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Ann Thorac Surg 2001;72:906-907
© 2001 The Society of Thoracic Surgeons
a Pulmonary Hypertension Center, Division of Pediatric Cardiology, New York Presbyterian Medical Center, 3959 Broadway, BHN-2, New York, NY 10032, USA
e-mail: rjb3{at}columbia.edu
Yamauchi and colleagues report "successful" repair of an atrial septal defect in a 35-year-old female patient with an atrial septal defect and pulmonary vascular disease, who apparently improved postoperatively clinically as well as hemodynamically from the standpoint of her pulmonary arterial hypertension. Although I would agree with the authors that patients who have pulmonary vascular disease associated with congential heart defects should be repaired if they are "operable," the criteria for "operability," specifically with respect to complete repair versus leaving a residual defect, such as a small "atrial septal defect" remains unclear. Our experience has been that many patients with severe pulmonary hypertension associated with atrial septal defects, such as the patient that the authors report, do significantly improve clinically for a number of years after closure of the atrial septal defect, although more often than not, they subsequently clinically deteriorate, eg, approximately 5 years after surgical repair, although we have seen clinical deterioration earlier than 5 years as well as later following repair. In addition, although decreasing the "sheer stress" by decreasing the pulmonary artery pressure due to a reduction in the pulmonary blood flow may be efficacious in slow progression of the pulmonary vascular disease, this remains to be established. Despite this patient’s pulmonary artery pressure decreasing postoperatively, her pulmonary vascular resistance did not significantly improve, although this should not be interpreted as a contraindication to consideration of surgical repair in patients such as this woman.
Based on our experience with similar patients, we recommend "aggressive" medical therapy for a patient and if on chronic vasodilator therapy, including continuous intravenous prostacyclin, we demonstrate a significant left to right shunt with no right to left shunt at rest, as well as, no right to left shunt with exercise, we partially close the defect leaving a small residual "atrial septal defect" as a "pop-off" safety valve for the patient. As discussed by Rosenzweig and colleagues (Reference 5 in the case report by Yamauchi and colleagues), we treated a young girl who had a large atrial septal defect and was "inoperable" from a pulmonary vascular disease standpoint with chronic intravenous prostacyclin, which increased her QP:QS (pulmonary blood flow:systemic blood flow) from 2.8:1 to 4.1:1 and subsequently we partially closed her atrial septal defect. At the time of repair, lung biopsy was remarkable for demonstrating plexogenic arteriopathy. The child clinically and hemodynamically improved on chronic intravenous prostacyclin, and postoperatively continued on chronic intravenous prostacyclin with further documented clinical and hemodynamic improvement. Obviously, this is only one child and our recommendation is to continue to carefully evaluate these patients, which hopefully in the not too distant future, will give us additional information on a larger number of patients to allow us to make recommendations for these patients.
We agree with the authors that patients considered "inoperable" years ago, may now be "operable," but despite this, the goal of long-term therapy, whether medical or surgical, is to improve the long term natural history of pulmonary vascular disease by improving overall quality of life as well as survival. We are optimistic that with novel therapeutic modalities currently being investigated, we will be able to further our understanding of the pathogenesis and pathophysiology of pulmonary vascular disease associated with congenital heart defects, and ultimately improve the long-term outlook for these patients. A word of caution is needed with respect to pulmonary hypertensive patients who have associated congenital heart defects, eg, these patients most likely represent a very heterogeneous group of patients and their responses to therapeutic interventions may be extremely variable. Whether this is based on some of these patients in fact having "primary pulmonary hypertension" with associated congenital heart defects versus "the classic Eisenmenger Syndrome" awaits further study. We anticipate that genetic studies currently being carried out will give us additional insight into these patients.
In conclusion, our experience suggests that long term vasodilator therapy may play an important role in the treatment of patients with pulmonary hypertension and associated congenital heart disease, just as it does for primary pulmonary hypertension patients, although the biologic variability, as well as, variability in the etiology of the pulmonary vascular disease with these patients, requires critical investigation to determine why some patients markedly improve with chronic vasodilator therapy and other patients do not.
Related Article
Ann. Thorac. Surg. 2001 72: 905-906.
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