Development of pulmonary hypertension after placement of a ventriculoatrial shunt

The ventriculoatrial shunt (VAS) was developed to control hydrocephalic syndromes effectively. Several complications, however, have been described after the procedure. One of the most serious consequences is the 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 experience with three patients in whom severe pulmonary hypertension developed after a VAS procedure. In two patients, refractory heart failure developed, an outcome that caused death within a brief period. The third patient underwent atrial thrombectomy and then pulmonary thromboendarterectomy; recovery was complete. Scientific evidence shows that initial embolization predisposes pulmonary vessels to develop further in situ thrombosis; thus, the vascular lung disease progresses despite removal of the embolic source. A review of the literature revealed that in patients with a VAS, pulmonary embolism and pulmonary hypertension were clinically diagnosed in only 0.4% and 0.3% of the cases, respectively, whereas postmortem diagnoses of pulmonary embolism and pulmonary hypertension were established in 59.7% and 6.3%, respectively. These discrepancies point out the difficulty of establishing the diagnosis of these serious pulmonary vascular complications while the patient is alive.