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Chronic thromboembolic pulmonary embolism not preceded by clinical thromboembolic event(s) in a 50 year-old male with a history of three-year dyspnea on exertion was detected. Proximal chronic pulmonary embolism, mean pulmonary artery pressure 32 mm Hg, and pulmonary vascular resistance of 444 dyn x s(-1) x cm(-5) were found. We describe a 11-year clinical course of anticoagulated patient with monitoring of functional status and echocardiography and with control of pulmonary haemodynamics at the end of follow-up.

Pulmonary hypertension is characterized by a progressive increase in pulmonary arterial pressure in association with dilatation and hypertrophy of the right ventricle, causing gradual reduction in ejection fraction. The increase in mean pulmonary arterial pressure may be passive, due to increased downstream pressure, hyperkinetic due to increased cardiac output, or due to increased pulmonary vascular resistance resulting from changes in the pulmonary vessels. In an advanced stage of pulmonary hypertension there may be right ventricular dilatation and hypertrophy, tricuspid regurgitation and septal deviation, with consequent effects on cardiac function. Clinical symptoms are not specific. Until recently, the treatment of pulmonary hypertension was limited to anticoagulation, supplementary oxygen and high-dose calcium channel blockers, in association with diuretics and digoxin where indicated. Recently approved treatments are nitric oxide, sildenafil--a phosphodiesterase-5 inhibitor, analogs of prostacyclin, and nonselective and selective endothelin receptor inhibitors. Surgery and anaesthesia pose a significant risk for patients with pulmonary hypertension. Right ventricular failure, persistent postoperative hypoxia and coronary ischaemia are among the potential postoperative complications.

Unlabelled: Thirty patients (24F, 6M, aged 40.5 +/- 15.4 years) with chronic precapillary pulmonary hypertension (primary pulmonary hypertension--PPH)--20 pts, associated with connective tissue disease--6 pts, due to thromboembolism--2 pts and Eisenmenger syndrome--2 pts) were submitted to 5 min inhalation of 10 and 20 ppm of nitric oxide (NO) during right heart catheterization. For the whole group this test resulted in minimal changes in directly assessed hemodynamics. However, individual reactions differed markedly between patients. Assuming three levels of criteria of preserved reactivity (liberal, standard, demanding) 11/30 (37%) patients had fall in PAP and PVR > or = 20% from baseline, 4/30 (13%) decreased PAP and PVR by > or = 20% and 30% respectively and 2 (7%) patients normalized PAP and decreased PVR > or = 50% from baseline, respectively. Interestingly, even liberal criteria were fulfilled positive test was observed exclusively in patients with primary PH. In one patient with connective tissue disease and systemic hypertension acute pulmonary congestion appeared during NO testing, with concomitant increase in systemic arterial pressure and pulmonary wedge pressure. No other complications were noted and no rebound PH after discontinuation of NO was observed in any of the patients., Conclusion: 1. Acute inhaled nitric oxide test appears to be safe in patients with PH and no coexisting left heart dysfunction. 2. It allows for stratification of patients according to the degree of preserved pulmonary vascular reactivity. However, criteria which would permit to select optimal treatment based on the results of NO test require further studies.

A case of 11 year old girl with the anomalous origin of the right coronary artery from pulmonary trunk is presented. Initially the patient was treated conservatively, however, after demonstrating myocardial ischaemia during SPECT, she was selected for surgical treatment.

Diagnosis of acute pulmonary embolism is difficult. The aim of the study was analysis of electrocardiographic (ECG) changes in patients with acute pulmonary embolism and analysis of correlations between electrocardiographic changes and pulmonary angiography and pulmonary artery pressure. ECG in 22 patients aged 47 +/- 13 years old (9 women and 13 men) with confirmed pulmonary embolism and without pre-existing cardiorespiratory diseases were analyzed. Most frequently tachycardia, negative T wave in III, aVF and precordial leads, dextrogyria and dextrogram in ECG were observed. In 20 patients 3 or more criteria were found. Index Milleri--an quantitative method of estimation of pulmonary embolism correlated with pulmonary artery pressure. Conclusion of this study is that ECG is one of the important diagnostic tests in patients with pulmonary embolism: it gives us information about changes in pulmonary arteries.

20 consecutive patients with thromboembolic pulmonary hypertension underwent pulmonary thromboendarterectomy. Mean pulmonary artery pressure decreased from mean 49.9 +/- 9.8 to 25.1 +/- 8.8 mm Hg (p < 0.0001), pulmonary vascular resistance decreased from mean 5.58 +/- 2.58 to 1.62 +/- 0.79 mm Hg/l*min-1 (p < 0.0001) and cardiac output increased from 3.71 +/- 1.18 to 6.92 +/- 1.64 l/min. Sixteen patients had marked clinical improvement. Two patients died in early and 2 patients in late postoperative period due to unrelieved pulmonary hypertension and postoperative complications.

