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Vulnerability of pulmonary capillaries in heart disease.

The pulmonary blood-gas barrier presents a dilemma. It must be extremely thin for efficient gas exchange. However, it also needs to be immensely strong because the stresses in the pulmonary capillary wall become extremely high when the capillary pressure rises. Stress failure of the capillaries occurs in several pathological conditions. It causes high-permeability edema as in neurogenic pulmonary edema or high-altitude pulmonary edema; alveolar hemorrhage, which occurs in all galloping racehorses; or a combination of the two as in severe congestive heart failure. The vulnerability of the capillary wall to increased mechanical stress has not previously been sufficiently appreciated.

Animals↗

The use of high-resolution computed tomography in the evaluation of pulmonary hemodynamics in patients with congenital heart disease: in pulmonary vessels larger than 1 mm in diameter.

High-resolution computed tomography (HRCT) was carried out in 36 patients with congenital left-to-right shunt disease and 10 normal control subjects to assess the feasibility of CT in the evaluation of pulmonary hemodynamics. The patients had a left-to-right or a bidirectional shunt and the hemodynamic data obtained by cardiac catheterization in these patients were compared to the information obtained by CT imaging. The pulmonary/systemic blood flow (Q(p)/Q(s)) ratio and pulmonic/systemic resistance (R(p)/R(s)) ratio had a significant correlation with the pulmonary artery/bronchus (PA/Br) ratio (r = 0.54 and r = -0.37, respectively) and pulmonary vein/bronchus (PV/Br) ratio (r = 0.66 and r = -0.66, respectively), and the R(p)/R(s) and mean PA pressure also showed a significant correlation with the PA/PV ratio (r = 0.53 and r = -0.61, respectively) in the mid-lung field when accompanying bronchi were 4. 0-5.9 mm in diameter. There was no correlation between the hemodynamic data and the size of the central and hilar PA or with the rate of PA tapering. With HRCT, it is possible to evaluate pulmonary hemodynamics in patients with congenital heart disease with a left-to-right or bidirectional shunt, particularly R(p)/R(s) and mean PA pressure, which have been very difficult to obtain noninvasively. The small-sized pulmonary vessel/Br ratio or the small-sized PA/PV ratio could offer very useful information, but the dimension of the central PA provided the least useful information.

Adolescent↗

Combination therapy of prostacyclin for pulmonary hypertension in congenital heart disease.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart disease. Despite differences in etiology and pathophysiology, successful therapy for idiopathic PAH may benefit in patients with congenital heart disease. We theorized that combination of oral and aerosolization prostacyclin will benefit this group of patients in long-term. MATERIAL AND METHOD: The study design was single group and open label study with intention to treat for patients with congenital heart disease with pulmonary artery (PA pressure) more than 50% of systemic pressure. All patients were given a combination of orally given beraprost sodium and inhalation of iloprost for 12 months. Data were collected prospectively consisting of functional class, O2 saturation, 6-minute walk test and right ventricular systolic pressure (RVSP). RESULTS: There were 23 patients with an average right ventricular systolic pressure (+/- SD) of 94.8 +/- 14.5 mmHg and with average age of 27.8 +/- 14.9 years (2.5 to 50 years). The average oxygen saturation was 87.9 +/- 7 %. There were 12 patients with post surgical repair or cardiac catheterization interventional procedure and 11 with and Eisenmenger's syndrome. There were significant improvement of 6-minute-walk test from an average of 268 +/- 70 meters to 308 +/- 57 meters at the end of 12 months. The functional class of patients was also improving. However, there were no significant different in oxygen saturation. CONCLUSION: Combination therapy of oral and inhalation of aerosolized vasodilators is a fascinating concept in the therapy of pulmonary hypertension. Treated patients showed an improvement in exercise capacity and right ventricular systolic pressure without a worsening in oxygen saturation.

Administration, Inhalation↗

Hemodynamic effects of ketamine in children with congenital heart disease.

Pulmonary and systemic vascular responses to ketamine (2 mg X kg-1, intravenously) were studied during cardiac catheterization in 20 children with congenital heart lesions. Pulmonary and systemic resistances (Rp, Rs), ratios between pulmonary and systemic flows (Qp/Qs), and left to right (L----R) and right to left shunts (R----L) were calculated before and after ketamine administration. Statistically significant (P less than 0.05) but clinically minor increases in heart rate (106.8 to 109.9 beats/min), mean pulmonary artery pressure (20.6 to 22.8 mm Hg), and Rp/Rs (0.12 to 0.14) were seen after ketamine. There were no significant changes in systemic arterial pressure, Rs, Qp/Qs, L----R, R----L, or arterial oxygen or carbon dioxide tensions. No patient had any major untoward effects from ketamine administration. It is concluded that the hemodynamic alterations after ketamine administration in children undergoing cardiac catheterization are small and do not alter the clinical status of the patients or the information obtained by cardiac catheterization.

