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Biomedical subjects

F Parent

Publications and source records attributed to F Parent.

At least 37 records · Page 2Linked to original sources

Thrombotic risk factors in pulmonary hypertension.

Thrombotic lesions are consistently observed in chronic thromboembolic pulmonary hypertension (CTEPH) and frequently found in primary pulmonary hypertension (PPH). It remains unknown, however, whether thrombosis is related to defects of the antithrombotic pathway or to previous vascular injury. This study therefore analysed the frequency of both hereditary and acquired thrombotic risk factors in CTEPH and PPH. One hundred and forty-seven consecutive patients with CTEPH investigated in the author's institution were compared to 99 consecutive patients with PPH. In 116 CTEPH patients and 83 PPH patients, phospholipid-dependent antibodies (antiphospholipid antibodies and lupus anticoagulant) were analysed by both immunological and clotting assays. In patients enrolled since 1994 (46 CTEPH and 64 PPH), hereditary thrombotic risk factors were also determined. Antithrombin, protein C and protein S activities were measured by functional assays. Mutations of factor V and factor II were identified by polymerase chain reaction. The prevalence of hereditary thrombotic risk factors was not increased in patients with either PPH or CTEPH. In contrast, a high frequency of phospholipid-dependent antibodies was observed in PPH (10%) and more notably in CTEPH (20%). Moreover, in PPH, antibodies were present only in low titre whereas in CTEPH, half of the patients with antiphospholipid antibodies had high titres. In addition, in CTEPH all but one of the patients with lupus anticoagulant also had antiphospholipid antibodies. The most striking finding of this study was the high prevalence of phospholipid-dependent antibodies but their clinical relevance appears to be different in primary pulmonary hypertension and chronic thromboembolic pulmonary hypertension. In primary pulmonary hypertension, these antibodies in low titre probably reflect endothelial dysfunction. In contrast, in chronic thromboembolic pulmonary hypertension the presence of antibodies in high titre associated with lupus anticoagulant, underlines the role of thrombosis in the pathogenesis of this condition.

Antibodies, Antiphospholipid↗

Assessment of hospital morbidity, mortality, and cost-effectiveness of a nutritional program for children under 5 years of age in Pala, Chad.

This paper analyses the effectiveness of case management at referral level for malnourished sick children in a rural district health system in the south of Chad (Pala district). The methodology followed was based on a cohort study of malnourished children as well as a cost-effective analysis of alternative options for nutritional rehabilitation strategies. Results show that effective case management at hospital level is possible with few resources as long as the nutritional rehabilitation programme is implemented on an integrated basis including early diagnosis of malnutrition for all children admitted to hospital and not to wait for a normal weight-for-height but discharge when the hospital is no longer the best place to avoid further mortality.

Case Management↗

Severe pulmonary hypertension in histiocytosis X.

Diminished exercise capacity in advanced pulmonary histiocytosis X does not appear to be related to ventilatory limitation but may be related to pulmonary vascular dysfunction. Pulmonary hemodynamics and respiratory function were studied in 21 consecutive patients with advanced pulmonary histiocytosis X, and compared with parameters of patients with other severe chronic lung diseases (29 patients with chronic obstructive pulmonary disease and 14 patients with idiopathic pulmonary fibrosis). All patients with pulmonary histiocytosis X displayed severe pulmonary hypertension: mean pulmonary arterial pressure, 59 +/- 4 mm Hg; cardiac index, 2.6 +/- 0.8 L/min/m(2); and total vascular pulmonary resistance, 25 +/- 3 IU/m(2) (p < 0.05, as compared with patients with other chronic lung diseases). Pa(O(2)) was similar in the three groups, whereas FEV(1) was lower in patients with other chronic lung diseases (p < 0.05). In contrast to other chronic lung diseases, the degree of pulmonary hypertension was not related to variables of pulmonary function in pulmonary histiocytosis X. Histopathology was available for 12 patients with pulmonary histiocytosis X and revealed proliferative vasculopathy involving muscular arteries and veins, with prominent venular involvement. Two consecutive lung samples (taken before and after the occurrence of pulmonary hypertension) were available for six patients with pulmonary histiocytosis X, and showed that pulmonary vasculopathy worsened, whereas parenchymal and bronchiolar lesions remained relatively unchanged. These results indicate that pulmonary hypertension in pulmonary histiocytosis X might be related to an intrinsic pulmonary vascular disease, in which the pulmonary circulation is involved independent of small airway and lung parenchyma injury.

