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[Pathogenetic interactions between veno-occlusive disease and sideroelastosis pulmonum? (author's transl)].

The clinicopathologic findings in a case of a 66-year-old woman who died 19 days after admission to the hospital for treatment of chronic bronchitis and cor pulmonale are presented. Pathoanatomic investigation revealed striking features of pulmonary sideroelastosis, including fragmentation and iron impregnation of pulmonary elastic fibers. Simultaneously, the pulmonary veins showed alterations similar to those described as "veno-occlusive disease": numerous small and medium-sized pulmonary veins were stenosed or completely obliterated by fibrous intimal connective tissue. The etiology of the pulmonary lesions is discussed on the basis of previously published cases. The possibility of a causal relationship between sideroelastosis and veno-occlusion is considered.

Aged↗

Inhaled nitric oxide for children with congenital heart disease and pulmonary hypertension.

BACKGROUND: Endothelium-derived nitric oxide (NO) is a potent vasodilator and a major mediator of pulmonary vascular tone. METHODS: Five infants underwent a trial of inhaled NO with hemodynamic monitoring in the operating room after atrioventricular canal repair. An additional 15 patients with congenital heart disease and refractory pulmonary hypertension were treated with inhaled NO for 1 day to 10 days postoperatively. RESULTS: In the 5 infants with atrioventricular canal, corrective surgical intervention and conventional therapy (hyperventilation, inspired oxygen fraction of 0.80, and inotropic agents) lowered mean pulmonary artery pressure from 49.5 +/- 10.5 to 20.0 +/- 2.2 mm Hg (p < 0.001). Adding inhaled NO further decreased mean pulmonary artery pressure to 18.0 +/- 2.8 mm Hg (p = not significant). Inhaled NO had no effect on ventricular function curves (inflow occlusion) in this group. In the 15 patients with refractory postoperative pulmonary hypertension, 11 had a favorable response to inhaled NO, with a decrease in mean pulmonary artery pressure from 30.9 +/- 5.8 to 23.1 +/- 5.4 mm Hg (p < 0.01) in 8 patients with pulmonary artery catheters. CONCLUSIONS: These studies demonstrate that inhaled NO has minimal beneficial effect on pulmonary artery pressure or cardiac output in infants after repair of atrioventricular canal. Inhaled NO is effective in decreasing PAP postoperatively in select patients with congenital heart disease and pulmonary hypertension refractory to conventional therapeutic modalities.

Administration, Inhalation↗

Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy.

BACKGROUND: Endothelin receptor antagonism has been introduced as an effective oral therapy of patients with idiopathic pulmonary arterial hypertension. In view of the pathophysiologic and histologic similarities between idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), there is a rationale for treating these patients with the oral dual (ET(A)/ET(B)) endothelin receptor antagonist bosentan. METHODS: Thirty-three patients with PAH-CHD (43 +/- 14 years, 23 with Eisenmenger syndrome) were treated with bosentan for a mean of 2.1 +/- 0.5 years. Efficacy was assessed by a panel of tests, including New York Heart Association functional class, 6-minute walking distance, and echocardiographic and hemodynamic parameters. RESULTS: Mean 6-minute walking distance increased from 362 +/- 105 to 434 +/- 68 m (P = .001). New York Heart Association class also improved significantly (3.1 to 2.4, P = .0001). This was associated with slight trends in improvements of transcutaneous oxygen saturation (86% +/- 7% to 88% +/- 7%, P = .13) and maximum oxygen uptake (13.2 +/- 4.0 to 14.9 +/- 2.5, P = .18). Right ventricular systolic pressure measured by echocardiographic decreased from 111 +/- 32 to 106 +/- 22 mm Hg (P = .001). Bosentan treatment was well tolerated by all patients. CONCLUSIONS: Long-term bosentan treatment in adult patients with PAH-CHD was well tolerated and improved functional status as well as exercise capacity. These findings have to be corroborated by controlled studies that are presently ongoing.

Adolescent↗

[Electrocardiography and echocardiography in the diagnosis of chronic cor pulmonale].

