The Natural History of Isolated Pulmonary Stenosis

Abstract

The natural history of isolated pulmonary stenosis is largely unknown. Knowledge of this subject has been gained mostly from studying groups of patients of different ages, or from necropsy records, procedures clearly less satisfactory than following up individual cases. Only two authors have reported follow-up studies; Barritt (1954) observed 33 cases (8 have been catheterized) over periods of 5 to 21 years, and Fabricius (1959) followed up 47 of 75 fully-documented cases for periods of between 2 and 10 years, but in both series the patients were mainly children. The advent of successful surgical treatment has made such studies more difficult to pursue.In this paper we present the findings in 75 cases of isolated pulmonary stenosis with special reference to 59 who have been observed for periods ranging from 2 to 23 years. SUBJECTS AND METHODSThe records were examined of all patients who have attended the University Department of Cardiology, Manchester Royal Infirmary, during the past 23 years, in whom a diagnosis of isolated pulmonary stenosis was made. We were able to trace all patients in whom this diagnosis seemed likely from a study of the case notes; many had been attending at regular intervals and 24 had undergone surgical treatment. One patient diagnosed clinically died in 1952 and some died as a result of operation; all the remainder who had not been seen in the last 2 years were recalled and re-examined.Cardiac catheterization was performed in 59 of the 75 patients; those not investigated by catheterization were only included in the series if a confident clinical diagnosis could be made. In all except one, who probably had associated aortic stenosis, pulmonary stenosis was the only lesion present. None were clinicially cyanosed. The stenosis was valvular in all except 4 patients where it was due to a localized subvalvular obstruction. Repeat catheterization after an interval of six to eight years was carried out in 3 patients.Patients were classified into mild, moderate, and severe cases according to Wood's criteria (1956). Those subjected to cardiac catheterization were graded according to the height of the right ventricular pressure, mild cases having pressures of 30-50 mm. Hg, moderate between 50 and 100 mm. Hg, and severe over 100 mm. Hg. Those not catheterized were graded on a combination of clinical, radiological, and electrocardiographic data as suggested by Wood, and which correlate closely with the hamodynamic changes.The clinical, electrocardiographic, and radiological findings at the first and subsequent attendances were noted and compared. For the purposes of this study, the period of follow-up of surgical cases ended at the time of operation. Effort tolerance was graded according to the nomenclature adopted by the New York Heart Association (1953).The electrocardiograms were classified as follows: Grade I: normal.Grade II: (i) mean frontal axis of +90°to + 1100 and/or(ii) R/S or R'S ratio in lead Vl greater than 1-0 with R or R' less than 0 5 millivolts. 151 1i on 10 May 2018 by guest. Prot...

Functional and haemodynamic assessment of mild-to-moderate pulmonary valve stenosis at rest and during exercise

“…Consequently, exercise capacity is reduced due to an inability to increase stroke volume with the increase in heart rate being the sole contributor to an increase in cardiac output 5 6. Conversely, the natural evolution of mild PV stenosis is considered to be benign without progression of the valve gradient after adolescence and no deaths are observed during an 8-year follow-up period 3 7. Because of this, no restrictions in physical activity are imposed 8 9…”

The Natural History of Pulmonary Stenosis

“…T inker et al [8] reported the follow-up of 59 cases. No evidence of deterioration was found in the patients with mild or moderate stenosis, three of whom had a second cardiac catheterization after intervals of 6-8 years when considerable growth occurred.…”

“…Surgical treatment is widely used in severe and moderate cases, and only few long-term follow-up studies have been reported [1][2][3][4][5][6][7][8]. In 1959 F abricius [3] from this laboratory published his monograph on 75 fully documented cases.…”

Regional right ventricular remodeling and function in children with idiopathic pulmonary arterial hypertension vs those with pulmonary valve stenosis: Insights into mechanics of right ventricular dysfunction

“…4 Moreover, mortality vastly differs between the groupshigh in iPAH and very low in PS. 4,5 Although the duration and possibly character of increased RV pressure differs between the two conditions, the RV response also seems very different. A study by Jurcut et al 7 demonstrated this difference in adult iPAH and PS patients, showing global RV dilation and decreased global RV free wall (RVFW) longitudinal deformation in iPAH vs PS patients.…”

“…4,5 While a large proportion of iPAH patients present with, or rapidly develop, symptomatic heart failure, 5,6 PS patients are often asymptomatic or present with only mild exercise intolerance. 4 Moreover, mortality vastly differs between the groupshigh in iPAH and very low in PS. 4,5 Although the duration and possibly character of increased RV pressure differs between the two conditions, the RV response also seems very different.…”