A tale of two cases of pulmonary arteriovenous malformation: How they fared after cardiac transplantation.

BACKGROUND: In single ventricle patients, aortopulmonary collaterals (APCs) and pulmonary arteriovenous malformations (PAVMs) following superior cavopulmonary shunt (CPS) can complicate orthotopic heart transplant (OHT) by cyanosis and hemoptysis. Although PAVMs 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 PAVMs after CPS were cyanotic following OHT. One patient with predominantly unilateral left PAVMs 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 PAVMs regressed, the flow restrictor stent was dilated to eliminate the constriction. The second patient with PAVMs had cyanosis and severe hemoptysis from APCs post-OHT. The APCs required an extensive coil embolization, while the cyanosis responded to oxygen and pulmonary vasodilators. Both recipients did well with gradual resolution of PAVMs 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 APCs or PAVM following cardiac transplantation.

Long-term outcome and impact of surgery on adults with coronary arteries originating from the opposite coronary cusp.

BACKGROUND: An anomalous coronary artery from the opposite sinus of Valsalva may increase sudden death risk in children and young adults, and surgical intervention is often recommended. The impact of this lesion when recognized in the adult and its management are ill defined. METHODS AND RESULTS: We reviewed 210 700 cardiac catheterizations performed over a 35-year period at a single institution and identified 301 adults with an anomalous coronary artery from the opposite sinus of Valsalva, either anomalous right coronary artery from the left cusp or anomalous left main coronary artery from the right cusp. Patients were stratified by the pathway of the anomalous artery and the chosen treatment. Among the 301 patients with anomalous coronary artery from the opposite sinus of Valsalva (0.14% of the cohort), 79% had anomalous right coronary artery from the left cusp, and 18% had an interarterial course (IAC). Patients with IAC were younger (52±13 versus 59±13 years; P=0.001) and more likely to undergo surgical intervention (52% versus 27%; P<0.001), but mortality was not increased with IAC. Among the 54 patients with IAC, 28 underwent surgical repair with no perioperative deaths. Patients evaluated since 2000 were significantly more likely to be referred for surgery (P=0.004). Surgical patients were more likely to have abnormal stress tests (90% versus 43%; P=0.01) and had more extensive atherosclerosis but less diabetes mellitus (0% versus 23%; P=0.01). Long-term survival at 10 years appeared similar in both groups. CONCLUSIONS: In this single-center cohort study of patients with an anomalous coronary artery from the opposite sinus of Valsalva, surgical management appears to have been favored recently. Despite no perioperative mortality, a positive impact on long-term survival was not observed. The impact of surgery in older adults with anomalous coronary arteries arising from the opposite coronary sinus with IAC deserves further study.