Lung and heart-lung transplantation in pulmonary arterial hypertension

Abstract

BackgroundReal use of lung (LT) and heart-lung (HLT) transplantation in pulmonary arterial hypertension (PAH) is unknown. The objectives were to describe the indication of these procedures on PAH treatment in a national cohort of PAH patients, and to analyze the potential improvement of its indication in severe patients.MethodsEligibility for LT/HLT was assessed for each deceased patient. Incident patients from REHAP diagnosed between January 2007 and March 2015 and considered eligible for LT/HLT were grouped as follows: those who finally underwent transplantation (LTP) and those who died (D-Non-LT).FindingsOf 1391 patients included in REHAP, 36 (3%) were LTP and 375 (27%) died. Among those who died, 36 (3%) were D-Non-LT. LTP and D-Non-LT were equal in terms of age, gender, and clinical status. Ten percent of those who died were functional class I-II. Patients functional class IV were less likely to undergo LT (8.3% LTP vs. 30.6% D-Non-LT, p = 0.017). Patients with idiopathic and drug/toxin-associated PAH were more likely to undergo LT (44.4% LTP vs. 16.7% D-Non-LT, p = 0.011).ConclusionsThe present results show that the use of LT/HLT could double for this indication. Relevant mortality in early functional class reflects the difficulties in establishing the risk of death in PAH.

Management of Idiopathic Pulmonary Arterial Hypertension in a Patient in Trinidad: A Case Report

“…Oxygen therapy would be added if SpO2 <90% [ 31 ]. Judicious use of diuretics is crucial to reduce volume overload without consequent hypotension (due to reduced cardiac output as the right heart is preload dependent), arrhythmias (due to hypokalemia), and metabolic alkalosis [ 23 , 25 , 27 , 29 ]. As such, renal function and blood biochemistry were regularly monitored when bumetanide and metolazone were added to our patient’s regimen, and she was treated with supplemental potassium as necessary.…”

“…As such, renal function and blood biochemistry were regularly monitored when bumetanide and metolazone were added to our patient’s regimen, and she was treated with supplemental potassium as necessary. Studies have shown that acutely administered inotropic drugs, such as digoxin, have improved cardiac output in IPAH; however, its long-term effects remain unknown [ 25 , 27 , 30 ]. The role of anticoagulation in IPAH is controversial as the evidence in favor was derived from small, retrospective, and single-center studies [ 25 , 26 ].…”

“…Nonetheless, in light of our patient's high BMI and sedentary lifestyle, as well as the theoretical risk for intrapulmonary thrombosis in an already compromised pulmonary vascular bed, she was treated with apixaban. In contrast, the literature recommends warfarin [23,[25][26][27][28].…”

“…As there were no protocol-mandated PAH treatments in the past, conventional or supportive therapy such as anticoagulants, diuretics, and cardiac glycosides were prescribed as necessary, all of which were included in our patient's treatment regimen [4,8,9,[23][24][25][26][27][28][29][30]. Oxygen therapy would be added if SpO2 <90% [31].…”

Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

“…There is growing evidence to support the use of PAH therapies in PAH associated with CHD (PAH-CHD) population, but despite recent advances, mortality remains high. Patients with severe PAH with an inadequate response to PAH therapy should be considered for transplantation [4]. We present the case of a patient with severe PAH and a large secundum atrial septal defect (ASD), who was referred to transplantation due to inadequate response to PAH therapy.…”