scholarly journals Vicious circle between progressive right ventricular dilatation and pulmonary regurgitation in patients after tetralogy of Fallot repair? Right heart enlargement promotes flow reversal in the left pulmonary artery

Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew N. Redington ◽  
Lars Grosse-Wortmann
Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew Redington ◽  
Lars Grosse-Wortmann

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.


2015 ◽  
Vol 16 (suppl 2) ◽  
pp. S56-S58 ◽  
Author(s):  
R Cordina ◽  
H Bellsham-Revell ◽  
J Melero Ferrer ◽  
Valverde Perez ◽  
R Pietrzak ◽  
...  

2015 ◽  
Vol 17 (S1) ◽  
Author(s):  
Israel Valverde ◽  
Annalisa Paolino ◽  
Maria Pilar Serrano Gotarredona ◽  
Silvia Navarro ◽  
Nieves Romero ◽  
...  

2018 ◽  
Vol 39 (4) ◽  
pp. 731-742 ◽  
Author(s):  
Miyuki Shibata ◽  
Keiichi Itatani ◽  
Taiyu Hayashi ◽  
Takashi Honda ◽  
Atsushi Kitagawa ◽  
...  

2014 ◽  
Vol 3 ◽  
pp. 28-31 ◽  
Author(s):  
Shamus O’Meagher ◽  
Madhusudan Ganigara ◽  
David J. Tanous ◽  
David S. Celermajer ◽  
Rajesh Puranik

2013 ◽  
Vol 24 (2) ◽  
pp. 369-373 ◽  
Author(s):  
Nikolaus A. Haas ◽  
Thorsten K. Laser ◽  
Axel Moysich ◽  
Ute Blanz ◽  
Eugen Sandica

AbstractThere is ongoing debate regarding the initial management of symptomatic neonates with tetralogy of Fallot. Although neonatal repair can be performed with low mortality, it is associated with increased morbidity and long-term impact on right ventricular performance. Traditionally, the modified Blalock–Taussig shunt remains the palliative procedure of choice. Differential pulmonary artery flow may occur and subsequently result in underdevelopment and distortion of pulmonary vessels. Transcatheter therapy was previously limited to balloon valvulotomy when the obstruction is predominantly at the pulmonary valve level. Stenting of the right ventricular outflow tract can enable adequate forward flow; however, pulmonary regurgitation may impact on right ventricular performance and cardiac output. Stenting of the right ventricular outflow tract with valve sparing placement of the stent thus treating the underlying pathophysiology of the hypercyanotic spells provides a safe and effective management strategy, improving arterial oxygen saturation, avoiding pulmonary regurgitation and encouraging pulmonary artery growth.


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