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Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P

Studies on ventricular septal defects closure by catheterization confirm its feasibility without reporting clearly the indications and difficulties encountered.From 2001 to end-2006, 22 patients benefited from 26 ventricular septal defects closure (15 muscular and 7 membranous) at a median age and weight of 2.1 years and 12.5 kg, respectively. A perventricular catheterization was performed in 2 cases. Eighteen patients (82%) benefited from 21 prostheses with success. The closure was associated to surgery in 9 cases (41%) whereas it substituted surgery in the other 13 cases (59%). The median duration of the procedure was significantly longer in case of muscular ventricular septal defects (215 min (175-510) vs. 170 min (120-225), p=0.04). Major complications are reported in 5 cases out of 26 catheterization (19%), including one death related to conduction block, occurring after the implantation of two prostheses in a patient with aortopulmonary transposition. All other associated cardiac diseases have been corrected. A prosthetic emboli occurred in one case, 1.5 months after implantation. It had been retrieved by catheterization. Two patients died afterwards from non-procedure-related causes. After a median follow-up of 1.1 years, the 17 other patients remained asymptomatic. One child with a perimembranous prosthesis presents a paroxystic atrio-ventricular block.Even though indispensable for the curative treatment of several congenital cardiac diseases including non-operable ventricular septal defects, this procedure is related to a substantial rate of mortality and morbidity. The risk of atrio-ventricular block must be adequately considered in case of membranous ventricular septal defects.

For 3 years we have used extracorporeal assistance in intensive care frequently. This study evaluates our results.We studied the patient records for those treated between January 2002 and January 2005. The method used, indications and morbidity/mortality were analysed.We performed 24 circulatory assistance procedures in 20 patients (median age: 5 months), arterio-venous with oxygenation (n=18), veino-venous with oxygenation (n=3) or biventricular (n=3). The indications were post cardiotomy cardio-respiratory failure (Group I; n=20, 16 patients), pure respiratory failure (Group II: n=1), or pre-transplant/recovery (Group III: n=3). Five procedures (4 from group I and 1 from group III) required cardiac massage (no fatalities). The average duration of assistance was 7 +/- 6 days (2 to 20 days). Treatment was successfully discontinued in sixteen patients 80%), one of them thanks to heart transplant. Four (20%) died during assistance. The morbidity essentially consisted of further surgery for haemostasis, multiple transfusions, and infections. Three patients (15%) died later (1 at 17 months after discontinuation) from complications unrelated to the assistance. No neurological sequelae were noted in the survivors.These results confirm the usefulness of circulatory assistance when medical treatment has failed, particularly in the post-operative period of paediatric cardiac surgery or while awaiting transplantation.

The authors report a rare case of coexistence of a pulmonary arteriovenous fistula with rheumatic mitral disease. The diagnosis was suspected clinically and with the chest X-ray, and was confirmed by the cardiac catheterization showing mitral disease and a pulmonary arteriovenous fistula with arterial desaturation (70%). Successful surgery (aneurysmal excision and closure of abnormal communication together with mitral valve repair) was undergone under cardiopulmonary bypass. In the literature, 7 cases of this association have been reported, with surgery in 4. None of the cases had a procedure similar to the above-mentioned and simultaneous surgery was not recommended. The authors show that the anatomic situation of the case reported allowed simultaneous surgery, and that cardiopulmonary bypass rendered the procedure easy.

Left ventricular endomyocardial fibrosis accompanied by severe mitral insufficiency occurring in 4 adolescent African patients is reported. Mitral valve repair was successfully performed in all 4 cases including annuloplasty in 3 and chordal shortening in one. Short-term follow-up of up to 10 months indicated substantial clinical improvement and decrease of the cardiothoracic ratio in all. It is emphasized that mitral valve repair appears to be the method of choice in treating mitral incompetence in the localized form of endomyocardial fibrosis, where endocardiectomy is not required in the area of the papillary muscles.