Double aortic arch with double aneuploidy—rare anomaly in combined Down and Klinefelter syndrome

A 14-month-old boy with double aneuploidy and a double aortic arch suffered from frequently recurrent severe feeding and respiratory problems. Chromosomal analysis showed a 48,XXY + 21 karyotype: a double aneuploidy of Down syndrome (DS) and Klinefelter syndrome (KS). Only four cases of double aneuploidy (DS + KS) associated with congenital heart defects have been published of which none had a double aortic arch. Our case report should draw attention to the possibility of a double aortic arch in patients with severe feeding and respiratory problems and a double aneuploidy

Down-sizing of cryopreserved homografts is a valuable technique, but do not make them too small

Cardiolog

Fifteen years' experience with the use of artificial chords for valve reconstruction in children

OBJECTIVES: To retrospectively review our experience with artificial chords in mitral and tricuspid reconstructive surgery in children. METHODS: All consecutive paediatric ( <18 years) patients who underwent mitral or tricuspid valve reconstruction with the use of artificial chords in our centre in the past 15 years were retrospectively analysed. RESULTS: Thirty-nine patients (age 3 days to 17 years) underwent reconstruction of the mitral (n = 27) or tricuspid (n = 12) valve using artificial chords. Mean number of chords was 3.5 +/- 1.7. In 26 of 27 mitral valve patients, chords were placed on the anterior leaflet, in one on the posterior leaflet. In 10 of the 12 tricupid valve, patients chords were placed on the anterior leaflet and in 2 on the septal leaflet. All mitral patients underwent annuloplasty (10 bilateral Wooler-Kay and 17 rigid ring annuloplasty). Ten of the 12 tricuspid patients underwent annuloplasty (1 rigid ring and 9 commissural plication). Follow-up was after 8.7 +/- 5.5 years. There was no early or late mortality. The actuarial freedom from reoperation rates at 1, 5 and 10 years were 95%, 91% and 81%, respectively. No reoperations occurred in the tricuspid group. In the mitral group, there were 2 early failures and 3 late reoperations due to mitral stenosis. Restricted leaflet motion probably caused by the artificial chords was seen in only 1 patient. CONCLUSIONS: Our data show that long-term durability of mitral and tricuspid valve reconstruction using artificial chords in children is good. Despite patient growth, restricted leaflet motion by the artificial chords does not seem to form a major proble

Hybrid branch pulmonary artery stent placement in adults with congenital heart disease

Valuable treatment modalities for branch pulmonary artery (BPA) stenoses are surgical patch angioplasty, percutaneous BPA stenting and hybrid stent placement. The purpose of this study was to report our institutional experience with hybrid stent placement to relieve BPA stenoses. Between August 2007 and May 2014, 7 adults (5 females) with congenital heart disease (CHD) had elective intraoperative BPA stent placement. All 7 patients had undergone previous surgery [6 tetralogy of Fallot repairs and 1 arterial switch operation for transposition of the great arteries (TGAs)]. A total of 7 stents (4 right pulmonary artery, 3 left pulmonary artery) were implanted under direct vision, concomitant with a pulmonary homograft implantation (tetralogy of Fallot patients), or because percutaneous stenting was not feasible (TGA patient). Retrospective analysis of clinical data, procedural details and outcomes was performed. Overall, the mean age was 35 ± 7.2 years. Stent implantation was successful in all procedures. The mean postinflation stent diameter was 13.3 ± 2.0 mm. No stent migration, fracture, stent thrombosis, reintervention or deaths occurred. In 1 case the procedure was complicated by a right pulmonary artery tear just distal to the stent which was repaired by surgical patch angioplasty. At a mean follow-up of 55.6 ± 26 months no patient required catheter reintervention or surgery. Echocardiography (mean follow-up 47.1 ± 22 months), MRI (mean follow-up 43.8 ± 37 months) and CT data (mean follow-up 14.8 ± 10 months) demonstrate a BPA diameter increment from a mean 5.57 ± 2.29 to 10.71 ± 2.56 mm. Hybrid intraoperative BPA stent placement is safe and effective and can be used as an alternative for surgical patch angioplasty or if percutaneous BPA stenting is not feasible. Short- to mid-term results are goo

Influence of age on scar tissue: A retrospective study on the differences in scar tissue development between children and adults

Thoracic Surger

Continuous Postoperative Pericardial Flushing: A Pilot Study on Safety, Feasibility, and Effect on Blood Loss

Background: Prolonged or excessive blood loss is a common complication after cardiac surgery. Blood remnants and clots, remaining in the pericardial space in spite of chest tube drainage, induce high fibrinolytic activity that may contribute to bleeding complications. Continuous postoperative pericardial flushing (CPPF) with an irrigation solution may reduce blood loss by preventing the accumulation of clots. In this pilot study, the safety and feasibility of CPPF were evaluated and the effect on blood loss and other related complications was investigated. Methods: Between November 2011 and April 2012 twenty-one adult patients undergoing surgery for congenital heart disease (CHD) received CPPF from sternal closure up to 12 h postoperative. With an inflow Redivac drain that was inserted through one of the chest tube incision holes, an irrigation solution (NaCl 0.9% at 38 °C) was delivered to the pericardial cavity using a volume controlled flushing system. Safety aspects, feasibility issues and complications were registered. The mean actual blood loss in the CPPF group was compared to the mean of a retrospective group (n = 126). Results: CPPF was successfully completed in 20 (95.2%) patients, and no method related complications were observed. Feasibility was good in this experimental setting. Patients receiving CPPF showed a 30% (P = 0.038) decrease in mean actual blood loss 12 h postoperatively. Conclusions: CPPF after cardiac surgery was found to be safe and feasible in this experimental setting. The clinically relevant effect on blood loss needs to be confirmed in a randomized clinical trial

Tissue chimerism in human cryopreserved homograft valve explants demonstrated by in situ hybridization

Background. The presence of viable cells may contribute to increased homograft valve durability. These cells may be of infiltrating recipient or persisting donor origin. In this study, in situ hybridization was used to assess the origin of cells in cryopreserved homograft valve explants. Methods. A total of 10 homografts with a donor-recipient gender mismatch were acquired from patients whose graft had been explanted at reoperation or at autopsy. The period of implantation varied from 14 days to 70 months. Frozen sections were made and alternately examined with hematoxylin and eosin staining and in situ hybridization. Male cells were distinguished from female using a biotinylated Y-chromosome-specific deoxyribonucleic acid probe. Results. No endothelial cells were found. Thirty percent of the leaflets showed large acellular zones and 30% were completely acellular. The homograft arterial wall was occupied by a vast majority of penetrating host fibroblasts in 80% of the studied specimens. Donor and recipient cells were coexistent in the wall in 60% of the studied specimens and in 50% of the leaflets. In 30% only host cells could be identified. Conclusions. This finding of tissue chimerism may lead to new insights in homograft pathology. The technique of in situ hybridization may provide an indispensable contribution in further homograft research