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(Circulation. 2000;102:2611.)
© 2000 American Heart Association, Inc.

Clinical Investigation and Reports

Severely Impaired Baroreflex-Buffering in Patients With Monogenic Hypertension and Neurovascular Contact

Jens Jordan, MD; Hakan R. Toka, MD; Karsten Heusser, MD; Okan Toka, MD; John R. Shannon, MD; Jens Tank, MD; Andre Diedrich, MD; Christine Stabroth, MD; Mandy Stoffels, RN; Ramin Naraghi, MD; Wolfgang Oelkers, MD; Herbert Schuster, MD; Hans P. Schobel, MD; Hermann Haller, MD; Friedrich C. Luft, MD

From the Clinical Research Center, Franz Volhard Clinic, Medical Faculty of the Charité, Humboldt-University, and the Department of Endocrinology, Klinikum Benjamin Franklin, Free University, Berlin, and the Department of Internal Medicine and Nephrology and Department of Neurosurgery, Friedrich-Alexander University, Erlangen, Germany; and the Autonomic Dysfunction Service, Vanderbilt University, Nashville, Tenn.

Correspondence to Jens Jordan, MD, Clinical Research Center, Franz-Volhard-Clinic, Humboldt University, Wiltbergstraße 50, 13125 Berlin, Germany. E-mail jordan{at}fvk-berlin.de

Background—We identified a family with a monogenic syndrome of hypertension, brachydactyly, and neurovascular contact of the brain stem. Neurovascular contact of the ventrolateral medulla may lead to arterial hypertension by interfering with baroreflex function.

Methods and Results—In 5 patients with monogenic hypertension (18 to 34 years old), we conducted detailed autonomic function tests. Blood pressure during complete ganglionic blockade was 134±4.9/82±4.1 mm Hg and 90±6/49±2.4 mm Hg in patients and in control subjects, respectively. During ganglionic blockade, plasma vasopressin concentration increased 24-fold in control subjects and <2-fold in patients. In patients, cold pressor testing, hand-grip testing, and upright posture all increased blood pressure excessively. In contrast, muscle sympathetic nerve activity was not increased at rest or during cold pressor testing. The phenylephrine dose that increased systolic blood pressure 12.5 mm Hg was 8.0±2.0 µg in patients and 135±35 µg in control subjects before ganglionic blockade and 5.4±0.4 µg in patients and 13±4.8 µg in control subjects during ganglionic blockade.

Conclusions—In patients with monogenic hypertension and neurovascular contact, basal blood pressure was increased even during sympathetic and parasympathetic nerve traffic interruption. However, sympathetic stimuli caused an excessive increase in blood pressure. This excessive response cannot be explained by increased sympathetic nerve traffic or increased vascular sensitivity. Instead, we suggest that baroreflex buffering and baroreflex-mediated vasopressin release are severely impaired.

Key Words: baroreceptors • genetics • hypertension • nervous system, autonomic • receptors

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