Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated anomalies and outcome.

OBJECTIVE: To assess the accuracy of prenatal diagnosis, the association with genetic and extracardiac anomalies, and outcome in fetuses with isolated pulmonary atresia with ventricular septal defect (PA-VSD). DESIGN AND SETTING: Retrospective study in a tertiary centre for fetal cardiology. PATIENTS AND OUTCOME MEASURES: Echocardiographic video recordings of 27 consecutive fetuses with PA-VSD were reviewed for: (1) intracardiac anatomy; (2) presence of confluence and size of the branch pulmonary arteries; (3) source of pulmonary blood supply; and (4) side of the aortic arch. Postmortem and postnatal data were added. Karyotyping was performed in 25 patients and, in 23 of these, fluorescent in situ hybridisation to identify 22q11.2 deletion. RESULTS: PA-VSD was correctly diagnosed in 19 of 21 patients (90%) with postnatal or autopsy confirmation of diagnosis. Central pulmonary arteries were correctly identified in 79% (15/19), the source of pulmonary blood supply in 62% (13/21) and major aortopulmonary collateral arteries in 44% (4/9). Aneuploidy was detected in 4 of 25 patients (16%) and 22q11.2 deletion in 6 of 23 patients (26%). Five of 27 patients (19%) had extracardiac anomalies. Eleven pregnancies were interrupted. Eleven of 16 liveborn babies survived. Neonatal survival was 15 of 16 (94%, 95% confidence interval (CI) 70 to 100), one-year survival was 9 of 12 (75%, 95% CI 43 to 95) and two-year survival was 5 of 9 (56%, 95% CI 21 to 86). CONCLUSION: PA-VSD can be diagnosed by fetal echocardiography with a high degree of accuracy. However, it can be difficult to determine the morphology of the central pulmonary arteries and to locate the source of pulmonary blood supply. In most liveborn infants, complete surgical repair can be achieved.

Successful outcome in a fetus with an extremely low heart rate due to isolated complete congenital heart block.

Isolated complete congenital heart block (CCHB) in a fetus is usually associated with the presence of autoantibodies to SSA (Ro) and SSB (La) antigens in the maternal circulation. Although the prognosis for the majority of fetuses is good, it is less favorable in fetuses with a ventricular rate < 55 bpm in early pregnancy or with a decrease in the ventricular rate by >/= 5 bpm during pregnancy. It is not known if the same prognostic criteria apply for the occasional fetus with isolated non-autoimmune CCHB. We report a case of a single fetus with an isolated non-autoimmune CCHB with an extremely low ventricular rate (37 bpm) in which the outcome was favorable. Dilated cardiomyopathy is a rare complication in patients with isolated CCHB, despite early institution of cardiac pacing, and is usually recognized after several months of relative well-being. It is assumed that in the majority of patients it represents a sequel to in utero autoimmune or postnatal reactivation myocarditis. However, the possibility of a tachycardia-induced cardiomyopathy caused by an excessively high pacing rate should also be taken into consideration, as was clearly demonstrated in our patient.

Autologous placental blood transfusion after a planned neonatal pacemaker implantation.

A fetal third-degree atrio-ventricular block was diagnosed in a 22-year-old woman at 24 weeks of gestation. During clinical follow-up a mild to moderate tricuspid regurgitation was detected at 35 weeks and maternal connective tissue diseases were excluded. Early postnatal cardiac pacing therapy was planned and autologous placental blood transfusion was proposed for the treatment of probable blood loss due to pacemaker implantation. A male infant was delivered at 38 weeks vaginally and 87 mL of placental blood was collected from the undelivered placenta. The placental blood was negative for viral markers and syphilis. Subsequent tests for bacteriological cultures were also negative. Within 6 h of delivery, the baby underwent cardiac pacemaker implantation and received 45 mL of autologous placental blood. Autologous placental blood transfusion was successfully used for the treatment of predicted blood loss after a planned neonatal surgical procedure.

[A biomechanical solution for permanent anchoring of artificial hip joint acetabula]. / Eine biomechanische Lösung zur dauerhaften Verankerung künstlicher Hüftgelenkspfannen.

This paper describes models of artificial acetabula which are better adapted to the structural conditions of the fixation bed than the prostheses used hitherto. The most important design feature is the increased area of surface contact between the artificial acetabulum and bone by means of stepped anchoring pins, which extend radially into the spongiosa from the surface of the acetabulum. The location and length of these small anchoring pins depends on the bone architecture of the acetabulum.