Clinical InvestigationPediatricsAtrial septostomy improves survival in select patients with pulmonary hypertension
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
References (29)
- et al.
Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation
Chest
(1987) - et al.
Experimental studies of the surgical treatment of primary pulmonary hypertension
J Thorac Cardiovasc Surg
(1964) - et al.
Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension
Am J Cardiol
(1983) - et al.
Usefulness of atrial septostomy as a treatment for primary pulmonary hypertension and guidelines for its application
Am J Cardiol
(1997) - et al.
Graded balloon atrial septostomy in severe primary pulmonary hypertension
J Am Coll Cardiol
(1998) - et al.
Hemodynamic data and survival in children with pulmonary hypertension
J Am Coll Cardiol
(1997) - et al.
New predictors of outcome in idiopathic pulmonary arterial hypertension
Am J Cardiol
(2005) - et al.
Atrial septostomy for pulmonary hypertension
Clin Chest Med
(2001) - et al.
Survival in patients with primary pulmonary hypertension. Results from a national prospective registry
Ann Intern Med
(1991) - et al.
Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin
Ann Intern Med
(1994)
Outcomes in children with idiopathic pulmonary arterial hypertension
Circulation
The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension
N Engl J Med
Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion
J Am Coll Cardiol
Survival in primary pulmonary hypertension. The impact of epoprostenol therapy
Circulation
Cited by (84)
The Evolution of Pulmonary Artery Denervation for Treatment of Pulmonary Arterial Hypertension
2023, Interventional Cardiology ClinicsPerioperative Decision-Making in Pulmonary Hypertension
2023, Heart Lung and CirculationOutcomes of atrial septostomy and effect on long-term survival in patients with idiopathic pulmonary arterial hypertension: A single-center cohort
2023, International Journal of CardiologyCitation Excerpt :As these procedures are complex and associated with high risk, including substantial procedure-related mortality, they may only be considered in centres with experience in the techniques [3,14]. Since the first report of septostomy in PAH 30 years ago [15], BAS is used to alleviate RHF symptoms and/or syncope [7,16]. Despite the use and purported benefits of BAS [17,18], it remains a palliative and invasive therapy.
Pulmonary artery denervation for pulmonary arterial hypertension
2021, Trends in Cardiovascular MedicineCitation Excerpt :Exercise-based rehabilitation programs are safe and effective in PH, and can result in clinically relevant improvements in exercise capacity [9]. Invasive treatment strategies include percutaneous or surgical creation of pulmonary-to-systemic shunts aimed at offloading the RV and enhancing cardiac output (atrial septostomy, or a Potts shunt between the descending aorta and left PA) [10–12], and balloon pulmonary angioplasty for patients with CTEPH [13,14]. Various forms of endovascular autonomic system modification have been proposed in animal models of PH, including sympathetic ganglion blockade and catheter-based renal denervation [15–18].
Interventional Therapies in Pulmonary Hypertension
2018, Revista Espanola de CardiologiaIdiopathic pulmonary hypertension
2018, Diagnosis and Management of Adult Congenital Heart Disease: Third Edition