Serial hemodynamic assessment using Doppler echocardiography in a fetus with left ventricular aneurysm presented as fetal hydrops.

A 22-week fetus presented with a large left ventricular aneurysm, 24 × 21 × 18 mm in size, detected by abnormal four-chamber view, and severe fetal hydrops with pericardial effusion, ascites and skin edema. The aneurysm was thin-walled, hypokinetic, and had enlarged with gestational age, causing compression of the lung. Although the left ventricular function had progressively impaired as expressed by increase in Tei index, hydrops had resolved by 32 weeks of gestation, probably because of maternal digoxin therapy and successful compensation by the right ventricle, as represented by retrograde blood flow in the distal aortic arch via the patent arterial duct. Because of the significant risk of severe cardiorespiratory failure, we transported the mother to a neonatal cardiac surgical center at 38 weeks of gestation. Indeed, the baby showed severe cardiopulmonary failure after birth, showing 100% of cardiothoracic ratio on the chest X-ray film, but was saved by the successful Dor procedure, including surgical resection of the aneurysm at 10 h of life. In this case, serial echocardiographic evaluation can allow us to monitor the hemodynamics and lead to successful postnatal management.

Hypogammaglobulinaemia in a patient with ring chromosome 21.

An 8 year old boy with ring chromosome 21 who was susceptible to sinorespiratory infections due to hypogammaglobulinaemia is reported. He presented with the characteristic features of monosomy 21 syndrome, such as psychomotor retardation, hypertonia, large saccular ears, prominent nasal bridge, micrognathia, thrombocytopenia, and patent ductus arteriosus. His serum IgG concentration was less than 1.5 g/l at 3 years and 6 months of age after repeated hospitalisations with pneumonia, otitis media, and convulsions. Regular replacement of intravenous gammaglobulin effectively reduced such infectious episodes. A predisposition to infection in patients with ring chromosome 21 may be explained by hypogammaglobulinaemia and merit treatment with gammaglobulin.

Influence of pulmonary blood pressure and flow on endothelin-1 production in humans.

To investigate the regulation of endothelin-1 (ET-1) production, cardiac catheterization was performed in young patients with congenital heart disease (27 +/- 5 months old, mean +/- SEM), and plasma levels of ET-1 in the inferior vena cava were measured by a sandwich-enzyme immunoassay. Plasma ET-1 levels of age-matched healthy controls were 1.55 +/- 0.07 pg/ml (n = 6). In patients with atrial septal defect [without pulmonary hypertension (PH)] who had volume but not pressure overload to the pulmonary circulation (PC), plasma ET-1 levels were significantly lower than in controls. In patients with PH due to ventricular septal defect (VSD) who had both volume and pressure overload to PC, the levels were significantly higher than in controls. In patients with PH and severe pulmonary congestion due to pulmonary venous stenosis (PVS), plasma ET-1 levels were significantly higher than in those with VSD, suggesting that ET-1 production is also augmented by pulmonary congestion. The present findings suggest that ET-1 production is increased by pressure overload to PC but decreased by volume overload to PC.

Arterial switch operation for transposition of the great arteries: surgical techniques to avoid complications.

From June 1984 to November 1990, 109 patients with transposition of the great arteries underwent arterial switch operation. There were 5 deaths, yielding a mortality rate of 4.6%. During this period, modifications of the surgical technique were devised to minimize intra- and postoperative problems, such as bleeding, kinking of the coronary arteries, aortic regurgitation and pulmonary stenosis. The surgical refinements that evolved include (1) a more distal division of the ascending aorta, (2) a punch technique for reimplantation of the coronary arteries in a medially rotated position, approximating the commissure, and superior to the upper border of the sinus of Valsalva, and (3) removal of left coronary ostia by incision down from the transected site to include a button of aortic wall, avoiding the free margin of the aorta and patch enlargement of the neopulmonary artery. Since instituting these refinements: (1) the time consumed for hemostasis after termination of the bypass considerably decreased from 111 +/- 59 to 87 +/- 51 minutes (p less than 0.05), (2) the incidence of kinking of the coronary arteries decreased from 29% (4/14) to 7% (6/88) (p less than 0.05), and (3) the occurrence of aortic insufficiency 1 year after correction was reduced from 36% (5/14) to 8% (5/66) (p less than 0.02). However, the occurrence of pulmonary stenosis with a pressure gradient greater than 30 mmHg did not decrease significantly despite aggressive modifications of surgical techniques, and its incidence in the most recent series of 32 patients was still a high 19%.

