Clinical Investigation
Pediatrics
Atrial septostomy improves survival in select patients with pulmonary hypertension

https://doi.org/10.1016/j.ahj.2007.02.019Get rights and content

Background

Atrial septostomy is a palliative treatment for patients with pulmonary hypertension (PHTN) refractory to vasodilator therapy. Limited data exist in the pediatric population and in patients with repaired congenital heart defects.

Methods

We performed a retrospective analysis of hemodynamic and symptomatic changes in patients with PHTN who underwent an atrial septostomy at our institution.

Results

Forty-six atrial septostomies were performed on 43 patients. Patient ages ranged from 0.3 to 30 years (median 12.5). Of 43 patients, 29 had primary PHTN, 10 had PHTN associated with repaired congenital heart defects, and 4 had other secondary causes of PHTN. Mean baseline pulmonary vascular resistance was 35 ± 17 Wood units, and mean pulmonary artery pressure was 74 ± 19 mm Hg. Patients surviving ≥30 days had immediate improvement in cardiac index (from 2.3 to 2.9 L · min−1 · m−2, P < .0001), right atrial pressure (RAp) (from 9.9 to 8.3 mm Hg, P < .05), and oxygen delivery (from 424 to 491 mL O2 per minute, P < .01), with a decrease in systemic oxygen saturation (from 93% to 86%, P < .001). Pulmonary artery pressure was unchanged (P = .3). New York Heart Association class and symptoms of syncope improved (P < .01). Event-free survival at 1, 2, and 3 years was 84%, 77%, and 69%, respectively. Using the National Institutes of Health Registry model, predicted survival probability significantly improved (P < .001). Ten patients (22%) died within 30 days of catheterization. Mortality was associated with preceding decompensations in the intensive care unit (6/10, P < .001) and a higher RAp (21.4 vs 9.8 mm Hg, P < .001).

Conclusions

Atrial septostomy provides symptomatic and hemodynamic improvement in cardiac index and RAp. Risk of septostomy is increased in patients with preceding decompensation or a RAp >18 mm Hg.

Section snippets

Patients

Approval from the institutional review board of the Baylor College of Medicine, Houston, TX, was obtained. A retrospective analysis of catheterization data was performed for all patients who underwent atrial septostomy for the palliation of either primary or secondary PHTN at the Texas Children's Hospital, Houston, TX, from 1981 to 2005. Patients were offered atrial septostomy if they had syncope or NYHA Class III or IV symptoms despite maximum medical therapy available at that time.

Procedure

After a

Baseline characteristics

There were 43 patients who underwent atrial septostomy as summarized in Table I. Twenty-nine patients in this cohort had primary PHTN, and 10 patients had previously undergone corrective surgery for congenital heart disease. Two patients had an associated rheumatologic condition (juvenile rheumatoid arthritis and sarcoidosis). Two siblings presented shortly after birth with primary PHTN of the newborn, diagnosed later at autopsy with alveolar-capillary dysplasia, although the diagnosis was

Discussion

Atrial septostomy is a palliative option for patients with PHTN with syncope or worsening heart failure symptomatology, refractory to vasodilator therapy. Since the earliest report of septostomy as a palliative therapy for PHTN >20 years ago, the number of published reports has been small. All published studies on this procedure have been retrospective analyses, limited by insufficient numbers and short duration of follow-up.

In this study, we describe the largest and youngest series of patients

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