Partitioning of pulmonary vascular resistance in primary pulmonary hypertension

OBJECTIVES: This study sought to determine the site of increased pulmonary vascular resistance (PVR) in primary pulmonary hypertension by standard bedside hemodynamic evaluation. BACKGROUND: The measurement of pulmonary vascular pressures at several levels of flow (Q) allows the discrimination between active and passive, flow-dependent changes in mean pulmonary artery pressure (Ppa), and may detect the presence of an increased pulmonary vascular closing pressure. The determination of a capillary pressure (Pc') from the analysis of a Ppa decay curve after balloon occlusion allows the partitioning of PVR in an arterial and a (capillary + venous) segment. These approaches have not been reported in primary pulmonary hypertension. METHODS: Ppa and Pc' were measured at baseline and after an increase in Q induced either by exercise or by an infusion of dobutamine, at a dosage up to 8 microg/kg body weight per min, in 11 patients with primary pulmonary hypertension. Reversibility of pulmonary hypertension was assessed by the inhalation of 20 ppm nitric oxide (NO), and, in 6 patients, by an infusion of prostacyclin. RESULTS: At baseline, Ppa was 52+/-3 mm Hg (mean value+/-SE), Q 2.2+/-0.2 liters/min per m2, and Pc' 29+/-3 mm Hg. Dobutamine did not affect Pc' and allowed the calculation of an averaged extrapolated pressure intercept of Ppa/Q plots of 34 mm Hg. Inhaled NO had no effect. Prostacyclin decreased Pc' and PVR. Exercise increased Pc' to 40+/-3 mm Hg but did not affect PVR. CONCLUSIONS:ns. These findings are compatible with a major increase of resistance and reactivity at the periphery of the pulmonary arterial tree.     

Characteristics of pulmonary artery pressure waveform for differential diagnosis of chronic pulmonary thromboembolism and primary pulmonary hypertension

OBJECTIVES: The accurate diagnosis of chronic pulmonary thromboembolism (CPTE) is a prerequisite for life-saving surgical interventions. To help in the differential diagnosis of CPTE and primary pulmonary hypertension (PPH), we characterized the configuration of the pulmonary artery pressure waveform. BACKGROUND: Because CPTE predominantly involves the proximal arteries, whereas PPH involves the peripheral arteries, we hypothesized that patients with CPTE would have stiff or high resistance proximal arteries, whereas those affected by PPH would have high resistance peripheral arteries. These differences in the primary lesions would make arterial pulsatility relative to mean pressure larger in CPTE than in PPH. METHODS: In 34 patients with either CPTE (n = 22) or PPH (n = 12) whose pulmonary systolic pressure was > or = 50 mm Hg, we measured pulmonary artery pressure using a fluid-filled system that included a balloon-tipped flow-directed catheter. RESULTS: To quantify the magnitude of pulsatility relative to mean pressure, we normalized pulse pressure by mean pressure, hereinafter referred to as fractional pulse pressure (PPf). PPf was markedly higher in CPTE than in PPH (mean [+/-SD] 1.41 +/- 0.20 and 0.80 +/- 0.18, respectively, p < 0.001) and was diagnostic in separating the two groups without overlap. Similarly, the coefficient of variation of pulmonary artery pressure also separated the two groups without overlap (0.45 +/- 0.06 and 0.25 +/- 0.06, respectively, p < 0.001). Fractional time to half the area under the pressure curve separated the two groups reasonably well (0.35 +/- 0.02 and 0.43 +/- 0.03, respectively, p < 0.001). CONCLUSIONS: The analysis of pulsatility of pulmonary artery pressure is useful in the differential diagnosis of CPTE and PPH.     

A comparative analysis of pulmonary perfusion scans with pulmonary angiograms

Pulmonary angiograms and pulmonary lung perfusion scans on 162 patients with pulmonary embolism were comparatively analyzed. Among the expert angiographic panel members who independently evaluated the studies there was consistent agreement on the diagnosis, size of the emboli, and severity. Consistency of agreement among the expert pulmonary lung perfusion scan panelists was considerably less. These data demonstrate that, in addition to the lack of specificity of the lung perfusion scan for the diagnosis of pulmonary thromboemboli, there is a considerable problem of interpretation in this patient population.     