Adult↗

Short-term and long-term epoprostenol (prostacyclin) therapy in pulmonary hypertension secondary to connective tissue diseases: results of a pilot study.

Continuous intravenous epoprostenol improves exercise capacity, haemodynamics, and survival in severe primary pulmonary hypertension. Pulmonary hypertension can also be life-threatening in patients with connective tissue diseases. In a prospective open monocentre uncontrolled study, the effects of epoprostenol were evaluated in patients with severe pulmonary hypertension secondary to connective tissue diseases who were unresponsive to oral vasodilators (including calcium channel blockers) and continued to be in the New York Heart Association (NYHA) functional class III or IV despite conventional medical therapy. Seventeen patients received epoprostenol administered by a portable infusion pump associated with conventional therapy (oral anticoagulants, diuretics, supplemental oxygen). During the first six weeks of therapy, two (12%) patients died, of pulmonary oedema (n = 1) and severe sepsis (n = 1). In the fifteen remaining subjects, clinical and haemodynamic parameters improved significantly at six weeks. These patients were subsequently monitored for 80+/-48 (range 14-154) weeks after initiation of epoprostenol. Five (33%) patients died, of right heart failure (n = 2), severe sepsis (n = 2) or syncope (n = 1) and two patients were successfully transplanted 24 and 52 weeks after initiation of epoprostenol. Seven of the remaining eight patients had a persistent clinical improvement. Short-term epoprostenol therapy is effective in some patients with connective tissue diseases as demonstrated by better clinical status and haemodynamics at six weeks. However, this study reports several cases of early and late major complications including severe sepsis and pulmonary oedema. Additional information is needed to evaluate the benefit: risk ratio of long-term epoprostenol therapy in pulmonary hypertension secondary to connective tissue diseases.

Adult↗

High prevalence of detectable deep venous thrombosis in patients with acute pulmonary embolism.

STUDY OBJECTIVES: Because specific studies are unavailable, the exact prevalence of detectable "residual" deep venous thrombosis (DVT) in patients with acute pulmonary embolism (PE) is unknown. DESIGN: Review of clinical records and radiologic documents of consecutive patients. SETTING: Pulmonary diseases and radiology departments at a university hospital. PATIENTS: All patients hospitalized in the Department of Pulmonary Diseases with a diagnosis of acute PE during a 5-year period (1984 to 1988). During this period, the diagnosis of PE was based exclusively on pulmonary angiography, and bilateral lower limb venography was routine in patients with proven acute PE. MEASUREMENTS AND RESULTS: Among 228 consecutive patients with angiography-proven PE, 213 underwent bilateral lower limb venography within 48 h of the diagnosis. Venography demonstrated DVT in 174 patients (81.7%; 95% confidence interval, 76.5 to 86.9%), including 128 patients (60%) with proximal DVT. Signs or symptoms of DVT were present in only 72 patients (42%) with DVT. The prevalence of detectable DVT was significantly lower in patients with recent pelvic surgery or delivery (6 of 12, 50%) than in the other patients, whatever their individual risk factors (p < 0.05). The mean pulmonary vascular obstruction was significantly lower in patients with normal venography than in patients with detectable DVT (37.6 +/- 20.9% vs 48.4 +/- 21.7%; p = 0.007). CONCLUSIONS: Lower limb venography demonstrates a high prevalence (82%) of residual DVT in patients with angiography-proven PE. These data should be taken into account in the diagnostic and therapeutic management of patients with suspected or proven PE.

