Affiliations: *From the Departments of Cardiology and Angiology (Drs. Althoff, Knebel, Franke, Baumann, and Borges) and Pulmology and Infectiology (Dr. Witt), Charité Universitätsmedizin Berlin, Berlin, Germany; Pulmonary & Critical Care Medicine Section (Drs. Panda and McArdle), Department of Internal Medicine, Yale University School of Medicine, New Haven, CT; and Department of Cardiology (Dr. Gliech), HUMAINE Klinikum Bad Saarow/Fürstenwalde, Bad Saarow, Germany.,
†These authors contributed equally to this work.
Correspondence to: Till F. Althoff, MD, Department of Cardiology and Angiology, Charité Universitätsmedizin Berlin, Campus Mitte, Charité-Platz 1, 10177 Berlin, Germany; e-mail: email@example.com
Pulmonary arterial hypertension (PAH) is a progressive disease, with right-heart failure being the main cause of death. In patients refractory to conventional drug therapy, atrial septostomy can serve as palliative treatment or as a bridge to transplantation. A 41-year-old woman with a 15-year history of PAH associated with a corrected atrial septal defect presented with severe deterioration of symptoms. Echocardiography confirmed reocclusion of an atrial septal stoma that had been created several months before. After performing a repeat atrial septostomy, we implanted a custom-made atrial septostomy device, an Amplatzer septal occluder that had been fenestrated to serve as a custom-made atrial septostomy device. This resulted in an improvement in cardiac output and a marked symptomatic relief. During the 6-year follow-up, the patient was clinically stable with limited but constant exercise tolerance, under specific medical therapy. Repeated echocardiography confirmed long-term patency of the device.
Pulmonary arterial hypertension (PAH) is characterized by a progressive increase of pulmonary vascular resistance eventually leading to right-heart failure. PAH includes idiopathic PAH and pulmonary hypertension associated with various conditions such as congenital systemic-to-pulmonary shunts. Prognosis to a large extent depends on the underlying etiology. Before the availability of targeted medical therapies, the median survival for patients with idiopathic PAH was 2.8 years.1Patients with Eisenmenger syndrome have a more favorable prognosis (3-year survival rates > 75%).2Treatment consists of pulmonary vasodilator therapy, long-term oxygen therapy, anticoagulation, and lung transplantation or, at times, heart-lung transplantation.3–4 In recent years, percutaneous atrial septostomy has been established as palliative treatment or bridge to transplantation in patients with severe right-heart failure refractory to conventional therapy.5
A now 41-year-old woman with PAH first diagnosed in 1985 has been followed up by us since 1999. In 1988, an operative closure of a secundum atrial septum defect (preoperative hemodynamics: left-to-right shunt, 1.6:1; right atrial pressure, 3 mm Hg) had been performed. At that time, pulmonary artery pressure (PAP) was 90/50 mm Hg with a beginning shunt reversal. In 2000, her symptoms started deteriorating despite long-term oxygen therapy, and she was admitted for recurrent hemoptysis. PAP then was 106/41 mm Hg (mean PAP, 66 mm Hg). The patient rejected the recommended IV therapy with epoprostenol and did not tolerate inhalational prostanoid application. Since she had not shown any acute vasoreactivity during invasive hemodynamic testing, we performed a blade balloon atrial septostomy, which resulted in an augmentened cardiac index and a marked symptomatic relief (neither endothelin antagonists nor posphodiesterase-5 inhibitors were approved for this indication on the German market at that time).
In November 2001, the patient was admitted to the hospital with complaints of severe dyspnea, orthopnea, and orthostatic vertigo. Transthoracic echocardiography with Echovist contrast (Bayer Schering Pharma; Berlin, Germany) revealed no atrial shunt, suggesting a spontaneous closure of the septal stoma. Thus, a repeat atrial septostomy was performed associated with an augmentation of the cardiac index (2.2 to 2.5 L/min/m2). In order to maintain patency and to ensure an adequate shunt area, we ordered a fenestrated septostomy device (Fig 1
). This custom-made device based on an Amplatzer septal occluder has two additional 6-mm holes in the stent area, without Dacron patches (AGA Medical; Golden Valley, MN; and Drabo; Cologne, Germany).
