Pulmonary Hypertension in Hereditary Hemorrhagic Telangiectasia

Autor: Garvan C. Kane, Karen L. Swanson, Michael J. Krowka, Robert P. Frantz, Melissa A. Lyle, Michael D. McGoon, Eric R. Fenstad
Rok vydání: 2016
Předmět:
Male
Pulmonary and Respiratory Medicine
Cardiac Catheterization
medicine.medical_specialty
Hypertension
Pulmonary
Hemodynamics
030204 cardiovascular system & hematology
Critical Care and Intensive Care Medicine
03 medical and health sciences
Folic Acid
0302 clinical medicine
Internal medicine
medicine.artery
medicine
Humans
Pulmonary Wedge Pressure
Telangiectasia
Pulmonary wedge pressure
Vinca Alkaloids
Retrospective Studies
business.industry
Central venous pressure
Middle Aged
medicine.disease
Pulmonary hypertension
medicine.anatomical_structure
030228 respiratory system
Heart catheterization
Pulmonary artery
Ventricular Function
Right
Vascular resistance
Cardiology
Female
Telangiectasia
Hereditary Hemorrhagic
Vascular Resistance
medicine.symptom
Cardiology and Cardiovascular Medicine
business
Follow-Up Studies
Zdroj: Chest. 149:362-371
ISSN: 0012-3692
Popis: Background A subset of patients with hereditary hemorrhagic telangiectasia (HHT) develops pulmonary hypertension (PH) by mechanisms including pulmonary arterial hypertension, high flow, and elevated pulmonary arterial wedge pressure (PAWP). We aimed to describe echocardiographic and hemodynamic characteristics of patients with coexisting HHT and PH. Methods We conducted a single-center cohort study of patients with confirmed HHT who underwent right-sided heart catheterization (RHC) and transthoracic two-dimensional echocardiography for suspected PH between June 1, 2003 and September 1, 2013 at Mayo Clinic Rochester, Minnesota. Results Of 38 patients with confirmed HHT who underwent RHC and echocardiography, 28 (74%) had a mean pulmonary artery pressure (MPAP) ≥ 25 mm Hg. Of those 28, 12 (43%) had pulmonary arterial hypertension. Two patients had normal PAWP and pulmonary vascular resistance (PVR), with PH secondary to either an atrial septal defect or high cardiac flow. Fourteen patients (50%) had elevated PAWP (≥ 15 mm Hg), nine with evidence of high flow. RHC in all 28 patients demonstrated a MPAP of 41 ± 11 mm Hg, PAWP of 17 ± 10 mm Hg, and PVR of 4.5 ± 4.2 Wood units. Echocardiography demonstrated moderate/severe right ventricular dysfunction in nine patients (32%). The presence of PH trended toward worse survival ( P = .06). Conclusions PH in patients with HHT occurs by different mechanisms, and there is a trend toward worse survival in patients who develop PH despite the mechanism. The equal predilection toward all subtypes of PH illustrates the necessity of RHC to clarify the hemodynamics.
Databáze: OpenAIRE
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