Atrial Septal Defect - Natural History

E-chocardiography Journal: Alphabetical List / Chronological List / Images / Home Page

Indian Heart J. 2005 Jan-Feb;57(1):35-8.
Natural history of secundum atrial septal defect revisited in the era of transcatheter closure.
Saxena A, Divekar A, Soni NR.
Department of Pediatrics, Health Sciences Centre, Winnipeg, Canada.

BACKGROUND: Several reports in the past have described the natural history of atrial septal defects, most dealing with a decrease in size or spontaneous closure of the defect. Some recent articles have also described an increase in size of the defect in a sizable number of cases which may be important in the current era of transcatheter closure. We analyzed the data of 52 consecutive cases diagnosed to have secundum atrial septal defect in the first year of life, seen over the last six years at our center. METHODS AND RESULTS: All infants with a defect size of > or =4 mm on echocardiography were included. The first and the last echocardiographic images with a minimum interval of 6 months were used for analysis. Cases were divided into three groups depending upon the defect diameter (small: 4-5 mm, moderate: 6-8 mm and large: > or =9 mm). The age ranged from one day to 12 months (mean 2.9 +/- 3.2 months). On a follow-up of 0.7 to 7.0 years (mean 2.9 +/- 1.4 years), the septal defect reduced in size in 24 (46%) cases with complete closure in 14 of these. The size remained same in 13 (25%) and enlarged in 15 (29%) cases. The likelihood of closure was highest in small defect group as compared to the large defect group (p < 0.05). Similarly, enlargement was more often seen in large defects. CONCLUSIONS: Small atrial septal defects of 4 mm to 5 mm are very likely to decrease in size or completely close on follow-up. Larger defects, on the other hand may remain large or enlarge further in a significant proportion of cases. A close observation is required for these cases if being considered for transcatheter closure.

Kardiologia Polska 3/2004
streszczenie artykulu:
Ocena zmian w ukladzie krazenia u ciezarnych kobiet z ubytkiem przegrody miedzyprzedsionkowej typu otworu drugiego
Kardiol Pol 2004; 60: 224-228
autorzy: Wojciech Piesiewicz, Aleksander Goch, Zbigniew Binikowski, Urszula Faflik, Grzegorz Krasomski, Jan Goch,

Background: The secundum atrial septal defect (ASD II) is, after bicuspid aortic valve, the second most frequent congenital heart disease. In the majority of females with ASD II the course of pregnancy is uncomplicated, however, pregnancy-related cardiovascular changes may affect haemodynamical parameters of the defect. Aim: To assess cardiovascular changes in pregnant females with ASD II. Methods: The study group consisted of 54 pregnant females with ASD II and 30 control healthy pregnant women. NYHA class, standard ECG, 24-hour ambulatory ECG monitoring and echocardiography were analysed in the second and third trimester of pregnancy as well as during puerperium. Results and conclusions: (1) Due to the relatively small sample of population studied and limitations of the usage of NYHA classification in pregnant women, the relationship between ASD II and the development of heart failure during pregnancy could not be definitely proved. (2) An increase in the right ventricular and right atrial enlargement was significantly greater in pregnant women with ASD II compared with healthy pregnant females. Also indirect parameters of the right ventricular strain (paradoxical systolic movement of the interventricular septum or tricuspid regurgitation) were more frequent in patients with ASD II rather than in controls. These alterations were accompanied by a significant decrease in the mean value of the Qp/Qs index which may suggest pregnancy-related favourable changes in the haemodynamical consequences of the defect - a decrease in the left-to-right shunt. (3) Paroxysmal supraventricular tachycardia was the most frequent cardiac arrhythmia in pregnant women with ASD II.

Rev Esp Cardiol. 2002 Sep;55(9):953-61.
Surgical closure of atrial septal defect before or after the age of 25 years. Comparison with the natural history of unoperated patients
Oliver JM, Gallego P, Gonzalez AE, Benito F, Sanz E, Aroca A, Mesa JM, Sobrino JA.
Unidad de Cardiopatias Congenitas del Adulto, Hospital Universitario La Paz, Madrid, Spain.

