Pulmonary arterial changes in patients with ventricular septal defects and severe pulmonary hypertension.

In 25 patients, aged eight months to 31 years, with ventricular septal defect (VSD; isolated in 15, the others with atrial septal defect, PDA, coarctation or patent ductus arteriosus + coarctation), each with severe pulmonary artery hypertension (pulmonary artery systolic pressure [Ppa] at least 75% of systemic and an elevated pulmonary vascular resistance), we related morphologic and morphometric data from open-lung biopsy to hemodynamic measurements obtained at cardiac catheterization during the same hospital admission. Of the hemodynamic features measured, only the ratios of pulmonary-to-systemic flow and pulmonary-to-systemic resistance correlated significantly with structure. Neither pulmonary artery pressure (Ppa) nor pulmonary vascular resistance correlated significantly with any structural feature studied. The increased external diameter of respiratory bronchiolar arteries in those with the more advanced Heath-Edwards grades reflects dilatation and suggests that it is in the small arteries of the distal arterial bed that the changes of pulmonary hypertension are most significant. Neither age nor body weight correlated significantly with the degree of structural or hemodynamic abnormality. In the ten patients who underwent VSD closure, Ppa was measured postoperatively. The Heath-Edwards grade (no more than one grade-III lesion) and arterial density (at least one-half that normal for age) were the best correlates of the difference between preoperative Ppa and Ppa immediately after corrective surgery. The presurgical catheterization data, including pulmonary resistance and the resistance ratio, did not correlate significantly with change in Ppa following VSD closure.(ABSTRACT TRUNCATED AT 250 WORDS)

[Role of lung biopsy in a heart surgery center (a new method for the quantitative assessment of pulmonary hypertension by biopsy of the lung of patients with congenital heart defects)]. / Rol' biopsii legkogo v kardiokhirurgicheskom tsentre (novyi metod kolichestvennoi otsenki legochnoi gipertenzii v biopsii legkogo u bol'nykh vrozhdennymi porokami serdtsa).

Forty-three lung biopsies taken from 42 patients with various congenital heart malformations with high pulmonary hypertension (aged from 8 months to 34 years) were studied by morphological and morphometric methods. Changes in the pulmonary vessels were evaluated according to Heat-Edwards' classification and morphometric classification of Rabinovitch , et al. The morphometric data give some additional information on the growth and development of peripheral vessels, the degree of hypertrophy of tunica media muscularis, a decrease in the size of arteries. The demonstration of considerable reduction in the number of peripheral arteries by morphometry constitutes a serious prognostic sign of further progression of sclerotic changes in the pulmonary vessels even in the early correction of the heart malformation.

Aortopulmonary septal defect: hemodynamics, angiography, and operation.

Twenty-five patients with malseptation of the aortopulmonary trunk resulting in aortopulmonary septal defect (window) were evaluated in a unique combined surgical series assembled from two institutions participating in the USA-USSR Health Exchange Program. Typical aortopulmonary septal defect or window (type I) with the connection between the ascending aorta and main pulmonary artery was found in 21 patients. Four had a more cephalad defect (type II) between the ascending aorta and the origin of the right pulmonary artery. Hemodynamics were the consequence of a large left-to-right shunt (mean ratio of pulmonary to systemic flow, 3.0) with right ventricular hypertension (mean right ventricular pressure, 86 mm Hg) and increased pulmonary vascular resistance (mean, 7.4 U) (mean ratio of pulmonary to systemic vascular resistance, 0.33). Angiography may provide patterns that allow preoperative distinction between the two types of aortopulmonary septal defect and provide information important in planning the details of corrective operation. Operative techniques included ligation, direct suture, and patch closure of the aortopulmonary septal defect. The best method appeared to be patch closure by the transaortic approach; real and potential problems were associated with other techniques.

[Problem of pulmonary hypertension in determining the indications for an operation in congenital heart defects]. / Problema legochnoi gipertenzii pri opredelenii pokazanii k operatsii u bol'nykh vrozhdennymi porokami serdtsa.

The authors studied the indications for surgical treatment of patients with congenital heart diseases and increased pulmonary blood flow, depending on the degree of pulmonary hypertension. Particular attention is focused on the indications for operation in ventricular septal defect. The work is based on the experience of the workers of the institute, embracing 3,521 operations performed on this category of patients among whom there were 863 children under 3 years of age. The indications for surgery are determined in conformity with clinico-hemodynamic groups according to a classification elaborated at the institute. The late results studied in 213 patients in follow-up periods of up to 12 years show that the main evidence of the efficacy of surgical treatment are the hemodynamic data, which determine the prognosis and the subsequent working capacity of the patients.