Echocardiographic Features of Primary Pulmonary Hypertension*,**,*

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Abstract

Primary pulmonary hypertension (PPH) is essentially a diagnosis of exclusion and usually is made late because of the nonspecific nature of the early signs and symptoms. Echocardiography is a key screening test in the diagnostic algorithm of patients with suspected PPH. The purpose of this study was to define the echocardiographic Doppler features in patients with PPH at the time of diagnosis. From 1992 to 1997, 51 patients were diagnosed with PPH at our institution. All underwent a standardized transthoracic echocardiographic examination, including a contrast study and transthoracic echocardiographic examination if indicated. Pulmonary artery systolic pressure was calculated from the tricuspid regurgitation jet. The majority of patients had pulmonary artery systolic pressure greater than 60 mm Hg (96%) associated with systolic flattening of the interventricular septum (90%), enlarged right atrium (92%) and ventricle (98%), and reduced right ventricular systolic function (76%). There was an increase in the interventricular septal thickness (>1.2 cm) in 21 (43%) of 49 patients, accompanied by a septal/posterior wall ratio greater than 1.3 in 11 (22%) of 49. Although a reduction in both left ventricular systolic and diastolic volumes was noted, global left ventricular systolic function was preserved in all patients. Mitral E/A ratio was less than 0.7 in 7 (22%) patients studied. Color Doppler revealed moderate to severe tricuspid regurgitation and pulmonic insufficiency in 41 (80%) of 51 and 16 (31%) of 51 of cases, respectively. Pericardial effusion (7 small and 1 moderate) and patent foramen ovale (n = 12) were also frequently detected. At the time of initial diagnosis, PPH is associated with secondary cardiac abnormalities in the majority of patients. (J Am Soc Echocardiogr 1999;12:655-62.)

Section snippets

Study Population

We analyzed 51 consecutive patients referred to the University of Michigan for evaluation of known or suspected pulmonary hypertension or who were initially diagnosed to have PPH at the University of Michigan after referral for another presumed diagnosis (Table 1).All patients underwent a detailed history, physical examination, electrocardiography, chest radiography, and comprehensive transthoracic echocardiography (TTE), including Doppler studies. The diagnosis of PPH was established by use of

Patient Demographics

Demographic data are presented in Table 1. The female-to-male ratio was 7.5:1. Of the total patients, 78% were in New York Heart Association functional class III or IV. The mean time from the onset of the initial symptoms to diagnosis was 20.6 ± 20.2 months (median 12), with a range from 1 to 84 months, confirming that the diagnosis of PPH is usually made late in the course of disease.

TTE was available in all 51 subjects. Additionally, contrast TTE was available in 24 and TEE in 5. TEE

DISCUSSION

PPH is a rare disease of the pulmonary vasculature that primarily affects otherwise healthy young adults. It usually follows a rapidly fatal course and has a median survival of only 2.8 years after diagnosis. Five-year survival rate is 34%.23 Because of the nonspecific symptoms and the subtlety of the signs of less advanced disease, the mean length of time from the onset of symptoms to definitive diagnosis averages 2 years.1 A high level of clinical suspicion is important for prompt diagnosis

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    *

    From the Division of Cardiology, Department of Internal Medicine, University of Michigan, Ann Arbor, Mich.

    **

    Reprint requests: William F. Armstrong, MD, University of Michigan Hospital, Division of Cardiology, Women’s L3119, 1500 E Medical Center Dr, Ann Arbor, MI 48109-0273. E-mail: [email protected].

    *

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