Repair of congenital pulmonary incompetence by bicuspidization of the pulmonary valve

We report the successful repair of pulmonary incompetence in an adult due to the congenital absence of the posterior leaflet of the pulmonary valve. The repair consisted of bicuspidization of the pulmonary valve, which achieved competence and eliminated the symptoms and echocardiographic manifestations of right ventricular overload while preserving the native valve.     

Anomalous left pulmonary artery without pulmonary artery sling

Although most news surrounding the declining dollar has concentrated on its impact on Canadian shoppers, economists say it is bound to affect the financially strapped health care system too. They point out that many of the good purchased by Canadian hospitals come from the US, and the weak loonie means their price will rise.     

Idiopathic pulmonary hilar fibrosis: an unusual cause of pulmonary hypertension

A 37-year-old man with progressive exertional dyspnea had pulmonary hypertension associated with pulmonary arterial and venous obstruction. An autopsy revealed that the cause of death was idiopathic pulmonary hilar fibrosis, a variant of mediastinal fibrosis. Pulmonary hilar fibrosis can mimic thromboembolic pulmonary hypertension, pulmonary veno-occlusive disease, and pulmonary venous hypertension.     

Sonographic observation of pulmonary infarction and early infarctions by pulmonary embolism

Fifty-eight consecutive patients with clinical symptoms of pulmonary embolism/infarction were examined by ultrasound as the first imaging modality. The diagnosis was confirmed in 35 patients by ventilation-perfusion scintigraphy; 13 underwent pulmonary angiography for verification of clinical diagnosis. Seven patients died, necropsy was performed and the diagnosis of pulmonary embolism was confirmed in six cases; three patients were submitted to transthoracic lung biopsy. Intercostal space and an additional small pleural effusion in 48% of the examined patients served as a sonic window for the 5 MHz sector scanner. In 42 of the 54 cases with a final diagnosis of pulmonary embolism/infarction a total of 69, hypoechoic, lesions with a pleural basis were detected. These were conspicuous, predominantly triangular, of a mean size 4.6 x 3.7 cm (range 9 x 8 to 2 x 1.5). A hyperechoic structure with reverberation artefacts suggestive of air was frequently visible in the centre: a sign of segmental involvement. The ultrasound examination yielded a true positive result in 41 cases. The overall sensitivity was 98% and the specificity 66%. The prevalence of pulmonary embolism was 83% and the diagnostic accuracy 90%. This suggests that chest sonography can be an efficient technique in the detection of pulmonary infarction.     

Non-invasive estimation of pulmonary arterial hypertension in chronic obstructive pulmonary disease

The feasibility and reliability of the combination of several noninvasive methods using a multivariate method of analysis to predict pulmonary artery hypertension (PAH) is evaluated in 20 patients with chronic obstructive pulmonary disease. These methods comprised arterial blood gases (Pao2, Paco2), pulmonary functional parameters (FEV1), echo-Doppler parameters (tricuspid regurgitation jets, acceleration time on pulmonary valve), computed tomography measurements (transhilar distance, hilar thoracic index, and measurement of the descending branch of the right pulmonary artery to the lower lobe). A multiple stepwise regression analysis (including one Doppler parameter, two parameters of arterial blood gases, and one functional parameter) revealed a coefficient of determination (R2) equal to 0.954 for mean pulmonary artery pressure (MPAP) with a standard error of estimate (S.E.E.) of 5.25 mmHg. A stepwise regression analysis including computed tomography and radiographic parameters revealed an R2 equal to 0.970 for PAP with a S.E.E. of 4.26 mmHg. Logistical regression analysis classified correctly 80% of patients with PAH using noninvasive methods such as the diameter of the main pulmonary artery and the diameter of the left pulmonary arterial branch calculated by computed tomography. Not only the presence of PAH but also the level of MPAP can be estimated by the combination of multiple stepwise and logistical regression analyses.     

