Ann Thorac Surg 2001;72:905-906
© 2001 The Society of Thoracic Surgeons
Case report
Reduction in recalcitrant pulmonary hypertension after operation for atrial septal defect
Hitoshi Yamauchi, MDa,b,
Shigeo Yamaki, MDa,b,
Masahiro Fujii, MDa,b,
Hideyuki Iwaki, MDa,b,
Shigeo Tanaka, MD, PhDa,b
a Division of Cardiovascular Surgery, Department of Surgery II, Nippon Medical School, Tokyo, Japan
b Department of Cardiology, Katta General Hospital, Shiroishi, Japan
Accepted for publication May 24, 2000.
Address reprint requests to Dr Yamauchi, Division of Cardiovascular Surgery, Department of Surgery II, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
e-mail: yamauchi_hitoshi/surg2{at}nms.ac.jp
 |
Abstract
|
|---|
We present the case of a patient with atrial septal defect and severe pulmonary hypertension with pulmonary artery peak pressure greater than 110 mm Hg. Open lung biopsy was done prior to the corrective operation, and pathological findings in the small pulmonary arteries included "musculoelastosis" and complete occlusion of 70% of these small arteries and arterioles. The atrial septal defect was closed, and long-term oral prostacyclin therapy was initiated. Pulmonary artery peak pressure decreased to 65 mm Hg 2 years after the operation. This case demonstrates that in a patient with 70% complete occlusion of small pulmonary arteries and arterioles resulting from "musculoelastosis," not only is surgical intervention possible but also pulmonary artery pressure decreases in the long term after operation.
|
Introduction
|
|---|
It is not often that severe pulmonary hypertension occurs in patients with atrial septal defect (ASD), but once it does, the prognosis is not favorable. The hemodynamic determination of operability in patients with ASD and severe pulmonary hypertension is problematic [1]. It is our policy to perform open lung biopsy prior to a corrective surgical procedure in patients with ASD and pulmonary vascular resistance greater than 8 U/m2 or pulmonary artery peak pressure greater than 70 mm Hg. We then determine candidacy for operation on the basis of the pathological findings.
A 35-year-old woman was referred to our hospital in August 1996 because of shortness of breath and palpitations. When she was 3 years old, the diagnosis of ASD had been made, but she had not undergone intracardiac repair. On this admission, cardiac catheterization was performed. The pulmonary artery pressure and pulmonary vascular resistance were 110/31 mm Hg (mean value, 65 mm Hg) and 8 U/m2 in room air and 105/34 mm Hg (mean value, 61 mm Hg) and 4.8 U/m2 during pure oxygen inhalation, respectively (Table 1). We performed an open lung biopsy. Pathological findings in the small pulmonary arteries involved "musculoelastosis," a term used by Yamaki and associates [1, 2] to mean "intimal proliferation of longitudinal smooth muscle bundles and elastic fibers." In our patient, there was severe proliferation of these muscle bundles and elastic fibers in the intima, and 70% of all small pulmonary arteries and arterioles were completely occluded (Fig 1). The Heath-Edwards classification was grade III. We determined operability on the basis of histopathological criteria [1].
View larger version (104K):
[in this window]
[in a new window]
|
Fig 1. (A) Completely obstructed lumen of small pulmonary artery because of proliferation of longitudinal smooth muscle bundles and elastic fibers ("musculoelastosis"). (B) Both peripheral small pulmonary arteries are obstructed and destroyed by "musculoelastosis." Elastica Goldner stain; A, x200; B, x400; both before 63% reduction.
|
|
Direct closure of the ASD was accomplished uneventfully. The pulmonary artery pressure immediately after operation was 80/34 mm Hg (mean value, 52 mm Hg). After 2 years of oral prostacyclin (PGI2) therapy, pulmonary artery pressure was 65/35 mm Hg (mean value, 48 mm Hg) (Table 1). Surgical intervention and long-term PGI2 therapy started immediately after operation improved the New York Heart Association functional status of the patient from class II with full medication (digitalis, diuretics, and calcium-channel blocker) to class I with PGI2 only.
