Early parental adjustment to visible congenital disfigurement

Summary When a baby is born with a visible disfigurement, then parents need to adjust to the loss of the anticipated 'perfect' child and thus accept their baby. The impact of the birth on the parents is described in the context of a measure which identifies areas of potential difficulty. The two groups studied were parents of children with cleft palates and parents of children with congenital hand deficit. A wide range of adjustment was found. There was no significant difference between the two groups in terms of their overall adjustment, but there were individual differences in adjustment which did not relate to the severity or type of anomaly. The only significant variable found to relate to parental adjustment was perceived family support.     

Success determining factors in percutaneous transluminal balloon valvuloplasty of pulmonary valve stenosis

Twenty-two percutaneous transluminal balloon valvuloplasty procedures were performed on 21 patients with congenital pulmonary valve stenosis. The peak systolic pressure gradient was immediately reduced from 79.1 +/- 7.4 to 22.2 +/- 1.8 mmHg, (P less than 0.0001) and follow-up cardiac catheterization at 5.3 +/- 0.4 months in 19 patients revealed no further significant change in gradient (23.5 +/- 3.2 mmHg). The best results were obtained when balloons larger than the pulmonary annulus were used, i.e. an immediate residual transvalvular gradient of 22.0 +/- 2.2 mmHg with a balloon/annulus ratio greater than 1, as opposed to 44.2 +/- 5.4 with a balloon/annulus ratio = 1 (P less than 0.001). The angiographically determined cusp thickness of the stenotic pulmonary valves was significantly greater than that of the control group of 24 patients without pulmonary valve stenosis (1.21 +/- 0.09 vs 0.59 +/- 0.02 mmHg, P less than 0.00001). The relationship between this parameter and the residual transvalvular gradient at follow-up was found to be significant (r = 0.77, P less than 0.001). It is concluded that balloon size is a determinant factor in achieving good results with percutaneous balloon valvuloplasty although cusp thickness, a factor to which scant regard has hitherto been paid, also plays a significant role in the residual transvalvular gradient measured at follow-up.     

先天性一侧肺动脉缺如的电子束CT诊断

目的 评价电子束CT(EBCT)诊断先天性一侧肺动脉缺如的价值。方法 对经平片、超声心动图检查后拟诊为肺血管疾病或原发性肺动脉高压的患者行EBCT检查，EBCT诊断先天性一侧肺动脉缺如的11例患者入选，并与超声心动图、核素通气灌注扫描、心血管造影的检查结果作进一步的比较及评估。结果 单发一侧肺动脉缺如4例，均为女性成年人。合并多发心血管畸形7例，其中合并复杂畸形3例，均为男性儿童和左肺动脉缺如；合并单发心血管畸形4例，其中3例为右肺动脉缺如。结论 (1)儿童时期明确诊断的一侧肺动脉缺如多合并有心血管畸形，且左肺动脉缺如多见，成年人明确诊断的单发一侧肺动脉缺如多为右肺动脉缺如。(2)EBCT对先天性一侧肺动脉缺如的诊断有较高的实用价值，较之多普勒超声更为准确，与心血管造影各具独特优势，但EBCT为无创检查是其特点。     

Cosmetic benefits of lower midline skin incision for pediatric open heart operation

OBJECTIVE: Since we developed the procedure in 1996, we have now performed 100 pediatric open heart operations using a lower midline skin incision and a minimal sternotomy approach. METHODS AND RESULTS: To elucidate the benefits of this approach, we analyzed these 100 cases retrospectively. There was no death, and no major complication, caused by this approach, and the resulting scarring in each patient is difficult to be seen under a common undershirt. CONCLUSION: This review shows that the technique of a lower midline skin incision and minimal sternotomy approach is a safe reliable and cosmetically advantageous method for a pediatric cardiac operation.     

先天性心脏病患儿肺动脉,上腔静脉血一氧化氮含量检测及意义

目的:探讨一氧化氮(NO)与先天性心脏病(CHD)引起的肺动脉高压(PH)发病间的关系。方法:应用NO试剂盒检测了CHD患儿肺动脉及上腔静脉血浆中NO含量。结果;(1)伴PH组肺动脉血浆NO含量明显高于不伴肺动脉高压组(37.58±9.99μmol/L:19.03±15.25μmol/L,P<0.01);(2)在PH组中,肺动脉血浆NO含量明显高于上腔静脉血(P<0.01);而不伴PH组,肺动脉和上腔静脉血浆NO含量无显著性差异(P>0.05)。结论:(1)伴PH的先心患儿肺动脉血浆NO含量升高;(2)NO可能介入了CHD引起的PH发病过程。     

Cohen syndrome: fertility in a female patient

In this report we describe fertility in an adult female with Cohen syndrome. She gave birth to a son, now 1.5 years old, with discrete facial stigmata and slight psychomotor retardation.     

Persistent left superior vena cava draining to left atrium with normal coronary sinus

In embryology, a persistent connection of the left superior vena cava to the left atrium rarely coexists with a coronary sinus. We herein report an unusual case of persistent left superior vena cava terminating in a left atrium with normal coronary sinus, which was revealed at the time of permanent pacemaker implantation after a second operation for recurrent left atrioventricular valve regurgitation. Because this anomaly had gone undiagnosed at the first operation, we were unable to diagnose it prior to the second operation, because the preoperative coronary angiogram clearly demonstrated a coronary sinus that was not dilated. We would have repaired the anomaly using a patch or other procedure had it been diagnosed before the second operation in order to prevent cyanosis or brain complications secondary to right-to-left shunting. One should always be on guard for this rare condition.