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Minimally invasive surgeries for pediatric patients have been proposed for decades, with different approaches in mind. Minimal right axillary thoracotomy (MRAT), proposed two decades ago, allows the preservation of patients\' safety alongside faster aesthetic and functional recovery. The MRAT did not become widely adopted due to the prejudice that to follow a minimally invasive approach, safety and efficacy must be compromised. With this study, we aim to compare MRAT to the standard median sternotomy approach with a focus on safety and clinical outcomes. Between January 2017 and April 2021, 216 patients diagnosed with ASD, pAVSD, or PAPVD underwent surgical repair with different approaches in the same period. MRAT was used for 78 patients, and median sternotomy was used for 138 patients. In this last group, standard median sternotomy (SMS) was used for 116 patients, while a minimal skin incision (SMS mini) was used for 22 patients. There were no major complications overall nor in each specific approach. MRAT enabled the successful repair of simple heart defects, providing similar post-operative and cardiological recovery. MRAT does not compromise patients\' safety and does not prolong the duration of surgery once the learning curve is overcome, which is generally after 15-20 consecutive operations.

Ellis-van Creveld syndrome is a rare autosomal recessive disorder caused by mutations in the EVC and EVC2 genes. The four principal manifestations are chondrodysplasia, polydactyly, ectodermal dysplasia, and congenital heart defects. We describe the case of a 7-year-old girl with Ellis-van Creveld Syndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well. The correct diagnosis of this disorder in early life is essential in the overall prognosis of the syndrome. Clinical follow-up at regular intervals is very important in these patients to institute proper managements and prevent further complications.

Limited data are available on long-term outcome after repair of partial atrioventricular septal defects (pAVSD) in adults. We sought to review our experience. Between January 1, 1957 and December 31, 2016, 179 adult patients [median age (IQR) 34 (18, 72) years] underwent primary repair of pAVSD. The most common associated defects were secundum atrial septal defect (n = 38) and ventricular septal defect (VSD) (n = 7). Left atrioventricular valve (LAVV) zone of apposition (ZOA) was complete in 47 patients and LAVV regurgitation (≥moderate) was present in 73 patients. Autologous pericardium (n = 79, 45%) and polytetrafluoroethylene felt (n = 56, 32%) were mainly used for pAVSD closure.  Repair techniques for LAVV regurgitation included: ZOA suture closure (n =  142), suture annuloplasty (n = 10) and posterior band annuloplasty (n = 9). Six had LAVV replacement. There were 61 deaths over a median follow-up of 21 years (IQR 10, 38), with only 4 early deaths. In a limited subset of patients with 80 paired measurements (n = 40), median right ventricular systolic pressure declined from 43 mm Hg (IQR 35-51) to 33 mm Hg (IQR 30-44) postoperatively (P < 0.001), and this improvement was sustained over long-term follow-up (P = 0.513). A total of 34 patients underwent a reoperation (recurrent LAVV regurgitation, n = 26; left ventricular outflow-tract obstruction, n = 7; LAVV stenosis, n = 4; patch dehiscence, n = 1) with cumulative incidence of 6% and 16% at 10 and 15 years, respectively. Repair of pAVSD in adults can be done safely with low early mortality and good long-term outcomes. Postrepair reduction of pulmonary artery pressure is significant. Despite the low re-operation rates, long-term surveillance remains essential.

We report a case of a 55-year-old male who had been diagnosed with mitral regurgitation and atrial septal defect 5 years earlier. He was referred to our institution because of worsening of mitral regurgitation accompanied by exertional dyspnea. As an echocardiography showed atrioventricular valve regurgitation and ostium primum atrial septal defect, but without ventricular septal defect, he was diagnosed as having partial atrioventricular septal defect (pAVSD). An operation was performed through median sternotomy. The anterior atrioventricular leaflet had a cleft and thickening with calcification. Suturing the cleft could not control the regurgitation. Incomplete coaptation was seen at the edge of the anastomosis site of the cleft, where the severe calcification had been identified. A rough zone including a part of the chordae tendineae was sutured in order to compensate for the gap. The atrioventricular septal defect was closed with an autologous pericardial patch. He was discharged uneventfully on the 24th postoperative day and has been followed up without complications for 1.5 years.

