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. Patients with Eisenmenger syndrome have a more favorable prognosis (3-year survival rates > 75%). Treatment consists of pulmonary vasodilator therapy, long-term oxygen therapy, anticoagulation, and lung transplantation or, at times, heart-lung transplantation.- 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.
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 posphodi-esterase-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 X 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.
Figure 1. Fenestrated device with two holes 6 mm in diameter in the central region of an Amplatzer septal occluder (AGA Medical, Golden Valley, MN; and Drabo; Cologne, Germany).
Figure 2. Cine recording showing correct position of the fenestrated Amplatzer device.
Figure 3. Transesophageal echocardiographic image demonstrating correct position of the fenestrated Amplatzer device.
Figure 4. Transesophageal echocardiographic image indicating patency of the device: right-to-left shunt through the stent holes. RA = right atrium; LA = left atrium.