Screening for pulmonary arteriovenous malformations using transthoracic contrast echocardiography: a prospective study.

Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of the present study was to prospectively establish the diagnostic value of transthoracic contrast echocardiography (TTCE) as a screening technique for PAVM using chest high-resolution computed tomography (HRCT) as the gold standard for PAVMs. All consecutive adult patients referred for HHT screening underwent a chest HRCT (n = 299), TTCE (n = 281), arterial blood gas analysis (n = 291), shunt fraction measurement (n = 111) and chest radiography (n = 296). TTCE was positive in 87 (58.8%), 12 (16.7%) and four (6.7%) patients, and chest HRCT was positive in 54 (36.5%), three (4.2%) and zero (0%) patients with a definite, possible and negative clinical diagnosis of HHT, respectively. Two patients with a negative TTCE were diagnosed with PAVMs after computed tomography; in both cases the PAVMs were too small to be treated by embolotherapy. The sensitivity of TTCE was 97% (95% confidence interval (CI) 93.6-98.3) and negative predictive value 99% (95% CI 96.9-99.8). The other diagnostic tests showed a considerable lower diagnostic value. The present prospective study shows that transthoracic contrast echocardiography has an excellent diagnostic value and can be used as an initial screening procedure for pulmonary arteriovenous malformations. The high false-positive rate of transthoracic contrast echocardiography possibly represents microscopic pulmonary arteriovenous malformations.

Pulmonary arteriovenous malformations associated with migraine with aura.

Migraine with aura (MA) is associated with cardiac right-to-left shunt. We prospectively studied the association between pulmonary arteriovenous malformations (PAVMs) and MA in hereditary haemorrhagic telangiectasia (HHT). All 220 consecutive HHT patients who underwent high-resolution chest computed tomography for PAVM screening were included prospectively. Prior to screening, a structured validated headache questionnaire was completed by 196 patients (57% female; mean+/-sd age 44.6+/-15.2 yrs). Two neurologists diagnosed migraine according to the International Headache Society Criteria. A PAVM was present in 70 (36%) patients. The prevalence of MA was 24% in the presence of a PAVM compared with 6% in the absence of a PAVM (OR 4.6, 95% CI 1.84-11.2; p = 0.001), and MA was an independent predictor for the presence of PAVM using multivariate analysis (OR 3.6, 95% CI 1.21-10.5; p = 0.02). A PAVM was present in 68% of the patients with MA compared with 32% in the non-migraine controls (OR 4.6, 95% CI 1.84-11.2; p = 0.001), and a PAVM was an independent predictor for MA using multivariate analysis (OR 3.0, 95% CI 1.00-9.20; p = 0.05). In conclusion, PAVMs are associated with MA in HHT patients.

Pulmonary arteriovenous malformations and migraine: a new vision.

Migraine is a common neurological disorder with a great impact on the quality of life and social activities. Pulmonary arteriovenous malformations (PAVMs) are mostly congenital, with a prevalence of 5-50% in patients with hereditary hemorrhagic telangiectasia (HHT). A high prevalence of PAVMs is found in patients with HHT and migraine. Embolization of PAVMs seems to decrease the prevalence of migraine. Different pathophysiological hypotheses have been proposed to explain the association between migraine and the different right-to-left shunts. This review article describes the association between a pulmonary right-to-left shunt and the occurrence of migraine.

[Subarachnoid haemorrhage presenting clinically as circulatory arrest in acute myocardial infarction]. / Subarachnoïdale bloeding met de klinische presentatie van een circulatiestilstand bij acuut myocardinfarct.

A 59-year-old woman and a 41-year-old man were both brought to the Cardiac Emergency Clinic with circulatory arrest on the basis ofpulseless electrical activity. The first patient had had no prodromal symptoms and the second patient had visited his general practitioner 2 weeks earlier because of pain in the head and neck. In both patients, electrocardiography and echocardiography suggested acute myocardial infarction. However, both patients proved to be suffering from a subarachnoid haemorrhage (SAH) and both died. One-third ofthe patients with SAH are comatose at presentation. Cardiac manifestations such as ECG-abnormalities, cardiac arrhythmias, cardiopulmonary arrest, elevated troponin values, and signs of left ventricular dysfunction are common. These findings can be misleading and may have catastrophic consequences if anticoagulant therapy is initiated because of a presumed myocardial infarction. Low-threshold CT-scanning of the brain is therefore advised for patients who remain comatose after resuscitation for cardiac arrest in the presence of an atypical anamnesis.