Atrial Flow Regulator (AFRr) -Device as „Bridge to lung-transplant“ in a 9-year-old girl with hereditary BMPR 2 related pulmonary arterial hypertension (HPAH)

Hintergrund Pulmonary arterial hypertension (PAH) is a scarce, devastating disease with a rising number of patients requiring lung transplantation. Due to the rare availability of donors, these children experience deterioration between listing and transplantation or even die before having the chance of transplantation. Therefore, the need for advanced strategies to overcome this burden is essential.

Methoden In PAH low cardiac output is caused by restricted transpulmonary blood flow. Syncope is a severe symptom of pulmonary hypertension due to the acute complete reduction of transpulmonary blood flow. One option besides targeted medical therapy can be the creation of an intra-atrial shunt to decompress the right atrium, enable a right-to-left shunt and thereby sustain cardiac output. Unrestricted transatrial blood flow may be dangerous due to complete desaturation, hence a secured decompression with restricted blood flow is warranted and can be achieved by using a dedicated interatrial shunt device (i.e. AFR device).

Ergebnisse A 7-year-old girl was diagnosed with PAH. She initially presented with syncopy. Cardiac catheterisation showed severe systemic PAH and reduced cardiac output. Therefore, pharmacological treatment was initiated and extended to targeted quadruple therapy, including i.v. prostacyclin. Due to rapid disease progression and further deterioration, she was listed for lung transplantation. As syncopes persisted, an AFR device (8 mm device size, AFR®​ device, Occlutech, Germany) was implanted in 03/2023. There was mild desaturation and improvement of the clinical status without additional syncopes.To stabilize her RV function later on, we started levosimendan i.v. therapy every 4 weeks. After 3 months, she received a successful bilateral lung transplantation.

Schlussfolgerung Our treatment decision was encouraged by the experience of several other cases of PAH treated successfully with an AFR device in our institution. Based on the pathophysiology of severe PAH with substantial reduction of transpulmonary blood flow and thereby reduction of cardiac output, the AFR device is beneficial in cases with overt right heart failure (RA pressure always > LA pressure) as well as in those with compensated right heart function and syncope. Adequate device sizing is crucial.

In conclusion, interventional implantation of an AFR device contributes to successfully bridging patients for lung transplantation by maintaining cardiac output in decompensated right heart failure and pulmonary hypertensive crises.

Publication History

Article published online:
22 February 2024