|
Comment
|
|---|
Changes related to ASD in the small pulmonary arteries were grouped into four classifications as follows [1, 2]: plexogenic arteriopathy; thromboembolism; "musculoelastosis"; and combinations of the first and second or the first and third. In patients with the finding "musculoelastosis," even if the vascular lumens of the small pulmonary arteries and arterioles are completely occluded by the longitudinal smooth muscle bundles with thrombi of fibrin deposit in the intima, our experience leads us to recommend ASD closure. Wagenvoort and associates [3] also indicated that thickening of the intima, if comprised of only longitudinal muscles, is a benign vascular lesion. Yamaki and coauthors [4] previously proposed an index of pulmonary vascular disease as a measure to describe the severity of plexogenic arteriopathy. However, in the case of "musculoelastosis," this index is not effective, and the occlusion or the stenosis of the lumen is important.
In one patient, we did not expect the postoperative pulmonary artery pressure to be substantially lower, but our pathological experience assured us that clinical symptoms would decrease and exercise ability would improve. Rosenzweig and colleagues [5] reported that a long-term regimen of PGI2 improves hemodynamics and quality of life in patients with pulmonary hypertension with associated congenital heart defects as previously demonstrated in patients with primary pulmonary hypertension. In one patient, 2 years of PGI2 therapy markedly improved hemodynamics and quality of life.
In conclusion, even in a patient with ASD and associated "musculoelastosis" and complete occlusion of 70% of the small pulmonary arteries, surgical intervention and long-term PGI2 therapy can reduce pulmonary artery pressure and improve quality of life.
|
References
|
|---|
-
Yamaki S., Horiuchi T., Miura M., Haneda K., Ishizawa E., Suzuki Y. Secundum atrial septal defect with severe pulmonary hypertension. Open lung biopsy diagnosis of operative indication. Chest 1987;91:33-38.[Abstract/Free Full Text]
-
Yamaki S., Horiuchi T., Miura M., Suzuki Y., Ishizawa E., Takahashi T. Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension. Chest 1986;89:694-698.[Abstract/Free Full Text]
-
Wagenvoort C.A., Keutel J., Mooi W.T., Wagenvoort N. Longitudinal smooth muscle in pulmonary arteries: occurrence in congenital heart disease. Virchows Arch [A] 1984;404:265-274.
-
Yamaki S., Mohri H., Haneda K., Endo M., Akimoto H. Indications for surgery based on lung biopsy in cases of ventricular septal defect and/or patent ductus arteriosus with severe pulmonary hypertension. Chest 1989;96:31-39.[Abstract/Free Full Text]
-
Rosenzweig E.B., Kerstein D., Barst R.J. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-1865.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
K. Hoetzenecker, H. J. Ankersmit, D. Bonderman, W. Hoetzenecker, R. Seitelberger, W. Klepetko, and I. M. Lang
Atrial septal defect repair after a 10-month treatment with bosentan in a patient with severe pulmonary arterial hypertension: a case report.
J. Thorac. Cardiovasc. Surg.,
March 1, 2009;
137(3):
760 - 761.
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. S. Sachweh, S. H. Daebritz, B. Hermanns, B. Fausten, S. Jockenhoevel, S. Handt, and B. J. Messmer
Hypertensive Pulmonary Vascular Disease in Adults with Secundum or Sinus Venosus Atrial Septal Defect
Ann. Thorac. Surg.,
January 1, 2006;
81(1):
207 - 213.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Yamaki, M. Kumate, S. Yonesaka, K. Maeda, M. Endo, and K. Tabayashi
Lung Biopsy Diagnosis of Operative Indication in Secundum Atrial Septal Defect With Severe Pulmonary Vascular Disease
Chest,
October 1, 2004;
126(4):
1042 - 1047.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
