Disease characteristics and surgical effect of 22 children with Shone′s syndrome / 中华实用儿科临床杂志

Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.

Estudo anatômico do posicionamento do anel aórtico em relaçäo ao anel mitral / Anatomic study between aortic and mitral rings

Objetivo Avaliar as relações entre os anéis aórtico e mitral. Métodos Cem corações humanos normais, em 61% de indivíduos brancos, em 84%, de homens e em 85%, na faixa de 11 a 40 anos. O ponto de referência no anel aórtico foi a comissura entre os seios não coronariano e coronariano esquerdo; no anel mitral foi o meio caminho entre os trígonos anterior e posterior. A coincidência dos pontos de referência caracterizou a posição média, a referência aórtica mais próxima do trígono posterior, a posição posterior e a mais próxima do trígono anterior, a anterior ou muito anterior. Resultados Observamos 47% de posição média, 27% de muito anterior, 22% de anterior e 4% de posterior. Estes percentuais não foram influenciados por sexo e idade. Os corações de indivíduos negros evidenciaram maior freqüência de posição posterior do que os demais. Conclusão Corações considerados normais apresentam variações na interrelação dos anéis mitral e aórtico.

Purpose Evaluate the relations between aortic and mitral rings. Methods One hundred hearts were studied, in 84% men, 61% white and 85% were between 11 and 40 years old. The commissure between non coronary/left coronary sinus in the aortic ring and the middle point between anterior (AT) and posterior trigone (PT) in the mitral ring were used as references. When these points werencoincidents the position was middle; when the aortic point (AP) was closer the PT the position was posterior and when the AP was closer the AT the position was anterior or much anterior. Results Forty seven (47%) showed middle position, twenty seven (27%) much anterior, twenty two (22%) anterior and only four (4%) posterior. These arent related to sex and age. The hearts of black people showed more posterior position than the others. Conclusion Variations in aortic/mitral rings relations may be observed in normal hearts

Mitral Ring Motion and Transmitral Blood Flow Velocity in Dilated Cardiomyopathy

Mitral ring motion and indices of left ventricular diastolic filling were measured by M-mode and Doppler echocardiography in apical 4 chamber view in 11 dilated cardiomyopathy patients and 9 normal subjects without clinical evidence of heart disease. The mean age of patients was 52 years and average heart rate was 76 beats/min. The parameters of mitral annulus motion include earley relaxation amplitude(ER), late atrial contraction amplitude(AC) and A2-peak excursion(A2-PE). Transmitral flow velocity parameters include peak flow velocity of early diastolic flow velocity(PFVE), peak flow velocity of late atrial contraction(PFVA), the ratio between early and late peak flow velocity(PFVE/PFVA), Acceleration rate of early diastolic peak flow(AR), deceleration rate of early diastolic peak flow(DR), time velocity integral of early diastolic flow velocity(TVIE), time velocity integral of late atrial contraction flow velocity(TVIA) and ratio between early diastolic and late atrial flow velocity integral(TVIE/TVIA). In patients with dilated cardiomyopathy, ER(4.5+/-2.3mm) and AC(2.3+/-1.6mm) were significantly decreased than normal(10.7+/-2.6mm, 6.6+/-1.6mm, p<0.01, p<0.01, respectively), whereas ER/AC(1.7+/-0.7) was not significantly different than normal subjects(1.6+/-0.5). A2-PE(100+/-80 msec) was significantly delayed in dilated cardiomyopathy patients than normal subjects(35+/-25 msec, p<0.01). In analysis of transmitral flow velocities, PFVE, PFVA and PFVE/PFVA, etc were not significantly different compared to normal subjects in patients with dilated cardiomyopathy. Mitral ring motion amplitude was decreased and A2-peak excursion time interval(A2-PE) was delayed in patients with dilated cardiomyopathy, but transmitral flow velocities were not significantly different from normal subjects in patients with dilated cardiomyopathy. These results reflect the facts that early diastolic relaxation amplitude is decreased by the change of compliance of LV and late atrial contractin amplitude is decreased by decrease of atrial contractility and increased stiffness of LA and LV. Despite of decreased mitral ring motion, transmitral flow velocity is not significantly different compared to normal subjects in patients with dilated cardiomyopathy. From these evidences, not only transmitral flow velocity affected by multiple factors but also mitral ring motion affected by LA and LV function are considered in assessment of LV diastolic dysfuction.