Adolescent↗

[Pulmonary embolism and pregnancy].

Pulmonary emboli are frequent during pregnancy and the post partum period and represent one of the major causes of maternal mortality during this period. It is estimated that there is an increased risk of thromboembolic venous disease during pregnancy at 1:1000 to 1:2000 pregnancies. This is explained by changes in the physiology and biology of coagulation factors and fibrinolysis. The risk is increased in those aged more than 35, the presence of a past history of thromboembolic venous disease; hereditary or acquired thrombophilia or delivery by caesarean section. The diagnosis should be confirmed with the same rigor as outside pregnancy. No diagnostic examination is contraindicated in those pregnant but the diagnostic strategy should be in favor of non-invasive examinations with ultrasonic venography of the lower limbs and/or pulmonary scintigraphy being the investigations of choice. The treatment of pulmonary emboli during pregnancy rests on heparin, coumarin being strictly contraindicated due to the risk of fetal malformation. Low molecular weight heparin is not currently used during pregnancy but initial data in the literature suggests that they may be used during pregnancy without increased risk compared to non-fractionated heparin. The indications for anticoagulant prophylaxis remains poorly characterized.

Adult↗

A clinical trial of vena caval filters in the prevention of pulmonary embolism in patients with proximal deep-vein thrombosis. Prévention du Risque d'Embolie Pulmonaire par Interruption Cave Study Group.

BACKGROUND: The efficacy and safety of vena caval filters in the prevention of pulmonary embolism in patients with proximal deep-vein thrombosis is still a matter of debate. METHODS: Using a two-by-two factorial design, we randomly assigned 400 patients with proximal deep-vein thrombosis who were at risk for pulmonary embolism to receive a vena caval filter (200 patients) or no filter (200 patients), and to receive low-molecular-weight heparin (enoxaparin, 195 patients) or unfractionated heparin (205 patients). The rates of recurrent venous thromboembolism, death, and major bleeding were analyzed at day 12 and at two years. RESULTS: At day 12, two patients assigned to receive filters (1.1 percent), as compared with nine patients assigned to receive no filters (4.8 percent), had had symptomatic or asymptomatic pulmonary embolism (odds ratio, 0.22; 95 percent confidence interval, 0.05 to 0.90). At two years, 37 patients assigned to the filter group (20.8 percent), as compared with 21 patients assigned to the no-filter group (11.6 percent), had had recurrent deep-vein thrombosis (odds ratio, 1.87; 95 percent confidence interval, 1.10 to 3.20). There were no significant differences in mortality or the other outcomes. At day 12, three patients assigned to low-molecular-weight heparin (1.6 percent), as compared with eight patients assigned to unfractionated heparin (4.2 percent), had had symptomatic or asymptomatic pulmonary embolism (odds ratio, 0.38; 95 percent confidence interval, 0.10 to 1.38). CONCLUSIONS: In high-risk patients with proximal deep-vein thrombosis, the initial beneficial effect of vena caval filters for the prevention of pulmonary embolism was counterbalanced by an excess of recurrent deep-vein thrombosis, without any difference in mortality. Our data also confirmed that low-molecular-weight heparin was as effective and safe as unfractionated heparin for the prevention of pulmonary embolism.

Aged↗

Three-dimensional measurement of wedged scoliotic vertebrae and intervertebral disks.

