Invalidating headaches as a symptom of pulmonary embolism in a Dandy-Walker syndrome with ventriculoatrial shunt

Abstract

Ventriculoatrial shunt (VAS) is an accepted treatment for hydrocephalus, submitting intra-cranial pressure to right atrial pressure. Several pathological situations can increase right atrial pressure, frequently as a consequence of pulmonary hypertension, and lead to the recurrence of intra-cranial hypertension. We report an unusual aetiology of invalidating headaches in a 26-year-old woman with history of Dandy-Walker syndrome treated initially by multiple ventriculoperitoneal shunts (VPS) switch 2 years ago by a VAS. Congenital heart disease was excluded during the childhood. She was current smoker, obese (body mass index 32.5 kg/m 2 ) and took combined oral contraceptive pill. She also reported a stage II NYHA class dyspnoea aggravated for 2 weeks. On physical examination, the heart rate was 95 beats per minute and the blood pressure 120/70 mmHg. She had an elevated jugular venous pressure and an accentuated pulmonary component of the second heart sound. Cerebral computed tomography (CT) showed a DandyWalker cyst in the posterior fossa (Fig. 1a). ECG reported a sinus rhythm with tachycardia, S1Q3 pattern and negative T waves in the right precordium (Fig. 1b). Arterial blood gas showed hypoxemia (PaO 2 70 mmHg) and hypocapnia (PaCO 2 32 mmHg; pH 7.43). CT pulmonary angiogram confirmed lingular pulmonary emboli with chronic pulmonary embolism signs as pulmonary artery enlargement and pulmonary vascular thickening (Fig. 1c). There was no abnormality of VAS catheter confirmed by transesophageal echocardiography but there was an interauricular septum curve inversion with a right ventricle dilatation (Fig. 1d), and transthoracic echocardiography confirmed a pulmonary hypertension with 65 mmHg systolic pulmonary arterial pressure. There was no intra-cardiac thrombosis. Bilateral leg venogram ultrasound was normal. The final diagnosis was pulmonary emboli with chronic pulmonary heart complicated by exacerbation of intracranial hypertension because of right atrium pressure overload. Search of thrombophilia was negative. Finally, the VAS was replaced by a ventriculoperitoneal shunt, permitting the retrocession of the pain. Retrospective test of the device didnÕt shown dysfunction. Two years later, she is still alive. She takes an oral anticoagulation and reports a NYHA stage 2 dyspnoea. Transthoracic echocardiography follow-up shows pulmonary hypertension stability. DiscussionVentriculoatrial shunt became accepted treatment for hydrocephalus in the late 1950s [1]. Previous studies reported development of severe pulmonary hypertension after placement of a VAS [2][3][4]. In patients with a VAS, pulmonary embolism and pulmonary hypertension were recognized clinically in 0.4% and 0.3%, respectively, whereas, at necropsy the frequency of these complications was 59.7% and 6.3% respectively [2]. Several hypotheses could explain this phenomenon: latent shunt infection could induce a persistent activation of clotting factors, which leads to recurrent pulmonary embolism [5]; other hypothesis suggests a possible role for cerebrosp...

Thrombosis associated with ventriculoatrial shunts

“…When the tip of the catheter moves out of the atrium and into the great vessels, increasing the risk of thrombosis [38]. Other thromboembolic risk factors include SVC stenosis, current smoking, oral contraceptive, obesity, parenteral nutrition, and malignancy [21,[39][40][41][42]. Distal tip thrombsis may occur more commonly in patients with these factors because the blood is in hypercoagulative state.…”

“…It means the total incidence of PE during life may be higher, with most emboli remaining clinically undetected, which may lead to potentially increased incidence of PH and cor pulmonale. In VAS patients, PH were recognized clinically in 0.3%, whereas postmortem diagnoses of PH were established in 6.3% [ 10 , 21 ].…”

“…Bacteria are known to attract complement and clotting factors [ 14 ]. Therefore, the presence of latent infection at the site of the shunts could induce a persistent activation of thrombotic factors and coagulation cascade, leading to thromboembolic events [ 21 , 29 , 30 ]. Hyperinflammation with cytokines storm could also be potential mechanism of shunting infection-related hypercoagulable state [ 31 ].…”

“…Some pathological situations can increase right atrial pressure, frequently as a consequence of PH, and lead to the recurrence of intracranial hypertension characterized by headache, nausea, vomiting, etc. [ 21 ]. Patients with normal pressure hydrocephalus may present with the “classic” triad of symptoms of hydrocephalus including psychomotor retardation, gait unsteadiness, and urinary incontinence associated with ventricular dilation [ 44 ].…”

“…It is important to note that a VAS may be unsuitable for those patients who are complicated by heart disease, respiratory disease, coagulation disturbance, hereditary hypercoagulability diseases, history of thrombosis, and scarring at the insertion site in the neck [ 74 ]. In addition, because of the high prevalence of PE and PH, indication of VAS has to be retained considering thromboembolic risk factors as SCV stenosis, current smoking, oral contraceptive, obesity, or intravenous alimentation [ 3 , 21 , 39 ].…”