Pathophysiology and Natural History Congenital Heart Disease

We examined the cases of 702 patients found to have isolated atrial septal defect of the secundum or sinus venosus type at catheterization from 1953 to 1978. Forty patients (6%), 34 women and six men, had pulmonary vascular obstructive disease, with a total pulmonary resistance greater than 7 U/M2; of these patients 26 (mean age 47 years) underwent surgical closure and 14 (mean age 44 years) received medical treatment. All patients were followed for at least 4 years, with a median follow-up of 12 years. At the most recent follow-up, 17 of the 40 patients were dead. Of the 22 surgically treated patients with total pulmonary resistance less than 15 U/M2, 19 were alive with significant regression of symptoms. All four surgically treated patients with total pulmonary resistance greater than or equal to 15 U/M2 were dead. Of the five medically treated patients with total pulmonary resistance less than 15 U/M2, four had died, and one was alive with significant progression of symptoms. Of the nine medically treated patients with total pulmonary resistance greater than or equal to 15 U/M2, six had died and the three survivors had progression of symptoms. In the surgically treated group, the following variables correlated with survival: total pulmonary resistance (p < .00001), pulmonary arteriolar resistance (p < .00001), pulmonary-to-systemic resistance ratio ( p = .004), systemic arterial oxygen saturation (p = .005), and pulmonary arterial oxygen saturation (p = .007). In conclusion: (1) Atrial septal defect with high total pulmonary resistance is uncommon and predominates in adult female patients. (2) Total pulmonary resistance (or pulmonary arteriolar resistance) is the best predictor of surgical outcome. In patients with total pulmonary resistance less than 15 U/m2, surgical treatment is advised. (3) In patients with borderline total pulmonary resistance, the systemic arterial oxygen saturation provides a good prediction of surgical outcome. Circulation 76, No. 5, 1037-1042, 1987. PATIENTS with moderate or large congenital ventricular septal defects become symptomatic and develop pulmonary hypertension with an increased pulmonary vascular resistance in childhood. In contrast, patients with an isolated atrial septal defect (of ostium secundum or sinus venosus type) usually do not become symptomatic until middle or old age. A fraction ofthese patients go on to develop pulmonary vascular obstructive disease." 2 Surgical correction, in patients with isolated atrial septal defect, is usually recommended when there is a significant shunt (pulmonary-to-systemic flow ratio of at least 1.5: 1) and no evidence of severe pulmonary vascular obstructive disease.3 Furthermore, in the absence of significant pulmonary vascular obstructive disease, many studies have reported good surgical results even in the elderly.4 It has often been stated that the risks and benefits of surgery depend mainly on the presence of pulmonary hypertension, 2, 5,6 but the point at which surgery should be avoided remains uncertain. No large studies have reported on the long-term follow-up of patients with atrial septal defect and pulmonary vascular obstructive disease and the effect of surgery. This study addressed that question with particular reference to the prediction of outcome after surgical correction. From the Division of Cardiology, Department of Medicine, The Mount Sinai School of Medicine of the City University of New York, New York, and the Mayo Clinic, Rochester, MN. Address for correspondence: Dr. Valentin Fuster, Division of Cardiology, Mount Sinai Medical Center, One Gustave L. Levy P1., New York, NY 10029. Received Feb. 6, 1987; revision accepted July 16, 1987. Methods Study patients. Between 1953 and 1978, 702 patients at the Mayo Clinic were found to have isolated atrial septal defect of the ostium secundum or sinus venosus type at cardiac catheterization Ofthese 702 patients, 40 (6%) had pulmonary vascular obstructive disease, defined as a total pulmonary resistance of Vol. 76, No. 5, November 1987 1037 by gest on A ril 4, 2017 http://ciajournals.org/ D ow nladed from