Successful palliation of primary pulmonary hypertension by atrial septostomy

doi:10.1016/0002-9149(90)91016-Y

The American Journal of Cardiology

Volume 65, Issue 15, 15 April 1990, Pages 1045-1046

https://doi.org/10.1016/0002-9149(90)91016-Y Get rights and content

Abstract

Primary pulmonary hypertension is an idiopathic disease characterized by a progressive increase in pulmonary vascular resistance with consequent pulmonary hypertension, right ventricular failure and death.¹ Several series have suggested that the findings of right ventricular failure manifested by high right ventricular filling pressures and decreased cardiac output imply a poor prognosis with death in less than a year.^2,3 We describe a patient who had rapidly progressive primary pulmonary hypertension and hemodynamic parameters suggestive of a poor prognosis who underwent successful atrial septostomy with marked clinical improvement.

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Cited by (52)

Potts shunt as an effective palliation for patients with end stage pulmonary arterial hypertension
2021, Indian Heart Journal
Citation Excerpt :
Unlike atrial septostomy, Potts shunt does not create arterial desaturation in the upper part of the body including cerebral and coronary circulation and the shunt remains open throughout the cardiac cycle.18 Improvement in functional class, reduction in need for PAH specific medications and improvement in right ventricular function has been demonstrated after creation of the Potts shunt.19–21 Timing of the Potts shunt, as well as preoperative stabilization, is critical for successful outcome.
Potts shunt has been suggested as an effective palliative therapy for patients with pulmonary artery hypertension (PAH) not associated with congenital heart disease.
This is a prospective single-center study performed to assess outcomes of Potts shunt in patients with PAH who are in functional class III or IV.
52 patients in functional class III/IV with pulmonary arterial hypertension without significant intra or extracardiac shunt on maximal medical therapy were evaluated and counseled for undergoing Potts shunt/patent ductus arteriosus (PDA) stenting. 16/52 patients (13 females) consented for the procedure; 14 patients underwent surgical creation of Potts, and 2 underwent transcatheter stenting of PDA, which physiologically acted like a Potts shunt. Standard medical therapy was continued in patients who did not consent for the procedure. 12/16 patients survived the procedure. Patients who did not survive the procedure were older, with severe right ventricular systolic dysfunction, and functional class IV. Patients who survived the procedure were followed up in the pulmonary hypertension clinic. The Median follow-up was 17 months (1–40 months). 11/13 patients discharged after the operation showed sustained clinical, echocardiographic, and biochemical improvement, which reduced need for pulmonary vasodilator therapy in 10/13 patients. There was one death in the follow-up period 16 months post-surgery due to lower respiratory tract infection.
Potts shunt is feasible in patients with PAH without significant intra or extracardiac shunts. It can be done safely with an acceptable success rate. Patient selection, preoperative stabilization, and meticulous postoperative management are essential. It should be performed at the earliest sign of clinical, echocardiographic, or laboratory deterioation for optimal outcomes.
Long-term follow-up is required to see a sustained improvement in functional class and the need for a lung transplant in the future.
Hypoxemia and PFO
2019, Patent Foramen Ovale Closure for Stroke, Myocardial Infarction, Peripheral Embolism, Migraine, and Hypoxemia
Although the association between patent foramen ovale (PFO) and stroke has been well established, PFO-mediated hypoxemia remains underrecognized and undertreated. PFO as a culprit of hypoxemia has been described in numerous observational studies, with a complete absence of published randomized trials evaluating the efficacy and safety of percutaneous PFO closure for treating pulmonary and nonpulmonary hypoxemic conditions that are attributed to right-to-left shunting (i.e., sleep apnea, platypnea-orthodeoxia, chronic obstructive pulmonary disease, pulmonary hypertension, pulmonary arteriovenous malformation, high-altitude pulmonary edema, and exercise desaturation). PFO-mediated hypoxemia occurs when deoxygenated venous blood enters and mixes with oxygenated systemic blood. This may result in profound hypoxemia out of proportion to underlying primary lung disease, even with normal right-sided cardiac pressures. Intracardiac right-to-left shunting can exacerbate underlying hypoxemic pulmonary disorders; in a subset of these patients, percutaneous PFO closure may improve symptoms and hypoxemia. This chapter will discuss the clinical association of PFO-mediated right-to-left shunting with hypoxemic medical conditions, and highlight the role of percutaneous device closure in treating the hypoxemia.
Interventional Therapies in Pulmonary Hypertension
2018, Revista Espanola de Cardiologia
A pesar de los avances en el tratamiento farmacológico de la hipertensión pulmonar, en particular de la hipertensión arterial pulmonar (HAP), sigue siendo una enfermedad mortal. La insuficiencia ventricular derecha (IVD) debida a HAP refractaria a tratamiento finalmente se produce y permanece como una causa importante de muerte en estos pacientes. Disminuir la impedancia pulmonar con diferentes fármacos específicos para HAP es el objetivo terapéutico obvio en la IVD secundaria a una poscarga crónicamente aumentada. Sin embargo, se puede esperar una ganancia clínica potencial a partir de los intentos para descargar el corazón derecho y aumentar el gasto cardiaco. La septostomía auricular, la anastomosis de Potts y la denervación de la arteria pulmonar son procedimientos intervencionistas que sirven para este propósito. La angioplastia pulmonar percutánea con balón, otra terapia intervencionista, ha resurgido en los últimos años como una alternativa clara para el tratamiento de pacientes con hipertensión pulmonar tromboembólica crónica distal no operable. En esta revisión se presentarán los antecedentes fisiológicos, la evidencia experimental y los posibles beneficios clínicos y hemodinámicos de todas estas terapias de intervención con respecto a su uso en el contexto de la IVD secundaria a hipertensión pulmonar grave.
