Advanced Therapy in Pulmonary Hypertension: Experience with Specific Shunt Devices and Pump-Less Lung Assist Device (PLAD)

Background: Pulmonary arterial hypertension (PAH) is a severe and rare disease with a devastating prognosis with childhood onset. The distribution of tautologies in pediatric PH differ quite significantly from that of adults, with a more significant predominance of idiopathic arterial hypertension (IPAH), pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), and developmental lung disease. In severe PAH, Syncope is a sign of acute pulmonary hypertension crisis with a dramatic reduction of transpulmonary blood flow, which occurs later than in adults. Even if current treatment options enable a 5-year survival rate of 75%. Further deterioration in these patients is joint and targeted pharmacological treatment options to a maximum are required. In this stage of the disease, the patients suffer from the low-cardiac output. At this stage of the disease, there is evidence to improve clinical symptoms by performing a balloon-atrial-septostomy (BAS) in adults. However, due to the uncontrolled size of the BAS and subsequent acute and severe desaturation, the long-term outcome of BAS is quite limited, especially in patients with elevated right atrial pressure. A defined shunt size might be an option to tackle this burden, so specific shunt devices showed lately convincing data regarding the long-term outcome of adult patients in this specific condition. In our department, two patients could successfully be transferred for bilateral lung transplantation with this device, two patients could tremendously improve their clinical condition, and bilateral lung transplantation could be postponed until now. Furthermore, one patient with end-stage pulmonary hypertension could be successfully transplanted on a pump-less lung assist device after 6 months.

Method: The specific shunt device with a fenestration diameter of 8 or 10 mm is 2019 been authorized in adults with severe and drug-resistant PAH and heart failure. Allowing a controlled interatrial right to left blood flow with decompression of the right ventricle and improvement of the cardiac output without significant systemic desaturation when device size is well chosen for the patient, this fenestrated self-expandable double-disc is developed. Easily to implant with a single attempt and in the absence of significant complications, the specific shunt device might be a safe option. Furthermore, the para-corporal lung assist device is a feasible option as a bridge to transplant instead of ECMO.

Conclusion: Specific shunt devices and the pump-less lung assist device show convincing outcomes in advanced end-stage pulmonary hypertension.

Publication History

Article published online:
28 January 2023