Pulmonary arterial hypertension in patients with prior atrial switch procedure for d-transposition of great arteries (dTGA)

Late Onset Postcapillary Pulmonary Hypertension in Patients With Transposition of the Great Arteries and Mustard or Senning Baffles

“…This likely reflects greater resilience of a mitral valve, which is normally subjected to systemic pressures, to leak when exposed to an increase in afterload. Although it could be argued that indirect echocardiographic signs may be useful metrics in assessing PH, we found no differences in ventricular chamber dimensions or function in patients with and without PH. Thus, echocardiographic signs seem to appear later in the evolution of PH in patients with D‐TGA and Mustard or Senning baffles because of the resiliency of the subpulmonary morphologic left ventricle and its mitral valve.…”

“…Although PH was postcapillary in all patients, a precapillary component (PVR >3 Wood units) was identified in 2 subjects. Earlier studies with shorter follow‐up periods reported a prevalence of PH ranging from 2.6% to 7% . Ebenroth et al described 4 (7%) patients with idiopathic PH, and although PCWP values were not available, indexed PVRs were high .…”

“…Ebenroth et al described 4 (7%) patients with idiopathic PH, and although PCWP values were not available, indexed PVRs were high . Yehra et al detected PH in 6 of 103 (5.6%) patients, mean age 29 years, by echocardiographic screening . Three had postcapillary PH.…”

“…Pulmonary hypertension (PH) has been described in patients with D‐TGA and atrial switch surgery and has been associated with impaired functional capacity and quality of life . Precapillary PH was first observed early after atrial switch surgery, with more recent reports describing late‐onset PH . The prevalence of late‐onset PH is poorly defined, with studies suggesting rates between 2% and 7%, an age at diagnosis ranging between 14 and 34 years, and a predominance of precapillary PH with high pulmonary vascular resistance (PVR).…”

Late-onset pulmonary arterial hypertension after successful early arterial switch surgery for simple transposition of the great arteries

“…Pre-operative systemic to pulmonary artery shunts, delayed reconstructive Mustard or Senning procedures, with mildly elevated base-line pulmonary pressures at early postoperative catheterisation, increase the risk of developing pulmonary arterial hypertension. (3)(4)(5)(6)(7)(8) Reversible conditions like pulmonary venous baffle obstruction, and branch pulmonary artery stenosis, must be addressed. (7)(8)(9) Late-onset pulmonary arterial hypertension after neonatal arterial switch surgery for simple d-transposition without remaining defects is however of right ventricular systolic pressures with magnetic resonance using the left ventricular septal-to-free wall curvature ratio according to Dellegrottaglie, et al estimated this to be 108mmHg (Figure 2b).…”