Despite advances in drug therapy, pulmonary hypertension—particularly arterial hypertension (PAH)—remains a fatal disease. Untreatable right heart failure (RHF) from PAH eventually ensues and remains a significant cause of death in these patients. Lowering pulmonary input impedance with different PAH-specific drugs is the obvious therapeutic target in RHF due to chronically increased afterload. However, potential clinical gain can also be expected from attempts to unload the right heart and increase systemic output. Atrial septostomy, Potts anastomosis, and pulmonary artery denervation are interventional procedures serving this purpose. Percutaneous balloon pulmonary angioplasty, another interventional therapy, has re-emerged in the last few years as a clear alternative for the management of patients with distal, inoperable, chronic thromboembolic pulmonary hypertension. The current review discusses the physiological background, experimental evidence, and potential clinical and hemodynamic benefits of all these interventional therapies regarding their use in the setting of RHF due to severe pulmonary hypertension.
Patent Foramen Ovale Closure for Hypoxemia
2017, Interventional Cardiology Clinics
Patent foramen ovale in idiopathic pulmonary arterial hypertension: Long-term risk and morbidity
2016, Respiratory Medicine
Citation Excerpt :
However, Nootens et al. in their study of 58 patients with PH of varied etiologies, found PFO had no correlation with any hemodynamic parameter as measured by RHC during rest [22]. As seen in patients with atrial septostomy, one may expect decreased right-sided pressures due to shunting at the expense of worsened hypoxemia, as well as improved cardiac output [14,16]. Our study supports correlation of PFO with greater severity of hypoxemia, but did not find improved right-sided pressures in comparison to non-PFO patients, indeed right-sided pressures appeared to be greater.
Little is known about the presence of patent foramen ovale in idiopathic pulmonary arterial hypertension. While there is suspected worsening of hypoxemia confounding assessment and management of pulmonary hypertension, as well as possible increased morbidity from paradoxical emboli, there may be theoretical relief of worsening right-sided pressures by the same mechanism of right-to-left shunting.
Retrospective review of consecutive patients diagnosed with idiopathic pulmonary arterial hypertension (WHO Group 1) via right heart catheterization, from 1998 to 2010. All patients also underwent a four chamber transthoracic echocardiogram with agitated saline contrast for the evaluation of patent foramen ovale. Primary clinical data was collected and compared between patients with and without patent foramen ovale along with univariable and multivariable predictors of long term survival.
One hundred and fifty five patients were included in the study, 42 with patent foramen ovale (27%). Patients with patent foramen ovale were younger at pulmonary arterial hypertension diagnosis and trended towards higher right ventricular systolic pressures on echocardiography and mean pulmonary arterial pressures by right heart catheterization. Predictors of mortality included age, diffusing capacity for carbon monoxide, and severe hypoxemia. Only diffusing capacity and age were predictive of mortality after adjustment for a priori covariables.
Patent foramen ovale is seen in a quarter of patients with idiopathic pulmonary arterial hypertension and associated with increased prevalence of severe hypoxemia but had no effect on long term survival.
Interventional and surgical therapeutic strategies for pulmonary arterial hypertension: Beyond palliative treatments
2015, Journal of Cardiology
Citation Excerpt :
Interestingly, despite all these limitations, the experience with AS has increased in the past few years. In the most recent review of the worldwide experience [22], 372 procedures performed in 324 patients were identified; 304 have been reported in case series [15–17,23–43], and 20 as case reports [18,19,44–61] (Table 1). AS has largely been performed in young people, mostly women (∼70%), and most of them with IPAH in functional classes III and IV (∼77%).
Despite significant advances in pharmacological treatments, pulmonary arterial hypertension remains an incurable disease with an unreasonably high morbidity and mortality. Although specific pharmacotherapies have shifted the survival curves of patients and improved exercise endurance as well as quality of life, it is also true that these pharmacological interventions are not always accessible (particularly in developing countries) and, perhaps most importantly, not all patients respond similarly to these drugs. Furthermore, many patients will continue to deteriorate and will eventually require an additional, non-pharmacological, intervention. In this review we analyze the role of atrial septostomy and Potts anastomosis in the management of patients with pulmonary arterial hypertension, we summarize the current worldwide clinical experience (case reports and case series), and discuss why these interventional/surgical strategies might have a therapeutic role beyond that of a “bridge” to transplantation.

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Case reportSuccessful palliation of primary pulmonary hypertension by atrial septostomy

Abstract

Am J Med

Am J Cardiol

Case report
Successful palliation of primary pulmonary hypertension by atrial septostomy