It is not clear whether right ventricle to pulmonary artery coupling is modified by the site of vascular obstruction in patients with chronic severe pulmonary hypertension. We compared invasively (Swan Ganz) and non-invasively (echo/ /Doppler) assessed hemodynamics between two groups of patients with severe chronic thromboembolic pulmonary hypertension (CTEPH)--(n = 6; 52 +/- 24 yrs) and pulmonary arterial hypertension (PAH) (n = 5; 42 +/- 9 yrs) who had similar invasively measured right ventricular systolic pressure (CTEPH: 78 +/- 14 mm Hg; PAH: 83 +/- 17 mm Hg; p = NS), mean pulmonary arterial pressure (CTEPH: 51 +/- 10; PAH 56 +/- 11 mm Hg, p = NS) and pulmonary vascular resistance (CTEPH: 15.6 +/- 4.4 l/min; PAH: 19.2 +/- 6.1; p = ns). Patients with CTEPH have significantly shorter acceleration time corrected to ejection time (RVET): (AcT/RVET % = 24 +/- 5% vs 32 +/- 6% in PAH; p = 0.04) as well as AcT corrected by RR distance was highly significantly shorter (8 +/- 2% vs 12 +/- 2%; p = 0.006). AcT in the CTEPH group was shorter than in the PAH (60 +/- 5 vs 75 +/- 15; p = 0.047). The mid-systolic deceleration was significantly more frequent in the CTEPH group than in the PAH group (88% vs 30%; p = 0.005). If the mid-systolic deceleration was present in patients with PAH, the time to mid-systolic deceleration (t-N) had tendency to be longer in CTEPH group (118 +/- 22 ms vs 150 +/- 28 ms in PAH; p = 0.09). Significant differences appeared after correction t-N to RVET (t-N/RVET % = 46 +/- 9% vs 61 +/- 4%; p = 0.027) and to RR interval (t-N/RVET % = 16 +/- 2% vs 24 +/- 1%; p = 0.002). Doppler derived RV index proposed by Tei was slightly higher in CTEPH (0.81 +/- 0.18 vs 0.65 +/- 0.32 in PAH) but not significantly. Taken together our observations indicate that dynamical coupling between RV and pulmonary arteries is more disturbed in CTEPH than in PPH despite similar levels of chronically increased PAP.

We aimed to investigate relations between PAP increase on exercise and long-term evolution of pulmonary hypertension and survival. 43 COPD pts were investigated. Initially they with mild pulmonary hypertension and abnormally high increase in PAP on exercise (40 W, 5 min). All pts were followed-up for 3-5 years. During follow-up 21 pts. died (group I), mean survival time was 28 +/- 17 month, and 22 survived (group II), mean observation period was 59 +/- 22 month. Survivors were recatheterized after 3-7 yrs (mean 5 yrs). PAP increased by 3.4 +/- 4.9 mm Hg and correlated better with initial PAP (r = 0.91, p < 0.001) than with delta PAP on exercise (r = 0.61, p < 0.02). Survivors differed from dead in FEV1 (1.59 +/- 55 vs 1.17 +/- 0.33 L, p < 0.02), VC (3.54 +/- 1.0 vs 2.8 +/- 0.84 L, p < 0.02), resting PaO2 (39.1 +/- 9.0 vs 46.3 +/- 11.5 mm Hg, p < 0.05) resting heart rate (82.5 +/- 13.6 vs 93.8 +/- 16.1 beats/min p < 0.02) resting PAP (20.7 +/- 8.2 vs 32.3 +/- 13.2 mm Hg, p < 0.002), and resting PW (7.6 +/- 2.0 vs 11.6 +/- 7.4 mm Hg, p < 0.05).

The aim of this study was to test if the lung compliance and other indices of pulmonary function correlated with hemodynamic parameters in patients with secondary pulmonary hypertension due to mitral stenosis. 36 patients (mean age 50 years) with mitral stenosis (mean mitral valve area-1.2 cm2), without history of lung diseases were analyzed in the study. 16 patients (group A) were in the II-nd and 20 patients (group B) were in the III-rd and IV-th NYHA class. All patients underwent Swan-Ganz catheterization with evaluation of pulmonary pressures, resistances and pulmonary veins compliance. Pulmonary function tests (spirometry, plethysomography, lung compliance) were also performed. In both analyzed groups the pulmonary artery pressure and pulmonary vein compliance correlated significantly with pulmonary compliance.

