DEVELOPMENT OF PULMONARY-HYPERTENSION AFTER PLACEMENT OF A VENTRICULOATRIAL SHUNT

The ventriculoatrial shunt (VAS) was developed to control hydrocephali c syndromes effectively. Several complications, however, have been des cribed after the procedure. One of the most serious consequences is th e development of severe pulmonary hypertension attributed to multiple and recurrent pulmonary embolization caused by the catheter of the VAS ; however, the frequency is exceedingly low. Herein we describe the ex perience with three patients in whom severe pulmonary hypertension dev eloped after a VAS procedure. In two patients, refractory heart failur e developed, an outcome that caused death within a brief period. The t hird patient underwent atrial thrombectomy and then pulmonary thromboe ndarterectomy; recovery was complete. Scientific evidence shows that i nitial embolization predisposes pulmonary vessels to develop further i n situ thrombosis; thus, the vascular lung disease progresses despite removal of the embolic source. A review of the literature revealed tha t in patients with a VAS, pulmonary embolism and pulmonary hypertensio n were clinically diagnosed in only 0.4% and 0.3% of the cases, respec tively, whereas postmortem diagnoses of pulmonary embolism and pulmona ry hypertension were established in 59.7% and 6.3%, respectively. Thes e discrepancies point out the difficulty of establishing the diagnosis of these serious pulmonary vascular complications while the patient i s alive.