Idiopathic scoliosis involves complex spinal intrinsic deformations such as the wedging of vertebral bodies (VB) and intervertebral disks (ID), and it is obvious that the clinical evaluation obtained by the spinal projections on the two-dimensional (2D) radiographic planes do not give a full and accurate interpretation of scoliotic deformities. This paper presents a method that allows reconstruction in 3D of the vertebral body endplates and measurement of the 3D wedging angles. This approach was also used to verify whether 2D radiographic measurements could lead to a biased evaluation of scoliotic spine wedging. The 3D reconstruction of VB contours was done using calibrated biplanar X-rays and an iterative projection computer procedure that fits 3D oriented ellipses of adequate diameters onto the 3D endplate contours. "3D wedging angles" of the VB and ID (representing the maximum angle between adjacent vertebrae) as well as their angular locations with respect to the vertebral frontal planes were computed by finding the positions of the shortest and longest distances between consecutive endplates along their contour. This method was extensively validated using several approaches: (1) by comparing the 3D reconstructed endplates of a cadaveric functional unit (T8-T9) with precise 3D measurements obtained using a coordinate measuring machine for 11 different combinations of vertebral angular positions; (2) by a sensitivity study on 400 different vertebral segments mathematically generated, with errors randomly introduced on the digitized points (standard deviations of 0.5, 1, 2, and 3 mm); (3) by comparing the clinical wedging measurements (on postero-anterior and lateral radiographs) at the thoracic apical level of 34 scoliotic patients (15 degrees < Cobb < 45 degrees) to the computed values. Mean errors for the 11 vertebral positions were 0.5 +/- 0.4 mm for VB thickness, less than 2.2 degrees for endplate orientation, and about 11 degrees (3 mm) for the location of the maximum 3D wedging angle along the endplate contour. The errors below 2 mm (introduced on the digitized points) slightly affected the 3D wedging angle (< 2 degrees) and its location (< 4 degrees) for the ID. As for the clinical evaluation, average angular errors were less than 0.4 degrees in the radiographic frontal and lateral planes. The mean 3D wedged angles were about 4.9 degrees +/- 1.9 degrees for the VB and 6.0 degrees +/- 1.7 degrees for the ID. Linear relations were found between the 2D and the 3D angles, but the 3D angles were located on diagonal planes statistically different than the radiographic ones (between 100 degrees and 221 degrees). There was no statistical relation between the 2D radiographic angles and the locations of the 3D intervertebral wedging angles. These results clearly indicate that VB and ID endplates are wedged in 3D, and that measurements on plain radiographs allow incomplete evaluation of spinal wedging. Clinicians should be aware of these limitations while using wedging measurements from plain radiographs for diagnosis and/or research on scoliotic deformities.

Algorithms↗

[Pulmonary thromboendarterectomy with video-angioscopy and circulatory arrest: an alternative to cardiopulmonary transplantation and post-embolism pulmonary artery hypertension].

The best predictor of poor or suboptimum outcome from pulmonary thromboendarterectomy (PTE) is insufficient relief of obstruction, especially in the lower lobes. The aim of this study is to emphasize that the use of video-assisted angioscopy may increase the quality of PTE and thus improve outcome. PTE included a median sternotomy, intrapericardial dissection limited to the superior vena cava, institution of cardiopulmonary bypass, deep hypothermia and sequential circulatory arrest periods. PTE was always bilateral and performed through two separate arteriotomies of both main intrapericardial pulmonary arteries. A rigid 5 mm angioscope connected to a video camera was introduced through the arteriotomy into the lumen to increase the visibility and perform the video-assisted endarterectomies of all obstructed segmental branches, including normally inaccessible anterior segmental branches. Between January 1996 and December 1997, 48 patients with severe postembolic pulmonary hypertension had PTE. Patients were in New York Heart Association (NYHA) class II (n = 2), III (n = 28) or IV (n = 18) with the following hemodynamics: mean pulmonary arterial pressure (PAP) 53 +/- 13 mmHg, cardiac index 2.16 +/- 0.5 L/min/m2, pulmonary vascular resistances (PVR): 1,152 +/- 414 dyne.s-1.cm-5. Six patients died from alveolar hemorrhage (n = 1), high residual pulmonary pressure and rethrombosis (n = 4) and hypoxic cardiac arrest (n = 1). The functional outcome in surviving patients was as follows: (NYHA) class I (n = 24), II (n = 16) or III (n = 2) with improved hemodynamics: mean pulmonary arterial pressure: 30 +/- 9 mmHg, cardiac index: 2.78 +/- 0.5 L/min/m2, pulmonary vascular resistances (PVR): 484 +/- 159 dynes.s-1.cm-5. Video-assisted angioscopy allows much improved quality and degree of pulmonary endarterectomy. This expands the indications to include patients with previously inaccessible distal disease and candidates for heart-lung transplantation.

