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

Abstract

BackgroundThere is a paucity of data regarding late‐onset pulmonary hypertension (PH) in patients with transposition of the great arteries and atrial switch surgery.Methods and ResultsA retrospective cohort study was conducted on 140 adults with transposition of the great arteries and atrial switch surgery, age 37.3±7.8, 37.1% female, in order to assess the prevalence and characteristics of late‐onset PH and explore associated factors. Patients were followed for a median of 32.3 years after atrial switch surgery and 10.0 years after their first referral visit. PH was detected in 18 of 33 (54.5%) patients who had invasive hemodynamic studies. Average age at diagnosis of PH was 33.9±8.1 years. PH was postcapillary in all, with a mean pulmonary artery pressure of 36±12 mm Hg and mean pulmonary capillary wedge pressure of 28±8 mm Hg. PH was diagnosed in 13 of 17 (76.5%) patients who had cardiac catheterization for heart failure or decreased exercise tolerance. In multivariable analyses, systemic hypertension (odds ratio 9.4, 95% confidence interval 2.2‐39.4, P=0.002) and heart failure or New York Heart Association class III or IV symptoms (odds ratio 49.8, 95% confidence interval 8.6‐289.0, P<0.001) were independently associated with PH. Patients with PH were more likely to develop cardiovascular comorbidities including atrial (P=0.001) and ventricular (P=0.008) arrhythmias, require hospitalizations for heart failure (P<0.001), and undergo tricuspid valve surgery (P<0.001). Mortality was significantly higher in patients with PH (hazard ratio 9.4, 95% confidence interval 2.1‐43.0], P<0.001).ConclusionsLate‐onset postcapillary PH is highly prevalent in adults with transposition of the great arteries and atrial switch surgery and is associated with an adverse prognosis.

Differential myocardial fibrosis of the systemic right ventricle and subpulmonary left ventricle after atrial switch operation for complete transposition of the great arteries

“…While the cause of worse subpulmonary LV fibrotic remodeling in our patients is not immediately obvious, chronic volume unloading of the eccentrically compressed left ventricle related to septal shift and impaired LV diastolic function as shown previously by our group [30] may be contributive. Furthermore, the potential role of the recently described highly prevalent postcapillary pulmonary hypertension on subpulmonary LV adaptation and fibrosis in these patients warrants further evaluation [31] .…”

Pulmonary arterial hypertension in adults with systemic right ventricles referred for cardiac transplantation

“…Pulmonary vascular disease is a well‐documented phenomenon found in 20% of lung biopsy specimens in infants with D‐TGA, especially those with shunts at the ventricular or great artery level . Late‐onset PAH has been reported in 2.6%‐7% of patients with D‐TGA status post‐atrial switch operation (Mustard or Senning procedure) whereas post‐capillary PH is highly prevalent, appearing in 54.5% of these adults . Distinct from post‐capillary PH due to systemic ventricular dysfunction, tricuspid regurgitation, or pulmonary venous baffle obstruction, the mechanism of late‐onset PAH is not well‐understood but is thought to be related to altered hemodynamics in the fetal circulation and pulmonary vascular bed.…”

“…17 Late-onset PAH has been reported in 2.6%-7% of patients with D-TGA status post-atrial switch operation (Mustard or Senning procedure) [18][19][20] whereas post-capillary PH is highly prevalent, appearing in 54.5% of these adults. 21 Distinct from post-capillary PH due to systemic ventricular dysfunction, tricuspid regurgitation, or pulmonary venous baffle obstruction, the mechanism of late-onset PAH is not well-understood but is thought to be related to altered hemodynamics in the fetal circulation and pulmonary vascular bed. This, coupled with history of pulmonary overcirculation, older age at surgery, as well as pulmonary venous hypertension from the failing RV, may predispose patients to adverse vascular remodeling and PH later in life.…”

Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries

“…Progressive tricuspid regurgitation occurs in the context of RV failure. Finally more recently late occurrence of pulmonary arterial hypertension has been recognized as an important long-term problem in patients after the atrial switch (30). Monitoring LV systolic pressures is essential part of the follow-up.…”