In December 2001, the patient was readmitted for elective implantation of the device. Intraprocedurally, the septal stoma was still patent with a stretched diameter of 9 mm facilitating probing. Subsequently, the device was implanted followed by dilation of the preformed holes using a 6 × 40-mm balloon. Correct position as well as a right-to-left shunt through the stent holes were demonstrated by cine recordings (Fig 2
) and intraprocedural transesophageal echocardiography (Fig 3, 4
). Augmentation of the shunt volume due to the procedure was reflected by a significant fall of aortic oxygen saturation (90.1 to 76.1%), and a further increase of cardiac index (3.1 to 3.6 L/min/m2). Systemic and pulmonary pressures were equalized with a PAP of 104/40 mm Hg (mean, 64 mm Hg).
Symptoms further improved after the procedure and have remained unchanged since that time. Meanwhile, the pulmonary vasodilators bosentan and sildenafil have been initiated. The patient solely complains about intermittent vertigo and dyspnea on exertion (New York Heart Association class III). This is consistent with the results of repeated cardiopulmonary exercise testing with the peak oxygen uptake varying between 13 and 15 mL/kg/min. Furthermore, she suffers from paroxysmal atrial fibrillation and arterial hypertension. During the 5-year follow-up, all transthoracic and transesophageal echocardiographies showed a correct position and patency of the device without thrombotic depositions and persistence of the right-to-left shunt. Systemic and pulmonary pressures are still equalized with a PAP of 113/44 mm Hg (mean PAP, 68 mm Hg). Medical therapy now includes oral anticoagulation (international normalized ratio 2 to 3); bosentan, 125 mg bid; and sildenafil, 20 mg tid.
In patients with PAH, increasing PAP eventually leads to right-sided heart failure and often to sudden cardiac death.1 Available treatments consist of anticoagulation and pulmonary vasodilators such as phosphodiesterase-5 inhibitors, endothelin-receptor antagonists, calcium-channel blockers, or prostanoids, but patients with severe right-heart failure and low cardiac output are often refractory to these agents.4
In recent years, atrial septostomy has emerged as palliative therapy or bridge to transplantation. In severe PAH, this procedure creates a right-to-left shunt reducing right ventricle preload. It has been shown to result in an increased cardiac output and an augmentation of systemic oxygen transport despite the fall in systemic arterial oxygen saturation.5A major drawback of the procedure is the high incidence of spontaneous closure or decrease in orifice size, with repeat septostomy being required in 3 to 17% depending on the performed technique.6 Oversizing of the orifice represents another hazard, leading to severe hypoxia or left ventricular failure due to an excessive shunt volume.5
The rationale to develop a fenestrated septostomy device was to improve long-term patency and to allow for control of the shunt area, the latter bearing the potential to tailor the device to the patient, taking individual hemodynamic and oximetric parameters into account. The only case report7 on a septostomy device based on a septal occluder reported a spontaneous closure after 6 months. To our knowledge, our case represents the only reported implantation of such a device in this setting that has proven successful in long-term follow-up.
The septal occluder on which the device is based has been used in the percutaneous treatment of atrial septum defects for years, thus providing sophisticated design and a great amount of experience regarding implantation procedure and management of complications. We assume that the procedural risk associated with the atrial septostomy device is similar to that associated with the septal occluder and thus justifiable in the setting of severe pulmonary hypertension.
Even though fenestrated devices to be distributed serially are under development, today only custom products are available. Intraprocedural modification of septal occluders has been shown to be a feasible alternative.8 Devices can be prepared during the procedure according to the patient’s individual needs, obviating the time-consuming process of ordering a customized device and the need for an additional catheterization. Cost of the Amplatzer-based devices add up to approximately $5,000. However, these costs are relativized by the high annual costs of the specific medication (> $50,000/yr for bosentan and sildenafil).
We believe that with the advantages mentioned above, implantation of a septostomy device constitutes a strong alternative to conventional balloon atrial septostomy. It has to be questioned though if a statistical proof of superiority is feasible because the limited number of affected patients makes it nearly impossible to create a control group for a prospective randomized trial. Accordingly, we regard a registry-based evaluation as more appropriate. At this point, we do not believe that data are sufficient to support the first-line use of fenestrated devices instead of conventional atrial septostomy. We would rather suggest its application when septostomy has failed in terms of spontaneous closure or oversizing of the orifice. Depending on further data, fenestrated devices might become the standard method.
Abbreviations: PAH = pulmonary arterial hypertension; PAP = pulmonary artery pressure
The authors have no conflicts of interest to disclose.
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