One of the most critical problems of neonatology persistent pulmonary hypertension in the newborn, is discussed. This syndrome is characterized by increased resistance in pulmonary vessels leading to hypertension in pulmonary arteries and a recurrence of R > L shunting at the ductus arteriosus and foramen ovale. This leads to deep hypoxia and hypoxemia. It is most important to rule out a congenital cyanotic heart defect in the differential diagnosis. The aim of the treatment is to establish normal ventilation and intrapulmonary shunting as well as to control the symptoms leading to pulmonary hypertension that persist over time. The goal preceding treatment should be to determine the initial cause of the persistent pulmonary hypertension.

We present our 7 years experience with 135 pts aged 1.4-44 years (mean 10-yrs) in whom percutaneous balloon valvuloplasty (BPV) for congenital pulmonary stenosis was attempted. In 4 pts we failed to place the balloon at valvar level, in another four BPV was repeated so, 135 procedures in 131 pts were performed. Balloon diameter/pulmonary anulus diameter ratio (BD/PD) ranged from 0.9 to 1.85 (mean 1.37). In 10 cases double balloon technique was used. Immediate results: in the whole group of 131 pts the right ventricular-pulmonary artery gradient (RV-PAG) was reduced from 74 +/- 42 to 34 +/- 34 mm Hg, and right ventricular systolic pressure (RVSP) decreased from 92 +/- 41 to 54 +/- 34 mm Hg just after BPV. 86 pts (65.6%)--group I, had good immediate result of BPV (RVSP less than 50 mm Hg). 4 of them had dysplastic pulmonary valves (DPVs). RV-PAG in 82 pts with normal valves decreased from 53 +/- 25 to 15 +/- 7 mm Hg right after BPV. In 45 pts (34.4%)--group II, immediate result of valvuloplasty was recognised as unsatisfactory (RVSP greater than or equal to 50 mm Hg): in 4 of them BD/PD was smaller or equal 1.2 (subgroup IIa); 34 others had significant subpulmonary obstruction (SPO) that appeared or increased after BPV (subgroup IIb); and in remaining 7, DPVs were noticed (subgroup II c).(ABSTRACT TRUNCATED AT 250 WORDS)

Patients with Raynaud's phenomenon can demonstrate arterial spasm not only in hands but also in other organs. The aim of this study was to investigate pulmonary function in 15 patients with Raynaud's disease and in 8 healthy controls before and after cold pressor test. Before the cold pressor test no significant differences in pulmonary parameters between the patients and the controls were observed. After cold pressor test, a statistically significant decrease in diffusing capacity (DLCO) was observed in the study group, but not in the controls. Total lung capacity (TLC) was also slightly decreased. No significant changes of specific diffusing capacity (D/VA) and flow-volume curve parameters (FEF50, FEF25) were noticed.

The purpose of the study was to analyse relationships between the presence and magnitude of a regurgitant wave from the left ventricle to the left atrium and the state of the pulmonary circulation in patients with primary dilated cardiomyopathy. The study population consisted of 97 patients divided into three subgroups I--41 patients without the regurgitant wave, II--31 patients with the first-degree regurgitant wave, III--25 patients with the second-degree regurgitant wave. Diagnosis was based upon non invasive studies, cardiac catheterization and left ventriculography. Average values of the left ventricular systolic and end-diastolic pressures were similar in all subgroups, whereas the cardiac index was the lowest in the subgroup of patients with a large regurgitant wave to the left atrium. There were no differences in mean values of the pulmonary artery pressures, total pulmonary resistance and pulmonary vascular resistance between the patients without the regurgitant wave (subgroup II). In contrast the patients with a large regurgitant wave (subgroup III) showed statistically significantly higher pulmonary artery pressures, higher total pulmonary resistance and pulmonary vascular resistance. In 64% of the patients of this subgroup the left atrium was said to be large. The results of the present study indicate that even not a large regurgitant wave (second-degree) from the left ventricle to the left atrium leads to secondary pulmonary hypertension in patients with primary dilated cardiomyopathy.

In 47 patients with chronic obstructive pulmonary disease and 19 patients with idiopathic pulmonary fibrosis pulmonary haemodynamics were studied. Pulmonary arterial pressure was compared with non-invasive lung function tests, ecg and the chest X-ray. Correlations between pulmonary arterial pressure and some of those parameters were found. Investigations were repeated after 3 or 5 years depending on severity of pulmonary hypertension initially found. The progression of pulmonary hypertension was found. It was accompanied by intensification of non-invasive indices of pulmonary hypertension. More than half of patients with initially severe pulmonary hypertension (> or = 30 mmg Hg) did not survive 3 years.