Adult↗

The flexible contour backrest: a new design concept for wheelchairs.

Studies have shown that the use of a conventional sling backrest in a wheelchair may have a negative impact on the posture and health of users. To avoid such problems, this backrest is often replaced by a back cushion on a rigid surface. However, the use of the latter deprives many wheelchair users of the advantages provided by the sling backrest (easy folding, lightweight, low cost, unobtrusive, simplicity of design). In an effort to maintain these features while providing adequate posture, a new backrest was designed according to specific criteria. The "flexible contour backrest" has been developed so that it can be adapted to a person's back contour with stays, adjustable straps, and curved back posts. Preliminary comparisons done by some experts with commercially available backrests showed that design criteria were reached at 62.9% by the back cushion on a rigid surface, at 64% by the sling backrest, and at 67.7% by the adjustable-tension backrest. On the other hand, design criteria were reached at 80% by the new flexible contour backrest because its design brings together most of the positive features provided by these other backrests (easy to fold, light weight, unobtrusive, airy) as well as providing adequate posture and better fit to the user's morphology.

Equipment Design↗

Inhaled nitric oxide as a screening agent for safely identifying responders to oral calcium-channel blockers in primary pulmonary hypertension.

In a subset of patients with primary pulmonary hypertension (PPH), high doses of oral calcium-channel blockers (CCB) produce a sustained clinical and haemodynamic improvement. However, significant side-effects have been reported during acute testing with CCB. Therefore, to identify accurately patients who may benefit from long-term CCB therapy, there is a need for a safe, potent and short-acting vasodilator. The aim of this study was to compare the acute response to inhaled nitric oxide (NO) and oral high doses of CCB in 33 consecutive patients with PPH. A significant acute vasodilator response was defined by a fall in both mean pulmonary artery pressure and total pulmonary resistance by >20%. Ten patients responded acutely to NO, nine of whom responded acutely to CCB, without any complications. The 23 other patients failed to respond to NO and CCB. In these nonresponders, nine serious adverse events were observed with CCB (38%). There was no clinical or baseline haemodynamic feature predicting acute vasodilator response. Long-term oral treatment with CCB was restricted to the nine acute responders and a sustained clinical and haemodynamic improvement was observed in only six patients. In primary pulmonary hypertension, the acute response rate to high doses of calcium-channel blockers is low (27%). Serious adverse reactions to high doses of calcium-channel blockers during acute testing are frequently observed in nonresponders. It is concluded that nitric oxide may be used as a screening agent for safely identifying patients with primary pulmonary hypertension who respond acutely to calcium-channel blockers and may benefit from long-term treatment with these agents.

Administration, Inhalation↗

Epidural corticosteroid injections for sciatica due to herniated nucleus pulposus.

BACKGROUND: Although epidural corticosteroid injections are commonly used for sciatica, their efficacy has not been established. METHODS: In a randomized, double-blind trial, we administered up to three epidural injections of methylprednisolone acetate (80 mg in 8 ml of isotonic saline) or isotonic saline (1 ml) to 158 patients with sciatica due to a herniated nucleus pulposus. All patients had Oswestry disability scores higher than 20 (on a scale of 1 to 100, with scores of 20 or less indicating minimal disability, and higher scores greater disability). RESULTS: At three weeks, the Oswestry score had improved by a mean of -8.0 in the methylprednisolone group and -5.5 in the placebo group (95 percent confidence interval for the difference, -7.1 to 2.2). Differences in improvements between the groups were not significant, except for improvements in the finger-to-floor distance (P=0.006) and sensory deficits (P=0.03), which were greater in the methylprednisolone group. After six weeks, the only significant difference was the improvement in leg pain, which was greater in the methylprednisolone group (P=0.03). After three months, there were no significant differences between the groups. The Oswestry score had improved by a mean of -17.3 in the methylprednisolone group and -15.4 in the placebo group (95 percent confidence interval for the difference, -9.3 to 5.4). At 12 months, the cumulative probability of back surgery was 25.8 percent in the methylprednisolone group and 24.8 percent in the placebo group (P=0.90). CONCLUSIONS: Although epidural injections of methylprednisolone may afford short-term improvement in leg pain and sensory deficits in patients with sciatica due to a herniated nucleus pulposus, this treatment offers no significant functional benefit, nor does it reduce the need for surgery.

