S Ram Kumar1, Grace Kung2, Nathan Noh2, Novel Castillo2, Brian Fagan2, Winfield J Wells2, Vaughn A Starnes2. 1. From Heart Institute, Children's Hospital Los Angeles, CA (S.R.K., G.K., N.N., N.C., B.F., W.J.W., V.A.S.); and Departments of Surgery (S.R.K., W.J.W., V.A.S.) and Pediatrics (S.R.K., G.K.), Keck School of Medicine of the University of Southern California, Los Angeles. rsubramanyan@chla.usc.edu. 2. From Heart Institute, Children's Hospital Los Angeles, CA (S.R.K., G.K., N.N., N.C., B.F., W.J.W., V.A.S.); and Departments of Surgery (S.R.K., W.J.W., V.A.S.) and Pediatrics (S.R.K., G.K.), Keck School of Medicine of the University of Southern California, Los Angeles.
Abstract
BACKGROUND: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. METHODS: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected. RESULTS: Twenty-seven patients (13, 48% boys) underwent the Starnes procedure at 7 (5-9) days of life. All were intubated and on prostaglandin, 24 (89%) were inotrope dependent, and 22 (81%) had no antegrade flow from the right ventricle. Three patients underwent nonfenestrated right ventricular exclusion, 2 (67%) of whom died. Of the remaining 24, 3 (13%) died during the same hospitalization. The 22 neonatal survivors have been followed for 7 (6-8) years: 1 patient is awaiting a Glenn procedure; 1 died after undergoing a Glenn procedure; and the remaining 20 patients have successfully undergone Fontan completion. Their indexed pulmonary vascular resistance was 1.8 (1.2-2.3) W/m2, and mean pulmonary pressure was 12 (9-18) mm Hg. At last follow-up, 1 patient had died, and the remaining patients had normal left ventricular function, and all but 1 have New York Heart Association class I symptoms. Two patients have required pacemaker implantation, whereas the rest are in sinus rhythm. Survival for the entire cohort at 1, 5, and 10 years is 81±4%, 81±5%, and 76±3%, respectively, whereas for those with fenestrated right ventricular exclusion, survival at 1, 5, and 10 years is 87±2%, 87±2%, and 81±4%, respectively. CONCLUSIONS: Long-term single-ventricle outcomes among neonatal survivors of the modified Starnes procedure are excellent. There is reliable remodeling of the excluded right ventricle and good function of the left ventricle.
BACKGROUND: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. METHODS: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected. RESULTS: Twenty-seven patients (13, 48% boys) underwent the Starnes procedure at 7 (5-9) days of life. All were intubated and on prostaglandin, 24 (89%) were inotrope dependent, and 22 (81%) had no antegrade flow from the right ventricle. Three patients underwent nonfenestrated right ventricular exclusion, 2 (67%) of whom died. Of the remaining 24, 3 (13%) died during the same hospitalization. The 22 neonatal survivors have been followed for 7 (6-8) years: 1 patient is awaiting a Glenn procedure; 1 died after undergoing a Glenn procedure; and the remaining 20 patients have successfully undergone Fontan completion. Their indexed pulmonary vascular resistance was 1.8 (1.2-2.3) W/m2, and mean pulmonary pressure was 12 (9-18) mm Hg. At last follow-up, 1 patient had died, and the remaining patients had normal left ventricular function, and all but 1 have New York Heart Association class I symptoms. Two patients have required pacemaker implantation, whereas the rest are in sinus rhythm. Survival for the entire cohort at 1, 5, and 10 years is 81±4%, 81±5%, and 76±3%, respectively, whereas for those with fenestrated right ventricular exclusion, survival at 1, 5, and 10 years is 87±2%, 87±2%, and 81±4%, respectively. CONCLUSIONS: Long-term single-ventricle outcomes among neonatal survivors of the modified Starnes procedure are excellent. There is reliable remodeling of the excluded right ventricle and good function of the left ventricle.
Authors: Lindsay R Freud; Doff B McElhinney; Brian T Kalish; Maria C Escobar-Diaz; Rukmini Komarlu; Michael D Puchalski; Edgar T Jaeggi; Anita L Szwast; Grace Freire; Stéphanie M Levasseur; Ann Kavanaugh-McHugh; Erik C Michelfelder; Anita J Moon-Grady; Mary T Donofrio; Lisa W Howley; Elif Seda Selamet Tierney; Bettina F Cuneo; Shaine A Morris; Jay D Pruetz; Mary E van der Velde; John P Kovalchin; Catherine M Ikemba; Margaret M Vernon; Cyrus Samai; Gary M Satou; Nina L Gotteiner; Colin K Phoon; Norman H Silverman; Wayne Tworetzky Journal: J Am Heart Assoc Date: 2020-10-20 Impact factor: 5.501