Blood Gas Analysis↗

Patterns of pulmonary arterial anatomy and blood supply in complex congenital heart disease with pulmonary atresia.

To identify the pattern of pulmonary anatomy and pulmonary blood supply, we analyzed the angiocardiograms of 40 patients with pulmonary atresia and complex congenital heart disease. All patients but two (95%) had confluent pulmonary arteries supplied by a patent ductus arteriosus. These two patients (5%) had nonconfluent pulmonary arteries, one with major systemic-pulmonary collateral arteries and absent patent ductus arteriosus and the other with a patent ductus arteriosus supplying the right lung and small collateral arteries supplying the left. The low incidence of pulmonary arterial abnormalities and the uniform pattern of pulmonary blood supply make the patients with pulmonary atresia and complex congenital heart disease potential candidates for palliative operation without cardiac catheterization in the neonatal period.

Adolescent↗

[Chronic cor pulmonale].

Cor pulmonale is defined as "hypertophy of the right ventricle resulting from diseases affecting the function and/or structure of the lungs, except when these pulmonary alterations are the result of diseases that primarily affect the left side of the heart, as congenital heart disease". Pulmonary hypertension is a frequent hemodynamic complication associated with a wide variety of respiratory systems disorders whose only common physiologic abnormalities are alveolar hypoxia and consequent arterial hypoxemia of longterm duration. The sustained elevation in pulmonary arterial hypertension is thought to be mediated through two pathophysiologic vascular mechanism: 1) persistent vasoconstriction and 2) vascular structural remodeling. The combination of these processes causes vascular luminal narrowing and vessel obliteration that reduce pulmonary vascular surface area to the critical degree necessary for the development of the pulmonary hypertension. Cor pulmonale may be difficult to diagnose, particularly early in its course, when they symptoms manifested may be interpreted as representing progression of an underlying pathophysiological state, such as chronic obstructive airways disease. The treatment of cor pulmonale is directed toward reversing the pathogenetic process that can be directly treated, while at the same time relieving the hypoxemia, hypercapnia or acidosis. At present long-term oxygen therapy is the best treatment for pulmonary hypertension. Heart failure in cor pulmonale is usually transient once the initiating mechanism is controlled. The usual therapeutic measures for heart failure apply: a low-salt regimen, and diuretics.

Chronic Disease↗

Hemodynamic effects of prostaglandin E1 in patients with congenital heart disease and pulmonary hypertension.

Investigations were performed to determine whether prostaglandin E1 (PGE1) would cause a favorable change in pulmonary hemodynamics in pediatric patients with congenital heart disease and elevated pulmonary vascular resistance. Twenty-seven patients (ages 0.5 months to 19 years) were studied. PGE1 caused a significant decrease in mean pulmonary artery pressure with 0.05 microgram/kg/min and 0.1 microgram/kg/min infusions (p = .0004). There were significant increases in pulmonary blood flow (p = .0007), and decreases in pulmonary vascular resistance (p = .0001); changes in systemic pressure, resistance, oxygen consumption, and heart rate were minimal. Twenty-three patients underwent heart surgery after catheterization, nineteen of these survived. The four nonsurvivors had higher initial pulmonary resistance (PVR) and less decline in PVR with PGE1 than survivors. This study shows that PGE1 is an effective pulmonary vasodilator in patients with congenital heart disease and elevated pulmonary vascular resistance.

Adolescent↗

[Contractility parameters of the right heart in patients with chronic obstructive lung diseases without pulmonary hypertension].

By means of the radionuclide ventriculography certain contractility parameters of the right heart are presented in 15 patients with chronic obstructive bronchitis without pulmonary hypertension. The results confirm that also without increase of the pulmonary arterial blood pressure the ejection fraction of the right ventricle in rest is decreased in possible dependence upon the size hypoxaemia. The increase of the ejection fraction of the right ventricle under stress is insufficient in 7 out of 15 patients, in which case a connection with the left-ventricular ejection fraction is observed. A characteristic valuation of the right- and left-ventricular ejection fraction for pathophysiology and prognosis of the pulmonary heart disease is discussed. The necessity of further investigations is emphasized.

Adult↗