BACKGROUND: Partial atrioventricular canal defects (PAVC) are preferred to be repaired when diagnosed and before an operation would interfere with school. There were rare previous studies about partial atrioventricular canal defect operations in adult patients. In this single-center retrospective study, we mean to review the mid-term follow-up outcomes of late diagnosed and repaired partial atrioventricular canal defects in adult patients.
METHODS: 46 adult partial atrioventricular canal defect patients who underwent operation in West China Medical Center from 2009 to 2017 were included. Required data were obtained from operation notes, patient charts and the outpatient records.
RESULTS: Among 46 patients, 10(21.7%)were male and mean age at operation was 37.6 ± 12.4 years. 11 patients had prior arrythmia, including 8 atrial fibrillations, 2 atrioventricular blocks and 1 left bundle branch block. There were 41 patients with tricuspid valve regurgitation and 22 underwent tricuspid valvuloplasty. All the patients had mitral regurgitation. 6 patients with valve incrassation and shrinkage underwent mitral valve replacement, and the rest underwent mitral repair surgery. There was one early death post operation and no more mortalities in the following follow-up years. According to the follow-up outcomes, heart function of the patients recovered significantly, dilation of atriums and ventricles, except for left atriums, were reversed to a large extent and all but one patients\' tricuspid valve regurgitations were reduced to mild and below. 4(8.7%) patients underwent reoperation and the main reasons were arrythmia and recurrent severe mitral valve regurgitation.
CONCLUSIONS: Partial atrioventricular canal defect repair in adult patients can achieve good results. Compared with the results of patients underwent operations in preschool years, though delayed surgery timing seems to bring more preoperative complications and influences heart function, the mortality and reoperation rate are excellent.

The exact incidence and risk factors for reoperation in partial and transitional atrioventricular septal defects are unclear. The goal of this study was to assess risk factors for left atrioventricular valve and left ventricular outflow tract reoperation in partial and transitional atrioventricular septal defects.
All patients undergoing partial and transitional atrioventricular septal defects repair between 1995 and 2017 were reviewed. Patients were classified as infants (<1 year), toddlers (1-3 years), children (3-17 years), and adults (≥18 years). Survival and reoperation were assessed using log-rank test and Cox models for univariate and multivariable analyses, respectively.
Overall, 265 patients underwent partial and transitional atrioventricular septal defects repair (partial: 177 [67%]). Median age was 2 years. The cohort included 73 infants (28%), 85 toddlers (32%), 94 children (35%), and 13 adults (5%). Trisomy 21 was present in 76 patients (29%), and in 216 patients (83%), the zone of apposition was completely closed. Perioperative mortality was 0.8%. Complete heart block did not develop in any patients. Ten-year survival and freedom from reoperation were 98% and 81%, respectively. On multivariable analysis, trisomy 21 (hazard ratio [HR], 0.16) and older age compared with infants (toddlers: HR, 0.35; children: HR, 0.25) were protective for any reoperation, whereas heterotaxy (HR, 3.43) was a risk factor. For left atrioventricular valve reoperation, toddlers (HR, 0.35), children (HR, 0.25), and trisomy 21 (HR, 0.16) remained protective, whereas left atrioventricular valve anomaly was a risk factor (HR, 2.61). Likewise, for left ventricular outflow tract reoperation, toddlers (HR, 0.24) and children (HR, 0.06) were protective.
Mortality after partial and transitional atrioventricular septal defects repair is minimal, yet reoperation for left atrioventricular valve disease and left ventricular outflow tract obstruction remains significant. Patients requiring repair during infancy are at higher risk of reoperation.