We wanted to verify if the acute effect of oxygen on pulmonary arterial pressure (PPA) is related to survival on LTOT as was suggested recently in the literature. We studied 46 COPD patients qualified for LTOT. The acute effects of O2 on pulmonary haemodynamics were assessed by pressure and flow measurements before and after 30 mins of O2 breathing via 28% Ventimask. Thirty nine patients reacted with a fall of the mean PPA of less than 5 mm Hg. These were termed nonresponders (NR). In seven patients mean PPA fell > 5 mm Hg. They were called responders (R). After the initial investigations patients were followed up on LTOT for 2 years or until death. During two years of LTOT 15 patients died (12 from NR and 3 from R groups). The first two years survival rate was 69% in NR and 57% in R groups respectively. We conclude that survival on LTOT is not related to the acute effect of oxygen on the pulmonary arterial pressure in COPD patients investigated in the steady-state period of the disease.

In a 22 year old female with primary pulmonary hypertension Doppler echocardiography revealed a systolic gradient between the right atrium and ventricle of 11.3 kPa (85 mmHg). A trial of high dose nifedipine therapy was started. It's efficacy was assessed by monitoring the tricuspid gradient. During therapy variations of the gradient were seen but without a favorable, steady, decrease trend. Although nifedipine did not produce any improvement during the "acute trial"--nifedipine therapy was continued--initially 60 mg, followed by 80 mg and finally 100 mg per day. Efficacy of this therapy was also controlled by Doppler echocardiography. Nifedipine was discontinued after 8 months due to lack of improvement in the patient's state. The patient died 30 months from the initial symptoms. Pathomorphological examination confirmed the clinical diagnosis. The use of Doppler echocardiography in assessing the vasodilators in primary pulmonary hypertension is discussed.

Pulmonary artery pressure was measured in 70 patients with chronic bronchitis. Those with pulmonary hypertension underwent two hours oxygen therapy (flow 10-12 l/min). Those in whom a decrease of pulmonary artery pressure was observed were qualified for long term oxygen therapy (LTOT). In 32 patients which were qualified for LTOT, after a two week period of 18 hour per day oxygen therapy a decrease of systolic pulmonary arterial pressure from 336.1 mm Hg to 30.9 mm Hg and a decrease of mean pulmonary artery pressure from 25.9 to 23.2 mm Hg was observed.

In 41 patients with cardiac defects cardiac catheterization and echocardiographic examination were performed to evaluate the usefulness of Doppler echocardiography for estimation the grade of pulmonary hypertension. Parameter for noninvasive estimation of mean pulmonary artery pressure was the acceleration time (AcT) calculated basing on pulsed Doppler recordings of blood flow through the right ventricular outflow tract. Technics of examination and calculation of mean pulmonary artery pressure (MPAP) were concordant with Kitabatake's methodology. Patients were divided into 3 groups according to MPAP values measured by the invasive method. MPAP values obtained using Doppler method were not identical with results of direct measurements, but in the majority of patients (85%) Doppler method allowed to properly qualify patients to a respective group--with normal, moderate or very high pulmonary artery pressure. We think that the presented method is a semiquantitative one and useful for monitoring of pulmonary pressure in patients with cardiac defects.

In 40 patients aged 25-52 years (mean 36 years) with mitral valve disease but without overt circulatory failure haemodynamic studies and echocardiographic examinations were carried out recording the blood flow in the pulmonary artery and the blood flow through the tricuspid valve by pulsating and continuous-wave methods. From the obtained curves the time was calculated from the beginning of the flow to its maximum (TPV), the pre-ejection time (RPEP) and the right-ventricular ejection time (RVET), and the indices TPV/RVET and RPEP/RVET. Moreover, in 14 patients with coexistent insufficiency of the tricuspid valve the systolic pressure in the pulmonary artery was calculated by determining the systolic gradient across the tricuspid valve. On the basis of the results of haemodynamic examination the patients were divided into two groups: with normal (24 patients) and with raised (16 patients) pressure in the pulmonary artery. In the group of mitral valve disease and pulmonary hypertension a significantly shorter TPV, lower values of the TPV/RVET index and greater values of the RPEP/RVET index were found as compared with the patients with mitral disease and normal value of the systolic arterial pressure in the pulmonary artery. The systolic arterial pressure in the pulmonary artery determined on the basis of Doppler echocardiography with measurement of the regurgitation wave in tricuspid valve insufficiency showed a high agreement (r = 0.94) with the pressure recorded during cardiac catheterization.

The study concerned certain aspects of pulmonary hypertension. The most frequent factors in the aetiology of the syndrome are: 1) pressure rise in pulmonary capillaries and/or left atrium, 2) considerable rise of blood flow in the pulmonary artery, 3) reduced total blood flow in the pulmonary vascular bed. Pulmonary hypertension is divided into passive and hyperkinetic. Moreover, pulmonary hypertension of unknown aetiology exists, so called primary pulmonary hypertension, which may occur as one of three types of the disease: thromboembolic pulmonary hypertension, primary pulmonary arteriopathy, primary pulmonary thrombophlebitis.