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.