Adult↗

Morphometric evaluations of personalised 3D reconstructions and geometric models of the human spine.

In the past, several techniques have been developed to study and analyse the 3D characteristics of the human spine: multi-view radiographic or biplanar 3D reconstructions, CT-scan 3D reconstructions and geometric models. Extensive evaluations of three of these techniques that are routinely used at Sainte-Justine Hospital (Montréal, Canada) are presented. The accuracy of these methods is assessed by comparing them with precise measurements made with a coordinate measuring machine on 17 thoracic and lumbar vertebrae (T1-L5) extracted from a normal cadaveric spine specimen. Multi-view radiographic 3D reconstructions are evaluated for different combinations of X-ray views: lateral (LAT), postero-anterior with normal incidence (PA0 degree) and postero-anterior with 20 degrees angled down incidence (PA20 degrees). The following accuracies are found for these reconstructions obtained from different radiographic setups: 2.1 +/- 1.5 mm for the combination with PA0 degree-LAT views, and 5.6 +/- 4.5 mm for the PA0 degree-PA20 degrees stereopair. Higher errors are found in the postero-anterior direction, especially for the PA0 degree-PA20 degrees view combination. Pedicles are found to be the most precise landmarks. Accuracy for CT-scan 3D reconstructions is about 1.1 +/- 0.8 mm. As for a geometric model built using a multiview radiographic reconstruction based on six landmarks per vertebra, accuracies of about 2.6 +/- 2.4 mm for landmarks and 2.3 +/- 2.0 mm for morphometric parameters are found. The geometric model and 3D reconstruction techniques give accurate information, at low X-ray dose. The accuracy assessment of the techniques used to study the 3D characteristics of the human spine is important, because it allows better and more efficient quantitative evaluations of spinal dysfunctions and their treatments, as well as biomechanical modeling of the spine.

Evaluation Studies as Topic↗

Prognostic value of pulmonary hypertension in patients with chronic interstitial lung disease referred for lung or heart-lung transplantation.

BACKGROUND: The purpose of this study was to identify precise and reliable prognostic parameters in patients affected by serious chronic interstitial lung disease, who were undergoing screening for lung or heart-lung transplantation. METHODS: Hemodynamic and respiratory function parameters of 67 patients (43 with idiopathic pulmonary fibrosis, 18 with histiocytosis X, and 6 with lymphangioleiomyomatosis) undergoing clinical screening for lung transplantation. RESULTS: Statistical analysis showed that hemodynamic and respiratory function parameters in patients affected by histiocytosis X and idiopathic pulmonary fibrosis were not related to survival time. Moreover, the degree of pulmonary hypertension showed no correlation between respiratory function parameters in all the groups of diseases examined. Patients affected with histiocytosis X, even with higher degrees of pulmonary hypertension, had a better survival rate (p < 0.0005) compared with patients with idiopathic pulmonary fibrosis. CONCLUSIONS: Hemodynamic and respiratory parameters obtained during the clinical screening for lung transplantation do not predict survival and cannot be used as prognostic indicators.

Adult↗

Lung perfusion scans and hemodynamics in acute and chronic pulmonary embolism.

