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Original Research: Pulmonary Vascular Disease |

Grade of Pulmonary Right-to-Left Shunt on Contrast Echocardiography and Cerebral ComplicationsPulmonary Right-to-left Shunt Grade: A Striking Association

Sebastiaan Velthuis, MD; Elisabetta Buscarini, MD, PhD; Marco W. F. van Gent, MD, PhD; Pietro Gazzaniga, MD, PhD; Guido Manfredi, MD, PhD; Cesare Danesino, MD, PhD; Wouter J. Schonewille, MD, PhD; Cornelis J. J. Westermann, MD, PhD; Repke J. Snijder, MD; Johannes J. Mager, MD, PhD; Martijn C. Post, MD, PhD
Author and Funding Information

From the Departments of Cardiology (Drs Velthuis, van Gent, and Post), Neurology (Dr Schonewille), and Pulmonology (Drs Westermann, Snijder, and Mager), St. Antonius Hospital, Nieuwegein, The Netherlands; the Departments of Gastroenterology (Drs Buscarini and Manfredi) and Cardiology (Dr Gazzaniga), Maggiore Hospital, Crema, Italy; and Genetic Institute (Dr Danesino), University of Pavia, Pavia, Italy.

Correspondence to: SebastiaanVelthuis, MD, Department of Cardiology, St. Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands; e-mail: S.Velthuis@antoniusziekenhuis.nl


Funding/Support: The authors have reported to CHEST that no funding was received for this study.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(2):542-548. doi:10.1378/chest.12-1599
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Background:  A pulmonary right-to-left shunt (RLS) carries the risk of cerebral paradoxical embolization and severe neurologic complications. Recognizing patients at risk is important to facilitate appropriate management strategies, but a direct relation between pulmonary shunt size and risk of complications remains controversial. This study evaluated the potential relation between pulmonary shunt grade on transthoracic contrast echocardiography (TTCE) and prevalence of cerebral manifestations in patients screened for hereditary hemorrhagic telangiectasia (HHT).

Methods:  We conducted a two-center, cross-sectional study of all consecutive patients screened for HHT between 2004 and 2011. Pulmonary shunt grading on TTCE (grade 0, no microbubbles; grade 1, < 30 microbubbles; grade 2, 30-100 microbubbles; grade 3, > 100 microbubbles) was performed according to contrast opacification of the left ventricle. Cerebral complications were defined as ischemic stroke, transient ischemic attack, or brain abscess diagnosed by a neurologist and confirmed by appropriate imaging techniques.

Results:  A pulmonary RLS was present in 530 out of 1,038 patients (51.1%; mean age, 44.3 ± 15.6 years; 58.6% women). The presence of a cerebral manifestation (n = 51) differed significantly among pulmonary shunt grades on TTCE: 1.4%, 0.4%, 6.5%, and 20.9% for grades 0, 1, 2 and 3, respectively. A pulmonary shunt grade 1 was not associated with an increased prevalence of cerebral manifestations (OR, 0.44; 95% CI, 0.05-4.13; P = .47), whereas pulmonary shunt grade 2 (OR, 4.78; 95% CI, 1.14-20.0; P = .03) and grade 3 (OR, 10.4; 95% CI, 2.4-45.3; P = .002) were both independent predictors for the prevalence of a cerebral ischemic event or brain abscess.

Conclusions:  The pulmonary RLS grade on TTCE is strongly associated with the prevalence of cerebral complications in patients screened for HHT.

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