Marked pulmonary function abnormalities in a case of HIV-associated pulmonary hypertension

Recent reports have suggested an association between primary pulmonary hypertension and human immunodeficiency virus (HIV) infection. This appears to be an accelerated syndrome, associated with a relatively brief duration of symptoms, yet prominent right ventricular failure and severe pulmonary hypertension on presentation. We present a case of a primary pulmonary hypertension in a 35-year-old HIV-seropositive hemophiliac. His accelerated clinical course is consistent with previously reported cases of HIV-related pulmonary hypertension. However, this patient's pulmonary function tests revealed marked hyperinflation, a decreased diffusing capacity, and no airflow obstruction. To our knowledge, this very usual constellation of pulmonary function changes has not been described previously in this syndrome.     

Hyperplasia of pulmonary neuroendocrine cells in a case of childhood pulmonary emphysema

Pulmonary emphysema is very uncommon in children in the first decade of life. The few cases documented in the literature were all due to alpha1-antitrypsin deficiency. We present the case of a 6-year-old white boy with chronic cough and dyspnea on exertion. Lung biopsy showed panacinar type emphysema with patent airways and diffuse hyperplasia of pulmonary neuroendocrine cells revealed after immunostaining for bombesin, a peptide produced by these cells. We speculate that idiopathic diffuse hyperplasia of bombesin-producing pulmonary neuroendocrine cells may contribute to the pathogenesis of unusual COPD in childhood.     

Limits of pulmonary resection

Foreign body aspiration in children may present with a variety of symptoms. We report here an unusual case of laryngeal foreign body in an infant masquerading as laryngeal web in its clinical presentation as well as radiological findings. The diagnosis was only made at endoscopy, thus emphasising the limited sensitivity of radiographic studies in identifying this condition.     

Imaging of pulmonary sarcoidosis

Imaging plays a fundamental role in every step of the management of thoracic sarcoidosis. It contributes to the positive and differential diagnosis and assessment of the prognosis. It is able to distinguish between inflammatory lesions and fibrosis. It allows follow-up of the course of the disease with or without treatment and detection of any complications. Computed tomography provides very useful complementary information at each of these steps.     

Regional chemotherapy via the pulmonary artery for pulmonary metastases

Regional chemotherapy with Adriamycin via the pulmonary artery produces significantly higher tissue levels in the infused canine lobe than systemic administration. Seven patients with soft tissue sarcomas who had received the maximum dose of Adriamycin and had shown metastatic tumor recurrence to the lungs, received small doses of 10 to 20 mg of Adriamycin in the lobar arteries supplying areas with tumor via a Swan-Ganz catheter, temporarily occluding with its inflated balloon the infused artery. One partial objective regression was noted. A total of 56 injections of Adriamycin was given through individual lobar arteries in the seven patients. This preliminary experience indicates the feasibility and relative safety of the use of the pulmonary artery for regional chemotherapy of pulmonary malignant tumors and suggests further cautious exploration of this method.     

Rupture of an abnormal pulmonary artery with a flow-directed pulmonary artery catheter

Most authors believe that biliary pancreatitis rarely progresses to chronic pancreatitis. Here, we present a case of an 18-yr-old white male with a history of gallstone pancreatitis and pancreatic pseudocyst resulting in radiographic and pathologic evidence of pancreatic calcification over a 16-mo period.     

Diagnosis of pulmonary thromboembolism in a cat using echocardiography and pulmonary scintigraphy

A 10-year-old male cat was presented with sudden onset of respiratory difficulties. Clinical examination revealed an acute dyspnoea with cyanosis associated with a left systolic heart murmur. Standard thoracic radiographs excluded pulmonary oedema and showed very few pulmonary changes given the intensity of the respiratory compromise. Echocardiographic examination revealed hypertrophic cardiomyopathy and a thrombus in the right pulmonary artery. Pulmonary scintigraphy confirmed a pulmonary thromboembolism with hypovascularisation of the left cranial lobe and of the ventral segment of the right lobe. Conservative treatment was instituted using an antibiotic (doxycycline), anticoagulants (heparin, coumadine) and a calcium inhibitor (diltiazem). The cat was given absolute rest. The general condition of the animal improved.