UNLABELLED: To assess the relationship between pulmonary vascular obstruction and hemodynamic status in acute pulmonary embolism (APE) and in chronic thromboembolic pulmonary hypertension (CTEPH), perfusion lung scan and hemodynamic measurements were obtained in 31 consecutive patients with APE and in 45 with CTEPH. METHODS: Lung scans were scored independently by two experience observers who determined the percentage of vascular obstruction (PVOs). Mean pulmonary artery pressure (PAP) and total pulmonary resistance (TPR) were obtained during right heart catheterization. In patients with APE, measurements were recorded within a 1-hr interval before and 12 hours after thrombolysis. This yielded 62 paired PVOs values with concomitant PAP and TPR measurements. In patients with CTEPH, data were recorded within a 3-day interval. RESULTS: Mean PVOs (%) values were similar in APE and CTEPH patients (59 +/- 13 vs. 58 +/- 15), whereas PAP and TPR were significantly higher in CTEPH patients (51 +/- 17 mmHg and 23 +/- 11 U/m2, respectively) than in APE patients (23 +/- 8 mmHg and 9 +/- 5 U/m2, respectively, p < 0.001). In APE patients, significant hyperbolic correlations were found linking PVOs with PAP and TPR (r = 0.75, p < 0.01 for PAP; r = 0.71, p < 0.01 for TPR). In CTEPH, there were no significant correlations between PVOs and PAP or TPR. For the same level of PVOs, patients with CTEPH had higher PAP and TPR value than patients with APE. CONCLUSION: In APE without prior cardiopulmonary disease, increases in PAP and TPR are correlated in a nonlinear fashion with the degree of pulmonary vascular obstruction as assessed by lung scanning. In CTEPH patients, the higher PAP and TPR values as compared to APE patients with comparable degrees of PVOs are consistent with previous reports that pulmonary hypertension in CTEPH is due not only to the obstruction of proximal pulmonary arteries but also to remodeling of small distal arteries in nonoccluded areas.

Acute Disease↗

Clinical significance of the pulmonary vasodilator response during short-term infusion of prostacyclin in primary pulmonary hypertension.

BACKGROUND: The short-term vasodilator response to prostacyclin (PGI2) in patients with primary pulmonary hypertension (PPH) is not only unpredictable but also extremely variable in magnitude. In this retrospective study, we attempted to evaluate in a nonselected population of patients with PPH the degree of vasodilatation achieved during short-term infusion of PGI2 and to investigate whether patients with PPH differed in terms of baseline characteristics and prognoses, according to the level of vasodilatation achieved during initial testing with PGI2. METHODS AND RESULTS: Between 1984 and 1992, 91 consecutive patients with PPH underwent catheterization of the right side of the heart with a short-term vasodilator trial with PGI2 (5 to 10 ng.kg-1.min-1). According to the level of vasodilatation achieved during PGI2 infusion, patients were divided into three groups: nonresponding (NR, n = 40), moderately responding (MR, n = 42), and highly responding (HR, n = 9) patients. All three groups were defined by a decrease in total pulmonary resistance index (TPRi) of < 20%, between 20% and 50%, and > 50%, respectively, relative to control values. Prolonged oral vasodilator therapy was subsequently started only in MR and HR patients. All patients had long-term oral anticoagulant therapy. The survival rate at 2 years (transplant recipients excluded) was significantly higher in HR patients compared with NR and MR patients (62% versus 38% and 47% survivors, respectively; P < .05). Comparisons between groups showed no significant differences in baseline hemodynamics or clinical characteristics except for a longer time between onset of symptoms and diagnosis (ie, first catheterization) of PPH in HR patients than in NR and MR patients (71 +/- 61 versus 35 +/- 34 and 21 +/- 21 months, respectively; P < .05). CONCLUSIONS: In this study, patients with PPH exhibiting a decrease in TPRi > 50% during short-term PGI2 challenge at the time of diagnosis had longer disease evolutions and better prognoses than patients with a lower vasodilator response.

Adolescent↗