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A tale of two cases of pulmonary arteriovenous malformation: How they fared after cardiac transplantation
Author(s) -
Wisotzkey Bethany L.,
Magyar Dari L.,
Jones Thomas K.,
Boucek Robert J.,
Permut Lester C.,
Kemna Mariska S.,
Law Yuk M.
Publication year - 2018
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/ctr.13183
Subject(s) - medicine , pulmonary artery , cardiology , hypoxemia , ventricle , right pulmonary artery , shunt (medical) , fontan procedure , left pulmonary artery , surgery
Background In single ventricle patients, aortopulmonary collaterals ( APC s) and pulmonary arteriovenous malformations ( PAVM s) following superior cavopulmonary shunt ( CPS ) can complicate orthotopic heart transplant ( OHT ) by cyanosis and hemoptysis. Although PAVM s can regress with the restoration of hepatic venous flow to the pulmonary circulation, the effects of hypoxemia on the “unconditioned” allograft are not known. Cases Two patients with significant PAVM s after CPS were cyanotic following OHT . One patient with predominantly unilateral left PAVM s had arterial saturation levels less than 70% despite pulmonary vasodilators and ventilation. A custom flow restrictor‐covered stent was deployed in the pulmonary artery of the affected side, redirecting the blood flow to the contralateral lung, immediately improving cyanosis. When the PAVM s regressed, the flow restrictor stent was dilated to eliminate the constriction. The second patient with PAVM s had cyanosis and severe hemoptysis from APC s post‐ OHT . The APC s required an extensive coil embolization, while the cyanosis responded to oxygen and pulmonary vasodilators. Both recipients did well with gradual resolution of PAVM s within 8 months. Conclusions Despite cyanosis from right‐to‐left intrapulmonary shunting, allograft function recovered. Novel transcatheter interventions can play a role in patients with significant APC s or PAVM following cardiac transplantation.