality n n n n n P 4 Table 4 n Characteristic Deceased Alive Odds ratio (95% CI) Adjusting for weight and age at time of IM surgery OR (95% CI) Major CCVD 9 28 1.8 (0.6–5.7) 5.0 (1.2–20.7) Minor CCVD 6 34 Syndrome/association 7 34 0.7 (0.2–2.2) 1.0 (0.3–3.4)  No syndrome or association 8 28 Other congenital defects 13 52 1.3 (0.2–6.4) 1.1 (0.2–5.9) No other congenital defects 2 10 Physical CCVD signs prior to IM-surgery 7 28 1.1 (0.3–3.4) 1.3 (0.4–4.4) No physical CCVD signs prior to IM-surgery 7 30 Intestinal ischemia 3 2 7.6 (1.1–51.2) 5.9 (0.9–42.4) No intestinal ischemia 11 56 Post-IM-operative complications 1 27 11.3 (1.4–92.3) 8.7 (1.0–75.7) No post-IM-operative complications 13 31 Post-IM operative cardiovascular complications 7 6 5.5 (1.5–20.5) 4.5 (1.1–18.4) No post-IM-operative cardiovascular complications 8 51 Values in bold represent statistical significant results CCVD  IM  Discussion 2 17 18 4 19 20 21 24 23 24 25 9 26 27 6 8 2 6 8 28 26 27 6 27 29 8 27 30 30 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 (yet) 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. 31 Conclusion The prevalence of CCVD in patients with IM is high (27.1%). 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.