Primary repair for complete atrioventricular canal: recommendation for early primary repair.

Forty patients with complete atrioventricular canal (CAVC) underwent primary repair at Fukuoka Children's Hospital in Fukuoka, Japan, between August 1, 1981 and July 31, 1989. The age at repair ranged from 2 months to 6 years (mean 19 months); weight ranged from 2.3 to 22 kg. The surgical mortality was 2.5%. Justification for early primary repair was examined. Eleven patients underwent repair before 6 months of age (Group 1), 12 patients, between 7 and 11 months of age (Group 2), and 17 patients, after 12 months of age (Group 3). Degenerative changes in the atrioventricular valve increased significantly as age at repair increased (p less than 0.05 Group 1 versus Group 3). The incidence of residual mitral regurgitation tended to increase in the order of Group 1, 2 and 3, though the degree ranged from trivial to mild. Study of the left atrium/aorta ratio by echocardiography revealed that stable values of around 1.1 in Groups 1 and 2 and around 1.3 in Group 3 continued during the follow-up period of 3 years. Assessment of the diameter of the repaired mitral valve in the mean interval of 26 months in groups 1 and 2 revealed normal growth of the mitral valve annulus. The angle between the repaired mitral valve and ventricular septum, which can be affected by the growth of the ventricular septum, converged to normal range in the mean interval of 26 months. Postoperative pulmonary vascular resistance in Groups 2 and 3 was higher at 4.4 +/- 2.3 and 6.3 +/- 2.2, respectively, than in Group 1 at 3.3 +/- 2.2 (p less than 0.01 versus Group 3).(ABSTRACT TRUNCATED AT 250 WORDS)

[Evaluation of left ventricular function after Jatene's operation for transposition of the great arteries--influence of age at repair].

The postoperative left ventricular function (LV) of Jatene's operation for transposition of the great arteries (TGA) was evaluated by angiocardiography and echocardiography in 39 patients. In 16 patients repaired at less than 3 months of age, left ventricular endodiastolic volume (LVEDV) was significantly decreased at the angiography 2 months after repair: form 215 +/- 685% normal of preoperative LVEDV to 120 +/- 14% normal postoperatively (p less than 0.05) in 7 patients with simple TGA (Group IA), and from 220 +/- 64% normal to 130 +/- 33% normal (p less than 0.05) in 8 patients associated with ventricular septal defect (Group IIA). On the other hand, among 24 patients repaired at 3 or more months of age, 19 patients with simple TGA (Group IB) showed the significant increase of LVEDV (from 159 +/- 40% normal to 183 +/- 23% normal, p less than 0.05), and 5 patients associated with ventricular septal defect (Group IIB) showed no remarkable change (from 208 +/- 96% normal to 193 +/- 23% normal). There was no significant difference of postoperative pulmonary artery (PA) wedge pressure among the four groups. Postoperative left ventricular diastolic dimension (LVDd) revealed the tendency of normalization in all groups during the first postoperative year. Group IA and IIA completed the normalization of LVDd in 2 months and Group IIB in 6 months after the repair. However, LVDd in Group IB could not decrease to normal range even in 12 months after repair. There was a significant correlation between postoperative LVDd and the age at PA banding in 14 patients of Group IB, who had the preparatory PA banding for LV training.(ABSTRACT TRUNCATED AT 250 WORDS)

[Anatomic correction of transposition of the great arteries].

The present status of the surgical treatment of transposition of the great arteries was described by showing the analysis of our results of an arterial switch operation in addition to the review of the literatures. The arterial switch operation recently has become the operation of first choice because of its low surgical mortality (author 4.5%) and low incidence of postoperative complication. One-stage anatomic correction has been performed with low mortality less than 10% in centers dealing high volumes of neonatal surgery (author: 7%). One-stage correction is preferable and it can be accomplished with the cooperative treatment plan of surgeon and pediatrician, starting immediately after birth.