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At least 991 records · Page 55Linked to original sources
PubMed2003

Intravenous sildenafil is a potent pulmonary vasodilator in children with congenital heart disease.

BACKGROUND: Increased pulmonary vascular resistance (PVR) because of congenital heart disease (CHD) may be caused by a dysfunction in endogenous pulmonary endothelial nitric oxide (NO) production. In other forms of pulmonary vascular disease with increased PVR, an elevated activity of a phosphodiesterase type 5 (PDE-5), responsible for the degradation of cyclic guanidine monophosphate (cGMP), the second messenger of endothelially produced NO, has been demonstrated. This study compares the effects of inhaled NO before and after the specific inhibition of the PDE-5 by intravenous sildenafil (Viagra) in pre- and postoperative children with increased PVR because of CHD. METHODS AND RESULTS: 12 children with congenital heart disease (age 0.2 to 15.7 years, median 2.4 years) and increased mean pulmonary arterial pressure, and 12 postoperative children (age 0.11 to 0.65 years, median 0.32 years) with increased PVR (8.3+/-1.0 Wood Units*m2) were studied during cardiac catheterization ("cath laboratory"), or within 2 hours after return from cardiac surgery ("post op"), respectively. All were sedated, tracheally intubated and paralyzed. During alveolar hyperoxygenation (FiO2=0.65), the effects of inhaled NO (20 ppm) were compared before and after the stepwise infusion of sildenafil ("cath laboratory", 1 mg/kg; post op, 0.25 mg/kg). Intravenous sildenafil more effectively reduced PVR than NO (11.5% versus 4.3% in the "cath laboratory" patient group, P<0.05, and 25.8% versus 14.6% in the post op patient group, P=0.09. The increase in cGMP in response to NO was potentiated (2- to 2.4-fold) by PDE-5 inhibition. While the vasodilating effects of sildenafil showed pulmonary selectivity, its infusion was associated with increased intrapulmonary shunting in the postoperative patients (Qs/Qt=16.5+/-4.7% to 25.5+/-18.2% P=0.04). CONCLUSIONS: Intravenous sildenafil is as effective as inhaled NO as a pulmonary vasodilator in children with congenital heart disease. Although clinically insignificant in this study, increased intrapulmonary shunting with sildenafil may be disadvantageous in some patients after CHD surgery.

3',5'-Cyclic-GMP Phosphodiesterases↗
PubMed1990

[Quantitative evaluation of right ventricular overload in cor pulmonale using 201T1 myocardial SPECT].

In patients with right ventricular overload, the free wall of the right ventricle is often visualized by 201T1 myocardial scintigraphy. To determine quantitatively the discriminant and characteristics of cor pulmonale, 201T1 myocardial perfusion single photon emission computed tomography (SPECT) was performed in 16 patients with chronic obstructive pulmonary disease (COPD) and seven with restrictive pulmonary disease (RPD). We selected one section of the short-axis image of SPECT in which the right ventricle was most clearly visualized, and defined the T1-score as the ratio of the sum of counts in the region of interest (ROI) at the anterior, mid and posterior regions of the right ventricular free wall to the sum of counts in the ROI at the posterior, lateral, anterior walls of the left ventricle, and the anterior and posterior regions of the interventricular septum. Correlation of the T1-score with mean pulmonary arterial pressure (mPAP), systolic pulmonary arterial pressure (sPAP), and total pulmonary vascular resistance (TPR), as obtained by right cardiac catheterization, arterial oxygen tension (PaO2), and arterial carbon dioxide tension (PaCO2) were evaluated. The results were as follows: 1. In the patients with COPD, the T1-score correlated positively with mPAP (r = 0.71, p less than 0.005), TPR ( r = 0.69, p less than 0.005), and PaCO2 (r = 0.63, p less than 0.01), and it correlated inversely with PaO2 (r = -0.58, p less than 0.05). However, there was no significant correlation between the T1-score and mPAP, TPR, PaCO2, and PaO2 in the patients with RPD.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1976

Pulmonary congestion in chronic heart disease. Radiologic, clinical and hemodynamic relationships.

Pulmonary congestion in chronic heart disease was evaluated by lung auscultation and roentgen examination of the chest and compared with pressure recording of the pulmonary circulation in 49 cases. Whereas auscultation appeared not to be correlated to the pressures, the radiologic findings proved to be a reliable measure of increased pressures. The radiologic assessment was based on isolated flow shift to the upper lobes of the lungs, indicating slight pulmonary venous hypertension, and flow shift in association with blurring of the lower lobe vessels, indicating pulmonary interstitial oedema. In distinction to the results from acute cardiac failure interlobular septa were often demonstrated in association with pulmonary interstitial oedema, and pulmonary alveolar oedema rarely demonstrated in spite of marked pulmonary venous hypertension.

Adolescent↗
PubMed1964

BRONCHIOLAR EMPHYSEMA. A REPORT OF A NECROPSIED CASE OF DIFFUSE BRONCHIOLECTASIS AND REVIEW OF THE LITERATURE.

Bronchiolar emphysema is a rare, insidious, progressive, bilateral pulmonary fibrosis with diffusion defect. Death follows in five to 10 years after the onset of symptoms because of respiratory failure, cor pulmonale or pneumothorax. In the 20 cases reviewed, the lungs typically showed bosselation of their pleural surfaces, giving the appearance and consistency of a liver involved by Laennec's cirrhosis. Cross sections showed honeycombing chiefly along the lung margins. Microscopically, cystic dilatation of terminal bronchioles was present, with obliteration of the remaining peripheral lung tissue by a dense pulmonary fibrosis. Hyperplasia of smooth muscle and elastica was prominent.Bronchiolar emphysema is a clinicopathologic entity, the etiology of which is unknown and likely multiple. Bronchiolectasis and superimposed recurrent infection are essential in its pathogenesis. Thickening of alveolar walls may play a decisive role in determining the characteristic site of the bronchiolar dilatation.

Autopsy↗
PubMed1984

[Value of the vectorcardiogram in the evaluation of right heart hypertrophy in chronic obstructive lung diseases].

In 41 patients with severe chronic obstructive lung disease vector cardiographic and electrocardiographic results and the level of pulmonary arterial pressure (pap) are compared. We observed a positive correlation between normal results from vector cardiography, electrocardiography, and pulmonary arterial pressure. In cases of hypertrophy of the right heart, type I and type II according the method of Lichtlen, pulmonary arterial hypertension was found in 72% of the cases.

Cardiomegaly↗