The study was aimed at evaluating the possibilities of electrocardiography (ECG) and echocardiography in establishing the diagnosis of chronic pulmonary heart disease (CPHD). Sixty patients with a chronic obstructive pulmonary disease (COPD) and clinical symptoms of cardiac decompensation were observed. The examined patients ranged from 26 to 74 years of age, the mean age being 59.25 years. The diagnosis of CPHD was established by electrocardiography in 43 patients (78.18%) while in 57 patients (95%) it was done by ultrasound cardiography. Morphologic changes of the right ventricle (RV) were quantified, i.e. the diagnosis of chronic pulmonary heart disease was confirmed by echocardiography parameters such as right ventricle free wall thickness--RVFWT (0.82 +/- 0.09 cm), enddiastolic right ventricle dimension--ERVD (3.78 +/- 0.73 cm) and tricuspid regurgitation--TR (2.9 +/- 0.59, i.e. 2.58 +/- 0.55 after therapy). The information on the right ventricle function was provided by the values of echocardiography parameters, right ventricle ejection fraction--RVEF (30.98 +/- 5.88%, i.e. 34.1 +/- 5.64 after therapy) and right ventricle systolic pressure--RVSP (60.41 +/- 16.98 mmHG, i.e. 51.50 +/- 12.76 after therapy). Monitoring of these values after therapy, as well as of the RTO changes gave a good insight into the therapy effects. Echocardiography has been concluded to be of better sensitivity than ECG in diagnosing CPHD and both methods, noninvasive and easily applicable, have an important role in examining cardiac changes in patients with COPD.

Adult↗

A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow.

A new angiographic method for quantitative standardization of cross-sectional area of bilateral pulmonary arteries, the PA-index, and retrospective analysis of the PA-index in different types of operative procedures are presented. This study included 40 subjects in the normal control group, 46 patients in the tetralogy group, 26 patients in the Rastelli group, and 15 patients in the Fontan group. The normal value of the PA-index was 330 +/- 30 mm2/BSA and was consistent in a wide range of body surface areas from infancy to adolescence. The PA-index in the tetralogy and Rastelli groups ranged from 100 to 400 mm2/BSA. There were no early deaths in the tetralogy group, but the incidence of low cardiac output was higher in patients with a smaller PA-index, especially when the PA-index was less than 150 mm2/BSA. Low cardiac output was more severe in the Rastelli group. The operative mortality was significantly affected by the PA-index. In the Rastelli group, all of the patients with a PA-index of less than 200 mm2/BSA died, whereas the mortality rate in patients with a PA-index of more than 200 was only 6% (p less than 0.01). The mortality rate was not influenced by any other factors, such as aortic cross-clamp time or age at operation. In the Fontan group, two patients with a PA-index of less than 250 mm2/BSA died of severe heart failure, and 12 of 13 patients with a PA-index of more than 250 survived (p less than 0.01). Our results indicated the validity of the PA-index in predicting the postoperative prognosis of the various entities. In tetralogy, all patients with a PA-index over 100 mm2/BSA can undergo correction safely; in Rastelli operation, those with a PA-index under 200 should have a palliative procedure first, whereas those with a PA-index over 250 can be considered good candidates for the Fontan procedure. The PA-index may also serve a useful guide in comparing surgical results from different institutions with patients having anomalies of varying severity.

Adolescent↗

[Perioperative changes in plasma endothelin and calcitonin gene-related peptide in congenital heart disease with pulmonary hypertension].