INTRODUCTION: Surgical closure of an atrial septal defect (ASD) before the age of 25 years has been demonstrated to reduce complications during adulthood. However, the outcome for patients operated after the age of 25 is still debated. METHODS: In a retrospective study we examined the outcome of early and late surgical repair of ASD in adults, as compared with the natural evolution of unoperated patients. The study population was 280 patients (mean age 40 18 years) with non-restrictive ASD: 102 patients (group 1) underwent surgery before the age of 25 years, 90 patients (group 2) underwent surgery after the age of 25 years, and 88 unoperated patients were older than 25 years at the time of study (group 3). The variables analyzed were left ventricular systolic function, left atrial dimensions, systolic pulmonary pressure, right ventricular dimensions, the degree of mitral and tricuspid regurgitation, and the prevalence of late atrial fibrillation. RESULTS: Left ventricular systolic function and the degree of mitral regurgitation were not statistically different between groups. Compared with the patients in group 2, the patients in group 1 had a significantly lower systolic pulmonary arterial pressure (p < 0.001) and less dilated right ventricle (p < 0.001) and left atrium (p < 0.001). The degree of tricuspid regurgitation (p < 0.001) and prevalence of atrial fibrillation (p < 0.001) were significantly higher in the patients of group 2. Compared with group 3, the patients in group 2 had a significantly lower systolic pulmonary arterial pressure (p < 0.001) and less dilated right ventricle (p < 0.001). However, the left atrial dimensions, degree of tricuspid regurgitation, and prevalence of atrial fibrillation did not differ in a statistically significant way between the two groups. CONCLUSIONS: Surgical repair of an atrial septal defect in patients over 25 years of age does not fully prevent hemodynamic deterioration and the development of atrial arrhythmias. Therefore it seems that the surgical closure of ASD before adulthood should be strongly recommended.

Heart. 2002 Mar;87(3):256-9.
Natural history of growth of secundum atrial septal defects and implications for transcatheter closure.
McMahon CJ, Feltes TF, Fraley JK, Bricker JT, Grifka RG, Tortoriello TA, Blake R, Bezold LI.
The LillieFrank Abercrombie Division of Pediatric Cardiology, Texas Children's Hospital and Baylor College of Medicine, Houston, 6621 Fannin, Texas 77030, USA.

OBJECTIVE: To investigate the natural history of secundum atrial septal defects (ASDs) over several years using serial echocardiographic studies. METHODS: All patients with isolated secundum ASDs who had serial transthoracic echocardiograms at Texas Children's Hospital, Houston, Texas, from January 1991 to December 1998 were identified. Patients with fenestrated or multiple ASDs, other congenital heart defects, or less than a six month interval between echocardiograms were excluded. There were 104 patients eligible for inclusion in the study. Studies were reviewed by two echocardiographers (blinded) and the maximal diameter was recorded. Defects were defined as small (> 3 mm to < 6 mm), moderate (> or = 6 mm to < 12 mm), or large (> or = 12 mm). ASDs that grew > or = 20 mm were defined as having outgrown transcatheter closure with the device available to the authors' institution. RESULTS: ASD diameter increased in 68 of 104 patients (65%), including 31 patients (30%) with a > 50% increase in diameter. Spontaneous closure occurred in four patients (4%). Thirteen defects (12%) increased to > or = 20 mm. One fifth of the patients studied had an insufficient atrial rim by transthoracic echocardiogram to hold an atrial septal occluder. The only factor associated with significant growth of ASDs was initial size of the defect. ASD growth was independent both of age at diagnosis and when indexed to body surface area. CONCLUSIONS: Two thirds of secundum ASDs may enlarge with time and there is the potential for secundum ASDs to outgrow transcatheter closure with specific devices. Further development in devices and general availability of devices capable of closing larger ASDs should circumvent this problem.

G Ital Cardiol. 1999 Nov;29(11):1302-7.
Should the patient with an interatrial defect recognized in adulthood always be operated on?
Favilli S, Zuppiroli A, Mori F, Santoro G, Manetti A, Dolara A.
U.O. Cardiologia 2, Azienda Ospedaliera Careggi, Firenze.

