INTRODUCTION:Idiopathic pulmonary arterial hypertension (IPAH) is a progressive disease with poor prognosis. In patients refractory to conventional drug therapy, atrial septostomy can serve as palliative treatment or as a bridge to transplantation.
CASE PRESENTATION:A now 41-year-old female with IPAH first diagnosed in 1985 has been followed by us since 1999. In 1988 an operative closure of an atrial septal defect had been performed with patch plastic. The systolic PAP was initially around 50 mmHg and had been constant since. In 2000 her symptoms started deteriorating and she was admitted for recurrent hemoptysis. PAP then was 106/41 mmHg (PAPmean 66 mmHg). The patient did not show any vasoresponsiveness to neither the prostanoid Iloprost nor the endothelin antagonist tetsosentan. Therefore a blade balloon atrial septostomy was performed which resulted in an augmentened cardiac index and a marked symptomatic relief. In November 2001 the patient presented with severe dyspnea, orthopnea and orthostatic vertigo. Transthoracic echocardiography did not demonstrate an atrial shunt, suggesting a spontaneous closure of the septostoma. Thus a repeat blade balloon atrial septostomy was performed. In order to maintain patency we ordered a fenestrated septostomy device. This custom made device based on an Amplatzer septal occluder has two additional 6 mm-holes in the stent area, without dacron patches. In December 2001 the patient was readmitted for elective implantation of the device. Intrainterventionally the septostoma proved still patent with a stretched diameter of 9 mm facilitating probing. Subsequently the device was implanted and its correct position demonstrated by intrainterventional transesophageal echocardiography. Eventually dilation of the preformed holes was performed using a 6x 40mm balloon. Augmentation of the shunt volume was reflected by a significant fall of aortic oxygen saturation from 90,1% to 76,1%. Cardiac output increased from 4.8 to 5.5. Systemic and pulmonary pressures were equalized with a PAP of 104/40 (PAPmean 64 mmHg). Symptoms further improved after the procedure and remained constant since. Meanwhile an additional therapy with Bosentan has been initiated since the PDE-5-inhibitor Vardenafil could not prove a significant effect in hemodynamic testing. The patient solely complains about intermittent vertigo and dyspnea on exertion. This is consistant with the results of repeated cardiopulmonary exercise testing with the peak oxygen uptake (VO2max) varying between 13 and 15 ml O2/kg/min. During the 5-year follow-up all transthoracic and transesophageal echocardiographies showed a correct position and good patency of the device without thrombotic depositions and could demonstrate a sufficient right-to-left shunt. Systemic and pulmonary pressures are still equalized with a PAP of 113/44 (PAPmean 68 mmHg). Medical therapy now includes oral anticoagulation and Bosentan 125 mg twice per day.
DISCUSSIONS: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 septal occluder on which the device is based has been used in the percutaneous treatment of atrial septal defects for years, thus providing sophisticated design and a great amount of experience regarding implantation procedure and management of complications.
CONCLUSION:To our knowledge our case represents the only reported implantation of such a device that proved successful in long-term follow-up. Though most reported trials suggest a beneficial effect, the impact of atrial septostomy on long-term survival has not been shown in prospective and controlled studies yet. Furthermore the optimal timing of the procedure as well as the optimal amount of right to left shunt remain to be determined.
DISCLOSURE:Alexander Panda, No Financial Disclosure Information; No Product/Research Disclosure Information