OBJECTIVE: To assess the perioperative changes in plasma endothelin (ET) and calcitonin gene-related peptide (CGRP) in children with congenital heart disease complicated by pulmonary hypertension. METHODS: In 33 patients, radioimmunoassay was used to measure plasma ET and CGRP levels at different time points: Preoperative, immediate post-op, 3 hrs, 24 hrs, 72 hrs after cardiac surgery with cardiopulmonary bypass. RESULTS: Preoperative plasma ET levels in two groups were markedly increased. The level of ET in group II (severe pulmonary hypertension patients) was significantly higher than those [(138+/-25 compared with 77+/-19)pg/ml, P<0.01] in group I (mild pulmonary hypertension patients). In group I, the plasma ET level rose immediately post-op (P<0.001) and retumed to the initial level after one day (P>0.05). However, in group II plasma ET level was only normalized 3 days post-op (P<0.05). The CGRP level of both group I and group II increased at the first post-op day [(187+/-131 330+/-187)pg/ml], then began to decline by the 3rd day [(88+/-85, 150+/-95) pg/ml]. CONCLUSION: Endothelin and calcitonin gene-related peptide was involved in the pathophysiologic process of pulmonary hypertension and correlated with the degree of pulmonary hypertension. The imbalance of ET and CGRP after cardiopulmonary bypass may affect the prognosis.

Adolescent↗

Clinical aspects of cardio-respiratory homeostasis.

Starting from general assumptions on the clinical aspects of homeostasis and on the functional correlations lung-heart in physiological and pathological states, the difficulty to diagnose an individual pulmonary heart disease is stressed, as well as the necessity to differentiate it from the cases of coronary heart disease, when respiratory failure aggravates the latent cardiac ischemia and induces a global cardiac failure. The diagnosis criteria of the two distinct pathological pictures are established, emphasizing the importance of this differentiation for clinical practice and epidemiological research.

Coronary Disease↗

Pulmonary embolism, pulmonary hemorrhage and pulmonary infarction.

We compared 41 patients with angiographic proof of pulmonary embolism and clinical signs of pulmonary infarction (as evidenced by an infiltrate on x-ray study and pleuritic pain in the area of the embolus) with 24 patients with pulmonary embolism but without infarction. Only 18 of the 41 patients with pulmonary infarction had associated heart disease. Pulmonary infarction was uncommon when emboli obstructed central arteries but frequent when distal arteries were occluded. Follow-up x-ray examination showed that the infiltrates resolved in the patients with pulmonary infarction without heart disease, but persisted when heart disease was present. We suggest that obstruction of distal arteries results in pulmonary hemorrhage owing to an influx of bronchial arterial blood at systemic pressure. Hemorrhage causes symptoms and x-ray changes usually attributed to pulmonary infarction. However, hemorrhage resolves without infarction in patients without, but progresses to infarction in those with, heart disease.

Angiography↗

[Effect of tetramethylpyrazine on endothelin, von Willebrand factor and thromboxane A2 during cardiopulmonary bypass in patients of congenital heart disease with pulmonary hypertension].

OBJECTIVE: To study the effect of tetramethylpyrazine (TMP) on the vascular endothelial cell (VEC) related humoral factors, including endothelin (ET), factor VIII related antigen (i.e. von Willebrand factor, vWF) and thromboxane A2(TXA2) in patients of congenital heart disease with pulmonary hypertension (CHD-PH) during cardiopulmonary bypass (CPB), and explore the clinical physiopathologic significance of them. METHODS: Thirty non-cyanotic patients of CHD-PH were randomly divided into the control group and the treated group. TMP was given to the treated group by intravenous dripping 3 mg/kg after anesthesia induction and adding 1 mg/kg in oxygenator during CPB. Blood samples were collected from radial artery at the time points of after anesthesia induction, 15 min after beginning CPB, 5 min after opening aorta, 20 min, 6 hrs and 24 hrs after stopping CPB, to determine the plasma contents of ET and vWF, as well as TXB2, the stable metabolite of TXA2. The pulmonary vascular reactivity 6 hrs (6h-PVR) after CPB and the mechanical ventilatory support time (VST) after operation were calculated. RESULTS: Levels of ET, vWF and TXB2 increased obviously during CPB, but the degree of increasing in the treated group was lower than that in the control group (P < 0.05), and the 6h-PVR and VST in the former were also lower than those in the latter respectively. CONCLUSION: TMP could obviously reduce the production of ET, vWF and TXB2 during CPB and relieve the pulmonary vascular reactivity after operation, indicating that TMP could reduce the injury of CPB on VEC, and is benefit to enhance the efficacy of treatment.

Adolescent↗