BACKGROUND: Atrial septal defect (ASD) can be recognized in adult age, mostly in asymptomatic or scarcely symptomatic patients. These patients differ from patients in "historical" clinical series, in whom diagnosis was done on the basis of clinical evidence, and their natural history is probably different. AIM OF THE STUDY: Our aim was to verify retrospectively results of surgery versus medical follow-up in an adult population with ASD with age at first diagnosis > or = 30 years. PATIENTS AND METHODS: Seventy-two patients with ASD, 52 females (72%), observed at our Institution since 1978, were considered. Mean age at diagnosis was 48 +/- 12 years (range 30-79); 36 patients (50%, group A) are still on medical therapy, 36 patients (group B) were operated. As groups A and B did not differ significantly in any demographic, clinical or echocardiographic parameter, they were compared for the incidence of complications. RESULTS: During follow-up (100 +/- 70 months, range 12-240), the incidence of major clinical events showed no significant differences in the two groups, as cardiac death or cardiovascular complications (cerebral ischemic events, severe mitral insufficiency, reoperation) occurred in 4 patients in group A (11%) and in 4 patients in group B (11%). Worsening of NYHA class was observed in 3 patients from group A (8%) and 2 patients from group B (5.5%; p = ns). New onset of supraventricular arrhythmias occurred more frequently in group B (14 patients, 39%) than in group A (5 patients, 14%) (p = 0.01; OR = 3.9; CI 95%: 1.2-12.6). CONCLUSIONS: In an adult population affected with asymptomatic or mildly symptomatic ASD and age at first diagnosis > or = 30 years, surgical closure of the defect did not modify morbidity and mortality at a mid-term follow-up. We suggest that, mostly in older asymptomatic patients, surgery should not be a routine choice and clinical decision-making should be individualized in each case.

Heart 1999;81:271-275 ( March )
Outcome of pregnancy in women with congenital shunt lesions M Zuber, N Gautschi, E Oechslin, V Widmer, W Kiowski, R Jenni Division of Echocardiography, University Hospital, Raemistrasse 100, CH-8091 Zurich, Switzerland

OBJECTIVE: To evaluate the outcome of pregnancy in women with congenital shunt lesions. SETTING: Retrospective study in a tertiary care centre. METHODS: Pregnancy history was obtained by a standardised questionnaire and medical records were reviewed. PATIENTS: 175 women were identified, at a mean (SD) age of 42 (14) years. Pregnancies occurred in 126 women: 50 with an atrial septal defect, 22 with a ventricular septal defect, 22 with an atrioventricular septal defect, 19 with tetralogy of Fallot, and 13 with other complex shunt lesions. RESULTS: 309 pregnancies were reported by 126 woman (2.5 (1.6) pregnancies per woman). The shortening fraction of the systemic ventricle was 40 (8)%, and 98% were in New York Heart Association class I-II at last follow up. Spontaneous abortions occurred in 17% of pregnancies (abortion rate, 0.4 (0.9) per woman). Gestational age of the 241 newborn infants was 8.8 (0.8) months. There were no maternal deaths related to pregnancy. Pre-eclampsia and embolic events were observed in 1.3% and 0.6%, respectively of all pregnancies. Women with complex shunt lesions more often underwent caesarean section (70% v 15-30%, p = 0.005) and gave birth to smaller babies for equivalent gestation (2577 (671) g v 3016 (572) to 3207 (610) g, p < 0.05). The recurrence risk of congenital heart disease was 2.5%. CONCLUSIONSThe outcome of pregnancy is favourable in women with congenital shunt lesions if their functional class and their systolic ventricular function are good. Such patients can be reassured.

Br Heart J. 1994 Mar;71(3):224-7; discussion 228.
Comment in: Br Heart J. 1994 Mar;71(3):219-23.
Natural history of secundum atrial septal defect in adults after medical or surgical treatment: a historical prospective study.
Shah D, Azhar M, Oakley CM, Cleland JG, Nihoyannopoulos P.
Department of Medicine (Division of Cardiology), Hammersmith Hospital, London.

OBJECTIVE--To compare outcome in patients with medically treated secundum atrial septal defect (ASD) first diagnosed after the age of 25 with the long-term outcome in a similar group of patients after surgical closure. DESIGN--A historical, prospective, unrandomised study. SETTING--A tertiary referral centre. PATIENTS--All patients with ASD followed up since 1955 who fulfilled the entry criteria and had reached a current age of over 45 years--that is, 34 medical and 48 surgical patients with a mean follow up of 25 years. MAIN OUTCOME MEASURES--Survival, symptoms, and complications. RESULTS--There was no difference in survival or symptoms between the two groups and no difference in the incidence of new arrhythmias, stroke or other embolic phenomena, or cardiac failure. No patient in either group developed progressive pulmonary vascular disease. CONCLUSION--Outcome in adults with ASD was not improved by surgical closure. Because progressive pulmonary vascular disease did not develop in any of these patients its prevention is not a reason for advising closure of ASD in adults.

Jpn Circ J. 1991 Aug;55(8):791-3.
Natural histories of atrial septal defect with pulmonary hypertension, and ventricular septal defect with pulmonary hypertension.
Hashimoto A, Momma K, Hayakawa H, Hosoda S.
Department of Cardiovascular Surgery, Tokyo Women's Medical College, Japan.

A study of the natural history of 51 adult patients with atrial septal defect with pulmonary hypertension (ASD + PH) was performed. ASD + PH of less than 14 Um2 of pulmonary artery resistance (PVR) was considered an indication for surgery. The prognosis of surgically treated patients was favorable, but that of medically treated patients was poor. For patients with ventricular septal defect with pulmonary hypertension (VSD + PH), surgery was considered for pulmonary-systemic vascular resistance ratio (Rp/Rs) less than 0.5, and for patients under than 10 years and, ideally, under 2 years of age.

N Engl J Med. 1990 Dec 13;323(24):1645-50.
Comment in: N Engl J Med. 1990 Dec 13;323(24):1698-700.
N Engl J Med. 1991 May 2;324(18):1286-7.
Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years.
Murphy JG, Gersh BJ, McGoon MD, Mair DD, Porter CJ, Ilstrup DM, McGoon DC, Puga FJ, Kirklin JW, Danielson GK.
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, MN 55905.

BACKGROUND. Atrial septal defects have been surgically correctable for more than 30 years. The long-term survival rates among patients treated in the early era of cardiac surgery are poorly documented, but such data are of critical importance to the future medical care, employability, and insurability of these patients. METHODS. To determine the natural history of surgically corrected atrial septal defects, we studied all 123 patients who underwent repair of an isolated defect (ostium secundum or sinus venosus) at the Mayo Clinic between 1956 and 1960, 27 to 32 years after the procedure. The follow-up status of all patients was determined by written questionnaires and telephone interviews. Hospital records and death certificates were obtained if interim hospitalization or death had occurred. RESULTS. The overall 30-year actuarial survival rate among survivors of the perioperative period was 74 percent, as compared with 85 percent among controls matched for age and sex. The perioperative mortality was 3.3 percent (four deaths). Actuarial 27-year survival rates among patients in the younger two quartiles according to age at operation (less than or equal to 11 years and 12 to 24 years) were no different from rates among controls--97 percent and 93 percent, respectively. In the two older quartiles (25 to 41 years and greater than 41 years), 27-year survival rates were significantly less (P less than 0.001)--84 percent and 40 percent, respectively--than in controls (91 and 59 percent). Independent predictors of long-term survival according to multivariate analysis were age at operation (P less than 0.0001) and systolic pressure in the main pulmonary artery before operation (P less than 0.0027). When repair was performed in older patients, late cardiac failure, stroke, and atrial fibrillation were significantly more frequent. CONCLUSIONS. Among patients with surgically repaired atrial septal defects, those operated on before the age of 25 have an excellent prognosis, but older patients require careful, regular supervision.

Jpn Circ J. 1981 Feb;45(2):249-59.
Effects of corrective surgery on natural history of atrial septal defect of secundum type.
Tsuchioka H, Iyomasa Y, Kakihara R, Takeuchi E, Suenaga Y, Kitoh S, Watanabe T.

Two hundred and seventy-eight patients with atrial septal defect of secundum type were operated on surgical closure of the defect. The patients were divided into 5 groups according to age at surgery, and preoperative complaints and laboratory findings were analyzed to evaluate natural history. As year passed, incidence of complaints increased. Cardiothoracic ratio (CTR), systolic pressure of the right ventricle (PRVS), end-diastolic pressure of the right ventricle (RVEDP), mean pressure of the right atrium (PRAm), ratio of pulmonary to systemic systolic pressure (PPA/PS), right ventricular dimension (RVD) and left atrial dimension (LAD) increased significantly, while frontal axis of the QRS complex, RV1, SV1 +RV5 and LVD decreased. No chronological changes were seen in ratio of pulmonary to systemic blood flow (QPA/QS) and ratio of pulmonary to systemic vascular resistance (RPA/RS). The first and second decades of life were considered to be stable stages of the disease. Postoperatively, changes in the above-mentioned parameters were compared in each age group, and effects of surgical repair on natural history were evaluated. Of 265 survivors, 168 were followed-up for more than one year, the longest period being 22 years. CTR, frontal axis, RV1, PRVS, RVEDP, PPA/PS and RVD decreased significantly, while SV1 + RV5, RPA/RS and LVD increased. No changes were seen in LAD. Surgical effects appeared most significantly in the first decade and least in the fifth or more. From these findings it would be concluded that corrective surgery should be best carried out in the first decade, at latest in the second, though not to be contraindicated by age alone. Otherwise, postoperative improvement of parameters delays or hemodynamical abnormalities may persist regardless of considerable clinical improvement.

Cardiology. 1978;63(2):73-8.
Natural history of atrial septal defect of secundum type in the middle-aged. Medical versus surgical therapy.
Forfang K.

10 middle-aged patients with atrial septal defect of the secundum type (ASD II) were referred to surgery, but for several noncardiological reasons the operation was not performed. 5 patients were reexamined after an interval from 6 1/2 to 15 years. During the follow-up period only relatively minor changes in pulmonary arteriolar resistance had occurred and all patients were still acceptable candidates for surgical treatment. The other 5 patients died after a period of time ranging from 2 weeks to 16 1/2 years from conditions directly or indirectly related to the cardiac malformation. During the same period of time, 84 ASD II patients underwent surgery with a mortality rate of 8.3% after 74 months, including 2 hospital deaths. These results suggest that middle-aged patients with ASD II without obstructive pulmonary hypertension should be operated on without delay, but that in a significant number of cases, long-term conservative treatment is not associated with an increase in pulmonary arteriolar resistance.

Am Heart J. 1976 Sep;92(3):302-7.
The natural history of small atrial septal defects; long-term follow-up with serial heart catheterizations.
Andersen M, Moller I, Lyngborg K, Wennevold A.

Thirty-nine patients with a small ASD of the secundum type were followed clinically for 5 to 21 years (mean 11.6 years); no evidence of deterioration was found. In 26 of these cases recatheterization was carried out with a mean follow-up period of 9.8 years. No significant changes were found in most patients; in four patients, however, the left-to-right shunt had increased significantly. Our recommendations are that we will continue to advise surgery in patients with large ASD's, whereas we still do not recommend surgery in patients with small ASD's; the latter patients should be followed for longer periods to ensure that no deterioration occurs. The decision as to whether an ASD should be regarded as large or small in our opinion not only should be based on a chosen limit of pulmonary-to-systemic flow ratio, but clinical factors such as diastolic flow murmurs, ECG changes, the heart size, and the pulmonary vascular markings should also be taken into consideration.

Circulation. 1968 May;37(5):805-15. Natural history and prognosis of atrial septal defect. Craig RJ, Selzer A.

Back to E-chocardiography Home Page.

The contents and links on this page were last verified on May 16, 2006.